pulmonary veno-occlusive disease

WordNet

1. an impairment of health or a condition of abnormal functioning
2. caused by or altered by or manifesting disease or pathology; "diseased tonsils"; "a morbid growth"; "pathologic tissue"; "pathological bodily processes" (同)morbid, pathologic, pathological
3. tending to occlude

PrepTutorEJDIC

1. (体の)『病気』,疾患 / (精神・道徳などの)病気,病弊
2. 女性の話術芸人 =diseur
3. 病気にかかった / 病的な,不健全な(morbid)
4. 肺の;肺を冒す

Wikipedia preview

出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2016/04/25 18:30:48」(JST)

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Pulmonary veno-occlusive disease
Pulmonary veno-occlusive disease-Intimal fibrosis with marked narrowing of lumen of a large pulmonary vein
Classification and external resources
ICD-10	I27.2
OMIM	265450
MeSH	C14.907.690

Pulmonary veno-occlusive disease (PVOD) is a rare form of pulmonary hypertension caused by progressive blockage of the small veins in the lungs. The blockage leads to high blood pressures in the arteries of the lungs, which, in turn, leads to heart failure. The disease is progressive and fatal, with median survival of about 2 years from the time of diagnosis to death. The definitive therapy is lung transplantation.

Signs and symptoms

The symptoms for pulmonary veno-occlusive disease are the following:

• Shortness of breath
• Fatigue
• Fainting
• Hemoptysis
• Difficulty breathing ( lying flat)
• Chest pain
• Cyanosis
• Hepatosplenic congestion

Cause

The genetic cause of pulmonary veno-occlusive disease is mutations in EIF2AK4 gene. Though this does not mean other possible causes do not exist, such as viral infection and risk of toxic chemicals (chemotherapy drugs).

Pathophysiology

Pulmonary veno-occlusive disease may have a genetic basis, published reports have indicated fatal occurrences that appeared to possess a familial pattern, more to the point, a germline mutation. The pathophysiology of veno-occlusive disease culminates in occlusion of the pulmonary blood vessels. This could be due to edematous tissue (sclerotic fibrous tissue). Thickening is identified in lobular septal veins, also dilatation of lymphatics happens. Furthermore, alveolar capillaries become dilated (due to back-pressure).

Diagnosis

Pulmonary veno-occlusive disease can only be well diagnosed with a lung biopsy. CT scans may show characteristic findings such as ground-glass opacities in centrilobular distribution, and mediastinal lymphadenopathy, but these findings are non-specific and may been seen in other conditions. However, pulmonary hypertension (revealed via physical examination), in the presence of pleural effusion (done via CT scan) usually indicates a diagnosis of pulmonary veno-occlusive disease. The prognosis indicates usually a 2 year (24 month) life expectancy after diagnosis.

Treatment

Lung transplant rejection - intermed mag

Treatments for primary pulmonary hypertension such as prostacyclins and endothelin receptor antagonists can be fatal in people with PVOD due to the development of severe pulmonary edema, and worsening symptoms after initiation of these medications may be a clue to the diagnosis of pulmonary veno occlusive disease.

The definitive therapy is lung transplantation, though transplant rejection is always a possibility, in this measures must be taken in terms of appropriate treatment and medication.

Epidemiology

Pulmonary venoocclusive disease is rare, difficult to diagnose, and probably frequently misdiagnosed as idiopathic pulmonary arterial hypertension. Prevalence in parts of Europe is estimated to be 0.1-0.2 cases per million.

PVOD appears to occur as frequently in men as in women, and age at diagnosis ranges from 7-74 years with a median of 39 years. PVOD may occur in patients with associated diseases such as HIV, bone marrow transplantation, and connective tissue diseases. PVOD has also been associated with several chemotherapy regimens such as bleomycin, BCNU, and mitomycin.

References

Further reading

• Lung Diseases—Advances in Research and Treatment: 2013 Edition. ScholarlyEditions. 2013-06-21. ISBN 9781481677332. 
• Mandel, Jess; Mark, Eugene J.; Hales, Charles A. (2000-11-01). "Pulmonary Veno-occlusive Disease". American Journal of Respiratory and Critical Care Medicine 162 (5): 1964–1973. doi:10.1164/ajrccm.162.5.9912045. ISSN 1073-449X. 

UpToDate Contents

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• 1. Epidemiology, pathogenesis, clinical evaluation, and diagnosis of pulmonary veno-occlusive disease in adults
• 2. Treatment and prognosis of pulmonary veno-occlusive disease in adults
• 3. 同種造血細胞移植後の肺合併症 pulmonary complications after allogeneic hematopoietic cell transplantation
• 4. 全身性硬化症（強皮症）における肺合併症の概要 overview of pulmonary complications of systemic sclerosis scleroderma
• 5. 成人における肺高血圧症の概要 overview of pulmonary hypertension in adults

English Journal

• HbA(1c) in pulmonary arterial hypertension: A marker of prognostic relevance?

• Belly MJ, Tiede H, Morty RE, Schulz R, Voswinckel R, Tanislav C, Olschewski H, Ghofrani HA, Seeger W, Reichenberger F.SourceDepartment of Internal Medicine, University of Giessen Lung Center, Giessen, Germany.
• The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation.J Heart Lung Transplant.2012 Oct;31(10):1109-14. doi: 10.1016/j.healun.2012.08.014.
• BACKGROUND: Patients with pulmonary arterial hypertension (PAH) exhibit impaired glucose metabolism and increased insulin resistance. The clinical consequences of these metabolic changes are not known.METHODS: We assessed HbA(1c) levels in 115 patients newly diagnosed with PAH (79 females and 36 mal
• PMID 22975101

Japanese Journal

• Beneficial Effects of Imatinib in a Patient with Suspected Pulmonary Veno-Occlusive Disease

• Sato Haruka,Tatebe Shunsuke,Satoh Kimio,Shimokawa Hiroaki,Sugimura Koichiro,Miura Masanobu,Konno Ryo,Kozu Katsuya,Yaoita Nobuhiro,Shimizu Toru,Yamamoto Saori,Aoki Tatsuo
• The Tohoku Journal of Experimental Medicine 247(2), 69-73, 2019
• … <p>Pulmonary veno-occlusive disease (PVOD) is a rare form of pulmonary hypertension (PH). … Imatinib is a tyrosine kinase inhibitor specific for platelet-derived growth factor receptor and is expected as a treatment option for pulmonary arterial hypertension (PAH). … Because the patient had no left heart disease, we diagnosed that she had PAH associated with severe right heart failure. …
• NAID 130007585081

• Pulmonary Veno-occlusive Disease: A Surgical Lung Biopsy-proven and Autopsied Case Radiologically Mimicking Hypersensitivity Pneumonitis at the Time of a Transbronchial Lung Biopsy

• Minomo Shojiro,Kitaichi Masanori,Arai Toru,Matsui Hideo,Akira Masanori,Inoue Yoshikazu
• Internal Medicine, 2019
• … <p>Pulmonary veno-occlusive disease (PVOD) is a rare disease in the subgroup of conditions known as pulmonary arterial hypertension. … Although a histological examination is needed for a definitive diagnosis, a non-invasive diagnosis is required for patients with pulmonary hypertension because a lung biopsy is deemed risky. …
• NAID 130007525249

• 肺静脈閉塞性疾患/肺毛細血管腫症の診断・治療はここまで進歩した (特集 肺高血圧症 : 診断と治療の新展開)

• 小川 愛子
• 最新醫學 = The medical frontline 72(8), 1141-1145, 2017-08
• NAID 40021294169

Related Pictures

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★リンクテーブル★

リンク元	「肺静脈閉塞症」「肺静脈閉塞性疾患」
関連記事	「disease」「occlusive」「pulmonary」

「肺静脈閉塞症」

　　[★]

英

pulmonary veno-occlusive disease

関

肺静脈閉塞性疾患

「肺静脈閉塞性疾患」

　　[★]

英

pulmonary veno-occlusive disease

関

肺静脈閉塞症

「disease」

　　[★]

• n.

疾患：illnessより厳密な概念。「ある臓器に明確な障害が確認され、それによって症状が出ているとはっきり説明できる場合」 (PSY.9)

特定の原因、病態生理、症状、経過、予後、病理組織所見が全てそろった場合 (PSY.9)

• something that is very wrong with people's attitudes, way of life or with society.

関

ail、ailment、disease entity、disorder、ill、illness、malady、sick、sickness

• 注意

disease ≠ illness ≠ disorder

　 　 　

「occlusive」

　　[★]

• 閉塞性の

関

constrictive、obliterans、obliterative、obstructive、occlusively

　

「pulmonary」

　　[★]

• adj.

• 肺の、肺性の

関

lung、pneumo