Sickle cell nephropathy with diffuse proliferative lupus nephritis: a case report

<p/> <p>Background</p> <p>Sickle cell nephropathy (SCN) is an important cause of mortality in patients with sickle cell disease. SCA with systemic lupus erythematosus (SLE) is known in children and less common in adults, however diffuse proliferative lupus nephritis (DPLN) with SCN has rarely been reported in adults. It requires early diagnosis and aggressive management.</p> <p>Case presentation</p> <p>We present here a 35 years old lady with sickle cell disease who presented with edema, dyspnoea on exertion, pyuria and had raised s. creatinine of 7 mg%. Her biopsy revealed SCN with DPLN. She is on maintenance hemodialysis after 2 months of diagnosis.</p> <p>Conclusion</p> <p>DPLN with SCN is a rare entity with poor prognosis, which may be overlooked and needs aggressive management.</p

Urinary Tract Infection in Renal Allograft Recipents

Renal replacement therapy in the form of renal transplantation (RT) is the treatment of choice in these patients. Various factors influence the graft survival, infections being most common. Infections account for 16% of patient deaths and 7.7% of death censored graft failure in renal transplant patients. Urinary tract infection (UTI) is the most common infectious complication accounting for 45–72% of all infections. According to few studies UTI may have a negative impact over the long term survival of renal allograft. There are multiple factors that predispose these patients to UTI. Elderly age group, female gender, increased duration of catheterization and anatomical abnormalities of the urinary tract are most common predisposing factors. E. coli is the most frequently isolated organisms from the urine of these patients. We would proceed further with two cases which presented as UTI in post-transplant period. The first patient transplanted (living donor related) for diabetes induced end stage renal disease had developed UTI 4 years post-transplant. The other patient underwent deceased donor renal transplant for adult polycystic disease related chronic kidney disease, presented 2 years post-transplant with UTI

Pathology Associated with Hormones of Adrenal Cortex

Adrenal gland is an endocrine organ comprising of an outer cortex and inner medulla. These secrete various hormones that have a vital role in maintaining the normal homeostasis of the body. Lesions of adrenal cortex are quite common to encounter and most of these are related to the hormones secreted by three layers of adrenal cortex: the zona glomerulosa, the zona fasciculata, and the zona reticularis. Also it is very infrequent to encounter metastatic lesions in the adrenal glands too. So it is very important as a part of a clinician as well as a pathologist to know the pattern in which these hormones are secreted along with their physiological roles. Thus this chapter includes the disease that are related to excess as well as deficiencies of the hormones secreted by adrenal cortex. The chapter also includes various genetic syndromes that are associated with the disorders associated with hormones of adrenal cortex. The last part of the chapter includes a brief description of various benign as well as malignant lesions, the pathological as well as the etiological aspects and the hormonal abnormalities associated. This chapter thus mainly focuses on the pathology associated with the adrenal cortex and hormones secreted by the various layers of adrenal cortex

Mitochondrial Cytopathies of the Renal System

Mitochondria are major intracellular organelles with a variety of critical roles like adenosine triphosphate production, metabolic modulation, generation of reactive oxygen species, maintenance of intracellular calcium homeostasis, and the regulation of apoptosis. Mitochondria often undergo transformation in both physiological and pathological conditions. New concepts point that mitochondrial shape and structure are intimately linked with their function in the kidneys and diseases related to mitochondrial dysfunction have been identified. Diseases associated with mitochondrial dysfunction are termed as “mitochondrial cytopathies”. Evidence support that there is a role of mitochondrial dysfunction in the pathogenesis of two common pathways of end-stage kidney disease, namely, chronic kidney disease (CKD) and acute kidney injury (AKI). Mitochondrial cytopathies in kidneys mainly manifest as focal segmental glomerular sclerosis, tubular defects, and as cystic kidney diseases. The defects implicated are mutations in mtDNA and nDNA. The proximal tubular cells are relatively vulnerable to oxidative stress and are therefore apt to suffer from respiratory chain defects and manifest as either loss of electrolyte or low-molecular-weight proteins. Patients with mitochondrial tubulopathy are usually accompanied by myoclonic epilepsy and ragged red muscle fibers (MERRF), and Pearson’s, Kearns-Sayre, and Leigh syndromes. The majority of genetic mutations detected in these diseases are fragment deletions of mtDNA. Studies have shown significantly increased ROS production, upregulation of COX I and IV expressions, and inactivation of complex IV in peripheral blood mononuclear cells of patients with stage IV–V CKD, thereby demonstrating the close association between mitochondrial dysfunction and progression to CKD. Furthermore, the mechanisms that translate cellular cues and demands into mitochondrial remodeling and cellular damage, including the role of microRNAs and lncRNAs, are examined with the final goal of identifying mitochondrial targets to improve treatment of patients with chronic kidney diseases

Extrarenal retroperitoneal angiomyolipoma with oncocytoma

The simultaneous presence of renal angiomyolipoma and oncocytoma is a rare occurrence. Extrarenal retroperitoneal angiomyolipoma is an even more rare neoplasm, and its simultaneous presence with renal oncocytoma has not been documented. We present herein the first case to be reported in English literature

Effects of a Tolerance Induction Protocol in Renal Allograft Recipients — the Ahmedabad Experience

BackgroundThe most interesting recent development in transplantation immunobiology is the clinical implementation of tolerance induction. We report our experience of megadose hematopoietic stem cell transplantation with non-myeloablative minimum conditioning in renal allograft recipients.MethodsThis was a retrospective, single-center study of 159 renal allograft biopsies from two groups of patients: one group underwent a tolerance induction protocol (TIP) before renal transplantation; the other underwent renal transplantation directly. Biopsies were classified into two subgroups to differentiate between acute and late rejection: 127 biopsies, comprising 64 from patients who underwent a TIP and 63 from controls, performed within 180 days after transplantation; and 32 biopsies, comprising 26 from patients who underwent a TIP and six from controls, performed 180 days after transplantation. All patients received cyclosporine 7 mg/kg/day, tapered to 3 mg/kg/day 3 months after transplantation, and subsequently continued at the latter dosage.ResultsThere was markedly less immunologic injury (i.e. generally fewer and milder rejection episodes) evident in biopsies from patients who underwent TIP than in biopsies from controls. Cyclosporine toxicity was considerably greater in patients from the TIP versus control group (82.9% vs 40.6%).ConclusionTIP protects renal allografts from immunologic injury and has an unexplained cyclosporine-sparing effect

Clinicopathological study of nondiabetic renal disease in type 2 diabetic patients: A single center experience from India

Diabetic nephropathy (DN) is a major complication of diabetes mellitus (DM), leading to chronic kidney disease/end-stage renal disease. Wide spectrum of nondiabetic renal diseases (NDRD) is reported in type-2 diabetes (type-2 DM). We carried out this single-center study to find clinical, laboratory, and histological features of NDRD in type-2 DM patients and to assess the prevalence of NDRD in India. A single-center retrospective study which included analysis of renal biopsies from patients with type-2 DM, performed between January 2008 and September 2016. Biopsy findings were categorized into three groups, Group-I (isolated NDRD); Group-II (NDRD superimposed on underlying DN); and Group-III (isolated DN). Out of 152 diabetic patients (111 males and 41 females), 35 (23.03%) patients were of Group-I (isolated NDRD), 35 (23.03%) of Group-II (NDRD superimposed on underlying DN), and 82 (53.95%) of Group-III (isolated DN). The mean age (in years) was 55.08 ± 10.71, 55.65 ± 8.71, and 54.45 ± 9.01 respectively in Group-I, II, and III. Nephrotic syndrome (NS) was the most common clinical presentation in all groups. Duration of DM was significantly shorter in Group-I than in Group-II. Diabetic retinopathy was absent in Group-I. Proteinuria was more in Group-III than Group-I. Low serum C3 and/or C4 levels was observed in five (14.29%) cases of Group-I and Group-II each and two (2.43%) cases of Group-III. Nearly, 70 (46.05%) patients were found to have NDRD either in isolated form or as combined lesions. The most common histological types of NDRD were acute tubulointerstitial nephritis (38.57%) followed by benign nephrosclerosis (15.72%), membranous nephropathy (10%), IgA nephropathy (7.14%), and membranoproliferative glomerulonephritis (7.14%). The incidence of NDRD (with/without DN) in type-2 DM is very high. Shorter duration of diabetes, hematuria, absence of retinopathy, low serum complement levels, and nephrotic range proteinuria are predictors of NDRD