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1.
Hosp Pract (1995) ; 46(3): 144-151, 2018 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-29648482

RESUMO

Acute chest syndrome (ACS) is a leading complication of sickle cell disease (SCD) with significant morbidity and mortality. ACS is the most common cause of death and the second most common cause of hospitalization in patients with SCD. Delineating the specific cause of ACS is often difficult, and multiple risk factors that precipitate ACS frequently coexist. The prominent risk factors include infection, hypoxia, bronchial hyperresponsiveness, the SCD genotype, and opioid use. The key to the successful treatment of ACS is early recognition and initiation of treatment without delay. The main goal is to prevent and treat acute respiratory failure and, thus, minimize irreversible lung damage. This review focuses on the risk factors, pathogenesis, clinical presentation, and management of ACS.


Assuntos
Síndrome Torácica Aguda/etiologia , Anemia Falciforme/complicações , Síndrome Torácica Aguda/prevenção & controle , Síndrome Torácica Aguda/terapia , Anemia Falciforme/prevenção & controle , Anemia Falciforme/terapia , Feminino , Humanos , Masculino
2.
PLoS One ; 13(4): e0196455, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-29694434

RESUMO

During hemolysis, hemoglobin and heme released from red blood cells promote oxidative stress, inflammation and thrombosis. Plasma haptoglobin and hemopexin scavenge free hemoglobin and heme, respectively, but can be depleted in hemolytic states. Haptoglobin and hemopexin supplementation protect tissues, including the vasculature, liver and kidneys. It is widely assumed that these protective effects are due primarily to hemoglobin and heme clearance from the vasculature. However, this simple assumption does not account for the consequent cytoprotective adaptation seen in cells and organs. To further address the mechanism, we used a hyperhemolytic murine model (Townes-SS) of sickle cell disease to examine cellular responses to haptoglobin and hemopexin supplementation. A single infusion of haptoglobin or hemopexin (± equimolar hemoglobin) in SS-mice increased heme oxygenase-1 (HO-1) in the liver, kidney and skin several fold within 1 hour and decreased nuclear NF-ĸB phospho-p65, and vaso-occlusion for 48 hours after infusion. Plasma hemoglobin and heme levels were not significantly changed 1 hour after infusion of haptoglobin or hemopexin. Haptoglobin and hemopexin also inhibited hypoxia/reoxygenation and lipopolysaccharide-induced vaso-occlusion in SS-mice. Inhibition of HO-1 activity with tin protoporphyrin blocked the protections afforded by haptoglobin and hemopexin in SS-mice. The HO-1 reaction product carbon monoxide, fully restored the protection, in part by inhibiting Weibel-Palade body mobilization of P-selectin and von Willebrand factor to endothelial cell surfaces. Thus, the mechanism by which haptoglobin and hemopexin supplementation in hyperhemolytic SS-mice induces cytoprotective cellular responses is linked to increased HO-1 activity.


Assuntos
Anemia Falciforme/prevenção & controle , Haptoglobinas/uso terapêutico , Heme Oxigenase-1/metabolismo , Hemopexina/uso terapêutico , Inflamação/prevenção & controle , Aldeídos/análise , Anemia Falciforme/patologia , Animais , Monóxido de Carbono/farmacologia , Citocinas/análise , Modelos Animais de Doenças , Feminino , Expressão Gênica/efeitos dos fármacos , Haptoglobinas/farmacologia , Hemopexina/farmacologia , Molécula 1 de Adesão Intercelular , Masculino , Metaloporfirinas/farmacologia , Camundongos , Microssomos Hepáticos/metabolismo , Protoporfirinas/farmacologia , Pele/metabolismo , Pele/patologia , Fator de Transcrição RelA/metabolismo , Molécula 1 de Adesão de Célula Vascular/metabolismo
3.
Sultan Qaboos Univ Med J ; 18(1): e24-e29, 2018 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-29666677

RESUMO

Due to the high rate of consanguineous marriages in Oman, there is a correspondingly high prevalence of hereditary blood disorders, particularly sickle cell disease and ß-thalassaemia. This article proposes the possibility of implementing mandatory premarital carrier screening for blood disorders in Oman, while giving due consideration to potential social and cultural obstacles. Although the implementation of such legislation would require collaboration between different sectors and may negatively affect the autonomy of certain individuals, mandatory premarital screening would help to alleviate the burden of hereditary blood disorders on the national healthcare system, as well as reduce avoidable suffering among carriers and their families.


Assuntos
Programas de Rastreamento/métodos , Exames Pré-Nupciais/métodos , Anemia Falciforme/genética , Anemia Falciforme/prevenção & controle , Consanguinidade , Testes Genéticos/métodos , Humanos , Programas de Rastreamento/tendências , Omã , Exames Pré-Nupciais/tendências , Prevalência , Talassemia beta/genética , Talassemia beta/prevenção & controle
4.
Int J Immunogenet ; 44(5): 219-224, 2017 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-28815969

RESUMO

Cytotoxic T-lymphocyte-associated antigen 4 (CTLA-4) molecule is expressed on T-lymphocyte membrane and negatively influences the antigen-presenting process. Reduced expression of CTLA-4 due to gene polymorphisms is associated with increased risk of autoimmune disorders, whose physiopathology is similar to that of post-transfusion red blood cell (RBC) alloimmunization. Our goal was to evaluate if polymorphisms of CTLA-4 gene that affect protein expression are associated with RBC alloimmunization. This was a case-control study in which 134 sickle cell disease (SCD) patients and 253 non-SCD patients were included. All patients were genotyped for the polymorphisms 49A/G and -318C/T of CTLA-4 gene. The genotype frequency of -318C/T differed significantly between alloimmunized and nonalloimmunized SCD patients, irrespective of clinical confounders (p = .016). SCD patients heterozygous for -318T allele presented higher risk of alloantibody development (OR: 5.4, CI: 1.15-25.6). In conclusion, the polymorphism -318C/T of CTLA-4 gene is associated with RBC alloimmunization among SCD patients. This highlights the role played by CTLA-4 on post-transfusion alloantibody development.


Assuntos
Anemia Falciforme/genética , Doenças Autoimunes/genética , Antígeno CTLA-4/genética , Eritrócitos/imunologia , Adulto , Anemia Falciforme/sangue , Anemia Falciforme/imunologia , Anemia Falciforme/prevenção & controle , Células Apresentadoras de Antígenos/imunologia , Células Apresentadoras de Antígenos/patologia , Doenças Autoimunes/sangue , Doenças Autoimunes/imunologia , Doenças Autoimunes/patologia , Antígeno CTLA-4/imunologia , Eritrócitos/patologia , Feminino , Predisposição Genética para Doença , Genótipo , Humanos , Imunização/efeitos adversos , Isoanticorpos/imunologia , Masculino , Pessoa de Meia-Idade , Polimorfismo de Nucleotídeo Único/genética , Fatores de Risco , Linfócitos T/imunologia , Linfócitos T/patologia
5.
Pediatr Blood Cancer ; 64(11)2017 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-28556441

RESUMO

Sickle cell disease (SCD) results in end organ damage and a shortened lifespan. Both the pathophysiology of the disease and the social determinants of health affect patient outcomes. Randomized controlled trials have been completed among this population and resulted in medical advances; however, the gestation of these advances and the lack of penetrance into clinical practice have limited advancements in clinical improvements for many people with SCD. We discuss the role of implementation science in SCD and highlight the need for this science to shorten the length of time to implement evidence-based care for more people with SCD.


Assuntos
Anemia Falciforme/diagnóstico , Anemia Falciforme/prevenção & controle , Antidrepanocíticos/uso terapêutico , Humanos , Prognóstico
6.
Recurso na Internet em Português | LIS - Localizador de Informação em Saúde | ID: lis-LISBR1.1-45233

RESUMO

Página da Federação Nacional das Associações de Pessoas com Doença Falciforme (FENAFAL) com informações e orientações para pessoas portadoras da doença.


Assuntos
Anemia Falciforme/prevenção & controle , Doenças Hematológicas
7.
Int J Stroke ; 12(6): 580-588, 2017 08.
Artigo em Inglês | MEDLINE | ID: mdl-28440126

RESUMO

Background Sickle cell disease (SCD) is one of the most common causes of stroke in children worldwide. Based on the results of the Stroke Prevention Trial in Sickle Cell Anemia (STOP), annual transcranial Doppler ultrasound (TCD) screening for affected children is standard practice. However, the need for TCD surveillance programs could override the accuracy of the screening, affecting the correct stratification of stroke risk and subsequent clinical management of the target population. Aims To shed light on this issue, a systematic review of the literature on TCD screening for children and adolescents with SCD was carried out (CRD42016050549), according to a list of clinically relevant questions, with a particular focus on screening practices in European countries. Quality of the evidence was rated using the grading of recommendations assessment, development and evaluation. Summary of review Thirty-three studies published in English or French were included (5 randomized controlled trials, 8 experimental non-randomized, and 20 observational studies). The quality of the retrieved evidence ranged between low and high, but was rated as moderate or high most of the times. TCD is effective as a screening tool for the primary prevention of stroke in SCD children. There is no high-quality evidence on the effectiveness of alternative screening methods, such as imaging-TCD with or without angle correction or magnetic resonance angiography. No evidence was found on effectiveness of the screening on children on hydroxyurea and with genotypes other than HbSS and HbS/ß0. No European data were found on screening rates or adherence of screening practices to the STOP protocol. Conclusions High-quality studies on alternative screening methods that are currently used in real-world practice, and on screening applicability to specific subgroups of patients are urgently needed. Considering the low awareness of the disease in European countries and the lack of data on screening practices and adherence, clinicians need up-to-date guidelines for more uniform and evidence-based surveillance of children with SCD.


Assuntos
Anemia Falciforme/complicações , Prevenção Primária , Acidente Vascular Cerebral/prevenção & controle , Ultrassonografia Doppler Transcraniana , Anemia Falciforme/prevenção & controle , Criança , Humanos , Angiografia por Ressonância Magnética/métodos , Risco , Acidente Vascular Cerebral/complicações , Ultrassonografia Doppler Transcraniana/métodos
9.
Brasília; Ministério da Saúde; dez. 2016. 42 p. ilus, tab.
Monografia em Português | LILACS, PIE | ID: biblio-971877

RESUMO

A doença falciforme é uma das doenças hereditárias mais comuns no mundo. No estado de São Paulo, a incidência de doença falciforme é de um para quatro mil (1:4.000) nascidos vivos. Uma mutação no gene que produz a hemoglobina A origina, por meio de herança recessiva, a hemoglobina S (HbS). Em situações de ausência ou diminuição da tensão de oxigênio, a HbS altera drasticamente a morfologia do eritrócito (forma de foice ou meia lua), causando vaso-oclusão e, consequentemente, isquemia, dor, necrose, disfunções, danos permanentes aos tecidos e órgãos, além de hemólise crônica. Devido às possíveis manifestações logo no primeiro ano de vida, é de suma importância o diagnóstico precoce na triagem neonatal.


Assuntos
Humanos , Adolescente , Anemia Falciforme/prevenção & controle , Anemia Falciforme/terapia , Traço Falciforme/terapia , Saúde do Adolescente , Serviços de Saúde do Adolescente , Medicina do Adolescente , Antidrepanocíticos/uso terapêutico , Assistência Integral à Saúde , Medicina Baseada em Evidências , Adesão à Medicação , Aceitação pelo Paciente de Cuidados de Saúde , Assistência ao Paciente , Planejamento de Assistência ao Paciente
10.
BMC Health Serv Res ; 16: 304, 2016 07 26.
Artigo em Inglês | MEDLINE | ID: mdl-27461265

RESUMO

BACKGROUND: Sickle cell disease (SCD) constitutes a major public health problem in sub-Saharan Africa (SSA). Newborn screening and early subsequent clinical intervention can reduce early mortality and increase life expectancy, but have not been widely implemented in SSA. This analysis assesses the cost-effectiveness of a newborn screening and prophylactic intervention (NSPI) package for SCD in 47 SSA countries. METHODS: A lifetime Markov model with annual cycles was built with infants either being screened using isoelectric focusing (IEF) or not screened. Confirmed positive cases received interventions including insecticide-treated mosquito bed nets, folic acid supplementation, prophylactic antimalarial and penicillin therapy, and vaccinations against bacterial infections. Estimates for the local incidence of SCD, the life expectancy of untreated children, the SCD disability weight, and the cost of screening laboratory tests were based on published sources. Among treated infants, the annual probability of mortality until 30 years of age was derived from a pediatric hospital-based cohort. The outcome of interest included a country-specific cost per Disability Adjusted Life Year (DALY) averted. RESULTS: Of 47 modeled countries in SSA, NSPI is almost certainly highly cost-effective in 24 countries (average cost per DALY averted: US$184); in 10 countries, it is cost-effective in the base case (average cost per DALY averted: US$285), but the results are subject to uncertainty; in the remaining 13, it is most likely not cost-effective. We observe a strong inverse relationship between the incidence rate of SCD and the cost per DALY averted. Newborn screening is estimated to be cost-effective as long as the incidence rate exceeds 0.2-0.3 %, although in some countries NSPI is cost-effective at incidence rates below this range. In total, NSPI could avert over 2.4 million disability adjusted life years (DALYs) annually across SSA. CONCLUSIONS: Using IEF to screen all newborns for SCD plus administration of prophylactic interventions to affected children is cost-effective in the majority of countries in SSA.


Assuntos
Anemia Falciforme/prevenção & controle , Triagem Neonatal/métodos , Adolescente , África ao Sul do Saara , Anemia Falciforme/economia , Antimaláricos/economia , Antimaláricos/uso terapêutico , Criança , Pré-Escolar , Análise Custo-Benefício , Ácido Fólico/administração & dosagem , Ácido Fólico/economia , Humanos , Incidência , Lactente , Recém-Nascido , Expectativa de Vida , Malária/prevenção & controle , Triagem Neonatal/economia , Anos de Vida Ajustados por Qualidade de Vida , Estudos Retrospectivos
11.
Proteomics Clin Appl ; 10(8): 816-30, 2016 08.
Artigo em Inglês | MEDLINE | ID: mdl-27193514

RESUMO

Sickle cell disease (SCD) is an autosomal recessively inherited ß-hemoglobinopathy causing a sickling hemoglobin (HbS) to be expressed in the erythrocyte. Due its unique biophysical properties and tendency to form polymers in deoxygenated conditions, HbS causes dramatic erythrocyte deformation and damage ultimately leading to diffuse hemolysis, vasco-occlusion, and vasculopathy in affected individuals. Albeit SCD was the first molecular disease identified in the human several decades ago, the progress in caring for patients with SCD has been globally limited and faces considerable biological, medical, psychological, and economic challenges. The aim of this review is to illustrate the opportunities lying ahead for proteomic discovery in SCD. It also establishes a tentative conceptual framework for clinically oriented investigations. The ultimate target is the translation of findings into validated and actionable improvements at the bedside. Thanks to significant technological advances, proteomics is poised to play an important role for patients affected by hematological disorders, and SCD could be a paradigm for impactful research.


Assuntos
Anemia Falciforme/metabolismo , Proteômica/métodos , Anemia Falciforme/epidemiologia , Anemia Falciforme/prevenção & controle , Anemia Falciforme/terapia , Humanos , Internacionalidade
13.
BMJ Clin Evid ; 20162016 Jan 22.
Artigo em Inglês | MEDLINE | ID: mdl-26808098

RESUMO

INTRODUCTION: Sickle cell disease causes chronic haemolytic anaemia, dactylitis, and painful acute crises. It also increases the risk of stroke, organ damage, bacterial infections, and complications of blood transfusion. In sub-Saharan Africa, up to one third of adults are carriers of the defective sickle cell gene, and 1% to 2% of babies are born with the disease. METHODS AND OUTCOMES: We conducted a systematic overview, aiming to answer the following clinical question: What are the effects of pharmaceutical interventions to prevent sickle cell crisis and other acute complications in people with sickle cell disease? We searched: Medline, Embase, The Cochrane Library, and other important databases up to January 2015 (Clinical Evidence overviews are updated periodically; please check our website for the most up-to-date version of this overview). RESULTS: At this update, searching of electronic databases retrieved 369 studies. After deduplication and removal of conference abstracts, 136 records were screened for inclusion in the overview. Appraisal of titles and abstracts led to the exclusion of 99 studies and the further review of 37 full publications. Of the 37 full articles evaluated, three already included systematic reviews were updated, two systematic reviews, two RCTs, and one subsequent RCT were added at this update. We performed a GRADE evaluation for 12 PICO combinations. CONCLUSIONS: In this systematic overview, we categorised the efficacy for five interventions based on information about the effectiveness and safety of antibiotic prophylaxis in children aged under 5 years, antibiotic prophylaxis in children aged 5 years or older, hydroxyurea, malaria chemoprophylaxis, and pneumococcal vaccines.


Assuntos
Anemia Falciforme/terapia , Anemia Falciforme/prevenção & controle , Antibioticoprofilaxia , Transfusão de Sangue , Humanos , Hidroxiureia/uso terapêutico , Vacinas Pneumocócicas
14.
Pediatr Blood Cancer ; 63(1): 168-70, 2016 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-26275168

RESUMO

Sickle cell disease is among hereditary diseases with evidence that early diagnoses and treatment improves the clinical outcome. So far sickle cell disease has not been included in the German newborn screening program despite immigration from countries with populations at risk. To determine the birth prevalence we tested 17,018 newborns. High pressure liquid chromatography and subsequent molecular-genetic testing were used for the detection and confirmation of hemoglobin variants. The frequency of sickle cell disease-consistent genotypes was one in 2,385 newborns. Duffy-blood group typing showed evidence that affected children were likely of Sub-Saharan ancestry. An inclusion of sickle cell disease into the German newborn screening seems reasonable.


Assuntos
Anemia Falciforme/epidemiologia , Anemia Falciforme/prevenção & controle , Triagem Neonatal , Genótipo , Alemanha/epidemiologia , Humanos , Recém-Nascido , Triagem Neonatal/métodos , Prevalência
15.
Am J Hematol ; 91(1): 5-14, 2016 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-26547630

RESUMO

Over the past 40 years, public health measures such as universal newborn screening, penicillin prophylaxis, vaccinations, and hydroxyurea therapy have led to an impressive decline in sickle cell disease (SCD)-related childhood mortality and SCD-related morbidity in high-income countries. We remain cautiously optimistic that the next 40 years will be focused on meeting current challenges in SCD by addressing chronic complications of SCD to reduce mortality and improve quality of life in a growing population of adults with SCD in high-income countries, while simultaneously decreasing the disparity of medical care between high and low-income countries.


Assuntos
Anemia Falciforme/terapia , Antibacterianos/uso terapêutico , Antidrepanocíticos/uso terapêutico , Transfusão de Sangue , Transplante de Células-Tronco Hematopoéticas , Adulto , Anemia Falciforme/complicações , Anemia Falciforme/mortalidade , Anemia Falciforme/prevenção & controle , Antibacterianos/administração & dosagem , Antibioticoprofilaxia , Antidrepanocíticos/administração & dosagem , Criança , Pré-Escolar , Doença Crônica , Humanos , Hidroxiureia/uso terapêutico , Lactente , Recém-Nascido , Mortalidade/tendências , Triagem Neonatal , Penicilinas/uso terapêutico , Acidente Vascular Cerebral/etiologia , Acidente Vascular Cerebral/prevenção & controle
16.
Mass Spectrom Rev ; 35(1): 71-84, 2016 Jan-Feb.
Artigo em Inglês | MEDLINE | ID: mdl-25952022

RESUMO

Tandem mass spectrometry (MS/MS) has become a leading technology used in clinical chemistry and has shown to be particularly sensitive and specific when used in newborn screening (NBS) tests. The success of tandem mass spectrometry is due to important advances in hardware, software and clinical applications during the last 25 years. MS/MS permits a very rapid measurement of many metabolites in different biological specimens by using filter paper spots or directly on biological fluids. Its use in NBS give us the chance to identify possible treatable metabolic disorders even when asymptomatic and the benefits gained by this type of screening is now recognized worldwide. Today the use of MS/MS for second-tier tests and confirmatory testing is promising especially in the early detection of new disorders such as some lysosomal storage disorders, ADA and PNP SCIDs, X-adrenoleucodistrophy (X-ALD), Wilson disease, guanidinoacetate methyltransferase deficiency (GAMT), and Duchenne muscular dystrophy. The new challenge for the future will be reducing the false positive rate by using second-tier tests, avoiding false negative results by using new specific biomarkers and introducing new treatable disorders in NBS programs.


Assuntos
Espectrometria de Massas/métodos , Triagem Neonatal/métodos , Espectrometria de Massas em Tandem/métodos , Adrenoleucodistrofia/diagnóstico , Adrenoleucodistrofia/prevenção & controle , Anemia Falciforme/diagnóstico , Anemia Falciforme/prevenção & controle , Guanidinoacetato N-Metiltransferase/deficiência , Degeneração Hepatolenticular/diagnóstico , Degeneração Hepatolenticular/prevenção & controle , Humanos , Recém-Nascido , Transtornos do Desenvolvimento da Linguagem/diagnóstico , Transtornos do Desenvolvimento da Linguagem/prevenção & controle , Doenças por Armazenamento dos Lisossomos/diagnóstico , Doenças por Armazenamento dos Lisossomos/prevenção & controle , Transtornos dos Movimentos/congênito , Transtornos dos Movimentos/diagnóstico , Transtornos dos Movimentos/prevenção & controle , Imunodeficiência Combinada Severa/diagnóstico , Imunodeficiência Combinada Severa/prevenção & controle
19.
Soc Work Public Health ; 30(7): 592-612, 2015.
Artigo em Inglês | MEDLINE | ID: mdl-26458116

RESUMO

Sickle cell disease (SCD) is a hereditary blood disorder affecting the shape of the red blood cells that block blood vessels leading to organ damage and frequent erratic painful episodes. SCD is a global public health issue affecting millions of people throughout the world. This article explores (a) what SCD is; (b) the physical and psychosocial impacts of the disease;


Assuntos
Anemia Falciforme/prevenção & controle , Educação em Saúde , Anemia Falciforme/fisiopatologia , Anemia Falciforme/psicologia , Saúde Global , Política de Saúde , Humanos , Saúde Pública , Serviço Social
20.
Med Hypotheses ; 85(4): 424-32, 2015 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-26141635

RESUMO

Sickle Cell Disease (SCD) is a genetic condition which manifests as altered hemoglobin (Hb) protein that can aggregate under hypoxic conditions. The resultant sickled erythrocytes experience premature hemolysis, releasing an estimated 10g of free Hb (fHb) into the intravascular space. FHb participates in redox reactions creating various reactive oxygen species which rapidly and irreversibly scavenge nitric oxide, thereby attenuating its vasodilatory, antithrombotic, and anti-inflammatory properties. FHb also induces endothelial expression of adhesion molecules, triggering leukocyte margination at the vessel wall. These mechanisms participate in diverse SCD-associated clinical events including nephropathy, pulmonary hypertension, chronic leg ulceration, and ischemic events. FHb also exerts a direct reno-toxic effect contributing to albuminuria which is an early, frequent manifestation of glomerular injury. Under normal conditions, fHb is effectively scavenged by the Hb-scavenging mechanism (HSM); this involves binding to haptoglobin (Hp), uptake via the Hb-scavenging receptor (CD163) on monocytes and metabolism by heme-oxygenase-1. This culminates in increased CD163 expression and release of anti-inflammatory by-products e.g. interleukin-10 (IL-10). In SCD, the Hb-binding capacity is overwhelmed by chronic hemolysis; our previous research shows serum Hp as the depleted component. This deficiency could result in the harmful consequences of circulating fHb going unbridled. The hypothesis we explore here is that Hp infusions, in excess of fHb concentration, will allow the HSM to remain functional, and thereby achieve improved clinical outcomes, tracking albuminuria as a sentinel. Albuminuria was selected because of its high prevalence in SCD and its relative ease of diagnosis and monitoring. The hypothesis may be evaluated in four phases: Phase 1 will determine the concentration of Hp needed to trigger the HSM as measured by induction of CD163 and IL-10 and the recovery of hemopexin. Phase 2 will investigate the half-life of HSM induction by analyzing the time-course of CD163 expression and IL-10 and hemopexin serum concentration. Phase 3 will determine patient eligibility for therapy, whether as treatment or prevention. Phase 4 will test the efficacy of Hp transfusions in a randomized control trial as measured by correction of albuminuria. Angiotensin converting enzyme inhibitors (ACEi) are currently the first-line treatment for SCD nephropathy, however hyperkalemia limits its use. Hydroxyurea, which has therapeutic value in many SCD adverse events, has yielded inconsistent effects on albuminuria. We are proposing the addition of an intervention more proximal in the hemolytic cascade. Boosting the exhausted Hb-scavenging capacity via Hp replacement therapy has the potential to modify multiple downstream clinical events.


Assuntos
Anemia Falciforme/tratamento farmacológico , Anemia Falciforme/prevenção & controle , Haptoglobinas/administração & dosagem , Haptoglobinas/uso terapêutico , Albuminúria/diagnóstico , Anti-Inflamatórios , Antígenos CD/metabolismo , Antígenos de Diferenciação Mielomonocítica/metabolismo , Biomarcadores/metabolismo , Criança , Pré-Escolar , Hemoglobinas/uso terapêutico , Hemólise , Hemopexina/metabolismo , Humanos , Hidroxiureia/uso terapêutico , Infusões Intravenosas , Interleucina-10/metabolismo , Modelos Teóricos , Espécies Reativas de Oxigênio/metabolismo , Receptores de Superfície Celular/metabolismo , Resultado do Tratamento
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