Primary hyperoxaluria (PH) is a very rare genetic disorder; it is characterized by total or partial deficiency of the enzymes related to the metabolism of glyoxylate, with an overproduction of calcium oxalate that is deposited in different organs, mainly the kidney, leading to recurrent lithiasis, nephrocalcinosis and end stage renal disease (ESRD). In patients with ESRD that receive kidney transplantation alone, the disease has a relapse of 100%, with graft loss in a high percentage of patients in the first 5 years of transplantation. Three molecular disorders have been described in PH: mutation of the gene alanin glioxalate aminotransferase (AGXT); glyoxalate reductase/hydroxy pyruvate reductase (GRHPR) and 4-OH-2-oxoglutarate aldolase (HOGA1). We present two cases of patients with a history of renal lithiasis who were diagnosed with primary hyperoxaluria in the post-transplant period, manifested by early graft failure, with evidence of calcium oxalate crystals in renal biopsy, hyperoxaluria, hyperoxalemia, and genetic test compatible; they were managed with proper diet, abundant oral liquids, pyridoxine, hydrochlorothiazide and potassium citrate; however, they had slow but progressive deterioration of their grafts function until they reached end-stage chronic renal disease.
a b s t r a c tIn this work we present the results of a study on pulse energy noise dynamics of the IBR-2M; the study employs statistical methods of time series (pulse energy) processing and hierarchical cluster analysis. It is shown that the power spectrum changes of the pulse energy fluctuations per cycle ($11 days) has a transition region duration of $3 days that takes place after the reactor has reached the nominal power of 2 MW. The power noise is subsequently divided into four stable clusters, with three of which describe the noise transition region. The fourth cluster constitutes a stable structure that does not depend on noise level (amplitude of the power spectrum) or on reactor operation time. The noise transition region is formed by the vibration of the moving reflectors once the reactor has been reached.
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