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1.
Ann Hematol ; 99(1): 41-47, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31760485

RESUMO

In the present study, the possible activation of cellular immunity in SCD patients was investigated. As immune activation parameters, neopterin concentrations and kynurenine/tryptophan ratio for tryptophan degradation in 35 pediatric patients with sickle cell disease (31 HbSS and 4 HbSß) were determined. Our results have shown that neopterin levels (both urinary and serum) are increased in pediatric patients with sickle cell disease. The increase in neopterin concentration was accompanied by significantly increased biopterin, kynurenine concentration and kynurenine/tryptophan ratio. The mechanism of immune activation and the effects of inflammatory mediators in sickle cell disease are poorly understood, especially in terms of cell-mediated immunity. Further in-vivo and in-vitro studies are required to illuminate the association between neopterin levels and neutrophil activation in sickle cell disease.


Assuntos
Anemia Falciforme/sangue , Anemia Falciforme/urina , Neopterina/sangue , Neopterina/urina , Adolescente , Anemia Falciforme/imunologia , Criança , Pré-Escolar , Feminino , Humanos , Inflamação/sangue , Inflamação/imunologia , Inflamação/urina , Masculino , Neopterina/imunologia , Ativação de Neutrófilo , Neutrófilos/imunologia , Neutrófilos/metabolismo
2.
Blood Cells Mol Dis ; 80: 102369, 2020 02.
Artigo em Inglês | MEDLINE | ID: mdl-31677454

RESUMO

People with sickle cell disease often report severe bone pain with repeated bouts of vaso-occlusive crises, but the extent of skeletal injury incurred during these painful episodes remain unclear. We sought to quantify bone degradation by comparing urinary concentrations of carboxyterminal cross-linked telopeptide of type I collagen (CTX-1), a well-described marker of bone resorption, in a prospective cohort of 52 adults with sickle cell disease enrolled in the Sickle Cell Pain Markers Study. We also questioned if changes in urinary CTX-1 concentrations correlated with changes in hemolysis and inflammatory markers measured both during and after resolution of a painful vaso-occlusive episode. Thirty-one of the 52 adults enrolled in the study had paired urine samples for CTX-1 analysis. Urinary CTX-1, corrected for urine creatinine, significantly decreased from a mean of 3.45 µg/mmol during vaso-occlusive crises to 2.62 µg/mmol at recovery (p = 0.01). Thus, increased bone loss appears to correlate with acute vaso-occlusive crises in sickle cell disease. Our finding that urinary CTX-1 can be used to probe bone degradation in sickle cell disease provides an important new tool for diagnosing and monitoring response to therapy for people with sickle cell-related bone loss.


Assuntos
Anemia Falciforme/complicações , Anemia Falciforme/urina , Biomarcadores , Reabsorção Óssea/etiologia , Reabsorção Óssea/urina , Colágeno Tipo I/urina , Dor/etiologia , Peptídeos/urina , Adulto , Anemia Falciforme/diagnóstico , Feminino , Humanos , Masculino
3.
Ann Hematol ; 98(12): 2653-2660, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31641850

RESUMO

Sickle cell disease (SCD) is a hereditary condition characterized by homozygosis of the hemoglobin S (HbS) gene. Marked morbimortality is observed due to chronic hemolysis, endothelial injury, and episodes of vaso-occlusion, which leads to multi-organ damage. Renal impairment is common and may have different presentations, such as deficiency in urinary acidification or concentration, glomerulopathies, proteinuria, and hematuria, frequently resulting in end-stage renal disease (ESRD). Novel biomarkers of renal function, such as kidney injury molecule 1 (KIM-1), and neutrophil gelatinase-associated lipocalin (NGAL) and monocyte chemoattractant protein 1 (MCP-1) are being studied in order to enable early diagnosis of kidney damage in SCD.


Assuntos
Anemia Falciforme/urina , Quimiocina CCL2/urina , Receptor Celular 1 do Vírus da Hepatite A/metabolismo , Falência Renal Crônica/urina , Rim/metabolismo , Lipocalina-2/urina , Anemia Falciforme/complicações , Biomarcadores/urina , Humanos , Falência Renal Crônica/etiologia
4.
Physiol Rep ; 7(8): e14066, 2019 04.
Artigo em Inglês | MEDLINE | ID: mdl-31033226

RESUMO

Sickle cell disease (SCD)-induced urinary concentration defect has been proposed as caused by impaired ability of the occluded vasa recta due to red blood cell sickling to serve as countercurrent exchangers and renal tubules to absorb water and solutes. However, the exact molecular mechanisms remain largely unknown. The present studies were undertaken to determine the effects of SCD on vasopressin, aquaporin2 (AQP2), urea transporter A1 (UTA1), Na-K-Cl co-transporter 2 (NKCC2), epithelial Na channels (ENaC), aquaporin1 (AQP1), nuclear factor of activated T cells 5 (NFAT5) and Src homology region-2 domain-containing phosphatase-1 (SHP-1), an important regulator of NFAT5, in the Berkeley SCD mouse kidney medulla. Under water repletion, SCD only induced a minor urinary concentration defect associated with increased urinary vasopressin level alone with the well-known effects of vasopressin: protein abundance of AQP2, UTA1 and ENaC-ß and apical targeting of AQP2 as compared with non-SCD. SCD did not significantly affect AQP1 protein level. Water restriction had no further significant effect on SCD urinary vasopressin. NFAT5 is also critical to urinary concentration. Instead, water restriction-activated NFAT5 associated with inhibition of SHP-1 in the SCD mice. Yet, water restriction only elevated urinary osmolality by 28% in these mice as opposed to 104% in non-SCD mice despite similar degree increases of protein abundance of AQP2, NKCC2 and AQP2-S256-P. Water-restriction had no significant effect on protein abundance of ENaC or AQP1 in either strain. In conclusion, under water repletion SCD, only induces a minor defect in urinary concentration because of compensation from the up-regulated vasopressin system. However, under water restriction, SCD mice struggle to concentrate urine despite activating NFAT5. SCD-induced urinary concentration defect appears to be resulted from the poor blood flow in vasa recta rather than the renal tubules' ability to absorb water and solutes.


Assuntos
Anemia Falciforme/metabolismo , Aquaporina 2/metabolismo , Canais Epiteliais de Sódio/metabolismo , Rim/metabolismo , Proteínas de Membrana Transportadoras/metabolismo , Membro 1 da Família 12 de Carreador de Soluto/metabolismo , Vasopressinas/metabolismo , Anemia Falciforme/urina , Animais , Aquaporina 1/metabolismo , Aquaporina 2/genética , Canais Epiteliais de Sódio/genética , Feminino , Masculino , Proteínas de Membrana Transportadoras/genética , Camundongos , Proteína Tirosina Fosfatase não Receptora Tipo 6/genética , Proteína Tirosina Fosfatase não Receptora Tipo 6/metabolismo , Membro 1 da Família 12 de Carreador de Soluto/genética , Fatores de Transcrição/metabolismo , Regulação para Cima , Equilíbrio Hidroeletrolítico
6.
Ann Biol Clin (Paris) ; 77(1): 79-86, 2019 02 01.
Artigo em Inglês | MEDLINE | ID: mdl-30799301

RESUMO

OBJECTIVES: The objective of this study was to assess the prevalence of albuminuria in sickle cell disease patients at the Campus University Hospital of Lome. PATIENTS AND METHOD: Albuminuria was assessed by the urinary albumin-to-creatinine ratio (UACR) in sickle cell disease individuals who attended the outpatient consultation in their steady state. RESULTS: The prevalence of albuminuria was 21% (14/67). Albuminuria was more frequent (32% vs 13%, p=0,054) and occurred earlier (6 years vs 21 years) among the 28 SS/Sß0-thalassemia sickle-cell diseases individuals than the 39 SC ones. Albuminuria was associated with high counts of leukocytes (p=0.033) and neutrophils (p=0.008). It was negatively correlated with hemoglobin level (p=0.032) and positively with LDH (p=0.002), SGOT (p=0.002), leukocytes (p=0.003), neutrophils (p< 0.001) and thrombocytes (p=0.010) counts for all sickle cell patients without statistical confirmation for each sickle cell phenotype apart from neutrophils in SS/Sß0-thalassemia. Defining albuminuria as an UACR greater than 20 mg/g had a specificity of 100% and a sensibility and 90% when the UACR was compared to the 24-hours urines albumin quantification. CONCLUSION: The assessment of albuminuria should begin at age 5 years in SS/Sß0-thalassemia sickle-cell anemia patients and from 20 years old in SC patients by the UACR.


Assuntos
Albuminúria/epidemiologia , Anemia Falciforme/epidemiologia , Adolescente , Adulto , Albuminúria/urina , Anemia Falciforme/complicações , Anemia Falciforme/urina , Criança , Pré-Escolar , Estudos Transversais , Feminino , Hospitais Universitários , Humanos , Masculino , Pessoa de Meia-Idade , Prevalência , Togo/epidemiologia , Adulto Jovem
10.
Hematology Am Soc Hematol Educ Program ; 2017(1): 406-411, 2017 12 08.
Artigo em Inglês | MEDLINE | ID: mdl-29222285

RESUMO

Chronic pain affects one-half of adults with sickle cell disease (SCD). Despite the prevalence of chronic pain, few studies have been performed to determine the best practices for this patient population. Although the pathophysiology of chronic pain in SCD may be different from other chronic pain syndromes, many of the guidelines outlined in the pain literature and elsewhere are applicable; some were consensus-adopted in the 2014 National Heart, Lung, and Blood Institute SCD Guidelines. Recommended practices, such as controlled substance agreements and monitoring of urine, may seem unnecessary or counterproductive to hematologists. After all, SCD is a severe pain disorder with a clear indication for opioids, and mistrust is already a major issue. The problem, however, is not with a particular disease but with the medicines, leading many US states to pass broad legislation in attempts to curb opioid misuse. These regulations and other key tenets of chronic pain management are not meant to deprive adults with SCD of appropriate therapies, and their implementation into hematology clinics should not affect patient-provider relationships. They simply encourage prudent prescribing practices and discourage misuse, and should be seen as an opportunity to more effectively manage our patient's pain in the safest manner possible. In line with guideline recommendations as well as newer legislation, we present five lessons learned. These lessons form the basis for our model to manage chronic pain in adults with SCD.


Assuntos
Anemia Falciforme/terapia , Dor Crônica/terapia , Manejo da Dor/métodos , Adulto , Anemia Falciforme/urina , Dor Crônica/urina , Humanos , Manejo da Dor/normas , Guias de Prática Clínica como Assunto
11.
Hematology Am Soc Hematol Educ Program ; 2017(1): 423-430, 2017 12 08.
Artigo em Inglês | MEDLINE | ID: mdl-29222288

RESUMO

Sickle cell disease (SCD) is an autosomal recessive disease in which homozygosity for a single point mutation in the gene encoding the ß-globin chain produces hemoglobin S molecules that polymerize within the erythrocyte during deoxygenation; the result is sustained hemolytic anemia and vaso-occlusive events. As patients live to adulthood, the chronic impact of sustained hemolytic anemia and episodic vaso-occlusive episodes leads to progressive end-organ complications. This scenario culminates in the development of 1 or more major cardiovascular complications of SCD for which there are no approved or consensus therapies. These complications include elevated pulmonary artery systolic pressure, pulmonary hypertension, left ventricular diastolic heart disease, dysrhythmia, sudden death, and chronic kidney disease with associated proteinuria, microalbuminuria, and hemoglobinuria. In patients with advancing age, cardiopulmonary organ dysfunction and chronic kidney injury have significant effects on morbidity and premature mortality. Over the last 15 years, a number of tests have been validated in multiple replicate cohort studies that identify patients with SCD at the highest risk of experiencing pulmonary and systemic vasculopathy and death, providing for screening strategies tied to targeted, more aggressive diagnostic and therapeutic interventions.


Assuntos
Anemia Falciforme , Doenças Cardiovasculares , Anemia Falciforme/complicações , Anemia Falciforme/terapia , Anemia Falciforme/urina , Doenças Cardiovasculares/etiologia , Doenças Cardiovasculares/terapia , Doenças Cardiovasculares/urina , Humanos , Proteinúria/etiologia , Proteinúria/terapia , Proteinúria/urina , Insuficiência Renal Crônica/etiologia , Insuficiência Renal Crônica/terapia , Insuficiência Renal Crônica/urina
13.
Pediatr Blood Cancer ; 64(12)2017 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-28612449

RESUMO

BACKGROUND: Renal damage is a progressive complication of sickle cell disease (SCD) that begins in childhood and may progress to renal failure and early mortality in 12% of adults with hemoglobin SS (HbSS) SCD. Early sickle nephropathy is characterized by hyperfiltration and microalbuminuria; therefore, urine albumin to creatinine ratio (ACR) is an effective screening tool for its detection. PROCEDURE: This study investigated the effect of hydroxyurea (HU) therapy on urine ACR levels among children with SCD. A retrospective review was conducted to identify all patients with HbSS or HbSß0 thalassemia of age 7-18 years who began HU therapy in 2011-2013; a control group of patients not on HU were matched by age and baseline hemoglobin. All urine ACR measurements ≤24 months prior to and ≥24 months after HU initiation were recorded. RESULTS: There were 63 eligible patients on HU and 13 (25%) with albuminuria prior to HU initiation. Among those with baseline albuminuria, the median ACR was 96 mg/g prior to HU, 39 mg/g at 1 year (P = 0.02), and 25 mg/g at 2 years (P = 0.03). Albuminuria normalized in 37.5% (6/16) after 1 year and 61% (8/13) after 2 years of HU therapy. Among those without albuminuria prior to HU, 13% (6/47) developed albuminuria during HU therapy. Sixteen percent (13/80) of control patients had albuminuria in the beginning of study period, which normalized in 15% (two of 13) of patients at 1-year follow up. CONCLUSION: Introduction of HU is associated with significant decreases in urine ACR in children with SCD and albuminuria.


Assuntos
Albuminúria/urina , Anemia Falciforme/tratamento farmacológico , Creatinina/urina , Hidroxiureia/uso terapêutico , Adolescente , Anemia Falciforme/urina , Criança , Feminino , Humanos , Masculino , Estudos Retrospectivos
14.
Am J Hematol ; 92(9): E520-E528, 2017 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-28589652

RESUMO

Nephropathy is a common and progressive complication of sickle cell anemia (SCA). In SCA mice, we found that hyperangiotensinemia in the absence of hypertension underlies nephropathy, and its downregulation by losartan, an angiotensin-II-receptor-1 blocker, reduced albuminuria and progression of nephropathy. Therefore, we performed a phase-2 trial of oral losartan, given for 6 months, to explore whether it reduced albuminuria in children and adults with SCA. Participants were allocated to groups defined by class of baseline urinary albumin-to-creatinine ratio (UACR): no albuminuria (NoA), microalbuminuria (MicroA), and macroalbuminuria (MacroA). The primary endpoint was a ≥25% reduction UACR from baseline. There were 32 evaluable participants (mean age 24 years; NoA = 14, MicroA = 12, MacroA = 6). The primary endpoint was met in 83% of the MacroA group (P < 0.0001) and 58% of the MicroA group (P < 0.0001). Median fold-change in UACR was -0.74 for MacroA and -0.46 for MicroA. In MacroA and MicroA, UACR classification improved in 50% but worsened in 11%. Urine osmolality and estimated glomerular filtration rate (eGFR) did not change significantly. Losartan was discontinued in three participants [leg cramps, N = 1; decline in eGFR >25% (142➝104 mL/minute/1.73 m2 ), N = 1; rise in serum creatinine >50% (0.2➝0.3 mg/dL), N = 1]. Albuminuria was associated with diastolic dysfunction and impaired functional capacity, although cardiopulmonary status was unchanged after 6 months of losartan therapy. In summary, losartan decreased urinary albumin excretion in most participants with albuminuria. Those with macroalbuminuria had the greatest benefit. This study forms the basis for a phase-3, randomized, placebo-controlled trial of losartan for the nephropathy of SCA.


Assuntos
Albuminúria , Anemia Falciforme , Losartan/administração & dosagem , Adolescente , Adulto , Fatores Etários , Albuminúria/tratamento farmacológico , Albuminúria/etiologia , Albuminúria/fisiopatologia , Albuminúria/urina , Anemia Falciforme/complicações , Anemia Falciforme/tratamento farmacológico , Anemia Falciforme/fisiopatologia , Anemia Falciforme/urina , Criança , Feminino , Taxa de Filtração Glomerular , Humanos , Masculino , Pessoa de Meia-Idade
16.
Cochrane Database Syst Rev ; (6): CD009191, 2015 Jun 04.
Artigo em Inglês | MEDLINE | ID: mdl-26041152

RESUMO

BACKGROUND: Sickle cell disease is a group of disorders characterized by deformation of erythrocytes. Renal damage is a frequent complication in sickle cell disease as a result of long-standing anemia and disturbed circulation through the renal medullary capillaries. Due to the improvement in life expectancy of people with sickle cell disease, there has been a corresponding significant increase in the incidence of renal complications. Microalbuminuria and proteinuria are noted to be a strong predictor of subsequent renal failure. There is extensive experience and evidence with angiotensin-converting enzyme (ACE) inhibitors over many years in a variety of clinical situations for patients who do not have sickle cell disease, but their effect in people with this disease is unknown. It is common practice to administer ACE inhibitors for sickle nephropathy due to their renoprotective properties; however, little is known about their effectiveness and safety in this setting. This is an update of a Cochrane Review first published in 2013. OBJECTIVES: To determine the effectiveness of ACE inhibitor administration in people with sickle cell disease for decreasing intraglomerular pressure, microalbuminuria and proteinuria and to to assess the safety of ACE inhibitors as pertains to their adverse effects. SEARCH METHODS: The authors searched the Cochrane Cystic Fibrosis and Genetic Disorders Group's Hameoglobinopathies Trials Register comprising references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings.Date of the most recent search: 03 June 2015. SELECTION CRITERIA: Randomized or quasi-randomized controlled trials of ACE inhibitors designed to reduce microalbuminuria and proteinuria in people with sickle cell disease compared to either placebo or standard treatment regimen. DATA COLLECTION AND ANALYSIS: Three authors independently applied the inclusion criteria in order to select studies for inclusion in the review. Two authors assessed the risk of bias of studies and extracted data and the third author verified these assessments. MAIN RESULTS: Five studies were identified through the searches, only one met our inclusion criteria. The included study randomized 22 participants (seven males and 15 females) having proteinuria or microalbuminuria with sickle cell disease and treated the participants for six months (median length of follow up of three months) with captopril or placebo. The overall quality of the outcomes reported was high, since most aspects that may contribute to bias were regarded to be of low risk, although allocation concealment was not reported. At six months, the study reported no significant difference in urinary albumin excretion between the captopril group and the placebo group, although the mean urinary albumin excretion in the captopril group was lower by a mean difference of -49.00 (95% confidence interval -124.10 to 26.10) compared to that of placebo. However, our analysis on the absolute change score showed significant changes between the two groups by a mean difference of -63.00 (95% confidence interval -93.78 to -32.22). At six months albumin excretion in the captopril group was noted to decrease from baseline by a mean of 45 ± 23 mg/day and the placebo group was noted to increase by 18 ± 45 mg/day. Serum creatinine and potassium levels were reported constant throughout the study. The potential for inducing hypotension should be highlighted; the study reported a decrease of 8 mmHg in systolic pressure and 5 mmHg in diastolic and mean blood pressure. AUTHORS' CONCLUSIONS: There is not enough evidence to show that the administration of ACE inhibitors is associated with a reduction of microalbuminuria and proteinuria in people with sickle cell disease, although a potential for this was seen. More long-term studies involving multiple centers and larger cohorts using a randomized-controlled design are warranted, especially among the pediatric age group. Detailed reporting of each outcome measure is necessary to allow a clear cut interpretation in a systematic review. One of the difficulties encountered in this review was the lack of detailed data reported in the included study.


Assuntos
Albuminúria/tratamento farmacológico , Anemia Falciforme/complicações , Inibidores da Enzima Conversora de Angiotensina/uso terapêutico , Captopril/uso terapêutico , Proteinúria/tratamento farmacológico , Anemia Falciforme/urina , Creatinina/sangue , Feminino , Humanos , Masculino , Potássio/sangue , Ensaios Clínicos Controlados Aleatórios como Assunto , Insuficiência Renal/prevenção & controle
17.
Hematology ; 20(7): 422-8, 2015 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-25431929

RESUMO

OBJECTIVES: As populations with sickle cell disease (SCD) live longer, it is likely that the burden of renal dysfunction will be an increasing challenge for patients. In this study, we aim to determine the prevalence of renal dysfunction and its possible predictors in persons with SCD. METHODS: Ninety-eight patients with the homozygous SCD (SS disease;55 females, 43 males; mean age 34 ± 2.3 years) in their steady state had measurements of glomerular filtration rate (GFR) using 99mTc-DTPA nuclear renal scan, serum creatinine, and urinary albumin: creatinine ratio. Other haematological and biochemical measurements and data on clinical events were completed for each individual. RESULTS: Chronic kidney disease (CKD) stages 3 and above was present in 6% of the study population, and 65.3% had albuminuria. Hyperfiltration occurred in 24.5% patients with two-thirds having albuminuria as well. Serum creatinine was an insensitive marker of renal dysfunction as started rising after measured GFR fell below 50 mls/min/1.73 m(2). Multiple regression modelling showed serum creatinine and height to be significantly associated with GFR. Serum creatinine was also significantly associated with albuminuria, and age was not a predictor in any of the models. There was no association with markers of haemolysis. CONCLUSION: We conclude that the burden of renal dysfunction is quite high in this young cohort with SS disease. Serum creatinine is a late and insensitive marker of worsening glomerular function, and screening for albuminuria could begin early in life. Longitudinal studies will continue to increase our understanding of pathophysiological mechanisms that lead to CKD in this specific population.


Assuntos
Albuminúria , Anemia Falciforme , Taxa de Filtração Glomerular , Nefropatias , Rim , Albuminúria/sangue , Albuminúria/diagnóstico por imagem , Albuminúria/etiologia , Albuminúria/fisiopatologia , Albuminúria/urina , Anemia Falciforme/sangue , Anemia Falciforme/complicações , Anemia Falciforme/diagnóstico por imagem , Anemia Falciforme/fisiopatologia , Anemia Falciforme/urina , Biomarcadores/sangue , Biomarcadores/urina , Creatinina/sangue , Creatinina/urina , Estudos Transversais , Feminino , Humanos , Jamaica , Rim/diagnóstico por imagem , Rim/metabolismo , Rim/fisiopatologia , Nefropatias/sangue , Nefropatias/diagnóstico por imagem , Nefropatias/etiologia , Nefropatias/fisiopatologia , Nefropatias/urina , Masculino , Prevalência , Radiografia
18.
Blood Cells Mol Dis ; 54(3): 297-301, 2015 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-25500149

RESUMO

BACKGROUND: The aim of this study was to evaluate the monocyte chemoatractant protein-1 (MCP-1) as a novel biomarker of renal lesion in sickle cell disease (SCD) and correlate it with oxidative stress. METHODS: This is a prospective study with SCD patients followed at a tertiary center in Brazil. Urine samples were collected to dosage of protein, MCP-1, malondialdehyde (MDA) and urinary creatinine. Patients taking hydroxyurea (SSHU) were compared to those not taking the drug (SS). Patients' data were also compared to a control group of 15 healthy blood donors. RESULTS: MCP-1 dosage was increased in SCD patients (Control: 42.12±27.6; SSHU: 168.2±90.1 and SS: 231.4±123.7 p<0.0001). SS patients presented higher levels of MCP-1 in comparison to SSHU group (SSHU: 168.2±90.10 and SS: 231.4±123.7; p=0.023). The same results were observed for MDA (Control: 2:29±1:13; SSHU: 5.60±2.39 and SS: 7.23±2.64, p<0.0001) and NO (control: 2.25±1.9; SSHU: 56.54±9.1 and SS: 39.1±9.02, p<0.0001). A positive correlation was obtained between MCP-1 and MDA (r=0.34, p=0.01); albuminuria (r=0.5, p=0.03); NO (r=0.39, p=0.005). CONCLUSION: The outcomes of the study suggest that MCP-1 is a predictive biomarker of renal lesion that can also reflect damage caused by oxidative stress present in SCD.


Assuntos
Anemia Falciforme/complicações , Anemia Falciforme/urina , Quimiocina CCL2/urina , Nefropatias/etiologia , Estresse Oxidativo , Adulto , Albuminúria/etiologia , Albuminúria/metabolismo , Anemia Falciforme/epidemiologia , Anemia Falciforme/metabolismo , Brasil/epidemiologia , Feminino , Humanos , Rim/metabolismo , Rim/patologia , Nefropatias/metabolismo , Nefropatias/urina , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Adulto Jovem
19.
Pediatr Hematol Oncol ; 32(4): 250-7, 2015 May.
Artigo em Inglês | MEDLINE | ID: mdl-23987825

RESUMO

OBJECTIVES: Renal involvement is common in sickle cell disease (SCD). Early demonstration of renal injury and commencement of appropriate treatment will increase survival and quality of life in these patients. We investigated renal manifestations in our pediatric and adult SCD patients and evaluated the role of cystatin C, Beta2 microglobulin (B2M), retinol-binding protein (RBP), N-acetyl-beta-D-glucosaminidase (NAG), and endothelin-1 (ET-1) to indicate renal damage. METHODS: The study involved 45 pediatric and 10 adult patients with SCD and 20 healthy children and 10 healthy adults as a control. All the patients were questioned for possible renal manifestations. 24-hour urine samples were collected and glomerular filtration rates (GFRs) were calculated by using creatinine (GFR(creatinine)), Schwartz formula (GFR(Schwartz)), and cystatin C (GFR(cystatin C)). Blood and urine samples were collected and serum cystatin C, urine B2M, RBP, NAG, and ET-1 levels were measured. RESULTS: Nocturnal enuresis and proteinuria were the most common renal manifestations in SCD patients. When the groups were compared in terms of GFR, GFR(creatinine) and GFR(Schwartz) levels were higher in group 1 and 2 patients than in control 1 and 2 patients (P < .05). Cystatin C, B2M, RBP, NAG, and ET-1 values were normal in both the patient and the control groups. However, B2M/creatinine levels were higher than 160 µg/mg creatinine levels in 10 patients. CONCLUSIONS: Serum cystatin C, urine NAG, RBP, and ET-1 levels were found to be insufficient for the evaluation of SCD nephropathy. Increased B2M/creatinie levels can be valuable in estimating possible glomerular and tubular damage in SCD.


Assuntos
Acetilglucosaminidase , Anemia Falciforme , Cistatina C , Endotelina-1 , Nefropatias , Proteínas Celulares de Ligação ao Retinol , Microglobulina beta-2 , Acetilglucosaminidase/sangue , Acetilglucosaminidase/urina , Adolescente , Adulto , Anemia Falciforme/sangue , Anemia Falciforme/complicações , Anemia Falciforme/urina , Criança , Pré-Escolar , Creatinina/sangue , Cistatina C/sangue , Cistatina C/urina , Endotelina-1/sangue , Endotelina-1/urina , Feminino , Humanos , Lactente , Nefropatias/sangue , Nefropatias/complicações , Nefropatias/urina , Masculino , Pessoa de Meia-Idade , Proteínas Celulares de Ligação ao Retinol/sangue , Proteínas Celulares de Ligação ao Retinol/urina , Microglobulina beta-2/sangue , Microglobulina beta-2/urina
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