Abstract | OBJECTIVE: DESIGN:
Serum proteins were analyzed in 26 biochemically active AIP patients that were classified according to the EPI (European Porphyria Initiative) guidelines as follows: (i) patients who presented a single acute attack having remained so far free of clinical symptoms; (ii) patients who present recurrent attacks or chronic symptoms associated with exacerbations of AIP. RESULTS: Most of the serum proteins were within normal limits, however insulin-like growth factor 1 (IGF-1) was decreased in 53.8% of AIP patients (z-score = -2.86 +/- 0.37) and transthyretin ( prealbumin) was found significantly decreased in 38.5% of them. The IGF-1 z-score was lower in group B versus group A patients (-2.66 vs. -1.43; P = 0.024). The coincident decrease of both IGF-1 and transthyretin was associated with worsening of the clinical condition. CONCLUSIONS: This first study in humans suggests that the clinical expression AIP is associated with a state of under-nutrition and/or with hepatic inflammation due to the sustained accumulation of heme-precursors. We propose the use of both IGF-1 and transthyretin as biomarkers of disease morbidity/severity for the clinical follow-up of AIP patients.
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Authors | C Delaby, J To-Figueras, J C Deybach, R Casamitjana, H Puy, C Herrero |
Journal | Journal of internal medicine
(J Intern Med)
Vol. 266
Issue 3
Pg. 277-85
(Sep 2009)
ISSN: 1365-2796 [Electronic] England |
PMID | 19570056
(Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
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Chemical References |
- Biomarkers
- Prealbumin
- Insulin-Like Growth Factor I
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Topics |
- Adult
- Biomarkers
(blood)
- Body Mass Index
- Case-Control Studies
- Female
- Follow-Up Studies
- Humans
- Insulin-Like Growth Factor I
(analysis)
- Male
- Middle Aged
- Morbidity
- Porphyria, Acute Intermittent
(blood)
- Prealbumin
(analysis)
- Statistics, Nonparametric
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