Growth Hormone Studies in Lysinuric Protein Intolerance

Overview

Journal Eur J Pediatr

Specialty Pediatrics

Date 1984 Feb 1

PMID 6428892

Citations 8

Authors

I Goto

T Yoshimura

Y Kuroiwa

Affiliations

Soon will be listed here.

Abstract

Our two patients with lysinuric protein intolerance, a 14-year-old boy and his 12-year-old sister, showed growth retardation and their bone ages were retarded. Growth hormone secretion responded to glucagon-propranolol and showed a good response to arginine. However, growth hormone showed little response to insulin. After the oral administration of arginine hydrochloride, growth hormone showed a good response to insulin and glucagon-propranolol, and gain in height and weight accelerated. This result may suggest that an adequate supply of arginine is effective in improving the growth retardation in lysinuric protein intolerance.

Citing Articles

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Children with lysinuric protein intolerance: Experience from a lower middle income country.

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Lysinuric protein intolerance: Pearls to detect this otherwise easily missed diagnosis.

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A global Slc7a7 knockout mouse model demonstrates characteristic phenotypes of human lysinuric protein intolerance.

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Growth Hormone Deficiency and Lysinuric Protein Intolerance: Case Report and Review of the Literature.

Evelina M, Grazia M, Francesca O, Marta C, Paolo C, Rossella G JIMD Rep. 2015; 19:35-41.

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