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1.
Artigo em Inglês | MEDLINE | ID: mdl-38327222

RESUMO

BACKGROUND/AIMS: Two earthquakes on February 6th, 2023 destroyed ten cities in Türkiye. We report our experience with pediatric victims during these catastrophes, with a focus on crush syndrome related-acute kidney injury (Crush-AKI) and death. METHOD: A web-based software was prepared. Patient demographics, time under rubble (TUR), admission laboratory data, dialysis, and kidney and overall outcomes were asked. RESULTS: 903 injured children (median age: 11.62 years) were evaluated. Mean TUR was 13 h (Interquartile range-IQR: 32.5), max 240 h). 31 of 32 patients with a TUR of >120 h survived. The patient who rescued after ten days survived.Two-thirds of the patients were given 50 mEq/L sodium-bicarbonate in 0.45% sodium-chloride solution on admission day. 58% of patients were given intravenous fluid (IVF) at a volume of 2000-3000 mL/m2 body surface area (BSA), 40% of 3000-4000 mL/m2 BSA, and only 2% of >4000 mL/m2 BSA. 425 patients had surgeries, 48 suffered from major bleeding. Amputations were recorded in 96 patients. Eighty-two and 66 patients required ventilator and inotropic support, respectively.Crush-AKI developed in 314 patients (36% of all patients). 189 patients were dialyzed. Age > 15 years, creatine phosphokinase (CK)≥20 950 U/L, TUR≥10 h, and the first-day IVF volume < 3000-4000 mL/m2 BSA were associated with Crush-AKI development. 22 deaths were recorded, 20 of 22 occurred in patients with Crush-AKI and within the first 4 days of admission. All patients admitted after 7 days survived. CONCLUSIONS: This is the most extensive pediatric kidney disaster data after an earthquake. Serum CK level was significantly associated with Crush-AKI at the levels of >20 950 U/L, but not with death. Adolescent age and initial IVF of less than 3000-4000 mL/m2 BSA were also asscoiated with Crush-AKI. Given that mildly injured victims can survive longer periods in the disaster field, we suggest uninterrupted rescue activity for at least 10 days.

2.
Clin Exp Rheumatol ; 42(1): 194-201, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-37497697

RESUMO

OBJECTIVES: Biological drugs are one of the most effective treatment methods for systemic juvenile idiopathic arthritis (SJIA) and can significantly prevent morbidity and mortality. This study aimed to evaluate the efficacy and safety of biologics in patients with SJIA and provide real-life data that might help improve the outcomes. METHODS: TURSIS was a retrospective multicentre study carried out in patients with SJIA for whom a biological treatment had been initiated between 1st March 2013 and 30th December 2018. Data include patients' characteristics, laboratory-clinical results, outcomes, and safety-related variables. The 24-month follow-up data of the patients and the efficacy and safety of biological drugs were evaluated. RESULTS: 147 patients were enrolled. The clinical course of the disease was as follows; it was monocyclic in 38.1%, polycyclic in 49%, and persistent in 12.9% of patients. First-choice biologics were interleukin (IL)-1 blockers in the majority of patients (56.5%), followed by the anti-IL-6 (25.2%) and anti-TNF-alpha drugs (18.4%). Anakinra was the most preferred biologic agent in patients with macrophage activation syndrome (MAS), and tocilizumab was used more frequently in patients with persistent type (p=0.000 and p=0.003). The most frequent switch rate was seen in patients receiving anakinra (n=40/68, 58.8%), and it was most frequently switched to canakinumab (n=32/40, 80%). Better physician's global assessment scores were achieved in patients treated with anakinra in Month 3, compared to other treatments (p=0.04). CONCLUSIONS: The results of our study support the efficacy of biological drugs in particular anti-IL-1 and anti-IL-6 drugs, in the treatment of SJIA. These treatments resulted in improvement in activity of disease and provide a considerable decrease in the frequency of MAS.


Assuntos
Artrite Juvenil , Produtos Biológicos , Síndrome de Ativação Macrofágica , Humanos , Artrite Juvenil/diagnóstico , Artrite Juvenil/tratamento farmacológico , Proteína Antagonista do Receptor de Interleucina 1/efeitos adversos , Turquia , Inibidores do Fator de Necrose Tumoral/uso terapêutico , Interleucina-1 , Produtos Biológicos/efeitos adversos , Síndrome de Ativação Macrofágica/induzido quimicamente
3.
Pediatr Nephrol ; 39(5): 1435-1446, 2024 May.
Artigo em Inglês | MEDLINE | ID: mdl-38041748

RESUMO

BACKGROUND: C3 glomerulopathy (C3G) is a complement-mediated disease. Although genetic studies are not required for diagnosis, they are valuable for treatment planning and prognosis prediction. The aim of this study is to investigate the clinical phenotypes, kidney survival, and response to mycophenolate mofetil (MMF) treatment in pediatric C3G patients with and without mutations in complement-related genes. METHODS: Sixty pediatric C3G patients were included, divided into two groups based on complement-related gene mutations. Demographic and clinical-pathological findings, treatment modalities, and outcome data were compared, and Kaplan-Meier analysis was performed for kidney survival. RESULTS: Out of the 60 patients, 17 had mutations. The most common mutation was in the CFH gene (47%). The mean age at diagnosis was higher in the group with mutation (12.9 ± 3.6 vs. 11.2 ± 4.1 years, p = 0.039). While the patients without mutation most frequently presented with nephritic syndrome (44.2%), the mutation group was most likely to have asymptomatic urinary abnormalities (47.1%, p = 0.043). Serum parameters and histopathological characteristics were similar, but hypoalbuminemia was more common in patients without mutation. During 45-month follow-up,10 patients progressed to chronic kidney disease stage 5 (CKD5), with 4 having genetic mutation. The time to develop CKD5 was longer in the mutation group but not significant. MMF treatment had no effect on progression in either group. CONCLUSIONS: This study is the largest pediatric C3G study examining the relationship between genotype and phenotype. We showed that the mutation group often presented with asymptomatic urinary abnormalities, was diagnosed relatively late but was not different from the without mutation group in terms of MMF treatment response and kidney survival.


Assuntos
Glomerulonefrite Membranoproliferativa , Glomerulonefrite , Nefropatias , Falência Renal Crônica , Humanos , Criança , Complemento C3/genética , Ácido Micofenólico/uso terapêutico , Glomerulonefrite Membranoproliferativa/patologia , Mutação , Glomerulonefrite/tratamento farmacológico , Nefropatias/tratamento farmacológico
4.
Lupus ; 30(12): 1983-1990, 2021 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-34459313

RESUMO

Introduction: Systemic lupus erythematosus (SLE) may present with features of several systems, including hematological manifestations. In this study, we aimed to evaluate the characteristics of hematological involvement and assess possible associations and correlations in pediatric SLE patients. Method: This is a retrospective multi-center study. The medical records of pediatric SLE patients followed between January 2000 and June 2020 were analyzed. All children fulfilled the criteria of the Systemic Lupus International Collaborating Clinics. Results: The study included 215 children with SLE, 118 of whom had hematological manifestations. Concomitant renal involvement and low C3 levels were significantly more frequent in patients with hematological involvement (p = 0.04, p = 0.008, respectively). Also, anti-cardiolipin, anti-beta-2-glycoprotein I (anti-ß2 GP1), and anti-Sm antibody positivity, and the presence of lupus anticoagulant were more common in the group with hematological findings (p = 0.001 for anti-cardiolipin antibody positivity and p < 0.001 for the positivity of anti-ß2 GP1 antibody, anti-Sm antibody, and lupus anticoagulant). The most common hematologic abnormality was anemia (n = 88, 74.5%), with autoimmune hemolytic anemia constituting the majority (n = 40). Corticosteroids followed by IVIG were the mainstay of treatment. In patients resistant to corticosteroid and IVIG treatments, the most preferred drug was rituximab. Low levels of C3, high SLEDAI score, high incidence of renal involvement, and positive antiphospholipid antibodies were associated with hematological involvement in the univariate analysis. The presence of antiphospholipid antibodies and high SLEDAI score were independently associated with hematological involvement in multivariate analysis (OR: 4.021; 95% CI: 2.041-7.921; p < 0.001 and OR: 1.136; 95% CI: 1.065-1.212; p < 0.001). Conclusion: Hematological abnormalities are frequently encountered in pediatric SLE. Positive antiphospholipid antibodies and high SLEDAI scores were associated with hematological involvement.


Assuntos
Anemia Hemolítica Autoimune/epidemiologia , Lúpus Eritematoso Sistêmico/complicações , Administração Oral , Adolescente , Corticosteroides/administração & dosagem , Corticosteroides/uso terapêutico , Anemia Hemolítica Autoimune/tratamento farmacológico , Anticorpos Antifosfolipídeos , Criança , Feminino , Humanos , Hidroxicloroquina/uso terapêutico , Imunoglobulinas Intravenosas , Inibidor de Coagulação do Lúpus , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Lúpus Eritematoso Sistêmico/epidemiologia , Masculino , Estudos Retrospectivos
5.
J Pediatr Hematol Oncol ; 43(3): e452-e456, 2021 04 01.
Artigo em Inglês | MEDLINE | ID: mdl-32205782

RESUMO

Various autoimmune diseases may be associated with primary immune deficiencies. We reported a case with a loss-of-function mutation in DNASE1L3, a gene described previously in families with systemic lupus erythematosus. In addition, the patient showed a novel homozygous missense variant in DOCK8, a gene known to be responsible for the hyper-IgE recurrent infection syndrome (HIES). A 3-year-old girl born to consanguine parents presented with chronic urticarial rash, hemolytic anemia, pulmonary hemorrhage, and hypovolemic shock findings. She had a low hemoglobin level, a positive direct antiglobulin test, antinuclear antibody and anti-double stranded DNA, low C3 and C4, third-degree tricuspid regurgitation, and severe enlargement of the right ventricle on echocardiography, suggesting pulmonary embolism. Despite treatment with intravenous immunoglobulin, pulse metilprednisolone, rituximab, and supportive treatment for shock, the patient died on the seventh day. Whole-exome sequencing indicated a homozygous stop variant c.537G>A (p. Trp179Ter) in DNASE1L3. In addition, a possibly pathogenic homozygous missense variant in the HIES gene DOCK8 was detected. The occurrence of potentially clinically relevant, genetic variants in several genes posed various challenges with respect to diagnosis, treatment, and prognosis.


Assuntos
Anemia Hemolítica Autoimune/genética , Endodesoxirribonucleases/genética , Fatores de Troca do Nucleotídeo Guanina/genética , Anemia Hemolítica Autoimune/patologia , Pré-Escolar , Feminino , Humanos , Mutação com Perda de Função , Mutação de Sentido Incorreto
6.
Mod Rheumatol ; 31(3): 718-724, 2021 May.
Artigo em Inglês | MEDLINE | ID: mdl-32815445

RESUMO

OBJECTIVES: Familial Mediterranean fever (FMF) may present with various concomitant diseases. This study aims to evaluate the clinical characteristics of patients with FMF with Juvenile Spondyloarthropathy (jSpA). METHOD: Thirty-two patients diagnosed with FMF/jSpA, sixty-four with FMF, and fifty-four with jSpA were included in this retrospective study. Three patient groups were compared in terms of clinical and laboratory features. RESULTS: The mean ages of patients in the FMF/jSpA, FMF and jSpA groups were 15.75(11.50-19.83), 15,41(6.83-21.50), and 16(9-22) years, respectively. Chronic arthritis (OR: 0.11, p = .049), erythrocyte sedimentation rate values (OR:1.07, p = .011), and C-reactive protein values (OR:1,08, p: .039) of the patients in remission period were found higher, the international severity scores for FMF (ISSF) before and after colchicine treatment (OR: 1.16, p: .021, OR: 2,21, p: .012) were higher in the FMF/jSpA group compared to FMF. Plantar fasciitis was more common and HLA-B27 positivity rate was lower in the FMF/jSpA group (OR:0.08, p = .024), (OR:4.71, p = .002) compared to jSpA. FMF/jSpA patients were divided as previous diagnosed FMF and jSpA.The diagnosis of jSpA was at a younger age(p = .002), Juvenile arthritis damage index-articular(p = 0.022) and extraarticular(p = .026), and the rate of biologic drug usage(p = .015) were higher in the previous jSpA group. The number of FMF attacks before colchicine was lower in the previous jSpA group(p = .02). CONCLUSION: Our findings suggest that both classical FMF and jSpA findings were lower in patients with FMF/jSpA. Patients who were diagnosed with jSpA at an early age and who had enthesitis and plantar fasciitis should also be evaluated in terms of FMF.


Assuntos
Artrite Juvenil/complicações , Febre Familiar do Mediterrâneo/complicações , Espondilite Anquilosante/complicações , Adolescente , Adulto , Artrite Juvenil/diagnóstico , Sedimentação Sanguínea , Proteína C-Reativa , Febre Familiar do Mediterrâneo/diagnóstico , Feminino , Humanos , Masculino , Espondilite Anquilosante/diagnóstico
7.
Fetal Pediatr Pathol ; 39(5): 381-389, 2020 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31490094

RESUMO

Background: A new semiquantitative classification (SQC) for pediatric Henoch-Schönlein nephritis (HSN) was defined recently. The outcomes of pediatric HSN patients are reevaluated according to the new classification. Methods: Primary kidney biopsies from 80 HSN patients were scored using the new SQC. The International Study of Kidney Disease in Children (ISKDC) and SQC classifications were compared in terms of the patient outcomes. Outcomes were defined as: Outcome A (n = 44) patients with no sign of renal disease, Outcome B (n = 32) patients with minor urinary abnormalities, and Outcome C (n = 4) patients with active renal disease. Results: The patients with outcome C had significantly higher biopsy scores and chronicity indices than patients in group A. There was no significant difference in areas under the curve between total biopsy SQC scores and ISKDC findings. Conclusions: Our results suggest that the modified SQC is not more sensitive than ISKDC classification for predicting the outcome in HSN cases.


Assuntos
Vasculite por IgA , Nefrite , Vasculite , Biópsia , Criança , Humanos
9.
Biomarkers ; 23(6): 558-562, 2018 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-29611726

RESUMO

OBJECTIVE: Endothelial microparticles (EMPs) are considered as markers of endothelial dysfunction. In this study, we aimed to examine whether there is endothelial dysfunction in children with familial Mediterranean fever (FMF), hypothesizing that endothelial dysfunction would be present especially with acute-phase response in the active period of the disease. METHODS: This cross-sectional study included 65 FMF patients (41 attack free, 24 attack period) and 35 healthy controls. Circulating EMPs, serum amyloid A (SAA), and other inflammation markers were measured in all groups. Circulating EMPs were measured using flow cytometry. Study groups were compared for circulating EMP and inflammatory markers. The relationship between EMPs and the activation of the disease was evaluated. RESULTS: The levels of CD144+ and CD146+ EMPs in the FMF attack period group were significantly higher than those of the control group (p < 0.05). The levels of inflammation markers in the attack period group were significantly higher than those of the control and attack-free groups (p < 0.05). In the FMF attack group, the CD144+ and CD146+ EMP were significantly correlated with CRP. CONCLUSIONS: Our results suggest that endothelial damage is present especially in the active period of the disease in children with FMF. The endothelial dysfunction becomes an overt parallel with inflammation.


Assuntos
Biomarcadores/sangue , Micropartículas Derivadas de Células/metabolismo , Endotélio Vascular/metabolismo , Febre Familiar do Mediterrâneo/sangue , Adolescente , Antígenos CD/sangue , Proteína C-Reativa/análise , Antígeno CD146/sangue , Caderinas/sangue , Criança , Estudos Transversais , Endotélio Vascular/fisiopatologia , Febre Familiar do Mediterrâneo/fisiopatologia , Feminino , Humanos , Inflamação/sangue , Inflamação/fisiopatologia , Masculino , Proteína Amiloide A Sérica/análise
10.
Ren Fail ; 39(1): 146-152, 2017 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-27846788

RESUMO

AIM: we aimed to establish reference values for urinary oxalate to creatinine ratios in healthy children aged 6-15 years and to investigate the relationship between their nutritional habits and oxalate excretion. MATERIALS AND METHODS: Random urine specimens from 953 healthy children aged 6-15 years were obtained and analyzed for oxalate and creatinine. Additionally, a 24-h dietary recall form was prepared and given to them. The ingredient composition of the diet was calculated. The children were divided into three groups according to age: Group I (69 years, n = 353), Group II (10-12 years, n = 335), and Group III (13-15 years, n = 265). RESULTS: The 95th percentile of the oxalate to creatinine ratio for subjects aged 6-9, 10-12, and 13-15 years were 0.048, 0.042, and 0.042 mg/mg, respectively. The oxalate to creatinine ratio was significantly higher in Group 1 than in Group 2 and Group 3. Urinary oxalate excretion was positively correlated with increased protein intake and negatively correlated with age. A significant positive correlation was determined between urinary oxalate excretion and the proline, serine, protein, and glycine content of diet. Dietary proline intake showed a positive correlation with the urine oxalate to creatinine ratio and was found to be an independent predictor for urinary oxalate. CONCLUSIONS: These data lend support to the idea that every country should have its own normal reference values to determine the underlying metabolic risk factor for kidney stone disease since regional variation in the dietary intake of proteins and other nutrients can affect normal urinary excretion of oxalate.


Assuntos
Creatinina/urina , Dieta , Oxalatos/urina , Adolescente , Criança , Feminino , Voluntários Saudáveis , Humanos , Masculino , Valores de Referência , Análise de Regressão , Fatores de Risco , Turquia
12.
Clin Exp Rheumatol ; 33(6 Suppl 94): S156-62, 2015.
Artigo em Inglês | MEDLINE | ID: mdl-26393894

RESUMO

OBJECTIVES: To develop and assess the validity and reliability of an adherence scale concerning medical treatment in paediatric FMF patients. METHODS: The Medication Adherence Scale in FMF Patients (MASIF) is a 18-item questionnaire that evaluates adherence to medication in four domains. Validation of the instrument was accomplished in paediatric FMF patients (aged 2-18 years) under medication at least for 6 months. The first step was to build up the scale through qualitative approach (with interviews using semi-structured questions). Validation analyses included assessment of feasibility, face and content validity; construct validity, internal consistency and test-retest reliability. RESULTS: One hundred and fifty patients with FMF were enrolled in the study. The mean age of the patients was 11.11±4.02 years and 48.7% of them were male. The MASIF was found to be feasible and valid for both face and content. It correlated with the Morisky Medication Adherence Scale as a gold standard thereby demonstrating good construct validity (r=0.515, p<0.001). Assessment of content validity identified four subscales. The internal consistency, Cronbach's alpha was 0.728. There was a positive and significant correlation between test and retest scores (r=0.843; p<0.001). Also, a significant correlation between parents' and children's reports (r=0.781, p<0.001). CONCLUSIONS: Based on these results, the use of this scale to assess and follow up the adherence to treatment in paediatric FMF patients under medical treatment is recommended.


Assuntos
Febre Familiar do Mediterrâneo/tratamento farmacológico , Imunossupressores/uso terapêutico , Adesão à Medicação , Inquéritos e Questionários , Adolescente , Fatores Etários , Criança , Pré-Escolar , Febre Familiar do Mediterrâneo/diagnóstico , Febre Familiar do Mediterrâneo/epidemiologia , Estudos de Viabilidade , Feminino , Humanos , Entrevistas como Assunto , Masculino , Pesquisa Qualitativa , Reprodutibilidade dos Testes , Resultado do Tratamento , Turquia/epidemiologia
13.
Eur J Pediatr ; 173(9): 1157-60, 2014 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-24687251

RESUMO

UNLABELLED: The aim of this paper is to compare the effect of nebulized magnesium sulfate to nebulized salbutamol and salbutamol/magnesium sulfate on successful discharge from the emergency department. A total of 56 infants were included in this double-blinded, prospective study. Infants were grouped according to the nebulized treatment they received: group 1-salbutamol/normal saline, group 2-magnesium sulfate and normal saline, and group 3-salbutamol plus magnesium sulfate. Heart beat, bronchiolitis, clinical severity scores (CSS), and oxygen saturation of the patients were determined before and after nebulization (0, 1, 4 h). The patients were monitored for adverse reactions. Post-treatment mean CSS results were significantly lower than pre-treatment scores in all groups at 4 h with no significant difference within groups. CSS scores were lower in the salbutamol/magnesium sulfate group when compared with the magnesium sulfate and salbutamol groups (3.4 (2.4-4.3), 4.7 (3.8-5.7), 4.0 (3.2-4.3)). CSS were significantly lower than those from the magnesium sulfate group. CONCLUSION: Nebulized magnesium sulfate plus salbutamol may have additive effects for improving the short-term CSS.


Assuntos
Albuterol/administração & dosagem , Bronquiolite/tratamento farmacológico , Broncodilatadores/administração & dosagem , Sulfato de Magnésio/administração & dosagem , Nebulizadores e Vaporizadores , Bronquiolite/diagnóstico , Bronquiolite/fisiopatologia , Pré-Escolar , Método Duplo-Cego , Quimioterapia Combinada , Feminino , Frequência Cardíaca/efeitos dos fármacos , Hospitais Universitários , Humanos , Lactente , Unidades de Terapia Intensiva Pediátrica , Masculino , Veículos Farmacêuticos , Estudos Prospectivos , Solução Salina Hipertônica/administração & dosagem , Índice de Gravidade de Doença , Resultado do Tratamento
14.
Cardiol Young ; 24(3): 510-4, 2014 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-23694855

RESUMO

INTRODUCTION: A variety of patch materials have been used in the repair of intracardiac defects. We evaluated the short- and mid-term clinical and echocardiography results of glutaraldehyde-preserved bovine pericardium patches used to repair intracardiac defects in our clinic. METHODS AND RESULTS: This study examines the short- (up to 30 days post-operatively) and mid-term (up to 24 months post-operatively) results of 533 patients with intracardiac defects who underwent surgical correction with glutaraldehyde-preserved bovine pericardium patches between 2004 and 2010 at a university clinic. Short- and mid-term post-operative echocardiographic studies showed no evidence of calcification, thrombus, or aneurysmal dilatation on the patch. Vegetation developed in two (0.37%) of the 533 patients. CONCLUSION: These results that have been obtained from a group of large number of patients imply that the glutaraldehyde-preserved bovine pericardium patches may be preferable in the closure of cardiac defects because of their low complication rates and ease of use.


Assuntos
Bioprótese , Cardiopatias Congênitas/cirurgia , Xenoenxertos , Pericárdio/transplante , Animais , Bovinos , Criança , Pré-Escolar , Feminino , Glutaral , Humanos , Lactente , Masculino , Soluções para Preservação de Órgãos , Estudos Retrospectivos , Fatores de Tempo , Resultado do Tratamento
15.
Ren Fail ; 36(9): 1428-35, 2014 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-25110139

RESUMO

AIM: The purpose of this study was to compare the possible healing effects of intraperitoneal (IP) and intravenous (IV) mesenchymal stem cell (MSC) transplantation on ultrafiltration failure (UFF) in a chronic rat model of peritoneal dialysis (PD). METHODS: Rats were initially divided into two groups. The UFF-group received once-daily IP injections of 20 mL of 3.86% glucose PD solution for six weeks to stimulate the development of UFF, and a control group received no injections. The UFF group was sub-divided into four groups: an UFF-C group, a MSC-IP group, a MSC-IV group and a placebo (P) group. Peritoneal equilibration tests (PETs) and peritoneal biopsies were performed in the control and UFF-C groups. MSCs were administered by IP injection in the MSC-IP group and by IV injection in the MSC-IV group. The P group received IP injection of placebo. PETs and peritoneal biopsies were performed in the MSC-IP, MSC-IV and P groups at the three weeks after receiving MSCs or placebo. RESULTS: When compared with the control group, ultrafiltration capacity significantly decreased, and the submesothelial thickness increased in the UFF-C and P group, but there were no differences between the control and MSC-IP and MSC-IV groups. The rate of glucose transport was high in the UFF-C and P group compared with the control group, and D/PCr rates in the UFF-C and P group were lower than in the control group. However, D/D0glucose was higher and D/PCr was lower in the MSC-IP group than in the UFF-C and P groups, but D/D0glucose rate of MSC-IV group similar to UFF-C and P groups and there was no difference between MSC-IV group and the other groups in terms of D/PCr rates. The MSC-IP, MSC-IV and P groups had significantly decreased tumor necrosis factor α concentrations compared with the UFF-C group. MSC-IP group had lower levels of TGF-ß1 compared with the P group; MSC-IP group had also lower levels of interleukin-6 compared with UFF-C group. CONCLUSION: The UFF group had a high permeability UFF. These results showed that IV and IP MSC transplantation exerted positive effects on UFF in a chronic rat model of PD. However, healing effect of small solute transport in MSC-IP group was better than MSC-IV group. IP MSC transplantation may be more effective than IV MSC transplantation for the renewal of the peritoneum in chronic PD patients with UFF.


Assuntos
Administração Intravenosa , Glucose/metabolismo , Injeções Intraperitoneais , Transplante de Células-Tronco Mesenquimais/métodos , Diálise Peritoneal/métodos , Peritônio/metabolismo , Ultrafiltração/métodos , Animais , Transporte Biológico , Modelos Animais de Doenças , Masculino , Microscopia de Fluorescência , Ratos , Ratos Wistar , Falha de Tratamento
16.
Ren Fail ; 36(4): 562-6, 2014 May.
Artigo em Inglês | MEDLINE | ID: mdl-24456204

RESUMO

BACKGROUND: Original peritoneal equilibration test (PET) is an implementation that requires hard work for peritoneal dialysis (PD) staff. Therefore, several authors have attempted to validate short and fast PET protocols, with controversial results. The aim of this study was to evaluate the concordance between the mini-PET and original PET in children. METHODS: In 26 stable continuous ambulatory PD patients, we performed an original PET with 2.27% (4 h) and a mini-PET with 3.86% glucose PD fluid (1 h) and compared ultrafiltration (UF) and small solute transports obtained with the two methods. RESULTS: Twenty-six children, 14 males, mean age 11.4 ± 5.6 (range 2.5-19 years), were included. Meantime on PD at time of enrollment was 35.2 ± 24.5 months (range 6-84 months). Based on the 4-h creatinine D/P data, the number of the patients within each transport category was as follow: high, 5; average, 18; low, 3. Kappa test showed a significant concordance between original PET and mini-PET (k=0.610). Based on the 4-h glucose D/D0 data, the number of the patients within each transport category was as follow: high, 5; average, 17; low, 4. Kappa test showed a moderate agreement between original PET and mini-PET (0.514, p=0.000). When Pearson correlation analysis between original PET and mini-PET was performed, there were significant positive correlations between original 2.27% PET and mini-PET (r=0.720, p=0.000, r=0.638, p=0.000, respectively). When comparing the numeric results of mini-PET and 4 h of original PET for D/Creatinine, by simple regression analysis, we found statistically significant correlation among PETs. CONCLUSIONS: In this study, we showed concordance between the mini-PET and original PET. The 3.86% mini-PET is simple and fast methods to assess free water transport. This also gives information about total UF and small solute transports and it is in good agreement with the original PET.


Assuntos
Soluções para Diálise/farmacocinética , Diálise Peritoneal , Peritônio/metabolismo , Adolescente , Transporte Biológico , Criança , Pré-Escolar , Protocolos Clínicos , Feminino , Humanos , Masculino , Permeabilidade , Estudos Prospectivos , Fatores de Tempo , Ultrafiltração , Adulto Jovem
17.
Nephrol Dial Transplant ; 28(10): 2493-501, 2013 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-23880789

RESUMO

BACKGROUND: The purpose of this study was to investigate possible healing effects of intraperitoneal (IP) mesenchymal stem cell (MSC) transplantation on ultrafiltration failure (UFF) in a chronic rat model of peritoneal dialysis (PD). METHODS: Rats were initially divided into two groups. The APUF group received once-daily IP injections of 20 mL of 3.86% glucose PD solution for 6 weeks to stimulate the development of UFF and a control group received noinjections. The PUF group was sub-divided into three groups: a PUF-C group, an MSC group and a Placebo (P) group. Peritoneal equilibration tests (PETs) and peritoneal biopsies were performed in the control and PUF-C groups. MSCs were administered by IP injection in the MSC group and the PUF-C and P groups received IP injection of placebo. PETs and peritoneal biopsies were performed in the MSC and P groups at the first [P-1 (and MSC-1 groups] and second [P-2 and MSC-2 groups] week after receiving MSCs or placebo. RESULTS: When compared with the control group, ultrafiltration capacity significantly decreased and the submesothelial thickness increased in the PUF-C and P groups (P-1, P-2) (P < 0.05), but there were no differences between the control and MSC groups (MSC-1, MSC-2). The rate of glucose transport was high in the PUF-C and P-2 groups compared with the control group, and D/PCr rates in the PUF-C and P-2 groups were lower than in the control group (P < 0.05). However, D/D0(glucose) was higher and D/P(Cr)was lower in the MSC-2 group than in the PUF-C and P-2 groups (P < 0.05). Transforming growth factor-ß (TGF-ß) levels were lower in the MSC groups than in the P and PUF-C groups (P < 0.05). CONCLUSION: The PUF-C group had a high permeability UFF. These results showed that MSC transplantation exerted positive effects on UFF in a chronic rat model of PD. MSC transplantation may provide new options for the renewal of the peritoneum in chronic PD patients with UFF.


Assuntos
Permeabilidade da Membrana Celular , Falência Renal Crônica/terapia , Transplante de Células-Tronco Mesenquimais , Diálise Peritoneal/efeitos adversos , Ultrafiltração/efeitos adversos , Animais , Transporte Biológico , Doença Crônica , Soluções para Diálise , Masculino , Peritônio/metabolismo , Ratos , Ratos Wistar , Fator de Crescimento Transformador beta/metabolismo , Falha de Tratamento
18.
World J Urol ; 31(5): 1117-22, 2013 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-22258667

RESUMO

OBJECTIVE: Urolithiasis in infants is not a very rare situation in Turkey, and the incidence has been increasing in recent years. The purpose of this paper was to investigate the clinical characteristics, metabolic and anatomic risk factors for urolithiasis and microlithiasis in infants. METHODS: The cases of 178 infants (63 girls, 115 boys), who were referred to our department between 1999 and 2009 with urolithiasis, were evaluated. RESULTS: The mean age at diagnosis of stone disease was 11.5 months (range, 10 days-24 months). The mean follow-up duration was 33.6 months (1.2-110 months). The major clinical symptoms of our patients were restlessness in 24 children (13.5%) and vomiting in 23 (13%). Thirty-five infants (19.7%) had a urinary tract abnormality; vesico-ureteral reflux was the most common abnormality (12.9%). Hypercalciuria and hyperuricosuria were detected in 46 and 56%, respectively. Stone analysis was performed in 56 infants, and calcium oxalate was determined in 36 patients (64.3%). A family history of urolithiasis, presenting symptoms and underlying metabolic abnormalities were similar for patients with microlithiasis and those with larger stones. However, infants with microlithiasis had higher ratios for history of vitamin D administration and feeding with formula. Surgical treatment was performed in 42 infants and extracorporeal shock wave lithotripsy in 30 infants. CONCLUSION: Our results showed that urolithiasis in infants may present nonspecific symptoms and may even be asymptomatic and that a positive family history for urolithiasis, urologic abnormalities, metabolic disorders, urinary tract infections, vitamin D administration and feeding with formula may increase the occurrence of urolithiasis in infants.


Assuntos
Urolitíase/epidemiologia , Urolitíase/etiologia , Pré-Escolar , Feminino , Humanos , Incidência , Lactente , Recém-Nascido , Litotripsia , Masculino , Estudos Retrospectivos , Fatores de Risco , Urolitíase/terapia , Procedimentos Cirúrgicos Urológicos
19.
J Pediatr Endocrinol Metab ; 25(11-12): 1111-7, 2012.
Artigo em Inglês | MEDLINE | ID: mdl-23329757

RESUMO

BACKGROUND: Obesity in children increases the risk of atherosclerosis. Endothelial dysfunction is an important factor in the pathogenesis of atherosclerosis, and endothelial microparticles (EMPs) are considered as markers of endothelial dysfunction. In this study, we aimed to evaluate circulating EMPs in obese and overweight children and to disclose the measure of obesity with the strongest relation with circulating microparticles and carotid atherosclerosis. METHODS: This prospective study included 55 obese and overweight children and 23 healthy controls. Insulin resistance was studied. Both in vivo and in vitro human umbilical vein endothelial cell evaluations were used for the study. Circulating EMPs (CD144 and CD146) were measured by flow cytometry. The carotid artery intima-media thickness (cIMT) and left ventricular mass index (LVMI) were measured using ultrasound and echocardiography, respectively. Study groups were compared for anthropometric measurement, insulin resistance, circulating EMP, cIMT, and LVMI. The relationship among overweight, obesity, and circulating EMPs were investigated. RESULTS: Blood pressure, CD144+EMP levels, and LVMI were statistically higher in the patients group than in the control group. The multiple logistic regression analysis and the backward elimination method showed that CD144+EMP and systolic blood pressure had a linear relationship with overweight and obesity. CONCLUSION: Our results suggest that endothelial damage starts in the early stage of childhood obesity and that obese and overweight children have increased circulating CD144+EMPs, showing that endothelial dysfunction and increased CD144+EMPs may be related to obesity.


Assuntos
Doenças das Artérias Carótidas/diagnóstico , Micropartículas Derivadas de Células/patologia , Endotélio Vascular/patologia , Obesidade/diagnóstico , Adolescente , Antígenos CD/metabolismo , Antígenos CD/farmacologia , Antígeno CD146/metabolismo , Antígeno CD146/farmacologia , Caderinas/metabolismo , Caderinas/farmacologia , Doenças das Artérias Carótidas/metabolismo , Doenças das Artérias Carótidas/fisiopatologia , Micropartículas Derivadas de Células/metabolismo , Criança , Ecocardiografia , Eletrocardiografia , Endotélio Vascular/metabolismo , Feminino , Células Endoteliais da Veia Umbilical Humana/efeitos dos fármacos , Humanos , Hipertrofia Ventricular Esquerda/diagnóstico por imagem , Hipertrofia Ventricular Esquerda/patologia , Masculino , Obesidade/metabolismo , Obesidade/fisiopatologia , Estudos Prospectivos
20.
Turk J Pediatr ; 54(4): 393-402, 2012.
Artigo em Inglês | MEDLINE | ID: mdl-23692721

RESUMO

The aim of this study was to evaluate the mid-term clinical status of patients following tetralogy of Fallot (TOF) repair. We performed a cross-sectional observational analysis of 57 postoperative TOF patients and 58 age-matched controls. Patients were examined with myocardial performance index (MPI), which was obtained by tissue Doppler imaging (TDI) in addition to the conventional methods. Compared with controls, patients had significantly higher right ventricular (RV) dimensions and volumes, RV MPI and left ventricular (LV) MPI, and significantly lower RV ejection fraction (EF), LV EF, exercise duration, and maximum heart rate with exercise (p<0.001 for all parameters). A positive correlation was found between RV MPI and LV MPI (r=0.541, p<0.001). There was a negative correlation between LV MPI and exercise duration (r=0.260, p=0.034). After total surgical correction of TOF, the patients had a good clinical status at the mid-term follow-up; decreased exercise capacity and impaired functions in both ventricles related to the degree of pulmonary regurgitation were found. By using MPI, impaired cardiac functions can identify such conditions before they become clinically symptomatic.


Assuntos
Procedimentos Cirúrgicos Cardíacos , Tetralogia de Fallot/cirurgia , Adolescente , Estudos de Casos e Controles , Distribuição de Qui-Quadrado , Criança , Pré-Escolar , Estudos Transversais , Ecocardiografia Doppler , Eletrocardiografia , Teste de Esforço , Feminino , Humanos , Masculino , Estatísticas não Paramétricas , Resultado do Tratamento , Adulto Jovem
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