Pulmonary function in children with chronic renal failure

Lung volumes were measured in 45 children with chronic renal failure and compared to 10 healthy controls. Six patients were receiving conservative treatment (CT), 11 were undergoing regular haemodialysis (HD), 8 were on continuous ambulatory peritoneal dialysis (CAPD) and 20 were post transplantation (TP). We measured vital capacity (VC) and forced expiratory volume in 1 s (FEV-1) with a bell spirometer. In addition residual volume (RV) was determined in CAPD patients. VC and FEV-1 values below the lower limit of predicted normal values from healthy children with the same body height were found in 38% and 52% of all patients respectively (P<0.05). Median values of VC and FEV-1 were lowest in CT and highest in TP patients. Median FEV-1 was significantly reduced to 79% of predicted values in CT and to 82% in HD patients (P<0.05). No correlation was found between FEV-1 and haemoglobin levels or the concomitant use of beta-blocking agents. During a HD session mean FEV-1 increased significantly. In CAPD patients the routine filling of the abdomen was followed by an 11% decrease of RV (non significant) while the other parameters remained stable. It is concluded that lung volumes are frequently reduced in chronic renal failure but remain essentially stable during the dialysis procedures.     

腹膜透析在新生儿心脏直视术后的应用

目的 探讨心脏直视术后腹膜透析在治疗新生儿急性肾功能衰竭方面的应用.方法 2006年1月至2008年9月,我院共有131例新生儿先天性心脏病患儿接受体外循环下心脏直视手术,其中男88例,女43例;年龄1 h至28 d;体重1.13～5.10kg,平均(3.34±0.54)kg;其中13例患儿在术后因罹患急性肾功能衰竭而接受腹膜透析治疗,男9例,女4例;年龄1 h至28 d;体重为1.13～4.80kg,平均(3.25±0.72)kg.3例患儿在术中经胸骨正中切口下方的腹膜切口间接放置腹膜透析管,另外10例患儿则在术后经脐旁腹壁切口直接放置.透析液采用百特专用腹膜透析液,并根据病情的需要选择不同渗透压的液体.常规透析方案为:首次透析选用1.5%腹膜透析液,剂量为10～30 ml/kg;输入15min,滞留30 min,引出15min;根据患儿病情变化调整腹膜透析液的浓度、滞留时间比.结果全部患儿均无腹膜透析禁忌证.11例肾功能恢复,2例死亡.无腹膜透析并发症发生.结论 腹膜透析是治疗新生儿心脏直视术后急性肾功能衰竭的有效方法 ,并发症发生率低,操作简单,经济实用.     

慢性心力衰竭患儿心型脂肪酸结合蛋白的变化及临床意义

目的：通过前瞻性观察慢性心力衰竭（CHF）患儿血浆心型脂肪酸结合蛋白（h-FABP）的水平变化及其与心功能的关系,探讨其在CHF中的临床价值。方法：36例CHF患儿（CHF组）纳入研究,其中心内膜弹力纤维增生症16例（EFE组）,扩张型心肌病20例（DCM组）。选择同期30例健康儿童作为对照组。血浆h-FABP浓度测定采用酶联免疫吸附法（ELISA）,并以心脏超声心动图测量心脏指数、左室短轴缩短率及左室射血分数。结果：CHF患儿血浆h-FABP浓度明显高于对照组（21.7±4.3 ng/mL vs 6.2±1.7 ng/mL, P＜0.01）,且心力衰竭程度越重,h-FABP浓度升高越明显（P＜0.01）。EFE、DCM两组h FABP水平均高于对照组（P＜0.01）。CHF患儿h-FABP水平与左室射血分数、心脏指数及左室短轴缩短率均呈明显负相关（分别r=-0.65、-0.64、-0.71,均P＜0.01）。结论：CHF患儿的血浆h-FABP浓度明显升高,血浆h FABP浓度与心力衰竭的严重程度相关。血浆h-FABP浓度可作为评估心力衰竭及其严重程度的一个指标。     

Aggressive angiomyxoma in a child with chronic renal failure

Aggressive angiomyxoma (AAM) is a rare and nonmetastasizing soft-tissue tumor predominantly found in the female pelvis and perineum. It has a high risk of local recurrence. We report the unusual case of a 15-year-old boy with an AAM presenting as a slowly enlarging scrotal mass. The patient had had chronic renal failure since 1997 and had needed hemodialysis for the previous 11 months. He presented with a 12-month history of a nontender soft mass in the right scrotum. Ultrasound examination revealed a solid mass in the scrotum. After surgical resection, pathological analysis disclosed spindle-shaped neoplastic cells widely separated by a myxoid stroma rich in collagen fibers and prominent irregularly shaped blood vessels; the histological examination confirmed an AAM.     

Energy and nutrient intake of patients with mild-to-moderate chronic renal failure compared with healthy children: An Italian multicentre study

Nutritional counselling is important in the management of children with chronic renal failure (CRF). In 1988, a controlled European multicentre study was started to evaluate the effects of a low-protein diet on the progression of CRF in children. To assess the energy, macro- and micronutrient intake, 4-day weighed dietary records were obtained from 50 children with low to moderate CRF (creatinine clearance 65 to 15 ml/min per 1.73 m²) and from 93 healthy children. The mean energy intake was 90%–93% of the recommended dietary allowance for Italian children in controls and 76%–88% in CRF patients. The mean protein intake was 2.1–3.1 g/kg per day in controls and 1.6–2.7 g/kg per day in CRF patients. Overall, the energy intake was 10% and the protein intake 33% lower in CRF patients than in healthy children. Children with CRF consumed less cholesterol, calcium and phosphorus than healthy children. The lower spontaneous intake of energy, protein and other nutrients should be taken into account when planning the nutrition of children with CRF.     

91所医院1990～2002年小儿慢性肾衰竭1268例调查报告

目的　调查我国 1990年 1月～ 2 0 0 2年 12月间 0～ 14岁住院小儿中慢性肾衰竭 (CRF)病例的年龄、病因、临床病理特点、治疗情况及转归。方法　由中华医学会儿科学分会肾脏病学组统一组织领导 ,全国 4个直辖市、13个省及 2个自治区共 91所医院参加调查。CRF诊断以内生肌酐清除率 (CCr) <5 0ml/(min·1 73m2 )为标准。采用填写调查表形式 ,对诊断为慢性肾衰竭的住院患儿进行回顾性病例登记 ,进行相关资料统一汇总、分析。结果　 1990年 1月～ 2 0 0 2年 12月间 ,91所医院0～ 14岁住院小儿中共诊断CRF 16 5 8例 ,每年在住院的泌尿系统疾病患儿中所占比例为 0 72 %～1 75 % ,平均 1 31% ,呈逐渐上升趋势。 1997～ 2 0 0 2年与 1990～ 1996年比较 ,CRF平均年诊断例数和占泌尿系统疾病的百分数均显著增加 (P <0 0 0 1)。 12 6 8例完整资料分析显示 :男女比例 1 4 9∶1,平均发病年龄 8 2岁 ,平均确诊前病程 2 5年。主要原发病为慢性肾炎和肾病综合征 ,占 5 2 7% ,先天 /遗传性疾病约 1/4,以肾发育异常和肾囊性病为主。确诊时主要临床表现为贫血、胃肠反应、水肿、高血压和体格发育落后 ;平均血清肌酐 (SCr) 5 94 7μmol/L ,BUN 39 1mmol/L ,肾功能分级≥Ⅳ级者占 80 % ;1/3有肾萎缩 ,部分见囊性病变。多     

Acute non-oliguric kidney failure and cholestatic hepatitis induced by ibuprofen and acetaminophen: a case report

The combined use of acetaminophen with ibuprofen has long been in clinical use because the target of action of each drug is different and they do not interfere with each other. Appropriate dosing and managing of these drugs do not likely lead to organ toxicity. However, both acetaminophen and ibuprofen can induce liver problems and acute kidney failure, respectively, if administered at high doses. We report the case of a female child, in treatment with both acetaminophen and ibuprofen, administered at therapeutic antipyretic doses in condition of volume depletion, who suffered acute kidney and liver failure.
Conclusion: The combined ibuprofen and acetaminophen treatment, even if administered at therapeutic dosages and in a reduced number of doses, may be dangerous in conditions of volume depletion.     

慢性肾衰竭肾脏替代治疗现状调查多中心研究

目的 调查我国2007年至2011年≤18岁住院患儿中慢性肾衰竭病例的肾脏替代治疗情况及转归.方法 制定统一的儿童慢性肾衰竭肾脏替代治疗现状调研表,分发至28家参研医院进行回顾性病例登记,进行相关资料统一汇总、分析.结果 2007年1月1日至2011年12月31日28间医院住院患儿确诊慢性肾衰竭1033例,474例患儿接受肾脏替代治疗,占45.9％.380例慢性肾衰竭患儿接受血液透析治疗,133例次出现急性并发症,占35.0％;119例次出现慢性并发症,占31.3％.177例患儿接受腹膜透析治疗,79例次出现并发症,占51.0％.47例患儿接受肾移植治疗,9例出现排斥反应.结论 我国儿童慢性肾衰竭肾脏替代治疗水平有较大提高,但发展不平衡.维持性透析仍以血液透析为主,腹膜透析发展较为滞后.儿童肾移植治疗仍在起步阶段.     

甲亢危象并发多器官衰竭1 例报告

目的探讨儿童甲亢危象的临床特征。方法回顾分析1例甲亢危象并发多器官衰竭患儿的临床资料。结果女性患儿,13岁,既往无甲状腺疾病史。患儿以恶心、呕吐、神萎起病,入院时以心律失常、心源性休克、心力衰竭为主要表现,考虑为暴发性心肌炎。有轻度突眼。游离T3、游离T4明显增高,促甲状腺激素明显降低。入院后相继出现呼吸衰竭、肾衰竭及肝衰竭,甲亢危象评分120分,确诊为甲亢危象。经积极抗心律失常、应用血管活性药物、呼吸支持、丙硫氧嘧啶、碘剂、糖皮质激素、血液净化等综合治疗,患儿好转出院。结论甲亢危象在儿童较为罕见,但可以作为儿童甲状腺疾病的首发症状出现。甲亢危象表现隐匿,早期识别、及早综合治疗是治疗成功的关键。     

Gastrointestinal bleed in a toddler secondary to chronic renal failure and hyper-gastrinemia

A 3-year-old girl with H. Pylori negative duodenal ulcer with hypergastrinemia secondary to chronic renal failure presenting with upper gastrointestinal bleed as the cardinal manifestation is unusual in toddlers and the case is presented for its rarity.     

Nesiritide Improves Hemodynamics in Children with Dilated Cardiomyopathy: A Pilot Study

Background This study aimed to obtain hemodynamic measurements of nesiritide in children with dilated cardiomyopathy. Methods A prospective, randomized, double-blinded, placebo-controlled pilot study was conducted in the pediatric intensive care unit at the University of California, Los Angeles. All subjects younger than 21 years admitted to the pediatric intensive care unit with a diagnosis of dilated cardiomyopathy and submitted to cardiac catheterization were randomized to receive either nesiritide or placebo. Right heart catheterization with Swan-Ganz catheter placement was performed. Nesiritide was infused over 24 h. Hemodynamic data were obtained before, during, and after the 24-h nesiritide infusion. The measures obtained included pulmonary capillary wedge pressure (PCWP), central venous pressure, mean pulmonary arterial pressure (MPAP), systolic arterial blood pressure (SBP), cardiac index, and systemic vascular resistance. Results The study included 20 children: 9 randomized to nesiritide and 11 to placebo. At 24 h, the mean decreases in PCWP, MPAP, and SBP were significantly greater for nesiritide than for placebo: PCWP (–5.3 vs. 1.2 mmHg; p = 0.02), MPAP (–8.0 vs. 0.4 mmHg; p = 0.006), SBP (–7.9 vs. 2.6 mmHg; p = 0.04). Conclusions Nesiritide significantly decreases PCWP, MPAP, and SBP in children with dilated cardiomyopathy.     

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目的探讨钙磷代谢与慢性肾功能不全患儿心血管结构与功能关系。方法研究对象为1998—2004年在哈尔滨医科大学附属第一医院住院的44例慢性肾功能不全非透析患儿(非透析组)、另选40例维持性透析患儿(透析组),40例健康志愿者设为对照组。测量指标包括入选对象血液生化指标、超声心动图及颈动脉超声。结果与对照组比较,非透析组及透析组患儿均出现明显的颈动脉增厚、左心室肥厚、舒张功能减退。透析组动脉厚度及心室肥厚指标高于非透析组,且其动脉硬化较其他两组明显。两个肾脏疾病组颈动脉壁增厚与钙磷乘积增加呈正相关,动脉硬化、心室肥厚及舒张功能减退与血清磷及全段甲状旁腺素增加有关。结论慢性肾脏疾病患儿可合并心血管结构与功能异常;血液透析患儿心血管疾病的加重与钙磷代谢异常有关。     

慢性心力衰竭儿童血清PTX-3、syndecan-4水平变化及意义

目的 探讨血清pentraxin-3(PTX-3)、syndecan-4在儿童慢性心力衰竭(chronic heart failure,CHF)中的变化及意义.方法 选取收治的CHF患儿40例为心衰组,另选取同期门诊健康体检儿童30例为对照组.分别测定并比较血清PTX-3、syndecan-4、氨基末端脑钠肽前体(N-...     

纽约大学儿童心力衰竭指数在儿童慢性心力衰竭中的应用

目的 探讨纽约大学小儿心力衰竭指数(NYU PHFI)在小儿慢性心力衰竭中的应用价值及其对不同程度心力衰竭的诊断界值.方法 各种原因导致的慢性心力衰竭患儿及具有器质性心脏病但尚无心力衰竭症状的患儿共105例,分别应用改良Ross标准、NYU PHFI及血浆NT-proBNP进行诊断,以改良Ross标准作为参考标准,评估NYU PHFI对小儿慢性心力衰竭的诊断价值.进一步根据改良Ross标准的分度标准作为参考标准,分别绘制ROC曲线,计算曲线下面积,寻找出NYUPHFI对于心力衰竭分度标准的最佳临界值.结果 NYU PHFI的评分分值随着改良Ross标准的评分分值增加而增加,两者之间呈正相关(r=0.909,P=0.000),并且NYU PHFI分值在基于改良Ross标准的不同程度心力衰竭组间差异有统计学意义(F=80.034,P=0.000).血浆NT-proBNP水平与改良Ross标准及NYU PHFI的评分之间均呈正相关,相关系数分别为0.752及0.909,血浆NT-proBNP水平与患儿NYU PHFI评分之间的相关性较血浆NT-proBNP水平与改良Ross标准的相关性更好.分别以改良Ross标准评分0～2分为无心力衰竭、3～6分为轻度心力衰竭、7～9分为中度心力衰竭及10～12分为重度心力衰竭作为参考标准,则NYU PHFI诊断心力衰竭是否存在、轻度心力衰竭与中度心力衰竭及中度心力衰竭与重度心力衰竭三种诊断内容的ROC曲线下面积分别为0.982、0.942和0.918.经ROC曲线分析表明,诊断心力衰竭是否存在的界值为6分、诊断轻度与中度心力衰竭的界值为10分,诊断中度与重度心力衰竭的界值为13分.分别以NYU PHFI评分0～6分为无心力衰竭、7～10分为轻度心力衰竭、11～13分为中度心力衰竭及14～30分为重度心力衰竭为标准,分别比较不同程度心力衰竭患儿血浆NT-proBNP水平,其差异具有统计学意义(F=53.31,P<0.001),即无心力衰竭患儿的血浆NT-proBNP水平显著低于轻度心力衰竭患儿,其差异具有统计学意义;轻度心力衰竭患儿的血浆NT-proBNP水平显著低于重度心力衰竭患儿,其差异具有统计学意义(P<0.001).结论 NYU PHFI是评价小儿慢性心力衰竭的良好标准,可将0～6分、7～10分、11～13分及14～30分分别作为有无心力衰竭、轻度心力衰竭、中度心力衰竭及重度心力衰竭的分度参考标准.     

Transumbilical embolization of hepatic arteriovenous malformation in a neonate with heart failure

A newborn infant presented with severe heart failure due to a large hepatic arteriovenous malformation (AVM). Umbilical artery (UA) access was safely used for immediate diagnosis and for embolization.     

血清嗜铬粒蛋白A、尾加压素Ⅱ在小儿慢性心力衰竭中的变化及意义

目的探讨嗜铬粒蛋白A(CgA)、尾加压素Ⅱ(UⅡ)在慢性心力衰竭(CHF)患儿血清中的变化及意义。方法选取58例CHF患儿为心衰组,其中心内膜弹力纤维增生症17例,扩张型心肌病41例;另选取门诊健康体检儿童20例为对照组。采用酶联免疫吸附法(ELISA法)测定血清CgA及UⅡ水平;采用双向侧流免疫法测定氨基末端脑钠肽前体(NT-proBNP)水平;超声心动图测定心室重塑指标;Pearson相关或Spearman秩相关分析血清CgA、UⅡ与心室重塑的相关性。结果心功能Ⅱ级患儿的血清CgA、NT-proBNP水平与对照组的差异无统计学意义(P0.05);CgA、NT-proBNP水平在心功能Ⅲ级、Ⅳ级患儿中高于对照组,并且随着心功能损害加重而升高(P0.05)。UⅡ浓度在心功能Ⅱ级、Ⅲ级、Ⅳ级患儿中均低于对照组,并且随着心功能损害加重而逐渐降低,差异有统计学意义(P0.05)。心内膜弹力纤维增生症与扩张型心肌病患儿间血清CgA、UⅡ水平的差异无统计学意义(P0.05)。血清CgA浓度分别与左心室质量分数(LVMI)、NT-proBNP、心功能分级成正相关(r分别为0.279、0.649及0.778,P0.05),与左室射血分数(LVEF)、左室短轴缩短率(LVFS)、UⅡ成负相关(r分别为-0.369、-0.322及-0.718,P0.05)。血清UⅡ分别与NT-proBNP、心功能分级成负相关(r=-0.472、-0.591,P0.05),而与LVMI、LVEF、LVFS无明显相关性(P0.05)。结论 CgA可能参与CHF患儿心室重塑,血清CgA和UⅡ有可能为心衰的诊断和心功能判断提供参考。     

Growth hormone treatment enhances bone mineralisation in children with chronic renal failure

Dual energy X-ray absorptiometry of the whole body and the lumbar spine was performed to study bone mineralisation before and after 1 year of recombinant human growth hormone (rhGH) treatment in ten children with chronic renal failure. At the start, median age was 7.3 years (range 2.0–8.8 years) and median glomerular filtration rate 15 ml/min per 1.73 m² (range 7–41 ml/min per 1.73 m²). Total body mineral content (TBMC), lumbar spine mineral content (LBMC), total body bone mineral density (TBMD) and lumbar spine mineral density (LBMD) improved significantly (P < 0.05) after 1 year of treatment. Bone mineral data before and after treatment were compared with two groups of controls, i.e. ten healthy children matched for age and ten healthy children matched for height. Patients' TBMC, LBMC, TBMD and LBMD data before treatment were no different from those of height-matched controls; the same was true after 1 year of treatment except for the patients' significantly better LBMD (P < 0.05). When compared with age-matched controls, patients had significantly lower baseline TBMC and LBMC levels before treatment; after treatment LBMC was no longer different. However, there were no differences in TBMD or LBMD between patients and age-matched controls at baseline or after rhGH. Conclusion Recombinant human growth hormone treatment for 1 year results in a significant increase in both growth velocity and bone mineralisation. Comparison with height-matched controls shows a similar bone mineralisation at baseline and a better bone mineral density after treatment. Received: 10 August 2000 and in revised form 10 November 2000 and 5 January 2001 /  Accepted: 8 January 2001     

慢性心力衰竭患儿血清卵泡抑素样蛋白1的变化及临床意义

目的 探讨卵泡抑素样蛋白1(FSTL1)在慢性心力衰竭(CHF)患儿血清中的变化及与左心室重构的相关性。方法 选取2014年5月至2015年5月CHF患儿45例为心衰组,其中心内膜弹力纤维增生症21例,扩张型心肌病24例;另选取门诊健康儿童30例为健康对照组。采用酶联免疫吸附法测定血清FSTL1水平;采用放射免疫分析法测定氨基末端脑利钠肽前体;超声心动图测定左心室重构指标;Pearson相关或Spearman秩相关分析血清FSTL1与左心室重构指标的相关性。结果 心衰组治疗前血清FSTL1水平高于健康对照组(PPP=0.176)。血清FSTL1与左心室舒张末径(r=0.485,P=0.001)、左心室质量(r=0.322,P=0.031)、左心室质量分数(r=0.353,P=0.017)、氨基末端脑利钠肽前体(r=0.562,Pr=-0.436,P=0.003)、左室短轴缩短率(r=-0.436,P=0.003)呈负相关。结论 FSTL1可能参与CHF患儿左心室重构,血清FSTL1可以作为儿童CHF临床诊断、病情评估的客观指标之一。     

Liver transplantation in an adolescent with acute liver failure from acute lymphoblastic leukemia

The most common identifiable causes of acute liver failure in pediatric patients are infection, drug toxicity, metabolic disease, and autoimmune processes. In many cases, the etiology of acute liver failure cannot be determined. Acute leukemia is an extremely rare cause of acute liver failure, and liver transplantation has traditionally been contraindicated in this setting. We report a case of acute liver failure in a previously healthy 15‐yr‐old male from pre‐B‐cell acute lymphoblastic leukemia. He underwent liver transplantation before the diagnosis was established, and has subsequently received chemotherapy for pre‐B‐cell acute lymphoblastic leukemia. He is currently alive 31 months post‐transplantation. The published literature describing acute lymphoblastic leukemia as a cause of acute liver failure is reviewed.     

比较不同肾小球滤过率计算方法评价儿童慢性肾脏病肾功能的适用性

目的 肾小球滤过率(GFR)是评价肾功能的最好指标,可用直接检测法和公式估算法获得GFR,后者在临床实践中尤为常用,如24 h内生肌酐清除率、Schwartz公式以及Filler公式,但少有对这些计算方法在我国儿童慢性肾脏病(CKD)患者的适用性的研究.方法 选择2002年至2005年在我院住院、且符合入选标准和排除标准的CKD患儿为研究对象.将24 h内生肌酐清除率、Schwartz公式以及Filler公式估算的GFR(eGFR)与99mTc-DTPA肾动态显像(Gates法)测得的GFR(rGFR)进行比较.结果 入选30例患儿(男18例、女12例),平均年龄为9.4岁.CKD1期13例、CKD2期8例、CKD3期8例、CKD4期1例.CKD病因包括结构异常、肾小球肾炎、血管性疾病、肾病综合征和泌尿系结核.Bland-Altman分析显示24 h内生肌酐清除率的eGFR与rGFR的一致性相对最好;由24 h肌酐清除率和Schwartz公式得出的eGFR的准确性较Filler公式好.CKD1期时,由24 h内生肌酐清除率和Schwartz公式得出的eGFR过高估计rGFR,而由Filler公式得出的eGFR过低估计rGFR;CKD2期时,由24 h内生肌酐清除率得出的eGFR过低估计rGFR,而由Schwartz公式和Filler公式得出的eGFR过高估计rGFR.结论 3种公式获得的GFR的估计值与标准方法测得的GFR值之间存在显著差异,有待于今后开发更适用于我国儿童的肾功能估算方法.