Random Peds stuff Flashcards
What are the complications of Kawasaki disease?
1) coronary artery aneurysms, 2) myocardial infarction and ischemia
Dilated arteries are prone to thrombotic occlusion. Risk is higher in those with delayed diagnosis.
What is the triad for hereditary spherocytosis?
1) hemolytic anemia
2) jaundice
3) splenomegaly
What is the treatment of x-linked agammaglobulinemia
immunoglobulin replacement therapy. Prophylactic antibiotics if severe.
Guillian-Barre syndome (GBS)
acute, immune-mediated polyneuropathy accompanied by ascending muscle weakness and areflexia. Distal paresthesias are common
What is choanal atresia?
congenital nasal malformation caused by failure of the posterior nasal passage to canalize completely leaving either a bony or membranous obstruction. Narrowing at the level of the pterygoid plate in the posterior nasal cavity. Can be found isolated or as part of the CHARGE syndrome
- sudden intermittent abdominal pain
- currant jelly stools (bloody stool)
- sausage-shaped abdominal mass
- lethargy or altered mental status
intussusception
what is oligohydramnios?
deficiency of amniotic fluid
What bacteria causes hemolytic uremic syndrome ?
infection with shiga toxin by E.coli (O157:H7)
what is the inheritance pattern for hereditary spherocytosis?
autosomal dominant mutation of the ankyrin gene (decreased ankyrin in the red blood cell membrane)
- low-grade fever
- maculopapular rash with rapid cephalocaudal spread
- posterior auricular and suboccipital lymphadenopathy
Rubella. Can also present with arthralgia and arthritis that can persist after the rash resolves.
What is the gold standard for diagnosis for DMD?
genetic testing showing deletion of the dystrophin gene on Xp21
What test should be done on a patient with kawasaki disease?
echocardiography at time of diagnosis and repeated 6-8 weeks later to assess change
What is the treatment of obsessive-compulsive disorder in children ?
SSRIs and cognitive-behavioral psychotherapy
What is the definitive treatment for choanal atresia?
repairing the obstruction with surgery or endoscopy
what is the typical age for stranger anxiety?
6-2 years
at what age can a child build a tower with 6 cubes?
2 years
What is the most common muscular dystrophy in children?
Duchenne muscular dystrophy (DMD)
What is the classic triad of hemolytic uremic syndrome?
1) microangiopathic hemolytic anemia (anemia and schistocytes)
2) thrombocytopenia
3) acute kidney injury (poor urine output, edema, elevated creatinine and BUN)
pretend play is a developmental milestone of what age?
18 months
how is a diagnosis of PUV confirmed?
1) voiding cystourethrogram (an x-ray study of bladder and urethra using contrast to visual bladder and also done while voiding) and
2) cystoscopy (examine bladder lining and urethra with camera)
3-hour-old boy with cyanosis. Cyanotic and tachypneic at rest but turns pink when cries. Cyanosis worsens when feeding. What is the most likely diagnosis?
choanal atresia
What are the classic findings on prenatal U/S for a baby with posterior urethral valves?
1) bladder distension
2) bilateral hydroureters
3) bilateral hydronephrosis
how is the diagnosis of choanal atresia confirmed?
CT scan
A congenital abnormality in which the penile urethra opens on the ventral surface of the penis rather than the tip
hypospadias - able to urinate but difficulty controlling urinary stream
What does henoch-schonlein purpura present with?
- purpura
- arthritis
- renal disease
an atretic duodenum can lead to polyhydramnios or oligohydramnios?
polyhydramnios because obstructs amniotic fluid clearance.
When can a child balance and hop on 1 foot?
4 years
What is X-linked agammaglobulinemia (XLA) a.k.a bruton agammaglobulinemia
Caused by a defect in tyrosine kinase that prevents the development of mature B cells.
What is the treatment for ADHD?
1) stimulants (methylphenidate, amphetamines)
2) nonstimulants (atomoxetine, alpha-2 adrenergic agonists)
3) behavioural therapy
What is the clinical presentation of laryngomalacia?
inspiratory stridor that worsens when supine and peaks at age 4-8 months
How do most patients with wilms tumor present?
at age 2-5 years with a large, palpable flank mass but no other symptoms
What is the CHARGE syndrome?
C - coloboma H - Heart defects A - Atresia choanae R - Retardation of growth/development G - genito-urinary anomalies E - ear abnormalities/deafness
What is the difference in indication for ordering a spirometry test vs a peak flow meter test?
Spirometry - evaluates pulmonary function (FVC, forced expiratory volume in 1 second)
Peak flow meter - assessment of airflow out of the lungs (peak expiratory flow rate) Less accurate than spirometry
What test is used to diagnose Meckel’s diverticulum?
Technetium-99m scan to identify ectopic gastric tissue
What test is used to determine intussusception if the diagnosis is equivocal?
U/S - target sign
At what ages does cooperative play occur?
4 years
What is the most common renal malignancy in childhood?
wilms tumor (nephroblastoma)
At what age does a child use 2 word phrases
2 years
during screening for muscular dystrophies, what things are normally elevated?
serum creatine phosphokinase and aldolase levels
What causes laryngomalacia
caused by floppy supraglottic structures that collapse during inspiration.
What is the treatment for laryngomalacia ?
reassurance for most cases and supraglottoplasty for severe symptoms
What is the most important next step in management once a diagnosis of GBS is suspected?
spirometry to measure forced vital capacity (gold standard)
What is the most common cause of urinary tract obstruction in newborn boys?
Posterior urethral valves. Abnormal folds in the distal prostatic urethra obstruct urinary flow.
What is kawasaki disease
an acute vasculitis of small and medium arteries.
How is the diagnosis of laryngomalacia confirmed?
- clinical but confirmed with flexible laryngoscopy for moderate/severe cases
What is the treatment for intussusception?
1) air or saline enema (u/s guided) - diagnostic and therapeutic
2) surgery for removal of lead point
At what age does DMD usually present?
age 2-5
What is the potter sequence?
flat faces, limb deformities, pulmonary hypoplasia due to oligohydramnios.
Kawasaki disease features
Fever ≥ 5 days, conjunctivitis (bilateral, nonexudative, spares limbus), oral mucosal changes (strawberry tongue, erythema, fissured lips), rash, extremity changes (erythema, deem, desquamation of hands and feet), cervical lymphadenopathy
What is the most life-threatening complication in patients with gillian barre syndrome?
neuromuscular respiratory failure
What is the treatment for hereditary spherocytosis
folic acid supplementation, blood transfusions, splenectomy
What is the first step in management of choanal atresia
placing an orophrangeal airway and orogastric tube feeding
at what age can a child use a pincer grasp?
12 months
a child can run and kick a ball at what developmental age?
18 months
What are the lab findings for hereditary spherocytosis?
increased mean corpuscular hemoglobin concentration, spherocytes on peripheral smear, negative coombs test, increased osmotic fragility , abnormal eosin-5-maleimide binding test.
How is amniotic fluid removed by the fetus?
fetal swallowing
What are the treatment options for PUV?
1) PUV ablation
2) urinary diversion
What are the 4 categories for developmental milestones?
gross motor, fine motor, language, social/cognitive
Describe the pathophysiology of Hemolytic Uremic Syndrome
systemic vascular endothelial injury –> platelet microthrombi formation –> thrombocytopenia and schistocytes as they flow through small vessels
What is anorectal manometry?
a test used to analyze the motility and pressure in the distal bowel. Often used to diagnose Hirschsprung disease
What is the treatment of kawasaki’s disease?
aspirin plus IVIG - intravenous immunoglobulin to reduce systemic inflammation
What are the clinical manifestations of x-linked agammaglobulinemia
- recurrent sinopulmonary and gastrointestinal infections after age 6 months (e.g H.influenzae, Strep pneumoniae)
- absence of lymphoid tissue on examination
At what age can a child throw a ball?
12 months
Henoch-schonlein purpura is associated with what GI complication?
intussusception
What condition is likely on the differential when a patient presents with bilateral calf pseudohypertrophy and Gower sign ?
Duchenne muscular dystrophy
