Peritoneal Dialysis in Maple Syrup Urine Disease

Peritoneal dialysis in maple-syrup-urine disease: Studies on branched-chain amino and keto acids

“…According to our data and also to reports of other authors [I, 8,11,[19][20][21], peritoneal dialysis is an effective treatment in life-threatening situations in MSUD with markedly increased metabolite levels. It is unfurtunate, however, that the most dangerous metabolites, i.e., leucine and KICA, have unfavourable kinetics not only in renal excretion but also in peritoneal dialysis.…”

Comparison of temporary and permanent catheters for acute peritoneal dialysis.

“…Since then it has been used successfully for the short term treatment of 2 3 4 acute renal failure, intoxication states, and metabolic abnormalities.5 6 9 4…”

“…Since then it has been used successfully for the short term treatment of 2 3 4 acute renal failure, intoxication states, and metabolic abnormalities.5 6 Until recently nephrologists relied on rigid polyethylene catheters placed percutaneously such as the Trocath (McGaw Laboratories) for acute dialysis. In many centres these have now been replaced by the flexible silicone rubber catheter introduced by Tenckhoff and Schechter in 1968,7-9 which was originally designed for chronic dialysis.…”

Maple syrup urine disease: Treatment of the acutely ill newborn

“…It took almost one week of therapy before patient D. K. decisively improved and the possibility can not be excluded that this slow recovery has had a lasting effect on the future mental development of the child. Therefore more effective procedures have been introduced at the beginning of treatment to eliminate the toxic agents as quickly as possible [1,6,9,18,20,22,23,25,27].…”

“…3), it can be concluded that multiple exchange transfusions rapidly improve the clinical and biochemical status in the acute phase of MSUD [23,27]. Instead of blood exchange transfusions, peritoneal dialysis has also been used sucessfully to rapidly eliminate the toxic agents [1,6,9,18,20,22,27]. At the moment it cannot be definitely decided which of these two procedures is the more effective form of-therapy.…”