The relationship between iron overload, clinical symptoms, and age in 410 patients with genetic hemochromatosis

✍ Scribed by P. C. Adams; Y. Deugnier; R. Moirand; P. Brissot

• Publisher: John Wiley and Sons
• Year: 1997
• Tongue: English
• Weight: 203 KB
• Volume: 25
• Category: Article
• ISSN: 0270-9139
• DOI: 10.1002/hep.510250130

✦ Synopsis

The aim of this study was to investigate the relationprevalence of symptoms was retrospectively reviewed in 410 ship between iron overload, age, and clinical symptoms patients with genetic hemochromatosis. in genetic hemochromatosis. The relationship was studied between clinical symptoms and liver iron concentration, serum ferritin, and iron removed in a retrospective PATIENTS AND METHODS study of 410 homozygotes diagnosed using strict criteria. Hemochromatosis was diagnosed at two tertiary referral centers No significant relationship was found between liver iron between 1962 and 1995. The median year of diagnosis was 1987 in concentration, iron removed by venesection, and serum 167 Canadian patients and 1992 in 243 French patients. The diagnoferritin level with age. The prevalence of cirrhosis, diasis of hemochromatosis was established by one of the following critebetes, cardiac disease, pigmentation, and fatigue inria. (1) Liver biopsy results consistent with a histological diagnosis creased as liver iron concentration increased. The most of hemochromatosis with a hepatic iron index (hepatic iron concen- common presentations at diagnosis were fatigue or as tration/age) of ú1.9 6 (n Å 281). (2) Iron removed by venesection ther- an incidental finding in all age groups. Twenty-seven apy, ú5 g. Patients were treated by weekly venesection of 300 to 500 percent of patients (110 of 410) had no clinical symptoms mL until the serum ferritin level was approximately 50 mg/L. Iron of hemochromatosis. Iron accumulation is highly variremoved was calculated by the number of liters of blood removed to achieve iron depletion 10.5 g (n Å 53). (3) Siblings who were HLA able in patients with genetic hemochromatosis. The sigidentical and did not meet above criteria (n Å 76), including HLAnificant relationship between liver iron concentration identical siblings with elevated serum ferritin levels who did not and cirrhosis, diabetes, cardiac disease, pigmentation, undergo a liver biopsy (n Å 62) or had õ5 g of iron removed.