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Ascorbic acid status in idiopathic hemochromatosis
Insights
Idiopathic hemochromatosis patients show vitamin C deficiency, particularly when untreated. Treatment for this iron overload condition effectively restores vitamin C levels.
Area of Science:
- Biochemistry
- Hematology
- Nutritional Science
Background:
- Idiopathic hemochromatosis is a genetic disorder characterized by excessive iron absorption and storage.
- Vitamin C (ascorbic acid) plays a crucial role in iron metabolism and antioxidant defense.
- Altered vitamin C status has been anecdotally reported in various chronic diseases.
Purpose of the Study:
- To investigate vitamin C status in patients with idiopathic hemochromatosis.
- To compare vitamin C levels between untreated and treated idiopathic hemochromatosis patients and control groups.
- To explore the relationship between iron overload and vitamin C deficiency in this condition.
Main Methods:
- Leukocyte ascorbic acid concentrations were measured as an indicator of vitamin C status.
- Study included 67 idiopathic hemochromatosis patients (44 untreated, 25 treated), 31 normal subjects, and 37 alcoholic cirrhosis patients.
- Statistical analysis compared mean vitamin C levels across different groups.
Main Results:
- Untreated idiopathic hemochromatosis patients exhibited significantly lower vitamin C levels (19.5 +/- 1.7 microgram/10(8) WBC) compared to normal controls (34.4 +/- 1.9 microgram/10(8) WBC).
- Treated idiopathic hemochromatosis patients showed restored vitamin C levels (34.3 +/- 2.3 microgram/10(8) WBC), comparable to normal subjects.
- Alcoholic cirrhosis patients had reduced vitamin C levels (22.0 +/- 1.8 microgram/10(8) WBC).
Conclusions:
- A significant vitamin C deficiency is present in untreated idiopathic hemochromatosis.
- Iron overload appears to be the primary cause of vitamin C deficiency in idiopathic hemochromatosis.
- This deficiency can lead to underestimation of desferrioxamine-induced urinary iron excretion in these patients.
Abstract:
Vitamin C status was studied, by means of leucocyte ascorbic acid concentrations, in 67 cases of idiopathic hemochromatosis subdivided into 44 untreated and 25 treated cases (2 patients belonging to both subgroups) and compared to 31 normal subjects and 37 alcoholic cirrhosis patients. The control groups exhibited the following mean levels (+/- SEM): 34.4 +/- 1.9 microgram/10(8) WBC in normals and 22.0 +/- 1.8 microgram/10(8) WBC in alcoholic cirrhosis. In idiopathic hemochromatosis the mean levels were: for the untreated group 19.5 +/- 1.7 microgram/10(8) WBC and for the treated group 34.3 +/- 2.3 microgram/10(8) WBC. These results (1) affirm an important vitamin C deficiency in the untreated disease; (2) suggest that iron overload is the main causal factor in view of the striking difference--to date unreported--between untreated and treated cases of idiopathic hemochromatosis. Besides its possible theoretical interests, this vitamin C deficiency is responsible in idiopathic hemochromatosis for a significant underestimation of the desferrioxamine-induced urinary iron excretion.