USMLE FA QA Flashcards

1
Q

Bitemporal hemianopia, typ- ically caused by lesions in the sella turcica im- pinging on the optic chiasm. In children the most common tumor in this location is a cra- niopharyngioma, derived from the remnants of

A

Rathke’s pouch.

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2
Q

The three most common forms of pituitary adenoma are:

A

prolactinomas (which are derived from lactotrophs and secrete pro- lactin, causing galactorrhea and amenorrhea), growth hormone-secreting tumors (somato- trophs, causing acromegaly), and ACTH-pro- ducing tumors (corticotrophs, causing Cush- ing’s disease).

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3
Q

Fetuses exposed to what virus during the first trimester may experience intrauterine growth retardation and be afflicted with central nervous system damage, with hearing and sight impairments. Mental retardation will occur along with microcephaly. A characteristic “blueberry muffin rash” is usually present.

A

CMV

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4
Q

the persistence after birth of part of the omphalomesenteric duct (vitelline duct or yolk stalk).

A

Meckel’s diverticulum

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5
Q

what is the most common cause of death in infants born with congenital diaphragmatic her- nia. When the pleuroperitoneal folds fail to fuse with the other components of the dia- phragm during development, a hole is created that allows bowel into the thorax.

A

Pulmonary hypoplasia

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6
Q

What maternal condition increases the risk for transposition of great vessels in the child? What are the signs?

A

maternal diabetes increases the risk of having a child with transposition. Finally, the common symptoms of diabetes must be known: polydip- sia, polyuria, and blurred vision.

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7
Q

The allantois and urachus form what mature adult structure?

A

the median umbilical ligament.

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8
Q

This patient’s inability to pass meconium, as well as the subsequent digital disimpaction, is consistent with what disease?

A

Hirschsprung’s disease, or congenital megacolon.

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9
Q

This disorder is characterized by the absence of ganglion cells in the large bowel, leading to functional obstruction and colonic dilatation proximal to the affected segment. Signs and symptoms of the disease re- sult from failure of neural crest cell migration into the bowel wall during development.

A

Hirschsprung’s disease, or congenital megacolon.

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10
Q

4 components of tetralogy of fallot

A

Pulmonary valve stenosis (as evidenced here by the left upper sternal border systolic murmur), Right ventric- ular hypertrophy, an Overriding aorta, and a Ventricular septal defect are the four compo- nents of the tetralogy of Fallot

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11
Q

This condition results from abnormal migration of neural crest cells and results in the aorticopulmonary septum being displaced anterosuperiorly. Life-threaten- ing hypoxia may develop. The child will often squat in an effort to relieve hypoxia; squatting increases the systemic pressure, thereby reduc- ing the right-to-left shunt and improving oxy- genation. Treatment is corrective surgery.

A

Tetralogy of fallot

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12
Q

In the fetus, what structure serves to shunt blood from the left pulmonary artery to the aorta, allowing the blood to avoid the high-resistance lungs and delivering more oxygenated blood to the rest of the body.

A

the ductus arteriosus

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13
Q

Failure of the ductus arterio- sus to close is common in premature babies, as in this case as well as in cases of maternal ru- bella during pregnancy. What drug can be given?

A

Indomethacin, a non- steroidal anti-inflammatory drug, blocks the production of PGE1 and can be used to close a PDA.

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14
Q

What muscles elevate the palate? embryological origin?

A

The muscles that ele- vate the palate are derived from branchial arch 3 (the stylopharyngeus) and branchial arch 4 (the levator veli palatini). These are innervated by cranial nerves IX and X, respectively.

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15
Q

Caused by a complete defi- ciency in UDP-glucuronosyltransferase, the hepatic enzyme necessary to conjugate biliru- bin. This disorder produces a severe unconju- gated (indirect) hyperbilirubinemia that causes death within the first few years of life.

A

Crigler-Najjar syn- drome type I

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16
Q

benign disorder caused by a mutation in the promoter region of UDP-glucuronyltransferase, leading to diminished expression of the gene. Patients with this syndrome develop a mild un- conjugated hyperbilirubinemia but are usually asymptomatic and have a normal life expec- tancy.

A

Gilbert’s syndrome

17
Q

occurs when the developing bile ducts close com- pletely and fail to recanalize. Appropriate ther- apy of biliary atresia involves anastomosis of the small bowel directly to intrahepatic bile ducts, a maneuver known as the Kasai procedure.

A

Congenital extra hepatic biliary atresia

18
Q

Until week 5, what location is solely responsible for hematopoiesis. By week 8, what location has taken over as the pre- dominant location.

A

yolk sac, liver