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https://www.readbyqxmd.com/read/27910218/octreotide-use-and-safety-in-infants-with-hyperinsulinism
#1
Ann W McMahon, Gerold T Wharton, Paul Thornton, Diva D De Leon
BACKGROUND: Octreotide is a synthetic peptide analog of naturally occurring somatostatin. Octreotide is used off-label in children <6 years of age for hyperinsulinism, chylothorax, and gastrointestinal bleeding. There is a lack of controlled data on efficacy or potential adverse events from this off-label use. METHODS: Three pediatric hospitals participated in this study. Patients were hospitalized January 2007-December 2010 and administered octreotide for congenital hyperinsulinism (CHI) at least 1 day...
December 2, 2016: Pharmacoepidemiology and Drug Safety
https://www.readbyqxmd.com/read/27908292/conservatively-treated-congenital-hyperinsulinism-chi-due-to-k-atp-channel-gene-mutations-reducing-severity-over-time
#2
Maria Salomon-Estebanez, Sarah E Flanagan, Sian Ellard, Lindsey Rigby, Louise Bowden, Zainab Mohamed, Jacqueline Nicholson, Mars Skae, Caroline Hall, Ross Craigie, Raja Padidela, Nuala Murphy, Tabitha Randell, Karen E Cosgrove, Mark J Dunne, Indraneel Banerjee
BACKGROUND: Patients with Congenital Hyperinsulinism (CHI) due to mutations in K-ATP channel genes (K-ATP CHI) are increasingly treated by conservative medical therapy without pancreatic surgery. However, the natural history of medically treated K-ATP CHI has not been described; it is unclear if the severity of recessively and dominantly inherited K-ATP CHI reduces over time. We aimed to review variation in severity and outcomes in patients with K-ATP CHI treated by medical therapy. METHODS: Twenty-one consecutively presenting patients with K-ATP CHI with dominantly and recessively inherited mutations in ABCC8/KCNJ11 were selected in a specialised CHI treatment centre to review treatment outcomes...
December 1, 2016: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/27905047/endoscopic-ultrasound-guided-fine-needle-aspiration-biopsy-eus-fna-in-insulinomas-indications-and-clinical-relevance-in-a-single-investigator-cohort-of-47-patients
#3
Peter Herbert Kann, Roland Moll, Detlef Bartsch, Andreas Pfützner, Thomas Forst, Gianluca Tamagno, Joachim N Goebel, Verena Fourkiotis, Simona Raluca Bergmann, Maike Collienne
PURPOSE: This study was aimed to investigate the role and relevance of endoscopic ultrasound-guided fine-needle aspiration biopsy in the diagnostic work-up of insulinomas. METHODS: We have analysed the frequency, clinical indications, success rate (obtaining diagnostic tissue), diagnostic accuracy (in comparison to the pathological diagnosis after surgery), complications, and tolerability of endoscopic ultrasound-guided fine-needle aspiration biopsy and the localization and size of the lesions in 47 consecutive patients (29 females, 18 males; 46 ± 15 years) who had surgery for insulinoma following fasting test and were explored by single investigator EUS 1994-2015...
November 30, 2016: Endocrine
https://www.readbyqxmd.com/read/27808506/identification-of-a-novel-activator-of-mammalian-glutamate-dehydrogenase
#4
Hong Qiu Smith, Thomas James Smith
Glutamate dehydrogenase (GDH) catalyzes the oxidative deamination of L-glutamate and in animals is highly regulated. GDH in Hyperinsulinism/hyperammonemia syndrome (HHS) patients lack GTP inhibition resulting in hypersecretion of insulin upon protein consumption. This suggests insulin secretion could be stimulated with GDH activators. A high throughput screen yielded one potent activator N1-[4-(2-aminopyrimidin-4-yl)phenyl]-3-(trifluoromethyl)benzene-1-sulfonamide (75-E10). 75-E10 is ~1,000 fold more efficacious than the synthetic activator, BCH, and is at least as effective as ADP...
November 3, 2016: Biochemistry
https://www.readbyqxmd.com/read/27795707/arterial-calcium-stimulation-with-hepatic-venous-sampling-in-the-localization-diagnosis-of-endogenous-hyperinsulinism
#5
Paloma Moreno-Moreno, María Rosa Alhambra-Expósito, Aura Dulcinea Herrera-Martínez, Rafel Palomares-Ortega, Luis Zurera-Tendero, Juan José Espejo Herrero, María Angeles Gálvez-Moreno
Objective. The aim of this study was to assess the utility of arterial calcium stimulation with hepatic venous sampling (ASVS) in the localization diagnosis of endogenous hyperinsulinism. Patients and Methods. A retrospective descriptive study was performed including patients with endogenous hyperinsulinism who underwent ASVS. The histopathological diagnosis in patients who underwent a surgical procedure was used as the reference for the statistical study of the accuracy of this technique. Results. 30 patients were included with endogenous hyperinsulinism and nonconclusive imaging diagnosis was included...
2016: International Journal of Endocrinology
https://www.readbyqxmd.com/read/27777708/persistent-hyperinsulinism-in-kabuki-syndrome-2-case-report-and-literature-review
#6
Hobia Gole, Raymond Chuk, David Coman
Kabuki syndrome is a clinically and genetically heterogeneous congenital malformation syndrome with protean clinical manifestations. This reflects the important epigenetic role in embryonic development of the two genes currently known to be associated with Kabuki syndrome i.e., KMT2D and KDM6A, which are responsible for Kabuki syndrome 1 and Kabuki syndrome 2, respectively. Hypoglycemia is thought to be a rare manifestation of Kabuki syndrome; however it may be under diagnosed. Herein we describe the case of a 5-year-old girl with Kabuki syndrome 2 in whom persistent hyperinsulinism was diagnosed at 4 years of age...
August 8, 2016: Clinics and Practice
https://www.readbyqxmd.com/read/27754802/diagnosis-of-abcc8-congenital-hyperinsulinism-of-infancy-in-a-20-year-old-man-evaluated-for-factitious-hypoglycemia
#7
Amichai Gutgold, David J Gross, Benjamin Glaser, Auryan Szalat
CONTEXT: Hypoglycemia is a rare event in healthy adults, and the differential diagnosis includes many diseases some of which are rare and easily missed. Description of the case: A 20 year-old male military paramedic was referred to our Emergency Department for the investigation of recurrent hypoglycemia episodes during the last months. Factitious hypoglycemia was excluded and organic hyperinsulinemic hypoglycemia was diagnosed by prolonged fast. Imaging studies (endoscopic ultrasound and triple-phase computed tomography) were normal...
October 18, 2016: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/27753189/congenital-hypoglycemia-disorders-new-aspects-of-etiology-diagnosis-treatment-and-outcomes-highlights-of-the-proceedings-of-the-congenital-hypoglycemia-disorders-symposium-philadelphia-april-2016
#8
Diva D De Leon, Charles A Stanley
Hypoglycemia continues to be an important cause of morbidity in neonates and children. Prompt diagnosis and management of the underlying hypoglycemia disorder is critical for preventing brain damage and improving outcomes. Congenital hyperinsulinism (HI) is the most common and severe cause of persistent hypoglycemia in neonates and children. Recent discoveries of the genetic causes of HI have improved our understanding of the pathophysiology, but its management is complex and requires the integration of clinical, biochemical, molecular, and imaging findings to establish the appropriate treatment according to the subtype...
October 18, 2016: Pediatric Diabetes
https://www.readbyqxmd.com/read/27747338/robust-regulation-of-hepatic-pericentral-amination-by-glutamate-dehydrogenase-kinetics
#9
Soumen Bera, Sanjay Lamba, Mubasher Rashid, Anuj K Sharma, Alexander B Medvinsky, Claudia Acquisti, Amit Chakraborty, Bai-Lian Li
Impaired glutamate dehydrogenase (GDH) sensitivity to its inhibitors causes excessive insulin secretion by pancreatic beta-cells and defective ammonia metabolism in the liver. These symptoms are commonly associated with hyperinsulinism/hyperammonemia syndrome (HI/HA), which causes recurrent hypoglycaemia in early infancy. Hepatic localization of GDH amination and deamination activities linked with the urea cycle is known to be involved in ammonia metabolism and detoxification. Although deamination activities of hepatic GDH in the periportal zones of liver lobules and its connection to the urea cycle have been exhaustively investigated, physiological roles of GDH amination activity observed at pericentral zones have often been overlooked...
October 17, 2016: Integrative Biology: Quantitative Biosciences From Nano to Macro
https://www.readbyqxmd.com/read/27691052/mtor-inhibitors-for-the-treatment-of-severe-congenital-hyperinsulinism-perspectives-on-limited-therapeutic-success
#10
Marie Szymanowski, Maria Salomon Estebanez, Raja Padidela, Bing Han, Karolina Mosinska, Adam Stevens, Lena Damaj, Florence Pihan-Le Bars, Emilie Lascouts, Rachel Reynaud, Catherine Ferreira, Claire Bansept, Pascale de Lonlay, Cécile Saint-Martin, Mark J Dunne, Indraneel Banerjee, Jean-Baptiste Arnoux
CONTEXT: Congenital hyperinsulinism (CHI) is the commonest cause of persistent hypoglycemia in neonates and infants. In medically unresponsive CHI, subtotal pancreatectomy is performed to achieve euglycaemia with consequent diabetes in later life. Sirolimus, a mammalian target of rapamycin (mTOR) inhibitor has been reported to obviate the need for pancreatectomy, but experience is limited. OBJECTIVE: We have investigated the efficacy and adverse effect profile of mTOR inhibitors in the treatment of severe CHI...
October 3, 2016: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/27682711/genetic-characteristics-and-long-term-follow-up-of-11-patients-with-congenital-hyperinsulinism-followed-in-a-single-center
#11
Katharina Warncke, Franziska Falco, Wolfgang Rabl, Ilse Engelsberger, Julia Saier, David Flores-Rodriguez, Stefan Burdach, Walter Bonfig
BACKGROUND: Congenital hyperinsulinism (CHI) is a rare disease with an estimated incidence of 1:40,000 live births. Here, we characterize 11 patients treated at Munich Children's Hospital Schwabing. METHODS: We analyzed data on birth, treatment and laboratory results including genetic testing and evaluated the long-term course with a follow-up visit. RESULTS: All patients had severe, diazoxide-(DZX)-resistant hypoglycemia, beginning immediately after birth...
October 1, 2016: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://www.readbyqxmd.com/read/27681997/molecular-and-clinical-features-of-katp-channel-neonatal-diabetes-mellitus-in-japan
#12
Yukiko Hashimoto, Sumito Dateki, Masakazu Hirose, Kenichi Satomura, Hirotake Sawada, Haruo Mizuno, Shigetaka Sugihara, Koichi Maruyama, Tatsuhiko Urakami, Hidenori Sugawara, Kenji Shirai, Tohru Yorifuji
BACKGROUND: There are few reports pertaining to Asian patients with neonatal diabetes mellitus (NDM) caused by activating mutations in the ATP-sensitive potassium channel genes (KATP-NDM). OBJECTIVES: To elucidate the characteristics of Japanese patients with KATP-NDM. METHODS: By the amplification and direct sequencing of all exons and exon-intron boundaries of the KCNJ11 and ABCC8 genes, 25 patients with KATP-NDM were identified from a total of 70 patients with NDM...
September 29, 2016: Pediatric Diabetes
https://www.readbyqxmd.com/read/27672547/coffin-siris-syndrome-with-caf%C3%A3-au-lait-spots-obesity-and-hyperinsulinism-caused-by-a-mutation-in-the-arid1b-gene
#13
Fatma Mujgan Sonmez, Eyyup Uctepe, Mehmet Gunduz, Zeliha Gormez, Seval Erpolat, Murat Oznur, Mahmut Samil Sagiroglu, Huseyin Demirci, Esra Gunduz
Coffin-Siris syndrome (CSS) (MIM 135900) is characterized by developmental delay, severe speech impairment, distinctive facial features, hypertrichosis, aplasia or hypoplasia of the distal phalanx or nail of the fifth digit and agenesis of the corpus callosum. Recently, it was shown that mutations in the ARID1B gene are the main cause of CSS, accounting for 76% of identified mutations. Here, we report a 15 year-old female patient who was admitted to our clinic with seizures, speech problems, dysmorphic features, bilaterally big, large thumb, café-au-lait (CAL) spots, obesity and hyperinsulinism...
August 2016: Intractable & Rare Diseases Research
https://www.readbyqxmd.com/read/27627078/-hypoglycemia-as-a-symptom-of-cancer-in-adults
#14
Zdeněk Fryšák, David Karásek
UNLABELLED: Decrease of blood glucose levels below 3 mmol/l is in fully developed cases accompanied by neuroglycopenic symptoms that may even lead to altered state of consciousness. The treating physician frequently faces a complicated situation. This may be due to inappropriately administered drugs including cases motivated by self-harm intentions (insulin, insulin secretagogues), or alcohol abuse. Undernourished people, or those afflicted with a serious systemic infection, end-stage liver or kidney diseases or with a failing heart, belong to a risk group...
2016: Vnitr̆ní Lékar̆ství
https://www.readbyqxmd.com/read/27624297/the-treatment-of-hyperinsulinemic-hypoglycaemia-in-adults-an-update
#15
M V Davi, A Pia, V Guarnotta, G Pizza, A Colao, A Faggiano
BACKGROUND: Treatment of hyperinsulinemic hypoglycaemia (HH) is challenging due to the rarity of this condition and the difficulty of differential diagnosis. The aim of this article is to give an overview of the recent literature on the management of adult HH. METHODS: A search for reviews, original articles, original case reports between 1995 and 2016 in PubMed using the following keywords: hyperinsulinemic hypoglycaemia, insulinoma, nesidioblastosis, gastric bypass, autoimmune hypoglycaemia, hyperinsulinism, treatment was performed...
September 13, 2016: Journal of Endocrinological Investigation
https://www.readbyqxmd.com/read/27573238/pharmacological-correction-of-trafficking-defects-in-atp-sensitive-potassium-channels-caused-by-sulfonylurea-receptor-1-mutations
#16
Gregory M Martin, Emily A Rex, Prasanna Devaraneni, Jerod S Denton, Kara E Boodhansingh, Diva D DeLeon, Charles A Stanley, Show-Ling Shyng
ATP-sensitive potassium (KATP) channels play a key role in mediating glucose-stimulated insulin secretion by coupling metabolic signals to β-cell membrane potential. Loss of KATP channel function due to mutations in ABCC8 or KCNJ11, genes encoding the sulfonylurea receptor 1 (SUR1) or the inwardly rectifying potassium channel Kir6.2, respectively, results in congenital hyperinsulinism. Many SUR1 mutations prevent trafficking of channel proteins from the endoplasmic reticulum to the cell surface. Channel inhibitors, including sulfonylureas and carbamazepine, have been shown to correct channel trafficking defects...
October 14, 2016: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/27557839/how-to-use-a-controlled-fast-to-investigate-hypoglycaemia
#17
S Sreekantam, M A Preece, S Vijay, J Raiman, S Santra
Controlled fasts can play a valuable role in the diagnosis and management of hypoglycaemia in paediatric clinical practice, but are no substitute for the collecting of appropriate critical samples at the time of hypoglycaemia for metabolic and endocrine studies. Fatty acid oxidation defects, hyperinsulinism and adrenal insufficiency should always be excluded prior to organising controlled fasts. Controlled fasts are safe if conducted in an experienced setting with strict protocols in place. Failure to adhere to protocol can defeat the purpose of the study and can potentially be dangerous...
August 24, 2016: Archives of Disease in Childhood. Education and Practice Edition
https://www.readbyqxmd.com/read/27555915/a-case-of-low-serum-insulin-levels-in-a-patient-with-insulinoma
#18
Chun-Han Lo, Ding-Ping Sun
UNLABELLED: Insulinomas are the most common cause of hypoglycemia resulting from endogenous hyperinsulinism. Traditionally, inappropriately elevated levels of insulin in the face of hypoglycemia are the key to diagnosis. However, contradictory levels of insulin and C-peptide do not necessarily exclude the diagnosis. A 50-year-old female was brought to our emergency department because of conscious disturbance on the previous night. She had no history of diabetes mellitus, and was not using any medications or alcohol...
2016: Endocrinology, Diabetes & Metabolism Case Reports
https://www.readbyqxmd.com/read/27552834/prolonged-episodes-of-hypoglycaemia-in-hnf4a-mody-mutation-carriers-with-igt-evidence-of-persistent-hyperinsulinism-into-early-adulthood
#19
S Bacon, M P Kyithar, E M Condron, N Vizzard, M Burke, M M Byrne
AIMS: HNF4A is an established cause of maturity onset diabetes of the young (MODY). Congenital hyperinsulinism can also be associated with mutations in the HNF4A gene. A dual phenotype is observed in HNF4A-MODY with hyperinsulinaemic hypoglycaemia in the neonatal period progressing to diabetes in adulthood. The nature and timing of the transition remain poorly defined. We performed an observational study to establish changes in glycaemia and insulin secretion over a 6-year period. We investigated glycaemic variability and hypoglycaemia in HNF4A-MODY using a continuous glucose monitoring system (CGMS)...
August 23, 2016: Acta Diabetologica
https://www.readbyqxmd.com/read/27466042/a-novel-factor-for-primary-arteriovenous-fistula-failure-hyperinsulinism
#20
Davut Akin, Sehmus Ozmen, Ramazan Kaya
INTRODUCTION: Dysfunction of vascular access is an important reason of morbidity for dialysis patients and it is a major factor affecting the economical burden of hemodialysis. The preferred type of vascular access is creation of an arteriovenous fistula (AVF). However, the problem of fistula maturation rate is still a challenge. Herein, we tried to search the role of hyperinsulinism and insulin resistance as a new predictor of primary AVF failure (pAVFF) that may be a cause of intimal damage...
September 2016: Renal Failure
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