1 topic 1 differential diagnosis of pneumonia in children. complications of pneumonia ... ( pdf drive )

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Topic 1: Differential diagnosis of pneumonia in children. Complications of pneumonia.
Emergency care in acute respiratory failure in children.
Thematic chapter: Differential diagnosis of the most common respiratory tract diseases in
childhood. Emergency care in common emergency conditions.
Academic hours: 6
Self-education: 4
1. SIGNIFICANCE
Primary infection of the parenchyma of the lung is much less common than secondary
bacterial infection complicating the acute viral bronchitis that occurs during minor upper
respiratory infection. Bacterial pneumonia during childhood and recurrent pneumonia in the
absence of an underlying chronic illness, such as cystic fibrosis or immunologic deficiency, is
quite unusual.
Therefore, student encountered pneumonia should think of the patient as if he/she has
accompanying condition to rule out any possibilities of subjecting the patient at risk of
complications.
2. PREREQUISITES
The skills listed below will not be taught in this lesson but are necessary to perform physical
examination of the patient practical training. Therefore, before beginning this lesson, one has to
be sure of the ability to: inspect the thorax and its respiratory movements and note rate, rhythm,
depth, and effort of breathing; observe retractions of the supraclavicular areas of contractions of
the sternomastoid muscles on respiration; observe shape of the child’s chest; auscultate to child’s
breathing for increased white noise and wheezes; palpate the chest for respiratory expansion,
tactile fremitus; percuss the chest in the standard areas, comparing one side with the other at each
level; auscultate to the chest with stethoscope in order to evaluate breath sounds and not any
adventitious sounds.
3. EDUCATIONAL OBJECTIVES
Student should know:
- etiology, pathogenesis, classification, clinical manifestation, differential diagnosis and
treatment principles for acute bacterial and viral pneumonia in children and infants.
Student should be able:
- to identify the child with acute pneumonia, make correct decisions during physical examination
of the patient with given conditions, take appropriate actions based on those decisions,
demonstrate skills to develop management and follow up measures.
4. INTERDISCIPLINARY INTEGRATION
Discipline Student should know Student should be able to
Normal anatomy,
Physiology
Anatomic and physiologic
features of the respiratory
system in children of different
age groups
Use knowledge of anatomic and
physiologic features of the
respiratory system in children
for evaluation of clinical
findings
Biochemistry Normal ranges for the routine
biochemical blood analysis
Assess blood biochemistry and
comment on deviations from

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normal in a clinical context
Pathology Histologic and histochemical
presentation of the respiratory
tract illnesses in children
Use knowledge of histologic
and histochemical presentation
of the respiratory tract illnesses
in children for evaluation of
clinical findings
Pathologic physiology Pathophysiologic mechanisms of
the respiratory failure
Recognize symptom and signs
of respiratory failure
Microbiology Sampling of sputum, pleural
excudate, blood for bacterial
cultures
Assess microbiologic findings
in clinical context
Propedeutics of pediatric
diseases
Physical examination of the
respiratory system in children.
Correct performance of
pulmonary tests.
Perform physical examination
of the respiratory system (gross
inspection, palpation,
percussion, auscultation),
Assess the results of pulmonary
tests
Imaging studies Indications and methods of
imaging studies in respiratory
tract illnesses
Assess radiologic examination
of the chest
Intensive care Symptoms and signs of
respiratory failure of different
stages, its etiology, and
principles of intensive care
Recognize respiratory failure,
assess its severity, provide
emergency care
5. ABSTRACT FOR PRE-WORKSHOP SELF-EDUCATION
1. Pneumonia in children.
Etiology. Neonates Group B Streptococcus, mechanically ventilated and gram-negative. Ages 2
mo to 2 y S. pneumonia. Type B Haemophilus influenza. Bordetella pertussis (unimmunized).
Ages 2– 5 y S. pneumonia. Type B Haemophilus influenza. Staphylococcus. Age >5 y
Mycoplasma pneumonia. Nosocomial in institutionalized children. Anaerobic bacteria. S. aureus.
Predisposing conditions:
 Congenital anomalies (cleft palate, tracheoesophageal fistula, sequestration).
 Congenital defects of immune system, sickle cell disease.
 Cystic fibrosis.
 Otherwise normal, healthy children, with younger children 2 y of age more susceptible.
 May follow epidemic of viral infections.
 Human transmission via droplet spread more common in winter.
Symptoms.
 Cough, fever, chest pain, dyspnea.
 Abdominal pain, nausea, vomiting: less common.
Signs.
 Increased respiratory rate, grunting, retractions.
 Dullness on percussion.
 Crackles, abdominal pain, distention, dehydration.
Investigations.
 Chest radiography: anteroposterior and lateral.
 Decubitus: if suspect effusion.

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Note: Chest radiograph resolution can lag behind physical findings.
To assess severity.
 Arterial blood gas: low PO2, inversed PCO2, decreased pH.
 Pulse oximetry: helpful for O2 saturation.
 Complete blood count: white blood cell count >15,000 often found (<5000 can be associated
with severe infection).
To assess cause.
 Blood culture: will be positive in less than 15% of cases.
 Serology: cold agglutinins (mycoplasma).
 Acute: convalescent titers for specific antibodies.
 Serum stain and culture of sputum: usually difficult to obtain.
Complications.
 Respiratory failure.
 Pleural effusion.
 Lung abscess.
 Pneumothorax.
 Empyema.
 Pneumatocele.
 Bacteremia.
 Sepsis.
Differential diagnosis.
Acute anemia, acute respiratory distress syndrome, afebrile pneumonia syndrome,
agammaglobulinemia, airway foreign body, alveolar proteinosis, aortic stenosis, aspiration
syndromes, asthma, atelectasis, pulmonary, bacteremia, bronchiectasis, bronchiolitis, bronchitis,
bronchogenic cyst, cardiomyopathy, hypertrophic, chronic anemia, chronic granulomatous
disease, combined B-cell and T-cell disorders, common variable immunodeficiency, congenital
diaphragmatic hernia, congenital pneumonia, congenital stridor, cystic fibrosis, ebstein anomaly,
empyema, foreign body aspiration, gastroesophageal reflux, goodpasture syndrome,
hemosiderosis, hemothorax, hypersensitivity pneumonitis, IgA and IgG subclass deficiencies,
legionella infection, meningitis, bacterial, patent ductus arteriosus, pediatric respiratory distress
syndrome, pertussis, pleural effusion, pneumococcal infections, pneumococcal infections,
pneumonia, aspiration, pneumonia, bacterial, pneumonia, empyema and abscess, pneumonia,
immunocompromised, pneumonia, mycoplasma, pneumothorax, pulmonary atresia with
ventricular septal defect, pulmonary hypoplasia, pulmonary sequestration, respiratory distress
syndrome.
Treatment aims:
 To improve oxygenation.
 To achieve rapid resolution.
 To maintain nutritional support.
Pharmacologic treatment.
 Oxygen to maintain PO2 >60 mmHg.
 Oral or parenteral fluids to correct dehydration.
 Initial treatment with antibiotics is empirical.
 Oral antibiotics are appropriate in mild infection, parenteral if severe infection or if vomiting.
 Bronchodilator may be of benefit.
 Treatment is for at least 7 days.
Infants
Standard dosage. Ampicillin, 25– 50 mg/kg i.v. every 6 h. Gentamicin, 2.5 mg/kg i.v. every 12
(0– 7 d of age) to 8 (>7 d of age) h. After sepsis or meningitis is ruled out, the antibiotic regimen
may be adjusted to the specific organism isolated and/or site involved.
Older children.

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Standard dosage Empiric therapy until etiology established (especially in children <5 y old).
Cefuroxine, 100– 150 mg/kg/d i.v. or i.v. divided into every 8-hour doses, or Ampicillin, 150
mg/kg/d i.v. or i.v. divided into every 6-hour doses plus chloramphenicose, 50– 75 mg/kg/d i.v.
divided into every 6-h doses, or Ceftriaxone, 50 mg/kg/d i.v. or i.v. once daily. The antibiotic
regimen is adjusted according to organism isolated, resistance pattern, and types/sites of
infection finally identified.
Outpatient.
Standard dosage Penicillin V, 25– 50 mg/kg divided every 8 h. Erythromycin, 20– 40 mg/kg
divided doses every 8 h. Second generation cephalosporin or semisynthetic macrolides.
Clarithromycin, 15 mg/kg divided in doses every 12 h, or Azithromycin, 10 mg/kg first day, then
5 mg/kg/d for 4 d.
 Specific treatment Mycoplasma: erythromycin.
 Gram-negative pneumonia: must consider aminoglycoside.
 Anaerobic: penicillin or clindamycin.
Prevention.
Prophylactic antibiotics and vaccinations for children at elevated risk (H. influenza type B for all
children; pneumococcal vaccine for immunocom-promised and sickle cell patients).
Prognosis.
 Pyrexia usually resolves within 48 h of beginning antibiotics.
 Radiographic findings are slow to clear and lag behind clinical recovery.
 Prognosis is excellent in uncomplicated cases of Pneumococcal pneumonia, but longer
recovery with other pathogens.
 Bacterial resistance and poor response to choice of antimicrobial requires reassessment.
Follow-up and management.
 Patients should be seen routinely following treatment completion.
 Follow-up chest radiography indicated for child with recurrent pneumonias, persistent
symptoms, severe atelectasis, unusually located infiltrates, pneumothorax, or effusion.
 Admit to hospital if 1) patient presents dehydration, hypoxia, or significant respiratory distress;
2) outpatient management fails (24– 72 hours), or if 3) less than 6 mo of age.
2. Pleural effusion
Etiology.
There is normally 1– 15 cm3
of fluid in the pleural space. Alterations in the flow and/or
absorption of this fluid leads to accumulation. Six mechanisms influence fluid flow:
 Increased capillary hydrostatic pressure.
 Decreased pleural space hydrostatic pressure.
 Decreased plasma oncotic pressure.
 Increased capillary permeability.
 Impaired lymphatic drainage from the pleural space.
 Passage of fluid from the peritoneal cavity through the diaphragm to the pleural space.
There are two types of pleural effusion are described:
 Transudate: mechanical forces of hydrostatic and oncotic pressures are altered favoring liquid
filtration.
 Exudate: damage to the pleural surface occurs that alters its ability to filter pleural fluid
lymphatic drainage is diminished.
Epidemiology.
For empyema, major organisms include: Staphylococcus aureus, 28%. Streptococcus
pneumoniae, 20%. Haemophilus influenzae, 13%.
Symptoms.

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Basic disease determines most of systemic symptoms. Patients may be asymptomatic until
amount of fluid is large enough to cause cardiorespiratory distress. Dyspnea, cough, fever, and
pleuritic pain.
Signs.
Dependent on the size of the effusion; may be normal in small effusions.
Pleural rub: during early phase; resolves as fluid accumulates the in pleural space.
Decreased thoracic wall excursion, dull or flat percussion, decreased tactile and vocal fremitus,
fullness of intercostal spaces, decreased breath sounds on affected side, trachea and cardiac apex
displaced toward the contralateral side, tachycardia, tachypnea, shortness of breath, and
respiratory distress.
Investigations.
 Chest radiograph: to determine the size of the effusion.
 Upright films: anteroposterior projection can see >400 cm3
; lateral projection can see >200 cm3
 Lateral decubitus films: can check for free-flowing pleural fluid; can see as little as 50 cm3
of
fluid.
 Ultrasound: can diagnose small (>10 cm3
) small amount of pleural fluid; useful as a guide for
thoracentesis; can distinguish between pleural thickening and pleural effusion.
 Computed tomography scan: useful for defining extent of demarcated effusions; visualizes the
underlying lung parenchyma.
 Thoracentesis: indicated whenever etiology is unclear or the patient is symptomatic.
 Pleural fluid analysis.
 Sedimentation rate (ESR): to follow degree of inflammation and response to therapy.
 Pleural biopsy: if thoracentesis is non-diagnostic; most useful for diseases that cause extensive
involvement of the pleura (ie, tuberculosis, malignancies) confirms neoplastic involvement in
40%– 70% of cases.
Complications.
 Hypoxia.
 Respiratory distress.
 Trapped lung (secondary to a constrictive fibrosis) with restrictive lung diseases.
 Decreased cardiac function.
 Shock: secondary to blood loss in cases of hemothorax.
 Malnutrition: seen in chylothorax.
Transudate: Cardiovascular: congestive heart failure, constrictive pericarditis. Nephrotic
syndrome with hypoalbuminemia. Cirrhosis. Atelectasis.
Exudate: Infections: parapneumonic effusions (Staphylococcus aureus is most common
organism), tuberculous effusion, viral effusions (adenovirus, influenza), fungal effusions (most
not associated with effusions; Nocardia and Actinomyces are most common), and parasitic
effusions. Neoplasms: uncommon in children; seen mostly in leukemia and lymphoma.
Connective tissue disease. Pulmonary embolus: intraabdominal disease, subdiaphragmatic
abscess, pancreatitis. Others: sarcoidosis, esophageal rupture, hemothorax, chylothorax, drugs,
chemical injury, post-irradiation effusion.
Pleural fluid analysis
Test Transudate Exudate
pH
Protein, g/100 cm 3
Pleural/serum protein
Pleural/serum LDH*
LDH, IU
Pleural/serum amylase
Glucose, mg/dL
7.4
<3.0
<0.5
<0.6
<200
<1
>40
<7.3
>3.0
>0.5
>0.6
>200
>1
<40

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Red blood cell count, mm3
White blood cell count, mm3
<5000
<1000
>5000
>1000
*LDH— lactate dehydrogenase
Treatment aims:
 To remove pleural fluid.
 To decrease respiratory distress.
 Earlier intervention is associated with a better prognosis.
 Prevent long term complications, such as trapped lung.
Diet and lifestyle.
 In general, no special precautions are necessary.
 If a chylothorax is present, patient’s diet should include medium chain triglycerides as the
primary source of fat for 4– 5 weeks.
Antibiotics are administered depending on the organism identified and degree of illness. Clinical
improvement usually occurs in 48– 72 h.
 Staphylococcus aureus: 3– 4 wk minimum.
 Streptococcus pneumoniae: 2 wk minimum.
 Haemophilus influenzae: 2 wk minimum.
Patients should remain on i.v. antibiotics until afebrile. Complete remainder of therapy on oral
antibiotics (usually between 2– 4 wk total of i.v. and oral treatment).
Drainage.
 Thoracentesis: for diagnosis and relief of dyspnea or cardiorespiratory distress.
 Chest tube drainage: for large effusions; reduces reaccumulation; drainage of empyema.
Drainage should be stopped when patient is asymptomatic. Thick, loculated empyema requires
prolonged drainage.
 Fiberoptic thoracoscopy: to remove the pleural rind to aid with chest tube drainage; helpful if
loculated effusions are present.
 Open thoracotomy with rib resection: performed less now that fiberoptic thoracoscopy is
available.
 Decortication: symptomatic chronic empyema; relief of thick fibrous peal. Pleurectomy:
chylothorax, malignant effusions.
Prognosis.
Prognosis is dependent on underlying disease process. If properly treated for infectious etiology:
excellent. If malignancy: poor.
Follow-up management.
Clinical improvement usually occurs within 1– 2 wk. Fever spikes may last up to 2– 3 wk. It
may take up to 6 mo for the chest radiograph to normalize, dependent on the extent and etiology
of the effusion (serial films are helpful at following the patient’s course). Pulmonary function
tests will start to normalize as the underlying cause resolves; dependent on the extent and
etiology of the effusion.
3. Treatment of acute respiratory failure
Management of acute respiratory failure begins with a determination of the underlying etiology.
For extrathoracic airway obstruction, such as croup, the following measures may be helpful:
 Inspired humidity to liquefy secretions
 Heliox (helium and oxygen gas mixture) to decrease work of breathing
 Racemic epinephrine 2.25%, an aerosolized vasoconstrictor
 Systemic corticosteroids to decrease airway edema
Endotracheal intubation.

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It is needed to maintain airway patency in certain cases (e.g., epiglottitis, thermal burns to the
airway, severe croup).
Tubes:
 In neonates and infants younger than 6 months, an endotracheal tube with an inner diameter
(ID) of 3.5-4 mm is appropriate.
 In infants aged 6-12 months, a tube with a 4-4.5 mm ID is appropriate.
 Weight is the traditional guide to determine appropriate endotracheal tube size in infants and
children, and many emergency departments have a color-coded emergency equipment cart
organized by weight for easy access.
 A useful bedside or field guideline for appropriate endotracheal tube size is approximately the
size of the patient’s fifth finger.
The mnemonic MSOAPP can be used to remember the preparation essential for a safe tracheal
intubation procedure, as follows:
M - Monitors (heart rate, blood pressure, pulse oximetry, capnography for CO2 detection)
S - Suction and catheters
O - Oxygenation with a bag-valve mask
A - Apparatus (laryngoscope, endotracheal tubes appropriate for the patient's age and a half-size
smaller and larger, stylets, oral airways)
P - Pharmacy (medications for amnesia and paralysis)
P = People (respiratory therapist, nurse, a skilled set of hands)
 The initial treatment for hypoxemia is to provide supplemental oxygen.
 High-flow (>15 L/min) oxygen delivery systems include a Venturi-type device that places an
adjustable aperture lateral to the stream of oxygen.
 Oxygen is mixed with entrained room air, and the amount of air is adjusted by varying the
aperture size.
 The oxygen hoods and tents also supply high gas flows. Low-flow (< 6 L/min) oxygen
delivery systems include the nasal cannula and simple face mask.
6. MATERIALS FOR METHODOLOGICAL BACKGROUND OF THE WORKSHOP
6.1. Quiz
1. What is etiology of bacterial pneumonia in ages 2 mo to 2 yr?
2. What is etiology of bacterial pneumonia in ages 2-5 yr?
3. What is etiology of bacterial pneumonia in age above 5 yr?
4. What conditions predispose to bacterial pneumonia?
5. What will you do to assess severity of bacterial pneumonia?
6. What will you do to assess cause of acute bacterial pneumonia?
7. List complications of an acute bacterial pneumonia?
8. Explain pharmacologic treatment of an acute bacterial pneumonia.
9. How neonates with acute bacterial pneumonia are treated?
10. How older children with acute bacterial pneumonia are treated?
11. What is outpatient treatment of acute bacterial pneumonia?
12. What is the drug of choice for M. pneumoniae?
13. What is the drug of choice for acute bacterial pneumonia of anaerobic etiology?
14. What prevention measures can be applied for pneumonia?
15. What is etiology of viral pneumonia?
16. What is epidemiology of viral pneumonia?
17. List symptoms of viral pneumonia.
18. Do these differ from ones in bacterial pneumonia?
19. What is investigation in viral pneumonia?
20. List complications brought by viral pneumonia?
21. What is differential diagnosis for viral pneumonia?

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22. What is the treatment aims in viral pneumonia?
23. What is pharmacologic treatment for viral pneumonia?
24. Can viral pneumonia be prevented?
6.2. Multi-choice questions
1. What is specific pathogen which causes acute pneumonia in neonate?
A. S. pneumoniae
B. Group B Streptococcus*
C. Type B H. influenzae
D. M. pneumoniae
E. Clamidia trachomatis
6.3. Sample case report
The boy 15 y.o. presents with a chief complaint of coughing, dyspnea. The patient
experienced coughing and a runny nose which later led to a fever Within a few days the fever
had increased, the patient had a rapid heart beat, chest pain with difficulty breathing, shaking
chills and crushing fatigue. His cough began producing mucous and his father started him on a
regime of ampicillin. On physical exam skin is pale; RR 20/min.; chest shape is normal with
symmetric expansion on respiration with use of accessory muscles, fremitus reduced to the left
subscapular are on palpation, percussion is reduced resonant to the left subscapular area. On
auscultation regular clear breathing with vesicular sound heart over most of lung fields with fine
crackles to the left subscapular area.
Labs. WBC 3.7x G/L; Eosinophils 1%; Bands 15%; Segment 61%; Lymphocytes 21%;
Monocyte 2%; ESR 40 mm/h; RBC 3.78 x 1012
/L; Hb 12.1 g/dL ; Hct36.2% (normal 35 -
45%); MCV 90.5 fl (normal 78 - 98 fl); MCH 32.0 pg (normal 25-35 pg); MCHC 35.4 g/dL
(normal 31 -36 g/dL); PLT 190x 109
/L (normal 180-320 x 109
/L); Biochemical analysis: K+
3.5 mmol/l; Na+ 140 mmol/l; Alt 35.1 IU; Ast 39.6 IU; glucose 5.2 mmol/l; bun 3.8 mmol/l;
creatinine 74.5 mcmol/l bilirubin 5.8 mcmol/l.
Chest X–ray: At background of the increased markings, there are foci of infiltration in the
lower part of the left lung. Diaphragmatic dome is clear, sinuses without liquid. Left pulmonary
root is slightly deformed.
1. What is the diagnosis?
2. What is the differential diagnosis?
3. What is the follow-up?
Suggested reading
1. Sandora T.J., Sectish T.C. Community acquired pneumonia / in Nelson Textbook of
Pediatrics 19th ed. by Kliegman R.M., Stanton B., Geme J.S. / Saunders, 2011.- P. 1474-
1479.
2. Winnie G.B., Lossef S.V. Pleurisy, pleural effusions, empyema / in Nelson Textbook of
1509.
3. Sarnaik A.P., Clark J.A. Respiratory distress and failure / in Nelson Textbook of
333.
4. Bennett N.J., Domachowske J. Pediatric pneumonia /
http://emedicine.medscape.com/article/967822
Additional reading

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1. Barnett ED, Klein JO. Bacterial infections of the respiratory tract. Remington JS, Klein
JO, eds. Infectious Diseases of the Fetus and Newborn Infant. 6th ed. Philadelphia, Pa:
Elsevier Saunders Co; 2006. 297-317.
2. Bradley J.S., Byington C.L., Shah S.S., et al. The management of community-acquired
pneumonia in infants and children older than 3 months of age: clinical practice guidelines
by the Pediatric Infectious Diseases Society and the Infectious Diseases Society of
America // Clin. Infect. Dis.- 2011.- 53(7).- P. 25–76.
3. Mandell L. Community acquired pneumonia: new guidelines on management in primary
care // BMJ.- 2010.- 341.- P. 59–60.
4. Ruuskanen O., Lahti E., Jennings L.C., et al. Viral pneumonia // Lancet.- 2011.- 377.- P.
1264–1274.
5. Wunderink R.G., Waterer G.W. Community-acquired pneumonia // N. Engl. J. Med. –
2014.- 370.- P. 543–550.

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Topic 2: Differential diagnosis of bronchial obstruction in children. Differential approach
to treatment of bronchial obstruction in children. Emergency care in status asthmaticus.
Academic hours: 6
Self-education: 4
1. SIGNIFICANCE
Differential diagnosis of bronchial obstruction in children is one of the challenging issues in
pediatrics as etiology is very variable that dictates quite different approaches to the treatment.
However, bronchitis, bronchiolitis, and asthma remain the most common problems of bronchial
obstruction and should serve a background in differential diagnosis. There are a number of acute
and chronic disorders that present with coughing and wheezing, therefore the student should be
careful in clinical evaluation of the child with bronchitis or bronchiolitis.
2. PREREQUISITES
be sure of the ability to: inspect the thorax and its respiratory movements and note rate, rhythm,
depth, and effort of breathing; observe retractions of the supraclavicular areas of contractions of
the sternomastoid muscles on respiration; observe shape of the child’s chest; auscultate to child’s
breathing for increased white noise and wheezes; palpate the chest for respiratory expansion,
tactile fremitus; percuss the chest in the standard areas, comparing one side with the other at each
level; auscultate to the chest with stethoscope in order to evaluate breath sounds and not any
- differential diagnosis in a context of etiology, pathogenesis, classification, clinical
manifestation of for the obstructive disorders in children and infants.
- to identify the child with obstructive disorder, make correct decisions during physical
examination of the patient with given conditions, take appropriate actions based on those
decisions, demonstrate skills to develop management and follow up measures.
Normal anatomy,
Physiology
features of airways in children
of different age groups
findings

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presentation of
bronchoobstructive illnesses in
children
of bronchoobstructive illnesses
clinical findings
diseases
pulmonary tests.
tests
imaging studies in
bronchoobstructive illnesses
of the chest
emergency care
1. Differential diagnosis of bronchial obstruction in children
Airway Foreign Body History of a distinct coughing or choking episode: occurs in a majority
of cases.
Sudden onset of respiratory distress.
Acute or chronic cough.
Hoarseness or aphonia.
• Some patients with small subglottic foreign bodies may be
asymptomatic.
Laryngeal or tracheal foreign bodies
• Total obstruction results in severe respiratory distress with cyanosis,
supraclavicular and substernal retractions, aphonia, ineffective cough,
and absent or very diminished breath sounds, with or without loss of
consciousness.
• Partial obstruction results in stridor, cough, dysphonia, drooling and
supraclavicular and/or substernal retractions.
Bronchial foreign bodies
• The classic triad of cough, wheezing, and focally decreased breath
sounds occurs in only 30% of patients.
• Respiratory distress is manifested as tachypnea, intercostal
retractions, and cough.
• 20% of patients are asymptomatic at presentation.
Allergic Rhinitis Symptoms
Ocular pruritus.
Ocular discharge and tearing.
Photophobia.
Above with sneezing.
Nasal itching and/or congestion.

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Rhinitis.
Signs
Eyelid edema: as lacrimation.
Chemosensitivity: with injection with nasal edema and secretions.
Aspergillosis Patients with allergic bronchopulmonary aspergillosis (ABPA) often
have histories of worsening respiratory symptoms in association with
asthma or cystic fibrosis (CF).
ABPA occurs in approximately 11% of patients with CF.
The main complaints of these patients are wheezing and cough.
As the disease progresses, patients may expectorate mucous plugs
containing eosinophils, and they may develop bronchiectasis.
Exacerbation and remission characterize the natural history of disease.
Progression to respiratory failure may occur occasionally because of
irreversible airway obstruction and pulmonary fibrosis.
It may mimic pneumonia with mucopurulent bloody sputum, fever,
and respiratory distress.
Predominant wheezing may be the only manifestation suggesting an
exacerbation of bronchial asthma.
Aspergillomas may remain asymptomatic until hemoptysis occurs.
Bronchiectasis Should include differential diagnosis of:
Aspiration Pneumonia
Bacterial Pneumonia
Chronic Obstructive Pulmonary Disease
Emphysema
Parapneumonic Pleural Effusions and Empyema Thoracis
Pediatric Asthma
Pediatric Bronchitis
Pediatric Cystic Fibrosis
Tuberculosis
Bronchiolitis SEE DETAILS BELOW
Bronchopulmonary
Dysplasia
Abnormal findings on physical examination, and chest radiography
Initial findings observed shortly after birth consistent with respiratory
distress syndrome (RDS).
Persistence of these abnormalities can be associated with an increased
risk of bronchopulmonary dysplasia.
Physical examination may reveal tachypnea, tachycardia, increased
work of breathing (with retractions, nasal flaring, and grunting),
frequent desaturations, and significant weight loss during the first 10
days of life.
Infants with severe bronchopulmonary dysplasia are often extremely
immature and had a very low birth weight.
Their requirements for oxygen and ventilatory support often increase
in the first 2 weeks of life
Cystic Fibrosis Presence of typical clinical features (respiratory, gastrointestinal, or
genitourinary)
OR
A history of CF in a sibling
OR
A positive newborn screening test
PLUS
Laboratory evidence for CF transmembrane regulator (CFTR)
dysfunction:

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Two elevated sweat chloride concentrations obtained on separate days
OR
Identification of two CF mutations
OR
An abnormal nasal potential difference measurement
Gastroesophageal
Reflux
See relevant topic in the manual
Primary Ciliary
Dyskinesia
Clinical manifestations vary.
Chronic persistent rhinorrhea, sensation of local fullness, and sinus
pain
Anosmia, nasal character of speech, and halitosis
Recurrent acute otitis
Chronic otitis
Recurrent sinusitis
Male infertility (common)
Chronic productive cough and respiratory distress, especially in infants
Bronchospastic symptoms (e.g., wheeze and cough), usually
responsive to bronchodilator therapy
Recurrent or persistent atelectasis or pneumonia
Abnormal histology findings
2. Bronchiolitis.
Etiology.
Bronchiolitis is an acute inflammatory process causing obstruction of the small conducting
airways and a manifestation of lower respiratory tract obstruction. RSV is the most common
cause (70% of bronchiolitis cases and 40% of pneumonia in younger children). Other infectious
etiologies include parainfluenza virus (second most common cause of bronchiolitis; more often
associated with croup, tracheobronchitis, and laryngitis), influenza virus, adenovirus, rhinovirus,
Mycoplasma pneumoniae, Chlamydia, and ureaplasma. Pneumocystis carinii is rarely associated
with wheezing in infancy. RSV causes epithelial damage and elicits a mononuclear cell infiltrate
and peribronchiolar edema. Those predisposed to the development of reactive airways (asthma)
may develop RSV-specific immunoglobulin-E (IgE) responses, presumably because of a high
IgE-responder phenotype. Notably, 30%– 40% of patients who develop severe wheezing with
RSV later show a tendency to wheeze repeatedly.
Epidemiology.
RSV epidemic peaks from late fall to early spring. Up to 40% of primary infections result in
febrile pneumonitis, but only 1% require hospitalization. Family studies indicate that nearly 70%
of children are infected in the first year of life; by 24 months, nearly all children have been
infected at least once. Beyond the first year, the clinical severity diminishes, changing from
bronchiolitis and pneumonia to predominantly trachea-bronchitis and reactive airway events.
Transmission is by droplets or fomites. The virus can remain infectious for
hours on surfaces. Hospital-acquired infections are common.
Symptoms.
 Prodrome: 1– 2 days of fever, rhinorrhea, mild cough.
 Apnea: may occur early before full intensity of chest symptoms.
 Persistent, increased cough: may later be productive.
 Rapid respirations.
 Skin color changes: rashes (rare).
 Poor feeding, lethargy.
Signs.
 Findings of rhinitis: occasionally otitis, conjunctivitis.
 Pharyngitis, hoarseness.

14
 Tachypnea with usually shallow respirations.
 Tachycardia: especially when hypoxemia present.
 Fever: usually milder later in course.
 Nasal flaring, retractions, and hyperinflation.
 Wheeze, increased expiratory phase, and rales and/or rhonchi.
 Palpable liver and/or spleen: secondary to hyperinflation.
 Cyanosis: Note: poor correlation with hypoxemia.
 Vomiting (post-tussive).
 Evidence for dehydration may be present: secondary to poor oral intake.
Investigations.
 Complete blood count: with differential (no specific findings).
 Pulse oximetry.
 Arterial blood gas if respiratory failure appears imminent: severe hypoxia, raised or rising
partial pressure of carbon dioxide.
 Rapid viral identification: usually a ―respiratory panel‖ is available to include common
seasonal respiratory pathogens by antigen detection.
 Viral culture: results delayed but may be useful to identify causative organism.
 Serologic diagnosis: paired samples needed; rarely indicated as clinical diagnosis is usually
evident.
Chest radiograph.
 Interstitial pneumonitis: the most typical finding; usually diffuse but may be segmental.
 Hyperaeration: also typical and may be the only finding.
 Peribronchial thickening: common but may not be related to the primary infection.
 Consolidation: occasional in hospitalized patients; usually is subsegmental.
Complications.
 Otitis media: is most common (secondary; bacterial).
 Pneumonia: secondary, bacterial; occurs in < 1% of hospitalized cases.
 Apnea.
 Respiratory failure.
 Cardiac failure: secondary to pulmonary disease or rarely myocarditis.
 Bronchiolitis obliterans: rare; usually associated with adenovirus-induced
bronchiolitis/pneumonia.
Differential diagnosis
Pneumonia (viral, bacterial), Chlamydia pneumonitis, asthma, foreign body, cystic fibrosis,
and pertussis. Gastroesophageal reflux. The diagnosis is evident when, during an epidemic
period, an infant presents with tachypnea, diffuse wheeze, and hyperinflation (radiograph) after a
febrile upper respiratory illness.
Treatment.
Treatment aims:
 To adequately monitor until resolution.
 To maintain oxygenation.
 To assess reversibility of airway obstruction (bronchodilator response).
 To avoid complications of treatment.
 To identify high-risk patients.
During the acute illness, routine health maintenance and respiratory care is provided at home
or in hospital. Home management for mildly symptomatic patients is recommended. Adequate
hydration should be assured. For significant wheeze or work of breathing, bronchodilator
treatment (e.g., albuterol) may be tried. Reassess infant with increased respiratory distress and
tachycardia (oxygen saturation).

15
Corticosteroids: although controversial, a trial is reasonable for hospitalized patients in
whom bronchodilator responsiveness is documented. Theophylline: not useful as a
bronchodilator but may be helpful for management of apnea. Antibiotics: not indicated unless
secondary bacterial infection detected. Ribavirin treatment: this is controversial because of
concerns regarding cost, benefit, safety and quite variable clinical efficacy (conflicting chemical
trials); may be considered for patients who are at risk for severe or fatal infections, but no
definitive indications have been established.
Immunoprophylaxis: RSV-intravenous immunoglobulin (RSV-IVIg) is approved for
prevention of RSV disease in 1) children less than 2 years of age with bronchopulmonary
dysplasia (BPD) who have been oxygen dependent at least 6 months prior to oncoming RSV
season, and 2) selected infants with prematurity (gestational age < 32 weeks at birth) without
BPD.
Intravenous hydration: monitor intake and output; avoid excessive hydration and aspiration.
Consider use in severely immunodeficient patients (primary disorder such as severe combined
immunodeficiency or severe HIV). RSV-IV-Ig is given at a dose of 750 mg/kg once per month
beginning just before and monthly during the RSV season.
Nonpharmacologic treatment.
Infant showing inability to feed, severe respiratory distress, and/or hypoxemia should be
hospitalized. Supplemental oxygen for saturation < 92%; titrate inspired oxygen to achieve
>95% saturation. Note: nasal cannula may not effectively deliver oxygen if nasal passage not
patent or patient mouth breaths. Bronchodilator trial: all hospitalized patients should receive a
trial of an aerosolized beta-2-agonist for potential relief of obstruction. Monitor oxygen
saturation concurrently as hypoxemia may worsen in some patients. Improvement suggests
continuation may be beneficial, and that airway hyperreactivity associated with asthma may be
present. Careful monitoring of vital signs and clinical status as patient may worsen during
inpatient stay; use both electronic instruments and direct visual contact; include oximetry, and
confirm progression to respiratory failure with arterial blood gas. Hospitalized patients should be
isolated and may be cohorted in the same room. Use gown, gloves, and careful hand washing.
Additional control measures may be advisable beyond patient isolation measures. Consider
laboratory screening for RSV infection in patients, cohorting medical staff, exclusion of infected
staff from contact with high-risk patents, and limitations on visitation.
Prognosis.
Acute severe obstructive symptoms usually resolve in 3– 5 days, but cough and fatigue may
last up to 14 days. Complete recovery expected for most patients.
Patients with recurrent episodes of wheeze (obstruction) often found to have reactive airway
disease. Chronic lung disease or other complications are rare in otherwise normal hosts.
Follow-up and management.
Most cases are uncomplicated and require no follow-up unless reactive air-way disorder is
uncovered, complications arise, or the patient has another disorder that causes increased risk for
severe or fatal RSV infection.
3. Differential approach to the treatment airway of obstruction
Cystic Fibrosis Pulmonary therapy - to clear secretions from airways and to control
infection;
Inhalation therapy - to deliver medications and hydrate the lower
respiratory tract;
The basic aerosol solution is 0.9% saline;
In reactive airways, albuterol or other β agonists are added;
When the airway pathogens are resistant to oral antibiotics or when
the infection is difficult to control at home, aerosolized antibiotics

16
may reduce symptoms, improve pulmonary function, and alleviate the
need for hospitalization;
Human recombinant DNase for mucopurulent exudate;
N-acetylcysteine, is toxic to ciliated epithelium, and repeated
administration should be avoided;
Hypertonic saline aerosols are reported to increase mucus clearance
and improve pulmonary function. Benefit is quite variable and
inferior, on average, to that achieved with DNase;
Airway clearance therapy - chest percussion combined with postural
drainage;
Antibiotic therapy - to control progression of lung infection
Airway Foreign Body Bronchodilators and corticosteroids should not be used
Chest physical therapy with postural drainage may dislodge the
material to an area where it may cause more harm
Endoscopic removal with a rigid bronchoscope
Endoscopist may observe enough focal swelling after the material is
removed to recommend a short course of systemic corticosteroids
Antibiotics are not necessary
Allergic Rhinitis The removal and avoidance of offending allergens
Sealing the mattress, pillows, and covers in allergen-proof encasings
Wash bed linens and blankets every week in hot water (>65°C).
Staying in a controlled environment in pollen allergy.
Oral antihistamines as needed for ild, intermittent symptoms of
sneezing and rhinorrhea
Nasal spray ipratropium bromide may be used for serous rhinorrhea.
Intranasal decongestants should be used for <3–5 days, not to be
repeated >1 cycle a month.
Severe symptoms require intranasal corticosteroids, which are the
most effective therapy for allergic rhinitis
Specific allergen immunotherapy
Aspergillosis Deepends on the form: in an immunocompromised host or allergic
disease that includes allergic bronchopulmonary aspergillosis (ABPA)
Voriconazole has now become the drug of choice for invasive
aspergillosis. This is due to the increased efficacy and significantly
less toxicity compared to amphotericin B.
Caspofungin is a newer antifungal agent that is effective against
invasive aspergillosis but more pediatric studies are needed prior to its
widespread use. Currently caspofungin has been approved for use as
salvage therapy for invasive aspergillosis that does not respond to
existing antifungals.
Treatment duration has not been well defined and is based on the
clinical response and the tolerance to the drug. Continue therapy 4-12
weeks or longer.
Itraconazole is used as prophylaxis in some cancer centers for
immunocompromised patients.
ABPA exacerbations are treated with corticosteroids.
The desired goal is to reduce serum immunoglobulin E (IgE) levels to
a range consistent with levels obtained from patients with asthma
(without ABPA) living in the same geographic area.
Reinstitution of corticosteroid therapy may be required if the serum
IgE levels rise to twice this level or higher.
Immediately obtain IgE levels after corticosteroid therapy.

17
For asthma exacerbation, as indicated, administer other agents, such as
beta-adrenergic agonists, high-dosage inhaled corticosteroids, and,
possibly, nedocromil or theophylline.
Administer prednisone as a single morning dose for 2 weeks and then
convert to an alternate-day dosage for 3 months.
Systemic antifungal therapy is not indicated for ABPA
Bronchiectasis Inhaled β agonists - for patients who respond, the medication should
be continued, especially during high-risk periods when triggers are
present, such as an upper respiratory infection or hot humid days;
β-agonists may worsen the air exchange in infants with BPD and
airway malacia;
These patients may benefit from alternative bronchodilators such as
inhaled ipratropium or oral methylxanthines;
Inhaled glucocorticoids and leukotriene-modifying agents may be
considered in patients with frequent inflammatory triggers;
Adequate caloric intake;
Fluid balance;
Prevention of respiratory viral illness
Bronchiolitis SEE DETAILS BELOW
Bronchopulmonary
Dysplasia
Inhaled β agonists - for patients who respond, the medication should
be continued, especially during high-risk periods when triggers are
present, such as an upper respiratory infection or hot humid days;
β-agonists may worsen the air exchange in infants with BPD and
airway malacia;
These patients may benefit from alternative bronchodilators such as
inhaled ipratropium or oral methylxanthines;
Inhaled glucocorticoids and leukotriene-modifying agents may be
considered in patients with frequent inflammatory triggers;
Adequate caloric intake;
Fluid balance;
Prevention of respiratory viral illness
Primary Ciliary
Dyskinesia
Pulmonary therapy - to clear secretions from airways and to control
infection;
Inhalation therapy - to deliver medications and hydrate the lower
respiratory tract;
The basic aerosol solution is 0.9% saline;
In reactive airways, albuterol or other β agonists are added;
When the airway pathogens are resistant to oral antibiotics or when
the infection is difficult to control at home, aerosolized antibiotics
may reduce symptoms, improve pulmonary function, and alleviate the
need for hospitalization;
Human recombinant DNase for mucopurulent exudate;
N-acetylcysteine, is toxic to ciliated epithelium, and repeated
administration should be avoided;
Hypertonic saline aerosols are reported to increase mucus clearance
and improve pulmonary function. Benefit is quite variable and
inferior, on average, to that achieved with DNase;
Airway clearance therapy - chest percussion combined with postural
drainage;
Antibiotic therapy - to control progression of lung infection

18
3. Treatment of bronchial asthma
Treatment aims to:
 Find minimum treatment necessary to suppress symptoms and limit side effects.
 Maintain (near) ―normal‖ pulmonary function.
 Avoid loss of time from school and other activities; avoid parental loss of work time (quality
of life measures).
 Enable patient and/or family to take responsibility for day to day management of asthma.
 Reduce the frequency of exacerbations and to avoid hospital admissions.
 Exercise optimal environmental control measures.
Lifestyle management.
Excellent conditioning and a normal lifestyle is encouraged. The patient should not alter
goals or activities based on asthma. Diet should be altered only if food, food additive (e.g.,
sulfite), or drug (e.g., ASA, nonsteroidal anti-inflammatory drugs) has been identified as a
trigger.
Long-term control medications. Inhaled corticosteroids: anti-inflammatory; dosage
dependent on severity and formulation; can be used for daily maintenance routine and at
increased dosage for short-term management of exacerbations; use of spacer and mouth rinsing
with water. Systemic corticosteroids: anti-inflammatory; for short-term ―burst‖ to control
exacerbation and for longer term in severe, persistent cases; main side effects include increased
activity, appetite; Cushingoid appearance; growth suppression; osteoporosis. Systemic
(methylprednisolone, prednisolone, prednisone): anti-inflammatory mechanism; for moderate to
severe exacerbations (usually 3– 5 day courses; up to 10 may be indicated); tapering the dose
following improvement not indicated. Severe attacks should be treated in hospital with careful
monitoring, oxygen, hydration, aerosol beta-2-agonist (intermittent or continuous), systemic
corticosteroid (oral or parenteral, but initiated early); theophylline for those on maintenance.
Cromolyn and nedocromil: anti-inflammatory; safety is primary advantage of these agents;
nebulizer delivery of cromolyn (20 mg/ampule) may be more effective than MDI (1 mg/puff).
Methylxanthines: predominantly bronchodilator mechanisms; standard dosage adjusted to yield
blood concentration of 1015 mg/L; has a narrow therapeutic margin, multiple drug interactions;
oral dosing may aid compliance; additive benefit with inhaled steroids.
Long-acting beta-2-agonists. Salmeterol (inhaled): bronchodilator; not to be used for acute
symptoms or exacerbations because of slow onset of action; does not replace anti-inflammatory
medication; tolerance can occur, but clinical significance is unknown.
Oral agents: inhaled route is preferred.
Leukotriene modifiers: new classes of anti-inflammatory drugs with great potential
advantages; clinical experience in children is just beginning; leukotriene-receptor antagonists
(multiple entities) and a 5-lipoxygenase inhibitor (Zileuton); special note: age indications vary.
Quick-relief medications. Short-acting inhaled beta-2-agonists. Albuterol, bitolterol,
pirbuterol, terbutaline: bronchodilator mechanism; drugs of choice for intermittent symptom
control and management of acute bronchospasm; regularly scheduled daily use not
recommended; oral route not recommended.
Anticholinergics. Ipratropium bromide: bronchodilator mechanism (reduces vagal tone);
may provide some additive effect to beta-2-agonist as supplement but not indicated as a primary
drug.
4. Summary for the asthma treatment
Step 4: Severe persistent asthma
Continuos symptoms Daily medication required

19
Frequent exacerbations
Physical activities limited
PEF (peak expiratory flow readings)
<60% predicted
variability >30%
to maintain control
Multiple daily controller medications: long-
acting bronchodilator and oral corticosteroids
long term
Step 3: Moderate persistent
Symptoms daily
Exacerbations affect activity and sleep
Nighttime asthma symptoms >1 week
Daily use of inhaled short-acting beta-agonists
<60% predicted
variability >30%
Daily medication required
to maintain control
Daily controller medications: inhaled
corticosteroids and long-acting bronchodilator
especially for nighttime symptoms
Step 2: Mild persistent
Symptoms > 1 time a week but < 1 a day
Exacerbations may affect activity and sleep
Nighttime asthma symptoms >2 a month
60-80% predicted
variability 20-30%
to maintain control
One daily controller medication: possibly add a
long-acting bronchodilator to anti-inflammatory
medication especially for nighttime symptoms
(Cromones, corticosteroids)
Step 1: Intermittent
Intermittent symptoms <1 time a week
Brief exacerbations (from few hours to few
days)
Nighttime asthma symptoms <2 a month
Asymptomatic and normal lung function
between exacerbations
>80% predicted
variability <20%
to maintain control
Intermittent reliever medication taken as needed
only short-acting beta-agonist
Intensity of treatment depends on the severity of
exacerbations. Corticosteroids, cromones may
be required
The long-term management of asthma: treatments in the stepwise
approach for infants and young children
Step 4: Severe persistent asthma
Controller
Daily medication
Nebulized budesonide 1 mg bid
If needed add oral steroids - lowest possible
doses on an alternate day early morning
schedule
Reliever
Inhaled short-acting bronchodilator: inhaled
beta2-agonist or ipratropium bromide or oral
beta2-agonist as needed not to exceed 3-4 times
daily
Step 3: Moderate persistent
Controller
Daily medication
Nebulized budesonide 1 mg bid
Reliever
daily
Step 2: Mild persistent
Controller
Daily medication
Nebulized budesonide or sodium cromoglycate
Reliever

20
daily
Step 1: Intermittent
Controller
No controller therapy is needed
Reliever
week
At each step of therapy avoidance or control of trigger factors are required
5. Emergency care of status asthmaticus
 Admission to the hospital
 Supplemental oxygen
 Oxygen can be provided via nasal cannula or face masks.
 In significant hypoxemia, non-rebreathing masks may be used to deliver as much as 98%
oxygen.
 The goal of supplemental oxygen therapy is oxygen saturation above 90%.
 Inhaled beta-agonists can be administered intermittently or as continuous nebulized aerosol
in a monitored setting.
 Corticosteroids, such as methylprednisolone prednisolone or prednisone, are critical in the
therapy of status asthmaticus
 Ipratropium bromide (Atrovent), a quaternary amine that does not cross the blood-brain
barrier, is the recommended sympathomimetic agent of choice.
 Further therapy:
 Magnesium sulfate
 Intravenous beta-agonists
 Ketamine
 Methylxanthines
 Inhaled anesthetic agents, such as halothane, isoflurane, and enflurane
 Extracorporeal membrane oxygenation (ECMO)
6.1. Quiz
1. How do you differentiate airway foreign body aspiration?
2. How do you differentiate allergic rhinitis with bronchial obstruction?
3. How do you differentiate pulmonary aspergillosis?
4. How do you differentiate bronchiectasis?
5. Define bronchiolitis.
6. What is the most common pathogen for bronchiolitis?
7. List all pathogens involved into the development of bronchiolitis.
8. How many children develop recurrent wheezing after acquizisition of RSV infection?
9. When do RSV peak?
10. At what age do clinical severity of bronchiolitis diminishes?
11. How do RVS transmit?
12. What is and how long the prodrome does last?
13. Depict clinical manifestation of the bronchiolitis.
14. What are laboratory tests usually required in bronchiolitis?
15. What will chest radiograph show in acute bronchiolitis?
16. What are the complications for the bronchiolitis?
17. What is the differential diagnosis for the bronchiolitis?
18. What are the treatment aims?

21
19. What does the hospital care imply?
20. List drugs effective for the treatment of bronchiolitis.
21. What is the immunoprophylaxis for bronchiolitis?
22. What does nonpharmacological treatment of bronchiolitis include?
23. How rapidly do acute severe obstructive symptoms usually resolve in bronchiolitis?
24. What is follow-up in bronchiolitis?
25. What are treatment aims for bronchial asthma in children?
26. What is lifestyle management?
27. What is diet therapy?
28. Which inhaled corticosteroids do you know?
29. What is their action in bronchial asthma?
30. What adverse effects can be expected from the use of local corticosteroids?
31. What are systemic adverse effects can be expected from the use of local corticosteroids at
high dosages?
32. What is primary advantage of cromolyn and nedocromil over local corticosteroids?
33. What is mechanism of action for these drugs?
34. Spell MDI abbreviation?
35. What do you know of a new class of anti-inflammatory drugs?
36. What groups of methyxanthines do you know?
37. What are adverse effects of methyxanthines?
38. What is safe serum concentration for methyxanthines?
39. What are drug interactions for methyxanthines?
40. What long-acting beta-2-agonists do you know?
41. What short-acting beta-2-agonists do you know?
42. How are these administered?
43. What are systemic adverse effects from the use of beta-2-agonists
44. How do anticholinergics exert beneficial effect on to asthma?
45. List anticholinergic drugs you know.
46. What are controllers and relievers for severe persistent asthma?
47. What are controllers and relievers for moderate persistent asthma?
48. What are controllers and relievers for mild persistent asthma?
49. What are controllers and relievers for intermittent asthma?
50. What clinical picture indicates the need in severe persistent asthma therapy?
51. What clinical picture indicates the need in moderate persistent asthma therapy?
52. What clinical picture indicates the need in mild persistent asthma therapy?
53. What clinical picture indicates the need in intermittent asthma therapy?
1. What may lead to the bronchiolitis?
A. Staphylococcal infection
B. Streptococcal infection
C. Mycoplasma pneumoniae*
D. Neisseria meningitidis
E. E.coli
A 13 years old boy came to the doctor with symptoms of the pneumonia. In the anamnesis a
boy had recurrent left sided lower lobe pneumonia and chronic cough in early childhood. On the
physical examination decreased breath sound and dull area were revealed over the left lung base.
Laboratory data showed leucocytosis. On X-ray was seen dense opacity of the left lower lobe.
After the effective treatment of pulmonary infection with appropriate antibiotics, X-ray was
repeated. It shows a mass in the posterior basal segment of the left lower lobe. CT scan with

22
contrast confirms a mass in posterior basal segment of the left lower lobe with blood supply from
the thoracic aorta.
3. What is the follow-up?
Suggested reading
1. Genie E. Roosevelt. Infectiuos upper airway obstruction / in Nelson Textbook of
Pediatrics, 2-Volume Set, 20th ed. by Kliegman R.M., Bonita M.D. Stanton MD, Geme
J.S., Nina F Schor MD / Elsevier Inc., 2016. P. 2031-2035.
2. James W. Schroeder Jr. and Lauren D. Holinger. Laryngotracheal stenosis and subglottic
stenosis / in Nelson Textbook of Pediatrics, 2-Volume Set, 20th ed. by Kliegman R.M.,
Bonita M.D. Stanton MD, Geme J.S., Nina F Schor MD / Elsevier Inc., 2016. P. 2041-
2042
3. Bria M. Coates, Lauren E. Camarda, and Denise M. Goodman. Whezzing in infants:
Bronchiolitis / in Nelson Textbook of Pediatrics, 2-Volume Set, 20th ed. by Kliegman
R.M., Bonita M.D. Stanton MD, Geme J.S., Nina F Schor MD / Elsevier Inc., 2016. P.
2044-2048.
4. Lauren E. Camarda and Denise M. Goodman. Bronchitis / in Nelson Textbook of
Pediatrics, 2-Volume Set, 20th ed. by Kliegman R.M., Bonita M.D. Stanton MD, Geme
J.S., Nina F Schor MD / Elsevier Inc., 2016. P. 2048-2049
5. Federico MJ, Hoch HE, Anderson WC 3rd, Spahn JD, Szefler SJ Asthma Management
for children: Risk Identification and prevention./
http://www.advancesinpediatrics.com/article/S0065-3101(16)30010-X/pdf
Additional reading
1. Sveum R., Institute for Clinical Systems Improvement, Bergstorm J., Brottman G.,
Hanson M., Heiman M., Johns K., Malkiewicz J., Manney S., Moyer L., Myers N.,
O’Brien M., Uden D.. et.al. Health care guideline: diagnosis and management of asthma /
Sveum R. http://www.sign.ac.uk/pdf/pat141_CHILDREN.pdf
2. Kam-Lun Hon, Alexander K.C. Leung, MBBS, FRCPC, FRCP(UK&Irel), FRCP, FAAP
et al. Severe Childhood Respitarory Viral Infections // Advances in Pediatrics 56 (2009)
47-73
3. Monica l. Federico, Heather E. Hoch, William C. Andercon III, Joseph D. Spahn, Stanley
J. Szefler et.al Asthma Managments for Children: Risk Identification And Prevention //
Advances in Pediatrics Vol. 63, Issue 1 (2016), p103-126.
4. Susan Gage, Peiyi kan, henry C. Lee, Jeffrey B. Gould, David K. Stevenson, Gary M.
Shaw, Hugh M. O’Brodovich et al. Maternal Asthma, Preterm Birth, and Risk of
Bronchopulmonary Dysplasia // The Journal of Pediatrics, Vol. 167, Issue 4,(2015) p875-
880.e1

23
Topic 3: Differential diagnosis of hereditary, congenital, and chronic disease of the
bronchopulmonary system in children.
Academic hours: 6
Self-education: 4
1. SIGNIFICANCE
Congenital and chronic disease of bronchial tree usually manifest in infancy or in childhood.
Some of them produce debilitating condition that potentially can be prevented in case of making
timely diagnosis. That is why it is important to remember that the most common case of cough
may not be only frequent respiratory viral infections or asthma, but illnesses with specific
organic structural changes in the broncho-pulmonary tree, much rare, including bronchomalacia
and tracheomalacia, α1-antitrypsin deficiency and emphysema, pulmonary hypoplasia,
pulmonary sequestration, bronchogenic cysts, lung hernia, pulmonary hemosiderosis, congenital
pulmonary lymphangiectasia.
However, the most prevalent cause of chronic pulmonary disease in children, after bronchial
asthma, is cystic fibrosis (CF). It takes a significant proportion of children who require long-term
support on the part of medical personnel, a large among of antibiotics and other treatments and
health related costs involved. The diagnosis of the disease can be easily obscured by a number of
CF like disease, that is why it is important to the student to familiarize with clinical and
laboratory presentation of the disease, make a clear differential diagnosis, and be aware of the
contemporary treatment protocols for CF.
2. PREREQUISITES
be sure of the ability to:
 Inspect the thorax and its respiratory movements and note rate, rhythm, depth, and effort of
breathing;
 Inspect for retractions of the supraclavicular areas of contractions of the sternomastoid
muscles on respiration;
 Observe shape of child`s chest;
 Auscultate child`s breathing for increased white noise and wheezes;
 Palpate the chest for respiratory expansion, tactile fremitus;
 Percuss the chest in the standard areas, comparing one side with the other at each level;
 Auscultate the chest with stethoscope in order to evaluate breath sounds and not any
- know the way of differential diagnosis between bronchomalacia and tracheomalacia, α1-
antitrypsin deficiency and emphysema, pulmonary hypoplasia, pulmonary sequestration,
bronchogenic cysts, lung hernia, pulmonary hemosiderosis, congenital pulmonary
lymphangiectasia, CF and other chronic bronchopulmonary diseases in infants and children.
- to identify the child with bronchomalacia and tracheomalacia, α1-antitrypsin deficiency
and emphysema, pulmonary hypoplasia, pulmonary sequestration, bronchogenic cysts, lung

24
hernia, pulmonary hemosiderosis, congenital pulmonary lymphangiectasia, make correct
decisions during physical examination of the patient with given conditions, take appropriate
actions based on those decisions, demonstrate skills to develop management and follow up
measures.
Normal anatomy,
Physiology
features of the respiratory
age groups
findings
presentation of the respiratory
tract illnesses in children
of the respiratory tract illnesses
clinical findings
Microbiology Sampling of sputum, pleural
excudate, blood for bacterial
cultures
Assess microbiologic findings
in clinical context
diseases
pulmonary tests.
tests
imaging studies in respiratory
tract illnesses
of the chest
emergency care
1. Diffrenential diagnosis of chronic and inherited anomalies of pulmonary system
Bronchomalacia and
tracheomalacia
Primary tracheomalacia and bronchomalacia are principally disorders of
infants, with a male: female ratio of 2: 1.
The dominant finding, low-pitched monophonic wheezing, is most
prominent over the central airways.
Parents often describe persistent respiratory congestion even in the
absence of a viral respiratory infection.
When the lesion involves only one main bronchus (more commonly the

25
left), the wheezing is louder on that side.
In cases of tracheomalacia, the wheeze is loudest over the trachea.
Hyperinflation and/or subcostal retractions do not occur unless the patient
also has asthma or another cause of small airways obstruction.
In the absence of asthma, patients with tracheomalacia and
bronchomalacia are not helped by administration of a bronchodilator.
Acquired tracheomalacia and bronchomalacia are seen in association with
vascular rings (and may persist after surgical correction),
tracheoesophageal fistula, and cardiomegaly, and after lung
transplantation.
The definitive diagnosis of tracheomalacia and bronchomalacia is
established by flexible or rigid bronchoscopy.
Other important diagnostic modalities include MRI and CT scanning.
MRI is especially useful when there is a possibility of vascular ring and
should be performed when a right aortic arch is seen on plain film
radiography.
Cystic fibrosis See below
Α1-Antitrypsin
Deficiency and
Emphysema
Little or no detectable pulmonary disease during childhood.
A few have early onset of chronic pulmonary symptoms, including
dyspnea, wheezing, and cough, and panacinar emphysema
It is probable that these findings occur secondarily to infection, causing
inflammation with consequent early disease.
Smoking greatly increases the risk of emphysema developing in mutant Pi
types.
Physical examination in childhood is usually normal.
It very rarely reveals growth failure, an increased anteroposterior diameter
of the chest with a hyperresonant percussion note, crackles if there is
active infection, and clubbing.
Severe emphysema can depress the diaphragm, making the liver and
spleen more easily palpable.
Serum immunoassay measures low levels of α1-antitrypsin; normal serum
levels are 180–280 mg/dL.
Serum electrophoresis reveals the phenotype, and genotype is determined
by polymerase chain reaction
Chest CT may show more hyperexpansion in the lower lung zones, with
occasional bronchiectasis.
Pulmonary
hypoplasia
Secondary to other intrauterine disorders that produce an impairment of
normal lung development
Deformities of the thoracic spine and rib cage (thoracic dystrophy),
pleural effusions with fetal hydrops, cystic adenomatoid malformation,
and congenital diaphragmatic hernia physically constrain the developing
lung.
Any condition that produces oligohydramnios (fetal renal insufficiency or
prolonged premature rupture of membranes) can also lead to diminished
lung growth.
Pulmonary hypoplasia involves a decrease in both the number of alveoli
and the number of airway generations.
The hypoplasia may be bilateral because of the presence of bilateral lung
constraint, as occurs in oligohydramnios or thoracic dystrophy.
Pulmonary hypoplasia is usually recognized in the newborn period due to

26
either the respiratory insufficiency or the presentation of persistent
pulmonary hypertension.
Later presentation (tachypnea) with stress or respiratory viral infection
can be seen in infants with mild pulmonary hypoplasia.
Pulmonary
sequestration
Area of dullness to percussion and decreased breath sounds over the
lesion.
During infection, crackles may also be present.
A continuous or purely systolic murmur may be heard over the back. If
findings on routine chest radiographs are consistent with the diagnosis,
further delineation is indicated before surgical intervention
CT with contrast can demonstrate both the extent of the lesion and its
vascular supply.
Magnetic resonance angiography (MRA) is also useful.
Ultrasonography can help rule out a diaphragmatic hernia and
demonstrate the systemic artery.
Surgical removal is recommended.
Intrapulmonary sequestration is generally found in a lower lobe
Patients usually present with infection.
In older patients, hemoptysis is common.
A chest radiograph during a period when there is no active infection
reveals a mass lesion; an air-fluid level may be present.
During infection, the margins of the lesion may be blurred.
Bronchogenic cysts Fever, chest pain, and productive cough are the most common presenting
symptoms.
Dysphagia may be present; some bronchogenic cysts are asymptomatic.
A chest radiograph reveals the cyst, which may contain an air-fluid level
CT scan or MRI is obtained in most cases to better demonstrate anatomy
and extent of lesion before surgical resection.
Treatment of symptomatic cysts is surgical excision after appropriate
antibiotic management.
Symptomatic cysts are generally excised in view of the high rate of
infection
Lung hernia Cervical hernia (―Sibson hernia‖) is usually a neck mass noticed while
straining or coughing.
Some lesions are asymptomatic and detected only when a chest film is
taken for another reason.
Findings on physical examination are normal except during Valsalva
maneuver, when a soft bulge may be noticed in the neck. In most cases,
no treatment is necessary.
These hernias may cause problems, however, during attempts to place a
central venous catheter through the jugular or subclavian veins.
Spontaneous resolution can occur.
Paravertebral or parasternal hernias are usually associated with rib
anomalies.
Intercostal hernias usually occur parasternally, where the external
intercostal muscle is absent
Straining, coughing, or playing a musical instrument may have a role in
causing intercostal hernias, but in most cases, there is probably a pre-
existing defect in the thoracic wall.

27
Pulmonary
hemosiderosis
Hemorrhage may be significant without remarkable symptomatology.
Hemoptysis may not occur.
Bleeding may occasionally be recognized by the presence of alveolar
infiltrates on a chest radiograph.
It should be noted that the absence of an infiltrate does not rule out an
ongoing hemorrhagic process.
Wheezing, cough, dyspnea, and alterations in gas exchange, reflecting
bronchospasm, edema, mucous plugging, and inflammation.
On physical examination, the patient may be pale with tachycardia and
tachypnea.
During an acute exacerbation, children are frequently febrile.
Examination of the chest may reveal retractions and differential or
decreased aeration, with crackles or wheezes.
The patient may present in shock with respiratory failure from massive
hemoptysis.
Children in particular may present with symptoms of chronic anemia,
such as failure to thrive.
Pulmonary hemorrhage is associated with a decrease in hemoglobin and
hematocrit. The classic finding is a microcytic, hypochromic anemia. The
reticulocyte count is elevated
White blood count and differential should be evaluated for evidence of
infection and eosinophilia.
A stool specimen can be heme-positive secondary to swallowed blood.
Renal and liver function should be reviewed. A urinalysis should be
obtained to assess for evidence of nephritis.
A coagulation profile, quantitative immunoglobulins (including IgE), and
complement studies are recommended.
Testing for antinuclear antibody (ANA), anti–double stranded DNA,
rheumatoid factor, antiphospholipid antibody, and anti–glomerular
basement membrane antibody (antiGBM) evaluates for a number of
primary and secondary etiologies of DAH.
Sputum or pulmonary secretions should be analyzed for significant
evidence of blood or hemosiderin-laden macrophages (HLM).
Congenital
pulmonary
lymphangiectasia
Children with pulmonary venous obstruction or severe pulmonary
lymphangiectasia present with dyspnea and cyanosis in the newborn
period.
Chest radiographs reveal diffuse, dense, reticular densities with
prominence of Kerley B lines.
If the lung is not completely involved, the spared areas appear
hyperlucent.
Respiration is compromised because of impaired diffusion and decreased
pulmonary compliance.
The diagnosis can be suggested by CT scan and cardiac catheterization;
definitive diagnosis requires lung biopsy (either thoracoscopic or open).
2. Cystic fibrosis.
Abnormal CFTR gene in 1:of 25 individuals in the Caucasian population. Incidence of
cystic fibrosis 1:2500 in Caucasian population. 1:17,000 in African-American population (rarely
seen in African blacks and Asians).

28
Etiology.
 The most common severe inherited disease in the Caucasian population (autosomal recessive
in inheritance).
 Cystic fibrosis transmembrane regulator (CFTR): functions as a cyclic AMP-activated
 chloride channel, which allows for the transport of chloride out of the cell. It is accompanied
by the passive passage of water, which keeps secretions well hydrated.
 In cystic fibrosis, an abnormality in CFTR blocks chloride transport and inadequate
hydration of the cell surface results in thick secretions and organ damage.
 The CFTR gene is 250,000 base pairs long and located on the long arm of chromosome
7.The most common deletion is three base pairs, which results in the absence of phenylalanine
at codon 508 (seen in over 70% of the cystic fibrosis population in North America).
Clinical presentation.
Symptoms.
 Chronic cough, recurrent pneumonia, bronchorrhea, nasal polyps, and chronic pansinusitis.
 Pancreatic insufficiency: occurs in 85% of patients. Fat malabsorption may lead to failure to
thrive or pancreatitis.
 Rectal prolapse: occurs in 2% of the patients.
 Meconium ileus: 15%–20% of patients present with this symptom.
 Distal obstruction: of the large intestine may be seen in older children.
 Hyponatremic dehydration.
 Hypochloremic metabolic alkalosis.
Signs.
 Cough (frequently productive of mucopurulent sputum), rhonchi, rales, hyper-resonance to
percussion, nasal polyps, and cyanosis (in later stages).
 Digital clubbing, hepatosplenomegaly in patients with cirrhosis, growth retardation,
hypertrophic osteoarthropathy, and delayed puberty, amenorrhea, irregular menstrual periods
(in teenage patients).
Laboratory work-out.
 Sweat test: ―gold standard‖ for the diagnosis of cystic fibrosis (CF). Sweat chloride >60
mEq/L is considered abnormal. False positives are seen in severe malnutrition, ectodermal
dysplasia, adrenal insufficiency, nephrogenic diabetes insipidus, hypothyroidism,
hypoparathyroidism, mucopolysaccharidoses. False negatives are seen in patients with edema
and hypoproteinemia.
 Genetic testing: over 600 identified CF genotypes, but only 20–70 of the most common are
tested; thus, the lack of a positive genotype reduces (but does not eliminate) the possibility that
a CF sample can be obtained from blood or buccal cell scraping. Sputum cultures: frequent
pathogens include Staphylococcus aureus, Pseudomonas aeruginosa (mucoid and nonmucoid),
Burkholderia cepacia.
 Pulmonary function tests: usually reveal obstructive lung disease.
 Pancreatic function tests: 72-hour fecal fat measurement, measurement of serum para-
aminobenzoic acid (PABA) levels, stool trypsin levels, serum immunore-active trypsin (IRT).
 Chest radiography: typical features include hyperinflation, peribronchial thickening,
atelectasis, cystic lesions filled with mucus, and bronchiectasis.
 Sinus radiography: typically shows pansinusitis.
Complications.
 Respiratory: recurrent bronchitis and pneumonia, chronic sinusitis, pneumothorax,
hemoptysis
 Gastrointestinal: include pancreatic insufficiency; patients usually have steatorrhea;
decreased levels of vitamins A, D, E and K; poor growth and failure to thrive; meconium ileus
equivalent; rectal prolapse; and clinically significant hepatobiliary disease (cirrhosis of the
liver, esophageal varices and splenomegaly).

29
 Reproductive: include sterility in 98% of males and 75% of females.
 Endocrine: abnormal glucose tolerance; diabetes mellitus.
 Pulmonary: recurrent pneumonia, chronic bronchitis, immotile cilia syndrome, severe
asthma, aspiration pneumonia.
 Gastrointestinal: gastroesophageal reflux, celiac sprue, protein-losing enteropathy.
 Other: Failure to thrive (secondary to neglect, poor caloric intake or feeding problems).
Immune deficiency syndromes. Nasal polyposis. Male infertility. Hyponatremic dehydration.
Treatment aims
 To maintain good nutritional status (good nutrition is associated with a better prognosis).
 To slow pulmonary deterioration as much as possible.
 To maintain a normal lifestyle.
Treatment.
High calorie diet with nutritional supplements (given orally, via nasogastric tube feedings or
through gastrostomy tube feeding). Vitamin supplements: multivitamins and fat soluble vitamin
replacement (usually E and K).
Pancreatic enzyme-replacement therapy can be used in patients who are pancreatic
insufficient. Dosage is adjusted for the frequency and character of the stools and for growth
pattern.
Stool softeners treat constipation or meconium ileus equivalent and include mineral oil, oral
N-acetylcysteine, lactulose, enemas.
 Antibiotic therapy (based on sputum culture results). Oral antibiotics: cephalexin, cefaclor,
trimethoprim-sulfamethoxazole, chloram-phenicol, ciprofloxacin. Intravenous antibiotics
(given for 2–3-week course): Staphylococcus aureus: oxacillin, nafcillin; Pseudomonas
aeruginosa or Burkholderia cepacia: semisynthetic penicillin (ticarcillin, piperacillin) or a
cephalosporin (ceftazidime) plus an aminoglycoside (gentamicin, tobramycin, or amikacin) to
obtain synergistic action.
 Aerosolized bronchodilator therapy: albuterol
 Mucolytic agents: N-acetyl-cysteine, recombinant DNase.
 Chest physiotherapy with postural drainage.
Prognosis
 Long-term prognosis is poor.
 The course of the illness is variable; it is impossible to predict the course of
 the disease in a specific person.
 The current mean life span is 29 years.
 Due to new antibiotics, enzyme-replacement therapy, and maintenance of good pulmonary
toilet with chest physiotherapy and bronchodilators, the mean age of survival has been
increasing for the past three decades.
Follow-up and management
• Routine care should be at a Cystic Fibrosis Center.
• Frequency of visits is dependent on severity of illness: usually every 2–4 m.
• Usually lifelong nutritional support is required.
• Duration of antibiotic therapy is controversial. Chronic use is eventually required as the
patient’s pulmonary function deteriorates.
• All siblings should have a sweat test
6.1. Quiz
1. What is the diagnosis and differential diagnosis for bronchomalacia and tracheomalacia?

30
2. What is the diagnosis and differential diagnosis for α1-antitrypsin deficiency and
emphysema?
3. What is the diagnosis and differential diagnosis for pulmonary hypoplasia?
4. What is the diagnosis and differential diagnosis for pulmonary sequestration?
5. What is the diagnosis and differential diagnosis for bronchogenic cysts?
6. What is the diagnosis and differential diagnosis for lung hernia?
7. What is the diagnosis and differential diagnosis for pulmonary hemosiderosis?
8. What is the diagnosis and differential diagnosis for congenital pulmonary
lymphangiectasia?
9. Define CF
10. What is the epidemiology of the disease?
11. In which population one may encounter the most severe cases of CF?
12. What is disorder seen in CF patients?
13. What is pathophysiology of the disease?
14. Why does the mucus become dense in CF?
15. What does CFTR gene represent?
16. What pulmonary changes are seen in CF?
17. What are the symptoms in CF?
18. What are the signs of pancreatic insufficiency in children with CF?
19. What is the clinical presentation of meconium ileus?
20. How will you treat hyponatremic dehydration in patient with CF?
21. How will you treat hypochloremic metabolic alkalosis in a patient with CF?
22. What are the signs of pulmonary involvement in CF?
23. What findings can be expected after the examination of the abdominal cavity of the
patient with CF?
24. What is the gold laboratory standard for making the diagnosis of CF?.
25. Which level of sweat chloride we consider as pathologic?
26. In which clinical circumstances we may expect false positive results of sweat chloride
test?
27. In which clinical circumstances we may expect false negative results of sweat chloride
test?
28. Which pathegens are most commonly cultered from the sputum?
29. What do pulmonary tests usually reveal in CF?
30. What are the laboratory tests to check pancreatic function?
31. What are the findings on X-ray in CF patients?
32. What are respiratory complications of CF?
33. What are gastrointertinal complications of cystic fibrosis
34. What is the differencial diagnosis in CF?
35. What are the treatment aims in CF?
36. Describe diet therapy in CF?
37. List pancreatic enzymes preparations you would employ in CF?
38. What stool softeners do you knwp?
39. What is antibiotic therapy in CF?
40. What is the prognosis in CF?
A 14 year old girl was complaining on decreased exercise tolerance during sport classes and
intermittent breathlessness. Chest radiography showed a homogenous soft tissue mass at right
lower lobe of the lung. Thoracic echocardiography showed normal cardiac structure. As the
mother mentioned that the family is positive for hereditary hemorrhagic teleangiectasia, doctor
directly send a girl for computer tomography pulmonary angiogram. What is the most common
finding in this case?

31
1. Pulmonary sequestration
2. Bronchogenic cyst
3. Pulmonary arteriovenous malformation*
4. Pulmonary embolism
5. Pnumonia
A 6 years old female child from a poor socioeconomic status family, born of a non
consanguineous marriage presented with cough, respiratory difficulty, recurrent hemoptysis and
pallor with easy fatigability beginning at the age of four years. There was no history of recurrent
fever, skin lesions, bleeding from any other site, arthritis, bone pain, significant drug intake or
prolong exposure to toxin. Her appetite, bladder & bowel habits were normal. She had received
antibiotics, bronchodilators treatment along with iron prophylaxis and total six blood
transfusions. At the time of admission the child had marked pallor, respiratory distress,
tachypnea (respiratory rate = 46/min) and tachycardia (pulse = 120/min). Other vital parameters
were normal. Her weight was 15 kg and height was 105.5 cm. Examination of chest revealed
crepitations bilaterally. Other examination findings were normal. On investigation hemoglobin
was 4.6gm% with hypochromic microcytic anemia, total leukocyte count was 8700/cumm
(polymorph 58%, lymphocyte 30%, eosinophil 07%, monocyte 03%), platelets were adequate,
serum ferritin level was low (4mg/l), total iron binding capacity was 500 mg/dl (Normal = 250-
400 mg/dl) suggestive of iron deficiency anemia. Clotting time, prothombin time, activated
partial thromboplastin time, liver function test, renal function test were normal. Antinuclear
antibody (ANA), anti neutrophil cytoplasmic antibody (ANCA) and Mantoux test were negative.
Chest radiograph revealed bilateral pulmonary infiltrate. CT scan sowed bilateral ground glass
opacity. Bronchoalveolar lavage demonstrated significant number of hemosiderin-loaded
macrophages. As no cause was identified the child was diagnosed as idiopathic pulmonary
hemosiderosis. The patient was given blood transfusion and oral prednisolone in the dose of 2
mg/kg along with iron. Her condition improved after 7 days of therapy. After 1 month of
treatment, chest radiograph was clear and there was no further hemoptysis for last 6 months.
3. What are the characteristic laboratory tests?
Suggested reading
1. James W. Shroeder Jr. and Lauren D. Holinger. Congenital anomalies of the larynx,
trachea, and bronchi / in Nelson Textbook of Pediatrics, 2-Volume Set, 20th ed. by
Kliegman R.M., Bonita M.D. Stanton MD, Geme J.S., Nina F Schor MD / Elsevier Inc.,
2016. P. 2036-2039.
2. Joshua A. Blatter and Jonathan D. Finder. Congenital disorders of the lung / in Nelson
Textbook of Pediatrics, 2-Volume Set, 20th ed. by Kliegman R.M., Bonita M.D. Stanton
MD, Geme J.S., Nina F Schor MD / Elsevier Inc., 2016. P. 2057-2061.
3. Anne G. Griffiths and Thomas P. Green. Chronic or reccurent respiratory sympthoms / in
Nelson Textbook of Pediatrics, 2-Volume Set, 20th ed. by Kliegman R.M., Bonita M.D.
Stanton MD, Geme J.S., Nina F Schor MD / Elsevier Inc., 2016. P. 2027-2031.
4. Susanna A. McColley. Extrapulmonary diseases with pulmonary manifestations / in
Nelson Textbook of Pediatrics, 2-Volume Set, 20th ed. by Kliegman R.M., Bonita M.D.
Stanton MD, Geme J.S., Nina F Schor MD / Elsevier Inc., 2016. P. 2055-2057.
Additional reading

32
1. Brenda B. Poindexter, Alan H. Jobe et.al. The diagnostic Conundrum of
Bronchopulmonary Dysplasia // The Journal of Pediatrics, Vol. 167, Issue 3,(2014) p517-
518.
2. Tom Watson, Catherine M. Owens et.al. Computer tomography in children with lung
disease, How, when and why? Myths and mystery unraveled // Pediatrics and Child
Health, Vol. 23, Issue 3, (2013) p125-132.
3. Diana L. Diesen, Steve Megison et.al. Congenital Diaphragmatic Hernia with Associated
Pulmonary Sequestration // The Journal of Pediatrics, Vol. 167, Issue 3,(2014) p517-518.

33
Topic 4: Differential diagnosis of cardiomegaly in children
Thematic chapter: Differentail diagnosis of the most common disease of the blood
circulatory system in children. Emergency care in common emergency conditions
Academic hours: 6
Self-education: 4
1. SIGNIFICANCE
Differential diagnosis of cardiomegaly, seen commonly in cardiomyopathies, constitutes a
significant diagnostic challenge even for the experienced physician and rarely bring much
satisfaction from the therapy. Student should be always alert in respect of these illnesses and
suspect it in any cases when changes in the myocardium cannot be easily explained.
2. PREREQUISITES
examination of the patient in the intensive care unit during practical training. Therefore, before
beginning this lesson, you have to be sure of the ability to:
 Palpate the radial pulse and note heart rate and rhythm
 Palpate the carodit artery pulse and note amplitude, any variations in latter. contour, any
thrills
 Measure maximal and minimal blood pressure
 Identify the jugular venous pulsations
 Identify the apical heart impulse and note location, diameter, amplitude, duration
 Auscultate heart with stethoscope and identify if murmurs are present
- etiology, pathogenesis, classification, clinical manifestation, differential diagnosis and
treatment principles for the cardiomyopathies in children and infants.
- to identify the child with myocardial disease, make correct decisions during physical
examination of the patient with listed conditions, take appropriate actions based on those
decisions, demonstrate skills to develop immediate and long-term care plan, foresee outcomes.
Normal anatomy,
Physiology
features of the cardio-vascular
age groups
cardio-vascular system in
children for evaluation of
clinical findings
presentation of the cardiomegaly
in children
of cardiomegaly in children for
evaluation of clinical findings

34
the cardiac failure
of cardiac failure
diseases
cardio-vascular system in
children.
Physiology of
electrocardiogram.
Assess the results of
electrocardiography
Imaging studies Indications for the heart
ultrasound
of the chest
Intensive care Symptoms and signs of cardiac
failure of different stages, its
etiology, and principles of
intensive care
Recognize cardiac failure,
emergency care
1. Differential diagnosis of cardiomegaly in children
Cardiomegaly is a condition wherein the heart enlarges in a cardiothoracic ratio of more than
0.50. It is when the heart is more than 50 percent bigger than the inner diameter of one’s rib
cage.
Cardiomegaly is assumed to be the direct effect of the thickening of the heart muscles and
that happens when the heart is given an increased workload. This increase workload on the other
hand, may be due to other health conditions present in the body. Viral illnesses and previous
heart attacks can cause the heart to overwork. Drug abuse, inflammation of the heart, and
uncontrolled hypertension are the known issues that may give rise to cardiomegaly.
Exercise is also a factor. It is believed that most athletes have enlarged hearts, but in this case,
theirs is not considered to be a medical condition at all. So in essence, cardiomegaly is not
always bad, at least not for sports people. But to a regular person, having an enlarged heart is not
normal at all.
The heart’s right or left ventricle may be enlarged, and in some instances, both ventricles
can be affected. This is called cardiomegaly due to ventricular hypertrophy. If the left heart is
affected, it is called left ventricular hypertrophy. This condition is very common to people who
have chronic systolic heart failure or cardiomyopathies. Cardiomegaly can also be due to
dilation.
The summary below helps to evaluate different types of cardiomegaly
Hypertrophic
cardiomyopathy
Inheritance is autosomal dominant.
Pathology: asymmetric hypertrophy of the ventricular myocardium, without
chamber dilation; microscopic hypertrophy of cardiac myocytes with
abnormal intercellular connections; intramural coronary arteries thickened
with narrowed lumens; the anterior mitral valve leaflet may abut the septum
causing LV outflow obstruction in systole.
Pathophysiology: impaired filling, relaxation; hyperdynamic systolic
function. LV outflow tract gradient +/- present.
Patients may be asymptomatic, with or without history; may occur during
childhood, but especially in teenagers, young adults.
Palpitations: premature ventricular contractions (PVCs), ventricular
tachycardia (VT); exercise-induced chest pain; dizziness, syncope: especially
with stress or activity; dyspnea on exertion: approximately 50% of patients.

35
Harsh systolic ejection murmur along the left sternal border: diminished with
squatting, accentuated by Valsalva maneuver); systolic murmur of mitral
regurgitation (MR) at LV apex; gallop rhythm, ectopic beats on examination
(PVCs); rales: if significant pulmonary edema is present.
Differential diagnosis: murmur: fibrous subaortic stenosis or valvar aortic
stenosis; LVH: secondary effects of systemic hypertension;
ECG: PVCs with normal QT interval, LV hypertrophy by voltage criteria, T-
wave flattening or inversions diffusely.
Chest radiography: hypertrophic: normal in approx 50% of patients;
Echocardiography: left atrial enlargement (LAE); MR; systolic anterior
motion (SAM) of the mitral valve; asymmetrical, thick ventricular septum or
other ventricular wall; abnormal LV diastolic performance. A resting LV
outflow tract gradient may or may not be demonstrated by Doppler
echocardiography;
Holter monitoring: check for PVCs, VT, SVT or atrial fibrillation in dilated
cardiomyopathy (embolic risk).
Cardiac catheterization: 1) to check degree of LV outflow obstruction at rest
and provocative testing (ie, dobutamine); 2) to perform myocardial biopsy to
confirm diagnosis; and 3) to assess LV outflow gradient with verapamil,
beta-blockade and ventricular pacing;
Hypertrophic
cardiomyopathy
―Idiopathic‖ postviral (coxsackie B and A, echovirus) myocarditis,
mitochondrial disorders, carnitine deficiency; HIV;
Congestive heart failure: exercise intolerance,
Dyspnea on exertion, altered growth, sweating; palpitations, chest pain. May
occur in all age groups from infancy on.
May get a history of preceding febrile illnesses, viruses, rashes.
Tachycardia: with or without gallop rhythm; tachypnea for age: with or
withour rales; systolic murmur of mitral regurgitation (MR) at LV apex;
hepatomegaly: with late dependent edema
Acquired inflammatory carditis (Kawasaki disease, rheumatic fever)
Anomalous origin of the left coronary artery from the pulmonary artery
(ALCAPA).
Acquired LV dysfunction: toxin-mediated post-anthracycline therapy,
hemochromatosis, or related to untreated tachyarrhythmias.
ECG: PVCs with normal QT interval, LV hypertrophy by voltage criteria, T-
wave flattening or inversions diffusely.
Chest radiography: cardiomegaly with left atrial and LV enlargement,
pulmonary edema.
Echocardiography: globular dilated left ventricle with poor systolic
contractility, normal LV wall thickness, mitral annular dilation with MR and
LAE. Look for valve disease or regional wall motion abnormalities.
Holter monitoring: check for PVCs, VT, SVT or atrial fibrillation in dilated
cardiomyopathy (embolic risk).
Cardiac catheterization: 1) to check ventricular end-diastolic pressure (worst
prognosis >25 mmHg) and cardiac index, 2) to perform myocardial biopsy,
and 3) to perform coronary arteriography to rule out congenital or acquired
coronary disease (ie, anomalous coronary origin or aneurysms).
Subaortic
stenosis or
valvular aortic
Symptoms depend on the severity of the obstruction.
Severe aortic stenosis - left ventricular failure and signs of low cardiac
output.

36
stenosis Heart failure, cardiomegaly, and pulmonary edema
Pulses are weak in all extremities
Skin may be pale or grayish
Urine output may be diminished
If cardiac output is significantly decreased, the intensity of the murmur at the
right upper sternal border may be minimal.
Most children with less severe forms of aortic stenosis remain asymptomatic
and display normal growth and development.
The murmur is usually discovered during routine physical examination.
Rarely, fatigue, angina, dizziness, or syncope may develop in an older child
with previously undiagnosed severe obstruction to left ventricular outflow.
Sudden death has been reported with aortic stenosis but usually occurs in
patients with severe left ventricular outflow obstruction in whom surgical
relief has been delayed.
In mild stenosis, the pulses, heart size, and apical impulse are all normal.
With increasing degrees of severity, the pulses become diminished in
intensity and the heart may be enlarged, with a left ventricular apical thrust.
Mild to moderate valvular aortic stenosis is usually associated with an early
systolic ejection click, best heard at the apex and left sternal edge.
Normal splitting of the 2nd heart sound is present in mild to moderate
obstruction.
The chest radiograph frequently shows a prominent ascending aorta, but the
aortic knob is normal.
Heart size is typically normal. Valvular calcification has been noted only in
older children and adults.
Echocardiography identifies both the site and the severity of the obstruction.
Glycogen storage
disease (Pompe)
Deficiency of acid α-glucosidase (acid maltase)
Cardiomegaly, hypotonia, hepatomegaly;
Onset: birth–6 mo
Cardiorespiratory failure leading to death by age 2 yr
Kawasaki
disease
Principal Diagnostic Criteria for Kawasaki Disease
Fever of at least 5 days' duration
Presence of four of the following:
Changes in extremities consisting of induration of the hands and feet with
erythematous
palms and soles
Polymorphous rash
Bilateral conjunctival injection
Erythematous mouth and pharynx, strawberry tongue, and red, cracked lips
Cervical lymphadenopathy
Tachycardia
Cardiomegaly
with rheumatic
fever
Modified Jones criteria for the diagnosis of acute rheumatic fever
Major manifestations
Carditis
Polyarthritis
Chorea
Erythema marginatum
Subcutaneous nodules
Minor manifestations
Arthralgia

37
Fever
Elevated acute phase reactants: ESR and C-reactive protein
Prolonged PR interval
Supporting evidence
Positive throat culture for group A Streptococcus or positive, rapid
streptococcal test, elevated or rising streptococcal antibody titer
Cardiomegaly in
SLE
Associated with:
Malar (butterfly) Rash
Discoid-Lupus Rash
Photosensitivity
Oral or Nasal Mucocutaneous Ulcerations
Nonerosive arthritis
Pleuritis or Pericarditis
Cytopenia
Positive Antinuclear Antibody Test
Nephritis**
• Proteinuria >0.5g/day
• Cellular Casts
Encephalopathy**
• Seizures
• Psychosis
Positive Immunoserology**
• Antibodies to nDNA
• Antibodies to Sm Nuclear Antigen
• Positive LE-Cell Preparation
• Biologic False-positive Test for Syphilis
Cardiomegaly
due to systemic
hypertension
May present with congestive heart failure
Cardiomegaly
with bacterial
endocarditis and
aortic stenosis
History of:
Fever and sweating.
Easy fatiguability, malaise.
Palpitations.
Weight loss and anorexia.
Splenomegaly.
Splinter hemorrhages
Pulmonary embolism.
Blood cultures: at least 2–3 over a 24-hour period (not only with temperature
spike!) prior to instituting antibiotic therapy
Acute phase reactants: sedimentation rate (ESR), C-reactive protein (CRP).
Urinanalysis: hematuria.
Echocardiography: standard transthoracic echocardiography may adequately
image a moderate to large valve vegetation, and also demonstrate leaking
valves;
Remember: a negative echocardiogram does not rule out endocarditis!
6.1. Quiz
1. What is the differential diagnosis of hypertrophic cardiomyopathy?
2. What is the differential diagnosis of dilated cardiomyopathy?

38
3. What is the differential diagnosis of congenital subaortic stenosis?
4. What is the differential diagnosis of cardiomegaly associated with Pombe disease?
5. What is the differential diagnosis of cardiomegaly associated with Kawasaki disease?
6. What is the differential diagnosis of cardiomegaly associated with rheumatic fever?
7. What is the differential diagnosis of cardiomegaly associated with SLE?
8. What is the classification of cardiomyopathies?
9. Explain etiology of hypertrophic cardiomyopathies.
10. Explain etiology of dilated cardiomyopathies.
11. Which are the peculiarities in epidemiology of dilated cardiomyopathies?
12. Which are the peculiarities in epidemiology of hypertrophic cardiomyopathies?
13. What are the symptoms and signs of the hypertrophic cardiomyopathies?
14. What are the symptoms and signscof the dilated cardiomyopathies?
15. What investigation would you prescribe for the patients with cardiomyopathies?
16. What are complications of the cardiomyopathies?
17. What are diseases ruled out in hypertrophic cardiomyopathy?
18. What are diseases ruled out in dilated cardiomyopathy?
19. What the treatment aims for cardiomyopathies?
20. What are measures concerning lifestyle and diet in cardiomyopathies?
21. What is pharmacologic treatment for hypertrophic cardiomyopathy?
22. What is pharmacologic treatment for dilated cardiomyopathy?
23. What is nonpharmacologic treatment for dilated cardiomyopathy?
24. What is nonpharmacologic treatment for hypertrophic cardiomyopathy?
25. What is prognosis for hypertrophic cardiomyopathy?
26. What is prognosis for dilated cardiomyopathy?
Hypertrophic cardiomyopathy:
1. Inherits as an autosomal dominant trait*
2. Has postviral (coxsakie B and A echovirus) etiology
3. Develops for the carnitin deficiency
4. Adenoviral infection may play a role in
5. Develops after bacterial endocarditis
A 16-year-old male patient was admitted to our hospital for syncope that was not preceded
by symptoms. The status of the patient had already improved by the time the ambulance arrived.
He had a regular heart rate and was perfectly orientated. Before this event he had been in New
York Heart Association functional class III (dyspnea after climbing one floor, no dyspnea at rest
and no angina). Over the past 3 years, he had experienced recurrent episodes of pre-syncope. On
heart ultrasound he presented with enlargement of all the heart chambers with a left ventricular
ejection fraction of 20%. On arrival at hospital his blood pressure was 100/60 mmHg, with a
heart rate of 76 beats, min- 1. ECG demonstrated a complete left bundle branch block. There was
no ST-segment elevation. During the hospital stay 24-h Holter monitoring was conducted,
demonstrating a normal sinus rhythm with no pauses and no ventricular arrhythmia <1%
premature ventricular contractions, and no symptoms. The chest radiograph demonstrated a
normal pulmonary interstitial structure, with the pulmonary bases clear of any interstitial
effusion. The cardiothoracic index was 55%, left heart catheterization showed normal coronary
arteries.
1. What is a complete diagnosis?
2. What is the treatment?

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