Do not minimize the effects that diet has on metabolism
Do not minimize the effects that diet has on metabolism: Excerpt from Avoiding Common Pediatric Errors
Author:
Nickie Niforatos, MD
What to Do - Interpret the Data
Some diets are medications, too.
Aclinicianshouldneverunderestimatetheroleofdietinachild'shealth.
A child's diet must meet basic metabolic functions but also provide adequate
nutrition to allow for appropriate growth and development. A pediatrician's
role is to ensure that the child's diet meets all metabolic and nutritional requirements in the prevention and management of disease, such as iron and
vitamin D deficiencies. Diet, as source of treatment, is particularly important for a number of diseases, including phenylketonuria (PKU), epilepsy,
diabetes, and renal disease.
Children with inborn errors of metabolism have unique metabolic and
dietary requirements. A commonly screened inborn error of metabolism
includes PKU. Patients with PKU lack the enzyme used to convert phenylalanine to tyrosine. As serum levels of phenylalanine rise, excessive levels
of phenylalanine are transmitted across the blood–brain barrier, leading to
deficiencies in the transfer of other essential amino acids. The resultant
amino acid deficiencies lead to reduced neuronal protein and neurotransmitter synthesis, impaired brain development, and mental retardation. The
mainstay of treatment for patients with PKU is diet; phenylalanine intake
is limited with a low-protein diet, and a specific amino acid "cocktail" to
provide sufficient amounts essential amino acids. In addition, patients with a
metabolicdisordermustbemonitoredforsignscatabolism(e.g.,intercurrent
illness, overexertion, anorexia), as they promote the breakdown of internal
protein stores, lead to toxic amounts of a specific substrate, like phenylalanine in PKU. Even a simple viral infection in patients with inborn errors of
metabolism can lead to severe acidosis or hyperammonemia, which, in turn,
can lead to coma or death. These patients should be treated aggressively at
the first sign of illness to avoid (or treat) a metabolic crisis.
On the other end of the dietary protein-intake spectrum is the ketogenic diet, which encourages protein intake and limits carbohydrates. Ketogenic diets benefit patients with severe, refractory epilepsy, infantile spasms,
or certain neurodegenerative disorders. Patients on ketogenic diets often
require vitamin and mineral supplements, especially iron, calcium, and folic
acid; however, vitamin supplements and medications cannot be placed in
typical suspensions, which often contain a carbohydrate base. Be sure to
prescribe all medications and supplements in the form of sugarfree drops or
tabs.
Patients with diabetes mellitus must also pay close attention to their
diet, in maintaining glycemic control. Using an insulin sliding scale in conjunction with carbohydrate counting leads to better glycemic control and the
avoidance of both hypo-and hyperglycemia.
Renal disease is another condition that requires dietary modification. A
high-protein diet can exacerbate uremia, a salt-losing nephropathy may requiresodiumsupplementation,whereascomorbidhypertensionmayrequire
sodium restriction. Vitamin D deficiency, due to renal disease, often leads to
hyperphosphatemia and hypocalcemia and the need for dietary adjustments.
Severe hyperkalemia may lead to fatal cardiac arrhythmias. Fluid retention
may lead to ascites, pulmonary edema, or impaired cardiopulmonary function. Depending on the patient's unique fluid and electrolyte status, a renal
diet must be individualized to each patient.
An inadequate diet may contribute to various diseases, as well. A low-
iron diet in infancy may impair brain development. Anemia can result from
a variety of dietary deficiencies, including iron deficiency, vitamin B12 deficiency, or folate deficiency. Infants who are exclusively breastfed, especially
infants with darker skin and limited exposure to sunlight, may have inadequate vitamin D intake that can result in rickets.
Because of its importance in the maintenance of health and in the treatment of disease, a patient's diet should be monitored regularly to maximize
health and to allow for early intervention should disease occur.
Suggested Readings
Goodman SI, Greene CL. Metabolic disorders of the newborn. Pediatri Rev. 1994;15(9):
359–365.
Haslam RH. Nonfebrile seizures. Pediatr Rev. 1997;18(2):39–49.
Kossof EH, Pyzik PL, McGrogan JR, et al. Efficacy of the ketogenic diet for infantile spasms.
Pediatrics. 2002;109(5):780–783.
Weiss RA, Edelmann CM Jr. End-stage renal disease in children. Pediatr Rev. 1984;5(10):295–
304.
Book Source Details
- Book Title: Avoiding Common Pediatric Errors
- Author(s): Anthony D Slonim MD, DrPH; Lisa Marcucci MD
- Year of Publication: 2008
- Copyright Details: Avoiding Common Pediatric Errors, Copyright © 2008 Lippincott Williams & Wilkins.
More About Metabolic disorders
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Copyright notice for book excerpts: Copyright © 2008 Lippincott Williams & Wilkins. All rights reserved.
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More About This Book:
Title: Avoiding Common Pediatric Errors
Authors: Anthony D Slonim MD, DrPH; Lisa Marcucci MD
Publisher: Lippincott Williams & Wilkins
Copyright: 2008
ISBN: 0-7817-7489-6
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