40 research outputs found

    Diagnóstico, tratamento e seguimento do carcinoma medular de tireoide: recomendações do Departamento de Tireoide da Sociedade Brasileira de Endocrinologia e Metabologia

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    R\ue9sultats des ex\ue9reses laparoscopiques des tumeurs surrenaliennes voluminouses et potentiallement malignes

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    INDICATIONS AND RESULTS OF VIDEOASSISTED PARATHYROIDECTOMY BY LATERAL APPROACH IN PATIENTS WITH PRIMARY HYPERPARATHYROIDISM

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    Abstract: Background. Different minimally invasive techniques of parathyroidectomy have been described. We performed a retrospective study to evaluate the indications and results of video-assisted parathyroidectomy by lateral approach (VAPLA) in the management of our patients with primary hyperparathyroidism (PHPT). Methods. From December 1997 to December 2000, we operated on 293 patients with PHPT. VAPLA was proposed for patients with sporadic PHPT a single adenoma was localized by means of sonography or sestamibi scanning, or both. VAPLA was performed on the anterior border of the sternosonography muscle. A quick parathormone (PTH) assay was used during the surgical procedures. Results. Of the 293 patients, 127 (43.3 %) were not eligible for VAPLA: ipsilateral previous neck surgery (28 cases), associated nodular goiter (59 cases), suspicion of multiglandular disease (15 cases), no preoperative localization (17 cases), and miscellaneous causes (8 cases). VAPLA was performed in 166 patients (56.7 %). Conversion to conventional parathyroidectomy was required in 26 patients (15.6 %). Morbidity included 2 local hematomas, 1 definitive recurrent nerve palsy, and 4 capsular fractures. All of the 166 patients were normocalcemic, with follow-up ranging from 3 to 33 months. Conclusions. VAPLA is safe and effective. It should be reserved for patients with sporadic PHPT, with a small single adenoma clearly localized preoperatively

    Clinical and economic aspects of newborn screening for severe combined immunodeficiency: DEPISTREC study results

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    PURPOSE: Severe combined immunodeficiency (SCID) refers to a group of genetic disorders characterized by greatly compromised cellular and humoral immunity. Children with SCID are asymptomatic at birth, but they die from infections within the first months of life if not treated. Quantification of T-cell receptor excision circles is an extremely sensitive screening method for detecting newborns who may have SCID.The goal of the DEPISTREC study was to evaluate the feasibility of nationwide newborn screening for severe T-cell lymphopenia in France as well as its economic and clinical utility. METHODS: The test universally used for neonatal screening for SCID was the quantification of TRECs on Guthrie cards. We compared a group of 190,517 babies from 48 maternities across the country who underwent newborn SCID screening with a control group of 1.4 million babies out of whom 28 were diagnosed with SCID without such screening during the course of the study. RESULTS: Within the screening group, 62 babies were found to be lymphopenic, including three with SCID. The cost of screening ranged from 4.7euro to euro8.15 per newborn. The average 18-month cost was euro257,574 vs euro204,697 in the control group. CONCLUSIONS: In this large-scale study, we demonstrate that routine SCID screening is feasible and effective. This screening offers the additional benefit of aiding in the diagnosis of non-SCID lymphopenia. Economic evaluation allowed us to calculate the cost per test. Newborn screening may also prevent death by SCID before any curative treatment can be administered. The difference in cost between screened and control children could not be ascertained because of the very low numbers and death of one of the children tested

    Laparoscopic adrenalectomy in children: experience of the GECI in 16 cases

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    Background: Conventional adrenalectomy usually necessitates a large incision. In some cases, laparoscopic adrenalectomy offers a less aggressive solution to the patient's problem. Patients and Methods: Laparoscopic adrenalectomy for clearly defined lesions was performed in five girls and nine boys with a mean age of 9 years. The indication was incidental discovery in four patients, hypertension in six (two of whom had von Hippel Lindau syndrome), Cushing's disease in two, and gynecomastia or premature puberty in one patient each. The lesion was on the right side in five patients and the left side in six and was bilateral in three. The mean diameter was 35 mm. The urinary concentrations of vanillylmandelic acid and homomandelic acid were normal in five patients with neurogenic tumors and elevated in five patients with pheochromocytoma. An anterior transabdominal approach was used in nine patients and a lateral retroperitoneal approach in seven. Results: The operating time ranged from 35 to 320 minutes. There were three conversions to open surgery (20%). There were no perioperative or postoperative complications. Histologic examination revealed 13 benign lesions (7 pheochromocytomas, 3 ganglioneuromas, 2 cortical adenomas, and 1 hyperplasia), 1 malignant lesion (N-Myc-negative neuroblastoma, which was removed after conversion to open surgery), and two indeterminate lesions. Of the latter lesions, one was an N-Myc-negative gangioneuroblastoma, and the other was an adrenocortical tumor that was treated initially by local excision and 1 month later by total adrenalec via an open approach. There were no tumor remnants in the specimen. All patients are alive without evidence of recurrence with follow-up of 6 months to 4 years. Conclusions: The laparoscopic approach should be reserved for well-defined lesions <0.5 cm that are probably benign, although the nature of the lesion can be difficult to define preoperatively. The indications can be extended to stage I nonsecreting neuroblastoma in some situations and, for some surgeons, to tumors of stage IV or V. There seems to be no age or weight limit. A left-sided lesion usually is resected via a lateral retroperitoneal approach and a right-sided lesion by an anterior transperitoneal approach, but the details of the technique with the anatomy and the surgeon's experience. Oncologic principles must be respected, and the rate of conversion to open surgery is high

    Efficacy of assisted reproductive technology after ovarian tissue transplantation in a cohort of 11 patients with or without associated infertility factors

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    International audiencePurpose IVF treatment in women with grafted frozen-thawed ovarian tissue is associated with poor reproductive outcomes. The aim of this study was to evaluate the efficacy of ovarian tissue transplantation (OTT) followed by assisted reproductive technology (ART) in women with or without associated infertility factors. Methods This is a prospective cohort study with retrospective data collection including eleven women, four of whom having an infertility factor (IF), who had undergone OTT in one university center between 2005 and 2017, followed by ART in six in vitro fertilization (IVF) centers. Results In total, 25 of the 85 cycles initiated (29%) were canceled, resulting in 60 oocyte retrievals. Ninety-five oocytes were retrieved: 36 were abnormal or immature, 29/39 fertilized (74%) after ICSI and 13/20 (65%) after IVF. Thirty-five embryos were transferred in seven patients (5/7 patients without IF and 2/4 patients with IF). After ART, one patient with IF experienced two pregnancies, one resulting in a live birth. For all patients, pregnancy rates and live birth rates were 7.4% and 3.7% per embryo transfer, respectively. Nine pregnancies and four live births occurred after spontaneous conception in five patients without IF, none in the infertility group. Conclusion This study confirms that IVF treatment in women with grafted frozen-thawed ovarian tissue is associated with poor outcomes. However, the chances of natural conception are high in women without IF. Patients with IF, without the possibility of spontaneous pregnancy, should be informed of poor reproductive outcomes after OTT followed by ART
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