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Biomedical subjects

B Wechsler

Publications and source records attributed to B Wechsler.

At least 361 records · Page 20Linked to original sources

Multiple pulmonary arterial aneurysms in Behcet's disease and Hughes-Stovin syndrome.

Four case studies of patients with angiographically documented pulmonary arterial aneurysms are presented. In two cases, Behcet's disease was diagnosed; one case corresponded to the syndrome described by Hughes and Stovin, that is, venous thrombosis especially of the vena cava accompanied by singular or multiple pulmonary arterial aneurysms in young patients; and the last case could best be described as an association of the two. Our observations lead us to question the existing notions concerning the relationship between Behcet's disease and Hughes-Stovin syndrome-the clinical, angiographic and histologic aspects of the vascular manifestations are comparable. Typically the two diseases run similar courses with death resulting frm the rupture of the aneurysms and massive hemoptysis. These case studies cast certain doubts as to the effectiveness of the corticosteroid treatment usually prescribed. Finally, we suggest that Hughes-Stovin syndrome might be, in fact, a manifestation of Behcet's disease.

Adult↗

[Superior vena cava syndrome: a rare complication of endocavitary cardiac pacing].

The superior vena cava syndrome is an exceptionally rare complication of endocavitary cardiac pacing, asymptomatic venous thrombosis being much more common. Two cases wee observed as late complications of pacemaker implantation. In the first case the clinical signs regressed with the development of a collateral circulation under heparin therapy after failure of fibrinolytic drugs. In the second one, the severity of the superior vena cava syndrome and the poor quality of the collateral circulation led to the implantation of a Gore-Tex prosthesis. This surgical bypass led to a rapid and complete regression of the clinical signs. It would seem to be a valuable alternative when anticoagulant therapy fails.

Cardiac Pacing, Artificial↗

[21-hydroxylase deficit revealed by hyperkalemia in the absence of renal failure in an insulin-dependent diabetic (author's transl)].

The authors report a case of severe hyperkalemia (7 mmol/l) in an insulin-dependent diabetic in the absence of renal failure. This hyperkalemia was due to hypoaldosteronism caused by inadequate hormone biosynthesis in the absence of 21-hydroxylase. Replacement therapy allowed normalization of clinical and laboratory parameters. The various causes of hyperkalemia in the absence of renal failure in insulin-dependent diabetes are discussed, notably the renin deficiency hypoaldosteronism syndrome. This case study is remarkable for a number of reasons, including the very limited degree of virilization, the occurrence of a pregnancy, and the degree of salt excretion. This is a new observation in the diabetic, and is apparently a coincidental association since deficits in 21-hydroxylase are not usually associated with insulin-dependent diabetes.

Adrenal Hyperplasia, Congenital↗

[Lupus, insulin-resistant diabetes and acanthosis nigricans (author's transl)].

The authors report the case of a 22-year-old Guatemalan in whom lupus was diagnosed 8 months after a second pregnancy. The diagnosis of lupus met the criteria of the ARA: Raynaud's syndrome, alopecia, arthralgia, thrombophlebitis, facial erythema, antinuclear factor at 1/100, Farr at 75 p. 100 and immunofluorescent demonstration of IgM binding in healthy skin. Two months after the beginning of the lupus, there was onset of insulin-resistant ketosic diabetes without overweight. The serum insulin was 1.140 mu U/ml. Acanthosis nigricans was noted and confirmed by a biopsy. Insulin-resistance can be attributed to anti-membrane receptor antibodies titrating at 1/200 (R. Khan). The short-term progress of the disease was favorable on corticosteroid treatment. Insulin could be stopped, but high insulin serum levels persisted. This case meets criteria for type B as defined by Flier, Khan and Roth, and is the first European case of lupus with a complete presentation. Short-term progress was favorable, and there is no evidence to affirm that there will be a tardive progression towards hypoglycemia which is, however, possible due to the persistence of elevated serum insulin levels.

Acanthosis Nigricans↗

[Aneurism in a persisting sciatic artery. One case (author's transl)].

The authors report on the first case of aneurism in a persisting sciatic artery observed in France. The condition was complicated by truncular embolism and fissuration and was successfully treated by endo-aneurismorrhaphy. Restoration of arterial continuity is nevertheless necessary in most cases of aneurysm affecting a complete persistant sciatic artery (providing the vascular supply for the whole lower limb) which represents the commonest anatomical type.

Aged↗

[Compressive extradural lipomatosis as an unusual complication of corticosteroid therapy: one case (author's transl)].

In a 43 year old man we reported a spinal cord compression by extradural lipomatosis associated with long term corticosteroid therapy for periarteritis nodosa. Decompressive surgery at the 16th month of corticosteroid treatment resulted in regression of symptoms and signs. Corticosteroid therapy was felt to be responsible for this neurological complication, which is unknown in periarteritis nodosa, but has been seen in association with intraspinal lipomas.

Adult↗

[Ruptured spleen during bacterial endocarditis. 2 cases (author's transl)].

Splenectomy was performed in two patients amongst a group of 120 suffering from bacterial endocarditis. In the first case, rupture of the spleen followed rupture of a partially thrombosed splenic vein and bacteriological specimens were negative despite the presence of multiple infarcted areas, certain of which were suppurative. In the second case surgery was possible before rupture of the spleen. The latter was the site of numerous abcesses, one of which was in the process of fissuration, and bacteriological specimens were positive. The course in both cases was favourable. Twenty two other cases have been found in the literature with 15 deaths. Whilst the prevalence of such complications remains low, their severity is such that routine surveillance of the kidney should be the rule in infectious endocarditis. The methods of such surveillance are discussed, the mechanism of such complications not being fully elucidated: the respective role of splenic infarction, splenic abcess and immunological factors remains to be determined.

Adolescent↗

[Glomerulopathy during Behçet's syndrome. Immunofluorescent and electron microscopy studies (author's transl)].

Full renal investigation were conducted in 11 patients with Behçet's syndrome. Biological tests revealed the presence of mild proteinuria in 5 of them. Needle biopsy showed extra-membranous or mesangial fibrinoid deposits in 6 cases, associated in 1 case with segmental and focal glomerulitis lesions. Immunofluorescence studies demonstrated glomerular deposits of mesangial and extra-membranous C3, sometimes with immunoglobulins (IgA-AgG). Ultrastructural examination was carried out in 7 cases and showed "hump" deposits attached to the external face of the basal membrane and rounded nodules of 7 to 15 mu included in the mesangium. The presence of immune complexes in the renal glomeruli, therefore, is not all unusual in this disease, which is probably auto-immune in nature, and in fact appears to be a quite frequent occurrence. These deposits, which were probably well-tolerated by most of the patients in this series, might be involved in the production of progressive glomerulopathies in chronic cases, as observed in one of the cases studied.

Adolescent↗

[The association of a digestive carcinoma and subacute endocarditis. 4 cases (author's transl)].

Attention has recently been drawn to the association of digestive carcinoma and streptococcus bovis endocarditis. The authors studied 130 records of patients with endocarditis and an association with a digestive tumour (3 neoplasms, one villous tumour) was found in four. Streptococcus bovis was demonstrated in one case, streptococcus faecalis in two and streptococcus durans in one. The association of carcinoma and subacute endocarditis would thus seem to not solely relate to digestive commensual organisms. The question of common factors favourising carcinoma and endocarditis is raised.

Adenocarcinoma↗

[Scleroderma presenting as oedema (authors transl)].

The authors report 8 cases of scleroderma with oedema in the initial stages which could be divided amongst three groups on the basis of their clinical, biological, histological characteristics and course: normal systemic scleroderma, mixed connective tissue disease, Shulmans syndrome. The relationship between one of these cases and Buschke's scleroderma is discussed. These cases of scleroderma are characterized by diagnostic difficulties in the initial phase and by their relatively favourable vital prognosis (one death only). Corticosteroids may be effective to some extent in the presence of mixed connective tissue disease of Shulman's syndrome.

Adrenal Cortex Hormones↗