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Alpha 1-antitrypsin deficiency panniculitis: a histopathologic and immunopathologic study of four cases.

Four cases of alpha 1-antitrypsin deficiency associated with panniculitis were reviewed. The following histopathologic characteristics were typically present: (a) large areas of normal panniculus adjacent to severe necrotic panniculitis; (b) acute panniculitis--masses of neutrophils causing necrosis and replacing fat lobules; (c) chronic inflammation and hemorrhage at the periphery of acute panniculitis; (d) focal collection and proliferation of histiocytic cells and lipophages; (e) secondary leukocytoclastic vasculitis in areas of necrosis and lymphocytic vasculitis in areas of severe inflammation, but no evidence of primary vasculitis; (f) phlebothrombosis; and (g) in partial (heterozygous) deficiency, pronounced lipophages and giant-cell replacement of fat cells. Endarteritis obliterans was noted. Direct immunofluorescence study showed C3 deposition in the blood vessels of the panniculus or dermis (or both) in all four cases, and IgM was present in the blood vessels of three. Weak granular deposits of IgM or C3 were seen at the epidermal basement membrane zone in two cases. C3 in endothelial cells of the blood vessels was detected in one case. In general, the immunoreactants in the blood vessels were not dense and probably represent secondary vasculitis. We conclude that all patients with severe panniculitis and ulceration should have alpha 1-antitrypsin levels determined. A deep excisional biopsy specimen with abundant panniculus tissue is required for histopathologic diagnosis.

Adipose Tissue↗

Predominant right ventricular infarction in malignant renovascular hypertension in rats.

Malignant hypertension was induced in Wistar rats of both sexes by complete aortic ligature just above the origin of the left renal artery. An acute and a late phase of hypertension, 4 days and 28 days after the aortic ligature respectively, were defined to study the relationship between the severity of hypertension and injury of the small arteries and myocardial necrosis. The most frequent finding in the heart was the presence of right ventricular infarction. In acute-phase hypertension most of the animals showed acute infarction of the right ventricle, but only focal left ventricular infarction was found. Lesions in the large coronary arteries were not observed. However, the small arteries and arterioles of the right ventricle presented fibrinoid necrosis lesions. In late-phase hypertension, the intramyocardial small vessels showed proliferative endarteritis and fibrinoid necrosis lesions, but this time they appeared in both ventricles. The severity of the myocardial infarcts and the percentage of small arteries and arterioles injured in the right ventricle were significantly higher than in the left ventricle despite the maintenance of blood pressure almost to the same level. No changes in the right ventricular pressure were observed in either phase. These observations suggest that in malignant renovascular hypertension the intramyocardial arteriolar lesions appear to be an important factor in the production and the severity of myocardial infarcts with predominance in the right ventricle.

Animals↗

[Method of surgical treatment of aneurysms of the peripheral vessels].

The author advocates active surgical tactics in aneurysm both in the stage of pulsatile hematoma and in immature aneurysm since only operation may prevent the development of such complications as hemorrhage, suppuration of the aneurysm, thrombosis of a major vessel, bacterial endarteritis, and bacterial endocarditis. The development of specialized medical service makes it possible to conduct a skilled operation, which greatly reduces the term of the patient's disability.

Adult↗

Radiation enteritis.

As the population receiving radiation therapy grows, so does the incidence of chronic radiation enteritis. A review of the pathology of chronic radiation enteritis reveals fibrosis, endarteritis, edema, fragility, perforation, and partial obstruction. Conservative management of patients with this disease is common. Because the obstruction is only partial, decompression is easily achieved with nasogastric suction and parenteral support. The patient is then often discharged on a liquid-to-soft diet. This therapeutic strategy does nothing for the underlying pathology. The problem, sooner or later, will return with the patient further depleted by the chronic radiation enteritis. We think surgical intervention is appropriate when the diagnosis of chronic radiation enteritis is assumed. The surgery in relation to this disease is high risk with a 30% mortality and 100% expensive morbidity. Early intervention seems to decrease these figures. All anastomoses, if possible, should be outside the irradiated area. Trapped pelvic loops of intestine should be left in place and a bypass procedure with decompressing enterostomies accomplished. The surgery should be performed by a surgeon with extensive experience with all kinds of bowel obstruction as well as experience in performing surgery in radiated tissue.

Enteritis↗

Mixed connective tissue disease with pulmonary hypertension: a clinical and pathological study.

Clinical and autopsy findings of 2 cases with mixed connective tissue disease (MCTD) associated with severe pulmonary hypertension are reported. Both cases showed marked intimal and medial thickening in small and medium sized pulmonary arteries. In one of the cases plexiform lesions, endarteritis obliterans, and fibrous intimal thickening of the pulmonary veins were recognized. In some cases with MCTD, pulmonary hypertension develops due to pulmonary vascular lesions which are similar to those of primary pulmonary hypertension.

Adult↗

Malignant or accelerated hypertension in IgA nephropathy.

A retrospective analysis of 66 adult patients diagnosed as having IgA nephropathy by renal biopsy revealed that 24 (36%) were hypertensive when first seen. Of these hypertensive patients, 10 (15%) had malignant or accelerated hypertension. All patients but one were male and had no knowledge of their renal disease and sought medical advice for symptoms due to hypertension. Five patients had no history of gross hematuria. Histological vascular findings showed, in three proliferative endarteritis and fibrinoid necrosis, in five arteriolosclerosis and in two vascular hypertrophy. In spite of good blood pressure control, six patients reached terminal uremia within a maximum of 14 months. In summary, the incidence of malignant hypertension in adults with IgA nephropathy is higher than previously reported, and its true incidence can only be known if more histopathologic studies of patients with malignant hypertension are performed. Patients with this association reach end stage renal failure in a short period of time.

Adult↗

Lyme arthritis. Spirochetes found in synovial microangiopathic lesions.

In 17 patients with Lyme disease, synovial specimens, obtained by synovectomy or needle biopsy, showed nonspecific villous hypertrophy, synovial cell hyperplasia, prominent microvasculature, lymphoplasmacellular infiltration, and sometimes lymphoid follicles. The larger surgically obtained specimens also showed striking deposition of fibrin in synovial stroma and a form of endarteritis obliterans. In 2 patients, spirochetes were seen in and around blood vessels by the Dieterle silver stain. Compared with 55 cases of other synovial disease, obliterative microvascular lesions were seen only in Lyme synovia, but marked stromal deposition of fibrin seemed nonspecific. These findings imply that the Lyme spirochete may survive for years in affected synovium and may be directly responsible for the microvascular injury.

Adolescent↗

[Prinzmetal's angina. Histological study of peroperative coronary specimens. Apropos of 2 cases].

The results of histological examination of two coronary artery biopsy specimens removed at operation are presented. There were unusual findings, especially of a highly cellular and oedematous fibroblastic endarteritis, of the lack of calcification, and of the presence of marked medial sclerosis. These unusual histological findings are the result of solitary variable stenoses of the coronary arterial tree, and very different from the histological findings in the atheromatous lesions which are usually found when Prinzmetal's angina is super-imposed on lesions which are multiple and diffuse.

Angina Pectoris↗

Osteolysis of the ribs in progressive systemic sclerosis.

In a patient with progressive systemic sclerosis (PSS), osteolysis of the posterior portion of the rib cage developed in an insidious fashion, without symptoms or preceding trauma. Six previous examples of rib resorption in PSS are reviewed. The destructive mechanism is unknown but may be related to endarteritis and ischemia.

Bone Resorption↗

[Frequency, etiological factors and the morphological characteristics of myocardial infarct in youth].

Examinations were carried out on 827 deceased with myocardial infarction (MI), 27 (3.26%) out of them being at a rather early age (26-44). The average age of those examined with MI at that early age is 39. The males are affected more frequently than females -- ratio 3.5:1. In 89 per cent of the examined the coronary insufficiency was manifested clinically and morphologically before the age of 40 and two or more MI, with a different duration were established. The most frequent causes of the development of MI are coronary atherosclerosis in 48.15 per cent, rheumatism (coronary embolism) in 14.81 per cent, endarteritis obliterans in 7.14 per cent and leutic coronaritis in 3.70 per cent. In 25.93 per cent of the examined only lipoidosis or completely intact coronary arteries were established. Almost in all of the examined cardiac hypertrophia was present, the average weight of the heart being 434 g. In a part of the examined, morphological changes in microcirculation were observed -- namely -- formation of microthrombosis, manifested intimal cushions, fibrosis of the walls and perivasal fibrosis of the muscular arteries and arteriols.

Adult↗

[Aneurysms in Takayasu disease].

Takayashu's inflammatory panarteritis essentially results in stenotic lesions of the supra aortic axes and the collateral vessels of the abdominal aorta. Fusiform, segmental post-stenotic dilatation is commonly observed. Three particular types of aneurysms are described, each illustrated by a case report: --aneurysm of the ascending aorta causing severe aortic regurgitation by dilatation of the aortic ring: the diagnosis of Takayashu's disease was made in a young West Indian female on the evidence of associated aortic lesions and calcification of the descending thoracic aorta. The patient underwent replacement of the ascending aorta and aortic valve replacement. This form is very rare, although mild aortic regurgitation in Takayashu's disease has been reported in about ten cases by different workers; --large saccular aortic aneurysm. This lesion of the descending thoracic aorta with parietal calcification and without intrasaccular thrombosis was associated with a long, irregular stenosis of the paroxismal segment of the aorta, giving rise to signs of coarctation, and with multiple stenoses of the supra-aortic axes in a 31 year old Algerian. Surgical cure was realised by occluding the orifice in order to avoid replacing the aorta at the origin of the main medullary arteries. An ascending aorta-abdominal aorta bypass was performed at the same time, together with correction of the stenoses of the supra-aortic vessels. A few similar cases of large succular aneurysms of the thoracic or abdominal aorta or of the large collateral vessels have previously been described; --pseudo-aneurysm due to arterial rupture and formation of a large hematoma. A 20 year old Algerian presented with a polylobulated saccular aneurysm of the superficial femoral artery in the femoral triangle. Several stenotic lesions typical of Takayashu's disease (including a particularly rare stenosis of the contra lateral superficial femoral artery) were associated. At surgery, the lesion was found to be a pseudo-aneurysm with no true arterial wall on histological examination. The role of an infective endarteritis is discussed and excluded. This type of lesion does not appear to have been previously reported. These three varieties of aneurysm were the presenting signs of Takayashu's disease in the cases reported.

Adult↗

[The radiolesions of the small bowel (author's transl)].

The irradiation of the pelvic abdominal cancers extends beyond the centre of the tumour and may induce actinic digestive lesions. The bowel and more rarely the small bowel--which is the subject-matter of our study--are concerned by those radiolesions that are favoured by therapeutic overdose, post-operative adhesions fastening the bows, radio-surgical or chemicostatic associations, and lastly by vascular or nutritive deficiencies. One may distinguish between two kinds of lesions, depending on the lapse of time before their coming out and on the symptoms. The early or acute types are characterized by a radio-mucitis and give an exsudative enteropathy with anorexia, vomiting, diarrhoea and loss of weight, of which the diagnosis is easy because it occurs during the irradiation and lessens at the end of the treatment. The late radiolesions of the small bowel are characterized by sclerosis and chronic endarteritis and, after a longlasting period of latency, give varied symptoms: disordered intestinal transit which sometimes is irreversible, perforation, fistula, syndrome of malabsorption, giving often rise to be mistaken for a recurrence of the cancer. The treatment varies whether the lesion is segmental or diffuse. In the first case, the failure of the medical means accounts for the surgical cutting away or the internal derivation; in the second case, the digestive mutilation which would result from an enlargement of the lesion commands to be more cautions and to call for the methods of parenteral feeding and digestive setting to rest.

Humans↗

Conditions associated with relapse of amphotericin B-treated disseminated histoplasmosis.

Progressive disseminated histoplasmosis (PDH) is a rare consequence of infection with Histoplasmia capsulatum. Usually fatal if untreated, PDH generally is cured by appropriate amphotericin B treatment. Of 31 persons with uncomplicated PDH treated with amphotericin B, we found that relapse occurred in five (16%) after an interval of up to nine years after initial therapy. Review of these five cases and 31 additional relapsing cases from the literature indicates that fungal endocarditis or endarteritis without surgical treatment, underlying lymphoreticular neoplasm, and amphotericin B dosage of less than 2 g appear to be associated with relapse of PDH.

Aged↗

Causes and consequences of placental growth retardation.

Placental growth retardation caused 84 fetal and neonatal deaths per 100,000 births. Its frequency increased as mothers' diastolic blood pressure levels increased, an effect augmented by proteinuria. The perinatal deaths also increased with advancing maternal age, anemia, and poverty. Maternal weight gains were low in the involved pregnancies, and the fetuses and neonates who died had a pattern of growth retardation characteristic of fetal undernutrition. Microscopic abnormalities in the decidua and placenta were characteristic of inadequate perfusion of the placenta from the uterus. They included fibrinoid changes in the arteries and arterioles of the decidua, villous cytotrophoblastic hyperplasia, and an obliterative endarteritis in fetal stem arteries of the placenta.

Decidua↗

Histoplasma infection of abdominal aortic aneurysms.

Fungal endarteritis resulting from progressive disseminated histoplasmosis may cause arterial aneurysms, or lead to infection of pre-existing aneurysms. Three patients with Histoplasma capsulatum infections of abdominal aortic aneurysms are reported. All had previous disseminated histoplasmosis and atherosclerotic peripheral vascular disease. All were considered cured of systemic infection when their aneurysms were discovered. Atherosclerotic vascular lesions may become infected during the course of systemic fungal disease and may serve as a haven for viable organisms in patients whose dissemination recurs despite seemingly adequate antifungal therapy. In treating these patients, resection of all infected arterial tissue, revascularization through uninfected tissues, and long-term antimicrobial therapy are recommended.

Adult↗

Allergic complication in operated gastroduodenal ulcers: pathogeny and morphoclinical significance.

1660 gastric and duodenal ulcers were examined, of which 61 per cent showed allergic complications. The eosinophilic infiltrate associated with endarteritis and fibrinoid necrosis in the area, more accentuated than in ulcers without eosinophils is produced through a mechanism of atopic hypersensitization to food antigens. A more frequent appearance of allergic complications in the gastrointestinal tract explains why some ulcers do not heal and why certain groups of food tolerated by some patients, are rejected by others. It also imposes in some cases a desensitization treatment concomitantly with the antiulcerous one.

Eosinophils↗

Dirofilariasis with arteriosclerosis in a horse.

Arteriosclerosis caused by Dirofilaria immitis adult parasites was diagnosed in a 20-month-old Quarter horse stallion that died from cantharidin toxicosis. Microscopically, the pulmonary vascular changes were typical of those described as "proliferative endarteritis" in D immitis-infected dogs.

Animals↗