I Malkani

Louisiana State University, Baton Rouge, Louisiana, United States

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Publications (2)4.72 Total impact

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    ABSTRACT: The pathogenesis of veno-occlusive disease (VOD) of the liver appears to be secondary to endothelial damage of terminal hepatic venules, which leads to activation of the coagulation cascade, fibrin deposition, and eventual fibrous obliteration of the hepatic venules. Patients with VOD usually present with jaundice, hepatomegaly, weight gain, and ascites. This complication is usually associated with a high mortality rate. We report here the frequency and treatment of VOD in our autologous bone marrow transplant (BMT) patient population. Three of 15 (20%) children (median age 9 years) developed VOD and were treated with recombinant tissue plasminogen activator (rt-PA). Two of these three patients were prepared for BMT with busulfan (16 mg/kg) and cyclophosphamide (Cytoxan, 200 mg/kg), while the other child received cytosine arabinoside (ARA-C 18 g/m2), Cytoxan (3,600 mg/m2) and total body irradiation (TBI, 1,400 y). VOD developed between days 7-24 posttransplant. Clotting studies obtained pretransplant and during VOD included prothrombin time (PT), partial thromboplastin time (PTT), fibrinogen, fibrin-degradation product (FDP), proteins C and S, and platelet count. There was no correlation between the incidence of VOD and coagulation status. All patients had normal pretransplant clotting studies. However, protein C levels were noted to be consistently low for those patients at the time of VOD. All three patients received rt-PA at a dose of 0.25-0.5 mg/kg for 4 days. This dose produced increased levels of FDP but did not significantly prolong PT nor PTT. Two of the patients had dramatic responses and had complete resolution of VOD within 6-12 days from the start of therapy. The other patient died of fulminant hepatic failure. It seems that rt-PA is effective in VOD of the liver, which may be associated with low protein C level.
    American Journal of Hematology 08/1994; 46(3):194-8. · 4.00 Impact Factor
  • I Malkani, R P Warrier, L C Yu, D L Ode
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    ABSTRACT: Retinoblastoma is the most common primary ocular malignancy in childhood, usually occurring below the age of five. Recent advances in molecular biology have enabled understanding of retinoblastoma tumorigenesis as well as detecting carriers of the mutant retinoblastoma allele. It is possible now to predict prenatally whether a child carries the retinoblastoma genome. This has enabled early detection of retinoblastoma and improved outcome. Also these children are at risk for mesenchymal tumors in early adult life necessitating life long vigilance. Great strides have been made in the management of retinoblastoma. Introduction of a new pathological staging system and addition of combination chemotherapy for extraocular disease has led to more than 80% long term disease free survivors in this group of patients, who earlier had a very poor outcome. For localized intraocular disease, local treatment seems to be all that is necessary. Newer radiation techniques have helped preserve useful vision and reduced radiation related side effects.
    The Indian Journal of Pediatrics 01/1993; 60(2):227-36. · 0.72 Impact Factor

Publication Stats

21 Citations
4.72 Total Impact Points

Institutions

  • 1994
    • Louisiana State University
      Baton Rouge, Louisiana, United States
  • 1993
    • Louisiana State University Health Sciences Center New Orleans
      • Department of Pediatrics
      New Orleans, LA, United States
    • The Children’s Medical Group
      Poughkeepsie, New York, United States