PubMed · 3917530
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N J McCarthy. 1985-01-07. Factor VIII.. https://doi.org/10.5694/j.1326-5377.1985.tb113298.x
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The nature of the mutation in the factor IX gene is an important factor in determining whether a patient with hemophilia B will develop an inhibitor. In a series of 62 Swedish families with hemophilia B, including 30 with the severe form, approximately one third of the families exhibiting deletions or nonsense mutations contained one member who developed an inhibitor. The risk for inhibitor development in family members carrying missense mutations was virtually zero.
Prothrombin complex concentrates (PCCs) have been used for the treatment of hemorrhagic episodes in patients with hemophilia B but have been associated with a high incidence of thrombotic complications. Newer, ultrapure concentrates of factor IX contain less extraneous proteins than PCCs and are less thrombogenic in vitro. In this report, the results of clinical studies with Mononine (Armour Pharmaceutical Company), a purified factor IX concentrate prepared by monoclonal affinity chromatography, are described. Two studies were performed, a phase I/II clinical trial in 10 patients and a compassionate use study in 72 patients. The pharmacokinetics of Mononine were found to be similar to the pharmacokinetics of factor IX after administration of PCCs. In both studies, Mononine was well-tolerated and hemostatically effective when used in doses of up to 161 IU/kg in treatment courses of up to 67 infusions of Mononine. Patients with hepatitis and a prior history of thrombosis with PCCs tolerated Mononine with no evidence of thrombosis. The only thrombotic complication attributed to Mononine in the two studies was an episode of phlebitis at the site of an intravenous line in one patient. These studies indicate that Mononine is safe and effective in the treatment of hemorrhagic episodes in patients with hemophilia B.
We report pelvis calculi fragmentation through the use of extracorporeal shock wave lithotripsy in a patient with mild hemophilia B. The EDAP LT 01 lithotriptor was used without incident. We review other reported cases in the literature.