Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0019079 (hemoptysis)
 6,129 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Primary pulmonary hypertension, as defined by the World Health Organization, specifically includes three entities: primary plexogenic arteriopathy, pulmonary veno-occlusive disease, and silent recurrent thromboembolism. Pulmonary hemorrhage may occur in each of these syndromes but is not usually a prominent symptom. The hemorrhage is probably the result of a variety of causes and related to the specific morphologic characteristics of the pulmonary vasculature in these diseases. In primary plexogenic arteriopathy, hemoptysis and pulmonary hemosiderosis occur mainly in advanced disease with dilatation lesions and plexiform lesions. In veno-occlusive disease, pulmonary hemorrhage occurs chiefly in adult patients and is almost certainly capillary in origin. In recurrent thromboembolism, hemoptysis is considerably more common in the symptomatic form than in the silent form, probably because hemorrhagic infarction and anticoagulant therapy play a greater role in the former situation.
 ...
PMID:[Arterial hypertension and veno-occlusive disease. Connections with pulmonary hemorrhage]. 281 4

Invasive pulmonary haemangiomatosis is a recently described disease in which exceedingly thin-walled vessels of capillary or venous dimensions infiltrate the lung parenchyma and pulmonary blood vessels. The angiomatous vessels, of obscure origin, infiltrate the media and intima of muscular pulmonary arteries, pulmonary veins and venules. The occlusion of the veins and venules by the thin-walled vessels, and the reactive intimal fibrosis they provoke, leads to pulmonary capillary dilatation, collections of intra-alveolar siderophages, fibrosis of alveolar walls and osseous nodules. This secondary pulmonary veno-occlusive disease in turn leads to hypertensive pulmonary vascular disease. Hence invasive pulmonary haemangiomatosis represents a fourth cause of 'unexplained pulmonary hypertension', the other three being unexplained plexogenic pulmonary arteriopathy, recurrent pulmonary thromboembolism, and pulmonary veno-occlusive disease. Two previously reported cases of invasive pulmonary haemangiomatosis presented with recurrent haemoptysis and the gradual development of chronic respiratory insufficiency associated with diffuse infiltrates in the chest radiograph. In one of these cases a haemothorax had developed. Such clinical features may be of importance in coming to the correct diagnosis.
 ...
PMID:Invasive pulmonary haemangiomatosis. 401 59

Segmental mediolytic arteriopathy (SMA) is defined as non-inflammatory arteriopathy with mediolysis due to segmental loss of media and consecutive formation of vascular gaps. Up to now, less than 40 cases of visceral and cerebral SMA and, to our knowledge, only one case of pulmonary SMA have been reported. We present the history of a 21 year old female patient, admitted to hospital with hemoptysis, but without other symptoms. Apart from two lesions in the sixth and tenth pulmonary segment, documented by CT and interpreted as colliquations, there were no other clinical and laboratory findings. Repeated bronchoscopy supplied no further information. Histomorphology of the resected lesion revealed SMA without evidence of vasculitis. Wegener's disease could be excluded. The aetiology of the disease is still unknown. Acute vasospasm (due to inappropriate reactions to catecholamine or endothelial dysfunction), as well as SMA as a precursor or subtype of fibromuscular dysplasia, are two theories still under discussion.
 ...
PMID:[Pulmonary segmental mediolytic arteriopathy]. 1645 8

A large proportion of patients with congenital heart disease (CHD), in particular those with relevant systemic-to-pulmonary shunts, will develop pulmonary arterial hypertension (PAH) if left untreated. Persistent exposure of the pulmonary vasculature to increased blood flow, as well as increased pressure, may result in pulmonary obstructive arteriopathy, which leads to increased pulmonary vascular resistance that, if it approaches or exceeds systemic resistance, will result in shunt reversal. Eisenmenger's syndrome, the most advanced form of PAH associated with CHD, is defined as CHD with an initial large systemic-to-pulmonary shunt that induces severe pulmonary vascular disease and PAH, with resultant reversal of the shunt and central cyanosis. The histopathological and pathobiological changes seen in patients with PAH associated with congenital systemic-to-pulmonary shunts, such as endothelial dysfunction of the pulmonary vasculature, are considered similar to those observed in idiopathic or other associated forms of PAH. A pathological and pathophysiological classification of CHD with systemic-to-pulmonary shunt leading to PAH has been developed that includes specific characteristics, such as the type, dimensions and direction of the shunt, extracardiac abnormalities and repair status. A clinically oriented classification has also been proposed. The prevalence of PAH associated with congenital systemic-to-pulmonary shunts in Western countries has been estimated to range between 1.6 and 12.5 cases per million adults, with 25-50% of this population affected by Eisenmenger's syndrome. Clinically, Eisenmenger's syndrome presents with multiple organ involvement, with progressive deterioration of function over time. The signs and symptoms of Eisenmenger's syndrome in the advanced stages include central cyanosis, dyspnoea, fatigue, haemoptysis, syncope and right-sided heart failure. Survival of patients with Eisenmenger's syndrome is clearly less than that of the general population, but appears to be better than that of patients with idiopathic PAH in a comparable functional class. The treatment strategy for patients with PAH associated with congenital systemic-to-pulmonary shunts and, in particular, those with Eisenmenger's syndrome is based mainly on clinical experience rather than being evidence based. General measures include recommendations for physical activity, pregnancy, infections, air travel, exposure to high altitudes and elective surgery, and that psychological assistance be provided as necessary. Phlebotomies are required only when hyperviscosity of the blood is evident, usually when the haematocrit is >65%. The use of supplemental oxygen therapy is controversial and it should be used only in patients in whom it produces a consistent increase in arterial oxygen saturation. Oral anticoagulant treatment with warfarin can be initiated in patients with pulmonary artery thrombosis and absent, or only mild, haemoptysis. The following three classes of drugs targeting the correction of abnormalities in endothelial dysfunction have been approved recently for the treatment of PAH: (i) prostanoids; (ii) endothelin receptor antagonists; and (iii) phosphodiesterase-5 inhibitors. The efficacy and safety of these compounds have been confirmed in uncontrolled studies in patients with PAH associated with corrected and uncorrected congenital systemic-to-pulmonary shunts, as well as in patients with Eisenmenger's syndrome. One randomized controlled trial reported favourable short- and long-term outcomes of treatment with the orally active dual endothelin receptor antagonist bosentan in patients with Eisenmenger's syndrome. Lung transplantation with repair of the cardiac defect or combined heart-lung transplantation are options for Eisenmenger's syndrome patients with a poor prognosis. A treatment algorithm based on the one used in the treatment of PAH patients is proposed for patients with PAH associated with corrected and uncorrected congenital systemic-to-pulmonary shunts and Eisenmenger's syndrome.
 ...
PMID:Management of pulmonary arterial hypertension associated with congenital systemic-to-pulmonary shunts and Eisenmenger's syndrome. 1848 98