Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0033687 (proteinuria)
 24,015 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report a case of Klinefelter's syndrome who developed a decrease of serum gonadotropin levels, particularly LH, after CyA treatment for complicated focal glomerulosclerosis (FGS). A 38-year-old man suffering from general malaise and pretibial edema was diagnosed FGS by renal biopsy in October 1988, and was referred to our hospital for further evaluation and treatment for FGS in December 1988. He was not married, and closer anamnesis revealed that he had had impaired seminal ejaculation from the age of 30. The physical examination showed 37% obesity, scanty body hair, pretibial edema and small bilateral testes (3.0 x 1.5cm). Laboratory findings included marked proteinuria (5.3g/day) and mild renal dysfunction (serum creatinine 1.3mg/dl, glomerular filtration rate 57.2ml/min). Endocrinologically, high basal levels of LH and FSH (133.6mIU/ml and 93.7mIU/ml, respectively) and the hyperresponses of LH and FSH to LH-RH stimulation were found, but the other pituitary hormone levels, thyroid and adrenal status, were in the normal range. In testicular biopsy, nodularly proliferated Leydig cells and no seminal tubules could be seen. The chromosome analysis showed 47,XXY karyotype, which confirmed the diagnosis of Klinefelter's syndrome in this patient. From 9 January 1989, CyA (6mg/Kg.day) was orally administered for 4 weeks in order to treat for FGS. After CyA administration, basal levels of LH and FSH remarkably decreased, particularly LH, and their decrease lasted for at least 6 weeks after cessation of CyA (final levels; LH 28.2mIU/ml, FSH 69.8mIU/ml). On the other hand, serum testosterone level was low normal or slightly under normal, and no apparent changes could be seen during CyA treatment.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Cyclosporine A (CyA)-induced decrease of serum gonadotropin levels in a case of Klinefelter's syndrome. 190 51

The lumbar spine bone density (BMD) was assessed by single energy quantitative tomography in 16 young patients with non traited Klinefelter's syndrome (19 +/- 2.2 yr) and in 16 age weight- and height-matched control males. The BMD were significantly lower in the patients than in the control group (175 +/- 26 mg/cm3 K2HPO4 vs 204 +/- 26; p < 0.02). The authors found a significant correlation between BMD and plasmatic levels of testosterone and estradiol suggesting the hormonal origin of the osteopenia whereas no correlation was found with serum calcium, phosphorus or prolactin levels or hydroxy-proteinuria. Such osteopenia in young patients with Klinefelter's syndrome supports early androgenic treatment of these patients.
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PMID:[Early spinal bone loss in Klinefelter syndrome. X-ray computed tomographic evaluation in 16 cases]. 816 26

We report a case of a middle-aged male who presented with pyrexia of unknown origin, oral ulcers, rash and bicytopenia. His past medical history included unexplained proteinuria, infertility and joint pain. Initial workup showed a large retroperitoneal soft tissue mass, which has appeared stable for 10 years. He subsequently developed neuropsychiatric symptoms with imaging findings of meningoencephalitis. Concurrent investigations showed elevated ANA, anti-double-stranded DNA (dsDNA) and ESR levels, as well as low complement levels. Bone marrow aspirate samples revealed karyotype of chromosome 47, XXY. The unifying diagnosis was therefore Klinefelter's syndrome (KS) with retroperitoneal teratoma and systemic lupus erythematosus (SLE), with manifestations of cytopenias, myositis, serositis, cutaneous vasculitis, encephalitis and nephritis. Upon commencing immunosuppressive therapy, there was dramatic improvement in his mental state and resolution of imaging findings. This case illustrates that males with KS may develop severe SLE manifestations; hence, increased diagnostic vigilance of KS in males with SLE is important in order to recognize effects of hypogonadism and other associations with KS such as retroperitoneal teratoma.
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PMID:Klinefelter's syndrome with lupus encephalitis and retroperitoneal teratoma. 2975 49