Subject(s)
Anemia, Sickle Cell/drug therapy , Anti-Bacterial Agents/therapeutic use , Gastrointestinal Microbiome/drug effects , Rifaximin/therapeutic use , Adult , Analgesics, Opioid/therapeutic use , Anemia, Sickle Cell/blood , Anemia, Sickle Cell/microbiology , Anti-Bacterial Agents/pharmacology , Cellular Senescence , Female , Hemoglobin C Disease/blood , Hemoglobin C Disease/drug therapy , Hemoglobin C Disease/microbiology , Humans , Hydroxyurea/therapeutic use , L-Selectin/analysis , Leukocyte Count , Male , Middle Aged , Neutrophils/chemistry , Pain/drug therapy , Pain/etiology , Quality of Life , Receptors, CXCR4/analysis , Rifaximin/pharmacology , Sickle Cell Trait/blood , Sickle Cell Trait/drug therapy , Sickle Cell Trait/microbiology , Vascular Diseases/etiology , Young Adult , beta-Thalassemia/blood , beta-Thalassemia/drug therapy , beta-Thalassemia/microbiologySubject(s)
Anemia, Sickle Cell/microbiology , Bacteroidetes/isolation & purification , Dysbiosis/microbiology , Firmicutes/isolation & purification , Gastrointestinal Microbiome , Proteobacteria/isolation & purification , Adolescent , Adult , Aged , Anemia, Sickle Cell/blood , Anemia, Sickle Cell/complications , Anemia, Sickle Cell/immunology , Bacterial Translocation , Blood Cell Count , Blood Sedimentation , Case-Control Studies , Child , Female , Fetal Hemoglobin/analysis , Hemoglobin, Sickle/analysis , Humans , Male , Middle Aged , Neutrophil Activation , Sickle Cell Trait/blood , Sickle Cell Trait/complications , Sickle Cell Trait/immunology , Sickle Cell Trait/microbiology , Young AdultSubject(s)
Hemoglobins, Abnormal , Meningitis, Pneumococcal/complications , Sickle Cell Trait/microbiology , Tuberculosis, Meningeal/complications , Adult , Antitubercular Agents/therapeutic use , Humans , Male , Meningitis, Pneumococcal/drug therapy , Sickle Cell Trait/complications , Tuberculosis, Meningeal/drug therapyABSTRACT
BACKGROUND: Pulmonary infections continue to be a major cause of morbidity and mortality in patients with sickle cell disease (SCD). METHODS: In this study cell-mediated immunity in vitro was evaluated in 62 SCD patients (62 steady state and 16 with acute pneumonia) and compared with 44 normal controls (30 healthy and 14 with acute pneumonia). Lymphocyte blastogenic responses to phytohemagglutinin, tetanus toxoid and Candida albicans antigen were assessed in all subjects. In addition production of tumor necrosis factor, alpha- and gamma-interferon (IFN) were assayed. RESULTS: The results revealed comparable blastogenic responses to all three stimuli in all subjects except SCD patients with pneumonia. This group showed poor responses to all stimuli. The mean counts per minute were decreased 65 to 90% when compared with the other patients. Cytokine production of IFN-alpha and TNF was equivalent in all subjects. Conversely IFN-gamma production in both SCD groups, steady state (35 +/- 6 U/ml) and SCD with pneumonia (14 +/- 6 U/ml), was significantly decreased when compared with those in normal healthy controls (65 +/- 14 U/ml) and with pneumonia (48 +/- 17 U/ml). On analysis of individual titers 15 of 62 (24%) steady state and 10 of 16 (63%) SCD patients with pneumonia were deficient in IFN-gamma production in vitro. CONCLUSIONS: Acute pulmonary infections seem to have a profound effect on cell-mediated immunity in SCD. IFN-gamma deficiency, along with quantitative and qualitative T cell abnormalities, may represent significant factors to explain the frequent and severe infections seen in SCD.
Subject(s)
Interferon-alpha/biosynthesis , Interferon-gamma/biosynthesis , Lymphocyte Activation , Pneumonia, Bacterial/immunology , Sickle Cell Trait/complications , Tumor Necrosis Factor-alpha/biosynthesis , Adolescent , Candida albicans/immunology , Child , Child, Preschool , Female , Humans , Infant , Interferon-alpha/blood , Interferon-gamma/blood , Lymphocytes/chemistry , Male , Phytohemagglutinins/immunology , Sickle Cell Trait/immunology , Sickle Cell Trait/microbiology , Tetanus Toxoid/immunologyABSTRACT
Acute hematogenous osteomyelitis was detected in a 23-year-old sickle-cell patient after standard radiographs of the right femur showed gas within the medullary cavity. Three anaerobic organisms were cultured from surgically aspirated materials, but no source of infection was found. After surgical drainage and six weeks of intravenous clindamycin therapy, there was clinical improvement and radiographic resolution of the gas formation.