Consensus guidelines for diagnosis and management of anemia in epidermolysis bullosa.
Anemia
Chronic anemia of inflammation
Epidermolysis bullosa
Iron deficiency
Recessive dystrophic epidermolysis bullosa
Journal
Orphanet journal of rare diseases
ISSN: 1750-1172
Titre abrégé: Orphanet J Rare Dis
Pays: England
ID NLM: 101266602
Informations de publication
Date de publication:
23 02 2023
23 02 2023
Historique:
received:
07
02
2022
accepted:
17
07
2022
entrez:
24
2
2023
pubmed:
25
2
2023
medline:
3
3
2023
Statut:
epublish
Résumé
Anemia is a common complication of severe forms of epidermolysis bullosa (EB). To date, there are no guidelines outlining best clinical practices to manage anemia in the EB population. The objective of this manuscript is to present the first consensus guidelines for the diagnosis and management of anemia in EB. Due to the lack of high-quality evidence, a consensus methodology was followed. An initial survey exploring patient preferences, concerns and symptoms related to anemia was sent to EB patients and their family members. A second survey was distributed to EB experts and focused on screening, diagnosis, monitoring and management of anemia in the different types of EB. Information from these surveys was collated and used by the panel to generate 26 consensus statements. Consensus statements were sent to healthcare providers that care for EB patients through EB-Clinet. Statements that received more than 70% approval (completely agree/agree) were adopted. The end result was a series of 6 recommendations which include 20 statements that will help guide management of anemia in EB patients. In patients with moderate to severe forms of EB, the minimum desirable level of Hb is 100 g/L. Treatment should be individualized. Dietary measures should be offered as part of management of anemia in all EB patients, oral iron supplementation should be used for mild anemia; while iron infusion is reserved for moderate to severe anemia, if Hb levels of > 80-100 g/L (8-10 g/dL) and symptomatic; and transfusion should be administered if Hb is < 80 g/L (8 g/dL) in adults and < 60 g/L (6 g/dL) in children.
Sections du résumé
BACKGROUND
Anemia is a common complication of severe forms of epidermolysis bullosa (EB). To date, there are no guidelines outlining best clinical practices to manage anemia in the EB population. The objective of this manuscript is to present the first consensus guidelines for the diagnosis and management of anemia in EB.
RESULTS
Due to the lack of high-quality evidence, a consensus methodology was followed. An initial survey exploring patient preferences, concerns and symptoms related to anemia was sent to EB patients and their family members. A second survey was distributed to EB experts and focused on screening, diagnosis, monitoring and management of anemia in the different types of EB. Information from these surveys was collated and used by the panel to generate 26 consensus statements. Consensus statements were sent to healthcare providers that care for EB patients through EB-Clinet. Statements that received more than 70% approval (completely agree/agree) were adopted.
CONCLUSIONS
The end result was a series of 6 recommendations which include 20 statements that will help guide management of anemia in EB patients. In patients with moderate to severe forms of EB, the minimum desirable level of Hb is 100 g/L. Treatment should be individualized. Dietary measures should be offered as part of management of anemia in all EB patients, oral iron supplementation should be used for mild anemia; while iron infusion is reserved for moderate to severe anemia, if Hb levels of > 80-100 g/L (8-10 g/dL) and symptomatic; and transfusion should be administered if Hb is < 80 g/L (8 g/dL) in adults and < 60 g/L (6 g/dL) in children.
Identifiants
pubmed: 36823529
doi: 10.1186/s13023-022-02448-w
pii: 10.1186/s13023-022-02448-w
pmc: PMC9948325
doi:
Substances chimiques
Iron
E1UOL152H7
Types de publication
Journal Article
Review
Research Support, N.I.H., Extramural
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Pagination
38Subventions
Organisme : NICHD NIH HHS
ID : K23 HD091295
Pays : United States
Informations de copyright
© 2023. The Author(s).
Références
Am J Hematol. 2017 Oct;92(10):1068-1078
pubmed: 28612425
Br J Dermatol. 2023 Feb 10;188(2):306-307
pubmed: 36763865
J Health Serv Res Policy. 1999 Oct;4(4):236-48
pubmed: 10623041
BMJ. 2017 Jun 15;357:j2513
pubmed: 28620083
Gut. 2004 Aug;53(8):1190-7
pubmed: 15247190
Gastroenterology. 2020 Sep;159(3):1085-1094
pubmed: 32810434
Cochrane Database Syst Rev. 2014 Dec 31;(12):CD010640
pubmed: 25550190
Pediatr Dermatol. 2020 Sep;37(5):817-820
pubmed: 32677046
Clin Toxicol (Phila). 2005;43(6):553-70
pubmed: 16255338
AMIA Annu Symp Proc. 2006;:359-63
pubmed: 17238363
J Eur Acad Dermatol Venereol. 1999 Mar;12(2):181-2
pubmed: 10343955
Int J Womens Dermatol. 2015 Feb 18;1(1):37-40
pubmed: 28491953
CMAJ. 2010 Dec 14;182(18):E839-42
pubmed: 20603348
World J Gastroenterol. 2009 Oct 7;15(37):4638-43
pubmed: 19787826
Pediatr Dermatol. 2018 Sep;35(5):e319-e320
pubmed: 29974505
J Gen Intern Med. 1992 Mar-Apr;7(2):145-53
pubmed: 1487761
Br J Dermatol. 2012 Feb;166(2):354-61
pubmed: 21895617
Lancet Haematol. 2017 Nov;4(11):e524-e533
pubmed: 29032957
Rev Peru Med Exp Salud Publica. 2017 Apr-Jun;34(2):201-208
pubmed: 29177377
J Grad Med Educ. 2013 Dec;5(4):541-2
pubmed: 24454995
Hemodial Int. 2017 Jun;21 Suppl 1:S83-S92
pubmed: 28371203
Am J Clin Nutr. 2001 Jan;73(1):93-8
pubmed: 11124756
Br J Dermatol. 1999 Apr;140(4):773
pubmed: 10233353
J Pediatr. 1998 May;132(5):871-3
pubmed: 9602203
J Am Acad Dermatol. 2009 Sep;61(3):367-84; quiz 385-6
pubmed: 19700010
Nutrients. 2018 Mar 09;10(3):
pubmed: 29522446
Br J Dermatol. 2020 Jun;182(6):1437-1448
pubmed: 31487386
Ann N Y Acad Sci. 2019 Aug;1450(1):126-146
pubmed: 30652320
Dermatol Clin. 2010 Apr;28(2):271, ix
pubmed: 20447491
Pediatr Dermatol. 2006 Nov-Dec;23(6):580-5
pubmed: 17156003
Clin Dermatol. 2010 Nov-Dec;28(6):627-43
pubmed: 21034987
Am Fam Physician. 2013 Jan 15;87(2):98-104
pubmed: 23317073
J Blood Med. 2017 Oct 19;8:175-184
pubmed: 29089792
PLoS One. 2015 Feb 20;10(2):e0117383
pubmed: 25700159
Pharmaceuticals (Basel). 2018 Oct 04;11(4):
pubmed: 30287781
Br J Dermatol. 2020 Oct;183(4):614-627
pubmed: 32017015
Curr Opin Hematol. 2016 May;23(3):189-97
pubmed: 26886082
Dermatol Clin. 2010 Apr;28(2):289-301, x
pubmed: 20447494
Br J Clin Pharmacol. 2021 May;87(5):2256-2273
pubmed: 33188534
Pediatr Dermatol. 2010 May-Jun;27(3):238-43
pubmed: 20609141
Clin Exp Dermatol. 2011 Aug;36(6):579-83; quiz 583-4
pubmed: 21671991
Blood. 1977 Dec;50(6):1093-7
pubmed: 922160
Haematologica. 2020 May;105(5):1232-1239
pubmed: 31413088