Cargando…

A Case Report of Doege–Potter Syndrome: A Rare Cause of Hypoglycemia in a Patient without Diabetes

Hypoglycemia in patients without diabetes is a diagnostic challenge for the endocrinologist. Sometimes it is related to rare causes such as Doege–Potter Syndrome (DPS). DPS is caused by an abnormal insulin-like grow factor 2(IGF-2) that retains part of the E domain during the production process, res...

Descripción completa

Detalles Bibliográficos
Autores principales: Corsano, Chiara, Paradiso, Matteo, Laudadio, Ester Daniela, Sollitto, Francesco, Lamacchia, Olga
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10299076/
https://www.ncbi.nlm.nih.gov/pubmed/37373678
http://dx.doi.org/10.3390/jcm12123984
_version_ 1785064274212683776
author Corsano, Chiara
Paradiso, Matteo
Laudadio, Ester Daniela
Sollitto, Francesco
Lamacchia, Olga
author_facet Corsano, Chiara
Paradiso, Matteo
Laudadio, Ester Daniela
Sollitto, Francesco
Lamacchia, Olga
author_sort Corsano, Chiara
collection PubMed
description Hypoglycemia in patients without diabetes is a diagnostic challenge for the endocrinologist. Sometimes it is related to rare causes such as Doege–Potter Syndrome (DPS). DPS is caused by an abnormal insulin-like grow factor 2(IGF-2) that retains part of the E domain during the production process, resulting in a longer peptide called “big-IGF-2”. We present a case report of DPS with emphasis on the diagnosis and especially on the difficulties in interpreting the biochemical findings. An elderly patient with an intrathoracic neoplasm and hypoglycemia underwent various tests: insulin autoantibodies and fasting test were both negative. She had low values of IGF-1 and normal values of IGF-2 that apparently excludes a diagnosis of DPS. The evaluation of the IGF-2/IGF-1 ratio is the most important test because a ratio >10 is widely considered to be indicative of non-islet cell tumor hypoglycemia (NICTH). Glucose infusion and steroid therapy were used to control the hypoglycemia, but the definitive treatment was surgery, which almost immediately reversed the hypoglycemia. The differential diagnosis of hypoglycemia should include rare causes such as DPS, and the IGF-2/IGF-1 ratio is a useful tool.
format Online
Article
Text
id pubmed-10299076
institution National Center for Biotechnology Information
language English
publishDate 2023
publisher MDPI
record_format MEDLINE/PubMed
spelling pubmed-102990762023-06-28 A Case Report of Doege–Potter Syndrome: A Rare Cause of Hypoglycemia in a Patient without Diabetes Corsano, Chiara Paradiso, Matteo Laudadio, Ester Daniela Sollitto, Francesco Lamacchia, Olga J Clin Med Case Report Hypoglycemia in patients without diabetes is a diagnostic challenge for the endocrinologist. Sometimes it is related to rare causes such as Doege–Potter Syndrome (DPS). DPS is caused by an abnormal insulin-like grow factor 2(IGF-2) that retains part of the E domain during the production process, resulting in a longer peptide called “big-IGF-2”. We present a case report of DPS with emphasis on the diagnosis and especially on the difficulties in interpreting the biochemical findings. An elderly patient with an intrathoracic neoplasm and hypoglycemia underwent various tests: insulin autoantibodies and fasting test were both negative. She had low values of IGF-1 and normal values of IGF-2 that apparently excludes a diagnosis of DPS. The evaluation of the IGF-2/IGF-1 ratio is the most important test because a ratio >10 is widely considered to be indicative of non-islet cell tumor hypoglycemia (NICTH). Glucose infusion and steroid therapy were used to control the hypoglycemia, but the definitive treatment was surgery, which almost immediately reversed the hypoglycemia. The differential diagnosis of hypoglycemia should include rare causes such as DPS, and the IGF-2/IGF-1 ratio is a useful tool. MDPI 2023-06-12 /pmc/articles/PMC10299076/ /pubmed/37373678 http://dx.doi.org/10.3390/jcm12123984 Text en © 2023 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Case Report
Corsano, Chiara
Paradiso, Matteo
Laudadio, Ester Daniela
Sollitto, Francesco
Lamacchia, Olga
A Case Report of Doege–Potter Syndrome: A Rare Cause of Hypoglycemia in a Patient without Diabetes
title A Case Report of Doege–Potter Syndrome: A Rare Cause of Hypoglycemia in a Patient without Diabetes
title_full A Case Report of Doege–Potter Syndrome: A Rare Cause of Hypoglycemia in a Patient without Diabetes
title_fullStr A Case Report of Doege–Potter Syndrome: A Rare Cause of Hypoglycemia in a Patient without Diabetes
title_full_unstemmed A Case Report of Doege–Potter Syndrome: A Rare Cause of Hypoglycemia in a Patient without Diabetes
title_short A Case Report of Doege–Potter Syndrome: A Rare Cause of Hypoglycemia in a Patient without Diabetes
title_sort case report of doege–potter syndrome: a rare cause of hypoglycemia in a patient without diabetes
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10299076/
https://www.ncbi.nlm.nih.gov/pubmed/37373678
http://dx.doi.org/10.3390/jcm12123984
work_keys_str_mv AT corsanochiara acasereportofdoegepottersyndromeararecauseofhypoglycemiainapatientwithoutdiabetes
AT paradisomatteo acasereportofdoegepottersyndromeararecauseofhypoglycemiainapatientwithoutdiabetes
AT laudadioesterdaniela acasereportofdoegepottersyndromeararecauseofhypoglycemiainapatientwithoutdiabetes
AT sollittofrancesco acasereportofdoegepottersyndromeararecauseofhypoglycemiainapatientwithoutdiabetes
AT lamacchiaolga acasereportofdoegepottersyndromeararecauseofhypoglycemiainapatientwithoutdiabetes
AT corsanochiara casereportofdoegepottersyndromeararecauseofhypoglycemiainapatientwithoutdiabetes
AT paradisomatteo casereportofdoegepottersyndromeararecauseofhypoglycemiainapatientwithoutdiabetes
AT laudadioesterdaniela casereportofdoegepottersyndromeararecauseofhypoglycemiainapatientwithoutdiabetes
AT sollittofrancesco casereportofdoegepottersyndromeararecauseofhypoglycemiainapatientwithoutdiabetes
AT lamacchiaolga casereportofdoegepottersyndromeararecauseofhypoglycemiainapatientwithoutdiabetes