Nutrition/GI Flashcards

1
Q

A 20-month-old, previously healthy boy is being evaluated for poor weight gain for 4 to 5 months, and watery diarrhea, bloating, and poor appetite for 3 weeks. He currently passes stools 4 to 6 times daily, wakes up with stool in his diaper, and has a significant diaper rash. He has had no vomiting or hematochezia. The boy prefers to drink whole milk (generally 20 oz/day) and water. He does not eat many sugary foods and does not drink juice. His father has lactose intolerance and his older brother has type 1 diabetes. The boy’s physical examination findings demonstrate a weight of 10 kg (7th percentile), length of 87 cm (54th percentile), and weight/length less than the 1st percentile. He appears tired and has thin extremities. His abdomen is soft, moderately distended, and nontender, without mass or organomegaly. Moderate diaper dermatitis is present.

Stool testing shows the following results:

Laboratory Test Result
Fecal ova and parasite examination Negative

Giardia antigen Negative

Fecal bacterial culture Negative

Fecal fat 43% ( reference range <20%)

Pancreatic elastase >500 μg/g ( reference range >200)

Fecal occult blood Negative

Fecal white blood cells Negative

Of the following, the MOST likely cause of this boy’s diarrhea is

A.	celiac disease
 	B.	functional diarrhea
 	C.	Shwachman Diamond syndrome
 	D.	ulcerative colitis
A

Celiacs Disease

Signs of malabsorption:
poor weight gain/failure to thrive, watery diarrhea, abdominal distention, bloating, and diaper dermatitis. abdominal pain, anemia, edema, and easy bleeding

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2
Q

A 6-month-old male infant who is limp and exhibiting obtundation in the morning is brought to the emergency department. Bedside blood analysis reveals a significant metabolic acidosis, including an elevated lactate level and a capillary blood glucose level of 35 mg/dL (1.9 mmol/L) and 3+ ketones in the urine. His newborn screening results were normal. His parents report that he only recently has been able to sleep through the night without waking for a feeding and that he has had a few episodes of morning lethargy that improved after his first morning feeding. The infant appears small and nearly comatose. He has chubby cheeks, a short nose, and a relatively small chin. He has a temperature of 37.8°C, a heart rate of 155 beats/min, a respiratory rate of 15 breaths/min, and a blood pressure of 75/45 mm Hg. Cardiac examination reveals no murmur and a normal S1 and S2. The liver edge is palpable 3 cm below the right costal margin. The remainder of the physical examination findings are unremarkable.

Of the following, the MOST likely diagnosis is

A. fatty acid oxidation disorder

B.	glycogen storage disorder
 	C.	mitochondrial disorder
 	D.	organic acid disorder
A

glycogen storage disorder

obtundation after prolonged overnight fasting and hypoglycemia with lactic acidosis and ketosis, most likely has a glycogen storage disorder (GSD). Glycogen storage disease type 1a (also known as von Gierke disease) is caused by a deficiency in glucose-6-phosphatase.

Inborn errors of metabolism are generally insidious; they involve abnormalities of carbohydrate, lipid, or protein processing that result in a toxic accumulation of energy precursors or metabolites or in a deficiency of nutritional elements.
Glycogen storage disease often presents in infancy and results in hepatomegaly, growth failure, and recurrent episodes of hypoglycemia with ketosis.
Children with organic and amino acid disorders often experience neonatal lethargy, vomiting, coma, strokes, and, if unrecognized, even death.
A hallmark of fatty acid oxidation disorders is the development of hypoglycemia without the concurrent development of ketones.

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ASSESSMENT PROGRESS:Total Questions: 246 Questions Answered: 30 Correct Answers: 21
Question 30
A 12-year-old boy with myotonic dystrophy requires nocturnal ventilator support via tracheostomy. He takes oral nutrition supplemented with feedings via gastrostomy tube. He has had intermittent episodes of fever associated with increased tracheal secretions. A modified barium swallow study demonstrates oropharyngeal dysphagia; the vocal cords are penetrated by thin liquids but not by semisolids.

Of the following, the MOST appropriate next management step for this boy is
A. limitation of oral intake to solids and semisolids
B. pharmacologic manipulation of oral secretions
C. placement of a cuffed tracheostomy tube
D. use of a Passey-Muir speaking valve

A

limitation of oral intake to solids and semisolids

Most patients with a tracheostomy experience at least minimal aspiration of liquids.
Children with a tracheostomy may safely swallow feedings of some consistencies; a modified swallow study should be performed to assess swallowing ability.
A cuffed tracheostomy tube does not prevent aspiration.

Barium and methylene blue dye studies have shown that 30% to 50% of patients with tracheostomies aspirate oral liquids, and 50% to 75% of these aspirations are silent, with no cough or attempt to clear the aspirate.

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4
Q

A 3-year-old boy with trisomy 21 and autism is seen for a health supervision visit. His mother reports that their neighbor, who is a naturopath, suggested administering high doses of vitamin B9 (folic acid) supplements 3 times a day. The mother asks about the benefits and risks of this high intake.

Of the following, the BEST response is that this treatment may

A.	improve his IQ
B.	increase his risk of malignancy	 
    C.	mask vitamin B12 deficiency
D.	prevent worsening of autistic behaviors
A

mask vitamin B12 deficiency

High doses of vitamin B9 (folic acid) may mask vitamin B12 deficiency.
Providers should proactively ask children and families about their usage of vitamins and other supplements.
Providers should counsel children and families on the risks of hypervitaminosis.

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5
Q

A 3-year-old boy is brought to the emergency department after sustaining a scald burn to the majority of his torso. His family states that he reached up to the stove and pulled on the handle of a pot of boiling water that spilled on the anterior surface of his chest. The total body surface area burned is 40%. After the patient has undergone initial resuscitation and fluid management, the burn service asks about the plan for nutritional support.

Of the following, the MOST appropriate nutritional regimen for this patient is administration of

A.	dextrose-containing intravenous fluids at 30% above maintenance rate for 24 hours, followed by initiation of half-strength enteral formula with daily multivitamin	B.	full enteral feedings with high-protein formula supplemented with vitamins C and A and zinc sulfate to provide 30% above resting energy expenditure
C.	isotonic saline at maintenance rate with bowel rest for 72 hours followed by trophic feedings with 30% of full-volume enteral formula
D.	total parenteral nutrition with the recommended dietary allowance of protein, carbohydrates, and fats to meet the daily resting energy expenditure
A

full enteral feedings with high-protein formula supplemented with vitamins C and A and zinc sulfate to provide 30% above resting energy expenditure

Adequate nutritional support is required for optimal wound healing in pediatric patients with burns, and the enteral route is preferred, unless otherwise contraindicated, to ensure positive nitrogen balance and reduce gastrointestinal bacterial translocation.
For children with large total body surface area burns, caloric needs will be higher than usual resting energy expenditure.
Vitamins C and A and zinc sulfate should be prescribed in burn management to ensure adequate wound healing.

Children with burns over less than 10% of their total body surface area (TBSA) have REE that is nearly normal; however, those with burns that affect more than 40% TBSA have REE that may be as high as 1.5 times the normal REE. In children with significant TBSA burns, adequate nutrition is required, and the current recommendations include initiation of enteral nutrition as soon as possible to ensure positive nitrogen balance and reduce gastrointestinal bacterial translocation

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6
Q

A 3-year-old girl is brought to the clinic for concerns of rectal bleeding. Her symptoms began about 6 months ago. Her parents had noticed bright red blood intermittently on the toilet paper and in the toilet; however, over the last few weeks, the bleeding has intensified. Now the girl has blood with each bowel movement. She has no history of constipation and has a soft bowel movement 1 to 2 times every day. She is toilet trained and does not seem to experience painful bowel movements. She is otherwise healthy, with excellent growth and development. Her physical examination findings demonstrate normal vital signs; the abdomen is soft and nontender, without masses or hepatosplenomegaly; digital rectal examination findings are normal and there is no gross blood present. Meckel scan result is negative.

Laboratory data include the following:

Hemoccult

Positive

White blood cell

9,000/µL (9.0 × 109/L)

Hemoglobin

11.8 g/dL (118 g/L)

Hematocrit

32.8%

Platelet

310 × 103/µL (310 × 109/L)

Erythrocyte sedimentation rate

3 mm/h

Of the following, the MOST likely cause of this girl’s findings is
A. cow milk protein allergy
B. infectious colitis
C. juvenile polyp
D. very early-onset inflammatory bowel disease

A

juvenile polyp

Gastrointestinal bleeding may present as hematemesis, melena, and/or hematochezia.
Hemodynamic status should be quickly assessed in children with gastrointestinal bleeding.
Causes of painless rectal bleeding include polyps, Meckel diverticulum, and vascular malformations.

Neonates may exhibit significant rectal bleeding because of coagulopathies, necrotizing enterocolitis, Hirschsprung disease, midgut volvulus, or vascular malformations. mild symptoms may have hematochezia because of swallowed maternal milk, milk protein allergy, or an anal fissure.

infants and toddlers, causes of severe rectal bleeding include Meckel diverticulum, intussusception, midgut volvulus, Henoch-Schönlein purpura, and vascular malformations. Infants and toddlers with mild symptoms may have milk protein allergy or anal fissure.

Older children can have severe bleeding with Meckel diverticulum, inflammatory bowel disease, vascular malformations, or Henoch-Schönlein purpura. Older children

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7
Q

A 5-week-old male infant is brought to the emergency department after 2 days of vomiting. He is not tolerating 2-oz feedings. His vomit is projectile and yellow. He has not had diarrhea, constipation, or abdominal distension. He is alert and active. He has a heart rate of 136 beats/min, a respiratory rate of 20 breaths/min, and a blood pressure of 86/52 mm Hg. His mucous membranes are dry, and his capillary refill time is less than 2 seconds. His abdomen is not distended. The liver edge is palpable 2 cm below the costal margin, and bowel sounds are present. Abdominal ultrasonography shows thickening of the pylorus muscle with no other abnormality.

Of the following, the MOST likely laboratory finding in this infant is

A.	high serum chloride level and metabolic acidosis
B.	high urine chloride level and metabolic alkalosis	C.	low serum chloride level and metabolic alkalosis
D.	low urine chloride level and metabolic acidosis
A

low serum chloride level and metabolic alkalosis

The excessive vomiting leads to loss of large amounts of gastric hydrochloric acid (hydrogen and chloride ions).

Low serum chloride levels and metabolic alkalosis are seen in hypertrophic pyloric stenosis.
High serum chloride levels and metabolic acidosis are seen in diarrhea and renal tubular acidosis.
High urine chloride levels and metabolic alkalosis are seen in diuretic use, Bartter syndrome, and Gitelman syndrome.

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8
Q

A physician is conducting a follow-up visit with a 12-year-old girl who has been experiencing painless vomiting. The girl has a 6-month history of effortless, nonbloody, nonbilious regurgitation occurring 30 min after meals at least once per day. She has had a 4-kg weight loss in the last 6 months. She reports that she enjoys food and is not intentionally losing weight. She reports no nausea, abdominal pain, bloating, diarrhea, or constipation. She was prescribed a proton pump inhibitor for 6 weeks without improvement. On physical examination, her heart rate is within normal range and her dentition is normal.

Of the following, the MOST likely diagnosis is
A. cyclic vomiting syndrome
B. eating disorder
C. Helicobacter pylori gastritis
D. rumination syndrome

A

rumination syndrome

Rumination syndrome should be considered in patients who experience effortless and painless vomiting for 2 months.
Rumination syndrome can be treated with behavioral therapy.

effortless and painless nonbilious, nonbloody regurgitation of food within minutes to hours of eating, after which partially digested food is then rechewed, re-swallowed or expulsed

Cyclic vomiting syndrome is a diagnosis of exclusion characterized by recurrent, discrete stereotypical episodes of vomiting and baseline health between episodes. Cyclic vomiting syndrome is often triggered by psychological or infectious events

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9
Q

A 16-year-old adolescent is being evaluated for concerns about 6 months of worsening fatigue, dizziness, nausea, and abdominal pain. The girl describes daily generalized abdominal pain with nausea, which is worse with dairy intake. She has had intermittent nonbilious nonbloody emesis. She reports no diarrhea, constipation, hematochezia, oily-appearing stools, or significant weight loss. She has a history of well-controlled asthma and normal menses. Physical examination findings demonstrate a blood pressure of 122/58 mm Hg, heart rate of 91 beats/min, weight of 46 kg (11th percentile), height of 154 cm (8th percentile), and body mass index of 19 kg/m2 (33rd percentile for age). She is very pale. The remainder of her findings are within normal limits.

Laboratory data are as follows:

White blood cell

5,110/µL (5.11 × 109/L)

Hemoglobin

4.4 g/dL (44 g/L)

Hematocrit

17.5%

Mean corpuscular volume

64 fL

Platelets

842 × 103/µL (842 × 109/L)

Erythrocyte sedimentation rate

<1 mm/h

Alanine aminotransferase

26 U/L

Aspartate aminotransferase

24 U/L

Albumin

3.8 g/dL (38 g/L)

Upper endoscopy is performed (Item Q82). Biopsy specimens obtained from the small intestine demonstrate severe villous atrophy with increased intraepithelial lymphocytes.

Of the following, the test MOST likely to confirm this adolescent’s diagnosis is

A.	fecal elastase 1
B.	stool polymerase chain reaction for detection of Shigella
C.	sweat chloride
D.	tissue transglutaminase antibody
A

tissue transglutaminase antibody

Celiac testing should be considered in individuals with gastrointestinal symptoms (including abdominal pain, diarrhea, and bloating) and extraintestinal symptoms (including anemia, poor growth, and dermatitis herpetiformis).

Initial screening for celiac disease should include both tissue transglutaminase antibody and total IgA testing.

Individuals with celiac disease should adhere to a strict gluten-free diet, avoiding gluten-containing grains including wheat, barley, and rye

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10
Q

A 2-month-old infant is brought to the clinic for evaluation of jaundice noted last week by her family members. She has been otherwise well with normal growth and development. She is breastfed exclusively. She has multiple stools daily; her parents describe the color as green and brown. The infant was born at term; the pregnancy and delivery were uncomplicated. She had mild jaundice for the first week after birth, which resolved without phototherapy. Her newborn screening results were normal.

Her physical examination reveals a weight of 4.4 kg (25th percentile for age), length of 55 cm (25th percentile for age), and weight-length ratio at the 40th percentile for age. She has scleral icterus. Her abdomen is soft and nondistended, with a liver edge palpable 2 to 3 cm below the right sternal costal margin. There is no splenomegaly. The remainder of her physical examination findings are normal. Item Q107 shows the appearance of her stool in the diaper.

Laboratory data are as follows:

Laboratory Test

Result

Alanine transaminase

83 U/L

Aspartate transaminase

147 U/L

γ-glutamyltransferase

1,662 U/L

Total bilirubin

9.7 mg/dL (166 μmol/L)

Direct bilirubin

6.5 mg/dL (111 μmol/L)

White blood cell count

12,200/µL (12.2 × 109/L)

Hematocrit

36.4%

Platelet count

530 × 103/µL (530 × 109/L)

Urinalysis, urine culture, and urine cytomegalovirus testing results are pending.

Of the following, the best NEXT test to perform is

A.	abdominal ultrasonography
B.	liver biopsy
C.	morning cortisol level
D.	sweat chloride
A

abdominal ultrasonography

The presence of jaundice and acholic stools in a neonate/young infant should prompt urgent assessment.
Evaluation of cholestasis, evidenced by an elevated conjugated (direct) bilirubin level of >1.0 mg/dL (17.1 µmol/L), should include abdominal ultrasonography and urgent consultation with a pediatric gastroenterologist and pediatric surgeon.
Biliary atresia is the most common cause of neonatal cholestasis; initial management with a Kasai hepatoportoenterostomy is most efficacious in infants younger than 60 days.

The initial evaluation for the cause of cholestasis in this infant should include obtaining α1-antitrypsin phenotype and level, thyroid function tests, abdominal ultrasonography, and reviewing the newborn screen results.

In infants who appear to have jaundice after age 2 to 3 weeks, particularly in the presence of acholic stools, serum total and direct (conjugated) bilirubin levels should be measured. A direct bilirubin level greater than 1.0 mg/dL (17.1 μmol/L) is abnormal

Evaluation for BA includes:

liver function tests (including markers of liver synthetic function, such as albumin, glucose, prothrombin time, and ammonia)
γ-glutamyltransferase (which is often very elevated in BA)
liver biopsy (looking for evidence of fibrosis, bile duct plugging, and bile ductular proliferation).

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