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  • Phacomatosis  (1)
  • sarcoidosis  (1)
  • 1
    ISSN: 1437-7799
    Keywords: renal failure ; sarcoidosis ; interstitial nephritis ; corticosteroid therapy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Chronic renal failure due to granulomatous interstitial nephritis in sarcoidosis is a rare phenomenon, and its response to corticosteroid therapy is not well known. We report a patient with sarcoidosis who presented with chronic renal failure and hypercalcemia, but who did not exhibit nephrocalcinosis. Renal histology findings showed the presence of noncaseating granuloma and heavy interstitial nephritis. Although hypercalcemia was remarkably improved by corticosteroid therapy, chronic renal failure, due to interstitial fibrosis and scarring, remained unchanged. This case reinforces evidence supporting the effectiveness of corticosteroid therapy in granulomatous interstitial nephritis of sarcoidosis, and suggests the need for early initiation of the therapy to avoid permanent renal dysfunction.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Virchows Archiv 400 (1983), S. 155-162 
    ISSN: 1432-2307
    Keywords: Hemihypertrophy ; Hemimegalencephaly ; Klippel-Trénaunay-Weber syndrome ; Phacomatosis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary A 17-year-old Japanese girl had right hemihypertrophy, vascular nevus of the right side of the body, right hemimegalencephaly, multiple haemangiomatosis, varicosis and chronic proliferative glomerulonephritis. The hypertrophied side of the cerebrum showed no malformations. Quantitative studies showed no significant differences between the two cerebral hemispheres in size, form, density or DNA content of nerve and glial cells. The hemimegalencephaly seemed to be due to an increase in the absolute number of nerve and glial cells in the ipsilateral cerebral hemisphere. Eight cases of hemihypertrophy with hemimegal-encephaly have been reported to date, in all of them hemihypertrophy and hemimegalencephaly were on the same side. This condition seems to be due to unilateral overproduction of neuroblasts and glioblasts or hindrance of the normal loss of excess neurons, which may be induced by hemihypertrophy of the mesenchyme surrounding the CNS.
    Type of Medium: Electronic Resource
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