Articles: function.
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Sickle cell anemia (SCA) is a hereditary blood disorder characterized by the presence of abnormal hemoglobin, leading to the formation of sickle-shaped red blood cells. While much research has focused on the molecular and cellular mechanisms underlying the pathophysiology of SCA, recent attention has turned to the role of apoptosis, or programmed cell death, in the disease progression. This review aims to elucidate the intricate mechanisms of apoptosis in SCA patients and explore its implications in disease severity, complications, and potential therapeutic interventions. ⋯ Various factors, including oxidative stress, inflammation, and altered cell signaling pathways, converge to modulate the apoptotic response in SCA. Furthermore, the interaction between apoptotic cells and the vascular endothelium contributes to endothelial dysfunction, promoting the pathogenesis of vasculopathy and organ damage seen in SCA patients. In conclusion, unraveling the complexities of apoptosis in SCA provides valuable insights into the disease pathophysiology and offers novel avenues for therapeutic interventions.
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Review Case Reports
Adrenomyeloneuropathy manifesting as adrenal insufficiency and bilateral lower extremity spastic paraplegia: A case report and literature review.
Adrenomyeloneuropathy (AMN) is a variant type of X-linked adrenoleukodystrophy, and it is a genetic metabolic disease with strong clinical heterogeneity so that it is easily misdiagnosed and underdiagnosed. Moreover, most patients with AMN have an insidious clinical onset and slow progression. Familiarity with the pathogenesis, clinical features, diagnosis, and treatment of AMN can help identify the disease at an early stage. ⋯ The clinical manifestations of AMN are diverse. When patients with adrenocortical dysfunction complicated with progressive spastic paraplegia of lower limbs are involved, AMN should be highly suspected, and the determination of very long-chain fatty acids and genetic testing should be performed as soon as possible to confirm the diagnosis because early treatment can help prevent or delay the progression of the disease.
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Decompressive craniectomy (DC) is a well-established neurosurgical intervention in patients with high intracranial pressure who fail to respond to medical treatment. Data on predictive factors for functional outcomes in patients with DC who have malignant middle cerebral artery (MCA) infarction as opposed to intracranial hemorrhage (ICH) are scarce. Eighty-four patients who underwent DC treatment for ICH and malignant MCA infarction were examined. ⋯ The postsurgical midline shift (MLS) (ICH group) showed the strongest correlation (ρ = 0.434; P = .006), as in the MCA infarction group as well (ρ = 0.46; P = .005). Further analyses using binary logistic regression with postsurgical basal cistern status and ∆ MLS, and it was observed to be statistically significant (odds ratios: 0.067, 95% CI: 0.007, 0.67; P = .021). The initial Glasgow coma scale, postsurgical MLS, basal cistern status, and ∆ are Measurable variables that can be used to predict outcomes in the groups with ICH and MCA infarction.
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Cardiovascular diseases (CVDs) are a very common occurrence in patients with chronic kidney disease (CKD) and that was the main cause of mortality in these patients. The aims of the present study were to examine the effects of inflammation, malnutrition, and an oxidative stress in patients undergoing long-term hemodialysis or/and kidney transplant patients or patients with late-stage CKD, with its coherent consequences during a 38-month follow-up period. The present study included 137 patients with renal insufficiencies (48 patients had CKD, 29 patients had kidney transplants, and 60 CKD patients underwent hemodialysis [HD] and 39 normal individuals [controls]; aged 49 ± 20 years, 96 males and 80 females). ⋯ As the disease progressed, the survival function decreased to 30% due to the malnutrition in patients with CKD. Lipid oxidation and malnutrition/inflammation are associated with in various stages of CKD patients. With progressing CKD patients' biomarkers of lipid oxidation and malnutrition/inflammation show an increasing trend.