腎臓学と治療学ジャーナル

Objectives: Studies evaluating geographic barriers to home dialysis utilization in the United States have shown increased rates of utilization of home therapies in rural areas and increased distance to a home dialysis unit (HDU). These large studies may not capture unique regional variation in access to home dialysis. We performed this study to evaluate geographic barriers to home dialysis in the rural Southeastern US.
Methods: We extracted residential zip code and dialysis modality on point-prevalent dialysis patients living in Alabama, Mississippi, and Tennessee. Facility location was identified as well. Data were stratified by patient zip code using Rural Urban Continuum Codes (RUCC) and by circumferential zones differentiated by distance around existing (HDU). 2010 U.S. census data was used for socioeconomic characteristics of each zone. Poisson regression was used to compare prevalence of home dialysis by circumferential zone and RUCC.
Results: Patients more rural or remote from a HDU had higher end-stage renal disease rates, lower median per capita income, higher poverty levels, and were older compared to those within 20 miles of a HDU or urban (all p values <0.001). The adjusted prevalence ratio of home modalities (PD and HHD) was 1.14(95%CI 1.03-1.26) in RUCC codes 4-7, and 1.00 (95%CI, 0.80-1.24) for RUCC code 8-9 when compared to the most urban (RUCC 1-3).There was no difference in adjusted prevalence ratio of home modality utilization regardless of distance to the closest HDU (1.02; 95%CI 0.90-1.16, 0.87; 95% CI 0.72-1.05, and 0.96; 95% CI 0.69-1.34 for 20-30,30-40, and >40 miles respectively) when compared to patients within 20 miles of an HDU.
Conclusions: Prevalence ratio of home dialysis did not differ with distance from an HDU and did not increase in rural areas versus metropolitan areas. Further studies are needed to address if regional variation exists in access to home dialysis care.

Introduction: Most forms of human glomerulonephritis (GN) result from immunologic mechanisms that are mediated by the actions of multiple elements of both the innate and adaptive immune systems, thereby resulting in different clinical manifestations. The treatment of immune-mediated kidney disease is based on steroids and immunosuppressive drugs that interfere with the immune processes. These groups of drugs have led to significant benefits, but severe side effects are still frequent. Monoclonal antibodies directed against molecules of inflammation or several cellular components have emerged in clinical practice. Plasmapheresis and new methods to reduce the risks associated with the procedure with standard therapies may be combined. Moreover new therapeutic options have been proposed, as the use of natural anti-inflammatory cytokines or intracellular signaling reducing inflammation. Materials and Methods: We conducted a systematic review on the pathogenetic mechanisms of glomerulonephritis and their therapies.
Results: We analized all RCTs and quasi-RCTs evaluating the current knowledge about pathogenetic mechanisms of glomerulonephritis and related therapy were considered.
Conclusion: The pathogenetic mechanisms of glomerulonephritis are complex and strongly influenced by immunogenetic factors. Several clinical trials to identify the best therapeutic options for glomerulonephritis have been conducted, but currently, although significant advancements over the last 10 years have been obtained, important questions are still unanswered. Moreover, we need to consider many and important side effects that have the main therapies for glomerulonephritis. Therefore the development of a web enabled data base to assist nephrologists for the treatment of patients with glomerulonephritis is strongly suggested.

Renal involvement especially glomerulonephropathy in polymyositis or dermatomyositis is considered rare. Crescentic glomerulonephritis is even more so. Herein, we report a patient who was diagnosed with concurrent polymyositis and myeloperoxidase-related crescentic glomerulonephritis. We discuss the association between polymyositis or dermatomyositis and renal involvement, glomerulonephropathy, and crescentic glomerulonephritis. This is the first report of a case of polymyositis and myeloperoxidase-related crescentic glomerulonephritis with a clear temporal correlation.

Early diagnosis of monogenic forms of urolithiasis is important to prevent associated renal injury and other treatable disease manifestations, but is often delayed due to lack of knowledge of these rare disorders. Adenine phosphoribosyltransferase (APRT) deficiency is an under-recognized autosomal recessive disorder causing 2,8 dihydroxyadenine (2,8-DHA) urolithiasis and crystalline nephropathy secondary to intratubular 2,8-DHA crystalline precipitation. Patients often present with kidney stones but may also present with renal failure in the absence of stones or nephrocalcinosis. The disease can be efficiently treated by inhibitors of xanthine dehydrogenease (XDH), which makes early diagnosis and treatment essential to prevent recurrence of urolithiasis and nephropathy. Here, we reviewed 67 patients from 56 families with complete APRT deficiency identified at Necker Universitary Hospital, Paris, France, between 1978 and 2014. The initial clinical presentation was urolithiasis in all symptomatic pediatric patients. In adult patients, recurrent nephrolithiasis was a much more common presentation (73%) than crystalline nephropathy (27%). Unfortunately, APRT deficiency was often misdiagnosed and irreversible loss of renal function occurred in 20% of cases. APRT gene sequencing was performed in 54 patients (81%) in 46 families: 25 pediatric patients in 22 families and 29 adult patients in 26 families. 98 mutated alleles were found out of 108 analyzed (91%). Twenty seven different mutations were identified. A single T insertion at the intron 4 splice donor site (c.400+2dup) leading to a truncated protein, accounted for 36 percent of mutated alleles for pediatric and adult cases. This review summarizes the genetic data of a large cohort of pediatric and adult patients along with the issues for diagnosis and management of APRT deficiency and highlights the underdiagnosis associated with the potential severity of APRT deficiency. Early diagnosis is essential for treatment initiation and prevention of renal complications.

We report a case of a 45-year-old woman who presented for nephrotic syndrome and anemia. Her kidney biopsy confirmed the diagnosis of class V membranous lupus nephritis. She had also criterias for antiphospholipid syndrome: miscarriages and antiphospholipid antibodies. She received as immunosuppression treatment corticosteroids and mycophenolate mofetil. Her kidney function deteriorated and three bolus of cyclophosphamide were given. She developed a severe pneumonia and became uremic. She underwent two sessions of acute hemodialysis and developed deep venous thrombosis in her leg. Anticoagulation was then started and her kidney function stabilized for a few months until she was put on chronic dialysis three years and a half after her initial presentation. We emphasize on the factors that worsen the renal prognosis in membranous lupus nephritis patients. We also highlight the importance of recognizing early the antiphospholipid syndrome in lupus nephritis in order to treat it and prevent eventually end-stage renal failure.

The impaired bone mineral metabolism followed byVascular Calcification (VC) will be presentat the beginning stage of Chronic Kidney Disease (CKD). VC can be considered as two major types, which are intimal calcification, associated with atherosclerosis, and medial calcification that involves damaged vascular smooth muscle cells (VSMCs), which leads to increase vascular stiffness and decrease vascular elasticity. Many factors control the mechanisms, and they are imbalances in serum calcium and phosphate, systemic inflammation, hyperparathyroidism, increased matrix degradation, VSMC apoptosis, decreased matrix glutamate protein, etc. These will make VSMCs Trans differentiation to phenotypic osteoblastic cells. In addition, patients with CKD usually have bone turnover problems. For a high turnover status, secondary hyperparathyroidism increases calcium and phosphate release from the bone, but for a low turnover status in a dynamic bone disorder, circulating phosphate and calcium cannot enter the bone to cause serum calcium and phosphate levels to frequently maintain at high levels. This is caused by the fact that the bone can no longer buffer the increases in phosphate and calcium load, and these conditions will cause the possibility of VC. Interestingly, the VC process will secrete sclerostin, a hormone that may act not only locally in the artery wall to reduce mineralization but also destroy bone mineralization. These problems will lead to reduced bone mass with a cycle between bone turnover and VC that only leads to problems. This article will describe the complex relationship between the rate of bone turnover and VC in CKD.

Although the concept of nephroprotection is relatively new but evidences are accumulating to demonstrate that the drugs described to be useful in various renal disorders because of their nephroprotective and nephrotonic effect as described in Unani or other traditional medicines, have diverse therapeutic uses and can be used to manage many renal diseases and their complications or at least to arrest their progression. The traditional systems of medicines would offer some novel drugs, which may be effective in different pathological conditions of the kidney. The drugs of traditional systems of medicines are frequently used to protect the renal function and to delay the progression of renal diseases to CKD and ESRD. In recent past it has been shown that a number of drugs from herbal medicine possess promising nephroprotective effect. The researchers are keen to develop an effective, safe and low cost drug from alternative medicine. However, the drugs identified to be effective in renal diseases on the basis of age-old practices of traditional medicines and few scientific studies need to be evaluated for their pharmacological profile and wide therapeutic potential. This paper gives a detailed account of the traditional drugs useful in renal diseases and the scientific studies conducted so far to validate them.