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Background: Cardiorespiratory fitness is significantly reduced in children with end-stage renal disease. The role of renal transplantation in improving cardiorespiratory fitness has not been thoroughly investigated., Methods: In this work, we wanted to assess whether, in children after a successful renal transplant, the amount of weekly physical exercise affects cardiorespiratory fitness and left ventricular mass (LVM). The study was conducted on 16 children after renal transplantation and 36 matching healthy controls. Four groups were formed according to the weekly amount of physical exercise; all children received an echocardiogram and underwent a treadmill exercise test according to the Bruce protocol., Results: Cardiorespiratory fitness is worst in renal transplant children with a weekly physical exercise<3 h; renal transplant children with a physical exercise of 3-5 h per week attain a cardiorespiratory fitness comparable to controls with a sedentary lifestyle (<3-h exercise per week); healthy controls with a weekly physical exercise of 3-5 h per week show the highest levels of cardiorespiratory fitness; the LVM assessed via echocardiography follows the same pattern., Conclusions: In children with a successful renal transplant, a weekly physical exercise of 3-5 h significantly improves the cardiorespiratory fitness and the LVM, up to the level of matching healthy controls with a sedentary lifestyle (<3 h exercise per week).

Methylmalonic acidemia (MMA) is an inborn error of organic acid metabolism. Patients with severe disease develop many complications despite treatment; often, the disease progresses to severe damage of the central nervous system or to end-stage renal disease (ESRD). When medical treatment is ineffective, liver, kidney, or combined liver and kidney transplantation is advocated. At present, there are no definite guidelines as for the organ to be transplanted, and results are inconsistent. We report on a 27-year-old woman with MMA MUT0. The clinical symptoms developed at age 4 months. She progressed to ESRD and received a kidney transplant in November 1996 at age 17 years. One hundred and twenty months after transplant, renal function is normal; although urinary levels of methylmalonic acid are above normal limits, no episodes of metabolic decompensation have been observed after transplantation. Although liver is the major site of methylmalonyl-CoA mutase activity, this case and similar ones in the literature suggest that the smaller mutase activity present in the transplanted kidney may be sufficient to ensure partial correction of the metabolism of organic acids sufficient to prevent the onset of episodes of metabolic decompensation. It is worth investigating whether kidney transplant can be a safer and more satisfactory alternative to liver transplantation in cases of MMA unresponsive to medical treatment although urine MMA excretion remains significantly elevated.

Background: actual literature suggests that children of methylmalonic acidemia patients are mostly healthy, but data are only partial, especially regarding long-term outcome. Therefore, our aim was to evaluate the possible long-term neurological effects of fetal exposure to high levels of methylmalonic acid in a child of a renal transplant recipient. Methods: we retrospectively evaluated the clinical and neurological records of a girl whose mother is a kidney transplant recipient affected by methylmalonic acidemia. Subsequently, we compared our results with the ones already published. Results: the girl’s weight and stature were within the normal range in the first years of life but, starting from 4 years of age, she became progressively overweight. Regarding the neurodevelopment aspects, for the first time we performed a complete and seriated neuropsychological evaluation, highlighting a mild but significant weakness in the verbal domain, with a worsening trend at three-year revaluation. Conclusions: since children of MMA patients are exposed to methylmalonic acid, the efforts of the physicians caring for these children should be directed on careful evaluation of growth, prevention of obesity and regular neurological examination together with structured neuropsychological tests to achieve a better insight in possible complications of pregnancy in patients suffering from this condition. [ABSTRACT FROM AUTHOR]