Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2022 Aug 19;12(8):1270.
doi: 10.3390/life12081270.

Dental and Periodontal Health in Acute Intermittent Porphyria

Affiliations

Dental and Periodontal Health in Acute Intermittent Porphyria

Elin Storjord et al. Life (Basel). .

Abstract

In the inherited metabolic disorder acute intermittent porphyria (AIP), high sugar intake prevents porphyric attacks due to the glucose effect and the following high insulin levels that may lower AIP disease activity. Insulin resistance is a known risk factor for periodontitis and sugar changes diabetogenic hormones and affects dental health. We hypothesized differences in homeostasis model assessment (HOMA) scores for insulin resistance in AIP cases vs. controls and in those with periodontitis. Our aim was to systematically study dental health in AIP as poor dental health was previously only described in case reports. Further, we aimed to examine if poor dental health and kidney failure might worsen AIP as chronic inflammation and kidney failure might increase disease activity. In 47 AIP cases and 47 matched controls, X-rays and physical examination of clinical attachment loss (CAL), probing pocket depth (PPD), and decayed missing filled teeth (DMFT) were performed. Dietary intake was evaluated through a diet logbook. Plasma cytokines and diabetogenic hormones were measured using multiplex technology and urine porphobilinogen and kidney and liver function by routine methods. An excel spreadsheet from the University of Oxford was used to estimate HOMA scores; beta cell function, HOMA%B (%B), insulin sensitivity, HOMA%S (%S), and insulin resistance HOMA-IR (IR), based on glucose and plasma (P) C-peptide. The Wilcoxon matched-pairs signed rank test, the Mann−Whitney U-test, and Spearman’s non-parametric correlation were used. Insulin (p = 0.007) and C-peptide (p = 0.006) were higher in the AIP cases with periodontitis versus those without. In AIP patients, the liver fibrosis index 4 correlated with DMFT (p < 0.001) and CAL ≥4 mm (p = 0.006); the estimated glomerular filtration rate correlated with DMFT (p < 0.001) and CAL ≥4 mm (p = 0.02). CAL ≥4 mm was correlated with chemokine ligand 11 and interleukin (IL)-13 (p = 0.04 for both), and PPD >5 mm was correlated with plasminogen activator inhibitor-1 (p = 0.003) and complement component 3 (p = 0.02). In conclusion, dental health in AIP cases was correlated with insulin resistance, inflammatory markers, and biomarkers of kidney and liver function, demonstrating that organ damage in the kidney and liver are associated with poorer dental health.

Keywords: cytokines; delta aminolevulinic acid; immunity; inflammation; innate immunity; periodontal disease; periodontitis; porphobilinogen; systemic health.

PubMed Disclaimer

Conflict of interest statement

Amy Dickey reports receiving honoraria from Alnylam Pharmaceuticals for speaking engagements and porphyria-related consulting, and reports consulting honorarium from Recordati. No other potential conflict of interests were reported by the authors.

Figures

Figure 1
Figure 1
Possible pathways between acute intermittent porphyria (AIP) and dental health. Bård Ove Karlsen created Figure 1 in Adobe Illustrator version CC 2019 (23.0.0), Available online: https://www.adobe.com/products/illustrator.html (accessed on 10–27 June 2019).

Similar articles

Cited by

References

    1. Strand L.J., Felsher B.F., Redeker A.G., Marver H.S. Heme biosynthesis in intermittent acute prophyria: Decreased hepatic conversion of porphobilinogen to porphyrins and increased delta aminolevulinic acid synthetase activity. Proc. Natl. Acad. Sci. USA. 1970;67:1315–1320. doi: 10.1073/pnas.67.3.1315. - DOI - PMC - PubMed
    1. Puy H., Gouya L., Deybach J.C. Porphyrias. Lancet. 2010;375:924–937. doi: 10.1016/S0140-6736(09)61925-5. - DOI - PubMed
    1. Bissell D.M., Anderson K.E., Bonkovsky H.L. Porphyria. N. Engl. J. Med. 2017;377:862–872. doi: 10.1056/NEJMra1608634. - DOI - PubMed
    1. Floderus Y., Shoolingin-Jordan P.M., Harper P. Acute intermittent porphyria in Sweden. Molecular, functional and clinical consequences of some new mutations found in the porphobilinogen deaminase gene. Clin. Genet. 2002;62:288–297. doi: 10.1034/j.1399-0004.2002.620406.x. - DOI - PubMed
    1. Pischik E., Kauppinen R. An update of clinical management of acute intermittent porphyria. Appl. Clin. Genet. 2015;8:201–214. doi: 10.2147/TACG.S48605. - DOI - PMC - PubMed

LinkOut - more resources