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Dhondt Jean-Louis - - 2010
In addition to being hyperphenylalaninemic, patients lacking tetrahydrobiopterin (BH4) are deficient in the neurotransmitters whose synthesis depends on the normal activity of tetrahydrobiopterin-dependent tyrosine and tryptophan hydroxylases. Consequently, these patients have to be rapidly recognized among hyperphenylalaninemic babies, since they need specific and early substitutive therapy. Since 1980, BH4 metabolism ...
Chinnappa Shanmugakumar - - 2010
BACKGROUND: Detection of iron deficiency in patients with end-stage renal disease (ESRD) remains challenging due to the lack of reliable markers. The immature reticulocyte fractions (IRF) RET-Y and RBC-Y may serve as useful novel markers. We investigated the ability of IRF to detect functional iron deficiency in ESRD patients in ...
Wood John C - - 2010
We present results from a prospective, multicenter, open-label, single-arm study evaluating response of cardiac and liver iron to deferasirox therapy for 18 months. Twenty-eight patients with abnormal T2* and normal left ventricular ejection fraction were enrolled from 4 US centers. All patients initially received deferasirox doses of 30 to 40 ...
Landy J - - 2010
BACKGROUND: UK guidelines1 suggest investigation with bidirectional endoscopy (BDE) (upper endoscopy and colonoscopy) in the same sedative episode is a reasonable approach for the investigation of iron deficiency anaemia. There are few data regarding such investigation for iron deficiency anaemia (IDA) in young men. OBJECTIVE: To assess the use of ...
Valenti L - - 2011
Increased ferritin and body iron stores are frequently observed in nonalcoholic fatty liver disease (NAFLD), associated with heightened susceptibility to vascular damage. Conflicting data have been reported on the role of iron in atherosclerosis, with recent data suggesting that excess iron induces vascular damage by increasing levels of the hormone ...
Al-Shohaib S - - 2010
Recombinant human erythropoietin (rHuEpo) has revolutionized the management of renal anemia, significantly improving patient quality of life. Great attention has been paid lately on how to optimally use this potent anti-anemic agent. Aiming to overview anemic patient management with Epotin (Julphar's rHuEpo) according to the new guidelines, we included in ...
Ferrari Paolo - - 2010
AIM: To assess whether pentoxifylline improves anaemia of chronic kidney disease (CKD) via suppression of interleukin-6 (IL-6) and improved iron mobilization. BACKGROUND: CKD patients may have elevated IL-6 and tumour necrosis factor alpha levels. These cytokines can increase hepcidin production, which in turn reduces iron release from macrophages resulting in ...
Hankins Jane S - - 2010
The rate and pattern of iron deposition and accumulation are important determinants of liver damage in chronically transfused patients. To investigate iron distribution patterns at various tissue iron concentrations, effects of chelation on hepatic iron compartmentalization, and differences between patients with sickle cell disease (SCD) and thalassemia major (TM), we ...
Riccioni Maria Elena - - 2010
BACKGROUND: In around 30% of iron deficiency anaemia (IDA) cases a definite diagnosis cannot be made. AIM: To investigate the role of capsule endoscopy (CE) in detecting lesions in patients with unexplained IDA after a negative endoscopic, serologic and haematologic diagnostic work up and its possible role in influencing clinical ...
Lasocki Sigismond - - 2010
PURPOSE: Inflammation-induced anemia is frequent among critically ill patients and can be aggravated by true iron deficiency (ID) resulting from blood losses. The serum hepcidin level controls the availability of iron for erythropoiesis, and its determination offers new perspectives for the diagnosis of ID in the presence of inflammation. We ...
Zacharski L R - - 2010
Two male first cousins with mild haemophilia A had baseline factor VIII levels of 12-15% and experienced bleeding requiring coagulation factor infusion therapy with trauma and surgical procedures. Both the patients with haemophilia A also had electrocardiographically documented symptomatic paroxysmal atrial fibrillation (PAF) for several years that had become resistant ...
P?rez de la Ossa Natalia - - 2010
BACKGROUND AND PURPOSE: Iron plays a detrimental role after experimental intracerebral hemorrhage (ICH). This study investigates whether high-serum ferritin levels are associated with poor outcome in patients with ICH. METHODS: We studied 92 consecutive patients with primary hemispheric ICH within the first 12 hours from onset of symptoms (median, 3.3 ...
Zhou Jing-Guo - - 2010
This is a cross-sectional study to assess the prevalence and causes of anemia in the primary Sjögren's syndrome (pSS). One hundred and thirty-two consecutive patients with pSS were enrolled into the study. Standard hematological and immunological tests and examination of bone marrow were performed. Anemia occurred in 45 (34.1%) patients. ...
Poggi M - - 2010
Dysfunction of GH-IGF-I axis has been described in many patients affected by β-thalassemia major (TM), especially in children and in adolescents. Recent studies have demonstrated the necessity to evaluate adult patients affected by TM to establish the presence of this alteration which could be relevant in the pathogenesis of cardiac ...
Varma A - - 2010
AIM: The aim of this study was to assess cardiac mortality in patients with reduced ejection fraction (EF< or =45%) and anemia (Hb< or =12 g/dL) undergoing coronary stenting and to investigate whether iron-deficiency anemia influenced outcome when compared to non-anemic patients or patients with other types of anemia. METHODS: ...
Fracanzani Anna Ludovica - - 2010
The clinical presentation of hereditary hemochromatosis has changed markedly in recent years. The aim of this study was to analyze a large series of consecutive Italian patients with hemochromatosis diagnosed between 1976 and 2007 to determine whether the genetic background and the presence of acquired risk factors influenced the severity ...
Jayaranee S - - 2010
We evaluated the usefulness of RET-Y and RBC-Y in distinguishing functional iron deficiency from iron-deficiency anaemia (IDA) in patients with anaemia of inflammation (AI). Sixty healthy blood donors constituted the control group. We studied RET-Y and RBC-Y in 115 patients with hypochromic/microcytic anaemia. Of these 42 patients had uncomplicated IDA ...
Richette Pascal - - 2010
To determine the effects of iron depletion on serum levels of joint biomarkers and on joint symptoms in patients with hereditary haemochromatosis (HH). Levels of biomarkers were measured in 18 patients with HH at the time of diagnosis and after iron depletion. The markers were type II collagen degradation (Coll2-1) ...
Majhail Navneet S - - 2010
We report the results of a single-center, prospective evaluation for iron overload and subsequent treatment in 147 adult allogeneic hematopoietic cell transplantation (HCT) recipients who survived beyond 1 year after transplantation. Patients were screened by serum ferritin level; those with ferritin >1000 ng/mL underwent liver R2 magnetic resonance imaging to ...
Cuijpers Maria L H - - 2010
Myelodysplastic syndromes (MDS) are a heterogeneous group of clonal haematopoietic stem cell malignancies. A subgroup, the so-called sideroblastic MDS, shows ring sideroblasts in the bone marrow aspirate that represent mitochondrial iron accumulation. Patients with sideroblastic MDS also develop systemic iron overload and generally have a low-risk MDS. Therefore it is ...
Ostrvica Edin - - 2010
INTRODUCTION: Anemia is an early sign of chronic kidney dysfunction, caused by many different factors, but the insufficient erythropoietin synthesis is the crucial factor in its development. OBJECTIVES: The objective of our study was to compare effectiveness of epoietin alpha and beta application in the treatment of renal anemia in ...
Koren Ariel - - 2010
BACKGROUND: Blood transfusions are the standard of care in b thalassemia and transfusions are also indicated in sickle cell disease (SCD) patients with hypersplenism, recurrent vaso-occlusive crises and for stroke prevention. Iron overload caused by blood transfusions in thalassemia and in SCD may affect morbidity and mortality. Recent studies of ...
Miura Yasuo - - 2010
Iron chelation therapy (ICT) has been applied for the patients with iron overload-associated liver dysfunction since it is one of the causes of death in patients with intractable hematological diseases requiring multiple red blood cell transfusions. Recently, deferasirox (DSX), a novel, once-daily oral iron chelator, was demonstrated to have similar ...
De Nicola Luca - - 2010
we evaluated prevalence and prognosis of mild anemia, defined as Hb (g/dl) 11-13.5 in males and 11-12 in females, in a prospective cohort of stage 3-5 chronic kidney disease (CKD) patients. we enrolled 668 consecutive patients in 25 renal clinics during 2003. Patients with frank anemia (Hb <11 or erythropoiesis-stimulating ...
Pavlović Momcilo - - 2010
Coeliac disease (CD) is a permanent intolerance of gluten, i.e., of gliadin and related proteins found in the endosperm of wheat, rye and barley. It is characterized by polygenic predisposition, autoimmune nature, predominantly asymptomatic or atypical clinical course, as well as by high prevalence in patients with Down's syndrome (DS) ...
Jonckheere Stijn - - 2010
BACKGROUND: European guidelines stress that iron status should be regularly assessed for the optimal management of renal anemia. These guidelines include the hemoglobin content of reticulocytes and the percentage of hypochromic RBC as markers for functional iron deficiency. Recently, equivalents of these indices have become available on the automated hematology ...
Schwenk Michael H - - 2010
Ferumoxytol is an intravenous iron preparation for treatment of the anemia of chronic kidney disease (CKD). It is a carbohydrate-coated, superparamagnetic iron oxide nanoparticle. Because little free iron is present in the preparation, doses of 510 mg have been administered safely in as little as 17 seconds. Two prospective, randomized ...
Hozumi Isao - - 2010
Patients with marked calcification of the basal ganglia and cerebellum have traditionally been referred to as having Fahr's disease, but the nomenclature has been criticized for including heterogeneous etiology. We describe 3 patients with idiopathic bilateral striatopallidodentate calcinosis (IBSPDC). The patients were a 24-year-old man with mental deterioration, a 57-year-old ...
Anees Muhammad - - 2010
INTRODUCTION: Anemia and hyperhomocysteinemia are risk factor of mortality of patients on dialysis. This study was conducted to assess the relationship of hemoglobin and homocysteine levels and mortality of patients on hemodialysis. MATERIALS AND METHODS: Fifty patients on hemodialysis and 20 healthy individuals were enrolled in the study. Blood samples ...
Burgetova A - - 2010
The aim of this work was to quantify the accumulation of iron in the basal ganglia in multiple sclerosis (MS) patients and in a control group, and to investigate the relationship between iron accumulation and other parameters assessed in MS, i.e. lesion load (LL) and brain parenchymal fraction (BPF). Magnetic ...
Hayashi Hisao - - 2010
In chronic hepatitis C, iron might play an important role as a hepatotoxic co-factor. Therefore, venesection, a standard treatment for hemochromatosis, has been proposed as an alternative for patients who respond poorly to anti-viral therapy. To improve our understanding of iron-induced hepatotoxicity, we compared the responses to venesection between patients ...
Coppo Paul - - 2010
Severe ADAMTS13 deficiency occurs in 13% to 75% of thrombotic microangiopathies (TMA). In this context, the early identification of a severe, antibody-mediated, ADAMTS13 deficiency may allow to start targeted therapies such as B-lymphocytes-depleting monoclonal antibodies. To date, assays exploring ADAMTS13 activity require skill and are limited to only some specialized ...
Dever John B - - 2010
There are limited data on the phenotypic differences between patients with hereditary hemochromatosis (HH) and other forms of iron overload. To describe and compare patients suspected of having iron overload disease. Patients were evaluated at a university iron overload clinic over a 5-year period. Biochemical and clinical profiles of patients ...
Matsumoto Mika - - 2010
BACKGROUND: The etiology of anemia is still unclear in patients with chronic heart failure (CHF). Hepcidin is an iron regulatory peptide that is synthesized in the liver to suppress iron absorption and utilization. Hepcidin synthesis is suppressed by anemia, hypoxia and erythropoiesis, and induced by inflammation. Inflammatory cytokines, such as ...
Roghi Alberto - - 2010
Cardiac involvement in patients with thalassemia intermedia (TI) is characterized by a high-output state and pulmonary hypertension, with systolic left ventricle function usually being preserved. Myocardial iron overload in patients with TI has not been extensively studied. We conducted a cross-sectional study of 49 Italian patients with TI. Patient charts ...
Stancu Simona - - 2010
Anemia is iron responsive in 30 to 50% of nondialysis patients with chronic kidney disease (CKD), but the utility of bone marrow iron stores and peripheral iron indices to predict the erythropoietic response is not settled. We investigated the accuracy of peripheral and central iron indices to predict the response ...
Fenaux Pierre - - 2009
Anaemia is prevalent in patients with myelodysplastic syndromes (MDS), and most patients with MDS receive regular red blood cell transfusions, which can lead to iron overload. Some patients may already have iron overload before transfusions begin, as a result of ineffective erythropoiesis. Iron overload has been linked to hepatic, cardiac, ...
U?arda? Derya - - 2009
BACKGROUND/AIMS: Anemia, especially due to iron deficiency, is a frequent feature in celiac disease. In this study, we aimed to define the prevalence of celiac disease in Turkish patients with iron deficiency anemia of obscure origin. METHODS: One thousand four hundred and eighty-six consecutive patients with iron deficiency anemia were ...
Varma A - - 2009
AIM: The aim of this study was to assess cardiac mortality in patients with reduced ejection fraction (EF</=45%) and anemia (Hb</=12 g/dL) undergoing coronary stenting and to investigate whether iron-deficiency anemia influenced outcome when compared to non-anemic patients or patients with other types of anemia. METHODS: One hundred twenty consecutive ...
Anker Stefan D - - 2009
BACKGROUND: Iron deficiency may impair aerobic performance. This study aimed to determine whether treatment with intravenous iron (ferric carboxymaltose) would improve symptoms in patients who had heart failure, reduced left ventricular ejection fraction, and iron deficiency, either with or without anemia. METHODS: We enrolled 459 patients with chronic heart failure ...
Lambourne Jonathan - - 2009
BACKGROUND: Invasive aspergillosis is a devastating infection with attributable mortality of 40% despite antifungal therapy. In animal models of aspergillosis, deficiency of mannose-binding lectin (MBL), a pattern recognition receptor that activates complement, is a susceptibility factor. MBL deficiency occurs in 20%-30% of the population. We hypothesized that MBL deficiency may ...
Farmaki Kallistheni - - 2010
Cardiac and endocrine disorders are common sequelae of iron overload in transfused thalassaemia patients. Combined chelation with desferrioxamine (DFO) and deferiprone (DFP) is well tolerated and produces an additive/synergistic effect superior to either drug alone. 52 thalassaemia major patients were transitioned from DFO to combined chelation with DFO and DFP. ...
Rath Thomas - - 2010
Intravenous iron supplementation is a basic principle in the therapy of haemodialysis (HD) patients with renal anaemia. In the Iron Dextran in Renal Anaemia (IDIRA) study, we analysed the efficacy of a therapy with low-molecular-weight iron dextran (LMW-ID) in stable HD patients with adequate iron stores previously treated with ferric ...
Compilato D - - 2010
BACKGROUND: Recurrent aphthosis is a common oral ulcerative condition consisting also of a subset of similar ulcers, properly named 'aphthous-like' ulcers (ALU), linked to systemic diseases and among these, to iron, folic acid and vitamin B(12) deficiencies. OBJECTIVES: The main objectives of this study were: (i) to evaluate the association ...
Rosner Mitchell H - - 2009
Iron deficiency anemia is a common occurrence in patients with chronic kidney disease and many patients do not respond well to supplementation with oral iron. There are now five intravenous iron preparations available for use in the chronic kidney disease patient, with ferumoxytol being the most recently approved agent. As ...
Roggero Simona - - 2009
Chronic iron overload is a serious complication in transfusion-dependent patients. Few studies have addressed this issue in Diamond-Blackfan anemia (DBA). We describe a retrospective analysis of iron overload, and its related complications in 31 transfusion-dependent Italian DBA patients whose records included one or more evaluation of liver iron concentration (LIC) ...
Kwapisz Justyna - - 2009
Iron deficiency is a common complication in patients with polycythemia vera (PV). Hepcidin is a principal regulator of iron homeostasis. The aim of our study was to assess prohepcidin, a hepcidin precursor, and other iron status parameters in the serum of PV patients. The study was performed in 60 patients ...
Anker Stefan D - - 2009
Iron deficiency (ID) and anaemia are common in patients with chronic heart failure (CHF). The presence of anaemia is associated with increased morbidity and mortality in CHF, and ID is a major reason for the development of anaemia. Preliminary studies using intravenous (i.v.) iron supplementation alone in patients with CHF ...
Muñoz M - - 2009
Obesity-induced chronic inflammation leads to activation of the immune system that causes alterations of iron homeostasis including hypoferraemia, iron-restricted erythropoiesis, and finally mild-to-moderate anaemia. Thus, preoperative anaemia and iron deficiency are common among obese patients scheduled for bariatric surgery (BS). Assessment of patients should include a complete haematological and biochemical ...
Awasthi R - - 2010
BACKGROUND AND PURPOSE: It has been reported that iron concentration influences DTI metrics in deep gray matter nuclei. We hypothesized that increased FA in the deep gray nuclei may indicate abnormal iron accumulation in patients with PKAN and their siblings. MATERIALS AND METHODS: Seven patients with the characteristic "eye-of-the-tiger sign," ...
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