AI Article Synopsis
- - The study aimed to characterize patients with Autoimmune Polyglandular Syndrome (APS) type 4 at a specialized reference center, examining clinical records of affected individuals.
- - 111 patients were identified, with a mean onset age of 23.1 years; type I diabetes and celiac disease were the most common associated conditions, diagnosed either at the first evaluation or after a significant delay.
- - APS type 4 affects about 9 in 100,000 people, with type I diabetes being a crucial indicator; however, the findings highlight an unpredictable progression of the syndrome and note that 5% of affected women experienced premature ovarian failure.
Article Abstract
Purpose: To characterize patients with APS type 4 among those affected by APS diagnosed and monitored at our local Reference Center for Autoimmune Polyglandular Syndromes.
Methods: Monocentric observational retrospective study enrolling patients affected by APS diagnosed and monitored in a Reference Center. Clinical records were retrieved and analyzed.
Results: 111 subjects (51 males) were affected by APS type 4, mean age at the onset was 23.1 ± 15.1 years. In 15 patients the diagnosis of APS was performed during the first clinical evaluation, in the other 96 after a latency of 11 years (range 1-46). The most frequent diseases were type I diabetes mellitus and celiac disease, equally distributed among sexes.
Conclusions: The prevalence of APS type 4 is 9:100,000 people. Type I diabetes mellitus was the leading indicator of APS type 4 in 78% subjects and in 9% permitted the diagnosis occurring as second manifestation of the syndrome. Our data, showing that 50% of patients developed APS type 4 within the first ten years, don't suggest any particular follow-up time and, more importantly, don't specify any particular disease. It is important to emphasize that 5% of women developed premature ovarian failure.
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Source |
|---|---|
| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC10627240 | PMC |
| http://dx.doi.org/10.3389/fendo.2023.1236878 | DOI Listing |
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