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Fibrosing mediastinitis causing pulmonary arterial hypertension without pulmonary venous hypertension. Clinical and necropsy observations
Pulmonary arterial hypertension
Clinical and morphologic observations are described in two patients with severe pulmonary arterial hypertension without pulmonary venous hypertension from fibrosing mediastinitis. In one patient, both right and left main pulmonary arteries and one major pulmonary vein were severely narrowed by dense fibrous tissue; in the second patient, only the right main pulmonary artery was severely narrowed. Both patients had normal intrapulmonary arteries and pulmonary parenchyma. Although usually due to obstruction of multiple large pulmonary veins and to secondary changes in small intrapulmonary arteries, fibrosing mediastinitis can cause severe pulmonary hypertension purely by obstructing the right or both main pulmonary arteries.
Arnett EN, Bacos JM. Macher AM, Marsh HB, Savage DD, Fulmer JD, Roberts WC: Fibrosing mediastinitis causing pulmonary arterial hypertension without pulmonary venous hypertension. Clinical and necropsy observations. Am J Med 63: 634-643, 1977.
Flbroslng mediastinitis
Massive medial calcific deposits are described in the peripheral and visceral arteries, and similar but small-sized deposits in the coronary arteries of a 41 year old woman with diabetes mellitus. Although observed by roentgenogram fairly commonly during life in the muscular arteries of the legs in middle-aged men, medial calcinosis infrequently involves the visceral arteries and has never, to our knowledge, been documented in the coronary arteries. Although it may be associated with intimal atherosclerosis, medial calcinosis does not obstruct the lumens of the arteries and, therefore, does not lead to symptoms or signs of limb or organ ischemia. The cause of medial calcinosis remains a mystery, but it appears to affect people with diabetes more frequently than those without.
Lachman AS, Spray TL, Kerwin DM, Shugoll GI, Roberts WC: Medial calcinosis of Monckeberg. A review of the problem and a description of a patient with involvement of peripheral, visceral and coronary arteries. Am J Med 63: 615-622, 1977.
Peripheral arteries Medial calcinosis of Miinckeberg Visceral arteries Coronary arteries Diabetes mellitus
Idiopathic aortitis
Takayasu’s arteritis
Menetrier’s disease
Gastric carcin
Described here is a 33 year old man who presented with epigastric pain, weight loss and h matemesis. Roentgenograms of the upper gastrointestinal tract and endoscopy revealed gia rugal folds, and the diagnosis of hypertrophic gastropathy was confirmed by operative biops This patient’s symptoms and roentgenographic abnormalities persisted until his death 16 yea later. A review of 120 previously reported cases of hypertrophic gastropathy with special attentic to its natural history reveals that the chronic nature of this patient’s illness is typical. In additio in these patients the chance of gastric carcinoma developing during long-term follow-up appreciable. Since the reported lonpterm results of operative therapy appear favorable, patien with persistent and sufficiently distressing symptoms should be considered for gastric rese tion.
Scharschmidt BF: The natural history of hypertrophic gastropathy (Menetrier’s disease). Report of a case with 16 year follow-up and review of 120 cases from the literature. Am J Med 63: 644-652, 1977.
Gastric surgery
Hypertrophic gastropathy
Clinical and morphologic features are described in two relatively young adults with aortic gurgitation secondary to chronic aortitis. The regurgitation in each was severe enough to requ aortic valve replacement. Both patients had normochromic, normocytic anemia, consideral weight loss despite congestive cardiac failure, and negative serologic tests for syphilis. The systemic manifestations in association with the aortitis suggest that both had Takayasu’s arteril In addition, one patient had total occlusion at the origin of one subclavian artery (classic pulsele disease). Takayasu’s arteritis must be added to the list of causes of severe aortic regurgi tion.
Honig HS, Weintraub AM, Gomes MN, Hufnagel CA, Roberts WC: Severe aortic regurgitation secondary to idiopathic aortitis. Am J Med 63: 623-633, 1977.
Aortic regurgitation
Clinical and morphologic observations are described in two patients with severe pulmonary arterial hypertension without pulmonary venous hypertension from fibrosing mediastinitis. In one patient, both right and left main pulmonary arteries and one major pulmonary vein were severely narrowed by dense fibrous tissue; in the second patient, only the right main pulmonary artery was severely narrowed. Both patients had normal intrapulmonary arteries and pulmonary parenchyma. Although usually due to obstruction of multiple large pulmonary veins and to secondary changes in small intrapulmonary arteries, fibrosing mediastinitis can cause severe pulmonary hypertension purely by obstructing the right or both main pulmonary arteries.
Arnett EN, Bacos JM. Macher AM, Marsh HB, Savage DD, Fulmer JD, Roberts WC: Fibrosing mediastinitis causing pulmonary arterial hypertension without pulmonary venous hypertension. Clinical and necropsy observations. Am J Med 63: 634-643, 1977.
Flbroslng mediastinitis
Massive medial calcific deposits are described in the peripheral and visceral arteries, and similar but small-sized deposits in the coronary arteries of a 41 year old woman with diabetes mellitus. Although observed by roentgenogram fairly commonly during life in the muscular arteries of the legs in middle-aged men, medial calcinosis infrequently involves the visceral arteries and has never, to our knowledge, been documented in the coronary arteries. Although it may be associated with intimal atherosclerosis, medial calcinosis does not obstruct the lumens of the arteries and, therefore, does not lead to symptoms or signs of limb or organ ischemia. The cause of medial calcinosis remains a mystery, but it appears to affect people with diabetes more frequently than those without.
Lachman AS, Spray TL, Kerwin DM, Shugoll GI, Roberts WC: Medial calcinosis of Monckeberg. A review of the problem and a description of a patient with involvement of peripheral, visceral and coronary arteries. Am J Med 63: 615-622, 1977.
Peripheral arteries Medial calcinosis of Miinckeberg Visceral arteries Coronary arteries Diabetes mellitus
Idiopathic aortitis
Takayasu’s arteritis
Menetrier’s disease
Gastric carcin
Described here is a 33 year old man who presented with epigastric pain, weight loss and h matemesis. Roentgenograms of the upper gastrointestinal tract and endoscopy revealed gia rugal folds, and the diagnosis of hypertrophic gastropathy was confirmed by operative biops This patient’s symptoms and roentgenographic abnormalities persisted until his death 16 yea later. A review of 120 previously reported cases of hypertrophic gastropathy with special attentic to its natural history reveals that the chronic nature of this patient’s illness is typical. In additio in these patients the chance of gastric carcinoma developing during long-term follow-up appreciable. Since the reported lonpterm results of operative therapy appear favorable, patien with persistent and sufficiently distressing symptoms should be considered for gastric rese tion.
Scharschmidt BF: The natural history of hypertrophic gastropathy (Menetrier’s disease). Report of a case with 16 year follow-up and review of 120 cases from the literature. Am J Med 63: 644-652, 1977.
Gastric surgery
Hypertrophic gastropathy
Clinical and morphologic features are described in two relatively young adults with aortic gurgitation secondary to chronic aortitis. The regurgitation in each was severe enough to requ aortic valve replacement. Both patients had normochromic, normocytic anemia, consideral weight loss despite congestive cardiac failure, and negative serologic tests for syphilis. The systemic manifestations in association with the aortitis suggest that both had Takayasu’s arteril In addition, one patient had total occlusion at the origin of one subclavian artery (classic pulsele disease). Takayasu’s arteritis must be added to the list of causes of severe aortic regurgi tion.
Honig HS, Weintraub AM, Gomes MN, Hufnagel CA, Roberts WC: Severe aortic regurgitation secondary to idiopathic aortitis. Am J Med 63: 623-633, 1977.
Aortic regurgitation