PMID- 15840043
OWN - NLM
STAT- MEDLINE
DCOM- 20050822
LR  - 20181201
IS  - 0085-2538 (Print)
IS  - 0085-2538 (Linking)
VI  - 67
IP  - 5
DP  - 2005 May
TI  - Anemia is a new complication in Fabry disease: data from the Fabry Outcome 
      Survey.
PG  - 1955-60
AB  - BACKGROUND: The prevalence and causes of anemia among patients with Fabry disease 
      are unknown. METHODS: In a cross-sectional study we examined hemoglobin 
      concentrations of patients with Fabry disease using a large international 
      database, the Fabry Outcome Survey (FOS), and analyzed the association of renal 
      function, heart failure, gastrointestinal symptoms, and inflammation, with anemia 
      (hemoglobin <12 g/dL in females and <13 g/dL in males). RESULTS: Anemia was 
      present in 34% of 345 patients with Fabry disease. Median hemoglobin in 158 
      females was 12.9 g/dL and the median hemoglobin of 187 male patients was 13.2 
      g/dL. The prevalence of anemia among females was 20%, and among males 47%. Among 
      patients with normal renal function [estimated glomerular filtration rate (GFR) 
      >90 mL/min/1.73 m(2)] and anemia, heart failure [New York Heart Association 
      (NYHA) class II to IV] and/or elevated C-reactive protein (CRP) levels were 
      documented in 82% of patients. Up to 67% of patients with decreased estimated GFR 
      presented with anemia. There was also a trend for lower hemoglobin levels among 
      patients with signs of inflammation (defined by an elevated CRP level). We 
      observed no association of the presence of gastrointestinal symptoms with anemia. 
      Analyses in 53 patients receiving enzyme replacement therapy for up to 2 years, 
      suggest no effect on anemia. CONCLUSION: The results of this study point to a 
      high prevalence of anemia among patients with Fabry disease that is in most 
      instances related to impaired renal function, heart failure, and inflammation. 
      This finding may be of clinical relevance, because anemia is a major risk factor 
      for patients with kidney disease, heart failure, or stroke, which are important 
      manifestations of Fabry disease.
FAU - Kleinert, Julia
AU  - Kleinert J
AD  - Division of Nephrology and Dialysis, Department of Medicine III, Medical 
      University Vienna, Vienna, Austria.
FAU - Dehout, Francois
AU  - Dehout F
FAU - Schwarting, Andreas
AU  - Schwarting A
FAU - de Lorenzo, Abelardo Garcia
AU  - de Lorenzo AG
FAU - Ricci, Roberta
AU  - Ricci R
FAU - Kampmann, Christoph
AU  - Kampmann C
FAU - Beck, Michael
AU  - Beck M
FAU - Ramaswami, Uma
AU  - Ramaswami U
FAU - Linhart, Ales
AU  - Linhart A
FAU - Gal, Andreas
AU  - Gal A
FAU - Houge, Gunnar
AU  - Houge G
FAU - Widmer, Urs
AU  - Widmer U
FAU - Mehta, Atul
AU  - Mehta A
FAU - Sunder-Plassmann, Gere
AU  - Sunder-Plassmann G
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PL  - United States
TA  - Kidney Int
JT  - Kidney international
JID - 0323470
RN  - 0 (Hemoglobins)
RN  - 0 (Isoenzymes)
RN  - 9007-41-4 (C-Reactive Protein)
RN  - EC 3.2.1.22 (alpha-Galactosidase)
SB  - IM
MH  - Adult
MH  - Anemia/blood/*etiology
MH  - C-Reactive Protein/metabolism
MH  - Cross-Sectional Studies
MH  - Data Collection
MH  - Databases, Factual
MH  - Europe
MH  - Fabry Disease/blood/*complications/drug therapy/physiopathology
MH  - Female
MH  - Gastrointestinal Diseases/blood/etiology
MH  - Glomerular Filtration Rate
MH  - Heart Failure/blood/etiology
MH  - Hemoglobins/analysis
MH  - Humans
MH  - Isoenzymes/therapeutic use
MH  - Male
MH  - Middle Aged
MH  - alpha-Galactosidase/therapeutic use
EDAT- 2005/04/21 09:00
MHDA- 2005/08/23 09:00
CRDT- 2005/04/21 09:00
PHST- 2005/04/21 09:00 [pubmed]
PHST- 2005/08/23 09:00 [medline]
PHST- 2005/04/21 09:00 [entrez]
AID - S0085-2538(15)50674-1 [pii]
AID - 10.1111/j.1523-1755.2005.00294.x [doi]
PST - ppublish
SO  - Kidney Int. 2005 May;67(5):1955-60. doi: 10.1111/j.1523-1755.2005.00294.x.