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https://www.readbyqxmd.com/read/27922496/metabolic-myopathies
#1
Mark A Tarnopolsky
PURPOSE OF REVIEW: Metabolic myopathies are genetic disorders that impair intermediary metabolism in skeletal muscle. Impairments in glycolysis/glycogenolysis (glycogen-storage disease), fatty acid transport and oxidation (fatty acid oxidation defects), and the mitochondrial respiratory chain (mitochondrial myopathies) represent the majority of known defects. The purpose of this review is to develop a diagnostic and treatment algorithm for the metabolic myopathies. RECENT FINDINGS: The metabolic myopathies can present in the neonatal and infant period as part of more systemic involvement with hypotonia, hypoglycemia, and encephalopathy; however, most cases present in childhood or in adulthood with exercise intolerance (often with rhabdomyolysis) and weakness...
December 2016: Continuum: Lifelong Learning in Neurology
https://www.readbyqxmd.com/read/27921434/-actual-trends-in-diagnostics-and-treatment-of-congenital-hyperinsulinism
#2
Juraj Staník, Martina Škopková, Monika Rosoľanková, Iwar Klimeš, Daniela Gašperíková
Congenital hyperinsulinism (CHI) is the most common cause of severe persistent hypoglycemia in neonates and infants. Early diagnosis and effective treatment (based on the principles of pharmacogenetics) play the key role for the prognosis. The DNA anlysis, which can identify mutation in one of the 11 genes causing MODY, is crutial in the diagnostics. Moreover, The genotype determines also the optimal therapy approach (medicaments, diet or rarely surgery). There was a large progress of novel medicaments treating particularly most severe (diazoxide-resistant) forms of CHI...
2016: Vnitr̆ní Lékar̆ství
https://www.readbyqxmd.com/read/27920549/management-of-hypoglycemia-in-nondiabetic-palliative-care-patients-a-prognosis-based-approach
#3
REVIEW
Victor C Kok, Ping-Hsueh Lee
Hypoglycemia due to underlying terminal illness in nondiabetic end-of-life patients receiving palliative care has not been fully studied. For example, we do not have adequate information on the frequency of spontaneous hypoglycemia in patients as occurs during the different stages of palliative care. Depending on the case-mix nature of the palliative care ward, at least 2% of palliative care patients may develop hypoglycemia near the end of life when the remaining life expectancy counts down in days. As many as 25%-60% of these patients will neither have autonomic response nor have neuroglycopenic symptoms during a hypoglycemic episode...
2016: Palliative Care
https://www.readbyqxmd.com/read/27918858/d10-in-the-treatment-of-prehospital-hypoglycemia-a-24-month-observational-cohort-study
#4
H Gene Hern, Matthew Kiefer, Derex Louie, Joseph Barger, Harrison J Alter
INTRODUCTION: Prehospital first responders historically have used an IV bolus of 50 mL of 50% dextrose solution (D50) for the treatment of hypoglycemia in the field. A local Emergency Medical Services (EMS) system recently approved a hypoglycemia treatment protocol of IV 10% dextrose solution (D10) due to occasional shortages and higher cost of D50. We use the experience of this EMS system to report the feasibility, safety, and efficacy of this approach. METHODS: Over the course of 104 weeks, paramedics treated 1,323 hypoglycemic patients with D10 and recorded patient demographics and clinical outcomes...
December 5, 2016: Prehospital Emergency Care
https://www.readbyqxmd.com/read/27918639/evaluation-of-an-outpatient-protocol-in-the-treatment-of-canine-parvoviral-enteritis
#5
Emilee C Venn, Karolina Preisner, Pedro L Boscan, David C Twedt, Lauren A Sullivan
OBJECTIVE: To compare 2 treatment protocols (standard in-hospital versus modified outpatient) in affecting the duration of treatment or survival of dogs with parvoviral enteritis. DESIGN: Prospective, randomized study. SETTING: University teaching hospital. ANIMALS: Client-owned dogs with naturally acquired parvovirus were randomized to receive either an inpatient (n = 20) or outpatient (n = 20) treatment protocol. INTERVENTIONS: Both groups received intravenous (IV) fluid resuscitation and correction of hypoglycemia at hospital admission...
December 5, 2016: Journal of Veterinary Emergency and Critical Care
https://www.readbyqxmd.com/read/27916774/non-islet-cell-tumor-hypoglycemia-caused-by-a-big-insulin-like-growth-factor-ii-producing-hepatocellular-carcinoma-an-autopsy-case-report
#6
Hirohito Naruse, Norihiko Shimoyama, Tsugumi Satoh, Yoshiya Yamamoto, Kazuteru Hatanaka, Taiki Kudo, Masayoshi Ohno, Kazuharu Suzuki, Hajime Hirata, Marina Suzuki
A 73-year-old man with a hepatocellular carcinoma was admitted to our hospital. He suffered from recurrent severe hypoglycemia. An autopsy was performed after his death. Anti-insulin-like growth factor II (IGF-II) monoclonal antibody immunostaining of the hepatocellular carcinoma was positive. Western immunoblot analysis of the serum revealed highly elevated IGF-II. Therefore, we diagnosed this case as a non-islet cell tumor hypoglycemia caused by a big IGF-II-producing hepatocellular carcinoma.
2016: Nihon Shokakibyo Gakkai Zasshi, the Japanese Journal of Gastro-enterology
https://www.readbyqxmd.com/read/27916054/-how-to-recognize-and-treat-hypoglycemia-in-type-2-diabetes
#7
J H Huang, Y Z Liang
No abstract text is available yet for this article.
December 1, 2016: Zhonghua Nei Ke za Zhi [Chinese Journal of Internal Medicine]
https://www.readbyqxmd.com/read/27914297/long-term-efficacy-and-safety-of-vildagliptin-add-on-therapy-in-type-2-diabetes-mellitus-with-insulin-treatment
#8
Ippei Kanazawa, Ken-Ichiro Tanaka, Masakazu Notsu, Sayuri Tanaka, Nobuaki Kiyohara, Sayo Koike, Yuko Yamane, Yuko Tada, Motofumi Sasaki, Mika Yamauchi, Toshitsugu Sugimoto
BACKGROUND: The use of dipeptidyl peptidase (DPP)-4 inhibitors in patients with type 2 diabetes treated with insulin may be beneficial. However, the long-term efficacy and safety of vildagliptin add-on therapy in these patients remains unclear. SUBJECTS AND METHODS: A total of 73 patients with type 2 diabetes treated with insulin were randomly assigned to receive either add-on therapy of vildagliptin (n=37) or conventional therapy without DPP-4 inhibitors (n=36) for glucose control...
November 20, 2016: Diabetes Research and Clinical Practice
https://www.readbyqxmd.com/read/27913575/lowest-glucose-variability-and-hypoglycemia-are-observed-with-the-combination-of-a-glp-1-receptor-agonist-and-basal-insulin-variation-study
#9
Harpreet S Bajaj, Karri Venn, Chenglin Ye, Avril Patrick, Shivani Kalra, Hasnain Khandwala, Nadeem Aslam, David Twum-Barima, Ronnie Aronson
OBJECTIVE: There is a dearth of published literature comparing glucose variability (GV) between different insulin regimens in type 2 diabetes. This cohort study compares GV using continuous glucose monitoring (CGM) in patients with well-controlled type 2 diabetes using four common insulin regimens: basal insulin + oral drugs (BO), basal insulin + glucagon-like peptide 1 receptor agonist (GLP-1 RA) (BGLP), premixed insulin (PM), and basal-bolus insulin (BB). RESEARCH DESIGN AND METHODS: Consecutive patients from three endocrinology clinics who met study criteria-type 2 diabetes, age 18 to 80 years, BMI ≤ 45 kg/m(2), stable insulin regimen for a minimum of 6 months, and stable A1C value ≤7...
December 2, 2016: Diabetes Care
https://www.readbyqxmd.com/read/27913302/to-the-editor-combined-immunodeficiency-and-hypoglycemia-associated-with-mutations-in-hypoxia-up-regulated-1
#10
Emma M Haapaniemi, Christopher L Fogarty, Salla Keskitalo, Shintaro Katayama, Helena Vihinen, Mette Ilander, Satu Mustjoki, Kaarel Krjutškov, Markku Lehto, Timo Hautala, Ove Eriksson, Eija Jokitalo, Vidya Velagapudi, Markku Varjosalo, Mikko Seppänen, Juha Kere
We describe recessive mutations in hypoxia up-regulated 1 (HYOU1) in a patient that presented with generalized susceptibility to bacterial and herpetic infections as well as hypoglycemic episodes.
November 29, 2016: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/27909992/roles-for-orexin-hypocretin-in-the-control-of-energy-balance-and-metabolism
#11
Paulette B Goforth, Martin G Myers
The neuropeptide hypocretin is also commonly referred to as orexin, since its orexigenic action was recognized early. Orexin/hypocretin (OX) neurons project widely throughout the brain and the physiologic and behavioral functions of OX are much more complex than initially conceived based upon the stimulation of feeding. OX most notably controls functions relevant to attention, alertness, and motivation. OX also plays multiple crucial roles in the control of food intake, metabolism, and overall energy balance in mammals...
December 2, 2016: Current Topics in Behavioral Neurosciences
https://www.readbyqxmd.com/read/27908250/hypoglycemia-and-cardiac-arrhythmia-mechanisms-evidence-base-and-current-recommendations
#12
Sitara Gulrukh Khan, Mohammed Shahriar Huda
BACKGROUND: With the use of intensive insulin therapy and insulin secretagogues to optimize glycemic control in diabetes, hypoglycemia continues to present a clinical challenge. Hypoglycemia has been implicated in nocturnal sudden death in type 1 diabetes, and the mechanism underlying this is postulated to be cardiac arrhythmia. OBJECTIVE: This article reviews the evidence surrounding hypoglycemia and cardiac arrhythmia. METHODS: A structured search of the Pubmed bibliographic database was undertaken, and relevant peer-reviewed articles on the topic were included in the review...
December 1, 2016: Current Diabetes Reviews
https://www.readbyqxmd.com/read/27906953/what-s-tramadol-got-to-do-with-it-a-case-report-of-rebound-hypoglycemia-a-reappraisal-and-review-of-potential-mechanisms
#13
Charles A Odonkor, Akhil Chhatre
BACKGROUND: Tramadol has gained traction as an analgesic of choice among pain practicing physicians. However some concerns regarding a previously unlabeled adverse reaction - hypoglycemia - have cast it in a dim light. Prior reports have noted an associated risk of hospitalization for hypoglycemia after tramadol use, but whether tramadol is the main causal agent is poorly understood and the underlying mechanisms are not well delineated. We present a unique case of rebound hypoglycemia as a variation of the theme of tramadol's adverse effect profile in a patient with type 1 diabetes mellitus, and reappraise potential mechanisms underlying this underappreciated phenomenon...
November 2016: Pain Physician
https://www.readbyqxmd.com/read/27906880/hypoglycemia-in-patients-with-insulin%C3%A2-treated-diabetes
#14
Janusz Gumprecht, Katarzyna Nabrdalik
Hypoglycemia is the major barrier for optimal glycemic control in patients on maintenance insulin therapy. It is widely known that good glycemic control leads to prevention of or delay in the development of microvascular complications, and can reduce macrovascular events. It is thought that hypoglycemia may predispose patients to cognitive deterioration and may negatively affect the cardiovascular system. Hypoglycemia per se can contribute to a blunted counterregulatory response and disabling hypoglycemia, while hypoglycemia avoidance restores normal response to low blood glucose levels...
October 11, 2016: Polskie Archiwum Medycyny Wewnętrznej
https://www.readbyqxmd.com/read/27906547/late-diagnosis-of-pomc-deficiency-and-in-vitro-evidence-of-residual-translation-from-allele-with-c-11c-a-mutation
#15
Aleksandra S Anisimova, Petr M Rubtsov, Kseniya A Akulich, Sergey E Dmitriev, Elena Frolova, Anatoly Tiulpakov
CONTEXT: Loss-of-function mutations in the POMC gene are associated with the syndrome of adrenal insufficiency, obesity and red hair. We describe here a case of POMC deficiency, in which adrenal insufficiency was not treated until the fourth year of life. One of the disease-causative POMC mutations was characterized in vitro using a new approach. CASE DESCRIPTION: A boy presented in the first year of life with red hair, growth acceleration, moderate obesity and recurrent cholestasis, which was followed by two episodes of hypoglycemia at ages of 1...
December 1, 2016: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/27905899/a-case-of-extragastrointestinal-stromal-tumor-complicated-by-severe-hypoglycemia-a-unique-presentation-of-a-rare-tumor
#16
Zeb Saeed, Solaema Taleb, Carmella Evans-Molina
BACKGROUND: Non-Islet Cell Tumor Hypoglycemia (NICTH) is a rare paraneoplastic cause of hypoglycemia arising from excess tumor production of insulin-like growth factor. The objective of this report is to describe an unusual case of Extragastrointestinal Stromal Tumor (EGIST) associated NICTH. CASE PRESENTATION: A 64 year-old African female was brought to the emergency room with a 1-month history of recurrent syncope, weight loss, and abdominal bloating. Serum blood glucose was discovered to 39 mg/dL, when insulin, proinsulin, and C-peptide were suppressed...
December 1, 2016: BMC Cancer
https://www.readbyqxmd.com/read/27904111/restoration-of-the-hypothalamic-pituitary-adrenal-response-to-hypoglycemia-in-type-2-diabetes-by-avoiding-chronic-hypoglycemia
#17
Shin-Ichi Tsuda, Kazunori Konishi, Toshiki Otoda, Takako Nagai, Ai Takeda-Watanabe, Megumi Kanasaki, Munehiro Kitada, Atsushi Nakagawa, Makoto Nishizawa, Keizo Kanasaki, Daisuke Koya
An impaired ability to sense and respond to drug-induced hypoglycemia is a common and serious complication in diabetic patients. The hypothalamic-pituitary-adrenal (HPA) axis activity plays a critical role in the counterregulatory response to hypoglycemia. We herein report a case that experienced restoration of a blunted HPA axis by avoiding hypoglycemia with the use of the DPP-4 inhibitor sitagliptin.
2016: Internal Medicine
https://www.readbyqxmd.com/read/27903941/prep-t1-preteen-re-education-with-parents-type-1-diabetes-feasibility-intervention-results
#18
Susan Sullivan-Bolyai, Sybil Crawford, Kim Johnson, Neesha Ramchandani, Diane Quinn, Bianca D'Alesandro, Kailyn Stern, Terry Lipman, Gail Melkus, Randi Streisand
There has been a 2% to 3% increase in Type 1 diabetes (T1D) in children below 11 years old. Preteens (9-12 years old) with T1D are often overlooked regarding future diabetes self-management (DSM) expectations because parents are still in the "driver's seat." The study purpose was to explore feasibility/ability to recruit and conduct a two-arm trial on reeducation, collaboration, and social support. One component of DSM was reviewed (hypoglycemia) with preteens (n = 22) and parents (n = 22). The experimental preteens discussed hypoglycemia management with a teen mentor and nurse educator using a human patient simulator for practice, and working collaboratively with parents...
November 2016: Journal of Family Nursing
https://www.readbyqxmd.com/read/27899870/cognition-and-type-1-diabetes-in-children-and-adolescents
#19
Allison Cato, Tamara Hershey
IN BRIEF In children and adolescents with type 1 diabetes, exposure to glycemic extremes (severe hypoglycemia, chronic hyperglycemia, and diabetic ketoacidosis) overlaps with the time period of most active brain and cognitive development, leading to concerns that these children are at risk for cognitive side effects. This article summarizes the existing literature examining the impact of glycemic extremes on cognitive function and brain structure in youth with type 1 diabetes and points out areas for future research...
November 2016: Diabetes Spectrum: a Publication of the American Diabetes Association
https://www.readbyqxmd.com/read/27899494/incidence-trends-and-predictors-of-hospitalization-for-hypoglycemia-in-17-230-adult-patients-with-type-1-diabetes-a-danish-register-linkage-cohort-study
#20
Kazi Ishtiak-Ahmed, Bendix Carstensen, Ulrik Pedersen-Bjergaard, Marit E Jørgensen
OBJECTIVE: This study aimed to examine nationwide incidence trends and predictors of hospitalization for hypoglycemia (HH) in the adult population with type 1 diabetes in Denmark. RESEARCH DESIGN AND METHODS: All 17,230 patients with type 1 diabetes aged 16 years and above registered in the Danish Adult Diabetes Database (DADD) from 2006 were followed to 2012 by linkage of registers. Incidence rates of HH were modeled by Poisson regression by calendar time, taking sex, age, diabetes duration, clinical variables, and previous HH into account...
November 29, 2016: Diabetes Care
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