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Biomedical subjects

P Godeau

Publications and source records attributed to P Godeau.

At least 451 records · Page 25Linked to original sources

[Neuropathy associated with ichthyosis and splenomegaly in an adult patient. Possible relationship with altered glycosphingolipid metabolism (author's transl)].

The authors report on the case of a 40-year-old woman presenting with ichtyosiform erythroderma, central and peripheral neuropathy, splenomegaly and low serum haptoglobin levels. The patient's blood and urine contained large amounts of various ceramides polyhexoside including, in particular, a ceramide tetrahexoside containing fucose. The possibility of a new form of sphingolipidosis is discussed.

Adult↗

[Aseptic osteonecrosis during corticosteroid treatment of Behçet's disease (author's transl)].

Four patients with Behçet's disease (a 42-year-old woman and 3 men aged 24, 32 and 39 respectively), who had received high doses of prednisone (0.5 to 1 mg/kg/day) during a mean period of 21 months developed aseptic osteonecrosis of the femoral heads and condyles which obliged to reduce steroid dosage and to introduce additional drugs. Corticosteroids are useful in Behçet's disease, as in other systemic diseases, but they probably act also on the underlying immune vascularitis. The occurrence of pain in weight-bearing joints during treatment should suggest osteonecrosis and in such cases, beside radiography, scintigraphy with technetium and/or phlebography with pressure measurement might provide an early diagnosis.

Adrenal Cortex Hormones↗

[Efficacy of captopril in periarteritis nodosa with arterial hypertension and renal failure (author's transl)].

In a patient with periarteritis nodosa failure of antihypertensive treatment with beta-blockers and diuretics encouraged the authors to use captopril in doses of 150 mg per day. Blood pressure rapidly returned to normal levels. The hypotensive effect of captopril is ascribable to its action on the renin-angiotensin system, since plasma renin activity was particularly high in this patients.

Angiotensin I↗

[Periarteritis nodosa associated with hepatitis B virus. 42 cases (author's transl)].

HBs antigen was detected by immunodiffusion or radioimmune assay in the blood of 19 out of 42 patients with periarteritis nodosa examined between 1970 and 1978. Histological or biochemical signs of hepatitis were present in 6% of HBs--positive patients and 1% of HBs-negative patients. The incidence of clinical symptoms was similar in both groups, except for multiple neuritis, which was much more frequent in HBs-positive patients (89%) than in HBs-negative patients (52%; p less than 0.001), suggesting a close relationship between neuritis and HBs and HBs antigen. There was a negative correlation between myalgias and skin lesions and the presence of the antigen. The prognosis of periarteritis nodosa was similar in both groups. The course of the disease seemed to be more prolonged in HBs-positive patients, but there was no significant difference in mortality rate.

Adult↗

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↗

Pulmonary involvement in Behcet disease.

The radiologic appearance of pulmonary involvement in six cases of Behcet disease is described. Chest radiographs in five patients showed infiltrates and/or rounded opacities followed by excavation in two cases and by pleural rupture in one. Repeat chest films on four of these five patients 3 weeks to 9 months after diagnosis showed resolution of the infiltrates and the subpleural opacities. The other findings from chest radiography in three patients were rounded or lobulated opacities near the hila. Four of the six patients underwent pulmonary angiography, which in all cases showed wide-spread occlusions of pulmonary arteries, accompanied in three cases by segmental or lobular pulmonary artery aneurysms corresponding to the proximal opacities visible on the plain films. Two of the three patients who displayed pulmonary artery aneurysms died of massive hemoptysis 3 and 13 months after angiography. In the third patient, progress under medical treatment was favorable; chest film 10 months after treatment started showed complete resolution of the aneurysms. Repeat angiogram also showed partial recanalization of the occluded arteries.

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↗

[The complement system in familial Mediterranean fever: studies in 41 families (author's transl)].

A total increase of blood complement components, particularly C4, is found in subjects with familial Mediterranean fever both before and after colchicine therapy. This effect differs from the serum haptoglobin and orosomucoïd concentration decreases detected after identical therapy, confering diagnostic value to this inflammatory syndrome. This could be of both hepatic and extrahepatic origin. For the latter, it is possible that up take of circulating monocytes, macrophage precursors, by the connective tissue of the serum sub-mesothelial layer is responsible for the lesion.

Complement C1 Inactivator Proteins↗

[Cardiac manifestations of the hypereosinophilia syndrome: a report on fifteen cases (author's transl)].

A retrospective study was conducted in fifteen patients with the hypereosinophilia syndrome to assess the cardiac manifestations of the affection. Clinical, electrical, and radiological findings, together with the results of ultrasonography (8 cases), hemodynamic tests (6 cases), and pathological examinations (7 cases) were used to classify the fifteen patients into four groups: 7 cases with endomyocardial fibrosis (EMF), 5 with non-obstructive cardiomyopathy (NOCM), 2 with pre-existing cardiopathies, and 1 with a normal heart. The manifestations in the 7 EMF cases varied according to the extent of the fibrosis present: 2 adiastolies, 3 valve incompetences, and 2 asymptomatic forms. Cardiac insufficiency can occur in the absence of fibrosis, pathological examination in one case demonstrating that it was due to a myocarditis, with infiltration of eosinophils and necrotic foci. The results of this study underline the close relationship between Loëffler's fibroplastic endocarditis and Davies' fibrous endomyocarditis. The presence of vascular lesions in the hypereosinophilia syndrome leads to difficulties in nosological distinction with some forms of necrotizing angitis especially Churg and Strauss' syndrome.

Adult↗

[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↗

Polycythemia vera and primary hyperparathyroidism.

A patient with the clinical manifestations of polycythemia and hypercalcemia underwent an extensive examination for occult neoplasm. No malignancy was found; however, a parathyroid adenoma was excised. After its removal, the hypercalcemia and polycythemia disappeared during a two-year follow-up period.

Adenoma↗

[Disorders of conduction in lupus erythematosus : frequency and incidence in a group of 112 patients (author's transl)].

A retrospective study of 112 cases of lupus erythematosus, 103 acute disseminated lupus erythematosus (ADLE) and 9 chronic discoid lupus (CDL), was conducted to determine the incidence of disorders of conduction (DC), and to study their prognosis and discuss their pathogenicity. The mean age of the group was 38 +/- 16 years, and the mean follow-up period after discovery of the DC was 53 months. Cardiac lesions were present in 49.5 p. cent of the 103 patients with ADLE : pericarditis in 27 p. cent, murmur from lupus endocarditis or cardiomyopathy in 23 p. cent, heart failure in 4.8 p. cent, and hypertension in 17 p. cent. Disorders of conduction were present in 18 (17.5 p. cent) of the 112 patients studied. These included 5 partial right bundle-branch blocks (no complete right bundle-branch block), 2 complete and 3 partial left bundle-branch blocks, 5 complete, 2 first degree, and 1 second degree atrioventricular blocks (AVB). The atrioventricular blocks were usually located in the truncal or fascicular regions, but in 2 cases they were nodal in origin. The 5 complete AVB were associated with ADLE in two cases and CDL in the three other cases. The AVB in the ADLE cases appeared 9 to 20 years after the onset of the lupus, these two patients developing pericardiomyocarditis unaccompanied by disorders of conduction. The three complete AVB occurring during CDL were detected 9 to 18 months after the diagnosis. A fatal outcome was noted in 13 (12.5 p. cent) of the ADLE patients and one of the 9 cases of CDL. Ten-year survival curves showed no difference in prognosis for the groups with or without disorders of conduction, but mortality increased in patients with DC after 10 years. As disorders of conduction were more frequently observed in patients with lupus than in a control population, they can be attributed to either a lupus myocarditis or prolonged administration of synthetic antimalarial agents. Disorders of conduction, and particularly complete AVB are, in fact, observed in patients without pericardiomyocardial lesions, and when they exist usually develop a long time after the onset of the cardiac lesion. All patients had been treated with antimalarials, however, and the onset of the DC was associated with a chloroquine myopathy in some of them. Three of the five complete AVB were observed during the course of CDL in patients without cardiac lesions, this being a supplementary argument for implicating synthetic antimalarials.

Adult↗