Postgraduate School of Pediatrics, University of Florence, Meyer Children's Hospital, Viale Pieraccini 24, 50139, Florence, Italy.
NEUROFARBA Department, University of Florence, Meyer Children's Hospital, Florence, Italy.
Eur J Pediatr. 2021 Mar;180(3):679-688. doi: 10.1007/s00431-020-03782-6. Epub 2020 Aug 15.
Paediatric Menetrier disease (PMD) is a protein-losing gastropathy, presenting with generalized oedema and abdominal symptoms. PMD commonly has an acute course and may be associated with Cytomegalovirus (CMV) infection. The aim of this retrospective study is to assess the epidemiological and clinical data, diagnostic procedures, treatment and outcome of CMV-associated PMD. The medical charts of the patients with PMD and CMV infection diagnosed at our hospital have been reviewed. Then, a systematic literature's review of all the cases of PMD and a selection of those associated with CMV infection have been performed. Three previously healthy boys were admitted for vomiting and oedema. Endoscopy showed hypertrophic gastric folds and CMV infection was diagnosed. Albumin was administered in all cases, with clinical resolution within few weeks. In literature, PMD has been described in 150 children and the association between CMV and PMD was found in 89 cases. Clinical and laboratory data, radiological and histological exams, therapy and outcome were reviewed.Conclusions: Basing on the present experience and on the current knowledge, PMD has a benign course without long-term sequelae. Although PMD is rare in children, we recommend paediatricians to consider CMV-related PMD when facing children with vomiting and diffuse oedema. What is Known: • Paediatricians should consider Menetrier disease (MD) when facing oedematous child complaining of abdominal symptoms with hypoalbuminemia, without proteinuria and liver dysfunction. • Typical ultrasound features (hypertrophic gastric folds) suggest such condition which requires endoscopy and biopsy for definitive diagnosis. What is New: • A familial susceptibility to CMV gastric infection has been recently suggested; thus when suspecting MD, the family history of gastric diseases should be investigated. • Menetrier disease has been found associated with other unusual conditions either benign (such as gastric bezoar) or malign as neoplasms (acute lymphatic leukaemia and adenocarcinoma) even in children.
小儿 Menetrier 病(PMD)是一种蛋白丢失性胃病,表现为全身性水肿和腹部症状。PMD 通常具有急性病程,可能与巨细胞病毒(CMV)感染有关。本回顾性研究旨在评估与 CMV 相关的 PMD 的流行病学和临床数据、诊断程序、治疗和结局。回顾性分析我院诊断为 PMD 并伴有 CMV 感染的患者的病历。然后,对所有 PMD 病例进行了系统的文献回顾,并选择了一些与 CMV 感染相关的病例。3 名既往健康的男孩因呕吐和水肿入院。内镜检查显示胃黏膜皱襞肥厚,诊断为 CMV 感染。所有患者均给予白蛋白治疗,数周内临床缓解。文献中已描述了 150 例小儿 PMD,发现 89 例与 CMV 相关。回顾了临床和实验室数据、影像学和组织学检查、治疗和结局。结论:基于目前的经验和现有知识,PMD 具有良性病程,无长期后遗症。虽然 PMD 在儿童中罕见,但当儿科医生遇到呕吐和弥漫性水肿的儿童时,建议考虑与 CMV 相关的 PMD。已知情况:儿科医生在遇到出现腹部症状伴低白蛋白血症、无蛋白尿和肝功能异常的水肿儿童时,应考虑 MD 。典型的超声特征(胃黏膜皱襞肥厚)提示这种情况,需要内镜和活检进行明确诊断。新情况:最近有人提出 CMV 胃感染存在家族易感性;因此,当怀疑 MD 时,应调查家族胃病史。MD 还与其他异常情况相关,良性(如胃石)或恶性(如急性淋巴细胞白血病和腺癌),甚至在儿童中也如此。