Adult Fanconi syndrome and multiple myelomatosis.
نویسندگان
چکیده
A case is described of a 59-year-old woman presenting with multiple renal tubular defects. The aminoaciduria was of a generalized type. When investigated initially the only feature of myelomatosis was urinary Bence-Jones protein. Two years later radiologically classical multiple myelomatosis developed and rapidly progressed to the patient's death nine months later.
منابع مشابه
Isoelectric points of urinary light chains in myelomatosis: analysis in relation to nephrotoxicity.
Measurements of the isoelectric point (pI), sialic acid content, and polymerisation were made of 43 light chains isolated from the urine of patients with myelomatosis excreting large amounts of light chains. The pIs ranged from 3.5-9.5, and 23 were greater than 7.0. Sialylation was detected in 62% of light chains, and 69% showed microheterogeneity of charge. There was no clear association betwe...
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A case of adult Fanconi syndrome is described in which there was urinary excretion of kappa light chains. After 13 years the patient developed overt myeloma. She also developed an adenocarcinoma of the colon and an adenocarcinoma of the parathyroid gland. These findings are discussed in relation to the known association between adult Fanconi syndrome, renal damage, and myeloma.
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Two adult cases of the Fanconi syndrome are described, in each of which there was abnormal urinary excretion of immunoglobulin kappa-chain. The significance of this finding is discussed in relation to the recognized association between multiple myeloma and the Fanconi syndrome.
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Fanconi syndrome was first described in 1927 by G.Fanconi. Fanconi syndrome with congenital aplastic anemia is a hereditary disorder in which peripheral blood Pancytopenia and bone marrow hypoplasiais often associated with multiple somatic congenital malformations such as microcephaly, skeletal anomalies (absence or hypoplasia of radial or thumb or both of them), brown hyperpigmentation of the ...
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CREETH, J. M., KEKWICK, R. A., FLYNN, F. V., HARRIS, H., and ROBSON, E. B. (1963): An Ultracentrifuge Study of Urine Proteins with Particular Reference t,o the (Proteinuria of Renal Tubular Disorders, Clin. Chim. Acta, 8, 406. DENT, C. E., and HARRIS, H. (1951): The Genetics of Cystinuria, Ann. Eugen. (Lond.), 16, 60. ENGLE, R. L. Jr., and WALLIS, L. A. (1956): Multiple Myeloma and the Adult Fa...
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ورودعنوان ژورنال:
- Journal of clinical pathology
دوره 22 4 شماره
صفحات -
تاریخ انتشار 1969