药物治疗Dent病15例的临床疗效

Clinical efficacy of medicinal treatment in 15 children with Dent disease

目的探讨枸橼酸钾联合氢氯噻嗪及肾素血管紧张素转换酶抑制剂（ACEI）治疗Dent病患儿的药物疗效，为Dent病的药物治疗提供临床依据。方法选取2006年7月至2014年3月北京军区总医院附属八一儿童医院随访的Dent病患儿。根据尿蛋白、尿钙及血钾水平，予枸橼酸钾联合氢氯噻嗪及ACEI类药物治疗。比较分析其治疗前后各项指标的变化。结果15例患儿均为男性；发病年龄3个月～11岁[（2．62±3．11）岁]；病程0．50～9．50（2．81±2．34）年。共随访0．50～7．50年（3．61±2．26）年。治疗后，尿钙／肌酐水平与治疗前比较差异有统计学意义[（0．41±0．19）mg／mg比（0．26±0．12）mg／mg，t=2．603，P=0．021]，24h尿钙定量差异有统计学意义[（6．76±2．05）mg／kg比（4．34±1．97）mg／kg，t=5．265，P=0．000]；24h尿蛋白定量差异无统计学意义[（0．96±0．62）g／24h比（0．87±0．44）g／24h，t=1．016，P=0．327]。肾脏超声：原有1例肾结石患儿超声显示结石消失，原有5例存在。肾脏钙化灶患儿治疗后肾脏钙化灶消失。结论枸橼酸钾联合氢氯噻嗪及ACEI类药物治疗可降低Dent患儿的尿钙水平，使肾脏钙化灶或结石消失，可能具有保护肾功能的作用。但贝那普利在降低尿蛋白水平上作用不明显，对低分子蛋白尿无明显改善。

Objective To investigate the drug effect of children with Dent disease who received therapy of po- tassium citrate and thiazide diuretics and angiotensin - converting enzyme inhibitors （ ACEI ） , and to provide the refe- rence for the clinical treatment. Methods Dent disease patient who were followed - up in Bayi Children＇s Hospital Af- filiated to Beijing Military Region General Hospital during the period of July 2006 and March 2014 were selected. The patients were administered a therapy of potassium citrate associated with thiazide diuretics and ACEI according to the level of proteinuria and calciuria and serum potassium. The underlying changes before and after the treatment were com- pared and analyzed. Results In 15 children with Dent disease, they were all male cases, onset age ranged from 3 months to 11 years old [ （2.62± 3. 11 ） years old ], and the disease duration ranged from 0.50 to 9.50 years old [ （ 2.81 ± 2.34 ） years ]. The patients were followed up for 0.50 to 7.50 years [ （ 3.61 ± 2.62） years ]. There was a sig- nificantly statistical difference in calcium/creatinine and daily Ca -creat ratio in contrast to before treatment [ （0.41 ± 0.19） mg/mg vs（0.26 ±0.12） mg/mg,t =2. 603,P =0. 021 ; （6.76 ±2.0） mg/kg vs （4.34 ± 1.97） mg/kg,t = 5. 265 ,P = 0. 000 ], there was no significantly statistical difference in 24 -hour urinary protein quantity in contrast to before treatment [ （0.96 ± 0.62 ） g/24 h vs （0.87±0.44 ） g/24 h, t = 1.016, P = 0. 327 ]. One case with kidney stone and 5 cases with nephrocalcinosis had a negative result of renal ultrasound after treatment. Conclusions Treatment of potassium citrate combination with thiazide diuretics and ACEI can significantly decrease urinary calcium excretion, make a disappearance of kidney stone and nephrocalcinosi, and it may have a role in protecting renal function. Treat- ment of benazepril can not significantly decrease the proteinuria and has no substantial improvement in low molecular weight protein urine.