RESUMO
OBJECTIVE: To evaluate the natural history of MEN1-related bronchial endocrine tumors (br-NETs) and to determine their histological characteristics, survival and causes of death. br-NETs frequency ranges from 3 to 13% and may reach 32% depending on the number of patients evaluated and on the criteria required for diagnosis. METHODS: The 1023-patient series of symptomatic MEN1 patients followed up in a median of 48.7 [35.5-59.6] years by the Groupe d'étude des Tumeurs Endocrines was analyzed using time-to-event techniques. RESULTS: br-NETs were found in 51 patients (4.8%, [95% CI 3.6-6.2%]) and were discovered by imaging in 86% of cases (CT scan, Octreoscan, Chest X-ray, MRI). Median age at diagnosis was 45 years [28-66]. Histological examination showed 27 (53%) typical carcinoids (TC), 16 (31%) atypical carcinoids (AC), 2 (4%) large cell neuroendocrine carcinomas (LCNEC), 3(6%) small cell neuroendocrine carcinomas (SCLC), 3(6%) TC associated with AC. Overall survival was not different from the rest of the cohort (HR 0.29, [95% CI 0.02-5.14]). AC tended to have a worse prognosis than TC (p = 0.08). Seven deaths were directly related to br-NETs (three AC, three SCLC and one LCNEC). Patients who underwent surgery survived longer (p = 10-4) and were metastasis free, while 8 of 14 non-operated patients were metastatic. There were no operative deaths. CONCLUSIONS: Around 5% of MEN1 patients develop br-NETs. br-NETs do not decrease overall survival in MEN1 patients, but poorly differentiated and aggressive br-NETs can cause death. br-NETs must be screened carefully. A biopsy is essential to operate on patients in time.
Assuntos
Neoplasias Brônquicas/patologia , Neoplasia Endócrina Múltipla Tipo 1/patologia , Tumores Neuroendócrinos/patologia , Adulto , Idoso , Neoplasias Brônquicas/diagnóstico , Neoplasias Brônquicas/mortalidade , Causas de Morte , Estudos de Coortes , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Neoplasia Endócrina Múltipla Tipo 1/diagnóstico , Neoplasia Endócrina Múltipla Tipo 1/mortalidade , Tumores Neuroendócrinos/diagnóstico , Tumores Neuroendócrinos/mortalidade , Análise de SobrevidaRESUMO
INTRODUCTION: Laparoscopy has become the gold-standard approach for excision of benign adrenal tumors but the question of its safety for malignant lesions is still controversial. Our aim was to evaluate the oncologic outcome of laparoscopic adrenalectomy for adrenal metastasis and to look for predictors of a negative surgical outcome. PATIENTS AND METHODS: We retrospectively reviewed the charts of all patients who underwent laparoscopic adrenalectomy for suspicion of adrenal metastasis between 2007 and 2013 at a single academic institution. Recurrence-free survival (RFS) and cancer-specific survival (CSS) were estimated using the Kaplan-Meier method. Univariate analysis was performed to determine risk factors of negative surgical outcome (positive surgical margins, complications, conversion, significant blood loss) and predictors of RFS and CSS. RESULTS: Thirteen patients underwent 14 laparoscopic adrenalectomies. All patients were operated by a single highly experienced surgeon. Complications occurred in 2 patients (15%): 2 blood transfusions (Clavien-score=2). There were 3 positive surgical margins (21%). Mean length of hospital stay was 4.3 days. Unadjusted RFS and CSS were respectively 48.4% and 83.3% at 1 year, 39.5% and 66.7% at 5 years. In univariate analysis, tumor size was the only risk factor of complication (P=.009) and conversion (P=0.009). Capsule invasion and tumor size were risk factors of positive surgical margins (P=0.01 and P<0.0001). One hundred percent of complications, conversion and positive surgical margins occurred in tumor>7.5 cm on preoperative CT-scan. No predictors of RFS and CSS was found in univariate analysis. CONCLUSION: Laparoscopic adrenalectomy for adrenal metastasis achieves good surgical and oncologic outcomes. When performed by highly experienced surgeon, complications and positive surgical margins occur only in tumors>7.5 cm. These patients may benefit from an open surgical approach.
Assuntos
Neoplasias das Glândulas Suprarrenais/secundário , Neoplasias das Glândulas Suprarrenais/cirurgia , Adrenalectomia/métodos , Laparoscopia/efeitos adversos , Neoplasias das Glândulas Suprarrenais/diagnóstico por imagem , Neoplasias das Glândulas Suprarrenais/mortalidade , Adrenalectomia/efeitos adversos , Idoso , Perda Sanguínea Cirúrgica , Transfusão de Sangue/estatística & dados numéricos , Conversão para Cirurgia Aberta , Intervalo Livre de Doença , Feminino , Humanos , Estimativa de Kaplan-Meier , Masculino , Prontuários Médicos , Pessoa de Meia-Idade , Invasividade Neoplásica , Cintilografia , Estudos Retrospectivos , Fatores de Risco , Resultado do TratamentoRESUMO
Automated closed-loop (CL) insulin therapy has come of age. This major technological advance is expected to significantly improve the quality of care for adults, adolescents and children with type 1 diabetes. To improve access to this innovation for both patients and healthcare professionals (HCPs), and to promote adherence to its requirements in terms of safety, regulations, ethics and practice, the French Diabetes Society (SFD) brought together a French Working Group of experts to discuss the current practical consensus. The result is the present statement describing the indications for CL therapy with emphasis on the idea that treatment expectations must be clearly defined in advance. Specifications for expert care centres in charge of initiating the treatment were also proposed. Great importance was also attached to the crucial place of high-quality training for patients and healthcare professionals. Long-term follow-up should collect not only metabolic and clinical results, but also indicators related to psychosocial and human factors. Overall, this national consensus statement aims to promote the introduction of marketed CL devices into standard clinical practice.
Assuntos
Diabetes Mellitus Tipo 1 , Sistemas de Infusão de Insulina , Insulina , Adolescente , Adulto , Criança , Diabetes Mellitus Tipo 1/tratamento farmacológico , França , Humanos , Hipoglicemiantes/administração & dosagem , Insulina/administração & dosagemRESUMO
The use by diabetes patients of real-time continuous interstitial glucose monitoring (CGM) or the FreeStyle Libre® (FSL) flash glucose monitoring (FGM) system is becoming widespread and has changed diabetic practice. The working group bringing together a number of French experts has proposed the present practical consensus. Training of professionals and patient education are crucial for the success of CGM. Also, institutional recommendations must pay particular attention to the indications for and reimbursement of CGM devices in populations at risk of hypoglycaemia. The rules of good practice for CGM are the precursors of those that need to be enacted, given the oncoming emergence of artificial pancreas devices. It is necessary to have software combining user-friendliness, multiplatform usage and average glucose profile (AGP) presentation, while integrating glucose and insulin data as well as events. Expression of CGM data must strive for standardization that facilitates patient phenotyping and their follow-up, while integrating indicators of variability. The introduction of CGM involves a transformation of treatment support, rendering it longer and more complex as it also includes specific educational and technical dimensions. This complexity must be taken into account in discussions of organization of diabetes care.
Assuntos
Automonitorização da Glicemia , Educação de Pacientes como Assunto , Guias de Prática Clínica como Assunto , Diabetes Mellitus Tipo 1/sangue , Diabetes Mellitus Tipo 1/terapia , Diabetes Mellitus Tipo 2/sangue , Diabetes Mellitus Tipo 2/terapia , França , Humanos , Estudos RetrospectivosRESUMO
The popularity of continuous subcutaneous insulin infusion (CSII), as a way for achieving long term strict glycaemic control in diabetic patients, has increased over the last ten years. Most reports on technical faults, often leading to metabolic emergencies, mainly ketoacidosis, have been published in the 1980s. Obstruction of infusion set and infection of infusion site are the most frequent events. Insulin precipitation or aggregation is thought to be one of the precipitating factors. Few data are available about failures of the pump itself. We report our experience of pump malfunctions recorded between 2001 and 2004 in 376 pumps used by patients treated with CSII therapy in Brittany. Recent studies indicate a decrease of metabolic complication frequency during CSII. This suggests technical improvements and/or a greater experience of physicians in selecting and educating patients. We report instructions for monitoring insulin pump therapy that should be included in a formal educational program for pump users. Clinical studies using newly available devices should reassess technical risks associated with CSII.
Assuntos
Sistemas de Infusão de Insulina/efeitos adversos , Glicemia/metabolismo , Automonitorização da Glicemia , Cetoacidose Diabética/epidemiologia , Falha de Equipamento , Humanos , Hipoglicemia/epidemiologia , Inflamação , Fatores de RiscoRESUMO
OBJECTIVE: Our objective was to compare statistical and clinical methods for the evaluation of five self-monitoring blood glucose (SMBG) meters. RESEARCH DESIGN AND METHODS: Two successive capillary blood glucose measurements were performed, and a simultaneous laboratory venous glucose measurement was used as the reference value. Accuracy was studied by comparing each of the two successive meter values with the reference value by 1) a Spearman's correlation test, 2) a Wilcoxon's paired test, 3) the percentage of values within the 10% interval of the reference value according to the American Diabetes Association consensus statement, and 4) the error grid analysis. RESULTS: The first two methods did not discriminate between the SMBG systems: r was >0.92 for the five meters, and a significant difference between the meter and reference values was found for all but one meter. The two other methods allowed classification of the devices into three groups according to their accuracy: good (two meters), acceptable (two meters), and unacceptable (one meter). These two methods gave consistent results and both had a good reproducibility, because the classification was similar for the two successive measurements. CONCLUSIONS: Both the Spearman's and Wilcoxon's paired tests, although commonly used, are inappropriate to evaluate SMBG systems. The percentage of SMBG values within the +/-10% interval and the error grid analysis are more accurate, because they consistently classified the five glucose meters tested in our study with a high degree of reproducibility.
Assuntos
Automonitorização da Glicemia/instrumentação , Adulto , Automonitorização da Glicemia/normas , Automonitorização da Glicemia/estatística & dados numéricos , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Valores de Referência , Reprodutibilidade dos TestesRESUMO
AIM: The benefits of retrospective continuous glucose monitoring (retroCGM) recording have been widely explored in clinical studies, and many diabetes physicians routinely use this examination. However, the method of interpretation of CGM recordings has never been precisely described. METHOD: An expert French panel of physicians met for two days to discuss several aspects of retroCGM use and to produce a position statement. RESULTS: The guidelines cover the indications for retroCGM, the general organization and practical implementation of CGM recordings, a description of the different devices available and guidelines for the interpretation of retroCGM recordings. CONCLUSION: This consensus document should help clinicians in the proper use of retroCGM.
Assuntos
Automonitorização da Glicemia , Glicemia/análise , Diabetes Mellitus , HumanosRESUMO
BACKGROUND: MEN1, which is secondary to the mutation of the MEN1 gene, is a rare autosomal-dominant disease that predisposes mutation carriers to endocrine tumors. Most studies demonstrated the absence of direct genotype-phenotype correlations. The existence of a higher risk of death in the Groupe d'étude des Tumeurs Endocrines-cohort associated with a mutation in the JunD interacting domain suggests heterogeneity across families in disease expressivity. This study aims to assess the existence of modifying genetic factors by estimating the intrafamilial correlations and heritability of the six main tumor types in MEN1. METHODS: The study included 797 patients from 265 kindred and studied seven phenotypic criteria: parathyroid and pancreatic neuroendocrine tumors (NETs) and pituitary, adrenal, bronchial, and thymic (thNET) tumors and the presence of metastasis. Intrafamilial correlations and heritability estimates were calculated from family tree data using specific validated statistical analysis software. RESULTS: Intrafamilial correlations were significant and decreased along parental degrees distance for pituitary, adrenal and thNETs. The heritability of these three tumor types was consistently strong and significant with 64% (s.e.m.=0.13; P<0.001) for pituitary tumor, 65% (s.e.m.=0.21; P<0.001) for adrenal tumors, and 97% (s.e.m.=0.41; P=0.006) for thNETs. CONCLUSION: The present study shows the existence of modifying genetic factors for thymus, adrenal, and pituitary MEN1 tumor types. The identification of at-risk subgroups of individuals within cohorts is the first step toward personalization of care. Next generation sequencing on this subset of tumors will help identify the molecular basis of MEN1 variable genetic expressivity.
Assuntos
Neoplasias das Glândulas Suprarrenais/genética , Neoplasias Brônquicas/genética , Neoplasia Endócrina Múltipla Tipo 1/genética , Tumores Neuroendócrinos/genética , Neoplasias Pancreáticas/genética , Neoplasias das Paratireoides/genética , Neoplasias Hipofisárias/genética , Neoplasias do Timo/genética , Adolescente , Neoplasias das Glândulas Suprarrenais/epidemiologia , Adulto , Distribuição por Idade , Neoplasias Brônquicas/epidemiologia , Criança , Pré-Escolar , Estudos de Coortes , Feminino , Predisposição Genética para Doença , Humanos , Lactente , Recém-Nascido , Masculino , Pessoa de Meia-Idade , Tumores Neuroendócrinos/epidemiologia , Neoplasias Pancreáticas/epidemiologia , Neoplasias das Paratireoides/epidemiologia , Linhagem , Neoplasias Hipofisárias/epidemiologia , Neoplasias do Timo/epidemiologia , Adulto JovemAssuntos
Diabetes Mellitus Tipo 1/tratamento farmacológico , Falha de Equipamento , Sistemas de Infusão de Insulina/efeitos adversos , Overdose de Drogas/epidemiologia , Desenho de Equipamento/normas , Falha de Equipamento/estatística & dados numéricos , Humanos , Sistemas de Infusão de Insulina/estatística & dados numéricos , Estudos Prospectivos , Software , Fatores de Tempo , Falha de TratamentoRESUMO
The aim of this study was to investigate the frequencies of clinical diabetes and humoral markers of anti-pancreatic autoimmunity in a homogeneous population of 600 Caucasian patients with recently diagnosed Graves' disease (GD), in order to characterize the specific features of this group of endocrine patients among subjects at risk of diabetes. Ten were already diabetic at GD diagnosis. Among the 590 non-diabetic patients, 29 had islet cell antibodies (ICA), including 15 with low titre ICA and only 1 ICA-positive subject with a familial history of diabetes. Twenty-four patients had insulin autoantibodies, including three in association with ICA. Glutamic acid decarboxylase (GAD)/64 kDa antibodies were found in 16 of the 150 tested sera, including 13 of the 29 ICA-positive sera. Four ICA-positive patients displayed 37/40 kDa antibodies, including three in association with GAD/64 kDa antibodies. During follow-up, one of the ICA-positive patients developed insulin-dependent diabetes, 14 years after the GD diagnosis. To summarize, this anti-pancreatic autoimmunity study was focused on a large but specific and homogeneous group of subjects at risk for diabetes: recently diagnosed GD patients. This population was characterized by a high prevalence of GAD/64 kDa antibodies but also by a low frequency of evolution towards diabetes and the slowness of the process which could be due to the fact that only a minority of subjects possessed a sufficient combination of anti-pancreatic markers at the same time.
Assuntos
Autoimunidade/imunologia , Doença de Graves/imunologia , Pâncreas/imunologia , Adulto , Autoanticorpos/análise , Biomarcadores/análise , Estudos de Coortes , Complicações do Diabetes , Feminino , Glutamato Descarboxilase/imunologia , Doença de Graves/complicações , Doença de Graves/etnologia , Humanos , Insulina/imunologia , Ilhotas Pancreáticas/imunologia , Masculino , Pessoa de Meia-Idade , População BrancaRESUMO
Acute insulin responses to glucose (AIRG), glucagon (AIRGln), and arginine (AIRArg) were evaluated prospectively in nine subjects positive for islet-cell antibodies (ICAs) who later progressed to type I diabetes or impaired glucose tolerance (IGT) (progressors), 64 ICA-positive subjects at risk who did not develop type I diabetes, 365 ICA-negative relatives of diabetic patients who also remained free of the disease, and 89 control subjects. Seven progressors already had a low AIRG at entry into the study, and the other two became low responders 3 to 9 months before diabetes or IGT, with a progressive decline of AIRG over serial intravenous (IV) glucose tolerance tests. At entry into the study, the group of progressors displayed lower AIRG, AIRGln, and AIRArg than the other three groups (P<.001). However, AIRArg was less altered than AIRG. During the course of the prediabetic phase, there was a progressive decline in AIRG and AIRGln analyzed as a function either of time (P<.006) or of basal glycemia (P<.05), ie, two different ways of estimating worsening of the disease process. Conversely, there was no significant decrease in AIRArg with time or with increasing basal glycemia, so that AIRArg was not totally blunted in these prediabetic subjects even a few months before the onset of diabetes. The persistence of a substantial response to arginine, ie, higher than the fifth control percentile, even at a late stage, was confirmed in five of nine diabetic patients tested either at onset of the disease or during non-insulin-receiving remission. Whereas AIRG deteriorates during prediabetes, AIRArg appears to be less altered with time and increased basal glycemia, remaining substantial even at the onset of the disease. This reinforces the supposition that the prediabetic state may be associated with a glucose-specific beta-cell functional abnormality in addition to a decreasing beta-cell mass.
Assuntos
Arginina/farmacologia , Diabetes Mellitus Tipo 1/metabolismo , Glucagon/farmacologia , Glucose/farmacologia , Ilhotas Pancreáticas/efeitos dos fármacos , Adolescente , Adulto , Criança , Pré-Escolar , Feminino , Humanos , Insulina/metabolismo , Secreção de Insulina , Ilhotas Pancreáticas/fisiopatologia , Masculino , Pessoa de Meia-IdadeRESUMO
The purpose of this study was to determine the prevalence of thyroperoxidase (TPO) and thyroglobulin (Tg) antibodies, using a sensitive and specific radioimmunoassay method in a large cohort of 254 first-degree relatives of Type 1 diabetic patients with or without other autoimmune endocrinopathy, and to evaluate the predictive value of thyroid antibodies for impaired thyroid function in these groups. TPO and Tg antibodies were found at similar frequencies (12%) in the 254 relatives, and both antibodies were present in 23 cases (9%). Seven subjects displayed subclinical thyroid dysfunction without an abnormal free T4 level. Among first-degree relatives of probands with Type 1 diabetes alone, TPO or Tg antibodies were found in 8 subjects (6%), including 6 with both antibodies. The prevalence of TPO antibodies was significantly greater among relatives of TPO-positive than TPO-negative probands (p < 0.01). In relatives of diabetic patients with other endocrinopathy, frequencies of TPO (20%), Tg (19%) and a combination of both antibodies (15%) were significantly higher than in relatives of Type 1 diabetic patients without endocrinopathy (p < 0.001). TSH levels were abnormal in only one relative of the group without endocrinopathy but occurred in 6 relatives of the proband with overt endocrinopathy-associated diabetes (p < 0.02) in marked association with TPO antibodies (p < 10(-4). It is concluded that relatives of probands with overt endocrine autoimmune disease-associated diabetes, unlike those of probands with diabetes alone, showed increased prevalence of thyroid antibodies and thyroid dysfunction. These results argue for a different risk of thyroid autoimmunity and clinical disease in families of diabetic patients without or with overt endocrine disease. A screening of thyroid autoimmunity is highly recommended for the latter group.
Assuntos
Autoanticorpos/sangue , Diabetes Mellitus Tipo 1/imunologia , Doenças do Sistema Endócrino/imunologia , Tireoglobulina/imunologia , Doenças da Glândula Tireoide/imunologia , Adolescente , Adulto , Doenças Autoimunes/imunologia , Criança , Pré-Escolar , Feminino , Humanos , Iodeto Peroxidase/imunologia , Masculino , Pessoa de Meia-Idade , Prevalência , Radioimunoensaio , Doenças da Glândula Tireoide/epidemiologiaRESUMO
STUDY: The aim of our study was to study therapeutic results after thyroidectomy in patients positive for predictive genetic analysis and with preoperative calcitonin (CT) response to pentagastlin (Pg) < 150 pg/ml. MATERIAL AND METHODS: 36 patients (13 F, 23 M) were selected: 13 F-MTC from 8 families, 22 MEN 2A from 15 families and 1 MEN 2B. They were positive for direct RET mutation analysis. CT was assayed by immunoradiometric method before and after Pg. Pg test results before and after thyroidectomy, age at operation and histologic results were analysed. RESULTS: Mean preoperative peak CT was 82.5 +/- 34.0 pg/ml (22-133): among these 36 patients preoperative basal and peak CT were normal in 16 and 2 patients respectively. F-MTC and MEN 2A patients were different according to their preoperative peak CT levels (58.1 +/- 24.0 vs 97.6 +/- 31.3) pg/ml, p < 0.01) and age at thyroidectomy (20.4 +/- 10.5 vs 11.6 +/- 7.6 years, p < 0.01 by Mann-Whitney test). Total thyroidectomy was performed in all patients at a mean age of 14.8 +/- 9.8 years (2.5-41.7) and was associated with lymph node dissection in 30 cases. The 2 F-MTC patients with normal preoperative peak CT levels had bilateral C-cell hyperplasia (CCH) associated with uni or bilateral micro-MTC. Other patients had uni or bilateral micro MTC except 4 who had isolated CCH without carcinoma. The age of two MEN-2A and 1 MEN 2B patients with micro-MTC ranged from 2.5 to 4.7 yr. Micro MTC was present in 100% of MEN-2A cases after the age of 10 yr. There were no lymph nodes metastases. During postoperative survey, the last PG tests (n = 33) were performed 27.5 months (1-92) after thyroidectomy: peak CT values were always < 10 pg/ml. IN CONCLUSION: Thyroidectomy should be performed at a very young age in RET mutation carriers, regardless of the plasma CT values. This choice is justified in NEM-2A and NEM-2B patients but must be discussed in F-MTC families with less aggressive forms of the disease.
Assuntos
Carcinoma Medular/genética , Carcinoma Medular/cirurgia , Neoplasias da Glândula Tireoide/genética , Neoplasias da Glândula Tireoide/cirurgia , Adolescente , Adulto , Fatores Etários , Calcitonina/sangue , Carcinoma Medular/sangue , Causalidade , Criança , Pré-Escolar , Análise Mutacional de DNA , Feminino , Seguimentos , Triagem de Portadores Genéticos , Predisposição Genética para Doença , Humanos , Excisão de Linfonodo , Masculino , Pessoa de Meia-Idade , Neoplasias da Glândula Tireoide/sangue , Tireoidectomia , Resultado do TratamentoRESUMO
The frequency of recurrence of thyroid nodule following hemithyroidectomy is variably assessed according to the individual authors. Few studies put a figure to ultrasonographic recurrence, clinical recurrence only being taken into account: its percentage ranges from 10 to 20%, increasing with the time lapse after thyroidectomy. Attempts to prevent recurrence are based on an approach of the physiopathology of thyroid nodules. Clinical and epidemiological studies of patients with goiters suggest that TSH may play a role in the generation of goiters, as has been confirmed by experimental work. However, several other local autocrine or paracrine growth factors may also be involved in the generation of goiters, including IGF I and IGF II (Insulin Growth Factors), FGF (Fibroblast Growth Factor), EGF (Epidermal Growth Factor), TGFB (Transforming Growth Factor beta). On the basis of the possible role of TSH in goiter generation, some teams have advocated a postoperative course of thyroid hormones aimed at limiting thyreostimulin production. These studies, most of them retrospective studies, do not allow drawing a meaningful conclusion because their methods are open to criticism and the results observed are disparate. On a whole, although thyroid hormone therapy is often used after hemithyroidectomy, the physiopathological bases of its prescription are disputable, and the results obtained in terms of recurrence of nodules are not conclusive enough. It is therefore desirable to set up randomized studies.
Assuntos
Hormônios Tireóideos/uso terapêutico , Nódulo da Glândula Tireoide/prevenção & controle , Nódulo da Glândula Tireoide/cirurgia , Tireoidectomia/efeitos adversos , Humanos , Recidiva , Nódulo da Glândula Tireoide/epidemiologia , Nódulo da Glândula Tireoide/fisiopatologia , Tireoidectomia/métodos , Tireotropina/fisiologiaRESUMO
The diagnosis of thyroid dysfunction is often late in type 1 diabetic population. So, the aims of this study were 1) to evaluate the prevalences of thyroperoxydase (TPO) and thyroglobulin (Tg) autoantibodies detected by highly sensitive radioimmunological method in a cohort of 258 adult type 1 diabetic patients without evidence of clinical thyroid disease; 2) to determine whether or not measurement of TPO and/or Tg antibodies can identify subjects at risk of clinical or infraclinical thyroid dysfunction by measuring TSH in the entire group. TPO antibodies were found in 45 of the 258 diabetic patients (17%). The prevalence of TPO antibodies was not influenced by the following factors: gender, duration of disease, age at screening and at diabetes diagnosis, positivity of familial history. Tg antibodies were found in 19 patients (7%), including 13 cases with TPO antibodies. All patients without TPO antibody (n=213), including Tg-positive patients displayed TSH values in normal range. Among the 45 TPO-positive patients, 11 patients displayed infraclinical thyroid dysfunction. At the end of the 5-year follow-up, only 2/45 patients became anti-TPO negative. Thirteen of the 45 patients developed subclinical or clinical thyroid diseases (4 Graves'disease and 9 thyroiditis with hypothyroidism). By contrast, none of 45 TPO negative patients, sex and age matched with the TPO-positive patients, developed during follow-up anti-TPO positivity and/or infraclinical thyroid dysfunction. In conclusion, the determination of TPO antibodies by a highly sensitive method allows identifying diabetic patients with thyroid autoimmunity and at risk of subsequent impaired thyroid function, whatever age at diagnosis and diabetes duration. By contrast, anti-Tg determination did not give further information about subsequent thyroid dysfunction. In TPO antibody positive patients repeated thyroid clinical examination and TSH determination could be recommended to detect infraclinical thyroid dysfunction.
Assuntos
Anticorpos/sangue , Doenças Autoimunes/diagnóstico , Ensaios Enzimáticos Clínicos , Diabetes Mellitus Tipo 1/enzimologia , Iodeto Peroxidase/imunologia , Doenças da Glândula Tireoide/diagnóstico , Adolescente , Adulto , Doenças Autoimunes/sangue , Biomarcadores/sangue , Diabetes Mellitus Tipo 1/complicações , Feminino , Humanos , Iodeto Peroxidase/sangue , Masculino , Pessoa de Meia-Idade , Tireoglobulina/sangue , Tireoglobulina/imunologia , Doenças da Glândula Tireoide/sangue , Tiroxina/sangue , Tri-Iodotironina/sangueRESUMO
We report a new case of primary adrenal lymphoma with latent adrenal insufficiency and long-term remission after hydrocortisone replacement therapy. We have analyzed 29 other cases described in the literature. This disease with poor prognosis can be revealed by an incidentally discovered, frequently bilateral, adrenal mass. Adrenal insufficiency may be latent and the diagnostic procedure should include both cortisol and ACTH determination with an additional ACTH stimulation test if appropriate. Early adrenal substitution can improve patient survival.
Assuntos
Neoplasias das Glândulas Suprarrenais/patologia , Insuficiência Adrenal/patologia , Linfoma/patologia , Neoplasias das Glândulas Suprarrenais/complicações , Neoplasias das Glândulas Suprarrenais/tratamento farmacológico , Insuficiência Adrenal/etiologia , Idoso , Terapia de Reposição de Estrogênios , Humanos , Hidrocortisona/uso terapêutico , Linfoma/complicações , Linfoma/tratamento farmacológico , Imageamento por Ressonância Magnética , Masculino , Tomografia Computadorizada por Raios XRESUMO
CONTEXT: Multiple endocrine neoplasia type 1 (MEN1) disease is an autosomal dominant syndrome that is believed to equally affect men and women. This assumption has never been confirmed. OBJECTIVE: The aims of this study were to evaluate the impact of gender on the prevalence of MEN1 lesions, on their lifetime probability of occurrence, and on the diagnosis of MEN1. DESIGN: Data regarding a study of 734 cases of MEN1 from the multicenter 'Groupe d'étude des Tumeurs Endocrines' were analyzed. RESULTS: There were 57.8% females. The prevalence and probability of pancreatic tumors were higher in males than in females (P=0.06, P=0.0004). This difference was due to gastrinomas. The prevalence and probability of developing pituitary tumors were significantly greater in females (P<0.001, P<0.0001). Thymic tumors were exclusively found in men. There were no significant gender differences in the prevalence and the probability of developing hyperparathyroidism, or adrenal and bronchial tumors, or in the proportion of positive genetic tests. A family history of MEN1 was more frequently found in men than in women at the time of diagnosis (P=0.02). In the case of pituitary tumor, the proportion of patients diagnosed with MEN1 at the time of the first lesion was lower in women (44.2%) than in men (67.3%). CONCLUSION: The phenotype expression of the MEN1 disease gene was different in males and females. In female patients, the possibility of MEN1 is not sufficiently taken into account. Any patient presenting a lesion that belongs to the MEN1 spectrum, such as a pituitary tumor, should be closely questioned about their family history and should be tested for hypercalcemia.