Renal Autotransplantation in a Patient with Bilateral Renal Artery Stenosis Secondary to Takayasu Arteritis.

Involvement of the renal artery is common in Takayasu arteritis. We, herein, present on a patient with Takayasu arteritis causing severe renal failure and a successful auto-transplantation. This case shows that early diagnosis and immediate appropriate interventions are life-saving in patients with Takayasu arteritis. Renal auto-transplantation performed in selected cases increases dialysis-free survival.

Clinical assessment of primary and secondary hypertension in children and adolescents.

The aim of this study was to compare the clinical features of patients with elevated blood pressure and to detect variables associated with the diagnosis of primary hypertension. We identified 383 (69%) hypertensive children (197 [51.5%] with primary hypertension, and 186 [48.5%] with secondary hypertension) out of 553 children referred to our clinic with a history of elevated blood pressure. The primary hypertension group was significantly older and had higher BMI, positive family history of hypertension, and lower prevalence of preterm birth compared with those with secondary hypertension. No difference was found between the two groups in terms of the frequency of target organ damage. Multiple regression analysis showed that a family history of hypertension, obesity, age over 10 years, elevated uric acid, and presence of higher systolic blood pressure values at admission were independent predictors of primary hypertension; therefore, these parameters can be considered important clues for diagnosing primary hypertension.

Primary Hyperoxaluria Type 1: A Cause for Infantile Renal Failure and Massive Nephrocalcinosis.

Primary hyperoxaluria type 1 is a rare autosomal-recessive disease caused by the deficient activity of the liver specific enzyme alanine-glyoxylate aminotransferase. Increased endogenous oxalate production induces severe hyperoxaluria, recurrent urolithiasis, progressive nephrocalcinosis and renal failure. Here we report a 6 month old boy who presented with vomiting and decreased urine volume. He was diagnosed with chronic kidney failure at 4 months of age and peritoneal dialysis was introduced at a local hospital. His parents were third degree cousins and family history revealed 2 maternal cousins who developed end stage renal disease during childhood. When he was admitted to our hospital, laboratory studies were consistent with end stage renal disease, ultrasound showed bilateral massive nephrocalcinosis. As clinical presentation was suggestive for primary hyperoxaluria type 1, plasma oxalate was determined and found extremely elevated. Genetic testing proved diagnosis by showing a disease causing homozygous mutation (AGXT-gene: c.971_972delT). The patient was put on pyridoxine treatment and aggressive dialysis programme. In conclusion; progressive renal failure in infancy with massive nephrocalcinosis, especially if accompanied by consanguinity and family history, should always raise the suspicion of PH type 1. Increased awareness of the disease would help physicians in both treating the patients and guiding the families who have diseased children and plan to have further pregnancies.