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{{GS}} '''Assosciate Editor(s)-In-Chief:''' [[User: Prashanthsaddala|Prashanth Saddala M.B.B.S]]
{{GS}} '''Assosciate Editor(s)-In-Chief:''' [[User: Prashanthsaddala|Prashanth Saddala M.B.B.S]]



Revision as of 00:03, 30 July 2012

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Laron syndrome
Growth hormone
ICD-10 E34.3
ICD-9 259.4
OMIM 262500 245590
DiseasesDB 7262
MeSH D046150

Template:Search infobox

WikiDoc Resources for Laron syndrome

Articles

Most recent articles on Laron syndrome

Most cited articles on Laron syndrome

Review articles on Laron syndrome

Articles on Laron syndrome in N Eng J Med, Lancet, BMJ

Media

Powerpoint slides on Laron syndrome

Images of Laron syndrome

Photos of Laron syndrome

Podcasts & MP3s on Laron syndrome

Videos on Laron syndrome

Evidence Based Medicine

Cochrane Collaboration on Laron syndrome

Bandolier on Laron syndrome

TRIP on Laron syndrome

Clinical Trials

Ongoing Trials on Laron syndrome at Clinical Trials.gov

Trial results on Laron syndrome

Clinical Trials on Laron syndrome at Google

Guidelines / Policies / Govt

US National Guidelines Clearinghouse on Laron syndrome

NICE Guidance on Laron syndrome

NHS PRODIGY Guidance

FDA on Laron syndrome

CDC on Laron syndrome

Books

Books on Laron syndrome

News

Laron syndrome in the news

Be alerted to news on Laron syndrome

News trends on Laron syndrome

Commentary

Blogs on Laron syndrome

Definitions

Definitions of Laron syndrome

Patient Resources / Community

Patient resources on Laron syndrome

Discussion groups on Laron syndrome

Patient Handouts on Laron syndrome

Directions to Hospitals Treating Laron syndrome

Risk calculators and risk factors for Laron syndrome

Healthcare Provider Resources

Symptoms of Laron syndrome

Causes & Risk Factors for Laron syndrome

Diagnostic studies for Laron syndrome

Treatment of Laron syndrome

Continuing Medical Education (CME)

CME Programs on Laron syndrome

International

Laron syndrome en Espanol

Laron syndrome en Francais

Business

Laron syndrome in the Marketplace

Patents on Laron syndrome

Experimental / Informatics

List of terms related to Laron syndrome

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Phone:617-632-7753 Assosciate Editor(s)-In-Chief: Prashanth Saddala M.B.B.S

Synonyms and keywords: Growth Harmone Receptor Deficiency; Growth hormone insensitivity syndrome; pituitary dwarfism II; Laron syndrome due to postreceptor defect; growth hormone insensitivity due to postreceptor defect; Laron-type dwarfism

Overview

Laron syndrome is a autosomal recessive disorder characterized by an insensitivity to growth hormone (GH), caused by a variant of the growth hormone receptor. It causes short stature.

Historical perspective

It is named after Zvi Laron, the Israeli researcher who, with A. Pertzelan and S. Mannheimer, first reported the condition in 1966[1][2], based upon observations which began in 1958.[3]

Resistance to GH was first reported by Laron in 1966. Since then, severe resistance to GH, characterized by grossly impaired growth despite normal levels of GH in serum, has been termed Laron syndrome.

Homo floresiensis

Recent publications have proposed that Homo floresiensis represented a population with widespread Laron syndrome.[4][5]

Pathophysiology

Molecular genetic investigations have shown that this disorder is mainly associated with mutations in the gene for the GH receptor. These can result in defective hormone binding to the ectodomain or reduced efficiency of dimerization of the receptor after hormone occupancy. There are exceptionally low levels of insulin growth factor (IGF-1) and its principal carrier protein, insulin-like growth factor binding protein 3. Laron syndrome is a autosomal recessive disorder.

Epidemiology and Demographics

The majority of reported cases have been of Mediterranean or semitic origin, with numerous patients in Israel, Ecuador, Turkey and in the Bahamas.

Diagnosis

History and Symptoms

The principal feature of Laron syndrome is abnormally short stature (dwarfism). Physical symptoms include:

Treatment

Treatment may include administration of IGF-1.

References

  1. Template:WhoNamedIt
  2. Laron Z, Pertzelan A, Mannheimer S (1966). "Genetic pituitary dwarfism with high serum concentation of growth hormone--a new inborn error of metabolism?". Isr. J. Med. Sci. 2 (2): 152–5. PMID 5916640.
  3. Laron Z (2004). "Laron syndrome (primary growth hormone resistance or insensitivity): the personal experience 1958-2003". J. Clin. Endocrinol. Metab. 89 (3): 1031–44. PMID 15001582.
  4. Hershkovitz I, Kornreich L, Laron Z (2007). "Comparative skeletal features between Homo floresiensis and patients with primary growth hormone insensitivity (Laron syndrome)". Am. J. Phys. Anthropol. 134 (2): 198–208. doi:10.1002/ajpa.20655. PMID 17596857.
  5. Culotta E (2007). "Paleoanthropology. The fellowship of the hobbit". Science. 317 (5839): 740–2. doi:10.1126/science.317.5839.740. PMID 17690271.
  6. Laron Z, Ginsberg S, Lilos P, Arbiv M, Vaisman N (2006). "Body composition in untreated adult patients with Laron syndrome (primary GH insensitivity)". Clin. Endocrinol. (Oxf). 65 (1): 114–7. doi:10.1111/j.1365-2265.2006.02558.x. PMID 16817829.
  7. Shevah O, Kornreich L, Galatzer A, Laron Z (2005). "The intellectual capacity of patients with Laron syndrome (LS) differs with various molecular defects of the growth hormone receptor gene. Correlation with CNS abnormalities". Horm. Metab. Res. 37 (12): 757–60. doi:10.1055/s-2005-921097. PMID 16372230.

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