Background: Sickle cell disease (SCD) is a life-threatening genetic disorder due to the formation of sickle hemoglobin molecule (HbS) that polymerizes in hypoxic conditions leading to SCD-related complications. Different approaches have been used in the management of SCD including symptomatic management, supportive management, and preventive management.
Objectives: To assess the management of SCD in pediatric patients in Gaafar Ibnauf Referral Hospital in Khartoum locality, Sudan.
Method: A descriptive, retrospective, hospital-based study was conducted in Gaafar Ibnauf Hospital using a data collection sheet. The study included all medical files of pediatric patients with SCD attending the hospital during the period from the first of April 2018 to the first of July 2018. The data were analyzed using descriptive statistics and the chi-square test. P < 0.05 was considered statistically significant.
Results: Out of 207 pediatric patients, 53.1% were females (mean age of 7.5 ± 3.1 years), with a 1.1 : 1 female:male ratio and low socioeconomic status. Only 4.3% of participants had health insurance. The Messeryia tribe in western Sudan had the highest prevalence of the disease among the Sudanese tribes (11.1%). Vaso-occlusive crisis (33.3%), infections (13.5%), and neurological complications (10.6%) were the most frequent complications reported during routine visits. After initiation of management, only 3.4% of pediatric patients had hemolytic crises, and 1.4% of the anemic patients had splenomegaly. 100% of patients received folic acid, 73.9% used hydroxyurea, and 69.6% underwent blood transfusion for the management of SCD. Prophylactic penicillin was prescribed for 15% of patients, and 41.1% were immunized with pneumococcal vaccine (PPSV23). Most patients had been scheduled for planned follow-up visits every 3-6 months (93.2%). Hydroxyurea and blood transfusion significantly reduced fever and vaso-occlusive crisis.
Conclusion: The SCD treatment protocol in Gaafar Ibnauf Children's Hospital, involving preventive and symptomatic therapy, is consistent with the internationally implemented protocols for SCD management. However, immunization and prophylactic penicillin approaches are deficient.
Introduction: Sickle cell disease is an autosomal recessive inherited disorder due to the mutation of a gene coding for the globin beta chain. The aim of this study is to update the epidemiological data on hemoglobinoses, in particular sickle cell disease in newborns in Congo.
Materials and methods: This was a descriptive cross-sectional study, conducted from October 1, 2019, to March 31, 2020, throughout the Congolese national territory. It involved all full-term newborns, without distinction of nationality, aged 5 days or less, and whose parents consented to participate in the study. The blood samples, taken at the heel and collected on Whatman blotting paper, were analyzed using the HPLC Variant NBS machine.
Results: In 2897 newborns (NN) screened, hemoglobin abnormalities were found in 603 NN (20.81%). The mean age of these newborns was 1 day (extremes 0 and 5 days). The male-to-female ratio was 1.03. Abnormal hemoglobins were mainly Hb S (n = 597 (97.71%)); Hb C (n = 5 (0.82%)); and variants (n = 7 (1.15%)). The national prevalence of major sickle cell (MSC) syndromes and sickle cell trait was 1.35% and 19.43%, respectively. The prevalence ranged from 1.77% to 2.56% for MSS in four departments and from 20.5% to 25.8% for the sickle cell trait in six other departments.
Conclusion: Data on homozygous sickle cell disease remain consistent with previous studies. However, further studies should clarify the molecular anomalies of the variants observed in our samples.
Aims: Sickle cell disease (SCD) is an upcoming global health problem with rapid progress in therapy especially since 2017. However, systematic reviews found no clinical trials on the dental treatment of sickle cell disease (SCD). This article aims to outline the oral features of the sickle disease and discuss oral management strategies that can serve as guidelines for dental professionals. Material and Methods. A comprehensive literature review was conducted using PubMed, Google Scholar, and Web of Science. The search strategies were developed to cover publications from January 2010 to March 2020. With the help of keywords, multiple abstracts were identified. These abstracts were further reviewed, which included the information about the SCD manifestation, particularly about the oral health features. Based on all these articles and clinical experience, a narrative review was constructed, which summarizes all the aspects of the oral manifestation in people with SCD.
Results: The results of this study demonstrate that there is distinct evidence available, indicating the developmental enamel defect leading to hypoplasia and increasing susceptibility to dental caries. Another important result of this review found that people with SCD have a vaso-occlusive crisis in the microcirculation in the dental pulp leading to symptomatic and asymptomatic pulpal necrosis without any signs of odontogenic pathology in an apparently healthy tooth. The study also found that early detection, intervention, and prevention are crucial for improving oral health care, and involving a multidisciplinary approach plays an important role in managing people with SCD.
Conclusion: Patients with sickle cell disease have chronic overall health problems. The hematological disorder becomes their main concern and impaired oral health becomes secondary, increasing the risk for dental caries at the most. This paper broadly describes the oral manifestations of SCD, additionally; this paper also provides recommendations for better dental management of patients with SCD. Patients with SCD are often misjudged and, due to lack of knowledge and guidelines, dental providers are not able to provide adequate care. This paper attempts to highlight the essential measures to provide better dental care.
Background: Thalassemia is a hereditary disease, and severe anemia is the main phenotype of major thalassemia. Furthermore, the most important method in the management of this disease is red blood cell transfusion. Regular transfusions administered 1 or 2 times every month improve prognosis and survival. However, there is higher risk of infections and iron overload, especially in transfusion-dependent thalassemia (TDT). Infections are the second leading cause of death in adult TDT, after heart failure. Higher risk of infection is also influenced by multiple blood transfusions which causes alteration in immune response due to alloimmunization, transfusion-related infections, and iron overload. Meanwhile, iron overload in TDT alters both innate and specific immune responses. Furthermore, previous studies have shown the correlation between ferritin with CD4, but this has not been carried out in Indonesia. Therefore, this study aims to determine the correlations between iron overload (serum ferritin and transferrin saturation) and specific immune cells (CD4).
Methods: This is a cross-sectional study, and a total number of 64 subjects were examined consecutively. Chest X-ray and blood sera were obtained. The total number of subjects was 64. The seromarkers HBsAg, anti-HCV, and anti-HIV were tested using the ELISA method. Serum ferritin and transferrin saturation was tested using ECLIA, and lymphocyte subsets were analyzed using flowcytometry. Meanwhile, the correlation between variables was determined using Spearman's test.
Results: The results showed that 4.9% subjects were HBsAg positive, 10.7% were anti-HCV positive, and none were anti-HIV positive. There were 4 subjects with lung tuberculosis based on the 41 chest X-ray. Meanwhile, there was a weak negative and insignificant correlation between serum ferritin with CD4 (p=0.75; r = -0.04) and a weak positive and insignificant correlation between transferrin saturation with CD4 (p=0.133; r = 0.19).
Conclusion: There were no correlations between iron overload (ferritin) and cellular immunity (CD4) in adult transfusion-dependent thalassemia.
Methods: In the current study, we investigated the morphological differences, proliferation capacity, population doubling time (PDT), surface marker profiling, trilineage differentiation potential, and immunosuppressive ability of BM Mesenchymal Stem Cells (BM-MSCs) from untreated aAA patients and in the same number of age- and gender-matched controls.
Results: We observed similar morphology, proliferation capacity, phenotype, trilineage differentiation potential, and immunomodulatory properties of BM-MSCs in aAA patients and control subjects.
Conclusion: Our results confirm that the basic and immunosuppressive properties of BM-MSCs from aAA patients do not differ from normal BM-MSCs. Our data suggest that BM-MSCs from aAA patients might not be involved in disease pathogenesis. However, owing to a smaller number of samples, it is not conclusive, and future studies with more exhaustive investigation at transcriptome level are warranted.
Thalassemia is a genetic disease caused by disruption of globin chain synthesis leading to severe anemia and thus regular blood transfusion is necessary. However, there have been known transfusions-related consequences, including iron overload and multi-organ damage. The aims of this study were to evaluate liver and cardiac function in youth and adult transfusion-dependent Indonesian thalassemic patients and to assess its correlation with serum ferritin levels, as well as T2 ∗ magnetic resonance imaging (MRI). Transfusion-dependent thalassemic (TDT) outpatients (n = 66; mean age, 21.5 ± 7.2 years) were carried out for the complete assessment consisting of blood test including liver enzyme and serum ferritin, followed by electrocardiography (ECG) and echocardiography. Subjects were also divided by serum ferritin levels into three groups: < 2500 ng/mL, 2500-5000 ng/mL, and >5000 ng/mL. Additionally, subgroup analysis in patients with T2∗ MRI assessment was conducted. In terms of age of first blood transfusion, subjects with ferritin >5000 ng/mL were the youngest among others. The alanine aminotransferase (ALT) levels in group with serum ferritin >5000 ng/mL were significantly higher than those of the group with serum ferritin <2500 ng/mL. Additionally, youth and adult TDT patients whose serum ferritin >5000 ng/mL had significantly lower tricuspid annular plane systolic excursion (TAPSE) when compared with those who had serum ferritin <2500 ng/mL. Similarly, TAPSE in patients with moderate cardiac siderosis based on cardiac T2∗ MRI was significantly lower than those without cardiac siderosis. There was significant, but only moderate correlation between serum ferritin and cardiac T2∗ MRI. Based on these findings, it is important to routinely monitor iron accumulation-related complications, including liver and cardiac damage in youth and adult TDT patients.
Background: Anaemia, a common nutritional deficiency, is a public health problem in the Mount Cameroon area. This study determined the prevalence and possible risk factors of microcytic and malarial anaemia in children less than ≤15 years residing in the Buea and Limbe municipalities in the Mount Cameroon area.
Methods: A total of 566 children were clinically examined in a cross-sectional study from December 2018 to August 2019 for anaemia and malaria parasites. Blood samples collected were used in evaluating full blood count with the aid of an automated haemoanalyser, and malaria parasite was confirmed by microscopy. Anaemia was defined based on WHO standards while microcytic anaemia and malarial anaemia were defined as microcytosis + anaemia and malaria + anaemia, respectively. Factors that showed significance in the bivariate analysis were entered into a multinomial logistic regression to determine risk factors for microcytic and malarial anaemia.
Results: The overall prevalence for anaemia, microcytosis, microcytic anaemia, and malarial anaemia was, respectively, 68.7%, 48.9%, 36.9%, and 19.6% with microcytic anaemia representing 53.7% of all anaemic cases. Risk factors for microcytic anaemia included child age of 1-5 years (P=0.007), forest ethnicity (P=0.019), parents being farmers (P=0.038) or jobless (P=0.009), and having moderate malaria parasitaemia (P=0.048) while those for malarial anaemia were child age of 6-10 years (P=0.008), parents' age of 26-35 years (P=0.049), parents being jobless (P=0.023), and consuming plantains 3-4 times (P=0.024) a week.
Conclusion: Microcytic anaemia is getting to be a severe public health concern while malarial anaemia is a mild public health issue in children residing in urban areas of Mount Cameroon. Parents' occupation was directly linked to all anaemia forms; hence, any intervention to curb anaemia should consider aspects that will raise the socioeconomic status of the population.

