Gut and Nutrition
Derm
Board Equations
Neonatology History
Genetics
Lung
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SLCH History
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100
The most common intestinal site(s) for NEC among preterm infants.
What are the terminal ileum and the ascending colon?
100
Name the condition
What is Harlequin syndrome?
100
The formula for calculating oxygenation index.
(MAP xx FiO2 xx 100)/(PaO2)
100
Dr. Virginia Apgar was trained in this specialty.
What is Anesthesiology?
100
Name the diagnosis.
What is Monosomy X /
Turner Syndrome?
100
Name the 5 stages of lung development (in order)
Embryonic
Pseudoglandular
Canalicular
Saccular
Alveolar
100
Term infant infant born to a 22 yo G1 mother. RN notes HR 75. Sats 98%. No respiratory distress. EKG shows this.
Identify the diagnosis.
Identify the cause / mechanism.
Describe the prognosis.
What is Complete heart block?
What is neonatal lupus? What are SSA/Ro and SSB/La antibodies?
–Present in 0.1-1.5% healthy women, 90% Sjogren, 20-30% SLE
–80-95% of severe heart block in the neonatal period
–7-16% of infants with seropositive moms have skin manifestations
What are irreversible changes to the conducting system?
100
B
100
What's wrong with this picture?
CDH with left arm in chest cavity
200
The most common maternal condition associated with small left colon syndrome.
What is maternal diabetes?
200
Name this and the most common genetic syndrome associated with this exam finding
What is cutis aplasia?
What is Trisomy 13?
200
Calculate the Odds Ratio.
What is 0.84?
(a/c) / (b/d)
or, equivalently:
(a*d) / (b*c)
200
Name the last child of Jackie and John F. Kennedy and describe her/his importance to neonatology.
Who is Patrick Bouvier Kennedy?
Born August 7, 1963 at 34 weeks, he had HMD, treated with hyperbaric oxygen at Boston Children’s Hospital, died at 2 days. JFK died 3 months later. His birth and death brought attention to neonatal intensive care.
200
Name the genetic condition.
What is Trisomy 13?
200
Name the structure that arises from the foregut and gives rise to the lungs
What is the respiratory diverticulum?
200
The infant pictured below has a large heart with pulmonary stenosis.
Identify the disease and as many genetic causes as you can.
Identify a novel pharmacologic treatment used in our NICU.
What is Noonan syndrome?
What are RAS-opathies: PTPN11, SOS1, RAF1, RIT1, KRAS; more rare NRAS, BRAF, MEK2, RRAS, RASA2, A2ML1, SOS2
What is tremetinib? Inhibitor of MEK1 and MEK2
200
L
200
What is the diagnosis?
Short rib polydactyly syndrome
200
Who was Martha May Eliot?
One of the first female residents at SLCH in 1919. She was turned down for residency at Hopkins. Went on to career in public health- head of US Children’s Bureau, a founder of UNICEF and WHO, faculty at Harvard and Yale. Went on to receive the American Pediatric Society’s John Howland medal in 1967- this award was named after the residency program at Hopkins who turned her down.
200
A 21-day-old male infant presents with history of recurrent oral thrush and eczematous skin rash.
The WBC counts are persistently low with normal morphology. Nitroblue tetrazolium test is negative. The most likely diagnosis is...
What is SCID?
300
Describe the caloric density of each component of TPN
What are:
Trophamine = 4 kcal/g
Dextrose = 3.4 kcal/g
Lipid = 9 kcal/g
300
Name this diagnosis and what would you find on the smear if you scraped.
What is erythema toxicum?
What are eosinophils?
300
The equation for fractional excretion of sodium.
What is
("Urine sodium" // "serum sodium") / ("urine creatinine" // "serum creatinine")
-or-
("Urine sodium" xx "serum creatinine" ) / ("urine creatinine" xx "serum sodium")
300
Number of beds in the original SLCH NICU.
What is 10?
300
Name the diagnosis and the gene?
Cornelia de Lange and NIPBL
300
Name the stage of lung development where TE fistulas arise.
Name the least common type of TEF.
What is the Embryonic stage?
What is B-type TEF?
Clements BS. Congenital malformations of the lung and airways. In: Pediatric Respiratory Medicine, 1st ed, Taussig LM, Landau LI (Eds), Mosby, St. Louis 1999. Vol 1, p.1115
300
Identify the goal saturation for an infant with unrepaired HLHS.
Describe the reason this is the goal saturation.
What is 75%?
Since aortic and PA sats are the same and sat Pv is hopefully 100%
(SpO2 - 50%) / (100% - SpO2) = 1
SpO2 - 50% = 100% - SpO2
SpO2 = 75%
300
A
300
Name the syndrome.
What is Zellweger Syndrome?
Scimitar-shaped stippled calcification at the patella, a pathognomonic finding of Zellweger syndrome.
300
What was the treatment for apnea or cyanosis in the early 1900's.
Spirit of ammonia and a small dose of whiskey
300
What is neonatal herpes?
400
Your patient has a direct bilirubin of 4.2 mg/dL. These updates are recommended for your TPN.
What are:
1) Reduce copper by 50%
2) Remove manganese and chromium
3) Consider transitioning lipids to Omegaven
400
Name this condition
What is neonatal lupus?
400
The formula to calculate the A-a gradient
What is
(P_"atm" - 47 mmHg)*FiO2 - ("PaCO2"/"RQ")
- PaO2
400
The prior name for ROP.
Name a famous person who has ROP.
The year in which ROP associated with liberal oxygen use.
What is Retrolental fibroplasia, most common cause of childhood blindness?
Who is Stevie Wonder, born at 34 weeks in 1950?
What is 1951? by Kate Cambell of Australia
400
Name the diagnosis and associated gene.
What is Neonatal Marfan’s Syndrome and FBN1?
400
FGF10 binds to FGFR2 receptor to initiate lung bud formation and is essential for branching.
Mutations in FGFR2 result in these genetic syndromes.
What are Apert, Pfeifer, Crouzon syndromes?
400
Describe all causes of differential and reverse differential cyanosis.
Differential - what are:
PPHN, coarctation of the aorta, interrupted aortic arch, severe hypotension with normal PA pressures
Reverse differential - what are:
TGA with PPHN, TGA with coarctation of aorta, supracardiac TAPVR with R->L PDA flow
400
N
400
What is the sign and associated syndrome?
Molar tooth sign, Jouberts syndrome
400
Where in the hospital where ‘infectious children’ cared for when the hospital first opened?
Nowhere. These children were turned away as they were health risks to other children.
500
A 500 gram 23 week infant at 36 hours of age has a Na of 155. UOP is 7 mL/kg/hr. In addition to maintenance fluids, you prescribe this much free water over the next 24 hours with a goal Na of this.
"Free water deficit" = "total body water" xx (Na_"now" - Na_"goal") / (Na_"goal")
TBW = "body water percent" xx weight
"Free water deficit" = "0.85" xx "0.5 kg" xx (155-145) / 145 = "29 mL"
500
Name the condition
What is congenital HSV?
500
The NEC rate in your unit is 3%. A drug rep claims their prebiotic reduces NEC to 50% of their previous rates.
You need to treat this many patients to see one less case of NEC.
What is 67 patients?
"RRR" = "1.5%"/"3%" = 0.5
"ARR" = "3%"-"1.5%" = "1.5%"
"NNT" = 1/"ARR"
500
The year donor milk first become available at SLCH.
What is 1919?
Wet nurses were employed by the hospital and offered to infants free of charge.
500
Name the diagnosis.
Name 1 of the associated genes.
What is Treacher Collins?
What are POLR1C, POLR1D, TCOF1?
500
1) The image on the right from a lung barium angiogram is illustrating this rare neonatal lung disease.
2) This gene is typically disrupted in this disorder.
What is Alveolar capillary dysplasia?
What is FOXF1?
500
Calculate the mL of oxygen per dL blood in a 1.4 kg 30 week infant with a Hgb of 9.2 g/dL, PaO2 70, SpO2 92%.
What is 1.34 mL O2 / g Hgb x 9.2 g/dL x 92% + 0.003 x 70
= 11.6 mL O2 per dL blood
500
K
500
What is the diagnosis/syndrome? What are associated problems?
Ectrodactyly
Can be associated with sensorineural hearing loss, cleft palate, deformities of lacrimal duct
500
Who was Apolline Blair?
Bonus: Who was the most recent Apolline Blair professor?
Widow of a civil war general and US senator, she was the first president of SLCH. She organized a group of 10 wealthy women and 4 homeopathic physicians to found the hospital.
500
What was the weight of the smallest baby that ever survived?
Bonus- earliest gestational age to survive?
260grams