Question Review Flashcards by John Nathanson

Signs of intentional burn injury

Zebra striping, lack of splash marks, well demarcated, sparing of flexor surfaces and creases.

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Two year old child language?

200 words, 50% intelligible by stranger, 2 word sentences

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Wiskott-Aldrich Syndrome cause and symptoms

X linked recessive in WASP gene. Features are eczema, microthrombocytopenia, and recurrent infections

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Bone pain worse at night but improved with ibuprofen?

Osteoid osteoma

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Genetics for Duchenne muscular dystrophy

X linked deletion of dystrophin due to a frameshift

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Genetics for Becker muscular dystrophy

X linked reduction in amount of dystrophin due to a nonsense mutation

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Genetics for myotonic dystrophy

Autosomal dominant expansion of CTG

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Comorbidities for duchenne muscular dystrophy

Scoliosis, cardiomyopathy

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Cause of death in friedrich’s ataxia?

Cardiomyopathy

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Newborns and varicella

Newborns are not at any increased risk for complications from varicella after day 2. If mom has varicella 5 days prepartum, give VZV ig

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Common complication of delivery

Broken clavicle presents as anterior shoulder bulge.

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Infectious contraindications to breast feeding?

Drug contraindications for breast feeding?

HIV, active pulmonary TB, typhoid, sepsis

Heroin, amphetamines, cocaine

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Complication of small-for-date infants

Congenital abnormalities

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PPHN hemodynamics

Increased pulm pressure and decreased systemic pressure lead to R to L shunt across PDA and cyanosis

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Apt-downey test?

Given to newborns with melena or hematemesis to distinguish if blood is maternal or fetal

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Derangements of infants born to diabetic mothers?

Immediate hypoglycemia followed by hypocalcemia 24-48 hours later

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Treatment for galactosemia?

Stop giving milk, switch to soy protein

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Injections to newborns?

Vitamin K, hepatitis B vaccine

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Most common TE fistule

Proximal esophageal atresia, distal fistulization

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McCune-Albrigt Syndrome

Sporadic mutation in GPCR

Precocious puberty, bone defects (polyostotic fibrous dysplasia), cafe au lait spots

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Earliest manifestation of sickle cell?

Dactylitis

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Jervell-Lange-Nielsen Syndrome

How to treat if symptomatic?

Long QT with sensorineural hearing loss. Autosomal recessive.
Treat with beta blockers and pacemaker if symptomatic

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Fanconi Anemia

Caused by defects in DNA repair, causes aplastic anemia, short stature, hypoplastic thumbs, strabismus, low set ears, hypo/hyper pigmented areas

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Most common cause of nephrotic syndrome in children? Treatment?

Minimal change disease. Treat with steroids

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Vitamin abnormalities with CF?

ADEK

B1 deficiency

Beri beri, peripheral neuropathy and cardiomyopathy

Workup for breath-holding spells?

CBC because they can be associated with iron deficiency anemia

How to treat a cat bite?

Augmentin, because want to prevent pasteurella cellulitis

Edwards syndrome

Trisomy 18 -- Clenched firsts, low birth weight, microcephaly, micrognathia, rockerbottom feet. VSD!!!!

Patau syndrome

Trisomy 13- cleft lip, polydactyly, hypotelorism, absent ribs

Cri-du-chat syndrome

5P deletion -- microcephaly, metpic suture prominence, cat-like-cry

Beckwith Wiedemann Syndrome, most common complications

Macrosomia, umbilical hernias, macroglossia. | Wilms tumor and hepatoblastomas.

Toxic ingestion that causes QRS widening? How to treat?

TCA ingestion. Treat with bicarb.

Liver biopsy of Reye syndrome?

Shows microvesicular fatty infiltration of liver.

How to treat nonbullous impetigo?

Topical muciprocin

How to treat gonococcal conjunctivitis?

ORAL erythromycin

Stridor caused by laryngomalecia vs vascular ring

Laryngomalecia is worse supine and improves prone. | Vascular ring improves with neck extension.

Any prophylaxis for patient with RF?

Yes, YEARS of Penicillin prophylaxis to prevent recurrent strep pharyngitis.

Causes of neonatal polycythemia

Maternal diabetes (poor gas exchange over placenta), maternal hypertension/preeclampsia, twin-twin transfusion

Symptoms of neonatal polycythemia

Fussiness, jitteriness, respiratory distress

How to treat neonatal polycythemia?

Hydration if asymptomatic, exchange transfusion if symptomatic.

New onset hearing loss with ear drainage despite antibiotics?

Cholesteatoma

Cause of death in fulminant meningococcemia?

Waterhouse friedrichsen syndrome with adrenal infarction

Complication of being large for gestational age?

Peripheral nerve injuries like Erb-Duchenne Palsy and phrenic nerve paralysis

Cause of recurrent hemoptysis and diagnosis?

Idiopathic pulmonary hemosiderosis, bronchoalveolar lavage

How to distinguish bacterial tracheitis from croup? How to treat bacterial tracheitis

Bacterial tracheitis is rapid onset after viral infection and has high fever. Treat with intubation and intravenous antibiotics. Croup has low grade fever.

How to treat ITP?

If just skin manifestations, observe. If bleeding, give IVIG.

Need to give pertussis prophylaxis to close contacts?

Yes, give macrolide prophylaxis

What type of nephrotic syndrome does hepatitis B cause?

Membranous nephropathy

Serum Sickness

Fever, hives, polyarthralgias 1 week after beta lactam antibiotics

Location of muscle weakness in myotonic dystrophy?

Distal muscle weakness.

IgA nephropathy

Nephritic syndrome 1 or 2 days after upper respiratory illness. Hematuria. Complement levels normal

Minimal change disease

Can happen after viral infection causes proteinuria and nephrotic syndrome.

PSGN time course vs iga nephropathy

Two weeks later as opposed to being synpharyngitic. PSGN has low C3 and low-to-low-normal C4

Baby with unibrow?

Cornelia De Lange Syndrome

CHARGE syndrome

``` Coloboma Heart disease Atretic choanae Retardation Genital abnormalities Ear abnormalities ```

Subgaleal hemorrhage

Firm swelling over scalp which extends to the ears and pushes them out.

Persistent posterior fontanelle, umbilical hernia, jaundice in a newborn?

Hypothyroidism

Harlequin skin

Newborn infant with one side pale and the other pink

Diamond Blackfan Anemia

Pure red cell aplasia.

Moyamoya

Finding in sickle cell patients on angiography that shows puff of smoke where there is new vessel formation

Which deficiency causes early onset SLE?

C2 deficiency

Job Syndrome

``` Hyper IgE, FATED Facies Abscesses (cold) Teeth (retained) igE Dermatologic manifestations (eczema) ```

Presentation of neuroblastoma

Midline abdominal mass, may present with opsoclonus

Venous hum

Murmur caused by draining of jugular veins. Low pitched murmur absent when patient supine or turns head.

Bacterial complication of galactosemia/

E coli bacteremia

Complication of prolonged immobilization?

Hypercalcemia

MELAS Syndrome

Mitochondrial encephalopathy, lactic acidosis and stroke

Macrophage activation syndrome

Seen in kids with systemic JIA where fever, pancytopenia, hepatosplenomegaly, and DIC occur.

What antibiotic shouldn't be used in kids under 8 years old?

Fluoroquinolones due to tendon issues. Treat lyme disease with amoxicillin.