Watery diarrhoea and ganglioneuroma with secretion of vasoactive intestinal peptide.

Swift, P G; Bloom, Stephen R.; Harris, F. Dee

doi:10.1136/adc.50.11.896

Cited by 77 publications

(19 citation statements)

References 7 publications

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“…To our knowledge, 63 cases of WDHA syndrome have been documented in children since the first report in 1975 by Swift et al [22]. There were 25 boys and 38 girls.…”

Section: Discussionmentioning

confidence: 91%

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

et al. 2008

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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. She had eight to ten abundant stools daily which is not improved by dietary treatment, resulting in an important weight loss. She weighed 6.8 kg (nl P50 at 6 months of age) and is 76 cm (nl P50 at 9 months of age) in height. Blood electrolytes showed 129 mmol/L sodium, 2.42 mmol/L potassium, 94 mmol/L chloride and 18.6 mmol/L bicarbonate; urinary catecholamines were normal. Computed tomography scan evidenced a left side paravertebral mass of 4 x 6 cm in the lower thoracic region leading to the blood determination of vasoactive intestinal polypeptide which amounted 830 pmol/L(normal < 25 pmol/L). Surgical removal showed a ganglioneuroma of 160 g and was associated with disappearance of the diarrhoea and normalization of VIP level below 20 pmol/L. Review of the 63 reported cases in children with WDHA showed that many of the cases presented with non-treatable watery diarrhoea, hypokalaemia. Achlorhydria is not necessarily part of the WDHA syndrome. The male to female ratio is 1:1.5. Ganglioneuroblastoma and ganglioneuroma are the commonest tumours. Location of the tumour is variable: abdomen, chest or neck. Abdominal distension, flushing, episodic hypertension and colonic dilatation, constipation and ataxia were the other associated features. Surgical resection is the treatment of choice of VIP-producing tumours.

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“…To our knowledge, 63 cases of WDHA syndrome have been documented in children since the first report in 1975 by Swift et al [22]. There were 25 boys and 38 girls.…”

Section: Discussionmentioning

confidence: 91%

“…Swift et al. [22] were the first to report a child with watery diarrhoea and ganglioneuroma with secretion of VIP in 1975. Since then, a number of cases have been reported.…”

Section: Introductionmentioning

confidence: 99%

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

et al. 2008

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“…Third, a blood pressure over the 99th centile on repeated measurements should, especially in combination with the other signs and symptoms, should remind the clinician to the possibility of a neural crest tumour. The patient in our report also presented with hypertension, but the child reported by Swift et al presented with a normal blood pressure [9]. Hypertension is however well known to be associated with neurogenic tumours in children [10].…”

Section: Discussionmentioning

confidence: 51%

VIP-Secreting Ganglioneuroblastoma as an Unusual Cause of Watery Diarrhoea in Childhood

Huysentruyt¹

2013

J Med Cases

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A 15-month-old boy with a 2-month history of watery diarrhoea presented with cachexia, hypokalaemia, hypochloraemia and metabolic acidosis. During a 2-week investigation period infection, celiac disease and cystic fibrosis were excluded and trial therapy with antibiotics, probiotics and dietary measures proved unsuccessful. This led to the suspected diagnosis of a vasoactive intestinal polypeptide (VIP)-secreting tumour with watery diarrhoea, hypokalaemia, achlorhydria (WDHA)-syndrome. MRI showed an infrarenal mass infiltrating the neuroforamen, which on pathological examination was consistent with a VIP-secreting neuroganglioblastoma. Serum VIP and urinary catecholamine levels were elevated. Treatment consisted of 2 courses of chemotherapy and a partial resection of the tumour, following which the diarrhoea disappeared. Refractory watery diarrhoea that persists during fasting in a young child should alert to the diagnosis of WDHA, and prompt investigations to diagnose a VIP-secreting tumour, one of the most common causes of secretory diarrhoea in the developed world. Timely diagnosis could avoid morbidity due to unnecessary investigations and protracted diarrhoea.

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“…Bloom et al first [4] noted the relationship between an elevated serum level of VIP and the watery diarrhea syndrome in patients with pancreatic tumors, and suggested the term pancreatic VIPoma. Swift et al [5] reported the first pediatric patient with a VIP-secreting ganglioneuroma; the tumor, in this case, was located at in the chest. Since this report, there has been numerous case reports of the WDHA syndrome caused by VIP-secreting tumors in children; the location of these tumors has been predominantly the usual location for neurogenic tumors including the adrenal glands, retroperitoneum, chest, and neck [6].…”

Section: Discussionmentioning

confidence: 78%