PubMed · 7648687
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L T Chappell, R Miranda, M Rubin, J P Carter, J Trowbridge. 1995-09-01. Chelation therapy.. https://pubmed.ncbi.nlm.nih.gov/7648687/
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BACKGROUND: To assess the literature for the impact of iron overload and infusion Iron Chelation Therapy (ICT) on patients' quality of life (QoL), and the availability of QoL instruments for patients undergoing infusion ICT. Also, to obtain patients' experiences of having iron overload and receiving infusion ICT, and experts' clinical opinions about the impact of treatment on patients' lives. METHODS: A search of studies published between 1966 and 2004 was conducted using Medline and the Health Economic Evaluation Database (HEED). Qualitative results from patient and expert interviews were analysed. Hand searching of relevant conference abstracts completed the search. RESULTS: Few studies measuring the impact of ICT with deferoxamine (DFO) on patients QoL were located (n = 15). QoL domains affected included: depression; fatigue; dyspnoea; physical functioning; psychological distress; decrease in QoL during hospitalization. One theme in all articles was that oral ICT should improve QoL. No iron overload or ICT-specific QoL instruments were located in the articles. Interviews revealed that the impact of ICT on patients with thalassemia, sickle cell disease, and myelodysplastic syndromes is high. CONCLUSION: A limited number of studies assessed the impact of ICT or iron overload on QoL. All literature suggested a need for easily administered, efficacious and well tolerated oral iron overload treatments, given the impact of current ICT on adherence. Poor adherence to ICT was documented to negatively impact survival. Further research is warranted to continue the qualitative and quantitative study of QoL using validated instruments in patients receiving ICT to further understanding the issues and improve patients QoL.
PURPOSE OF REVIEW: This article reviews the pathophysiology and clinical presentation of iron poisoning. Recently proposed guidelines for triage of children with iron ingestion will be discussed as well as diagnostic and treatment modalities. Finally, the potential impact of unit-dose packaging as a primary preventative measure will be discussed. RECENT FINDINGS: Carbonyl iron has a greater safety margin than the iron salts. There have been no published reports of serious or fatal poisoning from the ingestion of carbonyl iron products. Evidence-based consensus guidelines have determined that the threshold for referral to a healthcare facility is 40 mg/kg of elemental iron in the form of adult iron formulations. Unit-dose packaging of iron preparations appeared to decrease the number of exposures to iron and deaths in the United States during the period they were instituted. SUMMARY: Iron poisoning remains primarily a clinical diagnosis, although certain laboratory and radiological testing may provide helpful evidence to guide evaluation and management. Primary prevention is the best modality for decreasing morbidity and mortality for all poisonings including iron.
Hereditary hemochromatosis has been redefined over the past century, from a rare (but often fatal) disorder of iron overload known as "bronzed diabetes" to only biochemical evidence of altered iron metabolism, and recently to mere expression of the C282Y homozygous genotype of the HFE gene. The variable definitions of the disease, as well as the variable degree of penetrance of the C282Y homozygous genotype, have made it difficult to determine optimal screening strategies. Multiple studies performed since discovery of the C282Y mutation have led to the conclusion that overall penetrance of the genotype is low and that screening of asymptomatic general populations for hereditary hemochromatosis is not recommended. Screening for HFE mutations among certain patient groups, including patients with cirrhosis, however, may help target those who would benefit most from iron removal. For most patients, phlebotomy is the preferred treatment option; iron chelation therapies are available for patients unable to tolerate phlebotomy.