Beaudoin Laura, Bambic Gisela, Strasnoy Irene, García Chervo Laura, Balestracci Alejandro
Nephrology Unit, Hospital General de Niños Pedro de Elizalde, Montes de Oca 40, 1270, Autonomous City of Buenos Aires, Argentina.
Department of Nutrition and Diabetes, Hospital General de Niños Pedro de Elizalde, Montes de Oca 40, 1270, Autonomous City of Buenos Aires, Argentina.
Pediatr Nephrol. 2025 Feb;40(2):385-388. doi: 10.1007/s00467-024-06513-3. Epub 2024 Sep 9.
Exocrine pancreatic insufficiency (EPI) is an extremely rare complication of hemolytic uremic syndrome related to Shiga toxin-producing Escherichia coli (STEC-HUS) and, to our knowledge, only one patient has been reported to have received pancreatic enzyme replacement therapy (PERT). Furthermore, STEC-HUS is not usually included among EPI causes.
CASE DIAGNOSIS/TREATMENT: We report a 4-year-old girl with STEC-HUS who required dialysis and 4 days after admission developed acute pancreatitis (ACPAN) and diabetes mellitus (DM). Amylase and lipase normalized 15 days later but on the 73rd day of admission, she presented abdominal discomfort, bloating, and bulky and malodorous stools with a low fecal elastase-1 level (FE-1) of 15.74 µg/g confirming EPI diagnosis. She received 3 months of PERT until normalization of FE-1 levels.
In children with STEC-HUS with ACPAN or DM, a high index of suspicion for EPI is required, since its symptoms are often mild, nonspecific, or delayed. In addition, STEC-HUS should be further recognized as a cause of secondary EPI.
外分泌性胰腺功能不全（EPI）是溶血尿毒综合征（HUS）与产志贺毒素大肠杆菌（STEC-HUS）相关的一种极其罕见的并发症，据我们所知，仅有1例患者接受过胰酶替代疗法（PERT）。此外，STEC-HUS通常并不被列为EPI的病因之一。
病例诊断/治疗：我们报告了1例4岁患STEC-HUS的女孩，该患儿需要进行透析治疗，入院4天后并发急性胰腺炎（ACPAN）和糖尿病（DM）。淀粉酶和脂肪酶在15天后恢复正常，但在入院第73天时，她出现腹部不适、腹胀以及粪便量大且有恶臭，粪便弹性蛋白酶-1（FE-1）水平低至15.74μg/g，确诊为EPI。她接受了3个月的PERT治疗，直至FE-1水平恢复正常。
对于患有ACPAN或DM的STEC-HUS患儿，需要高度怀疑EPI，因为其症状通常较轻、不具特异性或出现延迟。此外，应进一步认识到STEC-HUS是继发性EPI的一个病因。
