Pecache N, Patole S, Hagan R, Hill D, Charles A, Papadimitriou J M
Princess Margaret and King Edward Memorial Hospitals, Neonatal Clinical Care Unit, Subiaco, Western Australia.
Postgrad Med J. 2004 Feb;80(940):80-3. doi: 10.1136/pmj.2003.007930.
Congenital microvillous atrophy (CMVA) is the leading cause of neonatal secretory diarrhoea with onset either in the first 72 hours of life (early onset) or at 6-8 weeks after birth (late onset). To date over 30 cases have been reported worldwide. The prognosis for this life threatening condition continues to be poor. Therapeutic agents like somatostatin and epidermal growth factor are either ineffective or of marginal benefit. Overall five year survival after small bowel transplantation is currently approximately 50%. The following brief review is aimed towards helping neonatologists/perinatologists in the early diagnosis, and management of CMVA and in counselling the parents appropriately.
先天性微绒毛萎缩（CMVA）是新生儿分泌性腹泻的主要原因，发病时间在出生后的前72小时（早发型）或出生后6至8周（晚发型）。迄今为止，全球已报告30多例。这种危及生命的疾病的预后仍然很差。生长抑素和表皮生长因子等治疗药物要么无效，要么只有微不足道的益处。目前，小肠移植后的总体五年生存率约为50%。以下简要综述旨在帮助新生儿科医生/围产医学专家早期诊断和管理CMVA，并适当地为患儿父母提供咨询。
