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Wolfram Syndrome 1 in Two Brothers Treated with Insulin Pump
BACKGROUND/OBJECTIVE: Wolfram syndrome (WS) is a rare genetic disorder, in which patients develop early-onset diabetes mellitus (DM), optic nerve atrophy, and neurodegeneration, which has no specific treatment available. Here, we report 2 brothers treated with an insulin pump to manage the alteratio...
Autores principales: | , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
American Association of Clinical Endocrinology
2023
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10382610/ https://www.ncbi.nlm.nih.gov/pubmed/37520764 http://dx.doi.org/10.1016/j.aace.2023.05.002 |
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author | Cardona, Manuela Ardila, Alejandro Gómez, Juan D. Román-González, Alejandro |
author_facet | Cardona, Manuela Ardila, Alejandro Gómez, Juan D. Román-González, Alejandro |
author_sort | Cardona, Manuela |
collection | PubMed |
description | BACKGROUND/OBJECTIVE: Wolfram syndrome (WS) is a rare genetic disorder, in which patients develop early-onset diabetes mellitus (DM), optic nerve atrophy, and neurodegeneration, which has no specific treatment available. Here, we report 2 brothers treated with an insulin pump to manage the alterations of the glycemic levels due to the DM. CASE REPORT: We present the case of 2 siblings diagnosed with Wolfram syndrome 1, they presented with typical endocrinological and neurodegenerative early manifestations, one brother was treated with a sensor-augmented insulin infusion system, and the other with an insulin pump. Both reached a better metabolic state and had improved quality of life. DISCUSSION: The management of WS is still a challenge; however, the use of a sensor-augmented insulin infusion system and the information that it provides may offer better care to patients who require frequent monitoring and adjustments in their treatment. It has been reported that the neurodegenerative progression of WS is also associated with high glucose peaks; therefore, it is necessary to control it, even when it is hard due to the difficult-to-manage DM. There is only 1 previous case report of WS with insulin pump that describes the benefits of continuous subcutaneous insulin infusion and tight metabolic control during pregnancy. CONCLUSION: The use of insulin pumps may be an effective treatment for DM in WS patients, mainly in terms of improving the prognosis of difficult-to-manage DM. |
format | Online Article Text |
id | pubmed-10382610 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2023 |
publisher | American Association of Clinical Endocrinology |
record_format | MEDLINE/PubMed |
spelling | pubmed-103826102023-07-30 Wolfram Syndrome 1 in Two Brothers Treated with Insulin Pump Cardona, Manuela Ardila, Alejandro Gómez, Juan D. Román-González, Alejandro AACE Clin Case Rep Case Report BACKGROUND/OBJECTIVE: Wolfram syndrome (WS) is a rare genetic disorder, in which patients develop early-onset diabetes mellitus (DM), optic nerve atrophy, and neurodegeneration, which has no specific treatment available. Here, we report 2 brothers treated with an insulin pump to manage the alterations of the glycemic levels due to the DM. CASE REPORT: We present the case of 2 siblings diagnosed with Wolfram syndrome 1, they presented with typical endocrinological and neurodegenerative early manifestations, one brother was treated with a sensor-augmented insulin infusion system, and the other with an insulin pump. Both reached a better metabolic state and had improved quality of life. DISCUSSION: The management of WS is still a challenge; however, the use of a sensor-augmented insulin infusion system and the information that it provides may offer better care to patients who require frequent monitoring and adjustments in their treatment. It has been reported that the neurodegenerative progression of WS is also associated with high glucose peaks; therefore, it is necessary to control it, even when it is hard due to the difficult-to-manage DM. There is only 1 previous case report of WS with insulin pump that describes the benefits of continuous subcutaneous insulin infusion and tight metabolic control during pregnancy. CONCLUSION: The use of insulin pumps may be an effective treatment for DM in WS patients, mainly in terms of improving the prognosis of difficult-to-manage DM. American Association of Clinical Endocrinology 2023-05-06 /pmc/articles/PMC10382610/ /pubmed/37520764 http://dx.doi.org/10.1016/j.aace.2023.05.002 Text en © 2023 AACE. Published by Elsevier Inc. https://creativecommons.org/licenses/by-nc-nd/4.0/This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/). |
spellingShingle | Case Report Cardona, Manuela Ardila, Alejandro Gómez, Juan D. Román-González, Alejandro Wolfram Syndrome 1 in Two Brothers Treated with Insulin Pump |
title | Wolfram Syndrome 1 in Two Brothers Treated with Insulin Pump |
title_full | Wolfram Syndrome 1 in Two Brothers Treated with Insulin Pump |
title_fullStr | Wolfram Syndrome 1 in Two Brothers Treated with Insulin Pump |
title_full_unstemmed | Wolfram Syndrome 1 in Two Brothers Treated with Insulin Pump |
title_short | Wolfram Syndrome 1 in Two Brothers Treated with Insulin Pump |
title_sort | wolfram syndrome 1 in two brothers treated with insulin pump |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10382610/ https://www.ncbi.nlm.nih.gov/pubmed/37520764 http://dx.doi.org/10.1016/j.aace.2023.05.002 |
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