Critical Appraisal of a mortality case presentation

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1. Critical Appraisal 20/07/2012 CASE 1 Dr. Saptharishi L G 2. What is Interstitial Lung Disease? What is the diagnostic criteria? What are the common etiologies? What…
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  • 1. Critical Appraisal 20/07/2012 CASE 1 Dr. Saptharishi L G
  • 2. What is Interstitial Lung Disease? What is the diagnostic criteria? What are the common etiologies? What have been ruled out in the index case? 1. Does the index case fit into ILD?
  • 3. Pediatric Interstitial Lung Disease Diffuse Lung Disease Chronic lung disease Chronic interstitial lung disease Chronic Interstitial Pneumonitis Pediatric Interstitial Lung Disease (PILD) Childhood Interstitial Lung disease (chILD) Chronic disease of Lung Parenchyma (CdoLP)
  • 4. Interstitial Lung disease • Incidence – 0.36 per 100,000 children • Large heterogeneous group • ‘Syndrome’ – • • • • • • Tachypnea, crackles (basal, ‘Velcro-type’) Clubbing & growth failure Impairment of pulmonary gas exchange Restrictive lung function Diffuse pulmonary infiltrates Inflammation in the interstitium with thickening of alveolar wall due to inflammatory cells/fibrosis • Diagnostic criteria • Lung biopsy based – Histo-pathological criteria • C-R-P diagnosis • Clinical – Radiological – Pathological correlation
  • 5. Etiologies of PILD
  • 6. Differential diagnosis for ILD
  • 7. Does this child fit into ILD ? YES YES • POINTS IN FAVOR: • Recurrent respiratory tract infections • Chronic presentation – tachypnea, bilateral diffuse crepitations, hypoxemia • Pulmonary infiltrates • HRCT picture – Ground-glass opacities with sparing of peripheral lung tissue • LOOSE ENDS: • Prominent mediastinal and hilar lymphadenopathy • Lung biopsy reports pending
  • 8. ILD data from China - 2011* • 93 patients from 11 pediatric hospitals • 39 (42%) – Bronchiolitis obliterans • 39 (42%) – Idiopathic Pulmonary Hemosiderosis • 7 (7.5%) – Idiopathic interstitial pneumonia • HRCT: • 56 (60%)– Ground glass opacifications (GGO) • 30 (32%) – mosaic appearance *Chen HZ et al. Clinical study on ILD in children of China. Zhonghua Er Ke Za Zhi. 2011 Oct; 49 (10): 734-9.
  • 9. Differential diagnosis for GGOs in the clinical setting of ILD Hypersensitivity Pneumonitis Diffuse Interstitial Pneumonitis Non-specific Interstitial Pneumonitis Acute interstitial pneumonia Chronic Organizing Pneumonia Bronchiolitis Obliterans Chronic Eosinophilic pneumonia Sarcoidosis Pulmonary alveolar proteinosis Lymphocytic Interstitial Pneumonia Extra-nodal marginal B cell lymphoma Exogenous lipoid pneumonia Recurrent aspirations
  • 10. Tracheo-Esophageal Fistula • • • • • A diagnostic possibility h/o recurrent pneumonia Feed-related respiratory worsening – multiple Treatable cause of recurrent aspirations Association with GER – well established Why was H-shaped TEF not considered in the differential diagnosis ?
  • 11. Is our index case equivalent to a child with CAP? Are there any specific infections associated with ILD? 2. Were the antibiotic decisions in this child justified?
  • 12. Antibiotic choice • Index case – • • • • Two previous hospital admissions in 6 months On home-based oxygen therapy Received oral steroids for > 3 months Received Methyl Prednisolone pulse steroid during previous admission • Grade IV malnutrition HIGH RISK FOR IMMUNOSUPPRESSION
  • 13. Likely infections in INDEX case Long term steroid therapy induced immunosuppression • • • • • • • • • • • S. aureus S. pneumoniae Legionella Gram negative organisms Listeria Pseudomonas aeruginosa Pneumocystis jirovecii Nocardia spp. Strogyloides stercoralis Varicella-zoster virus Fungal infections Interstitial Lung disease • CMV infections • EBV infection • Adenoviral
  • 14. Anti-fungal drugs • Should anti-fungal drugs have been considered in this child upfront? • If not on day one, on day 13-14 of hospital stay, when the child had secondary deterioration (child continued to be on full-dose steroids), why were anti-fungal drugs not considered?
  • 15. Anti-tubercular drugs • Child was started on ATT two days prior to demise. • What was the justification for starting ATT in this child ? • If indeed there was a clinical suspicion, should ATT have been started earlier?
  • 16. What is the role of Pneumococcal and influenza vaccines in children with ILD? 3. Vaccination in index case
  • 17. Prevention is better than cure? • Could we have considered Pneumococcal vaccine & Influenza vaccine when this child was under follow-up? • What is the unit’s policy on children with ILD? Is there a role for considering pneumococcal and influenza vaccines in these children?
  • 18. What does literature say regarding treatment of ILD? What other options are available? Could we have tried something that could have saved this child? Steroid use in septic shock in the index case. 4. Steroid use in Index case
  • 19. Treatment of ILD Ref: Pediatric Respiratory Reviews 2004.
  • 20. Literature regarding steroids • Desmarquest P et al. Chronic interstitial lung disease in children: response to high-dose intravenous methylprednisolone pulses. Pediatr Pulmonol. 1998 Nov;26(5):332-8. • 3 children treated with pulsed steroid @ 300 mg/m2 • 2 out of three responded • Deschildre A et al. Treatment with intermittent high dose corticotherapy in chronic interstitial pneumonia in an infant. A case report. Rev Mal Respir. 1994; 11 (5): 509-12. • RSV related chronic interstitial pneumonia • Oral steroid therapy followed by Methylpred boluses - monthly
  • 21. • 10 year old male – hypoxemia, progressive infiltration on chest radiograph, Biopsy showing desquamative interstitial pneumonia • Methyl Prednisolone pulses x 3 days every 4-6 weeks – administered concomitantly with oral prednisolone. • Acute deterioration while on pulse steroids, pulse dose hiked to 20 mg/kg pulses  SIGNIFICANT clinical improvement • At the end of 24 months, Normal lung function, blood gases, growth of 10 cm – Overall treatment success
  • 22. • Cyclophosphamide + prednisolone – better than steroids alone • Johnson et al – RCT – Cyclophosphamide + steroids Vs. steroids alone • Raghu et al – RCT – Azathioprine + steroids Vs. steroids alone PROTOCOL USED: • IV Methyl Prednisolone pulse for 3 consecutive days- weekly x 4 weeks • Cyclophosphamide + low dose prednisolone maintenance x 1 year
  • 23. Methyl Prednisolone Pulse therapy in ILD • Role of combination of daily steroids @ 2 mg/kg/day + Methyl Prednisolone high dose pulse therapy ? • Subsequently, post pulse steroid therapy, prednisolone was restarted at 2 mg/kg/day. Rationale?
  • 24. Steroid dose in septic shock Clinical situation Recommended steroid dosage Physiologic replacement 12.5 mg/m2/day Critical illness (stress dose) 50 mg/m2/day Shock dose (catecholamine resistant) Up to 50 mg/kg/day Ref: Pediatric Critical Care. Fuhrman & Zimmerman. 4 th Ed. After child developed shock requiring Dopamine/Adrenaline, why was oral maintenance dose of 2 mg/kg not hiked to shock dose of Hydrocortisone? Child had been receiving 2 mg/kg/day i.e. 17 mg/m2/day
  • 25. Indications for lung biopsy in ILD Sequence of events in index case 5. Justification for lung biopsy in index case
  • 26. Sequence of events in Index case • Fever spikes on D13-14 of admission with worsening respiratory distress and hypoxemia. Attributed to Nosocomial pneumonia Sepsis • Subsequently, intubated on day 16 of hospital stay. Two days later, child is started on Methylprednisolone pulse steroids (30 mg/kg/day) • Next day child is shifted for lung biopsy to Pediatric Surgery OT. Open lung biopsy under GA • On same day, child develops shock – requiring dopamine and adrenaline
  • 27. Note of appreciation • Diagnostic challenge – a relentlessly progressive respiratory condition – Poor prognosis • Extensive evaluation – diagnostic work-up (including Open lung biopsy) undertaken • Handling parents who were having difficulty accepting child’s clinical deterioration and prognosis – An uphill task • Getting parents to consent for AUTOPSY • Overall a worthy effort by the managing team
  • 28. Summary of queries • What sub-type of PILD does our index case fit into? (based on clinical + radiological details) • Was H-shaped TEF considered as a possibility in our case? • Role of anti-fungals and ATT in the index case • Vaccination in index case (Pneumococcal /Influenza) • Steroid protocol for chILD (childhood ILD) • Why were steroid doses not hiked to ‘SHOCK dose’? • Justification for lung biopsy in this case • Current status of pediatric lung transplantation in India
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