Health care provider and self-assessment of health status and sexual behaviour in HIV-seropositive young men with haemophilia

We hypothesized that persons with HIV who perceive themselves as having waning health status may participate in fewer sexual behaviours than HIV-infected persons without HIV-related health problems. The object-ives of this study were to compare health care provider responses with participant responses for health status and for sexual activity and to examine the relationship of HIV-related health status to the sexual behaviour of HIV-seropositive adolescents and young men with haemophilia. A detailed questionnaire designed by researchers from 11 participating US haemophilia treatment sites and CDC personnel was administered to 306 HIV-seropositive young men with haemophilia aged 12–25 years. A health care provider from the haemophilia treatment centre also completed a questionnaire on each respondent. Self-assessment of HIV-related health status was similar to provider assessment of health status. Providers accurat-ely assessed participant sexual activity (overall Kappa = 0.62). Participation in vaginal intercourse and condom use was independent of health status. Following extensive educational efforts, most HIV-seropositive adolescents and young men with haemophilia are aware of the relationship between their HIV seropositivity and health status. Waning health status does not reduce participation in penetrative sexual behaviours or increase use of condoms. HIV prevention efforts should continue for this population.     

Knee and hip arthroplasty infection rates in persons with haemophilia: a 27 year single center experience during the HIV epidemic

Total joint replacement (TJR) is an option for the management of chronic haemophilic arthropathy. Because surgery is technically challenging, there is a high rate of deep prosthetic infections, particularly in human immunodeficiency virus (HIV)-infected individuals. We determined the incidence of deep infection rates following total knee and hip arthroplasties in HIV-seropositive and HIV-seronegative persons with haemophilia. Fifty-one primary joint replacements were performed on 32 patients seen at a regional comprehensive haemophilia care center from 1975 to 2002. Thirty prostheses were placed in patients who were HIV-seropositive prior to surgery (n = 14) or seroconverted later (n = 16). Median age at the time of surgery was 33 years (range: 20-61) among 19 HIV-seropositive patients and 35 years (range: 26-74) among 13 HIV-negative patients. Median duration of follow-up was 83 months (range: 2-323). Rate of primary joint infection per artificial joint-year by HIV status was compared by Poisson regression. Main outcome measures were the incidence of primary replacement joint infections by HIV status. Deep infections developed in five (9.8%) of 51 replacement joints. There were two infections during 204.15 joint-years without HIV infection and three infections during 205.28 joint-years with HIV infection. The incidence rate of joint infection (0.98 vs. 1.46 per 100 joint-years) was not increased with HIV (relative risk, RR: 1.49, 95% CI: 0.25-8.93, P = 0.66). We conclude that HIV infection is not a contraindication to knee or hip replacement arthroplasty in the appropriate clinical setting.     

Partners of men with HIV infection and haemophilia: controlled investigation of factors associated with psychological morbidity.

The study was concerned with determining the prevalence of psychosocial problems in partners of men with haemophilia and HIV infection, and with identifying factors associated with psychological morbidity. Partners of 17 HIV positive and partners of 19 HIV negative men with haemophilia were assessed in a cross-sectional controlled investigation. Outcome measures included current psychological status and psychiatric history; coping and health beliefs; and social and sexual functioning. Partners of HIV positive men with haemophilia, the majority of whom were asymptomatic, were not found to suffer more psychological difficulties than the partners of HIV negative men with haemophilia. However, partners of haemophiliacs regardless of HIV status suffer from higher levels of psychological distress than comparable women in the community, and those with past psychiatric history and current social difficulties are particularly at risk. The sexual relationships of couples with HIV positive men were more adversely affected than those of HIV negative couples.     

A longitudinal study of immunological status in Chinese haemophiliacs: importance of the heat viral inactivation of factor concentrates. I. Immunological associations with the consumption of factor concentrates

Summary . Twenty-four of 117 cases of haemophilia A (20.5%) and none of 18 cases of haemophilia B reported in this study had an antibody to the human immuno-deficiency virus (HIV). Both groups of patients showed similar immunological alterations. HIV-seropositive haemophilia A patients had an increased CD8 cell count and a similarly decreased CD4/CD8 ratio as compared to HIV-seronegative haemophilia A patients. Multiple regression analysis for the association of CD4/CD8 ratio with HIV infection status and dosage of plasma products in haemophilia A and B patients, respectively, revealed that there was a significant negative association of ln(CD4/CD8) with dosage of factor VlII concentrates (P = 0.0435) and factor IX concentrates (P = O.O028), respectively. N o association occurred between CD4/CD8 ratio and HIV infection as well as dosage of other plasma products. These data indicate that the immunological abnormalities of our haemophilia A and B patients in their early years were primarily caused by various viral infections and/or a suppressive effect of allogeneic protein through infusion of factor concentrates and not caused simply by HIV infection.     

Low birth weight infants born to HIV-seropositive mothers and HIV-seronegative mothers in Chiang Rai, Thailand

The low birth weight (LBW) infant has a much higher risk of mortality and morbidity in infancy and early childhood. This study examined the effects of maternal HIV infection and other risk factors for LBW (< 2,500 g). A retrospective study of mothers who delivered at Mae Chan Hospital from 1997 to 2002 was conducted. Logistic regression was used to adjust for confounding factors. There were 266 infants born to HIV-seropositive mothers and 5,872 infants born to HIV-negative mothers. Low birth weight was significantly associated with maternal HIV status, gestational age, antenatal care, maternal age less than 20 years, and > 35 years. Maternal HIV positive status, young maternal age and gestational age were significant factors after adjusting for potential confounders. No significant effect of hilltribe on LBW was found. The results underline the need for nutritional surveillance and dietary counseling. HIV-seropositive women must receive early and continuing antenatal care for good pregnancy outcomes.     

T-helper cell responses to HIV envelope peptides in cord blood: protection against intrapartum and breast-feeding transmission

BACKGROUND: Acquired HIV-specific cell-mediated immune responses have been observed in exposed-uninfected individuals, and it has been inferred, but not demonstrated, that these responses constitute a part of natural protective immunity to HIV. This inference was tested prospectively in the natural exposure setting of maternal-infant HIV transmission in a predominantly breast-fed population. METHODS: Cord blood from infants of HIV-seropositive women in Durban, South Africa, were tested for in vitro reactivity to a cocktail of HIV envelope peptides (Env) using a bioassay measuring interleukin-2 production in a murine cell line. Infants were followed with repeat HIV RNA tests up to 18 months of age to establish which ones acquired HIV-infection. RESULTS: T-helper cell responses to Env were detected in 33 out of 86 (38%) cord blood samples from infants of HIV-seropositive women and in none of nine samples from seronegative women (P = 0.02). Among infants of HIV-seropositive mothers, three out of 33 with T-helper responses to Env were already infected before delivery (HIV RNA positive on the day of birth), two were lost to follow-up, and none of the others (out of 28) were found to be HIV infected on subsequent tests. In comparison, six out of 53 infants unresponsive to Env were infected before delivery, and eight out of 47 (17%) of the others were found to have acquired HIV infection intrapartum or post-partum through breast-feeding (P = 0.02). CONCLUSIONS: T-helper cell responses to HIV envelope peptides were detected in more than one-third of newborns of HIV-infected women; no new infections were acquired by these infants at the time of delivery or post-natally through breast-feeding if these T-helper cell responses were detected in cord blood.     

The Hemophilia Utilization Group Study (HUGS): determinants of costs of care in persons with haemophilia A

OBJECTIVE: The main objective of this study was to examine factors associated with utilization and costs for persons with haemophilia. STUDY DESIGN: Utilization data and patient characteristics were collected through medical record review of 336 patients receiving treatment for at least 90% of their haemophilia care at one of five comprehensive haemophilia treatment centres in California. PRINCIPAL FINDINGS: The range of factor VIII deficiency in our sample was similar to the distribution among haemophilic patients in the Western United States; 215 (64%) had severe FVIII deficiency. The mean age in our sample was 21.4 (SD = 16.2) years old and 114 (34%) were HIV-positive. In the multivariate model predicting the total cost of health care during 1995 (adjusted R2 = 0.40), total annual costs were significantly (P < 0.05) associated with being HIV-seropositive, infusing FVIII concentrate through a port vs. i.v. infusion, the number of comorbidities, moderate arthropathy (compared with no arthropathy), mild arthropathy, history of inhibitor to FVIII, and current prophylactic FVIII concentrate infusion. CONCLUSION: As expected, total health-care costs were correlated with comorbid medical conditions, such as HIV and sequelae of haemophilia such as arthropathy. Health policy should consider risk adjustment for the presence of complications such as arthropathy and HIV infection in the financing of haemophilia treatment to promote more equitable delivery of these services.     

Morbidity and mortality in ageing HIV‐infected haemophilia patients

Over 25 years of follow‐up is now available for HIV‐infected haemophilia patients. The aim of this study was to retrospectively asses the morbidity and mortality of HIV infection and the effects of HAART in these patients. Data on HIV infection, its treatment and all types of comorbidity were collected from medical records of all 60 HIV‐positive haemophilia patients who were treated at the Van Creveldkliniek since 1980 and compared with data from 152 HIV‐negative patients with severe haemophilia and the general age‐matched male population. AIDS developed in 27 patients (45%), while 31 patients died (52%). Death was solely or partially AIDS‐related in 71%. Development of AIDS and AIDS‐related deaths declined strongly after the introduction of HAART. Only one major ischaemic cardiovascular event occurred in our study population. Of the 27 patients who were still treated at our clinic in 2010, 25 (93%) were on HAART. They had more often hypertension and diabetes, but less often overweight and obesity and lower cholesterol levels than the general population. The occurrence of spontaneous intracranial bleeding was higher in HIV‐positive haemophilia patients on HAART than in HIV‐negative patients with severe haemophilia (16.6 vs. 1.2 per 1000 patient years). Since the introduction of HAART, the impact of HIV infection on morbidity and survival has decreased. The increased prevalences of hypertension and diabetes, however, warrant regular screening. HIV‐positive haemophilia patients on HAART appear to have an increased risk of spontaneous intracranial bleeding.     

Comparison of mycobacterial lymphadenitis among persons infected with human immunodeficiency virus and seronegative controls.

Lymphadenitis is a common extrapulmonary manifestation of mycobacterial disease in persons with human immunodeficiency virus (HIV) infection. We compared the clinical, mycobacterial, and diagnostic characteristics of mycobacterial adenitis in 11 HIV-seropositive and 29 HIV-seronegative patients. Ninety-three percent of the HIV-seronegative patients and 54% of the HIV-seropositive patients were foreign-born. In contrast to the HIV-seronegative patients, seropositive patients were more likely to be febrile and have negative purified protein derivative skin tests and abnormal chest roentgenograms. Sputum samples were rarely diagnostic in either group. Mycobacterium tuberculosis was the most commonly isolated organism in both groups, although United States-born patients with HIV infection were more likely to be infected with nontuberculous mycobacteria. In contrast to results for seronegative patients, fine-needle aspiration was usually diagnostic in the HIV-seropositive population, especially in those at risk for M. tuberculosis infection. Similarly, the rate at which smears were positive for acid-fast bacilli was significantly higher in the HIV-seropositive group, a circumstance suggesting a higher burden of organisms in this population. Finally, although preceding opportunistic infections were uncommon in the HIV-seropositive group, both tuberculous and nontuberculous adenitis were associated with advanced immunosuppression.     

The role of disease severity in influencing body mass index in people with haemophilia: a single‐institutional cross‐sectional study

The aim of this study was to evaluate the effect of haemophilia disease severity and potential intermediaries on body mass index (BMI) in patients with haemophilia. A secondary analysis of a cross‐sectional study of 88 adults with haemophilia was undertaken. On bivariate analysis, persons with severe haemophilia had 9.8% lower BMI (95% CI ?17.1, ?3.0) than persons with non‐severe haemophilia. The effect of haemophilia severity on BMI varied significantly by human immunodeficiency virus (HIV) status. Among HIV‐positive subjects, haemophilia severity was not associated with BMI (+5.0%, 95% CI ?22.4, 41.9). Among HIV‐negative subjects, severe haemophilia was associated with 15.1% lower BMI (95% CI, ?23.6, ?5.7). Older (>41 years) HIV‐negative subjects with severe haemophilia had a BMI that was 24.8% lower (95% CI ?39.1, ?7.0) than those with non‐severe haemophilia. No statistically significant association was detected between BMI and severe vs. non‐severe haemophilia for younger HIV‐negative subjects. Although joint disease, as measured by the World Federation of Hemophilia (WFH) joint score, did not influence the association between haemophilia disease severity and BMI, adjustment for the atrophy component of the WFH score reduced the association between haemophilia severity and BMI by 39.1–69.9%. This suggested that muscle atrophy mediated at least part of the relationship between haemophilia severity and BMI. Haemophilia disease severity is associated with BMI and appears to be mediated by muscle atrophy of surrounding joints. This association appears to be modified by HIV status and possibly age.     

Impact of human immunodeficiency virus type 1 on tuberculosis in rural Haiti

We conducted a case-control study to determine the relative and attributable risk of HIV seropositivity for bacillary-positive (smear and/or culture) pulmonary tuberculosis in Haiti. There were 274 patients with tuberculosis and an equal number of control subjects. Antibodies to HIV were present in 67 (24%) patients and eight (3%) control subjects. Odds ratios suggested that the risk of pulmonary tuberculosis was 15.7 times as great (95% confidence interval, 4.8 to 5.0; p less than 0.05) in patients 20 to 39 yr of age who were HIV-seropositive than in HIV-seronegative patients. In contrast, the relative risk in those 40 to 59 yr of age was elevated (3.0 times), though not significantly (lower 95% confidence interval, 0.8). In the 20- to 39-yr age group, 31% of tuberculosis was attributable to HIV infection (95% confidence interval between 23 and 39%). HIV-seropositive and HIV-seronegative patients did not differ with respect to sputum smear positivity. HIV-seronegative patients were twice as likely to be infected with resistant organisms, though this was not significant. We conclude that HIV infection is a major risk factor for pulmonary tuberculosis in young adult residents of Haiti. This, together with the fact that similar proportions of HIV-seropositive and HIV-seronegative patients were potentially infectious, suggests that without vigorous counteraction tuberculosis will become a greater problem for Haiti.     

HIV-infected children with hemophilia: one- and two-year follow-up of neuropsychological functioning

This report describes the absence of neuropsychologic change observed over a 2-year period for 25 HIV-seropositive (HIV+) children and adolescents with hemophilia and 33 HIV-seronegative (HIV-) controls. Efforts were made to match the groups on the basis of chronological age, race, and hemophilia severity. The baseline evaluation included blinded neuropsychologic measurement of motor, attention, language, visual processing, memory, and general intelligence. HIV+ and HIV-group means did not differ at baseline on any neuropsychologic domain, and this trend continued at the 2-year follow-up. Mixed models analyses did not indicate that the HIV+ group performed more poorly than the HIV- group on any of the neuropsychological domains, nor did they show different patterns of change over time on these variables for the HIV+ group. Consistent with emergent findings, it continues to be premature to attribute subtle neuropsychologic deficits in seropositive children with hemophilia directly to the central nervous system (CNS) effects of HIV infection.     

Low rates of child testing for HIV persist in a high-risk area of East Africa

Children in low- and middle-income countries (LMIC) are the least touched by recent successes in the diagnosis and treatment of HIV/AIDS globally. Early treatment is essential for a child's longer and higher quality of life; however, by 2011, only a small proportion of HIV-seropositive children in LMIC countries were receiving treatment, in part because of persisting low rates of diagnosis. This study of the prevalence and characteristics of children tested for HIV was embedded in the Coping with HIV/AIDS in Tanzania (CHAT) study in which HIV-seropositive and HIV-seronegative adults, and adults with unknown HIV status were asked about HIV testing for their children. Data were gathered from November 2009 to August 2010 during the scale-up of Prevention of Mother To Child Transmission and Early Infant Diagnosis programs in the region. Reports on 1776 children indicate that 31.7% of all children were reported to have been tested, including only 42.9% of children with an HIV-seropositive caregiver. In general, children more likely to be HIV tested were biological children of study participants, younger, of widowed adults, living in urban areas, and of HIV-seropositive parents/caregivers. Children belonging to the two indigenous tribes, Chagga and Pare, were more likely to be tested than those from other tribes. Rates of testing among children less than two years old were low, even for the HIV-seropositive caregiver group. The persistence of low testing rates is discussed in terms of the accessibility and acceptability of child testing in resource poor settings.     

Assessing health-related quality-of-life in individuals with haemophilia in China

Haemophilia is a group of inherited, congenital diseases present all through the life. Those disorders are associated with a chronic burden of morbidity punctuated by episodes of acute deterioration in health-related quality-of-life (HR-QL). The significances of assessing HR-QL in individuals with haemophilia as an important component of the clinical database have gained increasing recognition in recent years. To the best of our knowledge, reports on the HR-QL of Chinese patients with haemophilia are not available. The purpose of this paper was to assess the HR-QL of patients with haemophilia in China. In order to do this, 179 patients with haemophilia (age 16-70) were mailed China quality-of-life questionnaire, which was designed specially for this study taking into account the culture characters of Chinese. Compared with the normal male general population, individuals with haemophilia experienced significantly lower levels of HR-QL and within the subgroups of patients, there was a gradation of HR-QL from mild < moderate < severe haemophilia patients. Age, clinical severity, degree of arthropathy, hepatitis status, salary and mean annual expenditure for replacement therapy were predictors of HR-QL. Clinical severity of the disease manifestation rather than the severity of the disease based on molecular classification was a factor that negatively influenced the HR-QL of patients with haemophilia. Arthropathies of patients with haemophilia in China were more severe than that in western countries because of the poorer medical care and were associated with their lower income and therapy cost. A large number of patients never had hepatitis or HIV serological examination. The exact prevalence of hepatitis and HIV was difficult to obtain and the HR-QL of hepatitis and HIV positive patients required further study.     

Association of human immunodeficiency virus and anal human papillomavirus infection among homosexual men.

BACKGROUND--A previous study of men with proctitis, proctocolitis, or enteritis showed an association of anal human papillomavirus (HPV) infection with human immunodeficiency virus (HIV) infection. Because anorectal abnormalities may confound an observed association between anal HPV DNA and HIV seropositivity, the present study was undertaken among consecutive homosexual men seeking HIV serologic testing who were unselected for anorectal symptoms. METHODS--Consecutive homosexual men underwent a standardized interview, physical examination, and collection of specimens for HIV serologic testing and detection of anal HPV DNA. RESULTS--Anal HPV DNA was detected in eight (31%) of 26 HIV-seropositive men and in 10 (8%) of 119 HIV-seronegative men (odds ratio, 5.8; 95% confidence interval, 1.1 to 30.1, adjusted for history of sexually transmitted disease, current anorectal symptoms, and age). When men with anorectal symptoms were excluded from the analysis, anal HPV DNA was detected in 27% of seropositive men compared with 8% of seronegative men (odds ratio, 4.4; 95% confidence interval, 1.4 to 13.4). There was no difference between HIV-seropositive and HIV-seronegative men with respect to distribution of type of HPV DNA. Men with group II or III and group IV HIV disease were 4.1 and 10.9 times, respectively, more likely than HIV-seronegative men to have anal HPV DNA detected. CONCLUSIONS--Because HIV-seropositive men appear to be at increased risk for the detection of anal HPV DNA, the natural course of anal HPV infection should be compared among HIV-seropositive and HIV-seronegative homosexual men.     

Psychological status of men with haemophilia and HIV infection: two-year follow-up

This study aimed to assess the psychological status of men with haemophilia and HIV infection and to monitor changes in psychlogical status over time, in order to evaluate the need for psychological support. The study included 24 HIV seropositive men and a control group of 21 HIV seronegative men who attended the Haemophilia Centre in Bari (Italy). Subjects underwent psychological tests (STAl-Y: State and Trait Anxiety Inventory; SDS: Self-Rating Depression Scale) and completed a questionnaire on the emotional impact of AIDS. Assessment was repeated at 6-monthly intervals over a 2-year period. Contrary to expectation, HIV seronegative men with haemophilia had worse anxiety and depression scores, reported more confusion and fear, and had more reluctance towards the use of blood products (despite their present safety) than HIV seropositives. Possible reasons for these findings are considered, and their implications for clinical practice discussed.     

A case–control study assessing bone mineral density in severe haemophilia A in the UK

It has been shown that bone mineral density (BMD) may be lower in patients with haemophilia (PWH). A comparison to control subjects is required to thoroughly assess current BMD in PWH in the UK. The objective of this study was to test the hypothesis that BMD is lower in PWH than in controls, and in patients with more severely affected joints or lower activity levels. In this case–control study, 37 patients with severe haemophilia A were recruited from two haemophilia centres in the UK. A group of 37 age, gender and ethnicity‐matched control participants were recruited. All participants had a bone density scan, a musculoskeletal assessment, a blood test for vitamin D and completed a functional activity questionnaire. Of the case group, 5% had osteoporosis and 24% had BMD lower than expected for age. No control participants had osteoporosis, 3% had osteopenia and 14% had BMD lower than expected for age. Ninety one per cent of case participants and 92% of control participants had reduced 25(OH)D levels. Case participants had significantly lower BMD than control participants, and case participants with more severely affected joints, lower activity levels, HIV, history of hepatitis C or lower BMI had significantly lower BMD. Patients with severe haemophilia have a higher risk of low BMD than men without haemophilia. Patients with more severely affected joints and lower activity levels have lower BMD. It remains unclear whether patients with low BMD reached adequate peak bone mass. Low vitamin D may be present in the majority of PWH.     

HIV increases the risk of malaria in women of all gravidities in Kisumu,Kenya

OBJECTIVE: To study the importance of HIV infection for malaria in pregnancy in Kisumu, Kenya. SUBJECTS AND METHODS: Healthy women with an uncomplicated pregnancy of 32 weeks or more attending the prenatal clinic in the Provincial Hospital between June 1996 and March 1999 were tested for HIV and malaria after consent had been obtained. For participating women who delivered in the same hospital, a blood smear of the mother and the placenta were obtained. RESULTS: In the third trimester, 5093 women consented to testing: the prevalence of malaria and HIV was 20.1 and 24.9%, respectively. Among the 2502 screened women who delivered in the hospital, the prevalence of HIV, peripheral parasitaemia and placental malaria was 24.5, 15.2, and 19.0%, respectively. Compared with HIV-seronegative women, HIV-seropositive women were more likely to be parasitaemic, to have higher parasite densities, and to be febrile when parasitaemic. Placental infections in HIV-seropositive women were more likely to be chronic, as indicated by the presence of moderate to heavy pigment depositions. When adjusted by age, the typical gravidity-specific pattern of malaria in pregnancy disappeared in HIV-seropositive women; HIV-seropositive primigravidae had a similar risk of malaria as HIV-seropositive multigravidae. The excess malaria attributable to HIV in the third trimester increased from 34.6% among HIV-seropositive primigravidae, to 41.5% among HIV-seropositive secundigravidae, and 50.7% among HIV-seropositive gravidae with three or more pregnancies. CONCLUSION: HIV infection alters patterns of malaria in pregnant women; in areas with both infections, all pregnant women should use malaria prevention.     

Persistent hepatitis C virus RNA replication in haemophiliacs: role of co-infection with human immunodeficiency virus

Summary. In order to evaluate the evolution of transfusional hepatitis C in haemophiliacs, we performed a retrospective study of ALT levels and HCV viraemia with a RNA PCR assay in 57 patients. We found that the vast majority of HCV-infected patients remained viraemic (43/57=75%) and higher ALT levels correlated with HCV viraemia. Although indicators of the transfusional viral load (age, severity of haemophilia) and HBV co-infection did not correlate with HCV RNA replication, HIV seropositivity was strongly associated with persistence of HCV viraemia (23/25 = 92% in HIV-positive versus 20/32 = 62% in HIV-negative patients), without any correlation with CD4 counts. Genotyping of HCV in the 43 viraemic patients shows more frequent genotype 1 in the HIV-seropositive group (14/23) than in the seronegative group (6/20). Our data emphasize that besides the role of the immunodeficiency status, the genotypes of HCV might be involved in the differences observed in terms of HCV RNA replication between the HIV-seropositive and seronegative haemophiliacs.     

Emotional adjustment of children and adolescents with haemophilia in relation to the HIV threat

A Dutch nation-wide study on young haemophilia patients, whose former treatment placed them at risk for HIV infection, was done to examine the effect of HIV testing, HIV status, disclosure of HIV status and the child's experiences with disease-related information on emotional adjustment. In the Netherlands HIV tests are not routinely performed for paediatric haemophilia patients, but the number of HIV-infected children is estimated to be low (8%). Seventy-two boys with haemophilia, five HIV positive, 51 HIV negative and 16 not tested, and their mothers were interviewed and administered standardized questionnaires. Multiple regression analyses showed no differences in anxiety and depression between HIV-positive, HIV-negative or untested boys, nor between children who were informed or not informed about their HIV status. Conversely, children who experienced more difficulties to obtain information about the their disease were significantly more anxious and tended to be more depressed. No significant differences in emotional distress were found between the patients and a standardization sample of healthy peers. The data provide evidence that children and adolescents with haemophilia cope effectively with their disease and the emotional impact of the HIV threat. There seems to be no reason to protect children from testing or knowing their HIV status. In the absence of compelling medical reasons, however, health care workers should not forcefully advocate testing or early disclosure. Distinct disease and treatment-related factors as well as social factors modify disclosure practices.