Hypocholesterolemia in children and adolescents with β-thalassemia intermedia

Hartman, Corina; Tamary, Hannah; Tamir, Ada; Shabad, Evelyn; Levine, Carina; Koren, Ariel; Shamir, Raanan

doi:10.1067/mpd.2002.127498

Cited by 41 publications

(34 citation statements)

References 22 publications

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“…Hypocholesterolemia has been described in various hematological disorders [1], including thalassemia major [2], thalassemia intermediate [3], sickle cell disease [4], glucose-6-phosphate dehydrogenase (G6PD) deficiency [5], spherocytosis [6], aplastic anemia [7], and myelodysplastic syndrome [8]. The pathophysiology of the hypocholesterolemia in these settings remains obscure, although several mechanisms have been proposed: plasma dilution due to anemia, increased cholesterol requirement associated with erythroid hyperplasia, macrophage system activation with cytokine release, increased cholesterol uptake by the reticuloendotial system, and liver injury secondary to iron overload [2,3].…”

Section: Introductionmentioning

confidence: 99%

“…The pathophysiology of the hypocholesterolemia in these settings remains obscure, although several mechanisms have been proposed: plasma dilution due to anemia, increased cholesterol requirement associated with erythroid hyperplasia, macrophage system activation with cytokine release, increased cholesterol uptake by the reticuloendotial system, and liver injury secondary to iron overload [2,3]. Few small uncontrolled studies have also suggested a lower incidence of coronary artery disease in anemic patients with b-thalassemia and hereditary spherocytosis [9][10][11][12].…”

Section: Introductionmentioning

confidence: 99%

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Hypocholesterolemia in chronic anemias with increased erythropoietic activity

et al. 2006

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Hypocholesterolemia of unknown etiology has been previously described in various chronic anemias. Few small studies also suggested that those patients have a lower incidence of atherosclerotic events. The aim of our study was to determine the extent of hypocholesterolemia in various types of anemias. We studied 59 patients with chronic anemias associated with higherythropoietic activity (thalassemia intermedia, congenital dyserythropoietic anemia type I, congenital spherocytosis), 8 patients with low-erythropoietic activity anemias (acquired aplastic anemia, Fanconi anemia, and Diamond Blackfan anemia), and 20 healthy controls. Mean serum cholesterol, high-density lipoprotein (HDL) cholesterol, low-density lipoprotein (LDL) cholesterol, triglycerides, hemoglobin, serum ferritin, soluble transferrin receptor (STR), and serum erythropoietin levels were determined in each patient. All patients with chronic anemia and increased erythropoietic activity had hypocholesterolemia, whereas none of those with low erythropoietic activity was hypocholesterolemic. Mean serum cholesterol, HDL cholesterol, and LDL cholesterol levels were found to be significantly lower in the high-erythropoietic activity group (80 ± 19 mg/dl; 31 ± 10 mg/dl; 35 ± 14 mg/dl, respectively) compared with the control group (P < 0.001; 0.001; 0.001, respectively) and the low-erythropoietic activity group (P < 0.001; 0.001; 0.01, respectively). Significant inverse correlation (R 2 = 0.507) was observed between serum cholesterol and STR levels, which in the absence of iron deficiency reflect bone marrow activity. Taken together, our results imply that hypocholesterolemia accompanies anemias with high-erythropoietic activity. We suggest that the high-erythropoitic activity-associated hypocholesterolemia is due to increased cholesterol requirements by the proliferating erythoid cells. Further studies are needed to elucidate the exact mechanism and the possible clinical consequences of this phenomenon. Am. J. Hematol. 82:199-202, 2007. V V C 2006 WileyLiss, Inc.

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Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Hypocholesterolemia in chronic anemias with increased erythropoietic activity

et al. 2006

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“…Many of these studies have also tried to identify within-patient factors correlating with cholesterol level; 4,8,9 data analysis of most reported studies failed to show any influence on cholesterol level of age, sex, liver injury, haemoglobin, ferritin levels and the presence or absence of the spleen. However, in the study by Ricchi et al, 8 a lack of effect of single genotypes on cholesterol levels in thalassaemia major and intermedia patients was also reported.…”

Section: -10mentioning

confidence: 99%

Hypocholesterolaemia in Thalassaemia – Pathogenesis, Implications and Clinical Effects

Ricchi¹,

Ammirabile²,

Maggio³

2010

European Oncology &Amp; Haematology

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Free cholesterol is a constituent of cell membranes. Apart from nucleus-free erythrocytes, all cells of the human body are able to synthesise cholesterol de novo. More than 100 enzymatic processes are involved in the complete biosynthesis of cholesterol, which is a complex and energy-consuming process.For this reason, several tissues prefer to assume cholesterol from plasma lipoproteins rather than from their own intracellular synthesis.Cholesterol devoted to plasma lipoprotein is synthesised in the liver and in the distal part of the small intestine.1 Chylomicron remnants are the vehicle of cholesterol intake from the diet to the liver. The hepatic cholesterol pools originating from chylomicron remnants and de novo synthesised cholesterol are combined and excreted as verylow-density lipoprotein (VLDL). Low-density lipoprotein (LDL), the ultimate catabolic product of VLDL, is the main source of cholesterol for human tissue, especially those with high cell turnover. Cholesterol is not only a fundamental element of cell membranes but also the principal precursor for steroid and sexual hormone biosynthesis. Furthermore, cholesterol, through its intermediary products such as farnesyl diphosphate and geranylgeranyl diphosphate, is involved in the regulation of ras-protein intracellular signal transduction.1 Hypercholesterolaemia in ThalassaemiaA large body of evidence from prospective and retrospective studies has clearly shown that patients affected by thalassaemia have reduced levels of TC with respect to healthy age-and sex-matched controls. 3-10Hypocholesterolaemia has been reported in all phenotypes of β-thalassaemia and has also been described in various haematological disorders associated with high erythropoietic activity.11-14 Table 1 shows cholesterol levels found in different reports exploring lipid profiles in young and adult patients affected by all phenotypes of thalassaemia.The majority of these studies have evaluated cholesterol level in young patients with severe thalassaemia (thalassaemia major and intermedia). As shown in Table 1, low levels of plasma cholesterol are evident among all ages of patients with thalassaemia major and intermedia.Almost all studies agree with the observation that of all the forms of thalassemia, thalassaemia intermedia patients show the most marked alterations in lipid profile. Conversely, no studies have identified a cholesterol cut-off value that could clearly distinguish patients with thalassaemia intermedia from those affected by thalassaemia major.Furthermore, hypocholesterolaemia is only one aspect in the more complex alteration of lipid profile involving LDL and HDL level observed in all thalassemia patients. In fact, most of the abovementioned studies reported that thalassaemia patients also have lower HDL and LDL levels than those observed in control patients.Many of these studies have also tried to identify within-patient factors correlating with cholesterol level; 4,8,9 data analysis of most reported studies failed to show any influence on cholesterol level of a...

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“…Hypocholesterolaemia has been described in various anaemias with high erythropoietic activity [1][2][3][4][5][6][7] . In Nigerian children with sickle cell anaemia (SCA) and African-American patients with sickle cell disorder a significant reduction in plasma cholesterol; high density lipoprotein (HDL-c) and low density lipoprotein (LDLc) cholesterol concentration compared to normal controls have been reported 2,3 A study on adult with SCA reported low serum total cholesterol, LDL, HDL as well as lowered BMI compared to their controls.…”

Section: Introductionmentioning

confidence: 99%

Correlates of Steady State Lipid Profile and Anthropometric Parameters in Adult Sickle Cell Anaemia Patients in South-West Nigeria

Uche¹

2017

IOSR JDMS

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Total cholesterol (TC), High density lipoproteins (HDL), Low density lipoproteins (LDL), triglycerides and non-HDL-cholesterol were measured using Cholestech LDX (Cholestech Corporation U.S.A) from a single drop of capillary (35µ1) blood after an overnight fasting (9-l2hrs). Nutritional Assessment was measured with the aid of tape rule (for length and circumferences) while the skin fold thickness were measured using Caliper and weighing balance for weight. Data was analysed using excel and SPSS (version 20) statistical package. Result: The study showed significant reduction in TC, LDL-c and HDL-c in patients compared with controls while there was no variation in TG in

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Hypocholesterolemia in children and adolescents with β-thalassemia intermedia

Cited by 41 publications

References 22 publications

Hypocholesterolemia in chronic anemias with increased erythropoietic activity

Hypocholesterolemia in chronic anemias with increased erythropoietic activity

Hypocholesterolaemia in Thalassaemia – Pathogenesis, Implications and Clinical Effects

Correlates of Steady State Lipid Profile and Anthropometric Parameters in Adult Sickle Cell Anaemia Patients in South-West Nigeria

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