Background: This study investigated the impact of COVID-19 on patients with sickle cell crisis (SCC) using National Inpatient Sample (NIS) data for the year 2020. Methods: A retrospective cohort analysis was conducted utilizing International Classification of Diseases (ICD-10) codes to identify adults who were admitted with a principal diagnosis of sickle cell crisis. The primary outcomes examined were inpatient mortality, while the secondary outcomes assessed included morbidity, hospital length of stay, and resource utilization. Analyses were conducted with STATA. Multivariate logistic and linear regression analyses were used to adjust for confounding variables. Results: Of 66,415 adult patients with a primary SCC diagnosis, 875 were identified with a secondary diagnosis of COVID-19 infection. Unadjusted mortality rate was higher for SCC patients with COVID-19 (2.28%) compared to those without (0.33%), with an adjusted odds ratio (aOR) of 8.49 (p = 0.001). They also showed increased odds of developing acute respiratory failure (aOR = 2.37, p = 0.003) and acute kidney injury requiring dialysis (aOR = 8.66, p = 0.034). Additionally, these patients had longer hospital stays by an adjusted mean of 3.30 days (p < 0.001) and incurred higher hospitalization charges by an adjusted mean of USD 35,578 (p = 0.005). Conclusions: The SCC patients with COVID-19 presented higher mortality rates, increased morbidity indicators, longer hospital stays, and substantial economic burdens.

The management of acute chest syndrome (ACS) in sickle cell disease occurring concurrently with pulmonary embolism resulting from tricuspid valve endocarditis poses an atypical challenge. We present a case in which this complex interaction occurs and the prompt interventions that were utilized to give the best possible outcome.

Introduction Sickle cell anemia (SCA), a severe hematological disorder, is characterized by the presence of sickle-shaped erythrocytes that obstruct capillaries and restrict blood flow. This pathophysiology not only promotes systemic complications but may also influence cardiac function. Cardiac complications are a leading cause of mortality in SCA patients, yet the specific electrocardiographic (ECG) changes associated with disease severity are not thoroughly understood. This cross-sectional study aimed to explore ECG abnormalities in adults with SCA and correlate these findings with disease severity. Methods An observational cross-sectional study was conducted over 18 months, from January 2022 to June 2023, among 140 SCA patients at the Sickle Cell OPD of All India Institute of Medical Sciences, Raipur, Raipur, India. Steady-state SCA (HbS >50%) patients screened by high-performance liquid chromatography were enrolled. A history, physical examination, complete blood count, and ECG were done for all cases. The disease severity score was calculated using the Adegoke and Kuti severity scores, and their association with various ECG changes was studied. The chi-square test (Fisher\'s exact test, wherever applicable) was used for comparing the proportion. The correlation was done using the Pearson correlation coefficient or Spearman\'s rho. Results Out of 140 patients, the mean age of the study participants was 26 ± 6 years. More than half of the cases (80; 57%) fall under the 18-27 age group, with a male-to-female ratio of 4:3. A total of 99 (70.7%) of the participants had mild disease, and 41 (29.3%) had moderate disease. The QT interval was significantly higher among patients with mild disease compared to those with moderate disease (p-value: <0.01). QTc dispersion and prolonged QTc interval were significantly higher among patients with moderate disease compared to mild disease (p-value <0.01, 0.04, respectively). Sinus tachycardia and right ventricular hypertrophy with p-pulmonale were significantly higher in moderate severity (p < 0.01). A significant positive correlation was observed between QTc dispersion, P-wave dispersion, and severity (r: 0.19, 0.17; p-value: 0.02, 0.04, respectively). Conclusion As the disease severity progressed, the ECG changes studied had a higher distribution and significance. ECG is a readily and widely accessible investigation that can be used to screen all SCA patients for early recognition of various underlying cardiac complications.

It is known that an inherited blood condition called sickle cell disease (SCD) is a result of one gene. A number of blood and urine biomarkers have been determined in association with lab and clinical history for SCD patients. SCD has numerous interacting pathways associated with it, which have been identified by biomarkers. These mechanisms consist of some examples, such as endothelial vasodilation response, hypercoagulability, hemolysis, inflammation, oxidative stress, vascular dysfunction, and reperfusion injury among others. To effectively manage SCD, a comprehensive panel of validated blood and urine biomarkers must be established. Despite its monogenic inheritance, the complex nature of the SCD phenotype has impeded progress in its treatment. However, significant strides have been made in clinical biotechnology, paving the way for potential breakthroughs. In SCD, a panel of verified blood and urine biomarkers must be established, however. Despite monogenic inheritance, the great complexity of the SCD phenotype has hindered progress in its management. With few exceptions, clinical biomarkers of illness severity have been found through epidemiological investigations; nevertheless, systematic integration of these biomarkers into clinical treatment algorithms has not occurred. Furthermore, sickle cell crisis, the primary acute consequence of SCD, has been difficult to diagnose with the biomarkers now in use. Inadequate care and a lack of appropriate outcome measures for clinical research are the consequences of these diagnostic constraints. A new chapter in SCD customized treatment has begun with recent advancements in molecular and imaging diagnostics. Strategies in precision medicine are especially relevant now that molecular therapies are within reach. The significance of biochemical indicators linked to clinical manifestation and sub-phenotype identification in SCD is reviewed in this research.

Acute painful vaso-occlusive crisis (VOC) is the common presentation of sickle cell disease (SCD) leading to emergency room visits, admissions, morbidity, mortality, and negative impacts on quality of life. Among various treatment approaches commonly employed to manage the condition, intravenous (IV) hydration is also frequently used in emergency and inpatient settings. Although helpful to overcome dehydration, IV hydration often leads to adverse outcomes like fluid overload, pulmonary edema, increased length of stay, transfer to intensive care unit, new oxygen requirement, etc. Small-scale retrospective studies are conducted to study the outcomes of IV hydration but have failed to conclusively demonstrate its benefits as well as choice of IV fluids, rate of IV fluid replacement, etc. We conduct this review as an attempt to summarize the available evidence on the role and utility of IV hydration in sickle cell crises along with reported adverse outcomes.

We present the case of a 19-year-old male with a history of sickle cell anemia who presented to the hospital with worsening lower extremity pain. Given his acute presentation and history of recurrent pain crises, he was admitted to the hospital for management of a suspected acute pain crisis. However, due to continued pain, imaging was obtained which revealed a different diagnosis for the cause of his symptoms. MRI of the left lower leg revealed heterogenous T1 and T2 hyperintense signals within the proximal tibial diaphysis measuring 6.6 × 1.6 × 2.2 cm with a thick rim of peripheral irregular enhancement with surrounding periosteal reaction and soft tissue edema, concerning for osteomyelitis and developing Brodie\'s abscess. The patient underwent tibia irrigation and debridement with the placement of vancomycin and tobramycin beads. Perioperatively, no purulence was noted within the soft tissues, and no organisms were grown on tissue cultures. The patient\'s pain improved and he was discharged home with a plan to complete six weeks of intravenous antibiotics. This case represents the need to differentiate Brodie\'s abscess from a sickle cell crisis. Clinicians should also be aware that patients with sick cell disease are prone to Brodie\'s abscess and it should be a differential for symptoms of relenting bone pain.

Red cell exchanges (RCE) help in the treatment of complications of sickle cell anemia (SCA) by reducing the viscosity of blood and improving the oxygen-carrying capacity. We present a case of sickle cell crisis (SCC) managed with automated RCE and also reviewed the literature to assess the utilization and clinical efficiency of this therapy in India. A 19-year-old gentleman diagnosed with SCA presented with acute chest syndrome. Hemoglobin (Hb) was 8.8 g%, hematocrit (HCT) was 24%, and HbS was 90%. As there was worsening of symptoms with conventional management, the patient underwent two procedures of automated RCE. The clinical condition of the patient was improved, HbS was reduced to 16% and HCT was remained at 21% postprocedure. Articles on automated RCE in SCA conducted in India were reviewed and four articles were analyzed based on the search strategy. All the included articles concluded automated RCE as an effective procedure for complications of SCA. Common indication in India was SCA patients undergoing surgery as a prophylactic measure. Automated RCEs are promising as an acute treatment for indicated sickle cell complications. This therapy is underutilized in the Indian scenario, especially in patients with SCC.

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Sickle cell disease (SCD) is the most common hereditary hemoglobinopathy worldwide. It results in characteristic acute and chronic findings on postmortem computed tomography (PMCT), macroscopic and microscopic examinations. While the diagnostic imaging and macroscopic features are not specific for SCD on their own, when coupled with microscopic features such as sickled erythrocytes and evidence of chronic venous congestion (i.e., Gamna-Gandy bodies), these clues can help alert forensic pathologists to the presence of SCD. Despite the prevalence of the disease and the constellation of findings alluded to above, SCD is not often explored in forensic pathology literature. This case demonstrates classic acute and chronic features of SCD on PMCT, macroscopic and microscopic examinations. It explores the pathophysiology leading to sudden and unexpected death in a person with SCD and possible pitfalls in attribution of cause of death.

Sickle Cell Disease is an inherited autosomal recessive hemoglobinopathy associated with multiorgan damage. This single gene disorder involves one DNA base pair alteration, producing HbS. The sickle-shaped cells form when deoxygenated in the capillaries. The resulting RBC stasis leads to ischemia and pain, and acute and chronic organ damage. Patients with SCD presenting to a dental office need careful examination to rule out any current infections, neurologic deficits, or other organ involvement before formulating a dental treatment plan to avoid prolonged and complicated procedures. Early intervention and dental anxiety management are key to the dental treatment of patients with SCD.