Our study aims to observe the levels of knowledge, attitude, and practice (KAP) associated with sickle cell disease (SCD) and premarital genetic counseling (PMGC) in 351 Saudi adults. The relationships between KAP levels and sociodemographic characteristics (age, gender, marital status, and educational level) were observed. The study was conducted in King Khalid University Hospital between February 21, 2017, and March 7, 2018. A total of 351 Saudi participants attending the primary care clinic were selected using convenience sampling and were given a self-administered questionnaire. Overall, the 351 participants had the best attitude (41% scoring "good"), followed by knowledge (28.8%), and, lastly, practice (19.1%). Out of the sociodemographic characteristics, age group was the most statistically significant in all the three categories (knowledge, attitude, and practice). The > 50-year age group performed the worst in all the three categories. Despite the advancements in public healthcare measures in Saudi Arabia, our study revealed that there are still many gaps to be filled regarding the knowledge, attitude, and practice associated with SCD and PMGC.
Background: Abnormalities in lung function tests have been shown to commonly occur in a majority of patients with sickle cell disease (SCD) even at steady state. The prevalence and pattern of these lung function abnormalities have been described in other populations but this is unknown among our sickle cell cohort. There is generally little information available on risk factors associated with the lung function abnormalities and its relevance in patient care.
Method: This was an analytical cross-sectional study involving 76 clinically stable, hydroxyurea-naive adult Hb-SS participants and 76 nonsickle cell disease (non-SCD) controls. A structured questionnaire was used to obtain sociodemographic data and clinical history of the participants. Investigations performed included spirometry, pulse oximetry, tricuspid regurgitant jet velocity (TRV) measurements via echocardiogram, complete blood counts, free plasma haemoglobin, serum urea, and creatinine.
Results: Weight, BMI, mean FVC, and FEV1% predicted values were comparatively lower among the Hb-SS patients (p < 0.001). Abnormal spirometry outcome occurred in 70.4% of Hb-SS patients, predominantly restrictive defects (p < 0.001), and showed no significant association with steady-state Hb, WBC count, free plasma haemoglobin, frequency of sickling crisis, chronic leg ulcers, and TRV measurements (p > 0.05). The mean oxygen saturation was comparatively lower among Hb-SS patients (p < 0.001).
Conclusion: Measured lung volumes were significantly lower in Hb-SS patients when compared to non-SCD controls and this difference was not influenced by anthropometric variance. Lung function abnormalities, particularly restrictive defects, are prevalent in Hb-SS patients but showed no significant association with recognized markers of disease severity.
Background: Brown recluse spider (BRS) envenomation can lead to significant morbidity through severe local reaction and systemic illness including acute hemolytic anemia, rhabdomyolysis, disseminated intravascular coagulopathy (DIC), and even death. We aim to describe the clinical features and the roles of antibiotics and steroids in the treatment of loxoscelism.
Methods: We retrospectively identified nine patients (pts) at our institution who were admitted with moderate to severe loxoscelism. A chart review was performed to highlight important clinical features and effect of interventions.
Results: Nine pts (age 18 to 53) presented with fever (6), rash (9), pain/swelling (4), and jaundice (2). Of these, 6 pts had antecedent spider bites documented. Five pts were discharged from Emergency Room (ER) with oral antibiotics for "cellulitis" and were readmitted with severe systemic symptoms, with almost half (45%) of the pts being admitted to the intensive care unit. The most common admission diagnosis was sepsis secondary to cellulitis (6). Four pts developed worsening dermonecrosis, and 3 received prompt incision and drainage (I&D) with debridement. Hemolytic anemia developed around day 5 after spider bite (average); the lowest mean hemoglobin level was 5.8g/dL, with average drop of 3.1 g/dL. Direct antiglobulin test (DAT) (for both complement and surface immunoglobulin) was positive in 4 out of 9 patients. Four pts received glucocorticoid therapy for their hemolytic anemia. The use of steroid and intravenous immunoglobulin (IV Ig) did not seem to show a difference in the time of recovery although those who received steroids required less blood transfusion (2.1 units less). All pts had a complete recovery within two weeks.
Conclusion: Treatment of systemic loxoscelism involves aggressive supportive care including appropriate wound management, blood transfusions, intravenous fluid replacement, and appropriate antibiotic coverage. It is unclear at this time if glucocorticoids or IVIg has any beneficial impact on the treatment of severe loxoscelism.
Background: Globally, anemia, among people living with HIV/AIDS, is a major public health problem. It has a significant effect on the progression of HIV/AIDS to advanced stages and there are a number of factors that often affect anemia. However, there is little insight regarding factors affecting anemia among HIV/AIDS patients in developing countries, including Ethiopia.
Objective: This study aimed at investigating factors affecting anemia among people living with HIV/AIDS taking ART drug at Tikur Anbessa Specialized Hospital, Addis Ababa, Ethiopia.
Methods: A hospital based cross-sectional study design was used to assess factors affecting anemia among people living with HIV/AIDS. Structured checklist was used to gather information from charts of patients selected by simple random sampling method. We analyzed the data to identify factors associated with anemia among people with HIV/AIDS using logistic regression models.
Results: A total of 301 selected charts were reviewed. The median age was 38 ± 10.38. The majority (62.5%) of the patients were taking ZDV-containing ART drug (ZDV/3TC/NVP). The overall anemia prevalence was 34.6%, while about 5%, 15.6%, and 14% of the patients had severe, moderate, and mild prevalence of anemia, respectively. Factors that were found to affect anemia among these patients include gender (OR = 2.26 [95% CI: 1.22, 4.16]), occupation (OR: 0.57 [95%CI: 0.35, 0.92]), WBC count (OR = 2.30 [95% CI: 1.29, 4.09]), platelet count (OR = 2.89 [95% CI: 0.99, 8.41]), nutritional status (OR = 2.05 [95% CI: 0.69, 6.02]), and WHO clinical stage of HIV/AIDS (OR = 3.69 [95% CI: 1.86, 7.31]).
Conclusions: About one in three patients was found to be anemic. Intervention aimed at diagnosing and treating anemia among people living with HIV/AIDS should be considered.
Background: Sickle cell anaemia (SCA) is the most prevalent monogenic disease in Brazil. In SCA, haemoglobin S (HbS) is formed, which modifies red blood cell morphology. Intravascular haemolysis occurs, in which free Hb and free radicals degrade nitric oxide (NO) and release arginase, which reduces arginine levels. Because arginine is a substrate for NO formation, this decrease leads to reduced NO (vasodilator) synthesis. SCA treatment uses hydroxyurea (HU) to maintain high foetal haemoglobin (HbF) levels and reduces HbS to avoid haemolytic episodes.
Objective: To analyse the efficacy of L-arginine as an adjuvant in the treatment of SCA patients.
Setting: The State Blood Centre of Ceará, Brazil.
Methods: This was a randomized double-blind clinical study of adults with SCA with continuous use of HU at the State Blood Centre of Ceará. The clinical study enrolled 25 patients receiving HU + L-arginine (500 mg) and 25 patients receiving HU + placebo. The treatment was carried out over four months. Laboratory tests were performed to determine the levels of the following: (1) complete blood count; (2) nitrite + nitrate; (3) HbF; and (4) reticulocytes. The clinical experiments were performed by a haematologist. The main outcome measures were nitrite and pain.
Results: Statistical analysis showed that the levels of NO were increased in the study group, and there was also a reduction in pain frequency using a pain frequency scale by day, week, and month. The levels of nitrite plus nitrate in the group receiving placebo plus HU did not change among the times evaluated (38.27 ± 17.27 mg/L, 39.49 ± 12.84 mg/L, 34.45 ± 11.25 mg/L, p >0.05), but in the patients who received supplementation with L-arginine plus HU, a significant increase in nitrite plus nitrate levels was observed between M0 and M4 (36.55 ± 20.23 mg/L versus 48.64 ± 20.63 mg/L, p =0.001) and M2 and M4 (35.71 ± 15.11 mg/L versus 48.64 ± 20.63 mg/L, p <0.001). It is important to note that the increase in nitrite plus nitrate levels occurred only in the fourth month of follow-up of patients in the treatment group, showing that at least 4 months of supplementation with L-arginine is necessary to show an increase in these metabolites in the serum.
Conclusion: The use of L-arginine as a coadjuvant in the treatment of sickle cell anaemia may function as a potential tool for pain relief, consequently improving the life of patients.
Background: Limited data are available from real-world practices in Europe describing prevailing treatment patterns and outcomes in relapsed/refractory multiple myeloma (RRMM), particularly by cytogenetic risk.
Methods: A retrospective medical record review was conducted in 200 RRMM patients in France. From first relapse, patients were assessed on second-/third-line treatments, progression-free survival (PFS), overall survival (OS), and healthcare utilization.
Results: Fifty-five high risk and 113 standard risk patients were identified. Overall, 192 patients (96%) received second-line therapy after relapse. Lenalidomide-based regimens were most common (>50%) in second line. Hospitalization incidence in high risk patients was approximately twice that of standard risk patients. From Kaplan-Meier estimation, median (95% CI) second-line PFS was 21.4 (17.5, 25.0) months (by high versus standard risk: 10.6 [6.4, 17.0] versus 28.7 [22.1, 37.3] months). Among second-line recipients, 47.4% were deceased at data collection. Median second-line OS was 59.4 (38.8, NE) months (by high versus standard risk: 36.5 [17.4, 50.6] versus 73.6 [66.5, NE] months).
Conclusions: The prognostic importance of cytogenetic risk in RRMM was apparent, whereby high (versus standard) risk patients had decidedly shorter PFS and OS. Frequent hospitalizations indicated potentially high costs associated with RRMM, particularly for high risk patients. These findings may inform economic evaluations of RRMM therapies.
Background and objectives: Chronic myeloid leukemia (CML) is characterized by hyperproliferation of myeloid precursors, increased fibrosis, and neoangiogenesis in the bone marrow. Imatinib inhibits BCR-ABL tyrosine kinase produced due to reciprocal translocation t(9;22) in neoplastic CML cells. It reduces hyperproliferation of myeloid precursors and has been found to affect bone marrow fibrosis and angiogenesis. This study was done to assess the effect of imatinib on bone marrow morphology and angiogenesis in CML.
Methods: 31 newly diagnosed CML patients were evaluated before and after 3 months of imatinib therapy. A marrow morphological response (MMR) score was used to assess marrow cytological and histological features including grade of fibrosis. Mean microvessel density (MVD) was also assessed. Hematological parameters and BCR-ABL transcript levels were assessed in the peripheral blood.
Results: 86.21% of patients showed decrease in marrow cellularity with normalization of M:E ratio. 72.42% of patients had decrease in grade of fibrosis and 17.24% showed no change while 10.34% of patients showed progression of fibrosis grade. Patients with MMR score ≥ 2 (n=4) and those with progression of fibrosis grade (n=3) showed suboptimal molecular response (BCR-ABL transcripts > 10%). Pretherapy mean MVD of patients (14.69 ± 5.28) was higher than that of controls (6.32 ± 1.64). A significant reduction of 66.51% was observed in posttherapy mean MVD (4.98 ± 2.77) of CML patients (p<0.001).
Conclusion: Imatinib therapy in CML not only decreases marrow cellularity, but also helps towards normalization of bone marrow microenvironment by reducing fibrosis and angiogenesis.
Traceability is an essential tool for haemovigilance and transfusion safety. In Burkina Faso, the implementation of haemovigilance has been achieved as part of a pilot project from 2005 to 2009. Our study aims to evaluate the traceability of blood transfusions and reporting of adverse reactions over the 6-year postpilot phase. A cross-sectional study including all blood units ordered between 2010 and 2015 has been conducted in public and private health care facilities supplied with blood products by the transfusion center of Bobo-Dioulasso. The complete traceability was possible for 83.5% of blood units delivered. Adverse reactions were reported in 107 cases representing 2.1/1,000 blood units per annum. Transfusions of wrong blood to wrong patient were reported in 13 cases. Our study shows that the haemovigilance system in Burkina Faso must be improved. Healthcare workers have to be sensitized on how traceability and haemovigilance could impact the quality of care provided to patients.
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