Differential diagnosis of hereditary, congenital and

Differential diagnosis of hereditary, congenital and

chronic diseases of bronchopulmonary system

in children.

Hereditary and congenital

diseases of bronchopulmonary system in children.

               

 

                Actuality of study of hereditary and congenital chronic broncho-pulmonary diseases in children is significant due to:

1. The overall incidence of recurrent and chronic diseases of this system in children ranges from 0,85 to 1,45%  and the proportion of malformations and     hereditary diseases in the structure of this pathology is quite high (from 4.6 to 20% according to different authors).

2.  In 1-3% of deceased infants and 1/5-1/3 with chronic bronchopulmonary pathology congenital anomalies of the lungs is diagnosed. Moreover, with improved methods of diagnostics frequency of congenital and hereditary pathology has a tendency to increase.

 3. This group of diseases has usually unfavorable forecast, because that leads to disability, reduced life expectancy, and often to death in early age.

 4. A large number of pathological forms, lack of their study, low separate experience for each pediatrician, rarity of a disease make it difficult to establish diagnosis at time and prescribe treatment.

  5. There are no effective preventive measures.

             

                                         Common clinical symptoms of congenital lung diseases:

·        debut of the disease at an early age

·        prolonged, recurrent, chronic inflammation in the lungs

·        obstructive syndrome

·        persistent prolonged wheezing in the lungs, weakened breathing

·        “drumsticks”, pallor, cyanosis

·        physical retardation

·        “cor pulmonale”

·        breathlessness

·        bulging chest

·        asphyxia

·        loss of consciousness, convulsions

·        prolonged cough

·        pleural friction rub

·        combination with other symptoms

 

Classification of hereditary and congenital bronchopulmonary diseases  

·        Defects of the respiratory system:

·        abnormalities of the trachea and bronchi

·        anomalies of the lungs

·        Hereditary lung diseases.

·        Polyorganic hereditary disease with primary lesion of the lungs.

 

 

                         General diagnostic criteria

 

§  Early emergence of diseases of the respiratory system (the neonatal period, I year of life)

§  Physical retardation

§  Persistent obstructive syndrome

§  Recurrent nature of the respiratory system diseases

§  Increasing of sings of respiratory failure despite treatment

§  Inefficiency therapy

 

 

 

ABNORMALITIES OF TRACHEA AND BRONCHI

ü  Anomalies of branching tracheobronchial tree.

ü  Congenital lobar emphysema (lobar, obstructive, hypertrophic, gigant) is a malformation characterized by a sharp increase in the proportion of one lung due emphyzematous change and blowing

ü  Traheobronhomegalia / Mounier-Kuhn syndrome – 1932, France/ – congenital anomaly of the system of the trachea and bronchi, with their expansion due to underdevelopment of elastic cartilage and muscle structures of the tracheobronchial tree.

ü  Syndrome Williams-Campbell (I960) – congenital defect of the system due to generalized hypoplasia of cartilage and segmental subsegmental bronchi from 3rd to 8 th caliber, followed by the formation of secondary bronchiectasis, predominantly symmetric in the lower lobes.

ü  Tracheo(bronchi) – esophageal fistula – a severe malformation, which might result in a fatal outcome already in the early periods after birth

 

 ANOMALIES OF LUNG

 

ü Lung agenesia – absence of lung together with the main bronchus.

ü Lung aplasia   – absence of lung with the presence of rudimentary main bronchus.

ü Pulmonary Hypoplasia – underdevelopment simultaneous bronchi and lung parenchyma.

ü Cystic hypoplasia of the lungs /polycystitis/ – congenital malformation, which except reduction is accompanied by respiratory areas of cystic like formation of cavities and bronchiectasis.

ü Pulmonary sequestration – a section of lung placed inside or outside the pulmonary lobe and does not participate in gas exchange. Blood supply to the site is provided by the anomalous vessel from the thoracic or abdominal aorta or intercostal arteries.

 

                                    Malformations of lung

 

             Malformations of lung frequently underlie the second place amount inflammatory processes in the bronchopulmonary system and are evident more frequently in childhood and adolescence. They include, first of all, various options of cystic hypoplasia of the lung, congenital solitary cyst, pulmonary sequestration and rare abnormalities of the trachea and bronchi.

                               Normal structure of lungs

 

Agenesia, aplasia and hypoplasia of lungs

 

               Agenesia is the absence of lung together with the main bronchus.Until 1972 it was described 200 cases of unilateral agenesias and aplasias of the lungs.

 In lung agenesis, the entire lung and bronchial tree may be absent on one side. The bronchial tree may form without development of the alveoli. Pulmonary hypertension complicates lung agenesis because of a combination of factors: normal blood volume passing through reduced lung tissue, hypoxemia leading to pulmonary vasoconstriction, and any associated left-to-right shunting cardiac lesion.

 

Aplasia is the absence of lung tissue in the presence of rudimentary bronchus.

Hypoplasia is a state when the main and lobar bronchi terminate functionally insolvent rudiment, lung tissue is underdeveloped.

Intrathoracic or extrathoracic lesions can cause pulmonary hypoplasia. Therefore, prolonged rupture of membranes, renal dysplasia, neuromuscular diseases, and congenital diaphragmatic hernia can lead to lung hypoplasia. Reduced urine volume during fetal life may retard lung growth. Pulmonary aplasia leads to respiratory distress, which may vary according to the degree of alveolar involvement. Pulmonary hypoplasia may be primary when the entire lung or when one lobe is reduced in size.

Both pulmonary agenesis and hypoplasia may be accompanied by renal anomalies, which are usually apparent soon after birth and associated with respiratory distress. Cardiac defects occur in 50% of patients.

              Pulmonary agenesis is differentiated from lung aplasia by the absence of the carina in the latter. Lung agenesis is less common than aplasia, about 75% of cases affect the left side, and it is lethal in half of all patients. It may be associated with other manifestations of the syndrome of abnormalities of the vertebrae, anus, cardiovascular system, trachea, esophagus, renal system, and limb buds (VACTERL syndrome). The survival rate is better with left-sided lung agenesis than with right-sided agenesis because the right lung is the larger of the two.

    

Clinic

 

                 Asymptomatic clinic is rare. Children have physical retardation.  Respiratory failure is observed: dyspnea, cyanosis of varying severity. Cough and the allocation of purulent phlegm are associated with the accession of the inflammatory process. Sometimes there is a pain in the chest. On the lesion side thorax is flattened, and the healthy half is convex. At the site of the lesion there are observed shortening of percussion sounds, absent or weakened respiratory noises. The heart is shifted toward the lesion, which may erroneously be interpreted as dextracardia. When expressed hypoxia is observed for a long time the nail phalanx become thickened as “drumsticks.”

            Clinic of hypoplasia is less pronounced. The process proceeds by the type of chronic lung disease, vital lung capacity, GLC are decreased.

X-rays reveal the decrease of lung volume on the side of lesion, intense darkness, highstanding of diaphragm. The heart and mediastinum organs are removed so that the spine looks bare. However, there may be a “pneumocele” when healthy lung is prolaboring through the anterior mediastinum in the other direction.

           The final diagnosis is based on bronchography.

                In pulmonary hypoplasia, development of the distal lung tissue is incomplete. At earlier the delivery of a child the incidence of lung hypoplasia is higher. In babies delivered before 28 weeks’ gestation, the incidence approaches 20%. Pulmonary hypoplasia occur as a result of conditions that restrict lung growth, such as oligohydramnios, Potter syndrome (with bilateral renal agenesis or dysplasia), abnormalities of the thoracic cage, Scimitar syndrome (right-sided pulmonary hypoplasia), and diaphragmatic hernia (usually left-sided hypoplasia). More than 50% of patients have associated cardiac, gut, or skeletal malformations. They may have a small thoracic cage, decreased breath sounds on the affected side, and a mediastinal shift to the side of the lesion. Therefore, aplasia of the right lung can be confused with dextrocardia. Patients may present with lung infections, dyspnea upon exertion, and/or scoliosis.

 

 

Hypovascularity of the entire left lung in a 16-year-old patient with mild exercise intolerance. This patient had hypoplasia of the left lung

 

 

Bronchogram in simple hypoplastic left lung: the left lung is reduced in volume.

 

Displacement of mediastinum and heart to the left. Loculated translucencies in right middle and lower lung fields with flattened right diaphragm. (reprinted by permission from W. B. Saunders Company Ltd. Manual of Neonatal Emergency X-Ray Interpretation, 1994.)

 

                   Patients with pulmonary agenesis and pulmonary hypoplasia seem to have one of 3 presentations. The first group consists of patients with insufficient lung tissue who may have received mechanical ventilation for some time. However, ventilator-induced lung injury results in slow decompensation and death. The second group of patients is identified serendipitously when chest radiography is obtained to assess a minor complaint. These patients require no intervention. The third group does not have respiratory distress requiring mechanical ventilation, but they have respiratory limitations to activity or kinking of the airway with shift of the lung to the contralateral side of the chest. In addition to the aplasia or hypoplasia, congenital narrowing of the upper airway also affects many patients.

 

Cystic adenomatoid malformation

Cystic adenomatoid malformation is a defect in the development of the terminal bronchioles. A hamartomatous proliferation of cysts occurs and resembles bronchioles (airways without cartilage).

          

Clinic

                    In approximately 60% of patients, cystic adenomatoid malformation manifests soon after the neonatal period. It results in recurrent infections because the mucociliary clearance is poor. Malignancy can occur in the cystic adenomatoid malformation (pulmonary blastoma, rhabdomyosarcoma, and bronchoalveolar carcinoma).

                     The disease may occur immediately after birth or later, sometimes even in  teens agers, depending on the join of infection. Patients complain of a cough with purulent sputum, frequent colds, dyspnea during physical exertion, fatigue. Early the developing phalanges and nail changes in the form of drum sticks and watch glasses

occur. As result of the decline in lung maturation asymmetry of the chest is observed. Children suffer from malnutrition and have physical retardation. On auscultation over the affected areas of lung there are constantly listened dry and moist rales of various sizes.

 

X-ray picture depends on the level and extent of damage of bronchial tree. In hypoplasia of the lung there is marked shift of the mediastinum in the affected side, the high standing of the diaphragm. Affected lung is reduced in volume, sealed, sometimes with annular illumination. In hypoplastic lobe pattern develops equity fibroatelectasia with a decrease and compaction percentage, usually pressed against the mediastinum and therefore not always immediately visible. Adjacent healthy areas of the lung appear to be more transparent in comparison with the opposite lung due to hyperinflation.

 

 Cystic adenomatoid malformation of right lung          

 Polycystiс malformation of left lung   

Polycystiс malformation of the upper lobe of right lung

 

 

Most demonstrably picture is given at bronchography, and computed tomography (CT). They reveal multiple cystic cavities, which are the ends of lobe, segmental or smaller bronchi. At angiopulmonography may be found signs of malformation of the pulmonary blood vessels – their thinness, lack of contrasting small branches, the expansion of branching angles.

   

Treatment

             In cystic adenomatoid malformation, resection of even asymptomatic masses is recommended because of the risk for infection, hemorrhage, acute respiratory compromise (which may occur anytime), and neoplastic transformation. This disease is usually segmental; however, as noted for sequestration, lobectomy may reduce morbidity.

            During surgery, lung cysts are often found to be cystic adenomatoid malformations, though simple cysts do occur. Some lesions can be shelled out or unroofed. If they are not congenital but related to barotrauma, they may communicate directly with small bronchi. In this case, unroofing leads to major air leaks. These lesions can sometimes be controlled with figure-8 sutures, but wedge resection, segmentectomy, or even lobectomy may be required to avoid a bronchopleural fistula.

 

                  Congenital (real) solitary cysts

                   Bronchogenic cysts are also known as foregut duplication. They arise from an abnormal budding of the ventral foregut. Approximately 85% are mediastinal, and 15% are intrapulmonary. The peripheral cysts are multiple and appear late in gestation. They may be filled with air or fluid, or they may have air-fluid levels. The cysts can be central or peripheral. Many are asymptomatic, but incidental findings may be observed on chest radiography. Infection, hemorrhage, and, in rare cases, malignancy can occur. Respiratory distress may result in a stridor or wheeze. Airtrapping may lead to emphysema, atelectasis, or both. Dysphagia, chest pain, and epigastric discomfort can occur.

                Lung cysts are rare lesions that may arise from any of the parenchymal tissues of the lung. They can cause symptoms if they enlarge and occupy substantial space. Resection is performed to diagnose lung cyst and to stop the progression of symptoms.

                Bronchogenic cysts represent outpouchings of the ventral foregut in the early part of gestation. These outpouchings generally arise close to the bronchial tree. A cyst may become infected, or it may compress adjacent structures to produce signs and symptoms. Chronic infection and inflammation may predispose the patient to malignancy. Peripheral cysts appear late in gestation and are multiple.

     Bronchogenic cysts are most commonly mediastinal in a pericarinal, paratracheal, or retrocardiac location. The cysts are thin walled and lined with columnar epithelium. The common central cysts represent outpouchings of the ventral foregut in the early part of gestation.

                     

Clinics

                     Many cysts are asymptomatic, but incidental findings may be observed on chest radiography. Infection, hemorrhage, and, in rare cases, malignancy can occur. Respiratory distress may result in a stridor or wheeze. Airtrapping may lead to emphysema, atelectasis, or both. Dysphagia, chest pain, and epigastric discomfort can occur.

                      Unlike cystic hypoplasia solitary cysts usually do not have widespread reports of bronchi and therefore become infected less often and later than cystic. True cysts of the lung at birth are filled with mucus, often having a dark brown color (so-called chocolate cysts). Communicating with the small bronchi of the cyst may be complicated by valve mechanism (tense cysts), a breakthrough in the pleural cavity or infection.

       At infected cysts on the first place there are the symptoms of purulent intoxication: high fever, sweating, increasing weakness, lethargy, loss of appetite. For large scale cysts symptoms of respiratory failure may appear: shortness of breath, cyanosis of lips and limbs, which are more common in young children. Characteristic is   the appearance of dry or wet cough. At percussion over the cavity of the cyst when it is of sufficient size may be noted dullness of sound and auscultation with the weakening of breath and wheezing of various sizes.

       On plain radiograms and tomograms lung festering cysts are seen as round or oval hollows formations with, as a rule, the level of liquid and air over it. Unlike lung abscesses inner and outer contours of the cyst are clear and smooth, perifocal reaction is expressed slightly. Often cysts are a multi, and the upper edge of the liquid in individual cells may be at different levels.  Bronchi and vessels, clearly visible in contrasting and tomograms uniformly bend around the contours of the cyst, which is not at the case with lung abscess.

 Bronchogenic cyst. Conventional radiographs

 demonstrate a subcarinal mass

 

. Bronchogenic cyst. Media file shows a right paratracheal mass.

 

Bronchogenic cyst. CT scan demonstrates a thin-walled cyst in the right upper lobe.

 

           Review chest X-ray of the child with a congenital air cyst of the right lung in direct projection: annular shadow of the cyst is indicated by arrows.

 

  

Review chest X-ray of the child with a congenital air cyst of the right lung in lateral projection: annular shadow of the cyst is indicated by arrows.

 

 

 In the differential diagnosis of purulant cysts it is needed to remember about tuberculous cavity, the more so that their localization (mainly in the upper lobes) usually coincides. For tuberculous cavern characteristic features are rugged, “moth” inner contour and fibrose focal shadows on the periphery of the cavity. In addition, the cavity is characterized by the presence of shadows and enlarged lymph gland in the root of the lungs and shadow of draining bronchus, well visible on the tomograms.

 

PULMONARY SEQUESTRATION

 

               Pulmonary sequestration accounts for 6% of all congenital lung malformations and mostly occurs in the lower lobes. A sequestration is a bronchopulmonary tissue without a normal bronchial communication and with normal or anomalous vascular supply. Sequestered lung may be intralobar or extralobar.

                                           A pulmonary sequestration there is a benign mass of non-functioning lung tissue that appears during early lung development. This lesion has no connection with the airway and receives its blood supply from the systemic circulation usually off the abdominal or thoracic aorta. The most common type of BPS is formed within the normal lung itself and is referred to as intralobar. The other type of BPS is known as extralobar, and is formed outside the normal lung. This type of lesion is usually found in the chest cavity although rarely it may be found in the abdomen. There is a higher incidence of associated anomalies in babies with extralobar BPS.

                                        The involved lung segments can be classified on the basis of their pleural coverage into intrapulmonary or extrapulmonary types. Variants of pulmonary sequestration are described as disconnected or abnormally communicative bronchopulmonary masses with normal or anomalous vascular supply. The lesions may have some sort of communication with the gut.

 

                                      Clinic

                     About 50% of pulmonary sequestration cases are intrapulmonic, and 60% of intrapulmonic cases occur in the left lower lobe with equal sex distributions. Patients with intrapulmonary sequestration usually present late. They may have a chronic cough, recurrent pneumonias, or poor exercise performance. Systemic arterial flow may produce a murmur, and shunts may lead to congestive cardiac failure. Squamous cell carcinoma, adenocarcinoma, and rhabdomyosarcoma may arise in the sequestration.

                   Approximately 95% of extrapulmonary cases are left sided. Most extrapulmonary cases are detected in infancy, with boys affected 4 times more than girls. Infants usually present with a chronic cough and recurrent chest infections. Radiographs may reveal signs of consolidation. If communication with the gut is present, children may present with vomiting, failure to thrive due to poor oral intake, and abdominal pain.

         Clinical manifestations are due to inflammatory changes: cough, fever, shortness of breath, over the lesions listened small bubbling moist rales.

         There are three forms of the pathological process:

§   bronchiectasis, in which repeated inflammation leads to

              fusion of lung tissue and secondary connection with

                bronchial tree

§   pseudotumoral that is characterized by small clinical symptoms

§   local abscess formation or empyema

 

The main distinguishing feature of sequestration of the lung is an additional large vessel which deviates from the aorta and branching in the sequestered lung tissue. This vessel can be identified at aortography, tomography and CT. Sometimes   it is finding during the operation as an accident.

            Diagnose of pulmonary sequestration is based on angiography. Much less importance has bronchography. At radiograph may be darkening of the affected segment inflammation.

 Sequestration of the lung. There is cystic changed area of S10 of the lower lobe of the left lung.

 

                                       Aortogramme at sequestration of the lower

                                                                    lobe of right lung: the additional vessel (2)

                                                                    is going from the aorta (1)

                                                                    to the sequestered part of lung.

                

 

Review X-ray of the child with intrapulmonary sequestration in low medium sections of the right lung: in the zone sequestration there is shading lung tissue (indicated by arrow).

 

Surgical treatment: resection of the sequestered area.

                Resection is recommended, even in asymptomatic patients, to prevent infection, hemorrhage, shunting from arteriovenous anastomoses, or compression of normal lung mass leading to respiratory distress. Lobectomy can usually be performed. For patients with intralobar sequestration, segmentectomy may suffice. Segmentectomy is relatively difficult, but preserves additional functioning lung tissue.

 

Prognosis is  favorable.

 

 

Malformations of trachea and bronchi

 

                        

 

Clinic

                     Clinically there is the recurrent tracheobronchitis, in the course of which predominates bitonal irritating cough with prolonged sputum discharge.

                   Abnormalities of the trachea and bronchi may be suspected by the presence of the characteristic cough with metal, vibration shade, hard forced exhalation and the presence of hard discharged purulent sputum in children. On auscultation over the lungs there are listened many variegated wheezing, which do not disappear after cough. Radiographic examination often reveals signs of bronchiectasis, and in patients with acute inflammation the massive bilateral pneumonia, often with abscess formation.

          Frequent symptoms are chronic intoxication and hypoxemia, pallor, retarded physical development, the deformation of the fingers on the type of drum sticks. In the lungs, there are different changes in percussion sounds, a variety of wheezing.

          Studies of lung function and blood gas composition reveal pronounced degree of combined ventilatory insufficiency and hypoxemia. 

                   Bronchofibroscopy helps to establish the correct diagnosis. At tracheobronhomegalia there are determined a significant expansion of the lumen of the trachea and main bronchi, deformation of their walls with the curvature of the cartilaginous rings and deep, sac interchondral intervals, a thickening of the mucous membrane in the form of circular folds and the almost total collapse of the lumen by coughing and forced expiration, expiratory stenosis and the presence of inflammatory changes in the trachea and bronchi. Congenital stenosis of the major bronchi is defined as smooth wall sunken or membranous narrowing with a small round hole at the center.

Bronchoscopy – the examination of the bronchi (the main airways of the lungs) using a flexible tube (bronchoscope). Bronchoscopy helps to evaluate and diagnose lung problems, assess blockages, obtain samples of tissue and/or fluid, and/or to help remove a foreign body.

bronchoalveolar lavage – to remove cells from lower respiratory tract to help identify inflammation and exclude certain causes.

lung biopsy – to remove tissue from the lung for examination in the pathology laboratory.

 

  

 Bronchoscopical sings at tracheobronchomegalia (Moanier-Kuhn syndrome)

 

                Treatment is provided as at endobronchitis.

             Prognosis for life is favourable.

 

 

Williams– Campbell syndrome (generalized congenital bronchiectasis)

 

                  Syndrome is based on genetically inferiority of bronchial wall due to the bronchial cartilage defect. It arises under the influence of various pathological processes on formation of lungs in the embryonic period.

                    The morphological defect is associated with segmental and subsegmental bronchi, most of the lower lobes. As with lobar emphysema, lung tissue is air. Therefore it is believed that these two diseases occur as a result of one and the same process. It occurs with a frequency of 1:100 000.

 

                 

                 

              The clinical picture is the bronchial obstruction and bronchopulmonary infection. Usually in the first year of life an acute pneumonia occurs, and then eventually a chronic bronchopulmonary process is formed. Objectively chest is like hump.The cough is resistant with shortness of breath. At percussion of the lungs there is bandbox sound, at auscultation– dry and moist rales of various sizes. Phalanges and nails become “drumsticks”, “hour-glass”, a violation of external respiration is present.

                Radiological findings in the lungs are increased pulmonary pattern, the phenomenon of emphysema. At bronchography there are determined generalize bronchiectasis with balloon expansion during inspiration and collapse (by closing the walls) during expiration.

 

 

 

The scheme of the diameter changes of the bronchi at Williams – Campbell syndrome on inhalation (a) and expiration (b).

 

                         

Bronchogram of the left lung of the child with the Williams-Campbell syndrome.

 

            Prognosis is poor. Progression of syndrome leads to cardiopulmonary failure, which is the cause of death.

            Treatment is conservative.

 

                          Congenital lobar emphysema

         Congenital lobar emphysema (congenital localized emphysema, gigantic emphysema, tense emphysema) is characterized by stretching of the parenchyma of the lung lobe or a segment with manifestation mainly in early childhood. This anomaly is rare, but late diagnosis quickly leads to death of newborns.

          This disease is characterized by narrowing of the bronchus, it aplasia, dysplasia, and hypertrophy of the mucosa with the formation of folds, which act as valves. Amount of air, which gets into the lungs is more than that which gets out (valve mechanism).

         

                     Massive overinflation of one or more lung lobes occurs postnatally in congenital lobar emphysema. Causes include intrinsic absence or abnormality (bronchomalacia) of cartilaginous rings or external compression by a large pulmonary artery. (Compression of the cartilage usually leads to malacia.) Hyperexpansion of a pulmonary lobe is present after birth when, with negative inspiratory pressure, air can enter the lung. However, the air cannot exit easily because positive pressure causes the softened airway to collapse. The remaining normal lung is then compressed.

             Causes of congenital lobar emphysema include bronchial cartilage deficiency, extrinsic compression by a bronchogenic cyst, a large pulmonary artery, or mucus plugs. Lobar overdistention and airtrapping lead to compressive changes in the rest of the lung.

                                                 

                   Congenital lobar emphysema primarily involves the upper lobes. The left upper lobe is involved in 41% of patients; the right middle lobe, in 34%; and the right upper lobe, in 21%. Involvement of the lower lobes is rare, occurring in fewer than 5% of patients. Congenital cardiac anomalies may be present in as many as 10% of patients. Lesions most commonly occur in whites, in male individuals (male-to-female ratio, 3:1), and in young infants.

 

                                                     Clinic

                          Most patients with congenital lobar emphysema present before 6 months of life. Neonates may present with mild-to-moderate respiratory distress. Mediastinal shift may be present, with hyperresonance and decreased breath sounds on the involved side. Infants present with cough, wheezing, respiratory distress, and cyanosis. Older children may present with recurrent chest infections. On images obtained in neonates, the affected lobe may be slightly opacified, rather than lucent, because it is still filled with fluid. Associated cardiac anomalies occur in as many as 10% of patients.

            The most severe condition is in children in the first days of life. Iewborns there are increasing dyspnea, cyanosis, convulsions, loss of consciousness. One half of the thorax is protuberant. Here there is bandbox percussion sound. Breathing is weakened or absent on auscultation. Radiologically hyper aeration of one lung, mediastinum and heart are displaced in the opposite side. Lung pattern is scanty or absent. The diaphragm is flat, excursion of it is limited, possible mediastinal hernia.

 

 

 

 

 

                         

 

Congenital lobar emphysema on the right side of the chest in a neonate. There is  marked lucent hyperexpansion in the middle lobe of the right lung; this finding is consistent with lobar emphysema. The possibility of tension pneumothorax is unlikely because lung markings are seen in this region, with splaying of the pulmonary vessels. Compressive atelectasis is present in the left upper and right lower areas of the lungs. The mediastinum and heart are shifted to the left. The osseous structures are intact.

 

                       

 

                  Congenital lobar emphysema. Lateral view in the same patient. 

 

                       

 

                                            Congenital left side lobar emphysema.

 

 

               

 

                  Review chest X-ray of the child with congenital emphysema of the upper lobe of left lung: pulmonary tissue of upper lobe of left lung is increased lucent, in the lower areas – reduced by compression of the lower lobe, the mediastinum is shifted to the right.

 

                  

                   The mediastinum is flat and shifted to the right.

 

               The differential diagnosis is carried out with pneumothorax, cysts, diaphragmatic hernia, hypoplasia and aspiration syndrome.

               Prognosis is poor. Children are dying too early. In milder forms the flow can be subacute and chronic. Children are retard in physical development, constantly cough and shortness of breath are observed. There are described cases of the disease with little emphysema diagnosed by chance.

              Treatment. Surgical treatment is needed in the “stress syndrome”, which develops due to compression of the mediastinum. Milder forms are treated conservatively.

               Progressive airtrapping leads to respiratory and circulatory compromise in infancy. Emergency lobectomy may be required. A patient with respiratory distress whose chest radiograph reveals a hyperlucency on one side and mediastinal shift usually has a tension pneumothorax. However, one must consider congenital lobar emphysema, especially in the newborn. The diagnosis can usually be determined by looking at the edges of the hyperlucent area. In pneumothorax, the edges are convex and outline the chest wall, whereas in congenital lobar emphysema, they are concave and outline the cystic structure of an overexpanded lobe.

 

                Placing a chest tube in the hyperlucent airspace of congenital lobar emphysema decreases ventilation as air takes the path of least resistance out the chest tube from the bronchus rather than expanding the stiff infant lung in the remaining lobes. Prompt thoracotomy relieves the pressure inside a hyperexpanded lobe and allows the other compressed areas to ventilate. This overexpansion often stretches and dissects the bronchi and vessels, facilitating lobectomy. In cases that are detected early or surgically treated because of radiographic findings and not because of symptoms, the abnormal lobe may be difficult to identify during surgery. Therefore, in these cases, radiographs and CT scans must be carefully reviewed preoperatively.

 

 

                Tracheoesophageal and bronhoesophageal fistulas

 

 

                Tracheoesophageal and bronchoesophageal fistulas manifest themselves at the first feeding of the child by severe attack of asphyxia, cough and cyanosis. Quickly severe aspiration pneumonia occurs and is usually fatal in saved hernia. This defect is often combined with atresia of the esophagus. It is diagnosed with the introduction of X-ray contrast into the trachea and bronchi, or bronchoscopically with the introduction of contrast into the esophagus.

 

                                       Clinic

                  Clinical manifestations of this defect depend on the width of the message of the esophagus with the trachea. The wider the anastomosis, the more rapidly and early clinical symptoms arise. Any form of congenital isolated tracheoesophageal fistula mainly manifests is respiratory failure (cyanosis, shortness of breath, coughing, choking) that occur during breastfeeding. This relationship of breathing disorders in feeding is the cardinal symptom, distinguishing esophageal-endotracheal anastomosis from other types of diseases ieonates, occurring respiratory disorders. With a wide fistula respiratory failure is so severe that requires removal of the child on mechanical ventilation. Suction of gastric contents in this case from the intubation tube (tracheal) makes the diagnosis tracheoesophageal fistula uncertain.

                       

           Radiograph of the chest. Radiocontrast substance is flowing through the tracheoesophageal fistula from the esophagus to tracheobronchial tree.

 

              Surgical treatment must be performed as soon as possible. In this case the prognosis for life is good.

 

 

                Although congenital lung malformations are rare, they are important disorders because they may lead to considerable morbidity and mortality (eg, infection, hemorrhage, respiratory failure). Prognosis depends on the size of the lesion, and the degree of functional impairment. Small lesions may remain asymptomatic. Failure to recognize a malformation may lead to inappropriate intervention. 

 

 

                                 Hereditary lung diseases

 

                                 Kartagener syndrome

               Kartagener syndrome has autosomal dominant type of inheritance with 50% penentrance (frequency of 1:50 000) of the pathological gene. It occurs more frequently in kinship marriages.

            Kartagener’s syndrome – the combined congenital defect characterized by a triad of symptoms:

Ø sinus viscerus inversus

Ø chronic bronchopulmonary process

Ø pathology of paranasal sinuses (hypoplasia or chronic sinusitis).

 

             Syndrome reverse arrangement of lungs is always combined with dextrocardia, and sometimes the opposite arrangement of the abdominal cavity. Reverse position of internal organs is often combined with a violation of mucociliary clearance due to congenital disorders of motor function of the ciliated epithelium of the respiratory tract.

           

 

                                      Clinic

 

              Frequent respiratory infections, recurrent bronchitis, pneumonia in the first months of life are characteristic. There is early occurrence of chronic bronchitis and pneumonia with the development of bronchiectasis and bronchiectasis symptoms (retard physical development, intoxication symptoms, cough with purulent sputum, deformation of terminal phalanges of the type of drum sticks and nails in the form of watch glasses).

               Percussion and auscultation determine dextrocardia. In the lungs, predominantly in the lower parts, mostly on the right there is variety of moist and dry rales. Periods of exacerbation are accompanied by fever, deterioration of general condition and the growth of the symptoms of intoxication.

                 Nasal breathing is hard with purulent nasal discharge. Often, there are recurrent or chronic sinusitis, otitis, polyposis of the mucous membrane of the nose and sinus.

 

                               Diagnostic criteria

 

 

             The diagnosis of Kartagener syndrome is set on the basis of the following

                                       syndromes.

1. Respiratory Syndrome – cough with sputum (purulent).

2. Bronchopulmonary syndrome – a shortening of the sound on percussion over

    separate areas of the lungs; persistent wet fine bubbling rales on both sides.

3. Respiratory distress syndrome – shortness of breath at rest and at low physical

     activity.

4. Symptoms of chronic hypoxia: change of the terminal phalanges in the form of

    drumsticks, dystrophy.

5. The reverse arrangement of internal organs – dextrocardia, left-sided localization of

     the lungs, difficulty iasal breathing, sinusitis.

 

 

             Often patients with Kartagener syndrome have other defects and anomalies: polydactyly, heart, kidney anomalies, hypofunction of endocrine system etc.

              At the X-ray of lungs there are reverse position of internal organs, diffuse deformation of lung pattern, may be cysts in the lungs. The X-ray of paranasal sinuses reveales decrease lucency of sinuses.

                          

X-ray of the paranasal sinuses of girl 14 years old with chronic lung disease and polypous-purulent antritoetmoiditis.

 

                         

                                       X-ray of lungs of the same child.

 

 

                Bronchoscopical picture in patients with Kartagener syndrome is characterised by diffuse purulent endobronchitis. Bronchography reveales deformation of the bronchi, bronchiectasis and cysts.

 

 

         

         Bronchogram of the same patient.

 

         The differential diagnosis is carried out with chronic pneumonia, congenital abnormalities of bronchopulmonary system (agenesia, aplasia or hypoplasia of the right lung), in which heart is shifted into the right half of the thorax.

          The main method of treatment of Kartagener syndrome is a conservative therapy aimed at eliminating or reducing the activity of the inflammatory process in the bronchi and lungs, improving drainage and ventilation functions.

          Prognosis depends on the nature, incidence of bronchopulmonary process, the frequency of exacerbations, severity of the disease. With proper systematic treatment and regular rehabilitation prognosis is relatively favorable.

 

                   Idiopathic fibrosing alveolitis

                  (Hamman-Rich Syndrome)

 

(LV Hamman, Amer. Physician, 1877-1946; AR Rich, Amer. Physician, was born in 1893; synonyms: idiopathic pulmonary fibrosis) – a progressive inflammatory lung lesion of unknowature, which leads to the development of diffuse fibrosis, chronic respiratory and cardiopulmonary failure.

Hamman-Rich syndrome is a rare disease characterized by rapidly progressive diffuse pulmonary fibrosis with the development of respiratory failure, pulmonary hypertension and cor pulmonale.

 

                  

 

                                   Etiology.

 

-Hereditary disease with autosomal dominant type of inheritance.

 

                               

                                 Pathogenesis.

 

             Previously in the development of syndrome there was attached importance of auto-sensitization in connection with which diseases was entered to a group of connective tissue disease. Now there is considered that the syndrome occurs as the result of repeated inflammatory bronchopulmonary diseases. Morphological substrate is perialveolar fibrosis, which reduces the elasticity and pliability of lung tissue and thus worsens the excursion of the lungs. Due to the thickening of the interalveolar septa diffusion of gases in the blood is damaged, which leads to hypoxemia and hypoxia, hypercapnia.

 

                                  Clinic

                    The disease manifests itself mainly in school age children and adolescents, but the first signs of it (on history) almost half of the patients are detected in pre-school age. The disease begins gradually after an attack of influenza, repeated pneumonia, bronchitis and often measles. There are a spastic, dry cough, shortness of breath and cyanosis during physical exertion, sometimes chest pain, feeling of tightness in the chest.

                    Percussion defines an insignificant shortening of sound in the basal zones or no change, decrease of excursion of lungs, reducing difference of lung volume during inspiration and expiration. Auscultation reveals inconstant fine bubbling or crepitation moist rales, sometimes may be weakening of breathing in the lower areas.

                   Further there are increasing of cyanosis around the mouth, acrocyanosis, fingers take the form “drumsticks”. The child retards in weight and growth. Thorax is flattened, circumference of the neck disproportionately increases (due to increased contractility of the neck muscles that perform the role of auxiliary respiratory muscles during difficult breathing). Pulmonary heart syndrome is developing with the expansion of the cardiac dullness, signs of cardiovascular failure.

 

 

              In the blood – polycythemia, may be polyglobulinemia, ESR is increased, especially in acute periods, concentrations of gamma-globulin is elevated.

             The study of respiratory function reveal the decline of lung volumes, reduced lung compliance and difficulty in passing the oxygen through the alveolar-capillary membrane, and therefore change in the gas composition of the blood (hypoxemia, then hypercapnia) and indicators of acid-base status.

 

                    

                    After treatment with corticosteroids LVC, total lung capacity, the inspiratory volume is practically unchanged. Radiological investigation in the initial period reveals that the interstitial pattern is enhanced, later nodular formation appear on its background. In periods of exacerbations there is determined multiple shadowing, alternating with areas of particularly clear lung fields. Shadowing is usually localized in the roots and lower parts. Pneumothorax may be possible complication.

 

                                                                     

                                                   Representative CT scan image from a person with

                                                        idiopathic pulmonary fibrosis.

                                                                             

                                                                    Idiopathic fibrosing alveolitis.

Detail of chest radiograph with                                                                          interstitial pneumosclerosis with a primary lesion of the alveolar connective tissue (fibrosing alveolitis):  small meshy pattern of lung.

 

Micropreparations of lung at diffuse interstitial pneumosclerosis: interseptums are extremely thickened and sclerotic; coloured by Van Gieson; × 24.

 

 

 

A. Lung showing extensive interstitial and alveolar fibrosis.

    Note the increased interstitial cellularity with numerous fibroblasts.

B. Patchy areas of alveolar septal thickening and prominent hyaline membranes.

C. Higher magnification showing typical dense laminated hyaline membranes.

D. Alveolar septum showing prominent type II pneumocyte proliferation.

E. Abundant polymorphonuclear leukocytes fill alveolar spaces with focal destruction of alveolar septa.

F. Higher power magnification showing the antraalveolar exudate

composed mainly of polymorphonuclear leukocytes, red blood cells, and fibrin.

 

Original magnifications: A & B, x 50; C, x 100; D, x 158; E, x 50; F, x 158.

 

 

 

Electron-microscopic picture of fibrosed alveoli.

 

 

 

                    CT-image of meshy lung

 

 

Vacuolization of macrophages in amiodarone alveolitis. Materials obtained through bronchoalveolar lavage.

 

 

            The disease is undulating with periods of exacerbation and remission. However, remission time zone fibrotic changes do not disappear, and rates of respiratory still significantly reduced. Depending on the frequency and duration of periods of exacerbation, some authors distinguish subacute and chronic forms of course. In subacute form periods of exacerbation are more frequent, accompanied by fever reaction, while at chronic progression of pulmonary fibrosis occurs gradually and manifests the growth of respiratory failure. At present, long-term treatment of patients with corticosteroid differences within these two forms have become less pronounced. There are two clinical forms of idiopathic diffuse pulmonary fibrosis depending on diffusion of gases in the lungs. In most cases diffusion capacity is decreased, but in 10-15% of patients it is withiormal limits. In such patients the process is easier, retarded growth and weight are insignificant or absent.

               Diagnosis is mainly based on typical clinical signs of disease.

                  Diagnostic criteria of IFA

Clinical

·      progressive dyspnea

·      cyanosis

·      dry cough

·      progressive loss of weight

·      tender crepitation wheezing

·      thickening of hand phalanges nails

Radiographic

·      diffuse fibrosis

·      deformation of the pulmonary pattern

·      presence of focal shadows

Indicators of external respiration

·      decrease of lung volumes

·      hypoxemia

Indicators of regional lung function

·      diffuse irregular distribution of radionucleids

·      regional lung volume reduction

·      smoothing apex-basal ingredients

.

                       Differential diagnose.

 

                 Differential diagnosis should be carried out with exogenous allergic alveolitis caused by the inhalation of various organic antigens. Such condition is observed in children with close and prolonged contact with animals and birds. Clinically, the disease is very similar to idiopathic diffuse pulmonary fibrosis. Exogenous allergic alveolitis is diagnosed based on the detection of precipital antibodies against animal serum and excrements. Excluding the effects of antigen in combination with corticosteroid therapy in such patients may lead to recovery. In idiopathic pulmonary fibrosis diffused all lung volumes, lung elasticity during therapy with corticosteroids did not decreased even at satisfactory state of health of patients and the absence of exacerbations process.

 

                                           Treatment.

             Corticosteroid therapy is used. Originally prednisolone is prescribed at a dose of 1 mg / kg per day. The dose is gradually reducing, but maintenance therapy (5-10 mg prednisolone per day), continue for many months. In severe progressive course corticosteroids are combined with cytostatic drugs (azathioprine, etc.). During exacerbations, the accession of pneumonia, bronchitis, corticosteroids are combined with antibiotics. In addition, antihistamines, vitamins, symptomatic treatment is used.

 

                             Prognosis is unfavorable.

 

 

 

                    IDIOPATHIC PULMONARY HEMOSIDEROSIS (IPH)

 

 

              Idiopathic pulmonary hemosiderosis – (syn.: idiopathic pulmonary progressive induration, purpura immune pulmonary disease, Delen- Gellerstedt disease, etc.) – a disease in which the main symptom is recurrent hemorrhage in lung tissue and the subsequent development of fibrosis and hemosiderin deposition.

              At first the disease was described by Virhow in 1864 under the name “brown lung induration”. The basis of the disease is bleeding in lung alveoli, which has usually a diffuse character. Free iron as a result of the collapse of erythrocyte is absorbed by macrophages, which may be found in the sputum of patients (siderophages).

             It is considered that the anomaly of arteriolar-venular anastomoses and pathological structure of the connective tissue of the lungs is hereditary.

 

Pathogenesis.

 

                  As a result of an inherited defect forced hemocirculation of lung tissue occurs with a significant exit in it red blood cells (per diapedesis) and the progressive sensitization of the organism to them. Iron from haemolysed red cells becomes stable connected with pathologic sulfated mucopolysaccharides and dose not return to the blood, dose not include in the metabolism and synthesis of hemoglobin. Therefore, anemia accompanying IPH is related with iron deficiency. Iron deposition, hemorrhage, inflammation leads to pneumosclerosis, pulmonary hypertension and chronic pulmonary failure. Allergic lesions of the joints, skin, heart, kidneys may develop.

 

            Morphologically (based on open lung biopsy) there are revealed red blood cells in the cavity of the alveoli, hemosiderin-containing macrophages (siderophages), diffuse interstitial fibrosis, sclerosis of small vessels of the lungs.

 

Clinic

         The clinical picture of IPH is composed of the respiratory and hematological symptoms. At exacerbation cough appears, in older children accompanied by hemoptysis – the appearance of blood in the sputum. Small children do not expectorate sputum, they swallow it. With abundant pulmonary hemorrhage, they may have vomiting with ingested blood.

           Severity of the clinical picture in the lungs depends on the amount of haemorrhage into the alveoli. Together with cough and fever dyspnea and cyanosis occur. Wheezing in the lungs is listened. Radiographic changes appear in the form of large focus shadows in both lungs. The disease is often diagnosed as pneumonia, which leads to the appointment of inadequate treatment and the worsening of patient’s condition.

 

 

Laboratory data

1. Complete blood count: signs of anemia, aniso– and poicylocytosis, reticulocytosis, during exacerbation – leukocytosis, leukocyte shift to the left, increasing ESR.

2. Biochemical blood analysis: an increase of the level of indirect bilirubin and decrease of serum iron.

3. General sputum analysis: siderophages are found.

4. Investigation of lung biopsy: at a period of remission hemorrhage is weakly expressed, mainly in the cortical areas of the lung, pleura in these areas is thickened, in the alveoli, intralobulary, around vessels, peribronhially and in connective tissue there are many hemosiderophages, reactive fibrosis. At exacerbation alveolars are filled with fresh red cells, alveolar macrophages containing hemosiderin.

5. Peculiarity of radiological changes in the IPH is the rapid regression of foci shadows.

                                                                                                                

                

Review chest X-ray of patients with idiopathic pulmonary hemosiderosis: small meshy diffuse lung pattern deformation caused by compaction of interstitial lung tissue, multiple foci of diffused small monomorphic shadows.

 

                   

                  Idiopathic pulmonary hemosiderosis.

 

                    Hemosiderophages in hemoptysis of a patient with IPH.

                    Materials obtained through bronchoalveolar lavage.

 

                  In some cases, the X-ray of the chest indicates diffuse small shadows in both lungs, which are the reason of mistake diagnosis of miliary tuberculosis of the lungs.

             Changes in the lungs that maybe detected with X-rays can vary widely: from small to massive infiltration shadows accompanied by atelectasis, emphysema, and reaction from the lymph nodes of the roots of the lungs.

            Immediately after the exacerbation, which lasts 3-5 days, there is marked anemia – microcytic and hypochromic. The level of serum iron falls. In the biochemical analysis of blood there may be elevated levels of bilirubin. Since the regenerative bone marrow function does not suffer, in the peripheral blood reticulocytes appear. In young children in the fecal analysis blood test may be positive (swallowed by coughing phlegm with blood). Often there is hepatosplenomegaly.

           The course of idiopathic pulmonary hemosiderosis is undulating. Periods of crises alternate with periods of remission varying duration. Acute and subacute forms   are relatively distinguishing. At acute form of disease there are a significant worsening, weakness, dyspnea. Older children complain of chest pain, cough with small amount of sputum, relaxed breathing and wet wheezing are listened in lungs. Perhaps may be raising the temperature to febrile scores. There is rapidly increasing cell anemia.

          In the subacute form of IPH pallor of the skin, the symptoms of intoxication gradually develop. Exacerbations of the disease are more severe.

 

Diagnose.

 

                 Diagnostic significance has the discovery in sputum or endotracheal aspirate, and in some cases, in gastric lavage sidergophages. Puncture biopsy of the lung is accompanied with serious complications.

          Investigation of respiratory function detects or normal rates of ventilation, if the duration of the disease is little, or restrictive severe violations, reduced lung diffusion capacity, if the disease is prolonged with severe exacerbations.

           If repeated respiratory diseases occur every time with anemia, an unusual radiological pattern in the lungs and ineffectiveness of antiinflammatory therapy are present such a patient should be examined for the presence of IPH.

                    

                                                 Spirometer SPIROVIT SP-1

 Lung Volumes and capacities

 

 

 

                                           

 

          Differential diagnosis should be conducted with tuberculosis and fungal infections of the lungs.

                                            Treatment.

           Treatment of patients with IPH involves the appointment of corticosteroid drugs and symptomatic treatment. During periods of exacerbation oxygen therapy, glucocorticoids, antibiotics, disintoxication, vitamin and treatment of anemia are indicated. If you find high levels of precipients to cow’s milk this product is excluded from the diet. Prednisolone is appointed by the dose of 1 -1.5 mg / kg to achieve clinical and laboratory remission. There are reports that after splenectomy resistant prolonged remission may occur.

 

          Prognosis is serious. Half of the children die in the first five years after onset. Subsequent exacerbations are more difficulty to treat, the average life span of a sick is 2-3 years, rarely – more. Immediate causes of death often are acute massive pulmonary hemorrhage or progressive pulmonary heart failure. Perhaps there is a combination of both reasons. And to predict the probability of acute pulmonary hemorrhage is not possible.

 

 

Primary pulmonary hypertension (PPH)

 

          Primary pulmonary hypertension is a rise in pressure in the pulmonary artery and right ventricular hypertrophy which are not associated with congenital or acquired pathology of the heart and lungs.

          In 1901 Auerza described patient with marked cyanosis, right ventricular hypertrophy and chronic bronchitis. Family form of primary PH was first described in the report Clarke et al. in 1927. The authors have noted a similar clinic and morphological changes in autopsy material from two sisters, 5 – and 8-years of age and confirmed the presence of primary PH. There are more than 20 titles of the disease. Among them: Idiopathic right ventricular hypertrophy, primary pulmonary artery sclerosis, isolated pulmonary hypertension, a disease Aersa etc.

          Currently there is established the gene BMPR2, localized in the locus of the second chromosome 2q33, which regulates growth and proliferation of endothelial cells. This gene is responsible for the development of a family primary pulmonary hypertension. The disease is inherited in an autosomal-dominant type with incomplete penetrance, which manifests itself in a population with a frequency of 1-2 cases per 1 million people. Family nature of PPH is approximately in 6% of patients, the remaining cases are sporadic. Family form of PPH is not clinically different from sporadic, but after the first symptoms is usually diagnosed earlier. In sporadic cases diagnose of PPH is usually established in the advanced stages of disease.

                                          

                                       Pathomorphology.

            Pathomorphologic changes in primary pulmonary hypertension are clear. There is right ventricular hypertrophy, expansion of large branches of pulmonary artery with layer fibroelastosis of the intima, the presence of atriovenose anastomoses, thrombosis and fibrinous necrotizing arteritis of small branches of the pulmonary artery.

 

                                          Clinic.

                       In the initial stages the objective symptoms are mild, cardialgia in children is atypical. There is a poor tolerance to physical activity through the development of shortness of breath, sometimes accompanied by attacks of breathlessness. The appearance of syncope point at severe phase of the disease, they often appear at an exercise, RV heart failure increases.

           There are three stages of development of pulmonary hypertension in children. At I stage increased pulmonary pressure is the only hemodynamic abnormality, patients do not have distinct symptoms, there may be shortness of breath during physical activity, which often does not wary the doctors and patients for the presence of the disease and is usually associated with poor training of the body.

           When a decrease in cardiac output (II stage) occurs, there are detailed clinical symptoms in the form of hypoxemia, dyspnea and syncope. Pressure in the pulmonary artery remains stable at a high level.

           With the advent of right heart failure III stage of disease occurs: in this case, despite the consistently high values of pulmonary pressure, cardiac output falls sharply, there are venous congestion and peripheral edema.

            There is variability in the duration of different stages of the disease, an average from 6 months to 6 years or more from the stage of minimal hemodynamic changes to the lethal disease. The reason of death is the developing prolonged functional overload the right heart, destructive and sclerotic changes in lung tissue and myocardium.

      PPH affects mainly young patients, and the disease is usually fatal, although in the literature there are described isolated cases of spontaneous remission.

           It is assumed that the therapeutic activities of the patients of childhood will be more effective, as the remodeling of the pulmonary vessels in children can be prevented and even reversed. The development of new directions in treatment can increase survival and improve quality of life of patients.  

 

                     

                               The child 13 yrs old with PPH.

                       Weight – 33,5 kg (norm – 44,1 ± 8,68 kg), height – 144 cm (average 155,7 ± 6,57 cm). There are mixed apnea, tachypnea to 38 per minute. Skin is pale, cyanosis of the lips, acrocyanosis.

 

 

Deformation of the terminal phalanges as “drumsticks” and “watch glasses”, enlargement of the abdomen (ascites) in patient with PPH.

 

ECG of patient with PPH. Sinus tachycardia (cardiac rate 140 per minute).

Rightgram. Atrioventricular blockade Іst. (PQ–0,20″). Expressed hypertrophy of right

ventricular. Dilatation of the both auricles.

 

           

                             A                                                                         B

                                                                                                

                                   

           C                                                                 D

 

A. Hypertrophy of media and adventitia of small pulmonary arteries.

B. Concentric intimal fibrosis of pulmonary arteries with a diameter of 180 microns, there is also hypertrophy of the middle and the outer shell of the vessel.

C. Obliteration of the lumen of the pulmonary artery.

D. Plexiform defect. Note the absence of elastic membrane in the wall plexiform structure.

Increasing x 100. Coloured by Verhoeff’s – Van Gieson.

 

 

 

Muscularization of arterioles: a – hematoxylin and eosin. ґ400 b – immunohistochemical coloration on actin of smooth muscle (SMA). ґ200.

 

Necrotizing arteritis. Coloured                   Rechanaled thrombus of branch of

by hematoxylin and                                     pulmonary artery. Coloured                                         

eosin  ґ100..                                                  by hematoxylin and eosin  ґ40.                                                                     

  

 

EchoCG. A-wave is escaped.  

               Treatment is symptomatic: antihypertensives, anticoagulants, antiplatelet agents. Effects are minor.

               Prognosis is poor. Life expectancy is from several months to 10 years from onset of the first symptoms.

 

 

Hereditary polyorganic  diseases mainly affecting

the respiratory system

 

                                       Cystic fibrosis 

             Cystic fibrosis is a hereditary systemic disease caused by mutation of cystic fibrosis transmembrane regulator and characterized by exocrine glands disorders, severe impaired function of the respiratory and gastro-intestinal tract.

 

                     

Cystic fibrosis is the most common cause of chronic lung disease in children and young adults, and the most common fatal hereditary disorder affecting Caucasians in the US.

                                         Etiology and Pathogenesis

              The disease is the result of gene mutation. Pathological gene is localized in the middle of the long arm of chromosome 7. Cystic fibrosis is inherited in an autosomal-recessive type and is registered in most European countries with a frequency of 1:2000 – 1:2500 newborns. If both parents are heterozygous carriers of the mutated gene, the risk of birth of the child with cystic fibrosis is 25%. According to studies the frequency of heterozygous carriers of the pathological gene is 2-5%.

                                                

 

               This chart can help you determine the genetic probability of having a

                                child with cystic fibrosis

Parents	Chance of Unaffected Child	Chance of Child Carrier	Chance of Child with CF
Unaffected + Carrier	50%	50%	No Chance
Two Carriers	25%	50%	25%
Unaffected + CF Patient	No Chance	100%	No Chance
Carrier + CF Patient	No Chance	50%	50%

 

 

                     Currently, there are about 1000 identifiable gene mutations in cystic fibrosis. The consequence of gene mutation is a disturbance of the structure and function of the protein, known as the cystic fibrosis transmembrane regulator (CFTR). The result is a thickening of the secrets of exocrine glands, difficulty in evacuation secretion and changes in its physical and chemical properties, which, in turn, causes the clinical picture. Changes in the pancreas, respiratory and gastro-intestinal tract are recorded in the prenatal period and with the patient’s age are steadily increasing.

                Isolation of a viscous exocrine glands secretion leads to difficult output and stagnation with subsequent expansion of the excretory ducts of glands, atrophy of glandular tissue and the development of progressive fibrosis. Enzyme activity of the intestine and pancreas is significantly decreased. Along with the formation of sclerosis in the organs there is a violation of the functions of fibroblasts. It is established that fibroblasts of patients with cystic fibrosis produce ciliary factor, or M-factor, which has anticiliar activity – it disrupts the function of ciliar epithelium.

 

           

Schematic representation of proposed CFTR structure. CFTR is made up of five domains: two membrane-spanning domains that form the chloride ion channel, two nucleotide-binding domains that bind and hydrolyze ATP and a regulatory domain.

  

                              

 

                                     Pathological anatomy.

           Pathological changes in the lungs are characterized by symptoms of chronic bronchitis with the development of bronchiectasis and diffuse pneumosclerosis. In the bronchial lumen there is the viscous mucous-purulent content. Often findings are   atelectasis and areas of emphysema. In many patients during the pathological process in the lungs there are complications by joining the bacterial infection (pathogenic Staphylococcus aureus, Haemophilus influenzae and Pseudomonas aeruginosa) and the formation of destruction.

           In the pancreas there are revealed diffuse fibrosis, thickening of the interlobular connective tissue, cystic changes of small and medium-sized ducts. In the liver there are indicated focal or diffuse fatty and protein dystrophy of liver cells, bile stasis in the interlobular bile ducts, interlobular lymphohistiocytic infiltrates in intralobular layers, fibrous transformation and development of cirrhosis.

               At meconium ileus there is expressed atrophy of the mucous layer, mucous glands of the intestinal lumen are enlarged, filled with eosinophylic secretion masses, sometimes there is edema of submucosal layer, the expansion of lymphatic slits. Often cystic fibrosis is combined with various anomalies of gastrointestinal tract.

 

                   There are the following clinical forms of cystic fibrosis:

Ø  mostly pulmonary (respiratory, bronchopulmonary)

Ø  mostly enteric form

Ø  mixed form with simultaneous involvement of the gastrointestinal tract and

                respiratory system

Ø  meconium ileus

Ø  atypical and abortive forms (edematous-anemic, cirrhotic, and others).

 

 

                  Clinical features in 70% of cases of cystic fibrosis is detected during the first 2 years of life.                     

 

The signs and symptoms of CF in children and young adults may include:

§  Salty taste of the skin. People with CF tend to have two to five times the normal amount of salt (sodium chloride) in their sweat. This may be one of the first signs parents notice because they taste the salt when they kiss their child.

§  Blockage in the bowel.

§  Foul-smelling, greasy stools.

§  Delayed growth.

§  Thick sputum. It’s easy for parents to overlook this symptom because infants and young children tend to swallow their sputum rather than cough it up.

§  Coughing or wheezing.

§  Frequent chest and sinus infections with recurring pneumonia or bronchitis.

§  Growths (polyps) in the nasal passages.

§  Cirrhosis of the liver due to inflammation or obstruction of the bile ducts.

§  Displacement of one part of the intestine into another part of the intestine (intussusception) in children older than age 4.

§  Protrusion of part of the rectum through the anus (rectal prolapse). This is often caused by stools that are difficult to pass or by frequent coughing.

§  Enlargement or rounding (clubbing) of the fingertips and toes. Although clubbing eventually occurs in most people with CF, it also occurs in some people born with heart disease and other types of lung problems.

                   

 

                                              Meconium ileus

               At 30-40% patients cystic fibrosis is diagnosed in the early days of life in the form of meconium ileus. This form of the disease is due to a lack of trypsin, which leads to accumulation in the loops of the small intestine (usually in the ileocecal region) of dense, viscous meconium.

                 A healthy newborn first feces departs at first, less often – the second day after birth. In a sick child meconium is absent. By the second day of life the child becomes restless, abdomen is distended, regurgitation and vomiting are marked with an admixture of bile. After 1-2 days the state of newborn becomes worse: skin is dry and pale, expressed vascular pattern appears on the abdomen, tissue turgor is reduced, anxiety is replaced by lethargy and adynamia, signs of intoxication and dehydration occur.

                  An objective examination of patients reveals dyspnea and tachycardia, at the percussion of the abdomen – tympanic sound. Auscultation: peristalsis is not listening. Review radiograph of the abdominal cavity reveals swollen loops of the small intestine and sleeping units in the lower abdomen.

                   Complication meconium ileus may be perforation of the intestine with the development of meconium peritonitis. Frequently, the intestinal obstruction in cystic fibrosis patients on the 3-4-th day of life is associated with pneumonia, which has a protracted nature. Intestinal obstruction may also develop later.

 

                

A. Illustration of intestine blocked by meconium. B. Abdominal xray of a newborn infant with meconium ileus showing dilated loops of bowel.

 

                      

                                    Pulmonary (respiratory) form

 

                          

 

               The first symptoms of broncho-pulmonary forms of cystic fibrosis are fatigue, paleness of the skin, lack of weight gain with satisfactory appetite. In some cases (severe course) from the first days of life the patients have hacking cough, which gradually increases and becomes like pertussis. The cough is accompanied by excretion of the thick phlegm, which with presence of the bacterial flora is subsequently mucopurulent.

              The increased viscosity of bronchial secretion leads to the development mucostasis and occlusions of small bronchi and bronchioles, which contribute to the development of emphysema, while the total occlusion of the bronchi – the formation of atelectases. In very young children lung parenchyma becomes quickly involved in the pathological process, which leads to the development of severe, prolonged pneumonia with a tendency to abscess formation. Lung affection is always bilateral.

 

 

 

                         

                          

 

                       An objective examination indicated moist small-and medium bubbling rale, bandbox percussion sound. Patients may have toxemia, and even clinical shock on the background of diseases that occur with a high body temperature, or in the hot season in a significant loss of sodium and chloride through sweat. Later chronic pneumonia occurs with pneumosclerosis and bronchiectasis, symptoms of “cor pulmonale”, respiratory and cardiac failure.

                      At long course of disease there is involving in the pathological process nasopharynx: sinusitis, adenoidal vegetation, nasal polyps, chronic tonsillitis. Radiological examination of the lungs at cystic fibrosis reveals widespread peribronchial, infiltrative, sclerotic changes and atelectasis on the background of marked emphysema. At bronchography there is the presence of drop-shaped bronchiectasis, bronchial abnormalities and a decrease in the number of small branches, the bronchi 3-6-th calibre are in the form of beads. At bronchoscopy there is often found a small amount of thick viscous sputum, which resides in the form of threads in the lumens of the major bronchi.

                 Microbiological examination of sputum in cystic fibrosis patients can identify Staphylococcus aureus, Haemophilus influenzae and Pseudomonas aeruginosa. The presence of Pseudomonas aeruginosa in sputum is a poor prognostic sign for the patient.

                 There is specific appearance of the patient: pale-gray skin, acrocyanosis, general cyanosis, shortness of breath at rest, barrel chest, sternum deformation of type “wedge” and deformation of terminal phalanges of the type “drumsticks “,limitation of motor activity, decreased appetite and weight loss.

Symptom of “drumsticks ” and “watch glasses” at cystic fibrosis.

CT gram

Ultrasound of the chest. Bronchiectasis in a vacuum section of lung at cystic fibrosis.

               

Frontal chest X ray in CF shows diffuse interstitial disease with bronchiectasis and nodular densities of mucoid impaction.CF = cystic fibrosis.

 

                                 

At bronchography of the left lung in left lateral projections shows the decrease of the lower lobe of the left lung, convergence and expressed deformation of the bronchial tree, cylindrical bronchiectasis, lack of filling in small bronchial branches.

 

                The rare complications of cystic fibrosis are pneumo- and pyopneumothorax, pulmonary hemorrhage. In a more favorable course of cystic fibrosis, at which onset of the disease is observed at older age, bronchopulmonary pathology manifests slowly with progressive deforming bronchitis with moderate pneumosclerosis.

                              Intestinal form

 

                  

 

 

                Clinical symptoms of the intestinal form are caused by secretory insufficiency of the gastrointestinal tract. Violation of the enzymatic activity of the gastrointestinal tract is particularly pronounced after the transfer of the child to bottle-feeding or complementary feeding and manifest lack of splitting and absorption of proteins, fats and carbohydrates. Putrefactive processes is dominated in intestine, accompanied by the accumulation of gases, which leads to bloating. Frequent bowel movements, polifecalia (daily amount of feces 2-8 times exceed the age limit). In elder patiens with cystic fibrosis there is often marked prolapse of the rectum (in 10-20% of patients).

                 Patients complain of dry mouth, due to the high viscosity of saliva. Patients with difficulty chew dry food, and during meals drink a significant amount of fluid. Appetite in the first months is normal or even increased, but due to the violation of the digestive processes in patients hypotrophy and polyhypovitaminosis  rapidly occur. Muscle tone and tissue turgor are reduced.

                 Patients complain of abdominal pain of various kinds: cramping – with meteorism, muscle – after coughing, pain in right hypochondrium – the presence of right heart failure, pain in epigastric region due to the lack of neutralization of gastric juice into the duodenum at a reduced secretion of pancreatic bicarbonates.

                Violation of neutralization of gastric juice may cause the development of duodenal ulcer or ulcerative process in the small intestine. Intestinal complications of the cystic fibrosis may be secondary disaccharidase failure, intestinal obstruction, secondary pyelonephritis and urolithiasis on the background of metabolic disorders, latent diabetes in lesions of insular apparatus of the pancreas. Violation of protein metabolism leads to hypoproteinemia, which becomes the cause of development in some cases, infants edema.

                    Hepatomegaly (liver enlargement) occurs due to cholestasis. When clinical picture of biliary cirrhosis is present there may be observed jaundice, itching, signs of portal hypertension, ascites. Cirrhosis of the liver in some patients may develop without cholestasis.

 

                                         Mixed form

               Mixed form of cystic fibrosis is the most severe and includes clinical symptoms of pulmonary and intestinal forms. Usually the first week of life the patient has severe recurrent bronchitis and pneumonia with protracted, persistent cough, bowel syndrome, eating disorders and expressed dyspeptic syndrome. Clinical features of cystic fibrosis differ by considerable polymorphism, which determines the clinical variants of the disease. Severity of cystic fibrosis depends on the onset of the first symptoms. For the younger child the disease manifestation are severer and  prognosis is more unfavorable. Taking into account the polymorphism of clinical manifestations of cystic fibrosis severity is determined in most cases by the nature and extent of lesions of the broncho-pulmonary system.

 

        There are 4 stages of pathological changes in the bronchopulmonary system in cystic fibrosis:

• 1-stage – the stage of non-permanent functional changes, which is characterized by a dry cough without sputum, low or moderate dyspnea during physical exertion. Duration of this stage may be up to 10 years.

• 2-stage – stage of development of chronic bronchitis, which is characterized by the presence of cough with phlegm excreation, moderate dyspnea (increases with tense), the formation of the deformation of the terminal phalanges. Auscultation reveals moist, “booming” rales on the hard breathing. The duration of this stage may range from 2 to 15 years.

• 3-stage – stage of the progression of bronchopulmonary process with the development of complications. There are forming a zone of diffuse pulmonary fibrosis and focal pneumosclerosis, bronchiectasis, cysts and severe respiratory failure in combination with RV heart failure (cor pulmonale). The duration of the stage is from 3 to 5 years.

• 4-stage is characterized by severe cardio-respiratory insufficiency, which in the months is leading to the death of the patient.

 

                                            Diagnosis

                The diagnosis of cystic fibrosis is determined by the data of clinical and laboratory examination of a patient.

           For the diagnosis of the disease four basic criteria are necessary:

    chronic bronchopulmonary process

    bowel syndrome

    cases of cystic fibrosis in sibs

    positive results of sweat test.

 

                  Sweat for the study is collected after electrophoresis with pilocarpine. The minimum amount of sweat required to obtain reliable results, is 100 mg. The difference between the sodium and chlorine in the sample should not exceed 20 mmol / l, otherwise the study is repeated. At an acceptable methodology the determination of one of the ions is possible. In healthy children the concentration of sodium and chloride in the sweat caot exceed 40 mg / dl. Diagnostic criteria for cystic fibrosis is a reliable content of chloride ions more than 60 mmol / l  and sodium – more than 70 mmol / liter. To confirm the diagnosis there is required three positive sweat test s with chloride more than 60 mmol / liter.

               Important in the diagnosis of cystic fsbrosis has coprological study. In patients with cystic fibrosis in coprogram the most characteristic feature is the high content of neutral fat, but perhaps the presence of muscle fibers, cellulose and starch grains, which allows determine the degree of impairment of the enzymatic activity of the gastrointestinal glands. Under the supervision of data coprological research the dose of pancreatic enzymes is correcting.

                  Approximate methods for the diagnosis of cystic fibrosis are the identification of the proteolytic activity of feces by X-ray test, enzyme activity of the pancreas in the duodenal contents, the concentration of sodium iails and salivary gland secretion. As a screening test in the neonatal period the method of determining the high content of albumin in meconium – meconium test is used (normal albumin content does not exceed 20 mg per 1g of dry weight).

                 A special place in the diagnosis is molecular genetic testing. Today, the presence of known mutations is available identifying in 65-75% of patients with cystic fibrosis, which makes it impossible to use for verification of the diagnosis of the disease only by molecular-genetic examination.

                                          Differential diagnosis

                 The differential diagnosis of cystic fibrosis is conducted with whooping cough, obstructive bronchitis, bronchial asthma, congenital and acquired bronchiectasis, pulmonary fibrosis nonpancreatic origin. In the presence of a high rate of electrolytes in sweat cystic fibrosis is differentiated with such diseases as diabetes insipidus, adrenal insufficiency, hereditary ectodermal dysplasia, glycogenic disease, lack of glucose-1-phosphatase, hypoparathyreoidism, malnutrition, gargoilism, fucozidosis, dehydration, edema.

                                        Treatment

               Treatment of cystic fibrosis is symptomatic. Feeding is very important to the patient. Daily calories must be 10-30% higher than the age limit due to an increase in dietary protein component. Protein requirements are satisfied by the consumption of meat, fish, eggs, cottage cheese. Fat intake is significantly reduced. It is possible to use the fat, composed of fatty acids with an average size of the chain, because their digestion does not dependent on the activity of pancreatic lipase.

                At deficiency of disaccharidases in the small intestine the corresponding sugar (usually lactose) must be excluded from the diet. It is necessary to add salt to food, especially in the hot season and at high temperature, because of the large losses of salt through sweat.

 

                    A sufficient intake of fluids is provided to the patient. In the food should be included products containing vitamins, fruit and vegetable juices, butter.

                 It is obligatory to carry out correction of pancreatic function through the use of pancreatin or combined preparations containing pancreatin, along with other intestinal enzymes and lipotropics (Creon, Polizim, panzinorm, meksaza, etc.). The dose of enzyme preparations is picked individually, focusing on data of coprological study.

                Indicators of the optimal selection of a dose are normalization of stool and the disappearance of neutral fat in feces. The initial dose is 2-3grams a day. Dose is gradually raised until a positive effect. To liquefy the secrets of the gastrointestinal tract and improve their excreation the acetylcysteine is used in tablets and granules, which is indicated at cholestasis, viscous duodenal contents, impossibility of probe.

                                                         Treatment of pulmonary syndrome includes a complex of measures aimed at thinning of the sputum and removing it from the bronchi. For this purpose physical, chemical and instrumental methods are applied.

                 Mucus-thinning therapy is carried out daily throughout the patient’s life. The effectiveness of treatment increases with the simultaneous use of aerosol inhalation, exercise therapy, vibratory massage and postural drainage. The number and duration of inhaled medicines depend on the severity of the patient. As a mucus-thinning drugs may be used saline-alkaline mixture (1-2% solution– saline chloride and sodium carbonate), bronchodilators drugs acetylcysteine (one inhalation of 2-3 ml of 7-10% solution), pulmozim (dornaza alpha). Postural drainage is carried out every morning, vibrating massage – at least 3 times a day.

                   Therapeutic bronchoscopy with bronchial lavage with acetylcysteine and isotonic sodium chloride solution is indicated as emergency procedure in the absence of the effect of the previous therapy. During periods of exacerbation, in the presence of acute pneumonia or acute respiratory viral infection there are indications for  the using of antibacterial therapy.

                  Antibacterial drugs are injected parenterally (semi-synthetic penicillins, cephalosporins of second and third generation, aminoglycosides, Chinolones) and in the form of aerosols (aminoglycosides: gentamicin, tobramicin). As the pneumonia at cystic fibrosis has a prolonged duration, a course of antibiotics is not less than one month and sometimes more.

              In severe pneumonia, corticosteroids are used for 1,5-2 months. Prednisolone is prescribed in dose of 1,0-1,5 mg / kg / day for 10 – 15 days. Then the dose is gradually reduced.

              Antibiotics are used during the course of corticosteroid therapy. In addition to antibacterial and mucus-thinning therapy there are carried out a full range of therapeutic measures aimed to liquidate hypoxia, cardiovascular disorders, acid-base changes.

                    

 

                      The organization of dispensary observation of patients with cystic fibrosis in an outpatient setting it is necessary to monitor a feces and a patient’s body weight, regular (1 every 3 months) to conduct coprological study for correction of  doses of the pancreatic enzymes, in the spring and during exacerbation of the process to assign courses of vitamins (it is justified the appointment of double dose of fat-soluble vitamins A, E, D in water solutions).

                Relatives of the patient should be taught techniques of postural drainage, vibration massage and care for the patient. Along with exercise therapy, physical therapy recommends dosing exercise and sports. At sustained remission for 6 months there is allowed preventive vaccinations.

                       

 

 

                The prognosis for cystic fibrosis remains serious. Mortality is 50-60% among young children. Late diagnosis of disease and inadequate therapy significantly worsen the prognosis. Currently there is available the diagnostic of the disease in early pregnancy, and therefore becomes important medico-genetic counseling of families in which there are patients with cystic fibrosis.

 

                Chronic bronchopulmonary disease

                          in children.

 

                 Chronic bronchopulmonary pathology – is an inflammatory process of infectious origin (caused by the microflora), which is the recurring, usually bronchogenic spread and is based on functional and organic lesions of bronchi, vessels, parenchyma and interstitial tissue of the lungs, pleura and the subsequent development of sclerosis.

                                               Clinic

 

              The clinic is manifested as symptoms of a general nature and directly related with the bronchopulmonary system. It must be remembered that chronic bronchopulmonary pathology in 80% of cases occurs in the first 3 years of life and approximately 50% – in the first year of life, when its diagnosis at the initial stage is difficult. It is necessary to clarify the frequency of respiratory episodes in a child, and their duration. With increasing frequency of these episodes, their duration and the probability of chronization of the process increase.

               Coughing is a reflex act aimed at self-purification of the respiratory tract of mucus, pus, blood, foreign body, and other particles, which are normally not present in the bronchial tree. In chronic respiratory pathology there is occurrence of  the proliferation of the bronchial mucosa in relation to the inflammatory reaction, hypersecretion of glands, a violation of peristalsis, the accumulation of mucus and pus, compression of the bronchi and trachea by enlarged lymph nodes. Cough, mostly wet, accompanied by sputum, in the presence of emphysema is severe, unproductive, in remission – dry.

                Sputum is puromucous or purulent, more in the morning. It is not a three-layer, as in adults, in small quantities (30-50 ml), it is difficult to obtain it, because in most cases children caot expectorate. For bacteriological examination sputum is taken during bronchoscopic lavage or after digital pressure with a spatula on the tongue root, which causes reflex cough with expectoration.

              Cyanosis appears at exacerbation of chronic respiratory pathology or with the development of chronic pulmonary vascular disease, when it is constant and, depending on the activity of the pathological process, only its intensity changes. Often there is observed increase in body temperature, the phenomenon of hyperhidrosis.

              We must always pay attention at the form of a chest. Its deformation is evident in moderate and severe cases. It indicates a significant area of the pathological process in the lungs and long-term hypoxemia. “Chicken”, “pigeon” chest, depressed sternum are signs of chronic bronchopulmonary diseases in preschool age; barrel – in schoolchildren. The asymmetry of the chest, deformation, a marked decrease in the size of a half part in measuring by centimeter tape – all this makes it possible to analyze during the inspection the severity, duration and localization of process.

                        

                     

 

           

 

                Shortness of breath at chronic bronchopulmonary disease is mixed, sometimes expiration. Deformation of the fingers in the form of drum sticks and nails in the form of watch glass reflects the duration of hypoxemia.

 

 

                          

 

                    Percussion reveals bandbox sound (emphysema), at marked sclerotic changes shortening is detected.

                   Auscultation (at exacerbation) reveals diversity of dry and moist rales. Constant local fine bubbling moist rales is the criteria of chronic bronchopulmonary disease.

                   Cardiovascular system: expansion of the right heart, accent of the second tone in the pulmonary artery, weak tones and functional systolic murmur, the ECG changes shows myocardial hypoxia.                            

                Digestive system. Appetite is reduced, the liver is increased in size, its function is disrupted, there are disorders in hydrolysis and absorption of food, in severe cases, a child retards in the mass and growth. The skin is dry, visible pallor, reduced flexibility, opacity and the fragility of the hair.

           Sometimes there is short-term allergic rash, once rising of high temperature (due to a violation of drainage of purulent sputum). Headache, fatigue, irritability, aggression, memory decline indicate CNS violation. Pulmonary hemorrhage is very rare in children.

            X-rays changes are different: increased lung pattern, its deformation, cyst enlightenments, volume reduction of segment, lobe, mediastinal shift toward pathology, disateleсtasis (airless areas alternate with emphysema). Characteristic for this disease is that they are sustainable. They can only increase at exacerbation, but remain in remission.

 

 

 

Left side chronic bronchopulmonary                 Direct radiogram of chest.

disease.                                                                Decreasing of left lung field,

                                                                             shadowing in the middle areas.

 

 

 

Detail of chest radiograph at peribronchial pneumosclerosis in patient with chronic obstructive bronchitis: lung pattern is reinforced and deformed, there are clearly distinguished bronchial lumen, bordered by thickened walls.

 

 

Detail of chest radiographs in a direct projection at chronic bronchopulmonary disease: at low part of the right lung field lung pattern is reinforced and deformed, its radial direction caot be seen.

 

          At bronchographic observation (which is obligate) there are the deformation of the bronchi, cylindrical and saccular bronchiectasis, reducing the angle of the bronchi branching.

 

 

 

                        

 

 

Bronchiectasis. Coloured bronchogram (X-ray) of a human lung showing bronchiectasis.Bronchiectasis is a lung disorder in which the bronchi and bronchioles (airways of the lungs, red) are permanently dilated and distorted. In this case, some of the bronchi have terminal bulbous enlargements, a condition known as fusiform (saccular) bronchiectasis.

 

                     

Normal bronchogram.                                Varicose bronchoectasis.                           

 

 

 

                         

Representative CT scan image from a person with advanced CBPD shows emphysematous changes and subpleural blebs.

 

 

 

                Identification of chronic bronchopulmonary diseases at the level of deforming bronchitis without bronchiectasis may be conventionally considered  because the stabilization of the pathological process in these conditions is more likely. Clinical experience shows that bronchiectasis is less evident than deforming bronchitis.

 

                             

                           Deforming bronchitis.

                Deformations of the bronchi may be different: the broadening or narrowing of the lumen, presence of granulation and follicular polyps, false cysts, bronchiectasis (cylindrical, saccular, mixed), which lead to varying degrees of obstruction of small bronchi. Chronic process in the lung tissue in combination with deforming bronchitis or bronchiectasis becomes stable, leads to irreversible changes in the affected area of lungs.

              It is believed that chronic bronchopulmonary diseases are characterized by the local endobronchitis. However, at the severe forms with involvement in the pathological process of many segments of the left and right lungs endobronchitis becomes diffuse.

 

 

 

Referens:

 

A – Basic:

1.     Pediatrics. Textbook. / O. V. Tiazhka, T. V. Pochinok, A. N. Antoshkina et al. / edited by O. Tiazhka – Vinnytsia : Nova Knyha Publishers, 2011 – 584 pp. : il.

2.     ISBN 978-966-382-355-3Nelson Textbook of Pediatrics, 19th Edition Kliegman, Behrman. Published by Jenson & Stanton, 2011, 2608.  ISBN: 978-080-892-420-3.

3.     Illustrated Textbook of Paediatrics, 4th Edition.  Published by  Lissauer & Clayden, 2012, 552 p. ISBN: 978-072-343-566-2.

4.     Denial Bernstein. Pediatrics for medical Students. – Second edition, 2012. – 650 p.

 

B – Additional: 1.http://intranet.tdmu.edu.ua/data/kafedra/internal/pediatria2/classes_stud/шпитальна%20педіатрія/6%20курс/English/Theme%2001%20Differential%20diagnosis%20of%20pneumonia%20in%20children.htm

2. http://www.merckmanuals.com/professional/index.html

3. Lichtenstein, et al. Pediatric Pneumonia. Emergency medicine clinics of north America.  2010.

4. Barson.  Clinical manifestations and diagnosis of community-aquired pneumonia in children. UpToDate.com., 2009.