Abstract

Introduction: Acute thoracic syndrome is the appearance of a new pulmonary infiltrate on radiology associated with fever, desaturation or respiratory signs. It is the second leading cause of hospitalization and the first cause of death in sickle cell patients. It is an acute pulmonary complication whose pathophysiological mechanisms are still poorly understood. This study aims to study the epidemiological, clinical, therapeutic and evolutionary aspects of Acute Chest Syndrome in children at the Pediatrics Department of Donka National Hospital. Method: This is a prospective study of descriptive type for a period of 6 months from February 19 to August 19, 2019 on patients with sickle cell disease who developed an ATS in the pediatrics department of Donka National Hospital. Epidemiological, clinical, therapeutic and evolutionary data were studied and proportionate data were calculated. Results: The frequency of ATS was 39%. The mean age of our patients was 9.83 years with the extremes of 4 and 16 years. The age group from 6 to 10 years with a frequency of 66.7% was the most affected. Fever was the main clinical manifestation, followed by hepatomegaly. All our patients were homozygous SS and undergoing folic acid prophylaxis. 96% of our patients did not receive any specific vaccine. Antibiotic therapy, hyperhydration and analgesics were administered to all our patients. 96% of our patients were transfused with red blood cell concentrate. 96% of our patients were transfused with packed red blood cells. 96% of our patients were transfused with packed red blood cells and 96% of them had a favorable outcome. Conclusion: ATS is an acute complication of sickle cell disease responsible for significant mortality and morbidity in the pediatric population. Its treatment is symptomatic and must be started early. Emphasis must be placed on prevention to prevent or limit its occurrence.

Highlights

  • Acute thoracic syndrome is the appearance of a new pulmonary infiltrate on radiology associated with fever, desaturation or respiratory signs

  • This study aims to study the epidemiological, clinical, therapeutic and evolutionary aspects of Acute Chest Syndrome in children at the Pediatrics Department of Donka National Hospital

  • This is a prospective study of descriptive type for a period of 6 months from February 19 to August 19, 2019 on patients with sickle cell disease who developed an acute thoracic syndrome (ATS) in the pediatrics department of Donka National Hospital

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Summary

Introduction

Acute thoracic syndrome is the appearance of a new pulmonary infiltrate on radiology associated with fever, desaturation or respiratory signs It is the second leading cause of hospitalization and the first cause of death in sickle cell patients. Sickle cell disease is an autosomal recessive genetic disease linked to a hemoglobin abnormality, characterized by the replacement of glutamic acid by valine on the β chain [1] It is a real public health problem in the world because of its morbidity and mortality, especially in childhood. According to the WHO, 2 to 3 million children with sickle cell disease are born in the world every year It is the most common genetic disease and mainly affects black populations [2]. Acute thoracic syndrome (ATS) is the second leading cause of hospitalization and the first cause of death in patients with sickle cell disease [5] It combines chronic hemolytic anemia, susceptibility to bacterial infections, vaso-occlusive phenomena and hypercoagulability [3] [4].

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