Lecture 46: Lower Respiratory Disorders in Children

Primary Ciliary Dyskinesia (PCD)

-Autosomal Recessive

• Results from absent or disordered ciliary movement

• Most commonly due to defect in dynein arm which provide energy via

ATPase

Primary Ciliary Dyskinesia (PCD)

• Recurrent otitis media, sinusitis and bronchiectasis

• 50% are associated with Kartagener syndrome

• Situs inversus

• Chronic sinusitis

• Bronchiectasis

• Males are infertile due to immotile sperm

• Chronic untreated infections lead to bronchiectasis

Primary Ciliary Dyskinesia (PCD)

Primary Ciliary Dyskinesia (PCD)

• Confirmed by electron microscopy of respiratory cilia

• Obtained by scraping/biopsy of respiratory epithelium

• Measurement of nasal NO (nitric oxide) is used as a screening tool (Will be low or absent)

• Pressure equalizing tubes for chronic otitis

• Sinus surgery although questionable benefit

• Chest physiotherapy

• Treatment of recurrent bacterial infection

treatment of Primary Ciliary Dyskinesia (PCD)

Hemoptysis

Coughing up blood or blood in the presence of sputum

Pulmonary Hemorrhage

bleeding from intrathoracic source

Hemoptysis

Refers to expectoration of blood originating from the lower respiratory tract

children usually swallow their sputum

why is hemoptysis rare in children?

Pulmonary Hemorrhage

Hemoptysis is a sign of ______________________________

Pulmonary Hemorrhage

Pulmonary Hemorrhage

Causes of _____________________:

• Pulmonary embolism

• Arteriovenous (AV) malformations

• Iatrogenic

• Congenital heart defects

• Pulmonary hypertension

• Infection

• Autoimmune disorders

Bronchiectasis

_______________________ can cause hyperplasia, tortuosity and dilation of bronchial arteries which can erode or rupture and bleed

Pulmonary Hemorrhage

• Cough

• Wheeze

• SOB

• Pallor

• Fatigue

• Cyanosis

• Fever

• Bubbling sensation in the chest

• Increased work of breathing

Hematemesis

-acidic, coffee-grounds, contains food material

-unless massive bleeding and then may be bright red

Hemoptysis

alkaline, frothy, bright red or rust color

Pulmonary Hemorrhage

• Fever

• Weight loss

• Choking episodes

• Family illnesses

• Recent trauma

• Travel

• Hx of chronic lung disease

• Hx of congenital or rheumatic

heart disease

• Chest pain

• Calf pain

• Drug use

• Hematuria

Pulmonary Hemorrhage

• Local or diffusely decreased breath sounds, cyanosis, and crackles on auscultation

• Dullness to percussion

• Calf tenderness

• Clubbing

• Murmur

• Pallor

• Bruising or bleeding gums

• Signs of trauma

• Thorough oral and nasopharynx exam

• CBC, ESR, coags (PT, PTT, INR)

• Sputum culture

• Urinalysis

• ANA and evaluation for rheumatologic disease

• Sweat chloride test

What labs/tests would you order if you suspect Pulmonary hemorrhage

• Consider nasopharyngoscopy

• CXR, CT, CT angiogram, bronchoscopy, echocardiogram

diagnostic studies for pulmonary hemorrhage

• Supportive care: Supplemental O2 & blood transfusions

• Mechanical ventilation with PEEP to tamponade bleeding

• Bronchoscopy with balloon catheter, iced saline lavage

• Embolization for bronchial arterial bleeds

• Identify underlying cause and treat

treatment of pulmonary hemorrhage

Pertussis

• Caused by Bordetella Pertussis, gram negative bacillus

• Incubation period is 6 days

• Classically called whooping cough

Pertussis

• A vaccine preventable disease

• Countries such as United Kingdom and Japan had shown increase when the vaccination rates declined

• High mortality rate is associated with infants who are not completely vaccinated

-Catarrhal Stage

-Paroxysmal Stage

-Convalescent Stage

the three stages of pertussis

Catarrhal Stage

What stage of Pertussis?

• Non specific symptoms like low grade fever and nasal secretions for 1-2

weeks

Paroxysmal Stage

What stage of Pertussis?

• Coughing in paroxysms during expiration which lasts for 2-4 weeks

• May have cyanosis, apnea, and choking during paroxysms

• Post-tussive emesis common

• Between fits children appear well and are afebrile

• Characteristic whoop sound with the cough

Convalescent Stage

What stage of Pertussis?

• Gradual resolution of symptoms in 1-2 weeks

• Coughing decreases but can persist for months

Apnea

in a neonate, ________________ can be the first presenting sign of pertussis

• PCR and nucleic acid amplification for the organism

• Lymphocytosis

• Chest X ray findings

• Perihilar infiltrates

how to diagnosis pertussis?

Antibiotics:

• Azithromycin, Clarithromycin

• Erythromycin is avoided due to association with pyloric stenosis

Treatment for Pertussis

give macrolide and booster DTap if last dose was more than 3 years ago

Post-exposure prophylaxis for pertussis (under 7)

give macrolide and Tdap if not previously received

Post-exposure prophylaxis for pertussis (greater than 7)

Pertussis

• Apnea

• Hypoxia

• Seizures

• Encephalopathy (permanent disability)

• Secondary bacterial infections

• Strep pneumonia, Haemophilus influenza, Staph aureus

• Pneumomediastinum

• Pneumothorax

• Retinal hemorrhages

• Epistaxis

• Hernias

Pertussis

-2, 4, 6, and 15 months

-between 4-6 years

When do you give DTaP vaccine?

11 years; 10 years

Tdap is given at ___________ and every ___________ as a booster

27-36 weeks gestation

if pregnant and previously fully vaccinated, when do you give Tdap vaccine?

Bronchiolitis

• Viral respiratory infection

• Leading cause of hospitalizations in

infants

• Associated with respiratory tract

inflammation with airway

obstruction with cellular debris and

mucus plugging leading to poor air

exchange

• Can be life threatening

• Very contagious

• Spread by respiratory droplets

Bronchiolitis

Etiologies of ______________________:

• RSV*

• Adenovirus

• Parainfluenza virus

• Rhinovirus

• Influenza virus

• Human Metapneumovirus

• Coronavirus

• Prematurity

• Chronic lung disease

• Congenital heart disease

• Neuromuscular disorders

• Immunodeficiency

high risk groups for bronchiolitis

Bronchiolitis

• 50% of children under the age of two experience _________________________

• Peaks between 2-6 months

• Typically seen during Dec to March

Bronchiolitis

• Rhinorrhea

• Cough

• Raspy breathing

• Low grade temperature

• Apnea

• Intercostal, subcostal or supraclavicular retractions

• Diffuse wheezes/crackles

• Grunting

• Cyanosis

Bronchiolitis

• Mild leukocytosis

• Viral Culture (typically not done unless really ill)

• Venous, Capillary, Arterial blood gas

• Hyperinflation of lung fields

• Respiratory monitoring (Pulse ox)

• Oxygen to keeps sats > 92%

• Antipyretics

• Hydration

treatment of bronchiolitis

Warn parents that symptoms usually worsen days 3-5 and then improve

what should you tell parents when explaining treatment for Bronchiolitis?

Monthly Palivizumab, RSV monoclonal antibody vaccination

prevention of Bronchiolitis

Respiratory Distress Syndrome (RDS)

Also known as hyaline membrane disease

Respiratory Distress Syndrome (RDS)

-occurs at the onset of breathing due to an insufficiency of pulmonary surfactant

• Surfactant is produced starting at 20 weeks gestation by the type II cells

• Increases and starts maturing around 32-34 weeks gestation

• Prenatally the Lecithin/Sphingomyelin ratio is a good predictor of lung maturity (> 2:1 ratio)

Respiratory Distress Syndrome (RDS)

Pathophysiology:

1. Decreased surfactant

2. Pulmonary artery vasopasm

3. Atelectasis

4. More perfusion than ventilation

5. pulmonary shunting/hypoxemia

6. Increased atelectasis decreases lung compliance

7. retractions

8. Hypercapnia, acidosis, hypoxia

Respiratory Distress Syndrome (RDS)

• Cyanosis/ Hypoxemia

• Tachypnea

• Nasal flaring

• Intercoastal or subcoastal retractions

• Grunting

Respiratory Distress Syndrome (RDS)

Imaging shows characteristic low lung volume and diffuse reticulogranular ground glass appearance with air bronchograms

• Administration of betamethasone in mother's who are at risk for premature

delivery

• Intratracheal administration of exogenous surfactant

• Supported ventilation

• Antibiotics: Ampicillin/Gentamicin

treatment for RDS

• Bronchopulmonary dysplasia (BPD)

• Pulmonary air leaks

• Retinopathy of prematurity

complications of RDS

Bronchopulmonary Dysplasia (BPD)

• Condition of chronic lung disease due to disruption of pulmonary development and injury in preterm infants

• Infants with lung disease of prematurity who require supplemental oxygen >28 days

Bronchopulmonary Dysplasia (BPD)

• Grunting

• Nasal flaring

• Retractions

• Should be suspected in neonates who are still requiring oxygen even after

their due date

Bronchopulmonary Dysplasia (BPD)

• Pulmonary

Hypertension

• Cor Pulmonale

complications of BPD

• Ventilation

• Surfactant

• Nitric Oxide

• Corticosteroids

• Supportive: Feeding, Hydration,

Nutrition

treatment of BPD

Pneumonia

• Infection and inflammation of the lung parenchyma associated with infiltrates on CXR

• Patients often present with fever, cough, dyspnea

• Physical exam may reveal decreased breath sounds, crackles (rales), tachypnea, and respiratory distress

Pneumonia

Diagnosis is suggested by:

• Infiltrates on CXR (usually interstitial for viral and lobar for bacterial)

• Elevated WBC count (lymphocyte predominance for viral, neutrophil for bacterial)

Maternal Flora (Group B Strep) (S. pneumoniae)

etiology of pneumonia in neonates (0-3 months)

Viral Respiratory Infections

etiology of pneumonia in children 3 months to 5 years

Atypical Organisms (M pneumoniae and

Chlamydophila pneumonia)

etiology of pneumonia in school aged children (>5 yrs)

Mycoplasma pneumoniae

leading cause of bacterial pneumoniae in school aged children and young adults

-Antibiotics (if bacterial)

-Oxygen and Fluids

treatment of pneumonia