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“Giltelman syndrome (GS) is a recessive salt-losing tubulopathy of children or young adults caused by a mutation of genes encoding the human sodium chloride cotransporters and magnesium channels in the thiazide-sensitive segments of the distal convoluted tubule. The plasma biochemical picture is characterized by hypokalemia, hypomagnesemia, hypocalciuria, metabolic alkalosis and hypereninemic hyperaldosteronism. Hovever, patients with GS present some clinical and biochemical alterations see more resembling that observed in thiazide diuretics

abuse. On the pathophysiological point of view, GS represents a useful and interesting human model to better understand the clinical consequences of plasma hydro-electrolytes and acid-base derangements, associated with multiple hormonal alterations. The impact of this complex disorder involves cardiovascular, muscle-skeletal and some other physiological

functions, adversely affecting the patient’s quality of life. This review tries to summarize and better explain the linkage between the electrolytes, neurohormonal derangements and clinical picture. Moreover, the differential diagnosis between other similar electrolyte-induced clinical disorders and GS is also discussed.”
“Purpose: We elucidate the role of endopyelotomy as a primary and secondary intervention for ureteropelvic junction obstruction in children.

Materials and Methods: We retrospectively identified 79 pediatric patients who underwent endopyelotomy for ureteropelvic junction obstruction between 1986 selleck chemicals llc and 2011. Eleven patients were lost to followup and were excluded from analysis. PDE4B Patient demographics, operative information, complications and success rates were reviewed for the remaining 68 patients. Treatment success was defined as the absence of symptom recurrence and improved radiographic

features on ultrasound, computerized tomography, diuretic renogram or excretory urogram at most recent followup.

Results: Primary endopyelotomy data were analyzed in 37 patients with a median age of 11.1 years. The success rate was 65% at a median followup of 34 months (range 1.5 to 242). Treatment failure occurred in 13 patients with a median time to failure of 8 months (range 1.5 to 131). There were 8 cases of failure during 12 months of surgery. Secondary endopyelotomy data were analyzed in 31 patients with a median age of 6.5 years. The success rate was 94% at a median followup of 61 months (range 1 to 204). Treatment failure occurred in 2 patients at 1 and 6 months. Approximately two-thirds of all procedures used an antegrade approach.

Conclusions: Primary endopyelotomy is significantly less successful than pyeloplasty in the treatment of ureteropelvic junction obstruction in pediatric patients. However, secondary endopyelotomy following failed pyeloplasty represents a viable alternative to redo pyeloplasty.