WordNet

in or near a center or constituting a center; the inner area; "a central position"
a workplace that serves as a telecommunications facility where lines from telephones can be connected together to permit communication (同)telephone exchange, exchange
lacking interest or significance or impact; "an insipid personality"; "jejune novel" (同)jejune
a polygenic disease characterized by abnormally high glucose levels in the blood; any of several metabolic disorders marked by excessive urination and persistent thirst

PrepTutorEJDIC

『中心の』,中央の,中心からの / 『主要な』,中心的な(main) / (音声が)中舌音の / 電話交換局(《英》[telephone]exchange)
(飲食物が)味のない,まずい / (人・話などが)味気ない,無味乾燥な
糖尿病(尿に糖が異常に出る病気)

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2015/11/07 17:16:12」(JST)

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Neurogenic diabetes insipidus, more commonly known as central diabetes insipidus, is due to a lack of vasopressin production in the brain. Vasopressin acts to increase the volume of blood (intravascularly), and decrease the volume of urine produced. Therefore, a lack of it causes increased urine production and volume depletion.

It is also known as "neurohypophyseal diabetes insipidus".^[1]^[2] This condition has only polyuria in common with diabetes and although not mutually exclusive, with most typical cases, the name diabetes insipidus is a misleading misnomer.^[3] A better name might be "ADH-deficient polyuria".

Causes

Idiopathic

In at least 25% of cases (the most commonly occurring classification), neurogenic diabetes insipidus is idiopathic, meaning that the lack of vasopressin production arose from an unknown cause.^[4] It is also due to damage of the hypothalamus, pituitary stalk, posterior pituitary, and can arise from head trauma.

Acquired

The lack of vasopressin production usually results from some sort of damage to the pituitary gland. It may be caused due to damage to the brain caused by:

Benign suprasellar tumors (20% of cases)^[4]
Infections (encephalitis, tuberculosis etc.)
Trauma (17% of cases)^[4] or neurosurgery (9% of cases)^[4]
Non-infectious granuloma (sarcoidosis, Hand-Schuller Christian disease etc.)
Leukaemia
Autoimmune - associated with thyroiditis
Other rare causes which include hemochromatosis and histiocytosis.

Vasopressin is released by the posterior pituitary, but unlike most other pituitary hormones, vasopressin is produced in the hypothalamus. Neurogenic diabetes insipidus can be a failure of production at the hypothalamus, or a failure of release at the pituitary.^[5]

Genetic

The most rare form of central DI is familial neurogenic diabetes insipidus. This form of DI is due to an inherited mutation of the arginine vasopressin-neurophysin II (AVP-NPII) gene, inherited in an autosomal dominant manner.^[6] At one point, only 45 families worldwide were known to possess this genetic trait.^[7] It is now more widely recognized, although the precise number of people affected with this form of DI is unknown at the present time.

There is also an X-linked familial form.

Wolfram Syndrome (also called DIDMOAD) is characterised by DI, diabetes mellitus nerve deafness and optic atrophy.

Treatment

The disorder is treated with vasopressin analogs such as Desmopressin.

References

^ Chitturi S, Harris M, Thomsett MJ, et al. (December 2008). "Utility of AVP gene testing in familial neurohypophyseal diabetes insipidus". Clin. Endocrinol. (Oxf) 69 (6): 926–30. doi:10.1111/j.1365-2265.2008.03303.x. PMID 18494865.
^ Lee YW, Lee KW, Ryu JW, et al. (2008). "Mutation of Glu78 of the AVP-NPII gene impairs neurophysin as a carrier protein for arginine vasopressin in a family with neurohypophyseal diabetes insipidus". Ann. Clin. Lab. Sci. 38 (1): 12–4. PMID 18316776.
^ "The Pathogenesis of Diabetes Insipidus". Journal of the American Medical Association 49: 499. doi:10.1001/jama.1907.02530060049004.
^ ^a ^b ^c ^d "Diabetes Inspidus". Library of the National Medical Society. 2008. http://www.medical-library.org/journals4a/diabetes_insipidus.htm
^ "Central Diabetes Insipidus: Pituitary Gland Disorders: Merck Manual Home Health Handbook". Retrieved 2009-04-04.
^ "Diabetes Inspidus". The Doctor's Doctor. 2005. http://www.thedoctorsdoctor.com/diseases/diabetes_insipidus.htm
^ "Familial Neurogenic Diabetes Insipidus: a disease caused by a traffic jam?". The Diabetes Insipidus Foundation. 2006. http://diabetesinsipidus.org/4di_familial.htm

UpToDate Contents

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1. 中枢性尿崩症の治療 treatment of central diabetes insipidus
2. 中枢性尿崩症の臨床症状および原因 clinical manifestations and causes of central diabetes insipidus
3. 多尿および尿崩症の診断 diagnosis of polyuria and diabetes insipidus
4. 尿崩症における尿量 urine output in diabetes insipidus
5. 高ナトリウム血症の病因および評価 etiology and evaluation of hypernatremia

English Journal

Management and Outcome of Patients With Langerhans Cell Histiocytosis and Single-Bone CNS-Risk Lesions: A Multi-Institutional Retrospective Study.

Chellapandian D1, Shaikh F1, van den Bos C2, Somers GR3, Astigarraga I4,5, Jubran R6, Degar B7, Carret AS8, Mandel K9, Belletrutti M10, Dix D11, Visser J12, Abuhadra N1, Chang T13, Rollins B14, Whitlock J1, Weitzman S1, Abla O1.
Pediatric blood & cancer.Pediatr Blood Cancer.2015 Dec;62(12):2162-6. doi: 10.1002/pbc.25645. Epub 2015 Jul 14.
BACKGROUND: Children with Langerhans cell histiocytosis (LCH) and single-bone CNS-risk lesions have been reported to be at increased risk of diabetes insipidus (DI), central nervous system neurodegeneration (CNS-ND), and recurrence of disease. However, it is unknown whether the addition of chemother
PMID 26179251

AVP-NPII gene mutations and clinical characteristics of the patients with autosomal dominant familial central diabetes insipidus.

Turkkahraman D1, Saglar E2, Karaduman T2, Mergen H2.
Pituitary.Pituitary.2015 Dec;18(6):898-904. doi: 10.1007/s11102-015-0668-z.
BACKGROUND: Familial central diabetes insipidus (DI), usually an autosomal dominant disorder, is caused by mutations in arginine vasopressin-neurophysin II (AVP-NPII) gene that leads to aberrant preprohormone processing and gradual destruction of AVP-secreting cells.OBJECTIVE: To determine clinical
PMID 26134705

Desmopressin as an adjuvant to opioids or NSAIDs in treatment of renal colic: a nationwide register-based study.

Juul KV1, Schroeder MK2, Rittig S2, Nørgaard JP1.
Pharmacoepidemiology and drug safety.Pharmacoepidemiol Drug Saf.2015 Nov;24(11):1155-60. doi: 10.1002/pds.3875. Epub 2015 Sep 9.
AIMS: Desmopressin has been reported to be effective as an adjuvant to opioids or NSAIDs in management of pain in renal colic; however real-life data are lacking on the utilisation of desmopressin in this patient segment.METHODS: The Danish National Prescription Registry data-linked with Danish Nati
PMID 26349846

Japanese Journal

症例妊娠中期に発症し出産後も遷延した中枢性尿崩症の1例

内科 118(1), 145-148, 2016-07
NAID 40020888479

多発する中枢神経病変を認めたdisseminated juvenile xanthogranulomaの1例

日本小児血液・がん学会雑誌 53(2), 152-157, 2016
NAID 130005161928

Diabetes Insipidus as an Initial Presentation of Myelodysplastic Syndrome: Diagnosis with Single-Nucleotide Polymorphism Array-Based Karyotyping

The Tohoku Journal of Experimental Medicine 238(4), 305-310, 2016
NAID 130005145013

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リンク元	「中枢性尿崩症」「pituitary diabetes insipidus」「neurohypophyseal diabetes insipidus」
拡張検索	「idiopathic central diabetes insipidus」「partial central diabetes insipidus」
関連記事	「central」「insipid」「insipidus」「diabetes」