Journal de néphrologie et thérapeutique

Paraproteinemias are systemic disorders which cause renal lesions due to deposition of intact immumoglobulins or immunoglobulin fragments. These diseases are difficult to manage and can lead to end stage renal disease. The advances in the field include newer chemotherapy and hematopoietic stem cells which can reverse renal failure in some cases. This review seeks to provide a greater understanding of the trends and outcomes of renal replacement therapy including kidney transplant in patients with paraproteinemic kidney diseases.

This cross-sectional descriptive study was carried out at the hemodialysis unit of Karmouz Health Insurance Hospital, Alexandria, Egypt. The study was conducted on adult End-stage renal disease (ESRD) patients maintained on regular hemodialysis treatment for ≥1 year. Aim was to assess self-perception of ESRD patients undergoing regular Hemodialysis (HD) of their health-related quality of life (HRQOL) and explore resulted scores using kidney disease quality of life short form 36 instrument (KDQOL SF-36). A total of 81 eligible patients joined the study (49 males and 32 females). Aged 50.6 ± 12.7 years. Results showed that HRQOL of HD patients was very poor in all domains. The mean total score was below 50 (out of 100 point) with mean Physical Health Composite (PHC) = 35.57 ± 7.34 and mean Mental Health Composite (MHC) = 36.76 ± 10.22. In general, scores were relatively higher in males, younger age groups, high educational level, employed and/or higher family income. Regarding clinical prolife, this study showed that scores were lower among patients suffering anemia, dyslipidemia, hypoalbuminemia and/or those with comorbidities. Scores for general health survey were significantly lower in anemic patients (with hemoglobin level <11g/dL). Working patients had significantly higher scores than non-working or retired. The study also showed that employment was the only significant predictor for total HRQOL score in a multiple linear regression model was designed to predict total score, while controlling for hemoglobin level, age, duration of being on HD (p = 0.028).

Objective: The aim of this study is to evaluate the epidemiology, costs and outcome of children’s kidney failure in Brazil.
Methods: The study is an observational cohort study based in national govern database. Data were collected from all hospitals affiliated to Brazilian unified health system (SUS). We analyzed all children from neonatal period to 19 years old with diagnosis of renal failure based on the 10th revision of the International Classification of Diseases (ICD-10). Results: Demographic data were collected in eight cohorts from 1998 to 2007. There were 666.725 hospital admissions by kidney disease and 43,174 admissions by kidney failure. The mean of mortality rate, 4% in these cohorts. The admissions and mortality rate have been constant with high mortality under the age of 1 year since 1998. The incidence in boys was higher than in girls but was not significant (p > 0.05). The mean of length of stay was 9.55 days. The mean of annual costs was $5,075,641 million and $588 dollars per patient. In addition, the congenital malformations of the urinary system presented a coefficient of incidence in 1999 of the 0.08/1000 inhabitants or 4 fold less than 2005 (0.44/1000).
Conclusion: Kidney failure is a prevalent healthy problem that affects children from neonatal period to adolescence and presents high post natal mortality. Renal failure is associated to a large spectrum of different ethiologies and different levels of morbidity and consequent impact on the outcome. On account of this early diagnosis improvement and treatments would be established.

Chronic antibody mediated rejection (CAMR) is the main cause for late kidney transplant loss, and the results of its treatment are dissatisfying. In our one center study we evaluated the efficacy of combined treatment with plasma exchanges, intravenous immunoglobulin and rituximab on the top of standard immunosuppression in 24 patients with chronic transplant glomerulopathy (TG), compared to control group of 26 patients, who did not receive additional treatment. At the time of diagnosis baseline estimated glomerular filtration rate (eGFR) did not differ between treatment and control subgroups (44.9 ± 21.3 vs 41.2 ± 14.6 ml/min, P = 0.47), as well as any other laboratory or pathology data, and subsequent decline of allograft function was also found in both subgroups. However, the rate of eGFR decline was significantly lower in the patients from the treatment subgroup compared to the controls: -0.47 ± 0.6 ml/min/month and -1.31 ± 1.6 ml/min/month respectively (P = 0.02). Thus 3-year transplant survival turned to be 21.3% in the control subgroup vs 64.8% in the treatment subgroup (p = 0.01). Our study demonstrated, that TG, which is the most often variant of CAMR, is characterized by unfavorable prognosis regardless of its pathology features and activity at the time of diagnosis. Combined treatment, including plasma exchanges (PE), intravenous immunoglobulin (IVIG) and rituximab (Rtx) allows slowing down the rate of the disease progression at least in some proportion of patients with lately diagnosed CAMR.

Fungal infections are an increasing problem in immunocompromised patients. Members of the genus Penicillium rarely cause infections and are primarily limited to strains of the species Penicillium marnaffei. We are reporting a successfully treated case of culture proven pulmonary Penicillosis caused by Penicillium chrysogenum on the onset of Acute Kidney Injury in a patient of ademocarcinoma lung.

Chronic kidney disease (CKD) management requires a multidisciplinary approach. Although several treatment targets exist, the relationships between a number of clinical criteria and CKD progression have not been studied. Here, we investigated the association between renal dysfunction progression and a number of clinical parameters. We retrospectively enrolled 373 patients with mild impaired renal function indicated by a serum creatinine level > 2.0 mg/dL measured in 2012. We assessed clinical parameters both in 2009 and 2012, and analyzed whether each clinical parameter (e.g., hypertension, diabetes, dyslipidemia, and anemia) met therapeutic targets. We defined a 50% increase in serum creatinine level as baseline, and determined the progression and non-progression groups based on this definition. Systolic blood pressure (SBP), estimated glomerular filtration rate (eGFR), triglyceride, and urinary protein were significantly different between the progression and non-progression groups. The percentage of individuals in the non-progression group decreased with increasing proteinuria (<0.2 g/gCr: 83.3%, <0.3 g/gCr: 82.1%, <0.5 g/gCr: 78.3%, <1.0 g/gCr: 72.8%). In the multiple regression model, the number of clinical criteria achieved was significantly associated with renal progression. Moreover, the model including SBP, HbA1c, urinary protein, and triglyceride; e.g. intensive treatment, showed the strongest relationship (odds ratio 0.65, 95% confidence interval 0.53-0.82, p < 0.001). To prevent renal dysfunction progression, treatment with renin-angiotensin system inhibitor and statin are not sufficient in CKD patients. Intensive treatment of SBP, HbA1c, urinary protein, and triglyceride is essential. Even in patients with low eGFR, exacerbation of renal injuries was prevented with intensive treatment.

Hemolytic uremic syndrome (HUS), a common cause of acute renal failure (ARF) in children, consists of triad of microangiopathic haemolytic anemia (MAHA), thrombocytopenia, and ARF. The aim of the present article is to have a recent overview of HUS including its incidence, etiopathogenesis, clinical profile and management. It consists of two types a) Diarrhoea associated i.e. classical also called D+ HUS b) Non-diarrhoea associated i.e. atypical also called D- HUS. D+ HUS is caused mostly by Escherichia coli or occasionally by Shigella dysenteriae. The causes of HUS are infections, genetic defects, systemic diseases and drugs. The atypical form commonly presents with recurrent or chronic persistent attack. Thrombotic microangiopathy (TMA), the pathologic hallmark, includes HUS, atypical HUS (aHUS), thrombotic thrombocytopenic purpura (TTP) which is characterized by endothelial cell damage and microvascular injury. In the vast majority of aHUS susceptibility factors are familial and not acquired. In D+ HUS, following an attack of diarrhoea or dysentery the child abruptly develops pallor, irritability, swelling of body, oligoanuria, hematuria, and hypertension. Central nervous system disturbances like seizure, obtundation and encephalopathy may occur but less common than aHUS or TTP. Diagnosis of HUS is established by presence of MAHA in the peripherl blood smear i.e. schistocytes, burr cells, helmet cells etc. Thrombocytopenia is nearly always seen. ARF is reflected in elevated blood urea and creatinine level. Coagulation studies like prothrombin time (PT), activated partial thromboplastin time (APTT), will be normal unlike disseminated coagulopathy (DIC). D-dimer level will be raised in HUS similar to DIC. Interestingly, direct Coomb’s test will be positive in Streptococcus pneumonia induced D- HUS. With excellent supportive care and often dialysis D+ HUS will show remarkable recovery whereas in addition plasmapheresis or plasma infusion outcome is poor in most of the D-HUS cases. Though incidence of gastroenteritis is very high in our country, only few patients may develop HUS, the reason of which remains unclear. The incidence of HUS as a cause for ARF in India may be reviewed further. Indiscriminate use of antimicrobials is to be avoided as far as possible to prevent HUS related mortality. Facilities for dialysis may be enhanced in most of the health care facilities in our country as a life saving measure to reduce HUS related mortality and morbidity.