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بسم الله الرحمن الرحيم

بسم الله الرحمن الرحيم. ” بلي إن تصبروا و تتقوا ويأتوكم من فورهم هذا يمددكم ربكم بخمسة الآف من الملآكة مسومين * وما جعله الله إلا بشري لكم و لتطمئن قلوبكم به وما النصر إلا من عند الله العزيز الحكيم “. صدق الله العظيم آل عمران الأيه 125& 126. Childhood Interstitial lung Disease (chILD).

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بسم الله الرحمن الرحيم

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  1. بسم الله الرحمن الرحيم ” بلي إن تصبروا و تتقوا ويأتوكم من فورهم هذا يمددكم ربكم بخمسة الآف من الملآكة مسومين * وما جعله الله إلا بشري لكم و لتطمئن قلوبكم به وما النصر إلا من عند الله العزيز الحكيم“ صدق الله العظيم آل عمران الأيه 125& 126

  2. Childhood Interstitial lung Disease (chILD) Journey to solve the Mystery Dr. Malak Shaheen (PhD Pediatrics, MSc Critical Medicine)

  3. What is chILD Syndrom? “Pneumonitis”

  4. Persistent 3 of the following 4 ≥1 month: 1- Respiratory symptoms (cough, distress, exercise intolerence) 2- Evidence of impaired gas exchange (hypoxia &/or hypercapnia) 3- Diffuse/Patchy radiological chest abnormality (CXR or CTscan) 4- Adventitious sounds on auscultation (crepitation/wheeze) What are common features of chILD?

  5. Fan Staging of chILD • Asymptomatic • Symptomatic with normal oxyhemoglobin saturation • Symptomatic with nocturnal or exercise-induced desaturation • Desaturation at rest • Pulmonary hypertension

  6. Infectious Aspiration (GORD) Environmental (hypersensitivity pneumonitis) Drug-induced Neoplastic diseases (&LCH) Lymphoproliferative disorders (including HIV) Metabolic disorders Surfactant disorders Neurocutaneous syndromes Idiopathic pulmhemosidrosis Causes of chILD

  7. Collagen vascular disease Pulmonary vasculitis syndromes Radiation-induced Amyloidosis Graft-versus-host disease ARDS (recovering phase) Hypereosinophilic syndromes Pulmonary veno-occlusive disease Sarcoidosis With chronic liver, kidney, bowel diseases Causes of chILD cont….

  8. DD to rule out include: • Cystic fibrosis • Asthma • Cardiac (heart) disease • Primary ciliary dyskinesia • Scoliosis and chest wall abnormalities • Neuro-muscular disease/Neurocut dis • Immune deficiency • TB • Developmental abnormalities (BPD - Alveolar capillary dysplasia- Pulm hypoplasia)

  9. Does chILD differ from adILD? • Differences in the immune system responces, cytokine and growth factors • Histologic classification differs significantly • Specific types presented at children not adults • Rarer and less stereotyped than adult ILD • More difficult to treat than adults.

  10. Is there one test to diagnose chILD? • First round investigations: • Imaging (HRCT + ventilation control) • Lung functions (DLCO) • Oxygen saturation (rest /exercise) • Blood tests (CBC, ESR, Immune, Serology and PCR, RAST, ACE, HIV) • Resp secretions cultures/PCR • Sweat chloride test. • pH study/Contrast swallow • ECG and ECHO • Ciliary Brush Biopsy • Urine for CMV PCR

  11. CT scan

  12. Is there one test to diagnose chILD? • Second round investigations: • Bronchoscopy and BAL for cytology (LCH, iron laden macrophages, PAP) and cultures • Prone Oesophagram for H type fistula • Videofluroscopy for aspiration evidence • Cardiac Cath • Detailed lymphocyte function tests • TB –Elispot test

  13. Is there one test to diagnose chILD? • Third round investigations: • Lung biopsy (Transbronchial, percutaneous, thoracoscopic, open lung) CT guided from affected patch and unaffected patch. • Special stains (eg. Bompesin or PAS) • Immunoblotting for sufactant proteins at lung biopsy • Electron microscopy study of biopsy • DNA for mutations in SPB, SPC and ABCA3

  14. Histologic Classification of chILD * UIP: Usual interstitial pneuminitis (rare for children)

  15. Pulmonary Interstitial Glycogenosis

  16. Neuroendocrine cell hyperplasia of infancy (NEHI)

  17. CIP DIP Nonspecific interstitial pneumonitis

  18. Follicular bronchiolitis BOOP

  19. Surfactant Metabolism Dysfunction • Surfactant is a complex mixture of phospholipids and proteins (SP-A, -B, -C and -D)& ABCA3. • ABCA3 an ATP-binding transporter Of lipids. (chILD) due to ABCA3 gene mutations

  20. Further Readings …. First edition - 2008

  21. Further Readings …. www.childfoundation.info/chILD

  22. Keep the hat on! Thank You

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