ABSTRACT
INTRODUÇÃO: A doença falciforme (DF) compreende um grupo de anemias hemolíticas hereditárias cuja principal característica é a presença do gene da hemoglobina S (Hb S) que pode combinar-se com outras anormalidades sanguíneas hereditárias. O quadro clínico varia desde quase assintomático até formas graves. A Hidroxiuréia (HU) é a terapia de maior sucesso. É uma doença crônica, cuja prevalência e morbidade são altas no Brasil, dessa forma a qualidade de vida (QV) surge como um importante desafio para os pacientes, familiares e profissionais de saúde. OBJETIVOS: Avaliar a QV de pacientes portadores de DF, suas características sociodemográficas e clínicas. MÉTODOS: Os dados foram coletados através do questionário de QV abreviado (WHOQOL-Bref), questionário sociodemográfico e prontuário clínico. RESULTADOS: foram entrevistados 39 pacientes, entre 18 e 54 anos, tratados na Fundação Hemominas em Divinópolis - MG. A maioria dos pacientes eram homens (56%), homozigotos para a Hb S (80%), solteiros (67%), segundo grau completo (31%) e não usuários de HU (61%). Dentre os pacientes avaliados 53,8% considerou ter boa QV (escore médio 74,2 ±20,3). O escore relacionado à QV geral foi de 71,5. Os domínios físico e meio ambiente obtiveram os menores escores médio (61,5 e 59,0, respectivamente). Não foram encontradas diferenças significativas entre os que utilizavam ou não HU. CONCLUSÕES: Os pacientes com DF apresentaram boa QV, sendo mais comprometida pelos aspectos relacionados ao domínio meio ambiente, (como recursos financeiros) e físico (como dor e desconforto) que se correlacionam com as características clínicas e sociais relacionadas a DF.
Introduction: sickle cell disease (SCD) comprises a group of hereditary hemolytic anemias whose main characteristic is the presence of the hemoglobin S (Hb S) gene that can be combined with other hereditary blood abnormalities. The clinical picture ranges from almost asymptomatic to severe forms. Hydroxyurea (HU) is the most successful therapy. It is a chronic disease whose prevalence and morbidity are high in Brazil, thus quality of life (QoL) emerges as an important challenge for patients, family members and health professionals. Objectives: This study aimed to evaluate the QoL of patients with SCD, their sociodemographic and clinical characteristics. Methods: Data were collected through the abbreviated QoL questionnaire (WHOQOL-Bref), sociodemographic questionnaire and clinical chart. Results: It has interviewed at the Hemominas Foundation in Divinópolis - MG, 39 patients aged 18-54. Most of the patients were male (56%), homozygous for Hb S (80%), unmarried (67%), completed high school (31%) and non HU users (61%). Among the patients evaluated, 53.8% considered having a good QoL (mean score 74.2 ± 20.3). The overall QoL score was 71.5. The physical and environmental domains had the lowest mean scores (61.5 and 59.0, respectively). No significant differences were found among those using or not using HU. Conclusions: Patients with SCD presented good QoL, being more affected by aspects related to the environmental domain (such as financial resources) and physical (such as pain and discomfort) those correlate with clinical and social characteristics related to SCD.
Subject(s)
Humans , Male , Female , Adolescent , Adult , Middle Aged , Young Adult , Quality of Life , Anemia, Sickle Cell , Pain , Social Environment , Socioeconomic Factors , Hemoglobins/analysis , Surveys and Questionnaires , Environment , Anemia, Sickle Cell/psychology , Anemia, Sickle Cell/drug therapyABSTRACT
ABSTRACT Introduction: Hemophilia is a coagulopathy caused by a deficiency in coagulation factors VIII (hemophilia A) or IX (hemophilia B). It is a chronic disease and, hence, impairs the quality of life (Qol) of the patients. This study aimed to evaluate the Qol of patients with hemophilia using the WHOQOL-bref and the Haemo-A-Qol instruments, its relation to the clinical condition and its sociodemographic variables. Methods: This is a cross-sectional, epidemiological study, comprising 17 patients with hemophilia, registered at the hemocenter, who met the eligibility criteria. Data were collected using three questionnaires: a semi-structured clinical evaluation form, the WHOQOL-bref and the Haem-A-Qol. Results: The average age was 30 years old, and most participants declared themselves to be single (58.82%), without children (64.70%) and employed (58.82%). Hemophilia A was observed in 14 patients and the most severe form of the disease was more prevalent (64.70%). The average score of Qol, estimated by the WHOQOL-bref questionnaire was 74.3; being "social relations" the domain with the highest average. The Haem-A-Qol measured an average of 36.2 and the domain with the highest result was "Family Planning". Conclusion: Hemophilia had a higher negative impact upon the physical, sports and leisure features in the sample subjects. The analysis of the questionnaires did not reveal statistical agreement between them. Based on this, the Haem-A-Qol is considered the most recommended to evaluate the Qol, as it addresses factors more specifically related to the disease. No statistical significance was observed between the scores of Qol, as for the presence of comorbidities, gravity of the hemophilia and positive serology for infections.
Subject(s)
Humans , Adult , Middle Aged , Quality of Life , Surveys and Questionnaires , Hemophilia AABSTRACT
Abstract Objective: This study aimed to describe and analyze clinical and laboratory characteristics of patients with sickle cell anemia treated at the Hemominas Foundation, in Divinópolis, Brazil. Furthermore, this study aimed to compare the clinical and laboratory outcomes of the group of patients treated with hydroxyurea with those patients that were not treated with hydroxyurea. Methods: Clinical and laboratorial data were obtained by analyzing medical records of patients with sickle cell anemia. Results: Data from the medical records of 50 patients were analyzed. Most of the patients were female (56%), aged between 20 and 29 years old. Infections, transfusions, cholecystectomy, splenectomy and systemic arterial hypertension were the most common clinical adverse events of the patients. The most frequent cause of hospitalization was painful crisis. The majority of patients had reduced values of hemoglobin and hematocrit (8.55 ± 1.33 g/dL and 25.7 ± 4.4%, respectively) and increased fetal hemoglobin levels (12 ± 7%). None of the clinical variables was statistically significant on comparing the two groups of patients. Among hematological variables only hemoglobin and hematocrit levels were statistically different between patients treated with hydroxyurea and untreated patients (p-value = 0.005 and p-value = 0.001, respectively). Conclusion: Sickle cell anemia requires treatment and follow-up by a multiprofessional team. A current therapeutic option is hydroxyurea. This drug reduces complications and improves laboratorial parameters of patients. In this study, the use of the drug increased the hemoglobin and hematocrit levels of patients.
Subject(s)
Hemoglobin, Sickle , Hydroxyurea , Anemia, Sickle CellABSTRACT
A Eletroforese de Proteínas Séricas (EPS) é um método simples, que permite separar proteínas do plasma humano em frações. Sua interpretação traz informações úteis ao médico. Assim, ela é importante para a investigação e diagnóstico de diversas doenças. O exame consiste em aplicar a amostra do soro em um meio sólido e submetê-la a um potencial elétrico. As proteínas percorrem distâncias diferentes, formando bandas denominadas: albumina, alfa-1-globulina, alfa-2-globulina, betaglobulina e gamaglobulina. Essas bandas são, em seguida, quantificadas. Observa-se diminuição da concentração de albumina em situações que promovam sua perda, baixa ingesta protéica ou elevado catabolismo. As frações alfaglobulinas apresentam níveis aumentados em processos inflamatórios, infecciosose imunes. O aumento da betaglobulina é observado em situações de perturbação do metabolismo lipídico ou na anemia ferropriva. A ausência ou diminuição da bandagama indica imunodeficiências congênitas ou adquiridas. O seu aumento sugere elevação policlonal das imunoglobulinas associado às condições inflamatórias, neoplásicas ou infecciosas, além da elevação monoclonal observada no mieloma múltiplo e em outras desordens linfoproliferativas, como a macroglobulinemia de Waldenstrõm. O conhecimento dos principais componentes de cada banda eletroforética facilita o raciocínio clínico e auxilia na identificação de padrões eletroforéticos, característicos de algumas doenças.
Seric proteins electrophoresis (SPE) is a simple method that allows the separation of the human plasma proteins in fractions. Its interpretation gives useful information to the physician, which makes it an important tool for several diseases investigation and diagnosis. The exam consists in apllying the serum sample into a solid medium and put it under an electrical ptential...
Subject(s)
Humans , Blood Protein Electrophoresis , Blood Proteins/analysisABSTRACT
O objetivo do estudo é apresentar o trabalho de orientaçäo desenvolvido com uma criança pequena portadora da Síndrome Cornélia De Lange, atendida quinzenalmente. Säo discutidas as características genéticas e físicas da síndrome e suas implicaçöes no desenvolvimento geral da criança, Os resultsdos obtidos mostraram que esse tipo de intervençäo utilizada no processo terapêutico fonoaudiológico é viável e eficiente