Pediatric Board Review Practice Questions
2005
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QUESTION

89. Which of the following is a typical finding of ulcerative colitis?

A) Ileal involvement
B) Intestinal strictures
C) Transmural inflammation
D) Skip lesions
E) Crypt abscesses

ANSWER

89.     E     Crypt abscesses

Inflammatory bowel disease (IBD) includes Crohn disease and ulcerative colitis. It typically presents around adolescence and young adulthood. Crohn disease is a chronic inflammatory disease involving any part of the GI tract from mouth to anus. Inflammation is typically transmural, making fistulas more common, and discontinuous (skip lesions). Extraintestinal manifestations are more common with Crohn than with ulcerative colitis, and include oral aphthous ulcers, peripheral arthritis, erythema nodosum, digital clubbing, episcleritis, renal stones (from uric acid or oxalate), and gallstones. In contrast to Crohn disease, ulcerative colitis is limited to the colon and involves inflammation of the mucosa alone (not transmural). Crypt abscesses are commonly found and lesions are typically continuous, beginning distally. Extraintestinal manifestations more common with ulcerative colitis than with Crohn include pyoderma gangrenosum, sclerosing cholangitis, chronic active hepatitis, and ankylosing spondylitis. Risk for colon cancer is significantly higher with ulcerative colitis than with Crohn. In about 10% of patients with IBD, the presentation cannot be defined as either Crohn disease or ulcerative colitis but is instead termed indeterminate colitis.

1. Behrman RE, Kliegman RM, Jenson HB, Eds. Nelson Textbook of Pediatrics, 17th ed. Philadelphia, PA: Saunders, 2004:1248-1251.

QUESTION

179. A 4-year-old male is discovered face down, motionless, in a swimming pool. Initial assessment reveals that the patient is hypothermic, bradycardic, and apneic. He is intubated, stabilized, and transferred to a pediatric intensive care unit. Arterial blood gases reveal hypoxemia.

Respiratory management should include all of the following, EXCEPT:

A) Increased inspired oxygen concentration (FIO2)
B) Application of positive end-expiratory pressure
C) Hyperventilation
D) Beta-agonist therapy
E) Placement of nasogastric tube

ANSWER

179.     C      Hyperventilation

The combination of endotracheal intubation, supplemental oxygen, and the use of positive end-expiratory pressure can be effective in treating hypoxemia. Positive end-expiratory pressure (PEEP) keeps the alveoli from collapsing; it should be increased to help functional residual capacity, decrease intrapulmonary shunting, improve ventilation-perfusion matching, and may improve pulmonary compliance, but supra therapeutic PEEP levels can decrease venous return and affect cardiac output. The nasogastric tube is helpful in that it can decompress the stomach, reducing pressure on the lungs, and help prevent aspiration which will aggravate lung damage. Aspiration of 1-3 ml/kg can reduce lung compliance up to 40% and cause serious hypoxemia. While hypoventilation is not indicated, even moderate hyperventilation can contribute to cerebral hypoperfusion.

Death within 24 hours of submersion is considered drowning; after 24 hours of survival, it is considered near-drowning.

1. Kallas HJ. Drowning and near-drowning. In: Behrman RE, Kliegman RM, Jenson HB, Eds. Nelson Textbook of Pediatrics, 17th ed. Philadelphia, PA: Saunders, 2004:321-330.

QUESTION

225. A child is born to a 23-year-old mother with no known medical problems. He is small for gestational age, has overlapping fingers and abnormal arch patterns on his finger pads, microcephaly, and hydronephrosis.

He is MOST likely to have the genetic pattern:

A) Trisomy 8
B) Trisomy 13
C) Trisomy 18
D) Trisomy 21
E) Trisomy 23

ANSWER

225.     C     Trisomy 18

This baby has trisomy 18, also known as Edwards syndrome. This trisomy presents with low birthweight, clenched fists with overlapping fingers (2nd over 3rd and 4th over 5th), rocker-bottom feet, micrognathia, simple arch patterns on finger pads, and cardiac and renal malformations. Renal abnormalities include hydronephrosis and a small penis; a small penis can be seen with trisomy 21 as well. Trisomy 8, also known as mosaicism, occurs in 1/20,000 births and can present with a long face; wide, upturned nose; low-set ears; high arched and occasionally cleft palate. They have moderate mental retardation, and osteoarticular anomalies are seen. Trisomy 13, also known as Patau syndrome, occurs in 1/10,000 births and classically presents with midline cleft lip, microcephaly and/or holoprosencephaly, hypotelorism, polydactyly, bulbous nose, and cardiac malformations. Trisomy 21, also known as Down syndrome, occurs in 1/600-800 births, with increasing rates as mother ages. Other chromosomal anomalies are the cause of 5% of phenotypic Down syndrome. Trisomy 23 is not a recognized syndrome; the 23rd chromosome is the sex chromosome.

1. Hall JG. “Chromosomal clinical abnormalities.” In: Behrman RE, Kliegman RM, Jenson HB, Eds. Nelson Textbook of Pediatrics, 17th ed. Philadelphia, PA: Saunders, 2004:382-391.

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