PMID- 26992127
OWN - NLM
STAT- MEDLINE
DCOM- 20171024
LR  - 20191210
IS  - 1478-3231 (Electronic)
IS  - 1478-3223 (Linking)
VI  - 36
IP  - 10
DP  - 2016 Oct
TI  - The modified iron avidity index: a promising phenotypic predictor in HFE-related 
      haemochromatosis.
PG  - 1535-9
LID - 10.1111/liv.13121 [doi]
AB  - OBJECTIVE: Phenotypes of the HFE-related haemochromatosis vary considerably, 
      making it hard to predict the course of iron accumulation. The aim of this 
      retrospective study was to determine if the Iron Avidity Index (IAI) is a good 
      phenotypic predictor of the number of phlebotomies needed per year during 
      maintenance treatment (NPDMT) in patients with homozygous p.C282Y hereditary 
      haemochromatosis (HH). METHODS: Patients with HH homozygous for p.C282Y, on 
      maintenance treatment for at least 1 year were included. The IAI (ferritin level 
      at diagnosis/age at diagnosis) was calculated. RESULTS: Ninety-five patients were 
      included in the analysis. Linear regression analysis showed the confounding 
      effect of sex on the relationship between IAI and NPDMT. A modified IAI, adjusted 
      for sex, was calculated. As proton pump inhibitor (PPI) use was independently 
      associated with NPDMT, the group was split in PPI- and non-PPI-users. A positive 
      correlation between the modified IAI and the NPDMT was shown in both groups (PPI 
      r = 0.367, P = 0.023; non-PPI r = 0.453, P < 0.001). An ROC was computed to 
      measure the accuracy of the modified IAI to predict who needed 0-2 vs. >/=3 
      maintenance treatments per year. The AUROC in the PPI and non-PPI group were 
      respectively 0.576 (0.368-0.784) and 0.752 (0.606-0.899). CONCLUSION: The 
      modified IAI is a fairly good predictor in non-PPI-using homozygous C282Y HH 
      patients, to differentiate who needs >/=3 maintenance phlebotomies per year. 
      Therefore, this index might help to select patients that benefit from an 
      alternative less frequent therapy, e.g. erythrocytapheresis.
CI  - (c) 2016 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.
FAU - Verhaegh, Pauline L M
AU  - Verhaegh PL
AD  - Maastricht University Medical Center, Department of Internal Medicine, Division 
      of Gastroenterology and Hepatology, Maastricht, the Netherlands.
FAU - Moris, Wenke
AU  - Moris W
AD  - Faculty of Health, Medicine and Life Sciences, Maastricht University, Maastricht, 
      the Netherlands.
FAU - Koek, Ger H
AU  - Koek GH
AD  - Maastricht University Medical Center, Department of Internal Medicine, Division 
      of Gastroenterology and Hepatology, Maastricht, the Netherlands.
FAU - van Deursen, Cees Th B M
AU  - van Deursen CT
AD  - Maastricht University Medical Center, Department of Internal Medicine, Division 
      of Gastroenterology and Hepatology, Maastricht, the Netherlands. 
      c.van.deursen@atriummc.nl.
AD  - Zuyderland Medical Center, Department of Internal Medicine, Gastroenterology and 
      Geriatrics, Heerlen, the Netherlands. c.van.deursen@atriummc.nl.
LA  - eng
SI  - NTR/NTR4684
PT  - Journal Article
PT  - Validation Study
DEP - 20160406
PL  - United States
TA  - Liver Int
JT  - Liver international : official journal of the International Association for the 
      Study of the Liver
JID - 101160857
RN  - 0 (HFE protein, human)
RN  - 0 (Hemochromatosis Protein)
RN  - 0 (Proton Pump Inhibitors)
RN  - 9007-73-2 (Ferritins)
RN  - E1UOL152H7 (Iron)
SB  - IM
CIN - Liver Int. 2016 Nov;36(11):1713. PMID: 27124381
CIN - Liver Int. 2016 Nov;36(11):1714. PMID: 27744653
MH  - Aged
MH  - Female
MH  - Ferritins/*blood
MH  - Hemochromatosis/genetics/*therapy
MH  - Hemochromatosis Protein/genetics
MH  - Homozygote
MH  - Humans
MH  - Iron/*metabolism
MH  - Linear Models
MH  - Male
MH  - Middle Aged
MH  - Netherlands
MH  - Phlebotomy
MH  - Proton Pump Inhibitors/*therapeutic use
MH  - ROC Curve
MH  - Retrospective Studies
MH  - Severity of Illness Index
OTO - NOTNLM
OT  - C282Y
OT  - hereditary haemochromatosis
OT  - maintenance treatment
OT  - phenotypic predictor
OT  - phlebotomy
EDAT- 2016/03/19 06:00
MHDA- 2017/10/25 06:00
CRDT- 2016/03/19 06:00
PHST- 2015/10/29 00:00 [received]
PHST- 2016/03/07 00:00 [accepted]
PHST- 2016/03/19 06:00 [entrez]
PHST- 2016/03/19 06:00 [pubmed]
PHST- 2017/10/25 06:00 [medline]
AID - 10.1111/liv.13121 [doi]
PST - ppublish
SO  - Liver Int. 2016 Oct;36(10):1535-9. doi: 10.1111/liv.13121. Epub 2016 Apr 6.