Syndromes of ketosis-prone diabetes mellitus
Open accessAbstract
Ketosis-prone diabetes (KPD) is a widespread, emerging, heterogeneous syndrome characterized by patients who present with diabetic ketoacidosis or unprovoked ketosis but do not necessarily have the typical phenotype of autoimmune type 1 diabetes. Multiple, severe forms of beta-cell dysfunction appear to underlie the pathophysiology of KPD. Until recently, the syndrome has lacked an accurate, clinically relevant and etiologically useful classification scheme. We have utilized a large, longitudinally followed, heterogeneous, multiethnic cohort of KPD patients to identify four clinically and pathophysiologically distinct subgroups that are separable by the presence or absence of beta-cell autoimmunity and the presence or absence of beta-cell functional reserve. The resulting "Abeta" classification system of KPD has proven to be highly accurate and predictive of such clinically important outcomes as glycemic control and insulin dependence, as well as an aid to biochemical and molecular investigations into novel causes of beta-cell dysfunction. In this review, we describe the current state of knowledge in regard to the natural history, pathophysiology, and treatment of the subgroups of KPD, with an emphasis on recent advances in understanding their immunological and genetic bases.
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Individuals with autoantibody-negative diabetes of African or Asian ancestry may suffer from episodic DKA and exhibit varying degrees of insulin deficiency between episodes.
“Individuals with autoantibody-negative diabetes of African or Asian ancestry may suffer from episodic DKA and exhibit varying degrees of insulin de fi ciency between episodes (70).”
From 2. Diagnosis and Classification of Diabetes: <i>Standards of Care in Diabetes—2024</i> (2024) · cites this paper as [70]
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2. Classification and Diagnosis of Diabetes: <i>Standards of Medical Care in Diabetes—2022</i>
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- Authors
- Balasubramanyam, A., Nalini, R., Hampe, C. S., & Maldonado, M.
- Affiliations
- Baylor College of Medicine (US)
- University of Washington (US)
- Novartis (Switzerland) (CH)
- Corresponding author
- Balasubramanyam, Ashok
- Venue
- Endocrine Reviews
- Publisher
- The Endocrine Society
- Published
- 2008-02-21
- Volume
- 29
- Issue
- 3
- Pages
- 292-302
- ISSN
- 0163-769X (ISSN-L)
- 1945-7189
- PMC
- PMC2528854
- OpenAlex
- W2099270514
- Cited by
- 200
- References
- 55
- Citations by year
- 2026: 13 · 2025: 10 · 2024: 10 · 2023: 13 · 2022: 18 · 2021: 14 · 2020: 8 · 2019: 15 · 2018: 12 · 2017: 7 · 2016: 7 · 2015: 12 · 2014: 7 · 2013: 10 · 2012: 12
- FWCI
- 6.08
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- Endocrine Reviews (publishedVersion)
- PubMed Central (publishedVersion)
- PubMed (publishedVersion)
- Funders
- University of Washington
- National Heart, Lung, and Blood Institute (R01 HL73969)
- National Institute of Diabetes and Digestive and Kidney Diseases (R01DK026190)
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Diabetes and associated disorders
- Health Sciences › Medicine › Surgery › Pancreatic function and diabetes
- Health Sciences › Medicine › Endocrinology, Diabetes and Metabolism › Diabetes Management and Research
- Keywords
- KPD, diabetic ketoacidosis, beta-cell dysfunction, islet autoimmunity, disease subgroups, pathophysiology, natural history (disease), insulin dependence, glycemic control, genetic basis
- MeSH terms
- Adolescent, Adult, Diabetes Mellitus, Type 1, Female, Humans, Male, Middle Aged, Syndrome, Insulin-Secreting Cells
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