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Horm Res. 1994;42(4-5):198-202.

Laron syndrome: clinical features, molecular pathology and treatment.

Author information

1
Endocrinology and Diabetes Research Unit, Children's Medical Center of Israel, Petah Tikva.

Abstract

Primary growth hormone (GH) insensitivity (Laron syndrome) is a hereditary disease due to polymorphic defects in the GH receptor, or in the postreceptor mechanisms, leading to an inability to generate IGF-1. The clinical features and biochemical profiles are indistinguishable from isolated GH deficiency. A diagnostic feature is the lack of rise of serum IGF-1 in response to GH. In most patients growth hormone binding protein is low. Treatment of children with Laron syndrome by biosynthetic IGF-1 accelerates linear growth velocity and head circumference, reduces body fat, and stimulates kidney function.

PMID:
7868073
DOI:
10.1159/000184193
[Indexed for MEDLINE]

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