Effect of intracranial bleeds on the neurocognitive, academic, behavioural and adaptive functioning of boys with haemophilia.

Brain insults are a risk factor for neuropsychological and academic deficits across several paediatric conditions. However, little is known about the specific effects of intracranial haemorrhage (ICH) in boys with haemophilia. The study compared neurocognitive, academic and socio-emotional/behavioural outcomes of boys with haemophilia with and without a history of ICH. Of 172 consecutive patients seen at a Pediatric Comprehensive Care Hemophila Centre, 18 had a history of ICH. Sixteen boys between the ages of 3 and 17 years were available for study and were matched to controls with haemophilia of the same age and disease severity and on the basis of maternal education. Groups were compared on neuropsychological and academic outcomes. Attention, socio-emotional function and executive skills were compared using data from parent questionnaires. Differences were found in intellectual function, visual-spatial skill, fine motor dexterity and particularly language-related skills, including vocabulary, word reading and applied math problem solving. Despite these group differences, outcomes were within the average range for most boys with ICH. No group differences were found in behavioural and socio-emotional functioning. Although ICH in haemophilia is not benign, it was not associated with significant cognitive and academic consequences for most boys. Early neuropsychological assessment may be indicated when there is a history of ICH. Investigation of age at ICH and quantitative measures of brain in relation to neurocognitive outcomes in larger groups of boys with ICH would be useful.

Substance dependency in Iranian patients with hemophilia.

AIMS: The prevalence and predisposing factors of substance dependency in 100 Iranian hemophilic patients were investigated. MEASUREMENTS: A confidential questionnaire and DSM-IV criteria were used. Data were gathered from 100 randomly selected hemophilic patients. RESULTS: The mean age of studied patients was 23.42+/-9.67 years, ranging from 12 to 74 years. The rate of substance dependency was high in hemophilic patients (39%), particularly while comparing with normal population. Prevalence of substance dependency was significantly related to age, marital status, number of family members, and being another substance-dependent in the family. Intensity of disease and its associated physical problems were not the main factors moving the patients toward substance dependency. Release of tension and enjoyment were the leading reasons for substance dependency. The nicotine was the most prevalent form of used substance. CONCLUSIONS: The high rate of substance dependency among Iranian hemophilic patients is expected due to the characteristics of disease, loss of psychiatric supports and treatment facilities and the geographic locality of the country.

The role of the clinical health psychologist in the management of chronic paediatric illness. A report of 2 cases.

Behavioural problems in chronically ill children are common and adequate provision for holistic management is necessary. It appears preferable for psychological intervention to start not when the child is referred with behavioural problems but as soon as the medical condition is diagnosed. These children, and their families, are usually in need of psychological care from the earliest stages of the disease process. Two case reports are cited in this context. The role of the clinical health psychologist is emphasised.