Laron syndrome

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Laron syndrome Classification and external resources
Growth hormone
ICD-10	E34.3
ICD-9	259.4
OMIM	262500 245590
DiseasesDB	7262
eMedicine	ped/1277
MeSH	D046150

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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
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Blogs on Laron syndrome
Definitions
Definitions of Laron syndrome
Patient Resources / Community
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Healthcare Provider Resources
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Laron syndrome, or Laron-type dwarfism, 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.

Eponym

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

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.

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.

Clinical characteristics

The principal feature of Laron syndrome is abnormally short stature (dwarfism). Physical symptoms include: prominent forehead, depressed nasal bridge, under-development of mandible, truncal obesity^[1] and a very small penis. Seizures are frequently secondary to hypoglycemia. Some genetic variations have an impact upon intellectual capacity.^[1]

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

Treatment

Treatment may include administration of IGF-1.

Homo floresiensis

Recent publications have proposed that Homo floresiensis represented a population with widespread Laron syndrome.^[1][1]

References

External links

• MeSH Laron+syndrome

v • d • e Endocrine pathology: endocrine diseases (E00-35, 240-259)
Thyroid	Hypothyroidism (Iodine deficiency, Cretinism, Congenital hypothyroidism, Goitre, Myxedema) - Hyperthyroidism (Graves disease, Toxic multinodular goitre, Teratoma with thyroid tissue or Struma ovarii) - Thyroiditis (De Quervain's thyroiditis, Hashimoto's thyroiditis, Riedel's thyroiditis) - Euthyroid sick syndrome
Pancreas	Diabetes mellitus (type 1, type 2, coma, angiopathy, ketoacidosis, nephropathy, neuropathy, retinopathy) - Hypoglycemia - Hyperinsulinism - Zollinger-Ellison syndrome - insulin receptor (Rabson-Mendenhall syndrome)
Parathyroid	Hypoparathyroidism (Pseudohypoparathyroidism) - Hyperparathyroidism (Primary, Secondary, Tertiary)
Pituitary	Hyperpituitarism (Acromegaly, Hyperprolactinaemia, SIADH) - Hypopituitarism (Simmonds' disease/Sheehan's syndrome, Kallmann syndrome, Growth hormone deficiency, Diabetes insipidus) - Adiposogenital dystrophy - Empty sella syndrome
Adrenal	Cushing's syndrome (Nelson's syndrome, Pseudo-Cushing's syndrome) - CAH (Lipoid, 3β, 11β, 17α, 21α) - Hyperaldosteronism (Conn syndrome, Bartter syndrome) - Adrenal insufficiency (Addison's disease) - Hypoaldosteronism
Gonads	ovarian dysfunction (Polycystic ovary syndrome, Premature ovarian failure) - testicular dysfunction (5-alpha-reductase deficiency) - testosterone biosynthesis (17-beta-hydroxysteroid dehydrogenase deficiency) - general (Hypogonadism, Delayed puberty, Precocious puberty)
Other	Androgen insensitivity syndrome - Autoimmune polyendocrine syndrome - Carcinoid syndrome - Gigantism - Short stature (Laron syndrome, Psychogenic dwarfism) - Multiple endocrine neoplasia (1, 2) - Progeria - Woodhouse-Sakati syndrome

v • d • e WikiDoc Research Resources for Laron syndrome (Click show to right to view)
Articles on Laron syndrome	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 (Slides, Video, Images, MP3) on Laron syndrome	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 Regarding Laron syndrome	Cochrane Collaboration on Laron syndrome • Bandolier on Laron syndrome • TRIP on Laron syndrome
Cost Effectiveness of Laron syndrome	Cost Effectiveness of Laron syndrome
Clinical Trials Involving Laron syndrome	Ongoing Trials on Laron syndrome at Clinical Trials.gov • Trial results on Laron syndrome • Clinical Trials on Laron syndrome at Google
Guidelines / Policies / Government Resources (FDA/CDC) Regarding Laron syndrome	US National Guidelines Clearinghouse on Laron syndrome • NICE Guidance on Laron syndrome • NHS PRODIGY Guidance • FDA on Laron syndrome • CDC on Laron syndrome
Textbook Information on Laron syndrome	Books and Textbook Information on Laron syndrome
Pharmacology Resources on Laron syndrome	Dosing of Laron syndrome • Drug interactions with Laron syndrome • Side effects of Laron syndrome • Allergic reactions to Laron syndrome • Overdose information on Laron syndrome • Carcinogenicity information on Laron syndrome • Laron syndrome in pregnancy • Pharmacokinetics of Laron syndrome •
Genetics, Pharmacogenomics, and Proteinomics of Laron syndrome	Genetics of Laron syndrome • Pharmacogenomics of Laron syndrome • Proteomics of Laron syndrome
Newstories on Laron syndrome	Laron syndrome in the news • Be alerted to news on Laron syndrome • News trends on Laron syndrome
Commentary on Laron syndrome	Blogs on Laron syndrome
Patient Resources on Laron syndrome	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 on Laron syndrome	Symptoms of Laron syndrome • Causes & Risk Factors for Laron syndrome • Diagnostic studies for Laron syndrome • Treatment of Laron syndrome
Continuing Medical Education (CME) Programs on Laron syndrome	CME Programs on Laron syndrome
International Resources on Laron syndrome	Laron syndrome en Espanol • Laron syndrome en Francais
Business Resources on Laron syndrome	Laron syndrome in the Marketplace • Patents on Laron syndrome
Informatics Resources on Laron syndrome	List of terms related to Laron syndrome

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Acknowledgement and Attribution Regarding Sources of Content

Some of the initial content on this page may be incorporated in part from copyleft sources in the public domain including wikis such as Wikipedia and AskDrWiki. Drug information for patients came from the The National Library of Medicine. Infectious disease information may have come from the Centers for Disease Control (CDC). Differential Diagnoses are drawn from clinicians as well as an amalgamation of 3 sources: 1.The Disease Database; 2. Kahan, Scott, Smith, Ellen G. In A Page: Signs and Symptoms. Malden, Massachusetts: Blackwell Publishing, 2004:3; 3. Sailer, Christian, Wasner, Susanne. Differential Diagnosis Pocket. Hermosa Beach, CA: Borm Bruckmeir Publishing LLC, 2002:7 .