Low Serum 25-Hydroxyvitamin in Hereditary Hemochromatosis:Relation to Iron Status

Chow, Lok-Hi; Frei, J; Hodsman, Alex; Valberg, L. S.

doi:10.1016/s0016-5085(85)80001-9

Cited by 40 publications

(25 citation statements)

References 21 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…It was previously attributed to defective 25-hydroxylation of vitamin D in the liver, (1) as was found in hemochromatosis. (18) Although there was no correlation between serum ferritin or stainable iron surface and serum levels of vitamin D in our patients, the remarkable elevation of serum ferritin level and prominent iron staining in trabecular bone indicated heavy iron overload to the body, and maybe, to the liver. However, the contribution of vitamin D deficiency to the development of reduced bone mass is uncertain because no correlation between serum 25(OH) vitamin D or 1,25(OH) 2 vitamin D levels and BMD or histomorphometric parameters was found in our study.…”

Section: Resultscontrasting

confidence: 65%

“…Malnutrition is unlikely the cause as all the patients had normal dietary habit and no sign of other vitamin deficiency were found. It was previously attributed to defective 25‐hydroxylation of vitamin D in the liver, (1) as was found in hemochromatosis (18) . Although there was no correlation between serum ferritin or stainable iron surface and serum levels of vitamin D in our patients, the remarkable elevation of serum ferritin level and prominent iron staining in trabecular bone indicated heavy iron overload to the body, and maybe, to the liver.…”

Section: Discussionsupporting

confidence: 42%

See 1 more Smart Citation

Abnormalities in Bone Mineral Density and Bone Histology in Thalassemia

Domrongkitchaiporn

Sirikulchayanonta

Angchaisuksiri

et al. 2003

Journal of Bone and Mineral Research

View full text Add to dashboard Cite

This study demonstrated that there was extensive iron staining on trabecular surface and marked reduction in trabecular bone volume without significant alteration in bone formation and bone resorption rates as well as significant reduction in bone mineral density in 18 thalassemic patients. Serum IGF-I was reduced and may modulate the reduction of bone mass.Introduction: Bone histomorphometric studies in thalassemia to show alterations in bone histology and their relationship to biochemical parameters are very limited. Therefore, this study was systematically conducted to determine the alterations in thalassemia patients. Methods: Serum biochemical parameters, trans-iliac crest bone biopsy, and determination of bone mineral density of femur and lumbar spine were done in 18 thalassemic patients (10 females and 8 males). Results: Serum osteocalcin, carboxy terminal teleopeptide fragment of type I collagen, and parathyroid hormone levels were within normal limits, but serum 25(OH) vitamin D (19.3 Ϯ 1.6 ng/ml) and 1,25(OH) 2 vitamin D (33.77 Ϯ 1.51 pg/ml) levels were decreased. Serum insulin-like growth factor I (IGF-I; 145.2 Ϯ 20 ng/ml) was suppressed, whereas serum ferritin (1366.6 Ϯ 253.9 ng/ml) was markedly elevated. Reduced bone mineral density was found in all studied areas. Trabecular bone volume was significantly decreased (16.65 Ϯ 1.12%), whereas bone formation rate, eroded surface, and other bone histomorphometric parameters were within normal limits. The trabecular bone volume varied significantly with bone mineral density of total femur (r ϭ 0.48, p ϭ 0.04). There was an extensive stainable iron surface on the mineral front (9 -60%). Significant correlation between serum IGF-I, serum ferritin, stainable iron surface, and bone mineral density, lumbar spine, and total femur were found. Serum IGF-I correlated with trabecular bone volume (r ϭ 0.6, p ϭ 0.03), inversely with both serum ferritin level (r ϭ Ϫ0.6, p Ͻ 0.01), and inversely with stainable iron surface (r ϭ Ϫ0.53, p ϭ 0.02). Multiple regression analysis demonstrated that IGF-I was the only independent variable that determined bone mineral density of lumbar spine and total femur. Conclusion: Low bone mineral density and reduced trabecular bone volume with extensive iron deposition are the predominant findings in thalassemic patients. There was no evidence of increased bone resorption or mineralization defect. A reduction in circulatory IGF-I may modulate the reduction of bone mass.

show abstract

Section: Resultscontrasting

confidence: 65%

Section: Discussionsupporting

confidence: 42%

Abnormalities in Bone Mineral Density and Bone Histology in Thalassemia

Domrongkitchaiporn

Sirikulchayanonta

Angchaisuksiri

et al. 2003

Journal of Bone and Mineral Research

View full text Add to dashboard Cite

show abstract

“…The sharp decline in vitamin D25‐OH stores with age probably reflects a combination of decreased intake (dairy products) as well as a shift toward more indoor activities. Vitamin D is 25‐hydroxylated in the liver and haemosiderosis may impair vitamin D25‐OH production (Chow et al , 1985). Vitamin D1‐25OH levels were normal or elevated in all patients, similar to prior studies (Moulas et al , 1997; Napoli et al , 2006).…”

Section: Resultsmentioning

confidence: 99%

Vitamin D deficiency, cardiac iron and cardiac function in thalassaemia major

et al. 2008

View full text Add to dashboard Cite

Summary Vitamin D25‐OH and D1‐25OH levels were compared with cardiac R2* (1/T2*), left ventricular ejection fraction (LVEF), age, ferritin and liver iron in 24 thalassaemia major patients. Vitamin D25‐OH levels were reduced in 13/24 patients while vitamin D1‐25OH levels were often elevated. Vitamin D25‐OH levels decreased with age (r2 = 0·48) and with liver iron (r2 = 0·20). Cardiac R2* was inversely related with the ratio of D25‐OH to D1‐25OH levels (r2 = 0·42). LVEF was also proportional to the D25‐OH/D1‐25OH ratio (r2 = 0·49). Vitamin D deficiency may be associated with cardiac iron uptake and ventricular dysfunction in thalassaemia major patients.

show abstract

“…Vitamin D deficiency, overt osteomalacia, and rickets were previously reported in patients with thalassemia [16,20]. This had been attributed to impaired 25 hydroxylation of vitamin D in the liver, as was found in hemochromatosis [21]. However, with the use of effective chelating agents, other mechanisms of vitamin D deficiency, including decreased intakes, impaired absorption, and reduced skin production, should be explored.…”

Section: Discussionmentioning

confidence: 95%

BONE MINERAL DENSITY IN IRANIAN ADOLESCENTS AND YOUNG ADULTS WITH Β-Thalassemia MAJOR

Shamshirsaz

Bekheirnia

Kamgar

et al. 2007

Pediatric Hematology and Oncology

View full text Add to dashboard Cite

The authors investigated the prevalence of low bone mass in patients from Tehran, Iran, with beta-thalassemia major (n = 203), aged 10-20 years, and the potential risk factors for osteoporosis in this patient population. Prevalence of osteoporosis was 50.7% in lumbar spine, 10.8% in femur, and 7.9% in both regions with no significant difference between the two genders. The following factors were associated with low BMD: height for age and weight for age below 3rd percentile, delayed puberty or hypogonadism, age when Desferal (for iron chelation) was started, duration of Desferal therapy, and serum zinc. Low serum copper and 25(OH)D were not associated with low BMD.

show abstract

Low Serum 25-Hydroxyvitamin in Hereditary Hemochromatosis:Relation to Iron Status

Cited by 40 publications

References 21 publications

Abnormalities in Bone Mineral Density and Bone Histology in Thalassemia

Abnormalities in Bone Mineral Density and Bone Histology in Thalassemia

Vitamin D deficiency, cardiac iron and cardiac function in thalassaemia major

BONE MINERAL DENSITY IN IRANIAN ADOLESCENTS AND YOUNG ADULTS WITH Β-Thalassemia MAJOR

Contact Info

Product

Resources

About