Case Files Pediatrics, (LANGE Case Files) 4th Ed.

CASE 6

A 6-month-old child arrives for a well-child examination. His family recently moved to the United States from Turkey. His medical and family histories are unremarkable except that his sole source of nutrition is goat’s milk. He appears to be healthy on examination.

Image What hematologic problem is most likely to develop?

Image What nonhematologic concerns are considered in an infant fed on goat’s milk?

ANSWERS TO CASE 6: Megaloblastic Anemia

Summary: This is a 6-month-old child exclusively fed on goat’s milk.

• Likely complication: Megaloblastic anemia from folate or B12 deficiency.

• Other concerns: Brucellosis if milk is unpasteurized.

ANALYSIS

Objectives

1. Appreciate the benefits of breast-feeding.

2. Know the nutritional supplements recommended for breast-feeding mothers.

3. Understand the special needs of infants and toddlers fed on goat’s milk or vegan diets.

4. Appreciate the clinical syndromes resulting from vitamin excesses and deficiencies.

Considerations

A variety of feeding regimens exist for infants and toddlers—breast-feeding, goat’s milk, other types of nonformula milk, and commercial or handmade foods. Health-care providers can educate parents about the benefits and potential dangers of various diet choices.

APPROACH TO:

Infant Nutrition

DEFINITIONS

LACTOVEGETARIAN: Diet devoid of animal products but includes milk.

OMNIVORE: Diet includes both animal and vegetable products.

OVOVEGETARIAN: Diet devoid of animal products but includes eggs.

VEGAN: Vegetarian diet devoid of all animal products.

CLINICAL APPROACH

Infant formulas containing goat’s milk are not routinely available in the United States, but they are available elsewhere. Goat’s milk has lower sodium levels but more potassium, chloride, linoleic acid, and arachidonic acid than does cow’s milk. It is low in vitamin D, iron, folate, and vitamin B12; infants receiving goat’s milk as a primary nutrition source are given folate and vitamin B12 (to prevent megaloblastic anemia) and iron (to prevent iron deficiency anemia). Goat’s milk is boiled before ingestion; goats are particularly susceptible to brucellosis.

Breast milk is considered the ideal human infant food because it contains complete nutrition (with the possible exception of vitamin D and fluoride); iron levels are low but highly bioavailable and do not require supplementation until 4 to 6 months of age. In addition, it has antimicrobial properties and offers psychological advantages to mothers and infants. In developing countries, it is associated with lower infant morbidity and mortality, not only due to a reduction in diarrhea associated with contaminated water used in formula preparation but also because it contains high concentrations of immunoglobulin A (IgA), which reduces viruses and bacteria intestinal wall adherence, and macrophages, which inhibit Escherichia coli growth. Disadvantages include potential HIV (and other virus) transmission, occasional jaundice exacerbation due to increased unconjugated bilirubinemia levels (resolved with a 12- to 24-hour breast-feeding interruption), and its association with low vitamin K levels, contributing to hemorrhagic disease of the newborn (prevented by vitamin K administration at birth).

Formula feeding is substituted for breast-feeding for a variety of reasons. Commercial formula manufacturers strive to provide products similar to human milk. Infant growth rates with cow’s milk formula are similar to those in infants receiving breast milk. Improved sterilization procedures and refrigeration in developed and developing countries have reduced to some degree the gastrointestinal (GI) infections noted with formula feedings.

Formulas are available for special-needs infants. Infants with phenylketonuria require formulas low in phenylalanine, and those unable to digest protein require nitrogen in the form of amino acid mixtures.

Vegan diets supply all necessary nutrients if a variety of vegetables is selected. Some evidence suggests that high-fiber vegetarian diets lead to faster gastrointestinal transit time, resulting in reduced serum cholesterol levels, less diverticulitis, and a lower appendicitis incidence. Breast-feeding vegan mothers are given vitamin B12 to prevent the infant’s developing methylmalonic acidemia (an amino acid metabolism disorder involving a defect in the conversion of methylmalonyl-coenzyme A [CoA] to succinyl-CoA); patients can present with failure to thrive, seizure, encephalopathy, stroke, or other neurologic manifestations. Toddlers on a vegan diet are given vitamin B12 and, because of the high fiber content and rapid gastrointestinal transit time, are given trace minerals that can be depleted.

Vitamin deficiencies and excesses can result in a variety of clinical syndromes. Although rare, these syndromes usually can be averted with appropriate nutrition (Table 6-1).

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Table 6-1 • EFFECTS OF VITAMIN AND MINERAL DEFICIENCY OR EXCESS

COMPREHENSION QUESTIONS

6.1 A 2-day-old infant has significant nasal and rectal bleeding. The pregnancy was without complications; he was delivered by a midwife at home. His Apgar scores were 9 at 1 minute and 9 at 5 minutes. He has breast-fed well and has not required a health-care professional visit since birth. Which of the following vitamin deficiencies might explain his condition?

A. Vitamin A

B. Vitamin B1

C. Vitamin C

D. Vitamin D

E. Vitamin K

6.2 A 6-month-old infant has been growing poorly. His parents have changed his formula three times without success. His examination is remarkable for a pale, emaciated child with little subcutaneous fat and anterior fontanelle fullness. His laboratory test results are notable for a hemolytic anemia and prolonged bleeding times. Which of the following is the most appropriate next step?

A. Gather urine for pH and electrolytes.

B. Measure serum factor IX levels.

C. Measure serum immunoglobulins.

D. Obtain a sweat chloride concentration.

E. Perform a hemoglobin electrophoresis.

6.3 An exclusively breast-fed infant with poor routine care is switched at 6 months of age to whole milk and table foods. Screening laboratories at 9 months of age demonstrate the hemoglobin and hematocrit to be 8 mg/dL and 25%, respectively, and the lead level to be less than 2 µg/dL. A follow-up complete blood count (CBC) 2 weeks later shows the hemoglobin to be at 7.8 mg/dL, the hematocrit 25%, the mean corpuscular volume (MCV) 62%, the platelet count to be 750,000/mm3, and a reticulocyte count of 1%. Which of the following would be the next step in the management of this child?

A. Order a hemoglobin electrophoresis.

B. Obtain a bone marrow aspiration.

C. Initiate iron supplementation.

D. Refer to a pediatric hematologist.

E. Initiate soybean-based formula.

6.4 A 3-week-old is admitted for failure to thrive, diarrhea, and a septic appearance. He does well on intravenous fluids; when begun on routine infant formula with iron, his symptoms return. It is Saturday and the state health department laboratory is closed. You should begin feeds with which of the following?

A. Amino acid-based formula (Nutramigen or Pregestimil)

B. Low-phenylalanine formula (Lofenalac or Phenex-1)

C. Low-iron, routine infant formula (Similac with low iron or Enfamil with low iron)

D. Low-isoleucine, low-leucine, low-valine infant formula (Ketonex-1 or MSUD 1)

E. Soy-based formula (Isomil or ProSobee)

ANSWERS

6.1 E. Newborn infants have a relative vitamin K deficiency, especially if they are breast-fed; most infants are given vitamin K at birth to prevent deficiency-related bleeding complications.

6.2 D. The patient appears to have failure to thrive, with deficiencies of vitamin K (bleeding problems), vitamin A (fontanelle fullness), and vitamin E (hemolytic anemia). Cystic fibrosis (associated with vitamin malabsorption) would explain the condition.

6.3 C. The child in the question most likely did not get iron (or vitamin D) supplementation in the first 6 months of life while exclusively breast-feeding, and was switched to whole milk (low in iron) and to table foods (not supplemented with iron as are baby foods) at too young an age. All of the laboratory data are consistent with iron deficiency anemia; iron supplementation in this child with a resultant brisk erythrocyte response is both diagnostic and therapeutic. Failure of the child to respond to the iron therapy would require further evaluation.

6.4 E. This patient appears to have galactosemia; uridyl transferase deficiency is the cause, and the condition results in features of jaundice, hepatosplenomegaly, vomiting, hypoglycemia, seizures, lethargy, irritability, poor feeding and failure to thrive, aminoaciduria, liver failure, mental retardation, and an increased risk of E coli sepsis. Children with galactosemia are managed with a lactose-free formula. The low-phenylalanine formulas are for infants with phenylketonuria; low-iron formulas serve no purpose other than causing iron deficiency anemia; the low-isoleucine, low-leucine, low-valine infant formulas are useful for patients with maple syrup urine disease (MSUD); and the amino acid-based formulas are excellent for children with malabsorption syndromes.


CLINICAL PEARLS

Image Breast-feeding is associated with lower morbidity and mortality (especially in developing countries) mostly because of a reduction in enteric pathogens and diarrhea associated with contaminated water used in formula preparation.

Image Breast-feeding provides all the nutrients necessary for infant growth with the possible exceptions of vitamin D and fluoride, which usually are supplemented.

Image A breast-feeding vegan should supplement her infant’s or toddler’s diet with vitamin B 12 to prevent methylmalonic acidemia and trace minerals.


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