title: Depression in adults with Fabry disease: a common and under-diagnosed problem.
abstract: BACKGROUND
  Anderson-Fabry disease (AFD), an X-linked lysosomal storage disorder, leads to multi-organ dysfunction and premature mortality. Depression in adults with AFD has been reported, but no large study has been done. We have examined the adult Fabry population in the United Kingdom to describe the prevalence, associated factors and frequency of diagnosis of depression.
  
  
  METHODS
  Postal questionnaires were sent from four adult clinics to 296 AFD patients. A response rate of 62% (n = 184; 74 male, 110 female) formed the data set. Questionnaires collected demographic and Fabry-specific information. Depression status was assessed using the Centre for Epidemiological Studies depression scale (CES-D).
  
  
  RESULTS
  Responders were aged between 18 and 76 years (mean 44). The prevalence of depression was 46%, of which 28% were consistent with 'severe clinical depression'. Unlike the normal population, males with AFD report a higher prevalence of severe depression than females (36% males; 22% females). Interference of AFD symptoms with individuals' lives (particularly acroparaesthesiae or anhidrosis) showed the largest odds of association with depression. Relationship and financial status proved strong predictors of depression: 88% of those with mild-moderate depression and 72% with severe depression were undiagnosed.
  
  
  CONCLUSIONS
  Depression is common and under-diagnosed in AFD. Proper assessment of and treatment for depression could improve the quality of life of these patients.
authors.affiliation: Charles Dent Metabolic Unit, Box 92, The National Hospital for Neurology and Neurosurgery, Queen Square, London, WC1N3BG, UK. amy.cole@uclh.nhs.uk
authors.forename: A L
authors.initials: AL
authors.lastname: Cole
authors.forename: P J
authors.initials: PJ
authors.lastname: Lee
authors.forename: D A
authors.initials: DA
authors.lastname: Hughes
authors.forename: P B
authors.initials: PB
authors.lastname: Deegan
authors.forename: S
authors.initials: S
authors.lastname: Waldek
authors.forename: R H
authors.initials: RH
authors.lastname: Lachmann
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D003863:Depression / Q000150:complications* / Q000175:diagnosis* / Q000453:epidemiology; D000795:Fabry Disease / Q000150:complications* / Q000175:diagnosis* / Q000453:epidemiology; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D015995:Prevalence; D011788:Quality of Life; D012307:Risk Factors; D012737:Sex Factors; D012923:Social Class; D011795:Surveys and Questionnaires; D016896:Treatment Outcome; D006113:United Kingdom