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  • 1995-1999  (2)
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  • 1
    ISSN: 1432-0584
    Keywords: Key words Hepatic veno-occlusive disease ; VOD ; Treatment ; Pediatric ; Duplex ultrasound ; Prostaglandin E1
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract  Limited data exist on therapeutic options for established hepatic veno-occlusive disease (VOD) in pediatric patients after stem cell transplantation (SCT). In this report, we present data on the successful treatment of VOD in three children following allogeneic SCT and report the duplex ultrasound criteria for the confirmation of the diagnosis and for the evaluation of the treatment progress. All patients were 〈2 years at the time of transplantation and had received preparative regimens containing busulfan and cyclophosphamide. There were no known pretransplant risk factors for VOD. Allogeneic stem cell transplantation was performed from a sibling donor for CMML and from unrelated donors for Wiskott-Aldrich syndrome and familial hemophagocytic lymphohistiocytosis (FHL). The onset of first clinical symptoms of VOD (as defined by the Seattle and Baltimore criteria) was relatively late in all three patients (days +19, + 20, and +25, respectively). Time from onset of first symptoms until confirmation of diagnosis by serial duplex ultrasound examination was 4–11 days. Duplex ultrasound criteria are as follows: complete change of direction of blood flow in the portal vein, decrease of flow in the hepatic veins, and development of collateral circulation. Treatment was initiated upon confirmation of VOD by continuous infusion of prostaglandin E1 (initial dose 0.075 μg/kg/h) in addition to low-dose heparin (100 units/kg/d). Treatment was continued at the maximum tolerated dose of 0.3–0.5 μg/kg/h of PGE1. After 9, 14, and 25 days of treatment respectively, normal portal vein flow was restored and treatment could be discontinued. All three patients are alive and well without apparent sequelae.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    World journal of urology 14 (1996), S. 274-277 
    ISSN: 1433-8726
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Spermatogenesis is an orderly process whereby male germ cells pass through sequential phases of differentiation to develop into mature spermatozoa. At present, little is known about the factors that regulate this process. The POU domain represents a group of transcription-activator proteins with well-described roles in development. These proteins have been shown to regulate the tissue-specific expression of genes involved in cellular differentiation. The recent identification of Sperm-1, a testis-specific POU-domain protein expressed in primary spermatocytes just prior to the onset of meiosis, suggests that this protein may be involved in the regulation of male germ-cell differentiation. Continued evaluation of Sperm-1 and other related transcriptional activators may shed some light on the regulation of spermatogenesis.
    Type of Medium: Electronic Resource
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