Adherence to Hydroxyurea and Patients' Perceptions of Sickle Cell Disease and Hydroxyurea: A Cross-Sectional Study.

Background and Objectives: Hydroxyurea is a crucial treatment for sickle cell disease (SCD), but some patients' adherence to it remains suboptimal. Understanding patients' perspectives on SCD and HU is essential for improving adherence. This study aimed to assess hydroxyurea adherence and patients' perceptions of SCD and hydroxyurea among SCD patients in the Jazan region of Saudi Arabia. Materials and Methods: This cross-sectional study collected data from 217 SCD patients using self-administered questionnaires from August 2022 to January 2023. The survey covered patient demographics, SCD consequences, and other clinical data. We used the Brief Illness Perception Questionnaire (B-IPQ) to measure patients' disease perception and the 8-item Morisky Medication Adherence Scale (MMAS-8) to evaluate patients' adherence to HU. Data were analysed using descriptive, t-test, and chi-square tests, and the p-value was set at <0.05 for significance. Results: More than half of the patients were male, with a mean age of 28.09 ± 8.40 years. About 57.6% of the patients were currently using HU. About 81.6% of HU users reported low adherence. The adherence was lower among individuals with infections/recurrent infections and in patients who received repeated blood transfusions. ICU admission, blood transfusion, and certain SCD complications were associated with HU use. Male patients had a higher perception of SCD consequences, concern, and understanding. ICU-admitted and recurrent hospitalized patients had a higher perception of the SCD-related consequences, symptoms, concerns, and emotional responses. Conclusions: HU seems a well-established and efficacious disease-modifying agent, but its underutilization for SCD patients remains challenging. To overcome the adherence challenges, healthcare providers must educate SCD patients about the role of hydroxyurea in lowering disease severity and addressing side effects to obtain maximum benefits. Healthcare providers may consider tailored educational interventions to improve adherence, particularly for patients with infections, recurrent hospitalizations, or repeated blood transfusions. Further research is needed to identify strategies for improving hydroxyurea adherence and patient education among SCD patients.

Recent advances in the management of immune thrombocytopenic purpura (ITP): A comprehensive review.

Autoimmune disorders place a substantial burden on the healthcare system all over the world affecting almost 3% to 8% of the population. Immune thrombocytopenic purpura (ITP), also known as idiopathic thrombocytopenic purpura, is a blood disorder in which the body immune system destroys platelets, leading to low platelet counts in the blood (peripheral blood platelet count < 150 × 109/L). Although the pathophysiology of ITP is not fully understood, it is believed to result from a complex interplay between hereditary and environmental variables. Certain factors, such as a low platelet count, history of bleeding, and certain comorbidities can increase the risk of severe bleeding in patients with ITP. Corticosteroids, intravenous immunoglobulin (IVIG), immunosuppressants, rituximab, and thrombopoietin receptor agonists (TPO-RAs) are some of the advanced treatments for ITP. Although these therapies may be successful, they also carry the risk of negative effects. Recently, significant advancements have been made in the understanding and treatment of ITP. There is still much to learn about the disease, and new, more effective treatments are needed. This comprehensive review offers a comprehensive assessment of recent advancements in ITP management, with a focus on active research projects, novel therapeutic targets, new treatment modalities, and areas of uncertainty and unmet needs. According to research, it is crucial to develop individualized treatment plans for ITP patients based on their age, platelet count, risk of bleeding, and comorbidities. The article also looks at how future developments in gene editing, bispecific antibody therapies, and cellular therapy may completely change the treatment of ITP.

Impaired Awareness of Hypoglycemia and its Risk Factors among Diabetic Patients in Jazan, Saudi Arabia: A Cross-Sectional Study.

BACKGROUND: Impaired awareness of hypoglycemia (IAH) is related to a three- to sixfold increase in the risk of severe hypoglycemia in adults with type 1 diabetes mellitus (T1D). This study aimed to assess the prevalence of IAH and its risk factors and determine the frequency of hypoglycemic symptoms. OBJECTIVE: This study aimed to assess the prevalence of IAH and its risk factors and determine the frequency of hypoglycemic symptoms. METHODOLOGY: A cross-sectional study was conducted among T1D patients attending Jazan Endocrine and Diabetes Center in Jazan province, Saudi Arabia. A total of 151 patients participated, using the interview-based Clarke questionnaire, a validated eight-item questionnaire to evaluate IAH. Scoring four or more answers as reduced awareness categorizes the participant as having IAH. RESULTS: The prevalence of IAH was 25.2% among the T1D patients. IAH was significantly associated with body mass index (BMI; p = 0.034), occupation (p = 0.014), and blood glucose monitoring methods (p = 0.027). Shaking and sweating were the most commonly reported symptoms of hypoglycemia. A BMI of <25 kg/m2 was higher linked to hunger and speech difficulty compared to a BMI of ≥25 kg/m2 (p < 0.05). Changing the insulin injection site was associated with confusion, odd behavior, and speech difficulty (p < 0.05). Monitoring blood glucose four times daily was associated with sweating, odd behavior, and incoordination (p = 0.024) compared to monitoring less than four times daily (p < 0.05). A hemoglobin A1c (HbA1c) reading of ≥7 was linked to odd behavior compared to an HbA1c reading of <7 (p = 0.032). Patients committed to insulin injections were more likely to experience palpitations than non-committed patients (p = 0.038). Each one-unit increase in age, monitoring blood glucose, and income was associated with a decrease in the odds of IAH (OR of Age = 0.89, 95% CI: 0.83-0.95) (OR of income = 0.10, 95% CI: 0.01-0.55). Moreover, individuals with a Body Mass Index (BMI) greater than or equal to 25 (OR = 2.99, 95% CI: 1.13-8.25), employed individuals (OR = 18.2, 95% CI: 3.75-105), and diabetes duration of more than ten years (OR = 3.96, 95% CI: 1.31- 13.2) exhibited an increase in the higher risk of IAH. CONCLUSION: IAH was prevalent among T1D patients attending Jazan Endocrine and Diabetes Center. The main associated factors included BMI, blood glucose monitoring method, and occupation. Future research should investigate the underlying causes of the observed associations and explore strategies to enhance the awareness of hypoglycemia.

A Cross-Sectional Study on the Knowledge and Awareness of Lung Cancer and Screening in Jazan Region, Saudi Arabia.

Background: Lung cancer (LC) is the most common cause of cancer-related deaths worldwide. With lung cancer often diagnosed at advanced stages, understanding the local population's awareness levels is crucial for designing effective preventive strategies. By identifying gaps in knowledge, the research aims to inform targeted health education efforts, optimize resource allocation, influence policy development, and contribute to the limited body of research on lung cancer awareness in the region, ultimately fostering improved public health outcomes. Methods: This was a cross-sectional observational study conducted in Jazan region, Saudi Arabia, from July 2022 to June 2023, 671 participants over 18 years old, encompassing both genders, were gsurveyed. Data was collected through a questionnaire covering sociodemographic characteristics and LC-related awareness. SPSS 23 was used for analysis. Factors associated with knowledge scores were explored using independent t-tests and ANOVA, with the Tukey post-hoc test identifying specific group differences. Results: The study included 671 participants, most participants were between 18 and 35 years (73.5%), with 38.5% males and 61.5% females. Lung cancer (LC) awareness was high (95.1%), with 4.9% reporting a family history. Knowledge assessment revealed a mean score of 14.66, with 41.6% having low, 49.5% moderate, and 8.9% high knowledge levels. Correct responses were notable for recognizing LC as a common cancer, a leading cause of death, and associating smoking and shisha with risk. Symptoms were well identified. Screening awareness was at 63.5%, with 78.8% willing to undergo tests if at risk. Age, marital status, and occupation were associated with knowledge, while factors like gender, nationality, residency, education, income, and smoking status showed no significant associations. Conclusion: The findings indicate that there are knowledge gaps related to LC and its screening in Jazan region in Saudi Arabia. Effective awareness programs targeting specific sociodemographic groups are needed to improve the early detection and outcomes.

Overview of the Association Between the Pathophysiology, Types, and Management of Sickle Cell Disease and Stroke.

Sickle cell disease (SCD) is a genetic blood disorder that affects hemoglobin and increases stroke risk, particularly in childhood. This review examines the pathophysiological association between SCD and stroke, the classification of stroke types, risk factors, diagnosis, management, prevention, and prognosis. A comprehensive literature search was conducted via PubMed, Scopus, and Cochrane databases. Relevant studies on SCD and stroke pathophysiology, classification, epidemiology, diagnosis, treatment, and prevention were identified. Sickle cell disease causes red blood cells to become rigid and sickle-shaped, obstructing blood vessels. Recurrent sickling alters cerebral blood flow and damages vessel walls, often leading to ischemic or hemorrhagic strokes (HS). These occur most frequently in childhood, with ischemic strokes (IS) being more common. Key risk factors include a prior transient ischemic attack (TIA), low hemoglobin, and a high leukocyte count. Neuroimaging is essential for diagnosis and determining stroke type. Primary prevention centers on blood transfusions and hydroxyurea for those at high risk. Acute treatment involves promptly restoring blood flow and managing complications. However, significant knowledge gaps remain regarding stroke mechanisms, optimizing screening protocols, and improving long-term outcomes. This review synthesizes current evidence on SCD and stroke to highlight opportunities for further research and standardizing care protocols across institutions. Ultimately, a holistic perspective is critical for mitigating the high risk of debilitating strokes in this vulnerable patient population.

Effectiveness of Blood Glucose Time in Range to Reduce Risk of Blood Glucose Extrusion and Improve Blood Glucose Metrics in Type 1 Diabetic Patients.

BACKGROUND: With evolving diabetes technology, continuous glucose monitoring (CGM) and time in range have been advanced as critical measurements to assess complications. They have shown improvement in A1C levels and decreased episodes of blood glucose extrusion. AIMS: This study aimed to assess the awareness and utilization of blood glucose time in range and its effectiveness in reducing the risk of blood glucose extrusion and improving blood glucose metrics among patients with type 1 diabetes mellitus. METHODS: A retrospective study included 342 patients who met the inclusion criteria and were using the CGM, aiming for a TIR of 70% daily. Glycemic control was followed using TIR data, blood glucose extrusion frequency (including hyperglycemia and hypoglycemia events), active sensor time, average blood glucose, and glucose management indicator (GMI) levels. RESULTS: A total of 342 individuals participated in this study, the majority of whom were below 18 years of age (62.3%). The hypoglycemic frequency was significantly increased compared to the baseline, and most participants experienced hypoglycemia events (p = 0.0001). The incidences increased over time, with 90.9% and 93% having hypoglycemia at 60 and 90 days (p = 0.0001), respectively. The active scan and sensor time were not followed, which led to the blood glucose target not being achieved, with no improvement throughout the study. Consequently, no improvement occurred in glycemic control. CONCLUSION: CGM technology has been promising and proven effective in improving glycemic. However, our study did not show these benefits as expected, which could be explained by the underutilization and improper use of the CGM.

Awareness and Perception Toward Alzheimer's Disease Among Residents Living in the Jazan Province, Saudi Arabia: A Cross-Sectional Study.

BACKGROUND: Alzheimer's disease (AD) is a growing public health concern, yet misconceptions about the condition are common. This study assessed awareness and social perceptions of AD in Jazan. METHODS: A cross-sectional survey of 925 adults was conducted. Knowledge was assessed using a 30-item Alzheimer's Disease Knowledge Scale (ADKS). Social perceptions were evaluated using a 10-item questionnaire. RESULTS: Many had misconceptions about AD epidemiology, causes, management, and care. The mean ADKS score was 8.89 ± 5.17 out of 30. Knowledge was poorest for symptoms, risk factors, treatment, caregiving, and life impact. Knowledge was highest in those aged >45 years (p = 0.018), in those with income > 15K SR (p = 0.004), in retired individuals (p = 0.023), and in those who learned about AD from books (p = 0.001), healthcare professionals (p = 0.001), or had an affected relative (p = 0.001). However, knowledge was low across all domains, averaging only 29% correct answers. Most respondents held positive social perceptions, yet sizable minorities saw isolation, legal intervention, and institutionalization as appropriate. Additionally, a portion of respondents associated stigma with individuals affected by AD and expressed a sense of burden associated with the condition. CONCLUSIONS: There are substantial knowledge gaps and some stigmatizing attitudes about AD in Jazan. Awareness regarding the causes, diagnosis, and management of AD was low. Misconceptions exist that AD only affects older people. Improved public education, especially for higher-risk groups, is needed to address misconceptions and promote social inclusion for those with dementia. Healthcare professionals can play a crucial role.

Transfusion-Associated Graft-Versus-Host Disease in Adults.

Transfusion-associated graft-versus-host disease (TA-GVHD) is a rare but fatal complication of blood transfusion that usually develops two to 30 days following a blood transfusion giving rise to graft versus host disease (GVHD) clinical features that are consisting of fever, skin rash, jaundice, diarrhea, and pancytopenia. The disease is fulminant in most patients with a mortality rate of >90% of cases. The main aim of this review is to enhance awareness among medical practitioners about this fatal disease. Data were extracted manually from the main medical databases (Medline, Scopus, and Google Scholar) after the revision of selected articles and assessed for their contribution to the knowledge of TA-GVHD. TA-GVHD occurs when the viable donor T-cells in the blood or blood products attack the recipient's tissues which his/her immune system is incapable to destroy due to several reasons. The recipient's tissues that are usually involved in TA-GVHD include the liver, intestine, skin, lungs, and bone marrow. Any blood component either whole blood, packed red blood cells (RBCs), platelets, or fresh non-frozen plasma that contains viable T lymphocytes can cause TA-GVHD. Host immunodeficiency, transfusion of fresh blood, and partial human leukocyte antigen (HLA) matching between the donors and the recipients represent the major risk factors of TA-GVHD. Partial HLA matching includes immunocompetent recipients who receive blood from a first-degree relative also, seen in genetically homogenous populations because of high rates of consanguineous marriage. The diagnosis of TA-GVHD is mainly suspected based on clinical manifestations. However, a histopathological study of either skin or rectal biopsy is diagnostic. The treatment of TA-GVHD is generally not effective, unless the patient received emergency stem cell transplantation, while prevention via irradiation of blood or blood products represents the standard of care for this disease. In conclusion, medical practitioners should have a high index of suspicion for this disease. Moreover, future clinical trials targeting and comparing the outcomes of the different therapeutic options for TA-GVHD are required.

Crystal-Storing Histiocytosis: The Iceberg of More Serious Conditions.

Crystal-storing histiocytosis is a rare condition that is histologically characterized by intracellular cytoplasmic crystalline inclusions. It usually presents monoclonal immunoglobulins that deposit within histiocytes, which accumulate and affect different organs of the human body and are commonly associated with lymphoproliferative conditions, especially those with plasmacytic differentiation. The prognosis of this condition is variable and related to the underlying clinical disease. In this review article, we aim to describe and discuss the clinical and pathological characteristics of crystal-storing histiocytosis based on the available literature and to provide a thorough differential diagnosis.

A rare case report of gemcitabine-induced thrombotic microangiopathies.

A 65-year-old female patient with breast cancer and soft tissue sarcoma who experienced a gemcitabine-induced thrombotic microangiopathies manifestation visited the emergency department. The renal biopsy revealed endothelial cell swelling, focal reduplication of glomerular basement membrane, and narrowed capillary lumens with fibrin deposition and fragmented erythrocytes which are compatible with thrombotic microangiopathies. The patient was presented with organ injury, acute renal failure with the need for hemodialysis technique. The patient recovered after the appropriate treatment. Continuous observation of renal function during gemcitabine treatment regularly and withdrawal of treatment if acute kidney injury occurs is essential as acute kidney injury along with thrombotic microangiopathies may prove to be life-threatening.