Research & Literature

Pancreatic exocrine insufficiency following pancreatic resection.

Phillips ME

Pancreatology : official journal of the International Association of Pancreatology (IAP) ... [et al.] 2015; (15(5)):449-455 doi:10.1016/j.pan.2015.06.003.

Biomarkers of Insulin for the Diagnosis of Hyperinsulinemic Hypoglycemia in Infants and Children.

Ferrara C, Patel P, Becker S, et al.

The Journal of pediatrics 2016; (168()):212-219 doi:10.1016/j.jpeds.2015.09.045.

Persistent hyperinsulinemic hypoglycemia of infancy: a clinical and pathological study of 19 cases in a single institution.

Li J, Ma Y, Lv M, et al.

International journal of clinical and experimental pathology 2015; (8(11)):14417-24.

Late Presentation of Fulminant Necrotizing Enterocolitis in a Child with Hyperinsulinism on Octreotide Therapy.

Hawkes CP, Adzick NS, Palladino AA, De León DD

Hormone research in paediatrics 2016; (86(2)):131-136 doi:10.1159/000443959.

Subtotal Pancreatectomy for Congenital Hyperinsulinism: Our Experience and Review of Literature.

Redkar R, Karkera PJ, Krishnan J, Hathiramani V

The Indian journal of surgery 2015; (77(Suppl 3)):778-82 doi:10.1007/s12262-013-0999-9.

Thirty-day outcomes underestimate endocrine and exocrine insufficiency after pancreatic resection.

Lim PW, Dinh KH, Sullivan M, et al.

HPB : the official journal of the International Hepato Pancreato Biliary Association 2016; (18(4)):360-6.

Prolonged episodes of hypoglycaemia in HNF4A-MODY mutation carriers with IGT. Evidence of persistent hyperinsulinism into early adulthood.

Bacon S, Kyithar MP, Condron EM, et al.

Acta diabetologica 2016; (53(6)):965-972 doi:10.1007/s00592-016-0890-9.

Pharmacological Correction of Trafficking Defects in ATP-sensitive Potassium Channels Caused by Sulfonylurea Receptor 1 Mutations.

Martin GM, Rex EA, Devaraneni P, et al.

The Journal of biological chemistry 2016; (291(42)):21971-21983 doi:10.1074/jbc.M116.749366.

Genetic characteristics and long-term follow-up of 11 patients with congenital hyperinsulinism followed in a single center.

Warncke K, Falco F, Rabl W, et al.

Journal of pediatric endocrinology & metabolism : JPEM 2016; (29(10)):1187-1194.

[Congenital hyperinsulinism: Loss of B-cell self-control].

Lebl J, Roženková K, Průhová Š

Vnitrni lekarstvi 2016; (62(11 Suppl 4)):S72-76.

Asymptomatic Congenital Hyperinsulinism due to a Glucokinase-Activating Mutation, Treated as Adrenal Insufficiency for Twelve Years.

Morishita K, Kyo C, Yonemoto T, et al.

Case reports in endocrinology 2017; (2017()):4709262 doi:10.1155/2017/4709262.

Diagnosis of congenital hyperinsulinism: Biochemical profiles during hypoglycemia.

Sakakibara A, Hashimoto Y, Kawakita R, et al.

Pediatric diabetes 2018; (19(2)):259-264 doi:10.1111/pedi.12548.

Functional Imaging of Paragangliomas with an Emphasis on Von Hippel-Lindau-Associated Disease: A Mini Review.

Ilias I, Meristoudis G

Journal of kidney cancer and VHL 2017; (4(3)):30-36 doi:10.15586/jkcvhl.2017.92.

A new familial form of a late-onset, persistent hyperinsulinemic hypoglycemia of infancy caused by a novel mutation in KCNJ11.

Yang YY, Long RK, Ferrara CT, et al.

Channels (Austin, Tex.) 2017; (11(6)):636-647 doi:10.1080/19336950.2017.1393131.

18F-DOPA PET/CT and 68Ga-DOTANOC PET/CT scans as diagnostic tools in focal congenital hyperinsulinism: a blinded evaluation.

Christiansen CD, Petersen H, Nielsen AL, et al.

European journal of nuclear medicine and molecular imaging 2018; (45(2)):250-261 doi:10.1007/s00259-017-3867-1.

Challenging diagnosis of congenital hyperinsulinism in two infants of diabetic mothers with rare pathogenic KCNJ11 and HNF4A gene variants.

Huerta-Saenz L, Saunders C, Yan Y

International journal of pediatric endocrinology 2018; (2018()):5 doi:10.1186/s13633-018-0060-7.

Intraoperative Ultrasound: A Tool to Support Tissue-Sparing Curative Pancreatic Resection in Focal Congenital Hyperinsulinism.

Bendix J, Laursen MG, Mortensen MB, et al.

Frontiers in endocrinology 2018; (9()):478 doi:10.3389/fendo.2018.00478.

Therapies and outcomes of congenital hyperinsulinism-induced hypoglycaemia.

Banerjee I, Salomon-Estebanez M, Shah P, et al.

Diabetic medicine : a journal of the British Diabetic Association 2019; (36(1)):9-21 doi:10.1111/dme.13823.

Prevalence of Adverse Events in Children With Congenital Hyperinsulinism Treated With Diazoxide.

Herrera A, Vajravelu ME, Givler S, et al.

The Journal of clinical endocrinology and metabolism 2018; (103(12)):4365-4372 doi:10.1210/jc.2018-01613.

Hyperinsulinism-hyperammonemia Syndrome in an Infant with Seizures.

Strajnar A, Tansek MZ, Podkrajsek KT, et al.

Balkan journal of medical genetics : BJMG 2018; (21(1)):77-81 doi:10.2478/bjmg-2018-0014.

Pulmonary hypertension occurring with diazoxide use in a preterm infant with hypoglycemia.

Kylat RI

Drug, healthcare and patient safety 2019; (11()):7-10 doi:10.2147/DHPS.S198255.

Sirolimus: Efficacy and Complications in Children With Hyperinsulinemic Hypoglycemia: A 5-Year Follow-Up Study.

Maria G, Antonia D, Michael A, et al.

Journal of the Endocrine Society 2019; (3(4)):699-713 doi:10.1210/js.2018-00417.

Tracer Accumulation in Relation to Venous Thrombus on 18F-DOPA PET/CT in a Case of Persistent Hyperinsulinemic Hypoglycemia of Infancy.

Arora S, Damle NA, Passah A, et al.

Nuclear medicine and molecular imaging 2019; (53(2)):148-151 doi:10.1007/s13139-018-00568-5.

Clinical and enzymatic phenotypes in congenital hyperinsulinemic hypoglycemia due to glucokinase-activating mutations: A report of two cases and a brief overview of the literature.

Ping F, Wang Z, Xiao X

Journal of diabetes investigation 2019; (10(6)):1454-1462 doi:10.1111/jdi.13072.

Genotype and phenotype analysis of a cohort of patients with congenital hyperinsulinism based on DOPA-PET CT scanning.

Ni J, Ge J, Zhang M, et al.

European journal of pediatrics 2019; (178(8)):1161-1169 doi:10.1007/s00431-019-03408-6.

Current and Emerging Agents for the Treatment of Hypoglycemia in Patients with Congenital Hyperinsulinism.

De Cosio AP, Thornton P

Paediatric drugs 2019; (21(3)):123-136 doi:10.1007/s40272-019-00334-w.

Striking Visualization of Diffuse Congenital Nesidioblastosis on Ga-68 DOTATATE PET/CT

Canbaz F, Aydın M, Can Meydan B, et al.

Molecular imaging and radionuclide therapy 2019; (28(2)):83-85 doi:10.4274/mirt.galenos.2018.38039.

Novel dominant KATP channel mutations in infants with congenital hyperinsulinism: Validation by in vitro expression studies and in vivo carrier phenotyping.

Boodhansingh KE, Kandasamy B, Mitteer L, et al.

American journal of medical genetics. Part A 2019; (179(11)):2214-2227 doi:10.1002/ajmg.a.61335.

Nationwide survey of endogenous hyperinsulinemic hypoglycemia in Japan (2017-2018): Congenital hyperinsulinism, insulinoma, non-insulinoma pancreatogenous hypoglycemia syndrome and insulin autoimmune syndrome (Hirata's disease).

Yamada Y, Kitayama K, Oyachi M, et al.

Journal of diabetes investigation 2020; (11(3)):554-563 doi:10.1111/jdi.13180.

Surgical management of medically-refractory hyperinsulinism.

Zobel MJ, McFarland C, Ferrera-Cook CT, Padilla BE

American journal of surgery 2020; (219(6)):947-951 doi:10.1016/j.amjsurg.2019.09.003.

Congenital hyperinsulinism associated with Hirschsprung's disease-a report of an extremely rare case.

Shono T, Shono K, Hashimoto Y, et al.

Surgical case reports 2020; (6(1)):4 doi:10.1186/s40792-020-0778-3.

Using referral rates for genetic testing to determine the incidence of a rare disease: The minimal incidence of congenital hyperinsulinism in the UK is 1 in 28,389.

Yau D, Laver TW, Dastamani A, et al.

PloS one 2020; (15(2)):e0228417 doi:10.1371/journal.pone.0228417.

Congenital hyperinsulinsim: case report and review of literature.

Hasbaoui BE, Elyajouri A, Abilkassem R, Agadr A

The Pan African medical journal 2020; (35()):53 doi:10.11604/pamj.2020.35.53.16604.

A Novel HNF4A Mutation Causing Three Phenotypic Forms of Glucose Dysregulation in a Family.

Chandran S, Rajadurai VS, Hoi WH, et al.

Frontiers in pediatrics 2020; (8()):320 doi:10.3389/fped.2020.00320.

Case report: contradictory genetics and imaging in focal congenital hyperinsulinism reinforces the need for pancreatic biopsy.

Yau D, Marwaha R, Mohnike K, et al.

International journal of pediatric endocrinology 2020; (2020()):17 doi:10.1186/s13633-020-00086-2.

Detection of hepatocyte nuclear factor 4A(HNF4A) gene variant as the cause for congenital hyperinsulinism leads to revision of the diagnosis of the mother.

Vlachopapadopoulou EA, Dikaiakou E, Fotiadou A, et al.

Journal of pediatric endocrinology & metabolism : JPEM 2021; (34(4)):527-530 doi:10.1515/jpem-2020-0302.

Necrotizing Enterocolitis in Neonates With Hyperinsulinemic Hypoglycemia Treated With Diazoxide.

Keyes ML, Healy H, Sparger KA, et al.

Pediatrics 2021; (147(2)) doi:10.1542/peds.2019-3202.

Efficacy and safety of diazoxide for treating hyperinsulinemic hypoglycemia: A systematic review and meta-analysis.

Chen X, Feng L, Yao H, et al.

PloS one 2021; (16(2)):e0246463 doi:10.1371/journal.pone.0246463.

Congenital Hyperinsulinism.

Sims K

NeoReviews 2021; (22(4)):e230-e240 doi:10.1542/neo.22-4-e230.

Sirolimus in infants with congenital hyperinsulinism (CHI) - a single-centre experience.

Panigrahy N, Chirla DK, Bagga N, et al.

European journal of pediatrics 2022; (181(1)):407-412 doi:10.1007/s00431-021-04209-6.

[Clinical features of 123 patients with hyperinsulinemic hypoglycemia auxiliarily diagnosed by 18F-DOPA-PET CT scanning].

Zhang MY, Ni JW, Ge JJ, et al.

Zhonghua er ke za zhi = Chinese journal of pediatrics 2021; (59(10)):853-858 doi:10.3760/cma.j.cn112140-20210417-00326.

Birth weight and diazoxide unresponsiveness strongly predict the likelihood of congenital hyperinsulinism due to a mutation in ABCC8 or KCNJ11.

Hewat TI, Yau D, Jerome JCS, et al.

European journal of endocrinology 2021; (185(6)):813-818 doi:10.1530/EJE-21-0476.

Congenital hyperinsulinism: localization of a focal lesion with 18F-FDOPA positron emission tomography.

States LJ, Becker SA, De León DD

Pediatric radiology 2022; (52(4)):693-701 doi:10.1007/s00247-021-05206-5.

Early diagnosis of focal congenital hyperinsulinism: A fluorine-18-labeled l-dihydroxyphenylalanine positron emission tomography/computed tomography study.

Burroni L, Palucci A, Biscontini G, Cherubini V

World journal of nuclear medicine 2021; (20(4)):395-397 doi:10.4103/wjnm.wjnm_159_20.

F-18 DOPA PET/CT in pediatric patients with hyperinsulinemic hypoglycemia: A correlation with genetic analysis.

Sagar S, Arora G, Damle N, et al.

Nuclear medicine communications 2022; (43(4)):451-457 doi:10.1097/MNM.0000000000001526.

ATP-Sensitive Potassium Channels in Hyperinsulinism and Type 2 Diabetes: Inconvenient Paradox or New Paradigm?

Nichols CG, York NW, Remedi MS

Diabetes 2022; (71(3)):367-375 doi:10.2337/db21-0755.

Molecular imaging of endocrine neoplasms with emphasis on 18F-DOPA PET: a practical approach for well-tailored imaging protocols.

Fargette C, Imperiale A, Taïeb D

The quarterly journal of nuclear medicine and molecular imaging : official publication of the Italian Association of Nuclear Medicine (AIMN) [and] the International Association of Radiopharmacology (IAR), [and] Section of the Society of... 2022; (66(2)):141-147 doi:10.23736/S1824-4785.22.03450-1.

Congenital hyperinsulinism in a newborn presenting with poor feeding.

Mazloom K, Sanchez-Lara PA, Langston S, et al.

SAGE open medical case reports 2022; (10()):2050313X221083174 doi:10.1177/2050313X221083174.

Carbamazepine promotes surface expression of mutant Kir6.2-A28V ATP-sensitive potassium channels by modulating Golgi retention and autophagy.

Lin CH, Lin YC, Yang SB, Chen PC

The Journal of biological chemistry 2022; (298(5)):101904 doi:10.1016/j.jbc.2022.101904.

Congenital Hyperinsulinism International: A Community Focused on Improving the Lives of People Living With Congenital Hyperinsulinism.

Raskin J, Pasquini TLS, Bose S, et al.

Frontiers in endocrinology 2022; (13()):886552 doi:10.3389/fendo.2022.886552.

Congenital hyperinsulinism in clinical practice: From biochemical pathophysiology to new monitoring techniques.

Martino M, Sartorelli J, Gragnaniello V, Burlina A

Frontiers in pediatrics 2022; (10()):901338 doi:10.3389/fped.2022.901338.

Congenital Hyperinsulinaemic Hypoglycaemia-A Review and Case Presentation.

Krawczyk S, Urbanska K, Biel N, et al.

Journal of clinical medicine 2022; (11(20)) doi:10.3390/jcm11206020.

Hyperinsulinism-hyperammonemia syndrome in two Peruvian children with refractory epilepsy.

De Los Santos-La Torre MA, Del Águila-Villar CM, Lu-de Lama LR, et al.

Journal of pediatric endocrinology & metabolism : JPEM 2023; (36(2)):207-211 doi:10.1515/jpem-2022-0490.

New approaches to screening and management of neonatal hypoglycemia based on improved understanding of the molecular mechanism of hypoglycemia.

Stanley CA, Thornton PS, De Leon DD

Frontiers in pediatrics 2023; (11()):1071206 doi:10.3389/fped.2023.1071206.

Munchausen syndrome by proxy: a case report.

Alkhattabi F, Bamogaddam I, Alsagheir A, et al.

Journal of medical case reports 2023; (17(1)):148 doi:10.1186/s13256-023-03848-7.

Syndromic forms of congenital hyperinsulinism.

Zenker M, Mohnike K, Palm K

Frontiers in endocrinology 2023; (14()):1013874 doi:10.3389/fendo.2023.1013874.

Pathological features in non-neoplastic congenital and adult hyperinsulinism: from nesidioblastosis to current terminology and understanding.

Sempoux C, Klöppel G

Endocrine-related cancer 2023; (30(9)).

Congenital hyperinsulinism.

Velde CD, Reigstad H, Tjora E, et al.

Tidsskrift for den Norske laegeforening : tidsskrift for praktisk medicin, ny raekke 2023; (143(18)) doi:10.4045/tidsskr.23.0425.

Genotype-histotype-phenotype correlations in hyperinsulinemic hypoglycemia.

Larsen AR, Brusgaard K, Christesen HT, Detlefsen S

Histology and histopathology 2024; (39(7)):817-844 doi:10.14670/HH-18-709.

[Congenital hyperinsulinism : contributions of chemistry, therapeutic response, genetics and imaging].

Vandendaele C, Kaschten S, Parent AS, Fudvoye J

Revue medicale de Liege 2024; (79(3)):168-174.

Novel Treatment Options in Patients with Maturity-Onset Diabetes of the Young.

Müssig K

Experimental and clinical endocrinology & diabetes : official journal, German Society of Endocrinology [and] German Diabetes Association 2025; (133(1)):51-58 doi:10.1055/a-2436-7723.

Octreotide efficacy and safety in children with hyperinsulinism: evidence from two Chinese centers.

Ni J, Cao B, Zeng H, et al.

European journal of pediatrics 2024; (184(1)):33 doi:10.1007/s00431-024-05884-x.

Loss of β-Cell KATP Reduces Ca2+ Sensitivity of Insulin Secretion and Trpm5 Expression.

York NW, Yan Z, Osipovich AB, et al.

Diabetes 2025; (74(3)):376-383 doi:10.2337/db24-0650.

Reassessing Pancreatectomy in Diffuse Congenital Hyperinsulinism: A Tale of 2 Brothers With Homozygous KCNJ11 Variants.

Rafique HA, Lucas-Herald AK, Shaikh MG

JCEM case reports 2025; (3(4)):luaf045 doi:10.1210/jcemcr/luaf045.

Identification and Rescue of Congenital Hyperinsulinism-Associated ABCC8 Mutations that Impair KATP Channel Trafficking.

ElSheikh A, Kuo YY, Boodhansingh KE, et al.

bioRxiv : the preprint server for biology 2025; doi:10.1101/2025.05.18.654760.

Severe transient neonatal hyperinsulinism: First Peruvian case series.

Virú-Loza MA, Bermudez-Paredes F, Arauco-Carhuas IM

SAGE open medical case reports 2025; (13()):2050313X251347787 doi:10.1177/2050313X251347787.