KEGG   DISEASE: 下垂体前葉機能低下症
エントリ  
H01700                                                             
名称    
下垂体前葉機能低下症
  下位グループ
成長ホルモン分泌不全症 [DS:H00254]
低ゴナドトロピン性性腺機能低下症 [DS:H00255]
ACTH単独欠損症 [DS:H01011]
卵胞刺激ホルモン単独欠損症 [DS:H01253]
TSH 単独欠損症 [DS:H01699]
概要    
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.
カテゴリ  
内分泌代謝疾患
階層分類  
ICD-11 による疾患分類 [BR:jp08403]
 05 内分泌, 栄養, 代謝の疾患
  内分泌疾患
   下垂体ホルモン系の疾患
    5A61  下垂体機能低下症またはその他の明示された下垂体の疾患
     H01700  下垂体前葉機能低下症
指定難病 [jp08407.html]
 H01700
パスウェイ 
hsa04080  Neuroactive ligand-receptor interaction
hsa04935  Growth hormone synthesis, secretion and action
hsa04020  Calcium signaling pathway
病因遺伝子 
GHRH [HSA:2691] [KO:K05260]
TRH receptor [HSA:7201] [KO:K04282]
リンク   
ICD-11: 5A61.0
MeSH: D007018
文献    
  著者
Ascoli P, Cavagnini F
  タイトル
Hypopituitarism.
  雑誌
Pituitary 9:335-42 (2006)
DOI:10.1007/s11102-006-0416-5
文献    
  著者
Reynaud R, Saveanu A, Barlier A, Enjalbert A, Brue T
  タイトル
Pituitary hormone deficiencies due to transcription factor gene alterations.
  雑誌
Growth Horm IGF Res 14:442-8 (2004)
DOI:10.1016/j.ghir.2004.07.001
文献    
  著者
Kelberman D, Dattani MT
  タイトル
Hypopituitarism oddities: congenital causes.
  雑誌
Horm Res 68 Suppl 5:138-44 (2007)
DOI:10.1159/000110610
LinkDB    

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