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Rapid changes of serum vasoactive intestinal peptide after removal of ganglioneuroblastoma with watery-diarrhea-hypokalemia-achlorhydria syndrome in a child.

A 23-month-old girl with intractable diarrhea that had persisted for the past 6 months showed typical evidence of water-diarrhea-hypokalemia-achlorhydria (WDHA) syndrome. Serum vasoactive intestinal peptide (VIP)-like immunoactivity was very high, and urine homovanillic acid and noradrenaline were also abnormally increased. A calcified tumor in the right paravertebral region was noted radiologically. Soon after resection of the tumor, the diarrhea dramatically improved. A ganglioneuroblastoma was histologically confirmed. Serum VIP decreased to normal level at 1 h after the removal of the tumor. VIP activity in the tumor was extraordinarily high, and the cells were stained by the indirect immunofluorescence technique with anti-VIP serum. This is to report the rapid turnover of serum level of VIP after resection of ganglioneuroblastoma with WDHA syndrome in a child.