Pulmonary capillary haemangiomatosis PCH is a rare vascular proliferative condition that can lead to pulmonary hypertension. It is characterized by multiple angiomatous lesions composed of proliferating capillary vessels in the lung parenchyma and usually progresses to fatal pulmonary hypertension 1. At the time of initial writing, the etiology and inheritance pattern was not well known Pathologically, PCH is characterized by proliferation of benign thin-walled capillary-sized blood vessels within the lung parenchyma 5. Proliferating capillaries invade the pulmonary interstitium and alveolar septae and occlude the pulmonary vasculature.
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Summary and related texts. Related genes. Clinical signs. Check this box if you wish to receive a copy of your message. Detailed information Article for general public Svenska Professionals Review article English Additional information Further information on this disease Classification s 2 Gene s 1 Other website s 0. Health care resources for this disease Expert centres Diagnostic tests 2 Patient organisations 38 Orphan designation s and orphan drug s 1. Specialised Social Services Eurordis directory.
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Pulmonary capillary hemangiomatosis: an uncommon cause of pulmonary hypertension
Ma L, Bao R. Appl Clin Genet. Congenital pulmonary capillary hemangiomatosis: report of two cases and review of the literature. Pediatr Pulmonol. Pulmonary capillary hemangiomatosis associated with primary pulmonary hypertension.
Pulmonary capillary hemangiomatosis
We read with interest the recent publications in the Brazilian Journal of Pulmonology highlighting the prevalence of pulmonary hypertension PH in Brazil, particularly as a complication of sickle cell anemia 1 and schistosomiasis, 2 and discussing the role of imaging methods in the evaluation of this disease. A year-old male nonsmoker presented to the pulmonology department with a three-month history of rapidly progressive dyspnea. Physical examination revealed digital clubbing, and he was tachypneic and cyanotic, requiring supplementary oxygen. Heart auscultation and chest examination were unremarkable, and examination of the abdomen revealed a slightly enlarged liver. A transesophageal echocardiogram showed enlargement of the main pulmonary artery and right heart chamber. Arterial blood gas analysis showed the following results: pH, 7.
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Only comments seeking to improve the quality and accuracy of information on the Orphanet website are accepted. For all other comments, please send your remarks via contact us. Only comments written in English can be processed. Detailed information. Other search option s Alphabetical list. Suggest an update. Summary and related texts.
Pulmonary capillary hemangiomatosis PCH is a disease affecting the blood vessels of the lungs, where abnormal capillary proliferation and venous fibrous intimal thickening result in progressive increase in vascular resistance. Indeed, there is some evidence to suggest that PCH and pulmonary veno-occlusive disease are different forms of a similar disease process. These are non specific. Typical symptoms include dyspnea , cough , chest pain and fatigue. At least some cases appear to be due to mutations in the eukaryotic translation initiation factor 2-alpha kinase 4 EIF2AK4 gene. Lung biopsy is essential to make this diagnosis. This can be difficult if the pulmonary pressure is high.