KEGG   DISEASE: Hypopituitarism
Entry
H01700                      Disease                                
Name
Hypopituitarism
  Subgroup
Growth hormone deficiency [DS:H00254]
Hypogonadotropic hypogonadism [DS:H00255]
Adrenocorticotropic hormone deficiency [DS:H01011]
Isolated follicle-stimulating hormone deficiency [DS:H01253]
Isolated TSH deficiency [DS:H01699]
Description
Hypopituitarism is a chronic endocrine illness, and is the partial or complete insufficiency of anterior pituitary hormone secretion. Anterior pituitary is composed of five cell types that secrete growth hormone (GH), prolactin (PRL), follicle-stimulating hormone (FSH) and luteinizing hormone (LH), thyroid stimulating hormone (TSH) and corticotropin hormone (ACTH). Clinical manifestations of hypopituitarism are variable, often insidious in onset and dependent on the degree and severity of hormone deficiency. However, it is associated with increased mortality and morbidity. A variety of diseases may cause hypopituitarism and, accordingly, this disorder can be divided into two types depending on its cause. Primary hypopituitarism is caused by disorders of the pituitary gland itself and may be due to the loss, damage, or dysfunction of pituitary hormone-secreting cells. On the other hand, secondary hypopituitarism is the result of diseases of the hypothalamus or pituitary stalk interrupting the nerve or vascular connections to the pituitary gland, thereby reducing the secretion of the pituitary hormones. Hypopituitarism can be easily diagnosed by measuring basal pituitary and target hormone levels. MR imaging of the hypothalamo-pituitary region may provide essential information. The most common causes of primary hypopituitarism are pituitary adenoma and complications from surgery or radiation therapy for the treatment of pituitary adenoma. In rare cases, hypopituitarism has been observed when the infection occurs. Sheehan's syndrome is hypopituitarism caused by the postpartum hemorrhage of the pituitary gland. In still rarer cases, solitary or complicated pituitary hormone deficiency syndromes may occur due to genetic causes and typically affect children. The treatment of hypopituitarism typically involves a replacement of the deficient hormone but care must be taken because several studies have reported an increased incidence of cardiovascular disorders and number of deaths among these patients.
Category
Endocrine disease
Brite
Human diseases [BR:br08402]
 Endocrine and metabolic diseases
  Hypothalamus and pituitary gland diseases
   H01700  Hypopituitarism
Human diseases in ICD-11 classification [BR:br08403]
 05 Endocrine, nutritional or metabolic diseases
  Endocrine diseases
   Disorders of the pituitary hormone system
    5A61  Hypofunction or certain specified disorders of pituitary gland
     H01700  Hypopituitarism
Related
pathway
hsa04080  Neuroactive ligand-receptor interaction
hsa04935  Growth hormone synthesis, secretion and action
hsa04020  Calcium signaling pathway
Gene
GHRH [HSA:2691] [KO:K05260]
TRH receptor [HSA:7201] [KO:K04282]
Other DBs
ICD-11: 5A61.0
ICD-10: E23.0
MeSH: D007018
Reference
  Authors
Ascoli P, Cavagnini F
  Title
Hypopituitarism.
  Journal
Pituitary 9:335-42 (2006)
DOI:10.1007/s11102-006-0416-5
Reference
  Authors
Reynaud R, Saveanu A, Barlier A, Enjalbert A, Brue T
  Title
Pituitary hormone deficiencies due to transcription factor gene alterations.
  Journal
Growth Horm IGF Res 14:442-8 (2004)
DOI:10.1016/j.ghir.2004.07.001
Reference
  Authors
Kelberman D, Dattani MT
  Title
Hypopituitarism oddities: congenital causes.
  Journal
Horm Res 68 Suppl 5:138-44 (2007)
DOI:10.1159/000110610
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