目的 探讨恩格列净对糖原贮积病（glycogen storage disease, GSD）相关炎症性肠病（inflammatory bowel disease, IBD）的疗效及安全性。 方法 采用横断面研究的方法，纳入25例采用恩格列净治疗的GSD相关IBD患者作为研究对象。收集患者的一般资料，以及恩格列净治疗情况及不良反应等资料。比较恩格列净用药前后患者临床症状和生化指标的差异。 结果 纳入25例GSD相关IBD患者，中位确诊年龄为0.7岁，恩格列净的初始治疗年龄为（11±6）岁。恩格列净治疗的起始剂量为（0.30±0.13）mg/（kg·d），维持剂量为（0.40±0.21）mg/（kg·d），治疗时长为（34±6）个月。78%（18/23）的患者家长报告恩格列净治疗可降低感染和口腔溃疡的发作次数，增加中性粒细胞计数。临床数据显示，恩格列净治疗后厌食患者由12例减少至5例，30%的患者食欲改善（P<0.05）。治疗后腹泻、黏液便/血便、肛周病变及口腔溃疡的人数分别由19例、9例、11例和21例减少至7例、1例、0例和10例（均P<0.05）。实验室检查显示，恩格列净治疗后患者中性粒细胞绝对值较治疗前增加，血小板计数、乳酸和尿酸水平较治疗前降低（P<0.05）。7例（28%）在恩格列净治疗中发生了不良反应：2例发生在起始治疗阶段，表现为低血压或大汗、脱水，伴尿路感染，均停用恩格列净治疗；在维持治疗期间，发生尿路感染3例，低血糖2例（1例伴大汗）。 结论 恩格列净治疗可提高GSD相关IBD患者的中性粒细胞计数，降低感染和口腔溃疡的发生率，减轻腹泻和腹痛症状，增加食欲，改善血小板计数、乳酸和尿酸水平，对GSD相关IBD有明显治疗效果。尿路感染、低血糖、低血压、大汗和脱水是恩格列净治疗的可能的不良反应。

Objective To investigate the efficacy and safety of empagliflozin in patients with glycogen storage disease (GSD)-associated inflammatory bowel disease (IBD). Methods A cross-sectional study was conducted, enrolling 25 patients with GSD-associated IBD who received empagliflozin treatment. General data, details of empagliflozin use, and adverse events were collected. Clinical symptoms and biochemical parameters before and after empagliflozin therapy were compared. Results Twenty-five patients with GSD-associated IBD were included, with a median age at diagnosis of 0.7 years, and a mean age at initiation of empagliflozin therapy of (11 ± 6) years. The initial dose of empagliflozin was (0.30 ± 0.13) mg/(kg·d), with a maintenance dose of (0.40 ± 0.21) mg/(kg·d), and a treatment duration of (34 ± 6) months. Seventy-eight percent (18/23) of patients' parents reported that empagliflozin therapy reduced the frequency of infections and oral ulcers, and increased neutrophil counts. Clinically, the number of patients with anorexia decreased from 12 to 5 after treatment, and 30% showed improved appetite (P<0.05). The numbers of patients with diarrhea, mucus/bloody stools, perianal disease, and oral ulcers decreased from 19, 9, 11, and 21 before treatment to 7, 1, 0, and 10 after treatment, respectively (P<0.05). Laboratory findings showed that absolute neutrophil counts increased, while platelet counts, lactate, and uric acid levels decreased significantly after empagliflozin treatment (P<0.05). Adverse reactions occurred in 7 patients (28%) during empagliflozin treatment. Two cases occurred in the treatment initiation phase, presenting as hypotension or profuse sweating with dehydration, along with urinary tract infections (UTIs); empagliflozin was discontinued in both cases. During the maintenance phase, 3 cases of UTIs and 2 cases of hypoglycemia (one with profuse sweating) were reported. Conclusions Empagliflozin therapy can increase neutrophil counts, reduce the incidence of infections and oral ulcers, alleviate diarrhea and abdominal pain, improve appetite, and ameliorate platelet count, lactate, and uric acid levels in patients with GSD-associated IBD, demonstrating significant clinical benefit. UTIs, hypoglycemia, hypotension, profuse sweating, and dehydration may be potential adverse reactions associated with empagliflozin therapy.