Heterogeneity of glucagonomas due to differential processing of proglucagon-derived peptides

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Heterogeneity of glucagonomas due to differential processing of proglucagon-derived peptides. / Challis, Benjamin G; Albrechtsen, Nicolai J Wewer; Bansiya, Vishakha; Burling, Keith; Barker, Peter; Hartmann, Bolette; Gribble, Fiona; O'Rahilly, Stephen; Holst, Jens J; Simpson, Helen L.

I: Endocrinology, Diabetes & Metabolism Case Reports, Bind 2015, 150105, 2015, s. 1-7.

Publikation: Bidrag til tidsskriftTidsskriftartikelForskningfagfællebedømt

Harvard

Challis, BG, Albrechtsen, NJW, Bansiya, V, Burling, K, Barker, P, Hartmann, B, Gribble, F, O'Rahilly, S, Holst, JJ & Simpson, HL 2015, 'Heterogeneity of glucagonomas due to differential processing of proglucagon-derived peptides', Endocrinology, Diabetes & Metabolism Case Reports, bind 2015, 150105, s. 1-7. https://doi.org/10.1530/EDM-15-0105

APA

Challis, B. G., Albrechtsen, N. J. W., Bansiya, V., Burling, K., Barker, P., Hartmann, B., ... Simpson, H. L. (2015). Heterogeneity of glucagonomas due to differential processing of proglucagon-derived peptides. Endocrinology, Diabetes & Metabolism Case Reports, 2015, 1-7. [150105]. https://doi.org/10.1530/EDM-15-0105

Vancouver

Challis BG, Albrechtsen NJW, Bansiya V, Burling K, Barker P, Hartmann B o.a. Heterogeneity of glucagonomas due to differential processing of proglucagon-derived peptides. Endocrinology, Diabetes & Metabolism Case Reports. 2015;2015:1-7. 150105. https://doi.org/10.1530/EDM-15-0105

Author

Challis, Benjamin G ; Albrechtsen, Nicolai J Wewer ; Bansiya, Vishakha ; Burling, Keith ; Barker, Peter ; Hartmann, Bolette ; Gribble, Fiona ; O'Rahilly, Stephen ; Holst, Jens J ; Simpson, Helen L. / Heterogeneity of glucagonomas due to differential processing of proglucagon-derived peptides. I: Endocrinology, Diabetes & Metabolism Case Reports. 2015 ; Bind 2015. s. 1-7.

Bibtex

@article{4f369ed2411341099a751f06b8539a2f,
title = "Heterogeneity of glucagonomas due to differential processing of proglucagon-derived peptides",
abstract = "UNLABELLED: Pancreatic neuroendocrine tumours (pNETs) secreting proglucagon are associated with phenotypic heterogeneity. Here, we describe two patients with pNETs and varied clinical phenotypes due to differential processing and secretion of proglucagon-derived peptides (PGDPs). Case 1, a 57-year-old woman presented with necrolytic migratory erythema, anorexia, constipation and hyperinsulinaemic hypoglycaemia. She was found to have a grade 1 pNET, small bowel mucosal thickening and hyperglucagonaemia. Somatostatin analogue (SSA) therapy improved appetite, abolished hypoglycaemia and improved the rash. Case 2, a 48-year-old male presented with diabetes mellitus, diarrhoea, weight loss, nausea, vomiting and perineal rash due to a grade 1 metastatic pNET and hyperglucagonaemia. In both cases, plasma levels of all measured PGDPs were elevated and attenuated following SSA therapy. In case 1, there was increased production of intact glucagon-like peptide 1 (GLP-1) and GLP-2, similar to that of the enteroendocrine L cell. In case 2, pancreatic glucagon was elevated due to a pancreatic α-cell-like proglucagon processing profile. In summary, we describe two patients with pNETs and heterogeneous clinical phenotypes due to differential processing and secretion of PGDPs. This is the first description of a patient with symptomatic hyperinsulinaemic hypoglycaemia and marked gastrointestinal dysfunction due to, in part, a proglucagon-expressing pNET.LEARNING POINTS: PGDPs exhibit a diverse range of biological activities including critical roles in glucose and amino acid metabolism, energy homeostasis and gastrointestinal physiology.The clinical manifestations of proglucagon-expressing tumours may exhibit marked phenotypic variation due to the biochemical heterogeneity of their secreted peptide repertoire.Specific and precise biochemical assessment of individuals with proglucagon-expressing tumours may provide opportunities for improved diagnosis and clinical management.",
author = "Challis, {Benjamin G} and Albrechtsen, {Nicolai J Wewer} and Vishakha Bansiya and Keith Burling and Peter Barker and Bolette Hartmann and Fiona Gribble and Stephen O'Rahilly and Holst, {Jens J} and Simpson, {Helen L}",
year = "2015",
doi = "10.1530/EDM-15-0105",
language = "English",
volume = "2015",
pages = "1--7",
journal = "Endocrinology, Diabetes & Metabolism Case Reports",
issn = "2052-0573",
publisher = "BioScientifica Ltd.",

}

RIS

TY - JOUR

T1 - Heterogeneity of glucagonomas due to differential processing of proglucagon-derived peptides

AU - Challis, Benjamin G

AU - Albrechtsen, Nicolai J Wewer

AU - Bansiya, Vishakha

AU - Burling, Keith

AU - Barker, Peter

AU - Hartmann, Bolette

AU - Gribble, Fiona

AU - O'Rahilly, Stephen

AU - Holst, Jens J

AU - Simpson, Helen L

PY - 2015

Y1 - 2015

N2 - UNLABELLED: Pancreatic neuroendocrine tumours (pNETs) secreting proglucagon are associated with phenotypic heterogeneity. Here, we describe two patients with pNETs and varied clinical phenotypes due to differential processing and secretion of proglucagon-derived peptides (PGDPs). Case 1, a 57-year-old woman presented with necrolytic migratory erythema, anorexia, constipation and hyperinsulinaemic hypoglycaemia. She was found to have a grade 1 pNET, small bowel mucosal thickening and hyperglucagonaemia. Somatostatin analogue (SSA) therapy improved appetite, abolished hypoglycaemia and improved the rash. Case 2, a 48-year-old male presented with diabetes mellitus, diarrhoea, weight loss, nausea, vomiting and perineal rash due to a grade 1 metastatic pNET and hyperglucagonaemia. In both cases, plasma levels of all measured PGDPs were elevated and attenuated following SSA therapy. In case 1, there was increased production of intact glucagon-like peptide 1 (GLP-1) and GLP-2, similar to that of the enteroendocrine L cell. In case 2, pancreatic glucagon was elevated due to a pancreatic α-cell-like proglucagon processing profile. In summary, we describe two patients with pNETs and heterogeneous clinical phenotypes due to differential processing and secretion of PGDPs. This is the first description of a patient with symptomatic hyperinsulinaemic hypoglycaemia and marked gastrointestinal dysfunction due to, in part, a proglucagon-expressing pNET.LEARNING POINTS: PGDPs exhibit a diverse range of biological activities including critical roles in glucose and amino acid metabolism, energy homeostasis and gastrointestinal physiology.The clinical manifestations of proglucagon-expressing tumours may exhibit marked phenotypic variation due to the biochemical heterogeneity of their secreted peptide repertoire.Specific and precise biochemical assessment of individuals with proglucagon-expressing tumours may provide opportunities for improved diagnosis and clinical management.

AB - UNLABELLED: Pancreatic neuroendocrine tumours (pNETs) secreting proglucagon are associated with phenotypic heterogeneity. Here, we describe two patients with pNETs and varied clinical phenotypes due to differential processing and secretion of proglucagon-derived peptides (PGDPs). Case 1, a 57-year-old woman presented with necrolytic migratory erythema, anorexia, constipation and hyperinsulinaemic hypoglycaemia. She was found to have a grade 1 pNET, small bowel mucosal thickening and hyperglucagonaemia. Somatostatin analogue (SSA) therapy improved appetite, abolished hypoglycaemia and improved the rash. Case 2, a 48-year-old male presented with diabetes mellitus, diarrhoea, weight loss, nausea, vomiting and perineal rash due to a grade 1 metastatic pNET and hyperglucagonaemia. In both cases, plasma levels of all measured PGDPs were elevated and attenuated following SSA therapy. In case 1, there was increased production of intact glucagon-like peptide 1 (GLP-1) and GLP-2, similar to that of the enteroendocrine L cell. In case 2, pancreatic glucagon was elevated due to a pancreatic α-cell-like proglucagon processing profile. In summary, we describe two patients with pNETs and heterogeneous clinical phenotypes due to differential processing and secretion of PGDPs. This is the first description of a patient with symptomatic hyperinsulinaemic hypoglycaemia and marked gastrointestinal dysfunction due to, in part, a proglucagon-expressing pNET.LEARNING POINTS: PGDPs exhibit a diverse range of biological activities including critical roles in glucose and amino acid metabolism, energy homeostasis and gastrointestinal physiology.The clinical manifestations of proglucagon-expressing tumours may exhibit marked phenotypic variation due to the biochemical heterogeneity of their secreted peptide repertoire.Specific and precise biochemical assessment of individuals with proglucagon-expressing tumours may provide opportunities for improved diagnosis and clinical management.

U2 - 10.1530/EDM-15-0105

DO - 10.1530/EDM-15-0105

M3 - Journal article

C2 - 26693280

VL - 2015

SP - 1

EP - 7

JO - Endocrinology, Diabetes & Metabolism Case Reports

JF - Endocrinology, Diabetes & Metabolism Case Reports

SN - 2052-0573

M1 - 150105

ER -

ID: 152286555