Pediatric desquamative pneumonia
Introduction
Introduction to pediatric desquamate pneumonia Desquamative interstitial lpneumonia (DIP or D. histocytic I.P), also known as desquamative interstitial pneumonia, idiopathic pulmonary interstitial fibrosis, idiopathic interstitial pneumonia. Children are rarer than adults and can be found in infants and children of any age. The smallest case in the literature is one day after birth, and both men and women can develop the disease. basic knowledge The proportion of illness: male children are more common, the incidence rate is about 0.03% - 0.08% Susceptible people: children Mode of infection: non-infectious Complications: pulmonary hypertension chronic pulmonary heart disease
Cause
Causes of pediatric desquametic pneumonia
(1) Causes of the disease
Unexplained cause, foreign body reaction or autoimmune phenomenon or sequelae of infection, it is not clear, because rheumatoid factor, anti-nuclear antibody and lupus cells have been found, it was once considered a connective tissue disease, immune complex The presence and deposition of IgG and complement in the alveoli suggest that the disease is an immune disease, and some people believe that it is associated with alveolar proteinosis. It has also been reported that it is congenital after being infected with respiratory viruses such as adenovirus and mycoplasma infection. Reports of rubella have been reported in children with inflammatory bowel disease treated with Sulfesalazine, sometimes without any obvious cause.
(two) pathogenesis
The pathology of this disease is mainly characterized by the accumulation of a large number of rounded, oval or irregularly shaped type II epithelial cells in the alveolar cavity. The cytoplasm is obviously acidic stained in hematoxylin and eosin stained sections, and many epithelial cells. There are vacuoles of varying sizes in the cytoplasm, thickened alveolar walls, telangiectasia, congestion and edema, and infiltration of monocytes, lymphocytes and eosinophils.
Prevention
Pediatric desquamative pneumonia prevention
At present, there is no effective prevention method, and timely detection of timely treatment is better.
Complication
Pediatric desquamative pneumonia complications Complications pulmonary hypertension chronic pulmonary heart disease
Formation of complete fibrosis and vesicular cell lungs in the alveolar cavity, most patients with pulmonary fibrosis leading to pulmonary hypertension, pulmonary heart disease and right heart failure.
Symptom
Symptoms of pediatric desquametic pneumonia Common symptoms PAS staining positive hairpin dry cough heart rate increased vocal loss of appetite loss pulmonary fibrosis clubbing (toe) dyspnea pleural effusion
It can be divided into primary and secondary. The primary disease is more acute. After secondary to other diseases, the symptoms are similar to diffuse pulmonary fibrosis. The disease is more insidious, but it can also suddenly start. Mainly manifested as rapid breathing, progressive dyspnea, heart rate increase, cyanosis, dry cough, weight loss, weakness and loss of appetite, fever no more than 38 ° C, severe respiratory failure and heart failure, can suddenly die after eating Sometimes, you can see the clubbing, toe, lung signs are not obvious, sometimes the lower lung can hear the wet and wet voice, X-ray shows two lungs glass or mesh, flaky shadow, can be unclear The blurred triangle shadow, from the lung door along the heart to the lung base and the periphery of the release, sometimes visible emphysema bubble, pneumothorax and pleural effusion and other complications, long-term pulmonary disease, peripheral blood eosinophils can be seen increased.
Examine
Examination of children with desquamative pneumonia
There are no special findings in general laboratory tests.
1. Blood routine examination: white blood cells generally do not exceed 15000/mm3, and eosinophils may increase.
2. Blood biochemical examination: There is no change in serum protein, rheumatoid factor, anti-nuclear factor or lupus cells can be positive, and immunoglobulin levels are abnormal.
3. Blood gas analysis: There is hypoxemia.
4. Lung biopsy: desquamative interstitial pneumonia, a variant of idiopathic pulmonary fibrosis with similar symptoms, but the changes in lung tissue under the microscope are significantly different, with extensive scar formation.
Auxiliary inspection
1. Imaging characteristics
(1) Chest X-ray: 20% of patients have normal chest radiographs, 1/4 of patients have flaky blurred shadows or diffuse shadows, diffuse ground-glass changes in the middle and lower lung fields, and linear or reticular formation in the later stage. , nodular interstitial images.
(2) CT: A high-resolution CT scan of about 1/4 of the patients showed a diffuse ground-glass-like change in the middle and lower lung fields, and a linear, reticular, nodular interstitial image appeared later.
2. Pulmonary function test is a restrictive ventilatory disorder accompanied by decreased diffuse function and hypoxemia.
Diagnosis
Diagnostic identification of children with desquamative pneumonia
The diagnosis can be confirmed by bronchoscopy or thoracoscopic or thoracotomy for lung biopsy. The pathological diagnostic criteria proposed by Ashen et al. (1984) are as follows:
1. Macrophages containing PAS staining positive particles were abundantly aggregated in the alveoli.
2. Swelling and hyperplasia of type II epithelial cells in the alveoli.
3. There are lymphocytes, plasma cells and eosinophils in the interstitial, and there is mild interstitial fibrosis.
Differential diagnosis
1. Identification with other interstitial pneumonia: Clinical manifestations and other types of interstitial pneumonia are not easy to distinguish, the diagnosis depends mainly on lung biopsy.
2. Foreign body in the respiratory tract: Children with desquamative interstitial pneumonia with dry cough and progressive dyspnea as the main symptoms. During the physical examination, the lungs can smell dry sputum and blisters or respiratory sounds, which is easy to be confused with foreign bodies in the respiratory tract. point:
(1) The respiratory tract has a clear history of foreign body inhalation, while desquamative interstitial pneumonia can be induced by the cold and progresses rapidly.
(2) Chest X-ray: foreign body in the respiratory tract shows affected lung lobe, atrophy of lung segment, deformation and density change, mediastinal shift, mediastinal movement; and desquamative interstitial pneumonia manifests as interstitial changes in both lungs Diffuse, rapid onset of progressive, invasive lesions with macular changes or reticular shadows, antibiotic treatment is ineffective, difficult to use infection, allergy or connective tissue disease interpretation.
(3) Alveolar fluid tissue biopsy: characteristic pathological manifestations of pediatric desquamative interstitial pneumonia.
