IntroductionWatery diarrhea, hypokalemia and achlorhydria syndrome which was described by Verner and Morrison (1) in 1958 for the first time (WDHA) is a rare cause of chronic secretory diarrhea arising from a vasoactive intestinal peptide (VIP) secreting tumor. In adults, the majority of WDHA syndrome arises from pancreatic islet cell adenoma and hyperplasia (2, 3). In contrast, it mostly arises from VIP secreting tumor originating from the neural crest cells in the adrenal medulla or sympathetic ganglia in children (4). In this article, a 15-month old female patient who could not be diagnosed despite investigations made in different centers because of watery diarrhea, severe malnutrition and abdominal distention which had been lasting for four months and who was later diagnosed with VIP secreting gangliobeuroblastoma localized in the right side was presented.
CaseA 15-month old female baby presented to our hospital because of bulky watery diarrhea approximately 10-12 times a day which had been lasting for four months. She was investigated in two centers before because of chronic diarrhea and no response was obtained with the treatments administered. The patient who had a birth weight and height at the 50 th percentile had a weight of 7 080 g (-6,1 SD) and a height of of 64 cm (-4,9 SD) at presentation. Her vital findings were within the normal limits. She looked weak and cachectic and had abdominal distension and increased pigmentation on the skin. Other physical examination findings were found to be normal (Figure 1). Laboratory tests were as follows: hemoglobin 9.6 g/dL, WBC 16 100/mm³, platelets 611 000/mm³, serum sodium 130 mmol/L, potassium 2.3 mmol/L, Chlorine 104 mmol/L. Blood gas analysis revealed metabolic acidosis. Stool osmotic gap was found to be 32 mosm/L (secretory diarrhea) and reducing substance and fat were found to be negative in stool. Stool culture and parasite examination were found to be negative. Serum glucose, renal and hepatic function
AbstractWatery diarrhea, hypokalemia and achlorhydria syndrome is a rare cause of chronic secretory diarrhea arising from a vasoactive intestinal peptide releasing tumor. In this article, a 15-month old female patient with watery diarrhea and abdominal distension which lasting four months is presented. In different centers no diagnosis could be made although investigations. The patient was diagnosed with vasoactive intestinal peptide releasing ganglioneuroblastoma localized in the right surrenal gland. (Türk Ped Arş 2014; 49: 160-2)