3 7 730 100 3 8 730 s respiratory disorders concurrent … with unexplained neonatal respiratory...
TRANSCRIPT
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Respiratory DisordersThomas Lahiri MD
Professor of PediatricsLarner College of Medicine
University of Vermont
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Disclosure I have no relevant financial relationships
with the manufacturers(s) of any commercial products(s) and/or provider of commercial services discussed in this CME activity.
I do not intend to discuss an unapproved/investigative use of a commercial product/device in my presentation.
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Objectives Identify and distinguish causes of severe upper
airway infections in children Discuss the presentation and treatment of a
child with cystic fibrosis Know the typical presentation and treatment of
pneumothoraces Distinguish foreign body aspiration from
asthma
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Case 1: A 3 year old boy is seen for chronic wet-sounding
cough. Growth has been poor. There has been partial improvement with antibiotics. Albuterol does not help.
What is your next intervention? What is the likely diagnosis? How will this child’s condition be treated? What other organ systems are involved in his
disease process?
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Cystic Fibrosis Autosomal recessive condition CFTR (cystic fibrosis transmembrane
conductance regulator) dysfunction More than 2000 mutations identified
Principal manifestations Recurrent respiratory tract infections Failure to thrive/Malnutrition Meconium ileus (15-20% of cases)
Infants may present with severe malnutrition, low sodium/chloride, hypoproteinema
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Variable Presentation Recurrent pneumonia Meconium ileus Failure to thrive Rectal prolapse Malnutrition Steatorrhea Cholelithiasis Pneumothorax Nasal polyposis Clubbing Hemoptysis
Chronic sinusitis Pancreatitis Diabetes Cirrhosis Intussusception ABPA Infertility CBAVD Hypertrophic
osteoarthropathy Newborn screening
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CF Diagnosis NBS programs for CF have been approved
in 50 states More than 60% of new diagnosed by NBS Elevation of immunoreactive trypsinogen
(IRT) as a marker of pancreatic injury Sweat chloride testing is gold standard) CFTR mutation analysis indicated for all
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CF: Extrapulmonary Exocrine pancreatic insufficiency
Steatorrhea, rectal prolapse, vitamin deficiency Distal intestinal obstruction syndrome
Meconium ileus equivalent Hepatobiliary disease
May present with prolonged jaundice CF related diabetes
Increasing incidence with age Infertility
98% of males with obstructive aspermia
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Exocrine Pancreatic Insufficiency (PI)
Obstruction of pancreatic ducts by inspissated secretions
Damage to acinar cells, autodigestion 60% of CF infants have PI at birth Eventually 85-90% develop PI Leads to malabsorption of fat, protein,
decreased bicarbonate secretion
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Diagnosis of Exocrine Pancreatic Insufficiency
Congenital bowel obstruction Steatorrhea/large stools Failure to thrive Rectal prolapse Diagnosis confirmed with
Fecal elastase or coefficient of fat absorption
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Nutritional Management Fat soluble vitamins (A, D, E, K) 1/8 tsp of salt daily for infants Pancreatic enzyme replacement
1000-2500 units lipase/kg/meal
Adequate calories/high fat diet 1.5X RDA for calories, may need oral or
enteral supplementation
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Better Nutrition = Better Lung Function
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CF Hepatobiliary Disease Infants may have conjugated
hyperbilirubinemia or transaminitis Complicated by malnutrition, prolonged TPN
25-30% develop biliary tract involvement Dilation of ducts with periportal fibrosis May develop portal hypertension and
hepatic failure (very infrequent)
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CF: Pulmonary Early
CXR may be normal or show bronchial wall thickening
BAL fluid may show increased neutrophiliaeven in infancy
Late Bronchial dilation leading to bronchiectasis,
mucous plugging Marked neutrophilic inflammation
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CF Bronchiectasis
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Pulmonary Disease in CF Goal: prevent bronchiectasis/structural
lung disease and preserve function Airway clearance/exercise Abnormal mucus properties
DNase, hypertonic saline Inflammation
High dose ibuprofen, azithromycin Antibiotics: oral, inhaled, parenteral
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ManualFlutter
Airway Clearance
VestAcapella
And EXERCISE !
IPV
Frequencer
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CF Survival
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CFTR Modulators 2012: ivacaftor (Kalydeco) for patients
with at least 1 CFTR gating mutation (&R117H): ↑lung function/weight and fewer exacerbations
2015: lumicaftor/ivacaftor (Orkambi) for patients >12 yrs & homozygous for F508del (50% of CF pop.)
2016 Orkambi approved for 6-11 yrs
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Case 1
The same 3 year old boy with chronic wet cough, sweat chloride < 10 mmol/L.
What other diagnoses are in your differential?
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Non-CF Bronchiectasis
Pneumonia (usually unilobar) Chronic aspiration Foreign body (retained) Immunodeficiency Primary ciliary dyskinesia
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Primary Ciliary Dyskinesia Consider with unexplained neonatal
respiratory distress/hypoxemia Recurrent/chronic purulent otitis Chronic nasal congestion/rhinorrhea Heterotaxy, 50% with dextrocardia Diagnosis: clinical features, ciliary biopsy,
genetic testing, nasal nitric oxide (low)
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26
Primary Ciliary Dyskinesia
What’s Wrong?
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Case 2: Barking childThe father of a 2 year old previously healthy
boy calls you after his child awoke with a dry, harsh sounding cough.
1. What other history is important?2. What conditions are you concerned
about?3. What management, if any, is indicated?
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Croup Infectious (Viral)
laryngotracheobronchitis Fever URI symptoms Barking cough Inspiratory stridor Hoarseness
Non-infectious“spasmodic”
Afebrile No other signs of
illness Recurrent disorder Acute nocturnal onset Rapid resolution
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Laryngotracheobronchitis
Viral etiologies: parainfluenza type 1>2,3 RSV, influenza A & B, metapneumovirus
Very common (1-6% of kids under 6 yrs) Rarely requires hospitalization for
impending respiratory failure Usually resolves in 3-5 days
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Croup Differential Diagnosis Bacterial tracheitis Epiglottitis Retropharyngeal abscess Peritonsillar abscess Foreign body aspiration Caustic ingestion
Subglottic stenosis Vocal cord paralysis Anaphylaxis Tumor Hemangioma Diphtheria
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LTB Management Minimize child anxiety Symptom control/prevent hospitalization Racemic epinephrine or L-epinephrine
0.5 ml of 2.25% solution in NS (racemic) 5ml of 1:1000 (5 mg) L-epinephrine
Systemic steroids Dexamethasone 0.6mg/kg x 1 (oral or IM)
Nebulized steroids: Budesonide 2mg
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Croup
AP and lateral x-rays may show subglottic narrowing: “steeple sign”
Laboratory studies unhelpful
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Croup
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Case 2a2 year old boy is brought to the ED with T
of 40°C, toxic appearance, “brassy” cough, marked dyspnea, hoarseness, fails to respond to racemic epinephrine.
1. Now what are you worried about?2. How should you proceed?
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Emergent Airway Infections Bacterial Tracheitis
Rapid progression Often evolves from croup (biphasic illness) S.aureus, S.pyogenes
Supraglottitis (epiglottitis) Seen rarely in young children since Hib
vaccine Now seen more in older children with
nontypeable H.influenzae, S.pyogenes
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Bacterial Tracheitis
Assemble airway team Prepare for intubation/bronchoscopy in a
controlled setting (OR) Prompt coverage with parenteral
antibiotics (cover S.aureus) May require extended period of
mechanical ventilation
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Bacterial Tracheitis
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Supraglottitis Maintain child in upright position in
parent’s arms or lap Assemble airway team If concern for loss of airway, then proceed
with endoscopy/intubation in controlled setting rather than obtaining neck films
Quick resolution following intubation, antibiotics, steroids
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Supraglottitis
Smalhout B The Suffocating Child1980
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Supraglottitis
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Case 3: “His asthma isn’t getting any better”
18 month old boy presents with a 3 day history of cough and wheeze. No antecedent URI or fever. He has diffuse wheezing which responds poorly to albuterol.
What further evaluation is indicated? What are causes of chronic wheezing in
children?
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Foreign Body Aspiration
More common in toddlers 50% of children with foreign body
aspiration have no witnessed history Symptoms include sudden onset of cough,
choking, wheezing or shortness of breath Usually no signs of infection (unless
symptoms have persisted for weeks)
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Foreign Body Aspiration
Physical examination May be normal Unilateral wheezing or decreased aeration Asymmetric thorax +/- signs of respiratory distress
More likely with laryngeal foreign body
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Evaluation of FB Aspiration Chest radiographs may demonstrate
differential aeration Inspiratory/expiratory films can be
performed in cooperative children Bilateral decubitus films in infants Airway fluoroscopy can show air trapping Bronchoscopy with any clinical suspicion
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Foreign Body Aspiration
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Foreign Bodies
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Foreign Bodies
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FB Aspiration-Complications
Severe airway inflammation Scarring/granulation tissue Recurrent pneumonia Bronchiectasis Hemoptysis Bronchopleural fistula
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Differential:Chronic Wheeze Asthma Tracheobronchomalacia Foreign body aspiration Oral or gastric chronic aspiration Vascular ring Hemangioma Congenital malformation (CPAM)
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Normal Trachea Tracheomalacia
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Primary Tracheomalacia Common Symptoms include
stridor, wheeze, harsh cough, recurrent infections, reflex apnea
Abnormal cartilage to membrane ratio
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Case 4: My chest hurts…15 year old presents with a 12 hour history
of sudden left sided upper chest/shoulder pain, that began during basketball practice.
What is the usual presentation of a pneumothorax?
What is the appropriate management of pneumothoraces?
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Pneumothorax
Spontaneous Apical blebs More common in young, tall, thin males
Secondary Underlying pulmonary disease (asthma, cystic
fibrosis, pneumonia) Trauma
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Pneumothorax Symptoms:
Sudden onset Chest pain, worse with inspiration (pleuritic) Shortness of breath
Signs: Tachypnea Decreased aeration Hypoxemia Tension pneumothorax: unilateral absent BS, tracheal
deviation, bradycardia, ↓BP
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Pneumothorax Management Depends on severity Supplemental oxygen (FiO2 1.0) For worsening or persistent symptoms, large air
collection or underlying disease Thoracentesis Chest tube drainage Bleb resection Pleurodesis
High recurrence rate (>20%): ipsilateral and contralateral
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QUESTIONS ???