Abstract:
:The most recent elucidation of an inherited disorder of the pancreas concerns the Johanson-Blizzard syndrome (JBS). Positional cloning identified loss-of-function mutations in the UBRI gene on the long arm of chromosome 15 to be the cause of JBS in more than a dozen patients. In patients with JBS the absence of UBRI results in early prenatal destruction of the exocrine pancreas that involves impaired apoptosis, induced necrosis, and prominent inflammation. Knockout mice with absent UBR1 expression suffer from exocrine pancreatic insufficiency and increased susceptibility to experimental pancreatitis. The UBR1 protein substrate, presumably impaired degradation of which causes JBS, is not yet known.
journal_name
Endocrinol Metab Clin North Amjournal_title
Endocrinology and metabolism clinics of North Americaauthors
Zenker M,Mayerle J,Reis A,Lerch MMdoi
10.1016/j.ecl.2006.02.013subject
Has Abstractpub_date
2006-06-01 00:00:00pages
243-53, vii-viiiissue
2eissn
0889-8529issn
1558-4410pii
S0889-8529(06)00014-4journal_volume
35pub_type
杂志文章,评审abstract::The prevalence of low-risk differentiated thyroid cancer (DTC) is dramatically increasing because of superior diagnostic imaging technologies. Remote-access endoscopic thyroidectomy is becoming more popular for the lack of a noticeable neck scar. Transoral endoscopic thyroidectomy, vestibular approach (TOETVA) is the ...
journal_title:Endocrinology and metabolism clinics of North America
pub_type: 杂志文章,评审
doi:10.1016/j.ecl.2018.11.009
更新日期:2019-03-01 00:00:00
abstract::Obesity in adults is associated with excess mortality and excess risk of coronary heart disease, hypertension, hyperlipidemia, diabetes, gallbladder disease, certain cancers, and osteoarthritis. Overweight children often become overweight adults, and overweight in adulthood is a health risk. Although childhood overwei...
journal_title:Endocrinology and metabolism clinics of North America
pub_type: 杂志文章,评审
doi:10.1016/s0889-8529(03)00074-4
更新日期:2003-12-01 00:00:00
abstract::This article discusses several unusual forms of primary thyroid neoplasms. The TCV of PTC and insular thyroid carcinoma appears to have a more aggressive clinical behavior than DTC in most patient groups and may respond to thyroid hormone suppression and radioiodine. Anaplastic thyroid carcinoma, which may develop fro...
journal_title:Endocrinology and metabolism clinics of North America
pub_type: 杂志文章,评审
doi:10.1016/s0889-8529(05)70312-1
更新日期:1996-03-01 00:00:00
abstract::In this article, the literature is reviewed regarding the role of blood pressure variability and nocturnal nondipping of blood pressure as well as the presence of diabetic kidney disease (DKD), in the absence of albuminuria, as risk predictors for progressive DKD. The importance of glycemic and blood pressure control ...
journal_title:Endocrinology and metabolism clinics of North America
pub_type: 杂志文章,评审
doi:10.1016/j.ecl.2013.06.002
更新日期:2013-12-01 00:00:00
abstract::The regulation of energy balance is enormously complex, with numerous genetic, hormonal, neural/behavioral, and societal influences. Although the current epidemic of obesity has its underpinnings in the changes in culture during the last half century, the role of the neuroendocrine system in the genesis of obesity is ...
journal_title:Endocrinology and metabolism clinics of North America
pub_type: 杂志文章,评审
doi:10.1016/s0889-8529(05)70211-5
更新日期:2001-09-01 00:00:00
abstract::With building pressure for cost-containment of medical expenditures, the medical community is finding it increasingly necessary to justify its procedures and systems of care. The analysis of health care outcomes has been extensively applied to diabetes care as a means of determining the process by which optimal outcom...
journal_title:Endocrinology and metabolism clinics of North America
pub_type: 杂志文章,评审
doi:10.1016/s0889-8529(05)70262-0
更新日期:1997-09-01 00:00:00
abstract::All of the actions of atrial natriuretic factor (ANF) can be explained on the basis of ANF's interaction with specific receptors on the plasma membrane of cells in target tissues. These receptors have been identified. Binding to these receptors by ANF is time dependent and specific and occurs with high affinity. Numer...
journal_title:Endocrinology and metabolism clinics of North America
pub_type: 杂志文章,评审
doi:
更新日期:1987-03-01 00:00:00
abstract::The biologic actions of the peptides of the gastrointestinal tract indicate that they exert their effects by interacting with, in many instances, more than one receptor type and receptors on several tissues. Gut hormone profiles in pathophysiologic situations have not demonstrated a significant role for them in diseas...
journal_title:Endocrinology and metabolism clinics of North America
pub_type: 杂志文章,评审
doi:
更新日期:1993-12-01 00:00:00
abstract::CD4 + CD25 + regulatory T (TR) lymphocytes are essential to the maintenance of immunologic tolerance in the host. The discovery of Foxp3 as a transcription factor essential to the differentiation of TR ushered in detailed studies of the molecular mechanisms of TR cell development, peripheral homeostasis, and effector ...
journal_title:Endocrinology and metabolism clinics of North America
pub_type: 杂志文章,评审
doi:10.1016/j.ecl.2009.01.002
更新日期:2009-06-01 00:00:00
abstract::Adrenal venous sampling is the gold standard test to identify surgically curable primary aldosteronism, but it is markedly underused in clinical practice being perceived as a technically challenging and invasive procedure and, moreover, as difficult to interpret. This review provides updated information on current ind...
journal_title:Endocrinology and metabolism clinics of North America
pub_type: 杂志文章,评审
doi:10.1016/j.ecl.2019.08.012
更新日期:2019-12-01 00:00:00
abstract::Thyroid storm represents the extreme manifestation of thyrotoxicosis as a true endocrine emergency. Although Grave's disease is the most common underlying disorder in thyroid storm, there is usually a precipitating event or condition that transform the patient into life-threatening thyrotoxicosis. Treatment of thyroid...
journal_title:Endocrinology and metabolism clinics of North America
pub_type: 杂志文章,评审
doi:10.1016/j.ecl.2006.09.008
更新日期:2006-12-01 00:00:00
abstract::Prolactin-secreting pituitary tumors are not rare. The diagnosis of a patient with hyperprolactinemia and possible tumor should be carried out in an orderly fashion by first excluding secondary causes. If the patient has pathologic hyperprolactinemia, assessment of pituitary anatomy with a high resolution CT scan (or ...
journal_title:Endocrinology and metabolism clinics of North America
pub_type: 杂志文章,评审
doi:
更新日期:1987-09-01 00:00:00
abstract::Osteoporosis is prevalent in transplant recipients and is related to pre- and post-transplantation factors. Low bone density and fractures may antedate transplantation, related to traditional risk factors for osteoporosis, effects of chronic illness, and end-stage organ failure and its therapy, on the skeleton. Bone l...
journal_title:Endocrinology and metabolism clinics of North America
pub_type: 杂志文章,评审
doi:10.1016/j.ecl.2007.07.008
更新日期:2007-12-01 00:00:00
abstract::Insulin-dependent diabetes mellitus is associated with a growing number of immune abnormalities. At the time of clinical onset, most patients developing the disease as children or young adults have autoantibodies reactive with islet beta cells. Current autoantibody markers for IDDM are not sufficient to predict the di...
journal_title:Endocrinology and metabolism clinics of North America
pub_type: 杂志文章,评审
doi:
更新日期:1991-09-01 00:00:00
abstract::Surgical intervention is a necessary part of a multimodal approach to pituitary lesions. This article summarizes the surgical approach to different neoplastic processes affecting the sellar region. ...
journal_title:Endocrinology and metabolism clinics of North America
pub_type: 杂志文章,评审
doi:10.1016/j.ecl.2007.10.006
更新日期:2008-03-01 00:00:00
abstract::In this article, the cellular and molecular mechanisms of diabetic complications have been reviewed. Hyperglycemia-induced mechanisms that may induce vascular dysfunction in specific sites of diabetic microvascular damage include increased polyol pathway flux, altered cellular redox state, increased formation of diacy...
journal_title:Endocrinology and metabolism clinics of North America
pub_type: 杂志文章,评审
doi:10.1016/s0889-8529(05)70324-8
更新日期:1996-06-01 00:00:00
abstract::Glucocorticoids are critical in the management of chronic, noninfectious, inflammatory diseases. Bone loss is a most devastating side effect of these powerful medications. Glucocorticoids produce bone loss by altering calcium metabolism, suppressing gonadal hormone production, and inhibiting bone formation. Now that t...
journal_title:Endocrinology and metabolism clinics of North America
pub_type: 杂志文章,评审
doi:10.1016/s0889-8529(05)70017-7
更新日期:1998-06-01 00:00:00
abstract::The liver is key to glucose homeostasis. Any disruption of its metabolism, structural integrity, or intracellular dynamics may alter the liver's ability to maintain normal glucose homeostasis. When such disruption affects hepatic glucose output, hypoglycemia may eventuate. Multiple drugs including alcohol may alter th...
journal_title:Endocrinology and metabolism clinics of North America
pub_type: 杂志文章,评审
doi:
更新日期:1989-03-01 00:00:00
abstract::There are many pathways that might lead to decreased bone mass, skeletal fragility, and increased fracture risk in osteoporosis. Some of these have been clearly identified, such as estrogen deficiency. Others that were conceived on the basis of experimental findings and recent scientific discoveries such as abnormalit...
journal_title:Endocrinology and metabolism clinics of North America
pub_type: 杂志文章,评审
doi:10.1016/s0889-8529(02)00055-5
更新日期:2003-03-01 00:00:00
abstract::Glucocorticoids mainly act through binding to cytosolic receptors that translocate to the nucleus after ligand binding, and dimerize to affect gene transcription in multiple fashions. The liganded receptors may interact with DNA at specific glucocorticoid responsive-elements, may physically hinder the ability of other...
journal_title:Endocrinology and metabolism clinics of North America
pub_type: 杂志文章,评审
doi:
更新日期:1994-09-01 00:00:00
abstract::Glucocorticoid-remediable aldosteronism (GRA) is a hereditary form of primary hyperaldosteronism and the most common monogenic cause of hypertension. A chimeric gene duplication leads to ectopic aldosterone synthase activity in the cortisol-producing zona fasciculata of the adrenal cortex, under the regulation of adre...
journal_title:Endocrinology and metabolism clinics of North America
pub_type: 杂志文章
doi:10.1016/j.ecl.2011.01.012
更新日期:2011-06-01 00:00:00
abstract::Somatostatin receptor ligands (octreotide and lanreotide) are currently first-line pharmacotherapy for patients with acromegaly in whom surgery fails to control the disease or cannot be considered. The efficacy of a new pan somatostatin receptor ligand, pasireotide, has been investigated as a potential treatment of ac...
journal_title:Endocrinology and metabolism clinics of North America
pub_type: 杂志文章,评审
doi:10.1016/j.ecl.2014.10.004
更新日期:2015-03-01 00:00:00
abstract::Adrenocortical carcinoma (ACC) is rare but one of the most malignant endocrine tumors. This article reviews and summarizes the current knowledge about the treatment of ACC. The epidemiology and molecular events involved in the pathogenesis of ACC are briefly outlined. The different diagnostic tools to distinguish beni...
journal_title:Endocrinology and metabolism clinics of North America
pub_type: 杂志文章,评审
doi:10.1016/j.ecl.2015.02.008
更新日期:2015-06-01 00:00:00
abstract::Dyslipidemia now is recognized as a significant potential adverse event in HIV-infected patients who are receiving antiretroviral therapy. HIV-infected persons who have hyperlipidemia should be managed similarly to those without HIV infection in accordance with the National Cholesterol Education Program. Providers mus...
journal_title:Endocrinology and metabolism clinics of North America
pub_type: 杂志文章,评审
doi:10.1016/j.ecl.2008.11.009
更新日期:2009-03-01 00:00:00
abstract::CVD is the primary cause of morbidity and mortality in patients who have diabetes mellitus. Most such patients have at least one lipid abnormality. Managing these complex lipid disorders is a crucial component of comprehensive diabetes mellitus care and limits the risk for cardiovascular morbidity and mortality. With ...
journal_title:Endocrinology and metabolism clinics of North America
pub_type: 杂志文章,评审
doi:10.1016/j.ecl.2004.11.004
更新日期:2005-03-01 00:00:00
abstract::An approach to the diagnosis and follow-up of patients with osteoporosis is described. A system of classification of vertebral fractures is outlined, and the relative merits of different techniques for bone mineral density are discussed. The importance of identifying factors that accelerate bone loss is stressed. The ...
journal_title:Endocrinology and metabolism clinics of North America
pub_type: 杂志文章,评审
doi:
更新日期:1988-09-01 00:00:00
abstract::In this article, an overview is presented of hypophysitis in terms of current clinical and experimental findings, with discussion of the anatomic and histopathologic classification of primary hypophysitis and factors associated with secondary hypophysitis. In addition, discusses the pathophysiology, clinical features,...
journal_title:Endocrinology and metabolism clinics of North America
pub_type: 杂志文章,评审
doi:10.1016/j.ecl.2014.10.011
更新日期:2015-03-01 00:00:00
abstract::A wide variety of medications, devices, and surgical interventions are available to patients who have ED. These range from first-line oral agents to second-line therapy with injections or vacuum devices to third-line options, such as penile prosthesis implantation. In this article, we cover available treatments for er...
journal_title:Endocrinology and metabolism clinics of North America
pub_type: 杂志文章,评审
doi:10.1016/j.ecl.2007.02.001
更新日期:2007-06-01 00:00:00
abstract::Based on the 2010 version, the 2017 World Health Organization (WHO 2017) classification is for pancreatic neuroendocrine neoplasms (PanNEN). The WHO 2017 classification introduces the novel well-differentiated neuroendocrine tumor of high grade (NET G3). A sharp distinction between NET and poorly differentiated neuroe...
journal_title:Endocrinology and metabolism clinics of North America
pub_type: 杂志文章,评审
doi:10.1016/j.ecl.2018.04.008
更新日期:2018-09-01 00:00:00
abstract::Lifestyle modification is the cornerstone treatment of type 2 diabetes in the obese patient, and is highly effective at promoting glucose regulation. However, many individuals struggle over time to maintain optimal glycemic control and/or body weight with lifestyle modification. Therefore, additional therapeutic appro...
journal_title:Endocrinology and metabolism clinics of North America
pub_type: 杂志文章,评审
doi:10.1016/j.ecl.2016.04.007
更新日期:2016-09-01 00:00:00