[49,XXXXY syndrome with signs of precocious puberty].
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Biomedical subjects
Publications and source records attributed to T Bodrogi.
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The authors report on their experiences gained during follow-up studies in 3 children, who as neonates had had renal venous thrombosis, and had been treated with heparin. On the basis of the relevant literature and their own observations, the authors suggest heparinisation during the acute stage.
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A case is reported of systemic lupus erythematosus (SLE) associated with steroid-resistant nephrotic syndrome. Serial plasma exchanges (PE) combined with prednisolone treatment induced complete normalization of the immunological findings: the anti-DNA antibody, antinuclear antibody, LE cell phenomenon and circulatory immune complexes became negative. The prostacyclin (PGI2) production-supporting activity in the plasma increased to the control range; inhibitors of PGI2 production were eliminated. The creatinine clearance normalized, the urinary protein excretion decreased significantly, and the facial erythema disappeared. Continued treatment with chlorambucil + low-dose prednisolone led to a complete and stable remission of the nephrotic syndrome, and the C3 complement normalized. The low level of PGI2 production-supporting activity in the plasma may be explained by the inhibitor of PGI2 production. PE + immunosuppressive therapy might have beneficial effects on the immunological changes and PGI2 metabolism, and also on the remission of SLE-nephrotic syndrome.