Term
| 6 year old boy presents with sudden onset petechiae over trunk and extremities and epistaxis. S/p URI 3 weeks ago. Labs show 60k platelets. Dx? |
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Definition
- Immune thrombocytopenia. Usually following a URI, and may present with purpura, petechiae, hematuria, or GI bleed.
- The course is self limited, and most patients recover spontaneously.
- DOC is corticosteroids when platelets are less than 30k and for severe symptoms. |
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Term
| Swelling on a 10 hour old infant that is only on one cranial bone. |
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Definition
- Cephalohematoma. This is a subperiosteal hemorrhage.
- Most will resob sppontaneously within 2 weeks-3 months. |
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Term
| Child younger than 3 years with hoarseness and a barking cough. |
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Definition
- Croup. Caused by parainfluenza virus.
- Lateral x-ray may show subglottic narrowing. |
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Term
| deficiency of sphingomyelinase: |
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Definition
Niemann-Pick disease.
- cherry macula, hypotonia, HSM, protruding belly, regression of milestones. |
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Term
| Child with back pain, neurological deficit, and "step-off" in lumbosacral area. |
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Definition
| - Spondylolisthesis. Usually manifests in preadolescent children. |
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Term
| AA patient comes in at 9 months with swelling of the dorsum of his hands and feet bilaterally and a temperature. Dx? |
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Definition
| - Sickle cell. This is dactylitis, whihc is a vaso-occlusive phenonmenon that comes from vascular necrosis of the metacrapals and metatarsals. |
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Term
| Floppy baby born outside the hospital with poor apetite, choking spells, constipation, and apathy. |
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Definition
= Congenital hypothyroidism
- also look for a large tongue and abdominal hernia. |
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Term
| wtf is Beckwith-Wiedmann syndrome? |
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Definition
- neonate who presents with hypotonia, lethargy, and poor feeding.
- Big baby with enlarged tongue, microcephaly, prominent occiput, and omphalacele.
- viceromegaly, hypoglycemia, and hyperinsulinemia.
- |
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Term
| MCC of acute bacterial sinusitis: |
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Definition
1. S. pneumo
2. non-typable H. flu
3. Moraxella |
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Term
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Definition
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Term
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Definition
| - May be the initial presentation in cystic fibrosis; characterized by bilious vomiting, failure to pass meconium at birth, and ground glass appearance on abdominal x-rays. |
|
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Term
| wtf is Sturge-Weber syndrome? |
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Definition
| - a neurocutaneous syndrome characterized by congenital unilateral cavernous hemangioma along the trigeminal nerve distribution, and radiological evidence of intracranial calcifications that resemble a tramline. |
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Term
| 4 year-old girl is brought in due to seizures. She also has developmental delay, and a red, flat lesion covering the left eye area which does not blanch to pressure, and the parents report has been there since birth. She has other neuro defects, and skull X-ray shows tramline intra-cranial calcifications. |
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Definition
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Term
| Child with palpable purpura, hematuria, abdominal pain, and a swollen scrotum. |
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Definition
| - Henoch-Schonlein purpura. |
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Term
| What abdomial complication are children with HSP susceptible to? |
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Definition
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Term
| 2 week old infant with green vomiting and bloodstained stools. |
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Definition
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Term
| Child who presents with progressive morning headaches, unbalanced gait, trunk dystaxia, horizontal nystagmus, and papilledema. |
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Definition
| - Posterior vermis syndrome. Most likely a medulloblastoma. |
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Term
| - Child with macrocytic anemia, low retic count, and congenital anomalies. |
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Definition
- Suspect Diamond-Blackfan syndrome.
- Primary pathology is an intrinsic defect of erythroid progenitor cells which results in increased apoptosis. |
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Term
| A child presents with precocious puberty, cafe au lait spots, and multiple bone defects. |
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Definition
- McCune-Albright syndrome
- 3 p's: Precocious puberty, Pigmentation, Polyostotic fibrous dysplasia |
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Term
4 clinical features to distinguish Septic Joint from Transient Synovitis:
(if 3/4 criteria are met, further septic arthritis work-up is indicated) |
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Definition
1. WBC > 12,000
2. T > 102 (39)
3. ESR > 40
4. Refusal to bear weight
NB: a child with transient synovitis will limp, and may have a slight elevated white count and increased ESR. Look for the above criteria. |
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Term
| A newborn boy has just returned from circumscicion, and the nurses report that 'it just won't stop bleeding.' On exam, you notice petichiae as well as a bleeding wound, and red streaked stool in the diaper. Laboratory findings show thrombocytopenia, low IgM, with high IgE and IgA. Later in life, he gets recurrent infections with encapsulated organisms. Dx? |
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Definition
| - Wiskott-Aldrich syndrome |
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Term
| What immunoglobulin is HSP mediated by? |
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Definition
| IgA-mediated vasculitis of small vessels. |
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Term
| 1 week old infant with 'noisy breathing' on his back, better when prone with neck extended. On laryngoscopy, the epiglottis is rolled from side to side. |
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Definition
- Laryngomalacia. Aka: congenital flaccid larynz. This is the MCC of chronic inspiratory noise in infants.
- Infants should be held upright for 30 minutes after feeding, and never fed when lying down.
- Usually self limiting and resolves by 18 months. |
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Term
| Cyanotic newborn with left axis deviation: |
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Definition
- suspect tricuspid atresia.
- associated heart defects are needed for survival.
- Echo shows a fibromuscular membrane in place of the tricuspid valve, a small right ventricle, VSD, and a large left ventricle. |
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Term
| What congenital heart defect is a/w Edward's syndrome (trisomy 18)? |
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Definition
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Term
| _______ should always be tried in cases of croup before intubation. |
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Definition
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Term
| Pt with new onset headaches after otitis media, accompanied by focal neurological symptoms. |
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Definition
| - highly suspicious of a brain abscess. |
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Term
| What interveniton do you do for a newborn with respiratory distress and suspected diaphragmatic hernia? |
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Definition
| - orogastric tube placement to make sure the bowel doesn't dilate more and compress the lungs more. |
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Term
| What is anemia of prematurity? |
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Definition
- This is seen in hospitalized, premature, or low birth weight infants. Causes include:
1. transitions of erythropoiesis sites
2. shorter RBC lifespan
3. diminished fetoplacental transfusion
- Peripheral smear will show normocytic normochromic anemia, the retic count will be low, and there will be no other abnormalities.
Treat with iron supplementation, Hb checks, and transfusion if necessary. |
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Term
| Child with FTT and a normal anion gap metabolic acidosis: |
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Definition
| - Renal tubular acidosis. |
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Term
| 9 year old presentes with a wide-based gait and imbalance, with decreased vibratyr and position sense in the lower extremities. MRI brain and spine shows marked atrophy of the cervical spineal cord. She also has t wave inversions. |
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Definition
| - Friedreich Ataxia. This is an autosomal recessive condition that is progressive and fatal. |
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Term
| Which children need surgery for an umbilical hernia? |
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Definition
| - Surgery is advied if an umbilical hernia persists to the age of 3-4, exceeds 2 cm in diameter, causes symptoms, becomes strangulated, or enlarges progressively after 1-2 years. |
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Term
| Neonate with erythemetous papules and vesicles surrounded by patches of erythema. Microscopy reveals numerous eosinophils. |
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Definition
| - erythema toxicum. This is a benign condition that does not require treatment. |
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Term
| An adolescent presents with a 6 month h/o unstable gait and speech difficulty that are getting worse. On exam, he has scoliosis, and a foot deformity, dysarthria, dysmetria, nystagmus, and absent deep plantar reflexes. Dx? |
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Definition
- Friedrich ataxia. This is the MC type of spinocerebellar ataxia. It is AR, and usually onsets before 22 years. It is manifested by degeneration of the spinocerebellar, posterior columns, and pyrimidal tracts of the spinal cord. Non-neurologic compliations include concentric hypertrophy, diabetes, and skeletal deformities.
- the most frequent causes of death are cardiomyopathy and respiratory complications. |
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Term
| Apgar for color of the newborn: |
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Definition
0 - body and extremitis are blue/pale
1 - body is pink and extremities are blue
2 - body and extremities are pink |
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Term
|
Definition
0 - HR shows no activity
1 - HR < 100
2 - HR > 100 |
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Term
| Apgar for nasal stimulation: |
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Definition
0 - no response
1 - grimace
2 - active cough |
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Term
|
Definition
0 - limp
1 - some flexion of the extremities
2 - active flexion of extremities |
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Term
|
Definition
0 - absent
1 - slow and irregular
2 - good respiratory effor |
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Term
| CT of a newborn head that shows 'dilation of the entire ventricular system with distinct enlargement of the subarachnoid space over the cerebral cortex' is very suggestive of? |
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Definition
- nonobstructive or communicating hydrocephalus 2/2 SAH.
- SAH is acutally the most common cause of communicationg hydrocephalus. |
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Term
| Infant with FTT, bilateral cataracts, jaundice, and hypoglycemia. |
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Definition
- Galactosemia, caused by glactose-1-phosphate uridyl transferase deficiency, causing elevated blood levels of galactose.
- treat by elimination of galactose from the diet. |
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Term
| What type of anemia is a sickle cell patient at risk for? |
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Definition
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Term
| I say osmotic fragility test abnormal, you say: |
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Definition
| - hereditary spherocytosis |
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Term
| What treatment is indicated for pertussis? |
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Definition
| - regardless of age or disease onset, a macrolide antibiotic (erythromycin, azithromycin) is indicated to treat pertussis. Even if the cough has already occured. |
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Term
| What prophylaxis is indicated for the contacts of a patient with pertussis? |
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Definition
| - Erythromycin x 14 days is indicated as prophylaxis for any household contacts of a patient with pertussis. |
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Term
| What is the treatment for pinworms? |
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Definition
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Term
| What is the most significant risk factor for IVH? |
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Definition
| - low birth weight. The incidence of IVH is inversely proportional to birth weight. |
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Term
| Recurrent, self-limiting episodes of vomiting and nausea in a child, without any apparent casue: |
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Definition
| - cyclical vomiting. Cause unknown, more common in children with FH of migraine. |
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Term
| Small VSDs close spontaneously in __% of cases by 3 years, and __% of cases by 10 years. |
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Definition
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Term
| Adolescent takes amoxicillin for an apparent URI, and then develops a polymorphous rash. Dx? |
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Definition
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Term
| What specific finding is usually seen in the peripheral smear of a sickle cell patient (not sickled cells), and what are these? |
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Definition
| - Howell Jolly bodies. These are nuclear remnants fo RBCs which are generally removed by a functional spleen. Their presence suggests functional asplenia, which usually happens by 18-36 months of life. |
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Term
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Definition
| - aggregates of denatured hemoglobin, commonly seen in pt with hemolysis due to G6PD def and thalassemia. |
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Term
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Definition
| - A characterisitc appearance of cells after phagocytes have removed the Heinz bodies. |
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Term
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Definition
| - fragmented RBCs. The presence of helmet cells is suggestive fo traumatic hemolytic conditions such as DIC, HUS, and TTP. |
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Term
| What is basophilic stippling? |
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Definition
- ribosomal precipitates which appear as blue granules of various sizes dispersed throughout the cytoplasm of the red cell.
- these are often seen with thalassemias, as well as heavy metal poisoning. |
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Term
| What is the treatment for pediatric iron overdose? |
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Definition
- in an asymptomatic patient with an intact gag reflex: ipecac syrup to induce emesis.
- Severe intoxication: IV deferoxamine.
- Activated charcol and gastric lavage are not recommended in young children. |
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Term
| What is the major protein source in human milk? |
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Definition
| - whey, which is more easily digested than casein (also in breast milk) and helps improve gastric emptying. |
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Term
| Child with dubious immunization history, abrupt onset fevers, dysphagia, and drooling with increased respiratory effort. |
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Definition
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Term
| I say delayed separation of the umbilical cord, you say: |
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Definition
| - Leukocyte adhesion defect. This condition is characterized by increased immunoglobulins with impaired cell signaling. These patients will have recurrent bacterial infections, and necrotic peridontal infections. |
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Term
| What is the triad of toxoplasmosis? |
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Definition
| - chorioretinits, hydrocephalus, and intracranial calcifications. |
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