(Gut 2000;47:845-851)

No abstract

Menorrhagia is a common and major problem for patients with inherited bleeding disorders, especially vWD. Quality of life during menstruation was assessed in 99 patients with inherited bleeding disorders including vWD (n = 57), carriers of haemophilia A (n = 17), carriers of haemophilia B (n = 7) and FXI deficiency (n = 18), and comparison was performed with an age-matched control group (n = 69). A questionnaire was used that included four main sections: (i) general health, (ii) health and daily activities, (iii) dysmenorrhea and (iv) quality of life during the menstrual period. Although patients with inherited bleeding disorders felt that their health (in general) was very good, they had significantly poorer quality of life on all the scales used than controls. Thirty-nine per cent reported having cut down on the amount of time spent on work and other activities as a result of their menstruation; 47% felt that they accomplished less than they would like during this period, 38% felt that they were limited in the kind of work and other activities that they could do, and 40% found that it took extra effort to perform their work. Fifty-one per cent experienced moderate, severe or very severe dysmenorrhoea. Quality of life was statistically poorer in patients with vWD, menstrual scores > 100 according to the pictorial blood assessment chart (PBAC), those who had periods > or = 8 days and those who experienced flooding or passage of clots. In conclusion, menstruation has a negative effect on the quality of life in patients with inherited bleeding disorders especially in those with objectively confirmed menorrhagia.

Background-Many patients with haemophilia have developed cirrhosis or hepatocellular carcinoma due to transfusion acquired chronic viral hepatitis. Aims-To assess the long term outcome of all haemophilic patients reported to have undergone orthotopic liver transplantation. Methods-Transplant centres of patients identified by medical database search were contacted and survival data assessed by Kaplan-Meier analysis. Results-Twenty six haemophilic men (median age 46 years, range 5-63 years) underwent orthotopic liver transplantation in 16 centres between 1982 and 1996. Indications for transplantation were hepatitis C cirrhosis (69%), hepatitis B with or without C cirrhosis (15%), viral hepatitis related hepatocellular carcinoma (12%), and biliary atresia (4%). Six patients (23%) were infected with human immunodeficiency virus (HIV). Postoperatively, the median time to normal clotting factor levels was 24 hours (range 0-48 hours) and exogenous clotting factors were stopped at a median of 24 hours (range 0-480 hours). Four patients (15%) had bleeding complications. The one and three year survival of HIV positive recipients (67% and 23%) was significantly poorer (p=0.0003) than that of HIV negative recipients (90% and 83%). Coagulopathy was cured in all patients surviving more than 12 days post-transplant. Six of the 20 patients (30%) with hepatitis C cirrhosis pretransplant had evidence of disease recurrence at a mean of nine months post-transplant. Conclusions-Hepatitis C cirrhosis is the most common indication for orthotopic liver transplantation in patients with haemophilia. Transplantation results in long term cure of haemophilia but may be complicated by the eVects of HIV infection or recurrent viral hepatitis. (Gut 1998;42:744-749)

SUMMARYT cell subsets in the gut mucosa are disliiicl populations and their imbalance in HIV has specilic implications in infection. Alterations in T cell subsets in duodenal biopsies vvere investigated in 17 asymptomatic HIV patients, 24 AIDS patienis and 10 controls wilh non-ulcer dyspepsia. Immunohistochemislry and immunofluorescence using MoAbs to CD3, CD4, CDS, CD68, CD45RA, CD45RO and gp 120 were performed on frozen sections. In the lamina propria, there was a significant depletion of CD4'^ cells at all stages of HIV, bul the density of CDS lamina propria cells was increased. Iniraepithelial lymphocytes were decreased in AIDS patients. There was a signilieunl correlation between cellular density and mucosal CD3 ' lymphocytes, and between mucosal CD3' and CDS' lymphocytes. Allhotigh mucosal CD4,CD45RO* 'memory" cells were decreased, CD8,CD45RO^ 'memory cells were increased. MucosatCD4' lymphocyte depletion occurred early in HIV, and thus their role in mueosal proleciion against opportunistic infeetion should be revised. Mucosal CD8' lymphocytes initially increased, but decreased when CD4 blood counts were depicted, perhaps contributing to loss ofhost protection against infection. Intraepitheiial lymphocyte depletion may also eontHbute lo opportunisiic infeciion.