Therapeutics of Clinical Drugs 臨床藥物治療學 International Study of Kidney Disease in Children (ISKDC) % (minimal change disease, MCD) 2 (pre

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1 摘要麻豆新樓基督教醫院藥劑科藥師康建文 ( albumin 3.0 g/dl) 3.5 g ( > 200 mg/dl) (minimal change disease, MCD) childhood nephrotic syndrome minimal change disease (MCD) 壹前言 UpTodate 2013 review (hypoalbuminemia < 3.0 g/dl) (proteinuria > 50 mg/kg/day UP/UCr 3 mg) (edema ) (especially in hypercholesterolemia > 200 mg/dl) 一腎病症候群的成因 , ISOKD ( ) B ( ) (foot process) ( ) 30 2 Jun 藥學雜誌臨床藥物治療學疑似原發性腎病症候群之案例

2 Therapeutics of Clinical Drugs 臨床藥物治療學 International Study of Kidney Disease in Children (ISKDC) % (minimal change disease, MCD) 2 (prednisolone) 80% (complete remission) 3 4 圖一 The pathology of minimal change disease 二腎病症候群的預後 (MCD) ISKDC (steroid responsive) 5 ( urinary protein excretion < 4 mg/m 2 per hour or 100 mg/m 2 per day) % 16% MCD ( ) (frequent relapse) 6 4,5 表一 MCD Criteria 4 None of the following findings: hypertension, gross hematuria, and a marked elevation in serum creatinine Normal complement levels No extrarenal symptoms such as malar rash or purpura Age older than 1 year and younger than 10 years of age 70% 6 ISOKD % Cochrane database of systematic reviews 24 ( 1726 ) (RR = duration P = 0.03) 60% ( ) 33% 8 三腎病症候群的治療指引 2009 ( ) 9 prednisolone 86 THE JOURNAL OF TAIWAN PHARMACY Vol.30 No.2 Jun

3 臨床藥物治療 2 mg ( 60 mg/day) 25 (Grade 1B) ( ) 10 學 60 mg/day 表二美國 2009 年小兒腎病症候群治療共識會議 Initial therapy prednisone 2 mg/kg 1.5 mg/kg First relapse/ Prednisone 2 mg/kg Infrequent relapse 1.5 mg/kg Frequent relapses Prednisone 2 mg/kg 1.5 mg/kg mg/kg (prednisone or prednisolone) 12 (1B) (1B) 60 mg/m 2 2 mg/kg 60 mg/d (1D) 46 (1C) 40 mg/m mg/kg 40 mg/d (1D) 25 (1B) 60 mg/m 2 2 mg/kg 60 mg/d (2D) prednisone 40 mg/m mg/kg 40 mg/d (2C) prednisonee 3 (2C). frequently relapsing (FR) teroiddependent (SD) SSNS prednisone (2D) hours) (12 mg/kg/per dose) 貳案例報告 BMI ( 藥學雜誌 mg/kg ( 40 mg/day) MCNS prednisolone 60 mg/m 2 2 mg ( 80 mg/day) 40 mg/m 2 60 mg/day 4 (ISN) (KDIGO) 表三具類固醇敏感性之小兒腎病症候群臨床指引 (SSNS) Initial therapy First relapse/ Infrequent relapse Frequent relapses 四治療原則 % (< 2.5 g/dl) C3 C4 0.51g/kg over Jun

4 Therapeutics of Clinical Drugs 臨床藥物治療學 52 ) 4 ( 30 mg/ 1 30 mg/dl mg/dl mg/dl mg/dl) 1 ( > 3.5 g/ dl 386 mg/dl ( < 200 mg/dl) /min 24 /min 104/54 mmhg Scr 0.5 mg/dl KDIGO 60 mg/m 2 2 mg/kg 40 mg/m mg/kg 9/14 Prednisolone 5 mg 8# qod cimetidine albumin furosemide 49 表四病人住院期間的用藥紀錄 CaCO mg 1# tid Cimetidine 300 mg 1 tid Lovastatin 20 mg 1# qd Prednisolone 5 mg 8 bid Albumin25% 12.5 g/ml/50 ml 100 ml/ivd for 4 hrs qd 100 ml/ ivd for 4 hrs qd Furosemide 20 mg/2 ml 40 mg iv > 10 mim 40 mg iv > 10 mim Enalapril 5 mg 0.5 # qd 表五住院期間的檢驗數據的體重與體液變化 Input/0utput Alb g/dl < Chol < 200 mg/dl 386 Ca mg/dl Prot C3 (86160 mg/dl) 261 C4 (1745 mg/dl) 52.2 Bact 參討論 88 THE JOURNAL OF TAIWAN PHARMACY Vol.30 No.2 Jun

5 臨床藥物治療學疑似原發性腎病症候群之案例 PubMed Cochrane UpTodate 等搜尋相關絲球內壓力而減少蛋白尿的流失同時抑制治療準則以便探討個案用藥與治療目的腎臟發炎及纖維化臨床上使用時機當治除類固醇用藥明確外其他則以生理機轉說明療成效不佳時並用 ACEI 或 ARBs 能明顯可能的成因並記錄討論的問題改善尿蛋白的流失如案例當中當9/4加入一高血脂的成因體內的膽固醇濃度一般由肝臟調節合 Enalapril 後尿蛋白有明顯下降 (4 2) 三補體數值變化的意義成的膽固醇會以 LDL 的形式形成脂蛋白進目的為鑑別診斷發炎的原因評估是入血液當 LDL 受體數目增加時就會回否有自體免疫方面的疾病如狼瘡性腎炎慢收更多的 LDL 因此可能由於腎絲球通透性活動性肝炎或鏈球菌引起的心內膜炎及性增加導致血液中白蛋白減少造成肝臟藥物導致的腎絲球腎炎等而快速消耗補體分泌更多白蛋白同時也增加脂蛋白的數量 C3 C4 但從血清檢查中發現卻是上升造成血脂異常通常改善水腫與降低尿蛋白可能是多種炎症合併的現象則必須追蹤量血脂應能恢復正常但臨床上考量可 vital sign CBC/DC urine or blood culture 能對心血管造成損傷可選擇短效的 statins 評估可能的急性發炎或其他細菌性感染如腹不過 LDL 濃度的降低並未伴隨著蛋白尿量水引起自發性腹腔感染或凝血功能異常導致的減少及延緩腎功能的惡化因此降血脂藥血栓的可能等品仍未能被證明對腎臟病有明確的療效肆結論二使用 Enalapril 的目的慢性腎病症候群目前無法治癒因此治 ACEI 的作用機轉是在 RAAS (renin 療目標不是痊癒而是延緩病程與消除病人 angiotensin aldosterone system 腎素一血管不適 (通常即為主訴) 因此病人尿中蛋白還張力素一醛固酮) 抑制血管張力素轉化酶作未回到合理範圍1 但已降低50% (4 2) 用使 angiotensin I (血管張力素I) 無法轉換達部份緩解因此當水腫情況消失體重恢成可以強烈收縮血管的 angiotensin II (血管復到入院前的標準即已出院並後續追蹤治張力素II) 除了控制血壓外也有助於減輕腎療第 30 卷第 2 期 Jun 藥學雜誌第119冊 89

6 Therapeutics of Clinical Drugs 臨床藥物治療學A Case of Suspected Idiopathic Nephrotic Syndrome ChienWen Kang Department of Pharmacy, Madou Sinlau Christian Hospital Abstract Nephritic syndrome (NS) is a condition caused by renal diseases that increase the permeability across the glomerular filtration barrier, resulting in the loss of lots protein from blood into urine. In clinical, it is classically characterized by four features including hypoalbuminemia (serum albumin concentration < 3.0 g/dl) nephrotic range proteinuria (excretion greater than 3.5 g/day) edema and hyperlipidemia (especially cholesterol > 200 mg/dl). Primary or idiopathic nephritic syndrome (NS) is more common in younger children, particularly those less than six years of age and in them the minimal change disease (MCD) is the most common cause. In this type, the majority of childhood nephritic syndrome will respond well to steroid, however, symptomatic management is important in the early course of therapy as response to steroid therapy may take several weeks and it is the mainstay of therapy in children who fair to respond to steroid. Therefore, this paper attempts to analysis the case and explores the causes prognosis and treatment guideline about nephrotic syndrome to be a pediatric teaching plan for training pharmacy students. 參考資料 : 1. El Bakkali L, Rodrigues Pereira R, Kuik DJ, et al: Nephrotic syndrome in The Netherlands: a populationbased cohort study and a review of the literature. Pediatr Nephrol 2011; 26: Nephrotic syndrome in children: prediction of histopathology from clinical and laboratory characteristics at time of diagnosis. A report of the International Study of Kidney Disease in Children. Kidney Int 1978; 13: Kashgarian M, Hayslett JP, Siegel NJ. Lipid nephrosis and focal sclerosis distinct entities or spectrum of disease. Nephron 1974; 13: The primary nephrotic syndrome in children. Identification of patients with minimal change nephrotic syndrome from initial response to prednisone. A report of the International Study of Kidney Disease in Children. J Pediatr 1981; 98: Tarshish P, Tobin JN, Bernstein J, Edelmann CM Jr. Prognostic significance of the early course of minimal change nephrotic syndrome: report of the International Study of Kidney Disease in Children. J Am Soc Nephrol 1997; 8: Koskimies O, Vilska J, Rapola J, Hallman N. Longterm outcome of primary nephrotic syndrome. Archives of Disease in Childhood 1982;57(7): Anonymous. Minimal change nephrotic syndrome in children: deaths during the first 515 years' observation. A report of the International Study of Kidney Disease in Children. Pediatrics 1984;73(4): Hodson EM,Willis NS,Craig JC.Corticosteroid therapy for nephrotic syndrome in children. Cochrane Database of Systematic Reviews 2010, Issue Gipson DS, Massengill SF, Yao L, et al: Management of childhood onset nephrotic syndrome. Pediatrics 2009; 124: Kidney Disease International Global Outcomes of Clinical Practice Guideline for Glomerulonephritis Steroidsensitive nephrotic syndrome in children.official Journal of the International Society of Nephrology.Kidney International Supplements (2012) 2, THE JOURNAL OF TAIWAN PHARMACY Vol.30 No.2 Jun

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