Journal of nephropathology
Volume:3 Issue: 1, Jan 2014

Implication for health policy/practice/research/medical education: Reports on patients with steroid-dependent nephrotic syndrome and underlying minimal change disease or focal segmental glomerulosclerosis have shown promising results. There is a strong need for more trials conducted in a prospective, controlled manner to clearly recommend rituximab therapy in this indication on a regular basis.

Context: Antiphospholipid syndrome (APS) is a systemic autoimmune disorder which commonly affects kidneys. Evidence Acquisitions: Directory of Open Access Journals (DOAJ), Google Scholar, PubMed (NLM), LISTA (EBSCO) and Web of Science have been searched. There is sufficient epidemiological, clinical and histopathological evidence to show that antiphospholipid syndrome is a distinctive lesion caused by antiphospholipid antibodies in patients with different forms of antiphospholipid syndrome. It is now time to devise a classification for an accurate diagnosis and prognostication of the disease. Now that the morphological lesions of APSN are sufficiently well characterized, it is prime time to devise a classification which is of diagnostic and prognostic utility in this disease.

Context: Catastrophic antiphospholipid syndrome (CAPS) is a rare life-threatening autoimmune disease characterized by disseminated intravascular thrombosis resulting in multiorgan failure. Evidence Acquisitions: Directory of Open Access Journals (DOAJ), Google Scholar, PubMed (NLM), LISTA (EBSCO) and Web of Science have been searched. CAPS is due to antiphospholipid antibodies directed against a heterogeneous group of proteins that are associated with phospholipids. These autoantibodies activate endothelial cells, platelets, and immune cells, thereby promoting a proinflammatory and prothrombotic phenotype. Furthermore, antiphospholipid antibodies inhibit anticoagulants, impair fibrinolysis, and activate complements. Although CAPS can affect a variety of organs and tissues, the kidneys, lungs, central nervous system, heart, skin, liver, and gastrointestinal tract are most commonly affected. The systemic inflammatory response syndrome, likely to extensive tissue damage, accompanies CAPS. The most frequent renal manifestations are hypertension, proteinuria, hematuria, and acute renal failure. In the majority of patients with CAPS, a precipitating factor such as infection, surgery, or medication can be identified. Antiphospholipid antibodies such as lupus anticoagulant and antibodies against cardiolipin, β2-glycoprotein I, and prothrombin are serological hallmark of CAPS. Laboratory tests often reveal antinuclear antibodies, thrombocytopenia, and anemia. Despite widespread intravascular coagulation, blood films reveal only a small number of schistocytes. In addition, severe thrombocytopenia is uncommon. Histologically, CAPS is characterized by acute thrombotic microangiopathy. CAPS must be distinguished from other forms of thrombotic microangiopathies such as hemolytic-uremic syndrome, thrombotic thrombocytopenic purpura, disseminated intravascular coagulation, and heparin-induced thrombocytopenia. CAPS is associated with high morbidity and mortality. Therefore, an aggressive multidisciplinary treatment strategy is indicated. Anticoagulation, immunosuppression, plasma exchange, intravenous immunoglobulins, and anti-platelet agents, used in various combinations, have resulted in improved patient outcome.

Implication for health policy/practice/research/medical education: Transplantation is now a well-accepted therapy for end organ failure. However the recipients are required to take life–long immunosuppression to prevent rejection. This leads to immunosuppression associated morbidity in the form of viral/ fungal/ bacterial infections in addition to causing financial burden on the system. Over a long run these patients are at high risk to develop malignancies. In spite of all these efforts, the graft is lost over 7-10 years to chronic graft attrition/ rejection. The only answer to this problem is “Transplant tolerance” which means stable allograft function while maintaining third party immune response intact in absence of rejections on no immunosuppression. Since last 60 years transplanters across the globe are in search of this “Mackenna’s gold”. The following editorial discusses how far have we progressed in our search for the promised land of “Transplant Tolerance.”

Implication for health policy/practice/research/medical education: Much has been published on the epidemiology and clinicopathological characteristics of IgM nephropathy, but there is little information on the etiology, pathogenesis and specific therapy of the disease. Controversy still shrouds the definition and nosologic status of the disease. Well-coordinated and concerted international efforts and collaboration between researchers in the developing and developed countries are needed to make further progress on the above aspects of the disease.

Implication for health policy/practice/research/medical education: Amyloid A (AA) amyloidosis is a systemic form of amyloidosis secondary to chronic infections and inflammatory disorders such as recurrent suppurative skin infections secondary to subcutaneous administration of drugs (skin-popping). The diagnosis of AA amyloidosis is frequently overlooked due to the insidious nature of the disease. The renal manifestations of AA amyloidosis include proteinuria, tubular dysfunction, and progressive loss of renal function. Urinalysis and quantification of urinary protein excretion are important screening tests. Early diagnosis and treatment of AA amyloidosis can reverse end-organ damage.

Vitamin D is an important mediator of calcium metabolism. It has also been implicated as a potential contributor to the pathophysiology of various extra-skeletal conditions, consisting hypertension, renal disease, and insulin resistance. The primary objective of this study was to determine whether oral vitamin D (cholecalciferol) supplementation can lead to improvement of blood pressure in type 2 diabetes patients. Patients and This study was a double blind clinical trial conducted on 60 type 2 diabetes mellitus patients. Exclusion criteria were taking calcium, vitamin D supplements or any drugs effecting calcium and vitamin D metabolism in the past 6 months. Patients were administered weekly vitamin D supplementation (50000 units) for 12 weeks. Serum 25-Hydroxy vitamin D [25(OH)D] level was measured with ELISA method. Five patients (8.3%) had vitamin D deficiency, 27 (45%) had insufficient levels of vitamin D and in 28 (45%) patients vitamin D level was within normal limits. The means of systolic blood pressure (BP) and diastolic BP in patients before intervention were 121 and 80.5 mmHg; after intervention they were 110 and 76.3 mmHg, respectively. After intervention, systolic and diastolic blood pressure levels were significantly less than control group (p< 0.01). In this study we found that weekly vitamin D supplementation (cholecalciferol; 50,000 units for 12 weeks) had beneficial effect on the level of blood pressure in type 2 diabetic patients. Thus, oral vitamin D may help in improvement of hypertension in these patients.

Bitter Melon (BM) is known for its hypoglycemic effect and is commonly used in populations. This study examined the effects and safety of bitter melon fruit in laboratory mice. In this experimental study 70 male mice (25-30 gr) were randomly divided into 7 groups. The mice were injected intraperitoneally with single doses of 0, 100, 500, 1000, 2000 and 4000 mg/kg and multiple doses 500 mg/kg daily for 7 days. The mice were then observed for 72 hours before sacrificing. Immediately kidneys were taken out for histological examinations. Tubular cell vacuolization and flattening as well as hyaline casts, debris and dilatation of tubular lumen were the morphologic lesions which were assessed with scores from 0 to 4, while zero score addressed normal renal tissue. Serum samples were assayed for kidney function (creatinine; Cr and Blood Urea Nitrogen; BUN). Blood and bitter melon antioxidant activities were measured, too. Data were analyzed with Stata software (Stata Corp. 2011. Stata Statistical Software: Release 12. College Station, TX: Stata Corp LP) using ANOVA and Bonferroni tests. All single dose groups showed normal behavior after the dosing and no statistical changes were observed in blood parameters (p>0.05). Histological examinations revealed normal organ structures, however, the group treated for 7 days showed statistically a significant change in BUN (p=0.002) and a borderline significance in Cr (p=0.051). Administration of up to 4000 mg/kg did not have any effect on the mice kidney function and histology, however chronic administration were nephrotoxic. More studies with different dosage regimens are suggested.