[Cholangiocarcinoma in genetic hemochromatosis].
Explore the source record for details and available documents.
Biomedical subjects
Publications and source records attributed to J Barbier.
Explore the source record for details and available documents.
Primary hyperparathyroidism (PHPT) in multiple endocrine neoplasia (MEN) type IIa is rare, occurring in 20% to 30% of the patients. The aim of this study was to evaluate clinical findings, surgical therapy, and outcome for 56 patients affected by PHPT among 249 MEN-IIa patients collected from 84 families assembled by the Groupe d'Etude des Tumeurs á Calcitonine (GETC, French Calcitonin Tumors Study Group). This retrospective study was based on cases registered by the GETC (20 participating centers) from 1969 to 1994. Characteristics of PHPT in 56 patients (31 women, 25 men) with MEN-IIa were reviewed. All but two underwent cervicotomy. The median age at diagnosis was 37.6 years. PHPT was found concomitantly with medullary thyroid carcinoma (MTC) or pheochromocytoma in 43 patients (77%). PHPT was asymptomatic in 68% of the patients. Serum calcium levels ranged from 2.20 to 3.70 mmol/L (median 2.82 mmol/L; normal 2. 10-2.60 mmol/L). The number of parathyroid glands removed at surgery was 0 (n = 2), 1 (n = 24), 2 (n = 5), > 2 (n = 12), 4 (n = 11). Pathology (initial surgery) consisted of 24 adenomas, 4 double adenomas, and 25 hyperplasia. Cure after initial surgery was obtained in 89%, including a 22% incidence of hypoparathyroidism. There were 6 cases (11%) with persistent PHPT. With a mean follow-up of 6.4 years, five patients (9%) had recurrent PHPT. The results indicate that MEN-IIa-related PHPT is generally associated with mild, often asymptomatic hypercalcemia. Despite recurrences encountered 5 to 15 years after the first cervicotomy, resection of only macroscopically enlarged glands generally appears sufficient. Subtotal or total parathyroidectomy with autotransplantation is associated with a high rate of hypoparathyroidism.
After thyroidectomy, the anesthesiologist usually performs a laryngoscopy to detect laryngeal edema and nerve palsies. The goal of this study was to compare three different methods of laryngeal examination after tracheal extubation of the patients. For that purpose, between 1990 and 1995, a prospective series of 1608 patients operated for thyroidectomy has been studied. The series was divided into 4 groups. In group I (n = 200), four anesthesiologists have evaluated the efficiency of the immediate postextubation direct laryngoscopy. In group II (n = 100), one anesthesiologist has compared the direct, indirect, and flexible laryngoscopies in every patient in a fixed and timed fashion. In group III (n = 100), the four examiners have evaluated the flexible laryngoscopy at a different timing so as to eliminate the possible temporal relationship of the ease of visualization in group II. In group IV (n = 1208), the four examiners have evaluated flexible laryngoscopy, on a large scale, at any time during the 1-h stay in the recovery room. Special attention was directed to the patients with known cardiovascular diseases. Direct and indirect laryngoscopies were only effective in 76 and 73%, respectively, of the patients, whereas flexible laryngoscopy was effective in 99.6% of them. Flexible laryngoscopy was easy to perform in 96.5% of the patients versus 65 and 55% with direct and indirect laryngoscopies. Finally, variations in monitored cardiovascular parameters were significantly lower with flexible and indirect laryngoscopies than with direct laryngoscopy. These mild variations induced by flexible laryngoscopy were well tolerated by patients with known cardiovascular diseases. Flexible laryngoscopy is the best method for an immediate laryngoscopic examination after thyroidectomy.
We investigated the effects of inhibitions of protein phosphatases and protein kinases on thyrotropin (TSH) stimulation of cAMP accumulation in human thyroid cells. Okadaic acid (OA) and calyculin-A (CL-A), two potent inhibitors of type-1 (PP-1) and type-2A (PP-2A) protein phosphatases, had a biphasic concentration-dependent response on cAMP formation. An inhibitory effect (41.3% and 47.2% inhibition with OA and CL-A) was first observed at 1 microM OA and 10 nM CL-A, followed by a reduction of this effect with OA (24% inhibition) or by a complete reversal of inhibition with CL-A, at 10-fold higher concentrations of both products. Addition of purified PP-1 and PP-2A to crude membranes from cells preincubated with OA, reversed OA-induced adenylyl cyclase inhibition, confirming that these protein phosphatases regulate TSH-mediated cAMP production. Levels of protein incorporation of 32P were higher with 10 microM OA than with 1 microM OA and did not correlate with the biphasic effect of OA on cAMP production. These results support a dual action of protein phosphorylation in the control of adenylyl cyclase activity stimulated by TSH. H-7, an inhibitor of nucleotide- and calcium/phospholipid-dependent protein kinase (PKC), increased by 197% the stimulation of cAMP accumulation by TSH in thyroid cells. Phorbol 12-myristate 13-acetate (PMA) counteracted the effect of H-7 on cAMP levels, which suggests that PKC is involved in the action of H-7. Moreover, KT5926, an inhibitor of calcium/calmodulin-dependent protein kinase II and myosin light chain kinase, increased basal cAMP levels rather than cAMP levels stimulated by TSH. In light of these results, we suggest that phosphorylation/dephosphorylation cycles regulate basal and TSH-stimulated adenylyl cyclase activities in human thyroid.
BACKGROUND: Cathepsin D is a widely distributed lysosomal acidic endopeptidase. It is an estrogen-regulated protein that is a prognostic factor in breast cancer. The aim of this study was to measure cathepsin D concentrations in thyroid tissues and to correlate these concentrations with clinical and pathologic parameters. METHODS: Cathepsin D and thyroglobulin concentrations were measured in the cytosol of normal thyroid tissues (n = 14), benign nodules (n = 6), and thyroid carcinomas (n = 32) with an immunoradiometric assay. Statistical analysis was based on the Kruskal-Wallis and Wilcoxon tests and on the Spearman rank correlation coefficient. RESULTS: The mean level of cathepsin D, expressed as picomoles per milligram protein minus thyroglobulin, was higher in the 32 carcinomas, 29.1 +/- 15.5, than in the 14 normal thyroid tissues, 8.4 +/- 2.5 (p < 0.001) or in the 6 benign nodules, 11.2 +/- 7.3 (p = 0.003). Cathepsin D concentrations correlated with tumor size; Spearman rank correlation coefficient was rs = 0.44 (p = 0.012). No significant difference was found regarding histologic type. Cathepsin D concentrations were inversely correlated with the thyroglobulin level in the tumor; Spearman rank correlation coefficient was rs = -0.60 (p < 0.001). CONCLUSIONS: Cathepsin D concentration is higher in thyroid carcinoma than in normal thyroid tissue. Increased cathepsin D concentrations correlate with thyroid tumor size but not with histologic type. Further studies should be done to confirm the potential prognostic value of cathepsin D in patients with thyroid carcinomas.
We report the case of a 32-year-old man with a mycotic aneurysm of the left subclavian artery. This patient had immunosuppression caused by chemotherapy administered for treatment of leukemia. This aneurysm was revealed by two episodes of hemoptysis caused by a lung parenchyma fistulization. The patient was treated successfully by simple ligation and exclusion via a thoracotomy with partial lung resection. Histologic examination confirmed the presence of aspergilloma filaments in the false aneurysm. We suspect that aspergilloma could have been the cause of the mycotic aneurysm in this particular case. The literature on subclavian artery mycotic aneurysms is reviewed.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Negative initial cervicotomy for primary hyperparathyroidism can be due to one of two reasons: first, one gland has not been found: it was an missing adenoma on an ectopic gland. Secondly, four normal glands were found: a missing adenoma arising in a supernumerary and ectopic gland. Successful parathyroid surgery depends the on surgeon's experience, his knowledge of parathyroid gland embryology, and his perseverance to find the pathologic gland. After an unsuccessful cervicotomy, the necessity for reoperation must be discussed. Before reexploration, diagnosis of hyperparathyroidism must be reviewed, the operative notes and pathologic report of the previous operation must be studied, and localization studies must be performed in order to define the cervical or mediastinal surgical approach.
Explore the source record for details and available documents.
Two cases of torsion of the gallbladder in children (ages 5 and 7) are reported, and the relevant literature is reviewed. The condition is extremely rare in the pediatric population, and the correct diagnosis is rarely made preoperatively. However, the clinical presentation may be evocative of the diagnosis. Ultrasonography is helpful for the diagnosis, but ultrasonographic modifications of the gallbladder occur after the onset of the pain. The treatment of this condition is early cholecystectomy.
Complete transection of sympathic and parasympathic nerves is an inevitable consequence of intestinal transplantation. The aim of this experiment was to study if extrinsic denervation alters the motor function of the graft. Ten large-white piglets were used. In Group 1 control (5 animals), a segment of distal ileum was isolated on its intact neurovascular pedicle as a Thirty-Vella loop. In Group 2 study (5 animals), a Thiry-Vella loop of distal ileum was transplanted as a free autograft. The motility of intestinal loops was studied by intraluminal pressure recording at the 8th and 15th postoperative days, on conscious animals fasted for 24 hours. The measured patterns were period (P), duration (D), maximal amplitude (A) and speed of migration (S) of phases III of migratory myoelectric complexes (MMCs). One hundred and seven MMCs were recorded and compared between Group 1 and Group 2. The results were as follows: mean (+/- sem) of Group 1 vs mean (+/- sem) of Group 2. P (min) = 68.5 (+/- 8.1) vs 54.2 (+/- 3.2)(NS); D (min) = 5.9 (+/- 0.2) vs 5.9 (+/- 0.1) (NS); A (cmH2O) = 73.9 (+/- 4.1) vs 83.4 (+/- 3.2) (NS); S (cm/min) = 5 (+/- 1.1) vs 6.2 (+/- 1) (NS). The motor function of the loops was studied following intraluminal perfusion of a cholinergic compound (carbachol) at doses of 1, 2, 3, 4 and 5 mg. The mean and the maximal pressures were recorded during 3 minutes from the 2nd minute following injection of carbachol.(ABSTRACT TRUNCATED AT 250 WORDS)
It is important to recognize exceptional vascular events in celiosurgery because of their gravity. Most occur during pneumoperitoneal manoeuvres using a Palmer needle and rarely at the insertion of the first trocar. We performed a tomodensitometric study in 200 subjects free of abdominal pathology in order to determine the distance from the umbilicus to the aortic bifurcation, their projection over the spine and the superficial relations of the large vessels to the cutaneous umbilical cover. Generally, the projections of the umbilicus, and also the aortic bifurcation, lie over L4. The distance between the skin and the large retroperitoneal vesses is generally equal to one-third of the antero-posterior abdominal diameter. In thin subjects with a flat abdomen however, this distance may be smaller, even less than 3 cm. These findings emphasize the importance of "safety" manoeuvres during celiosurgery, especially during the learning period.
Surgery is usually indicated in elderly patients with acute lithiasic cholecystitis although single antibiotics are often used for patients over 75 with complex medical histories and major visceral lesions. In our series of 45 patients, no new biliary pathology was seen in 26 (57.8%) after a mean follow-up of 40 months. In 15 patients, acute cholecystitis recurred and in 4 a stone found in the common bile duct. No deaths were associated with biliary pathology. While antibiotics appear to have an immediate beneficial effect, medium and long term results are mediocre. Percutaneous cholecystectomie however would be a satisfactory technique for external drainage even though mortality related to other pathologies is high.
Volvulus of the superior mesenteric territory involving the left and transverse colon is rare. Congenital variations of intestinal fixation have been implicated. We report a case observed in an adolescent which illustrates this hypothesis. There was a fixation of pancreatosplenic region and the left colic angle. The duodenopancreas and the right, transverse and left colon were mobile. Human ontogenesis of the colon could provide a pathophysiologic explanation. During foetal development, the intestine results from unequal growth of the different segments which undergo pressure from the different intra-abdominal contents. The fixation of the different parts of the intestine are independent from each other. Specific circumstances are required for variations in congenital fixation of the colon to led to late events responsible for mechanical volvulus.
Explore the source record for details and available documents.
BACKGROUND: To investigate the significance of estrogen receptors (ER) in the pathogenesis of thyroid dysplasia, the authors analyzed, by analogy with breast cancers, ER and three estrogen-regulated proteins: progesterone receptor (PR), cathepsin D, and pS2 protein, in cytosols of 42 human thyroid tissues. METHODS: ER and PR were measured by an immunoenzymatic assay and cathepsin D and pS2 by an immunoradiometric assay. Tissue specimens included 7 normal tissues, 6 benign nodules, 8 toxic adenomas, 7 from patients with Graves disease, and 14 carcinomas. RESULTS: ER was present at very low concentrations, with no statistical difference between neoplastic and nonneoplastic tissues. The mean levels of cathepsin D, expressed as pmol/mg protein minus thyroglobulin, were higher in the 14 carcinomas (P = 0.0003), the 7 specimens from patients with Graves disease (P = 0.006), and the 8 toxic adenomas (P = 0.04) than in the 7 normal thyroid tissues. A significant difference also was observed between the carcinomas (P = 0.003) and six benign nodules. Compared to TNM parameters, cathepsin D concentrations correlated with tumor size: higher cathepsin D levels were found in pT4 than in pT2 and pT3 carcinomas. All the tissues tested were negative for PR and pS2 protein. CONCLUSIONS: The results clearly indicate a significant difference between neoplastic and normal thyroid tissue in terms of the amount of cathepsin D, but not that of ER. This suggests that cathepsin D probably is not regulated by estrogen but simply is a marker of protease activity during invasion by thyroid carcinomas.