X-linked acrogigantism

Orpha code: 300373OMIM code: 300942

Definition

A rare, genetic pituitary disease characterized by infantile-onset, rapid and excessive acceleration of linear growth and body size due to mixed growth hormone (GH)- and prolactin-secreting adenomas and/or pituitary hyperplasia. Patients present with gigantism and may have associated acromegalic features (e.g. coarse facial features, frontal bossing, prognathism, increased interdental space) as well as marked enlargement of hands and feet, soft tissue swelling, increased appetite and acanthosis nigricans.

Disease data
Classification

Disease

Synonyms
Familial infantile gigantism
Dziedziczny gigantyzm dziecięcy
Dziedziczny przerost przysadki
Gigantyzm dziecięcy z powodu hiperplazji przysadki
X-LAG (Gigantyzm kończyn sprzężony z chromosomem X)
Hereditary infantile gigantism
Hereditary pituitary hyperplasia
Infantile gigantism due to pituitary hyperplasia
X-LAG
ORPHA code
300373
OMIM code
300942
ICD10 code
E22.0
ICD11 code
-

No additional description.

Orphanet - interntowa baza danych dotyczących rzadkich chorób i sierochych leków. ©INSERM 1999 - Dostępna na stronie www.orphanet.pl