探讨以浮肿及低蛋白血症为主要表现的儿童嗜酸性粒细胞性胃肠炎(EGE)患儿的临床特征、治疗及转归情况。

选择2015年1月至2022年12月首都医科大学附属首都儿童医学中心诊治的以浮肿及低蛋白血症为主要表现的8例EGE患儿(患儿1~8)为研究对象。采用回顾性分析方法，收集其临床病例资料和随访结果，总结EGE患儿的临床特点。本研究经首都医科大学附属首都儿童医学中心伦理委员会批准(审批文号：SHERLLM2024010)。

本研究对8例EGE患儿的诊断、治疗、随访及转归结果如下。①男、女性各为4例，平均年龄为4岁7个月。②临床均以浮肿症状起病，包括3例单纯浮肿起病者，3例浮肿伴呕吐、腹痛、腹泻、腹胀者，1例浮肿伴面色苍白者，1例浮肿伴黑便者。③血清白蛋白水平为14.0~23.4 g/L(均较正常值降低)、球蛋白水平为9.1~14.5 g/L(均较正常值降低)。7例患儿外周血嗜酸性粒细胞(EOS)计数较正常值升高，2例血清总免疫球蛋白(Ig)E水平较正常值升高，4例低蛋白伴贫血。④影像学及内镜检查显示，5例患儿病变累及胃、十二指肠，1例累及回盲部，2例累及结肠、直肠。内镜检查结果无特异性，以组织黏膜充血肿胀、红斑、糜烂等多见，1例患儿内镜检查结果无明显异常。⑤消化道多部位活组织病理检查结果显示，胃、十二指肠、结肠、直肠黏膜慢性炎症，固有层EOS浸润。⑥对其住院期内均需补充白蛋白治疗；对7例患儿一线治疗方案选择饮食疗法，包括对5例患儿根据变态反应源检测结果采取针对性饮食回避，2例患儿回避6种常见易导致变态反应的食物，如海鲜、牛羊肉、坚果、鸡蛋及贝类等；患儿7因为年龄小，选择氨基酸配方奶粉治疗。除一线治疗外，7例患儿确诊EGE后，还加用糖皮质激素治疗。⑦所有患儿于上述治疗1~4周内，浮肿及其他消化道症状均消失，外周血EOS计数降至正常。⑧对8例患儿随访时间为12~18个月，其中7例患儿临床症状均已好转，患儿8目前随访15个月后症状仍有反复。

以浮肿、低蛋白血症为主要表现的EGE患儿，临床症状及内镜下征象缺乏特异性，胃肠黏膜组织病理学检查结果是诊断该病的关键，需多点深部取材以避免漏诊。EGE患儿对回避饮食及糖皮质激素治疗效果良好。

To summarize the clinical characteristics, treatment strategies and prognosis of pediatric eosinophilic gastroenteritis (EGE) in children presenting with edema and hypoproteinemia as the main clinical manifestations.

A total of 8 children with EGE (labeled as child 1 to child 8) presenting with edema and hypoproteinemia as the main clinical manifestations, who were diagnosed and treated at the Capital Center for Children′s Health, Capital Medical University from January 2015 to December 2022, were selected as the research subjects. Clinical medical records and follow-up data of all patients were retrospectively analyzed to summarize the clinical characteristics of EGE. This study was approved by the Ethics Committee of the Capital Center for Children′s Health, Capital Medical University (Approval No. SHERLLM2024010).

The diagnosis, treatments, follow-up and prognosis of the 8 children with EGE in this study were as follows. ① Among 8 children with EGE, 4 cases were boys and 4 cases were girls, with an average age of 4 years and 7 months. ② Every child initially presented with edema as the primary symptom: 3 cases had isolated edema; 3 cases had edema with vomiting, abdominal pain, diarrhea and bloating; 1 case had edema with pale complexion; 1 case had edema with black stools. ③ Laboratory testing showed that all patients had reduced serum albumin (14.0-23.4 g/L) and serum globulin (9.1-14.5 g/L). We also found elevated peripheral blood eosinophil (EOS) counts in 7 children, elevated total serum immunoglobulin (Ig)E in 2 children, and low protein levels with anemia in 4 children. ④ Imaging and endoscopic workups revealed that 5 children had lesions in the stomach and duodenum, 1 child in the ileocecal area, and 2 children in the colon and rectum. Endoscopic results were non-specific across the cohort, with the most common findings being mucosal congestion, swelling, redness and erosions; 1 child had no remarkable findings on endoscopy. ⑤ Biopsies taken from multiple parts of the digestive tract demonstrated chronic inflammation of the mucosa in the stomach, duodenum, colon and rectum, with EOS infiltrating the lamina propria in all cases. ⑥ All 8 children required albumin infusions during their hospital stay. For first-line treatment, 7 children were managed with dietary therapy, including 5 children followed a targeted elimination diet based on allergy testing, 2 children avoided 6 common high-allergy foods including seafood, red meat, nuts, eggs and shellfish, and child 7 was given an amino acid-based formula due to his young age. Alongside first-line treatment, 7 children were also prescribed glucocorticoids once EGE was definitively diagnosed. ⑦ Within 1 to 4 weeks of starting these treatments regimen, all children had complete resolution of edema and other gut symptoms, and their peripheral blood EOS levels dropped back to normal.⑧ We followed the 8 children for 12 to 18 months, 7 children had sustained improvement in their clinical symptoms, while child 8 continued to have symptom flares after 15 months of follow-up.

For children with EGE presenting with edema and hypoproteinemia as the main clinical manifestations, no specific clinical symptoms or endoscopic signs can be observed. The diagnosis of this disease mainly relies on the histopathological examination of the gastrointestinal mucosa, for which multiple deep biopsies from different sites should be performed to avoid missed diagnosis. Patient with this disease have a favorable therapeutic response to dietary avoidance therapy and glucocorticoid therapy.