Pediatric Examination and Board Review (82 page)

FIGURE 50-1.
Hepatic pathology of fulminant hepatic failure.
A.
Gross liver section demonstrating regenerative nodules after FHF.
B.
High-power view demonstrating necrosis with lack of hepatocytes and intact portal structures.
C.
High -power view with reticulin stain showing nodular liver regeneration. (Reproduced, with permission, from Hall JB, Schmidt GA, Wood LDH. Principles of Critical Care, 3rd ed. New York: McGraw-Hill; 2005: Fig. 83-1A-C.)

14.
(B)
In a patient who has been ill, acetaminophen toxicity must be included in the differential diagnosis. Parents who give acetaminophen frequently can inadvertently overdose a child. In a teenager, you must be concerned about intentional overdose. The viral illnesses listed would not cause liver disease in a previously healthy 8-year-old.

15.
(A)
The treatment for acetaminophen toxicity is Nacetyl-cysteine. The treatment is most effective if given within the first 10 hours after ingestion. Jaundice and increased liver numbers occur 3 days after the ingestion. Although the N-acetyl-cysteine may be less effective after that time, it is still given.

16.
(A)
The description of a 2-year-old with a large liver unexplained by a viral illness (eg, hepatitis) is characteristic of hepatic malignancy. The initial evaluations would include an ultrasound and a serum alpha-fetoprotein level. Wilson disease does not present before age 4-5 years. Autoimmune hepatitis rarely presents at 2 years old. The presentation for α
₁
antitrypsin deficiency or hepatitis B or C in this age group would be an asymptomatic elevation in the ALT and AST.

17.
(B)
The most common hepatic malignancy in this age group is hepatoblastoma. Hepatocellular carcinoma presents in older children and is usually associated with hepatitis B or C. Rhabdosarcomas and angiosarcomas are rare.

18.
(B)
The description is the classic presentation for a choledochal cyst in this age group. Choledochal cysts can present as neonatal cholestasis. Gallstones are incredibly rare in children. Sclerosing cholangitis and cholangiocarcinoma do not have acute presentations.

S
UGGESTED
R
EADING

Bezerra JA, Balistreri W. Cholestatic syndromes in infancy and childhood.
Semin Gastrointest Dis.
2001;12(2):54-65.

Schwarz K, Balistreri W. Viral hepatitis.
J Pediatr Gastroenterol Nutr.
2002;35(suppl 1):S29-S32.

Whitington PF, Soriano HE, Alonso EM. Fulminant hepatic failure. In: Suchy F, ed.,
Liver Disease in Children.
3rd ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2007.

CASE 51: A 6-YEAR-OLD WITH FECAL INCONTINENCE

A 6-year-old boy presents with a complaint of fecal incontinence. His mother reports he stools every other day in the toilet but in between he has 4-6 “accidents.” The mother denies abdominal distention, decreased appetite, or nausea.

Physical examination shows his height and weight to be at the 50th percentile. The abdomen is soft, with normal bowel sounds and no palpable liver or spleen. Examination of the head, ears, eyes, nose, and throat is normal. Similarly, the cardiac and respiratory examinations are normal.

SELECT THE ONE BEST ANSWER

1.
Your most important next step is

(A) barium enema

(B) rectal exam

(C) lumbar spine MRI

(D) to start a behavioral program for bowel training

(E) reassure Mom he will outgrow this problem

2.
If this was a low-segment Hirschsprung disease, you would expect

(A) increased rectal tone compared with functional constipation

(B) decreased rectal tone compared with functional constipation

(C) the same rectal tone compared with functional constipation

(D) normal rectal tone

(E) a presentation in infancy

3.
If the child has a sacral dimple, then associated clinical feature(s) may include

(A) urinary incontinence

(B) loss of lower extremity reflexes

(C) anterior placed anus

(D) A and B

(E) A, B, and C

4.
The most appropriate next step in the management for the patient in question 3 is

(A) rectal examination

(B) abdominal radiograph

(C) barium enema

(D) lumbosacral spine MRI

(E) to start a behavioral program for bowel training

5.
The child in the vignette has a large amount of hard stool and decreased tone on his rectal examination. The next diagnostic test is

(A) abdominal radiograph

(B) barium enema

(C) lumbar spine MRI

(D) colonoscopy

(E) none necessary

6.
The treatment for the child in question 5 includes

(A) dietary intervention

(B) enema

(C) osmotic laxatives

(D) behavioral intervention

(E) all of the above

7.
The length of time this child will need to be treated is

(A) 1 week

(B) 2 weeks

(C) 3 weeks

(D) more than 6 months

(E) once laxative therapy is initiated, it is lifelong

8.
The chance of successfully weaning all laxatives after a year is

(A) 10%

(B) 30-50%

(C) 50-75%

(D) 75-90%

(E) more than 90%

9.
If the child in the vignette had rectal prolapse with his constipation, the next step would be

(A) a sweat test

(B) a rectal biopsy

(C) to treat the constipation

(D) to do a detailed calorie count

(E) nothing

10.
Had the weight and height of the child in the vignette been less than the 5th percentile for age and there was rectal prolapse, your next step would be

(A) a sweat test

(B) a rectal biopsy

(C) to treat the constipation

(D) to do a detailed calorie count

(E) nothing

11.
Had the child in the vignette been treated for urinary incontinence by a urologist and his constipation began after his treatment

(A) the constipation and urinary incontinence are secondary to a spinal tumor

(B) urinary incontinence is usually treated with anticholinergics and the constipation is secondary to the drug

(C) the constipation was previously undiagnosed and the urinary incontinence is secondary to the constipation

(D) B or C

(E) none of the above

12.
Had the child in the vignette had painless bright red blood per rectum, the likely diagnosis would be

(A) juvenile polyp

(B) fissure

(C) hemorrhoid

(D) Meckel diverticulum

(E) colon cancer

13.
If this child was 6 weeks old, breast-fed, and stooled once every 5 days, the management would include

(A) reassurance

(B) mineral oil

(C) malt soup extract (a diastatic barley malt extract)

(D) rectal stimulation

(E) stop breastfeeding

14.
If the child in question 13 was in a good disposition but a poor feeder with constipation, your first step is

(A) a barium enema

(B) a rectal biopsy

(C) TSH and thyroxine (T
₄
) determinations

(D) a sweat test

(E) treat for reflux to help improve fluid intake

15.
Regarding Hirschsprung disease, which statement is true?

(A) male-to-female ratio is 4:1

(B) female-to-male ratio is 4:1

(C) there is an equal gender distribution