WordNet

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
arising in or stimulated by nerve tissues

PrepTutorEJDIC

(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）』「2015/08/21 16:29:12」(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. 中枢性尿崩症の臨床症状および原因 clinical manifestations and causes of central diabetes insipidus
2. 中枢性尿崩症の治療 treatment of central diabetes insipidus
3. 多尿および尿崩症の診断 diagnosis of polyuria and diabetes insipidus
4. 腎性尿崩症の治療 treatment of nephrogenic diabetes insipidus
5. 腎性尿崩症の臨床症状および原因 clinical manifestations and causes of nephrogenic diabetes insipidus

English Journal

[Case report: IgG4-related hypophysitis presenting with secondary adrenal insufficiency and central diabetes insipidus in a type 1 diabetes patient].

Kotera N, Isogawa A, Uchida L, Ishimoto Y, Tanaka M, Tanaka S, Kishi S, Mise N, Sugimoto T, Shiba T.SourceDepartment of Medicine, Mitsui Memorial Hospital, Japan.
Nihon Naika Gakkai zasshi. The Journal of the Japanese Society of Internal Medicine.Nippon Naika Gakkai Zasshi.2011 Apr 10;100(4):1044-7.
PMID 21626842

Japanese Journal

WFS1複合ヘテロ変異を認めたWolfram症候群女性の長期予後と医療管理

藤巻理沙,岩崎直子,山本弥生,花井豪,佐藤麻子,谷澤幸生,岩本安彦
東京女子医科大学雑誌 81(E2), E241-E246, 2011-03-31
… Wolfram症候群(diabetes insipidus and mellitus with optic atrophy and deafness; …
NAID 110008441475

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リンク元	「pituitary diabetes insipidus」「neurohypophyseal diabetes insipidus」「神経性尿崩症」「神経原性尿崩症」
関連記事	「neurogenic」「insipid」「insipidus」「diabetes」