Biomedical subjects
Publications and source records attributed to R Bouvier.
The authors report on the case of a child with the nephrotic syndrome complicated by thrombosis of superior longitudinal sinus, bilateral massive pulmonary embolism resulting in a sudden death. A dramatic deficiency in factor XII was demonstrated; the pathophysiology and management of such an abnormality are discussed.
A 17 day-old neonate with no history of broncho-pulmonary disease presented with a severe respiratory distress which was found to be related to a congenital tracheal diverticulum. An excision of the diverticulum was performed. Histologically it was characterized by a lining wall of stratified columnar ciliated epithelium and the presence of smooth muscle and cartilage in the wall. This rare entity was reported only once in the pediatric literature. It gives a history of chronic respiratory tract infections or acute respiratory distress. Bronchoscopy or bronchography and CT scan are necessary for the diagnosis. The treatment is surgical.
A review of renal tumors of childhood is presented with special emphasis on different patterns of nephroblastoma, the changes induced by preoperative chemotherapy and new related tumors. Their diagnosis and prognosis are discussed. A large place is given to the nephrogenic rests and nephroblastomatosis, in the light of the recent paper by Dr Beckwith.
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LFA-3, ICAM-1, HLA.ABC and HLA.DR expression was analyzed on 66 neuroblastoma specimens. HLA.ABC was expressed on 26 specimens, HLA.DR on 2, LFA-3 on 20 and ICAM-1 on 10. HLA.ABC and LFA-3 were positive on ganglioneuroblastoma or ganglioneuroma, but they were negative on neuroblastoma, independently of the clinical staging; HLA.ABC and LFA-3 were induced in vivo by chemotherapy in parallel with tumoral cell differentiation, in both the primary and the metastases. The expression of ICAM-1 was restricted to 5 of the 10 low-grade stage-1 or stage-2 specimens, 1 stage-3 specimen, and the primary tumors of 2 patients with stage-4 disease, analyzed hence at diagnosis and after chemotherapy (4 specimens); metastatic cells obtained in 1 of these patients were negative. HLA.ABC and LFA-3 expressed on both mycN-negative and -positive specimens, whereas ICAM-1 was restricted to MYCN-negative specimens. LFA-3 diffusely stained partially differentiated neuroblasts, Schwann cells and ganglion cells. The expression of HLA.ABC on differentiated neuroblasts varied from one sample to another and within the same tumor; Schwann cells were strongly positive, but ganglion cells were negative. In positive samples, ICAM-1 was expressed on differentiated neuroblasts and Schwann cells, but negative on ganglion cells; however, most of the differentiated tumors were ICAM-1-negative, suggesting ICAM-1 induction by unknown local signal. The 4 markers were negative on undifferentiated neuroblasts. The distribution of these 4 markers on clinical specimens was in agreement with their reactivity on fetal tissues, as well as with results obtained on neuroblastoma cell lines before and after in vitro treatment with IFN-gamma.
The localization of substance P (SP)-immunoreactive structures in the infant brainstem was investigated by immunohistochemistry using the peroxidase antiperoxidase technique. SP-immunoreactive structures are widely distributed throughout the brainstem region. SP-immunoreactive cell bodies are prominent in the superior colliculis, the central grey, the nucleus tractus solitarius and the reticular formation. A high density of SP-immunoreactive fibers is found in the nucleus tractus solitarius, the trigeminal nucleus and the dorsal horn of the spinal cord. Large SP-immunoreactive fibers are seen in the substantia nigra. In the present study, we also investigated the development of substance P-immunoreactive fibers in the infant brainstem during the first postnatal year. We note a qualitative increase in the density of SP-immunoreactivity in some brainstem regions such as colliculus superior and substantia nigra with respect to age.
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The use of leukocytes infiltrating bladder tumors as an internal standard for flow cytometric measurements of DNA and SG2M indices was investigated in comparison with the conventional use of chicken erythrocytes (chicken red blood cells; CRBC). The leukocytes were identified by indirect fluorescence, using a mouse anti-CD45 monoclonal antibody and a fluorescein isothiocyanate-conjugated goat anti-mouse Ig F(ab')2 fragment. DNA was stained with propidium iodide. The percentage of CD45-positive cells averaged 37% in 23 unimodal bladder tumors and 30% in 27 bimodal ones. The diploid peak of CD45-positive cells was always easily identified and permitted a more accurate calculation of the DNA index and the percentage of cells in SG2M phases than did the use of CRBC. Seven tumors that were classified as unimodal using CRBC as the standard did, in fact, contain a minor clone (with a DNA index of 1.80) that could be detected only when CD45-positive leukocytes were used as the internal standard.
Tissue lesions can be induced at the focal point of highly focused transducers with a frequency of 1 and 2.25 MHz for exposure times of less than 1 second. The energy generated by a high power amplifier (7.5 kilowatts effective at 1 MHz) is delivered in the form of series of impulses lasting between 10 and 1,000 milliseconds. The experimentation was conducted in the rat kidney (the left kidney, normally supplied by its vascular pedicle, was exteriorised during ultrasound treatment and then returned to the abdomen). The animal was sacrificed 3 days later and the lesions were studied by serial histological sections. 248 ultrasound shots were performed between January and September 1990. They allowed the definition of the time and intensity constants necessary to induce total destruction of the renal tissue at the focal point. Depending on the energy delivered, an elliptical cavity with a mean height of 1.2 to 4.6 mm and a mean diameter of 0.6 to 3 mm is observed at the focal point after a single shot. No cell structures were visible in the cavities and, in general, the cavity was prolonged by a cone-shaped region of coagulated necrosis with an inferior base. The mechanism responsible for this focused ultrasonic tissue destruction (FUTD) involves a variable combination of thermal and mechanical effects which depends on the ultrasound intensity delivered at the focal point of the transducer.
Human skeletal tissues fixed in Bouin's solution and embedded in paraffin were studied in order to identify cells responsible for production of types I and II collagens by in situ hybridization. The probes used were a double stranded cDNA fragment (type I collagen) and a synthetic oligonucleotide (type II collagen). The specificity of these probes labeled with 32P was proven in hybridizations to sections of human fetal femoral heads: fibroblasts and chondrocytes, known to produce respectively type I and type II collagen were only recognized by the corresponding probes. These results suggest that paraffin blocks available in pathology departments could be reexamined by in situ hybridization. Furthermore, the use of synthetic oligonucleotides without cloning the gene of interest could increase the usefulness of the technique in studies on human cartilage diseases and possibly on other diseases.
The authors report one case of Ollier's disease in a 12 year-old girl in whom the occurrence of an ascites revealed a secretant juvenile granulosa cell tumor some years later. The tumor was right-sided, homolateral to the hemicorporal side involved by enchondromatosis. Five other cases of this association were previously reported. Attention is drawn not only to the risk of chondrosarcomatous change but also to the possible recurrence of ovarian tumor in the year following ovariectomy.
Two siblings born from consanguineous parents experienced infantile nephrotic syndrome with ocular and neurological abnormalities; the boy also had a micropenis; both patients died before age 1 year. An initial renal biopsy followed by a two-step binephrectomy allowed good histological assessment of disease progression in one patient. The progression of the lesions was characterized by mesangial involvement, then an extensive extracapillary proliferation and tubular dilatations with a high mitotic activity of the epithelium and nuclei of unequal size. The main features involved major ultrastructural changes of the glomerular basement membrane. These two patients may represent a new disease entity or a severe form of diffuse mesangial sclerosis, with autosomal recessive inheritance.
A woman-aged 44-had corneal dystrophy anterior to Descemet's membrane. This corneal abnormality resembles Cornea Farinata with tiny, punctate, grey opacities localized in the entire thickness of the corneal stroma. We used light and electron microscopic to study this case. The corneal opacities were due to abnormal stromal keratocyte enlarged by intra-cytoplasmic vacuole containing lipid-like inclusions.
Isolated dissection of renal artery is uncommon; 150 cases were reported in the literature, whose 2/3 diagnosed during patient's life and 1/3 post mortem, during autopsy. From 1976 to 1988 4 cases of isolated dissection of the renal artery were diagnosed and treated in our Institution. Three men and one woman with age ranging from 39 to 46 yrs (mean age 41.75) were seen at our Institution for intense abdominal pain and blood hypertension (mean blood pressure 196.25/113.75 mmHg) of recent occurrence. In 2 cases angiography showed dissection of the left renal artery, with involvement of peripheral branches; in 1 case the dissection involved the right renal artery with complete occlusion of an upper pole branch and upper pole infarction; our youngest patients presented a bilateral dissection, limited to the main trunk on the right side and involving prepelvic and retropelvic branches on the left side. Surgical treatment consisted of renal autotransplantation in the iliac fossa after extracorporeal reconstruction of the arterial pedicule; a branched hypogastric graft was used in 3 patients; in the patient with bilateral dissection an hypogastric graft was used on the right side and a sapehenous graft on the left side (3 months later). Partial nephrectomy was also performed in the patient having right upper pole infarction. Histologic examination showed that dissection always started from lesions of the lamina media. All patient were alive from 6 to 48 months after renal revascularization, with normalization of blood pressure (mean BP 125/80 mmHg); postoperative angiographic control showed thrombosis of 2 small branches in 1 case and partial stenosis of 3 anastomoses in another patient.(ABSTRACT TRUNCATED AT 250 WORDS)
A hybridoma cell line secreting an IgM monoclonal antibody (MAb) was produced after immunizing a mouse with RT4 cells and a crude suspension of human bladder carcinoma cells (WHO grades II and III TCC). This MAb reacted with RT4 target cells derived from a human transitional bladder cancer but failed to react with a majority of non-bladder cancer cell lines. Immunohistological studies indicate that this MAb reacts inconstantly with normal bladder: in positive cases only a few superficial cells (5% to 10% umbrella cells) are stained but not intermediate or basal cells of the urothelium. This MAb was evaluated on 118 tumors: it reacted with tumor tissue in a majority of grade I (79.5%) and grade II papillary TCC (77.3%), less with grade III papillary TCC (45%) and very rarely with invasive non-papillary TCC (14%). In cases of flat lesions a strong reactivity of superficial, intermediate and/or basal layer cells was observed in 50% of moderate and severe dysplasia and in all cell layers of carcinomas in situ (CIS)(9/9).
Phenotyping of 76 bladder tumors (11 grade I, 33 grade II and 32 grade III) has been carried out by flow cytometry on cell suspensions with simultaneous determination of DNA content and surface immunofluorescence using G4 and 5 new monoclonal antibodies (10D1, 7C12, 6D1, 3C6 and 12F6) directed against bladder tumor cells. Ten normal bladder samples were used as control. Antibodies 6D1 and 12F6 were specific for tumor cells whereas the others also labelled umbrella cells. Cells from grade I tumors were labelled with 10D1, 6D1, 7C12 and 12F6 antibodies, and cells of grade II tumors with 7C12 and to a lesser degree with 12F6 but not with 10D1 and 6D1. Grade III tumor cells were specifically labelled with antibodies 3C6 and G4. Reactivity of antibodies with tissue sections was well correlated with cytometry results, except for the antibody 3C6. Finally, most of the cells stained by 3C6 and G4 were shown to have a DNA index greater than 1.0. In conclusion cells of low grade tumors can be identified with 10D1 and 6D1 antibodies, and antigens recognized by 3C6 and G4 antibodies are mostly expressed by aneuploid cells.
Southern blot analysis of neuroblastoma (NB) cell DNA from 52 patients (58 samples) allowed the detection of an N-myc amplification on three of the 13 BM samples and three of 13 tumor samples at diagnosis, on two of 17 tumor samples taken after induction therapy, on three of seven BM samples and two of the five local tumor samples taken after relapse. N-myc was amplified in two of the 15 patients with stage I to III NB and in 10 of the 36 patients with stage IV neuroblastoma over 1 year of age. Conclusions from the analysis are as follows: first tumor samples obtained on previously treated patients are histologically modified and detection of N-myc amplification is not accurate; consequently N-myc amplification must be defined at diagnosis. Surgical biopsies of the primary tumors can, however, be delayed and malignant cells obtained by ultrasound-guided punctures or analyzed on bone marrow samples if they represent more than 50% of the total population. Second, any attempt to define the prognostic value must be performed on a group of patients treated with a new aggressive protocol of chemotherapy; it will require a multiparametric analysis including, in particular, results of an extensive clinical staging at diagnosis and histological criteria.
Cytokeratin 18 expression in transitional cells of 76 bladder tumors (11 grade 1, 33 grade 2 and 32 grade 3) and 10 normal biopsies was quantified by flow cytometry after immunolabeling with the anti-cytokeratin 18 monoclonal antibody RGE 53. Combined cytometric analysis of deoxyribonucleic acid content and cytokeratin 18 expression was conducted. Of 76 tumor biopsies 38 had a unimodal deoxyribonucleic acid profile with a deoxyribonucleic acid index close to 1.0, while the other 38 showed a bimodal profile: 1 peak with an index of 1.0 and a second peak with an index of greater than 1.0. The 11 grade 1 tumors belonged to the first group, whereas 10 of the 33 grade 2 and 28 of the 32 grade 3 tumors belonged to the second group. Antibody RGE 53 reacted with 4 per cent of the normal bladder cells (umbrella cells), 14 +/- 3 per cent of the cells from grade 1, 33 +/- 8 per cent from grade 2 and 56 +/- 10 per cent from grade 3 tumors. Analysis of cytokeratin 18 expression according to deoxyribonucleic acid content at the single cell level in tumors with a bimodal deoxyribonucleic acid profile showed that cytokeratin 18 was detected frequently in cells with a high deoxyribonucleic acid index but much less so in cells with an index of 1.0. Therefore, expression of cytokeratin 18 can be recognized as a marker of aggressiveness in bladder tumors, since it increases in parallel with tumor grade and cell deoxyribonucleic acid content.