The Clinical Course of Minimal Change Nephrotic Syndrome With Onset in Adulthood or Late Adolescence: A Case Series.

BACKGROUND: Few studies have examined the treatment and outcome of adult-onset minimal change nephrotic syndrome (MCNS). We retrospectively studied 125 patients who had MCNS with onset in either adulthood or late adolescence. Presenting characteristics, duration of initial treatment and response to treatment, relapse patterns, complications, and long-term outcome were studied. STUDY DESIGN: Case series. SETTING & PARTICIPANTS: Patients with new-onset nephrotic syndrome 16 years or older and a histologic diagnosis of MCNS in 1985 to 2011 were identified from pathology records of 10 participating centers. OUTCOMES: Partial and complete remission, treatment resistance, relapse, complications, renal survival. RESULTS: Corticosteroids were given as initial treatment in 105 (84%) patients. After 16 weeks of corticosteroid treatment, 92 (88%) of these patients had reached remission. Median time to remission was 4 (IQR, 2-7) weeks. 7 (6%) patients initially received cyclophosphamide with or without corticosteroids, and all attained remission after a median of 4 (IQR, 3-11) weeks. 13 (10%) patients reached remission without immunosuppressive treatment. One or more relapses were observed in 57 (54%) patients who received initial corticosteroid treatment. Second-line cyclophosphamide resulted in stable remission in 57% of patients with relapsing MCNS. Acute kidney injury was observed in 50 (40%) patients. Recovery of kidney function occurred almost without exception. Arterial or venous thrombosis occurred in 11 (9%) patients. At the last follow-up, 113 (90%) patients were in remission and had preserved kidney function. 3 patients with steroid-resistant MCNS progressed to end-stage renal disease, which was associated with focal segmental glomerulosclerosis lesions on repeat biopsy. LIMITATIONS: Retrospective design, variable treatment protocols. CONCLUSIONS: The large majority of patients who had MCNS with onset in adulthood or late adolescence were treated with corticosteroids and reached remission, but many had relapses. Cyclophosphamide resulted in stable remission in many patients with relapses. Significant morbidity was observed due to acute kidney injury and other complications. Progression to end-stage renal disease occurred in a few patients and was explained by focal segmental glomerulosclerosis.

[A woman with ongoing anaemia]. / Een vrouw met persisterende anemie.

In a 65-year-old female with iron deficiency anaemia, video capsule endoscopy showed an ulcerative polyp in the proximal ileum. After histological examination we made the diagnosis 'granuloma pyogenicum', a rare cause of gastrointestinal bleeding. After partial resection of the small intestine, the anaemia was cured.

[Liver cirrhosis due to hepatitis E in a kidney transplant patient]. / Levercirrose door hepatitis E bij een niertransplantatiepatiënte.

Until recently, hepatitis E was considered to be an infectious disease that resolved without any long-term complications. We describe a 47-year-old woman who presented with a decompensated liver cirrhosis with ascites and peripheral oedema, 14 years after successful kidney transplantation. A previous extensive analysis of persistent liver enzyme disorders had not yielded a diagnosis, whereas now laboratory tests showed slightly abnormal liver results. The CT scan revealed ascites with signs of a decompensated liver cirrhosis. A liver biopsy revealed an active micronodular cirrhosis. Serological tests into the usual infectious causes of hepatitis provided no conclusive evidence but PCR on hepatitis E virus RNA and ELISA on virus-specific IgM and IgG were both positive on 2 occasions, suggestive for an active hepatitis E infection, the probable cause of the cirrhosis. The patient died several weeks later as a consequence of hepatic and renal failure. Hepatitis E was previously regarded as a travel-related disease from endemic areas. However, it is increasingly being observed in Western countries as well, where infection can take place due to intensive contact with animals and the consumption of infected meat.

Microbiological quality and quality control of purified water and ultrapure dialysis fluids for online hemodiafiltration in routine clinical practice.

During online hemodiafiltration, patients are directly infused with sterile substitution solutions to maintain fluid balance. Adequate water treatment and a well-organized quality control process are essential to provide non-pyrogenic fluids with consistent optimal quality. We sought to assess water quality, the water treatment system, and the methods for surveillance of microbiological water quality in 10 Dutch dialysis centers that routinely treat patients with hemodiafiltration. Microbiological monitoring results (micro-organisms and endotoxins) were collected over a 1-year period representing 11,258 hemodiafiltration sessions covering 97 patients. In all centers, water purification was based on a reverse osmosis module in combination with a second reverse osmosis and/or an electrodeionizer. All centers regularly and routinely monitored the microbiological purity of the dialysis water with adequate analytical methods but with variable monitoring frequency. Microbiological assessments were compliant with reference quality levels in 3923 of 3961 samples. Our study suggests that non-pyrogenic substitution fluids can be produced online for a prolonged period of time. It is likely that the current Dutch Quality of Care Guideline has contributed to high-quality water treatment and a well-organized control process.

Extracorporeal elimination in acute valproate intoxication.

Severe poisoning with valproate may result in coma and death. The management of valproate intoxication is principally supportive. Valproate is scarcely excreted renally and is mainly protein bound and, therefore, not considered to be amenable for extracorporeal elimination. Despite these unfavourable pharmacokinetic properties, several case reports showed successful treatment of valproate intoxication with haemodialysis and/or haemoperfusion. We describe a male patient (57 years) after ingestion of 64 g of valproate. The patient was successfully treated with haemodialysis for 6 h. Haemodialysis was followed by continuous venovenous haemodiafiltration (CVVH-D) for 18 h to prevent a rebound phenomenon. This report confirms the benefit of haemodialysis in serious valproate overdose. A review of the literature shows that haemodialysis followed by CVVH-D is the treatment of choice in severe valproate intoxication.

Urinary excretion of beta2-microglobulin and IgG predict prognosis in idiopathic membranous nephropathy: a validation study.

An accurate prediction of the prognosis of patients with idiopathic membranous nephropathy (iMN) should allow restriction of immunosuppressive treatment to patients who are at highest risk for ESRD. On the basis of retrospective studies, it has previously been suggested that the urinary excretions of beta2-microglobulin (Ubeta2m) and IgG (UIgG) are useful predictors of renal insufficiency in patients with iMN. The threshold values of 0.5 micro/min (Ubeta2m) and 250 mg/24 h (UIgG) have been validated in a new and larger patient cohort. From 1995 onward, 57 patients with iMN (38 men, 19 women; age 48 +/- 16 yr), a nephrotic syndrome, and a serum creatinine level 50%. Mean (+/-SD) follow-up was 53 +/- 23 mo. Thus far, 25 (44%) of the patients have reached the end point renal death. Multivariate analysis confirmed Ubeta2m as the strongest independent predictor for the development of renal insufficiency. Sensitivity and specificity were 88 and 91%, respectively, for Ubeta2m, and both were 88% for UIgG. When the excretions of both proteins were combined, specificity improved to 97%. It is concluded that the present data validate the accuracy of Ubeta2m and of UIgG in predicting renal outcome in patients with iMN. These markers can be used to guide decisions on the start of immunosuppressive treatment.