Laron syndrome: clinical features, molecular pathology and treatment.
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.
Laron
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Z
Endocrinology and Diabetes Research Unit, Children's Medical Center of Israel, Petah Tikva.
Klinger
B
B
D000293:Adolescent; D000328:Adult; D001827:Body Height / Q000187:drug effects; D002648:Child; D002675:Child, Preschool; D004351:Drug Resistance / Q000235:genetics; D005260:Female; D006128:Growth / Q000187:drug effects; D006130:Growth Disorders / Q000188:drug therapy / Q000235:genetics* / Q000378:metabolism; D013006:Growth Hormone / Q000172:deficiency*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007334:Insulin-Like Growth Factor I / Q000378:metabolism / Q000627:therapeutic use; D008297:Male; D011986:Receptors, Somatotropin / Q000235:genetics*; D015398:Signal Transduction / Q000235:genetics; D013577:Syndrome
D011986:Receptors, Somatotropin; D007334:Insulin-Like Growth Factor I; D013006:Growth Hormone