Successful liver transplantation in a patient with severe haemophilia A and a high-titre factor VIII inhibitor.

We present the case of a 61-year-old man with severe haemophilia A and a high-titre factor VIII inhibitor who underwent successful orthotopic liver transplantation (OLT) for hepatocellular carcinoma. Postoperatively, a modest early anamnestic response to FVIII was followed by immunological tolerance to FVIII. This case illustrates the technical feasibility of OLT in some patients with high-titre inhibitors to FVIII, and suggests that immune tolerance may be induced by endogenously produced FVIII from the transplanted organ.

The recent expansion of Pluto's atmosphere.

Stellar occultations--the passing of a relatively nearby body in front of a background star--can be used to probe the atmosphere of the closer body with a spatial resolution of a few kilometres (ref. 1). Such observations can yield the scale height, temperature profile, and other information about the structure of the occulting atmosphere. Occultation data acquired for Pluto's atmosphere in 1988 revealed a nearly isothermal atmosphere above a radius of approximately 1,215 km. Below this level, the data could be interpreted as indicating either an extinction layer or the onset of a large thermal gradient, calling into question the fundamental structure of this atmosphere. Another question is to what extent Pluto's atmosphere might be collapsing as it recedes from the Sun (passing perihelion in 1989 in its 248-year orbital period), owing to the extreme sensitivity of the equilibrium surface pressure to the surface temperature. Here we report observations at a variety of visible and infrared wavelengths of an occultation of a star by Pluto in August 2002. These data reveal evidence for extinction in Pluto's atmosphere and show that it has indeed changed, having expanded rather than collapsed, since 1988.

Iliopsoas haemorrhage in patients with bleeding disorders--experience from one centre.

Iliopsoas haemorrhage in patients with bleeding disorders is a potentially life-threatening condition, with significantly associated morbidity. Despite its clinical importance, little has been published on the frequency, complications or outcomes of this entity since the advent of modern therapies for haemophilia. In a retrospective review of 297 patients with bleeding disorders followed at our centre, we identified 46 episodes of iliopsoas haemorrhage in 31 patients. Patients presented primarily with thigh, hip and/or groin pain, and frequently had flexion hip contracture, femoral nerve paresthesia, and >2 g dL(-1) haemoglobin drop. The duration of symptoms prior to seeking medical attention was 3.8 +/- 4 days. Nineteen of 155 patients (12.3%) with haemophilia A had 28 episodes of iliopsoas bleed; 52.6% of these patients had severe haemophilia. Of these 19 patients with haemophilia A who had iliopsoas haemorrhage, seven (36.8%) had an inhibitor to factor VIII (FVIII), and accounted for one-half of the bleeding episodes. Nine of 66 patients (13.6%) with haemophilia B had 15 episodes of iliopsoas haemorrhage; 22.2% of these patients had severe haemophilia, including one patient with an inhibitor to FIX who had two iliopsoas bleeds. The mean duration of therapy was 18.7 +/- 11.9 days, and the duration of hospitalization was 12.3 +/- 9.1 days. The length of hospital stay was significantly longer in patients with inhibitors, when compared with patients without inhibitors (19.1 +/- 5.8 days vs. 7.6 +/- 7.8 days; P<0.0001) with higher factor consumption, although the total duration of therapy was not significantly different. Patients with inhibitors were over-represented in the cohort of haemophiliacs with iliopsoas bleed. Patients with inhibitors who had iliopsoas bleeds remained hospitalized longer, although the duration of therapy was the same as patients with no inhibitors. There was a low frequency of recurrent bleed (2.8%).

The use of implantable venous access devices (IVADs) in children with hemophilia.

PURPOSE: Implantable venous access devices (IVADs), either centrally or peripherally implanted, have become increasingly popular in children with hemophilia to assist in the early treatment of bleeding episodes and in the prevention of arthropathy. Their use has been associated with complications including thrombosis, thrombophlebitis, and infection. We attempted to better define whether the benefits associated with IVADs in this population outweight the associated risks. PATIENTS AND METHODS: We studied the medical records of 35 children from the University of Minnesota's Comprehensive Hemophilia Center who received IVADs between 1992 and 1996. RESULTS: There was no bleeding or thrombophlebitis associated with IVADs in our population. One patient required removal of a central IVAD due to thrombosis. The central IVADs were associated with local infection and bacteremia rates of 3% and 33%, respectively. The rates of local infection and bacteremia associated with peripheral IVADs were both 25%. The majority of infections were cleared with antibiotics, and ports remained intact. Both types of IVADs were associated with a high patient/parent satisfaction. CONCLUSION: Despite being associated with a significant incidence of infection, we believe the benefits of IVADs for children with hemophilia and their families outweigh the risks. Possible explanations for the observed infection rates are discussed.