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Biomedical subjects

B Wechsler

Publications and source records attributed to B Wechsler.

At least 307 records · Page 17Linked to original sources

[Myocardial infarction in Behçet's disease].

A case of myocardial infarction in a 23-year old male patient with Behçet's disease is reported. The infarction occurred 4 years after the onset of the disease, which had been marked by recurrent venous thrombosis. Coronary arteriography showed stenosis of the anterior interventricular artery and occlusion of the first diagonal artery; the other coronary vessels were normal. A search for vascular risk factors, including haemostasis, was undertaken, yielding only moderate cigarette-smoking. About 10 cases of myocardial infarction associated with Behçet's disease have been reported. They concerned young, usually male subjects. Infarction usually occurred late in the course of the disease, and vascular risk factors were seldom elicited. The leukocytoclastic vasculitis of Behçet's disease alone may be responsible for stenosis, thrombosis and false arterial aneurysms, as shown by anatomical studies. The physiopathological mechanisms involved (reduction of endothelial or systemic fibrinolytic activity, rise in fibrinogen and factor VIII) are still unclear; we believe that these abnormalities are inconstant. Behçet's disease may be regarded as a possible cause of myocardial infarction in young subjects.

Adult↗

[Lupus vasculitis].

Anatomical studies have demonstrated the high incidence of vasculitis in SLE, the appearances of which are variable and non-specific, ranging from necrotizing angiitis which is undistinguishable from periarteritis nodosa, to scarring lesions. Micro-angiitis is easily demonstrated in skin lesions and is also encountered to varying degrees in CNS, renal, cardiac, pulmonary and gastrointestinal localisations. Disease of large vessels is more rare and sometimes causes gangrene of the limbs. In SLE, vasculitis should be distinguished from thrombosis related to lupus anticoagulant and from atherosclerosis favoured by chronic steroid therapy but perhaps initiated by vascular deposits of immune complexes during the acute inflammatory stage. The treatment of lupic angiitis is mainly based on steroid therapy. The results are variable, probably due to the fibrous nature of some of the vascular lesions.

Humans↗

[Diffuse disease of elastic tissue and hyperphosphoremia].

Pseudoxanthoma elasticum (PXE) is exceptionally associated with abnormal phosphate-calcium metabolism. On the other hand, significant hyperphosphataemia is present in tumoral calcinosis, with an occasional elastopathy resembling that of PXE. We report here the first case of diffuse elastic tissue disease associated with hyperphosphataemia in the absence of tumoral calcinosis. The patient, a 29-year old Gabonese, had severe arteritis of the lower limbs with linear calcifications of the aorta and major arteries of the limbs. Angioid streaks and calcifications were present in the retinas. The skin appeared to be normal, but histological examination of specimens from flexures showed abnormalities of the elastic tissue with many fibres being broken into small pieces and curled up. In a morphometric study of cutaneous elastic tissue these abnormalities were compared with those observed in a PXE patient and with normal elastic tissue. The abnormalities in our patient and in the PXE patient were similar. Compared to normal elastic tissue, there was a slight decrease in the total number of oxytalan and elaunin fibres. The elastic fibres were distinctly wider, longer and separated from each other by smaller spaces, but not more numerous than normally. The histopathological appearance of an artery removed during bypass surgery was similar to that of a PXE artery. The hyperphosphataemia was due to an increase in tubular absorption of phosphates without abnormalities of parathyroid hormone, or resistance to its phosphaturic effect or any other tubular disorder. It was little altered by aluminium carbonate, and it partially decreased after addition of calcitonin.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

[Inappropriate secretion of antidiuretic hormone disclosing acute intermittent porphyria].

The authors report a case of acute intermittent porphyria in a 31-year old woman, revealed by inappropriate secretion of the antidiuretic hormone, a diagnosis confirmed by radioimmunoassay. Measurements of red cell urosynthetase made the diagnosis of porphyria certain and showed that the disease was familial, although clinically asymptomatic. Antidiuresis is not the only cause of hyponatraemia in acute intermittent porphyria. Similarly, psychic disorders are frequent in SIADH, but they are not necessarily due to acute intermittent porphyria.

Acute Disease↗

[Renal and digestive amyloidosis and Behçet's disease. Apropos of a case].

The authors report the case of a 33-year old male Tunisian who, 7 years after the onset of complete Behçet's disease (diagnosed on international criteria) developed a nephrotic syndrome diagnosed as renal amyloidosis on histological evidence. Attenuation of Congo red staining by potassium permanganate pointed to AA type amyloidosis. Despite treatment with colchicine and cyclophosphamide the patient's condition deteriorated. Amyloid deposits were found in a small bowel biopsy performed for persistent diarrhoea. The patient died 3 years after amyloidosis was diagnosed. The authors have reviewed the literature concerning this exceptional association: in all cases where it was typed the amyloidosis was AA. Attention is drawn to the failure of colchicine in this patient.

Adult↗

[Acute sphenoid sinusitis in adults].

Seven cases of acute sphenoid sinusitis were reviewed. Two happened after deep sea diving. Headache of variable location and fever were the most predominant presenting symptoms (5/7). Purulent discharge in the cavum was present in three. Correct diagnosis was delayed from six to twenty-four days after beginning of symptoms, when a neurologic deficit became apparent : mainly paralysis of the sixth and third cranial nerves. Computed axial tomography was the most useful radiologic procedure for demonstrating sinus opacification in every patient. Two patients died, three had complete recovery, one had persistent epileptic focus after brain abscess. Last patient had paraparesis and incontinence of urine after study of cerebrospinal fluid (C. S. F.) flow with methylene blue. Cannulation of the sphenoid sinus was performed in two patients and surgical drainage in three. Gram negative microorganisms and Staphylococcus aureus were isolated from the sphenoid sinus of only three patients. Blood cultures were negatives in every cases and C. S. F. in six. First choice antibiotics was an association of aminoglucosides and broad-spectrum penicillin in six cases. It always had to be changed.

Adult↗

[Toxoplasmosis and lupus. A review of the literature apropos of 4 cases].

The authors report 4 cases of toxoplasmosis in patients with acute disseminated lupus erythematosus (ADLE). In one case, a pregnant patient with serology indicative of chronic infection, infected the neonate who died of subacute toxoplasmosis. Although ADLE is a classical cause of immunodepression, toxoplasmosis is a rare complication; only 5 cases were found in a review of the literature. Toxoplasmosis infection may resemble an exacerbation of lupus; an accurate diagnosis is essential as the treatment of the two conditions is radically different. The problems of diagnosis of toxoplasmosis in immunodepressed patients are reviewed and the therapeutic approach, especially in pregnant patients, is discussed. In ADLE, the authors recommend checking toxoplasmosis serology before starting and during treatment with corticosteroids. Special attention should be paid to pregnant women with apparently chronic serological changes as neonatal infection may occur.

Adolescent↗

[Caval thromboses and systemic diseases].

Sixty-five patients presenting with a vena cava syndrome have been examined over a 7 years period; incidence of systemic diseases as an etiologic factor has thus been studied. A systemic disease was diagnosed in 17 cases, that is 25 p. 100 of cases. This incidence is higher in inferior vena cava syndromes (15 cases out of 45). Two diseases appear as the main factors: on one hand, Behçet disease, liable for 8 vena cava thrombosis, 7 inferior and 8 superior thrombosis; on the other hand, severe disseminated erythematous lupus liable for 5 cases of inferior vena cava thrombosis. Other etiologies are mentioned such as: Churg and Strauss disease (1 case), Horton disease (1 case) and hemorrhagic rectocolitis (1 case). In the patients presenting with lupus, two died from various pulmonary embolisms; a vena cava clip was required in 3 cases. As for lupus, the immuno-hepatologic exploration did demonstrate a thrombopenia in 5 cases, an hemolytic anemia with a positive Coombs test in 2 cases, and a circulating anti-coagulant (antiprothrombinase type) in one case. Though high frequency of systemic diseases in this series may depend on the patients' recruitment, systemic lupus as well as Behçet diseases are to be researched in case of vena cava thrombosis, above all if diagnosed in a young patient and failing to be explained by a congenital abnormality of hemostasis.

Adult↗

[Unexplained prolonged fever and inflammatory syndromes. Diagnostic value of abdominal X-ray computed tomography. Randomized prospective study of 36 cases].

A randomized study was carried out in 36 patients to determine the diagnostic value of abdominal computerized tomography (CT) in protracted fever of obscure origin and/or unexplained inflammatory syndromes. There were no clinical or biological signs pointing to a diagnosis, and 55% of the patients had already been unsuccessfully explored in other hospital departments. CT was performed initially in group I patients and after some of 56 predetermined conventional examinations (including 20 considered "invasive") carried out in a definite order in group II patients. An early diagnosis could be made within 2 months in 17 patients (47%): 7 in group E and 10 in group II. The underlying disease was diagnosed in 4 cases in group I and 1 case in group II (where 4 other cases could have been diagnosed, had CT been performed earlier); the difference was not significant. Nor was there any difference between the two groups in the number of examinations performed, the cost of investigation and the duration of stay in hospital. However, a number of reasons are in favour of early CT examination in protracted fever: the so-called "invasive" explorations were uncomplicated in group I and complicated in 2 patients of group II; following randomization, a diagnosis was made by CT within less than 3 days in the 4 patients of group I, and there were neither false-positive nor false-negative results of CT. If CT had been performed early in all patients, 9 diagnoses would have been made, representing 25% of the cases or 1/2 of early diagnoses. Abdominal CT proved unable to establish the 10 late diagnoses (i.e. after a mean delay of 18 months).

Adult↗