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537 Cards in this Set

  • Front
  • Back

what does APGAR stand for

appearance


pulse


grimace


activity


respiration

newborn care (x5)

vit K IM


prophylactic eye erythromycin


umbilical cord care


hearing test


newborn screening tests

skull fractures: linear vs depressed

linear -- most common due to pressure or foreceps; no tx and no symptoms




depressed -- elevate to prevent cortical injury

spinal regions:


erb-duchenne


klumpke

erb = C5-6 -- waiters tip




klumpke = C7-8 + T1 -- paralyzed hand and Horner's syndrome

caput succedaneum

diffuse edematous swelling of soft tissues of scalp; crosses suture lines



disappears in first few days of life; may lead to molding for weeks

cephalohematoma

subperiostal hemorrhage -- does not cross suture lines



may have underlying linear fracture


resolves in 2wk - 3mo; may calcify; jaundice

cutis marmorata

lacy, reticulated vascular pattern over most of body when baby is cooled


improves over first month

lacy, reticulated vascular pattern over most of body when baby is cooled




improves over first month



milia

firm, whie papules; inclusion cyst
on palate midline: ebstein pearls


spontaneous resolution

firm, whie papules; inclusion cyst


on palate midline: ebstein pearls




spontaneous resolution

salmon patch (nevus simplex)

pale, pink vascular macules


found in nuchal area, glabella, eyelids
usually disappears

pale, pink vascular macules




found in nuchal area, glabella, eyelids


usually disappears

mongolian spots

blue to slate-gray macules
seen on presacral, back, post thighs


> in nonwhite infants
arrested melanocytes
usually fade over first few years


ddx = CHILD ABUSE

blue to slate-gray macules


seen on presacral, back, post thighs




> in nonwhite infants


arrested melanocytes


usually fade over first few years




ddx = CHILD ABUSE

erythema toxicum neonatorum

firm, yellow-white papules/pustules with erythematous base


peaks on second day of life
contain eosinophils
benign

firm, yellow-white papules/pustules with erythematous base




peaks on second day of life


contain eosinophils


benign

hemangioma: superficial and deeper

superficial = bright red, protuberant, sharply demarcated; first 2mo of life
-face
-scalp
-back
-anterior chest


deeper = bluish hue, firm, cystic, less likely to regress (tx = steroids and pulsed laser)

superficial = bright red, protuberant, sharply demarcated; first 2mo of life


-face


-scalp


-back


-anterior chest




deeper = bluish hue, firm, cystic, less likely to regress (tx = steroids and pulsed laser)



neonatal acne

erythematous papules on face


high maternal androgens


no tx

preauricular tags/pits

look for hearing loss and genitourinary anomalies

look for hearing loss and genitourinary anomalies

coloboma of iris -- definition

cleft at "6 oclock"
CHARGE association

cleft at "6 oclock"


CHARGE association

CHARGE

leading cause of deaf/blindness


Coloboma of the eye
Heart defects
Atresia of the nasal choanae
Retardation of growth and/or dev
Genital and/or urinary abnormalities
Ear abnormalities and deafness.

leading cause of deaf/blindness




Coloboma of the eye


Heart defects


Atresia of the nasal choanae


Retardation of growth and/or dev


Genital and/or urinary abnormalities


Ear abnormalities and deafness.

aniridia

associated with Wilms tumor (WAGR)

associated with Wilms tumor (WAGR)

syndactyly

fusion of fingers/toes

polydactyly

>5 fingers/toes




no tx if good blood supply

finger tag

thin stalk, poor circulation




ties off at base -> autoamputation

PKU:


1. defect


2. presentation


3. associations


4. tx

1. phenylalanine hydroxylase (accumulation of PHE in body and CNS)


2. retard, vomit, growth retard, athetosis, seizures


3. fair hair, fair skin, blue eyes, normal baby at birth then gradual decline


4. low PHE for life

athetosis

a condition in which abnormal muscle contractions cause involuntary writhing movements. It affects some people with cerebral palsy, impairing speech and use of the hands.

classic galactosemia


1. defect


2. presentation


3. assoc


4. tx

1. gal-1-P uridylyltransferase def -- accum of gal-1-p with injury to kidney, liver, brain


2. jaundice (direct), heptomegaly, vomit, hypoglycemia, cataracts, retard, seizure


3. e.coli sepsis predisposition


4. no lactose (but never reverses neurodevelopmental problems

lanugo

fine, soft hair, especially that which covers the body and limbs of a human fetus or newborn.

fine, soft hair, especially that which covers the body and limbs of a human fetus or newborn.

low birth weight vs large birth weight (grams)

<2500 grams


>4500 grams

plethoric

ruddy -- polycythemia (diabetic mother)

diabetic mother -- congenital anomalies

VSD


ASD


transposition


small left colon syndrome


caudal regression syndrome



macrosomia


tachypnea


cardiomegaly (asymmetric)


septal hypertrophy


polycythemia


renal vein thrombosis


hypoglycemia

initial halmark of RDS?

hypoxemia




then.... hypercarbia and respiratory acidosis

RDS chest radiograph

ground glass appearance


atelectasis


air bronchograms

most likely to get transient tachypnea of the newborn

c-section


rapid second stage of labor

meconium aspiration:


1. xray


2. prevention


3. tx


4. other complications

1. patchy infiltrates, inc AP diameter, flattening of diaphragm


2. endotrachial intubation and airway suction of depressed infants with thick meconium


3. positive pressure ventilation


4. air leak (pneumothorax, pneumomediastinum)

necrotizing enterocolitis:


Define


Symp

transmural intestinal necrosis


symp with intro to feeding:


-bloody stools


-apnea


-lethargy


-abdominal distention once perforation occurs



***pneumatosis intestinalis on abd film

pneumatosis intestinalis

air in bowel wall

VACTERL

Vertebral anomalies


Anal atresia


Cardiac defects


Tracheoesophageal fistula and/or


Esophageal atresia


Renal & Radial anomalies


Limb defects




assoc with imperforate anus

kernicterus

unconjugated bilirubin in the basal ganglia and brain stem nuclei




hypotonia


seizures


delayed motor skills


choreoathetosis


SENSORINEURAL HEARING LOSS

choreoathetosis

occurrence of involuntary movements in a combination of chorea (irregular migrating contractions) and athetosis (twisting and writhing).

complications of phototherapy in infants

loose stools


erythematous macular rash


overheating -> dehydration


bronze baby (photo-induced change in porphyrins)



most common organisms of neonatal sepsis

group B strep


e. coli


listeria

tx of neonatal sepsis (w/ and w/o meningitis)

w/ = ampicillin and 3rd ceph (not ceftriaxone)




w/o = ampicillin and aminoglycoside until 48-72hr cultures are negative

symp of toxoplasmosis

jaundice/heptaosplenomegaly


thrombocytopenia/anemia


microcephaly


chorioretinitis


hydrocephalus


intracranial calcifications


seizures

congenital rubella symp

blueberry muffin spots


PDA


cataracts


congenital hearing loss


thrombocytopenia


hepatosplenomegaly

CMV symp in neonate

hepatosplenomegaly/jaundice


periventricular calcifications


IUGR


deaf


retard


microcephaly


thrombocytopenia


chorioretinitis

congenital syphilis symptoms


1. birth-2yrs


2. >2yrs

1. snuffles, maculopapular rash (palms/soles), jaundice, periostitis, osteochondritis, chorioretinits, congenital nephrosis




2. hutchinson teeth, clutton joints, saber shins, saddle nose, osteochondritis, rhagades

rhagades

assoc with syphilis
thickening and fissures of corners of mouth

assoc with syphilis


thickening and fissures of corners of mouth

clutton joints

assoc with syphilis


symmetrical swelling of joints

neonatal features in opiate abuse

inc low birth weight


inc stillborns


no inc in congenital abnormalities


early withdrawal symp (w/in 48hr)


tremors/hyperirritability


inc SIDS




diarrhea, apnea, poor feeding, high pitched cry, weak suck, weight loss, tachypnea, hyperacusis, seizures

neonatal features of cocaine abuse

no withdrawal symp


preterm labor, abruption, asphyxia


IUGR


impaired auditory processing


developmental delay


learning disabilities


CNS ischemic and hemorrhagic lesions


vasoconstriction -> other malformations

most common cardiac anomaly in trisomy 21

ECD = endocardial cushion defect

brushfield spots

speckling of iris
assoc with down's syndrome

speckling of iris


assoc with down's syndrome

trisomy 18 (survival rate and symp)

death by 1yr




retard


low-set ears


clenched hand (overlap)


short sternum


VSD, ASD, PDA


rocker-bottom feet


hammer toe


omphalocele

WAGR

wilms


aniridia


GU anomalies


retard

cubitus valgus

turner assoc

turner assoc

symp of fragile x

retard
large ears
large jaw
long face
large testes

retard


large ears


large jaw


long face


large testes

beckwith-wiedemann syndrome

IGF-2 disrupted


macrosomia
macroglossia (big tongue)
pancreatic beta cell hyperplasia -> hypoglycemia
omphalocele
hemihypertrophy -> inc Wilms risk


check AFP every 6mo for Wilms

IGF-2 disrupted




macrosomia


macroglossia (big tongue)


pancreatic beta cell hyperplasia -> hypoglycemia


omphalocele


hemihypertrophy -> inc Wilms risk




check AFP every 6mo for Wilms

angelgman syndrome -- communication deficiency

no speech or <6 words



use sign language

pierre-robin sequence

mandibular hypoplasia --> cleft palate


micrognathia
retroglossia (possible airway obstruction)
cleft palate

mandibular hypoplasia --> cleft palate




micrognathia


retroglossia (possible airway obstruction)


cleft palate

micrognathia



arachnodactyly

marfan syndrome

marfan syndrome



ehlers-danlos

type 1 is most common (6 total)
droopy ears
stretchy skin
joint hyperlaxity
blue sclera
ectopic lentis

type 1 is most common (6 total)


droopy ears


stretchy skin


joint hyperlaxity


blue sclera


ectopic lentis

ectopic lentis

marfan and ehlers-danlos

marfan and ehlers-danlos

cardiac anomalies in fetal alcohol syndrome

VSD > ASD


tetralogy of fallot

fetal hydantoin sydrome

growth deficiency
mild retardation
short neck
abnormal palmar crease
hirsutism
cupid's bow lips

growth deficiency


mild retardation


short neck


abnormal palmar crease


hirsutism


cupid's bow lips

fetal valproate syndrome

midface hypoplasia/cleft lip
cardiac defects
long thin fingers and toes
convex nails
meningomyelocele

midface hypoplasia/cleft lip


cardiac defects


long thin fingers and toes


convex nails


meningomyelocele

retinoic acid embryopathy (isotretinoin)

bilateral microtia/anotia (no ears)
facial nerve paralysis ipsilateral to ear
conotruncal malformations
CNS malformations
dec intelligence
thymic/parathyroid abnormalities

NO PROBLEMS IF STOPPED BY 15TH POSTMENSTRUAL DAY

bilateral microtia/anotia (no ears)


facial nerve paralysis ipsilateral to ear


conotruncal malformations


CNS malformations


dec intelligence


thymic/parathyroid abnormalities




NO PROBLEMS IF STOPPED BY 15TH POSTMENSTRUAL DAY

weight loss in first week of life

normal = up to 10% in first week of life due to elimination of extravascular fluid




regain birth weight by 2wks

infant weight gain and growth (until teen)

double at 6mo


triple at 1 yr




(myelination complete at 7yrs)


6-12yr = 3-6 growth spurts each year for 8wk periods each


10-20yr = acceleration


boy -- stop at 18yrs (peak at 13.5)


girls -- stop at 16yrs (peak at 11.5)

CI in breastfeeding

absolute:


antineoplastics


radiopharmaceuticals


ergot alkaloids


iodide/mercurials


atropine


lithium


chloramphenicol


cyclosporine


nicotine


alcohol




relative CI:


neuroleptics


sedatives


tranquilizers


metronidazole


tetracycline


sulfa drugs


steroids

side effect of early introduction of cow's milk (<1yr)

Fe deficiency

how many calories in breast vs cow milk

same -- 20 cal/oz

how to assess development in premature infants?

6mo born at 32 wks:




that is 2mo preterm so infant should be assessed as if a 4mo infant until the age of 2.

predisposing factors of pica

mental retardation and lack of parental nuturing




family disorganization


poor supervision


psychologic neglect




more common in...


-autism


-brain-behavior disorders


-low socio-economic status



inc risks in pica

lead poisoning


iron deficiency


parasitic infections

uveitis

third leading cause of blindness in kids


infection or HLA-B27


herpes/varicella




tx = steroids and methotrexate is aggressive




complications = cataracts, glaucoma, band keratopathy,macular edema and permanent vision loss

posterior uveitis

Floaters
Blurred vision
Photopsia or seeing flashing lights

Floaters


Blurred vision


Photopsia or seeing flashing lights

anterior uveitis

Redness of the eye
Blurred vision
Photophobia or sensitivity to light
Irregular pupil
Eye pain
Floaters
Headaches


Synechia = iris adheres to either the cornea

Redness of the eye


Blurred vision


Photophobia or sensitivity to light


Irregular pupil


Eye pain


Floaters


Headaches


Synechia = iris adheres to either the cornea

pharmacological method for nocturnal enuresis if behavioral therapy failed

imipramine (TCA)

define secondary enuresis

>6mo of a dry period before voluntary/involuntary peeing




girls




cause = psychological, UTI, constipation, diabetes

encopresis

passage of feces at inappropriate places




cause = psychological (toilet phobia), early toilet training, aggressive management of constipation, painful defecation, fissures

sleepwalking/sleep terrors

first third of night


during slow-wave sleep


no daytime sleepiness of recall


high arousal threshold when awoken


common family hx




tx = parental education, reassurance, safety

nightmares

last third of night


REM sleep


daytime sleepiness and vivid recall


easily awakened


no family hx




tx = none unless persistent (anxiety or abuse)

at what age is HiB stopped being given

>5yrs

bucket handle fracture

wrenching or pulling (child abuse)

wrenching or pulling (child abuse)

spiral fracture

child abuse!!!
twisting arm/leg

child abuse!!!


twisting arm/leg

immersion burns: abusive hot bath vs accidental

abuse:


demarcation is uniform


flexion creases spared (vs falling in)


no splash burns (held down)


hands and feet spared (baby raises them)



most common sexual abuse in pediatrics

brother-sister incest




also common to be father/stepfather

tx of croup

nebulized epinephrine followed by corticosteroids

epiglossitis

toxic-appearing
sniffing-position
difficulty swallowing
drooling

tx = intubate then abx

toxic-appearing


sniffing-position


difficulty swallowing


drooling




tx = intubate then abx

laryngomalacia

congenital
most freq cause of stridor in infants/kids
starts first 2wks of life

surgery = supraglottoplasty

congenital


most freq cause of stridor in infants/kids


starts first 2wks of life




surgery = supraglottoplasty

congenital subglottic stenosis

recurrent croup and stridor
tx = cricoid split or reconstruction

recurrent croup and stridor


tx = cricoid split or reconstruction

associations with vocal cord paralysis

meningomyelocele


chiari malformation


hydrocephalus

bronchiolitis:


symp


duration


tc

symp = mild URI, apnea, wheezing, work to breath


duration = 12 days (worst in first 2-3 days)


tx = supportive, no steroids

most likely organism of pneumonia:


<5yrs


>5yrs

< = viral (RSV)


> = bacterial (M., C., and S. pneumonia)

viral pneumonia symptoms


low grade fever


tachypnea


scattered crackles and wheezing

bacterial pneumonia symptoms


--sudden shaking chills with HIGH fever


--acute onset


--sign cough and chest pain


--splinting on affected side


--markedly diminished breath sounds and dullness to percussion

chlamydia trachomatis pneumonia symptoms


no fever or wheezing


1-3mo of age


w/ or w/o conjunctivitis


staccato cough


peripheral eosinophilia

chlamydia pneumonia and mycoplasma pneumonia symptoms


atypical


bronchopneumonia


gradual onset


persistent cough/hoarseness


dx and WBC of pneumonia


1. viral


2. pneumococcal


3. mycoplasma


4. chlamydia


1. hyperinflation with bilateral interstitial infiltrates and peribronchial cuffing -- <20k with lymphocyte dominance


2. confluent lobar consolidation -- 15-40k with neutrophil dominance


3. unilateral or bilateral lower-lobe interstitial pneumonia; looks worse than presentation -- 15-40k with neutrophil dominance


4. interstitial pneumonia or lobar; looks worse then presentation -- eosinophilia

genetic info of CF and pathogenesis


Aut rec


chromo 7


CFTR - transmembrane regulator


unable to secrete Cl -> cant clear mucus


inc salt in sweat

first presentation of CF


10% = meconium ileus (obstruction) -- dilated loops, air filled, ground glass in lower central abdomen


malabsorption of pancreatic exocrine insufficiency (freq bulky, greasy stools and failure to thrive)


ADEK deficiency


rectal prolapse


acute pancreatitis


inc DM

CF: determination of mortality and morbidity

rate of progression of lung disease

sweat test levels for CF

>60 mEq/L

xray results of CF


hyperinflation of chest


nodular densities, patchy atelectasis, confluent infiltrates, hilar nodea


w/progression = flattening of diaphragm, sternal bowing, narrow cardiac shadow, cysts, extensive bronchiectasis

SIDS


peak = 2-4mo; most by 6mo


males>females


smoking


pacifier DECREASES risk

allergic shiners and Dennie lines

allergic rhinitis

allergic rhinitis

physical examination of allergic rhinitis

allergic shiners
dennie lines
chemosis (edema) -- picture
transverse nasal crease
turbinate hypertrophy
pale nasal mucosa
postnasal drip
OME


allergic shiners


dennie lines


chemosis (edema) -- picture


transverse nasal crease


turbinate hypertrophy


pale nasal mucosa


postnasal drip


OME

ddx of allergic rhinitis (x5)


nonallergic inflammatory rhinitis (no IgE Ab)


vasomotor rhinitis (physical stimuli)


nasal polyps (CF)


septal deviation


overuse of topical vasoconstrictors

ddx of eosinophilia


neoplasm


allergy


asthma


Addison


parasite


collagen vascular disorder

desensitization -- immunotherapy should NOT be used in the following...


atopic dermatitis


food allergy


latex allergy


urticarial


kids <3

how to remove bee stinger

scraping

urticarial -- define

HIVES

in food allergies, is it more likely hypertension of hypotension

hypotension

hereditary angioedema


aut dom


c1 esterase-inhibitor deficiency


recurrent episodes of nonpitting edema

most common anaphylaxis and tx


peanuts


injectable epinephrine + oral liquid diphenhydramine


O2 and airway mangagement

atopic dermatitis (eczema)


half start by 1 year


PRURITIS


worse at night


scratching -> lesions


atopic dermatitis (eczema) skin patterns:


1. acute


2. subacute


3. chronic


1. erythematous papules, intensely pruritic, serous exudate, excoriation (open wound/ulcer)
2.erythematous, excoriated, scaling papules
3. lichenification (picture)


1. erythematous papules, intensely pruritic, serous exudate, excoriation (open wound/ulcer)


2.erythematous, excoriated, scaling papules


3. lichenification (picture)


distribution of eczema


1. infant


2. older


1. face, scalp, extensor


2. flexor


safe on face for eczema tx?


1. topical corticosteroids


2. tacromilus (what's MOA?)


1. no


2. yes -- calcineurin inhibitor

most common complication of eczema

Kaposi varicelliform eruption

Kaposi varicelliform eruption

normal onset of asthma in pediatrics


<6yrs


triggered by viral infections

risk factors for persistent asthma


perennial allergies


atopic dermatitis


allergic rhinitis


food allergy


LRI (ie pneumonia)


wheezing (exercise/emotions)


low birth weight


smoke exposure

duration of albuterol

4-6hr


symptom = recurrent infections


what can u exclude in following:


1. normal lymphocyte count


2. normal neutrophil count


3. normal platelet count


4. normal ESR


1. not T-cell


2. not congenital/acquired neutropenia and not leukocyte adhesion deficiency


3. no Wiskott-Aldrich


4. not chronic bacterial or fungal

what is recurrent if have phagocytic deficiency

recurrent staph and gr neg infections

bruton agammaglobinemia


--x-linked


--fine until 6-9mo


--pyogenic infections, hepatic viruses, enteroviruses


--lymphoid hypoplasia


--dec in all Ig and absent B cells

peritonsillar abscess

symptoms are 2-8d before visualization

uvula displaced

    Streptococcus, Staphylococcus and Haemophilus 


clindamycin or metronidazole in combination with benzylpenicillin (penicillin G)  

symptoms are 2-8d before visualization




uvula displaced




Streptococcus, Staphylococcus and Haemophilus




clindamycin or metronidazole in combination with benzylpenicillin (penicillin G)

common variable immune deficiency

hypoglammaglobinemia with phenotypically normal B cells




bact. infections plus enteroviral meningitis




normal or even increased lymphoid tissue




inc in lymphomas




males and females

IgA deficiency

most common


males and females


resp, GI, urogenital, skin infections


inc autoimmune diseases and malignancies




FATAL ANAPHYLACTIC RXNS IF GIVEN BLOOD WITH IgA

DiGeorge

third and fourth pharyngeal pouches -- 22q11.2


thymic hypoplasia


parathyroid hypoplasia -> hypocalcemia seizure


right aortic arch


ASD/VSD


esophageal atresia


short philtrum


hypertelorism


mandibular hypoplasia


fish mouth




low absolute lymphocyte count


inc fungi, virus, p.carinii infections


GVHD




rx = transplantation of thymic tissue of MHC compatible sibling or half-matched parental bone marrow

9p minus syndrome



Trigonocephaly (keel-shaped forehead caused by premature fusing of metopic suture) or minor ridge on forehead



  Choanal atresia (nasal passage narrow or blocked by tissue) 



Globalized hypotonia (low muscle tone)/Delayed gross moto...

Trigonocephaly (keel-shaped forehead caused by premature fusing of metopic suture) or minor ridge on forehead




Choanal atresia (nasal passage narrow or blocked by tissue)




Globalized hypotonia (low muscle tone)/Delayed gross motor development




Seizures


GERD


constipation


cardiac defects


scoliosis


kidney reflux


mental retardation

SCID

opportunistic and viral infections


diarrhea


pneumonia/AOM/sepsis/cut infections


GVHD from maternal incompetent T cells crossing placenta


severe lymphopenia


small or no thymus


no immunoglobins




x-linked (most common)


aut rec = ADA deficiency




rx = bone marrow transplant or death by 1 yr



wiskott-aldrich syndrome

x-linked rec




eczema


thrombocytopenia


recurrent infection


palpable adenopathy; splenomegaly




1. prolonged bleeding in circumcision




rare survival beyond teen if no bone marrow transplant

ataxia telangiectasia

mod dec in B and T cell 
dec CD3/CD4

hypoplastic thymus

mask-like facies
tics
drooling
irregular eye movement

cerebellar ataxia
telangectasias

high risk of malignancies - lymphoreticular and adenoCA

mod dec in B and T cell


dec CD3/CD4




hypoplastic thymus




mask-like facies


tics


drooling


irregular eye movement




cerebellar ataxia


telangectasias




high risk of malignancies - lymphoreticular and adenoCA

chronic granulomatous disease

x-linked and aut rec


catalase-positive microorganisms




lymphadenitis, pneumonia, skin infections


osteomyelitis




tx = bone marrow transplant

catalase positive bugs (x6)

s. aureus


norcardia


s. marcescens


b. cepacia


aspergillus


c.albicans

coloboma of iris

aut dom
ALWAYS INFERIOR

key appearance of iris; in lid, manifests as cleft

CHARGE association

aut dom


ALWAYS INFERIOR




key appearance of iris; in lid, manifests as cleft




CHARGE association

leucokoria

retinoblastoma
cataract

retinal detachment
retinopathy of prematurity

larval granulomatosis

retinoblastoma


cataract




retinal detachment


retinopathy of prematurity




larval granulomatosis

causes of cataracts (x6)

prematurity


aut dom


TORCH, measles, polio, flu, varicella, vaccina


galactosemia


chromosomal


steroids, contusions, penetrations (trauma)

ectopia lentis ddx

trauma = #1
uveitis
congenital glaucoma
cataract

systemic =
1. marfan
2. homocystinuria
3. ehlers

trauma = #1


uveitis


congenital glaucoma


cataract




systemic =


1. marfan


2. homocystinuria


3. ehlers

strabismus

misalignment due to muscles

dx = hirschberg corneal light reflex (should be symmetric and slightly nasal to center of pupil)

PATCH good eye to eliminate amblyopia then eye muscle surgery

misalignment due to muscles




dx = hirschberg corneal light reflex (should be symmetric and slightly nasal to center of pupil)




PATCH good eye to eliminate amblyopia then eye muscle surgery

pseudostrabismus

epicanthal folds and broad nasal bridge

caused by unique facial characteristics of infant

epicanthal folds and broad nasal bridge




caused by unique facial characteristics of infant

ophthalmia neonatorum

first 24hr of life

gonorrhea = 2-5d incubation

trachomatis = 5-14d incubation

first 24hr of life




gonorrhea = 2-5d incubation




trachomatis = 5-14d incubation

bacterial conjunctivitis

mucopurulent exudate


hyperemia


edema


eye discomfort




s. pneumonia


h. influenza


s. aureus




rx = warm compress and topical antibiotics

viral conjunctivitis

watery discharge, bilateral, usually with URI


adenovirus, enterovirus


good hand-washing




epidemic keratoconjunctivitis

epidemic keratoconjunctivits

adenovirus type 8

adenovirus type 8

keratitis

infection of cornea

h. simplex
adenovirus
s. pneumoniae
s. aureus
pseudomonas
chemicals

infection of cornea



h. simplex


adenovirus


s. pneumoniae


s. aureus


pseudomonas


chemicals

corneal abrasion

caused by foreign body

pain and photophobia

anesthetize the flourescein and blue-filtered light (woods lamp)

tx = pain relief and abx

caused by foreign body




pain and photophobia




anesthetize the flourescein and blue-filtered light (woods lamp)




tx = pain relief and abx

retinopathy of prematurity

-prematurity
-hyperoxia
-general illness

progressive vasoproliferative scarring and blinding retinal detachment

tx = cryosurgery or laser photocoagulation

-prematurity


-hyperoxia


-general illness




progressive vasoproliferative scarring and blinding retinal detachment




tx = cryosurgery or laser photocoagulation

retinoblastoma

most common primary malignant intraocular tumor




dx by 15 mo for bilat and 25 mo for unilat




initial sign = leucokoria


--- appears as white mass


--- strabismus




never biopsy cuz spreads easily

periorbital cellulitis

trauma
infected wound
abscess of lid
sinusitis
bacteremia

tx = oral or IV abx

inflammation of lids and periorbital tissue with NO ORBITAL INVOLVEMENT (normal vision)

trauma


infected wound


abscess of lid


sinusitis


bacteremia




tx = oral or IV abx




inflammation of lids and periorbital tissue with NO ORBITAL INVOLVEMENT (normal vision)

orbital cellulits -- symp, causes and tx

ophthalmoplegia (eyeball doesnt move)
chemosis (edema of conjunctiva)
inflammation
proptosis (exophthalmus)

paranasal sinusitis, direct infection frm woud, bacteremia

bugs = nontypable h. influ, s.aureus, beta hemolytic strep, s. pneumoniae

tx...

ophthalmoplegia (eyeball doesnt move)


chemosis (edema of conjunctiva)


inflammation


proptosis (exophthalmus)




paranasal sinusitis, direct infection frm woud, bacteremia




bugs = nontypable h. influ, s.aureus, beta hemolytic strep, s. pneumoniae




tx = IV abx and drainage

otitis externa


1. bugs


2. symp


3. malignant external otitis


4. tx and prevention

swimmer's ear


1. pseudomonas, s. aureus, staph, strep viridans


2. sign pain esp when move ear, conductive hearing loss, edema, erythema, thick otorrhea


3. invasive to temporal bone and skull base -> facial paralysis/vertigo --- needs surgery!


4. cortico and topical otic prep; ear plugs, drying of canal, 2% acetic acid after getting wet

otorrhea

ear discharge

otitis media

s. pneumo, h. influ, moraxella catarrhalis




mobility of TM = NOT OME




amoxicillin, NSAID/acetaminophen


azithromycin if allergy




>2yr, no fever or severe pain the observe 2-3d




cont pain = augmentin, cefuroxime, IV ceftriaxone, cefdinir

OME

monthly evaluation


>3mo --> send to ENT


no abx




tubes = bilateral and impaired hearing for >3mo

OME complications (x2)

acute mastoiditis -- displacement of pinna inferiorly and anteriorly inflammation of posterior auricular area; pain on percussion of mastoid




cholesteatoma -- cyst within middle ear/temporal bone lined with keratinizing strat sq epithelium

choanal atresia -- symp

unilat = asymp until first URI then persistent nasal discharge

bilat = cyanosis while trying to breathe through nose, then becoming pink with crying

CHARGE assoc

unilat = asymp until first URI then persistent nasal discharge




bilat = cyanosis while trying to breathe through nose, then becoming pink with crying




CHARGE assoc

clinical symp of foreign body in nose

unilateral purulent, malodorous bloody discharge

epistaxis - location and causes

anterior septum (most common -- keisselbach plexus)




digital trauma


dry air (esp winter)


allergy


inflammation (URI)


nasal steroid sprays


severe GERDin infants


clotting disorder


congential vascular anomalies

tx epistaxis

1. compress, upright, head forward


2. local oxymetazolone or phenylephrine


3. anterior nasal packing then maybe posterior


4. cautery




humidifier, saline drops, petrolatum for prevention

nasal polyps

most common = CF (<12yr -- first symp of CF)




may cause obstruction




glistening gray grape-like masses




tx = intranasal steroids/systemic steroids may provide some shrinkage (good in CF); may need surgery

samter triad

nasal polyps


ASA sensitivity


asthma

sinusitis bugs

s. pneumo


nontypable h. influ


m. catarrhalis


s. aureus in chronic cases

sinusitis symp and tx

congestion, discharge, fever (102 for 3d), cough, persistent URI without improvement for at least 10d




amoxicillin


alt = cefuroxime, cefpodoxime, azythromycin


7d treatment

acute pharyngitis presentation

strep = rapid onset, severe sore throat, fever, headache, GI symp, red pharynx with exudate, red swollen uvula, lymphadenopathy




scarlet fever = pharyngitis plus circumoral pallor, sandpaper rash, pastia's lines




viral = more gradual with URI symp, no pus


--coxsackie = hand-foot-mouth disease

pastia's lines

scarlet fever

scarlet fever

circumoral pallor

scarlet fever

scarlet fever

tx of strep pharyngitis

penicillin or erythromycin if allergis




early tx hastens recovery by 12-24hr and prevents acute RF if treated within 9d of illness

complications of strep throat

retropharyngeal and lateral pharyngeal abcess


-- drainage and 3rd ceph + amp/sul or clind




pertonsillar abcess


--needle aspiration, incision, drainage, tonsillectomy is recurrence

torticollis

congenital or retropharyngeal abscess (with muffled voice)

congenital or retropharyngeal abscess (with muffled voice)

tonsillectomy indications

>7 documented infections within past year


5/yr for 2 years


3/yr for 3 years

adenoidectomy indications

chronic nasal/sinus infection
recurrent/chronic OM in kids with tubes
persistent otorrhea
nasal obstruction with chronic mouth-breathing and snoring

chronic nasal/sinus infection


recurrent/chronic OM in kids with tubes


persistent otorrhea


nasal obstruction with chronic mouth-breathing and snoring



tonsillectomy + adenoidectomy indication

>7 infections


upper airway obstruction secondary to hypertrophy resulting in sleep-disordered breathing and complications

when to evaluate cardiac fxn in kids:


1. infant symp


2. child symp

1. feeding difficulty


easily fatigued


sweating while feeding


rapid respirations




2. SOB


dyspnea on exertion

heart murmur grading


1


2


3


4


5


6



1 - soft, difficult to hear


2 - easily heard


3 - louder but no thrill


4 - thrill


5 - thrill, audible with edge of stethoscope


6 - thrill, audible with stethoscope off chest

cardiac thrill

a vibration felt by the examiner on palpation

innocent murmur

30% children ages 3-7


easily heard during fever, infection, anxiety


never more than 2/6




reassurance

VSD

most common


when PVR>SVR --> Eisenmenger


harsh holosystolic murmur


dyspnea


feeding difficulties


poor growth


sweating


pulm infection and heart failure




surgery in first yr; small ones will close in first 1-2yrs of life

VSD complications (x3)

large defects lead to heart failure; failure to thrive




endocartitis




pulm HTN

ASD

most common = ostium secundum




few symp early in life


wide fixed splitting of S2




systolic ejection murmur along left mid to upper sternal border




ECG = right-axis deviation and RVH




tx = most symp dont appear till third decade


surgery if severe

ASD complications (x2)

dysrhythmia




low-flow lesion; does not require endocarditis prophylaxis

endocardial cushion defect

down's


ASD and VSD plus abnormal atrioventricular valves




wide fixxed split S2


pulm systolic ejection murmur, low-pitched diastolic rumble at left sternal border and apex





endocardial cushion defect:


surgery vs no surgery complciations

surgery = arrhythmias, congenital hear block




w/o surgery = death from heat failure

PDA

more common in girls


assoc = maternal rubella infection


common in premature infants




machine murmur


LV hypertrophy




tx = indomethacin, surgical closure

PDA complications (x2)

CHF


infective endocarditis

pulmonic stenosis

RV hypertrophy
first month of life

RV failure signs = hepatomegaly, edema, exercise intolerance

tx = balloon valvuloplasty then maybe surgery

RV hypertrophy


first month of life




RV failure signs = hepatomegaly, edema, exercise intolerance




tx = balloon valvuloplasty then maybe surgery

pulmonic stenosis complications (x3)

heart failure




endocarditis (lower risk)




secondary subvalvular muscular and fibrous hypertrophy

aortic stenosis

bicuspid aortic valve -- usually asymp


if severe... LV failure and dec CO






usually not discovered unless routine physical examination -- most dont know they have it




inc severity = dec pulses, inc heart size, LV apical thrust




early systolic ejection click at apex of left sternal border




tx = balloon valvuloplasty, surgery on valves, valve replacement

names of adult vs infantile coarctation of aorta

adult = discrete juxtaductal coarctation




infantile = tubular hypoplasia (preductal)

adult coarctation of aorta

discrete juxtaductal coarctation




narrowed at descending aorta


LV hypertrophy and HTN


delay in femoral pulse compared to radial




rib notching (dilated intercostal art)




tx = medicate for HTN and heart failure then follow with surgery

infantile coarctation of aorta

tubular hypoplasia




preductal (asc aorta)


PDA stays open


upper body is pink and lower is cyanotic




symp = lower body hypoperfusion, acidosis, severe heart failure with ductal closure, large heart, systolic murmur along left sternal border




tx = PGE1 then surgery

complications of coarctation of aorta (x4)

associated cerebrovascular disease


systemic HTN


endocarditis


aortic aneurysms

tetralogy of fallot

pulm stenosis


VSD


overriding aorta


RV hypertrophy




most common cyanotic lesion


squatting = inc vascular resistance and dec R-L shunt




either single S2 or soft pulmonic component

define tet spells (tetralogy)

paroxysmal hypercyanotic attacks




acute onset of hyperpnea and restlessness -> in cyanosis -> gasping -> syncope




syncope = inc infundibular obstruction with further R-L shunting




tx = place in lateral knee-chest position, give O2, inject subcut morphine, give b-blockers

tetralogy of fallot complications before surgery takes place (x4)

none of there are common cuz usually corrected surgically early in life




cerebral thromboses


brain abscess


bacterial endocarditis


heart failure

tricuspid atresia

no outlet from RA to RV
must have ASD to survive
pulmonary blood flow depends on size of VSD

severe cyanosis and inc LV impulse
holosystolic murmurs cuz of VSD

xray = pulmonary undercirculation

no outlet from RA to RV


must have ASD to survive


pulmonary blood flow depends on size of VSD




severe cyanosis and inc LV impulse


holosystolic murmurs cuz of VSD




xray = pulmonary undercirculation

ebstein anomaly

lithium
huge RA and tricuspid regurg

inc RA vol shunts blood through foramen ovale or ASD -> cyanosis

may have no symp till teen/adult
holosystolic murmur

tx = PGE, shunt, surgery

lithium


huge RA and tricuspid regurg




inc RA vol shunts blood through foramen ovale or ASD -> cyanosis




may have no symp till teen/adult


holosystolic murmur




tx = PGE, shunt, surgery

transposition of great arteries

PDA must stay open to survive
S2 usually single and LOUD, murmurs absent
"egg on a string" cxr

tx = PGE, balloon atrial septostomy, arterial switch surgery in first 2wks of life

PDA must stay open to survive


S2 usually single and LOUD, murmurs absent


"egg on a string" cxr




tx = PGE, balloon atrial septostomy, arterial switch surgery in first 2wks of life

truncus arteriosus

single arterial trunk

truncus overlies a VSD (ALWAYS PRESENT) and receives blood from both ventricles (total mixing)

SEM with loud thrill, single S2 and minimal cyanosis

inc pulm blood flow -> rapid heart failure

ECG = biventricular hypertrop...

single arterial trunk




truncus overlies a VSD (ALWAYS PRESENT) and receives blood from both ventricles (total mixing)




SEM with loud thrill, single S2 and minimal cyanosis




inc pulm blood flow -> rapid heart failure




ECG = biventricular hypertrophy




tx = tx heart failure and then surgery

TAPVR

total anomalous pulmonary venous return

TOTAL MIXING

enlarged: RA, RV, Pulmonary Artery

small: LA, LV

tx = PGE, surgery

total anomalous pulmonary venous return




TOTAL MIXING




enlarged: RA, RV, Pulmonary Artery




small: LA, LV




tx = PGE, surgery

TAPVR cxr

"snowman" appearance
pulm vascularity inc

"snowman" appearance


pulm vascularity inc



TAPVR ECG

right axis deviation
RVH
tall spiked P waves

right axis deviation


RVH


tall spiked P waves

hypoplastic heart syndrome

ATRESIA: MV or AV, LV, Asc Aorta

open ASD or foramen ovale

PDA supplies desc aorta

ECG = RVH and RAE with dec left-sided forces

tx = maybe nothing
or three-stage norwood procedure

ATRESIA: MV or AV, LV, Asc Aorta




open ASD or foramen ovale




PDA supplies desc aorta




ECG = RVH and RAE with dec left-sided forces




tx = maybe nothing


or three-stage norwood procedure

RAE -- define (cardiac

right atrial enlargement

describe 3-stage norwood procedure used in hypoplastic heart syndrome

mitral insufficiency

dilated annulus


abnormal chordea tendineae


deformed leaflets




high pitched holosystolic murmur at apex




ECG = bifid P waves, LVH


CXR = inc LA size, prominent LV




tx = mitral valvuloplasty

mitral valve prolapse

billowing of one or both leaflets into LA towards end of systole




girls>boys




apical late systolic murmur preceded by a click




normal CXR and ECG!


no tx.... not progressive

tricuspid insufficiency

associated with ebstein anomaly




RV dysfunction -- dilatation with enlargement of annulus (vol overload or intrinsic myocardial disease)




perinatal asphyxia in neonate (ischemia of papillary muscles)

infective endocarditis bugs

strep vir. and s. aureus are most common




vir. = dental


group D strep = large bowel surgery


pseudo. and serratia = IV drug users


fungi = open heart surgery


coag neg staph = IV catheters

infective endocarditis symp

prolonged intermittent fever


weight loss


new/changing heart murmur


osler nodes


janeway lesions


splinter hemorrhage


roth spots (retinal)

osler nodes

tender, pea sized intradermal nodules on pads of fingers and toes

tender, pea sized intradermal nodules on pads of fingers and toes

janeway lesions

painless, small erythematous or hemorrhagic lesions on palms and soles

painless, small erythematous or hemorrhagic lesions on palms and soles

HACEK

hemophilus


actinobacilus


actinomycetemcomitans


cardiobacterium hominus


eikenella corrodens


kingella kingae




slow-growing gr neg organisms that are part of normal flora

acute rheumatic fever -- bug? tx?

group a strep




most common form of acquired heart disease worldwide




tx = oral penicillin


ASA for arthritis (prednisone if have CHF hx)



JONES (rheumatic fever)




5 major criteria


5 minor criteria

Major:


carditis


polyarthritis (migratory)


erythema marginatum


chorea


subcutaneous nodules




Minor:


fever


arthralgia


elevated ESR and CRP


prolonged PR interval on ECG


plus evidence of preceding strep infection

HOCM:


define


symp


dx


tx

hypertrophic obstructive cardiomyopathy


SUDDEN DEATH OF ATHLETIC TEEN


obstructive left-sided congenital heart disease




palp, angina, dizzy, syncope, risk of sudden death




ECG = LVH + ST dep and T inversion


CXR = cardiomegaly




tx = no sports or hard exercise


b-blockers (propanolol) and CCB (verapamil)




DIGOXIN AND DIURESIS = CI



pericarditis:


define


most common cause


symp


dx

inflammation --> accum of fluid in pericardial space

viral is most common cause

pericordial pain!!
sharp, stabbing over heart, L shoulder, & back
relief when lean forward
friction rub is variable
cough, vomit, dyspnea, abd pain

ECG = low-volta...

inflammation --> accum of fluid in pericardial space




viral is most common cause




pericordial pain!!


sharp, stabbing over heart, L shoulder, & back


relief when lean forward


friction rub is variable


cough, vomit, dyspnea, abd pain




ECG = low-voltage QRS


CXR = water bottle appearance



what do u think of when its pediatric HTN?

RENAL CAUSES!!


UTI


glomerulonephritis


henoch-schonlein with nephritis


HUS


ATN


renal trauma


renal artery stenosis

secondary pediatric HTN: most common


1. newborn


2. early childhood


3. adolescent

1. umbilical artery catheters -> renal art thomb


2. renal disease, coarctation, endocrine, meds


3. essential HTN

Cleft lip and palate tx

Lip - surgery at 3mo


Palate - surgery at <1yr

Cleft lip and palate tx

Lip - surgery at 3mo


Palate - surgery at <1yr

Blood diarrhea. 5 common bugs (CASES)

Campylobacter


Amoeba (e. Historlytica)


Shigella


E. Coli


Salmonella

Most common viral acute diarrhea

Norovirus



Rotavirus


Enteric adenovirus


Astrovirus


Calcivirus

Most common parasitic acute diarrhea

Giardia

Should anti diarrheal med be used in pediatrics? Like loperamide?

Never!

Rotavirus


1. Assoc


2. Therapy

1. Watery diarrhea, vomiting, maybe fever



2. Supportive

Entamoeba histolytica


1. Assoc


2. Tx

1. Acute bloody diarrhea


2. Metronidazole

Giardia


1. Assoc


2. Tx

1. Anorexia, nausea, abd distension, watery diarrhea, weight loss


2. Metro or furazolidone

Cryptosporidium


1. Assoc


2. Tx

1. Mild diarrhea in immunocompromised infants, severe diarrhea in AIDS pts


2. Best tx is raise CD4 count


Supportive, may try rifabutin

Schwachman-diamond

Pancreatic insufficiency


Neutropenia


Malabsorption

Intestinal lymphangiectasia

Lymph fluid leaks into bowel lumen



Steatorrhea



Protein-losing enteropathy

Disaccardase deficiency

Osmotic diarrhea


Acidic stools

Abetalipoproteinemia

Severe fat malabsortion from birth



Acanthocytrs



Very low to absent plasma chooesterol, triglycerides, etc

Most useful sceening test for fat in stool

Sudan red stain

Three basic types of Esoph atresia and tracheoesoph fistula



Symp

Isolated EA


Isolated (h type) TEF


EA and distal TEF



Frothing


Bubbling


Cough


Cyanosis


Resp distress


Immediate regurg with feeding


Aspiration

GERD



Resolves by when?


Symp?

Resolves by 12-24mo



Postprandial regurg


Esophagitis


Obstructive apnea


Lower airway disease (cough/wheezing?

Enteropathogenic e. Coli



1. Assoc


2. Tx

1. Nurses, daycare


2. Supportive, severe -- neomycin or colostin

Etiology of pyloric stenosis

Firstborn white males

Describe dehydration in pyloric stenosis

Hypochloremic


Hypokalemic


Met alk

Enter tocopherol e. Cold


1. Assoc


2. Tx

1. Travelers diarrhea


2. Supportive, tmp-smx if severe

Enterohemorrhagic e. Coli


1. Assoc


2. Tx

1. Hemorrhagic colitis, HUS


2. No antimicrobial therapy cuz inc HUS!! Supportive only

Salmonella


1. Assoc


2. Tx

1. Infected animals and contaminated eggs/milk/poultry


2. Tx only if

Shigella


1. Assoc


2. Tx

1. Person to person spread, contaminated food


2. Tmp-smx

Campylobacter


1. Assoc


2. Tx

Person to person spread


Contaminated food



Self limiting; erythromycin speeds recovery or if severe

Yersinia enterocolitica


1. Assoc


2. Tx

1. Pets, contaminated food, arthritis, rash


2. No abx


Clostridium difficile


1. Assoc


2. Tx

1. Hx of abx use


2. Metronidazole or vancomycin and discontinuation of abx

Staph aureus


1. Assoc


2. Tx

1. Food poisoning--onset within 12hr


2. Supportive

duodenal atresia

--down's
--bilious vomiting without abd distention on first day of life (obstruction just distal to ampulla)
--polyhydramnios
--jaundice (inc enterohepatic circulation)

--double bubble with no distal bowel gas

--tx = nasogastric decompression, ...

--down's


--bilious vomiting without abd distention on first day of life (obstruction just distal to ampulla)


--polyhydramnios


--jaundice (inc enterohepatic circulation)




--double bubble with no distal bowel gas




--tx = nasogastric decompression, IV fluids, duodenoduodenostomy

jejunal and ileal atresia


1. causes


2. symp

1. cigarette or cocaine use


2. polyhydramnios, abd distantion at birth or with first feeds + vomiting, may be bilious, few with delayed or no meconium, jaundice



meconium ileus

proximal bowel dilated and filled with thick meconium impacted in ileum

"doughy" cordlike masses

tx = hypertonic water-soluble contrast enema to attempt wash-out
then laparotomy if failed

proximal bowel dilated and filled with thick meconium impacted in ileum




"doughy" cordlike masses




tx = hypertonic water-soluble contrast enema to attempt wash-out


then laparotomy if failed

gold standard confirmation for hirschprung disease

the suction rectal biopsy

delay in treating volvulus can result in...

short bowel syndrome

etiology and presentation for malrotation/volvulus


etiology = incomplete rotation, ladd bands may extend from cecum to RUQ to produce duodenal obstruction


presentation = bilious emesis, recurrent abd pain and vomiting, acute small bowel obstruction in a pt without previous bowel surgery is suspicious of volvulus


meckel diverticulum:


disease of 2's


remnant of embryonic yolk sac, lining similar to stomach


most freq congenital anomaly


2y/o


2% of population


2 types of tissue (true)


2inch in size


2ft from ileocecal valve


tx = surgical excision

intussusception:


etiology


clinical presentation


physical examination


dx


tx


telescoping (ileal-colic) from 3mo-6yr (80% are <2yrs)
assoc with henoch-schonlein purpura
black currant jelly 
stool
sudden onset of severe colicky pain
legs flexed
progressive weakness
lethargy/shock with fever
vomiting
slightly tender, sausag...


telescoping (ileal-colic) from 3mo-6yr (80% are <2yrs)


assoc with henoch-schonlein purpura


black currant jelly


stool


sudden onset of severe colicky pain


legs flexed


progressive weakness


lethargy/shock with fever


vomiting


slightly tender, sausage-shaped mass on R in cephalocaudal axis


US (doughnut appearance)


tx = resection

functional constipation


delay or difficulty for at least 2wks (usually 2y/o)


voluntary withholding to avoid pain


tx = relief of impaction (enema, stool softeners)


diet, behavioral, bowel training program

hirschsprung disease


most common reason for bowel obstruction in neonates


absence of ganglion cells in bowel wall beginning at internal anal sphincter and extending variably proximally


delay in passage of meconium

most common UTI bugs


e. coli


klebsiella


proteus


s. saprophyticus

types of UTI (x3)


cystitis


pyelonephritis


asymptomatic bacteriuria

cystitis symp & tx


dysuria, urgency, freq, suprapubic pain, incontinence, no fever (unless very young)


amoxicillin, TMP/SMX, methoxazole, nitrofurantoin

pyelonephritis symp & tx


abd or flank pain, fever, malaise, nausea, vomiting, diarrhea, nonspecific in newborns and infants


start with oral abx unless hospitalization and IV fluids

FU for UTI


Do urine culture 1 week after stopping abx to confirm sterility; periodic reassessment for next 1-2yrs


US for anatomy, suspected abscess, hydronephrosis, recurrent UTI


Voiding cystourethrogram (VCUG) in recurrent UTI or UTI with complications or abnormal US findings

VUR - vesicoureteral reflux
1. predisposition to pyelonephritis
2. scarring
3. reflux nephrology (HTN, proteinuria, renal insuff to ESRD)
4. impaired kidney growth
dx = VCUG

1. predisposition to pyelonephritis


2. scarring


3. reflux nephrology (HTN, proteinuria, renal insuff to ESRD)


4. impaired kidney growth


dx = VCUG


obstructive uropathy:


1. define


2. symp


3. dx


4. tx

1. obstruction of urinary outflow tract


2. hydronephrosis, upper abd pain, pyelonephritis, UTi recurrent, weak urine stream, failure to thrive


3. palpable abd mass -- VCUG, US


4. decompress bladder with catheter, abx, transurethral ablation or vesicostomy

obstructive uropathy etiology


ureteropelvic junction obstruction


ectopic ureter


ureterocele


posterior urethral valves

obstructive uropathy complications


if lesion is svere, may present with pulmonary hypoplasia (Potter seq)


prognosis dependent on lesion severity and recovery of renal fxn

ectopic ureter

drains outside bladder; causes continual incontinence and UTI

drains outside bladder; causes continual incontinence and UTI

ureterocele

cystic dilatation with obstruction from a pinpoint ureteral orifice; mostly girls

cystic dilatation with obstruction from a pinpoint ureteral orifice; mostly girls

posterior urethral valves

--most common cause of severe obstructive uropathy
--boys
--can lead to ESRD
--mild hydronephrosis to severe renal dysplasia
--palpable distended bladder and weak urinary stream


--most common cause of severe obstructive uropathy


--boys


--can lead to ESRD


--mild hydronephrosis to severe renal dysplasia


--palpable distended bladder and weak urinary stream


acute poststreptococcal glomerulonephritis


pathophys

lumpy-bumpy deposits of Ig and complement on glomerular basement membrane and in mesangium


acute poststreptococcal glomerulonephritis


1. presentation


2. dx


3. tx

1. 5-12y/o; 1-2wks after strep pharyngitis or 3-6wks after skin infection (impetigo)


edema, htn, hematuria


2. RBD casts, proteinuria, low C3, anti-DNase antigen


3.abx (penicillin 10d); Na restriction; fluids; control htn




95% patients = complete recovery


acute poststreptococcal glomerulonephritis


complications


HTN


ARF


CHF


electrolyte abnormalities


acidosis


seizures


uremia

Berger disease

IgA nephropathy
most common chronic glomerular disease worldwide

URI and GI infections

normal C3
mild proteinuria and mild/mod HTN

HTN = most important thing to control

IgA nephropathy


most common chronic glomerular disease worldwide




URI and GI infections




normal C3


mild proteinuria and mild/mod HTN




HTN = most important thing to control

alport syndrome

x-linked
renal biopsy = foam cells -- accumulation of neutral fats and mucopolysaccharide

asymp hematuria (usually 1-2d after URI)

hearing deficit (bilat sesorineural, never congenital)

ocular abnorm (extrusion of central part of lens into an...

x-linked


renal biopsy = foam cells -- accumulation of neutral fats and mucopolysaccharide




asymp hematuria (usually 1-2d after URI)




hearing deficit (bilat sesorineural, never congenital)




ocular abnorm (extrusion of central part of lens into anterior chamber)

1. most common nephrotic syndrome in adults


2. most common chronic glomerulonephtritis in older kids/young adults

1. membranous glomerulopathy


2. membranoproliferatic glomerulonephritis

henoch-schonlein purpura

small vessel vasculitis
good prognosis
purpurie rash
joint pain
abdominal pain
IgA deposits in kindey

steriods or spont resolve

small vessel vasculitis


good prognosis


purpurie rash


joint pain


abdominal pain


IgA deposits in kindey




steriods or spont resolve

HUS: symp and bugs

hemolytic uremic syndrome

most common ARF in young kids
1. microangiopathic hemolytic anemia
2. thrombocytopenia
3. uremia

e. coli 0157:H7
shigella
salmonella
campylobacter

hemolytic uremic syndrome




most common ARF in young kids


1. microangiopathic hemolytic anemia


2. thrombocytopenia


3. uremia




e. coli 0157:H7


shigella


salmonella


campylobacter

HUS: pathophys

how many days after infection does HUS occur?


Lab results?

5-10d = sudden pallor, irritability, weakness, oliguria, mild renal insuff




helmet cells, burr cells, fragmented cells


platelet = 20k-100k


hematuria and proteinuria

burr cells



HUS tx

FLUIDS AND ELECTROLYTES


tx HTN


aggressive nutritition


early peritoneal dialysis


maybe plasmaphoresis/fresh frozen plasma




no abx if e. coli 0157:H7 is suspected (will inc risk of developing HUS)

PKD -- aut rec (infantile)

large kidneys


microcysts --> progressive interstitial fibrosis and tubular atrophy --> renal failure




liver disease




symp = potter's, HTN, oliguria, ARF, pulm hypoplasia (if severe)




tx = dialysis and transplant; 80% have 10yr survival

PKD -- aut dom (adult)

most common hereditary kidney disease




enlarged kidney with cortical and medullary cysts




40-50s




macrocysts = kidney, liver, pancreas, spleen, ovaries, Berry




tx = HTN control and transplant

types of proteinuria (x3)

1. transient = from fever, exercise, dehydration, cold exposure, CHF, seizure stress




2. orthostatic = slightly inc in supine position and greatly inc in upright (esp school aged kids); most common




3. fixed = glomerular/tubular disorders; >1g/24hr or with HTN, hematuria, or renal dysfunction

1. most common nephrotic syndrome in kids




2. features of nephrotic syndrome

1. steroid-sensitive minimal change disease




2. proteinuria (>40mg/m2/hr)


hypoalbuminemia (<2.5 g/dL)


edema


hyperlipidemia

minimal change disease

most common between 2-6y/o
may follow minor infections
edema (eyes and lower extremities)
diarrhea, abd pain, anorexia
uncommon = HTN, gross hematuria

proteinuria
albumin <2.5
Cr normal or inc
inc chol and TG
C3 and C3 normal

tx = prednisone
Na...

most common between 2-6y/o


may follow minor infections


edema (eyes and lower extremities)


diarrhea, abd pain, anorexia


uncommon = HTN, gross hematuria




proteinuria


albumin <2.5


Cr normal or inc


inc chol and TG


C3 and C3 normal




tx = prednisone


Na restriction




multiple relapses but gradually dec

minimal change disease complication

infection: vacc for pneumococcus and varicella




most freq is spontaneous bacterial peritonitis (s. pneumo)




inc risk of thromboembolism

undescended testes

more common in preterm


should be descended by 4mo


usually stuck in inguinal canal




untreated = in seminoma malignancy


treated = bilateral (50% pt are fertile)


unilateral (85% pt are fertile)

testicular torsion

most common cause of testicular pain over 12y/o




acute pain


swelling


tenderness on palpation




ER surgery -- >90% of testicles survive if within 6h and <360 degree rotation

torsion of appendix testes

most common cause of testicular pain 2-11 y/o

gradual onset
3-5mm, tender, inflamed mass at upper pole of testes

blue dot through scrotal skin

most common cause of testicular pain 2-11 y/o




gradual onset


3-5mm, tender, inflamed mass at upper pole of testes




blue dot through scrotal skin

epididymitis

ascending, retrograde urethral infection -> acute scrotal pain/swelling

rare before puberty

main cause of acute scrotal swelling in sexually active men

pyuria

tx = bedrest and abx

ascending, retrograde urethral infection -> acute scrotal pain/swelling




rare before puberty




main cause of acute scrotal swelling in sexually active men




pyuria




tx = bedrest and abx

pyuria

the presence of pus/WBC in the urine, typically from bacterial infection

varicocele

dilatation of pampiniform plexus (valvular incompetence of spermatic vein)

tx = surgery 

"bag of worms" and painless

dilatation of pampiniform plexus (valvular incompetence of spermatic vein)




tx = surgery




"bag of worms" and painless

testicular tumors

65% malignant


palpable, hand mass that doesn't transilluminate


usually painless


dx = US, AFP, b-HCG


tx = radical orchiectomy

most common acquired hypopituitarism

craniopharyngioma

craniopharyngioma

most common hyperpituitarism

hormone-secreting adenomas

define precocious puberty and most common etiologies for boys/girls

female = <8; sporadic and familial


male = <9; hamartomas

hamartoma

composed of tissue elements normally found at that site, but which are growing in a disorganized mass




benign, focal malformation that resembles a neoplasm in the tissue of its origin

define thelarche, adrenarche, menarche

thelarche = boobs




adrenarche = hormones (adrenal production) -- hair (genital, axillary, inguinal) and sexual features (hips)




menarche = period

defect in thryoid hormone synthesis

goitrous


aut rec

cretinism

prolonged jaundice
large tongue
umbilical hernia
edema
retard
ant/post fontanels wide
mouth open
hypotonia

prolonged jaundice


large tongue


umbilical hernia


edema


retard


ant/post fontanels wide


mouth open


hypotonia

most common acquired hypothyroidism

hashimoto (caused by thyroiditis)


usually teens

first sign of acquired hypothyroidism

deceleration of growth




then myxedema, constipation, cold intol, dec energy, inc sleep, delayed osseous maturation, delayed puberty, headache, visual problems

myxedema

swelling of the skin and underlying tissues giving a waxy consistency

swelling of the skin and underlying tissues giving a waxy consistency

most common hyperthyroidism; peak age; findings

graves

11-15yrs

1. infiltration of thyroid and retro-orbital tissue with lymphocytes and plasma cella -> exopthalmos

2. lymphadenopathy and splenomegaly

3. thymic hyperplasia

graves




11-15yrs




1. infiltration of thyroid and retro-orbital tissue with lymphocytes and plasma cella -> exopthalmos




2. lymphadenopathy and splenomegaly




3. thymic hyperplasia

Graves HLA assoc in white people

HLA-B8


HLA-DR3




DR3 = Addisons, DM, MG, Celiac

autoimmune polyglandular disease


1. type 1


2. type 2

1. hypoparathyroidism


addisons


mucocutaneous candidiiasis


small # with autoimmune thyroiditis




2. addison


DM


w/ or w/o thyroiditis

Addison's disease

hypo vs hyperthyroidism symptoms

tx for acute symptoms of hyperthyroidism in thyroid storm

beta blockers!!

thyroid storm



symptoms of hypoparathyroidism

muscle cramps/pain


numbness


tingling


laryngeal and carpopedal (hands) spasm


seizures (hypocalcemic seizure in newborn -- think DiGeorge)

EKG in hypoparathyroidism

prolonged QT

rachitic rosary

vitamin D deficiency
(also see bowing and fractures)

vitamin D deficiency


(also see bowing and fractures)

21-hydroxylase deficiency -- labs plus tx

aut rec


dec cortisol -> inc ACTH -> adrenal hyperplasia




inc adnrogens


dec aldosterone and cortisol


inc 17-OH progesterone


inc K, renin


acidosis


low Na, glucose




tx = hydrocortisone


fludrocortisone if salt losing


inc both doses in times of stress


correct sex organ in females





cushing's syndrome

adrenocortical tumor (malig) = infants

disease = >7yrs

dexametasone-suppression test

adrenocortical tumor (malig) = infants




disease = >7yrs




dexametasone-suppression test

moon face

cushing's

cushing's

3-beta-hydroxysteroid deficiency

salt wasting


pseudohermaphrodites


precocious pubarche


inc 17-OH pregnenolone and DHEA

adrenal pathway

11-beta-hydroxylase deficiency

female pseudohermaphroditism


postnatal virilization


HTN


inc androgens


hypokalemia

17-alpha hydroxyl deficiency

male pseudohermaphroditism


sexual infantilism


HTN


hypokalemia

diabetic ketoacidosis

hyperglycemia


ketonuria


inc anion gap


dec HCO3


dec pH


inc serum osmolarity

metabolic syndrome

DM


hyperlipidemia


HTN


acanthosis nigricans

developmental dysplasia of the hip (DDH)




barlow vs ortolani

barlow = dislocate an unstable hip (most important test) -- clunk sound

ortolani = fixes dislocated hip; not possible after 2mo cuz of soft-tissue contractions

barlow = dislocate an unstable hip (most important test) -- clunk sound




ortolani = fixes dislocated hip; not possible after 2mo cuz of soft-tissue contractions

another picture of barlow vs ortolani

pavilk harness

tx = duration for 1-2months for developmental dysplasia of the hip

tx = duration for 1-2months for developmental dysplasia of the hip

legg-calve-perthes disease -- define

idopathic avascular necrosis of the capital femoral epiphysis in immature, growing kid

males
20% bilateral

idopathic avascular necrosis of the capital femoral epiphysis in immature, growing kid




males


20% bilateral

legg-calve-perthes disease -- symp; tx

mild intermittent pain in anterior thigh with plainless lump with restriction of motion




tx = containment with orthoses or casting


bedrest


abduction stretching exercises


if sig femoral deformity ppersits, surgical correction

Slipped capital femoral epiphysis (SCFE)

most common teen hip disorder

obese or delayed skeletal maturation or thin with recent growth spurt

tx = pinning

most common teen hip disorder




obese or delayed skeletal maturation or thin with recent growth spurt




tx = pinning



transient synovitis

viral
7-14d after URI
3-8 y/o

acute mild pain with limp and mild restriction movement
pain in groin, ant thigh, and knee

tx = bedrest and no weight bearing (<1wk) then 1-2wk of limited activity

viral


7-14d after URI


3-8 y/o




acute mild pain with limp and mild restriction movement


pain in groin, ant thigh, and knee




tx = bedrest and no weight bearing (<1wk) then 1-2wk of limited activity

metatarsus adductus

most common in firstborn

tx = serial plaster casts before 8mo, orthoses, corrective shoes

most common in firstborn




tx = serial plaster casts before 8mo, orthoses, corrective shoes

orthosis

brace (knee, foot, arm, etc)

talipes equinovarus

clubfoot

correction should be done by 3mo; if not then surgery needed

clubfoot




correction should be done by 3mo; if not then surgery needed

internal femoral torsion (femoral anteversion)

most common cause of intoeing >2yrs
most secondary to abnormal sitting habits (W-sitting)

tx = observation; take 1-3yr to resolve; surgery only if significant at >10yrs

most common cause of intoeing >2yrs


most secondary to abnormal sitting habits (W-sitting)




tx = observation; take 1-3yr to resolve; surgery only if significant at >10yrs

W-sitting

internal tibial torsion

most common <2yr

no tx -- resolves in normal growth/development  (takes 6-12mo)

most common <2yr




no tx -- resolves in normal growth/development (takes 6-12mo)

osgood-schlatter disease

overuse injury (traction apophysitis of tibial tubercle)

active teen

swelling, tender, inc prominence of tubercle

tx = rest, restriction of activities, knee brace

resolution = 1-2yrs

overuse injury (traction apophysitis of tibial tubercle)




active teen




swelling, tender, inc prominence of tubercle




tx = rest, restriction of activities, knee brace




resolution = 1-2yrs

scoliosis

most idiopathic
>11yrs
Adams test bending forward at hips

tx = brace if <30-45 degrees and surgery if >45 degrees

most idiopathic


>11yrs


Adams test bending forward at hips




tx = brace if <30-45 degrees and surgery if >45 degrees

hemivertebra

rare
causes scoliosis

rare


causes scoliosis

nursemaid elbow

hx of sudden traction or pulling on arm
radial head subluxation

hx of sudden traction or pulling on arm


radial head subluxation

osteomyelitis bugs

s. aureus (most common)


pseudomonas (puncture wound)


salmonella (sickle cell)

septic arthritis bug

almost always s. aureus

definitive dx for osteomyelitis vs septic arthritis

osteo = bone biopsy for culture and sensitivity


septic = US guided arthrocentesis for culture and sensitivity

osteogenesis imperfecta

most common genetic osteoporosis


aut dom




1. fragile bones


2. blue sclera


3. early deafness




defect of type 1 collagen

osteogenic sarcoma


1. age


2. M vs F


3. cxr


4. malignant?


5. metastases


6. tx


7. prognosis

1. second decade
2. males
3. sunburst (sclerotic destruction)
4. yes
5. lung, bone
6. chemo and surgery
7. 70% cure w/o mets (<20% w/ mets)

1. second decade


2. males


3. sunburst (sclerotic destruction)


4. yes


5. lung, bone


6. chemo and surgery


7. 70% cure w/o mets (<20% w/ mets)

ewing sarcoma


1. age


2. M vs F


3. cxr


4. malignant?


5. metastases


6. tx


7. prognosis

1. second decade
2. Males
3. onion skin -- lytic with laminar periosteal elevation
4. yes
5. lungs, bone
6. radiation and surgery
7. 60% cure w/o mets (20-30% w/ mets)

1. second decade


2. Males


3. onion skin -- lytic with laminar periosteal elevation


4. yes


5. lungs, bone


6. radiation and surgery


7. 60% cure w/o mets (20-30% w/ mets)

osteoid osteoma


1. age


2. M vs F


3. cxr


4. malignant?


5. metastases


6. tx


7. prognosis

1. second decade
2. 3x greater in males
3. central lucency with sclerotic margin
4. no
5. n/a
6. NSAID and surgery only if painful
7. resolve spontaneously
8. n/a

1. second decade


2. 3x greater in males


3. central lucency with sclerotic margin


4. no


5. n/a


6. NSAID and surgery only if painful


7. resolve spontaneously


8. n/a

juvenile idiopathic arthritis (JIA)


1. define


2. pathophys


3. symp


4. age/duration

1. iopathic synovitis or peripheral joints assoc with soft-tissue swelling and joint effusion




2. vascular endothelial hyperplasia and progressive erosion of cartilage and bone; DR8 and DR5




3. morning stiffness; easy fatigability; joint swelling/warm with decreased motion; NO REDNESS




4. <16yr and duration >6wks

juvenile idiopathic arthritis


3 types of onset

1. pauciarticular (<5 joints) = lower extremity but never hip; HLA B27
2. polyarticular (>5) = both small and large joints plus rheumatoid nodules on extensor surfaces of elbows and achilles tendon

3. systemic onset = visceral (hepatosplenomegal...

1. pauciarticular (<5 joints) = lower extremity but never hip; HLA B27


2. polyarticular (>5) = both small and large joints plus rheumatoid nodules on extensor surfaces of elbows and achilles tendon




3. systemic onset = visceral (hepatosplenomegaly, lymphadenopathy, serositis, iridocyclitis); daily temp spikes (102) for >2wks; salmon-colored evanescent rash

salmon-colored evanescent rash

linear or circular
mostly trunk and proximal extremities
systemic juvenile idiopathic arthritis

linear or circular


mostly trunk and proximal extremities


systemic juvenile idiopathic arthritis

JIA labs and tx

labs = RF+; inc ANA in 40-85% pts; inc ESR; inc anemia of chronic disease




tx = NSAID; methotrexate; maybe corticosteroirs

SLE


labs


M or F


symp:


-general


-skin


-renal


-neuro


-pulm


-hemato


-serositis


-GI

anti-dsDNA


HLA: B8, DR2, DR3


drug induced = anticonv, sulf, antiarry


females <8yr


gen = fever, fatigue, arthralgia, arthritis, rash


skin = malar, discoid lesions, livedo reticularis


renal = glomerulonephritis, nephrotic, HTN


neuro = seizures, stroke


pulm = pleuritic pain, pulm hemorrhage


hemato = coomb +, thrombocytopenia


serositis = pleural, pericardial, hepatospleno


GI = vasculitis (pain, diarr, bleeding, hepatitis)



raynaud phenomenon

SLE tx

NSAID if no renal disease


methotrexate


DMARDs


steroid for kidney disease and acute disease


cyclophosphamide for severe disease

kawasaki disease

affects medium arteries

leading cause of acquired heart disease in USA and Japan

fever >5d no improved by NSAID or acetaminophen

possible early myocarditis or pericarditis

inc ESR
sterile pyuria

tx = IVIg and high dose ASA (no Reye's syndrome)

affects medium arteries




leading cause of acquired heart disease in USA and Japan




fever >5d no improved by NSAID or acetaminophen




possible early myocarditis or pericarditis




inc ESR


sterile pyuria




tx = IVIg and high dose ASA (no Reye's syndrome)

Reye's syndrome

henoch-schonlein purpura

IgA vasculitis of small vessels


after URI


rash = red -> purple -> rusty brown


intussesception


occult blood; diarrhea


arthritis


nephrosis


hepatospleno




inc platelets, WBC, ESR, IgA, IgM


anemia




maybe anticardiolipin or antiphospholipid Ab




corticosteroids and symp tx

henoch-schonlein purpura complicaions

renal insufficiency/failure


bowel perforation


scrotal edema


testicular torsion


chronic renal insufficiency (<1%)

iron-deficiency anemia

typically anemic at 9-24mo due to cows milk




pallor, irritability, lethargy, pagophagia, tachycardia, systolic murmurs, long-tern neurodevelopmental effects

iron-deficiency labs

1. dec hemosiderin (bone marrow)


2. dec ferritin


3. dec serum iron and transferrin saturdation


4. inc TIBC


5. inc FEP (free erythrocyte protoporphyrin)


6. microcytosis, hypochromia, poikilocytosis

review blood cell morphology

poikilocytosis

lead poisoning

>5 ug/dL
housing before 1960

symp = behavioral (hyperactivity; aggression)
--cognitive (impaired growth)
--GI (constipation, anorexia, pain, vomit)
--CNS (cerebral edema, ICP)
--gingival lead lines

>70 = immediate hospitalization (EDTA and dime...

>5 ug/dL


housing before 1960




symp = behavioral (hyperactivity; aggression)


--cognitive (impaired growth)


--GI (constipation, anorexia, pain, vomit)


--CNS (cerebral edema, ICP)


--gingival lead lines




>70 = immediate hospitalization (EDTA and dimercaprol)


45-70 = chelation


20-44 = repeat1month


15-19 = tell health dep


5-14 = repeat 3month

Blackfan-Diamond: define and symp

congenital pure red-cell anemia

inc RBC programmed cell death -> profound anemia by 2-6 months  

short stature
craniofacial deformities
defects of upper extremities
triphalangeal thumbs

congenital pure red-cell anemia




inc RBC programmed cell death -> profound anemia by 2-6 months




short stature


craniofacial deformities


defects of upper extremities


triphalangeal thumbs

Blackfan-Diamond: labs and tx

labs = macrocytosis; inc HbF; inc ADA; very low reticulocyte count; inc iron




tx = corticosteroids; transfusions and deferoxamine; splenectomy




definitive = stem cell transplant

congenital pancytopenia: symp, labs, tx

most common is fanconi anemia (spont chromosomal breaks)

symp = hyperpigmented; cafe-au-lait; absent/hypoplastic thumbs; short

labs = dec RBC, WBC, platelts; inc HbF; bone marrow hypoplasia

tx = corticosteroids; androgens; bone marrow transplant

most common is fanconi anemia (spont chromosomal breaks)




symp = hyperpigmented; cafe-au-lait; absent/hypoplastic thumbs; short




labs = dec RBC, WBC, platelts; inc HbF; bone marrow hypoplasia




tx = corticosteroids; androgens; bone marrow transplant

cafe-au-lait

transient erythroblastopenia of childhood (TEC)

6mo-3y/o


transient immune suppression of erythropoiesis


dec reticulocytes ad bone-marrow precursors




recovery = 1-2months

anemia of chronic disease and renal disease

little/no inc in erythropoietin


mild dec in RBC lifespan


normochromic and normocytic


iron low w/o inc in TIBC


ferritin normal or slightly inc



folic acid deficiency

megaloblastic


peaks at 4-7months of age (goat milk feeding)


hypersegmented neutrophils



vitamin B12 deficiency

megaloblastic


vegans


sufficient stores for 3-5 years


first 4-5months of life if mothers deficient




weak; fatigue; failure to thrive; irritable; pallor; glossitis; diarrhea; vomiting; jaundice; neuro symptoms



hereditary spherocytosis and elliptocytosis

aut dom


spectrin def -> deformed -> early removal by spleen




symp = anemia, hyperbilirubinemia, hypersplenism, gallstones (biliary)




inc MCHC, inc bilirubin, inc reticulocytes




confirmatory test = osmotic fragility test




tx = transfusion or splenectomy (after 5-6yrs)

pyruvate kinase deficiency

pallor, jaundice, splenomegaly




inc reticulocytes, mild macrocytosis, polychromatophilia




tx = exchange transfusion for sig jaundice in neonate; transfusions; splenectomy

polychromatophilia

G6PD deficiency: 2 syndromes

episodic hemolytic anemia (most common)


chronic nonspherocytic hemolytic anemia

G6PD deficiency:


genetics


etiology


24-48hr after ingestion of an oxidant....



xlinked

mediterranean, middle eastern, african, asian

oxidant (ASA, sulfa, antimalarial, fava) or infection and severe illness --> rapid drop in Hb, hemoglobinuria, jaundice

heinz bodies

xlinked




mediterranean, middle eastern, african, asian




oxidant (ASA, sulfa, antimalarial, fava) or infection and severe illness --> rapid drop in Hb, hemoglobinuria, jaundice




heinz bodies





sickle cell anemia: age of onset and first presentation

hemolytic anemia over first 2-4 months


by age 5 -- all have functional asplenia




hand-foot syndrome (acute distal dactilitis)

hand-foot syndrome

symmetric painful swelling of hands and feet
ischemic necrosis of small bones

symmetric painful swelling of hands and feet


ischemic necrosis of small bones

acute painful crises (sickle)

younger = mostly extrmities


w/ inc age = head, chest, back abdomen




precipitated by illness, fever, hypoxia, acidosis, or without any factors (older)

vaso-occlusive crises (sickle)

--skin ulcers


--retinopathy


--avascular necrosis of hip/shoulder


--infarction of bone and marrow (salm osteomyelitis)


--splenic autoinfarction


--acute chest syndrome (most common mortality)


--stroke (peak @ 6-9yrs)


--priapism



priapism

painful boner that won't go away

sickle cell -- inc susceptibility to which bugs

encapsulated!!


-s. pneumonoccus


-h. influenza


-n. meningitidis


-salmonella osteomyelitis


-parvo --> aplastic crisis

sickle cell kidneys

proteinuria = first sign


UTI


papillary necrosis

sickle cell blood smear

what abx for sickle cell prophylaxis

penicillin at 2 months until age 5

age limit for bone marrow transplant in sickle cell

<16 y/o

alpha thalassemia--3 types:


etiology


type of anemia



1. alpha thalassemia trait (2 gene del)


african americans, mediterraneans


mild hypochromic, microcytic anemia w/o clinical problems




2. HgB H disease (3 gene del)


microcytosis and hypochromia with mild to mod anemia


SW asians


no transfusion/splenectomy required




3. alpha thalassemia major (del 4 gene)


transfusion is cure



beta thalassemia major (Cooley anemia)

excess alpha globin chains -> alpha tetramers form; inc in HbF

second month of life --> progressive anemia, hypersplenism and cardiac decompensation

excess alpha globin chains -> alpha tetramers form; inc in HbF




second month of life --> progressive anemia, hypersplenism and cardiac decompensation

beta thalassemia major labs

with HbF only




severe anemia, low reticulocytes, inc nucleated RBC, hyperbilirubinemia microcytosis




NORMAL BLOOD SMEAR




bone-marrow hyperplasia, Fe accumulates --> inc ferritin and transferrin saturation

tx of beta thalassemia major

transfusions


deferoxamine (asses iron overload with liver biopsy)


may need splenectomy


bone-marrow transplant curative

two diseases:


minor bleeds vs deep bleeds

minor = vWD


deep = hemophilia

intrinsic pathway vs extrinsic pathway

intrinsic = PTT, 8,9,11,12


extrinsic = PT, 7,13

INR normal range


INR on warfarin?

0.8–1.2


2.0–3.0

what does thrombin time measure

fibrinogen -> fibrin

ristocetin

previously used to treat staphylococcal infections




It is no longer used clinically because it caused thrombocytopenia and platelet agglutination

hemophilia A and B -- symp

A = factor 8 and B = factor 9

xlinked
easy bruising
hemarthroses (ankle first)

A = factor 8 and B = factor 9




xlinked


easy bruising


hemarthroses (ankle first)

hemarthroses

hemophilia A/B

hemophilia A/B

hemophilia A/B labs and tx

2x or 3x inc in PTT correction with mixing studies




tx = prophylaxis now recommended for young children -- prevents chronic joint disease


desmopressin


avoid ASA

vWD

most common bleeding disorder


aut dom


vW is carrier protein for factor 8




mucocutaneous bleeding (bruises; superficial)




lab = in BT and PTT




tx = DDAVP or also may need replacement



factors for vit K

factors 2,7,9,10,C,S

ITP -- symp and duration

1-4wks after viral infection
1-4 y/o

sudden onset of petechiae and purpura w/ or w/o mucous membrane bleeding

<1% w/ intracranial hemorrhage

resolve in 6mo (10-20% get chronic)

1-4wks after viral infection


1-4 y/o




sudden onset of petechiae and purpura w/ or w/o mucous membrane bleeding




<1% w/ intracranial hemorrhage




resolve in 6mo (10-20% get chronic)



ITP -- labs and tx/CI

labs = platelets <20k; platelet size normal to inc; bone marrow is normal to inc megakaryocytes




tx = transfusion CI!! (unless life-threatening bleeding); IV Ig for 1-2d if prednisone doesn't work; splenectomy if severe

ALL - early symp

acute lymphoblastic leukemia


bone and joint pain (esp lower extremities)


bone marrow failure (see other card)

signs of bone marrow failure

pallor


bruising


epistaxis


petechiae


purpura


mucous membrane bleeding


lymphadenopathy


hepatosplenomegaly


joint swelling

ALL (leukemia) dx and tx/complications

anemia


thrombocytopenia


leukemia cells not often seen early


WBC <10k


poor prognosis if WBC >100k


bone marrow aspirate --> lymphoblasts




tx = remission induction then CNS tx


maintenance for 2-3yrs


prognosis = >85% 5yr survival




relapse (15-20%) = ICP, isolated cranial nerve palsies, testicular relapse, pneumocystitis pneumonia, tumor lysis syndrome

tumor lysis syndrome

from initial chemotherapy (cell lysis)




hyper: uric, K


hypo: P, Ca (tetany, arryth, renal calcinosis)

Hodgkin Lymphoma

15-19 y/o
EBV
Reed-Sternberg cell

painless firm cervical/supraclavicular nodes

anterior mediastinal mass

B symptoms

90% cure if eary and >70% if late

15-19 y/o


EBV


Reed-Sternberg cell




painless firm cervical/supraclavicular nodes




anterior mediastinal mass




B symptoms




90% cure if eary and >70% if late

child with anterior mediastinal mass. dx?

hodgkin

hodgkin

4 types of Hodgkin lymphoma

1. lymphocytic predominant


2. nodular sclerosing


3. mixed cellularity


4. lymphocyte depleted

non-hodgkin lymphoma: types of cells


1. lymphoblastic


2. small, noncleaved cell lymphoma


3. large cell

1. T cell (mediastinal mass)


2. B cell


3. T, B, or indeterminate cell

second most frequent malignancy in children and mortality rate?

brain tumors


45% mortality




average age = <7y/o




infratentorial (cerebellum)

most common infratentorial tumor

juvenile pilocytic astrocytoma
80-100% survival
surgery, radiation, chemo
CEREBELLUM

juvenile pilocytic astrocytoma


80-100% survival


surgery, radiation, chemo


CEREBELLUM

name 5 infratentorial tumors

juvenile pilocytic astrocytoma


malignant astrocytoma (includes glioblastoma)


medulloblastoma (midline cerebellar)


brain stem tumors


ependymoma (posterior fossa)

most common supratentorial tumor

craniopharyngioma


calcification on cxr

major morbidity = panhypopituitarism, growth failure, visual loss

surgery and radiation (no chemo)

craniopharyngioma


calcification on cxr




major morbidity = panhypopituitarism, growth failure, visual loss




surgery and radiation (no chemo)

most frequent tumor of optic nerve and symp

optic nerve glioma
benign
slowly progressive

unilateral visual loss; proptosis; eye deviation; optic atrophy; strabismus; nystagmus

optic nerve glioma


benign


slowly progressive




unilateral visual loss; proptosis; eye deviation; optic atrophy; strabismus; nystagmus

wilms tumor: assoc, age

nephroblastoma


second most common malignant abdominal tumor




2-5y/o




assoc = hemihypertrophy, aniridia, genitourinary anomalies, WAGR





wilm tumor - symp and tx

asymptomatic abdominal mass

chemo, radiation, surgery. unilateral nephrectomy

asymptomatic abdominal mass




chemo, radiation, surgery. unilateral nephrectomy

proptosis

neuroblastoma: patho, symp

from neural crest cells, due to N-myconcogene (at any site)


8% of all kid malignancies




"dancing eyes and dancing feet" =


ataxia


opsomyoclonus


also Horner (maybe)




firm, palpable mass in flank or midline; painful; with calcification and hemorrhage



common locations of neuroblastoma

adrenal


retroperitoneal sympathetic ganglia


cervical, thoracic, or pelvic ganglia

neuroblastoma dx

inc HVA and VMA
cxr/CT/MRI

inc HVA and VMA


cxr/CT/MRI

pheochromocytoma: location, age, assoc, symp, tx

Epi and NE from chromaffin cells


adrenal medulla (usually, but can occur anywhere along abdominal sympathetic chain)


6-14y/o




aut dom


assoc = neurofibromatosis, MEN 2A, MEN 2B, tuberous sclerosis, sturge-weber, ataxia-telengiectasia




episodic severe HTN, palp, sweat, headache, dizzy, pallor, retinal papilledema, retinal hemorrhages/exudate




tx = give a- and b-blockers before removal

rhabdomyosarcoma location

head/neck = 40%


genitourinary = 20% -- grapes out vagina


extremities = 20%


retroperitoneal/other = 10%




inc freq in neurofibromatosis

neurofibromatosis

types of rhabdomyosarcoma (x4)

embryonal (60%) = intermediate prog




botryoid (grapelike) = vagina, uterus, bladder, nasopharynx, middle ear




alveolar (15%) = very poor prog, trunk and extremities




pleomorphic = adult form (rare in kids)

spina bifida occulta

inc AFP
midline defect w/o protusion
asymptomatic
lumbosacral

inc AFP


midline defect w/o protusion


asymptomatic


lumbosacral

tethered cord

inc AFP

ropelike filum terminale persists and anchors the conus below L2

abnormal tension = asymmetric lower extremity growth, deformities, bladder dysfxn, progressive scoliosis, diffuse pain, motor delay

MRI needed
surgery = tx

inc AFP




ropelike filum terminale persists and anchors the conus below L2




abnormal tension = asymmetric lower extremity growth, deformities, bladder dysfxn, progressive scoliosis, diffuse pain, motor delay




MRI needed


surgery = tx

meningocele

well covered with skin
meninges herniate

surgery

well covered with skin


meninges herniate




surgery

myelomeningocele

folate reduces risk
lumbosacral

lower sacral -- bowel/bladder incontinence, saddle anesthesia

folate reduces risk


lumbosacral




lower sacral -- bowel/bladder incontinence, saddle anesthesia

myelomeningocele assoc and tx

80% assoc with hydrocephalus; type 2 chiari malformation which may cause hindbrain dysfunction

tx = ventriculoperitoneal shunt and correction of defect

80% assoc with hydrocephalus; type 2 chiari malformation which may cause hindbrain dysfunction




tx = ventriculoperitoneal shunt and correction of defect

hindbrain dysfunction symp

feeding difficulty


choking


stridor


apnea


vocal cord paralysis


upper extremity spasticity

hydrocephalus: obstructive vs nonobstructive

obstructive = cerebral aqueduct (stenosis/gliosis) or lesions in 4th ventricle (tumor, malformation, hemorrhage)




non-obstructive = subarachnoid hemorrhage, meningitis

hydrocephalus symp: infant vs older kids

infant = inc head circ
bulging anterior fontanel
distended scalp veins
broad forehead
"setting sun" sign (eyes pointed down)
inc DTR
spasticity, clonus

older kid = subtle
irritable
lethergic
dec appetite
vomting
headache
papilledema
6th nerve palsy

infant = inc head circ


bulging anterior fontanel


distended scalp veins


broad forehead


"setting sun" sign (eyes pointed down)


inc DTR


spasticity, clonus




older kid = subtle


irritable


lethergic


dec appetite


vomting


headache


papilledema


6th nerve palsy

papilledema

dandy-walker malformation: define and assoc

cystic expansion of 4th ventricle due to absence of roof

assoc agenesis of posterior cerebellar vermis and corpus callosum

cystic expansion of 4th ventricle due to absence of roof




assoc agenesis of posterior cerebellar vermis and corpus callosum

dandy-walker malformation: symp

inc head size
prominent occiput
cerebellar ataxia
delayed motor

positive transillumination

inc head size


prominent occiput


cerebellar ataxia


delayed motor




positive transillumination

define epilepsy

at least 2 unprovoked seizures occur >24 hours apart

febrile seizures

6mo-5yrs


peak at 14-18mo


positive family hx


inc rapidly to 102 degrees


generalized tonic-clonic


<10-15min


brief postictal period




atypical = >15min, more than one day

postictal period

simple partial seizures

asynchronous tonic or clonic movements


most of face, neck, extremities




aura (some pt)


no postictal period




EKG = spike and sharp waves or multifocal spikes




tx = phenytoin usually

complex partial seizures

impaired consciousness


1/3 with aura


automatisms (lip-smacking)




EEG = anterior temporal lobe shows sharp waves or focal spikes




tx = carbamazepine

absence seizure

petit mal




blank stare/flickering eyes


girls


>5yrs


no aura


<30sec


no postictal period




EEG = 3/second spike and generalized wave discharge




tx = ethosuximide or valproic acid (2nd choice)

tonic-clonic seizures

aura


loss of consciousness


eyes roll back


apnea




tonic contraction then clonic rhythmic contractions




tongue-biting


loss of bladder control


semicomatose for up to 2 hrs w/ vomting and bilateral frontal headache




tx = valproic, phenobarbital, phenytoin, carbamazepine

myoclonic seizures

brief symmetric muscle contractions and loss of body tone with falling forward




tx = valproic acid

infantile spasms

hypoxic iscemia is most common (12-24hr after birth)




intraventricular hemorrhare = 1-7d after birth




tx = lorazepam, phenobarbital

NF-1: criteria

aut dom

2 criteria needed:
--5 cafe-au-lait >5mm
--axillar/inguinal freckling
-->2 iris Lisch nodules
-->2 neurofibromas or one plexiform neurofibroma
--osseous lesions (thinning of bone)
optic gliomas

aut dom




2 criteria needed:


--5 cafe-au-lait >5mm


--axillar/inguinal freckling


-->2 iris Lisch nodules


-->2 neurofibromas or one plexiform neurofibroma


--osseous lesions (thinning of bone)


optic gliomas

NF-1 complications

CNS = optic gliomas, hamartomas, malig cancer, TIA, generalized seizures, learning disab, ADD




renovascular HTN or pheochromocytoma




leukemia


rhabdomyosarcoma


wilms tumor

NF-2

bilateral acoustic neuromas


hearing loss


facial weakness


headache


unsteady gait


CNS tumors



tuberous sclerosis

aut dom
infantile spasms
ash-leaf macule (pic)
seizures
sebaceous adenoma
shagreen patch

complication = rhabdomyoma of heart
renal lesion in most -- wither hamartomas or polycystic kindeys

aut dom


infantile spasms


ash-leaf macule (pic)


seizures


sebaceous adenoma


shagreen patch




complication = rhabdomyoma of heart


renal lesion in most -- wither hamartomas or polycystic kindeys

shagreen patch

rough, raised lesion with orange-peel consistency

most in lumbosacral area (midline)

rough, raised lesion with orange-peel consistency




most in lumbosacral area (midline)

erythema toxium

50% of infants

aka body acne or body rash

clears within 2 weeks and up to 4 months

50% of infants




aka body acne or body rash




clears within 2 weeks and up to 4 months

sturge-weber syndrome symp

facial nevus
seizure
hemiparesis
intracranial calcification
retarded
glaucoma
seizure

facial nevus


seizure


hemiparesis


intracranial calcification


retarded


glaucoma


seizure

facial nevus

port-wine stain

what type of seizure in sturge-weber syndrome

focal tonic-clonic


contralateral to port-wine stain


becomes refractory and slowly develops hemiparesis and/or retardation

sturge-weber syndrome dx and tx

dx = occipital-parietal calcifications and intraocular pressure inc

unilateral cortical atrophy and hydrocephalus

tx = possible lobectomy (may prevent retard/seizures); tx seizures; pulsed laser for face

dx = occipital-parietal calcifications and intraocular pressure inc




unilateral cortical atrophy and hydrocephalus




tx = possible lobectomy (may prevent retard/seizures); tx seizures; pulsed laser for face

cerebral palsy

most born with uncomplicated labor and delivery




impaired voluntary muscles


maybe seizures, dec speech, vision, intellect




risks = low weight, intrapartum infection, intraventricular hemorrhage

friedrich ataxia

ataxia by <10y/o


progressive


loss of DTRs


plantar reflex


weak hands/feet


explosive dysarthric (weak) speech


hypertrophic cardiomyopathy --> CHF, death


aut rec GAA triplet repeat

DTR

deep tendon reflex

wilson disease: symp, MRI, tx

copper
aut rec

dystonia
tremors
basal ganglia problems
kayser-fleischer -- + neuropsych symp

MRI = dilated ventricles with atrophy of cerebrum and lesions in thalamus and basal ganglia

decreased ceruloplasmin

tx = penicillamine with chelation...

copper


aut rec




dystonia


tremors


basal ganglia problems


kayser-fleischer -- + neuropsych symp




MRI = dilated ventricles with atrophy of cerebrum and lesions in thalamus and basal ganglia




decreased ceruloplasmin




tx = penicillamine with chelation and liver transplant

tay-sachs disease

deficiency b-hexosaminidasa-A
Ashkenazi jews

normal developmental until 6 months the lag and lose milestones

seizures
hypotonia
blindness

cherry-red macula

deficiency b-hexosaminidasa-A


Ashkenazi jews




normal developmental until 6 months the lag and lose milestones




seizures


hypotonia


blindness




cherry-red macula

lesch-nyhan disease

x-linked


disorder of purine metabolism--excess uric




self-mutilation and dystonia


gouty arthritis


tophi


renal calculi




dx = analyze HPRT enzyme




tx = tx renal complications; anxiety; behavior



spinal muscle atrophy (SMA) -- types

1 = severe infantile (Werdnig-Hoffman)


2 = late infancy, slower progression


3 = chronic juvenile (Kugelber-Welander)

spinal muscle atrophy -- symp, dx, tx

aut rec




progressive hypotonia


generalized weakness (flaccid)


feeding difficulty


resp insuff


fasciculations of tongue and fingers


absent DTRs




dx = SMN gene; denervaion




tx = supportive; death by 2y/o

myasthenia gravis

ACh rec Ab -- block receptors


ptosis and extraocular muscle wekaness


dysphagia


facial weakness


poor head control


rapid muscle fatigue




tx = neostigmine; prednisone; thymectomy (if symp <2yrs)

transient neonatal myasthenia

neonates born to mother with myasthenia


hypotonia and weakness


feedin difficulties


respiratory insufficiency (days to weeks)




ventilation and nasogastric feedings




no inc risk of disease.... Ab fade out

ptosis

HSMSN -- whats it stand for

hereditary motor-sensory neuropathies

marie-charcot-tooth disease

peroneal muscle atrophy (and tibial)

aut dom

clumbsy
pes cavus (high arch)
foot drop
claw hand (severe)

sural nerve biopsy is diagnostic

tx = stabilize ankles; surgical ankle fusion; phenytoin/carbamaz

peroneal muscle atrophy (and tibial)




aut dom




clumbsy


pes cavus (high arch)


foot drop


claw hand (severe)




sural nerve biopsy is diagnostic




tx = stabilize ankles; surgical ankle fusion; phenytoin/carbamaz

guillain-barre syndrome bugs

campylobacter jejuni


mycoplasma pneumoniae




10 days later

guillain-barre syndrome symp

polyneuropathy


symmetric (ascending)


may have tender, pain, paresthesias early


bulbar involvement




autonomic: BP, bradycardia, asystole

define bulbar involvement

dysphagia


facial weakness


respiratory insufficiency

guillain-barre syndrome dx and tx

dx = inc CSF protein with normal glucose and no cells




tx = admit all patients!


supportive (watch resp)


IV Ig 2-5d

gower sign

duchenne muscular dystrophy

duchenne muscular dystrophy

duchenne muscular dystrophy (ages 1-10):


SYMPTOMS

1st sign = poor head control (maybe)


2 y/o = hip=girdle weakness


gower sign @ 3y/o or 5y/o


calf pseudohypertrophy




need orthotic device by age 12


sig accel of scoliosis

duchenne muscular dystrophy (ages 11-20):


SYMPTOMS

resp insuff


repeated pulm infections


pharyngeal weakness (aspiration)


contractures


scoliosis


cardiomyopathy


IQ <70 in most




death @ 18 from resp failure/cardiac failure



duchenne muscular dystrophy labs and tx

labs = CPK 15k-35k (norm <160)


dystrophin deficiency (muscle biopsy)




tx =digoxin; pulm tx; supportive

3 bugs in maternal vaginal flora

group B strep


e. coli


listeria

most common bugs 2mo - 12y/o

s. pneumo


n. meningitis


HiB (less cuz of vaccinations)

kernig sign

flexing of hip 90 degrees and subsequent pain with leg extension

meningitis

flexing of hip 90 degrees and subsequent pain with leg extension




meningitis

brudzinski sign

involuntary flexing of knees and hips after passive flexing of the neck while supine

meningitis

involuntary flexing of knees and hips after passive flexing of the neck while supine




meningitis

CI to immediate LP

--evidence of inc ICP


--severe cardiopylmonary problems requiring resuscitation


--infection of skin over site


--dont delay abx for the CT scan

meningitis: bacterial


cell #


cytology


glucose


protein


gram stain


culture


latex agglutination


pressure

200-5k


polymorphonuclear neutrophil


low


high


+


+


+


high

meningitis: partially treated


cell #


cytology


glucose


protein


gram stain


culture


latex agglutination


pressure

200-5k


mostly polymorphonuclear neutrophil


low


high


variable


variable


+


high

meningitis: TB


cell #


cytology


glucose


protein gram stain


culture


latex agglutination


pressure

100-500


lymphocytes


low


high


-


+


-


high

meningitis: aseptic (viral)


cell #


cytology


glucose


protein


gram stain


culture


latex agglutination


pressure

100-700


mostly lymphocytes


normal


normal/slightly high


-


-


-


normal

tx of meningitis (bacterial)

vancomycin plus cefotaxime/ceftriaxone

acute menigococcemia

any organ: vasculitis and thromboembolic dis

meningococcal rash (black with ring of red)

high dose IV penicillin

any organ: vasculitis and thromboembolic dis




meningococcal rash (black with ring of red)




high dose IV penicillin

acute menigococcemia -- fulminant

septic shock
DIC (purpura)
acidosis
adrenal hemorrhage
renal failure
heart failure

septic shock


DIC (purpura)


acidosis


adrenal hemorrhage


renal failure


heart failure

encephalitis

meningitis + mental status changes

anything that suggests temporal lobe involvement is highly suggestive of....

HSV! -- herpes




examples:


focal seizures


CT scan


MRI


EEG findings

pertussis: phases and tx

vacc wanes after 8-15 years




1. catarrhal phase (2wk) = coldlike


2. paroxysmal phase (2-5wks) = severe cough; whoop; facial petechiae; post-tussive emesis


3. convalescent phase (>2wk) = gradual resolution of cough




tx = supportive and 14d erythromycin (tx all family in household)

bartonella

most common cause of lymphadenitis lasting >3wks

incubation = 3-30d

red to white papules along the linear scratch

fever; malasie; headache; anorexia

atypical = perinaud oculoglandular syndrome

self-limiting and resolves in 2-4mo

most common cause of lymphadenitis lasting >3wks




incubation = 3-30d




red to white papules along the linear scratch




fever; malasie; headache; anorexia




atypical = perinaud oculoglandular syndrome




self-limiting and resolves in 2-4mo

perinaud oculoglandular syndrome

looks like pink eye but NOT

Bartonella henselae
Francisella tularensis
herpes simplex virus type 1
Paracoccidioides brasiliensis 

looks like pink eye but NOT




Bartonella henselae


Francisella tularensis


herpes simplex virus type 1


Paracoccidioides brasiliensis

positive PPD measurements

5,10,15 mm




5 = previously positive or AIDS pts


10 = in high risk pop


15 = in low risk pop

TB extrapulmonary (miliary)

spleen


liver


bone/joints (POTT)


meningitis -- CN 3,6,7 palsies


hydrocephalus

define miliary

disseminated

TB tx

latent = INH x 9mo


primary pulm disease = INH + rifampin x 6mo


------plus pyrazinamide in first 2mo




resistance? = + strptomycin, ethambutol/ethionamide




dec morbidity and mortality if add corticosteroids in meningitis

BCG vaccination

TB vaccination


variable efficacy


time-limited efficacy

perinatal TB

mom (+)PPD = get cxr




crx (-) = give mom INH for 9mo (no baby tx)




possible TB @ delivery = separate baby from mom until cxr obtained




crx (+) = give infant INH and mom full TB drugs until (-) for at least 3 months

lyme disease symp

hx of tick bite usually absent (don't notice)


erythema migrans (3-32d after bite)


--fever


--headache


--malaise




then uveitis and bell's palsy (bilateral); carditis; neuropathy; aseptic meningitis



erythema migrans

lyme dx and tx

dx = hx plus ELISA (Western confirms)




tx = doxycycline 14-21d (>8 y/o)


amoxicillin (<8 y/o)


ceftriaxone if meningitis/carditis

rickettsia rickettsii symp and tx

north carolina; SE
april-september

incubation 2-14d then headache; fever; anorexia; myalgias; GI symptoms

3d = RASH (distal first)

vasculitis and thromboses --> gangrene

hepatosplenomegaly; coma; delirium

tx = doxycycline or tetracycline (ch...

north carolina; SE


april-september




incubation 2-14d then headache; fever; anorexia; myalgias; GI symptoms




3d = RASH (distal first)




vasculitis and thromboses --> gangrene




hepatosplenomegaly; coma; delirium




tx = doxycycline or tetracycline (chloramphenical if allergy)

candida

thrush = nystatin




diaper dermatitis = topical nystatin and hydrocortisone




catheter-related fungemia = amphotericin B




chronic mucocutaneous candidiasis (T cell defect; plus DM usually)

cryptococcus neoformans

HIV pts -- soap bubble lesions in grey matter
meningitis
inhalation of spores (soil or pigeon poop)
usually just pneumonia or asymptomatic

latex agglutination test
india ink -- old method

HIV pts -- soap bubble lesions in grey matter


meningitis


inhalation of spores (soil or pigeon poop)


usually just pneumonia or asymptomatic




latex agglutination test


india ink -- old method

cryptococcus neoformans soap bubble lesions (picture)

only grey matter

only grey matter

histoplasmosis: location/source

bird poop and decayed wood


Midwest (Ohio and Mississippi River valleys)


bat poop in caves

histoplasmosis symp, dx, tx

symp:
acute pulmonary (usually asymp) pic =
--flulike in young kids
--hepatosplenomegaly
--patchy bronchopneumonia and hilar adenopathy
chronic = centrilobular emphysema
erythema nodosum

dx: serum/urine Ag

tx: none; maybe oral itraconazole; amp...

symp:


acute pulmonary (usually asymp) pic =


--flulike in young kids


--hepatosplenomegaly


--patchy bronchopneumonia and hilar adenopathy


chronic = centrilobular emphysema


erythema nodosum




dx: serum/urine Ag




tx: none; maybe oral itraconazole; amphotericin B if disseminated

erythema nodosum picture

coccidioidomycosis

aka san joaquin fever; valley fever

inhaled from dust (SW)

self-limiting usually
dry-nonproductive cough

disseminating = flu-like; chest pain; maculopapular rash; erythema nodosum

aka san joaquin fever; valley fever




inhaled from dust (SW)




self-limiting usually


dry-nonproductive cough




disseminating = flu-like; chest pain; maculopapular rash; erythema nodosum



measles

rubeola
RNA paramyxovirus is very contagious

incubation 10-12d

1. cough
2. coryza
3. conjunctivitis, then koplik spots

tx = support and vit A

rubeola


RNA paramyxovirus is very contagious




incubation 10-12d




1. cough


2. coryza


3. conjunctivitis, then koplik spots




tx = support and vit A

VERSES:


rubeola


rubella


roseola infantum

define coryza

head cold, fever, sneezing

measles complication

otitis media (most common)


pneumonia


encephalitis

rubella

rash begins on face then rest of body (faster rash then measles)

--retroauricular, posterior, and occipital lymphadenitis
--forscheimer spots (pic)
--polyarthritis

tx = supportive

rash begins on face then rest of body (faster rash then measles)




--retroauricular, posterior, and occipital lymphadenitis


--forscheimer spots (pic)


--polyarthritis




tx = supportive



roseola

aka exanthem subitum




high fever (106) for 3 days then URI




maculopapular rash on trunk, arms, neck, face AFTER FEVER

mumps

paramyxovirus
winter/spring
incubation = 12-24d

fever; headache; malaise

unilateral/bilateral parotid swelling

orchitis (one or both) and only if after puberty -- sterile if both

tx = support

paramyxovirus


winter/spring


incubation = 12-24d




fever; headache; malaise




unilateral/bilateral parotid swelling




orchitis (one or both) and only if after puberty -- sterile if both




tx = support

mumps complications

meningoencephalomyelitis


pancreatitis


thryroiditis


myocarditis


deaf


dacryoadenitis

dacryoadenitis

inflammation of lacrimal gland

complication of mumps

inflammation of lacrimal gland




complication of mumps

varicella -- progression of rash

incubation 10-21d

1. macule
2. papule
3. vesicle
4. open vesicle
5. crust

crops of lesions at same time

acyclovir and VZIG if immunocomp

incubation 10-21d




1. macule


2. papule


3. vesicle


4. open vesicle


5. crust




crops of lesions at same time




acyclovir and VZIG if immunocomp

fifths disease

parvovirus B19 (DNA)

--arthritis
--slapped cheek
--lacy, reticulated rash over trunk and extremities (no palms/soles!!)

rash = up to 40d

parvovirus B19 (DNA)




--arthritis


--slapped cheek


--lacy, reticulated rash over trunk and extremities (no palms/soles!!)




rash = up to 40d

fifths disease complications

aplastic anemia in sickle (hemolytic anemia)


hydrops fetalis if mom has it in first trimester

EBV

1. fatigue


2. pharyngitis


3. generalized adenopathy




incubation: 30-50d


splenomegaly (no sports)




prodrome for 1-2wks (fever, fatigue, headache, sore throat, abd pain)




get rash if given ampicillin or amoxicillin




tx = steroids

influenza virus symptoms

abrupt onset with coryza, conjunctivitis, parhyngitis, dry cough




fever (2-4d), myalgia, malaise, headache

coxsackievirus

oral ulcers

supportive care

oral ulcers




supportive care

adenovirus symptoms

fever


pharyngitis


conjunctivities


diarrhea

poliovirus

assymetric flaccid paralysis


URI symptoms

tx of AIDS (infant)

ZDV until confirmation that disease not present


TMP/SMX at 1mo




F/U = CBC, platelets, CD4, CD8

ascariasis

loeffler's syndrome (pulm eosinophilia) = cough and blood-stained sputum

poverty; human feces; preschool

obstructive intestinal/biliary disease

tx = albendazole, mebendazole, or pyrantel pamoate

loeffler's syndrome (pulm eosinophilia) = cough and blood-stained sputum




poverty; human feces; preschool




obstructive intestinal/biliary disease




tx = albendazole, mebendazole, or pyrantel pamoate

hookworm (2 bugs)

ancylostoma and necator

penetrate through skin --> veins --> lungs --> swallowed --> attach to mucosa up to 5 years

morbidity from blood loss
--iron def
--hypoalbuminemia (edema)
--cough, colicky abd pain, diarrhea, anorexia
--chlorosis (pictur...

ancylostoma and necator




penetrate through skin --> veins --> lungs --> swallowed --> attach to mucosa up to 5 years




morbidity from blood loss


--iron def


--hypoalbuminemia (edema)


--cough, colicky abd pain, diarrhea, anorexia


--chlorosis (picture); green-yellow




eosinophilia!!




tx = mebendazole or albendazole plus ferrous sulfate



enterobiasis (enterobius vermicularis)

perianal laying of eggs at night (anal itching)


restless sleep




tx = single oral dose of medendazole and repeat in 2wks



gonorrhea

asymptomatic pts are at higher risk for dissemination (fever, chills, arthritis)




purulent discharge




tx = single-dose ceftriaxone or single-dose azythromycin




tx partners

chlamydia

mucoid discharge or lymphogranuloma vernerum

tx = 

single-dose azythromycin or doxycycline for 7d

erythromycin in pregnant

mucoid discharge or lymphogranuloma vernerum




tx = single-dose azythromycin or doxycycline for 7d




erythromycin in pregnant

trichomonas

frothy-foul smelling
males asymptomatic
strawberry cervix

tx = metronidazole

motile protozoans

frothy-foul smelling


males asymptomatic


strawberry cervix




tx = metronidazole




motile protozoans

herpes tx

acyclovir


valacyclovir


famcyclovir

tzanck prep

HSV

HSV

open comedone vs closed

open = blackhead


closed = whitehead



tx of acne

benzoyl peroxide


tretinoin (most effective)


adapalene


erythromycin/clindamycin topicals




allow 4-8wks to assess effect before changing or adding medication




THEN.....


tetracycline


isoretinoin (teratogenic)


hormonal therapy




corticosteroid injections heal painful nodulocystic lesions




dermabrasion may help decrease visible scarring