Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019621 (Langerhans cell histiocytosis)
 3,250 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The endoscopic examination of the tracheobronchial tree is most helpful in the diagnosis and staging of bronchial carcinoma. Tumors that are endoscopically visible may be confirmed in more than 95% of the cases. In localized peripheral tumors, the diagnostic yield of bronchoscopy is significantly lower; for peripheral metastases, only about 10%. In diffuse interstitial pulmonary diseases other than malignancies, some infections, and histiocytosis X, bronchoscopy including transbronchial biopsy is less successful.
Cardiovasc Intervent Radiol
PMID:The diagnostic yield of bronchoscopy. 204 23

From July 1990 to April 1993, 36 lung transplantations in 33 patients were performed in our pediatric transplant program (0.25 to 23 years, mean age 10.3 years). Eight children had been continuously supported with a ventilator for 3 days to 4.5 years before transplantation and three were supported by extracorporeal membrane oxygenation. Indications for lung transplantation in this pediatric population included the following: cystic fibrosis (n = 13), pulmonary hypertension, and associated congenital heart disease (n = 10), pulmonary atresia, ventricular septal defect and nonconfluent pulmonary arteries (n = 3), pulmonary fibrosis (n = 6), and acute respiratory distress syndrome (n = 1). Three children underwent retransplantation for acute graft failure (n = 2) or chronic rejection (n = 1). Pulmonary fibrosis was related to complications of treatment of acute of myelogenous leukemia with bone marrow transplantation in two children and to bronchiolitis obliterans, bronchopulmonary dysplasia, interstitial pneumonitis, and Langerhans cell histiocytosis in four others. Thirteen children underwent lung transplantation and concomitant cardiac repair. Bilateral lung transplantation, ventricular septal defect closure and pulmonary homograft reconstruction of the right ventricular outflow tract to the transplanted lungs was performed in three children by means of a new technique that avoids the need for combined heart-lung transplantation. Two patients had ventricular septal defect closure and single lung transplant for Eisenmenger's syndrome, two had ligation of a patent ductus arteriosus and transplantation, three additional children underwent atrial septal defect closure and lung transplantation, and two underwent lung transplantation for congenital pulmonary vein stenosis. Eight early deaths and three late deaths occurred (actuarial 1-year survival 62%). Lung transplantation in children has been associated with acceptable early results, although modification of the adult implantation technique has been necessary. Lung transplantation and repair of complex congenital heart defects is possible; heart-lung transplantation may only be required for patients with severe left heart dysfunction and associated pulmonary vascular disease. Bronchiolitis obliterans remains a major concern for long-term graft function in pediatric lung transplant recipients.
J Thorac Cardiovasc Surg 1994 Apr
PMID:Pediatric lung transplantation. Indications, techniques, and early results. 815 51

Pulmonary Langerhans' cell histiocytosis is a relatively unusual, interstitial lung disease. Several organ systems may be involved, including the lung, bone, liver, lymph nodes and brain. It is known to occur preferentially in heavy smokers, and the cases such as ours presenting pneumothorax as the major clinical manifestation are rare.
Interact Cardiovasc Thorac Surg 2006 Aug
PMID:Pulmonary Langerhans' cell histiocytosis presented with recurrent pneumothorax. 1767 Jun 33

Langerhans cell histiocytosis (LCH) is a disease caused by the proliferation of Langerhans cells in various tissues or organs. A 43-year-old male patient presented with an anterior mediastinal mass in the thymus. Histological examination after a thymectomy revealed a bronchogenic cyst in the thymus, and multiple LCH and small thymic cysts were also incidentally observed in the thymus. Unifocal LCH in an adult occurring in the thymus is extremely rare. Furthermore, no cases of LCH with the coexistence of bronchogenic and thymic cysts in the thymus have been previously reported.
Gen Thorac Cardiovasc Surg 2008 Feb
PMID:Langerhans cell histiocytosis: coexistence of bronchogenic and thymic cysts in the thymus. 1829 62

Juvenile xanthogranuloma is the most frequent type of non-Langerhans cell histiocytosis. It most commonly presents in infancy and early childhood; manifesting as cutaneous lesions on the head, neck, and trunk that suddenly appear and usually undergo spontaneous regression. Extracutaneous involvement, although rare, may occur along with the cutaneous form or in isolation. It most frequently involves the eye, deep subcutaneous tissues, lung, and liver. Involvement of the heart is exceptionally rare, with only seven reports found in the English literature, all affecting infants. We present the first report of an intracardiac juvenile xanthogranuloma in an adult.
Cardiovasc Pathol
PMID:Intracardiac juvenile xanthogranuloma with presentation in adulthood. 2401 16

Erdheim-Chester disease (ECD) is a form of non-Langerhans cell histiocytosis that most commonly involves the skeletal system. We report an unusual case of ECD presenting as an anterior mediastinal tumor without skeletal involvement. A 60-year-old man with no remarkable medical history was referred for evaluation of a mediastinal mass. The patient underwent surgical excision of the tumor via video-assisted thoracoscopic surgery. Histologic examination revealed marked proliferation of atypical histiocytes with sclerosis, and the results of immunohistochemical staining were suggestive of ECD.
Korean J Thorac Cardiovasc Surg 2018 Jun
PMID:Erdheim-Chester Disease Presenting as an Anterior Mediastinal Tumor without Skeletal Involvement. 2985 71