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Pancreatic cholera

MedGen UID:
39692
•Concept ID:
C0086768
•
Neoplastic Process
Synonyms: Verner-Morrison syndrome; Watery diarrhea, hypokalemia, and achlorhydria syndrome; WDHA syndrome
SNOMED CT: Pancreatic cholera (39998009); Werner Morrison syndrome (39998009); Excessive vasoactive intestinal peptide secretion (39998009); Verner-Morrison syndrome (39998009); WDHA syndrome (39998009)
 
Monarch Initiative: MONDO:0004058

Definition

A rare syndrome characterized by severe watery diarrhea, hypokalemia, and achlorhydria. It is caused by the oversecretion of vasoactive intestinal peptide from the pancreatic islet cells. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPancreatic cholera

Professional guidelines

PubMed

Vasoactive intestinal peptide, the major mediator of the WDHA (pancreatic cholera) syndrome: value of measurement in diagnosis and treatment.
Bloom SR
Am J Dig Dis 1978 Apr;23(4):373-6. doi: 10.1007/BF01072425. PMID: 208414
Letter: Treatment of "pancreatic cholera".
Siegel SR, Muggia FM
N Engl J Med 1975 Jul 24;293(4):198. doi: 10.1056/NEJM197507242930414. PMID: 166314
Pancreatic cholera: beneficial effects of treatment with streptozotocin.
Kahn CR, Levy AG, Gardner JD, Miller JV, Gorden P, Schein PS
N Engl J Med 1975 May 1;292(18):941-5. doi: 10.1056/NEJM197505012921803. PMID: 163965

Recent clinical studies

Etiology

Pancreatic endocrine tumors: recent advances.
Jensen RT
Ann Oncol 1999;10 Suppl 4:170-6. PMID: 10436815

Diagnosis

VIPoma: an unsuspecting culprit of severe secretory diarrhoea in a human immunodeficiency virus-infected patient.
Vemuri KR, Balcerek MI, McGowan TE, Love A, Chapman PR
Intern Med J 2022 Jan;52(1):125-129. doi: 10.1111/imj.15655. PMID: 35060282
Pancreatic endocrine tumors: recent advances.
Jensen RT
Ann Oncol 1999;10 Suppl 4:170-6. PMID: 10436815
Chronic diarrhea of unknown origin.
Read NW, Krejs GJ, Read MG, Santa Ana CA, Morawski SG, Fordtran JS
Gastroenterology 1980 Feb;78(2):264-71. PMID: 7350049
Gastrointestinal hormones in clinical disease: recent developments.
Ann Intern Med 1979 May;90(5):817-28. doi: 10.7326/0003-4819-90-5-817. PMID: 219742
Intractable diarrhea. Intestinal perfusion studies and plasma VIP concentrations in patients with pancreatic cholera syndrome and surreptitious ingestion of laxatives and diuretics.
Krejs GJ, Walsh JH, Morawski SG, Fordtran JS
Am J Dig Dis 1977 Apr;22(4):280-92. doi: 10.1007/BF01072184. PMID: 857657

Therapy

Clinical use of the long acting somatostatin analogue octreotide in pediatrics.
Tauber MT, Harris AG, Rochiccioli P
Eur J Pediatr 1994 May;153(5):304-10. doi: 10.1007/BF01956407. PMID: 8033916
Intractable diarrhea. Intestinal perfusion studies and plasma VIP concentrations in patients with pancreatic cholera syndrome and surreptitious ingestion of laxatives and diuretics.
Krejs GJ, Walsh JH, Morawski SG, Fordtran JS
Am J Dig Dis 1977 Apr;22(4):280-92. doi: 10.1007/BF01072184. PMID: 857657
Indomethacin-responsive pancreatic cholera.
Jaffe BM, Kopen DF, DeSchryver-Kecskemeti K, Gingerich RL, Greider M
N Engl J Med 1977 Oct 13;297(15):817-21. doi: 10.1056/NEJM197710132971508. PMID: 197410
Pancreatic cholera.
Gardner JD
West J Med 1975 Oct;123(4):309-10. PMID: 173092Free PMC Article
Letter: Treatment of "pancreatic cholera".
Siegel SR, Muggia FM
N Engl J Med 1975 Jul 24;293(4):198. doi: 10.1056/NEJM197507242930414. PMID: 166314

Prognosis

Pancreatic endocrine tumors: recent advances.
Jensen RT
Ann Oncol 1999;10 Suppl 4:170-6. PMID: 10436815
Long-term survival after diagnosis of hepatic metastatic VIPoma: report of two cases with disparate courses and review of therapeutic options.
Nguyen HN, Backes B, Lammert F, Wildberger J, Winograd R, Busch N, Rieband H, Matern S
Dig Dis Sci 1999 Jun;44(6):1148-55. doi: 10.1023/a:1026672022917. PMID: 10389687

Clinical prediction guides

Cardiovascular effects of vasoactive intestinal peptide in healthy subjects.
Frase LL, Gaffney FA, Lane LD, Buckey JC, Said SI, Blomqvist CG, Krejs GJ
Am J Cardiol 1987 Dec 1;60(16):1356-61. doi: 10.1016/0002-9149(87)90619-9. PMID: 3687785
Effect of somatostatin on diarrhea and on small intestinal water and electrolyte transport in a patient with pancreatic cholera.
Ruskoné A, René E, Chayvialle JA, Bonin N, Pignal F, Kremer M, Bonfils S, Rambaud JC
Dig Dis Sci 1982 May;27(5):459-66. doi: 10.1007/BF01295657. PMID: 6122550
Effect of VIP infusion in water and ion transport in the human jejunum.
Krejs GJ, Fordtran JS, Fahrenkrug J, Schaffalitzky de Muckadell OB, Fischer JE, Humphrey CS, O'Dorisio TM, Said SI, Walsh JH, Shulkes AA
Gastroenterology 1980 Apr;78(4):722-7. PMID: 7353759
Intractable diarrhea. Intestinal perfusion studies and plasma VIP concentrations in patients with pancreatic cholera syndrome and surreptitious ingestion of laxatives and diuretics.
Krejs GJ, Walsh JH, Morawski SG, Fordtran JS
Am J Dig Dis 1977 Apr;22(4):280-92. doi: 10.1007/BF01072184. PMID: 857657
Pancreatic cholera. Sudies on tumoral secretions and pathophysiology of diarrhea.
Rambaud JC, Modigliani R, Matuchansky C, Bloom S, Said S, Pessayre D, Bernier JJ
Gastroenterology 1975 Jul;69(1):110-22. PMID: 1097288

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