Biomedical subjects
Publications and source records attributed to C Proye.
This article has been withdrawn at the request of the author(s) and/or editor. The Publisher apologizes for any inconvenience this may cause. The full Elsevier Policy on Article Withdrawal can be found at http://www.elsevier.com/locate/withdrawalpolicy.
AIM OF THE STUDY: To evaluate the efficiency of preoperative parathyroid ultrasonography and scintigraphy in the management of renal hyperparathyroidism. PATIENTS AND METHODS: The charts of the last consecutive 200 patients who underwent surgery for renal hyperparathyroidism from 1998 to 2003 were retrospectively reviewed to collect data concerning parathyroid gland function, results of preoperative ultrasonography and scintigraphy, as well as modalities and results of surgical exploration. RESULTS: Ultrasonography and scintigraphy sensibilities were 36.4% and 49.3%, respectively. Efficiency of both examinations was improved when they were combined (sensibility of 64.7%) and in those patients managed for recurrent hyperparathyroidism. Were more often detected by preoperative examinations glands with high weight and/or greatest diameter, orthotopic and inferior glands as well as glands exhibiting nodular hyperplasia content upon pathological examination. CONCLUSION: Parathyroid ultrasonography and scintigraphy are of poor interest in the management of renal hyperparathyroidism. In a preoperative setting, they should be performed only in patients with recurrent disease.
AIM OF THE STUDY: The allograft of pancreatic islets represents a potential alternative to insulin therapy in patients suffering from the most severe forms of Type 1 diabetes. Here we report our experience of pancreatic procurement for isolation and islet allograft. MATERIALS AND METHODS: Pancreata were procured in brain-dead donors. The islets were isolated using techniques developed and validated in pigs and men. Injection of a given preparation was decided after quantitative and qualitative controls. Islets were transplanted in Type 1 diabetic patients already grafted with a kidney or suffering from severe and/or unstable diabetes, after percutaneous or surgical settlement of an intra-portal catheter. Patients received an "Edmonton-like" immunosuppressive protocol. Grafts were repeated once or twice until a total quantity of 10,000 transplanted islet-equivalents was obtained. RESULTS: Twenty-nine pancreata were procured and 14 preparations were grafted to 7 patients. Eleven graftings were done percutaneously and three were surgical. The initial function of the 14 transplants was confirmed by secretion of C-peptide and decrease of insulin doses. Insulin therapy was completely interrupted in the 5 patients having received at least two grafts. CONCLUSION: These preliminary clinical results confirmed that the isolation technique of human islets and the technique of pancreas procurement are mastered by our team. If the results of this assay (assessment one year after graft) confirm our hopes, we will be able to offer islet allografts to an increasing number of patients with severe Type 1 diabetes.
THIS RETROSPECTIVE STUDY AIMS: To define a clinical and secretory profile of paragangliomas extra-adrenal chromaffin tumors. METHODS: From 1971 throughout 2002, 39 paragangliomas have been observed in 38 patients (22 male, 16 female, average age 41,2 years). RESULTS: Four were located above the diaphragm, 35 were sub-phrenic (6 of the organ of Zuckerkandl), 32 secreted catecholamines, 23 were hypertensive (with only one without hypersecretion of catecholamines). Among 29 (131)I-metaiodobenzylguanidine scans (MIBG) reviewed, 20 tumors took up the radiopharmaceutical. The treatment was surgical in 35 cases with addition of external radiotherapy and MIBG in one case each; two patients died before any treatment. Two patients with persistent disease after surgery were successfully treated by surgery or MIBG. Histologically, 20 were malignant and 17 were seemingly benign. All exclusive dopamine secreting paragangliomas were malignant. Six patients relapsed two of which for a tumor initially classified as benign. The treatment of recurrences was surgical, by MIBG or by external radiotherapy. Nine patients had a family history of chromaffin tumor(s). The genetic survey made in five of these nine patients was positive in all cases.
Appendicoliths are considered to be strong indicators of appendicitis and the complications of appendicitis. We report the case of a 29-year-old woman who underwent a laparoscopic appendectomy for appendicitis with an appendicolith. The appendix was divided with a single ligature at the appendiceal base, and an appendicolith escaped into the pelvis. Thereafter, the patient suffered recurrent pelvic abscess. The diagnosis of retained appendicolith was made by repeated CT scans that revealed a mobile spontaneous calcification within the abscess. This postoperative complication could have been avoided if a systematic division of the appendix had been performed between double ligatures.
AIM: The postoperative pancreatitis was a classical complication in the historical series of primary hyperparathyroidism (HPT), but the causal association was never demonstrated and even recent studies denied it. The aim of this study was to determine the augmentation of postoperative amylasemia, and its possible clinical traduction in patients operated for primary HPT. MATERIAL AND METHODS: Fifty consecutive patients operated for cure of a primary HPT were included in this study. Total amylase, as well as isoenzyme fractions P (pancreatic) and S (salivary), calcium, phosphorus and intact PTH serum concentrations were determined on the days prior and after parathyroidectomy. Fifteen normocalcemic patients operated for secondary HPT constituted the control group. RESULTS: The study deals with 42 female and eight male patients, their mean age was 58.5 years (range 19-89 years). All patients underwent parathyroidectomy for adenoma or hyperplasia. No patient had pancreatitis before parathyroidectomy. Postoperative amylasemia developed in four patients (8%), one with increased total amylase and P fraction, one with only increased total amylase, and two with increased total amylase and S fraction. No patients exhibited abdominal symptoms suggesting acute pancreatitis in the postoperative period. There was no correlation between pre- and post-operative calcium serum levels and pre- and post-operative amylasemia. In the secondary HPT group no significant diminution of the total amylasemia or of P and S fractions were observed. CONCLUSIONS: These results indicate that acute pancreatitis is an exceptional postoperative complication of primary HPT nowadays. The 8% incidence reported in the present study matches the incidence of hyperamylasemia reported postoperatively in non-abdominal or non-parathyroid surgery.
UNLABELLED: On July 2000, 127 gastrinomas (31.1%) were studied by the Endocrine Tumour Group (GTE) using a 408-patient cohort of Multiple Endocrine Neoplasia Type 1 patients. The aim of this study was to assess clinical, biological, surgical data as well as their trends over three periods (<1980-1980/1989->1990). A Zollinger-Ellison syndrome (SZE) was present in 96% of the cases. Mean age at the onset of the disease was 39.4 years. There were 55.9% of men. Synchronous liver metastasis was present in 7.1%. Taken independently, the positivity of the four main diagnosis tests decreased over the time. The diagnosis of oesophagitis increased (4.5-29.7%), as well as the size of the resected tumours (9.9-16.8 mm). There was an increase in the familial background diagnosis (73.1-80%), an increasing use of Octreoscan scintigraphy and transduodenal ultrasound with positive detection of metastasis and tumours in 81.3% and 92.3%, respectively after 1991. Patients were operated on less frequently (96-52.5%), less frequently from the pancreas (87.5-37.5%), and from the gastro-intestinal tract (70.8-30%). The relative percentage of major pancreatic resections increased (with at least removal of the duodenum and the pancreatic head) (10-26.7%). The operative mortality disappeared. Six out of the seven patients (85.7%) who benefited from major pancreatic resections normalized their gastrine level postoperatively versus 15% in less radical techniques. Overall 5 years survival was 90 +/- 4.4%. Survival increased after 1985 (85 +/- 4.8% versus 95 +/- 3.6, P = 0.1). CONCLUSION: SZE in NEM1 were diagnosed at an earlier stage and were less frequently operated on. Nevertheless, the incidence of synchronous metastasis did not change significantly. Patients were mainly operated on for gastric emergencies and pancreatic tumours in order to prevent metastasis without mortality after 1991.
AIMS: To analyse the results of re-operations for persistent (p) or recurrent (r) primary hyperparathyroidism (PHPT). PATIENTS AND METHODS: From 1965 throughout 2001, 1888 patients were operated on for PHPT. The cure rate after initial surgery was 97.6%. Seventy-seven (4.1%) were reoperated for p PHPT (n = 54) or r PHPT (n = 23). Thirty-two out of 77 (41%) had been primarily operated elsewhere. In 15 cases (20%) PHPT was genetically determined. The re-operation was undertaken on average 40.7 months after initial surgery (1 day-190 months). RESULTS: Two out of 77 were cases of familial hypocalciuric hypercalcaemia. Among the 75 patients reoperated for true PHPT, 23 (31%) had uniglandular disease (UGD) and 52 (69%) had multiglandular disease (MGD). There were two cases of recurrent parathyroid carcinoma. Overall 97 pathological glands were resected, 37% being orthotopic and 63% heterotopic. The re-operation was performed by a cervical approach in 80%, by a mediastinal approach in 15%, whereas 5% involved excision of antebrachial implants. In 96% of cases the parathyroid glands were in the cervical position. Among the preoperative localisations studies the sensitivity of scintigraphy utilising 2-methoxyisobutyl-isonitril (MIBI) was 61%. Utilising both MIBI and cervical ultrasound the sensitivity was 64%. Sixty-eight out of 75 (91%) were cured of their hypercalcaemia, but at the cost of permanent hypoparathyroidism in 9% of cases. No sporadic adenoma appears to have been missed. The seven failures after re-operation (9%) involved five cases of MGD, of which four were sporadic, two cases of carcinoma and one case of parathyreomatosis. 39 patients (51%) had more than four parathyroid glands and in 22/39 cases at least one supernumerary gland was pathological. CONCLUSION: The re-operations for PHPT were essentially due to MGD that was either sporadic or genetically determined. Often the offending supernumerary gland was not detected by imaging studies. Avoiding failures entails an initial bilateral cervicotomy with thymic exploration after MIBI scintigraphy to exclude a mediastinal focus.
Between 1971 and 2002, 80 patients underwent surgery for insulinoma at the Department of General and Endocrine Surgery of the Lille University Hospitals. The present report deals with 13 patients with proven multiple endocrine neoplasia type I (MEN I) or supposed genetic-related insulinomas. This entity differs from spontaneous insulinoma by the presence of multiple foci in the pancreas. Enucleation is not advised in this setting due to the strong likelihood of persistence or recurrence. Various studies suggest different strategies for preoperative localization and surgical approach. We analyzed retrospectively the surgical strategy proposed by the A.F.C.E. and G.E.N.E.M. The purpose of this study was to validate the strategy, integrate the contribution of genotypic diagnosis, simplify preoperative imaging studies, and re-evaluate the value of intraoperative baseline secretin-stimulated insulin measurements. We recommend preoperative endoscopic ultrasonography of the pancreatic head only and routine left pancreatectomy with enucleation of cephalic tumors under intraoperative hormone monitoring. Preoperative invasive localization studies are proposed only if the endoscopic ultrasonography is negative for the pancreatic head. Intraoperative secretin stimulation test can be useful in difficult cases, especially with concurrent nesidioblastosis or in case of secondary surgery. All but one of the 13 patients achieved long-term cure with this strategy.
Recent studies have focused on the occurrence of concomitant medullary-papillary thyroid carcinomas (MTC-PTC). The aims of this report were to compare the frequency of occult PTC in a population with MTC versus a control population that had undergone thyroidectomies and to check whether differences could be related to particular phenotype or genotype. To achieve these goals, we determined the frequency of occult PTC among patients operated for MTC (n = 82) or undergoing total thyroidectomy mainly for goiter and/or nodules (n = 7313) between 1994-2001. We then examined the clinical, histologic, and genetic characteristics (using a bio-chemical family inquiry and screening for RET germline mutations) of patients with associated PTC-MTC. Results show a significantly higher frequency of occult PTC in MTC (14.7%) than in total thyroidectomy (6.8%; p < 0.01). Seventeen cases of MTC or bilateral C-cell hyperplasia (CCH) and separate occult PTC were identified from 16 different families. Although common RET mutations providing evidence of familial forms of MTC were identified in only 3 of 16 families, clinical and histologic features usually seen in inherited forms of MTC such as young age of occurrence, bilateral CCH or associated case in family were found in 11 of the remaining 14 patients. In conclusion, results suggest that the association of MTC-PTC is not only a coincidence. Surprisingly, 11 of 17 MTC-PTC patients exhibited clinical, histologic, and/or family features usually encountered in familial forms despite the fact that no RET defect were present. This suggests the possible involvement of another gene or uncommon abnormality of RET gene.
Explore the source record for details and available documents.
Hyperparathyroidism (HPT) in the setting of multiple endocrine neoplasia type 1 (MEN I) is almost constant and occurs often early in the course of the disease. Underlying pathology is almost always multiglandular because of its genetic origin, and therefore, in case of less than subtotal parathyroidectomy, recurrence rate amounts to 20-40%. Operative strategy aims to find and check all parathyroid glands including possible supernumerary one(s) found in 30% of patients and to perform a subtotal parathyroidectomy. Combined transcervical thymectomy is a mandatory part of the procedure. Moreover HPT triggers the secretion of associated gastrinomas and its cure can thus delay the timing of duodenopancreatic surgery. In case of recurrent HPT, preoperative work-up yields to image the parathyroid remnant and possible supernumerary gland(s). Reoperation aims to remove all parathyroid tissue. Cryopreservation can be useful in case of permanent long-lasting symptomatic hypoparathyroidism.
Chylous ascites is a rare case of peritonitis. We report here a case arising in a 21-year-old lady.
In the situation of persistent hypercalcaemia after cervicotomy for medullary carcinoma of the thyroid (CMT), the concerns are radically different depending on whether the initial operation has been adequate or not. If it has been inadequate, it is necessary to reoperate via cervicotomy and facilitate, in all cases, a total thyroidectomy and a bilateral and central neck dissection. If the cervicotomy has been adequate, it is necessary to have a high index of suspicion for a locoregional recurrence and systemic disease, but the indications for reintervention must be respected. The essential problem is the difficulty in staging residual or recurrent disease. In this situation all the imagery available should be utilised, including laparoscopy to rule out the possibility of miliary metastatic liver disease. There is no hope of cure in the setting of systemic disease, but it is necessary to recall that an extremely elevated calcitonin can be well tolerated and compatible with a survival for several decades. The overall prognosis lies not in the level of elevation of the tumoral marker but the extent of local invasion and systemic disease. There is no hope of cure when the calcitonin level is superior to 1000 pg/ml. There is also no chance of localising recurrent disease when the calcitonin level is inferior to 50 pg/ml. Therefore, one should only utilise the various available localisation techniques when the level of calcitonin is between 50 and 1000 pg/ml. A mediastinal dissection via sternotomy is only indicated in the absence of distal metastases and in the setting of nodal involvement just caudal to the initial cervicotomy, and only after a laparoscopy to exclude hepatic metastases. The future hopes lie with radio-immunoguided surgery in cases of local invasive disease and radiolabelled immunochemotherapy for systemic disease.
PURPOSE: Multiple endocrine neoplasias (MEN) are autosomal dominant inherited syndromes characterized by the association of different glandular lesions in several members of the same kindred. The main clinical features of MEN 1 include primary hyperparathyroidism, pancreatic islet cell tumors and pituitary adenomas; less common features are adrenal adenomas, thymic and bronchial carcinoid tumors, lipomas and various cutaneous lesions. The MEN 2 syndromes (MEN 2A, MEN 2B and familial medullary thyroid carcinomas) are characterized by high penetrance of medullary thyroid carcinoma and differ in their variable expression of pheochromocytoma, hyperparathyroidism and other clinical features. CURRENT KNOWLEDGE AND KEY POINTS: MEN 1 tumor suppressor gene encodes a nuclear protein, menin, which interacts with different regulation transcription factors. The MEN 2 syndromes are caused by germ-line mutations of the RET proto-oncogene, which encodes a transmembrane tyrosine kinase. Genetic testing for mutations in these 2 genes allows identification of individuals predisposed to the disease, early diagnosis, and clinical and therapeutic management. FUTURE PROSPECTS AND PROJECTS: Fundamental approach will allow a best comprehension of physiopathogenic mechanisms of these disorders and the improvement of therapeutic management.
OBJECTIVE: Presence or occurrence of pancreas auto-antibodies (aAb) has been shown to be of poor prognosis for islet cell transplantation. The aim of the study was to monitor the kinetics of these aAb after sequential intra-portal islet plus kidney transplantation with pre-Edmonton immunosuppressive regimen in order to determine whether the sequential protocol of transplantation was involved in the occurrence of the immune response. PATIENTS AND METHODS: Three patients with IDDM and a previous (IAK) or simultaneous (SIK) kidney transplantation received 3 or 4 ABO compatible islet preparations. Islets (> 8 000 IEQ/kg post culture) were sequentially transplanted within a 12 day period via a per-cutaneous catheter. Immunosuppressive treatment included cyclosporine, steroïds and mycophenolate. Plasma ICAs, GAD 65, IA2 and C peptide (C-p) levels were monitored. Type II HLA phenotype was determined in donors and recipients. RESULTS: Patient #1 had high anti-GAD levels (26.5 UI/l) before the IAK, while anti-IA2 and ICA levels were low. After the transplantation, C-p levels increased to 4.9 ng/ml at one month before becoming undetectable at 2 months. GAD levels remained high, ICA and IA2 aAb were undetectable. Patients #2 and #3 did not have significant levels of aAb before the islet transplantation. A slight increase in GAD was observed with each islet transplantation, followed by an overt but transient increase in ICA. IA2 levels remained undetectable. Three months after the transplantation and 2 weeks after the increase of ICA, C-p levels, that were >3.4 ng/ml at one month, fell below 0.2 (N: 0.5-2). CONCLUSION: The immunosuppressive regimen used in kidney transplantation is unable to control perfectly anti-pancreas aAb production. Moreover, these results seem to indicate that the benefits of sequential islet transplantation lie more in the increased islet mass they provide than in potential immune benefit.
Explore the source record for details and available documents.
Explore the source record for details and available documents.