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Biomedical subjects

B Gosselin

Publications and source records attributed to B Gosselin.

At least 19 recordsLinked to original sources

[Transplantation for primary hyperoxaluria. Role of oxalate crystal deposits in the occurrence of kidney failure].

We analyzed the records of 3 patients transplanted for end-stage renal failure due to primary hyperoxaluria and evaluated on repeat biopsies the role played by oxalate deposits in the constitution of renal failure after isolated kidney graft, or combined liver and kidney transplantation. Early failure of the renal graft is frequent and often interpreted as the consequence of recurrence because of the presence of oxalate deposits on the graft biopsy. In fact, the decrease in oxalate deposits observed in our 2 cases of combined liver and kidney transplantation despite the progressive renal failure, indicates that crystal deposition is not responsible for the renal lesions. However, we cannot exclude that the oxalate molecule toxicity plays a role in the constitution of the diffuse sclerosis which occurred in these two cases after a primary renal non function, aggravating a hemodynamic process by using cyclosporin. On the other hand, as observed in our isolated kidney graft, renal crystal deposition occurring before the onset of renal failure suggests the true mechanism explaining the slow recurrence of renal oxalosis.

Adult

Immunopathological study of eosinophils in eosinophilic granuloma of bone: evidence for release of three cationic proteins and subsequent uptake in macrophages.

Eosinophils from two patients with eosinophilic granuloma of bone (EGB) were studied by combined immunohistochemical and immuno-ultrastructural methods with antibodies directed against three eosinophil granule proteins: major basic protein, eosinophil cationic protein, and eosinophil peroxidase. Immunohistostaining showed the presence and distribution of large numbers of eosinophils in the granuloma. Immuno-ultrastructural methods showed alterations of eosinophil fine structure associated with some steps in the release of granule proteins. No granule extrusion was seen, but rather cationic proteins diffused within cytoplasmic tubulo-vesicular structures. Furthermore, the three granule proteins were found within phagolysosomes of surrounding macrophages, suggesting an interaction between eosinophils and phagocytic cells at the destructive stage of EGB.

Adolescent

Prognosis and treatment of lymphoblastic lymphoma in adults: a report on 80 patients.

PURPOSE: We analyzed prognostic factors in 80 adult patients with lymphoblastic lymphoma (LBL). PATIENTS AND METHODS: Twenty-one patients received six monthly courses of cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) and maintenance chemotherapy for 12 months. The LNH-84 protocol (30 patients) consisted of four courses of high-dose CHOP followed by consolidation for 4 months. Both FRALLE (22 patients) and LALA (seven patients) protocols were two intensive chemotherapy regimens for acute lymphoblastic leukemia (ALL) that included an induction with daunorubicin, vincristine, cyclophosphamide, prednisolone (and asparaginase for the FRALLE regimen), consolidation, and maintenance chemotherapy that lasted for 2 years. RESULTS: Sixty-six patients (82%) achieved a complete remission (CR). The CR duration rate and overall survival rate at 30 months were estimated to be 46% and 51%, respectively, with a median follow-up of 55 months. Only two of 37 relapses occurred after 26 months. Chemotherapy protocol did not influence CR rate, CR duration, and survival. A higher CR rate was associated with an age of less than 40 years, lactic dehydrogenase (LDH) level of less than two times the upper limits of normal, and no or one extranodal site of disease. Short survival was associated with a failure to achieve CR, age older than 40 years, B symptoms, LDH level more than two times the upper limits of normal, and hemoglobin level of less than 100 g/L. Bone marrow involvement had no prognostic value. We could not evaluate precisely the prognostic value of Ann Arbor stage because inclusion criteria differed among treatment groups. CONCLUSION: Our findings suggest that age and LDH are two important pretreatment prognostic factors for adult LBL, and that the optimal prognostic staging system remains a controversial issue.

Adolescent

[Primary Aspergillus endocarditis. Apropos of a case and review of the international literature].

The authors report a case of primary aspergillus endocarditis with endophthalmitis and vertebral osteomyelitis. No underlying disease and no predisposing factors were found. Valve replacement plus combined antifungal chemotherapy proved to be effective as the patient is asymptomatic 18 months after the first symptoms. 48 cases of aspergillus endocarditis, without prior cardiac surgery have been reported in the literature. Aspergillus endocarditis was valvular or mural. Extracardiac dissemination was common but endophthalmitis and osteomyelitis were infrequent. In 11 cases, the diagnosis was made by histologic examination of embolectomy or ocular, skin biopsy tissue. All patients were febrile. Blood cultures showed no Aspergillus species. Clinical manifestations of endocarditis were described in less than fifty per cent of cases. Echocardiographic visualization of vegetations was obtained in 5 cases. Many patients experienced embolic phenomena. Mortality from Aspergillus endocarditis is extremely high (96%). Surgery is the main treatment, consisting of valve replacement. Antifungal chemotherapy should be combined. The proper duration and dosage and the combination of antifungal drugs have not been clearly defined.

Amphotericin B

c-ets1 proto-oncogene is a transcription factor expressed in endothelial cells during tumor vascularization and other forms of angiogenesis in humans.

The c-ets1 proteins are transcriptional activators expressed within endothelial cells during blood vessel development in chick embryos. The authors show by in situ hybridization that c-ets1 is transcribed in the endothelia during angiogenesis in human embryos, in granulation tissue, and especially during tumor vascularization. c-ets1 mRNAs were also detected in the fibrocytes of tumor stroma and in the spindle cells of Kaposi's sarcomas, regarded as cells of endothelial origin. It has been shown that the c-ets proteins activate transcription through a PEA3 motif that plays a role in the stimulation of transcription of urokinase-type plasminogen-activator (u-PA), stromelysin and collagenase genes. The authors demonstrate in vitro that the angiogenic factor TNF alpha increases transiently the amount of both c-ets1 and u-PA mRNA in confluent human umbilical vein endothelial cells. Therefore, the authors suggest that the c-ets1 proteins might regulate the transcription of the genes coding for matrix-degrading proteases, which are necessary for both angiogenesis and tumor invasion.

Adenocarcinoma

[The multifocal form of bronchocentric granulomatosis].

Bronchocentric granulomatosis is a rare entity and has only recently been characterised. We report a case of a forty-three year old man presenting with multifocal bronchocentric granulomatosis which was revealed following some oto-rhino-laryngological and bronchopulmonary symptoms. The open lung biopsy confirmed the diagnosis. Treatment was by bolus doses of corticosteroids (30 mg/kg) and of methotrexate in low dosage (7.5 mg/week, then 15 mg/week) which checked the initially unfavourable outcome.

Adrenal Cortex Hormones

Different patterns of spleen involvement in systemic and malignant mastocytosis. A histological and immunohistochemical study of three cases.

Three cases of splenic involvement in three different types of generalized mastocytosis (systemic mast cell disease) are reported. The macroscopic, histological and ultrastructural modifications of the spleen are described. Each case exhibited a different morphological pattern. Giemsa staining, fluorescence after acridine orange staining and naphthol ASD chloracetate esterase reaction are shown to be valuable for diagnosis. By comparison, immunohistochemistry seemed not to be very useful, because no specific antigens are expressed. These findings are compared to previously published cases. Their value for the diagnosis and the prognosis are discussed.

Adult

Cold blood cardioplegia and warm cardioplegic reperfusion in heart transplantation.

The major cause of early death after heart transplantation is graft failure. In 99 consecutive heart transplantations two protocols of myocardial protection were employed. In group 1 (n = 38) initial cold crystalloid cardioplegia combined with cold saline storage and peroperative surface cooling was used. In group 2 (n = 61) cold crystalloid cardioplegia was injected initially and cold blood cardioplegia (Buckberg) was infused every 30 min as soon as the graft arrived in the operating room. No surface cooling was used. Warm blood cardioplegic reperfusion was administered before removal of the aortic clamp. There were 8 early (within 30 days) deaths in group 1 and 6 in group 2 patients. In group 1 there were 5 cardiac deaths against 3 in group 2. Mean ischemic time was 153 +/- 37 min in group 1 and 158 +/- 51 min (p greater than 0.05) in group 2. The post-transplantation need for catecholamines was ten times higher in group 1 patients than in group 2. The first endomyocardial biopsy (after 1 week) showed cytologic lesions compatible with ischemia in 40% of group 1 and only 9% in group 2 patients. We conclude from this initial experience that intermittent cold blood cardioplegia and warm blood cardioplegic reperfusion are useful in heart transplantation in restoring the damage suffered by the graft during brain death and graft storage.

Adult

Metastatic prostatic adenocarcinoma-diagnosed by bronchoalveolar lavage and tumour marker determination.

We report a patient who presented with a diffuse interstitial lung disease in whom clinical and radiologic investigation led to the suspicion of lymphangitis carcinomatosa. Bronchoalveolar lavage (BAL) was performed and revealed the presence of prostatic specific antigen (PSA) positive cells. A prostatic needle aspiration confirmed the diagnosis of adenocarcinoma. This case demonstrates the value of tumour marker determination in BAL.

Adenocarcinoma

[Auricular chondritis, malignant glomerulonephritis and pulmonary hemorrhage].

Relapsing polychondritis is a rare connective tissue disorder characterized by inflammation of cartilagenous structures such as ears, nose, joints, trachea and larynx. Renal disease is unusual. A 41 years old man presented with auricular chondritis, subacute renal failure and lung hemorrhage. A renal biopsy disclosed a diffuse proliferative glomerulonephritis with extensive crescents. Immunofluorescent studies suggested a GNRP type III so called "non immunologic disease". He was treated by prednisone and plasmapheresis with rapid disappearance of pulmonary hemorrhages and good renal functional improvement.

Adult

[Leiomyosarcoma of the superior vena cava. Case report].

Leiomyosarcoma of the blood vessels is a rare malignant tumor, and its localization in the superior vena cava is exceptional. We had the opportunity to study such a case and we compared our clinical, histological and ultrastructural results with those of 5 other reported cases.

Aged

[Mediastinal adenopathy disclosing Castleman's disease. Apropos of a case].

Castleman's disease or pseudo-tumoral lymphoid hyperplasia is a rare entity. We report a case of Castleman's tumour in the antero-superior mediastinum which was revealed by a radiological examination in a young woman of 26. We also report immunohistochemical studies. There are two clinical presentations of Castleman's disease; a localised form (mediastinal or extrathoracic) and the multicentric variety. The localised form may be asymptomatic or can be accompanied by general features of inflammatory disease. Surgical excision of these localised forms both confirms the diagnosis and assures a cure. The multicentric variety has various clinical peculiarities (the patients are older and/or immunosuppressed and suffering from multiple enlarged glands also in the viscera) and the disease in above all progressive. There are associations with Kaposi sarcoma and lymphoproliferative complications are described in this multicentric form and their prognosis remains poor.

Adult

[Strictly epidermotropic "Ketron Goodman"-type lymphoma. Immunohistochemical and ultrastructural analysis of a case].

We report a case of T cell lymphoma which was exclusively located within the epidermis. The T cells of this lymphoma were all of suppressor-cytotoxic phenotype. They did not express the CD7 antigen and a molecular biology study showed T cell type genotypic rearrangements. Both immunohistochemical and ultrastructural studies showed close contacts between lymphoma and Langerhans cells.

Aged

Congenital cystic adenomatoid malformation of the lung.

Twenty cases of cystic adenomatoid malformation of the lung were observed: 2 had died in utero; the diagnosis was made at birth in 13 infants of which 3 were premature. A Bochdalek's hernia had been diagnosed before birth in 2 cases by echo-tomography. The correct antenatal diagnosis had been made in 2 cases. Two infants had no symptoms, 3 were dyspneic, 8 were in respiratory distress and had to be intubated and ventilated. Two had a prune belly syndrome. Eight infants had a thoracotomy during their first week of life. Pulmonary resections concerning those 13 patients comprised 10 lower lobectomies, 1 of which was associated with a lingulectomy, and 2 upper lobectomies, 1 of which was associated with a middle lobe resection. Five patients were diagnosed and operated upon between 10 months and 8 years of age; 4 had recurrent bronchitis and 1 was diagnosed during the treatment of a gastroenteritis. They had 3 lower and 2 upper lobectomies. Recovery was uneventful in all patients except for 1 who was reoperated upon for intestinal obstruction. Antenatal diagnosis of cystic adenomatoid malformation should become standard. The malformation may be mistaken for a pulmonary sequestration or bronchogenic cyst. Differential diagnosis of a congenital hernia is important.

Child

Successful autologous bone marrow transplantation in second remission of malignant histiocytosis.

This report describes an 18-year-old man with disseminated malignant histiocytosis (MH). The patient initially attained complete remission (CR1) with conventional chemotherapy and then relapsed 14 months later. In second complete remission (CR2) 2 years and 8 months after initial diagnosis, an autologous bone marrow transplantation (ABMT) was undertaken following conditioning with the BEAM regimen. Bone marrow collected in CR2 was incubated with mafosfamide at a dose adjusted to the individual sensitivity of normal CFU-GM according to our current protocol. At the time of writing, 4 years post-transplant, this patient remains disease free. This is the first report of ABMT with marrow treated in vitro by mafosfamide in MH.

Adolescent

[Alveolar proteinosis in a facade plasterer. A sequential study of bronchoalveolar lavage and thoracic computerized tomography].

Alveolar proteinosis is a rare disease. We report a case occurring in a patient who smoked and was a facade plasterer who was treated with repeated pulmonary lavage. A mineral analysis of the lavage fluid recovered a significant amount of silica and iron oxide confirming the massive exposure of the patient to these minerals. The favourable outcome over the next 7 years was associated as regards respiratory function, with a progressive improvement in lung volumes and pulmonary output and of CO diffusion capacity, as regards broncho-alveolar lavage there was a regular increase in the cellularity and finally as regards CT scanning (at high resolution) there was a progressive clearing of the alveolar opacities only leaving the persistent opacities in the interstitium.

Adult

[The lung in brain death. Anatomo-biological correlations for pulmonary transplantation].

Histopathological studies of the lung conducted during multiple organ procurement from 25 subjects in a state of brain death showed that 19 of them had lung lesions pre-existent to coma, viz. alveolitis and lung invasion by dusts increasing with the subject's age. Alveolar or interstitial emphysema and subpleural bullae caused by mechanical ventilation were present in 19 cases; signs of bronchial inertia were found in 6 cases and established superinfection in 3 cases. Finally, 17 subjects showed interstitial and vascular alterations induced by oedema and shock which were related to the time spent in intensive care units, to the cause of the coma and to the quantity and quality of intravenous received while in intensive care unit. There were histological correlations between these various factors, but precise criteria concerning the functional prognosis of the transplant could not be defined. However, this study seemed to confirm the need for a new approach to the intensive care of brain-dead subjects during lung procurement: the time elapsed before procurement should be shortened, the amount of medicinal solutions administered should be reduced, Ringer lactate and packed cells should be used in preference to albumin derivatives, and arterial blood pressure, rather than diuresis, should be maintained.

Adult