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176 Cards in this Set
- Front
- Back
Age for stranger anxiety
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6-9 months
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Common cause of severe combined immunodefiency
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adenosine deaminase deficiency (autosomal recessive)
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Small, pink, salmon-colored macular rash; high-spiking fevers, hepatospelenomegaly (HSM)
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Systemic (10-20%), "Still's disease"
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Treated with IV immunoglobulin (IVIG) and high-dose aspirin"
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Kawasaki disease
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treatment of croup
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supportive, but racemic epi and corticosteroids reduce length of time in ER and hospital
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Name the immunodeficiency syndrome characterized by cerebellar ataxia, oculocutaneous telangiectasia, decreased T-cell function, and low antibody levels.
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Ataxia telangiectasia
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Hot water should be less than
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110-120F
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Dx of HSV
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Tzanck smear, tx: acyclovir
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A 6 month old girl presents with optic atrophy, spasticity, and dies within 1 month. What is the most likely cause of death
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Krabbes disease
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Peak incidence of Wilms tumor in peds
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2 years
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CHF presentation in an infant
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weak, diaphoretic, poor weight gain, tacypnea w/ retractions. lungs: cracckles, wheezes.
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Roseola - rash
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Small, blanchable macules and papules
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Tuberous Sclerosis - description
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Rare inheritied Neurocutaneous disorder w/ cutaneous manifestations
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Presentation of TTP
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1) Thrombocytopenia
2) Schistocytosis 3) Elevated serum LDH levels 4) Absence of other disease entities that could explain the thrombocytopenia and microcytic hemolytic anemia |
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Age for social smile
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1-2 months
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At what age are you able to write script?
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8 years
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Aniridia and hemihypertrophy (one side of body larger then other)"
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Wilms Tumor
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characteristics that CMV and toxo share
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jaundice, hepatosplenomegaly, microcephaly,
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How do you diagnose absence seizures
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EEG will show the classic 3-Hz spike-and-wave pattern
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the type of ROP that has highly active ROP and must be considered for early treatment is
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prethreshold 1
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When does a child wave bye bye?
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10 months
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gross motor skills at 12 mo
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walks alone
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signs of autism may appear around the same time children receive a vaccine to prevent what dz
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MMR prevents rubella
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conjunctivitis with lymph nodes
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viral etiology: watery, red eyes with preauricular lymph nodes
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How does an annular pancreas form
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A band of pancreatic tissue (from a bifid ventral pancreatic bud) surrounds the duodenum
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clinical presentation of acute arsenic poisoning
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"n/v/d, abdominal pain
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Kallman Syndrome
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X-linked hypogonadotropic hypogonadism affecting males sand females, associated with anosmia, cleft lip/palate, and other midline defects
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complement deficiencies (C5 through C9) usually have
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Recurrent neisserial infections
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There is a prominent systolic ejection click just after S1, and a harsh systolic ejection murmur.
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pulmonic stenosis
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digitalis toxicity in infants
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vomiting
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the inheritance in 90% of cases of Stargartz (juvenile blindness=fundus flavimaculatus)
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AR
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Social smile"
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1 - 2 months
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how would you correct an amblyopic child with bilateral hyper of 7.0D with a large XP that tropes with full + correction
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partially correct
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how do you differentiate psoriasis from Reiter's syndrome witha psoriatic rash
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Reiter's syndrome will inolve the mucous membranes, Psoriasis does not.
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At what age do you use scissors?
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4 years old
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what is the most common cause of amblyopia
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strabismus
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Medications that are contraindicated in obstructive hypertrophic cardiomyopathy
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positive ionotropes
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What are the clinical manifestations of phagocytic immunodeficiency syndromes?
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Poor wound healing, abscess formation, and granulomas
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A 4 month old girl has a history of cyanosis while feeding. She has an x-ray that reveals a boot-shaped heart. Diagnosis?
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Tetralogy of Fallot
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tx of sinusitis
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antibiotics for 10-14 days
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what is seen in the serum of someone with a pneumonia caused by Mycoplasma?
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Cold Agglutinins
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PID treatment
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Doxycycline for 2 wks.
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vacterl
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vertebral
anal cv te renal limb |
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Most common primary immunodeficiency
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IgA deficiency
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Define failure to thrive
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growth below 5th percentile for age
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Features of X-linked agammaglobulinemia
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X-linked recessive, so seen in boys
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Lymph nodes and thymus in severe combined immunodefiency are usually
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hypoplastic or absent
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tx of clubfoot
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"bracing
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Features of peripheral smear in ITP
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Normal
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When does a child crawl?
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10 months
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Roseola - rash presentation?
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Sudden onset as high fever lysis in healthy infant
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What is the most common soft tissue tumor of childhood and adolescence
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Rhabdomyosarcoma
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gross motor skills at 1 mo old
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raises head
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Treatment of Osgood-Schlatter disease
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rest/activity restriction
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What is a milder form of dystrophinopathy that also contains a mutated dystrophin gene
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Becker's muscular dystrophy
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retinoscopy (over/under) est. hyperopia in small eyes. by how much is it over/under est?
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over by 1/3
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fine motor skills at 4 yrs
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catches ball
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What is the preschooler's concept of bodily injury?
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Fear of body mutilation
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When does growth end?
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Girl-15, boys-17
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Originates from cerebellar vermis and grows to the fourth ventricle
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Medulloblastoma
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caused by parvovirus B19. There is frequently an associated low-grade fever (100 to 101) with a rash appearing 7 to 10 days later. The characteristic rash starts as facial erythema -- the "slapped cheek" appearance. This can spread to the trunk and have an erythematous macular appearance. Central clearing of the rash appears, giving a lacy appearance. The rash often lasts longest on the extremities, where it has a lacy, reticular appearance.
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Erythema infectiosum - Also called fifth disease, t
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social milestones at 6 mo
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recognizes strangers
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What are the different types of VSD
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Membranous (most common)
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systemic manifest to CMV
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hearing loss, rash (blueberry baby)
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Which process, omphalocele or gastroschisis, has no umbilical ring
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omphalocele
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systemic manifest unique to toxo
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anemia, fever, intracran calcifications, seizures
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very low T- and B-cells (lymphopenia) with bacterial and fungal infections"
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SCID
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After a prodrome of fever (over 101), cough, coryza, and conjunctivitis, this maculopapular rash starts on the neck, behind the ears, and along the hairline. It spreads downward, and reaches the feet in 2-3 days. Immunization is very effective in preventing this infection.
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measles
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Treatment for meningitis caused by Cryptococcus
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"Amphotericin
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Common previous infection for TTP
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none
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mechanism behind bell's palsy in neonates
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forceps deliver... usually resovles
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imaging most specific for intussusception
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"barium (or air) enema
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TORCH cause of autism
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congenital rubella
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Separation in the sensorimotor stage of Cognitive development?
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Self from others-4-8 months:6-8 months is when it really kicks in
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Pyloric Stenosis
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"Pyloric stenosis is 3 P's:
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What is the cause of Hypoglycemia in a newborn from a mother with DM?
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Fetal Hyperinsulinemia
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List two of the most common T-cell immunodeficiency syndromes
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DiGeorge's syndrome and ataxia telangiectasia
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developmental dysplasia of the hip
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"abnml relationship between head of femur and acetabulum --> instabilility and dislocation of hip joint
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Pathology of Chediak-Higashi syndrome
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defect in microtubule polymerization
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embryology behind malrotation of midgut
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midgut undergoes partial rotation and --> abnormal position fo abdominal viscera, can be assoc with volvulus --> compromised blood flow and gangrene
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What is the most common congenital heart defect?
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VSD
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fine motor skills at 5 yrs
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ties shoes
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What is the germline deletion associated with Wilms tumor
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WT1.
Wilms tumor is the no. 1 cause of renal tumors in childhood |
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Holostyolic murmur next to sternum
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VSD
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Most common cause of delayed puberty
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constitutional delay (normal variant)
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bronchoalveolar lavage
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Bronchoalveolar lavage (BAL) is a medical procedure in which a bronchoscope is passed through the mouth into the lungs and fluid is squirted into a small part of the lung and then recollected for examination. BAL is typically performed to diagnose lung disease. In particular, BAL is commonly used to diagnose infections in people with immune system problems, pneumonia in people on ventilators, some types of lung cancer, and scarring of the lung (interstitial lung disease).
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Preoperational stage of Piaget's Cognitive Development?
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2 to 7 years
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X-ray finding for epiglottitis
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thumb sign on lateral neck x-ray
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usual location of chorioretinitis in HSV
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peripheral retina, well circumscribed hyperpigmnted scars
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What cardiac defect is associated with Turner's syndrome
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Coarctation of the aorta
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Solitary Mastocytoma - Lesion
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Solitary brown-pink plaque
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Name three glycogen storage diseases
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Type I- von Gierke
Type II Pompe Type VI Mcardle |
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A 2 year old girl presents with a flat, hypoplastic face, prominent epicanthal skin folds, small, low-set ears, stubby fingers, a transverse palmar crease, and mental retardation. What is the diagnosis and what cardiac malformation do you suspect
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Trisomy 21 (Down syndrome) with an endocardial cushion defect
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Industry vs. Inferiority stage of Erikson's psychosocial development?
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6 to 12 years
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Typical age for osteosarcoma
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10-20 year olds
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which 3 infections if already experienced by the mother prior to preg, pos noo further risk to the fetus
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primary rubella, varicella
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Adult-like thinking begins at what age?
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15
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Common previous infection for ITP
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viral, esp in children
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Inheritance of Wiskott-Aldrich deficiency
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X linked recessive
(almost exclusively males) |
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How much weight does a preschool child (3-5) gain each year/ inches grown each year?
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5 lbs and 2-3 inches/year
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Palpable purpura, abdominal pain, and hematuria"
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Henoch-Schonlein purpura
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Antisocial personality disorder must have what diagnosis as child
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conduct disorder
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erythematous macules and plaques appear in stocking and glove distribution 1-3 days after onset of fever. Spreads to involve trunk and extremieits w/in 2 daiys, lasts an average of 12 days
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Kawasaki's disease
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Apical Click followed by a late-systolic murmur"
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Mitral Prolapse
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fever and petechiae on palms and soles of feet
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RMSF
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when to do w/u for diarrhea in a child
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"in infant <3mo, do blood cx
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Most common kidney malignancy in children
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Wilms tumor
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short, Inc calcium, developmental delay, overy friendly, supravalvular aortic stenosis
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Williams syndrome
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Urticaria Pigmentosa - what happens when rubbed?
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Darier's sign of whealing
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strawberry tongue: erythematous tongue with prominent papillae
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streptococcal pharyngitis and Kawasaki disease
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until age 3.5, a partial correction is advised for astigmatism (T/F)
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TRUE
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Meningococcemia"
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"Adrenal Failure/Hemorrhage
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Major differential diagnosis of flank tumor in a child
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"- Wilms tumor (kidney)
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Name the three most common B-cell deficiency syndromes
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X-linked aggamaglobulinemia, common-variable immunodeficiency, and selective IgA deficiency
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What are the characteristics of Meckel's diverticulum
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"2% of the general population, 2 ft from the ileocecal valve, 2 in. in length, 2 years old or younger, typically
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causes of leukocoria
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"retinoblastoma (--> death and visceral mets in all cases)
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pus in middle meatus of the nose is draining from...
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maxillary sinux, frontal, ro anterior ethmoid sinuses
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unilateral nasal discharge, obstruction, and fould smelling discharge, purulent, maldorou,sb.oody discharge
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examine for a nasal foreign body
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Delayed passage of meconium, chronic constipation, FTT, air in bowel"
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Hirschprung's Dz
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recurrent pneumonia in an otherwise heatly child...think
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foreign body/anatomical blockade of airway
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What are the best nondrug measures to reduce pain in school age and adolescents?
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Guided imagery
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2 things that parvovirus B19 causes
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Erythema infectiousum aplastic crisis
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minor for RF
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arthalgias, fever or hx of RF, increased ESr, Pos CRP, increase WBC and anemai, prolonged PR and QT on ECg. most common finding is arthralgias
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Nevus Sebaceous - Location
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Usually on scalp
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fever, cough and tachypnea in a child with Sickle Cell Anemia
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manifestations of pneumonia, pulmonary thromboemboli, or sepsis. need to manage patient immediately.
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a child presents with a wide-based gait, decreased vibratory and position sense in lower extremities, absent ankle jerk bilaterally, atrophy of cervical spinal cord and T-wave inversions"
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Friedreich Ataxia
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gross motor skills at 24 mo
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walks well up and down stairs
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stage 5 ROP
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funnel shaped total retinal detachment.
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child with epistaxis, prolonged bleeding time, and nml platelet count
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von Willebrand's Disease
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Rubella - rash
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"Erythematous macules and papules
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battle's sign
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basilar skull fx that leads to bleeding/bruise behind the ear
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findings in classic hemophilia
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bleeding problems + hemarthrosis
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Skin finding in patients with severe combined immunodefiency
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usually have cutaneous anergy
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Treat refractory cases of enuresis with
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imipramine
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Number one preventable cause of mental retardation
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fetal alcohol syndrome
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Definition of delayed puberty in boys
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No testicular enlargement by 14 years old
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Rolls onto Back
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4 months
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left axis deviation and hypertrophy of the LV can happen in what cyanotic heart defect
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Tricuspid Atresia can cause a hypoplastic RV leading to left axis deviation. the rest cause right axis deviation
|
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what percentage of infants have astigmatism>1.0
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50%
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congenital cystoid adenomatous malformation
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congential dysfunction of the development of the bronchioles
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complication of neonatal lupus
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thrombocytopenia, neutropenia, rash, liver dysfunction, and congenital heart block
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combination of hyponatermia and hyperkalemia clue you into what congenital problem
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congenital adrenal hyperplasia: most commonly caused by 21-hyrdoxylase deficiency. can have severe salt wasting due to decreased aldosterone and cortisol. Females have ambiguosu genitalia.
|
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nursemaids elbow
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subluxation of radial head
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MC type of Ambiguous genitalia
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Congenital Adrenal Hyperplasia
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What should you look for in patient with frequent respiratory and GI infections?
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IgA deficiency
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Congenital Nevi - types
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"1. Giant Hairy Nevus
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When is encopresis considered abnormal?
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4 years old
|
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Combined Immunodeficiencies
|
SCID
|
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A 17 year old boy presents with lower abdominal cramping and bloody diarrhea. Colonoscopy reveals mucosal damage extending from the rectum proximally in a continuous fashion. What do you suspect
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Ulcerative colitis
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Optic atrophy, spasticity, Globoid bodies in brain
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Krabbe's Dz
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medical tx of hyperthyroidism
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Propylthiouracil 5-10 mg/kg/day q8h PO and Propranolol 0.5-1 mg/kg/day PO, thyroidectomy
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What is status asthmaticus?
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increasing distress despite treatment
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progression of intraparenchymal bleed in a newborn
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blood can flow into ventricles w/i 3 days of life
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most common location for intussusception
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ileocolic
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nocturnal abdomainal pain adn gi bleeding with pos fam hx, discomfort, vomiting, anorexia, weight loss, hematemeis or melena
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peptic ulcer disease. pain is most common
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umbilicated (dome-shaped), skin-colored or pearly-white papules. what is tx
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think molluscum contagiosum. it is ia self-liited, contagious, vrial infection transmitted by direct contact. it is caused by a poxvirus. tx: curettage, cryosurgery, sometimes observation
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inheritance of Werdnig-Hoffman disease
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autosomal recessive
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Infant gets repeated pneumonias at beginning of life due to exhaustion of maternal IgG in system"
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Transient Hypogammaglobinemia
|
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Hyperinsulinemia, omphalocele, macroglossia, organomegaly"
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Beckwith-Wiedemann syndrome
|
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Most common age for ITP
|
children or adults
|
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lymphedema in utero, which is the cause of many of the physical findings such as the webbed neck, low placement of ears, edema of the hands and feet, hyperconvex nails and a "shield" chest with widely spaced nipples. Coarctation of the aorta is found in about 20% of affected girls. Short stature is common, and some girls are not diagnosed until early adolescence when they present with short stature and delayed sexual maturation (due to gonadal dysgenesis). Most have a normal IQ.
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Turner's Syndrome
|
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Thumb sign on x-ray
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epiglottitis
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Immune deficiency in Wiscott-Aldritch
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dysfunction of many types of immune cells, such as T cells, B cells, dendritic cells, and natural killer cells.
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incidence of Wiskott-Aldrich syndrome
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between 1 and 10 cases per million males worldwide.
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Gene in W-A Syndrome
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Mutations in the WAS gene cause Wiskott-Aldrich syndrome. The WAS protein (WASP) is found in cells made from hematopoietic stem cells
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Median survival WAS
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6 years
|
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Rx WAS
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Bone marrow transplant
|
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enteroviruses
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There are more than 60 enteroviral serotypes, which are among the small RNA viruses within the Picornaviradae family. Enteroviral genera include the polioviruses, echoviruses, coxsackievirus A and B and newer enteroviruses
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viral myocarditis
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coxsackieviruses B1-5 and more rarely echovirus and coxsackievirus A
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antibiotics for bacterial dysentery
|
Unless the patient
is very young (less than 6 months of age), immunocompromised, or appears toxic, initial antibiotic therapy is not recommended. |
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CNS involvement in HUS
|
As many as 20% of children
with HUS may develop some form of CNS involvement including agitation or confusion, seizures, or cerebral edema. Rarely, patients may develop strokes and hemorrhage secondary to arterial occlusive disease. |
|
TTP consists
of the pentad of |
microangiopathic
hemolytic anemia, thrombocytopenia, fever, neurologic findings, and kidney disease. |
|
Idiopathic TTP appears to be related to a deficiency of
|
a metalloprotease,
ADAMTS13, which cleaves von Willebrand factor. |
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Mortalitiy in TTP
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As recently as
15 years ago, the mortality from an episode of TTP approached 90%.Discovery of the benefi t of plasma exchange has led to survival rates between 60% and 75%. |
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Painful
burning or tingling sensations in the hands and/or feet (acroparesthesias)... |
Fabry's disease
|
|
Pitted keratolysis is a relatively common bacterial infection,
|
and its unique appearance facilitates clinical diagnosis. Its is caused by Corynebacterium, Dermatophilus congolensis, or Micrococcus sedentarius, and it responds rapidly to topical erythromycin, clindamycin, or benzoyl peroxide.
|