OBJECTIVE: The aim of this study was to determine the association between dental tissues and sickle cell anemia (SCA) and how it impacts the quality of life related to oral health.
METHODS: It was a cohort study of 154 Congolese participants with and without SCA conducted in the dental service of SCA at the Yolo Center, Kinshasa, aged at least 6 years and without a history of clinically severe conditions (hospitalization and blood transfusion), who were regularly monitored. The inclusion criteria were the diagnosis confirmation of SCA at the health service in a period of at least 6 months before enrollment in this study. Dental tissues were assessed by a clinical examination using a dental mirror and probe. The index of Decayed-Missing-Filled Teeth (DMFT) was used to assess the dental state of the participants. For Oral Health-related Quality of Life (OHrQoL), the Congolese versions of the perception questionnaires, modified from the Oral Health Impacts Profile (OHIP-23), were used for participants. Each question had to be answered by yes or no, depending on whether the participant was satisfied (outcome = 1) or dissatisfied (outcome = 0) about an oral health-related quality of life.
RESULTS: Of the 154 participants, aged from 6 to 64 years, with a mean age of 19.5 ± 7 (SD) years, 96 presented with SCA and only 68 were correctly followed; 102 did not present SCA and only 86 were correctly followed. The DMFT and dmft indexes were higher in the SCA group, being 2.9 and 2.5, respectively. The difference between the SCA group and the control group was significant for decayed teeth, missing teeth, filled teeth and no caries. Of the different dimensions of quality of life that were compared between the SCA group and control group, 15 of 23 items were statistically significant.
CONCLUSIONS: The present study strongly confirmed an association between dental caries and missing teeth with sickle cell anemia. Secondly, the quality of life for SCA participants seems to be poor, compared to the control group.

Hemoglobin (Hb) abnormalities, such as thalassemia and structural Hb variants, are among the most prevalent inherited diseases and are associated with significant mortality and morbidity worldwide. However, there were not comprehensive reviews focusing on different clinical analytical techniques, research methods and artificial intelligence (AI) used in clinical screening and research on hemoglobinopathies. Hence the review offers a comprehensive summary of recent advancements and breakthroughs in the detection of aberrant Hbs, research methods and AI uses as well as the present restrictions anddifficulties in hemoglobinopathies. Recent advances in cation exchange high performance liquid chromatography (HPLC), capillary zone electrophoresis (CZE), isoelectric focusing (IEF), flow cytometry, mass spectrometry (MS) and polymerase chain reaction (PCR) etc have allowed for the definitive detection by using advanced AIand portable point of care tests (POCT) integrating with smartphone microscopic classification, machine learning (ML) model, complete blood counts (CBC), imaging-based method, speedy immunoassay, and electrochemical-, microfluidic- and sensing-related platforms. In addition, to confirm and validate unidentified and novel Hbs, highly specialized genetic based techniques like PCR, reverse transcribed (RT)-PCR, DNA microarray, sequencing of genomic DNA, and sequencing of RT-PCR amplified globin cDNA of the gene of interest have been used. Hence, adequate utilization and improvement of available diagnostic and screening technologies are important for the control and management of hemoglobinopathies.

Although risk factors such as cholelithiasis and vaso-occlusive crises exist in sickle cell disease, acute pancreatitis and its recurrence are considered rare complications manifesting as acute abdomen.
UNASSIGNED: A 33-year-old female with sickle cell disease and established cholelithiasis presented to the center with acute abdomen. After examination, investigation, and contrast enhanced computed tomography, acute pancreatitis was diagnosed. Conservative management was done and cholecystectomy was planned but delayed due to low hemoglobin. In the interval she presented again with similar features and diagnosed with of recurrence. After conservative management and after optimization of patient\'s hemodynamic status, laparoscopic cholecystectomy was finally performed. Postoperative period and follow-up visit after 2 months were uneventful.
UNASSIGNED: As the features of pancreatitis and vaso-occlusive crisis are similar, the former should be considered as a differential diagnosis of acute abdomen in sickle cell patients. Laparoscopic cholecystectomy is the treatment of choice for symptomatic cholelithiasis in these patients. However, some issues related to management such as preoperative transfusion and prophylactic cholecystectomy are still debated. This uncertainty caused delay in surgery which may have contributed to the recurrence of pancreatitis in our patient. As the risk of recurrence is possible after the first attack, standard guidelines are required for the definite management of the cause.
UNASSIGNED: This case report adds to the limited literature on recurrent acute pancreatitis in sickle cell patients and points out the need for studies on developing management guidelines in such patients and need for prophylactic treatment.

To define morphological changes in carotid and cerebral arteries in sickle cell transgenic mice (SS) as they age, a combination of ultrasound and microcomputed tomography of plastinated arteries was used to quantify arterial dimensions and changes in mice 4, 12, and 24 weeks of age. 12-week SS mice had significantly larger common carotid artery diameters than AS mice, which continued through to the extracranial and intracranial portions of the internal carotid artery (ICA). There were also side specific differences in diameters between the left and right vessels. Significant ICA tapering along its length occurred by 12- and 24-weeks in SS mice, decreasing by as much as 70%. Significant narrowing along the length was also measured in SS anterior cerebral arteries at 12- and 24-weeks, but not AS. Collectively, these findings indicate that sickle cell anemia induces arterial remodeling in 12- and 24-weeks old mice. Catalog of measurements are also provided for the common carotid, internal carotid, anterior cerebral, and middle cerebral arteries for AS and SS genotypes, as a reference for other investigators using mathematical and computational models of age-dependent arterial complications caused by sickle cell anemia.

Objectives: Although dental caries has been widely reported in individuals with sickle cell disease (SCD), there is still controversial in the literature regarding the association between SCD and dental caries. The aim of this systematic review was to investigate whether individuals with SCD have more dental caries than individuals with non-SCD. Methods: PubMed and Embase databases were searched for eligible studies. The parameters of the permanent decayed, missing and filled teeth (DMFT) index and the permanent decayed, missing and filled surface (DMFS) index were considered as outcome measures. The overall meta-analyses of the DMFT and DMFS index and various subgroup analyses (caries components, age, and genotypes) of DMFT index were performed to calculate the weighted mean differences (WMD) between patients with SCD and non-SCD individuals. Results: A total of 9 studies covering 1478 individuals were included in this meta-analysis. The results of overall meta-analyses indicated that the scores of the DMFT and DMFS index were not significantly different between patients with SCD and non-SCD participants. The results of subgroup analyses by caries components, age, and genotypes showed no significant difference in most items. The result of the missing teeth was significantly lower in patients with SCD than in non-SCD individuals (WMD, -0.14; 95% confidence interval [CI], -0.25 to -0.03; P = 0.01). Discussion and Conclusions: The results revealed that compared with non-SCD individuals, patients with SCD did not suffer from worse dental caries. Considering the limitations, further well-designed studies are necessary to reveal the association between SCD and dental caries.