Research and reports in endocrine disorders最新文献

{"title":"Diagnosis and management of insulinoma: current best practice and ongoing developments","authors":"Aida Taye, S. Libutti","doi":"10.2147/RRED.S86565","DOIUrl":"https://doi.org/10.2147/RRED.S86565","url":null,"abstract":"Insulinoma is a predominantly benign and rare neuroendocrine tumor. Patients with insulinoma typically present with neurologic symptoms from hypoglycemia, such as confusion, dizziness, and behavioral changes, as well as symptoms from a surge in catecholamine levels, such as palpitations, diaphoresis, and tachycardia. Symptomatic patients usually have glucose levels below 55 mg/dL and are relieved of their symptoms when carbohydrate is administered. The 48-hour test, performed by measuring blood glucose levels of insulin, C peptide, and proinsulin collected every 4-6 hours in fasting patients, accurately confirms the diagnosis of insulinoma in the majority of the patients. Once the diagnosis is confirmed, the next step in management involves identifying the location of the tumor and successfully removing it surgically. In the last two decades, clinicians have moved away from invasive angiography for preoperative localization. A multiphase computed tomography (CT) can be used to localize the lesion and evaluate for metastasis. If CT does not detect the lesion, selective arterial calcium stimulation test is recommended to identify the region of the lesion in the pancreas. Some argue that all preoperative localization techniques are superfluous. The combination of intraoperative ultra - sound and operative palpation has led to a nearly 100% success rate. Recently, in select cases, laparoscopic enucleations and resections of insulinomas have been performed with shorter length of stay and faster recovery time. Despite advances in imaging, a little over 10% of insulinoma patients undergo reexploration for missing lesions. Patients who are not candidates for tumor resections or awaiting surgery have had symptomatic relief from diazoxide and somatostatin analogs among various medical therapies. In patients with metastatic insulinoma, progression- free survival and overall survival are reported from newly approved chemotherapeutic agents. Liver-directed therapies, such as ablation and selective radiation, and cytoreductive surgery have also been performed for symptom control and prolonging survival.","PeriodicalId":90317,"journal":{"name":"Research and reports in endocrine disorders","volume":"5 1","pages":"125-133"},"PeriodicalIF":0.0,"publicationDate":"2015-08-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.2147/RRED.S86565","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"68478251","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The impact of low-carbohydrate diet on glycemic control in Native Americans","authors":"Shafaq Khairi, B. Sagvand, S. Nasser","doi":"10.2147/RRED.S91040","DOIUrl":"https://doi.org/10.2147/RRED.S91040","url":null,"abstract":"License. The full terms of the License are available at http://creativecommons.org/licenses/by-nc/3.0/. Non-commercial uses of the work are permitted without any further permission from Dove Medical Press Limited, provided the work is properly attributed. Permissions beyond the scope of the License are administered by Dove Medical Press Limited. Information on how to request permission may be found at: http://www.dovepress.com/permissions.php Research and Reports in Endocrine Disorders 2015:5 119–123 Research and Reports in Endocrine Disorders Dovepress","PeriodicalId":90317,"journal":{"name":"Research and reports in endocrine disorders","volume":"5 1","pages":"119-123"},"PeriodicalIF":0.0,"publicationDate":"2015-08-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.2147/RRED.S91040","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"68478381","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Managing congenital hyperinsulinism: improving outcomes with a multidisciplinary approach","authors":"Amanda M. Ackermann, A. Palladino","doi":"10.2147/RRED.S56608","DOIUrl":"https://doi.org/10.2147/RRED.S56608","url":null,"abstract":"Congenital hyperinsulinism (CHI) is the most common cause of persistent hypo- glycemia in pediatric patients and is associated with significant risk of hypoglycemic seizures and developmental delays. CHI results from mutations in at least nine genes that play a role in regulating beta-cell insulin secretion. Thus, patients with CHI have dysregulated insulin secretion that is unresponsive to blood glucose level. Each different genetic etiology of CHI is associated with particular clinical characteristics that affect management decisions. Given the broad phe- notypic spectrum and relatively rare prevalence of CHI, it is important that patients with CHI be evaluated by clinicians experienced with CHI and the multiple subspecialty services that are necessary for the management of the disorder. In this review, we summarize the pathophysiology and genetic causes of CHI and then focus primarily on the most common genetic cause (muta- tions in the ATP-gated potassium (K ATP ) channel) for further discussion of diagnosis, medical and surgical management, and potential acute and chronic complications. We provide insight from relevant published studies and reports, in addition to anecdotal information from our center's clinical experience in caring for over 400 patients with CHI. Careful assessment of each patient's individual pathophysiology is necessary to determine the appropriate treatment regimen, and continued close follow-up and monitoring of disease- and treatment-related complications are essential. Although significant improvements have been made in the past several years with regard to diagnosis and management, given the continued high morbidity rate in patients with CHI, improved diagnostic techniques and new therapeutic options would be welcomed.","PeriodicalId":90317,"journal":{"name":"Research and reports in endocrine disorders","volume":"5 1","pages":"103-117"},"PeriodicalIF":0.0,"publicationDate":"2015-07-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.2147/RRED.S56608","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"68477559","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Congenital hypothyroidism: current perspectives","authors":"D. Dayal, R. Prasad","doi":"10.2147/RRED.S56402","DOIUrl":"https://doi.org/10.2147/RRED.S56402","url":null,"abstract":"Congenital hypothyroidism (CH), the most common pediatric endocrine disorder with an estimated prevalence of 1:2,000 to 1:4,000, is an under-recognized problem in countries without routine newborn screening (NBS) programs. Thyroid dysgenesis (TD) is the most com- mon cause of primary CH accounting for approximately 85% of all cases; most of the remaining patients have dyshormonogenesis. Transient CH and CH with eutopic gland, are increasingly being identified after introduction of routine NBS. The clinical features of CH are often subtle resulting in delayed diagnosis and eventually poor intellectual outcome. In developed countries, detection by NBS and early initiation of treatment has largely eliminated the intellectual dis- ability caused by this disorder. The lower screening thyroid stimulating hormone (TSH) cutoff and changes in birth demographics in some countries have been associated with an increase in the reported incidence of CH. However, the additional cases detected by the lower TSH cutoff tend to have either milder or transient hypothyroidism. Diagnosis of CH is made on the basis of serum concentrations of TSH and thyroxine (T4). Thyroid ultrasound, radionuclide scintigraphy, serum thyroglobulin (TG) levels and specific genetic tests help ascertaining the exact etiological diagnosis. Non-availability of later tests should not deter the pediatrician from initiation of treat- ment. Age at initiation of treatment and starting dose of levothyroxine are critical factors that determine the long-term outcome. Higher doses of levothyroxine at 10-15 μg/kg/day are required in infants, with titration based on T4 and TSH levels, which are repeated frequently. Coexistence of other congenital anomalies in children with CH adds to the morbidity. Approximately 70% of babies worldwide are not born in an area with an established NBS program and hence are not detected and treated early. Consequently, the economic burden of mental retardation due to CH remains a significant public health challenge in countries without NBS. The health burden owing to CH continues to be high even in countries with well-developed NBS.","PeriodicalId":90317,"journal":{"name":"Research and reports in endocrine disorders","volume":"5 1","pages":"91-102"},"PeriodicalIF":0.0,"publicationDate":"2015-07-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.2147/RRED.S56402","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"68477505","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Profile of idursulfase for the treatment of Hunter syndrome","authors":"S. Sestito, Ferdinando Ceravolo, M. Grisolia, E. Pascale, L. Pensabene, D. Concolino","doi":"10.2147/RRED.S64347","DOIUrl":"https://doi.org/10.2147/RRED.S64347","url":null,"abstract":": Mucopolysaccharidosis type II (MPS II; Hunter syndrome) is a rare X-linked lysosomal storage disorder caused by deficiency of the enzyme iduronate-2-sulfatase (IDS). Enzyme replacement therapy (ERT) with recombinant human IDS, available since 2005, is currently the most appropriate treatment for this progressive, multisystemic, chronic, and life-threatening disease. Efficacy and safety of therapy with idursulfase have been assessed in several clinical trials, and confirmed in many clinical reports. Long-term follow-up of patients receiving ERT has demonstrated the importance of an early onset of treatment with idursulfase, before irreversible pathological changes occur. Intravenously administered idursulfase is not able to cross the blood–brain barrier, so neurological signs and symptoms cannot benefit from ERT, still remaining a major challenge in the treatment of MPS II.","PeriodicalId":90317,"journal":{"name":"Research and reports in endocrine disorders","volume":"5 1","pages":"79-90"},"PeriodicalIF":0.0,"publicationDate":"2015-07-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.2147/RRED.S64347","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"68477647","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Type 2 diabetes mellitus among government employees in Harar, Eastern Ethiopia: a cross-sectional study","authors":"D. A. Ayana, Yadeta Dessie Bachu, K. Roba, D. Kebede","doi":"10.2147/RRED.S82883","DOIUrl":"https://doi.org/10.2147/RRED.S82883","url":null,"abstract":"License. The full terms of the License are available at http://creativecommons.org/licenses/by-nc/3.0/. Non-commercial uses of the work are permitted without any further permission from Dove Medical Press Limited, provided the work is properly attributed. Permissions beyond the scope of the License are administered by Dove Medical Press Limited. Information on how to request permission may be found at: http://www.dovepress.com/permissions.php Research and Reports in Endocrine Disorders 2015:5 71–77 Research and Reports in Endocrine Disorders Dovepress","PeriodicalId":90317,"journal":{"name":"Research and reports in endocrine disorders","volume":"5 1","pages":"71-77"},"PeriodicalIF":0.0,"publicationDate":"2015-07-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.2147/RRED.S82883","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"68478206","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Current and emerging testosterone therapies for male hypogonadism","authors":"Blake A Wynia, J. Kaminetsky","doi":"10.2147/RRED.S46193","DOIUrl":"https://doi.org/10.2147/RRED.S46193","url":null,"abstract":"(unported, v3.0) License. The full terms of the License are available at http://creativecommons.org/licenses/by-nc/3.0/. Non-commercial uses of the work are permitted without any further permission from Dove Medical Press Limited, provided the work is properly attributed. Permissions beyond the scope of the License are administered by Dove Medical Press Limited. Information on how to request permission may be found at: http://www.dovepress.com/permissions.php Research and Reports in Endocrine Disorders 2015:5 59–69 Research and Reports in Endocrine Disorders Dovepress","PeriodicalId":90317,"journal":{"name":"Research and reports in endocrine disorders","volume":"5 1","pages":"59-69"},"PeriodicalIF":0.0,"publicationDate":"2015-04-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.2147/RRED.S46193","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"68477336","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Profile of pegvisomant in the management of acromegaly: an evidence based review of its place in therapy","authors":"I. Bernabéu, I. A. Rodriguez-Gomez, A. Ramos-Leví, M. Marazuela","doi":"10.2147/RRED.S78255","DOIUrl":"https://doi.org/10.2147/RRED.S78255","url":null,"abstract":"Pegvisomant (PEG) is a genetically engineered growth hormone (GH) analog able to bind and block the GH receptor. PEG blocks all metabolic effects of GH hypersecretion, normalizes insulin-like growth factor I (IGF-I) level and paradoxically produces an increase in GH secretion. When PEG was commercialized, there were some concerns regarding whether the increased GH secretion could cause growth of the residual tumor or cause the overcoming of receptor blockade with loss of efficacy. PEG commercialization was followed by the onset of two main prospective observational studies aiming to evaluate the safety and outcome of PEG long-term treatment: the German Pegvisomant Observational Study and ACROSTUDY. These observational studies, along with several independent studies have provided comprehensive information regarding the actual use, efficacy and safety of long-term treatment with PEG. The efficacy of PEG in clinical setting is somewhat lower than that reported in the pivotal studies, nevertheless PEG normalizes IGF-I levels ranging between 65% and 97% of cases. Side effects in observational studies were uncommon and rarely caused discontinuation of treatment. Liver dysfunction developed in 2.5% of cases, was usually transient and no permanent liver damage was reported. Increased tumor size was developed by about 2.2%-3.2% of acromegalic patients treated with PEG, without differences to that described for other modalities of treatment. Only one third of cases corresponded with true growth after initiation of PEG treatment. Involved mechanism is currently unknown. New modalities of treatments by the combined use of PEG with somatostatin analog or cabergoline have been developed with promising results. Recently, two clinical guidelines written to optimize the use of these treatment modalities and to monitor possible adverse events have been published.","PeriodicalId":90317,"journal":{"name":"Research and reports in endocrine disorders","volume":"5 1","pages":"47-58"},"PeriodicalIF":0.0,"publicationDate":"2015-02-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.2147/RRED.S78255","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"68477730","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The role of hormonal treatment in type 2 diabetes mellitus","authors":"S. K. Imam","doi":"10.2147/RRED.S49625","DOIUrl":"https://doi.org/10.2147/RRED.S49625","url":null,"abstract":"License. The full terms of the License are available at http://creativecommons.org/licenses/by-nc/3.0/. Non-commercial uses of the work are permitted without any further permission from Dove Medical Press Limited, provided the work is properly attributed. Permissions beyond the scope of the License are administered by Dove Medical Press Limited. Information on how to request permission may be found at: http://www.dovepress.com/permissions.php Research and Reports in Endocrine Disorders 2015:5 31–45 Research and Reports in Endocrine Disorders Dovepress","PeriodicalId":90317,"journal":{"name":"Research and reports in endocrine disorders","volume":"5 1","pages":"31-45"},"PeriodicalIF":0.0,"publicationDate":"2015-02-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.2147/RRED.S49625","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"68477368","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Diabetes reversal via gene transfer: building on successes in animal models","authors":"Dario Gerace, R. Martiniello-Wilks, A. Simpson","doi":"10.2147/RRED.S51644","DOIUrl":"https://doi.org/10.2147/RRED.S51644","url":null,"abstract":"License. The full terms of the License are available at http://creativecommons.org/licenses/by-nc/3.0/. Non-commercial uses of the work are permitted without any further permission from Dove Medical Press Limited, provided the work is properly attributed. Permissions beyond the scope of the License are administered by Dove Medical Press Limited. Information on how to request permission may be found at: http://www.dovepress.com/permissions.php Research and Reports in Endocrine Disorders 2015:5 15–29 Research and Reports in Endocrine Disorders Dovepress","PeriodicalId":90317,"journal":{"name":"Research and reports in endocrine disorders","volume":"5 1","pages":"15-29"},"PeriodicalIF":0.0,"publicationDate":"2015-01-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.2147/RRED.S51644","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"68477127","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}