role of Ischemia reperfusion damage on renal transplant, what are the new treatments?

Ischemia reperfusion damage usually occurs after renal transplantation. These injuries can stimulate the innate immune system, trigger an inflammatory response and ultimately activate the adaptive immune system. These events may result in rejection, graft fibrosis and chronicallograft nephropathy. Different mechanisms contribute to innate immune system activation following ischemia reperfusion injury in renal transplants. Some of these mechanisms are known and described by investigators while the remaining are still unknown. To clarify the precise mechanisms underlying the innate immune system activation and rejection progression helps us to plan therapeutic protocols to reduce immunologic responses to ischemic events and to improve the graft function and outcome. In this review, we will discuss how innate and adaptive immune systems are activated during an ischemic insult and thereafter discuss related therapeutic interventions to block the activating pathways

NPHS2 mutations in children with steroid-resistant nephrotic syndrome

Congenital nephrotic syndrome may be caused by mutations in NPHS1 and NPHS2, which encode nephrin and podocin, respectively. Since the identification of the NPHS2 gene, various investigators have demonstrated that its mutation is an important cause of steroid-resistant nephrotic syndrome. We aimed to evaluate frequency and spectrum of podocin mutations in the Iranian children with steroid-resistant nephritic syndrome. We examined 20 children with steroid-resistant nephritic syndrome referred to Ali Asghar Children's Hospital, in Tehran, Iran. Mutations in the 5th and 7th exons of NPHS2 were assessed. The mutational analysis of NPHS2 was performed by DNA sequencing. The mean age at the onset of proteinuria was 6.4 +/- 3.6 years. None of the children had mutations in the exons 5 or 7. Our study suggests that NPHS2 mutations in exons 5 and 7 are not seen in our children. Therefore, we cannot recommend NPHS2 [exons 5 and 7] mutation for screening in Iranian children with steroid-resistant nephritic syndrome. Other exons of podocin or other podocyte proteins in Iranian children may play a role in pathogenesis of steroid-resistant nephritic syndrome

Management and outcome of steroid-resistant nephrotic syndrome in children

Steroid-resistant nephrotic syndrome [SRNS] is uncommon in children, but often leads to ESRD. We report our experience with SRNS and its treatments and outcomes. We assessed 73 children with SRNS admitted to Ali Asghar Children Hospital in Tehran, Iran. Their clinical presentations, treatment, and disease courses were reviewed. The mean follow-up duration was 6.0 +/- 4.2 years. Moreover, survival times were calculated and the Cox regression method was used to determine variables able to predict survival of the kidneys. Age at the onset of the disease, sex, and hematuria were not predictive of the response to treatment with immunosuppressive drugs in the children with SRNS. The type of resistance [early or late] was associated with the responsiveness to immunosuppressives. Response to any of the immunosuppressive drugs determined the responsiveness to other immunosuppressive drugs. Cyclosporine was more effective than cyclophosphamide as initial therapy. The mean kidney survival time was 11.62 years. Kidney survival rates were 94.6%, 70.0%, 56.0%, and 34.0% at 1, 5, 10, and 15 years, respectively, in patients with initial resistance to steroid, while these were 100%, 100%, 83.0%, and 83.0% in those with late resistance, respectively [P = .03]. We showed that patients with late steroid resistance had better response to immunosuprressive drugs than patients with early resistance. We also showed that resistance to immunosuppressive therapies increased the risk of resistance to other immunosuppressive drugs. Achievement of complete or partial remission with any therapy reduced the risk of ESRD

Extensive myocardial infarction in a child with takayasu vasculitis: report of a case and literature review

Takayasu arteritis [TA] is an inflammatory arteritis involving large vessels, predominantly the aorta and its main branches. Angina pectoris or myocardial infarction may occur in 3-5% of patients due to coronary artery ostial narrowing from aortitis or coronary arteritis. We describe the case of an 11-year boy presented with hypertension and severe abdominal pain. After treatment he developed extensive myocardial infarction and died. Takayasu's disease should figure prominently amongst the causes of coronary artery disease in children and coronary arteriography should be undertaken more often during investigation of the arterial lesions of these patients