New insights on hyperglycemia in 17-hydroxylase/17,20-lyase deficiency
- PMID: 35937831
- PMCID: PMC9354396
- DOI: 10.3389/fendo.2022.917420
New insights on hyperglycemia in 17-hydroxylase/17,20-lyase deficiency
Abstract
Objective: The adrenal glands of patients with 17-hydroxylase/17,20-lyase deficiency (17OHD) synthesize excessive 11-deoxycorticosterone(DOC) and progesterone, and produce less amount of sex steroid production. Mineralocorticoids and sex hormones play an important role in regulating glucose homeostasis. This study aimed to describe the glucose metabolism in 17OHD patients diagnosed at Peking Union Medical College Hospital (PUMCH).
Design/methods: A total of 69 patients diagnosed with 17OHD after adolescence in PUMCH from 1995 to June in 2021. Among them 23 patients underwent a 3-hours oral glucose tolerance test (3hOGTT) after being diagnosed with 17OHD. Insulin response in patients with normal glucose tolerance (NGT) were further compared between the study two groups with different kalemia status. Another 19 patients were followed up to 30 years and older. All clinical data were obtained from the hospital information system of PUMCH.
Results: Baseline: (1) The average body mass index(BMI) of all patients at baseline was 20.3 ± 3.7kg/m2. Twenty-three patients underwent 3hOGTT, of whom three were diagnosed with diabetes mellitus, and one with impaired glucose tolerance (IGT). Positive correlation between the ratio of progesterone to upper limit of normal range (P times) and hyperglycaemia was exist(r=0.707, P=0.005). (2) In 19 NGT patients, the insulin concentrations at 0 minute, results of the homeostasis model assessment for β-cell function and insulin resistance were lower in the hypokalaemia group than in the normal kalemia group(7.0(5.8-13.2) vs 12.4(8.9-14.9) μIU/ml, P=0.017; 115.5(88.2-240.9) vs 253.1(177.2-305.8), P=0.048; 1.54(1.17-2.61) vs 2.47(1.91-2.98), P=0.022, respectively). Follow-up: Four patients had IGT, while seven patients had diabetes mellitus. Of the 19 patients,11 had hyperglycaemia. P times was significantly higher(7.6(5.0-11.0) vs 3.75(2.2-5.3), P=0.008) in hyperglycemia group than in the normal glucose group.
Conclusions: Abnormal glucose metabolism was common in 17OHD patients, which was possibly associated with hypokalaemia and high progesterone levels. Routine monitoring on glucose metabolism in 17OHD patient should be conducted.
Keywords: 17-hydroxylase/17; 20-lyase deficiency; diabetes; high progesterone; hypokelamia; impaired glucose tolerance.
Copyright © 2022 Xu, Lu, Tong, Chen, Li, Zhang, Ping and Li.
Conflict of interest statement
The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.
Similar articles
-
Glucose metabolic disorder in Klinefelter syndrome: a retrospective analysis in a single Chinese hospital and literature review.BMC Endocr Disord. 2021 Dec 1;21(1):239. doi: 10.1186/s12902-021-00893-5. BMC Endocr Disord. 2021. PMID: 34852815 Free PMC article. Review.
-
Steroid 17-hydroxylase and 17,20-lyase deficiencies, genetic and pharmacologic.J Steroid Biochem Mol Biol. 2017 Jan;165(Pt A):71-78. doi: 10.1016/j.jsbmb.2016.02.002. Epub 2016 Feb 6. J Steroid Biochem Mol Biol. 2017. PMID: 26862015 Free PMC article. Review.
-
Partial 17alpha-hydroxylase/17,20-lyase deficiency-clinical report of five Chinese 46,XX cases.Gynecol Endocrinol. 2008 Jul;24(7):362-7. doi: 10.1080/09513590802194051. Gynecol Endocrinol. 2008. PMID: 18645707
-
Metabolic characteristics of subjects with normal glucose tolerance and 1-h hyperglycaemia.Clin Endocrinol (Oxf). 2008 Oct;69(4):575-9. doi: 10.1111/j.1365-2265.2008.03209.x. Epub 2008 Jan 31. Clin Endocrinol (Oxf). 2008. PMID: 18248638
-
Impact of glucagon response on postprandial hyperglycemia in men with impaired glucose tolerance and type 2 diabetes mellitus.Metabolism. 2005 Sep;54(9):1168-73. doi: 10.1016/j.metabol.2005.03.024. Metabolism. 2005. PMID: 16125528
Cited by
-
Combined Effects of ESRα DNA Methylation and Progesterone on Glucose Metabolic Disorders: The Henan Rural Cohort Study.Nutrients. 2023 Mar 29;15(7):1659. doi: 10.3390/nu15071659. Nutrients. 2023. PMID: 37049500 Free PMC article.
References
-
- Kurnaz E, Kartal Baykan E, Türkyılmaz A, Yaralı O, Yavaş Abalı Z, Turan S, et al. . Genotypic sex and severity of the disease determine the time of clinical presentation in steroid 17α-Hydroxylase/17,20-Lyase deficiency. Horm Res Paediatr (2020) 93(9-10):558–66. doi: 10.1159/000515079 - DOI - PubMed
Publication types
MeSH terms
Substances
LinkOut - more resources
Full Text Sources
Medical