This document discusses the case of a 13-year-old boy with chronic lung disease who presented with worsening cough and difficulty breathing. He has a history of recurrent lung infections and symptoms since childhood. Differential diagnoses considered include post-infective bronchiectasis, cystic fibrosis, and childhood interstitial lung diseases. Investigations showed bronchiectasis and pseudomonas infection. The document reviews approaches to evaluating the etiology of non-cystic fibrosis bronchiectasis, managing bronchiectasis and its exacerbations, and discusses other conditions like bronchiolitis obliterans and childhood interstitial lung diseases.

1. APPROACH TO A CHILD WITH
CHRONIC LUNG DISEASE
DR SAMPATH MARAPANA
REGISTRAR IN PAEDIATRICS - THP

2. CASE HISTORY
• Master Dasun Prasanga, 13year and 6month
old boy who is known child with a chronic lung
disease, presented with worsened moist
cough and difficulty of breathing for 1 1/2
month duration.
• Transferred from TH-Anuradhapura to our unit
for further evaluation

3. • He was born by NVD, not cried at birth and
PBU care given for 3days
• Apparently well up to 7 years of age
• In 2013 he has developed symptoms
suggestive of bronchial asthma and allergic
rhinitis.
• Treated with MDI Salmeterol + Fluticasone
and MDI Salbutamol on SOS basis.
• But symptoms were not under controlled with
proper treatment for 6 months duration.

4. • In 2014 he has presented with worsening of
cough which was productive with purulent
sputum and cold for 2weeks duration without
fever.
• Managed as infective exacerbation of BA and
discharged.
• Followed up at routine clinic
– Found to have he had un resolving symptoms and
lung signs with adequate treatment with good
compliance

5. ? Differential diagnoses

6. ? Differential diagnoses
• Foreign body
• Structural abnormality –extra luminal and intraluminal
compressions, cysts, vascular rings
• Immunodeficiency
• TB
• Post infective BO
• Post infective Bronchiectasis
• Primary Cilliary dyskinesia
• CF
• Aspiration syndromes
• Allergic broncho-pulmonary aspergillosis

7. • CXR – R/S lower lobe consolidation
? Foreign body
• HRCT – collapse consolidation in medial
segment of R/middle lobe & postero basal
segment.
• No evidence of FB

8. • Broncoscopy –Noted thick purulent secretions
in R/main bronchi
? lung abscess
R/S Exploratory mini thoracotomy done
Findings - R/S Middle lobe and lower lobe were
shrunken and noticed very short ML vein
Resection was not done due to high risk of
vascular damage.

9. • Symptoms were subsided with treatment.
• In 2016 again presented with worsened
productive cough with yellowish sputum and
DOB.

10. Repeat HRCT – Bronchiectasis of R/Lower lobe
and L/Lingual lobe

11. • Bronchoscopy done
– Normal tracheobronchial tree
– BAL done and Pseudomonas aeruginosa isolated
– HIV –Negative
– Immunoglobulin levels –Normal
– Sweat chloride test arranged- not done

12. • This time again he has developed productive
cough with purulent sputum and difficulty of
breathing 1 ½ months back.
• Still he has no significant improvement and he
is now oxygen dependent.
• Still he has no etiological diagnosis

13. • Non CF Bronchiectasis
• CF Bronchiectasis
• Bronchiolitis obliterans
• Childhood Interstitial lung diseases (chILD)

14. BRONCHIECTASIS
Irreversible abnormal dilatation and anatomic
distortion of the bronchial tree

15. When to suspect bronchiectasis ?
• 01. Persistent, productive cough or moist cough
almost daily for 8 weeks without periods of
complete remission.
• 02. Children with difficult asthma, poorly
controlled with adequate treatment
• 03. Prolonged cough with worsening symptoms
• 04. Pertussis like illness which doesn’t resolve
after 6 months.
• 05. Incomplete resolution of symptoms , physical
signs or radiological changes following an episode
of severe pneumonia.

16. • 06. Recurrent pneumonia (≥2 episodes within
one year) – localized or multifocal
• 07. Chest deformity, finger clubbing, or persistent
crepitations without any obvious explanation
• 08. Unexplained CXR findings or persitent
abnormalities more than 12wks following the
initial illness
• 09. A positive culture for unususal organism
(Staphylococcus aureus, Pseudomonas aeruginosa, Non tuberculous
mycobacteria)
• 10. Recurrent respiratory symptoms in children
with GERD, swallowing problems or upper airway
abnormalities.
• 11. Unexplained haemoptysis

17. Non Cystic fibrosis Bronchiectasis
BRONCHIECTASIS
Cystic fibrosis bronchiectasis

18. Etiology of non-CF Bronchiectasis
• 01. Post infectious –commonest identified cause
• E.g. pneumonia, measles, pertusis, tuberculosis
• 02. Immune deficiency disorders (primary or
aquired)
• 03. Aspiration syndromes
• 04. Airway Obstruction – FB, lymphnodes,
tumours, cysts and vascular rings
• Congenital malformations – Pulmonary
sequestration and airway malformations
• Primary ciliary diskinesia (PCD)
• Allergic broncho-pulmonary aspergillosis (ABPA)

19. Etiological evaluation
• Post infectious
– History of severe /complicated respiratory tract
infection
– Hx suggestive of measles, pertusis and TB
Broncho-alveolar lavage samples for microbiology
testing
Sputum /gastric aspirate aspirate for AFB, TB culture
and GeneXpert MTB/RIF

20. • Immunodeficiency disorders
– Consangunity
– Family history of immune deficiency
– Delayed umbilical cord separation
– Recurrent pneumonia, ear infection, skin rashes,
recurrent abscess formation, oral thrush etc.
– HIV screening, serum Immunoglobulin levels and
lymphocytes subsets

21. • Aspiration syndromes
– Underlying neurological disorders
– Cough and choking episodes during feeding – TOF
– Gastro esophageal reflux disease – recurrent
vomiting and regurgitation
– Upper GI contrast study
– Milk scan , Extended esophageal pH monitoring

22. • Missed FB aspiration
– History of FB aspiration
– Sudden onset coughing, choking, respiratory
distress followed by persistent respiratory
symptoms
– CECT
– Broncoscopy

23. • Allergic broncho-pulmonary aspergillosis (ABPA)
– Allergic bronchopulmonary aspergillosis is the result
of hypersensitivity towards Aspergillus spp.
– Poorly controlled asthma despite adequate treatment
and good compliance
– Brown coloured sputum production
– BAL galactomannan – cell wall component
– Following criteria should be considered for diagnosis
– Obligatory criteria
• Total serum IgE > 1000IU/ml
• Positive Aspergillus fumigates specific IGE or skin prick test
– Other criteria
• Raised Aspergillus fumigatus IgG or precipitins
• Eosinophils > 500 cells/uL in peripheral blood
• Radiological features consistent with ABPA

24. • Mucoid impaction in dilated bronchi can appear
as mass like/sausage-shaped or blanching
opacities
–“Finger in glove sign”

25. • Primary ciliary diskinesia (PCD)
– Hx of neonatal respiratory distress in term baby
– Daily wet cough since early childhood
– Recurrent otitis media
– Persistent rhinosinusitis, nasal polyps
– Congenital heart defects and situs anomality
– Hx of abdominal surgeries
– Screening – Nasal nitric oxide level (nNO) <70 ppb
– Confirmation
• Nasal scraping for electron microscopy studies
• Genetic diagnosis

26. PICADAR SCORE

27. Predicted probability

28. Congenital forms
• William-Campbell syndrome – absence of
annular bronchial cartilage
• Marnier-Kuhn syndrome – congenital
tracheobronchomegaly
• Yellow nail syndrome- (Pleural effusion,
Lymphedema, discolored nails)
• Right middle lobe syndrome

29. CYSTIC FOBROSIS
When to suspect CF
• Respiratory-
– Recurrent sino-pulmonary desease
– Non/poorly resolving pneumonia
– Unexplained diffuse lung disease or bronchiectasis
– Unexplained neonatal respiratory distress
– Chronic infection seen with Staphylococcus aureus,
Haemophilus influenza, Pseudomonas aeruginosa and
Burkholderia capecia complex

30. When to suspect CF
• GI-
– Meconium ileus, prolonged neonatal jaundice
– Acute or chronic pancreatitis
– Rectal prolapse, malabsorption with fat soluble
vitamin deficiencies
• Unexplained failure to thrive
• Late onset haemorrhagic disease of newborn
• Salt depletion syndrome
• Family hx of CF

31. • Diagnosis
– New born screening
– Measurement of sweat chloride using pilocarpine
iontotophoresis – Gold standard
• <30mmol/L - Normal
• >60mmol/L - Diagnostic
• 30-60mmol/L correlate with clinical picture and repeat
thr test
There could be false positive and false negative test
results

32. Management of bronchiectasis
• Goals of therapy
– Reduced the frequency and severity of
exacerbations
– Prevent further lung damage and complications
– Preserve lung function
– Facilitate normal growth and development
– Improve quality of life

33. Management strategies
• General measures
– Maintain good hydration
– Adequate nutrition
– Parental awareness regarding condition
• Prevention of infections / exacerbations
– Additional immunization (pneumococcal and
influenza)
– Avoid exacerbating factors – smoke, irritants

34. • Airway Clearance Therapy (ACT)

35. 01. Breathing techniques
– Active cycle of breathing

36. • Autogenic drainage

37. 03. Manual techniques/assistant dependant
techniques
• Modified postural drainage

38. • Clapping, percussion &
vibration

39. • Device dependent techniques
Bottle PEP
Acapella

40. Fluttter - bouncing of the ball causes increased pressure inside
the chest and sends vibrations down through the airways
• increased pressure and vibrations helps the mucous move
from the lungs into the airways

41. • Chest wall oscillation

42. • Inhaled corticosteroids (ICS)
– Prescribed if there is associated wheezing
• Regular sputum cultures
– Done in symptomatic children to guide the treatment
– In all children during follow up
• Immune modulator therapy
– Azithromycin 10mg/kg/dose 3times /wk or
30mg/kg/dose weekly
• Prophylactic antibiotics
– Considered in severe recurrent infections
– In immunodeficiency disorders may need prophylactic
co-trimoxazole and itraconazole

43. Treatment of exacerbations
• Early identification
• Vigorous appropriate treatment (guided by
sputum culture sensitivity)
• Adequate duration essential to prevent
further lung damage

44. Surgical management
• Poorly controlled localized disease
• Recurrent hemoptysis
• Poor response or not amenable to
interventional radiological therapy
Lobectomy rarely pneumonectomy

45. Extra pulmonary management of CF
• Pancreatic enzyme replacement therapy
• No dietary restrictions
• Vitamin supplements
• Salt & fluid intake should be encouraged
• Multidisciplinary care
• Annual workup
– Anthropometry
– Lung functions, Liver functions
– USS abdomen, Echocardiogram
– FBS,OGTT,HBA1C

46. BRONCHIOLITIS OBLITERANS
• Occurs most commonly after an episode of acute
bronchiolitis or bronchopneumonia
• Fibrosing form of chronic obstructive lung disease
characterized by narrowing and /or completely
obliteration of small airways
• Initial insult – derangement in epithelial cell function or
local necrosis
• Accumulation of fibrino-purulent exudate induces
deposition of collagen and mucopolysaccharides from
myofibroblasts

48. Post infectious Adenovirus types 3,7,and 21
Influenza virus, Parainfluenza virus
RSV
Measles virus, Varicella virus
Mycoplasma pneumoniae
Post-transplant Chronic rejection
GVHD
Connective tissue disease Rheumatoid arthritis
Sjogren’s syndrome, SLE
Toxic fume inhalation NO
Ammonia
Chronic hypersensitivity
pneumonitis
Avian antigens
Mold
Aspiration GERD
Foreign bodies
Drugs Penicillamine
Cocaine

49. • In post – infective BO, gradual resorption of
the fibro-vascular connective tissue is possible
with restoration of normal airway caliber
• Generally leads to chronic non progressive
disease
Proliferative bronchiolitis
Constrictive bronchiolitis
BO

50. Diagnosis
• 01. History of an acute and severe bronchiolitis/ viral
pneumonia in a previously healthy child during first
three years of life
• 02. Evidence of persistent airway obstruction after acute
event after 2wks course of systemic corticosteroids and
broncho dialator.
• 03. CXR- features of obstructive lung disease , airway
wall thickening with or without bronchiectasis
• 04. CT- mosaic pattern and air trapping
• 05.Exclusion of other lung diseases (TB, CF, BPD, asthma,
immunodeficiency, alpha 1 antitrypsin def)
• PBO is diagnosed when all criteria mentioned above are met

51. Treatment
• Supportive treatment is mainstay since specific
treatment modalities are unknown
• Individualize according to age, severity, degree of
radiological involvement
– Nutritional optimization
– Avoidance of inhaled irritants
– Prevention of infection
– Vaccination (annual influenza and pneumococcal)
– Supplemental O2 for patient with hypoxaemia
– Oral Azithromycin prophylaxisc10mg/kg/dose, 3times/wk
– Corticosteroid therapy
– Treatment for GERD
– Airway clearance techniques, when complicated with
bronchiectasis.

52. CHILDHOOD INTERSTITIAL LUNG DISEASE
(chILD)
• The term “interstitial” may be misleading , because
other parenchymal components such as vessels,
epithelium, airways or pleura also involved
• Evaluation
– Birth Hx
– Detailed family Hx
– Environmental Hx
– Physical examination – nutritional indices, sino-pulmonary
disease, chest wall deformities, skin rashes, clubbing and
neurological deficit

53. Diagnosis
• 01. Respiratory symptoms such as coughing, rapid
breathing, or exercise intolerance
• 02. Physical signs such as crackles, adventitial
breathing sounds, digital clubbing, or intercostal
retractions
• 03.Low blood oxygen tension or hypoxaemia
• 04. Diffuse parenchymal abnormalities on chest
imaging
Three out of four without known underlying lung
disease

54. Disorders more prevalent in infancy
Diffuse developmental disorders Acinar dysplasia
Congenital alveolar dysplasia
Growth abnormalities Pulmonary hypoplasis
Broncho-pulmonary displasia in
premature babies
Specific conditions of undefined
aetiology
Pulmonary interstitial glycogenosis
Neuro-endocrine cell hyperplasia of
infancy
Surfactant dysfunction mutations and
related disorders

55. Disorders not specific to infancy
Disorders of the normal host Infection associated processes
Aspiration syndromes
Eosinophilic pneumonia
Disorders related to environmental agents-
hypersensitivity pneumonia, toxic inhalation
Disorders related to systemic
disease processes
Immune related disorders
Storage diseases
Sarcoidosis
LCH, Malignant infiltrates
Disorders of immune
compromised host
Opportunistic infections
Disorders related to transplantation and
rejection
Disorders misidentifying as ILD Cardiac disorders, Pulmonary arterial
hypertension, veno-occlusive disorders

56. • Investigations
– CXR
– HRCT – most helpful imaging modality
– Pulmonary function tests – classic pattern is
restrictive, ↓FEV1 and FVC
– Bronchoscopy and BAL
– Genetic testing - surfactant disorders
– Lung biopsy

57. THANK YOU