Hemochromatosis medical therapy: Difference between revisions
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* Limiting intake of alcoholic beverages, [[vitamin C]] (increases iron absorption in the gut), red meat (high in [[iron]]) and potential causes of food poisoning (shellfish, seafood). | * Limiting intake of alcoholic beverages, [[vitamin C]] (increases iron absorption in the gut), red meat (high in [[iron]]) and potential causes of food poisoning (shellfish, seafood). | ||
* Increasing intake of substances that inhibit iron absorption, such as high-[[tannin]] [[tea]], [[calcium]], and foods containing [[Oxalic acid|oxalic]] and [[phytic acid]]s (these must be consumed at the same time as the iron-containing foods in order to be effective.) | * Increasing intake of substances that inhibit iron absorption, such as high-[[tannin]] [[tea]], [[calcium]], and foods containing [[Oxalic acid|oxalic]] and [[phytic acid]]s (these must be consumed at the same time as the iron-containing foods in order to be effective.) | ||
====Screening and diagnosis of hemochromatosis.WT, wild type; HII, histologic iron index; CII, chemical iron index; HH, hereditary hemochromatosis<ref name="pmid22675794">{{cite journal| author=Bacon BR| title=Hemochromatosis: discovery of the HFE gene. | journal=Mo Med | year= 2012 | volume= 109 | issue= 2 | pages= 133-6 | pmid=22675794 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22675794 }}</ref><ref name="pmid20492323">{{cite journal| author=Asia-Pacific Working Party on Prevention of Hepatocellular Carcinoma| title=Prevention of hepatocellular carcinoma in the Asia-Pacific region: consensus statements. | journal=J Gastroenterol Hepatol | year= 2010 | volume= 25 | issue= 4 | pages= 657-63 | pmid=20492323 | doi=10.1111/j.1440-1746.2009.06167.x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20492323 }}</ref><ref name="pmid25976957">{{cite journal| author=Adams PC| title=Epidemiology and diagnostic testing for hemochromatosis and iron overload. | journal=Int J Lab Hematol | year= 2015 | volume= 37 Suppl 1 | issue= | pages= 25-30 | pmid=25976957 | doi=10.1111/ijlh.12347 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25976957 }}</ref><ref name="pmid25454304">{{cite journal| author=Salgia RJ, Brown K| title=Diagnosis and management of hereditary hemochromatosis. | journal=Clin Liver Dis | year= 2015 | volume= 19 | issue= 1 | pages= 187-98 | pmid=25454304 | doi=10.1016/j.cld.2014.09.011 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25454304 }}</ref><ref name="pmid23418762">{{cite journal| author=Crownover BK, Covey CJ| title=Hereditary hemochromatosis. | journal=Am Fam Physician | year= 2013 | volume= 87 | issue= 3 | pages= 183-90 | pmid=23418762 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23418762 }}</ref><ref name="pmid25864215">{{cite journal| author=Adams PC, Barton JC, Guo H, Alter D, Speechley M| title=Serum ferritin is a biomarker for liver mortality in the Hemochromatosis and Iron Overload Screening Study. | journal=Ann Hepatol | year= 2015 | volume= 14 | issue= 3 | pages= 348-53 | pmid=25864215 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25864215 }}</ref><ref name="pmid23862168">{{cite journal| author=Adams PC, McLaren CE, Speechley M, McLaren GD, Barton JC, Eckfeldt JH| title=HFE mutations in Caucasian participants of the Hemochromatosis and Iron Overload Screening study with serum ferritin level <1000 µg/L. | journal=Can J Gastroenterol | year= 2013 | volume= 27 | issue= 7 | pages= 390-2 | pmid=23862168 | doi= | pmc=3956024 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23862168 }}</ref><ref name="pmid25314357">{{cite journal| author=Lim A, Speechley M, Adams PC| title=Predicting C282Y homozygote genotype for hemochromatosis using serum ferritin and transferrin saturation values from 44,809 participants of the HEIRS study. | journal=Can J Gastroenterol Hepatol | year= 2014 | volume= 28 | issue= 9 | pages= 502-4 | pmid=25314357 | doi= | pmc=4205907 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25314357 }}</ref>==== | |||
{{familytree/start|summary=Algorithm for screening and diagnosis of hemochromatosis.WT, wild type; HII, histologic iron index; CII, chemical iron index; HH, hereditary hemochromatosis.}} | |||
{{familytree| | | | | | | | | | | | |A01| | | | | | | | | |A01=Serum Transferin Saturation<br>TS}} | |||
{{familytree| | | | | | | | | |,|-|-|-|^|-|-|-|.| | | | | | |}} | |||
{{familytree| | | | | | | | | |!| | | | | | | |!| | | | | |}} | |||
{{familytree| | | | | | | | |B01| | | | | |B02| | | | |B01=<50% premenupasal females<br><60% men, postmenupasal women|B02=≥50% premenupasal females<br>≥60% men, postmenupasal women}} | |||
{{familytree| | | | | | | | | |!| | | | | | | |!| | | | |}} | |||
{{familytree| | | | | | | | | |!| | | | | | |Q01| | | | |Q01=1 Repeat Transferin Saturation TS<br>2 Serum Feretin SF}} | |||
{{familytree| | | | | | | | | |!| | |,|-|-|-|-|^|-|-|.| |}} | |||
{{familytree| | | | | | | | |C01| |!| | | | | | | |!| | |C01=Repeat testing every 5 year}} | |||
{{familytree| | | | | | | | |,|-|-|-|'| | | | | | | |!| | |}} | |||
{{familytree| | | | | | | | |!| | | | | | | | | | | |!| |}} | |||
{{familytree| | | | | | | | |D01| | | | | | | | |D02| |D01=TS:<50% premenupasal females<br>TS: <60% men, postmenupasal women<br>SF: 20-250μg/L premenupasal females<br>SF: 10-120μg/L men, postmenupasal women|D02=TS:≥50% premenupasal females<br>TS: ≥60% men, postmenupasal women<br>SF:>200 μg/L premenupasal females<br>SF:>300 μg/L men, postmenupasal women}} | |||
{{familytree| | | | | | | | | |!| | | | | | |,|-|-|-|^|-|-|-|-|.|}} | |||
{{familytree| | | | | | | | |E01| | | | |E02| | | | | | |E03|E01=Repeat TS and SF every 2-3 year|E02=Serum Feretin<1000 μg/L|E03=Serum Feretin>1000 μg/L}} | |||
{{familytree| | | | | | | | | | | | | | | | |!|,|-|-|-|-|-|-|^|-|-|.|}} | |||
{{familytree| | | | | | | | | | | | | | | |F01| | | | | | | | |F02|F01=Geno-typing|F02=Liver biopsy}} | |||
{{familytree| | | | | | | | |,|-|-|v|-|-|-|^|-|-|v|-|-|.| | | | |,|-|^|-|.| |}} | |||
{{familytree| | | | | | | |G01| |G02| | | |G03| |G04| | |G05| |G06|G01=WT/WT genotype|G02=C282Y/WT genotype|G03=C282Y/H63D genotype|G04=C282Y/C282Y genotype|G05=Histological iron index<0.15<br>Chemical iron index<2.0|G06=Histological iron index>0.15<br>Chemical iron index>2.0}} | |||
{{familytree| | | | | | | | |`|-|V|-|'| | | | | |`|-|V|-|'| | | | |!| | |!|}} | |||
{{familytree| | | | | | | | | |H01| | | | | | | |H02| | | | |H03| |H04|H01=Secondray hemochromatosis|H02=Phelebotomy to maintain Serum Feretin|H03=Repeat TS and SF after 2-3 year|H04=Phelebotomy to maintain Serum Feretin}} | |||
{{familytree| | | | | | | | | | |!| | | | | | | | | |!| | |}} | |||
{{familytree| | | | | | | | | |K01| | | | | | | |K02|K01=Screen family with Transferin Saturation & Serum Feretin if atypical HH suspected|K02=Screen family with genotyping}} | |||
{{familytree| | | | | | | | | | | | | | | | | | | | |!| |}} | |||
{{familytree| | | | | | | | | | | | | | | | | | | |J01|J01=Moniter Transferin Saturation & Serum Feretin in subclinical members}} | |||
{{familytree/end}} | |||
==References== | ==References== |
Revision as of 03:35, 9 November 2017
Hemochromatosis Microchapters |
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:
Overview
Medical Therapy
- Treatment is initiated when ferritin levels reach 300 micrograms per litre (or 200 in nonpregnant premenopausal women).
- Treatment of organ damage (heart failure with diuretics and ACE inhibitor therapy).
- Limiting intake of alcoholic beverages, vitamin C (increases iron absorption in the gut), red meat (high in iron) and potential causes of food poisoning (shellfish, seafood).
- Increasing intake of substances that inhibit iron absorption, such as high-tannin tea, calcium, and foods containing oxalic and phytic acids (these must be consumed at the same time as the iron-containing foods in order to be effective.)
Screening and diagnosis of hemochromatosis.WT, wild type; HII, histologic iron index; CII, chemical iron index; HH, hereditary hemochromatosis[1][2][3][4][5][6][7][8]
Serum Transferin Saturation TS | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
<50% premenupasal females <60% men, postmenupasal women | ≥50% premenupasal females ≥60% men, postmenupasal women | ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
1 Repeat Transferin Saturation TS 2 Serum Feretin SF | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
Repeat testing every 5 year | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
TS:<50% premenupasal females TS: <60% men, postmenupasal women SF: 20-250μg/L premenupasal females SF: 10-120μg/L men, postmenupasal women | TS:≥50% premenupasal females TS: ≥60% men, postmenupasal women SF:>200 μg/L premenupasal females SF:>300 μg/L men, postmenupasal women | ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
Repeat TS and SF every 2-3 year | Serum Feretin<1000 μg/L | Serum Feretin>1000 μg/L | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
Geno-typing | Liver biopsy | ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
WT/WT genotype | C282Y/WT genotype | C282Y/H63D genotype | C282Y/C282Y genotype | Histological iron index<0.15 Chemical iron index<2.0 | Histological iron index>0.15 Chemical iron index>2.0 | ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
Secondray hemochromatosis | Phelebotomy to maintain Serum Feretin | Repeat TS and SF after 2-3 year | Phelebotomy to maintain Serum Feretin | ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
Screen family with Transferin Saturation & Serum Feretin if atypical HH suspected | Screen family with genotyping | ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
Moniter Transferin Saturation & Serum Feretin in subclinical members | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
References
- ↑ Bacon BR (2012). "Hemochromatosis: discovery of the HFE gene". Mo Med. 109 (2): 133–6. PMID 22675794.
- ↑ Asia-Pacific Working Party on Prevention of Hepatocellular Carcinoma (2010). "Prevention of hepatocellular carcinoma in the Asia-Pacific region: consensus statements". J Gastroenterol Hepatol. 25 (4): 657–63. doi:10.1111/j.1440-1746.2009.06167.x. PMID 20492323.
- ↑ Adams PC (2015). "Epidemiology and diagnostic testing for hemochromatosis and iron overload". Int J Lab Hematol. 37 Suppl 1: 25–30. doi:10.1111/ijlh.12347. PMID 25976957.
- ↑ Salgia RJ, Brown K (2015). "Diagnosis and management of hereditary hemochromatosis". Clin Liver Dis. 19 (1): 187–98. doi:10.1016/j.cld.2014.09.011. PMID 25454304.
- ↑ Crownover BK, Covey CJ (2013). "Hereditary hemochromatosis". Am Fam Physician. 87 (3): 183–90. PMID 23418762.
- ↑ Adams PC, Barton JC, Guo H, Alter D, Speechley M (2015). "Serum ferritin is a biomarker for liver mortality in the Hemochromatosis and Iron Overload Screening Study". Ann Hepatol. 14 (3): 348–53. PMID 25864215.
- ↑ Adams PC, McLaren CE, Speechley M, McLaren GD, Barton JC, Eckfeldt JH (2013). "HFE mutations in Caucasian participants of the Hemochromatosis and Iron Overload Screening study with serum ferritin level <1000 µg/L". Can J Gastroenterol. 27 (7): 390–2. PMC 3956024. PMID 23862168.
- ↑ Lim A, Speechley M, Adams PC (2014). "Predicting C282Y homozygote genotype for hemochromatosis using serum ferritin and transferrin saturation values from 44,809 participants of the HEIRS study". Can J Gastroenterol Hepatol. 28 (9): 502–4. PMC 4205907. PMID 25314357.