Affiliation:
1. Endocrinology Department, Universidad Militar Nueva Granada, Bogota, Colombia
2. Endocronology Department,
Hospital Militar Central, Bogota, Colombia
Abstract
Introduction:
Maturity-onset diabetes of the young (MODY) is a rare disease due to a
single gene mutation that affects several family members in most cases. The Krüppel-like factor 11
(KLF11) gene mutation is associated with decreased insulin sensitivity to high glucose levels. KLF
11 has been implicated in the pathogenesis of MODY type 7 but given its low prevalence, prolonged
subclinical period, and the emergence of new information, doubts are raised about its association.
Methods:
A literature search of the PubMed, Scopus, and EBSCO databases was performed. The
terms “Diabetes Mellitus, Type 2/genetics”, “Mason-Type Diabetes” , “Maturity-Onset diabetes of
the young”, “KLF11 protein, human”, and “Maturity-Onset Diabetes of the Young, Type 7” were
used”., “Diagnosis” The search selection was not standardized.
Results:
The KLF1 mutation is rare and represents <1% of the mutations associated with monogenic
diabetes. Its isolation in European family lines in the first studies and the emergence of new
variants pose new diagnostic challenges. This article reviews the definition, epidemiology, pathophysiology,
diagnosis, and treatment of MODY type 7.
Conclusion:
MODY type 7 diabetes represents a rare form of monogenic diabetes with incomplete
penetrance. Given its rarity, its association with impaired glucose metabolism has been questioned.
Strict evaluation of glycemic control and the appearance of microvascular complications are key
areas in the follow-up of patients diagnosed with MODY 7. More studies will be required to characterize
the population with KLF11 mutation and clarify its correlation with MODY.
Publisher
Bentham Science Publishers Ltd.
Subject
Endocrinology,Endocrinology, Diabetes and Metabolism
Cited by
1 articles.
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