Adenotonsillar hypertrophy and frequency of vaso-occlusive crisis in patients with sickle cell disease at Mulago hospital, Kampala: An unmatched case-control study.

Abstract

Objectives: This unmatched case-control study compared the mean frequency of vaso-occlusive crisis in sickle cell disease patients aged 2-17 years with adenotonsillar hypertrophy (ATH) and those without at Mulago Hospital, Kampala, Central Uganda.

Methods: From September to December 2022, sickle cell disease patients aged 2-17 years were examined using headlight illumination, a tongue depressor, and a post-nasal x-ray to diagnose adenotonsillar hypertrophy. Social-demographic characteristics, medical history, and physical examination findings were recorded using a data collection form. Patients who had bone pain requiring hospitalization in the previous 6 months were regarded as having a vaso-occlusive crisis. Those with a diagnosis of ATH (cases) were compared to those without (controls). Data were collected and managed using an open data kit (ODK) and analysed using STATA version 16. A T-test was used to compare the mean frequency of vaso-occlusive crisis between Cases and Controls.

Results: There was a significant difference in the mean frequency of painful vaso-occlusive crises suffered in the previous 6 months between cases and controls (p value = 0.018). Age of 15-18 years [OR(CI):1.56, AOR:2.59; p = 0.047], females [OR(CI):1.68, AOR: 2.35; p = 0.003], snoring [OR(CI):12.01, AOR:8.4; p < 0.0001], and children who predominately breathe through the mouth [OR(CI):12.92, AOR:5.14; p = 0.001] were all associated with increased odds of adenotonsillar hypertrophy. Among disease-modifying factors, patients who were taking hydroxyurea [OR(CI): 2.16, AOR: 2.33; p = 0.027] had higher odds of adenotonsillar hypertrophy.

Conclusion: Children with sickle cell disease and adenotonsillar hypertrophy are at risk of suffering more pain vaso-occlusive crises compared to their healthy counterparts. The presence of obstructive adenotonsillar hypertrophy in children with sickle cell disease increases sickle cell morbidity, so more attention to these children through routine screening, diagnosis, and timely therapeutic intervention could reduce the morbidity.