洛伐他汀对激素耐药性肾病综合征小儿高脂血症的疗效

目的观察降脂药物洛伐他汀对活动期激素耐药性肾病综合征（SRNS）患儿高脂血症的疗效及其安全性。方法对肝功能正常的活动期SRNS患儿37例应用洛伐他汀降脂治疗1个月，检测其治疗前后血清脂质、脂蛋白、清蛋白（Alb）、肝肾功能、24h尿蛋白定量的变化，并观察临床不良反应，本组患儿均接受激素正规治疗2个月以上且使用激素前后尿蛋白持续（＋＋＋-＋＋＋＋）。结果SRNS患儿血脂及脂蛋白水平在应用洛伐他汀2周后出现不同程度的变化，其中三酰甘油（TG）、24h尿蛋白定量明显下降（Pa〈0．05），总胆固醇（TC）、极低密度脂蛋白胆固醇（VLDL—C）、低密度脂蛋白胆固醇（LDL—C）下降，差异均在4周后出现明显变化（Pa〈0．05）。治疗前后其血Alb、ALT、血肌酐（Scr）差异均无统计学意义。除1例服药后出现胃肠不适外，余患儿均无腹痛、腹泻、皮疹等不良反应。结论对持续未缓解的SRNS患儿，尤其是以TG升高为主的高脂血症，短期应用洛伐他汀降脂治疗是安全有效的。     

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目的观察和分析原发性肾病综合征(PNS)的患儿脂质紊乱与尿β2-微球蛋白(β2-MG)间的关系,探讨高脂血症对PNS患儿肾小管功能的影响.方法检测广东省东莞市人民医院儿科1997年1月至2004年11月就诊的132例PNS患儿血脂及尿β2-MG水平.并将患儿随机分成治疗组与对照组,均予综合治疗,治疗组加服血脂康降脂治疗4周,比较治疗前后及两组间血脂与尿β2-MG的水平变化.结果 (1)132例PNS患儿尿β2-MG质量浓度均有不同程度升高,与胆固醇(TC),甘油三酯(TG),低密度脂蛋白(LDL),极低密度脂蛋白(VLDL)呈正相关(P<0.05).(2)降脂治疗4周后,治疗组血脂与尿β2-MG水平与对照组比较差异有显著性意义(P<0.01).(3)治疗组的PNS患儿尿β2-MG随血脂下降而下降,治疗前后尿β2-MG差异有显著性意义(P<0.01).结论 PNS患儿脂质紊乱与尿β2-MG关系密切,肾病综合征患儿高脂血症是损害肾小管功能的因素之一.     

原发性肾病综合征高脂血症对尿β2-微球蛋白影响的临床观察

目的观察和分析原发性肾病综合征(PNS)的患儿脂质紊乱与尿β2-微球蛋白(β2-MG)间的关系,探讨高脂血症对PNS患儿肾小管功能的影响。 方法检测广东省东莞市人民医院儿科1997年1月至2004年11月就诊的132例PNS患儿血脂及尿β2-MG水平。并将患儿随机分成治疗组与对照组,均予综合治疗,治疗组加服血脂康降脂治疗4周,比较治疗前后及两组间血脂与尿β2-MG的水平变化。 结果(1)132例PNS患儿尿β2-MG质量浓度均有不同程度升高,与胆固醇(TC),甘油三酯(TG),低密度脂蛋白(LDL),极低密度脂蛋白(VLDL)呈正相关(P<0.05)。(2)降脂治疗4周后,治疗组血脂与尿β2-MG水平与对照组比较差异有显著性意义(P<0.01)。(3)治疗组的PNS患儿尿β2-MG随血脂下降而下降,治疗前后尿β2-MG差异有显著性意义(P<0.01)。 结论PNS患儿脂质紊乱与尿β2-MG关系密切,肾病综合征患儿高脂血症是损害肾小管功能的因素之一。     

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高脂血症是儿童肾病综合征(NS)的重要特征,多于病程急性期出现,缓解期消失。但部分患儿可持续存在不同程度高脂血症,其中尤以频复发及激素耐药患儿最为明显。1儿童NS高脂血症特点总胆固醇(TC)、三酰甘油(甘油三酯,TG)、低密度脂蛋白胆固醇(LDL-Ch)、极低密度脂蛋白胆固醇(VLDL-C)及中密度脂蛋白胆固醇(IDL-C)浓度升高,出现氧化修饰LDL(Ox-LDL);高密度脂蛋白胆固醇(HDL-C)浓度可以升高、正常或降低,亚型分布异常:HDL2降低,HDL3增高。TC/HDL-C及TG/HDL-C比率升高时,HDL成熟被阻碍,颗粒趋于更小化,逆转运胆固醇的作用被…     

儿童肾病综合征脂质代谢紊乱研究进展

儿童肾病综合征常合并高脂血症，其原因主要与脂蛋白合成增加、分解代谢减少及控制载脂蛋白表现型的基因突变有关。高脂血症不仅影响肾脏病的预后，也易诱发心血管并发症的发生。重度脂代谢紊乱患儿，尤其是病程长、易反复、易复发及激素耐药的肾病高脂血症患儿应该加用降脂药物。     

儿童肾病综合征脂质代谢紊乱研究进展

儿童肾病综合征常合并高脂血症，其原因主要与脂蛋白合成增加、分解代谢减少及控制载脂蛋白表现型的基因突变有关。高指血症不仅影响肾脏病的预后，也易诱发心血管并发症的发生。重度脂代谢紊乱患儿，尤其是病理程长、易反复、蝗复发及激素耐药的肾病高脂血症患儿应该加用降脂药物。     

多烯康治疗儿童激素敏感型肾病综合征高脂血症疗效观察

目的：观察糖皮质激素治疗后(10周内)多烯康治疗儿童激素敏感型肾病综合征(SSNS)高脂血症的疗效。方法：SSNS患儿随机分为降脂治疗组(治疗组)和对照治疗组(对照组),均给予糖皮质激素标准疗程,治疗组加用多烯康。分别在治疗前,治疗后2～3周,5～6周,8～10周采空腹血观察4种血脂成分：总胆固醇(TC),三酰甘油(TG),高密度脂蛋白胆固醇(HDL-C)和低密度脂蛋白胆固醇(LDL-C)。结果：疗程2～3周治疗组LDL-C(3.19±2.08) mmol,TC(6.42±2.04) mmol均显著低于对照组(5.82±2.73) mmol/L,(10.0±4.75) mmol/L,(P均<0.05)。第5～6周治疗组LDL-C(2.83±1.50) mmol较对照组(4.94±2.04) mmol/L,提前下降至低水平(P<0.01),第8～10周治疗组LDL-C(2.30±0.46) mmol/L仍明显低于对照组（4.20±2.22）mmol/L,(P<0.01)。结论：多烯康能显著缩短SSNS严重高脂血症的时间,尤其是对降(LDL-C)有显著疗效,减轻了高脂血症对肾脏继发性损害的饿威胁。     

盐酸哌甲酯治疗儿童良性癫痫伴中央颞区棘波合并注意缺陷多动障碍的安全性研究

目的探讨盐酸哌甲酯治疗儿童良性癫痫伴中央颞区棘波(BECTS)合并注意缺陷多动障碍(ADHD)的安全性及对癫痫发作的影响。方法选取2007年4月至2008年10月广州市儿童医院神经内科确诊的40例BECTS合并ADHD患儿,经抗癫痫药物治疗临床无发作达6个月以上,加用盐酸哌甲酯,每6个月复查1次脑电图(EEG),评价患儿注意力和行为改善情况,记录癫痫发作次数、类型以及其他副反应,将患儿用药前后的癫痫发作情况进行自身对照研究。结果 40例中37例用药时间超过6个月,注意力、多动及对立违拗行为明显改善。4例发生副反应;2例用药1周内发作增多;1例用药1年后出现1次发作;1例用药2d内出现入睡困难,停药后发作及入睡困难停止。患儿服用盐酸哌甲酯前后癫痫发作及其他副反应出现情况差异无统计学意义(P0.05)。结论抗癫痫药控制临床发作的同时,在规范剂量范围内使用盐酸哌甲酯治疗BECTS合并ADHD具有安全性。     

异丙酚治疗小儿癫(癎)持续状态的安全性观察

目的探讨异丙酚治疗癫痫持续状态的安全性。方法对15例应用异丙酚治疗的癫痫持续状态的患儿,在用药前后检测肝肾功能、心肌酶、血气分析、血电解质、血脂及血、尿常规,并监测用药过程中呼吸、心率、血压的变化。结果出现一过性的肝酶升高5例,心肌酶一过性升高3例,其余均未见异常。结论异丙酚治疗癫痫持续状态相对安全,未见严重不良反应。     

儿童脂蛋白肾病1例报告并文献复习

目的探讨儿童脂蛋白肾病(LPG)的临床及预后。方法回顾性分析1例儿童LPG的临床资料,归纳总结国内外报道的儿童LPG的临床特点及预后。结果患儿,女,9岁,以尿频起病,初次尿检提示菌尿、血尿、蛋白尿,规律抗感染治疗1周后,仍有血尿、蛋白尿,血清白蛋白轻度降低,高脂血症,轻度贫血;肾脏组织活检,镜下可见肥大肾小球,扩张的肾小球毛细血管管腔,其内充以脂蛋白栓子,油红O染色阳性;电镜下多见层状或簇状"栓子"内含颗粒状脂质空泡。基因检测APOE Tokyo(Leu141-Lys143→0)。诊断为LPG,给予降脂治疗后病情明显缓解。结论儿童LPG罕见,血脂水平显著增高,激素治疗无效,肾脏穿刺活检是确诊的主要依据,基因检测提示其遗传背景;降脂治疗可缓解病情进展。     

原发性肾病综合征患儿血浆脂质及脂蛋白的变化

为了解小儿原发性肾病综合征（ＮＳ）时脂代谢的特点，观察了２０例原发性单纯型ＮＳ患儿的血脂、脂蛋白及载脂蛋白的变化。结果：（１）全部ＮＳ患儿血浆总胆固醇（ＴＣ）、低密度脂蛋白胆固醇（ＬＤＬ－Ｃ）和脂蛋白（ａ）［Ｌｐ（ａ）］均明显升高；兼有ＴＣ、甘油三酯（ＴＧ）升高者，其极低密度脂蛋白胆固醇明显升高，高密度脂蛋白胆固醇明显降低。（２）ＮＳ患儿２４小时尿蛋白定量与血浆ＴＣ、ＴＧ和ＬＤＬ－Ｃ呈正相关（Ｐ＜０．０１）。提示：（１）ＮＳ患儿存在脂代谢异常，兼有ＴＣ、ＴＧ同时升高者，伴有更严重的脂蛋白及载脂蛋白的紊乱，存在着多种致动脉硬化及肾损伤的因素。（２）ＮＳ患儿脂代谢异常的程度与蛋白尿严重程度高度正相关。     

辛伐他汀治疗难治性肾病综合征患儿高脂血症的疗效

目的 观察辛伐他汀治疗难治性肾病综合征(RNS)患儿高脂血症的疗效及其对预后的影响.方法 对在本科住院的27例RNS患儿应用辛伐他汀(年龄<10岁,0.3 mg·kg<'-1>·d<'-1>;≥10岁,10 mg·d<'-1>)降脂治疗2周,检测其治疗前后血清总胆同醇(TC)、三酰甘油(TG)、高密度脂蛋白(HDL)、低密度脂蛋白(LDL)、极低密度脂蛋白(VLDL)、PLT、ALT、SCr变化,并观察其临床不良反应情况.结果 辛伐他汀干预前,TC为(10.68±4.23)mmol·L<'-1>、PLT为(155.21±34.43)×10<'9>L<'-1>、HDL为(1.46±0.61)mmol·L<'-1>、VLDL为(2.47±1.31)mmol·L<'-1>、LDL为(6.74±3.96)mmol·L<'-1>、TG为(4.56±1.83)mmol·L<'-1>、ALT为(24.11±6.15)IU·L<'-1>、SCr为(91.32±6.15)μmol·L<'-1>.辛伐他汀干预1周后代为(9.36±3.46)mmol·L<'-1>,与干预前比较,差异有统计学意义(P<0.05),PLT为(123.34±31.32)×10<'9>L<'-1>、TG为(3.03±0.74)mmol·L<'-1>、VLDL为(1.36±0.33)mmol·L<'-1>、LDL为(6.21±3.21)mmol·L<'-1>、HDL为(1.82±0.90)mmol·L<'-1>、ALT为(25.32±4.14)IU·L<'-1>、SCr为(94.54±6.43)μmol·L<'-1>,与干预前比较,差异均无统计学意义(P<,a>>0.05).辛伐他汀干预2周后TC为(7.28±2.01)mmol·L<'-1>、PLT为(86.65±34.23)×10<'9>L<'-1>、TG为(2.36±1.16)mmol·L<'-1>、VLDL为(1.25±0.99)mmol·L<'-1>、LDL为(4.21±2.01)mmol·L<'-1>、HDL为(2.23±0.93)mmol·L<'-1>,与干预前比较,差异均有统计学意义(P<,a><0.05),ALT为(25.31±5.14)IU·L<'-1>、SCr为(94.53±6.23)μmol·L<'-1>,与干预前比较差异均无统计学意义(P<,a>>0.05).2例患儿服药后出现胃肠不适、1例患儿出现一过性ALT升高.该3例患儿停用辛伐他汀1周后均恢复正常,无其他不良反应.结论 辛伐他汀干预能使RNS高脂血症缓解,其高凝状态亦明显改善,并能够使病情缓解,改善预后,同时无明显不良反应.     

The effects of gemfibrozil on hyperlipidemia in children with persistent nephrotic syndrome

Persistent nephrotic syndrome is frequently accompanied by severe hyperlipidemia, and this may pose a substantial risk for cardiovascular disease. Lipid-lowering drugs are prescribed by many nephrologists for adult patients but rarely for nephrotic children. The present investigation was designed to evaluate the safety and efficacy of gemfibrozil in nephrotic children. Eight girls and four boys aged from 5 to 17 years were enrolled in this study. They were all steroid and immunosuppressive resistant patients with nephrotic range proteinuria. Placebo was administered to five patients and gemfibrozil was administered to seven patients for four months. Blood samples were taken for the determination of cholesterol, triglyceride, low-density lipoprotein (LDL), high-density lipoprotein (HDL), BUN, serum creatinine (Scr), ALT, AST, CPK, apolipoprotein A (apo A), apoliporotein B (apo B), and serum albumin levels during the initial and subsequent examinations. At the end of the fourth month, gemfibrozil reduced total cholesterol by 34%, LDL by 30%, apo B by 21% and triglycerides by 53% (p < 0.05). HDL cholesterol and apo A levels were not significantly altered. Renal function and urine protein excretion were not affected by gemfibrozil. In this study gemfibrozil therapy had no side effects and had favorable effects on the lipoprotein profile of nephrotic patients.     

Severe hyperlipoproteinemia in congenital nephrotic syndrome of the Finnish type: effect of dialysis and kidney transplantation

Two children with congenital nephrosis of the Finnish type were studied successively at the three stages of the disease: (A) nephrosis, (B) renal insufficiency/peritoneal dialysis and (C) post-transplantation; two additional patients were studied at two stages. Plasma lipoprotein profiles were determined by density gradient ultracentrifugation and lipids by enzymatic methods. Stage A was characterized by hyperchylomicronemia, low high density lipoprotein (HDL) cholesterol and the presence of dense low density lipoprotein (LDL) and HDL particles. Total cholesterol and triglycerides showed great daily variation (5–14 and 5–33 mmol/l, respectively). During stage B, hyperlipidemia weakened. Yet HDL concentration remained low and the concentration of intermediate density lipoproteins (IDL) increased. At stage C, hyperlipidemia had almost subsided, but the presence of IDL persisted. In conclusion, severe hyperlipoproteinemia of congenital nephrosis at the nephrotic stage is attenuated during renal insufficiency and dialysis, and essentially normalizes after kidney transplantation. Yet the presence of IDL implies an increased risk of atherosclerosis.     

单纯性肥胖儿童脂肪肝与血脂成分的关系

目的探讨单纯性肥胖儿童脂肪肝与血脂的关系及预防措施。方法48例2~16岁单纯性肥胖儿童。所有患儿分为脂肪肝组19例(38.78%)及无脂肪肝组29例(61.22%),对2组血脂成分进行分析。用生化分析仪检测血总胆固醇(TC)、三酰甘油(TG)、高密度脂蛋白(HDL)及低密度脂蛋白(LDL)。用超声诊断仪对肝脏进行超声检查。结果脂肪肝组血脂成分TG、TC、HDL及LDL均高于肥胖无脂肪肝组。与脂肪肝关系最密切者为TG(P<0.05)。当各自变量不独立时,TG、TC、HDL及LDL均是脂肪肝形成影响因素(Pa<0.05)。结论单纯性肥胖儿童可出现脂代谢紊乱,是儿童脂肪肝形成的主要影响因素,预防与肥胖有关的脂肪肝、高血压及心血管疾病,应从儿童甚至婴幼儿做起。     

Lipoprotein profile changes in children after renal transplantation in the modern immunosuppression era

There are little data on prevalence of dyslipidemia in pediatric kidney TX recipients in the modern IS era. LP profiles of 38 TX recipients receiving triple IS with MMF, prednisone, and tacrolimus were compared with those of 11 children on HD using mixed model multiple linear regression analysis of repeated measures after adjusting for age, sex, ethnicity, duration of ESRD, and BMI. TC and LDL levels were significantly higher in TX compared with HD, whereas there was no difference in the HDL, VLDL, and TG levels. TC and LDL in TX children had no association with age, sex, ethnicity, and duration of ESRD, stage of chronic kidney disease, DM, BMI percentile, and gain in percentage IBW. Five children treated with atrovastatin had a significant reduction in TC, LDL, VLDL, and TG at 3-6 months post-treatment compared with pretreatment levels, whereas there was no difference in HDL or tacrolimus levels after treatment. No side effects of therapy were observed. Although dyslipidemia remains a significant problem in pediatric renal TX recipients in the modern era, the prevalence may have decreased with use of newer IS drugs.     

Lipoprotein profiles at different stages of the nephrotic syndrome

We investigated lipoprotein profiles in 24 children with normal renal function at different stages of the idiopathic nephrotic syndrome (NS). Four groups of patients were studied: (I) steriod-resistant NS with persistent proteinuria; (II) untreated steroid-sensitive NS during a relapse; (III) steroid-sensitive NS in remission induced by steroid-treatment; (IV) steroid-sensitive NS in long-term remission with-out therapy. Triglycerides (TG), cholesterol (CHOL), and phospholipids (PLP) were measured in plasma as well as in the lipoprotein fractions of very low (VLDL), intermediate (IDL), low (LDL) and high density (HDL). Apoproteins (Apo) AI, AII, B and C-apoproteins were measured in patients of groups I and IV. Results were compared to those obtained in 24 healthy control subjects. All patients with active NS (groups I–III) had significantly elevated CHOL levels. TG and CHOL in the VLDL, IDL, LDL, and CHOL in HDL₂, but not HDL₃ were inversely correlated with the serum albumin level. Patients with active NS had increased concentrations of TG and CHOL in lipoprotein fractions of lower density. Total and fractionated HDL-CHOL was not significantly different from control levels in any group. Patients in group I had significantly reduced Apo AI levels, whereas an increase of Apo AI and Apo AII in HDL₃ and of most C-apoproteins in both HDL fractions was observed in patients of group IV. While changes in HDL apoprotein composition during longterm remission are of yet unknown clinical significance, our data indicate an increased risk of atherosclerosis only in those paediatric patients with persistent steroid-resistant NS.Abbreviations Apo A (B) apoprotein A (B) - CHOL cholesterol - GFR glomerular filtration rate - DHL high density lipoprotein - HLP hyperlipoproteinaemia - IDL intermediate density lipoprotein - LDL low density lipoprotein - MC minimal changes - NS nephrotic syndrome - PLP phospholipids - TG triglycerides - VLDL very low density lipoprotein     

Effect of carbamazepine on serum lipids and liver function tests

We prospectively studied the effect of carbamazepine (CBZ) therapy on serum lipids and liver function tests in 28 patients and 28 age and sex matched controls. The mean age of patients was 8.29 years, duration of therapy with CBZ 10.3 months and dose of CBZ 13.1 mg/dL. The patients and controls were comparable in weight, height and BMI. Mean +/- SD of cholesterol 162 +/- 25.8 mg/dL in patients was significantly more than controls 131+/- 25.2 mg/dL. Mean LDL cholesterol and HDL cholesterol were also significantly raised in patients. Values of mean VLDL, triglycerides, ratio of LDL HDL, TC HDLC bilirubin and SGPT were not significantly different in two groups. Blood Levels of alkaline phosphatase were significantly more in patients compared to controls. The long term implications of these findings need to be studied.     

Lipoprotein abnormalities are highly prevalent in pediatric heart transplant recipients

The role of hyperlipidemia in graft coronary artery disease (GCAD) is controversial although hyper-triglyceridemia is an independent risk factor. Recent studies show that 3-hydroxy-3-methylglutaryl-coenzyme A (HMG-CoA) inhibitors decrease the incidence of GCAD in adults. The incidence of GCAD in pediatric patients is lower than in adults; it is not clear whether age-related differences in lipid metabolism account for some of this protection. This study was performed to: characterize the lipoprotein profile in children after heart transplantation; demonstrate that total cholesterol (TC) is a poor marker for underlying lipoprotein abnormalities; and to compare lipid abnormalities in patients who had been converted from cyclosporin A (CsA) to tacrolimus. Seventy-one determinations of fasting lipoprotein profiles were performed in a cohort of 28 children. Each child had at least two determinations on separate occasions. TC, low-density lipoprotein (LDL), and serum triglyceride (TG) levels were categorized as abnormal if greater than the 75th percentile for age and gender. A high-density lipoprotein (HDL) level less than the 25th percentile was considered abnormal. Immunosuppression included CsA or tacrolimus, azathioprine, and prednisone. We found that 90% of the patients studied had abnormalities of either TG or HDL. In contrast, LDL tended to be normal when adjusted for age and gender. TC was a poor indicator of any underlying abnormality in TG, LDL, or HDL. In patients converted to tacrolimus, no significant differences were found in the levels of TG, LDL or HDL compared with each patient's respective values while receiving CsA. Hence, lipoprotein abnormalities among pediatric heart transplant recipients are highly prevalent. TC is a poor screening tool in the evaluation for lipid abnormalities. Lipoprotein profiles remain statistically unchanged after conversion from CsA to tacrolimus.     

瘦素及可溶性瘦素受体在原发性肾病综合征患儿血脂升高中的作用

目的探讨瘦素(Leptin)及可溶性瘦素受体(sOBR)在儿童原发性肾病综合征(PNS)血脂升高中的作用。方法检测23例未经治疗的PNS患儿空腹血清血脂、血浆清蛋白、Leptin、sOBR、胰岛素及尿Leptin、sOBR水平,并与在年龄、性别、体质量指数(BMI)均匹配的15例正常儿童比较。结果肾病组血胆固醇(TC)、三酰甘油(TG)、低密度脂蛋白(LDL)、载脂蛋白B(apoB)水平增高,血浆清蛋白、胰岛素水平较对照组降低。空腹血清总Leptin水平肾病组与对照组相当,而尿Leptin水平肾病组高于对照组;血清sOBR水平肾病组降低,尿sOBR水平二者无差异;血清游离Leptin指数(FLI)肾病组高于对照组。肾病组游离Leptin指数(FLI)与血浆清蛋白、HDL、apoA呈正相关,与LDL、胰岛素呈负相关。结论PNS患儿血清sOBR减少,游离瘦素增多可能为一抗高脂血症的代偿机制,但其纠正PNS脂代谢紊乱的能力并不完全。