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CASE 1

A 3-year-old boy was admitted to the hospital for examination to clarify the diagnosis.
With complaints of a constant wet cough with mucopurulent sputum discharge, impaired nasal
airflow.
Medical history
The child is sick from the first days of life: shortness of breath, mucopurulent discharge
from the nasal passages, frequent paroxysmal cough were noted. At the age of 6 months,
pneumonia was first diagnosed. At the age of 1.5 and 2 years there were episodes of pneumonia,
frequent bronchitis. There were 3 episodes of otitis during the first year of life.
The child from the second pregnancy, full-term. Birth weight 3500 g, length 51 cm.
Breast-fed up to 1.5 years. Poor weight gain was noted. Weight at 1 year - 9 kg, at 2 years - 10.5
kg. At the age of 1 year, dextracardia (heart is on the right) was revealed by echocardiography.
His older brother has chronic bronchitis.
Objective status
Body weight 10.8 kg. The child is lethargic, indifferent. Body temperature is increased to
38.0 °C. The skin is pale, marked cyanosis of the nasolabial triangle, acrocyanosis. Impaired
nasal breathing, mucopurulent discharge from the nose. Clubbing. BR - 40 per minute. Lung
percussion: dullness of percussion sound, mainly in basal areas. Lung auscultation: moist rales
on both sides.Apex beat is in the V intercostal space on the right midclavicular line. Heart
sounds are rhythmic, heard clearly on the right, mild systolic murmur is noted. Heart rate – 120
beats per minute. Liver +2 cm from under the edge of the right costal arch. The spleen is not
palpable. The abdomen is slightly enlarged, soft, painless.

Complete blood count:


Hb - 110 g / l,
red blood cells - 4.1 × 1012 / l,
platelets -270.0 × 109 / l,
white blood cells - 12.4 × 109 / l,
neutrophils: bands - 10%, segmented - 52%,
lymphocytes - 28%,
eosinophils - 1%,
monocytes - 9%,
ESR - 16 mm / h

1. Provisional diagnosis
2. What laboratory tests are indicated for this child?
3. What instrumental tests are indicated for this child?
4. What studies are recommended for confirmation of the diagnosis?
5. Differential diagnosis
6. Treatment
CASE 2

Pediatrician examines a 7-month-old boy at home. Complaints of coughing, difficulty


breathing, temperature.
Medical history
The child fell ill acutely 2 days ago: increased temperature up to 38.5 °C, nasal
congestion, coughing. Over the next few days, the cough began to increase, today there is a
difficulty in breathing, according to the mother, the child “suffocates”, refuses to drink.
The boy from the second pregnancy, full-term, body weight 3020 g, length 50 cm. Bottle-
fed from birth. Vaccinated. Until now, the child has not been sick. There are 5 members in the
family including a child at the age of 6 years old, healthy.
Objective status
Grave condition due to symptoms of respiratory failure. SpO2 92%. The child is anxious,
often coughing, cough is wet. Dyspnea (expiratory), tachypnea (BR 70 per minute), fine moist
rales and crepitation in the lungs on both sides are noted, rare wheezing is heard. A slight
bloating of the chest is noted, lung percussion - box-like sound. The auxiliary muscles take part
in the act of breathing. Intercostal reraction, nasal flaring. The abdomen is soft and painless on
palpation, the liver protrudes 1.5 cm from under the edge of the costal arch, the spleen is not
enlarged. Stool 2 times a day without pathological admixture. Urination without pathology.

1. Diagnosis
2. Risk factors for the development of the disease in this child
3. What lab tests are needed to confirm the diagnosis?
4. What is the most likely cause of a child’s disease?
5. Treatment
6. Prophylaxis
ANSWERS

CASE 1
1. Primary ciliary dyskinesia: Cartagener syndrome.
The diagnosis was made on the basis of a typical clinical manifestations (rhinitis,
bronchitis, clubbing, the reverse location of internal organs), and anamnesis (repeated otitis
media, bronchitis, pneumonia from an early age, growth retardation)

2. Microbiological examination (culture) of sputum.


A microbiological study (culture) of sputum or tracheal aspirate during an
exacerbation of the disease is recommended
In this case Haemophilus influenzae was detected in the culture

3. Chest x-ray, thoracic computed tomography, X-ray of the sinuses


To clarify the extent of damage to the bronchial tree and dynamic control,
computed tomography (CT) of the chest organs, radiography or CT of the paranasal
sinuses is recommended.

Chest x-ray.
In this case - Lungs hyperinflation, a few focal-like shadows, increased
pulmonary vascularity and deformation of the vascular pattern are noted in all
pulmonary fields. The roots of the lungs (lung hilum) are unstructured. Heart shadow is
on the right.
Thoracic computed tomography
In this case - Bilateral deformation of the bronchi, cylindrical bronchiectasis
X-ray of the sinuses
In this case - Bilateral opacity of the maxillary sinuses

4. The study of the motor activity of the cilia (ciliary beat and beating
pattern). Electron microscopy of the cilia of the epithelium (detection of ultrastructural
defects). Examination of NO in nasal expired air (for children older than 5 years).
Molecular genetic research to identify defective genes.

A combination of these tests is recommended for patients with chronic upper and
lower respiratory tract infections. None of these tests individually gives grounds to
confirm the diagnosis. Only their combination.

In this case - In the biopsy of the nasal mucosa, a significant decrease in the ciliary
beat frequency is noted (cilia are almost immotile). Electron microscopy revealed structural
defects in the microtubules of the cilia of the respiratory epithelium.

5. Cystic fibrosis, congenital malformations of the bronchial tree, primary


immunodeficiency conditions, asthma

6. Antibiotics, irrigation therapy, inhaled bronchodilators, mucolytic drugs


During the exacerbation of the chronic bronchopulmonary process, antibiotics
are prescribed in accordance with the sensitivity of the microflora. Antibacterial therapy
is also used for exacerbations of sinusitis, in the absence of the effect of irrigation
treatment. Hypertonic sodium chloride solution is used for irrigation therapy to relieve
nasal symptoms (nasal congestion, mucus secretion). In case of bronchial obstruction
inhaled bronchodilators are used (ipratropium bromide + phenoterol, salbutamol).
Mucolytics facilitate sputum discharge (ambroxol, acetylcysteine, carbocysteine).

CASE 2
1. Acute bronchiolitis. Respiratory failure II degree.
The diagnosis was made on the basis of typical clinical manifestations: low-grade fever,
cough, symptoms of respiratory failure, expiratory dyspnea, tachypnea, fine moist rales and
crepitation over the entire surface of the lungs, wheezing, chest bloating, participation in the act
of breathing of the auxiliary muscles, intercostal reraction, nasal flaring, SpO2 92%.
A characteristic feature of bronchiolitis is the development of dehydration with metabolic
acidosis, due to an increased need for fluid due to fever and tachypnea, a decrease in fluid intake
due to a child’s refusal to drink due to respiratory failure.

2. An older child in the family, a large family (≥ 4 members). Breastfeeding


≤ 2 months
Older children and adults can have URTI (rhinitis, pharyngitis), but in younger children
(<2 years) the same viruses can cause acute bronchiolitis. The lack of brestfeeding is associated
with impaired immunity.

3. None
Laboratory tests are not required as a rule. Complete blood count is not very informative
to establish a diagnosis. In acute viral bronchiolitis, lymphocytic leukocytosis is often observed.
An increase in the level of C-reactive protein (CRP) and procalcitonin is not typical.

4. Viral infection
In 60-70% of cases, the etiologic factor of bronchiolitis is respiratory syncytial virus
(RSV). Rhinovirus is a common cause as well. Influenza A and B viruses, parainfluenza,
adenovirus, coronavirus, metapneumovirus and human bokavirus are also considered as
causative factors of the disease.

5. Humidified oxygen
Inhalations with hypertonic (3%) sodium chloride solution
Most experts recognize the need for oxygen supply until this indicator reaches
95%. For hospitalized children consider the inhalation of a hypertonic (3%) sodium
chloride solution

6. Prevention of RS-virus infection - passive immunization with palivizumab


is recommended
In children of the first year of life from high-risk groups (prematurity, bronchopulmonary
dysplasia, congenital heart disease). Palivizumab is a monoclonal antibody directed against an
epitope in the A antigenic site of the F protein of RSV, inhibiting its entry into the cell and
thereby preventing infection. Palivizumab is dosed once a month via intramuscular injection, to
be administered throughout the duration of the RSV season (autumn-winter).

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