Biotin and carnitine deficiency due to hypoallergenic formula nutrition in infants with milk allergy

Hayashi, Hisako; Tokuriki, Shuko; Okuno, Takashi; Shigematsu, Yosuke; Akiba, Yasushi; Matsuyama, Go; Sawada, Ken; Ohshima, Yusei

doi:10.1111/ped.12319

Cited by 9 publications

(6 citation statements)

References 7 publications

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“…In Japan, skin abnormalities and hair loss are reported to be due to low contents of biotin in special formulas for inborn metabolic disease and milk allergy [28], [29]. In this regard, administration of biotin improved skin lesions found in PKU patients (personal communication).…”

Section: Discussionmentioning

confidence: 98%

Nutritional status of patients with phenylketonuria in Japan

Okano

Hattori

Fujimoto

et al. 2016

Molecular Genetics and Metabolism Reports

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Accumulating evidence suggests that hyperphenylalaninemia in phenylketonuria (PKU) can cause neuropsychological and psychosocial problems in diet-off adult patients, and that such symptoms improve after resumption of phenylalanine-restricted diet, indicating the need for lifetime low-phenylalanine diet. While limiting protein intake, dietary therapy should provide adequate daily intake of energy, carbohydrates, fat, vitamins, and microelements. We evaluated nutrient balance in 14 patients with classical PKU aged 4–38 years. Approximately 80–85% of the recommended dietary allowance (RDA) of protein in Japanese was supplied through phenylalanine-free (Phe-free) milk and Phe-free amino acid substitutes. Nutritional evaluation showed that the calorie and protein intakes were equivalent to the RDA. Phenylalanine intake was 9.8 ± 2.2 mg/kg of body weight/day, which maintained normal blood phenylalanine concentration by the 80% Phe-free protein rule. The protein, fat, and carbohydrate ratio was 9.5:23.9:66.6% with relative carbohydrate excess. Phe-free milk and amino acid substitutes provided 33.7% of carbohydrate, 82.1% of protein, and 66.7% of fat intake in all. Selenium and biotin intakes were 25.0% and 18.1% of the RDA and adequate intake (AI) for Japanese, respectively; both were not included in Phe-free milk. PKU patients showed low serum selenium, low urinary biotin, and high urinary 3-hydroxyisovaleric acid in this study. The intakes of magnesium, zinc, and iodine were low (71.5%, 79.5%, and 71.0% of the RDA, respectively) and that of phosphorus was 79.7% of the AI, although they were supplemented in Phe-free milk. PKU patients depend on Phe-free milk and substitutes for daily requirement of microelements and vitamins as well as protein and fat. Development of low-protein food makes it possible to achieve the aimed phenylalanine blood level, but this lowers the intake of microelements and vitamins from natural foods. The dietary habits vary continuously with age and environment in PKU patients. We recommend the addition of selenium and biotin to Phe-free milk in Japan and the need to review the composition of microelements and vitamins in A-1 and MP-11 preparations.

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Section: Discussionmentioning

confidence: 98%

Nutritional status of patients with phenylketonuria in Japan

Okano

Hattori

Fujimoto

et al. 2016

Molecular Genetics and Metabolism Reports

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“…The effect of not fortifying formula milk with inositol is unknown. 28 l-Carnitine is another essential ingredient in formula milk; its role in ensuring growth and development in infants is controversial; yet it should be administered exogenously to maintain a normal carnitine level in infants 39 because if it is not supplied or supplied in a small amounts to infants, they will develop carnitine deficiency, 40 which can cause a lot of abnormalities in infants such as failure to thrive, recurrent infections, hypotonia, encephalopathy, cardiomyopathy, and nonketotic hypoglycemia. 41 The result of this study showed that only Dielac formula milk was not fortified with l-carnitine, whereas S26 Gold and Guigoz were fortified with sufficient amounts of l-carnitine.…”

Section: Discussionmentioning

confidence: 99%

Comparison Among Commonly Available Infant Formula Milks in the Iraqi Market

Mikhael

2015

Global Pediatric Health

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Breast-feeding is the best method of feeding infants. In some cases, formula milk can be a suitable alternative, so this study aimed to compare the safety and nutritional adequacy of commonly available formula milks in the Iraqi market. An observational study for the commonly available formula milks was conducted in the largest supermarkets of Baghdad, Iraq, during January-March 2015. The macronutrient and micronutrient contents as presented in the label of each type of formula milk was compared with the standard requirement of formula milk according to the European Society for Paediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN) guidelines. Dielac formula milk is the commonest formula milk in the Iraqi market, with the lowest price when compared with other formula milks. All infant formula milks (Similac, Guigoz, and S-26 Gold) except Dielac have the mandatory contents within the specified ranges, according to the ESPGHAN guidelines. Dielac lacks more than 1 of the major mandatory contents besides lacking all optional contents in its formula. Guigoz formula milk lacks the optional ingredients arachidonic acid, docosahexaenoic acid, and nucleotides. Similac milk was supplemented with a higher-than-specified level of nucleotides, and its l-carnitine contents were not declared. Only S26 Gold formula milk contained all mandatory and optional ingredients within the specified range, according to the ESPGHAN guidelines. In conclusion, no formula milk can resemble breast milk; however, S26 Gold formula milk is the most acceptable formula, and Dielac formula milk is the worst. Therefore, it is recommended that Dielac be withdrawn from the Iraqi market.

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“…One randomized controlled trial (RCT) proved the efficacy of FMT in treating pseudomembranous colitis with a cure rate of 94%. 2 FMT also has been performed for UC, but its effectiveness has not been as promising. A previous systematic review of FMT for inflammatory bowel disease reported a remission rate of 63%.…”

Section: Disclosurementioning

confidence: 99%

“…1 Severe metabolic decompensation can be induced by biotin deficiency (BD) or carnitine deficiency (CD) in prolonged starvation, and supplementation of these nutrients has been recommended when treating milkallergic infants with amino acids or hydrolyzed formulas alone. 2 HF, however, are available without prescription, making it possible for parents of milk-allergic infants or infants without allergies to obtain HF at their discretion.…”

mentioning

confidence: 99%

Severe hypoglycemic encephalopathy due to hypoallergenic formula in an infant

Ogawa

Ishige

Takahashi

et al. 2016

Pediatrics International

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A 7-month-old girl was brought to hospital due to vomiting. Upon admission, she was in a convulsive state and stupor with extremely low blood glucose. Head computed tomography showed brain edema, and comprehensive treatment for acute encephalopathy was initiated immediately. Severe hypoglycemia, metabolic acidosis, elevation of ammonia and serum transaminases and creatine kinase suggested metabolic decompensation. Infusion of a high-glucose solution containing vitamins, biotin, and l-carnitine resolved the metabolic crisis quickly, but brain damage was irreversible. She was found to have been fed exclusively on a hypoallergenic formula (HF) for 7 months, although she was found later to be non-allergic. Evidence of inborn metabolic diseases was absent, therefore biotin deficiency and carnitine deficiency were concluded to be a consequence of reliance on a HF for a prolonged period. Health-care professionals should warn parents of the consequences of using HF.

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Biotin and carnitine deficiency due to hypoallergenic formula nutrition in infants with milk allergy

Cited by 9 publications

References 7 publications

Nutritional status of patients with phenylketonuria in Japan

Nutritional status of patients with phenylketonuria in Japan

Comparison Among Commonly Available Infant Formula Milks in the Iraqi Market

Severe hypoglycemic encephalopathy due to hypoallergenic formula in an infant

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