98 results match your criteria WDHA Syndrome

Octreotide reverses shock due to vasoactive intestinal peptide-secreting adrenal pheochromocytoma: A case report and review of literature.

World J Clin Cases 2018 Nov;6(14):862-868

Department of Emergency Medicine, Shengjing Hospital of China Medical University, Shenyang 110004, Liaoning Province, China.

Vasoactive intestinal peptide-producing tumors (VIPoma) usually originate in the pancreas and are characterized by diarrhea, hypokalemia, and achlorhydria (WDHA syndrome). In adults, nonpancreatic VIPoma is very rare. Herein, we report an unusual case of VIP-producing pheochromocytoma marked by persistent shock, flushing, and watery diarrhea and high sensitivity to octreotide. Read More

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November 2018

Verner-Morrison syndrome. Literature review.

Rom J Morphol Embryol 2017 ;58(2):371-376

Department of Pediatric Surgery, "Victor Babes" University of Medicine and Pharmacy, Timisoara, Romania; Department of Neonatology, "Victor Babes" University of Medicine and Pharmacy, Timisoara, Romania;

Chronic diarrhea in infants is a common condition for addressability to pediatric gastroenterologists. The causes are multiple and the delay in reaching the final diagnosis can lead to complications in the general condition of the child. The purpose of this review is to present the bio-clinical and histogenetic particularities of a rare clinical entity, characterized by tumoral causes of chronic diarrhea. Read More

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April 2018
25 Reads

Biochemical Testing in Patients with Neuroendocrine Tumors.

Dan Granberg

Front Horm Res 2015 14;44:24-39. Epub 2015 Aug 14.

Department of Medical Sciences, Uppsala University, Uppsala, Sweden.

Neuroendocrine tumors are usually slow-growing tumors. Many of these are capable of secreting peptide hormones or biogenic amines that may lead to endocrine syndromes. Nonfunctioning tumors can either secrete no hormones at all, or secrete hormones not giving rise to endocrine symptoms, such as chromogranin A, chromogranin B or pancreatic polypeptide. Read More

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June 2016
13 Reads

A rare case of watery diarrhea, hypokalemia and achlorhydria syndrome caused by pheochromocytoma.

BMC Cancer 2014 Jul 31;14:553. Epub 2014 Jul 31.

Department of Endocrinology and Metabolism, Zhongshan Hospital, Fudan University, 180 Fenglin Road, Shanghai 200032, P,R, China.

Background: A rare syndrome of watery diarrhea, hypokalemia and achlorhydria (WDHA) is usually caused by pancreatic endocrine tumors that secrete excessive vasoactive intestinal polypeptide (VIP). Here we report a rare case of WDHA caused by a pheochromocytoma.

Case Presentation: A 45-year old male presented with persistent and progressive watery diarrhea for half a year, and was treated with dialysis due to azotemia, hypokalemia, hypercalcemia and metabolic acidosis. Read More

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July 2014
9 Reads

A 45-year-old female with hypokalemic rhabdomyolysis due to VIP-producing composite pheochromocytoma.

Z Gastroenterol 2012 Jun 1;50(6):589-94. Epub 2012 Jun 1.

2nd Department of Internal Medicine, HELIOS-Klinikum Erfurt, Erfurt, Germany.

The watery diarrhea, hypokalemia and achlorhydria (WHDA) syndrome due to vasoactive intestinal polypeptide (VIP)-producing extra-pancreatic tumors is rare. We report on a 45-year-old woman who suffered from persistent secretory diarrhea for six years and who was admitted to hospital with complaints of muscular weakness and myalgia. Biochemical testing revealed pronounced rhabdomyolysis due to severe hypokalemia. Read More

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June 2012
1 Read

[Verner-Morrison syndrome: a case study].

Orv Hetil 2010 Jul;151(27):1111-4

Semmelweis Egyetem, Altalános Orvostudományi Kar, I. Belgyógyászati Klinika, Budapest.

Verner and Morrison described a syndrome of watery diarrhea, hypokalemia, and achlorhydria (WDHA) in 1958. VIPomas producing high amounts of vasoactive intestinal peptide (VIP) commonly originate from the pancreas. Typical symptoms play a momentous role in the diagnosis of VIPoma. Read More

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July 2010
6 Reads

Hypokalemic rhabdomyolysis due to watery diarrhea, hypokalemia, achlorhydria (WDHA) syndrome caused by vipoma.

South Med J 2009 Jul;102(7):761-4

Department of Gastroenterology, Wright State University School of Medicine, Dayton, OH 45428, USA.

Mild hypokalemia is common and encountered in a multitude of diseases, but severe hypokalemia leading to rhabdomyolysis is relatively rare. The watery diarrhea, hypokalemia, achlorhydria (WDHA) syndrome caused by vasoactive intestinal polypeptide (VIP)-producing tumors, is an extremely rare cause of hypokalemic rhabdomyolysis and the literature is limited to one case report. We report a second case of an adult who presented with rhabdomyolysis due to severe hypokalemia. Read More

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July 2009
12 Reads
1.120 Impact Factor

VIPoma with expression of both VIP and VPAC1 receptors in a patient with WDHA syndrome.

Endocrine 2009 Apr 30;35(2):143-6. Epub 2009 Jan 30.

Department of Internal Medicine (I), Osaka Medical College, 2-7 Daigakumachi, Takatsuki City, Osaka 569-0801, Japan.

We report a case of VIPoma in a 72-year-old female patient who presented with excessive diarrhea, severe hypokalemia, and acidemia. She had been referred to our hospital three times because of severe diarrhea. No primary tumor site was found by conventional techniques, including contrast-enhanced CT and MRI, angiography, endoscopy, and positron emission tomography (PET), but a tumor was subsequently found in the head of the pancreas by octreotide scanning. Read More

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April 2009
4 Reads

Tumor with watery diarrhoea, hypokalaemia in a 3-year-old girl.

Eur J Pediatr 2009 Jul 20;168(7):859-62. Epub 2008 Dec 20.

Department of Thoracic Surgery, Beijing Army General Hospital, Beijing, China.

Watery diarrhoea, hypokalaemia and achlorhydria (WDHA) syndrome was caused by vasoactive intestinal polypeptide (VIP)-producing tumour. A 3-year-old Chinese girl with watery diarrhoea, abdominal distension and hypokalaemia due to a thoracic paraspinal VIP-secreting ganglioneuroma is reported. The girl coughed, fevering up to 39 degrees C after a flu-like episode. Read More

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July 2009
3 Reads

A Pancreatic Polypeptide-Producing Pancreatic Tumor Causing WDHA Syndrome.

Case Rep Gastroenterol 2008 Jul 9;2(2):238-43. Epub 2008 Jul 9.

Department of Surgery, Philipps University Marburg, Marburg, Germany.

We report the case of a 46-year-old female patient with WDHA (watery diarrhea/hypokalemia/achlorhydria) syndrome caused by a pancreatic polypeptide-producing tumor in the head of the pancreas. Whereas VIP and other pancreatic endocrine hormones were in the normal range, only serum levels of pancreatic polypeptide were elevated. Imaging studies identified a pancreatic tumor in the head of the gland. Read More

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July 2008
6 Reads

[Severe diarrhea associated with X-linked lissencephaly with absent corpus callosum and abnormal genitalia: a case report of successful treatment with the somatostatin analogue octreotide].

No To Hattatsu 2007 Sep;39(5):379-82

Division of Child Neurology, Institute of Neurological Sciences, Tottori University School of Medicine, Yonago, Tottori.

We report the clinical course of a case of X-linked lissencephaly with absent corpus callosum and abnormal genitalia (XLAG) exhibiting severe diarrhea. The patient demonstrated lactose intolerance and his intractable seizures were relieved with lactose-free, extensively hydrolyzed whey protein formula. At the age of 2 years while being treated with the antiallergic formula, he was affected with severe diarrhea that resembled watery diarrhea-hypokalemia-acidosis syndrome (WDHA). Read More

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September 2007
6 Reads

Watery diarrhea, hypokalemia and achlorhydria syndrome due to an adrenal pheochromocytoma.

World J Gastroenterol 2007 Sep;13(34):4649-52

Department of Surgery, Meiwa General Hospital, Agenaruo 4-31, Nishinomiya, Hyogo, Japan.

Watery diarrhea, hypokalemia and achlorhydria (WDHA) syndrome caused by vasoactive intestinal polypeptide (VIP) -producing tumor only rarely occurs in patients with nonpancreatic disease. A 49-year-old woman was referred for evaluation of a right adrenal tumor incidentally diagnosed by abdominal ultrasound during the investigation of chronic watery diarrhea. Laboratory findings showed hypokalemia and excessive production of VIP and catecholamines. Read More

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September 2007
4 Reads

Liver embolization with trisacryl gelatin microspheres (embosphere) in patients with neuroendocrine tumors.

Acta Radiol 2007 Mar;48(2):180-5

Department of Endocrine Oncology, University Hospital, Uppsala, Sweden.

Purpose: To report our experience of liver embolization with trisacryl gelatin microspheres (Embospheretrade mark) in patients with metastatic neuroendocrine tumors.

Material And Methods: Fifteen patients underwent selective embolization of the right or left hepatic artery with Embosphere. One lobe was embolized in seven patients and both lobes, on separate occasions, in eight patients. Read More

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March 2007
4 Reads

Hypokalemic rhabdomyolysis due to WDHA syndrome caused by VIP-producing composite pheochromocytoma: a case in neurofibromatosis type 1.

Jpn J Clin Oncol 2005 Sep 18;35(9):559-63. Epub 2005 Jul 18.

Department of Internal Medicine, Asahikawa City Hospital, Hokkaido, Japan.

A 47-year-old woman with neurofibromatosis type 1 suffered from general muscle weakness and watery diarrhea. Laboratory findings showed elevated muscular enzymes, severe hypokalemia and excessive production of catecholamines and vasoactive intestinal polypeptide (VIP). A computed tomography scan showed a 10 cm left adrenal mass, in which [(131)I]-metaiodobenzylguanidine scintigraphy showed high uptake. Read More

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September 2005
6 Reads

Enteroendocrine tumors other than carcinoid: a review of clinically significant advances.

Gastroenterology 2005 May;128(6):1668-84

Gastrointestinal Division, Department of Medicine, The Mount Sinai School of Medicine, New York, New York 10128, USA.

Only relatively recently has there been an increased clinical recognition and characterization of the heterogeneous group of rare gastroenteropancreatic neuroendocrine neoplasms. Most have endocrine function and exhibit varying degrees of malignancy. This review summarizes the derivation of these tumors and the advances in their diagnosis and treatment over the past decade and a half. Read More

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May 2005
1 Read

[Intractable diarrhoea revealing a neuroblastoma hypersecreting the vasoactive intestinal peptide].

Arch Pediatr 2004 Apr;11(4):340-3

Clinique de pédiatrie, hôpital Jeanne-de-Flandre, 2, avenue Oscar-Lambret, 59037 Lille, France.

In children, the watery diarrhoea-hypokalemia-achlorhydria (WDHA) syndrome is uncommon and usually due to a neuroblastic tumour hypersecreting the vasoactive intestinal peptide (VIP). We report a case of WDHA syndrome secondary to hypersecretion of VIP that revealed a neuroblastoma in a 13-month-old girl. A secretory diarrhoea, characterised by the persistence of diarrhoea despite the cessation of oral feeding, led to the search of a neuroblastic tumour in the patient. Read More

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A calcitonin and vasoactive intestinal peptide-producing pancreatic endocrine tumor associated with the WDHA syndrome.

Int J Gastrointest Cancer 2003 ;33(2-3):99-102

Hokkaido University Graduate School of Medicine, Division of Cancer Medicine, Cancer Medicine, Surgical Oncology, Sapporo, Japan.

Background: Although pancreatic endocrine tumor can produce a variety of hormones, few pancreatic tumors produce a high systemic calcitonin concentration. Furthermore, calcitonin-producing pancreatic tumors rarely produce elevations of VIP in addition.

Methods: We evaluated and treated a 50-yr-old woman with the WDHA syndrome. Read More

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[WDHA syndrome by composite pheochromocytoma and ganglioneuroma].

Nihon Naika Gakkai Zasshi 2002 May;91(5):1585-8

Department of Endocrinology, Aomori Prefecture Central Hospital.

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May 2002
3 Reads

Dynamic gadolinium-enhanced MR imaging of pancreatic VIPoma in a patient with Verner-Morrison syndrome.

Eur Radiol 2001 ;11(10):1952-5

Department of Radiology, University Hospital Gent, De Pintelaan 185, 9000 Gent, Belgium.

We describe a rare case of a pancreatic VIPoma diagnosed in a patient presenting with watery diarrhea, hypokalemia, and achlorhydria, the so-called WDHA or Verner-Morrison syndrome. Emphasis is placed on the dynamic gadolinium-enhanced MR profile of the tumor, characteristics which have not been illustrated previously, to the best of our knowledge. Read More

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[Aged watery diarrhea, hypokalemia, achlorhydria (WDHA) syndrome].

Nihon Naika Gakkai Zasshi 2001 Jul;90(7):1333-5

Department of Internal Medicine, Takeda General Hospital, Aizuwakamatu.

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[Intractable diarrhea and neuroblastoma: report of a clinical case].

Pediatr Med Chir 2000 ;22(1):47-8

Divisione e Cattedra di Chirurgia Pediatrica, Istituto Giannina Gaslini, Genova, Italia.

One of the typical presentations of neuroblastoma is intractable diarrhea or wdha (watery diarrhea, hypokalemia, achloridria). The case admitted to our Pediatric Surgery Department presented watery diarrhea due to VIP hyperincretion caused by a stage 1 neuroblastoma, whose ablation allowed a complete resolution of the clinical conditions. This case report can be useful in the discussion of the differential diagnosis of the most common clinical pictures. Read More

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October 2001

Tumor markers in neuroendocrine tumors.

Digestion 2000 ;62 Suppl 1:33-8

Section for Endocrine Oncology, Department of Medical Sciences, University Hospital, Uppsala, Sweden.

Most neuroendocrine tumors produce and secrete a multitude of peptide hormones and amines. Some of these substances cause a specific clinical syndrome: carcinoid, Zollinger-Ellison, hyperglycemic, glucagonoma and WDHA syndrome. Specific markers for these syndromes are basal and/or stimulated levels of urinary 5-HIAA, serum or plasma gastrin, insulin, glucagon and vasoactive intestinal polypeptide, respectively. Read More

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September 2000
4 Reads

Tumour markers in neuroendocrine tumours.

Ital J Gastroenterol Hepatol 1999 Oct;31 Suppl 2:S160-2

Department of Medicine, University Hospital, Uppsala, Sweden.

Most of the neuroendocrine tumours produce and secrete a large number of peptide hormones and amines. Each of these substances cause a specific clinical syndrome: carcinoid, Zollinger-Ellison, hyperglycaemic, glucagonoma and WDHA syndrome. Specific markers for these syndromes are basal and/or stimulated levels of: urinary-5-HIAA, serum or plasma gastrin, insulin, glucagon, and VIP, respectively. Read More

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October 1999
3 Reads

Pancreatic endocrine tumors: recent advances.

R T Jensen

Ann Oncol 1999 ;10 Suppl 4:170-6

Digestive Diseases Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, MD, USA.

Pancreatic endocrine tumors (PET's) can be divided on a clinical and pathologic basis into ten classes [insulinomas, gastrinomas (Zollinger-Ellison syndrome), VIPomas (Verner-Morrison syndrome, WDHA, pancreatic cholera), glucagonomas, somatostatinomas, ACTH-releasing tumors (ACTHomas), growth hormone-releasing factor secreting tumors (GRFomas), nonfunctioning or pancreatic polypeptide secreting tumors (non-functioning PET), PET's causing carcinoid syndrome and PET's causing hypercalcemia)]. Recent reports suggest calcitonin-secreting PET's also rarely occur but whether they cause a distinct clinical syndrome is unclear. PET's resemble carcinoid tumors histologically; in their ability to synthesize and frequently secrete multiple peptides such as neuroendocrine cell markers (chromogranins); their biologic behavior and their tumor growth patterns. Read More

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September 1999

Metastatic pancreatic VIPoma: deteriorating clinical course and successful treatment by liver transplantation.

Z Gastroenterol 1998 Mar;36(3):239-45

Department of Medicine B, University of Münster, Germany.

Gastrointestinal neuroendocrine tumors are slowly growing and metastases are often limited to the liver. As a result of their favorable biological behavior these tumors have a relatively good prognosis even in metastatic stage. Due to a variety of therapeutic options patients with malignant neuroendocrine tumors may survive for extended periods of time up to ten years. Read More

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March 1998
2 Reads

Somatic mutations of multiple endocrine neoplasia type 1 gene in the sporadic endocrine tumors.

Lab Invest 1998 Apr;78(4):471-5

Department of Pathology, Wakayama Medical College, Wakayama City, Japan.

Endocrine tumors of the parathyroid and pancreas are encountered either as sporadic type or as part of multiple endocrine neoplasia type 1 (MEN 1). A high frequency of the loss of heterozygosity (LOH) has been observed in tumors of the sporadic type in the locus of the MEN 1 gene, which has recently been cloned and designated the menin gene. It would be of great interest to determine whether somatic mutations in the menin gene are responsible for the sporadic endocrine tumors. Read More

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[A case report of the VIP producing tumor with liver metastasis presenting WDHA syndrome].

Nihon Shokakibyo Gakkai Zasshi 1995 Aug;92(8):1217-22

Department of Internal Medicine, Kawasaki Medical School.

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WDHA (watery diarrhea, hypokalemia, achlorhydria) syndrome: clinical features, diagnosis, and treatment.

J F Grier

South Med J 1995 Jan;88(1):22-4

Department of Medicine, Louisiana State University Medical School, Shreveport 71130.

WDHA (watery diarrhea, hypokalemia, and achlorhydria) syndrome is an unusual paraneoplastic condition caused by excess vasoactive intestinal polypeptide (VIP) secreted by certain tumors. The onset of the syndrome is insidious, and diagnosis is usually delayed by months to years. Morbidity and mortality from untreated WDHA syndrome are related to long-standing dehydration and electrolyte and acid-base disturbances resulting in chronic renal failure. Read More

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January 1995
2 Reads

[WDHA syndrome].

Y Inoue T Kaneko

Nihon Rinsho 1994 Dec;Suppl 6:65-8

Third Department of Internal Medicine, Yamaguchi University School of Medicine.

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December 1994
1 Read

[WDHA syndrome].

Y Inoue T Kaneko

Ryoikibetsu Shokogun Shirizu 1994 (6):65-8

Third Department of Internal Medicine, Yamaguchi University School of Medicine.

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[Diagnosis of Verner-Morrison syndrome (VDHA) and its treatment with sandostatin an streptozocitin].

Orv Hetil 1993 Nov;134(47):2599-602

I. Számú Belgyógyászati Klinika, Szent-Györgyi Albert Orvostudományi Egyetem, Szeged.

A case report is presented of a man with Verner-Morrison syndrome of extreme severity, caused by an unresectable pancreatic VIPoma. The pathological role of vasoactive intestinal polypeptide (VIP) is discussed in the pathogenesis of Watery Diarrhoea, Hypokalaemia, Achlorhydria (WDHA) syndrome. The authors describe the typical symptoms of the syndrome and provide a diagnostic and therapeutic strategy. Read More

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November 1993
12 Reads

Multiple-hormone gene expression in ganglioneuroblastoma with watery diarrhea, hypokalemia, and achlorhydria syndrome.

Cancer 1993 May;71(9):2841-6

Department of Pathology, Tohoku University, School of Medicine, Sendai, Japan.

Background: It has been reported that vasoactive intestinal peptide (VIP)-producing tumors accompanied by watery diarrhea, hypokalemia, and achlorhydria (WDHA) syndrome often produce multiple hormones biochemically and immunohistochemically.

Methods: The authors examined the distribution of several peptides--VIP, peptide histidine methionine (PHM), neuropeptide Y (NPY), methionine-enkephalin (M-EK), somatostatin (SS), substance-P (Sub-P), corticotropin-releasing hormone, and tyrosine hydroxylase--with immunohistochemical studies and an in situ hybridization method in three ganglioneuroblastomas with WDHA syndrome. All patients had an extremely elevated plasma level of VIP. Read More

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Giant metastatic VIPoma in the liver.

Gastroenterol Jpn 1991 Aug;26(4):530-5

Department of Internal Medicine, Kyushu Koseinenkin Hospital, Kitakyushu, Japan.

A case of giant metastatic liver VIPoma manifesting WDHA syndrome is described. The patient was a 33-year-old Japanese male who was admitted because of persisting watery diarrhea and weight loss. Laboratory examinations revealed marked hypokalemia, mild hypercalcemia and highly elevated plasma vasoactive intestinal peptide (VIP) and glucagon levels. Read More

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August 1991
1 Read

Characterization of an alpha-amidating activity in a human pancreatic tumour secreting vasoactive intestinal peptide (VIP).

Clin Endocrinol (Oxf) 1990 Oct;33(4):467-80

Laboratoire de Neuroendocrinologie Expérimentale, INSERM U 297, Faculté de Médecine Nord, Marseille, France.

A case of watery diarrhoea hypokalaemia achlorhydria (WDHA) syndrome due to a pancreatic tumour and identified by VIP plasma level, VIP immunocytochemistry, and ultrastructural analysis of tumour sections, is reported. Since VIP is the mediator of the syndrome and is biologically active under its amidated form, the enzymatic alpha-amidating activity was investigated and characterized in tumour extract; using the synthetic substrate D-Tyr-Val-Gly, the enzyme displayed an optimal activity at pH 7.0, under aerobic conditions and with 35 microM CuSO4 and 3 mM ascorbate as co-factors. Read More

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October 1990

[Vipoma of the pancreas. Apropos of a case and review of the literature].

Rev Esp Enferm Dig 1990 May;77(5):377-83

Servicio de Digestivo, Hospital Nuestra Señora de Covadonga, Oviedo.

Pancreatic endocrine tumors are uncommon; of this type of tumors, the Verner-Morrison's syndrome, WDHA or vipoma is diagnosed very rarely. The present paper is a report of a pancreatic vipoma in a 60 year-old female; she presented with watery diarrhea, facial flushing, hypokalemia, hypochlorhydria, metabolic acidosis and reversible renal failure; these are the usual manifestations of the syndrome. The diagnosis was made on the basis of radiological imaging, CAT and arteriography as well as the finding or elevated levels of vasoactive intestinal peptide (VIP). Read More

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May 1990
2 Reads

A case of the watery diarrhea-hypokalemia-achlorhydria syndrome: successful preoperative treatment of watery diarrhea with a somatostatin analogue.

Jpn J Clin Oncol 1989 Sep;19(3):294-8

Department of Internal Medicine, Jou-etsu General Hospital, Niigata.

A 35-year-old man presenting with severe watery diarrhea was diagnosed as having the watery diarrhea, hypokalemia and achlorhydria (WDHA) syndrome with the elevation of plasma vasoactive intestinal peptide (VIP) level. Imaging diagnostic techniques revealed a hypervascular tumor at the tail of the pancreas as well as a solitary liver metastasis. During the patient's stay in hospital, he developed acute renal failure probably due to persistent dehydration and severe hypokalemia. Read More

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September 1989

[Chronic diarrhea caused by VIP-secreting ganglioneuroblastoma in children. Apropos of a case with a review of the literature].

Chirurgie 1989 ;115(3):156-61; discussion 161-2

A literature review was conducted in relation to a case of chronic diarrhea associated with a VIP (vasoactive intestinal polypeptide) producing ganglioneuroblastoma (GNB), in an 18-month old female baby. This is a rare entity characterized by premonitory, persisting diarrhea, causing fluid and electrolyte changes typical of the WDHA syndrome, associating watery diarrhea, hypokalemia, and achlorhydia. Elevated VIP plasma levels are an indication for an echographic and/or CT-scan search for the causal secreting tumor. Read More

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November 1989

Neuroendocrine pancreatic tumors. Clinical findings in a prospective study of 84 patients.

Acta Oncol 1989 ;28(3):373-7

Ludwig Institute for Cancer Research, University Hospital, Uppsala, Sweden.

Endocrine pancreatic tumors are slowly growing neuroendocrine neoplasms with a malignant potential which may cause symptoms such as hypoglycemia, multiple ulcers, diarrhea, flush, hyperglycemia and skin rash. A prospective study was performed on 84 patients with endocrine pancreatic tumors. In 59 patients (70%) the tumors were malignant. Read More

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[Effect of vasoactive intestinal peptide on the motility of guinea-pig colon in vitro].

M Ishizawa

Nihon Heikatsukin Gakkai Zasshi 1988 Jun;24(3):185-92

Laboratory of Physiology, School of Allied Health Professions, Sapporo Medical College.

The effects of vasoactive intestinal peptide (VIP) on longitudinal and circular muscle strips of guinea-pig proximal and distal colons, and on propulsive activity of guinea-pig distal colon were investigated in vitro. VIP (10(-9)-10(-6) M) produced relaxations of longitudinal and circular muscle strips in proximal colon and of circular muscle strip in distal colon, but produced a contraction of longitudinal muscle strip in distal colon. VIP-induced responses of the muscle strips were not influenced by indomethacin (10(-6) M). Read More

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Successful treatment of a VIPoma by continuous subcutaneous infusion of somatostatin analogue (SMS 201-995).

Aliment Pharmacol Ther 1988 Jun;2(3):269-79

Department of Internal Medicine, Sapporo Medical College, Japan.

A case with WDHA syndrome due to VIPoma is reported. Injection of somatostatin analogue SMS 201-995 was followed by prompt suppression of vasoactive intestinal polypeptide levels (VIP), decreased stool volume, and restoration of the serum potassium concentration to normal. Long-term treatment with SMS 201-995 for up to 20 weeks produced excellent clinical control and a decrease in tumour size. Read More

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The morphology and neuroendocrine profile of pancreatic epithelial VIPomas and extrapancreatic, VIP-producing, neurogenic tumors.

Ann N Y Acad Sci 1988 ;527:508-17

Department of Pathology, University of Pavia, Italy.

The histology, histochemistry, and ultrastructure of 43 VIP-producing tumors (34 from the pancreas, one jejunal, six retroperitoneal and two mediastinic), 37 of which were associated with the WDHA syndrome, have been investigated on paraffin sections of primary or metastatic tumor tissue. The pancreatic and jejunal tumors showed all structural and secretory patterns of epithelial endocrine tumors, including expression of cytokeratin, neuroendocrine markers like neuron-specific enolase, chromogranins and synaptophysin, peptides like VIP, PHM, GRH, PP, insulin, neurotensin, glucagon, somatostatin and enkephalin, secretory granules, small clear vesicles, peculiar osmiophilic bodies, and occasional formation of tubules or microacini with specialized luminal surfaces. All the remaining tumors were neurogenic, showing either neurons and nerve fibers together with Schwann cells (ganglioneuromas and ganglioneuroblastomas) or endocrine cells (pheochromocytomas) reacting with VIP, PHM, NPY, enkephalin, somatostatin, neuron-specific enolase, synaptophysin, and MAP2 (but not cytokeratin, PP, or GRH) antibodies. Read More

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A case of watery diarrhoea syndrome due to an adrenal phaeochromocytoma secreting vasoactive intestinal polypeptide with coincidental autoimmune thyroid disease.

Acta Endocrinol (Copenh) 1987 Mar;114(3):340-4

A 40 year old woman presented with a 10 year history of watery diarrhoea and an acute quadriparesis. On clinical examination there was severe muscle weakness and a nodule was palpable in the thyroid gland. Biochemical testing revealed a hypokalaemia at 1. Read More

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A multihormonal tumor of the pancreas producing neurotensin associated with the WDHA syndrome. Histology, histochemistry and origin.

Histol Histopathol 1986 Apr;1(2):187-95

Institute of Histology and General Embryology, University of Florence, Italy.

A pancreatic tumor associated with severe WDHA syndrome has been studied histologically and immunohistochemically. Light microscopy revealed that the growth pattern of the tumor varied greatly from zone to zone but with prevailing solid arrangement of the tumoral cells. The majority of the endocrine cells showed numerous eosinophilic, PTAH-positive, and argyrophilic secretory granules, that were ultrastructurally similar to those of normal and tumoral neurotensin-containing cells. Read More

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Characterization of immunoreactive forms of pancreatic polypeptide in islet cell tumors using antisera with different regional specificities.

Cancer 1985 May;55(9):1895-8

Tumor tissue from a heterogeneous group of patients with clinically and histologically diagnosed islet cell tumors has been assessed for pancreatic polypeptide (PP) content using radioimmunoassay. Immunoreactive forms of PP, obtained by gel filtration of tissue extracts, were detected using antisera with different regional specificities. Normal pancreatic tissue contained one form of PP coeluting with human PP whereas, tumor tissue from patients with insulinoma, Zollinger-Ellison, and WDHA (watery diarrhea hypokalemic achlorhydric) syndromes contained, in addition, higher molecular weight forms of immunoreactive PP. Read More

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Pancreatic endocrine tumours associated with WDHA syndrome. An immunohistochemical and electron microscopic study.

Virchows Arch A Pathol Anat Histopathol 1985 ;405(3):311-23

Nine pancreatic endocrine tumours of patients with watery diarrhoea hypokalaemia achlorhydria (WDHA) syndrome were examined by immunohistochemistry and electron microscopy. All cases revealed neoplastic proliferation of VIP (vasoactive intestinal peptide)-immunoreactive (IR) cells. Immunoreactivity to a novel peptide hormone PHM-27, which is processed from a common big precursor peptide of VIP (prepro VIP/PHM-27), was identified in VIP-IR cells of 8 tumours. Read More

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The exocrine pancreas of patients with WDHA syndrome. A light and electron microscopical study.

J Submicrosc Cytol 1984 Jul;16(3):569-76

The exocrine pancreas of two patients with WDHA syndrome was studied by light and electron microscopy and by morphometrical methods. Compared to the controls, centroacinar and ductular cells were increased in number and richer in organelles, and acinar cells were markedly degranulated with well-developed ergastoplasm and Golgi apparatus. These findings suggest enhanced secretion of bicarbonate and enzymes in patients with WDHA syndrome. Read More

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