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Biomedical subjects

G Chomette

Publications and source records attributed to G Chomette.

At least 379 records · Page 21Linked to original sources

Massive blastic infiltration of the liver: a cause of fulminant hepatic failure.

The clinical and pathological findings in four cases of fulminant hepatic failure due to massive infiltration of the liver by acute leukemia or lymphoma are reported. Liver abnormalities were found simultaneously with or led to the discovery of hematologic malignancies, and consisted of marked hepatomegaly and severe hepatocellular insufficiency associated with hyperlactatemia. The blood malignancies were peculiar in their fast cellular growth and large tumor mass. Evolution was rapidly fatal in all these cases. In another patient, marked hepatomegaly and hyperlactatemia revealed the presence of a widespread lymphoma before the appearance of hepatocellular insufficiency. Immediate chemotherapy was instituted, and complete remission without hepatic complication was obtained. It is suggested that malignant hematological diseases with fast cellular growth may present as fulminant hepatic failure. In order to avoid a rapidly fatal outcome secondary to liver failure and metabolic disorders, early recognition of these malignancies is necessary so as to assure prompt administration of appropriate chemotherapy.

Autopsy↗

[Buccal lymphomatoid granulomatosis].

A 57 year old man was referred to us with multiple long-standing, recurring and refractory mouth ulcers involving the palate, the intermaxillary folds, the inner side of the cheeks, the frenum of the tongue, a tonsil, the pharynx, the gums and a lower lip. Multiple biopsies had excluded a lymphoma, a carcinoma and infection. A new biopsy showed a polymorphous granuloma with up to 40% of atypical lymphoid cells. No angiitis was present, but an angiocentric pattern was present in the granuloma. The atypical cells stained positively with OKT3 and OKT4 antisera. The patient also had cardiac rhythm disturbances with defects on the myocardial scan and a proteinuria. Thus, we suspected the diagnosis of lymphomatoid granulomatosis, although there were no pulmonary nodules. Cyclophosphamide and prednisone were given with immediate success. As the doses were gradually decreased, a small relapse occurred. It was controlled by increasing the cyclophosphamide dosage for six months. The patient is now in complete remission more than four years after having discontinued the treatment. Thus, lymphomatoid granulomatosis can be diagnosed on the basis of a biopsy of buccal ulcers and in the absence of the classical pulmonary nodules. We found no evidence that the atypical cells were lymphomatous; rather, the very good response to the treatment points to a curable granulomatosis.

Cyclophosphamide↗

Behcet's syndrome and renal involvement: a histological and immunofluorescent study of eleven renal biopsies.

The finding of focal glomerulonephritis in a patient with Behcet's syndrome led us to perform systematic renal biopsies in ten other patients with the disease. None of the patients had symptoms of renal disease. Proteinuria was found in five, two of whom had associated leukocyturia. By light microscopy mesangial and extramembranous glomerular deposits were observed in eight patients. Arterioles in ten patients showed subendothelial and medial hyaline deposits. A granular pattern of fluorescent staining identified the presence of the third component of complement in these deposits. Circulating immune deposits were sought and found in six out of seven patients. The finding of circulating immune complexes and deposition of complement in glomerular and arteriolar tissues supports an immune complex mediated nephropathy and is consistent with the hypothesis of an immunological pathogenesis in Behcet's syndrome.

Adult↗

[Experimental autograft of the small intestine in the pig. Histological, histoenzymological and ultrastructural study].

In order to form an opinion of the value of certain precocious rejection tests with a view to ulterior intestinal allograftings, the authors intend first of all to determine the consequences of the operation itself and of the possibility resulting anoxia on the function of the intestinal mucosa. A concomitant biological (xylose test meal, quantitative analysis of the enzymes of the mucosa) and morphological study (lipid test meal, histologic, enzymologic and ultrastructural study of biopsic specimens) was carried out at various intervals after orthotopical intestinal autografting in 22 pigs. Most of the biological tests were strongly perturbated on the 7th day and some of them did not resume their initial value until the 15th day. The morphological and especially ultrastructural studies showed up to that time an important inflammation apparently caused not by the anoxia but by the lymph stasis. Solely conventional histology was only sightly modified (absence of ulcerations and of denudation of the villi) and this relative integrity, contrasting with the known severity of the lesions in case of precocious rejection, allows to consider this examination the only valuable until the 15th day, for the detection of immunologic incompatility reactions in allograftings.

Animals↗

[Myocardium in valvular cardiopathies. Ultrastructural and histoenzymological changes. Functional correlations].

The morphologic and hemodynamic datas are compared in 24 patients with mitroaortic valvular cardiopathy. The morphologic study was carried out on left ventricular biopsies and consisted first of all in ultrastructural analysis. Thus, 5 degrees of alterations were determined with regard to myofibrillar and, accessorily, to mitochondrial changes. The microscopic data, especially rarefaction of the Z material on semifine sections stained with Toluidine Blue and the demonstration of phospholipids by staining with acid hematein, are consistent with the ultrastructural gradient. Also, in cases with the most advanced electronic gradient, histoenzymology revealed important anomalies in the activity of the myofibrillar ATPase and of the S.D.H. The correlation between the so-established morphologic gradient and the preoperative hemodynamic data proved satisfying in the majority of cases which points out the interest of the morphologic tests for the appreciation of the functional value of the myocardium.

Adenosine Triphosphatases↗

[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↗

[Ultrastructural study of 6 cases of ameloblastoma].

Three cases of simple ameloblastoma were studied by electron microscopy. The classical ultrastructural features of these tumors are analysed and compared with reports in the literature and the development of the enamel organ. The authors stress the presence of two cell types (light and dark) probably corresponding to different degrees of maturity. One case of cystic ameloblastoma and one of granular cell ameloblastoma are then reported. The nature of the inclusions in this latter tumour is probably mixed: lysomal and secretory, perhaps ameloblastic. Finally, a malignant ameloblastoma is described under electron microscopy, for the first time. Closely related to simple ameloblastomas, it may be distinguished only by the presence of signs of cellular degeneration and numerous mitoses, sometimes abnormal.

Adult↗

Proteoglycans detected by monoclonal antibodies in adenoid cystic carcinoma of salivary glands.

Thirty-two cases of adenoid cystic carcinoma (ACC) of the salivary glands were examined immunohistochemically by monoclonal antibodies to proteoglycans (PG) such as chondroitin 6 sulfate (C6SPG), chondrotin 4 sulfate (C4SPG), dermatan sulfate (DSPG), heparan sulfate (HSPG) and keratan sulfate (KSPG) in conjunction with specific enzymatic digestion. The cribriform structure of ACC consisted of basaloid, myoepithelium-derived, and luminal tumor cells. The myoepithelial tumor cells were positive for PG, whereas luminal tumor cells were unstained. Occasional pseudocysts also gave positive staining for PG. Tubular structures consisting of modified myoepithelial cells indicated a high intensity reaction for C6SPG and C4SPG, and a slight one for DSPG, HSPG, and KSPG. Immunodepositions in solid and cluster structures were comparatively light in terms of PG. Basement membrane in ACC stained strongly for C4SPG, slightly for C6SPG, and very slightly for DSPG, HSPG, and KSPG. In ACC, immunohistochemical staining of PG was regularly positive in myoepithelium-derived tumor cells, but was irregular in other types of tumor cells.

Antibodies, Monoclonal↗

Chronic rejection in human heart transplantation.

The more prevalent complication in patients with a long survival rate after heart transplantation is chronic rejection, which was studied in a series of 80 necropsies and five cardiac grafts surgically removed for retransplantation after chronic rejection. In the material obtained at necropsy, 11 of 14 patients with a survival rate of more than 6 months died from chronic rejection. Clinically, the usual manifestation was heart failure. Anatomic angiograms were performed in several cases. They demonstrated narrowing and nonopacification of small coronary arteries, often accompanied by thrombosis and ischemic complications. The histologic study detected three types of rejection. (1) The more typical rejection is observed after 6 months. It is characterized by a stenosing fibrous endarteritis. (2) Another type of rejection occurs earlier and is associated with acute rejection; its anatomic substratum is an inflammatory panarteritis. (3) This type of rejection is accompanied by large atheromatous deposits. The significance and pathogenesis of these lesions are discussed in correlation with their clinical context and with the electron microscopic observations.

Adult↗

[Ventricular dysplasia. Nosology and sudden death].

The arrhythmia generating dysplasia of the right ventricle is a congenital anomaly suggesting a hereditary substratum. It usually manifests itself as follows: a young patient, with recurrent episodes of ventricular tachycardiac. There is no "pathognomonic sign" of dysplasia. The latter may only be determined by anatomical examination of the heart, macroscopically as well as microscopically. The most difficult differential diagnosis concerns minor or localized forms of dilated idiopathic myocardiopathy or sequelae of myocarditis with ventricular tachycardia originating in the right ventricle. It seems, currently, more appropriate to talk about "arrhythmia generating syndrome of the right ventricle" rather than arrhythmia generating dysplasia of the right ventricle, in these particular cases. The arrhythmia generating syndrome of the right ventricle could cause a sudden death in these young patients, especially during a violent physical exercise or practise of sports. The current nosological classification is based on an experience acquired by comparing a few isolated cases. The search for late potentials by summation-mean measurements and nuclear magnetic resonance could provide a new diagnostic approach.

Death, Sudden↗

Morphology of cyclosporine nephrotoxicity in human heart transplant recipients.

The morphologic findings of renal biopsies (four cases studied by electron microscopy) and renal specimens obtained from 44 autopsies of patients who died after heart transplantation are reviewed. Eight patients treated postoperatively with cyclosporine (serum concentration of 100 to 150 ng/ml), with a survival rate of 1 to 24 months, constantly had renal lesions of variable severity. Correlated with functional renal disturbances, these changes predominantly affect the proximal convoluted tubule: vacuolization, calcifications, and tubular atrophy. The interstitial fibrosis, increasing parallel to tubular alterations, occurs only after 3 months after transplantation. The hypertrophy of juxtaglomerular apparatus and the arteriolar sclerosis noted on and after the fourth month were associated with arterial hypertension. Among the other inconstant and mild lesions (attrition of vascular endothelium and arteriolar or glomerular thrombi), the partial sclerosis of glomerular flocculus, probably correlated with tubular lesions, seems more significant. Thus these findings corroborate the nephrotoxicity of cyclosporine (principally against the proximal convoluted tube), the increase of lesions with time, and the possible irreversibility of the lesions despite appropriately decreasing the dosage of cyclosporine to eliminate toxic side effects.

Acute Kidney Injury↗

[Neonatal primary hyperparathyroidism caused by clear cell hyperplasia].

Neonatal primary hyperparathyroidism is rare but must be evoke during respiratory distress with thoracic deformity and hypercalcemia. The plasma immunoreactive parathyroid hormone level allows the diagnostic. This case, with diffuse hyperplasia of water-clear cells type, develops rickets of vitamin D deficiency before surgery. A large parathyroidectomy (7/8) was performed and the child is normo-calcemic 2 years after. The time of surgery is function of calcium level.

Female↗