Ladapo Taiwo Augustina, Esezobor Christopher Imokhuede, Lesi Foluso Ebunoluwa
Department of Pediatrics, College of Medicine, University of Lagos, PMB 12003, Idi-Araba, Lagos, Nigeria; Lagos University Teaching Hospital, PMB 12003, Idi-Araba, Lagos, Nigeria.
Pan Afr Med J. 2016 Dec 29;25:258. doi: 10.11604/pamj.2016.25.258.9802. eCollection 2016.
Children with steroid resistant nephrotic syndrome usually require treatment with second-line agents and calcineurin inhibitors such as cyclosporine are now recommended as initial therapy. These agents only recently become available in our environment and their impact on care is unknown. We reviewed the short-term treatment outcomes of their use in comparison with previous outcomes.
Medical records of children managed for idiopathic steroid resistant nephrotic syndrome over a 5 year period were reviewed. Remission rates and improvement in renal function following use of various agents were compared.
Of 103 children with idiopathic nephrotic syndrome, 25(24.3%) were steroid resistant, of whom 17 received additional medications. Full remission rate for cyclosporine was 70% (7/10). Remission rates prior to the availability of cyclosporine were 40% (2/5) for cyclophosphamide and 66% (2/3), (partial remission only) with enalapril, an angiotensin converting enzyme inhibitor used in combination with alternate day prednisolone. One child with cyclophosphamide resistance subsequently achieved remission with cyclosporine. Remission was not related to sex , age , serum albumin or hypertension but to serum cholesterol . The estimated glomerular filteration rate (eGFR) among children treated with cyclosporine ranged from 30-167 ml/min/1.73m as follows: >90 (5); 60-89 (3); 30-59 (2) while the mean pre and post treatment eGFR in those with eGFR <90 were 60 and 104ml/min/1.73m respectively . Mortality rate was 10% (1/10) in children treated with cyclosporine compared with 28.6% (2/7) in those treated with other medications .
Cyclosporine resulted in improved treatment outcomes in children with idiopathic steroid resistant nephrotic syndrome.
患有激素抵抗型肾病综合征的儿童通常需要使用二线药物进行治疗，目前推荐将环孢素等钙调神经磷酸酶抑制剂作为初始治疗药物。这些药物最近才在我们所处的环境中可用，其对治疗的影响尚不清楚。我们回顾了使用这些药物的短期治疗结果，并与之前的结果进行比较。
回顾了5年间接受特发性激素抵抗型肾病综合征治疗的儿童的病历。比较了使用各种药物后的缓解率和肾功能改善情况。
在103例特发性肾病综合征儿童中，25例（24.3%）为激素抵抗型，其中17例接受了额外的药物治疗。环孢素的完全缓解率为70%（7/10）。在环孢素可用之前，环磷酰胺的缓解率为40%（2/5），而与隔日泼尼松联合使用的血管紧张素转换酶抑制剂依那普利的缓解率为66%（2/3）（仅部分缓解）。1例对环磷酰胺耐药的儿童随后使用环孢素实现了缓解。缓解与性别、年龄、血清白蛋白或高血压无关，但与血清胆固醇有关。接受环孢素治疗的儿童的估计肾小球滤过率（eGFR）范围为30 - 167 ml/min/1.73m²，具体如下：>90（5例）；60 - 89（3例）；30 - 59（2例），而eGFR <90的儿童治疗前和治疗后的平均eGFR分别为60和104 ml/min/1.73m²。接受环孢素治疗的儿童死亡率为10%（1/10），而接受其他药物治疗的儿童死亡率为28.6%（2/7）。
环孢素改善了特发性激素抵抗型肾病综合征儿童的治疗结果。
