Kartagener's syndrome and polycystic kidney disease

Kartagener's syndrome and polycystic kidney disease

Nephrotic syndrome and autosomal dominant polycystic kidney disease

BACKGROUND Autosomal dominant polycystic kidney disease (ADPKD) is an inherited disorder characterized by the development and growth of cysts in the kidneys and other organs. In ADPKD patients, nephrotic range proteinuria is unusual and needs to be investigated further to exclude coexisting glomerular disease. Among the anecdotal case reports of ADPKD associated with nephrotic syndrome, focal s...

Polycystic kidney disease.

A number of inherited disorders result in renal cyst development. The most common form, autosomal dominant polycystic kidney disease (ADPKD), is a disorder most often diagnosed in adults and caused by mutation in PKD1 or PKD2. The PKD1 protein, polycystin-1, is a large receptor-like protein, whereas polycystin-2 is a transient receptor potential channel. The polycystin complex localizes to prim...

Spinal anesthesia in Kartageners syndrome.

Kartageners syndrome, an autosomal recessive disorder is a combination of dextrocardia situs inversus, bronchiectasis and sinusitis. We report a 22-year-old woman with this syndrome scheduled for appendectomy. Spinal anesthesia was preferred for the patient with this rare disorder due to the relative advantages of the regional technique over general anesthesia.

MicroRNAs and Polycystic Kidney Disease.

Polycystic kidney disease (PKD), the most common genetic cause of chronic renal failure, is characterized by the presence of numerous fluid-filled cysts in renal parenchyma. Despite recent progress, no FDA-approved therapy is available to retard cyst growth. Here, we review current evidence implicating two groups of miRNAs - the miR-17~92 cluster and miR-200s - in the pathogenesis of PKD. We pr...