Management of steroid sensitive nephrotic syndrome: revised guidelines

JUSTIFICATION: In 2001, the Indian Pediatric Nephrology Group formulated guidelines for management of patients with steroid sensitive nephrotic syndrome. In view of emerging scientific evidence, it was felt necessary to review the existing recommendations. PROCESS: Following a preliminary meeting in March 2007, a draft statement was prepared and circulated among pediatric nephrologists in the country to arrive at a consensus on the evaluation and management of these patients. OBJECTIVES: To revise and formulate recommendations for management of steroid sensitive nephrotic syndrome. RECOMMENDATIONS: The need for adequate cortico-steroid therapy at the initial episode is emphasized. Guidelines regarding the initial evaluation, indications for renal biopsy and referral to a pediatric nephrologist are updated. It is proposed that patients with frequently relapsing nephrotic syndrome should, at the first instance, be treated with long-term, alternate-day prednisolone. The indications for use of alternative immunosuppressive agents, including levamisole, cyclophosphamide, mycophenolate mofetil and cyclosporin are outlined. The principles of dietary therapy, management of edema, and prevention and management of complications related to nephrotic syndrome are described. These guidelines, formulated on basis of current best practice, are aimed to familiarize physicians regarding management of children with steroid sensitive nephrotic syndrome.     

Phenotype of dent disease in a cohort of Indian children

Objective

To describe the clinical and genotypic features of Dent disease in children diagnosed at our center over a period of 10 years.

Design

Case series.

Setting

Pediatric Nephrology Clinic at a referral center in Northern India.

Methods

The medical records of patients with Dent disease diagnosed and followed up at this hospital from June 2005 to April 2015 were reviewed. The diagnosis of Dent disease was based on presence of all three of the following: (i) low molecular weight proteinuria, (ii) hypercalciuria and (iii) one of the following: nephrolithiasis, hematuria, hypophosphatemia or renal insufficiency, with or without mutation in CLCN5 or OCRL1 genes.

Results

The phenotype in 18 patients diagnosed with Dent disease during this period was characterized by early age at onset (median 1.8 y), and polyuria, polydipsia, salt craving, hypophosphatemic rickets and night blindness. Rickets was associated with severe deformities, fractures or loss of ambulation in six patients. Nephrocalcinosis was present in three patients, while none had nephrolithiasis. Generalized aminoaciduria was seen in 13 patients, two had glucosuria alone, and one had features of Fanconi syndrome. Over a median follow up of 2.7 years, one patient developed renal failure. Genetic testing (n=15) revealed 5 missense mutations and 3 nonsense mutations in CLCN5 in 13 patients. Five of these variations (p.Met504Lys, p.Trp58Cys, p.Leu729X, p.Glu527Gln and p.Gly57Arg) have not been reported outside the Indian subcontinent.

Conclusion

Our findings suggest a severe phenotype in a cohort of Indian patients with Dent disease.

     

纤维蛋白原检测的指南

英国血液学界通常通过纤维蛋白原的测定来判断纤维蛋白量的降低和质的异常,评估出血危险性。纤维蛋白原的升高通常预示各种缺血性事件的存在,建议进行纤维蛋白原检测就是基于这种观点。 纤维蛋白原的测定方法有多种,其中Clauss检测法(以凝血酶时间为基础)是英国医院最常采用的,它可选用多种检测试剂和测定方法。许多实验室配置了自动凝集仪,其中许多是根据光散射变化的差异或凝血酶原时问(PT-Fg)检测时光密度的变化来计算纤维蛋白原的量。PT-Fg法检测中还存在一系列的问题,     

Risk of cardiac arrhythmias and conduction abnormalities in patients with acute myocardial infarction receiving packed red blood cell transfusions

Purpose

Although transfusion has been linked to the development of atrial fibrillation (AF) in cardiac surgical patients, this association has not been investigated in patients with acute myocardial infarction (AMI). Evidence supports an inflammatory mechanism in the development of AF, and red cell transfusions also elicit an inflammatory response. We therefore sought to evaluate whether packed red blood cell transfusion increases the risk of AF, ventricular tachycardia (VT), and other arrhythmias and conduction abnormalities in patients with AMI.

Materials and Methods

This is a retrospective study on patients with AMI and no prior history of AF, admitted to a critical care area and entered in Project Impact database from 08/2003-12/2007. Primary outcome measures were new-onset cardiac arrhythmias or conduction disturbances.

Results

Transfused patients had significantly higher incidences of AF (4.7% vs 1.3%, P = .008), cardiac arrest (9.5% vs 1.7%, P < .001) and heart block (3.4% vs 0.1%, P < .001), and a trend toward a higher incidence of VT (3.4% vs 1.3%, P = .058). Multivariate regression analysis confirmed transfusion as an independent risk factor for “non-lethal” cardiac events (AF/heart block; odds ratio [OR], 4.7 [1.9-11.9]; P = .001), “lethal” events (VT/cardiac arrest; OR, 2.4 [1.1-5]; P = .016), and all cardiac events (OR, 2.8 [1.5-65.1]; P = .001). Transfused patients had significantly longer length of stay (P < .0001) and significantly higher mortality rates than nontransfused patients (OR, 3 [1.7-5.5]; P < .001).

Conclusions

Packed red blood cell transfusion is independently associated with an increased risk of new-onset cardiac arrhythmias and conduction abnormalities in the setting of AMI, even after controlling for traditional risk factors.     

Prevalence and predictors of peripheral neuropathy in nondiabetic children with chronic kidney disease

Introduction: This study sought to determine the prevalence and predictors of peripheral neuropathy in nondiabetic children with chronic kidney disease (CKD). Methods: Fifty‐one consecutive normally nourished children, 3–18 years of age, with CKD stages IV and V of nondiabetic etiology were enrolled from May to December 2012. Nerve conduction studies were performed in 50 children. Blood samples were analyzed for the biochemical parameters, trace elements, and micronutrients. Results: The prevalence of peripheral neuropathy in our cohort was 52% (95% confidence interval 37.65, 66.34). The majority (80.8%) of the children had axonal neuropathy, and 11.5% had demyelinating neuropathy. Isolated motor neuropathy was identified in 92.3% of the children, and sensorimotor neuropathy was identified in 7.6%. The significant risk factors associated with peripheral neuropathy were older age, low serum copper, and dialysis therapy. Discussion: Electrodiagnostic studies should be performed in children with CKD to assess for peripheral neuropathy for the purpose of optimizing medical care. Muscle Nerve 57 : 792–798, 2018     

Troubleshooting electromagnetic interference in a patient with centrifugal flow left ventricular assist device and subcutaneous implantable cardioverter defibrillator

A 25‐year‐old man with severe nonischemic dilated cardiomyopathy underwent subcutaneous implantable cardioverter defibrillator (S‐ICD) implant and subsequently underwent HeartWare ventricular assist device (HVAD) placement. Postoperative interrogation revealed both primary and secondary S‐ICD vectors inappropriately regarded sinus rhythm as “noise,” and the alternate vector significantly undersensed sinus rhythm. The S‐ICD was reinterrogated using high‐resolution capture to visually confirm EMI with a dominant frequency in both the primary and secondary vectors of 46.67 Hz that fell within the S‐ICD operational range of 9–60 Hz. The 46.67 Hz frequency correlated with the HVAD operational speed of 2,800 RPM. The HVAD pump speed was increased from 2,800 to 3,000 RPM, resulting in a dominant frequency of 50 Hz. The notch filter is nonprogrammable in S‐ICDs. However, the built‐in filter is 50 Hz for countries in European time zones as opposed to 60 Hz in US time zones due to differences in the anticipated noise from electrical sources within each continent. Thus, the S‐ICD time zone was reprogrammed from EST to GMT, which reduced the notch filter from 60  to 50 Hz, resulting in S‐ICD successfully eliminating EMI when the patient was in a supine position. The EMI interference was still intermittently present in the upright patient position. This case demonstrates the utility of high‐resolution electrogram capture to identify the source and frequency of EMI in S‐ICD and offers a potential avenue to troubleshoot dominant frequency oversensing by changing the device time zone.     

有症状门诊病人深静脉血栓形成的诊断,临床评价和D-二聚体分析可能减少对影像学诊断的需要

深静脉血栓形成(DVT)的年发病率为48-182／10万,一般估计为1／1000。DVT病死率为1%-5%,发病率和病死率与年龄密切相关。慢性疼痛、肿胀、偶尔腿部皮肤溃疡等血栓后综合征见于1／3发生过DVT的患者。血栓后综合征可出现较早,也可迟至10年才出现,总的发病率为2年23%,5年28%。患者如使用弹力加压袜至少2年以上,腿部病变的发生率可     

Pyelonephritis Presenting with Severe Acute Renal Failure

We describe an unusual case of an 8-yr-old child presenting with low grade fever and acute renal failure. Investigations showed blood urea 246 mg/dl, serum creatinine 6.4 mg/dl, microscopic hematuria and 2+ proteinuria. Renal biopsy was done in view of rapidly worsening kidney function and showed dense lymphoplasmacytic infiltrate and neutrophils with focal areas of interstitial necrosis, confirming acute pyelonephritis. Ultrasonography and MRI demonstrated multiple renal abscesses. He was managed with antimicrobial therapy and hemodialysis.