ヴェルナー・モリソン症候群、ヴァーナー・モリソン症候群、Verner-Morrison症候群

関: pancreatic cholera、VIPoma、VIPoma syndrome、watery diarrhea syndrome、watery diarrhea with hypokalemic alkalosis、WDHA

WordNet

United States suffragist in Wyoming (1814-1902) (同)Esther Morris, Esther Hobart McQuigg Slack Morris
United States statesman who led the committee that produced the final draft of the United States Constitution (1752-1816) (同)Gouverneur Morris
leader of the American Revolution who signed the Declaration of Independence and raised money for the Continental Army (1734-1806) (同)Robert Morris
English poet and craftsman (1834-1896) (同)William Morris
a pattern of symptoms indicative of some disease
a complex of concurrent things; "every word has a syndrome of meanings"
United States rock singer (1943-1971) (同)Jim Morrison, James Douglas Morrison
United States writer whose novels describe the lives of African-Americans (born in 1931) (同)Toni Morrison, Chloe Anthony Wofford
French writer who is considered the father of science fiction (1828-1905) (同)Jules Verne
Danish philologist (1846-1896) (同)Karl Adolph Verner

PrepTutorEJDIC

(疾患の徴候となる一群の)症徴候,症候群 / (事件・社会的状態などのパターンを示す)徴候形態

Wikipedia preview

出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2014/04/05 00:43:59」(JST)

wiki en

[Wiki en表示]

A VIPoma (also known as Verner Morrison syndrome, after the physicians who first described it)^[1] is a rare (1 per 10,000,000 per year) endocrine tumor,^[2] usually (about 90%) originating from non-β islet cell of the pancreas, that produce vasoactive intestinal peptide (VIP). It may be associated with multiple endocrine neoplasia type 1.

The massive amounts of VIP in turn cause profound and chronic watery diarrhea and resultant dehydration, hypokalemia, achlorhydria (hence WDHA-syndrome, or pancreatic cholera syndrome), acidosis, vasodilation (flushing and hypotension), hypercalcemia and hyperglycemia.^[3]

Symptoms and signs

The major clinical features are prolonged watery diarrhea (fasting stool volume > 750 to 1000 mL/day) and symptoms of hypokalemia and dehydration. Half of the patients have relatively constant diarrhea while the rest have alternating periods of severe and moderate diarrhea. One third have diarrhea < 1yr before diagnosis, but in 25%, diarrhea is present for 5 yr or more before diagnosis. Lethargy, muscle weakness, nausea, vomiting and crampy abdominal pain are frequent symptoms. Hypokalemia and impaired glucose tolerance occur in < 50% of patients. Achlorhydria is also a feature. During attacks of diarrhea, flushing similar to the carcinoid syndrome occur rarely.

Diagnosis

Besides the clinical picture, fasting VIP plasma level may confirm the diagnosis, and CT scan and somatostatin receptor scintigraphy are used to localise the tumor, which is usually metastatic at presentation.

Tests include:

Blood chemistry tests (basic or comprehensive metabolic panel)
CT scan of the abdomen
MRI of the abdomen
Stool examination for cause of diarrhea and electrolyte levels
Vasoactive intestinal peptide (VIP) level in the blood

Treatment

The first goal of treatment is to correct dehydration. Fluids are often given through a vein (intravenous fluids) to replace fluids lost in diarrhea.

The next goal is to slow the diarrhea. Some medications can help control diarrhea. Octreotide, which is a human-made form of the natural hormone somatostatin, blocks the action of VIP.

The best chance for a cure is surgery to remove the tumor. If the tumor has not spread to other organs, surgery can often cure the condition.

Some People seem to respond to a combination chemo called Capecitabine and Temozolomide but there is no report that it totally cured people from vipoma.

Outlook (prognosis)

Surgery can usually cure VIPomas. However, in one-third to one-half of patients, the tumor has spread by the time of diagnosis and cannot be cured.

References

^ Verner JV, Morrison AB (Sep 1958). "Islet cell tumor and a syndrome of refractory watery diarrhea and hypokalemia". Am J Med 25 (3): 374–80. doi:10.1016/0002-9343(58)90075-5. PMID 13571250.
^ "VIPoma" at Dorland's Medical Dictionary
^ Mansour JC, Chen H (Jul 2004). "Pancreatic endocrine tumors". J Surg Res 120 (1): 139–61. doi:10.1016/j.jss.2003.12.007. PMID 15172200.

Jensen RT, Norton JA. Endocrine tumors of the pancreas and gastrointestinal tract. In: Feldman M, Friedman LS, Brandt LJ, eds. Sleisenger and Fordtran's Gastrointestinal and Liver Disease . 9th ed. Philadelphia, Pa: Saunders Elsevier; 2010:chap 32.
National Cancer Institute. Islet cell tumors (pancreatic) treatment PDQ. Updated October 31, 2008.

UpToDate Contents

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1. VIP産生腫瘍症候群 the vipoma syndrome
2. 転移性の胃腸膵管系神経内分泌腫瘍：腫瘍増殖およびホルモン過剰分泌による症状を管理
するための全身治療の選択肢 metastatic gastroenteropancreatic neuroendocrine tumors systemic therapy options to control tumor growth and symptoms of hormone hypersecretion
3. 小児における慢性下痢の原因の概要 overview of the causes of chronic diarrhea in children
4. 成人の顔面紅潮に対するアプローチ approach to flushing in adults
5. 血管作動性腸管ポリペプチド vasoactive intestinal polypeptide

English Journal

Ventricular fibrillation resulting from electrolyte imbalance reveals vipoma in MEN1 syndrome.

Cavalli T1, Giudici F2, Santi R2, Nesi G2, Brandi ML2, Tonelli F2.
Familial cancer.Fam Cancer.2016 Oct;15(4):645-9. doi: 10.1007/s10689-016-9906-4.
Sporadic VIPoma is an exceedingly rare tumor with an annual incidence of 1:10 million people worldwide, yet it is described in approximately 5 % of MEN1 patients. The majority of VIPomas are malignant and radical surgery is the best therapeutic option. A 58-year-old man presented with cardiocircula
PMID 27071757

Epidemiological features of gastroenteropancreatic neuroendocrine tumors in Chengdu city with a population of 14 million based on data from a single institution.

Guo LJ1, Wang CH1, Tang CW1.
Asia-Pacific journal of clinical oncology.Asia Pac J Clin Oncol.2016 Sep;12(3):284-8. doi: 10.1111/ajco.12498. Epub 2016 May 12.
AIM: Recent studies on gastroenteropancreatic neuroendocrine tumors (GEP-NETs) in the United States as well as the European studies demonstrate an increasing GEP-NETs incidence. Most information on the epidemiology of neuroendocrine tumors comes from western countries. However, the epidemiological p
PMID 27170574

Medical management of secretory syndromes related to gastroenteropancreatic neuroendocrine tumours.

Dimitriadis GK1, Weickert MO2, Randeva HS2, Kaltsas G3, Grossman A4.
Endocrine-related cancer.Endocr Relat Cancer.2016 Sep;23(9):R423-36. doi: 10.1530/ERC-16-0200. Epub 2016 Jul 26.
Although recent epidemiological evidence indicates that the prevalence of non-functioning gastroenteropancreatic (GEP) neuroendocrine tumours (NETs) is rising, a significant number of GEP-NETs still present with symptoms related to the secretion of biologically active substances leading to the devel
PMID 27461388

Related Pictures

★リンクテーブル★

リンク元	「水様下痢低カリウム血症無胃酸症候群」「WDHA」「watery diarrhea with hypokalemic alkalosis」「ヴェルナー・モリソン症候群」「watery diarrhea syndrome」
関連記事	「Morris」「syndrome」

「水様下痢低カリウム血症無胃酸症候群」

VIPoma
	Classification and external resources
ICD-10	C25.4 or E16.8
ICD-O:	M8155/3
DiseasesDB	13877
MedlinePlus	000228
eMedicine	med/2379 med/2399 ped/2428
MeSH	D003969

　　[★]

英: watery diarrhea-hypokalemia-achlorhydria syndrome, watery diarrhea with hypokalemic alkalosis, watery diarrhea, hypokalemia and achlorhydria WDHA
同: WDHA症候群 WDHA症候群 WDHA syndrome、ヴェルナー-モリソン症候群 Verner-Morrison syndrome，膵性コレラ pancreatic cholera、VIP産生腫瘍症候群 VIPoma syndrome、VIP産生腫瘍？？？ VIPoma？？？
関: 膵島細胞腫瘍、VIP産生腫瘍 VIPoma。VIP

概念

血管作働性腸管ポリペプチド産生腫瘍(VIPoma)による症候群であり、水様下痢・低カリウム血症・無胃酸症を伴う症候群
水様性下痢 watery diarrhea　＋　低カリウム血症 hypokalemia ＋胃無酸症(無酸症) achlorhydria

-WDHA症候群

「WDHA」

　　[★] 水様下痢低カリウム血症無胃酸症候群

VIP産生腫瘍による症候群

関: pancreatic cholera、Verner-Morrison syndrome、VIPoma、VIPoma syndrome、watery diarrhea syndrome、watery diarrhea with hypokalemic alkalosis、watery diarrhea、hypokalemia and achlorhydria

「watery diarrhea with hypokalemic alkalosis」