Transcript Document
بسم هللا الرحمن الرحيم ” بلي إن تصبروا و تتقوا ويأتوكم من فورهم هذا يمددكم ربكم بخمسة اآلف من المآلكة مسومين * وما جعله هللا إال بشري لكم و لتطمئن قلوبكم به وما النصر إال من عند هللا العزيز الحكيم“ صدق هللا العظيم آل عمران األيه 126 &125 Childhood Interstitial lung Disease (chILD) Journey to solve the Mystery Dr. Malak Shaheen (PhD Pediatrics, MSc Critical Medicine) What is chILD Syndrom? “Pneumonitis” Alveoli & perialveolar tissues Impaired gas exchange What are common features of chILD? 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) Fan Staging of chILD 1. Asymptomatic 2. Symptomatic with normal oxyhemoglobin saturation 3. Symptomatic with nocturnal or exercise-induced desaturation 4. Desaturation at rest 5. Pulmonary hypertension Causes of chILD Infectious Aspiration (GORD) Environmental (hypersensitivity pneumonitis) Drug-induced Neoplastic diseases (&LCH) Lymphoproliferative disorders (including HIV) Metabolic disorders Surfactant disorders Neurocutaneous syndromes Idiopathic pulm hemosidrosis Causes of chILD Collagen vascular disease Pulmonary vasculitis syndromes Radiation-induced Amyloidosis Graft-versus-host disease cont…. ARDS (recovering phase) Hypereosinophilic syndromes Pulmonary venoocclusive disease Sarcoidosis With chronic liver, kidney, bowel diseases 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) Does chILD differ from adILD? 1. Differences in the immune system responces, 2. 3. 4. 5. 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. Children Adult Is there one test to diagnose chILD? First round investigations: 1. Imaging (HRCT + ventilation control) 2. Lung functions (DLCO) 3. Oxygen saturation (rest /exercise) 4. Blood tests (CBC, ESR, Immune, Serology and PCR, RAST, ACE, HIV) 5. Resp secretions cultures/PCR 6. Sweat chloride test. 7. pH study/Contrast swallow 8. ECG and ECHO 9. Ciliary Brush Biopsy 10. Urine for CMV PCR Is there one test to diagnose chILD? Second round investigations: 1. Bronchoscopy and BAL for cytology 2. 3. 4. 5. 6. (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 Is there one test to diagnose chILD? Third round investigations: 1. 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 Histologic Classification of chILD DIP Desquamative interstitial pneumonitis CIP Chronic pneumonitisof infancy NSIP Non-specific interstitial pneumonitis FB/LIP OP Follicular bronchiolitis /lymphoid Interstitial Pneumonia Organizing pneumonia (old BOOP) PIG Pulmonary interstitial glycogenosis NEHI Neuroendocrine cell hyperplasia of infancy * UIP: Usual interstitial pneuminitis (rare for children) Pulmonary Interstitial Glycogenosis Neuroendocrine cell hyperplasia of infancy (NEHI) DIP CIP Nonspecific interstitial pneumonitis Follicular bronchiolitis BOOP 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 Further Readings …. First edition - 2008 Further Readings …. www.childfoundation.info/chILD Keep the hat on! Thank You