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J L Peix

Publications and source records attributed to J L Peix.

At least 37 records · Page 2Linked to original sources

Effectiveness and limits of preoperative imaging studies for the localisation of pheochromocytomas and paragangliomas: a review of 282 cases. French Association of Surgery (AFC), and The French Association of Endocrine Surgeons (AFCE).

OBJECTIVE: To find out the optimal strategy for the preoperative location of pheochromocytomas and paragangliomas. DESIGN: Retrospective study. PATIENTS: 282 patients operated on for histologically confirmed pheochromocytoma in France between 1980 and 1991, the past decade. MAIN OUTCOME MEASURES: The results of imaging procedures, i.e. computed tomography (CT), (131)I meta-iodobenzylguanidine scintigraphy (MIBG) and magnetic resonance imaging (MRI) were reviewed. RESULTS: Pheochromocytomas were sporadic in 206 (73%). They were unilateral in 189 (67%), bilateral in 54 (19%) and extra-adrenal in 39 (14%). Overall sensitivity of the studies was 89% for CT, 98% for MRI, and 81% for (131)I-MIBG. In unilateral adrenal lesions sensitivity were 100% for CT and MRI, and 88% for (131)I-MIBG; in bilateral lesions 66% for CT, 100% for MRI, and 62% for (131)I-MIBG; in extra-adrenal lesions 64% for CT, 88% for MRI, and 64% for (131)I-MIBG. CONCLUSION: The accuracy with which pheochromocytomas and paragangliomas can be visualized questions nowadays the routine use of abdominal approach. In selected cases of sporadic unilateral chromaffin tumours, a posterior, lateral, or even laparoscopic approach should be considered.

Abdominal Neoplasms↗

Germ-line mutation analysis in patients with multiple endocrine neoplasia type 1 and related disorders.

Multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominant syndrome predisposing to tumors of the parathyroid, endocrine pancreas, anterior pituitary, adrenal glands, and diffuse neuroendocrine tissues. The MEN1 gene has been assigned, by linkage analysis and loss of heterozygosity, to chromosome 11q13 and recently has been identified by positional cloning. In this study, a total of 84 families and/or isolated patients with either MEN1 or MEN1-related inherited endocrine tumors were screened for MEN1 germ-line mutations, by heteroduplex and sequence analysis of the MEN1 gene-coding region and untranslated exon 1. Germ-line MEN1 alterations were identified in 47/54 (87%) MEN1 families, in 9/11 (82%) isolated MEN1 patients, and in only 6/19 (31.5%) atypical MEN1-related inherited cases. We characterized 52 distinct mutations in a total of 62 MEN1 germ-line alterations. Thirty-five of the 52 mutations were frameshifts and nonsense mutations predicted to encode for a truncated MEN1 protein. We identified eight missense mutations and five in-frame deletions over the entire coding sequence. Six mutations were observed more than once in familial MEN1. Haplotype analysis in families with identical mutations indicate that these occurrences reflected mainly independent mutational events. No MEN1 germ-line mutations were found in 7/54 (13%) MEN1 families, in 2/11 (18%) isolated MEN1 cases, in 13/19 (68. 5%) MEN1-related cases, and in a kindred with familial isolated hyperparathyroidism. Two hundred twenty gene carriers (167 affected and 53 unaffected) were identified. No evidence of genotype-phenotype correlation was found. Age-related penetrance was estimated to be >95% at age >30 years. Our results add to the diversity of MEN1 germ-line mutations and provide new tools in genetic screening of MEN1 and clinically related cases.

Amino Acid Substitution↗

[Malignant cortico-adrenal tumors with vena cava extension. Is surgical resection justified?].

Between January 1989 and December 1996, 6 patients (4 females and 2 males), ranging in age from 23 to 82, underwent surgery for malignant adrenocortical tumors with inferior vena cava extension. There were 5 adrenocortical carcinomas and 1 metastasis from a differentiated thyroid carcinoma. The tumor size ranges from 9 to 17 cm. 5 tumors involved the right adrenal and 1 involved the left adrenal. Four patients had a vena caval thrombus, extending into the right atrium in one case. Two patients had direct tumoral invasion of the IVC wall without thrombius. All patients underwent en bloc excision of the adrenal gland and regional nodes, with nephrectomy in 4 cases and right hepatectomy in one case. Thrombectomy was performed in 4 cases with cardio-pulmonary by-pass in one case. Venous invasion without thrombus required total resection of the infra-hepatic IVC ine one case and vascular reconstruction with prosthetic patch in another case. There were no peri-operative deaths. Two patients died from metastatic disease after 13 and 40 months respectively. Four patients were alive with a follow-up ranging from 3 to 31 months, 3 patient were free of disease. Surgical resection of adrenocortical carcinomas with IVC extension can be attempted. There is no increase in morbidity and mortality in comparison with surgical treatment of other adrenal carcinomas. In these particular cases, the risk of metastases and local recurrence is high, but long-term survival can be obtained after radical macroscopic resection.

Adrenal Cortex Neoplasms↗

[Thyroid cancer in children and adolescents. Clinical aspects, diagnostic problems and special therapeutics].

Twenty-five children undergoing surgery for thyroid carcinoma, were compared in a retrospective study. Clinical characteristics and disease course were detailed in three groups. In the first of 11 patients, a lobo-isthmusectomy was performed for a thyroid nodule, except two cases of total thyroidectomy (TT). Papillary carcinoma was diagnosed on histologic findings. Preoperative cytology and peroperative frozen section examination were inadequate for diagnosis. All children had a complete remission after treatment. In the second group, 10 children had evident thyroid carcinoma with cervical lymph node metastases, and lung metastases in two. Microscopicaly, there were diffuse sclerosing variant or multifocal papillary carcinoma. A TT with central node dissection was performed in all, with a lateral lymph node dissection in 9. Treatment included radioiodine therapy and thyroxin suppression therapy. Three patients had a postoperative recurrent laryngeal palsy, one hypoparathyroidism. Reoperation was carried out in 4 for cervicomediastinal lymph node recurrence. All patients are alive, with a complete remission, except for the two patients with lung metastases. In this group of aggressive tumors, a complete cure was possible with combination of extended, sometimes repeated, surgery and radioiodine therapy. In the last group, 4 patients had a medullary carcinoma. One presented with a thyroid nodule. The others were asymptomatic with a positive pentagastrin test during biological screening for familial disease. Two patients with lymph node metastases had a persistent pathologic calcitonin test despite a TT with complete central and lateral lymph node dissection. Early genetic screening in family members is the only way to perform a curative thyroidectomy in a prepathologic stage.

Adolescent↗

[Results of reoperations for goiter].

We report 47 reoperations for benign thyroid goiters between February 1994 and October 1995 out of a total of 1125 thyroidectomies. These operations concerned 42 women and 5 men with an average age of 54 years. The mean time between the 2 operations was 19 years. On 45 occasions, the reoperation included a totalisation of the previous partial thyroidectomy. In 2 cases a near total thyroidectomy was performed leaving a minimal thyroid remnent on one side. The mean weight of the resected gland was 73 grs. The morbidity included 2 temporary recurrent laryngeal nerve palsies resolving within 2 months and 7 cases of temporary hypocalcemia without permanent hypocalcemia. In the case of repeated surgery, the risks of recurrent laryngeal nerve damage and parathyroid gland resection are typically increased. The risk varies according to the initial surgical resection: unilateral lobectomy versus bilateral subtotal lobectomy. Surgical risks can be significantly decreased with a safe surgical technique. Indications for reoperations in case of benign thyroid goiter are rare and can probably still be reduced. Treatment with thyroxin is sometimes ineffective and is questionable after sub total thyroidectomy for nodular goiter. A primary total thyroidectomy can be indicated in such selected cases.

Female↗

[Excision of a parathyroid adenoma of the aorto-pulmonary window under thoracoscopy].

OBJECTIVES: Neck exploration is usually required in all cases of primary hyperparathyroidism. Without a precise localization preoperatively cervicotomy may be unsuccessful, especially in case of an ectopic adenoma. CASE REPORT: A patient with primary hyperparathyroidism due to a solitary adenoma localized in the middle mediastinum was identified on preoperative computed tomography and technetium-99m-sestamibi radionuclide scan. The tumor was successfully removed at thoracoscopy without neck exploration. DISCUSSION: Preoperative localization of primary hyperparathyroid tumors is not indicated in all patients. In some selected cases (acute hypercalcemia, reoperation, serious illness) prior neck exploration would be useful in guiding the surgeon.

Adenoma↗

[Role of total thyroidectomy in the treatment of benign thyroid diseases].

In 1990, total thyroidectomy (T.T.) was performed in 4% of all benign thyroid diseases and in 7% in case of diffuse benign thyroid diseases, operated on in our surgical unit. In 1995, this incidence of T.T. increased to 20% and 37% respectively. Several considerations can explain this rise in T.T. indications. Currently, the incidence of reoperations on benign goiters represents 4.5% of all our thyroid surgery. It can be considered as a failure of a first incomplete operation. Furthermore, most of goiter recurrences are medically treated and the recurrence rate is higher than the reoperation rate. In Grave's disease, surgery is indicated after medical treatment failure or for acute complications. In this case, the aim of surgery is radical cure of hyperthyroidism. Because evaluation of the weight of thyroid remnant for prevention of postoperative hypothyroidism is difficult, indication of T.T. with thyroxin replacement therapy can be proposed. Surgical excision of a multinodular goiter is indicated for large size lesions, tracheal compression or thyrotoxic evolution. Over the years subtotal thyroidectomy was performed for these benign lesions. Subtotal thyroidectomy leaves a diseased remnant gland. Attempts to suppress nodular recurrence by thyroxin treatment do not guarantee success. Then T.T. can be advocated since postoperative thyroxin therapy is often given after subtotal thyroidectomy to prevent hypothyroidism. Total thyroidectomy is an appropriate operation for the management of a benign thyroid disease because it precludes patients from requiring further surgery for recurrent diseases. However, it requires two conditions. In first, the patient must have the psychologic and economic capacity for a permanent medical therapy. The other is that the procedure be performed with low risks of complications in comparison with subtotal thyroidectomies. Currently recurrential palsy and hypoparathyroidism rates are evaluated in the literature between 0 and 3%.

Goiter↗

[Rapid peroperative assay of intact parathormone in primary hyperparathyroidism. Evaluation and comparison with the long method].

OBJECTIVES: To evaluate the efficacy of rapid peroperative assay of parathormone in patients with primary hyperparathyroidism compared with the longer standard assay. METHODS: Primary hyperthyroidism was diagnosed in 10 patients (8 males, 2 females, mean age 55 +/- 8.6 years, range 36-99) (preoperative tests: serum calcium 290 +/- 8.9 nmol/l, range 274-311; intact parathormone 137 +/- 31.3 pg/ml, range 87-250). First intention cervicotomy was performed. Blood samples were drawn at induction of anaesthesia, at palpation of the adenoma, and 15, 30 and 45 minutes after ablation. Each sample was assayed with a rapid radioimmunoassay and the longer standard laboratory methods. RESULTS: There was a good correlation between the two assay methods. In the 9 patients with a solitary adenoma, serum levels of parathormone were normalized 15 (n = 8) or 30 minutes (n = 1) after resection. CONCLUSION: Rapid radioimmunoassay is a reliable method for evaluating serum parathormone level peroperatively. Although financial implications may limit its use, this rapid assay is clearly indicated for patients in poor clinical condition undergoing first intention surgery and in those undergoing a second cervicotomy.

Adenoma↗

[Evaluation of results of surgery].

Surgical treatment of isolate thyroid nodules has a twofold aim: 1) obtaining an accurate diagnosis of benignancy or malignancy, which remains impossible on an extemporaneous basis in 5 to 10% of cases for technical reasons; 2) tailoring tumor exeresis according to the histological nature of the tumor. Scheduled lobo-isthmectomy is typically the standard treatment for benign thyroid nodules. While thyroid surgery on a whole is very benign today, surgery for isolate thyroid nodules is the most benign of all types. There is no mortality, and morbidity requires only allowing for a risk of recurrent paralysis in 0 to 2% of cases. Secondary recurrence of the nodule on the remaining lobe or compensating hypertrophy of said lobe requires second surgery in 5% of cases. The cost of surgery for thyroid nodules is low, but this statement should be qualified by the requirements for regular medical follow-up of the patients after surgery. Surgery for isolate thyroid nodules is both preventive and curative. For benign nodules, it allows avoiding the development of functional impairment caused by an increase in the volume of the nodule. In case of malignant nodules, which generally correspond to differentiated cancers, it allows treating the cancer. General extension of this exeresis surgery has resulted in the regression and even eradication of anaplastic cancers over the past thirty years.

Biopsy, Needle↗

[Primary hyperparathyroidism in 2 adolescents].

The authors report on two cases of primary hyperparathyroidism (PHP) in adolescents. In a 15-year-old boy, the clinical picture included skeletal, dental, renal and psychological manifestations. In the second case, the first symptom was urolithiasis at age 12; following a period without any important clinical event, the diagnosis was established when the patient was 18. In both cases, increased calcium and low phosphorus plasma levels associated to moderately elevated plasma parathyroid hormone levels were noted. CT scan and ultrasounds were normal but MRI showed an enlarged gland in the second case. The surgical exploration of the neck revealed an adenoma in the first case and a hyperplastic gland in the second one. Surgery was followed by an immediate improvement of both clinical and laboratory findings. These observations bring us to examine the spectrum of PHP in childhood, the difficulties in biological diagnosis and localizing techniques, and the recent results of surgical treatment.

Adolescent↗

[Treatment of sclerodermic esophagitis: value of duodenal diversion].

Between 1985 and 1990, six patients were operated on using duodenal diversion for reflux esophagitis with scleroderma. Duodenal diversion was performed twice as initial procedure and 4 times as treatment of unsuccessful antireflux procedure performed ten years previously. Duodenal diversion was associated with truncal vagotomy. In cases of reoperation the initial reconstruction procedure was removed. An esophageal stricture was resected in one case. One patient with previous truncal vagotomy and pyloroplasty underwent supra papillary diversion. There was no postoperative death. The mean follow-up was 28 months with one patient lost to follow-up. For 4 patients, the postoperative weight gain was 10 per cent. Abnormalities of lower esophageal motility in scleroderma account for the poor results after classical antireflux procedures. In these cases duodenal diversion is indicated as primary treatment. In cases of reoperation removal of the initial anti-reflux device is required in association with duodenal diversion.

Adult↗

[Prophylactic drainage after thyroidectomy: a randomized trial].

A randomized clinical trial of surgical drainage in thyroid surgery was performed with 97 patients. Using equivalence testing it is reported that morbidity was not significantly different between the two groups and the length of hospital stay was shorter in the undrained group. It is possible to perform thyroidectomy without drainage in a selected population.

Adult↗