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S Casanova

Publications and source records attributed to S Casanova.

At least 37 records · Page 2Linked to original sources

Phaeochromocytoma in multiple endocrine neoplasia type 2 A: survey of 100 cases.

OBJECTIVE: We report clinical, biochemical, morphological and histological data of phaeochromocytoma in 40 French families and in apparently sporadic cases of multiple endocrine neoplasia (MEN) type 2 A (medullary thyroid carcinoma, phaeochromocytoma, with or without hyperparathyroidism). DESIGN: This retrospective study was obtained from cases registered by the 'Groupe d'Etudes des Tumeurs à Calcitonine' from 1968 to 1990. We analysed the cases having sufficiently precise data on phaeochromocytoma with Pigas Software. PATIENTS: Characteristics of phaeochromocytoma in 100 patients with MEN 2 A were reviewed. Phaeochromocytoma was bilateral in 51%. The disease was inherited in 94 patients from 40 families (40 probands, 54 relatives), and was apparently sporadic in six. RESULTS: In this series, diagnostic circumstances were highly suggestive of phaeochromocytoma in 39.8% of the cases, whereas in 43.2%, diagnosis was made through systematic investigations of patients, either before (27.3%) or after (13.6%) thyroidectomy, or after discovery of hyperparathyroidism (2.3%). Fifteen per cent of patients were detected by family screening. Sudden death occurred in 8.9%, malignant phaeochromocytoma in 3%, and ectopic tissue in 4% of the cases. Urinary metanephrines appeared to be the most sensitive screening test. The extent of clinical symptoms was not associated with a particular hormonal pattern. Bilateral adrenalectomy was performed in 60% (in one step in 50%, in two steps in 10%). In these patients, bilateral histological lesions were observed in 92.5%. Simultaneous diagnosis for adrenal and thyroid disease was made in 73.4%, but phaeochromocytoma may be diagnosed before (9.6%) or after (17%) medullary thyroid carcinoma, with an interval greater than 2 years in 25 cases. CONCLUSION: Owing to variable clinical symptoms of phaeochromocytoma in these 100 cases of MEN 2 A, systemic biological adrenal assay is required. The search for phaeochromocytoma in medullary thyroid carcinoma (and vice versa) has to be systematically performed, even in apparently sporadic cases. Screening for phaeochromocytoma must be repeated for years, owing to the frequency of bilateral adrenal disease.

Adrenal Gland Neoplasms↗

Immunohistochemical distinction between amyloidosis and fibrillar glomerulopathy.

Six patients with glomerulonephritis and glomerular proteinaceous deposits constituted by fibrillar ultrastructures similar to those of amyloid but lacking the Congo red tinctorial affinity characterizing amyloid were studied. Clinically, these patients had proteinuria and hematuria; in addition, three patients had hypertension and one renal failure. Protein deposits in their kidney biopsy sections were evaluated by immunofluorescence, immunoperoxidase, and immunoelectron microscopic (protein A-gold) techniques, using antibodies against IgG, IgA, IgM, C3, C1q, fibrinogen, immunoglobulin kappa and lambda light chains, and against amyloid fibril proteins of different types, including AA, A lambda, A kappa, and AF. By immunofluorescence and immunoperoxidase, in all cases the deposits stained intensely with antibodies against IgG, C3, and kappa and lambda light chains; one case also showed C1q immunoreactivity. By contrast, none stained with antibodies against various amyloid fibril proteins. Immunoelectron microscopic findings corroborated this data, indicating that the nonamyloid fibrillar deposits studied are antigenically distinct from known amyloid deposits and that they contain IgG-derived material.

Amyloid↗

Bile acid active and passive ileal transport in the rabbit: effect of luminal stirring.

The intestinal absorption of bile acids (BA) with different chemical structure has been evaluated in the rabbit, after intestinal infusion of different concentrations (0.25-30 mM) of BA, by mesenteric blood sampling. Cholic (CA), chenodeoxycholic (CDCA), ursodeoxycholic (UDCA) acid, free and taurine (T-) conjugated, together with glycocholic (GCA) acid and deoxycholic acid (DCA) were studied. The apparent uptake parameters were calculated. All conjugated BA showed active transport (T max, nmol min-1 cm-1 int.), with Tmax values in the following order: TCA > TUDCA > TCDCA; unconjugated BA showed passive uptake, with values in the following order: DCA > CDCA > UDCA > CA. GCA and CA showed both passive uptake and active transport. For all BA studied the % uptake in the ileal segment considered was less than 10%, BA uptake being thus limited by transport and/or diffusion kinetics, rather than by flow velocity. The liquid resistance to BA radial diffusion inside the lumen was evaluated, and the infusate-to-blood uptake parameters corrected for it, in order to get the uptake parameters from the epithelium-to-liquid interface to mesenteric blood: the apparent Km decreased, passive uptake coefficient increased, while Tmax was unchanged. The passive component of the uptake, corrected for the luminal resistance, correlated with the BA hydrophobicity (r = 0.963; P < 0.01).(ABSTRACT TRUNCATED AT 250 WORDS)

Animals↗

Obstetric evolution of pregnancies obtained from donated oocytes.

Of 44 pregnancies obtained at the Tenon Maternity Hospital by in vitro fertilization (IVF) using donated oocytes, 25 have already been completed by spontaneous delivery or cesarean section: 22 (88%) single; 2 (8%) twins, and 1 (4%) triple. The most frequently seen complications of pregnancy were hypertension (16%) and preterm labor in the last 3 months (12%). However, as compared to a control group of 275 pregnancies resulting from classical IVF, the only difference was a very high percentage of cesarean sections (72%). No fetal deaths or neonatal malformations were reported.

Adult↗

Evaluation of the ileal absorption capacity for bile acids in the rabbit.

The intestinal absorption of bile acids has so far been studied from the measurement of the biliary secretion after their intestinal infusion. In the present investigation, the ileal absorption capacity for two different bile acids has been evaluated by means of an intestinal perfusion technique and the simultaneous blood drainage from the mesenteric vein corresponding to the isolated loop. Taurocholic acid and unconjugated cholic acid were infused at different concentrations (0.5-10.0 mM), at a flow rate of 4 ml/min/cm intest. Taurocholic acid absorption increased to a maximum with dose, then reaching a plateau, suggesting that a saturable process had taken place. Cholic acid uptake showed a saturable, possibly active, process, together with a nonsaturable one. These results are consistent with the physicochemical properties of the two bile acids. The technique seems so far suitable for the study of the intestinal absorption of substances undergoing the enterohepatic circulation, since it provides a direct measure of the absorbed fraction, without any distortion arising from the hepatic uptake and biliary secretion.

Animals↗

Glomerulonephritis with organized deposits: a new clinicopathological entity? Light-, electron-microscopic and immunofluorescence study of 12 cases.

Twelve cases of glomerulonephritis in patients without systemic diseases, displaying organized glomerular deposits, were reported. Microfibrils (11-30 nm diameter) were found in 9 patients and microtubules (20-35 nm diameter) in the other 3. Histochemical stainings for amyloid were always negative. By light microscopy, mesangial proliferative, membranous and membranoproliferative patterns were seen in 5, 3 and 4 patients, respectively. By immunofluorescence, granular deposits, mainly of IgG and C3, were found in all cases, either in the mesangium or in the mesangium and in the capillary walls. A second biopsy was performed in 2 patients. The number of hyaline glomeruli was increased, but the general pattern of glomerular changes remained unchanged. The commonest clinical findings were hypertension, microhematuria and proteinuria, often of nephrotic range. At variance to what is reported in the literature, 2 pediatric cases were found as well, and the overall prognosis (mean follow-up 54.3 months) was mostly favorable. The diagnostic relevance of these findings is pointed out, but further investigations are needed, before suggesting a new clinicopathological entity.

Actin Cytoskeleton↗

Production of interleukin-1 alpha and interleukin-2 by mononuclear cells from children with growth delay in relation to the degree of growth hormone deficiency: effects of substitutive treatment.

Interleukin-1 alpha (IL-1 alpha) and interleukin-2 (IL-2) levels were measured by radioimmunoassay in samples of conditioned medium from mononuclear cells taken from 20 normal subjects (14 adults ranging in age from 20 to 45 years and 6 children ranging in age from 3 to 11 years) and from 49 children with growth delay. Cultures were performed with 10(6) cells/ml in medium containing 1% normal human serum and 4.8 g/l phytohemagglutinin M. The incubation was performed for 48 h in an atmosphere containing 5% CO2. In normal subjects, the production of IL-1 alpha was 38.5 +/- 9.8 fmol/ml of conditioned medium (mean +/- SEM) in 14 adults and 41.6 +/- 3.0 fmol/ml in 6 children. The production of IL-2 was 46.9 +/- 6.5 and 57.3 +/- 10.5 fmol/ml, respectively. In the 16 patients with growth hormone (GH) deficiency studied before treatment, the production of ILs was significantly decreased in relation to the degree of deficiency. We observed a positive correlation between the production of IL-1 alpha and the values of insulin-like growth factor I but not with serum GH values. IL-1 alpha production was normalized after 15 days of substitutive GH therapy and IL-2 was normalized after 3 months of therapy. In 10 other patients with GH deficiency (4 with total and 6 with partial isolated GH deficiency) studied after long-term GH treatment (5 months or more), the mean of IL production was not significantly different from that of GH-deficient children treated for 3 months.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

Immunoreactive gastrin-releasing peptide in medullary thyroid carcinoma.

We have previously shown that immunoassayable concentration of somatostatin (SRIH) was elevated in 70% of 34 consecutive medullary thyroid carcinoma (MTC) tissue samples. In the present study gastrin releasing peptide (GRP)-like immunoreactivity was measured in tissue extracts from these 34 MTC (25 inherited, 7 sporadic, 2 unclassified) and in 7 normal thyroid tissue. Plasma SRIH, calcitonin (CT) and carcinoembryonic antigen were assayed in all patients. Normal thyroid tissue contained less than 61 pmol GRP per g wet weight; in contrast GRP concentration was elevated (62-7800 pmol/g) in 32/34 tumor extracts. The distribution of tissue GRP values were similar in sporadic as well as in familial MTC. We found no significant correlation between tumor GRP concentration and plasma SRIH (r = -0.05), plasma CT (r = -0.24), or plasma carcinoembryonic antigen levels (r = -0.21). Tumor concentrations of immunoreactive GRP and SRIH were positively correlated when logarithmic transformation was used (P less than 0.01). Thus GRP, as well as SRIH, is a major product of tumoral C cells in human MTC when systematically evaluated in a large number of cases.

Adolescent↗

Long-term survival patients with acute and severe renal failure due to multiple myeloma.

We selected 37 cases, followed-up for more than 36 months or until death, from a series of 45 patients affected by acute renal failure due to multiple myeloma in order to identify the parameters that could allow the outcome to be predicted. The patients were allocated to group 1, consisting of 27 patients who died within one year and to group 2, consisting of 10 patients who survived for more than 36 months. Renal failure was severe enough to require dialysis in 28 patients, 16 of whom were oliguric. Renal biopsy was performed in 23 cases, whereas light chain isoelectric point and serum beta 2-microglobulin levels were evaluated in each patient. All the patients underwent chemotherapy, which was associated with plasma exchange in 16 patients. Statistical analysis of the potential prognostic factors in the 2 groups showed that the incidence of hypercalcemia, infection, irreversible renal failure and severe tubulo-interstitial damage was significantly higher in group 1. Sex, tumor load, severity of renal failure and light chain isoelectric point had no prognostic significance. Finally, the number of patients treated by plasma exchange was significantly higher in group 2. Our results underline the prognostic role of both hypercalcemia and infection and justify aggressive treatment consisting of chemotherapy, plasma exchange and dialysis, even in cases of severe renal failure and high tumor load.

Acute Kidney Injury↗

Immunoelectron microscopic classification of amyloid in renal biopsies.

Recent classification of amyloidosis is based on the chemical type of amyloid protein involved. In this study, routinely embedded kidney biopsies from nine patients with generalized amyloidosis and renal involvement were tested by immunoelectron microscopy, using the protein A-gold technique, with a panel of antibodies against the following amyloid proteins: AA, A lambda, A kappa and AF. Among the antibodies, the anti-AA was monoclonal (mc1) and the others polyclonal. In all nine cases, only one type of antibody reacted with each amyloid type. Six cases were classified as AA and three cases as A lambda type. These classifications were in agreement with the clinical data and the results of serum and urine immunoelectrophoresis. The gold particles were always associated with amyloid fibrils. No reaction was evident when an amyloid type was stained by a non-corresponding antibody, or in the four control cases without amyloid. The results show that antigenic classification of amyloid is feasible on routinely processed ultra-thin epoxy sections by immunoelectron microscopy, and thus affords the possibility of retrospective studies.

Adolescent↗

Immunoelectron microscopic identification of human AA-type amyloid: exploration of various monoclonal AA-antibodies, methods of fixation, embedding and of other parameters for the protein-A gold method.

Using the postembedding protein-A gold technique ten monoclonal antibodies directed against amyloid-A protein (AA) were examined by immunoelectron microscopy to identify amyloid-A (AA) amyloid fibrils in plastic-embedded renal tissue of five patients and two controls. Two monoclonal antibodies (mc1, mc20) specifically labeled these amyloid deposits; two additional ones (mc4, mc13) bound with an intermediate rabbit anti-mouse IgG antiserum. These monoclonal anti-AA antibodies clearly separate amyloid fibrils from morphologically similar fibrils in the vicinity. Employing varying embedding media, fixation techniques, as well as etching and staining protocols, we adapted this method for the immunoelectron microscopic identification of AA-type amyloid fibrils and for the antigenic diagnosis of AA-type amyloid on routinely processed ultrathin sections.

Amyloidosis↗

Long-term outcome of Schönlein-Henoch nephritis in the adult.

Sixteen adult patients with Schönlein-Henoch nephritis, selected by strict inclusion criteria, were studied retrospectively. At the time of discovery of the nephropathy, 11 patients had normal plasma creatinine and 5 other patients had renal insufficiency. All patients had renal biopsies, which were studied by both light and immunofluorescence microscopy. After a mean follow-up of 90.5 +/- 59.1 months (range 16-261), 3 patients were in chronic dialysis (18.7%), 8 other patients had renal function deterioration (50%), with creatinine clearance ranging from 31 to 60 ml/min. Four other patients had mild urinary abnormalities with normal plasma creatinine (25%) and only 1 patient was in complete clinical remission (6%). No clinical features at onset were predictive for the clinical outcome of the disease, while in the biopsies the percentage of crescents was higher in patients who developed renal insufficiency. High IgA serum levels were correlated (p = 0.0242) with a favorable course. It is concluded that Schönlein-Henoch nephritis of the adult carries a high long-term risk of renal dysfunction.

Adolescent↗

Light chain nephropathy: histological and clinical aspects in 15 cases.

Fifteen patients aged 31-74 years (five male, ten female) on renal biopsy showed intense linear deposits of light chains along tubular basement membranes (TBM) by immunofluorescence, and/or granular dense deposits on electronmicroscopy. Multiple myeloma was diagnosed in ten patients. The onset of myeloma and nephropathy was simultaneous in six patients; nephropathy preceded or followed the diagnosis of myeloma in three and one patients respectively. The mode of onset of nephropathy was acute or rapidly progressive renal failure in five cases, chronic renal failure in seven, and heavy proteinuria in three. Only two patients had normal renal function at biopsy. Serum monoclonal component was kappa in five patients, IgG kappa in three, IgD kappa in one, IgG lambda in one, IgA lambda in one, absent in three, and not detected in one. On light microscopy eight cases had nodular glomerulosclerosis, three cast nephropathy and 14 TBM thickening. Immunofluorescence for monoclonal light chain(s) was positive in 11 of 13 cases. Electron microscopy showed finely granular deposits in the inner side of glomerular basement membranes (GBM) and the outer side of TBM in 11 of 11 tested cases. The evolution was towards chronic renal failure in 12 patients (six of whom required dialysis), death in two, unknown in one. Four patients died after a period of dialysis, from infections or cardiovascular complications.

Adult↗

Renal histological lesions and clinical syndromes in multiple myeloma. Renal Immunopathology Group.

This retrospective multicenter study, based on 42 patients affected by renal damage due to multiple myeloma, analyzes the renal biopsy results, the clinical data at the time of biopsy and the subsequent renal outcome in order to clarify the correlations existing between clinical and histological changes. Plasmocytoma components were Bence Jones alone in 55% of the patients and light-chain excretion was present in over 90%. Rapidly progressive renal failure was the most frequent clinical presentation (27 cases). The histological lesions directly attributable to multiple myeloma were subdivided into 3 basic categories: related to light-chains, direct tumor involvement of renal parenchyma and attributable to systemic effects of neoplastic disease. Light-chains seemed to cause renal lesions in 59.4% of the cases. Myeloma cast nephropathy was the prominent bioptic diagnosis established (20 cases). Among the clinical, laboratory and histological parameters studied, only the degree of tubular-interstitial damage was significantly correlated to the renal outcome in the 32 patients who had an adequately documented follow-up period.

Acute Kidney Injury↗