Growth and nutritional status of children with maple syrup urine disease (MSUD): A 6-months follow up study in institute of pediatric, hospital kuala lumpur (HKL)

Jian Pei Kong, Roslee Rajikan

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1 Citation (Scopus)

Abstract

Purpose – The purpose of this paper is a single-center six-month follow-up study to determine nutritional status of children with maple syrup urine disease (MSUD). Prolonged restriction on essential amino acid could cause malnutrition. By far, there is no study reported in the context of nutritional status among children with maple syrup urine disease (MSUD), who required life-long protein restriction. Design/methodology/approach – Atotal of 22 children with MSUD, aged from 1 to 12 years (6.54± 3.27) undergoing regular treatment in Institute of Pediatrics, Hospital Kuala Lumpur, were recruited. Body height, weight and head circumference were measured for anthropometry, whereas total protein, albumin and plasma branched-chain amino acid were measured for biochemical aspects. Clinical features diagnosed by pediatrician were recorded from children’s medical record. The 24-hour dietary recall was conducted to measure their nutrients intake. All assessments were repeated at six-month interval except clinical profile. Findings – There were no significant differences in all nutritional parameters from baseline to end of the visit. There was a prominence (particular noticeable of) of growth stunting (68; 64 per cent), undernutrition (35; 32 per cent) and microcephalic (57; 57 per cent) among children with MSUD. Nevertheless, children showed no significant improvement of anthropometric variables from baseline and after 6-month follow-up visit. Nearly all biochemical indicators were significantly (p < 0.05) higher than the reference value except valine. Intellectual disability was the most frequently (71 per cent) presenting symptoms among them. The finding also did not show any macro- or micronutrients fail to achieve above recommended nutrient intake in both visits. In conclusion, it is clear that no significant nutritional deficiency was induced by the use of MSUD dietary therapy; however, the findings indicated that MSUD children are at risk of malnutrition and regular nutritional assessment and monitoring should always be emphasized for optimal linear growth without affecting their amino acid profiles. Research limitations/implications – Multiple 24-hour recalls instead of single 24-hour recall should be used in this study for a better estimate of intake. Originality/value – Although there are retrospective studies targeted in presenting the clinical and biochemical profile of MSUD children which has been extensively examined, limited research has focused on prospective aspect of nutritional status of these children who are undergoing active and regular diet and medical nutrition therapy because of the absence of comprehensive reliable nutritional assessment data.

Original languageEnglish
Pages (from-to)286-301
Number of pages16
JournalNutrition and Food Science
Volume45
Issue number2
DOIs
Publication statusPublished - 9 Mar 2015

Fingerprint

maple syrup urine disease
Maple Syrup Urine Disease
Pediatric Hospitals
Nutritional Status
nutritional status
Growth
Malnutrition
malnutrition
diet therapy
Nutrition Assessment
nutrition assessment
nutrient intake
Growth Disorders
pediatricians
Nutrition Therapy
head circumference
Food
Branched Chain Amino Acids
Anthropometry
branched chain amino acids

Keywords

  • Growth
  • Hospital kuala lumpur
  • Maple syrup urine disease
  • Nutritional status

ASJC Scopus subject areas

  • Food Science
  • Nutrition and Dietetics

Cite this

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title = "Growth and nutritional status of children with maple syrup urine disease (MSUD): A 6-months follow up study in institute of pediatric, hospital kuala lumpur (HKL)",
abstract = "Purpose – The purpose of this paper is a single-center six-month follow-up study to determine nutritional status of children with maple syrup urine disease (MSUD). Prolonged restriction on essential amino acid could cause malnutrition. By far, there is no study reported in the context of nutritional status among children with maple syrup urine disease (MSUD), who required life-long protein restriction. Design/methodology/approach – Atotal of 22 children with MSUD, aged from 1 to 12 years (6.54± 3.27) undergoing regular treatment in Institute of Pediatrics, Hospital Kuala Lumpur, were recruited. Body height, weight and head circumference were measured for anthropometry, whereas total protein, albumin and plasma branched-chain amino acid were measured for biochemical aspects. Clinical features diagnosed by pediatrician were recorded from children’s medical record. The 24-hour dietary recall was conducted to measure their nutrients intake. All assessments were repeated at six-month interval except clinical profile. Findings – There were no significant differences in all nutritional parameters from baseline to end of the visit. There was a prominence (particular noticeable of) of growth stunting (68; 64 per cent), undernutrition (35; 32 per cent) and microcephalic (57; 57 per cent) among children with MSUD. Nevertheless, children showed no significant improvement of anthropometric variables from baseline and after 6-month follow-up visit. Nearly all biochemical indicators were significantly (p < 0.05) higher than the reference value except valine. Intellectual disability was the most frequently (71 per cent) presenting symptoms among them. The finding also did not show any macro- or micronutrients fail to achieve above recommended nutrient intake in both visits. In conclusion, it is clear that no significant nutritional deficiency was induced by the use of MSUD dietary therapy; however, the findings indicated that MSUD children are at risk of malnutrition and regular nutritional assessment and monitoring should always be emphasized for optimal linear growth without affecting their amino acid profiles. Research limitations/implications – Multiple 24-hour recalls instead of single 24-hour recall should be used in this study for a better estimate of intake. Originality/value – Although there are retrospective studies targeted in presenting the clinical and biochemical profile of MSUD children which has been extensively examined, limited research has focused on prospective aspect of nutritional status of these children who are undergoing active and regular diet and medical nutrition therapy because of the absence of comprehensive reliable nutritional assessment data.",
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T2 - A 6-months follow up study in institute of pediatric, hospital kuala lumpur (HKL)

AU - Kong, Jian Pei

AU - Rajikan, Roslee

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N2 - Purpose – The purpose of this paper is a single-center six-month follow-up study to determine nutritional status of children with maple syrup urine disease (MSUD). Prolonged restriction on essential amino acid could cause malnutrition. By far, there is no study reported in the context of nutritional status among children with maple syrup urine disease (MSUD), who required life-long protein restriction. Design/methodology/approach – Atotal of 22 children with MSUD, aged from 1 to 12 years (6.54± 3.27) undergoing regular treatment in Institute of Pediatrics, Hospital Kuala Lumpur, were recruited. Body height, weight and head circumference were measured for anthropometry, whereas total protein, albumin and plasma branched-chain amino acid were measured for biochemical aspects. Clinical features diagnosed by pediatrician were recorded from children’s medical record. The 24-hour dietary recall was conducted to measure their nutrients intake. All assessments were repeated at six-month interval except clinical profile. Findings – There were no significant differences in all nutritional parameters from baseline to end of the visit. There was a prominence (particular noticeable of) of growth stunting (68; 64 per cent), undernutrition (35; 32 per cent) and microcephalic (57; 57 per cent) among children with MSUD. Nevertheless, children showed no significant improvement of anthropometric variables from baseline and after 6-month follow-up visit. Nearly all biochemical indicators were significantly (p < 0.05) higher than the reference value except valine. Intellectual disability was the most frequently (71 per cent) presenting symptoms among them. The finding also did not show any macro- or micronutrients fail to achieve above recommended nutrient intake in both visits. In conclusion, it is clear that no significant nutritional deficiency was induced by the use of MSUD dietary therapy; however, the findings indicated that MSUD children are at risk of malnutrition and regular nutritional assessment and monitoring should always be emphasized for optimal linear growth without affecting their amino acid profiles. Research limitations/implications – Multiple 24-hour recalls instead of single 24-hour recall should be used in this study for a better estimate of intake. Originality/value – Although there are retrospective studies targeted in presenting the clinical and biochemical profile of MSUD children which has been extensively examined, limited research has focused on prospective aspect of nutritional status of these children who are undergoing active and regular diet and medical nutrition therapy because of the absence of comprehensive reliable nutritional assessment data.

AB - Purpose – The purpose of this paper is a single-center six-month follow-up study to determine nutritional status of children with maple syrup urine disease (MSUD). Prolonged restriction on essential amino acid could cause malnutrition. By far, there is no study reported in the context of nutritional status among children with maple syrup urine disease (MSUD), who required life-long protein restriction. Design/methodology/approach – Atotal of 22 children with MSUD, aged from 1 to 12 years (6.54± 3.27) undergoing regular treatment in Institute of Pediatrics, Hospital Kuala Lumpur, were recruited. Body height, weight and head circumference were measured for anthropometry, whereas total protein, albumin and plasma branched-chain amino acid were measured for biochemical aspects. Clinical features diagnosed by pediatrician were recorded from children’s medical record. The 24-hour dietary recall was conducted to measure their nutrients intake. All assessments were repeated at six-month interval except clinical profile. Findings – There were no significant differences in all nutritional parameters from baseline to end of the visit. There was a prominence (particular noticeable of) of growth stunting (68; 64 per cent), undernutrition (35; 32 per cent) and microcephalic (57; 57 per cent) among children with MSUD. Nevertheless, children showed no significant improvement of anthropometric variables from baseline and after 6-month follow-up visit. Nearly all biochemical indicators were significantly (p < 0.05) higher than the reference value except valine. Intellectual disability was the most frequently (71 per cent) presenting symptoms among them. The finding also did not show any macro- or micronutrients fail to achieve above recommended nutrient intake in both visits. In conclusion, it is clear that no significant nutritional deficiency was induced by the use of MSUD dietary therapy; however, the findings indicated that MSUD children are at risk of malnutrition and regular nutritional assessment and monitoring should always be emphasized for optimal linear growth without affecting their amino acid profiles. Research limitations/implications – Multiple 24-hour recalls instead of single 24-hour recall should be used in this study for a better estimate of intake. Originality/value – Although there are retrospective studies targeted in presenting the clinical and biochemical profile of MSUD children which has been extensively examined, limited research has focused on prospective aspect of nutritional status of these children who are undergoing active and regular diet and medical nutrition therapy because of the absence of comprehensive reliable nutritional assessment data.

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