Pediatric Sickle Cell Disease in Sudan: Complications and Management.

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.

Clinical manifestations, complications, and outcomes of patients with COVID-19 in Sudan: a multicenter observational study.

BACKGROUND: Coronavirus disease 2019 (COVID-19) is a pandemic caused by a newly discovered coronavirus. Although clinical manifestations of COVID-19 are mainly pulmonary, some patients have other systemic manifestations. This study aimed to describe the clinical finding and outcomes in Sudanese patients diagnosed with COVID-19. METHODS: This retrospective observational study is based on documented files that included patients diagnosed with COVID-19 in seven selected hospitals inside Khartoum. Clinical manifestations, complications and outcomes were extracted from patients' records using an extraction form designed for this study. RESULTS: Data of 243 patients diagnosed with COVID-19 were analyzed. The mean (SD) age in years was 55.8 (18.4). Out of 116 participants, 27 of them (23.3%) had severe disease, 15 (12.9%) were critically ill. 67.5% of patients were admitted to the hospital within 7 days from onset of symptoms; most of them were admitted to the wards (n = 140,72.5%). Fever (83.2%), cough (70.7%), and shortness of breath (69.2%) were the most commonly recorded clinical manifestations. Sepsis (9.8%) and acidosis (7.8%) were the most frequently reported complications. Death was the final outcome in 21.4% (56/243). Older age and presence of diabetes were found significantly associated with in-hospital death. The laboratory results showed high CRP in 85.6% (119/139), high ferritin in 88.9% (24/27), lactate dehydrogenase had a median of 409.0 (359-760), D-dimer had a median of 3.3 (1.2-16. 6), and 53/105 (50.5%) had low albumin. CONCLUSIONS: Fever was the most mentioned sign among the participants, followed by fatigue. Cough and shortness of breath were the most commonly recorded pulmonary symptoms manifested. Our study showed multiple variables were associated with in-hospital death. The mortality rate was high among severe and critically ill patients diagnosed with COVID-19.

Optimisation of T2 and T2* sequences in MRI for better quantification of iron on transfused dependent sickle cell patients.

This work aimed to investigate the effect of different shim techniques, voxel sizes, and repetition time (TR) on using theT2 and T2* sequences to determine their optimum settings to investigate the quantification of iron in transfused dependent sickle cell patients. The effect of each of these parameters was investigated on phantoms of different Gadolinium (Gd) concentrations, on 10 volunteers and 25 patients using a1 5T MRI Philips scanner. No significant difference between the three shim techniques was noticed in either T2 or T2* sequence measurements. Pixel sizes of 1 × 1 and 2 × 2 mm provided optimum results for T2 measurements. At 1 × 1 mm pixel size the T2* measurements experienced less error in measurements than the size of 2.5 × 2.5 mm used in the literature. Even though the slice thickness variation did not provide any changes in T2 measurements, the 12 mm provided optimum T2* measurements. TR variation did not yield significant changes on either T2 or T2* measurements. These results indicate that both T2 and T2* sequences can be further improved by providing more reliable measurements and reducing acquisition time.