Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0010200 (cough)
 23,843 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Lymphangioleiomyomatosis (LAM) is a rare disease of unknown etiology affecting woman, primarily in their reproductive years. It is characterized by non-neoplasic proliferation of atypical smooth muscle cells that may lead to airway, lymphatic and blood vessel obstruction over time.A nine-year followed-up case of LAM is described,which presents with some unusual details as worsening haemoptysis in the setting of pregnancy and absence of cough or pneumothorax along the course of the disease. Neither haemoptysis nor gradual pulmonary function deterioration seemed to be influenced by hormone therapy. A brief re-view of the disease is then presented.
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PMID:[Lymphangioleiomyomatosis: a case report]. 1651 17

A 44-year-old female patient diagnosed as pulmonary lymphangioleiomyomatosis with respiratory failure underwent bilateral sequential lung transplantation using an entire left lung and a post-lower-lobectomy right lung due to pneumonia. Persistent air leakage during cough was noted and bronchoscopy 12 days after transplantation demonstrated post-lobectomy bronchial stump dehiscence with a large bronchopleural fistula. We applied twice tissue glue instillation on the dehiscent bronchial stump through flexible bronchoscopy. The air leak improved and the patient was discharged 3 months after transplantation uneventfully. Two months after discharge, bronchoscopy showed complete healing of the dehiscent bronchial stump. Now, she lives independently without oxygen support.
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PMID:Bronchoscopic gluing for post-lung-transplant bronchopleural fistula. 1714 17

A 37-year-old woman presented with a cough and discomfort in the chest. Computed tomography revealed the right pleural effusion and a number of cysts in the lungs. Thoracentesis revealed LAM cell clusters (LCC) in chylous pleural effusion, confirmed by immunocytochemical examinations showing that the cells at the center of cluster were LAM cells positive for alpha-smooth muscle actin and HMB45 and the outer layer was lymphatic endothelium cells. When LCC were cultured in vitro, the loss of heterozygosity of TSC2 markers was detected. This case illustrates that LAM can be diagnosed by the identification of LCC without an invasive biopsy if complicated with chylous effusion.
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PMID:Lymphangioleiomyomatosis diagnosed by immunocytochemical and genetic analysis of lymphangioleiomyomatosis cell clusters found in chylous pleural effusion. 1787 49

Lymphangiomyomatosis (LAM) is a rare lung disease that is characterized by the progressive proliferation of atypical smooth muscle-like cells, which leads to severe respiratory impairment and death. Dyspnea, cough, recurrent pneumothorax, and hemoptysis are the most common clinical symptoms of LAM. We report a 29-year-old female patient with massive hemoptysis during laparoscopic gynecologic surgery under general anesthesia, who was diagnosed with pulmonary LAM.
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PMID:Lymphangiomyomatosis discovered by massive hemoptysis during general anesthesia -A case report-. 2255 5

Lymphangioleiomyomatosis (LAM) is a rare disease characterized by diffuse thin-walled cysts throughout the lungs on computed tomography and diffuse proliferation of abnormal smooth muscle-like cells (LAM cells) on lung biopsy. LAM affects women almost exclusively, predominantly in their reproductive age. The most typical presenting symptoms include dyspnea, spontaneous pneumothorax, cough and chylothorax. Abdominal findings represent less common initial manifestations of the disease and may pose diagnostic difficulties. The treatment of LAM has not been fully established. Recent studies report effectiveness of sirolimus in LAM patients. We report the case of a 45-year-old woman with sporadic LAM, successfully treated with sirolimus, in whom the first manifestation of the disease was chyloperitoneum and after three and nine years, respectively, lymphedema of the left lower extremity and right sided chylothorax occurred.
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PMID:Chyloperitoneum, chylothorax and lower extremity lymphedema in woman with sporadic lymphangioleiomyomatosis successfully treated with sirolimus: a case report. 2305 49

Lymphangioleiomyomatosis is an uncommon lung disease primarily affecting women of childbearing age. It is characterized by the progressive proliferation and infiltration of smooth muscle-like cells, which lead to cystic destruction of the lung parenchyma; obstruction of airways, blood vessels, and lymphatics; and loss of pulmonary function. We present the case of a 46-year-old female patient with chest pain, cough, sputum, and dyspnea on exertion for three weeks. Minimal pneumothorax was noted, and the patient was referred to our center for further investigation and treatment. High-resolution computed tomography revealed numerous bilateral thin-walled air cysts and interstitial thickening affecting the central and peripheral part of the upper zone of the lung. We performed an open-lung biopsy to confirm lymphangioleiomyomatosis. Our aim is to discuss the pathogenesis and other lesions noted in the differential diagnosis of this rare disease.
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PMID:Pulmonary lymphangioleiomyomatosis: a rare case. 2410 55

Lymphangioleiomyomatosis (LAM) is a rare, progressive systemic disease of unknown etiology, insidious onset, often fatal, and underdiagnosed. It is exclusively found among women. LAM mainly involves the lungs where, as its name suggests, lymphatic (lymph), blood vessel (angio), and airways are surrounded by smooth muscle (leiomyoma) proliferation. It may be associated with tuberous sclerosis with clinical manifestations varying from simple cough to the development of recurrent pneumothoraces, haemoptysis, and pleural effusions. There is currently no treatment or cure. We present a rare case of a 41-year-old female, who presented with recurrent pneumothoraces, whose high-resolution computed tomography findings that were suggestive of multiple bullous lung disease, and the thoracoscopic biopsy revealed features of LAM. She was offered video-assisted thoracoscopic surgery in form of bullectomy and pleurodesis to reduce the volume of abnormal tissue and treated with progesterone hormonal therapy. The present case aims to focus on clinical-radiologic and pathologic findings and the treatment modalities available in developing countries like India where lung transplantation is seldom performed.
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PMID:Palliative management of lymphangioleiomyomatosis: Using video-assisted thoracoscopic surgery. 2441 88

Disorders of the pulmonary lymphatic system include macro- and microcystic lymphatic malformations, primary or secondary lymphangiectasias, generalized lymphatic anomalies, diffuse pulmonary lymphangiomatosis, and combinations of lymphatic and other tissue anomalies, including lymphangioleiomyomatosis (LAM). We report a case of a patient with a newly defined entity classified as kaposiform lymphangiomatosis (KLA). This 50-year-old nonsmoking Hispanic woman presented with a 20-year history of cough, hemoptysis, chyloptysis, and pleuritic chest pain. Laboratory evaluation demonstrated a low normal platelet count, elevated d-Dimer, low normal fibrinogen, and elevated fibrin split products. Chest computerized tomography imaging showed enlarged hypodense lymph nodes in the mediastinum and hila, and peribronchovascular thickening, without evidence of cystic parenchymal lesions. Magnetic resonance imaging of the chest showed cystic mediastinal lymph nodes with heterogeneously increased T2 and decreased T1 signal intensity. Fiberoptic bronchoscopy revealed hyperemic mucosa with granular appearance suggestive of a submucosal infiltrative process. Pathological specimens revealed dilated, malformed lymphatic channels within the pleura, pulmonary septa, and bronchovascular bundles, and foci of perilymphatic and intralymphatic spindle cells which reacted with the Prospero homeobox protein 1 (PROX-1) immunostain. The morphology and immunohistochemistry results were consistent with a diagnosis of KLA. This newly recognized clinical-pathological entity among intrathoracic lymphatic anomalies is distinguished from generalized lymphatic anomaly and diffuse pulmonary lymphangiomatosis in part by characteristic hematological abnormalities and hemorrhagic complications, including hemoptysis, as experienced by our patient.
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PMID:Kaposiform lymphangiomatosis, a newly characterized vascular anomaly presenting with hemoptysis in an adult woman. 2446 Apr 39

We experienced a case of scheduled cesarean section under spinal anesthesia in a patient with LAM which had been missed in spite of preoperative medical examination and consultation with specialists but discovered because of perioperative hypoxia A 35-year-old woman, Gravida 1 Para 0, with breech presentation was scheduled to undergo cesarean section under spinal anesthesia at 38 weeks of gestation. She had no history of asthma or abnormal findings at annual medical examination. She had suffered from dry cough and nocturnal dyspnea for 7 weeks and an inhaled bronchodilator was administered with diagnosis of inflammatory airway disease by her respiratory physicians. Spinal anesthesia was performed with bupivacaine 12.5 mg. At the beginning of anesthesia SPO2 was 97% in supine position, but it rapidly decreased to less than 90% and 3 l x min(-1) oxygen was supplied with a facial mask. The anesthetic level was thoracal 4 bilaterally and her breathing was stable. The circulatory state, Apgar score and other vital signs were within normal ranges. Postoperative chest X-ray showed bilateral numerous grained spots and computed tomography scans showed multiple thin-walled cysts. The characteristic history and the fluoroscopic data gave her clinical diagnosis of LAM.
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PMID:[Case of lymphangioleiomyomatosis (LAM) discovered during cesarean section under spinal anesthesia]. 2460 Nov 17

The rare, severe lung disease lymphangioleiomyomatosis (LAM) usually presents with dyspnoea on exertion, cough and expectoration in females in the third decade and is worsened by pregnancy or hormone therapy. Lung transplant is often needed within ten years since no specific treatment is available. The patient in this case report was a mother of two, who had never received hormone therapy. Once, when she was 47-years-old, she had had a period of dyspnoea and pleural empyema and no diagnosis was made, but being 75-years-old she was admitted with moderate dyspnoea on exertion, and high resolution CT showed cysts of the lungs classical for LAM.
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PMID:[Unusually late debut of lymphangioleiomyomatosis]. 2561 86


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