HDR syndrome [hypoparathyroidism, sensorineural deafness and renal disease] accompanied by hirschsprung disease

HDR syndrome [hypoparathyroidism, sensorineural deafness and renal disease] is an autosomal dominant condition; defined by the triad hypoparathyroidism, renal dysplasia and hearing loss. Hirschsprung [HSCR] disease is a variable congenital absence of ganglion cells of the enteric nervous system resulting in degrees of functional bowel obstruction. Rarer chromosomal anomalies are reported in combination with Hirschsprung disease like DiGeorge syndrome, mosaic trisomy 8, XXY chromosomal constitution, partial duplication of chromosome 2q, tetrasomy 9p, and 20p deletion. Here, we describe an 8 year-old girl with HDR syndrome accompanied by Hirschsprung disease. Although the association of Hirschsprung disease with chromosomal anomalies has been reported, according to our knowledge, this is the first report of associated HSCR with HDR syndrome

Effect of Rowatinex on calculus clearance after extracorporeal shock wave lithotripsy

Our aim was to evaluate the effect of Rowatinex, an essential oil preparation of terpenic type, on kidney calculi clearance after extracorporeal shock wave lithotripsy [SWL]. A randomized controlled trial was performed at Hormozgan Hospital in Bandar Abbas, Iran, on 100 patients with 10-mm to 20-mm kidney calculi. They underwent SWL, and then, they were randomly assigned into 2 groups to receive Rowatinex, 100 mg, 3 times per day, or placebo after SWL. The patients were followed up with plain abdominal radiography, ultrasonography, and excretory urography [if required], 2 and 4 weeks postoperatively. Two weeks following SWL, 6 [12%] and 9 [18%] patients in the Rowatinex and control groups had fragmented calculi without clearance, 26 [52%] and 24 [48%] had less than 50% clearance, 9 [18%] and 15 [30%] had more than 50% but not total clearance, and 9 [18%] and 2 [4%] patients were stone free, respectively. Rowatinex had a significant effect on the stone-free rate [P=.02]. Four weeks post-SWL, 3 [7.3%] and 7 [14.6%] other patients in the Rowatinex and control groups became stone free, respectively. Overall, Rowatinex had no significant effect on the stone-free rate [P=.46]. No complications or differences between the two groups in symptoms and sign we reported. Rowatinex does not have a significant effect on clearance rate of kidney calculi after SWL. However, it can accelerate calculus passage after 2 weeks, and it does not have any significant adverse effects