Abstract
Objective: To study the pulmonary complications of major sickle cell syndromes in children aged 6 months to 15 years followed at the Department of Pediatrics of Gabriel Toure University Hospital. Materials and methods: This was a retrospective study from January 1st, 2017 to February 28, 2018 and a prospective study from March 1, 2018 to December 31, 2019, concerning sickle cell disease children followed at the Gabriel Touré University Hospital. All children with sickle cell disease confirmed by hemoglobin electrophoresis, aged 6 months to 15 years with a pulmonary complication and admitted to pediatrics were included. Were not included: 1) Infants and children with sickle cell disease not presenting a pulmonary complication coming only to their routine follow-up. 2) Sickle cell patients over 16 years of age and those whose parents do not consent. 3) Those who have not done electrophoresis. Results: During the study period, we were able to include 45 sickle cell children with a pulmonary complication. The frequency of pulmonary complications was 13.76%, involving homozygotes in 91.11% of cases. The age group 6 - 10 years predominated with 46.66% and the sex ratio was 0.45. Vaso-occlusive crisis was the most frequent reason for consultation with 35.56%. Respiratory distress (80%), fever (66.67%), crepitus rales (64.44%) and chest pain (60%) were the most frequent clinical signs. The main pulmonary complication was acute chest syndrome with 86.67%. Treatment was based on hydration (91.91%), analgesics (91.91%) and antibiotics (73.33%). Lethality was 4.44%. Conclusion: Pulmonary complications of sickle cell disease are serious and constitute the main cause of mortality in our context.
Highlights
IntroductionIn Mali, according to Center of management and research of Sickle cell disease called “Le Centre de Recherche et de Lutte contre la Drépanocytose (CRLD)”, there are 5000 to 6000 sickle cell births each year with a prevalence of 12%, including 3% for the major form [3]
Every year, nearly 300,000 children are born with a major hemoglobin abnormality, including more than 200,000 cases in Africa [1] [2].In Mali, according to Center of management and research of Sickle cell disease called “Le Centre de Recherche et de Lutte contre la Drépanocytose (CRLD)”, there are 5000 to 6000 sickle cell births each year with a prevalence of 12%, including 3% for the major form [3]
All children with sickle cell disease confirmed by hemoglobin electrophoresis, aged 6 months to 15 years with a pulmonary complication and admitted to pediatrics were included
Summary
In Mali, according to Center of management and research of Sickle cell disease called “Le Centre de Recherche et de Lutte contre la Drépanocytose (CRLD)”, there are 5000 to 6000 sickle cell births each year with a prevalence of 12%, including 3% for the major form [3]. They are subject to complications that can affect all organs, which makes sickle cell disease a disabling disease, with a 5-year probability of hospitalization for painful vaso-occlusive crisis (VOC) of 63%, for acute chest syndrome (ACS) of 45%, and for acute anemia of 30% [4]. Its presence increases the risk of occurrence of vaso-occlusive attacks (VOC) and acute chest syndrome (ACS) [6] [7]
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