keyword
https://read.qxmd.com/read/36835815/report-of-a-novel-molecular-profile-in-malignant-insulinoma
#21
JOURNAL ARTICLE
Laura Burns, Bita Naimi, Matthew Ronan, Huihong Xu, Horst Christian Weber
Pancreatic neuroendocrine tumors, or pNETs, represent a rare and clinically heterogenous subset of pancreatic neoplasms. One such pNET, the insulinoma, is found to be malignant in just 4% of all insulinomas. Due to the exceedingly uncommon occurrence of these tumors, there is controversy regarding the optimal evidence-based management for these patients. We therefore report on a 70-year-old male patient admitted with 3 months of episodic confusion with concurrent hypoglycemia. The patient was found to have inappropriately elevated endogenous insulin levels during these episodes, and somatostatin-receptor subtype 2 selective imaging revealed a pancreatic mass metastatic to local lymph nodes, spleen, and the liver...
February 6, 2023: Journal of Clinical Medicine
https://read.qxmd.com/read/36798937/a-newborn-with-a-pathogenic-variant-in-asxl2-expanding-the-phenotype-of-shapns-a-case-report-and-literature-review
#22
Meng Yuan, Yuanyuan Shan, Fanshu Xu, Lin Yang, Chengjun Sun, Ruoqian Cheng, Bingbing Wu, Zhehuan Zhang, Yun Cao, Rong Zhang, Wenhao Zhou, Guoqiang Cheng, Liyuan Hu
BACKGROUND: Shashi-Pena syndrome (SHAPNS) is a developmental disorder caused by mutations in additional sex combs-like Protein 2 ( ASXL2 ). Since 2016, only 12 cases from 10 families have been reported. However, neonatal period characteristics remain largely unknown. Herein, we report a case with a pathogenic variant in ASXL2 in a newborn. CASE DESCRIPTION: A newborn was diagnosed with a previously unreported de novo truncating mutation in ASXL2 (NM_018263.6) at 21 days and the clinical characteristics of all probands with ASXL2-related SHAPNS was reported in the literature...
January 31, 2023: Translational Pediatrics
https://read.qxmd.com/read/36742395/big-insulin-like-growth-factor-2-producing-multiple-solitary-fibrous-tumors-treated-with-debulking-surgery-a-case-report
#23
Yamato Keidai, Takaaki Murakami, Nana Yamamura, Shigeru Tsunoda, Atsushi Ikeda, Koya Hida, Mototsugu Nagao, Yosuke Yamada, Ayaka Fukui, Masahito Ogura, Izumi Fukuda, Yuji Nakamoto, Kazutaka Obama, Nobuya Inagaki
BACKGROUND: Non-islet cell tumor hypoglycemia (NICTH) is a rare paraneoplastic syndrome caused by a tumor-producing high molecular weight form of insulin-like growth factor 2 (IGF2) known as big IGF2. The only curative treatment for this condition is surgical resection of the responsible tumors. However, this may not be feasible in cases with multiple metastases at diagnosis of NICTH, and no standard treatment strategy for multiple tumors has been established. The effects of pharmacological therapies including somatostatin analogs are often inefficient and remain difficult to predict...
2023: Frontiers in Endocrinology
https://read.qxmd.com/read/36620829/predictors-of-the-need-to-use-medications-in-the-management-of-neonatal-hypoglycemia
#24
JOURNAL ARTICLE
Samira Al Housni, Khadija Al Ghaithi, Sathiya P Murthi, Hussain Alsaffar, Hilal Al Mandhari
Background and objective Neonatal hypoglycemia (NH) is one of the most common causes of admission to the neonatal intensive care unit (NICU). Persistent NH despite adequate feeding and intravenous dextrose may often require medications to maintain normal blood glucose levels (BGL). Several medications are used in the management of persistent NH, such as glucagon, diazoxide, and octreotide. In this study, we aimed to determine the factors that predict the need for medications to treat persistent NH. Methods This was a retrospective cohort study conducted at the Sultan Qaboos University Hospital (SQUH), Muscat, Oman...
December 2022: Curēus
https://read.qxmd.com/read/36494838/case-presentation-of-8-year-follow-up-of-recurrent-malignant-duodenal-insulinoma-and-lymph-node-metastases-and-literature-review-of-malignant-insulinoma-management
#25
REVIEW
Michelle P Walker, Vikram Shenoy, David C Metz, Charles A Stanley, Douglas Fraker, Vinay Chandrasekhara, Anastassia Amaro
BACKGROUND: Insulinoma is an uncommon insulin-secreting neuroendocrine tumor that presents with severe recurrent hypoglycemia. Although cases of extrapancreatic insulinomas have been reported, the majority of insulinomas occur in the pancreas. The number of reported cases of ectopic insulinomas with follow-up assessments is limited and they do not report disease recurrence. The current report presents the first documented case of recurrent extrapancreatic insulinoma with 8 years of follow-up, provides relevant literature review, and proposes surveillance and treatment strategies...
December 9, 2022: BMC Endocrine Disorders
https://read.qxmd.com/read/36409572/clinical-and-laboratory-evaluation-of-children-with-congenital-hyperinsulinism-a-single-center-experience
#26
JOURNAL ARTICLE
Semra Gundogdu, Mustafa Ciftci, Enver Atay, Akif Ayaz, Omer Ceran, Zeynep Atay
OBJECTIVES: To evaluate and present the data regarding clinical, laboratory, radiological and the results of molecular genetic analysis of patients with hyperinsulinemic hypoglycemia in our clinics. METHODS: A total of 9 patients with CHI followed at Istanbul Medipol University. Data related to gender, age at presentation, birth weight, gestational age, consanguinity, glucose and insulin levels at diagnosis, treatment modalities, response to treatment, the results of genetic analysis and radiological evaluation were gathered from the files...
November 22, 2022: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://read.qxmd.com/read/36377811/medical-and-surgical-treatment-of-postbariatric-hypoglycaemia-retrospective-data-from-daily-practice
#27
JOURNAL ARTICLE
Loek J M de Heide, Sterre H T Wouda, Vincent J T Peters, Mirjam Oosterwerff-Suiker, Victor A Gerdes, Marloes Emous, André P van Beek
AIM: To evaluate medical and surgical treatment of postbariatric hypoglycaemia (PBH) in daily practice. MATERIALS AND METHODS: Retrospective data were extracted from medical records from four hospitals. PBH was defined by neuroglycopenic symptoms together with a documented glucose <3.0 mmol/L in the postprandial setting after previous bariatric surgery. Data were scored semiquantitatively on efficacy and side effects by two reviewers independently. Duration of efficacy and of use were calculated...
March 2023: Diabetes, Obesity & Metabolism
https://read.qxmd.com/read/36282178/home-management-of-pediatric-sulfonylurea-ingestions
#28
REVIEW
Courtney Temple, Ruby Hoang, Shana Kusin
INTRODUCTION: Sulfonylureas are oral antidiabetic medications that act by stimulating insulin release from pancreatic beta cells. Unintentional pediatric ingestions may result in hypoglycemia. While guidelines often recommend up to a 24-hour hospital observation period for any ingestion, the Oregon Poison Center has historically managed select patients at home. This study aimed to describe outcomes of home-managed pediatric sulfonylurea exposures and characteristics of ingestions that are appropriate for home monitoring...
November 2022: Clinical Toxicology
https://read.qxmd.com/read/36248221/functional-localization-of-adult-onset-idiopathic-nesidioblastosis
#29
Jess C Hercus, Pouneh Pasha, Sadiq Al Lawati, Peter Kim, Andre Mattman, Douglas Webber, David M Thompson
Nesidioblastosis is a rare pancreatic disorder involving enlarged beta cells throughout the pancreas, causing elevated insulin production. We present the case of a 53-year-old woman with the initial symptom of fasting hypoglycemia. No pancreatic lesions were indicated on computed tomography and magnetic resonance imaging scans, and an octreotide scan was negative for insulinoma. Selective arterial calcium stimulation (SACST) showed increased insulin production from the stimulation of 3 out of 5 arteries. The SACST results suggested a diagnosis of nesidioblastosis, which was confirmed by histopathology after a subtotal distal pancreatectomy...
2022: Case Reports in Endocrinology
https://read.qxmd.com/read/36187120/case-report-a-particularly-rare-case-of-endogenous-hyperinsulinemic-hypoglycemia-complicated-with-pregnancy-treated-with-short-acting-somatostatin-analog-injections
#30
Ádám Barsi, Artúr Beke, Beatrix Sármán
Hyperinsulinemic hypoglycemia is a rare disease, and only two cases complicated with pregnancy were published previously when our patient became pregnant. We introduce a successful management of a pregnancy in a patient with endogenous hyperinsulinemic hypoglycemia, a condition also known as non-insulinoma pancreatogenous hypoglycemia syndrome or formerly as nesidioblastosis. A 29-year-old female patient was treated with endogenous hyperinsulinemic hypoglycemia since the age of 4 months, taking daily 3 × 75 mg diazoxide, which adds up to 225 mg per day...
2022: Frontiers in Endocrinology
https://read.qxmd.com/read/35838801/complete-remission-of-recurrent-multiple-insulin-producing-neuroendocrine-tumors-of-the-pancreas-with-somatostatin-analogs-a-case-report-and-literature-review
#31
JOURNAL ARTICLE
Andreas Tartaglia, Giulia Busonero, Lorenza Gagliardi, Valentina Boddi, Federica Pieri, Maurizio Nizzoli
Hyperinsulinemic hypoglycemia is most commonly caused by a single, sporadic insulinoma. Multicentric insulinoma disease (insulinomatosis) as well as metachronous neuroendocrine tumors of the pancreas, known also as neuroendocrine adenomatosis, represent a very rare condition, if not associated with multiple endocrine neoplasia type 1 syndrome (MEN1) or Von Hippel Lindau disease. We report a 9-year follow-up of a 41-year-old woman, initially presenting with hypoglycemic syndrome caused by two insulin-producing tumors, who underwent subtotal pancreasectomy in 2012, with histology compatible with multiple small neuroendocrine tumors...
July 15, 2022: Discover. Oncology
https://read.qxmd.com/read/35798301/case-report-describing-a-patient-with-diazoxide-resistant-congenital-hyperinsulinism-resulting-from-compound-heterozygous-mutations-in-the-abcc8-gene
#32
JOURNAL ARTICLE
Kyujung Park, Kyung In Lim, Young Bae Sohn, Hae Sang Lee, Jin Soon Hwang
Congenital hyperinsulinism (CHI) is an over-secretion of insulin by pancreatic ß-cells, causing hypoglycemia which can inhibit brain development in infants. CHI is primarily associated with mutations in the ABCC8 or KCNJ11 genes, which encode the SUR1 and KIR 6.2 subunits of the ATP-sensitive potassium (KATP) channel. Here, we report a case of hyperinsulinemic hypoglycemia with ABCC8 gene mutation in a full term, female, Korean infant who developed early onset hypoglycemia but was not subject to either genetic or metabolic workup...
June 29, 2022: Annals of Pediatric Endocrinology & Metabolism
https://read.qxmd.com/read/35356161/pasireotide-use-for-the-treatment-of-endogenous-hyperinsulinemic-hypoglycemia-refractory-to-conventional-medical-therapy-a-case-report-and-review-of-the-literature
#33
Hasan Husni, Sara A Khan, Buraq Alghaieb, Mohammed S Abusamaan, Thomas W Donner, Amir H Hamrahian
Insulinomas are rare neuroendocrine pancreatic tumors that can be associated with severe episodes of hypoglycemia, leading to significant morbidity and mortality. These tumors are often difficult to localize, and hypoglycemia control can be challenging since glucose levels can be resistant to conventional therapies. Pasireotide is a novel somatostatin analog with a high affinity to multiple somatostatin receptors. It has up to 40 times higher affinity for somatostatin receptor subtype 5 in comparison with octreotide, leading to a higher inhibition of insulin release from beta cells...
March 2022: Clinical Case Reports
https://read.qxmd.com/read/34765735/paraneoplastic-hypoglycemia-leading-to-insulin-independence-in-a-patient-with-type-1-diabetes
#34
Nami Safai Haeri, Hussain Mahmud, Mary T Korytkowski
Objective: Non-islet cell tumor hypoglycemia (NICTH) is an uncommon paraneoplastic syndrome associated with mesenchymal neoplasms such as gastrointestinal stromal tumors (GISTs). We report the case of a patient with type 1 diabetes (T1D) and recurrent GIST who not only required discontinuation of insulin therapy but also required continuous parenteral glucose infusions to prevent hypoglycemia. Methods: A 59-year-old woman with a 24-year history of T1D and recurrent GIST presented with frequent episodes of symptomatic hypoglycemia despite continuous reductions in her insulin therapy...
November 2021: AACE Clinical Case Reports
https://read.qxmd.com/read/34740273/octreotide-lar-is-a-useful-alternative-for-the-management-of-diazoxide-responsive-congenital-hyperinsulinism
#35
JOURNAL ARTICLE
Manjiri Pramod Karlekar, Vijaya Sarathi, Sneha Arya, Sarah E Flanagan, Virendra Patil, Anurag Lila, Nalini Shah, Tushar Bandgar
The data on the congenital hyperinsulinism (CHI) in Asian Indian patients is limited. Diazoxide is often unavailable in India, which poses challenge in managing CHI. The study was aimed to present our experience with CHI with a special focus on the effectiveness and cost-effectiveness of octreotide long-acting release (OCT-LAR) among diazoxide-responsive CHI. The data of 14 index cases with CHI registered at our center were retrospectively analyzed. The diagnosis of CHI was based on elevated serum insulin (3...
November 2021: Hormone and Metabolic Research
https://read.qxmd.com/read/34682101/novel-compound-heterozygous-variants-of-the-abcc8-gene-warrant-identification-of-pancreatic-histology-in-infant-with-diazoxide-unresponsive-congenital-hyperinsulinism
#36
Rana Al Balwi, Dalal Bubshait, Raed Al Nefily, Omar Al Ghamdi
Congenital hyperinsulinism (CHI) is characterized by dysregulated insulin secretion, resulting in severe hypoglycemia. Mutations in the ABCC8 and KCNJ11 genes encoding KATP channels in beta cells of the pancreas are common among patients with CHI. Autosomal recessive CHI with diffuse involvement is the most common type of CHI among Saudi patients. It is relatively common for patients with autosomal recessive CHI to be medically unresponsive and undergo pancreatectomy. In this case report, we describe novel compound heterozygous variants in the ABCC8 gene in a Saudi infant that caused diazoxide-unresponsive CHI...
September 23, 2021: Children
https://read.qxmd.com/read/34430127/steroid-refractory-insulin-autoimmune-syndrome-treated-with-rituximab-and-continuous-glucose-monitoring
#37
Chandar M Batra, Kiran Kumar, Monika Goyal
A 67-year-old female presented with severe hypoglycemia with a blood glucose of 34 mg/dl five hours after having dinner. She did not have diabetes and had no access to oral hypoglycemic agents, insulin, or any other drug known to cause hypoglycemia. She was a known case of primary hypothyroidism euthyroid on treatment. The physical examination was unremarkable. Her liver, renal functions, thyroid, and adrenal functions were normal. At a blood sugar level of 23 mg/dl, her serum insulin was 24,000 uU/ml (normal: <3 uU/ml) and C-peptide was 16...
July 2021: Curēus
https://read.qxmd.com/read/34292572/retrospective-analysis-of-23-chinese-children-with-congenital-hyperinsulinism-undergoing-pancreatectomy
#38
JOURNAL ARTICLE
Lin Zhang, Zi-Di Xu, Min Liu, Qiao Zeng, Rong-Min Li, Jie-Ying Wang, Huan-Min Wang, Long Li, Hong Qin, Jie Yan, Yu-Jun Wu, Cheng Zhu, Gui-Chen Ni, Yan-Mei Sang
INTRODUCTION: The aim of the study was to discuss therapeutic effect and prognosis of pancreatectomy in the treatment of congenital hyperinsulinism (CHI). MATERIAL AND METHODS: A total of 23 Chinese children with CHI, who had undergone pancreatectomy, were selected as the study objects. The clinical data, the results of the ¹⁸Fluoro-L-3-4 dihydroxyphenylalanine positron emission tomography/computerized tomography (¹⁸F-DOPA PET/CT) scanning, and the diagnosis, treatment, and follow-up were analysed retrospectively...
2021: Endokrynologia Polska
https://read.qxmd.com/read/34053707/toxicology-of-medications-for-diabetes-mellitus
#39
REVIEW
Kevin Baumgartner, Jason Devgun
Medications used to treat diabetes mellitus are heterogeneous, with widely differing safety profiles in therapeutic use and in overdose. Insulin overdose may produce severe and prolonged hypoglycemia. Sulfonylurea poisoning should be treated with octreotide, sparing intravenous dextrose where possible. Acute metformin overdose may lead to life-threatening acidosis with elevated lactate concentrations, which may require hemodialysis. Glucagon-like peptide 1 agonists and dipeptidyl peptidase 4 inhibitors are benign in overdose in diabetic patients but may produce profound hypoglycemia in nondiabetic patients...
July 2021: Critical Care Clinics
https://read.qxmd.com/read/34026158/a-case-of-insulinoma-with-hypoglycemia-that-was-better-managed-with-lanreotide-than-octreotide
#40
Keiko Yamaoka, Shuichi Nagashima, Nobukazu Okada, Nagisa Sawayama, Shinsuke Saito, Manabu Takahashi, Kenta Okada, Kazuhiro Endo, Masaru Koizumi, Hideki Sasanuma, Ken Ebihara, Atsuko Kasajima, Noriyoshi Fukushima, Naohiro Sata, Shun Ishibashi
Long-acting somatostatin analogs, including lanreotide slow release (LAN-SR) and octreotide long-acting release (OCT-LAR), can improve hypoglycemia in insulinoma. LAN-SR may be more beneficial in some patients with insulinoma than OCT-LAR.
May 2021: Clinical Case Reports
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