Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0016382 (flushing)
 6,387 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 3 year old Chinese girl with watery diarrhoea, abdominal distension and hypokalaemia due to a thoracic paraspinal vasoactive intestinal peptide (VIP) secreting ganglioneuroma is reported. The pre-operative serum VIP was 314 pmol/l (normal less than 30). Her diarrhoea stopped after the removal of the tumour. The VIP was 14 pmol/l 6 months post-operatively. Review of the 19 reported cases in children with documented elevated serum VIP showed that many of the cases presented with watery diarrhoea for prolonged duration before the diagnosis was made. Earliest age of onset was 2 weeks of age. The male to female ratio was 9:10. Ganglioneuroma and ganglioneuroblastoma were the commonest tumours. Pancreatic non-beta cell hyperplasia and neurofibroma were also reported. Location of the tumour was variable: neck, chest or abdomen. Increased urinary catecholamine excretion was reported in 50% of the cases. Abdominal distension, flushing, episodic hypertension and failure to thrive were the other associated features.
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PMID:Vasoactive intestinal peptide secreting tumours in children: a case report with literature review. 283 14