Alloys ; Cobalt ; Humans ; Lung ; pathology ; Lung Diseases ; diagnosis ; pathology ; Tungsten

An open lung biopsy was performed in 12 children with diffuse parenchymal lung disease. A definitive histopathological diagnosis was obtained from all procedures but determined treatment options in only 10 children (83%). Three (25%) children were ventilated for respiratory failure prior to the procedure. Four (44%) of the other 9 children required ventilatory support after the procedure. Three (25%) children developed post-op pneumothorax that resolved fully with chest tube drainage. There were no deaths as a direct result of the procedure. Open lung biopsy is useful in providing a definitive diagnosis in children with diffuse parenchymal lung disease and determining treatment in the majority of cases. The procedure was well-tolerated with minimal complications.
*Biopsy ; Lung/*pathology ; Lung Diseases/*pathology ; Scleroderma, Diffuse

Idiopathic interstitial pneumonias are currently classified into four categories: usual interstitial pneumonia, nonspecific interstitial pneumonia with fibrosis, acute interstitial pneumonia and desquamative interstitial pneumonia. The fibrotic process in interstitial pneumonias appears to result from a complex interaction between fibroblasts, other lung parenchymal cells and macrophages. The complex relationship between the local release of growth-promoting cytokines by alveolar macrophages and resident fibroblasts represents a necessary step for fibrosis or remodeling after lung injury. Injury to the epithelium and basement membranes is likely necessary for the fibrotic process to occur. Usual interstitial pneumonia, most frequent among interstitial pneumonias and has a poor prognosis, appears on high-resolution CT as patchy subpleural areas of ground-glass attenuation, irregular linear opacity, and honeycombing. Nonspecific interstitial pneumonia with fibrosis, the second most frequent and has a better prognosis than usual interstitial pneumonia, appears as subpleural patchy areas of ground-glass attenuation with associated areas of irregular linear opacity on CT. Acute interstitial pneumonia with high mortality rate presents as extensive bilateral airspace consolidation and patchy or diffuse bilateral areas of ground-glass attenuation. Desquamative interstitial pneumonia with good prognosis presents as patchy subpleural areas of ground-glass attenuation in middle and lower lung zones.
Human ; Lung Diseases, Interstitial/radiography ; Lung Diseases, Interstitial/physiopathology ; Lung Diseases, Interstitial/pathology*

The patient is a female infant, 4 months and 9 days old, who was admitted to the hospital due to recurrent fever, cough, and hepatomegaly for over a month. The patient was a healthy full-term infant with a normal birth history. At 2 months and 22 days after birth, she developed recurrent fever, cough, and respiratory distress. Chest imaging revealed diffuse bilateral lung lesions, and fiberoptic bronchoscopy showed interstitial changes in both lungs. These suggested the presence of interstitial lung disease. The patient also presented with hepatomegaly, anemia, hyperlipidemia, hypothyroidism, and malnutrition. Genetic testing indicated compound heterozygous variations in the MARS1 gene. This mutation can cause interstitial lung and liver disease, which is a severe rare disorder that typically manifests in infancy or early childhood. It is inherited in an autosomal recessive manner and characterized by early-onset respiratory insufficiency and liver disease in infants or young children. Since its first reported case in 2013, as of June 2023, only 38 related cases have been reported worldwide. This article reports the multidisciplinary diagnosis and treatment of interstitial lung and liver disease in an infant caused by MARS1 gene mutation.
Female ; Humans ; Infant ; Cough ; Hepatomegaly/pathology* ; Liver Diseases ; Lung/pathology* ; Lung Diseases, Interstitial/pathology* ; Mutation

Fungi ; Humans ; Lung Diseases, Fungal ; microbiology ; pathology

Congenital pulmonary lymphangiectasis (CPL) is a rare, poorly documented disease, characterized by abnormal dilatation of pulmonary lymphatics without lymphatic proliferation. This disease is seen almost exclusively in infancy and early childhood. It can usually be divided into primary (congenital) and secondary forms. The primary form presents in neonates, and the patients mostly die due to the respiratory distress, shortly after birth. The authors experienced two cases of primary CPL in a 13-day-old male neonate and a one-day-old male neonate, showing prominent lymphatic dilatation in the septal, subpleural, and peri-bronchial tissue throughout both lungs. The latter case was associated with congenital cardiac anomaly including single ventricle. These are unique cases of CPL in Korea of which the diagnosis was established through post-mortem examination. Therefore, the authors report these two cases with primary CPL with a review of the literature.
Humans ; Infant, Newborn ; Lung/pathology ; Lung Diseases/congenital/*pathology ; Lymphangiectasis/congenital/*pathology ; Lymphatic System/pathology ; Male

Cell aging is an extremely complex process, which is characterized by mitochondrial structural dysfunction, telomere shortening, inflammatory microenvironment, protein homeostasis imbalance, epigenetic changes, abnormal DNA damage and repair, etc. Aging is usually accompanied by structural and functional damage of tissues and organs which further induces the occurrence and development of aging-related diseases. Aging includes physiological aging caused by increased age and pathological aging induced by a variety of factors. Noteworthy, as a target organ directly contacting with the outside air, lung is more prone to various stimuli, causing pathological premature aging which is lung aging. Studies have found that there is a certain proportion of senescent cells in the lungs of most chronic respiratory diseases. However, the underlying mechanism by which these senescent cells induce lung senescence and their role in chronic respiratory diseases is still obscure. This paper focuses on the causes and classification of lung aging, the internal mechanism of lung aging involved in chronic respiratory diseases, and the application of anti-aging treatments in chronic respiratory diseases. We hope to provide new research ideas and theoretical basis for the clinical prevention and treatment in chronic respiratory diseases.
Aging/pathology* ; Cellular Senescence ; Humans ; Lung/pathology* ; Lung Diseases/pathology* ; Respiration Disorders/pathology* ; Telomere ; Telomere Shortening

Adult ; Calcinosis ; pathology ; Female ; Humans ; Lung Diseases ; pathology

Diagnosis, Differential ; Humans ; Lung Diseases, Interstitial ; diagnosis ; pathology ; Pathology, Clinical

We report a case of huge pulmonary chondroid hamartoma with multilocular cysts in a 38-yr-old male patient. The lobectomy specimen of the left lower lobe showed a large multilocular cystic mass, 11.5 x 10 cm in size. The mass had thin-walled, variable-sized cysts and areas of solid cartilaginous small nodules in the interstitium, which occupied the superior segment and the upper portion of the basal segment. There was no connection with bronchus or vessel. Microscopically, the cysts and cleft-like spaces were lined by ciliated columnar epithelium and the solid components were composed of cartilage, myxohyalinized connective tissue, and adipose tissue. Also seen were foci of calcification within the sclerotic stroma.
Adult ; Cysts/*pathology ; Hamartoma/*pathology ; Humans ; Lung/radiography ; Lung Diseases/*pathology ; Male ; Tomography, X-Ray Computed