Renal cystic diseases encompass a broad group of disorders with variable phenotypic expression. Cystic disorders can present during infancy, childhood, or adulthood. Often, but not always, they can be distinguished by the clinical features including age at presentation, renal imaging characteristics, including cyst distribution, and the presence/distribution of extrarenal manifestations. It is ...

OBJECTIVE The current study aimed to investigate the spectrum of etiologies and associated disorders of renal biopsy-proven thrombotic microangiopathy (TMA) patients. METHODS The clinical, laboratory, and renal histopathological data of patients with renal TMA from 2000 to 2012 in our institute were collected and reviewed. RESULTS One hundred and nine TMA patients were enrolled in this stud...

It is well known that statins exert their main effect by inhibiting cholesterol synthesis through the inhibition of the 3-hydroxy-3-methyl-glutaryl-CoA reductase enzyme. The pleiotropic effects of statins, which are independent of their inhibition of cholesterol synthesis, have explained many of the beneficial effects of these drugs in a variety of disorders such as malignancies, infection, and...

Renal acid-base balance may become deranged in a number of ways, some of which are the consequence of inherited disorders. Two main groups of distal renal acidopathies result either from a direct inability to secrete acid in the distal nephron (giving rise to type 1 renal tubular acidosis [RTA]) or as an inherited or functional consequence of hypoaldosteronism, leading to type 4 (hyperkalemic) ...

  Cartilage hair hypoplasia (CHH), is a rare cause of metaphyseal chondrodysplasia and short stature. Other features included hair abnormality, immunodeficiency, anemia, gastrointestinal disorders (Hirschsprung disease, celiac, …) and increased risk of cancer. The disease is an autosomal recessive disorder and previously has not been reported in Iran. We report a 9-year-old boy diagnosed as car...

introduction: the prevalence of rare bleeding disorders, including combined factor v+viii deficiency are higher in iran than in developed countries. there are only a few reports which have been written concerning kidney transplantation in the patients suffering from these disorders. case report: a 22-year old girl, with a known case of combined factor v+viii deficiency, a history of bladder sto...

At 30 minutes after intravenous administration of the glomerular renal agent TC-99m-DTPA, both right and left lateral views were obtained. We analyzed the ratio of optical densities (behind the ureter/In front of the ureter). In patients without gross renal failure or retroperitoneal disease, the ratio was always less than 1 (range 0.38 to 0.95, mean 0.68). This represents greater perfusi...

Hypophosphatemia is defined as a serum phosphate level of less than 2.5 mg/dL (0.8 mmol/L). Hypophosphatemia is caused by inadequate intake, decreased intestinal absorption, excessive urinary excretion, or a shift of phosphate from the extracellular to the intracellular compartments. Renal phosphate wasting can result from genetic or acquired renal disorders. Acquired renal phosphate wasting sy...

cartilage hair hypoplasia (chh), is a rare cause of metaphyseal chondrodysplasia and short stature. other features included hair abnormality, immunodeficiency, anemia, gastrointestinal disorders (hirschsprung disease, celiac, …) and increased risk of cancer. the disease is an autosomal recessive disorder and previously has not been reported in iran. we report a 9-year-old boy diagnosed as carti...