Title:Ketosis-prone Diabetes and Hypogonadism: A New Clinical Association to
be Aware of ?
Volume: 23
Issue: 12
Author(s): Andrea Graziani, Riccardo Maria Pollis*, Benedetta Maria Bonora, Carla Scaroni and Chiara Sabbadin
Affiliation:
- Department of Medicine, University of Padova, Padova, Italy
- Endocrine Disease Unit, University-Hospital of Padova,
Padova, Italy
Keywords:
Ketosis prone diabetes, diabetes, hypogonadism, klinefelter syndrome, primary ovarian failure, ketoacidosis.
Abstract:
Background: Ketosis-prone diabetes (KPD) is an emerging entity, sharing features of both
type 1 diabetes mellitus and type 2 diabetes mellitus. Patients with KPD usually present with diabetic
ketoacidosis without the classic phenotype of autoimmune type 1 diabetes. In most cases, they are
Afro-American adults, who require insulin therapy for the management of acute decompensation, then
usually encountering insulin-free remission for prolonged periods of time with diet or with non-insulin
agents. Meanwhile, hypogonadism is a known condition that could be associated with higher risk of
developing both type 1 and type 2 diabetes and could be a risk factor for decompensated diabetes. The
association of KPD and hypogonadism is reported for the first time in literature.
Case Presentation: Here we report two peculiar cases of young African patients, affected by KPD and
hypergonadotropic hypogonadism, respectively Klinefelter’s syndrome and primary ovarian failure.
Both patients were treated promptly for the ketoacidosis with intravenous fluids combined with continuous
insulin infusion, and then switched to subcutaneous regimen. After the correct clinical evaluation,
oral antidiabetic drugs were added.
Conclusion: KPD remains an under-recognized and under-diagnosed type of diabetes. As hypogonadism
is strongly linked to dysmetabolic disorders, the evaluation of sex hormones should be performed
at the onset of diabetes. Further studies should investigate the hypothalamic-pituitary-gonadal axis
and its role in the development of KDP and its manifestations and complications.
