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2.
PLoS One ; 14(5): e0216467, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31083675

RESUMO

Abnormal red blood cell (RBC) adhesion to endothelial αvß3 plays a crucial role in triggering vaso-occlusive episodes in sickle cell disease (SCD). It is known that epinephrine, a ß-adrenergic receptor (ß-AR) stimulator, increases the RBC surface density of active intercellular adhesion molecule-4 (ICAM-4) which binds to the endothelial αvß3. It has also been demonstrated that in human embryonic kidney 293 cells, mouse cardiomyocytes, and COS-7 cell lines, the ß-adrenergic and renin-angiotensin systems are interrelated and that there is a direct interaction and cross-regulation between ß-AR and angiotensin II type 1 receptor (AT1R). Selective blockade of AT1R reciprocally inhibits the downstream signaling of ß-ARs, similar to the inhibition observed in the presence of a ß-AR-blocker. However, it is not known if this mechanism is active in human RBCs. Here, we studied the effect of valsartan, an AT1R blocker, on the surface density of active ICAM-4 receptors in normal, sickle cell trait, and homozygous sickle RBCs. We applied single molecule force spectroscopy to detect active ICAM-4 receptors on the RBC plasma membrane with and without the presence of valsartan and epinephrine. We found that epinephrine significantly increased whereas valsartan decreased their surface density. Importantly, we found that pretreatment of RBCs with valsartan significantly impeded the activation of ICAM-4 receptors induced by epinephrine. The observed reduced expression of active ICAM-4 receptors on the RBC plasma membrane leads us to conjecture that valsartan may be used as a supporting remedy for the prevention and treatment of vaso-occlusive crisis in SCD.


Assuntos
Moléculas de Adesão Celular/metabolismo , Epinefrina/farmacologia , Membrana Eritrocítica/metabolismo , Eritrócitos Anormais/metabolismo , Traço Falciforme/metabolismo , Valsartana/farmacologia , Adolescente , Adulto , Animais , Células COS , Membrana Eritrocítica/ultraestrutura , Eritrócitos Anormais/ultraestrutura , Feminino , Células HEK293 , Humanos , Masculino , Camundongos , Microscopia de Força Atômica , Receptor Tipo 1 de Angiotensina/metabolismo , Traço Falciforme/patologia
3.
Pathol Int ; 69(4): 241-245, 2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-30843648

RESUMO

Renal medullary carcinoma (RMC) is a rare and aggressive cancer associated with the sickle cell trait. The diagnosis of RMC depends on recognition of its histologic features and immunohistochemical deficiency of INI1, but correct diagnosis is sometimes difficult, especially if a patient's information on race, past, and family medical history is unclear. At present, this is the first report on RMC in Japan.


Assuntos
Carcinoma Medular/diagnóstico por imagem , Carcinoma de Células Renais/diagnóstico por imagem , Neoplasias Renais/diagnóstico por imagem , Traço Falciforme/diagnóstico por imagem , Adulto , Carcinoma Medular/genética , Carcinoma Medular/patologia , Carcinoma de Células Renais/genética , Carcinoma de Células Renais/patologia , Diagnóstico Diferencial , Humanos , Imuno-Histoquímica , Japão , Neoplasias Renais/genética , Neoplasias Renais/patologia , Masculino , Proteína SMARCB1/metabolismo , Traço Falciforme/patologia , Tomografia Computadorizada por Raios X , Adulto Jovem
4.
Hematology Am Soc Hematol Educ Program ; 2018(1): 418-425, 2018 11 30.
Artigo em Inglês | MEDLINE | ID: mdl-30504341

RESUMO

Development of exercise guidelines for individuals with sickle cell trait (SCT) and sickle cell anemia (SCA) is hampered by the need to weigh the benefits against risks of exercise in these populations. In SCT, concern for exercise collapse associated with sickle cell trait has resulted in controversial screening of student athletes for SCT. In SCA, there exists unsubstantiated concerns that high-intensity exercise may result in pain and other complications. In both, finding the "right dose" of exercise remains a challenge for patients and their providers. Despite assumptions that factors predisposing to adverse events from high-intensity exercise overlap in SCT and SCA, the issues that frame our understanding of exercise-related harms in both are distinct. This review will compare issues that affect the risk-benefit balance of exercise in SCT and SCA through these key questions: (1) What is the evidence that high-intensity exercise is associated with harm? (2) What are the pathophysiologic mechanisms that could predispose to harm? (3) What are the preventive strategies that may reduce risk? and (4) Why do we need to consider the benefits of exercise in this debate? Addressing these knowledge gaps is essential for developing an evidence-based exercise prescription for these patient populations.


Assuntos
Exercício , Traço Falciforme/fisiopatologia , Feminino , Humanos , Masculino , Dor/patologia , Dor/fisiopatologia , Fatores de Risco , Traço Falciforme/patologia
5.
Anticancer Res ; 38(6): 3757-3761, 2018 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-29848739

RESUMO

Renal medullary carcinoma (RMC) is an aggressive high-grade renal cell carcinoma (RCC) associated almost exclusively with sickle cell trait or sickle cell disease. However, RCC with RMC features has rarely been reported in patients with no sickle cell trait or disease. Renal cell carcinoma unclassified with medullary phenotype (RCCU-MP) is a newly-coined term used by an international panel of experts to describe renal cell carcinoma showing morphologic and immunohistochemical features of renal medullary carcinoma in patients without sickle cell trait/disease. So far, only one study in the English literature has described five such cases. Here, we report a case with unique clinical and pathological features in a 76-year-old male patient without sickle cell trait. The patient had a history of colon cancer with liver and lung metastases and was found to have a new renal mass in his right kidney during the follow up. A right nephrectomy was performed and showed two separate masses (tumor 1 and tumor 2). Tumor 1 had histologic features of RMC and the tumor cells were positive for CK7, Pax8, and OCT4 and showed loss of nuclear INI1 expression. Tumor 1 was diagnosed as RCCU-MP (6.3 cm, pT3aNx, WHO/ISUP nuclear grade 3). Tumor 2 showed features of clear cell type of RCC (0.6 cm, pT1aNx, WHO/ISUP grade 2) with intact nuclear INI1 expression. Three-months post-nephrectomy, the patient developed lung metastasis of RCCU-MP. To the best of our knowledge, this was the first documented case with synchronous RCCU-MP and clear cell RCC presenting in a patient without sickle cell trait. Careful histologic assessment with a panel of immunohistochemical biomarkers was helpful to render a correct diagnosis for early aggressive treatment.


Assuntos
Carcinoma de Células Renais/patologia , Medula Renal/patologia , Neoplasias Renais/patologia , Rim/patologia , Idoso , Biomarcadores Tumorais/metabolismo , Carcinoma de Células Renais/metabolismo , Carcinoma de Células Renais/cirurgia , Humanos , Rim/metabolismo , Rim/cirurgia , Medula Renal/metabolismo , Medula Renal/cirurgia , Neoplasias Renais/metabolismo , Neoplasias Renais/cirurgia , Neoplasias Pulmonares/metabolismo , Neoplasias Pulmonares/secundário , Masculino , Nefrectomia/métodos , Fenótipo , Traço Falciforme/patologia
7.
Transl Res ; 197: 1-11, 2018 07.
Artigo em Inglês | MEDLINE | ID: mdl-29476712

RESUMO

Homozygosity for the hemoglobin (Hb) S mutation (HbSS, sickle cell anemia) results in hemoglobin polymerization under hypoxic conditions leading to vaso-occlusion and hemolysis. Sickle cell anemia affects 1:500 African Americans and is a strong risk factor for kidney disease, although the mechanisms are not well understood. Heterozygous inheritance (HbAS; sickle cell trait) affects 1:10 African Americans and is associated with an increased risk for kidney disease in some reports. Using transgenic sickle mice, we investigated the histopathologic, ultrastructural, and gene expression differences with the HbS mutation. Consistent with progressive glomerular damage, we observed progressively greater urine protein concentrations (P = 0.03), glomerular hypertrophy (P = 0.002), and glomerular cellularity (P = 0.01) in HbAA, HbAS, and HbSS mice, respectively. Ultrastructural studies demonstrated progressive podocyte foot process effacement, glomerular basement membrane thickening with reduplication, and tubular villous atrophy with the HbS mutation. Gene expression studies highlighted the differential expression of several genes involved in prostaglandin metabolism (AKR1C18), heme and iron metabolism (HbA-A2, HMOX1, SCL25A37), electrolyte balance (SLC4A1, AQP6), immunity (RSAD2, C3, UBE2O), fatty acid metabolism (FASN), hypoxia hall-mark genes (GCK, SDC3, VEGFA, ETS1, CP, BCL2), as well as genes implicated in other forms of kidney disease (PODXL, ELMO1, FRMD3, MYH9, APOA1). Pathway analysis highlighted increased gene enrichment in focal adhesion, extracellular matrix-receptor interaction, and axon guidance pathways. In summary, using transgenic sickle mice, we observed that inheritance of the HbS mutation is associated with glomerular and tubular damage and identified several candidate genes and pathways for future investigation in sickle cell trait and sickle cell anemia-related kidney disease.


Assuntos
Anemia Falciforme/patologia , Progressão da Doença , Glomérulos Renais/patologia , Túbulos Renais/patologia , Traço Falciforme/patologia , Anemia Falciforme/sangue , Anemia Falciforme/genética , Animais , Modelos Animais de Doenças , Regulação da Expressão Gênica , Hipertrofia , Glomérulos Renais/ultraestrutura , Túbulos Renais/ultraestrutura , Camundongos Transgênicos , Traço Falciforme/sangue , Traço Falciforme/genética
9.
Saudi J Kidney Dis Transpl ; 28(4): 909-911, 2017.
Artigo em Inglês | MEDLINE | ID: mdl-28748896

RESUMO

Sickled erythrocytes in patients of sickle cell trait with microscopic hematuria have rarely been reported so far. A 30-year-old female underwent delivery of a healthy full-term baby by cesarean section. However, postcesarean, she had pain in abdomen and fever, for which she was advised blood and urine examination. The hemogram suggested mild leukocytosis with neutrophilia and the urine showed red blood cells, some of which were sickled. The patient was advised hemoglobin electrophoresis which suggested sickle cell trait (Hb-AS). We conclude that sickled erythrocytes should not be ignored in a sample of urine as it may serve as an important clue to the diagnosis of sickle cell trait or disease.


Assuntos
Eritrócitos Anormais/patologia , Traço Falciforme/diagnóstico , Adulto , Eletroforese , Feminino , Hemoglobina Falciforme/análise , Humanos , Período Pós-Parto , Valor Preditivo dos Testes , Gravidez , Traço Falciforme/patologia , Traço Falciforme/urina , Urinálise , Urina/citologia
10.
J Hematol Oncol ; 10(1): 119, 2017 06 13.
Artigo em Inglês | MEDLINE | ID: mdl-28610635

RESUMO

BACKGROUND: Sickle cell disease (SCD) is a disorder of red blood cells (RBCs) expressing abnormal hemoglobin-S (HbS) due to genetic inheritance of homologous HbS gene. However, people with the sickle cell trait (SCT) carry a single allele of HbS and do not usually suffer from SCD symptoms, thus providing a rationale to treat SCD. METHODS: To validate gene therapy potential, hematopoietic stem cells were isolated from the SCD patient blood and treated with CRISPR/Cas9 approach. To precisely dissect genome-editing effects, erythroid progenitor cells were cloned from single colonies of CRISPR-treated cells and then expanded for simultaneous gene, protein, and cellular function studies. RESULTS: Genotyping and sequencing analysis revealed that the genome-edited erythroid progenitor colonies were converted to SCT genotype from SCD genotype. HPLC protein assays confirmed reinstallation of normal hemoglobin at a similar level with HbS in the cloned genome-edited erythroid progenitor cells. For cell function evaluation, in vitro RBC differentiation of the cloned erythroid progenitor cells was induced. As expected, cell sickling assays indicated function reinstitution of the genome-edited offspring SCD RBCs, which became more resistant to sickling under hypoxia condition. CONCLUSIONS: This study is an exploration of genome editing of SCD HSPCs.


Assuntos
Anemia Falciforme/genética , Anemia Falciforme/terapia , Sistemas CRISPR-Cas , Eritrócitos/metabolismo , Edição de Genes/métodos , Células-Tronco Hematopoéticas/metabolismo , Anemia Falciforme/patologia , Antígenos CD34/análise , Linhagem Celular , Células Cultivadas , Repetições Palindrômicas Curtas Agrupadas e Regularmente Espaçadas , Eritrócitos/citologia , Eritrócitos/patologia , Células Eritroides/citologia , Células Eritroides/metabolismo , Células Eritroides/patologia , Eritropoese , Terapia Genética/métodos , Genótipo , Células-Tronco Hematopoéticas/citologia , Células-Tronco Hematopoéticas/patologia , Hemoglobina Falciforme/análise , Hemoglobina Falciforme/genética , Hemoglobinas/análise , Hemoglobinas/genética , Humanos , Traço Falciforme/genética , Traço Falciforme/patologia
11.
Adv Colloid Interface Sci ; 249: 149-162, 2017 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-28515013

RESUMO

Atomic force microscopy (AFM) offers complementary imaging modes that can provide morphological and structural details of red blood cells (RBCs), and characterize interactions between specific biomolecules and RBC surface antigen. This review describes the applications of AFM in determining RBC health by the observation of cell morphology, elasticity and surface roughness. Measurement of interaction forces between plasma proteins and antibodies against RBC surface antigen using the AFM also brought new information to the immunohaematology field. With constant improvisation of the AFM in resolution and imaging time, the reaction of RBC to changes in the physico-chemistry of its environment and the presence of RBC surface antigen specific-biomolecules is achievable.


Assuntos
Doença da Artéria Coronariana/patologia , Diabetes Mellitus/patologia , Eritrócitos/ultraestrutura , Hipertensão/patologia , Malária Falciparum/patologia , Traço Falciforme/patologia , Anticorpos/metabolismo , Antígenos de Grupos Sanguíneos/análise , Proteínas Sanguíneas/imunologia , Proteínas Sanguíneas/metabolismo , Forma Celular , Doença da Artéria Coronariana/diagnóstico , Doença da Artéria Coronariana/imunologia , Diabetes Mellitus/diagnóstico , Diabetes Mellitus/imunologia , Elasticidade , Humanos , Hipertensão/diagnóstico , Hipertensão/imunologia , Malária Falciparum/diagnóstico , Malária Falciparum/imunologia , Malária Falciparum/parasitologia , Microscopia de Força Atômica , Ligação Proteica , Traço Falciforme/diagnóstico , Traço Falciforme/imunologia , Propriedades de Superfície
12.
Transl Res ; 181: 96-107, 2017 03.
Artigo em Inglês | MEDLINE | ID: mdl-27728824

RESUMO

Hemoglobin S (Hb-S) polymerization is the primary event in sickle cell disease causing irreversible damage to red blood cell (RBC) membranes over repeated polymerization cycles. A single polymerization triggered by a hypoxic environment was reported to result in reversibly (upon reoxygenation) decreased RBC deformability and increased mechanical fragility (MF). Individualized responses have not been reported, although RBC fragility can vary significantly even among healthy individuals. This study evaluates individual variability in response to a single hypoxia-induced sickling event, through changes in RBC MF. Blood was drawn from 10 normal (AA), 11 sickle cell (SS), and 7 sickle trait (AS) subjects-with Hb-S fraction, osmotic fragility, and medical history also collected. Mechanical stress was applied using a bead mill at 50-Hz oscillation for 0.5-30 minutes. MF profiles here give percent hemolysis upon successive durations of stressing. MF was measured for AA, SS, and AS cells-each equilibrated (1) with air, (2) with nitrogen in an anaerobic chamber, and (3) with air after the hypoxic event. While AA subjects exhibited significantly different changes in fragility upon hypoxia, in all cases there was recovery to close to the initial MF values on reoxygenation. For AS subjects, recovery at reoxygenation was observed only in about half of the cases. Fragility of SS cells increased in hypoxia and decreased with reoxygenation, with significantly variable magnitude of recovery. The variability of response for individual AS and SS subjects indicates that some are potentially at higher risk of irreversible hypoxia-induced membrane damage.


Assuntos
Anemia Falciforme/patologia , Eritrócitos/patologia , Traço Falciforme/patologia , Adulto , Área Sob a Curva , Hipóxia Celular , Feminino , Humanos , Masculino
13.
Sci Rep ; 6: 31698, 2016 08 22.
Artigo em Inglês | MEDLINE | ID: mdl-27546097

RESUMO

Sickle cell disease (SCD) is common across Sub-Saharan Africa. However, the investigation of SCD in this area has been significantly limited mainly due to the lack of research facilities and skilled personnel. Here, we present optical measurements of individual red blood cells from healthy individuals and individuals with SCD and sickle cell trait in Tanzania using the quantitative phase imaging technique. By employing a quantitative phase imaging unit, an existing microscope in a clinic is transformed into a powerful quantitative phase microscope providing measurements on the morphological, biochemical, and biomechanical properties of individual cells. The present approach will open up new opportunities for cost-effective investigation and diagnosis of several diseases in low resource environments.


Assuntos
Eritrócitos Anormais/patologia , Doença da Hemoglobina SC/diagnóstico , Doença da Hemoglobina SC/patologia , Traço Falciforme/diagnóstico , Traço Falciforme/patologia , Feminino , Humanos , Masculino , Microscopia de Contraste de Fase , Tanzânia
14.
Endocrinology ; 157(8): 3036-46, 2016 08.
Artigo em Inglês | MEDLINE | ID: mdl-27171384

RESUMO

Characterization of the bone phenotype of 24-week-old female transgenic sickle cell disease (SCD), sickle cell trait (SCT) revealed significant reductions in bone mineral density and bone mineral content relative to control with a further significant decreased in SCD compared with SCT. By microcomputed tomography, femur middiaphyseal cortical area was significantly reduced in SCT and SCD. Cortical thickness was significantly decreased in SCD vs control. Diaphysis structural stiffness and strength were significantly reduced in SCT and SCD. Histomorphometry showed a significant increase in osteoclast perimeter in SCD and significantly decreased bone formation in SCD and SCT compared with control with a further significant decrease in SCD compared with SCT. Collagen-I mRNA was significantly decreased in tibiae from SCT and SCD and osterix, Runx2, osteoclacin, and Dmp-1 mRNA were significantly decreased in tibiae of SCD compared with control. Serum osteocalcin was significantly decreased and ferritin was significantly increased in SCD compared with control. Igf1 mRNA and serum IGF1 were significantly decreased in SCD and SCT. IGF1 protein was decreased in bone marrow stromal cells from SCT and SCD cultured in osteogenic media. Crystal violet staining revealed fewer cells and significantly reduced alkaline phosphatase positive mineralized nodules in SCT and SCD that was rescued by IGF1 treatment. We conclude that reduced bone mass in SCD and SCT mice carries architectural consequences that are detrimental to the mechanical integrity of femoral diaphysis. Furthermore reduced IGF1 and osteoblast terminal differentiation contributed to reduced bone formation in SCT and SCD mice.


Assuntos
Anemia Falciforme/complicações , Densidade Óssea , Doenças Ósseas Metabólicas , Osso e Ossos/metabolismo , Fator de Crescimento Insulin-Like I/metabolismo , Traço Falciforme/complicações , Anemia Falciforme/sangue , Anemia Falciforme/metabolismo , Anemia Falciforme/patologia , Animais , Doenças Ósseas Metabólicas/sangue , Doenças Ósseas Metabólicas/metabolismo , Doenças Ósseas Metabólicas/patologia , Reabsorção Óssea/sangue , Reabsorção Óssea/metabolismo , Reabsorção Óssea/patologia , Células Cultivadas , Modelos Animais de Doenças , Feminino , Masculino , Camundongos , Camundongos Endogâmicos C57BL , Camundongos Transgênicos , Traço Falciforme/sangue , Traço Falciforme/metabolismo , Traço Falciforme/patologia
15.
Salvador; s.n; 2016. 61 p. ilus, tab, map.
Tese em Português | LILACS | ID: biblio-1001035

RESUMO

INTRODUÇÃO: A doença renal crônica (DRC) é uma doença grave que atinge cerca de 10% da população mundial. Devido à perda irreversível da função dos rins, os pacientes precisam do tratamento dialítico e desde 2010, no Brasil, a taxa de pacientes em diálise cresce de 3% cada ano. Cerca 93% do tratamento está financiado pelo SUS o que corresponde a 10% do orçamento do Ministério da Saúde. As principais causas de DRC no Brasil e no mundo são diabetes mellitus (DM) e hipertensão arterial sistêmica (HAS), seguido de glomerulopatias. As alterações podem ser complicadas por condições de hipóxia tecidual, as quais podem ser intensificadas pela doença falciforme. Os indivíduos com traço falciforme podem apresentar esse quadro clínico em condições extremas como um esforço físico intenso e prolongado. OBJETIVO: O objetivo deste estudo foi investigar a associação entre o traço falciforme e a progressão de DRC em Salvador-BA. MATERIAL E MÉTODOS: Foi desenvolvido um estudo de corte transversal, no qual no período de maio de 2014 até novembro de 2015...


INTRODUCTION: Chronic Kidney Disease (CKD) is a serious disease that affects about 10% of world population. It is due to irreversible loss of kidney function, so necessitating the patient’s need of dialysis treatment and since 2010, in Brazil, the rate of patients on dialysis is growing by 3% each year. About 93% of the treatment is funded by SUS which corresponds to 10% of the Health Ministry´s budget. The main causes of CKD in Brazil and in the world are diabetes mellitus and arterial hypertension, followed by glomerulopathies. The alterations can be complicated by conditions of tissue hypoxia, which can be intensified by the sickle cell disease. Individuals with sickle cell trait, although asymptomatic may present these clinical features in extreme conditions such as intense and prolonged physical activities. AIM: The aim of this study was to investigate the association between sickle cell trait and progression of CKD in patients on hemodialysis (HD) in Salvador, Bahia. MATERIAL AND METHODS: A cross-sectional cohort study was conducted from May 2014 to November 2015...


Assuntos
Humanos , Diálise Renal/métodos , Diálise Renal , Nefropatias/imunologia , Nefropatias/mortalidade , Nefropatias/patologia , Nefropatias/prevenção & controle , Traço Falciforme/diagnóstico , Traço Falciforme/patologia , Traço Falciforme/prevenção & controle
16.
Mil Med ; 180(8): e929-32, 2015 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-26226538

RESUMO

Sickle cell trait-related exertional deaths, although rare, are well-accepted in the field of forensic pathology; however, the increased risk of sudden unexpected deaths in persons with sickle cell trait undergoing strenuous physical activity may be an underappreciated acute phenomenon in the clinical realm. Herein, we report two cases of sickle cell trait-related exertional deaths of active duty military members, with a review of the literature including the pathophysiology of sickle cell trait-related deaths and current military screening guidelines.


Assuntos
Morte Súbita/etiologia , Traço Falciforme/patologia , Adulto , Autopsia , Morte Súbita/patologia , Humanos , Masculino , Militares , Traço Falciforme/complicações , Adulto Jovem
19.
Salvador; s.n; 2015. 61 p. ilus.
Tese em Português | LILACS | ID: biblio-870331

RESUMO

INTRODUÇÃO: A doença renal crônica (DRC) é uma doença grave que atinge cercade 10% da população mundial. Devido à perda irreversível da função dos rins, os pacientes precisam do tratamento dialítico e desde 2010, no Brasil, a taxa de pacientes em diálise cresce de 3% cada ano. Cerca 93% do tratamento está financiado pelo SUS o que corresponde a 10% do orçamento do Ministério da Saúde. As principais causas de DRC no Brasil e no mundo são diabetes mellitus (DM) e hipertensão arterial sistêmica (HAS), seguido de glomerulopatias. As alterações podem ser complicadas por condições de hipóxia tecidual, as quais podem ser intensificadas pela doença falciforme. Os indivíduos com traço falciforme podem apresentar esse quadro clínico em condições extremas como um esforço físico intenso e prolongado. OBJETIVO: O objetivo deste estudo foi investigar a associação entre o traço falciforme e a progressão de DRC em Salvador-BA. MATERIAL E MÉTODOS: Foi desenvolvido um estudo de corte transversal, no qual no período de maio de 2014 até novembro de 2015; foram incluídos 306 indivíduos portadores de DRC em programa de hemodiálise nos hospitais e clínicas de referência tais como, Instituto de Nefrologia e Diálise (INED), Hospital Ana Nery (HAN) e Hospital Geral Roberto Santos (HGRS) há no máximo três anos. cinco mililitros (mL) de sangue total foram coletados em cada paciente para a caracterização do perfil de hemoglobinas variantes pela técnica de cromatografia líquida de alta eficiência (HPLC). Como grupo controle, foram utilizados os resultados dos testes de triagem neonatal do APAE realizados em recém-nascidos em Salvador de 2012-2014. RESULTADOS: A frequência de HbAS foi significamente maior nos pacientes em hemodiálise (10,2%) em comparação ao grupo controle (5,05%) OR: 2,04 IC 95% (1,35–2,99). Quando comparamos os pacientes com DRC com e sem traço falciforme, não houve diferença em relação à distribuição do sexo (homens 57,6% vs 50%, respectivamente, p = 0,43). A média de idade não foi diferente entre os dois grupos (52 ± 1 anos vs 56 ± 2, p = 0,21).CONCLUSÕES: A frequência do traço falciforme é maior em pacientes portadores de DRC em programa de hemodiálise em comparação à população geral. Estudos que avaliam o impacto e fisiopatologia da doença renal em indivíduos portadores de traço falciforme podem fornecer informações importantes para desenvolvimento de estratégias de prevenção da progressão para estágio final da doença renal.


INTRODUCTION: Chronic Kidney Disease (CKD) is a serious disease that affects about 10% of world population. It is due to irreversible loss of kidney function, so necessitating the patient’s need of dialysis treatment and since 2010, in Brazil, the rate of patients on dialysis is growing by 3% each year. About 93% of the treatment is funded by SUS which corresponds to 10% of the Health Ministry´s budget. The main causes of CKD in Brazil and in the world are diabetes mellitus and arterial hypertension, followed by glomerulopathies. The alterations can be complicated by conditions of tissue hypoxia, which can be intensified by the sickle cell disease. Individuals with sickle cell trait, although asymptomatic may present these clinical features in extreme conditions such as intense and prolonged physical activities. AIM: The aim of this study was to investigate the association between sickle cell trait and progression of CKD in patients on hemodialysis (HD) in Salvador, Bahia. MATERIAL AND METHODS: A cross-sectional cohort study was conducted from May 2014 to November 2015. The subjects consisted of 394 of both sexes with chronic renal failure on hemodialysis sessions for up to three years and treated in hospitals and clinics of reference such as the Institute of Nephrology and Dialysis (INED), Ana Nery’s Hospital (HAN) and Roberto Santos General Hospital (HGRS). 5mls of whole blood was collected from each patient to characterize the hemoglobin variants profile by High Performance Liquid Chromatography (HPLC). As a control group, the results of neonatal screening tests of APAE performed on newborns in Salvador 2012-2014 were used. RESULTS: The frequency of HbAS was significantly higher in hemodialysis patients (10.2%) compared to the control group (5.05%) OR: 2.04 95% CI (1.35 to 2.99). When comparing patients with CKD with and without sickle cell trait, there was no difference in relation to the distribution of sex (men 57.6% vs 50%, respectively, p = 0.43). The mean age was not different between the two groups (52 ± 1 years vs 56 ± 2, p = 0.21)...


Assuntos
Humanos , Diálise Renal/métodos , Diálise Renal , Nefropatias/imunologia , Nefropatias/mortalidade , Nefropatias/patologia , Nefropatias/prevenção & controle , Traço Falciforme/diagnóstico , Traço Falciforme/patologia , Traço Falciforme/prevenção & controle
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