Journal of nephropathology
Volume:2 Issue: 1, Jan 2013

Context: Vasculitis is a clinicopathological entity characterized by inflammation and necrosis of blood vessels..Evidence Acquisitions: Directory of Open Access Journals (DOAJ), Google Scholar,Pubmed (NLM), LISTA (EBSCO) and Web of Science have been searched.. Two major autoantigens for ANCA are myeloperoxidase (MPO) and proteinase 3 (PR3), which are proteins in the primary granules of neutrophils and in the lysosomes of monocytes. They are expressed in mature neutrophils of patients with ANCA, while absent in healthy subjects.. The kidney is the most commonly affected vital organ in ANCA-associated vasculitis, and patient outcomes are largely determined by the severity of renal disease at diagnosis and by its response to treatment..

Context: Oxidative stress has crucial role in pathogenesis of diabetic nephropathy (DN). Despite satisfactory results from antioxidant therapy in rodent, antioxidant therapy showed conflicting results in combat with DN in diabetic patients..Evidence Acquisitions: Directory of Open Access Journals (DOAJ), Google Scholar,Pubmed (NLM), LISTA (EBSCO) and Web of Science have been searched.. Treatment of DN in human are insufficient with rennin angiotensin system (RAS) blockers, so additional agent ought to combine with this management. Meanwhile based on DN pathogenesis and evidences in experimental and human researches, the antioxidants are the best candidate. New multi-property antioxidants may be improved human DN that show high power antioxidant capacity, long half-life time, high permeability to mitochondrion, improve body antioxidants enzymes activity and anti-inflammatory effects.. Based on this review and our studies on diabetic rats, rosmarinic acid a multi-property antioxidant may be useful in DN patients, but of course, needs to be proven in clinical trials studies.

There is no information on the frequency and clinicopathological presentation of the variants of primary focal segmental glomerulosclerosis (FSGS) in adults presenting with idiopathic nephrotic syndrome (INS) in Pakistan.. The aim of this study was to determine the frequencies of different histologic variants of primary FSGS with INS at our center and to compare our findings with those published in literature..Patients and All consecutive adults (≥18 years) with INS, and diagnosis of FSGS on renal biopsies, were included. Their clinicopathological features at the time of presentation were retrieved and compared among the variants.. There were 120 (65.2%) males and 64 (34.8%) females. The mean age was 30.62±12.02 years. The mean 24-hr urinary protein excretion was 4.69±2.36 grams. Microscopic hematuria was found in 30 (16.3%) patients. The mean serum creatinine was 1.58±0.87 mg/dL. At presentation, 128 (69.6%) patients were normotensive, while 56 (30.4%) exhibited hypertension. FSGS, not otherwise specified (NOS) was the predominant variant, comprising 76.6% of all; collapsing variant comprised 12%, tip variant, 9.8%, perihilar, 1.1%, and cellular, 0.5%. The mean number of glomeruli involved by segmental scarring was 3.41±2.87 and there was significant difference among the variants (p= 0.001). Arteriolopathy was found in 23.4 % cases and fibrointimal thickening of arteries in 18.5%. Tubular atrophy and interstitial fibrosis (IF/TA) was noted in 93% of cases. There was no significant difference in vasculopathy and IF/TA among the variants.. Collapsing variant was the second most common variant following NOS and these findings are different from other regional studies..

Idiopathic steroid resistant nephrotic syndrome (ISRNS) represents about 10-20% of children with nephrotic syndrome with variable outcome.. To determine the histological patterns of ISRNS in Egyptian children and the histological details of the commonest types which might be the reason for the steroid resistance..Patients and The study included 53 cases with ISRNS. Their renal biopsies were retrieved from the archive of Electron microscopy unit and pathology department, Ain Shams University Specialized Hospital (ASUSH) in the duration from 2005-2011. The biopsies were examined histologically, with immunohistochemistry, and by electron microscopy.. They were 36 males (67.9%) and 17 females (32.1%), the age at diagnosis ranged from 1.5- 16 years with a mean of 6.71 years. Lower limb oedema was the commonest presentation (100%), haematuria was revealed in (17%) of cases. Histological examination showed three major patterns; Focal segmental glomerulosclerosis (FSGS) in 30.2%, minimal change glomerulopathy (MCG) in 24.5% and IgA nephropathy in 13.2 %. Mesangial hypercellularity was very common among MCG patients (85.3% ±6.7). Tubulointerstitial inflammation and fibrosis were common among cases with IgA nephropathy (40.4% ±11, 53.7% ±8 respectively).. ISRNS in Egyptian children could be attributed mainly to three major diseases (FSGS, MCG and IgA nephropathy). Mesangial hypercellularity and severe tubulointerstitial disease might be the major causes for steroid resistance in MCG and IgA nephropathy respectively. Renal biopsy with electron microscopy examination should be done for all children with nephrotic syndrome at first time of presentation for proper assignment of treatment protocol.

About 30-40% of diabetic patients who developed nephropathy have lipoprotein disorders, especially lipoprotein a [Lp(a)], which is related to atherosclerosis.. The aim of this study was to investigate the effect of N-acetyl cysteine (NAC) on the serum levels of Lp(a) and amount of proteinuria in a group of type 2 diabetic patients with diabetic nephropathy..Patients and A total of 40, type 2 diabetic (T2D) patients, patients with proteinuria, were randomly divided into two groups. The experimental group was treated by NAC (1200 mg/day) for two months in conjunction with conventional treatment for diabetes and hypertension. Control group received routine medications.. No significant change was identified in serum Lp(a) during treatment with NAC (P >0.05). However, NAC decreased the amount of proteinuria, serum triglyceride (TG) level and systolic blood pressure in experimental group compared to the control group (P <0.05).. These findings suggest that treatment with NAC has no significant effect on the serum level of Lp (a). However, it has beneficial effects on the reduction of proteinuria, serum TG level and systolic blood pressure in T2D patients with nephropathy. Further prospective studies are needed to determine its full role.

Hypertension (HTN), is a very common disorder with a substantial impact on public health because of its complications.. Little is known about the association of serum lipoprotein(a)[Lp(a)] elevation with essential hypertension(eHTN)..Patients and This study was conducted on a group of essential hypertensive patients. Laboratory exams consisting of serum creatinine and Lp(a) was measured.. 95 essential hypertensive patients was enrolled to the study. Serum Lp(a) in patients was 46.5±20. In patients an inverse correlation between seum Lp(a) with creatinine clearance was seen (r = -0.175 p<0.05).Significant correlation of serum Lp(a) with age (r =0.191 p<0.05) and also a significant correlation of serum Lp(a) with duration of HTN(known duration of hypertension period) (r =0.362 p<0.05) were found.. Our data suggest that Lp(a) may be involved as a cofactor in essential hypertension, with a mechanism that remains to be elucidated..

Isolated or predominant tubulointerstitial lupus nephritis is rare.. Here we report the case of a thirty eight years old male who was diagnosed with systemic lupus erythematosus (SLE) according to clinical and laboratory criteria and presented with impaired renal function and non nephrotic range proteinuria. Renal biopsy revealed normal glomeruli but interstitial momonuclear cell infiltration. Immunohiostochemistry (IHC) showed immune deposits in the tubular basement membranes (TBMs), and the peritubular capillary basement membranes (PTCBMs). He was started on high dose oral steroids, which were gradually tapered over one month. His renal functions improved over few days and normalized by the end of the first month of treatment. He was continued on low dose steroids and azathioprine with no evidence of relapse.. Predominant tubulointerstitial lupus can occur, although rarely; and it runs a favorable course with good response to treatment..

Antiphospholipid syndrome (APS) is a systemic disorder characterizes by recurrent arterial and venous thrombosis and/or pregnancy miscarriages and positive test for antiphospholipid antibodies..Case: Here we report a 31 year-old female whose main complain was symmetrical motor polyneuropathy and hand muscular weakness over a long period of time. Her clinical picture became more complex by Mitral valve regurgitation and renal dysfunction. Finally with the diagnosis of APS glucocorticoid therapy was started and her renal dysfunction improved profoundly, however her cardiac valvular involvement and peripheral neuropathy remained.. APS is a disease with wide clinical presentations and it continues to confound the clinicians..

Two different case reports, which have been published previously, suggested that bilateral nephrectomy can improve sever and refractory hemolytic uremic syndrome (HUS) in adults without a history of transplantation. At this study, kidney transplant nephrectomy in a patient with sever post transplant HUS was investigated..Case: Patient was a 55 years old man with a single small size kidney and end-stage renal disease (ESRD). He had received a kidney from an unrelated donor three months before admission. The patient was admitted with fever and acute renal failure. Clinical and laboratory evaluation wereconsistent with sever De novo hemolytic uremic syndrome (HUS). Different therapeutic regimens administered in this patient including intensive plasma exchange, plasma infusion, empirical antibiotics, and high doses of corticosteroid. Although Cyclosporine was changed to Tacrolimus. After 45 days of treatment, patient’s condition did not improve and sever thrombocytopenia (10000-15000/µL) developed. Patient was also suffered from severe hypersensitivity reaction (fever, chills, and itching) following each plasma exchange. Kidney transplant nephrectomy was done. However, sever post operativebleedingoccurred.HUS and thrombocytopenia did not improve and patient died two days after operation.. According to this experience, Kidney transplant nephrectomy may not be an effective treatment and is not recommended in the treatment of severe and refractory post transplant HUS..