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Biomedical subjects

C de Gennes

Publications and source records attributed to C de Gennes.

15 recordsLinked to original sources

Bone mastocytosis. A report of nine cases with a bone histomorphometric study.

Bone mastocytosis is characterized radiographically in some patients by diffuse osteosclerosis and in others by demineralization. The reason for these apparently conflicting bone features is unknown. Bone remodeling and marrow mastocytosis infiltration were studied in nine cases of mastocytosis with bone marrow involvement. Six men, ranging from 42 to 78 years of age, and three women, 43, 55, and 73 years old, comprised the series. Two patients had severe and diffuse osteosclerosis. Seven had diffuse demineralization, with crushed vertebrae in four, suggesting common osteoporosis. In three of the seven, cutaneous mastocytosis was absent. Bone biopsies were undecalcified and stained with toluidine blue. In the seven patients with demineralization, the number of marrow mastocytes was increased (154 +/- 24 versus 2 +/- 0.5/mm2 in normal postmenopausal osteoporosis). Mastocyte nodules covering 1-9% of the marrow area were present in all seven patients. These patients showed a significant increase in remodeling; bone formation rate was increased, coupled with a decrease in mean wall thickness. Concomitantly, osteoclast surfaces were increased, with an increased amount of bone resorbed. The two patients with diffuse osteosclerosis had a markedly different histology; mast cell infiltration was dramatically increased (mastocyte count greater than 1000/mm2) with diffuse marrow fibrosis. Bone volume was increased as well, and most of the bone was woven with an intratrabecular mineralization defect. High bone remodeling and decreased osteoblast activity can explain bone loss in mastocytosis with demineralization. Mastocytosis with osteosclerosis is characterized by a more extensive marrow mast-cell infiltration and fibrosis.

Adult

[Arterial embolism of the limbs in infectious endocarditis of the heart valves].

Nine cases of infective endocarditis (IE) on native valves, complicated by arterial embolism of the limbs (AEL), were collected between June 1974 and October 1988 (prevalence 4.3 percent). Among the 11 AEL recorded, 9 involved the lower limbs and 2 the upper limbs. The diagnosis, suspected in patients with acute ischaemia (n = 6), transient (n = 1) or pseudophlebitic (n = 1) pain, or discovered by systematic pulse examination (n = 3), was confirmed by Doppler ultrasound (n = 3), angiography (n = 2) or oscillometry (n = 4). AEL occurred 2.8 weeks on average after the onset of treatment; it appeared 6 months after the end of treatment in 1 case and preceded the diagnosis of IE by 1 to 6 weeks in 3 cases. The causative organisms isolated in 7 cases were: non-haemolytic streptococci (n = 4), Staphylococcus aureus (n = 1), Haemophilus parainfluenzae (n = 1) and enterococcus (n = 1). Vegetations were found in 6 of the 7 patients explored by echocardiography. Two cases of embolism of the femoral artery required embolectomy. Effective heparin anticoagulation was obtained in only one patient. Six patients underwent valve replacement in the acute phase of endocarditis. After a mean follow-up period of 32 months (range 3 to 120 months), only one patient has symptoms (claudication of the left upper limb); 5 patients are asymptomatic with a reduced (n = 5) or abolished (n = 2) pulse. Three embolisms have left no sequelae. Altogether, AEL are not uncommon in infective endocarditis. They rarely influence the functional prognosis and are detected by systematic palpation of the pulses. Anticoagulation in effective doses is discussed. Attempts at removing the obstruction should be made only in cases with poorly tolerated proximal embolism. In patients with multiple or recurrent embolic accidents, valve replacement may be considered.

Adolescent

[Prostatic location as the manifestation of lymphoma].

The authors report a case of non-hodgkin's lymphoma initially presenting with pallakiuria due to a prostatic localization of the tumour. The diagnosis was confirmed 6 months later following deterioration in the general condition with lymphomatous meningitis, after repeat examination of the prostatic slides. Less than twenty cases of prostatic lesions revealing lymphoma have been reported in the literature. There are no formal diagnostic arguments apart from the histology which is often difficult to interpret. In most cases the lymphomas are disseminated and composed of large cells or cleaved small cells with a poor prognosis and a mean survival of 14 months. Treatment primarily consists of chemotherapy, as the lymphoma is always disseminated. The role of local radiotherapy is questionable.

Aged

Temporal arteritis revealed by upper limb gangrene.

An 80-year-old white man presented with gangrenous lesions involving several distal phalanges of his left hand and an elevated erythrocyte sedimentation rate (ESR). Temporal artery biopsy showed patchy destruction of the internal elastic lamina by mononuclear cell infiltration, consistent with the diagnosis of temporal arteritis. After amputation of gangrenous lesions, he was discharged taking prednisone (60 mg/day). Twelve months after discharge there was no recurrence of ischemic manifestations and ESR was normal. Association of digital gangrene and elevated ESR should alert the clinician toward this diagnosis once other diseases such as atherosclerosis, scleroderma, lupus erythematosus, periarteritis nodosa have been ruled out.

Aged

[Wegener's granulomatosis in elderly subjects. 37 cases].

The clinical, biochemical and prognostic characteristics of 37 patients with Wegener's granulomatosis were studied in relation to age. Eleven patients were older than 60 years when the first sign of the disease appeared. Except for the fact that the renal lesions seemed to be more severe in these patients, there was no significant clinical or biochemical difference between them and patients under 60. Five patients over 60 years of age died during the first 2 years of Wegener's granulomatosis. Death was due to infection in 3 cases, to acute renal failure as complication of intra-alveolar haemorrhage in 1 case and to metastatic cancer in 1 case. Age-related comparisons of survival rates showed that the probability of surviving was lower in elderly subjects: 78 per cent at 1 year and 39 per cent at 3 and 5 years, as opposed to 96 per cent at 1 and 3 years and 84 per cent at 5 years in younger subjects (P less than 0.01). This poor prognosis is probably due to the renal lesions which constitute an important cause of death in the published series. Rapidly progressive glomerulonephritis being amenable to an early and intensive treatment, elderly patients should be treated as energetically as younger patients.

Adrenal Cortex Hormones

[Coccygodynia disclosing Tarlov's cysts].

In a 63-year old male patient coccygodynia, initially isolated then complicated by incomplete cauda equina syndrome, could be attributed to large perineurel meningeal cysts on the sacral nerve roots. The diagnosis was suspected at computerized tomography and nuclear magnetic resonance and confirmed by sacculoradiculography. Intradural injections of corticosteroids provided lasting pain relief. Arachnoid cysts are often asymptomatic, by they may be responsible for coccygodynia and/or incomplete cauda equina syndrome. Their presence is suggested by the characteristics of the symptoms which are paroxysmal, exacerbated in standing position, relieved in dorsal position and revived by percussing the sacrum. Treatment is medical in most cases. The decision to operate depends on the persistence and intensity of pain and on whether signs of neurological defecit are present.

Arachnoid Cysts

[Arterial manifestations of Behçet's disease. 12 cases].

Out of 196 patients with Behçet's disease, 12 (10 men and 2 women, mean age 34 +/- 7 years) had non-coronary arterial lesions. Behçet's disease was complete in 4 patients. The arterial lesions had appeared 8.6 +/- 8 years on average (20 years at most) after the first sign of the disease. Three patients showed evidence of stenosis or occlusion involving one or several arteries. Eight patients had both stenotic and aneurysmal lesions. One patient had an arteriovenous fistula. Another developed a false aneurysm at the site of introduction of a femoral catheter. Yet another patient developed an anastomotic aneurysm one year after implantation of an abdominal aortic graft. In 2 cases histology showed fragmentation of the media associated with vasculitis of the vasa vasorum. Two patients with pulmonary aneurysm died of massive haemoptysis. In 2 patients combined corticosteroid and cyclophosphamide therapy failed to prevent the development of aneurysmal lesions. Phlebitis was associated with arterial involvement in 7 patients. Comparison between patients with or without arterial lesions showed no significant difference in time of onset of Behçet's disease, sex, main clinical features and presence of HLA B5. Aneurysmal lesions respond poorly to medical treatment, and surgery is mandatory. Since recurrence at the site of anastomosis is possible, prolonged monitoring is required.

Adult

[Heart conduction disorders in long-term treatment with chloroquine. Two new cases].

Cardiac complications are exceptional in long-term chloroquine therapy; congestive heart failure and restrictive cardiomyopathy may develop, but disorders of conduction are more frequent. The characteristics of these disorders emerge from 12 cases in the literature and from 2 personal cases. The usual disorder is fascicular block which may become a complete, syncopal, atrioventricular block, as in one of our 2 patients. The time elapsed between the beginning of treatment and the occurrence of these disorders (2 to 30 years) and the total dose of chloroquine received (100 to 2,500 g) are extremely variable. Retinopathy or neuromyopathy is present in 64 and 35 percent of the cases respectively. The diagnosis is confirmed by endomyocardial biopsy with electron microscopic study which shows vaculoar myopathy with numerous large secondary lysosomes containing a dense material of lamellar structure (myelinic figures, curvilinear bodies). Regression of heart conduction disorders after withdrawal of chloroquine seems to be inconstant and incomplete. The rare occurrence of this complication raises the question of genetic predisposition. We believe that chloroquine therapy should be contra-indicated in patients with a history of conduction disorders and that a 6-monthly electrocardiographic control of these patients would be justified.

Biopsy

[Evolutive and prognostic aspects of Wegener's granulomatosis].

We have followed 45 patients affected with Wegener's granulomatosis and conducted a retrospective analysis of its evolutive aspects and prognostic factors. The study concerned 25 men and 20 women, with an age-range of 50 +/- 15 years at the onset of the disease. The time between occurrence of the first manifestations and the diagnosis was 16 +/- 21 months, the follow-up was 6.3 +/- 8 years. All patients except 3 were given steroids, associated in 39 cases with an immunosuppressor and in 9 cases with plasma exchanges. In 2 localized forms in the ENT area, remission was possible with cotrimoxazole alone. 15 deaths occurred, the causes of which are infection and renal insufficiency when death occurs early, and cardiovascular disorders and neoplasms when death occurs late. The survival probability is 93 p. cent at one year, 83 p. cent at 3 years and 75 p. cent at 5 years from the time of the first manifestations and 75 p. cent at one year, 53 p. cent at 3 years and 50 p. cent at 5 years from the time of the diagnosis. Relapses have been reported in 11 patients, some of which occurred several years after discontinuation of the treatment: they emphasize the need for prolonged monitoring of these patients. Presence of one of the poor prognostic factors: age 50 years (p less than 0.05), presence of renal insufficiency (p less than 0.05) or hemoglobinemia less than or equal to 10 g/cc (p less than 0.05), should be an indication for a more intensive treatment of Wegener's granulomatosis.

Adult