Measles-Associated Encephalopathy in Children with Renal Transplants

Two children, boys of 8 and 13 years, presented with measles-associated encephalopathy several years after kidney transplantation for congenital nephrotic syndrome. In the absence of prior clinical measles, the neurological symptoms initially eluded diagnosis, but retrospective analysis of stored samples facilitated the diagnosis of measles-associated encephalopathy without recourse to biopsy of deep cerebral lesions. Each had received a single dose of measles mumps and rubella vaccine before 12 months of age. Prior vaccination, reduction of immunosuppression and treatment with intravenous immunoglobulin and ribavirin may have contributed to their survival. Persistent measles virus RNA shedding, present in one child, was not controlled by treatment with i.v. ribavirin. Two years later, both patients continue to have functioning allografts with only minimal immunosuppression. These cases illustrate the difficulty in diagnosing measles-associated encephalopathy in the immunocompromised host, even in the era of molecular diagnostics, and highlight the renewed threat of neurological disease in communities with incomplete herd immunity.     

经皮肾囊穿刺封闭术治疗难治性肾病综合征的护理

对32例难治性肾病综合征患者行经皮肾囊穿刺封闭术治疗,术前加强心理护理,术中密切配合,术后严密监测生命体征,观察药物的不良反应.结果32例中,完全缓解18例,部分缓解9例,总有效率84.4%.     

经皮肾囊穿刺封闭术治疗难治性肾病综合征的护理

对32例难治性肾病综合征患者行经皮肾囊穿刺封闭术治疗，术前加强心理护理，术中密切配合，术后严密监测生命体征，观察药物的不良反应。结果32例中，完全缓解18例，部分缓解9例，总有效率84.4％。     

Renal Response to Atrial Natriuretic Peptide in Nephrotic Syndrome

In six patients with nephrotic syndrome of various aetiology,the increase in absolute and fractional sodium excretion (FE_Na)after a bolus injection of 100 µg -human atrial natriureticpeptide (ANP) was not different from the effect in normal healthysubjects at comparable sodium levels. Glomerular filtrationrate rose in normals as well as in patients. In two patients,however, baseline sodium excretion was very low and the natriureticresponse to ANP was proportionally blunted. The high baselinesodium reabsorption and blunted response to ANP may be explainedas due either to an intrinsic renal defect or to a circulatoryhypovolaemia. The finding of a low plasma ANP in these two patients,however, suggests involvement of a hypovolaemic component.     

Effect of Oral Fructose Load on Serum Uric Acid and Lipids in Kidney Transplant Recipients Treated With Cyclosporine or Tacrolimus

Hyperuricemia, frequently observed following kidney transplantation, may adversely affect graft survival. Although hyperuricemia is a well-known adverse effect of cyclosporine (CsA), a similar effect of tacrolimus (Tac) remains debatable. Hyperuricemia is also seen after oral fructose intake in beverages and processed foods. This sugar is blamed for the epidemic of obesity and metabolic syndrome. The aim of our study was to compare the effects of CsA and Tac on an acute oral fructose load in terms of plasma uric acid, serum lipids, and blood pressure in kidney transplant patients. Thirty-two kidney transplant recipients treated with CsA- or Tac-based triple (calcineurin inhibitor + mycophenolate mofetil + prednisone) immunosuppressive therapy displaying stable allograft function (mean glomerular filtration rate = 53 mL/min/1.73m²) received an oral challenge with 70 g of fructose. Serum uric acid, lipids, and blood pressure were measured before as well as 60, 120, 180, and 240 minutes after fructose administration. A significant increase in serum uric acid was observed in both groups after oral fructose administration (P < .001). A peak increase in serum uric acid was recorded at 120 minutes after fructose intake. Serum total, LDL, and HDL cholesterol also significantly decreased and serum triglycerides increased to a similar extent in both CsA and Tac groups. No significant changes in blood pressure were observed after fructose consumption. Oral fructose intake induced an acute rise in serum uric acid and triglycerides and decrease in serum cholesterol among kidney transplant recipients. Those changes were similar among patients treated with CsA or Tac.     

ACE Gene Polymorphism in Turkish Children with Nephrotic Syndrome

Since 1990, the role of angiotensin converting enzyme (ACE) gene polymorphism in various renal and cardiac diseases is still debated. This study comprised 71 pediatric patients with nephrotic syndrome, 47 males (66%) and 24 females (34%) with a mean age of 57.4 ± 37.6 months, and a control group of 83 healthy males (59%) and 57 healthy females (41%) with a mean age of 505 ± 160.5 months. The distribution of the ACE genotype in the control group was II, 11%; ID, 53%; and DD, 36%, and the nephrotic syndrome was II, 4%; ID, 78%; and DD, 18%. Angiotensin-converting enzyme genotypes were significantly different between patients and control groups (p<0.05). The study groups consisted of 52 (73%) with steroid-sensitive nephrotic syndrome (SNSS) and 19 (27%) with steroid-resistant nephrotic syndrome (SRNS). The distribution of the ACE genotype was II, 6%; ID, 75%; and DD, 19% in the SSNS population and ID, 84% and DD, 16% in the SRNS population. No statistically significant difference was found between steroid sensitivity and ACE genotypes (p=0.5). The results show that ACE I/D polymorphism does not contribute to the steroid resistance, even though this study indicates that the presence of the I/D genotype has a much higher risk—approximately 2.8 times—of having nephrotic syndrome. Further studies with a larger number of patients are needed.     

The Addition of Sirolimus to Cyclosporine and Steroids Inhibits the Anti-Equine Antibody Response in Renal Transplant Recipients Treated with Antithymocyte Globulin

Polyclonal antibodies, such as equine antithymocyte globulin (ATGAM), are known to induce antibody formation. This study evaluated the in vivo effect of sirolimus on antibody formation associated with the use of equine antithymocyte globulin in renal transplant recipients. Recipients of either a living-related donor or cadaveric renal allograft received azathioprine (AZA) (n = 15), mycophenolate mofetil (MMF) (n = 12), or sirolimus (n = 15) in addition to baseline immunosuppression with corticosteroids, cyclosporine, and equine antithymocyte globulin. Immediately before transplantation and weekly for at least 1 month, sequential serum specimens were tested for the presence of human anti-equine antibody using an enzyme-linked immunosorbent assay (ELISA). Anti-equine antibody formation was significantly different among the three treatment groups. Fewer patients receiving MMF (17%, p = 0.007 vs. AZA) and sirolimus (13%, p = 0.003 vs. AZA) developed anti-equine antibody compared with AZA (66%). There was no significant difference (p = 0.81) in the sensitization to equine antithymocyte globulin when comparing the patients receiving MMF or sirolimus. In the sensitized patients, high anti-equine antibody titers (>1 : 500) were more common in those receiving AZA (n = 3) than MMF (n = 0) or sirolimus (n = 1). Compared to AZA, sirolimus, when given in combination with cyclosporine A, significantly reduced anti-equine antibody formation to a degree similar to MMF.     

High-Density Lipoproteins in Untreated Idiopathic Nephrotic Syndrome Without Renal Failure

Serum apoprotein A-I and lipid composition of high-density lipoproteinsin 14 adults and 9 children with idiopathic untreated nephroticsyndrome without renal failure were studied. Serum apoproteinA-I in adults was significantly raised compared to a controlgroup (1.72±0.49 and 1.45±0.15 g/l, Mean±SD,respectively); a similar trend was observed in children (1.88±0.62and 1.52±0.21 g/l, respectively). High-density lipoproteincholesterol was normal in both groups of patients (1.19±0.30and 1.50±0.48mmol/l in adults and in children, respectively),whereas high-density lipoprotein phospholipids were decreasedin adults and increased in children. In addition, apoproteinA-I: high-density lipoprotein cholesterol ratios in both groupsof patients were significantly increased with respect to theircontrol groups. These alterations suggest the existence of qualitativechanges in high-density lipoprotein composition. Despite otherassociated risk factors, and particularly from the point ofview of the ‘high-density lipoprotein hypothesis’,our patients with untreated nephrotic syndrome without renalfailure seem not to be at a higher coronary risk.     

肾病综合征患儿家长的认知干预

目的减轻肾病综合征患儿家长的精神症状,提高其对疾病防护知识的了解程度.方法采用认知干预为主的方式,对38例肾病综合征患儿家长进行心理干预.采用自编疾病防护知识调查表及症状自评量表(SCL-90)分别于入院时(干预前)、入院2周(干预后)进行调查.结果肾病综合征患儿家长疾病防护知识缺乏,干预后,11项疾病防护知识了解率均达100.0%.干预前,除强迫症状、人际关系敏感、精神病性外,家长SCL-90其余各因子均显著高于国内常模(P<0.05,P<0.01);干预后,躯体化、抑郁、焦虑、敌对4个因子评分显著低于干预前(P<0.05,P<0.01),各因子与国内常模比较,差异无显著性意义(均P>0.05).结论心理干预可有效减轻肾病综合征患儿家长的精神症状,提高其对疾病防护知识的了解程度.     

Usefulness of Plasma Exchange in Recurrent Nephrotic Syndrome Following Renal Transplant

Abstract: Nephrotic syndrome due to focal segmental glomerulosclerosis (FSGS) frequently recurs even after transplantation and may cause failure of the renal al-lograft. We report a case in which plasma exchange (PE) was used to treat a 32-year-old patient with biopsy-proven recurrence of FSGS in a second renal transplant after the first allograft had failed. One year after the second renal graft, the patient presented with proteinuria of 5 g/day and a creatinine level of 1.46 mg/dl. A course of 9 PE was performed over a 15-day period. Proteinuria improved rapidly, and 20 days after the last PE, it was already down to 0.8 g/day. This level continued to decrease progressively (0.5 g/day after 3 months) and reached zero after approximately 6 months. Eleven months after PE treatment, the patient had no proteinuria and his plasma creatinine level was normal. In our opinion PE should be instituted early in recurrent nephrotic syndrome after renal transplantation. The optimum frequency of such treatment still has to be established, especially with regard to its use as long-term maintenance therapy.     

Renal Amyloidosis with Nephrotic Syndrome in a Spanish Subcutaneous Heroin Abuser

A 25-year-old drug addict who practiced subcutaneous injectionof heroin and crushed narcotic tablets, a practice known as‘skin popping’, developed nephrotic syndrome secondaryto renal amyloidosis. The patient had chronic inflammation ofthe skin in the form of abscesses and ulcerations. She had practicedsubcutaneous drug administration for only 2 years. Amyloidosishad the staining characteristics and tissue distribution ofthe secondary type, and was directly related to chronic skinsuppuration. To our knowledge, this represents the first reportedcase of renal amyloidosis in a subcutaneous drug abuser residingin Europe.     

Low-Density Lipoprotein Oxidizability and the Alteration of Its Fatty Acid Content in Renal Transplant Recipients Treated With Cyclosporine/Tacrolimus

Renal transplantation is widely used to treat patients with end-stage renal disease. Atherosclerosis is an important posttransplantation risk factor for renal transplant recipients. Subsequent to transplantation low-density lipoprotein (LDL) particles become susceptible to oxidative modification, which results in atherosclerosis. Therefore, the aim of our study was to investigate differences in the susceptibility of LDL particles to oxidation by analyzing LDL fatty acid levels among renal transplant recipients. The changes in lag phases and fatty acid levels of LDL were observed over 4 months among renal transplant recipients treated with Cyclosporine (CsA; n = 7) or Tacrolimus (FK-506; n = 9). We also analyzed cholesterol and triglyceride levels of patients and healthy controls. The lag phase at the 60th day after transplantation was significantly shorter than the results either before transplantation or among control subjects. In conclusion, a similar decrease in lag phase was observed in both above groups, but the FK-506-treated group showed a better lipid profile than the CsA-treated group.     

男性肾病综合征患儿行为及其影响因素的调查

目的 了解男性肾病综合征（NS）患儿的行为及其影响因素。方法 采用Achenbach’s儿童行为量表（CBCL）对32例男性NS患儿（NS组）及45名正常儿童（正常组）进行调查。结果 NS组体诉、社交退缩及攻击性行为因子评分显著高于对照组（p〈0．05，P〈0．01）。痰病病程、年均住院次数、年均复发次数及临床类型为影响NS患儿行为的因素（P〈0．05，P〈0．01）。结论 男性NS患儿的行为问题主要表现为体诉、社交退缩及攻击性，需早期行心理干预治疗。     

男性肾病综合征患儿行为及其影响因素的调查

目的 了解男性肾病综合征(NS)患儿的行为及其影响因素.方法 采用Achenbach's儿童行为量表(CBCL)对32例男性NS患儿(NS组)及45名正常儿童(正常组)进行调查.结果 NS组体诉、社交退缩及攻击性行为因子评分显著高于对照组(P＜0.05,P＜0.01).疾病病程、年均住院次数、年均复发次数及临床类型为影响NS患儿行为的因素(P＜0.05,P＜0.01).结论 男性NS患儿的行为问题主要表现为体诉、社交退缩及攻击性,需早期行心理干预治疗.