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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Laron syndrome


Other Names for this Disease
  • Growth hormone insensitivity syndrome
  • Growth hormone receptor deficiency
  • Laron dwarfism
  • Laron type pituitary dwarfism I
  • Pituitary dwarfism II
More Names
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Overview



What is Laron syndrome?

How might Laron syndrome be treated?


What is Laron syndrome?

Laron syndrome is a disorder chiefly characterized by marked short stature. Signs and symptoms in the newborn period typically include low blood sugar (hypoglycemia) and having an unusually small penis (micropenis). Affected children have slow and disproportionate growth, delayed motor development due to decreased muscle mass, and delayed puberty. Other signs and symptoms include distinctive facial features (protruding and high forehead, shallow eye sockets, underdeveloped nasal bridge and small chin); delayed teething; a high-pitched voice; thin bones and skin; and decreased sweating (hypohidrosis). It is often caused by mutations in the GHR gene and is inherited in an autosomal recessive manner. Treatment focuses on improving growth and includes injections of insulin-like growth factor 1 (IGF-1) and a diet with adequate calories.[1]
Last updated: 11/3/2011

How might Laron syndrome be treated?

Treatment for individuals with Laron syndrome mainly aims at improving growth.[1] The only specific treatment available for this condition is subcutaneous injections of insulin-like growth factor 1 (a growth-promoting hormone), often called IGF-1. IGF-1 stimulates linear growth (height) and improves brain growth as well as the metabolic abnormalities caused by long-term IGF-1 deficiency. It has also been shown to raise blood glucose levels, reduce cholesterol, increase muscle growth, and give other benefits.[2] The growth resulting from IGF-1 treatment is less than what is typically seen with human growth hormone (hGH) treatment in children with growth hormone deficiency. Treatment with IGF-1 reportedly stimulates growth by about 8cm in the first year and 4-5cm in the following years,[3] but more studies on the long-term effects on final height are needed. IGF-1 and GH levels should be closely monitored in individuals undergoing this treatment, because overdosage of IGF-I causes a variety of health problems.[4] Infants may also need frequent feedings in order to avoid hypoglycemia.[1]
Last updated: 11/3/2011

References
  1. J. Léger. Laron syndrome. Orphanet. November 2009; http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=633. Accessed 11/2/2011.
  2. Zvi Laron. Growth Hormone Insensitivity (Laron Syndrome). Reviews in Endocrine & Metabolic Disorders. 2002; 3:347-355.
  3. Laron Z. The essential role of IGF-I: lessons from the long-term study and treatment of children and adults with Laron syndrome. J Clin Endocrinol Metab. December 1999; 84(12):4397-4404.
  4. Laron Z. Insulin-like growth factor-I treatment of children with Laron syndrome (primary growth hormone insensitivity). Pediatr Endocrinol Rev. March 2008; 5(3):766-771.