Variables Discriminating Between Cryptogenic Glomerular Lesions in Adults with the Nephrotic Syndrome

A univariate analysis of individual clinical and biochemicalvalues of adult patients with cryptogenic nephrotic syndromehas shown that significant differences exist between patientswith proliferative glomerulonephritis, ‘minimal change’nephritis and membranous nephropathy. For any given adult patient with the condition, the most likelyclinical diagnosis is proliferative disease and the next, minimalchange. These two diagnoses together account for most cases.The best clinical discriminants between them are the systolicblood pressure and plasma cholesterol concentration. If thesystolic pressure is greater than 145 mm Hg proliferative diseaseis more likely, but if the cholesterol is greater than 530 mgm/100ml,a minimal change lesion is more likely. A scattergram for combiningthese variables in clinical practice is given, showing a zoneof uncertainty where renal biopsy would be indicated. Although single variables do not permit discrimination betweenmembranous nephropathy and the other two groups, it is suggestedthat analytical techniques where combinations of variables areused may be helpful, and should be developed.     

The Nephrotic Syndrome in Adults

Teratomas are predominantly tumors of infancy, usually detected during the first two years of life and occurring twice as frequently in male as in female children. Like other tumors of childhood, they often are benign but have serious potentialities of malignancy. The author describes several types of teratomas removed at the Children's Hospital, Louisville. He suggests microscopic diagnosis within 24 to 48 hours after a patient's admission to the hospital, with total excision the only logical procedure.     

Normal Glomerular Permeability and Its Modification by Minimal Change Nephrotic Syndrome

It has been suggested that the glomerular basement membrane restricts the passage of large molecules only, the barrier to filtration of smaller molecules being at the level of the epithelial slit pore. This hypothesis was investigated by measuring glomerular permeability to (125)I-labeled polydisperse polyvinyl pyrrolidone (PVP) in 16 children with idiopathic nephrotic syndrome (INS) and in 6 children of comparable age who had no evidence of renal disease. Studies were performed in the patients with INS before, during, and after treatment with steroids. PVP in blood and urine samples was separated according to molecular size by solumn chromatography, to permit the calculation of permeability to inert macromolecules of sizes ranging from 8,000 mol wt. In untreated INS, glomerular permeability to molecules > 40 A was normal; permeability to smaller molecules was markedly reduced, frequently to 20% or less of normal. There was an average decrease in inulin clearance (C(in)) of 24%. Glomerular permeability and C(in) returned to normal in INS treated with steroids only when proteinuria disappeared. The results support the concept, derived from studies with ultrastructural tracers, that the final barrier to filtration may be at the level of the epithelial slit pore. Thus fusion of the epithelial foot processed with obliteration of the slit pores was associated with impaired passage of smaller molecules of PVP into the urine. Reversal of the pathologic abnormality resulted in return of permeability to normal. The decreased C(in) seen in INS may not reflect true glomerular filtration rate, but may result from restricted passage of inulin molecules (mol wt 5,000) through the epithelial slit pore.     

The Medical Research Council Trial of Short-term High-dose Alternate Day Prednisolone in Idiopathic Membranous Nephropathy with Nephrotic Syndrome in Adults

We have assessed the medium-term effect of a short course ofhigh-dose, alternate-day prednisolone on adult nephrotic patientswith membranous nephropathy, using a randomized, prospective,double-blind, controlled trial. Patients were entered over theperiod 1981 to 1984 and were observed for a minimum of threeyears. One hundred and seven adult patients who had not previouslyreceived immunosuppressive treatment were included in the trial.One hundred and sixty further patients, excluded from the trial,but with membranous nephropathy were identified, followed andassesed retrospectively at the end of the trial. At 36 months there was no significant difference between controland treatment group in plasma creatinine, creatinine clearanceor 24-h excretion of protein. At between three and six monthsserum albumin concentrations were higher and protein excretionslower in the treatment group compared to controls. No significant benefit was therefore observer on renal functionin the medium term.     

肾病综合征患者肾小球中nephrin的表达意义

目的:检测肾病综合征(NS)患者肾小球组织中nephrin的表达,以探讨nephrin在肾病综合征患者蛋白尿发生机制中的可能作用。方法:免疫荧光染色图像分析及RT- PCR法检测NS、单纯血尿患者及正常对照组肾小球组织nephrin的表达水平。结果:免疫荧光染色定量分析NS患者肾小球组织nephrin的表达量显著低于单纯血尿患者(P<0 .0 5 )及正常对照组(P<0 .0 5 )。并且NS患者肾小球组织中nephrin的m RNA表达量也显著低于单纯血尿患者(P<0 .0 5 )及正常对照组(P<0 .0 5 )。结论:NS患者肾小球组织nephrin的表达减少,可能影响肾小球滤过屏障的通透性,导致蛋白尿的产生。     

Homocysteine Metabolism in Children with Idiopathic Nephrotic Syndrome

Background

Homocysteine metabolism is altered in children with idiopathic nephrotic syndrome. Hyperhomocysteinemia is a risk factor of early atherosclerosis and glomerulosclerosis and may occur at time of first occurrence of idiopathic nephrotic syndrome.

Methods

Thirty children with first episode of idiopathic nephrotic syndrome (FENS) aged 1–16 years along with 30 age‐ and sex‐matched healthy controls were enrolled in this study. Homocysteine and cysteine were measured with HPLC; vitamin B₁₂ and folic acid were measured with electro‐chemilumiscence immunoassay. Primary outcome measure was plasma homocysteine level in children with FENS and in controls. Secondary outcome measures were (1) plasma and urine homocysteine and cysteine levels in children with FENS at 12 weeks and 1 year (remission) and (2) plasma and urine levels of vitamin B₁₂ and folic acid in children with FENS, at 12 weeks and 1 year (remission).

Results

Plasma homocysteine and cysteine levels were comparable to controls in children with FENS, at 12 weeks and 1‐year remission. Plasma levels of vitamin B12 and folic acid were significantly decreased compared to controls in FENS due to increased urinary excretion, which normalize during remission at 12 weeks and 1 year. Urinary homocysteine and cysteine levels were significantly raised in FENS compared to controls and continued to be raised even at 12‐week and 1‐year remission.

Conclusion

Homocysteine metabolism is deranged in children with FENS. Renal effects of long‐term raised urinary homocysteine levels need to be studied.     

小儿肾病综合征并发血栓形成7例

对7例小儿肾病综合征并发血栓形成的诊断、治疗进行分析,发现小儿肾病综合征者血黏度普遍增高,应及时作相应检查,及时预防及抗凝治疗,防止血栓形成。     

The Everyday Life of Adults with Down Syndrome

Background Aspects of daily life have been considered in a population of people with Down syndrome, followed repeatedly from infancy to 21‐years old, and again at 30‐, 35‐ and 40‐years old. A control sample of non‐disabled babies were seen at the same ages. Method Parents (usually the mothers) and/or carers were interviewed about the people’s health, independence, occupation, leisure activities and social relationships. Results and Conclusions The health of the people with Down syndrome continued to be reasonably good though less good than that of the controls. Engagement in further education increased and attendance at social education centres decreased, while a minority were in employment. Over two‐thirds participated in at least one sport, and although participation decreased over time the fall‐off was no more than is seen in the general population. Fewer than half the people with Down syndrome were thought capable of being left in the house alone for over an hour or to go out alone beyond the garden, and social relationships for the majority were limited.     

Serum Ionized Calcium in Nephrotic Syndrome

Serum ionized calcium was measured in 12 patients with nephroticsyndrome and 14 nephrotic patients during clinical remission.All subjects had normal creatinine clearance and were not receivingtreatment at the time of study. The mean serum ionized calcium values of the two groups of patients,1·08±0·10 (SD) mmol/l ultrafiltrate and1·21±0·10 (SD) mmol/l ultrafiltrate respectively,are significantly lower than the normal mean (1·28±0·06(SD) mmol/l ultrafiltrate). The mean values of the patientsin remission is, however, significantly higher (P < 0·005)than that of the patients with manifest nephrotic syndrome. Elevated serum complexed calcium, and high serum protein-boundcalcium in relation to serum albumin, were encountered, beingmore apparent in patients with overt nephrotic syndrome.     

The Nephrotic Syndrome in Ghana: Clinical and Pathological Aspects

Clinical and pathological features of the nephrotic syndromewere studied in 36 adults and 25 children in Ghana. No evidencewas found to implicate Plasmodium malariae as a cause and inthe majority of patients the aetiology was not identified. Minimal change glomerulonephritis responsive to steroids wasdemonstrated in 14/25 children and 5/36 adults which was surprisingas this lesion has been reported only rarely from tropical Africa.The other major histological lesions were focal segmental glomerulosclerosis(12/61), diffuse proliferative glomerulonephritis (11/61) andmembranous glomerulonephritis (9/61).     

30例肾病综合征患儿的饮食干预

目的探讨饮食干预对肾病综合征患儿的效果。方法将30例肾病综合征患儿随机分为观察组16例,对照组14例。观察组由营养师根据患儿病情制定饮食,对照组由责任护士宣教进行传统饮食。比较2组患儿24h尿蛋白、血清白蛋白和胆固醇差异。结果住院2周后2组患儿24h尿蛋白均显著降低(p<0.01),且2组间比较有显著差异,观察组血清白蛋白明显升高,同时血清甘油三脂和胆固醇下降(p<0.05),而对照组与入院时比较,差异无显著性(p>0.05)。结论合理的饮食干预可显著降低肾病综合征患儿24h尿蛋白,并能提高血清白蛋白水平,降低血清油三脂和胆固醇。     

Osteomalacia and Hyperparathyroid Bone Disease in Patients with Nephrotic Syndrome

Patients with nephrotic syndrome have low blood levels of 25 hydroxyvitamin D (25-OH-D) most probably because of losses in urine, and a vitamin D-deficient state may ensue. The biological consequences of this phenomenon on target organs of vitamin D are not known. This study evaluates one of these target organs, the bone. Because renal failure is associated with bone disease, we studied six patients with nephrotic syndrome and normal renal function. The glomerular filtration rate was 113+/-2.1 (SE) ml/min; serum albumin, 2.3+/-27 g/dl; and proteinuria ranged between 3.5 and 14.7 g/24 h. Blood levels of 25-OH-D, total and ionized calcium and carboxy-terminal fragment of immunoreactive parathyroid hormone were measured, and morphometric analysis of bone histology was made in iliac crest biopsies obtained after double tetracycline labeling. Blood 25-OH-D was low in all patients (3.2-5.1 ng/ml; normal, 21.8+/-2.3 ng/ml). Blood levels of both total (8.1+/-0.12 mg/dl) and ionized (3.8+/-0.21 mg/dl) calcium were lower than normal and three patients had true hypocalcemia. Blood immuno-reactive parathyroid hormone levels were elevated in all. Volumetric density of osteoid was significantly increased in three out of six patients and the fraction of mineralizing osteoid seams was decreased in all. Evidence for an increase in active lacunae (bone-osteoclast interface) occurred in three out of six patients and in inactive (Howship's lacunae) bone resorption in six out of six. The data indicate that the loss of 25-OH-D in urine of patients with nephrotic syndrome and normal renal function may result in a decrease of blood levels of ionized calcium, secondary hyperparathyroidism and enhanced bone resorption. In addition, the vitamin D-deficient state causes osteomalacia as evidenced by defective mineralization and increased osteoid volume.     

环孢霉素A在儿童难治性肾病综合征的应用

目的 :探讨环孢霉素A治疗儿童难治性肾病 (RNS)的疗效。方法 :对 1998年 8月～ 2 0 0 2年 4月收住院的 14例RNS患儿进行CsA治疗后的临床分析。男性 12例 ,女性 2例 ,年龄 2 .5～ 12岁 ,病程 4个月～ 9年。激素依赖 12例 ,激素无效 2例。治疗前单用激素 4例 ,激素联合环磷酰胺 (Cyclophosphamide ,CTX) 10例。CsA4～ 5mg/kg .d ,强的松龙 1.0～ 1.5mg/kg隔日 ,疗程 12个月。结果 :12 / 14获完全缓解 ,2 / 14例部分缓解。根据肾活检病理分为微小病变 (MCNS)与IgM肾病 (IgMN)两组 ,完全缓解率分别为 83.3%和 87.5 % ,统计学无显著差异。血浆白蛋白和血胆固醇水平在治疗后与治疗前比较存在显著性差异 (P <0 .0 1)。 14例患儿CD4与CD8比值治疗前后增加具有统计学意义 (P <0 .0 1)。结论 :中小剂量CsA治疗RNS是安全和有效的 ,IgMNS和MCNS缓解率高     

肾病综合征患儿的韧性与抑郁的相关性

目的调查肾病综合征患儿的韧性和抑郁状况,并探讨两者之间的相关性。方法便利抽样法选取2009年4月至2010年2月在山东省5所三级甲等医院进行治疗的104名肾病综合征患儿为研究对象,采用自制的一般情况调查表、慢性病儿童韧性量表(chronic illness children’s resilience scale,CICRS)、儿童抑郁量表(children’s depression inventory,CDI)对其进行调查。结果肾病综合征患儿韧性水平均分为(99.53±14.33)分,韧性水平差者20例(19.23%);韧性水平较好者60例(57.69%);韧性水平很好者24例(23.08%)。肾病综合征患儿抑郁均分为(11.83±7.83)分,其中出现抑郁症状者18例(17.31%);无抑郁症状者86例(82.69%)。肾病综合征患儿韧性与抑郁评分之间呈中度负相关(r=-0.582,P<0.01)。各维度与抑郁总分均呈负相关(P<0.01)。结论医护人员应开展韧性的干预,以增强儿童的疾病管理行为,促进其心理健康。     

以肾病综合征为表现的毛细血管内增生性肾小球肾炎的临床与病理

目的探讨毛细血管内增生性肾小球肾炎并发肾病综合征的临床病理特点与发病机制。方法12例患者肾穿刺术后,标本经常规固定、包埋、切片,进行HE、六胺银、Masson和免疫荧光染色,其中3例予以电镜观察。结果肾小球系膜细胞、内皮细胞增生;小球内可见中性多核白细胞渗出。10例毛细血管襻和(或)灶性系膜区有免疫复合物沉积。3例上皮细胞足突融合消失,上皮下电子致密物沉积。结论EPGN发生大量蛋白尿的机制与肾小球滤过膜状态的改变关系密切,使其对蛋白的通透性大大增加。     

中医护理干预对肾病综合征的影响

目的:观察中医护理干预对肾病综合征患者协同增效及心理状态的影响。方法:将100例肾病综合征患者分为观察组和对照组各50例,对照组用常规护理及护肾、利尿等对症支持疗法。观察组除常规护理及治疗外,根据肾病综合征患者不同分型给予相应的辨证施护。结果:观察组总有效率88.0%,对照组总有效率74.0%,两组差异有统计学意义(P<0.05)。结论:中医护理干预对肾病综合征的治疗有协同增效作用,而且可以改善患者不良的心理状态。     

Calcium and Phosphorus Metabolism in Nephrotic Syndrome

Calcium and phosphorus balance studies were performed on 13nephrotic patients and eight patients during clinical remissionof the nephrotic syndrome. Marked impairment of intestinal absorptionof calcium was found among nephrotic patients, in eight of whomfaecal calcium equalled or exceeded dietary calcium. The meanfaecal :dietary calcium ratio of nephrotic patients, 1·06±0·23(SD), was significantly higher (p<0·005) than thatof patients in remission, 0·58±0·21 (SD).The mean 24-hour urinary excretion of calcium of nephrotic patients,0·68±0·68 (SD) mmol, was significantlylower (p<0·005) than that of patients in remission,3·02±1·91 (SD) mmol. Calciferol administeredto three nephrotic patients in the dosage of 1.25 mg per daydid not significantly influence intestinal absorption or renalexcretion of calcium. There was no difference between the two groups of patients inintestinal absorption or renal excretion of phosphorus; therewas net intestinal absorption in all subjects. Quantitative bone histology was studied in seven of the nephroticpatients. None had osteomalacia or osteitis fibrosa, while onlyone had evidence of mild osteoporosis.