KEGG   DISEASE: Pituitary gigantism
Entry
H01618                      Disease                                
Name
Pituitary gigantism
  Supergrp
Excessive secretion of growth hormone [DS:H01864]
Description
Pituitary gigantism is very rare conditions resulting from excessive secretion of growth hormone (GH). Most cases are due to benign pituitary adenomas. Nonadenomatous GH excess is exceptional but occasionally occurs in patients with multiple endocrine neoplasia syndrome type 1 (MEN1), Carney complex, or McCune-Albright syndrome. The clinical manifestations may include increased growth velocity with tall stature, enlargement of the hands and feet, excessive perspiration, coarsening of facial features, and headaches. It has been reported that duplication of GPR101 probably causes gigantism and acromegaly. Therapeutic modalities for the treatment of pituitary gigantism include surgery, medication and radiation.
Category
Endocrine and metabolic disease
Brite
Human diseases in ICD-11 classification [BR:br08403]
 05 Endocrine, nutritional or metabolic diseases
  Endocrine diseases
   Disorders of the pituitary hormone system
    5A60  Hyperfunction of pituitary gland
     H01618  Pituitary gigantism
Gene
GPR101 (duplication) [HSA:83550] [KO:K08423]
Comment
See also H00247 Multiple endocrine neoplasia syndrome (MEN), H01102 Pituitary adenomas, and H01483 Acromegaly.
Other DBs
ICD-11: 5A60.0
MeSH: D005877
OMIM: 300943
Reference
  Authors
Glasker S, Vortmeyer AO, Lafferty AR, Hofman PL, Li J, Weil RJ, Zhuang Z, Oldfield EH
  Title
Hereditary pituitary hyperplasia with infantile gigantism.
  Journal
J Clin Endocrinol Metab 96:E2078-87 (2011)
DOI:10.1210/jc.2011-1401
Reference
  Authors
Hannema SE, Savendahl L
  Title
The Evaluation and Management of Tall Stature.
  Journal
Horm Res Paediatr 85:347-52 (2016)
DOI:10.1159/000443685
Reference
  Authors
Trivellin G, Daly AF, Faucz FR, Yuan B, Rostomyan L, Larco DO, Schernthaner-Reiter MH, Szarek E, Leal LF, Caberg JH, Castermans E, Villa C, Dimopoulos A, Chittiboina P, Xekouki P, Shah N, Metzger D, Lysy PA, Ferrante E, Strebkova N, Mazerkina N, Zatelli MC, Lodish M, Horvath A, de Alexandre RB, Manning AD, Levy I, Keil MF, Sierra Mde L, Palmeira L, Coppieters W, Georges M, Naves LA, Jamar M, Bours V, Wu TJ, Choong CS, Bertherat J, Chanson P, Kamenicky P, Farrell WE, Barlier A, Quezado M, Bjelobaba I, Stojilkovic SS, Wess J, Costanzi S, Liu P, Lupski JR, Beckers A, Stratakis CA
  Title
Gigantism and acromegaly due to Xq26 microduplications and GPR101 mutation.
  Journal
N Engl J Med 371:2363-74 (2014)
DOI:10.1056/NEJMoa1408028
Reference
  Authors
Eugster E
  Title
Gigantism
  Journal
Endotext (2000)
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