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澳門鏡湖醫院兒童激素敏感、復發／依賴腎病綜合征診療現狀的研究
Study of current diagnostic and therapeutic status of pediatric patients with steroid- sensitive, frequently relapsing / steroid-dependent nephrotic syndrome in Kiang Wu Hospital, Macao
摘要
目的：了解鏡湖醫院兒童激素敏感、復發／依賴腎病綜合征（NS）的診療現狀。方法：對2010年～2018年8月於鏡湖醫院確診為激素敏感、復發／依賴腎病綜合征，年齡≤13歲納入研究，臨床分型均為單純型。結果：初發初治11例，其中復發8例（72.7%），頻復發1例（9.1%），激素依賴2例（18.2%）。確診時尿蛋白定性均3+～4+，24小時尿蛋白定量中位數為110mg/kg，平均血漿白蛋白水平為（15.2±4.3）g/L，血膽固醇水平為（9.3±2.3）mmo l/L。初發治療為口服足量潑尼松2mg/（kg.d），尿蛋白轉陰中位時間為11天，所有患兒均於4週內轉陰，足量激素使用的中位時間為35天，激素總療程的中位數為14個月。8例復發NS患兒，經調整激素用量，尿蛋白再次轉陰時間12天，復發NS患兒3例加用左旋咪唑。頻復發／激素依賴NS患兒，2例曾予甲潑尼龍靜脈衝擊治療（18.2%），3例加用免疫抑制劑，選擇環磷酰胺1例（9.1%），選擇霉酚酸酯2例（18.2%）。結論：鏡湖醫院兒童激素敏感、復發／依賴腎病綜合征診療情況與指南基本接軌。
並列摘要
Objective: To study the current status of diagnosing and treating pediatric patients with steroid-sensitive, relapsing/steroid-dependent nephrotic syndrome (SSNS, RNS/SDNS) in Kiang Wu Hospital, Macao. Methods: We retrospectively analyzed data from 2010 to 2018. Inclusion criteria were patients older than 13 years, diagnosed with SSNS, RNS/ SDNS, clinically categorized as purely nephrotic cases. Results: A total of 11 first presenting cases, including 8 relapsing cases (72.7%), 1 frequent RNS case (9.1%) and 2 SDNS cases (18.2%), were enrolled into this study. Test results upon diagnosis were as follows 3+~4+ proteinuria, median proteinuria level 110 mg/kg for 24 hours, mean plasma albumin level 15.2±4.3 g/L and mean plasma cholesterol level 9.3±2.3 mmol/L. Patients with first episode of NS were treated with 2mg/kg/day of oral Prednisone. The median time to proteinuria remission was 11 days, with all cases reaching complete remission within 4 weeks. The mean duration of full dose corticosteroid (GC) use and total period of GC use were 35 day and 14 months, respectively. 8 RNS cases reached remission after adjusting GC dosage with a median time to remission of 12 days. Three cases received concomitant treatment with Levamisole. For the three RNS/SDNS cases, two of them had received IV methylprednisolone challenge. All 3 cases of SDNS/frequent RNS received additional treatment with immunosuppressant, with CTX in 1 (9.1%) patient and the remaining 2 (18.2%) cases received Mycophenolate mofetil (MMF). Conclusion: Diagnosis and treatment of SSNS, RNS/ SDNS in Kiang Wu hospital, Macao was in accordance with the guideline publicized by The Subspecialty Group of Nephrology, Society of Pediatrics, Chinese Medical Association in 2009.