Nutritional Therapy Improves Growth and Protein Status of Children with a Urea Cycle Enzyme Defect

Phyllis B. Acosta; Steven Yannicelli; Alan S. Ryan; Georgianne Arnold; Barbara J. Marriage; Magda Plewinska; Laurie Bernstein; Joyce Fox; Vyonne Lewis; Marvin E. Miller; Antonio Velazquez

doi:10.1016/j.ymgme.2005.08.012

Nutritional Therapy Improves Growth and Protein Status of Children with a Urea Cycle Enzyme Defect

Phyllis B. Acosta
, Steven Yannicelli
, Alan S. Ryan
, Georgianne Arnold
, Barbara J. Marriage
, Magda Plewinska
, Laurie Bernstein
, Joyce Fox
, Vyonne Lewis
, Marvin E. Miller
, Antonio Velazquez

Pediatrics

Research output: Contribution to journal › Article › peer-review

Abstract

<p> <h4 id="x-x-absSec_1"> Background </h4> <p id="x-x-"> Poor growth has been described in patients with urea cycle enzyme defects treated with protein-restricted diets, while protein status is seldom reported. <h4 id="x-x-absSec_2"> Objective </h4> <p id="x-x-"> To assess the effects of nutritional therapy with a medical food on growth and protein status of patients with a urea cycle enzyme defect. <h4 id="x-x-absSec_3"> Methods </h4> <p id="x-x-"> A 6-mo multicenter outpatient study was conducted with infants and toddlers managed by nutrition therapy with Cyclinex-1 Amino Acid-Modified Medical Food with Iron (Ross Products Division, Abbott Laboratories, Columbus, OH). Main outcome variables were anthropometrics and plasma amino acids (selected), albumin, and transthyretin concentrations. <h4 id="x-x-absSec_4"> Results </h4> <p id="x-x-"> Seventeen patients completed the study. Mean (±SE) baseline age was 11.30 ± 3.20 months (median 4.40 months; range 0.22–38.84 months). Length and weight <em> z </em> -scores increased significantly during the 6-month study. Head circumference increased, but not significantly. Three patients were stunted and two were wasted (−2.0 <em> z </em> -score) at baseline while at study end, only one patient was both stunted and wasted. The majority of patients increased in length, head circumference, and weight <em> z </em> -scores during study. Mean (±SE) plasma albumin concentration increased from 34 ± 2 g/L at baseline to 38 ± 1 g/L at study end. Plasma transthyretin increased from a mean (±SE) of 177 ± 13 mg/L at baseline to 231 ± 15 mg/L at study end. No correlation was found between plasma NH <sub> 3 </sub> concentrations and medical food intake. Plasma NH <sub> 3 </sub> concentration was positively correlated with the percentage of Food and Agriculture Organization/World Health Organization/United Nations recommended protein ingested. <h4 id="x-x-absSec_5"> Conclusions </h4> <p id="x-x-"> Intakes of adequate protein and energy for age result in anabolism and linear growth without increasing plasma NH <sub> 3 </sub> concentrations. Medical food intakes did not correlate with plasma NH <sub> 3 </sub> concentrations. </p> </p> </p> </p> </p></p>

Original language	American English
Journal	Molecular Genetics and Metabolism
Volume	86
DOIs	https://doi.org/10.1016/j.ymgme.2005.08.012
State	Published - Dec 1 2005

Keywords

Growth
Nutrition Management
Protein Status
Urea Cycle Enzyme Defects

Disciplines

Medical Specialties
Medicine and Health Sciences
Pediatrics

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Cite this

@article{9463345c313349c9aa8722d5740fd191,

title = "Nutritional Therapy Improves Growth and Protein Status of Children with a Urea Cycle Enzyme Defect",

abstract = " Background Poor growth has been described in patients with urea cycle enzyme defects treated with protein-restricted diets, while protein status is seldom reported. Objective To assess the effects of nutritional therapy with a medical food on growth and protein status of patients with a urea cycle enzyme defect. Methods A 6-mo multicenter outpatient study was conducted with infants and toddlers managed by nutrition therapy with Cyclinex-1 Amino Acid-Modified Medical Food with Iron (Ross Products Division, Abbott Laboratories, Columbus, OH). Main outcome variables were anthropometrics and plasma amino acids (selected), albumin, and transthyretin concentrations. Results Seventeen patients completed the study. Mean (\±SE) baseline age was 11.30 \± 3.20 months (median 4.40 months; range 0.22\–38.84 months). Length and weight z -scores increased significantly during the 6-month study. Head circumference increased, but not significantly. Three patients were stunted and two were wasted (\−2.0 z -score) at baseline while at study end, only one patient was both stunted and wasted. The majority of patients increased in length, head circumference, and weight z -scores during study. Mean (\±SE) plasma albumin concentration increased from 34 \± 2 g/L at baseline to 38 \± 1 g/L at study end. Plasma transthyretin increased from a mean (\±SE) of 177 \± 13 mg/L at baseline to 231 \± 15 mg/L at study end. No correlation was found between plasma NH 3 concentrations and medical food intake. Plasma NH 3 concentration was positively correlated with the percentage of Food and Agriculture Organization/World Health Organization/United Nations recommended protein ingested. Conclusions Intakes of adequate protein and energy for age result in anabolism and linear growth without increasing plasma NH 3 concentrations. Medical food intakes did not correlate with plasma NH 3 concentrations. ",

keywords = "Growth, Nutrition Management, Protein Status, Urea Cycle Enzyme Defects",

author = "Acosta, \{Phyllis B.\} and Steven Yannicelli and Ryan, \{Alan S.\} and Georgianne Arnold and Marriage, \{Barbara J.\} and Magda Plewinska and Laurie Bernstein and Joyce Fox and Vyonne Lewis and Miller, \{Marvin E.\} and Antonio Velazquez",

year = "2005",

month = dec,

day = "1",

doi = "10.1016/j.ymgme.2005.08.012",

language = "American English",

volume = "86",

journal = "Molecular Genetics and Metabolism",

}

TY - JOUR

T1 - Nutritional Therapy Improves Growth and Protein Status of Children with a Urea Cycle Enzyme Defect

AU - Acosta, Phyllis B.

AU - Yannicelli, Steven

AU - Ryan, Alan S.

AU - Arnold, Georgianne

AU - Marriage, Barbara J.

AU - Plewinska, Magda

AU - Bernstein, Laurie

AU - Fox, Joyce

AU - Lewis, Vyonne

AU - Miller, Marvin E.

AU - Velazquez, Antonio

PY - 2005/12/1

Y1 - 2005/12/1

N2 - Background Poor growth has been described in patients with urea cycle enzyme defects treated with protein-restricted diets, while protein status is seldom reported. Objective To assess the effects of nutritional therapy with a medical food on growth and protein status of patients with a urea cycle enzyme defect. Methods A 6-mo multicenter outpatient study was conducted with infants and toddlers managed by nutrition therapy with Cyclinex-1 Amino Acid-Modified Medical Food with Iron (Ross Products Division, Abbott Laboratories, Columbus, OH). Main outcome variables were anthropometrics and plasma amino acids (selected), albumin, and transthyretin concentrations. Results Seventeen patients completed the study. Mean (±SE) baseline age was 11.30 ± 3.20 months (median 4.40 months; range 0.22–38.84 months). Length and weight z -scores increased significantly during the 6-month study. Head circumference increased, but not significantly. Three patients were stunted and two were wasted (−2.0 z -score) at baseline while at study end, only one patient was both stunted and wasted. The majority of patients increased in length, head circumference, and weight z -scores during study. Mean (±SE) plasma albumin concentration increased from 34 ± 2 g/L at baseline to 38 ± 1 g/L at study end. Plasma transthyretin increased from a mean (±SE) of 177 ± 13 mg/L at baseline to 231 ± 15 mg/L at study end. No correlation was found between plasma NH 3 concentrations and medical food intake. Plasma NH 3 concentration was positively correlated with the percentage of Food and Agriculture Organization/World Health Organization/United Nations recommended protein ingested. Conclusions Intakes of adequate protein and energy for age result in anabolism and linear growth without increasing plasma NH 3 concentrations. Medical food intakes did not correlate with plasma NH 3 concentrations.

AB - Background Poor growth has been described in patients with urea cycle enzyme defects treated with protein-restricted diets, while protein status is seldom reported. Objective To assess the effects of nutritional therapy with a medical food on growth and protein status of patients with a urea cycle enzyme defect. Methods A 6-mo multicenter outpatient study was conducted with infants and toddlers managed by nutrition therapy with Cyclinex-1 Amino Acid-Modified Medical Food with Iron (Ross Products Division, Abbott Laboratories, Columbus, OH). Main outcome variables were anthropometrics and plasma amino acids (selected), albumin, and transthyretin concentrations. Results Seventeen patients completed the study. Mean (±SE) baseline age was 11.30 ± 3.20 months (median 4.40 months; range 0.22–38.84 months). Length and weight z -scores increased significantly during the 6-month study. Head circumference increased, but not significantly. Three patients were stunted and two were wasted (−2.0 z -score) at baseline while at study end, only one patient was both stunted and wasted. The majority of patients increased in length, head circumference, and weight z -scores during study. Mean (±SE) plasma albumin concentration increased from 34 ± 2 g/L at baseline to 38 ± 1 g/L at study end. Plasma transthyretin increased from a mean (±SE) of 177 ± 13 mg/L at baseline to 231 ± 15 mg/L at study end. No correlation was found between plasma NH 3 concentrations and medical food intake. Plasma NH 3 concentration was positively correlated with the percentage of Food and Agriculture Organization/World Health Organization/United Nations recommended protein ingested. Conclusions Intakes of adequate protein and energy for age result in anabolism and linear growth without increasing plasma NH 3 concentrations. Medical food intakes did not correlate with plasma NH 3 concentrations.

KW - Growth

KW - Nutrition Management

KW - Protein Status

KW - Urea Cycle Enzyme Defects

UR - https://corescholar.libraries.wright.edu/pediatrics/5

U2 - 10.1016/j.ymgme.2005.08.012

DO - 10.1016/j.ymgme.2005.08.012

M3 - Article

VL - 86

JO - Molecular Genetics and Metabolism

JF - Molecular Genetics and Metabolism

ER -

Nutritional Therapy Improves Growth and Protein Status of Children with a Urea Cycle Enzyme Defect

Abstract

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