RESUMO
BACKGROUND: Common variable immunodeficiency (CVID) is considered the most symptomatic type of inborn errors of immunity in humans. Along with infectious complications, which have numerous consequences, noninfectious complications are a major challenge among CVID patients. METHODS: All CVID patients registered in the national database were included in this retrospective cohort study. Patients were divided into 2 groups based on the presence of B-cell lymphopenia. Demographic characteristics, laboratory findings, noninfectious organ involvement, autoimmunity, and lymphoproliferative diseases were evaluated. RESULTS: Among 387 enrolled patients, 66.4% were diagnosed with noninfectious complications and 33.6% with isolated infectious presentations. Enteropathy, autoimmunity, and lymphoproliferative disorders were reported in 35.1%, 24.3%, and 21.4% of patients, respectively. Some complications, including autoimmunity and hepatosplenomegaly, were reported to be significantly more frequent among patients with B-cell lymphopenia. As for organ involvement, the dermatologic, endocrine, and musculoskeletal systems were predominantly affected in CVID patients with B-cell lymphopenia. Among autoimmune manifestations, the frequency of rheumatologic, hematologic, and gastrointestinal autoimmunity was reported to be higher than that of other types of autoimmunity not associated with B cell-lymphopenia. Furthermore, hematological cancers, particularly lymphoma, were the most common type of malignancy. The mortality rate was 24.5%, and respiratory failure and malignancies were the most common causes of death, with no significant differences between the 2 groups. CONCLUSIONS: Considering that some of the noninfectious complications might be associated with B-cell lymphopenia, regular patient monitoring and follow-up with proper medication (in addition to immunoglobulin replacement therapy) are highly recommended to prevent sequelae and increase patient quality of life.
Assuntos
Linfócitos B , Imunodeficiência de Variável Comum , Linfopenia , Humanos , Imunodeficiência de Variável Comum/complicações , Imunodeficiência de Variável Comum/imunologia , Feminino , Masculino , Adulto , Estudos Retrospectivos , Linfócitos B/imunologia , Pessoa de Meia-Idade , Linfopenia/imunologia , Adulto Jovem , Autoimunidade , Adolescente , Idoso , CriançaRESUMO
BACKGROUND AND OBJECTIVE: Common variable immunodeficiency (CVID) is considered the most symptomatic type of inborn errors of immunity in humans. Along with infectious complications, which have numerous consequences, non-infectious complications are also a major challenge among CVID patients. METHODS: All registered CVID patients in the national database were included in this retrospective cohort study. Patients were divided into two groups based on the presence of B-cell lymphopenia. Demographic characteristics, laboratory findings, non-infectious organ involvements, autoimmunity, and lymphoproliferative diseases were evaluated. RESULTS: Among 387 enrolled patients, 66.4% were diagnosed with non-infectious complications; however, 33.6% had only infectious presentations. Enteropathy, autoimmunity, and lymphoproliferative disorders were reported in 35.1%, 24.3%, and 21.4% of patients, respectively. Some complications, including autoimmunity and hepatosplenomegaly, were reported to be significantly higher among patients with B-cell lymphopenia. Among organ involvement, dermatologic, endocrine and musculoskeletal systems were predominantly affected in CVID patients with B-cell lymphopenia. Among autoimmune manifestations, the frequency of rheumatologic, hematologic, and gastrointestinal autoimmunity was reported to be higher compared to other types of autoimmunity independent from the B cell-lymphopenia. Furthermore, hematological cancers, particularly lymphoma, were slightly introduced as the most common type of malignancy. Meanwhile, the mortality rate was 24.5%, and respiratory failure and malignancies were reported as the most common cause of death in our patients without significant differences between the two groups. CONCLUSION: Considering that some of the non-infectious complications might be associated with B-cell lymphopenia, therefore, regular patient monitoring and follow-up along with proper medications (besides immunoglobulins replacement therapy) are highly recommended to prevent further sequels and increase the patients' quality of life.
RESUMO
OBJECTIVE: To assess the quality of the pharmacological control of hypertension. DESIGN: A cross-sectional study. SUBJECTS: Primary health care centers-registered hypertensive patients. SETTING: Primary health care centers in Al-Khobar, Saudi Arabia. METHODS: Data was recorded, using a structured questionnaire, through direct interviewing of patients, and from their medical records. It included demographic characteristics, hypertension related, and doctors'-related variables; and patients' utilization of other health facilities and whether they had other chronic diseases. RESULTS: The proportion of patients with controlled hypertension was 37%. It was significantly increased with age below 55 years, with non-Saudis, duration of treatment shorter than 5 years; and with the use of monotherapy. Doctors' care-related variables did not show significant variation with the number of controlled patients which may imply that the quality of follow-up might not be adequate. CONCLUSIONS: The proportion of controlled patients cared for in the PHC centers is low. The factors associated with control were age, and the use of a single antihypertensive drug. The study questions the quality of follow-up of patients by the PHC physicians.