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1.
Acta Haematol ; 143(1): 26-32, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-31302652

RESUMO

OBJECTIVES: Non-anemic individuals may have undetected subclinical iron deficiency (SID). The aims of this study were to determine the prevalence of SID and identify the associated factors for SID. In addition, the screening performance of red blood cell (RBC) indices for SID in health check-ups was assessed. METHODS: This study was conducted with 16,485 non-anemic health examinees (3,567 males and 12,918 females) who underwent tests for iron variables (serum iron, total iron-binding capacity, ferritin, and iron saturation) at 16 health-promotion centers in 13 cities in Korea between January 2017 and June 2018. SID was defined as a decreased ferritin level (<24 µg/L in males and <15 µg/L in females) and either a decreased serum iron level (<44 µg/dL in males and <29 µg/dL in females) or a transferrin saturation of <20%. RESULTS: The prevalence rates of SID were 0.6 and 3.3% in males and females, respectively. In terms of age and sex, SID was most prevalent in males aged ≥70 years (7.8%) and females aged 15-49 years (7.6%). There were significant differences in the hemoglobin (Hb) level, white blood cell count, platelet count, mean corpuscular volume, mean corpuscular Hb (MCH), and RBC distribution width (RDW) between the SID and non-SID groups (p < 0.001). The factors associated with SID in males were older age (odds ratio, OR, 1.069, 95% confidence interval, CI, 1.03-1.109, p = 0.004), lower Hb (OR 0.58, 95% CI 0.345-0.976, p = 0.04), lower MCH (OR 0.433, 95% CI 0.298-0.629, p < 0.001), and higher RDW (OR 1.374, 95% CI 1.001-1.887, p = 0.049), while in females they were lower body mass index (BMI; OR 0.929, 95% CI 0.895-0.963, p < 0.001) and younger age (OR 0.954, 95% CI 0.945-0.963, p < 0.001), as well as lower Hb, lower MCH, and higher RDW. The AUC for the MCH (0.877, 95% CI 0.793-0.960 in males; 0.872, 95% CI 0.853-0.890 in females) indicates that the MCH at cut-offs of 29.2 and 29.3 pg are the best discriminators of SID in males and females, respectively (p < 0.001). CONCLUSIONS: Reproductive-age females with a lower BMI and elderly males are high-risk groups for SID. MCH is a reliable RBC index for the screening of SID. For the population with defined risk factors, including females with lower BMI and elderly males, screening for SID is needed to prevent the development of anemia.


Assuntos
Distúrbios do Metabolismo do Ferro/diagnóstico , Ferro/deficiência , Adolescente , Adulto , Idoso , Anemia Ferropriva/diagnóstico , Anemia Ferropriva/epidemiologia , Área Sob a Curva , Índice de Massa Corporal , Eritrócitos/citologia , Feminino , Hemoglobinas/análise , Humanos , Ferro/sangue , Distúrbios do Metabolismo do Ferro/epidemiologia , Masculino , Pessoa de Meia-Idade , Prevalência , Curva ROC , República da Coreia/epidemiologia , Estudos Retrospectivos , Fatores de Risco , Adulto Jovem
2.
BMJ Case Rep ; 12(11)2019 Dec 01.
Artigo em Inglês | MEDLINE | ID: mdl-31791989

RESUMO

Neonatal conjugated hyperbilirubinemia is a diagnostic challenge. A full term, small for gestational age boy presented with cholestasis, hypoglycemia, hyperferritinemia and severe bilateral deafness. Diagnostic work-up revealed two hereditary diseases: alpha-1-antitrypsin deficiency (PI*ZZ genotype) and autosomal recessive deafness type 3 (compound heterozygous MYO15A gene mutation). In addition, we found late hypoglycemia on full enteral feeding which complicated this case. Hyperferritinemia is an uncommon finding in newborn cholestasis without liver failure.


Assuntos
Colestase/diagnóstico , Perda Auditiva Neurossensorial/diagnóstico , Hipoglicemia/diagnóstico , Distúrbios do Metabolismo do Ferro/diagnóstico , Deficiência de alfa 1-Antitripsina/diagnóstico , Colestase/genética , Diagnóstico Diferencial , Ferritinas/sangue , Perda Auditiva Neurossensorial/genética , Humanos , Hipoglicemia/genética , Recém-Nascido , Recém-Nascido Pequeno para a Idade Gestacional , Distúrbios do Metabolismo do Ferro/genética , Miosinas/genética , Deficiência de alfa 1-Antitripsina/genética
3.
Curr Pharm Des ; 25(27): 2909-2918, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31686632

RESUMO

BACKGROUND: Ferritin is a molecule that plays many roles being the storage for iron, signalling molecule, and modulator of the immune response. METHODS: Different electronic databases were searched in a non-systematic way to find out the literature of interest. RESULTS: The level of ferritin rises in many inflammatory conditions including autoimmune disorders. However, in four inflammatory diseases (i.e., adult-onset Still's diseases, macrophage activation syndrome, catastrophic antiphospholipid syndrome, and sepsis), high levels of ferritin are observed suggesting it as a remarkable biomarker and pathological involvement in these diseases. Acting as an acute phase reactant, ferritin is also involved in the cytokine-associated modulator of the immune response as well as a regulator of cytokine synthesis and release which are responsible for the inflammatory storm. CONCLUSION: This review article presents updated information on the role of ferritin in inflammatory and autoimmune diseases with an emphasis on hyperferritinaemic syndrome.


Assuntos
Autoimunidade , Ferritinas/sangue , Distúrbios do Metabolismo do Ferro/sangue , Ferro/sangue , Síndrome Antifosfolipídica/sangue , Biomarcadores/sangue , Humanos , Síndrome de Ativação Macrofágica/sangue , Sepse/sangue , Doença de Still de Início Tardio/sangue
4.
Turk J Med Sci ; 49(5): 1560-1563, 2019 Oct 24.
Artigo em Inglês | MEDLINE | ID: mdl-31652037

RESUMO

Background/aim: Polycythemia Vera (PV) is a myeloproliferative disorder characterized by overproduction of morphologically normal red blood cells (RBCs), granulocytes, and platelets, a phenotype that is caused by a mutation (V617F) in Janus kinase 2 (JAK2). However, JAK2 V617F is also found in approximately 50% of patients with essential thrombocytosis and primary myelofibrosis, rendering its presence nonspecific as a diagnostic test. An increased red cell mass is a major criterion for the diagnosis of PV according to World Health Organization (WHO) 2016 criteria. High hemoglobin (Hgb) or Hematocrit (Hct) are universally used as indicators of an increased red cell mass for the diagnosis of PV. However, conditions such as iron deficiency (ID) with decreased mean cell volume may mask the diagnosis due to nonelevated Hct level. The aim of this study was to investigate the clinical characteristics of the patients with unclassifiable non-CML classical myeloproliferative disease with microcytosis (MPD/M) and nonelevated Hgb and Hct levels at diagnosis and to determine if some of these cases could be real PV cases masked due to ID-related microcytosis. Materials and methods: There were 23 MPD/M cases among 208 non-CML classical MPD cases (11%). Among 22 patients who had adequate test results related to the cause of microcytosis, ID and beta-thalassemia trait (TT) were the apparent causes of microcytosis in 15 and 1 cases, respectively. Results: Clinicopathological correlations revealed consistently positive JAK2 V617F mutation status (20/20, 100%), frequently elevated RBC count (17/23, 73.9%), and PV-compatible bone marrow findings (10/12, 83.3%). These findings are compatible with PV instead of essential thrombocytopenia or primary myelofibrosis. In spite of frequent cytoreductive treatment, 3 patients developed increased Hgb/Htc levels during median 58.2 (279­63) months' follow-up. Conclusion: These data show that the majority of MPD/M cases are PV patients masked due to ID-related microcytosis.


Assuntos
Eritrócitos Anormais , Distúrbios do Metabolismo do Ferro/sangue , Distúrbios do Metabolismo do Ferro/diagnóstico , Ferro/deficiência , Transtornos Mieloproliferativos/sangue , Policitemia Vera/sangue , Policitemia Vera/diagnóstico , Adulto , Idoso , Diagnóstico Diferencial , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos
5.
G Ital Cardiol (Rome) ; 20(10): 559-573, 2019 Oct.
Artigo em Italiano | MEDLINE | ID: mdl-31593159

RESUMO

Iron is an essential element for cardiomyocyte viability and contractility. Systemic iron deficiency, even without anemia, is reflected by iron deficiency in cardiomyocytes. As in other cells, there is here a complex, local and autonomous regulation of iron metabolism, based on two molecular systems: the hepcidin/ferroportin/transferrin receptor-1 axis; and the iron regulatory proteins-1,2 system. These molecular pathways allow cardiomyocytes to react to changes in serum iron availability. In mice, dietary manipulations of serum iron availability or cardio-specific deletions and mutations of regulatory genes for intracellular iron metabolism have clarified some aspects of the causal relationship between cardiomyocyte iron deficiency and the development of severe heart failure, prevented by intravenous iron treatment even without the occurrence of iron deficiency (sideropenic) anemia. The deleterious effects of iron deficiency and hypoxia on gene expression of the main regulators of intracellular iron and oxygen metabolism and on cardiac function are very similar in heart failure and in chronic stable ischemic heart disease, and conjure towards cardiomyocyte injury. We here hypothesize that in non-anemic patients with stable ischemic heart disease a chronic or acute serum iron deficiency can amplify the chronic activation of the cardiomyocyte hypoxia-inducible factor-1α. As a consequence, cardiac adaptative responses to chronic hypoxia/ischemia are significantly impaired, and cardiac dysfunction exacerbated. We hypothesize that, in such patients, iron replacement through forced iron supplementation may replete cardiomyocyte iron deficiency and improve ischemic heart disease. This hypothesis requires further experimental studies, but also, and already now, specific clinical trials.


Assuntos
Insuficiência Cardíaca/etiologia , Ferro/deficiência , Isquemia Miocárdica/etiologia , Animais , Doença Crônica , Modelos Animais de Doenças , Insuficiência Cardíaca/prevenção & controle , Humanos , Ferro/metabolismo , Ferro/uso terapêutico , Distúrbios do Metabolismo do Ferro/complicações , Distúrbios do Metabolismo do Ferro/tratamento farmacológico , Isquemia Miocárdica/prevenção & controle , Miocárdio/metabolismo , Miócitos Cardíacos/fisiologia
6.
Clin Ter ; 170(5): e373-e381, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31612196

RESUMO

Among the various pathologies of the oral cavity, the formation of "unsightly black spots" on the surface of the tooth, universally known as Black Stain (BS) has recently been acquiring more interest. Usually BS is typically found in individuals in prepubertal age, even though it has been identified in adults associated with microbial exchange and / or with iron metabolism disorders. Microbial exchange concerns the possible exchange of bacteria between family members which can take place directly, through effusions, or indirectly, through brushes, cutlery or glasses. For this reason, it is recommended that toothbrushes of family members not be left damp and in contact with each other. The bathroom, being a warm-humid environment, is in fact an optimal habitat for microbial proliferation. Of specific importance in BS is the accumulation of iron in tissues and secretions which, together with chromogenic bacteria, are the primary cause of this pathology. In fact, among the metabolic products synthesized by bacteria in the oral cavity, hydrogen sulfide is of considerable interest, since upon reacting with iron available in saliva, in pathological conditions (iron metabolism disorders), it forms black precipitates consisting of ferric sulfide. These precipitates bind to the surface of the teeth, tending to form a stria that usually follows the contour of the gingiva, with an unsightly and variable chromatic intensity. In physiological situations, iron homeostasis is defined as the state of equilibrium between iron present in tissues and in secretions and that which is present in the circulation. Instead, in pathological conditions, defined as iron metabolism disorders, there is an accumulation of iron in tissues and secretions and a lack of it in the circulation. It is also important to remember that subjects affected by BS are more protected from carious processes than healthy subjects, probably due to a significant predominance of chromogenic bacteria compared to those responsible for caries. It should also be remembered that in young subjects BS tends to regress with pubertal development and the transition to adult life. In any case, using common professional hygiene procedures, it is possible to remove BS as well as plaque and tartar deposits. In particular, with ultrasonic scalers, polishing pastes and powders carried by air and water jets, the surfaces of the teeth can be restored to their natural healthy state. All the techniques for removing the precipitates, are not enough however, to fix and permanently eradicate their appearance, as these precipitates last only for short periods and recur very frequently. Due to the frequent recurrences, new oral microbiota control therapies are emerging; among these the use of lactoferrin (Lf) in the dental field and particularly in the treatment of BS appears to be very promising. Taken togheter, here the effect of Lf in subjects affected by BS has been investigated.


Assuntos
Anti-Infecciosos/administração & dosagem , Cárie Dentária/dietoterapia , Distúrbios do Metabolismo do Ferro/tratamento farmacológico , Lactoferrina/administração & dosagem , Descoloração de Dente/diagnóstico , Adulto , Criança , Cárie Dentária/diagnóstico , Placa Dentária/diagnóstico , Feminino , Humanos , Distúrbios do Metabolismo do Ferro/diagnóstico , Masculino , Gravidez , Saliva/metabolismo , Descoloração de Dente/tratamento farmacológico
7.
Pediatr Hematol Oncol ; 36(6): 390-393, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31522592

RESUMO

Hyperferritinemia-cataract syndrome, characterized by high serum ferritin concentration and cataracts in early life, remains a less-known rare disease, with fewer than 100 families reported worldwide. Though benign, high ferritin levels frequently result in misdiagnosis with iron storage disease, and patients can be exposed to unnecessary, even invasive, evaluation and treatment procedures. The presence of cataract together with isolated serum ferritin elevation should alert clinicians to consider this syndrome. We herein present a new family with hyperferritinemia-cataract syndrome to increase clinical awareness.


Assuntos
Ferritinas/sangue , Adolescente , Catarata/congênito , Feminino , Humanos , Distúrbios do Metabolismo do Ferro/congênito
8.
BMJ Open Respir Res ; 6(1): e000454, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31548896

RESUMO

Hypoxia is common in many chronic lung diseases. Beyond pulmonary considerations, delivery of oxygen (O2) to the tissues and subsequent O2 utilisation is also determined by other factors including red blood cell mass and iron status; consequently, disruption to these mechanisms provides further physiological strains on an already stressed system. O2 availability influences ventilation, regulates pulmonary blood flow and impacts gene expression throughout the body. Deleterious effects of poor tissue oxygenation include decreased exercise tolerance, increased cardiac strain and pulmonary hypertension in addition to pathophysiological involvement of multiple other organs resulting in progressive frailty. Increasing inspired O2 is expensive, disliked by patients and does not normalise tissue oxygenation; thus, other strategies that improve O2 delivery and utilisation may provide novel therapeutic opportunities in patients with lung disease. In this review, we focus on the rationale and possibilities for doing this by increasing haemoglobin availability or improving iron regulation.


Assuntos
Anemia/complicações , Anemia/tratamento farmacológico , Hipóxia/tratamento farmacológico , Hipóxia/etiologia , Distúrbios do Metabolismo do Ferro/complicações , Distúrbios do Metabolismo do Ferro/tratamento farmacológico , Pneumopatias/tratamento farmacológico , Pneumopatias/etiologia , Doença Crônica , Humanos
9.
Artigo em Inglês | MEDLINE | ID: mdl-31489256

RESUMO

Background: Specific phenomenology and pattern of involvement in movement disorders point toward a probable clinical diagnosis. For example, forehead chorea usually suggests Huntington's disease; feeding dystonia suggests neuroacanthocytosis and risus sardonicus is commonly seen in Wilson's disease. Dystonic opisthotonus has been described as a characteristic feature of neurodegeneration with brain iron accumulation (NBIA) related to PANK2 and PLA2G6 mutations. Case report: We describe two additional patients in their 30s with severe extensor truncal dystonia causing opisthotonic posturing in whom evaluation revealed the diagnosis of NBIA confirmed by genetic testing. Discussion: Dystonic opisthotonus may be more common in NBIA than it is reported and its presence especially in a young patient should alert the neurologists to a possibility of probable NBIA.


Assuntos
Distonia/etiologia , Distúrbios do Metabolismo do Ferro/complicações , Distrofias Neuroaxonais/complicações , Postura/fisiologia , Adulto , Distonia/fisiopatologia , Humanos , Distúrbios do Metabolismo do Ferro/diagnóstico , Distúrbios do Metabolismo do Ferro/genética , Masculino , Músculo Esquelético/fisiopatologia , Distrofias Neuroaxonais/diagnóstico , Distrofias Neuroaxonais/genética , Tronco/fisiopatologia
10.
Biomed Pharmacother ; 118: 109068, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31404774

RESUMO

NBIA (Neurodegeneration with brain iron accumulation) is a group of inherited neurologic disorders characterized by marked genetic heterogeneity, in which iron atypical accumulates in basal ganglia resulting in brain magnetic resonance imaging changes, histopathological abnormalities, and neuropsychiatric clinical symptoms. With the rapid development of high-throughput sequencing technologies, ten candidate genes have been identified, including PANK2, PLA2G6, C19orf12, WDR45, FA2H, ATP13A2, FTL, CP, C2orf37, and COASY. They are involved in seemingly unrelated cellular pathways, such as iron homeostasis (FTL, CP), lipid metabolism (PLA2G6, C19orf12, FA2H), Coenzyme A synthesis (PANK2, COASY), and autophagy (WDR45, ATP13A2). In particular, PANK2, COASY, PLA2G6, and C19orf12 are located on mitochondria, which associate with certain subtypes of NBIA showing mitochondria dysregulation. However, the relationships among those four genes are still unclear. Therefore, this review is specifically focused on dysregulation of mitochondria in NBIA and afore-mentioned four genes, with summaries of both pathological and clinical findings.


Assuntos
Fosfolipases A2 do Grupo VI/genética , Distúrbios do Metabolismo do Ferro/genética , Mitocôndrias/patologia , Proteínas Mitocondriais/genética , Distrofias Neuroaxonais/genética , Fosfotransferases (Aceptor do Grupo Álcool)/genética , Transferases/genética , Humanos , Distúrbios do Metabolismo do Ferro/metabolismo , Distúrbios do Metabolismo do Ferro/patologia , Metabolismo dos Lipídeos/genética , Potencial da Membrana Mitocondrial/genética , Distrofias Neuroaxonais/metabolismo , Distrofias Neuroaxonais/patologia
11.
Adv Exp Med Biol ; 1173: 1-19, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31456202

RESUMO

Iron is the most abundant trace element in the human body. It is well known that iron is an important component of hemoglobin involved in the transport of oxygen. As a component of various enzymes, it participates in the tricarboxylic acid cycle and oxidative phosphorylation. Iron in the nervous system is also involved in the metabolism of catecholamine neurotransmitters and is involved in the formation of myelin. Therefore, iron metabolism needs to be strictly regulated. Previous studies have shown that iron deficiency in the brain during infants and young children causes mental retardation, such as delayed development of language and body balance, and psychomotor disorders. However, if the iron is excessively deposited in the aged brain, it is closely related to the occurrence of various neurodegenerative diseases, such as Alzheimer's disease, Parkinson's disease, and Friedreich's ataxia. Therefore, it is important to fully study and understand the mechanism of brain iron metabolism and its regulation. On this basis, exploring the relationship between brain iron regulation and the occurrence of nervous system diseases and discovering new therapeutic targets related to iron metabolism have important significance for breaking through the limitation of prevention and treatment of nervous system diseases. This review discusses the complete research history of iron and its significant role in the pathogenesis of the central nervous system (CNS) diseases.


Assuntos
Encéfalo/metabolismo , Doenças do Sistema Nervoso Central , Ferro/metabolismo , Humanos , Distúrbios do Metabolismo do Ferro , Doenças Neurodegenerativas
12.
Adv Exp Med Biol ; 1173: 33-44, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31456204

RESUMO

With the development of research, more and more evidences suggested that mutations in the genes associated with brain iron metabolism induced diseases in the brain. Brain iron metabolism disorders might be one cause of neurodegenerative diseases. This review mainly summarizes the normal process of iron entry into the brain across the blood-brain barrier, and the distribution and transportation of iron among neurons and glial cells, as well as the underlying regulation mechanisms. To understand the mechanisms of iron metabolism in the brain will provide theoretical basis to prevent and cure brain diseases related to iron metabolism disorders.


Assuntos
Encéfalo/metabolismo , Distúrbios do Metabolismo do Ferro , Ferro/metabolismo , Barreira Hematoencefálica , Humanos , Neuroglia/metabolismo , Neurônios/metabolismo
13.
Adv Exp Med Biol ; 1173: 179-194, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31456211

RESUMO

Iron has been proposed to be responsible for neuronal loss in several diseases of the central nervous system, including Alzheimer's disease (AD), Parkinson's disease (PD), stroke, Friedreich's ataxia (FRDA), multiple sclerosis (MS), amyotrophic lateral sclerosis (ALS). In many diseases, abnormal accumulation of brain iron in disease-affected area has been observed, without clear knowledge of the contribution of iron overload to pathogenesis. Recent evidences implicate that key proteins involved in the disease pathogenesis may also participate in cellular iron metabolism, suggesting that the imbalance of brain iron homeostasis is associated with the diseases. Considering the complicated regulation of iron homeostasis within the brain, a thorough understanding of the molecular events leading to this phenotype is still to be investigated. However, current understanding has already provided the basis for the diagnosis and treatment of iron-related CNS diseases, which will be reviewed here.


Assuntos
Distúrbios do Metabolismo do Ferro/diagnóstico , Distúrbios do Metabolismo do Ferro/terapia , Doenças Neurodegenerativas/diagnóstico , Doenças Neurodegenerativas/terapia , Homeostase , Humanos , Ferro
14.
Semin Liver Dis ; 39(4): 476-482, 2019 11.
Artigo em Inglês | MEDLINE | ID: mdl-31330553

RESUMO

Hyperferritinemia, observed in inflammation, iron overload as well as in combination of both, is found in ∼30% of nonalcoholic fatty liver disease (NAFLD) patients. The authors summarized the evidence regarding the potential cause of hyperferritinemia in NAFLD, as this may affect the indicated therapy. A systematic literature search was conducted in EMBASE, PubMed, MEDLINE, and the Cochrane library. In the majority of NAFLD patients, hyperferritinemia is due to inflammation without hepatic iron overload. In a smaller group, a dysmetabolic iron overload syndrome (DIOS) is found, showing hyperferritinemia in combination with mild iron accumulation in the reticuloendothelial cells. The smallest group consists of NAFLD patients with hemochromatosis. Phlebotomy is only effective with hepatocellular iron overload and should not be the treatment when hyperferritinemia is related to inflammation, whether or not combined with DIOS. Treatment with lifestyle changes is to date probably the more effective way until new medication is becoming available.


Assuntos
Inflamação/complicações , Distúrbios do Metabolismo do Ferro/complicações , Hepatopatia Gordurosa não Alcoólica/complicações , Exercício Físico , Proteína da Hemocromatose/genética , Hepatócitos/metabolismo , Homeostase , Humanos , Distúrbios do Metabolismo do Ferro/terapia , Mutação , Flebotomia
15.
Transfus Apher Sci ; 58(4): 416-421, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31281092

RESUMO

Anemia in elderly (AE), though often mild, is quite common and independently associated with important clinical outcomes, including decreased quality of life, risk of falls and fractures, cognitive decline, increased length of hospital stay, and even mortality. AE is generally overlooked, and hence undertreated, especially when comorbidities distract the attention of physicians and caregivers. This also partially reflects difficulties in dissecting the cause(s) of AE, which is typically multifactorial, as well as our limited diagnostic approach often categorizing AE as apparently "unexplained". Therapeutic approaches have been traditionally limited to transfusions, or supplementation with hematinics, including group B vitamins and iron. The latter has been largely underutilized, because of missing diagnosis of iron deficiency using inappropriate laboratory thresholds, as well as complex schedule and adverse effects associated with traditional preparations. After decades of stagnation, new oral and intravenous iron preparations look promising, particularly in the elderly. Moreover, a number of innovative anti-anemic drugs, like hepcidin modulators, Hypoxia Inducible Factor (HIF) stabilizers, and activin type II receptor agonists are entering the clinical arena and may substantially improve our therapeutic armamentarium to AE in the near future.


Assuntos
Anemia , Serviços de Saúde para Idosos , Hematínicos/uso terapêutico , Distúrbios do Metabolismo do Ferro , Ferro , Administração Intravenosa , Idoso , Idoso de 80 Anos ou mais , Anemia/sangue , Anemia/tratamento farmacológico , Humanos , Ferro/deficiência , Ferro/uso terapêutico , Distúrbios do Metabolismo do Ferro/sangue , Distúrbios do Metabolismo do Ferro/tratamento farmacológico
16.
Medicina (Kaunas) ; 55(7)2019 Jul 02.
Artigo em Inglês | MEDLINE | ID: mdl-31269687

RESUMO

Background and objectives: The etiology of anemia associated with heart failure is not fully understood, but there are data suggesting the involvement of multiple mechanisms, including various drug therapies used in patients with heart failure. Our primary objective was to evaluate the impact of beta blockers, angiotensin-converting enzyme inhibitors, and calcium-channel blockers on iron metabolism in patients with heart failure. Materials and Methods: This was a prospective observational study that included patients diagnosed with heart failure and iron deficiency (defined by ferritin <100 µg/L, or 100-300 µg/L with transferrin saturation <20%). Patients with anemia secondary to a known cause were excluded. Results: We found a statistically significant correlation between beta-blocker treatment and ferritin values (p = 0.02). Iron, hemoglobin, and hematocrit levels were significantly lower in the patients using calcium-channel blockers than those who were not. We also found a statistically significant indirect correlation (p = 0.04) between the use of angiotensin-converting enzyme inhibitors and hematocrit levels. Conclusion: The contribution of our study arises from the additional data regarding the drug-induced etiology of iron deficiency. Practitioners should be aware of the potential impact of therapeutic recommendations and this should imply a close monitoring of the biochemical parameters of iron deficiency in this category of patients.


Assuntos
Anemia/etiologia , Insuficiência Cardíaca/tratamento farmacológico , Distúrbios do Metabolismo do Ferro/etiologia , Antagonistas Adrenérgicos beta/efeitos adversos , Antagonistas Adrenérgicos beta/uso terapêutico , Idoso , Anemia/sangue , Anemia/complicações , Inibidores da Enzima Conversora de Angiotensina/efeitos adversos , Inibidores da Enzima Conversora de Angiotensina/uso terapêutico , Bloqueadores dos Canais de Cálcio/efeitos adversos , Bloqueadores dos Canais de Cálcio/uso terapêutico , Feminino , Insuficiência Cardíaca/sangue , Humanos , Ferro/análise , Ferro/sangue , Distúrbios do Metabolismo do Ferro/sangue , Masculino , Pessoa de Meia-Idade
17.
Am J Trop Med Hyg ; 101(3): 705-707, 2019 09.
Artigo em Inglês | MEDLINE | ID: mdl-31309922

RESUMO

A 43-year-old man was admitted to the intensive care unit and diagnosed with yellow fever. He presented with refractory bleeding, extreme hyperferritinemia, and multiple organ dysfunction syndrome, requiring renal replacement therapy, mechanical ventilation, and treatment with vasoactive drugs. Because the bleeding did not respond to fresh-frozen plasma administration, the patient received therapeutic plasma exchange, which was accompanied by a marked improvement of the clinical and biochemical parameters, including a significant decline in serum ferritin levels.


Assuntos
Distúrbios do Metabolismo do Ferro/diagnóstico , Distúrbios do Metabolismo do Ferro/terapia , Troca Plasmática , Febre Amarela/complicações , Febre Amarela/diagnóstico , Adulto , Hemorragia , Humanos , Unidades de Terapia Intensiva , Masculino , Insuficiência de Múltiplos Órgãos/terapia , Sepse/terapia
18.
Glia ; 67(9): 1760-1774, 2019 09.
Artigo em Inglês | MEDLINE | ID: mdl-31162719

RESUMO

Developmental iron deficiency (dID) models facilitate the study of specific oligodendrocyte (OL) requirements for their progression to a mature state and subsequent contribution to myelination. In the current work, we used the dID model in transgenic mice expressing green fluorescence protein under the CNPase promoter allowing the identification of cells belonging to the oligodendroglial lineage, and the visualization of the entire myelin structure and single OL morphology. The present work evaluates dID effects on OL complexity in different brain areas. Control animals showed an increase in OL complexity both during development and along the anterior-posterior axis. In contrast, dID animals exhibited an initial increase in CNPase+ cells with prevalence of immature-OL (i-OL), an effect later compensated during development by selective death of those i-OL. As a consequence, developmental behavior was impaired in terms of body balance, muscle response, and sensorimotor functions. To explore why i-OL fail to mature in dID, expression levels of transcriptional factors involved in the maturation of the OL lineage were studied. In nuclear fractions, dID animals showed an increase in Hes5, which prevents the maturation of i-OL, and a decrease in Sox10, a positive regulator of OL maturation. The cytoplasmic fractions showed a decrease in Olig1, which is critical for precursor cell differentiation into premyelinating OL. Overall, the expression levels of Hes5, Sox10, and Olig1 in dID conditions correlated with an unfavorable OL maturation profile. In sum, the current results provide further evidence of dID impact on myelination, keeping OL away from the maturational path.


Assuntos
Encéfalo/crescimento & desenvolvimento , Encéfalo/metabolismo , Distúrbios do Metabolismo do Ferro/metabolismo , Ferro/deficiência , Oligodendroglia/metabolismo , Fenômenos Fisiológicos da Nutrição Pré-Natal , Animais , Encéfalo/patologia , Modelos Animais de Doenças , Feminino , Proteínas de Fluorescência Verde/genética , Proteínas de Fluorescência Verde/metabolismo , Distúrbios do Metabolismo do Ferro/patologia , Masculino , Camundongos Endogâmicos C57BL , Camundongos Transgênicos , Oligodendroglia/patologia , Gravidez
19.
Rev Med Interne ; 40(10): 680-683, 2019 Oct.
Artigo em Francês | MEDLINE | ID: mdl-31213336

RESUMO

INTRODUCTION: Gaucher disease type 1 is a rare genetic disease. It can cause thrombocytopenia. Current guidelines do not support bone marrow examination in front of isolated thrombocytopenia if no evidence suggests malignant hemopathy. This strategy aiming at sparing unnecessary investigations makes such rare diseases more difficult to diagnose. CASE REPORT: A 31-year-old woman was diagnosed with immune thrombocytopenia according to current guidelines. She presented later with mild splenomegaly. Bone marrow aspirate smears showed Gaucher cells. Gaucher disease was then confirmed. Looking backward, initial biological clues (hyperferritinemia, hypergammaglobulinemia) should have enabled to consider the diagnosis. CONCLUSION: Gaucher disease type 1 can be responsible for apparently isolated thrombocytopenia. The disease must be looked for if the thrombocytopenia is associated with unexplained hypergammaglobulinemia or hyperferritinemia. Diagnosing immune thrombocytopenia without bone marrow sample requires to systematically pay attention to any clinical or biological abnormality, not to ignore rare differential diagnoses.


Assuntos
Doença de Gaucher/diagnóstico , Trombocitopenia/diagnóstico , Adulto , Diagnóstico Diferencial , Feminino , Ferritinas/sangue , Doença de Gaucher/sangue , Humanos , Hipergamaglobulinemia/diagnóstico , Distúrbios do Metabolismo do Ferro/diagnóstico , Gravidez , Complicações Hematológicas na Gravidez/sangue , Esplenomegalia/etiologia , Trombocitopenia/complicações , Trombocitopenia/imunologia
20.
Nutrients ; 11(5)2019 May 20.
Artigo em Inglês | MEDLINE | ID: mdl-31137583

RESUMO

BACKGROUND: Since haemodialysis is a lifesaving therapy, adequate control measures are necessary to evaluate its adequacy and to constantly adjust the dose to reduce hospitalisation and prolong patient survival. Malnutrition is common in haemodialysis patients and closely related to morbidity and mortality. Patients undergoing haemodialysis have a high prevalence of protein-energy malnutrition and inflammation, along with abnormal iron status. The haemodialysis dose delivered is an important predictor of patient outcome. AIM: To evaluate through haemodialysis adequacy, which parameter(s), if any, better predict Kt/V, among those used to assess nutritional status, inflammation response, and iron status. METHODS: We retrospectively studied 78 patients undergoing haemodialysis due to end-stage renal disease. As parameters of nutritional status, geriatric nutritional risk index (GNRI), transferrin levels, lymphocyte count, and albumin concentration were analysed. As signs of inflammation, C reactive protein (CRP) levels and ferritin concentrations were studied as well. Iron status was evaluated by both transferrin and ferritin levels, as well as by haemoglobin (Hb) concentration. RESULTS: The core finding of our retrospective study is that transferrin levels predict the adequacy of haemodialysis expressed as Kt/V; the latter is the only predictor (P = 0.001) when adjusting for CRP concentrations, a solid marker of inflammation, and for ferritin levels considered an iron-storage protein, but also a parameter of inflammatory response. DISCUSSION AND CONCLUSION: In keeping with the results of this study, we underline that the use of transferrin levels to assess haemodialysis quality combine into a single test the evaluation of the three most important factors of protein-energy wasting.


Assuntos
Inflamação/sangue , Distúrbios do Metabolismo do Ferro/sangue , Falência Renal Crônica/terapia , Desnutrição Proteico-Calórica/sangue , Diálise Renal , Transferrina/metabolismo , Idoso , Biomarcadores/sangue , Feminino , Avaliação Geriátrica , Humanos , Inflamação/diagnóstico , Inflamação/etiologia , Mediadores da Inflamação/sangue , Ferro/sangue , Ferro/deficiência , Distúrbios do Metabolismo do Ferro/diagnóstico , Distúrbios do Metabolismo do Ferro/etiologia , Falência Renal Crônica/sangue , Falência Renal Crônica/diagnóstico , Masculino , Pessoa de Meia-Idade , Estado Nutricional , Valor Preditivo dos Testes , Desnutrição Proteico-Calórica/diagnóstico , Desnutrição Proteico-Calórica/etiologia , Diálise Renal/efeitos adversos , Estudos Retrospectivos , Fatores de Risco , Resultado do Tratamento
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