BackgroundStudies on health-related quality of life are based on the increasingly evident need for medical care not to be limited to preventing death, but to focus instead on the value of health. ObjectiveThis study aimed to measure the health-related quality of life in hemophilia, using the Hemophilia- Specific Quality of Life (Haem-A-QoL) questionnaire and describe the socioeconomic characteristics and health conditions of these patients. MethodsThe Brazilian version of the Hemophilia-Specific Quality of Life questionnaire was administered to hemophiliac adults, treated in an on-demand regime at the Juiz de Fora Regional Blood Center - HEMOMINAS Foundation. The patients were interviewed about demographic and socioeconomic data and their understanding of the questionnaire. Clinical data were collected from medical records. The Mann-Whitney U test was used for statistical analysis. The level of significance was set for p-values < 0.05. Statistical analysis was performed using the Statistical Package for the Social Sciences (SPSS, version 15.0). ResultsThirty-nine patients were evaluated. The mean age was 36.8 years. 84.6% had hemophilia A; 20.5% of the patients had hemophilia classified as mild, 41% as moderate and 38.5% as severe. The records of 10.5% of the patients registered seropositivity for anti-HIV and 57.9% for anti-HCV. Target joints were detected in 69.2%. The mean total Hemophilia-Specific Quality of Life score was 35.55. 'Sports and leisure'and 'Physical health'were the most impaired dimensions and the dimension 'Relationship and partners'was the least impaired. The Hemophilia-Specific Quality of Life scores showed good discriminant validity for hemophilia severity (p-value = 0.001), HIV-infection (p-value = 0.02), HCV-infection (p-value = 0.01) and the presence of target joints (p-value < 0.001). ConclusionHealth-related quality of life in hemophilia, measured by the Hemophilia-Specific Quality of Life questionnaire, was influenced by the presence of arthropathy and infectious diseases transmitted by blood products. Rehabilitation measures should be encouraged in order to improve the quality of life of these patients.

Hemophilia A is an inherited disorder characterized by deficiency of coagulation factor VIII, which predisposes patients to bleeding events. Treatment is based on replacement of the deficient factor, in a therapeutic or prophylactic manner. Brazil is the country with the third largest population of people with hemophilia, for which the public health system provides free comprehensive care. Maintaining an updated registry of patients, documenting the prevalence of complications, and assessing the effectiveness of resource use are indispensable elements in the design of a well-coordinated national program. According to sociodemographic, clinical, and laboratory data collected by the computerized Brazilian system on coagulopathies, in June 2013, there were 9,122 registered patients with hemophilia A in Brazil, of which 36.1% had a severe form of the disease. Clotting factor inhibitors were present in 7.5%, but 25.7% of records did not provide this type of data. Around 70% of the patients belonged to the economically active population, being between 15 and 59 years old. Infection by the human immunodeficiency virus was present in 23.4% of the patients tested and infection by hepatitis C virus antibodies in 59.3%. Infection by the hepatitis B virus and human T-lymphotropic virus was also reported. The high percentage of incomplete records regarding serological data shows the fragility of the information system to date. There was also no information available on the prevalence of permanent or disabling joint damage. Although few hemophiliacs receive adequate care in developing countries, and despite Brazil exhibiting great social inequalities, the Ministry of Health has made significant advances in the treatment of hemophilia A. The gradual increase in importation of factor VIII concentrate enabled the implementation of primary and secondary modalities of prophylaxis, in addition to the induction of immune tolerance. There are also plans to set up a factory in the country, to ensure Brazilian self-sufficiency in the production of blood products.

No abstract

-We studied the incidence and prognosis of acute neurologic complications in 281 children under 13 years of age with a diagnosis of acute bacterial meningitis. All the patients were examined daily by the same group of neurologists, using a standardized neurological examination. Patients with signs of encephalic lesions, unsatisfactory response to antibiotics or decreased level of consciousness were submitted to brain computer tomography. The overall lethality rate was 20.3% and cases whose causative agent was identified presented a higher lethality rate (23.7%) than those in which the agent was not found. The most important neurological abnormalities were meningeal signs (88.3%) followed by decreased consciousness (47.7%), irritability (35.2%), seizures (22.4%), fontanel bulging (20.6%) and cranial nerve palsy (14.2%). Seizures, cranial nerve palsy and the absence of meningeal signs were related to higher rates of lethality. Diminished consciousness, seizures, subdural effusion, abscess and hydrocephalus were the most important complications, respectively. We can conclude that acute bacterial meningitis continues to be an important health problem in developing countries and that public health measures will be necessary to minimize the impact of sequelae and reduce the mortality rate in children with that pathology.KEY WORDS: pyogenic meningitis, bacterial meningitis, septicemia, meningitis in children.Although the use of antibiotics has dramatically changed the mortality of patients with pyogenic meningitis, management of neurological complications remains as a significant problem 6,7 . Fatalities may occur in spite of appropriate treatment and may be related to cerebral edema, vasculitis,