scholarly journals Effect of ascorbic acid deficiency on serum ferritin concentration in patients with beta-thalassaemia major and iron overload.

1982 ◽  
Vol 35 (5) ◽  
pp. 487-491 ◽  
Author(s):  
R W Chapman ◽  
M A Hussain ◽  
A Gorman ◽  
M Laulicht ◽  
D Politis ◽  
...  
Keyword(s):  
Ascorbic Acid ◽  
Iron Overload ◽  
Serum Ferritin ◽  
Thalassaemia Major ◽  
Ferritin Concentration ◽  
Ascorbic Acid Deficiency ◽  
Beta Thalassaemia ◽  
Serum Ferritin Concentration ◽  
Acid Deficiency

Extreme hyperferritinaemia; clinical causes

2013 ◽  
Vol 66 (5) ◽  
pp. 438-440 ◽  
Author(s):  
Martin A Crook ◽  
Patrick L C Walker
Keyword(s):  
Liver Disease ◽  
Iron Overload ◽  
Serum Ferritin ◽  
Malignant Disease ◽  
Hepatic Disease ◽  
Blood Transfusions ◽  
Still’S Disease ◽  
Ferritin Concentration ◽  
Serum Ferritin Concentration ◽  
Multiple Blood

There are many causes of raised serum ferritin concentrations including iron overload, inflammation and liver disease to name but a few examples. Cases of extreme hyperferritinaemia (serum ferritin concentration equal to or greater than 10 000 ug/l) are being reported in laboratories but the causes of this are unclear. We conducted an audit study to explore this further. Extreme hyperferritinaemia was rare with only 0.08% of ferritin requests displaying this. The main causes of extreme hyperferritinaemia included multiple blood transfusions, malignant disease, hepatic disease and suspected Still's disease.

scholarly journals Reduction of tissue iron stores and normalization of serum ferritin during treatment with the oral iron chelator L1 in thalassemia intermedia

Blood ◽  
1992 ◽  
Vol 79 (10) ◽  
pp. 2741-2748 ◽  
Author(s):  
NF Olivieri ◽  
G Koren ◽  
D Matsui ◽  
PP Liu ◽  
L Blendis ◽  
...  
Keyword(s):  
Iron Overload ◽  
Serum Ferritin ◽  
Iron Concentration ◽  
Transferrin Saturation ◽  
Dramatic Improvement ◽  
Thalassemia Intermedia ◽  
Ferritin Concentration ◽  
Iron Stores ◽  
Tissue Iron ◽  
Serum Ferritin Concentration

Abstract In patients with thalassemia intermedia in whom hyperabsorption of iron may result in serious organ dysfunction, an orally effective iron- chelating drug would have major therapeutic advantages, especially for the many patients with thalassemia intermedia in the Third World. We report reduction in tissue iron stores and normalization of serum ferritin concentration after 9-month therapy with the oral chelator 1,2- dimethyl-3-hydroxypyrid-4-one (L1) in a 29-year-old man with thalassemia intermedia and clinically significant iron overload (SF 2,174 micrograms/L, transferrin saturation 100%; elevated AST and ALT, abnormal cardiac radionuclide angiogram) who was enrolled in the study with L1 75 mg/kg/day after he refused deferoxamine therapy. L1-Induced 24-hour urinary iron excretion during the first 6 months of therapy was (mean +/- SD, range) 53 +/- 30 (11 to 109) mg (0.77 mg/kg), declining during the last 3 months of L1 to 24 +/- 14 (13–40) mg (0.36 mg/kg), as serum ferritin decreased steadily to normal range (present value, 251 micrograms/L). Dramatic improvement in signal intensity of the liver and mild improvement in that of the heart was shown by comparison of T1- weighted spin echo magnetic resonance imaging with images obtained immediately before L1 administration was observed after 9 months of L1 therapy. Hepatic iron concentration decreased from 14.6 mg/g dry weight of liver before L1 therapy to 1.9 mg/g liver after 9 months of therapy. This constitutes the first report of normalization of serum ferritin concentration in parallel with demonstrated reduction in tissue iron stores as a result of treatment with L1. Use of L1 as a therapeutic option in patients with thalassemia intermedia and iron overload appears warranted.

Hereditary Hemochromatosis in an Adult Due to H63D Mutation: The Value of Estimating Iron Deposition By MRI T2* and Dissociation Between Serum Ferritin Concentration and Hepatic Iron Overload

Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 4891-4891
Author(s):  
Mohamed A. Yassin ◽  
Ashraf T Soliman ◽  
Vincenzo Desanctis ◽  
Sandara Abusamaan ◽  
Ahmed Elsotouhy ◽  
...  
Keyword(s):  
Iron Overload ◽  
Basal Ganglion ◽  
Serum Ferritin ◽  
Normal Values ◽  
Hereditary Hemochromatosis ◽  
Transferrin Saturation ◽  
Iron Deposition ◽  
Ferritin Concentration ◽  
H63d Mutation ◽  
Serum Ferritin Concentration

Abstract Hereditary hemochromatosis (HH) is an autosomal recessive disorder characterized by excessive intestinal absorption of dietary iron, causing iron overload in different organs, especially the liver. Hemochromatosis may not be recognized until later in life. Patients are usually asymptomatic but may present with a variety of signs and symptoms. These include: hyper-pigmented skin, hepatomegaly, arthralgia, diabetes mellitusand/or heart failure/arrhythmia. The risk of HH related morbidity in HFE compound homozygotes patients (H63D /H63D) is considered rare, we report a male patient with H63D mutation who developed impaired glucose tolerance, and high hepatic enzymes due to significant iron accumulation in the liver as well as Parkinsonian-like syndrome due to iron deposition in the basal ganglia. A 40 year old Qatari male was referred for evaluation of a rise in hemoglobin and hematocrit values with normal MCV, total leucocyte and platelet counts. The patient was asymptomatic with normal vital signs, no depigmentation or hepato-splenomegaly. Hematologic findings included a hemoglobin concentration of Hb 16.5 g/dL, hematocrit 53%, mean corpuscular volume (MCV) 93 fL/red cell, leucocyte count of 7200/ μL and a platelet count of 199000/μL. His serum ferritin was 359 μg/l ( normal values: < 336 μg/l), serum iron: 37 μmol/l ( normal values <28.6μmol/l), fasting transferrin saturation: 64% (normal < 50%). A random glucose 6.5 and 6.4 mmol/L (normal values 5.5mmol/L ), A1C of 5,4 %, normal creatinine and electrolytes, alanine aminotransferase (ALT) of 66 U/l (normal < 40U/l), mild elevation of bilirubin 39 umol/l (normal <24umol/l), normal U&E Hepatitis B and C antibodies were negative. OGTT revealed impaired glucose tolerance. Thyroid function, morning serum cortisol, LH and FSH and serum total testosterone concentrations were in the normal range. A diagnosis of polycythemia vera was excluded on the basis of WHO Criteria 2008. The polymerase chain restriction assay was negative for the common mutation (C282Y) but positive for H63 D mutation. Family screening confirmed HH in his brother (homozygous), whereas his mother, two brothers and the sister were carriers (heterozygous). His four offspring were carriers. This suggested an autosomal recessive mode of inheritance. Conventional MRI study showed a normal liver size with diffuse fatty changes and focal areas of fatty sparing with some evidence of iron deposition. Whereas, T2-star (T2*) sequences showed a diffuse and significant decrease in liver signal intensity. A LIC liver concentration of 27 mg Fe/g dry wt was found (normalvalues:< 2 mg Fe/g dry wt; severe iron overload: ≥15 mg Fe/g dry wt). No significant iron deposition in the spleen, heart or pancreas was observed. At the age of 41 years the patient complained of tremors in both hands and arms while sitting or standing still (resting tremor) that improved with hands movements. A brain MRI revealed iron deposition in the basal ganglion. It was concluded that basal ganglionicn iron deposition mediated the neurological decline. Currently, the transferrin saturation and serum ferritin levels are within normal. Discussion: This is the first case of HH secondary to H63 D among an Arab family and the first reported case of Parkinsonism tremors secondary to this mutation. The H63D HFE variant is less frequently associated with HH, but its role in the neurodegenerative diseases has received a great attention. An accurate evaluation of iron overload is necessary to establish the diagnosis of HH and to guide iron chelation in HH by determination of liver iron concentration (LIC) by means of T2* MRI. Although serum ferritin concentration was only mildly increased a significant siderosis in the liver was detected by MRI T2* technique occurred. Liver siderosis was associated with mild impairment of liver function (increased serum ALT and bilirubin ). Conclusion: Our data further confirm that serum ferritin levels are not an accurate measure of total body iron stores in HH. Iron deposition in the liver and basal ganglion occurred despite mild elevation of ferritin. changes in basal ganglion may present by parkinsonian like tremors in these patients Use,T2* MRI should be encouraged in patients with HH for better evaluation of Iron overload and avoidance of Complications since serum ferritin can be misleading in these conditions. Disclosures Yassin: Qatar National research fund: Patents & Royalties, Research Funding. Aldewik:Qatar Ntional Research Fund: Patents & Royalties, Research Funding.

scholarly journals The effect of siderosis and ascorbic acid depletion on bone metabolism, with special reference to osteoporosis in the Bantu

1971 ◽  
Vol 25 (3) ◽  
pp. 367-376 ◽  
Author(s):  
A. A. Wapnick ◽  
S. R. Lynch ◽  
H. C. Seftel ◽  
R. W. Charlton ◽  
T. H. Bothwell ◽  
...  
Keyword(s):  
Ascorbic Acid ◽  
Bone Resorption ◽  
Bone Formation ◽  
Iron Overload ◽  
Bone Mineral ◽  
Guinea Pigs ◽  
Iron Concentration ◽  
Ascorbic Acid Deficiency ◽  
Acid Deficiency ◽  
Liver Storage

1. There is an association between iron overload, ascorbic acid deficiency and osteoporosis in middle-aged South African Bantu males. The iron overload contributes to the ascorbic acid deficiency by accelerating its oxidative catabolism. The object of the present investigation was to explore the possibility that the osteoporosis results from chronic ascorbic acid deficiency.2. On quantitative microradiography, percentage bone-formation surface was normal but percentage bone-resorption surface was significantly increased in ten osteoporotic subjects compared with seven control subjects.3. There was a significant inverse correlation between bone mineral density and liver storage iron concentration in thirty-five Bantu subjects. In thirteen individuals aged 39 years or less, liver storage iron concentration was significantly correlated with percentage bone-resorption surface.4. Guinea-pigs deprived of ascorbic acid for 21 d exhibited both significantly diminished percentage bone-formation surface and increased percentage bone-resorption surface.5. Guinea-pigs overloaded with iron by injections of iron dextran developed significantly reduced hepatic ascorbic acid concentrations and bone mineral densities; percentage bone-formation surface was significantly diminished and percentage bone-resorption surface significantly increased. Ascorbic acid injection largely prevented the bone changes.

Serum Ferritin in Ascorbic Acid Deficiency

1980 ◽  
Vol 45 (3) ◽  
pp. 459-466 ◽  
Author(s):  
H. P. Roeser ◽  
J. W. Halliday ◽  
D. J. Sizemore ◽  
A. Nikles ◽  
D. Willgoss
Keyword(s):  
Ascorbic Acid ◽  
Serum Ferritin ◽  
Ascorbic Acid Deficiency ◽  
Acid Deficiency

scholarly journals T2*-magnetic resonance relaxometry of the liver in the quantitative assessment of iron overload

2018 ◽  
Vol 20 (2) ◽  
pp. 55-58
Author(s):  
A M Titova ◽  
G E Trufanov ◽  
V A Fokin
Keyword(s):  
Magnetic Resonance ◽  
Iron Overload ◽  
Serum Ferritin ◽  
Quantitative Assessment ◽  
Accurate Estimate ◽  
The Body ◽  
Ferritin Concentration ◽  
Neoplastic Processes ◽  
Serum Ferritin Concentration ◽  
Reliable Marker

For objective quantitative assessment of iron overload in 60 patients with hemochromatosis of different genesis, T2*-magnetic resonance relaxometry of the liver was performed. The results of the latter were compared with the concentration of serum ferritin, which is by far the most common marker of iron overload. It has been established that T2*-magnetic-resonance relaxometry is a non-invasive, highly effective method of objective quantitative assessment of iron overload in patients of the hematological profile, in comparison with the serum ferritin concentration, which is not specific, since its change can be influenced by inflammatory and neoplastic processes in The organism, often found in patients with oncohematological profile. Thus, according to T2*-magnetic resonance relaxometry of the liver, all patients were divided into two groups: with iron overload and with no overload, the latter among the total number of subjects was 13,3%. The explanation of the increase in serum ferritin concentration in these patients was the presence of identified inflammatory foci in the body. In most patients (86,6%), iron overload was confirmed by T2*-magnetic resonance relaxometry measurements. However, at each degree of overload, there was no clear regularity in the proportional increase in serum ferritin concentration of the intensity of pathological changes revealed by T2*-magnetic resonance relaxometry. Thus, ferritin cannot remain a reliable marker for iron overload. In general, magnetic resonance T2 *-relaxometry provides the most accurate estimate of the degree of iron overload in the liver. T2*-magnetic resonance relaxometry should be included in the protocol of examination of patients with suspicion of the presence of iron overload.

scholarly journals Reduction of tissue iron stores and normalization of serum ferritin during treatment with the oral iron chelator L1 in thalassemia intermedia

Blood ◽  
1992 ◽  
Vol 79 (10) ◽  
pp. 2741-2748 ◽  
Author(s):  
NF Olivieri ◽  
G Koren ◽  
D Matsui ◽  
PP Liu ◽  
L Blendis ◽  
...  
Keyword(s):  
Iron Overload ◽  
Serum Ferritin ◽  
Iron Concentration ◽  
Transferrin Saturation ◽  
Dramatic Improvement ◽  
Thalassemia Intermedia ◽  
Ferritin Concentration ◽  
Iron Stores ◽  
Tissue Iron ◽  
Serum Ferritin Concentration

In patients with thalassemia intermedia in whom hyperabsorption of iron may result in serious organ dysfunction, an orally effective iron- chelating drug would have major therapeutic advantages, especially for the many patients with thalassemia intermedia in the Third World. We report reduction in tissue iron stores and normalization of serum ferritin concentration after 9-month therapy with the oral chelator 1,2- dimethyl-3-hydroxypyrid-4-one (L1) in a 29-year-old man with thalassemia intermedia and clinically significant iron overload (SF 2,174 micrograms/L, transferrin saturation 100%; elevated AST and ALT, abnormal cardiac radionuclide angiogram) who was enrolled in the study with L1 75 mg/kg/day after he refused deferoxamine therapy. L1-Induced 24-hour urinary iron excretion during the first 6 months of therapy was (mean +/- SD, range) 53 +/- 30 (11 to 109) mg (0.77 mg/kg), declining during the last 3 months of L1 to 24 +/- 14 (13–40) mg (0.36 mg/kg), as serum ferritin decreased steadily to normal range (present value, 251 micrograms/L). Dramatic improvement in signal intensity of the liver and mild improvement in that of the heart was shown by comparison of T1- weighted spin echo magnetic resonance imaging with images obtained immediately before L1 administration was observed after 9 months of L1 therapy. Hepatic iron concentration decreased from 14.6 mg/g dry weight of liver before L1 therapy to 1.9 mg/g liver after 9 months of therapy. This constitutes the first report of normalization of serum ferritin concentration in parallel with demonstrated reduction in tissue iron stores as a result of treatment with L1. Use of L1 as a therapeutic option in patients with thalassemia intermedia and iron overload appears warranted.

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