To analyze the prevalence and clinical significance of thyroid autoimmunity, thyroid volume and iodine status in patients with type 1 diabetes mellitus compared with age and sex matched healthy controls, in an iodine-deficiency improved area.Fifty-eight patients with type 1 DM, 30 female and 28 male, who attended the pediatric endocrinology clinic of Karadeniz Technical University Hospital were included into the study. They were compared with 58 healthy children matched for sex and age. Routine thyroid function parameters, thyroid autoantibodies (TPOAb, TGAb and TRAb) and urinary iodine excretion were measured and thyroid volume was determined by ultrasonography (US).Twenty-six patients (44.8%) in diabetic patients and 20 subjects (34.5%) in the control group had thyroid autoantibody positivity. TPOAb and TGAb positivity were significantly high in diabetic patients (P=0.01 and P=0.032, respectively). Thyroid US revealed a thyroid volume of 6.6±3.5 ml (median 6.4 ml, range 1.1-17.2 ml) in the diabetic patients compared with 3.7±2 ml (median 3.1 ml, range 0.8-8.6 ml) in the control group (P=0.0001). Median urinary iodine levels of both groups were clearly above the threshold level for iodine deficiency, but 26 patients with type 1 DM (44.8%) and 16 controls (27.5%) had urinary iodine excretion below 100 μg/L, and 21 (36.2%) of diabetic patients and two subjects (3.4%) of the control group were consistent with severe iodine deficiency. No significant differences were noted in diabetic patients in terms of age, duration and metabolic control of the disease and thyroid volume when compared according to the autoanti-body presence. Additionally, there were no significant differences between the iodine deficient and iodine sufficient diabetic patients in terms of age, sex, duration of disease, HbA1c, thyroid hormones and thyroid volumes. Thyroid autoimmunity was lower in patients with iodine deficiency (38.4% vs. 50%), but not statistically significant.We found that type 1 DM patients had larger thyroid volume compared with healthy control groups, and a large portion of them had the markers of autoimmune thyroid disease and iodine deficiency. Surprisingly, we found that a large portion of the healthy children had TRAb positivity. We proposed that TRAb must be considered in community surveys or prevalence studies of autoimmune thyroid disorders in iodine-replete areas. Additionally, prospective longitudinal studies are needed to determine the clinical significance of TRAb positivity in diabetic patients.Statut en iode, fonction, volume et auto-immunité thyroïdienne chez des enfants atteints de diabète de type 1 d'une région carencée en iodeAnalyser, chez des patients atteints de diabète de type1 (DT1), la prévalence et la signification clinique de l'autoimmunité anti-thyroïdiennne, le volume thyroïdien et le statut en iode, dans une région où la carence en iode a été corrigée.Cinquante-huit patients DT1 (30 filles) suivis dans le service d'endocrinologie pédiatrique du Karadeniz Technical University Hospital ont été comparés à 58 enfants non diabétiques, appariés selon l'âge et le sexe. Les hormones thyroïdiennes, les anticorps anti-TPO, anti-thyroglobuline (aTg), anti-récepteur de la TSH (TRAb) et l'iodurie ont été mesurés. Le volume thyroïdien a été déterminé par échographie.Des anticorps anti-thyroïdiens étaient présents chez 26 DT1 (44,8 %) et 20 témoins (34,5 %). Les anti-TPO et aTg étaient plus fréquents chez les DT1 (P = 0,01 et P = 0,032, respectivement). Le volume thyroïdien était de 6,6 ± 3.5 ml (médiane 6,4 ml, 1,1-17,2 ml) chez les DT1 et de 3,7 ± 2 ml (médiane 3,1 ml, 0,8-8,6 ml) dans le groupe témoin (P = 0,0001). Dans les deux groupes la médiane de l'iodurie était au-dessus du seuil de carence en iode mais 26 DT1 (44,8 %) et 16 témoins (27,5 %) avaient une iodurie inférieure à 100 μg/L; 21 DT1 (36,2 %) et deux témoins avaient une iodurie compatible avec une carence en iode sévère. Parmi les DT1 aucune différence n'a été observée concernant l'âge, l'ancienneté et la qualité du contrôle du diabète et le volume thyroïdien en fonction de la présence ou non d'anticorps anti-thyroïdiens. De même, parmi les DT1 il n'y avait aucune différence en terme d'âge, de sexe, d'ancienneté du diabète, d'HbA1c, d'hormones thyroïdiennes et de volume thyroïdien selon la présence ou non d'une carence iodée. L'auto-immunité anti-thyroïdienne semblait moins fréquente chez les patients ayant une carence en iode (38,4 % vs. 50 %) mais la différence n'était pas significative.Les patients atteints de DT1 avaient un volume thyroïdien plus élevé que celui des témoins et une grande proportion de ces patients avaient des marqueurs d'auto-immunité antithyroïdienne et une carence iodée. Nous avons trouvé une prévalence élevée de TRAb chez les enfants bien portants. Nous suggérons que des études de prévalence des TRAb et des maladies autoimmunes thyroïdiennes soient faites dans des régions sans carence en iode. De plus, des études prospectives sont nécessaires

Scintigraphy is employed almost systematically in current medical practice for initial thyroid nodules investigation. More and more authors observed that scintigraphy has lost importance confronted with others modern exam, but there are no statistical essay to demonstrate this fact. The aim of our work was to study, on 369 patients, the real contribution of scintigraphy to establish diagnosis and therapeutical strategy. Scintigraphy was truly useful only in 47 (12.7%) patients. We may conclude that thyroid scintigraphy ought to be indicated only in low TSH thyroid nodules, ectopic goiter and, eventually, in metastasis uptake radioactive iodine.

Multiple endocrine neoplasia type 1 (MEN 1) is a rare but misleading disease. The diagnosis is evocated when two main lesions are present (parathyroid, endocrine,pancreas, pitituary gland) but also when a family-tree shows recurrent lesions. Other lesions must be taken into account (adrenal glands, neuroendocrine thymic or bronchic lesions, cutaneous lesions, lipomas, nervous central system tumors). Any surgical cure without knowing the MEN1 background leads to failure. Specific treatment of each lesion is reviewed. Genetic diagnosis is possible but the mutation is not found in all cases. Nevertheless, when the mutation is known in a family, a negative genetic test allows to exclude the disease. Prognosis is related to hepatic metastases and to thymic neuroendocrine tumors which are rare (2.1%) but aggressive. As a general,rule, any apparently isolated endocrine lesion such hyperparathyroidism must prompt the surgeon to look for another endocrine lesion and to look for an abnormal family tree with reccurent monoglandular or pluriglandular lesions. (C) 2002 Editions scientifiques et medicales Elsevier SAS. All rights reserved.

Multiple endocrine neoplasia type 1 (MEN1) is a rare but misleading disease. The diagnosis is evocated when two main lesions are present (parathyroid, endocrine pancreas, pituary gland) but also when a family tree shows recurrent lesions. Other lesions must be taken into account (adrenal glands, neuroendocrine thymic or bronchic lesions, cutaneous lesions, lipomas, nervous central system tumors). Any surgical cure without knowing the MEN1 background leads to failure. Specific treatment of each lesion is reviewed. Genetic diagnosis is possible but the mutation is not found in all cases. Nevertheless, when the mutation is known in a family, a negative genetic test allows to exclude the disease. Prognosis is related to hepatic metastases and to thymic neuroendocrine tumors which are rare (2.1%) but aggressive. As a general rule, any apparently isolated endocrine lesion such hyperparathyroidism must prompt the surgeon to look for another endocrine lesion and to look for an abnormal family tree with recurent monoglandular or pluriglandular lesions.

AIM OF THE STUDY:To evaluate the real contribution of the thyroid radionuclid scanning in the preoperative evaluation of the thyroid nodules.MATERIAL AND METHOD:The authors prospectively studied 369 patients who underwent surgical intervention: 160 (43.4%) for isolated nodules and 209 (56.6%) for multinodular goiters.RESULTS:The scintigraphy of the thyroid was performed in 281 (76.2%) patients: 39 (13.8%) patients with nodules less than 1.5 cm in diameter and 242 (86.2%) patients with nodules more than 1.5 cm in diameter. In 234 patients (83.3%) the scintigraphic evaluation did not bring any utile information for the therapeutic decision. The scintigraphy proved its utility only in 47 patients, which represents 16.7% from the scintigraphic exams performed, or 12.7% from the patients that underwent a surgical procedure.CONCLUSIONS:In the presence of thyroid nodule or multinodular goiter, the scintigraphy is indicated only in decreased TSH level, suspicion of ectopic thyroid or retrosternal goiter.

Aim of the study. To evaluate the real contribution of the thyroid radionuclid scanning in the preoperative evaluation of the thyroid nodules.Material and method. The autors prospectively studed 369 patients who underwent surgical intervention: 160 (43.4%) for isolated nodules and 209 (56.6%) for multinodular goiters.Results. The scintigraphy of the thyroid was performed in 281 (76.2%) patients: 39 (13.8%) patients with nodules less than 1.5 cm in diameter and 242 (86.2%) patients with nodules more than 1.5 cm in diameter. In 234 patients (83.3%) the scintigraphic evaluation did not bring any utile information for the therapeutic decision. The scintigraphy proved its utility only in 47 patients, which represents 16.7% from the scintigraphic exams performed, or 12.7% from the patients that underwent a surgical procedure.Conclusions. In the presence of thyroid nodule or multinodular goiter, the scintigraphy is indicated only in decreased TSH level, suspicion of ectopic thyroid or retrosternal goiter. (C) 2002 Editions scientifiques et medicales Elsevier SAS. All rights reserved.

The extension of the resection for thyroid nodules depends both on nodules' nature and immediate or late postoperative complications risks. This clinical study analyzed the immediate complications appeared after partial thyroidectomy comparatively with those developed after total thyroidectomy. We studied 1411 patients operated in two clinics (from Romania and from France) which have two different attitudes concerning the width of the resection. Paralysis of recurrent laryngeal nerve occurred in 1.0% of patients with partial thyroidectomy and 3.0% of patients with total thyroidectomy, while only one patient (0.6%) developed permanent hypoparathyroidism after total thyroidectomy. In conclusion, total thyroidectomy can be performed by experimented surgeons with a recurrent or parathyroid injury risk similar to partial thyroidectomy. However, the surgeon should take into account the patient survey capacity and the discomfort produced by life substitutive treatment.

Two cases of acute necrotizing pancreatitis after bilateral laparoscopic adrenalectomy were observed in patients with an ectopic ACTH syndrome. Two reasons may be suspected: the difficulty of dissection in such patients and the specific morbidity in relation to hypercorticism.

STUDY AIM:The aim of this retrospective study conducted by the "Groupe d'étude des néoplasies endocriniennes multiples type 1" (GENEM) was to report a series of insulinomas associated with multiple endocrine neoplasias type 1 (NEM 1) and to determine the most appropriate strategy for the topographical exploration and surgical management.PATIENTS AND METHODS:From 1960 to 1996, 44 patients were included, 16 men and 28 women (mean age: 36.4 +/- 13.9 years). This study concerned morphological investigations, tumoral features, modalities and results of the treatment.RESULTS:Insulinomas were associated with other functional islet tumors in 15 patients: ZE syndrome (n = 8), glucagonoma (n = 6), vipoma (n = 1). Malignant lesions were present in 6 patients: isolated insulinomas (n = 2), insulinomas associated with ZE syndrome (n = 2) and vipoma (n = 1). The sensitivity of the preoperative imaging procedures was less than 70%. Fourty-one patients were operated on: subtotal pancreatectomy (n = 26 including cephalic enucleations in 8), enucleations (n = 8), total pancreatectomy (n = 3), pancreaticoduodenectomy with caudal enucleations (n = 1), and for 3 patients, no details were available. There was no postoperative mortality. Hyperinsulinism disappeared in 27 patients (including 2 after reoperation) with a mean follow-up of 9 years. Among 6 patients with malignant lesions, 3 were alive with a 3, 4 and 10 year-follow-up.CONCLUSION:Subtotal pancreatectomy with splenic conservation and enucleation of cephalic lesions is suggested as the procedure of choice in this group of patients. This procedure allows simplification of preoperative imaging investigations because lesions of the pancreatic head have only to be detected. Preoperative endoscopic ultrasonography and intraoperative ultrasonography are the best investigations.

But de l'étude Cette étude prospective initiée par l'AFCE a pour but d'analyser les indications, la voie d'abord et les résultats de toutes les surrénalectomies réalisées au cours de l'année 1997 dans 17 centres ayant une activité importante ou exclusive de chirurgie endocrinienne. Patients et méthodes En 1997, une surrénalectomie a été réalisée chez 247 patients, 149 hommes et 98 femmes (âge moyen: 51 ans). La lésion siégeait à droite dans 116 cas, à gauche dans 99; elle était bilatérale dans 28 cas et ectopique dans quatre. Les phéochromocytomes (n = 61), les adénomes de Conn (n = 50) et les adénomes ou hyperplasies bilatérales à l'origine d'un syndrome de Cushing (n = 48) ont été les lésions le plus souvent rencontrées. La surrénalectomie a été réalisée sous cœlioscopie chez 172 patients (70%) par voie intrapéritonéale dans tous les cas sauf un, et par une opération traditionnelle chez 75 patients (30%). Résultats Chez les patients opérés par cœlioscopie, la durée opératoire moyenne a été de 132 minutes, il y a eu une complication peropératoire dans 15 cas (8, 7%) le plus souvent hémorragique et le taux de conversion a été de 7%. Dans les suites opératoires, il y a eu deux décès (1,16%), l'un précoce chez un patient réopéré pour hémorragie, l'autre très tardif par pancréatite aiguë nécrosante. La durée moyenne d'hospitalisation a été de 5,8 jours. La taille moyenne de la tumeur était de 49 mm. Chez les patients traités en ≪ chirurgie ouverte ≫, la durée opératoire moyenne a été de 148 minutes. Il y a eu une complication peropératoire chez huit patients (10,6%) le plus souvent hémorragique. Il y a eu deux décès peropératoires par hémorragie incontrôlable. La durée moyenne de l'hospitalisation a été de 11 jours. La taille moyenne de la tumeur était de 72 mm. Conclusion La cœlioscopie a pris une part prédominante dans l'exérèse des tumeurs surrénaliennes. Certaines complications observées dans cette série correspondent pour plusieurs groupes à leur période d'apprentissage. La cœlioscopie constitue la voie d'abord de choix pour les tumeurs bénignes uni ou bilatérales, ne dépassant pas 6 ou 7 cm de diamètre. La chirurgie ≪ ouverte ≫ reste indiquée dans les tumeurs malignes, surtout les corticosurrénalomes et dans toutes les tumeurs volumineuses.

STUDY AIM:The aim of this prospective study conducted by the AFCE was to analyze the indications, approach and results of all adrenalectomies performed during the year 1997 in 17 centers, active or specialized in endocrine surgery. PATIENTS AND METHODS:During 1997, adrenalectomy was performed in 247 patients, 149 men and 98 women (mean age: 51 years). The lesion was located in the right side in 166 patients, in the left side in 99, in both sides in 28 patients and ectopic in four patients. Pheochromocytomas (n = 61), Conn adenomas (n = 50) and Cushing syndrome lesions (n = 48) were the most frequent in this series. Laparoscopic adrenalectomy was performed in 172 patients (70%) through a transperitoneal approach in all cases except one, and by 'open' surgery in 75 patients (30%). RESULTS:In patients operated on by the laparoscopic approach, the mean duration of surgery was 132 min, and there were peroperative complications in 15 patients (8.7%), mainly hemorrhages. Conversion rate into laparotomy was 7%. In the postoperative course, there were three reoperations and two deaths, an early one in a patient reoperated for bleeding and a very late one in relation with necrotising acute pancreatitis. Mean duration of hospitalization was 5.8 d. Mean tumoral size was 49 mm. In patients operated on by 'open' surgery, the mean duration of surgery was 148 min. There was a postoperative complication in eight patients (10.6%), mostly hemorrhages, and two intraoperative deaths in relation with uncontrollable bleeding. Mean duration of hospitalization was 11 d. Mean tumoral size was 72 mm. CONCLUSION:Laparoscopic adrenalectomy is now indicated for the majority of adrenal tumors. Several complications observed in this series were related to the learning curve in several centers. Laparoscopic adrenalectomy is the 'gold standard' in uni- or bilateral benign tumors no larger than 6 or 7 cm. 'Open' surgery is indicated in malignant tumors, especially in adreno-cortical carcinomas, and in all large tumors.

The European Consortium on MEN‐1. The search for the MEN1 gene (Minisymposium: MEN & VHL). J Intern Med 1998; 243 : 441–6. The search for the gene whose mutations predispose individuals to multiple endocrine neoplasia type 1 (MEN‐1) started in 1988 when the MEN1 locus was assigned to 11q13, close to PYGM . It came to an end with the recent identification of a gene expressed ubiquitously which harbours inactivating mutations associated with MEN‐1. During these nine years, the genetic linkage interval had been slowly reduced, and losses of heterozygosity (LOH) in MEN‐1 tumours had given strong indications that MEN1 was a tumour suppressor gene. It is ironic that MEN1 was finally found to be located less than 100 kb telomeric to PYGM . From the beginning, this gene was the most tightly linked genetically to MEN‐1. In addition, LOH had already shown (in 1990) that it was the most likely centromeric boundary of the MEN1 minimal region. We recently narrowed the critical region to 900 kb through meiotic mapping, and established a 1200‐kb sequence‐ready contig consisting of cosmids, bacterial artificial chromosomes (BACs) and P1‐derived artificial chromosomes (PACs), including three gene clusters (19 genes and 3 expressed sequence tags). Taking LOH results into account, the gene was likely to be present in the 300‐kb area telomeric to PYGM that we had covered with BACs. One of the novel genes that we have identified by cDNA selection in this region, SCG2 (Suppressor Candidate Gene 2), proved to be identical to the recently published MEN1 gene. Mutation analysis of SCG2 in 11 unrelated MEN‐1 families identified one nucleotide sequence polymorphism and 10 different mutations that segregated with the disease.

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.

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.

Les auteurs rapportent leur experience du traitement des insulinomes pancreatiques, au sujet de 21 patients traites depuis 10 ans. Il y avait 13 cas d'insulinome sporadique et 6 cas d'insulinomes associes a une neoplasie endocrine multiple de type 1 (NEM 1). Tous les insulinomes sporadiques etaient des tumeurs uniques. Tous les patients, d'un âge moyen de 55 ans, ont ete gueris par une exerese pancreatique limitee. Ils n'ont pas presente de recidive avec un recul moyen de 54 mois. Les cas de NEM 1 presentaient des lesions multiples lors du diagnostic, dans 5 cas sur 6, l'âge moyen des patients etait de 29 ans, il existait des antecedents familiaux ou personnels d'endocrinopathie dans 67 % des cas. 5 patients ont du subir une exerese pancreatique large pour controler localement la maladie, dont 2 pour recidive apres un premier traitement inadapte. Les aspects cliniques et anamnestiques doivent permettre de differencier ces deux maladies avant toute decision operatoire. L'echoendoscopie est le seul examen pre-operatoire qui puisse apporter des informations utiles. En cas de NEM 1, il nous semble que la pancreatectomie gauche subtotale est le traitement adapte a cette pathologie.