Malignant hypertension in a patient with end of stage renal disease (ESRD) treated by renal transplant

Control of hypertension is often a problem in the management of end stage renal disease (ESRD). Multiple modalities of treatment are required to prevent cardiovascular and cerebrovascular mortality and morbidity. These include fluid and salt restriction, multidrug regimes and dialysis. We report a case of young 25 years old patient, admitted with chronic renal failure, complicated by malignant and refractory hypertension, not responding to hemodialysis and antihypertensive agent. During stay in hospital, patient also had intracerebral hemorrhage, fits due to uncontrolled hypertension requiring ventilatory support followed. Renal transplant was considered to be the final therapeutic modality. After gradual recovery, a successful live-related renal transplant was performed. As soon as good graft was established, the blood pressure settled and 4 of the 5 antihypertensives were withdrawn. After 2 weeks, patient was discharged in a stable condition with a total stay of about 2 months.     

Interferon-beta reduces proteinuria in experimental glomerulonephritis

Interferon-beta (IFN-beta) is a multifunctional cytokine with immunomodulatory properties. We examined the effect of IFN-beta in three separate rat models of glomerular injury and in cultured human glomerular endothelial cells and podocytes. In nephrotoxic nephritis in WKY rats, recombinant rat IFN-beta started either at induction or after establishment of disease significantly reduced 24-h proteinuria by up to 73% and 51%, respectively, but did not affect serum creatinine. There was a slight reduction in numbers of glomerular macrophages, but no difference in glomerular or tubulointerstitial scarring. In Thy-1 nephritis in Lewis rats, IFN-beta started at induction of disease reduced proteinuria by up to 66% with no effect on numbers of glomerular macrophages, but a reduced number of proliferating cells. In puromycin nephropathy in Wistar rats, IFN-beta started at induction of disease reduced proteinuria by up to 93%, but had no effect on glomerular histology. In cultured cells, human IFN-beta-1a had a dramatic effect on barrier properties, increasing electrical resistance across monolayers of either glomerular endothelial cells or podocytes and decreasing trans-monolayer passage of albumin. In conclusion, these results show that IFN-beta reduces proteinuria in three different rat models of glomerular injury and that its anti-proteinuric action may result from direct effects on cells that comprise the glomerular filtration barrier. These data indicate that IFN-beta may have potential as a therapeutic agent in proteinuric renal disease.     

Consanguinity in primary immunodeficiency disorders; the report from Iranian Primary Immunodeficiency Registry

PROBLEM: Primary Immunodeficiency Disorders (PiD) are a heterogeneous group of genetic disorders, with different modes of inheritance. This study was accomplished in order to determine the frequency of consanguineous marriages in the families of patients with PiD. METHOD: In this study, the records 515 Iranian PiD patients were reviewed during a 25-year period. RESULTS: The mean proportion of consanguineous marriages was 65.6% among PiD patients, while the overall rate was 38.6% in the country. The rate of consanguinity was 77.8% in cellular immunodeficiencies, 75.8% in combined immunodeficiencies, 72.5% in defects of phagocytic function, 58.6% in other immunodefiiencies, 54.1% in predominantly antibody deficiencies, and 50% in complement deficiencies. Moreover all patients with immunodeficiency associated with other diseases had consanguineous parents. Such marriages were most common in the parents of patients with Chediak-Higashi syndrome, severe combined immunodeficiencies, primary CD4 deficiency, ataxia-telangiectasia, selective IgG class deficiencies, chronic granulomatous disease, and Schwachman syndrome. CONCLUSIONS: It is important to inform the general population about the dangers of consanguinity, which is very common in some areas such as Iran. Premarital examination to avoid genetic diseases could be suggested, especially in a community where the rate of consanguineous marriage is high.     

A twin study of cilioretinal arteries,tilted discs and situs inversus

Graefe's Archive for Clinical and Experimental Ophthalmology - To establish the prevalence and heritability of cilioretinal arteries (CRAs), tilted discs (TDs) and situs inversus (SI). Fundus...