Development of a new disease-specific quality-of-life questionnaire to adults living with haemophilia.

A haemophilia-specific health-related quality-of-life questionnaire (named 'Hemofilia-QoL') was developed to assess quality-of-life in adults with haemophilia, and was psychometrically tested. Seventy-three interviews with haemophilia patients and health care professionals were used to generate the items included in the questionnaire, and expert ratings on the items formulated were used to screen them for potential omission. This was followed by psychometric testing in a sample of 35 patients. Preliminary psychometric testing of the revised questionnaire version, which contains 10 domains (physical health, physical role, joint damage, pain, treatment satisfaction, emotional role, mental health, social support), showed acceptable reliability (alpha = 0.94 for the Hemofilia-QoL total score) and validity, and this will be examined in a subsequent study with a larger patient sample.

Cunninghamella bertholletiae infection (mucormycosis) in a patient with acute T-cell lymphoblastic leukemia.

Cunninghamella spp. are unusual opportunistic pathogens that have been identified with increased frequency in immunocompromised patients. Clinical infection by this fungus is almost always devastating and usually fatal. Infections with this group of organisms have been seen most frequently in patients with hematological malignancy. Here we report the case of a patient with acute leukemia who developed multiorganic failure as a consequence of hematological dissemination by Cunninghamella bertholletiae. The case highlights the mortality associated with this fungal infection in immunocompromised patients, confirms the risk factors associated with non-candida fungal infections and shows a clinical presentation mimicking myocardial infarct and cerebrovascular stroke.

[Evaluation of the type II diabetic population attended by a primary care team]. / Evaluación de la población diabética tipo II atendida en un equipo de atención primaria.

OBJECTIVES: To find the degree of control, the prevalence of complications and cardiovascular risk factors (CVRF), and the drugs treatment used for type II diabetics. DESIGN: A descriptive crossover study. SETTING: Rekaldeberri Health Centre, Bilbao. PATIENTS: 202 diabetes II patients selected at random. INTERVENTIONS: Data were gathered by interviews, physical examinations and further tests. MAIN RESULTS: 52.2% of the sample were women. Average age was 66.6 50% of the diabetics had been diagnosed for 8 years or more. Microalbuminuria was detected in 21.9% of the patients, Microangiopathy in 24.8%, Neuropathy in 11.4% and diabetic foot in 10.4%. The years of evolution and appearance of complications had a significant connection. 64.4% of patients had a good or acceptable metabolic control. Worse metabolic control of diabetes was connected with the appearance of retinopathy and neuropathy. 47.5% were hypertensive. 21.3% smoked, 35.6% had cholesterol figures over 250 mgr/dl. Their mean Body mass index was 28.1. 40.6% were treated exclusively by diet, 9.9% with insulin and 35.6% with medicines taken orally. CONCLUSIONS: A high percentage of diabetics had a good or acceptable control of their disease. Prevalence of complications was less than in other studies, whereas CVRFs were similar. We do not discount the presence of bias in the comparison because of different diagnostic methods.

[Granulocytic sarcoma of the base of the skull in chronic myeloid leukemia]. / Sarcoma granulocítico de base de cráneo en el curso de una leucemia mieloide crónica.

A 54 years old patient with Ph' negative chronic granulocytic leukaemia is reported, who presented rapidly progressive multiple cranial nerve palsies. A cerebral CT scan showed an invasive tumoral disease on the base of skull. Histologic examination of this mass was diagnostic for granulocytic sarcoma, which was the presentation form of an extramedullary blastic crisis. The general characteristics of granulocytic sarcoma are commented, special attention being paid to its appearance in the course of chronic granulocytic leukaemia.