The enigma of sickle cell hepatopathy: Pathophysiology, clinical manifestations and therapy.

Sickle cell disease (SCD) is one of the most common genetic disorders in the world predominantly affecting economically disadvantaged populations. There is a notable discrepancy between the growing adult SCD population and available diagnostic and therapeutic interventions for SCD. Sickle cell hepatopathy (SCH) is an all-inclusive term to describe the acute and chronic liver manifestations of SCD. The pathophysiology of SCH follows no defined pattern or sequence that poses challenges to clinicians and researchers alike. Evidence is lacking for this underreported disease at various levels from diagnostic to therapeutic options. This paper reviews the basic pathophysiology, clinical features, biochemical and radiological findings of various SCH manifestations and outlines the management of each condition. Old and new therapy options in SCD including hydroxyurea, red blood cell exchange transfusion, ursodeoxycholic acid, voxelotor, l-glutamine and crizanlizumab have been reviewed to investigate the role of these options in treating SCH. The role of liver transplant, haematopoietic stem cell transplant and gene therapy in SCH patients have been reviewed.

Effect of vitamin A on maternal, fetal, and neonatal outcomes: An overview of deficiency, excessive intake, and intake recommendations.

Vitamin A imbalance during pregnancy and lactation is a global public health concern with potentially negative consequences for fetuses and neonates. Inadequate vitamin A intake during this critical period can lead to anemia, weakened immune function, night blindness, and increased susceptibility to infections. Conversely, excessive intake of vitamin A can result in birth defects, hypercalcemia, and psychiatric symptoms. This review aims to identify risk factors contributing to vitamin A deficiency in pregnant women and its impact on maternal, fetal, and neonatal outcomes. It also examines the effects of high-dose vitamin A supplementation during pregnancy on offspring health. By analyzing existing literature and recommendations, the review emphasizes the significance of vitamin A in the development of various body systems and organs. It provides a comprehensive overview of the effects of vitamin A during pregnancy and lactation, encompassing deficiencies, excessive intake, and supplementation guidelines. The need for further research in this field is highlighted. In conclusion, maintaining a balanced vitamin A status is crucial during pregnancy to promote better outcomes for fetuses and newborns. Effective monitoring and intervention strategies are essential to address vitamin A deficiency and excess in pregnant women, thereby improving fetal and neonatal health.

Prevalence of Acute Coronary Syndrome and Various Risk Factors in Acute Stroke Patients.

Background Stroke is the second leading cause of death worldwide after acute coronary syndrome (ACS). Both diseases share many risk factors such as hypertension, diabetes, dyslipidemia, and smoking. Patients who experience acute coronary syndrome are at heightened risk of recurrent ischemic events such as ischemic strokes, one of the most feared cardiovascular events because of the risk of long-term disability. We tried to estimate the prevalence of underlying ACS among patients with acute stroke. Methods This cross-sectional study was done at the CPE Institute of Cardiology, Pakistan, and Nishtar Medical University and Hospital, Pakistan. A total of 160 acute stroke cases were selected by consecutive sampling technique and questionnaire forms were filled. Detailed history, investigations, and physical examinations were done. The primary outcome was the prevalence of ACS and secondary outcomes were the prevalence of hypertension, smoking, dyslipidemia, diabetes mellitus, and previous history of stroke in stroke patients. Results Most of the patients that presented to us were above 50 years of age with the mean age of 62 years (SD = 9.23 years). Male predominance was seen with a total of 115 (72%) cases. Out of 160 patients, 91 (57%, p < 0.05) had underlying ACS, with 45 cases (49%) with unstable angina, 20 (22%) with non-ST-elevation myocardial infarction (NSTEMI), and 26 (29%) with ST-elevation myocardial infarction (STEMI). Prevalence of risk factors in 160 cases were, hypertension (101, 63%), lack of exercise (91, 57%), smoking (70, 44%), diabetes mellitus (61, 38%), dyslipidemia (50, 31%). All these results were statistically significant (p < 0.05). Prevalence of obesity (35, 22%) and previous stroke history (19, 12%) was statistically insignificant (p > 0.05). Conclusion Acute coronary syndrome is still frequently present in patients with acute stroke. The need of the hour is to manage ACS efficiently so that its deadly complications such as stroke can be prevented.