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血管活性肠肽分泌肿瘤引起的慢性腹泻

Chronic Diarrhea Caused by Vasoactive Intestinal Peptide-Secreting Tumor.

作者信息

Belei Oana, Basaca Diana-Georgiana, Heredea Elena Rodica, Iacob Emil Radu, Olariu Laura, Folescu Roxana, Motoc Andrei Gheorghe Marius, Nanu Anda-Maria, Mărginean Otilia

机构信息

First Pediatric Clinic, Disturbances of Growth and Development on Children Research Center, "Victor Babeș" University of Medicine and Pharmacy, 300041 Timișoara, Romania.

Third Pediatric Clinic, "Louis Țurcanu" Emergency Hospital for Children, 300011 Timișoara, Romania.

出版信息

Life (Basel). 2023 Sep 27;13(10):1974. doi: 10.3390/life13101974.

Abstract

VIPomas are a type of neuroendocrine tumor that independently produces vasoactive intestinal peptide (VIP). VIPomas causing watery diarrhea, hypokalemia, and achlorhydria (WDHA) syndrome are not frequently observed in adult patients without pancreatic ailments. However, in children, the occurrence of a VIPoma originating in the pancreas is exceedingly uncommon. Instead, WDHA syndrome is more commonly associated with neurogenic tumors that secrete VIP, often located in the retroperitoneum or mediastinum. Among infants, chronic diarrhea is a prevalent issue that often necessitates the attention of pediatric gastroenterologists. The underlying causes are diverse, and delays in arriving at a definitive diagnosis can give rise to complications affecting the overall well-being of the child. The authors present the case of an infant with chronic watery diarrhea, subocclusion manifestations, mild hypokalemia, and metabolic hyperchloremic acidosis secondary to a VIPoma in the retroperitoneum that was diagnosed via abdominal ultrasound and tomography. The laboratory results revealed lowered potassium levels and an excessive secretion of VIP. Following the surgical removal of the tumor, the diarrhea resolved, and both electrolyte levels and the imbalanced hormone levels returned to normal. Immunohistochemical examination confirmed the diagnosis of ganglioneuroblastoma, with N-MYC negative on molecular biology tests. We present the clinical and histo-genetic aspects of this rare clinical entity, with a literature review.

摘要

血管活性肠肽瘤是一种能独立分泌血管活性肠肽(VIP)的神经内分泌肿瘤。在没有胰腺疾病的成年患者中,引起水样腹泻、低钾血症和无胃酸(WDHA)综合征的血管活性肠肽瘤并不常见。然而,在儿童中,起源于胰腺的血管活性肠肽瘤极为罕见。相反,WDHA综合征更常与分泌VIP的神经源性肿瘤相关,这些肿瘤通常位于腹膜后或纵隔。在婴儿中,慢性腹泻是一个普遍问题,常常需要儿科胃肠病学家的关注。其潜在病因多种多样,确诊延迟可能会引发影响儿童整体健康的并发症。作者报告了一例患有慢性水样腹泻、肠梗阻表现、轻度低钾血症以及因腹膜后血管活性肠肽瘤继发代谢性高氯性酸中毒的婴儿病例,该病例通过腹部超声和断层扫描得以确诊。实验室检查结果显示血钾水平降低以及VIP分泌过多。肿瘤手术切除后,腹泻症状消失,电解质水平和失衡的激素水平均恢复正常。免疫组织化学检查确诊为神经母细胞瘤,分子生物学检测显示N-MYC为阴性。我们结合文献回顾,介绍了这一罕见临床病例的临床和组织遗传学特征。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2672/10608053/33d2685c02b7/life-13-01974-g001.jpg

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