35 research outputs found
Crustal structure of the Betic–Rif system, western Mediterranean, from local earthquake tomography
La population de la Rioja au XVIe siècle
The present study of the population of Rioja (Old Castile) is based on several different sources, essentially population counts (seven in all for this century, but of varying quality) and parish registers, which are fairly numerous after 1550. The high population density is shown, then trends are examined : both the population counts and die baptism data indicate an upward trend which stopped around 1560-1570, followed by a population decline wich accelerated in the last decade. The lack of migration data and the relatively slight impact of the 1564-1567 and 1599-1600 epidemics do not enable the real causes of the growth pattern to be determined.The present study of the population of Rioja (Old Castile) is based on several different sources, essentially population counts (seven in all for this century, but of varying quality) and parish registers, which are fairly numerous after 1550. The high population density is shown, then trends are examined : both the population counts and die baptism data indicate an upward trend which stopped around 1560-1570, followed by a population decline wich accelerated in the last decade. The lack of migration data and the relatively slight impact of the 1564-1567 and 1599-1600 epidemics do not enable the real causes of the growth pattern to be determined.Lazaro Ruiz Mercedes, Gurria Garcia Pedro-A., Brumont Francis. La population de la Rioja au XVIe siècle. In: Annales de démographie historique, 1988. Les transitions démographiques. pp. 221-241
Structural Indicators for System Level Change for Access to Education for Marginalised Groups
Supplementary Material for: Multifocal Insulinoma as the Unique Presenting Feature of MEN1 in an Adolescent
Introduction
Multiple Endocrine Neoplasia Type 1 (MEN1) is an autosomal dominant inherited disorder defined by the presence of two of the following endocrinopathies: primary hyperparathyroidism, anterior pituitary tumors, and duodenopancreatic neuroendocrine tumors (NETs). NETs, which can secrete hormones including insulin, gastrin, and glucagon, among others, are common in patients with MEN1 and are a major cause of morbidity and premature death. NETs are more common later in life, with very few cases described in children. Here, we describe a unique case of an adolescent with multifocal pancreatic NETs as the single presenting feature of MEN1.
Case Presentation
A 13-year-old healthy male presented with severe weakness, altered mental status, and syncope in the setting of a venous blood glucose (BG) of 36 mg/dL. Workup showed an elevated insulin level (14 mcIU/mL) when BG was 39 mg/dL with positive response to glucagon, concerning for hyperinsulinism. Diazoxide and chlorothiazide were started but not well tolerated secondary to emesis. Three suspected NETs were identified by MRI and 68-Ga DOTATATE PET-CT imaging, including the largest, a 2.1 cm mass in the pancreatic head. A fourth mass in the pancreatic tail was identified via intraoperative ultrasound. All lesions were successfully enucleated and excised, and glucose levels normalized off diazoxide by post-op day 2. While the primary lesion stained for insulin and somatostatin by immunofluorescence (IF), consistent with his clinical presentation, the additional tumors expressed glucagon, somatostatin, pancreatic polypeptide, and chromogranin A but were negative for insulin. Genetic testing confirmed a pathogenic heterozygous mutation in MEN1 (c.969C>A, p.Tyr323). He had no other signs of MEN-associated comorbidities on screening.
Discussion/Conclusion
This case demonstrates that young patients with MEN1 can present with multifocal NETs. These NETs may have polyhormonal expression patterns despite a clinical presentation consistent with one primary hormone. Our patient had clinical symptoms and laboratory evaluation consistent with an insulinoma but was found to have four NETs, each with different IF staining patterns. Advanced pre-operative and intraoperative imaging is important to identify and treat all present NETs. Moreover, serum hormone levels pre- and post-treatment could help evaluate whether NETs are actively secreting hormones into the bloodstream or simply expressing them within the pancreas. Finally, this case highlights the importance of genetic testing for MEN1 in all young patients with insulinomas