Spectrum of childhood interstitial and diffuse lung diseases at a tertiary hospital in Egypt

Salma G Abdelhady; Eman M Fouda; Malak A Shaheen; Faten A Ghazal; Ahmed M Mostafa; Ahmed M Osman; Andrew G Nicholson; Heba M Hamza

doi:10.1183/23120541.00880-2020

Spectrum of childhood interstitial and diffuse lung diseases at a tertiary hospital in Egypt

ERJ Open Res. 2021 Jun 7;7(2):00880-2020. doi: 10.1183/23120541.00880-2020. eCollection 2021 Apr.

Authors

Salma G Abdelhady^{1

2}, Eman M Fouda^{1

2}, Malak A Shaheen^{1

2}, Faten A Ghazal^{3

4}, Ahmed M Mostafa^{5

6}, Ahmed M Osman⁷, Andrew G Nicholson^{8

9}, Heba M Hamza^{1

2}

Affiliations

¹ Dept of Paediatrics, Faculty of Medicine, Ain Shams University, Cairo, Egypt.
² Children's Hospital, Pulmonology Unit, Ain Shams University Hospitals, Cairo, Egypt.
³ Dept of Pathology, Faculty of Medicine, Ain Shams University, Cairo, Egypt.
⁴ Pathology Lab, Ain Shams University Hospitals, Cairo, Egypt.
⁵ Dept of Thoracic Surgery, Faculty of Medicine, Ain Shams University, Cairo, Egypt.
⁶ Cardiovascular and Thoracic Academy, Ain Shams University Hospitals, Cairo, Egypt.
⁷ Dept of Radiology, Faculty of Medicine, Ain Shams University, Cairo, Egypt.
⁸ Dept of Histopathology, Royal Brompton and Harefield NHS Foundation Trust, London, UK.
⁹ National Heart and Lung Institute, Imperial College, London, UK.

Abstract

Background: Childhood interstitial and diffuse lung diseases (chILD) encompass a broad spectrum of rare pulmonary disorders. In most developing Middle Eastern countries, chILD is still underdiagnosed. Our objective was to describe and investigate patients diagnosed with chILD in a tertiary university hospital in Egypt.

Methods: We analysed data of consecutive subjects (aged <18 years) referred for further evaluation at the Children's Hospital, Ain Shams University (Cairo, Egypt). Diagnosis of chILD was made in accordance with the ChILD-EU criteria. The following information was obtained: demographic data, clinical characteristics, chest computed tomography findings, laboratory studies, spirometry, bronchoalveolar lavage and histopathology findings.

Results: 22 subjects were enrolled over 24 months. Median age at diagnosis was 7 years (range 3.5-14 years). The most common manifestations were dyspnoea (100%), cough (90.9%), clubbing (95.5%) and tachypnoea (90.9%). Systematic evaluation led to the following diagnoses: hypersensitivity pneumonitis (n=3), idiopathic interstitial pneumonias (n=4), chILD related to chronic granulomatous disease (n=3), chILD related to small airways disease (n=3), post-infectious chILD (n=2), Langerhans cell histiocytosis (n=2), idiopathic pulmonary haemosiderosis (n=2), granulomatous lymphocytic interstitial lung disease (n=1), systemic sclerosis (n=1) and familial interstitial lung disease (n=1). Among the subjects who completed the diagnostic evaluation (n=19), treatment was changed in 13 (68.4%) subjects.

Conclusion: Systematic evaluation and multidisciplinary peer review of chILD patients at our tertiary hospital led to changes in management in 68% of the patients. This study highlights the need for an Egyptian chILD network with genetic testing, as well as the value of collaborating with international groups in improving healthcare for children with chILD.