A Case of WDHA(Watery Diarrhea Hypokalemia Achiorhydria) Syndrome |
Nam Seon Beck1, Jun Jai Kim1, Beom Soo Park1, Jeong Kee Seo1, Hyo Seop Ahn1, Hyung Ro Moon1, Je Geun Chi2 |
1Department of Pediatrics, Seoul National University College of Medicine, Seoul, Korea 2Department of Pathology, Seoul National University College of Medicine, Seoul, Korea |
WDHA 증후군 1례 |
백남선1, 김중재1, 박범수1, 서정기1, 안효섭1, 문형로1, 지제근2 |
1서울대학교 의과대학 소아과학교실 2서울대학교 의과대학 병리학교실 |
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Abstract |
VIP secreting tumors are rare in children but they produce a dramatic clinical picture, the most prominent feature of which is profuse, watery diarrhea and hypokalemia. A 5-year-old girl was brought to Seoul National University Children's Hospital for evaluation of profuse watery diarrhea. She presented with watery diarrhea, hypokalemia, and stunted growth, and had experienced these problems for four years prior to admission. A retroperitoneal tumor was noted by the ultrasound, abdominal CT and MRI scanning. Surgical exploration disclosed a well defined ovoid tumor in retroperitoneal space measuring 8¡¿6¡¿4.5cm, which was proven as ganglioneuroma histologically and the immunohistochemistry study of the tumor for VIP immunoreactivity was positive. Postoperatively, the patient recovered immediately from her symptoms and catched up growth with normal height and weight. |
Key Words:
Diarrhea, WDHA syndrome, VIPoma |
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