Hemolytic anemia due to G6PD deficiency and urate oxidase in a kidney-transplant patient.
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Biomedical subjects
Publications and source records attributed to G Verzetti.
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A patient with monoclonal gammopathy and renal failure due to kappa light chain deposition in the glomerular basement membrane is reported. After 8 months, he developed a progressive and severe renal failure requiring haemodialysis. 30 months later, the monoclonal gammopathy was unchanged. Clinical features, as well as kinetic and immunological studies, were consistent with a diagnosis of benign monoclonal gammopathy (BMG). This case suggests that the renal failure complicating a monoclonal gammopathy is not related to its malignancy.
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The present study points out that the CHRONIC RENAL FAILURE (CRF) represents a situation of decreased adrenal function: at least for delta 5 Steroids which are markedly reduced when compared with normal subjects. Peripheral plasma levels of Pregnenolone (delta 5 P) ranged in CRF between 190 and 860 pg/ml; Dehydroepiandrosterone-sulphate (DHA-S) 0.1-2.2 ng/ml and Dehydroepiandrosterone (DHA) 200-3100 pg/ml. Cortisol was in the normal range or slightly elevated (70-175 ng/ml). A significant correlation between basal concentration and the time of dialysis was observed. It is interesting to notice how the phase levels of delta 5 P, DHA-S and DHA are reduced ad from the beginning of the haemodialysis treatment and that during such a treatment a further progressive decrease occurs reaching concentrations with are similar to those found in pre-puberty. This phenomenon appears to be the evidence of a progressive metabolic involution of the adrenal gland due to the exhaustion of enzymatic activities and of receptorial structures. Furthermore, these results suggest speculation on interrelationship between adrenal and gonadal activity in these patients.
In 14 patients affected by chronic renal failure (7 males and 7 females) it has been evaluated the secretion of beta LPH, beta EP, ACTH and Cortisol in basal conditions and immediately after a dialytic treatment. For beta LPH and beta EP measurements on each thawed plasma a silic acid extraction and a successive peptides separation by a Sephadex G-75 column chromatography preceeded the two specific RIAs. Basal beta LPH plasma levels resulted significantly higher than in normal controls, while that of beta EP, ACTH and Cortisol were in the normal range. The dialytic treatment was able to increase ACTH and Cortisol plasma levels, without to modified beta LPH and beta EP plasma levels.
The serum levels of certain proteins were determined in 130 nephropathics collected into 8 different diagnostic groups, and in 20 normal subjects considered as a control group. The purpose was mainly to assess the clinico-diagnostic interest of such a measurement in nephrological practice. The results point to its clinical usefulness, particularly in differential diagnosis. It is possible to formulate interesting physiopathological interpretations notwithstanding the limitations of the eminently clinical features of the research.
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