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Outpatient Management of Sickle Cell Disease: Assessment of 13 Years Follow-Up of Pediatric Patients at the Sylvanus Olympio University Hospital

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Open Journal of Pediatrics 2022年第2期12卷 413-423页

作者： Koffi Mawuse Guedenon Djatougbe Ayaovi Elie Akolly essohana Padaro Ounoo Elom Takassi Etsè Akpako balakibawi esso Kokou Dzata Adama Dodji Gbadoe Department of Pediatrics CHU Sylvanus Olympio University of Lomé Lomé Togo Department of Hematology CHU Campus University of Lomé LoméTogo University of Maryland Medical Systems/Midtown Campus Baltimore MD USA

Objective: To evaluate thirteen years of ambulatory follow-up of patients with major forms of sickle cell disease. Methods: This was a study of 1055 records of sickle cell patients aged 6 months to 45 years followed between 2006 and 2018. Results: Six hundred and eighty-one (64.5%) homozygous SS, 283 (26.8%) SC, 86 (8.2%) SF, 4 (0.4%) S? thalassemia and 1 (0.1%) SD sickle cell patients were followed. The majority of the patients (97.9%) were living in the capital city of Lomé and its surrounding suburbs. Most children (67.3%) were less than 5-year-old when they were diagnosed with sickle cell disease, and only 21.3% of cases were discovered before the first year birthday of the patients. Osteo-articular vaso occlusive crisis (VOC) was the principal symptom at the onset of the diagnosis, approximately 28.9% of the cases, followed by severe anemia (19%), then hand-foot syndrome in 15.6%. The systematic screening was performed in 5.4%. Twenty-six percent were G6PD deficient, 4 were HIV immunocompromised, and 29.7% were transfused. Among the degenerative complications, 2.4% had retinopathy, 1.9% osteonecrosis of the femoral head, 1.4% leg ulcers, and 0.8% cardiomyopathy. Salmonella typhi was the main cause of infection, 51.4% of the cases. The mean baseline hemoglobin level (n = 27) was 7.67 ± 1.64 g/dl. Four (0.4%) patients had a splenectomy. The mortality rate was 2.4%. The primary cause of death was anemia (28%), followed by sepsis in 20% of the cases. One case of suicide was documented as the cause of death. Conclusion: Outpatient medical follow-up of sickle cell patients is essential in sub-Saharan African countries where neonatal screening does not exist, and diagnosis is often late. The improvement in quality of life observed should be studied.

关键词： Sickle Cell Disease Outpatient Follow-Up Lomé

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