RESUMO
OBJECTIVES: To develop and test a new multidimensional questionnaire for assessment of children with auto-inflammatory disease (AID) such as FMF, PFAPA, HIDS, TRAPS in standard clinical care. METHODS: The juvenile auto-inflammatory disease multidimensional assessment report (JAIMAR) includes 16 parent or patient-centered measures and four dimensions that assess functional status, pain, therapeutic compliance and health-related quality of life (physical, social, school, emotional status) with disease outcome. It is proposed for use as both a proxy-report and a patient self-report, with the suggested age range of 8-18 years for use as a self-report. RESULTS: 250 children with FMF were included in the study. Total of 179 forms were filled up by parents and patients, and 71 forms were filled up by parents having children less than 8 years. Completing and scoring the JAIMAR can be done in 15 minutes. For the JAIMAR's dimensions, the Cronbach's alpha coefficient for internal consistency was between 0.507-0.998. There was a significant and a positive correlation between the test-retest scale scores (ICC=0.607-0.966). Concerning construct validity, all factors loadings were above 0.30. For the criterion validity, the correlation level between each dimension and the related scale ranged from medium (r=0.329, p<0.0001) to large (r=0.894, p<0.0001). The parents' proxy-reported and children's self-reported data were outstandingly concordant (r=0.770-0.989). CONCLUSIONS: The development of the JAIMAR introduces a new and multi-dimensional approach in paediatric rheumatology practice. It is a new tool for children with auto-inflammatory dis-ease and it may help enhance their quality of care.
Assuntos
Febre Familiar do Mediterrâneo/diagnóstico , Febre/diagnóstico , Doenças Hereditárias Autoinflamatórias/diagnóstico , Inquéritos e Questionários , Adolescente , Fatores Etários , Criança , Pré-Escolar , Efeitos Psicossociais da Doença , Febre Familiar do Mediterrâneo/fisiopatologia , Febre Familiar do Mediterrâneo/psicologia , Febre Familiar do Mediterrâneo/terapia , Feminino , Febre/fisiopatologia , Febre/psicologia , Febre/terapia , Nível de Saúde , Doenças Hereditárias Autoinflamatórias/fisiopatologia , Doenças Hereditárias Autoinflamatórias/psicologia , Doenças Hereditárias Autoinflamatórias/terapia , Humanos , Masculino , Valor Preditivo dos Testes , Qualidade de Vida , Reprodutibilidade dos Testes , Índice de Gravidade de DoençaRESUMO
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/epidemiologiaRESUMO
OBJECTIVES: This was a prospective controlled study to determine the P-wave duration (Pdu) and P-wave dispersion (Pd) in patients with familial Mediterranean fever (FMF). METHODS: The study group consisted of 26 children with uncomplicated FMF and 25 age- and sex-matched healthy controls. We performed electrocardiography (ECG) with Doppler echocardiography on patients and controls. All participants underwent 12-lead electrocardiography under strict standards. Pdu and Pd were computed from a randomly selected beat and from an averaged beat constructed from 12 beats, included in a 10-s ECG. RESULTS: The left ventricle (LV) dimensions, LV ejection fraction (LVEF), and LV fractional shortening (LVFS) values, left atrium dimension, and aortic dimension were in normal range in both groups. There were significant differences between the groups regarding LV-isovolumic relaxation time (IRT), LV-isovolumic contraction time (ICT), right ventricle (RV)-ICT, RV-IRT, and Pd (all p < 0.0001). However, highly significant positive correlation was detected between LV-ICT, LV-IRT, RV-ICT, RV-IVT, C-reactive protein (CRP), and Pd (r = 0.505, p < 0.0001; r = 0.483, p < 0.0001; r = 0.433, p = 0.001; r = 0.421, p = 0.001; r = 0.452, p = 0.001; r = 0.478, p < 0.0001, respectively). CONCLUSIONS: Uncomplicated FMF children who are continuously treated with colchicine and do not develop amyloidosis have abnormal atrial dispersion and therefore seemingly have an increased electrocardiographic risk of atrial fibrillation.
Assuntos
Fibrilação Atrial/fisiopatologia , Febre Familiar do Mediterrâneo/fisiopatologia , Contração Miocárdica/fisiologia , Função Ventricular Esquerda/fisiologia , Adolescente , Fibrilação Atrial/complicações , Criança , Pré-Escolar , Eletrocardiografia , Febre Familiar do Mediterrâneo/complicações , Feminino , Humanos , Masculino , Estudos ProspectivosRESUMO
AIM: Pediatric surgeons frequently encounter children presenting with voiding dysfunction symptoms, including urgency, frequency, and incontinence. Antimuscarinic agents Oxybutynin) are the main drugs used to treat patients with overactive bladder (OAB) syndrome, defined as urgency, with or without urgency incontinence, usually with increased daytime frequency and nocturia. Increased QT dispersion is known to be the cause of ventricular arrhythmia in various systemic diseases and leads to increased mortality and morbidity. METHOD: This study represents a subset of a complete data set, considering only those children aged admitted to the Pediatric Surgery and Pediatric Nephrology Clinics during the period January 2011 to July 2012. RESULT: In this study, we have determined that the QT interval changes significantly depending on the use of oxybutynin. The QT changes increased cardiac arrhythmia in children. CONCLUSION: For this reason, children using such drugs should be closely monitored for cardiac arrhythmia.
Assuntos
Arritmias Cardíacas/induzido quimicamente , Eletrocardiografia/efeitos dos fármacos , Ácidos Mandélicos/efeitos adversos , Bexiga Urinária Hiperativa/tratamento farmacológico , Adolescente , Arritmias Cardíacas/fisiopatologia , Criança , Pré-Escolar , Feminino , Humanos , Masculino , Ácidos Mandélicos/uso terapêutico , Antagonistas Muscarínicos/efeitos adversos , Antagonistas Muscarínicos/uso terapêutico , Estudos RetrospectivosRESUMO
OBJECTIVES: Modified adult disease severity scoring systems are being used for childhood FMF. We aim to test the clinical consistency of two common severity scoring systems and to evaluate the correlation of scores with the type of FMF mutations in paediatric FMF patients since certain mutations are prone to severe disease. METHODS: Two hundred and fifty-eight children with FMF were cross-sectionally studied. Assessment of the disease severity was performed by using the modified scoring systems of Mor et al. and Pras et al. Genetic analysis was performed using PCR and restriction endonuclease digestion methods for the presence of 15 FMF gene mutations. FMF mutations were grouped into three based on well-known genotypic-phenotypic associations. Correlation between the mutation groups and the severity scoring systems was assessed. The consistency of the severity scoring systems was evaluated. RESULTS: The results of two scoring systems were not statistically consistent with each other (κ = 0.171). This inconsistency persisted even in a more homogeneous subgroup of patients with only homozygote mutations of M694V, M680I and M694I (κ = 0.125). There was no correlation between the mutation groups and either of the scoring systems (P = 0.002, r = 0,196 for scoring systems of Mor et al.; P = 0.009, r = 0.162 for Pras et al.). CONCLUSIONS: The inconsistency of the two scoring systems and lack of correlation between the scoring systems and mutation groups raises concerns about the reliability of these scoring systems in children. There is a need to develop a scoring system in children based on a prospective registry.
Assuntos
Febre Familiar do Mediterrâneo/diagnóstico , Índice de Gravidade de Doença , Adolescente , Fatores Etários , Criança , Pré-Escolar , Estudos Transversais , Febre Familiar do Mediterrâneo/genética , Feminino , Frequência do Gene , Predisposição Genética para Doença , Humanos , Masculino , Mutação , Reprodutibilidade dos TestesRESUMO
The aim of this prospective, multicenter study was to define the etiology and clinical features of acute kidney injury (AKI) in a pediatric patient cohort and to determine prognostic factors. Pediatric-modified RIFLE (pRIFLE) criteria were used to classify AKI. The patient cohort comprised 472 pediatric patients (264 males, 208 females), of whom 32.6% were newborns (median age 3 days, range 1-24 days), and 67.4% were children aged >1 month (median 2.99 years, range 1 month-18 years). The most common medical conditions were prematurity (42.2%) and congenital heart disease (CHD, 11.7%) in newborns, and malignancy (12.9%) and CHD (12.3%) in children aged >1 month. Hypoxic/ischemic injury and sepsis were the leading causes of AKI in both age groups. Dialysis was performed in 30.3% of newborns and 33.6% of children aged >1 month. Mortality was higher in the newborns (42.6 vs. 27.9%; p < 0.005). Stepwise multiple regression analysis revealed the major independent risk factors to be mechanical ventilation [relative risk (RR) 17.31, 95% confidence interval (95% CI) 4.88-61.42], hypervolemia (RR 12.90, 95% CI 1.97-84.37), CHD (RR 9.85, 95% CI 2.08-46.60), and metabolic acidosis (RR 7.64, 95% CI 2.90-20.15) in newborns and mechanical ventilation (RR 8.73, 95% CI 3.95-19.29), hypoxia (RR 5.35, 95% CI 2.26-12.67), and intrinsic AKI (RR 4.91, 95% CI 2.04-11.78) in children aged >1 month.
Assuntos
Injúria Renal Aguda/mortalidade , Criança , Feminino , Humanos , Recém-Nascido , Rim , Masculino , Análise Multivariada , Respiração Artificial/mortalidade , Fatores de Risco , Sepse/mortalidade , Resultado do TratamentoRESUMO
Familial Mediterranean fever (FMF) is an autosomal recessive disease characterized by self-limited attacks of fever and polyserositis. Articular involvement in early-onset FMF is a common finding characterized by non-erosive, generally asymmetric monoarthritis in large joints. Protracted FMF arthritis was reported in 2.6% of Turkish patients. An 8-year-old female who has a history of FMF for 5 years applied to our hospital with complaints of persistent swelling and pain of her left knee for 8 months. The patient had been tried to be managed with non-steroidal anti-inflammatory drugs as well as intra-articulary steroids and colchicine. However, arthritis and acute phase response persisted. With sulphasalazine, complete recovery was achieved. It is our belief that sulphasalazine can be a choice of medical treatment in protracted FMF arthritis.
Assuntos
Anti-Inflamatórios não Esteroides/uso terapêutico , Artrite/tratamento farmacológico , Febre Familiar do Mediterrâneo/complicações , Articulação do Joelho , Sulfassalazina/uso terapêutico , Artrite/diagnóstico , Artrite/etiologia , Criança , Feminino , Humanos , Imageamento por Ressonância Magnética , TurquiaRESUMO
In this study, we aimed to investigate whether neutrophil/lymphocyte ratio (NLR), platelet/lymphocyte ratio (PLR), and mean platelet volume (MPV) might be helpful in the diagnosis of subclinical inflammation of familial Mediterranean fever (FMF). Clinical, laboratory, and genetic results of the patients who were followed up with the diagnosis of FMF were obtained retrospectively from the hospital files and recorded to standardized form. Age- and sex-matched healthy subjects were included as the control group. Eighty-three of the 143 patients (58.0%) were male and 60 (42.0%) were female. The mean age of our patients was 164.62 ± 51.20 months and the mean age of the control group was 164.92 ± 51.10 months. The mean diagnosis age of our patients was 98.10 ± 49.11 months. The mean follow-up time of the patients was 66.03 ± 36.37 months. 91.60% of our patients had abdominal pain, 78.32% fever, and 28.67% joint pain. The mean NLR of the patients was significantly higher than the mean levels at attack-free period and the control group. However, no statistically significant difference was found between the mean levels at attack-free period and the control group. MPV levels were statistically significantly high during acute attack when compared with the control group. However, they showed no statistically significant difference between acute attack and attack-free period. NLR is a useful marker to predict inflammation in FMF patients. However, our results did not support the idea that MPV might reflect acute attack and attack-free period.
Assuntos
Febre Familiar do Mediterrâneo/sangue , Inflamação/sangue , Contagem de Linfócitos , Volume Plaquetário Médio , Neutrófilos , Contagem de Plaquetas , Adolescente , Estudos de Casos e Controles , Criança , Feminino , Humanos , Contagem de Leucócitos , MasculinoRESUMO
The aim is to investigate whether pediatric familial Mediterranean fever (FMF) patients have an increased risk of premature atherosclerosis and to determine the possible strength of association between atherosclerosis and Mediterranean fever (MEFV) gene mutation gene type. Demographic characteristics and MEFV mutations were defined in 49 children diagnosed with FMF (26 female, 23 male; mean age, 10.71 +/- 3.69 years). Twenty-six age-, sex-, and body-mass-index-matched healthy children constituted the control group. We evaluated the blood counts and acute-phase proteins during attack-free periods. Mean C-reactive protein (CRP), serum amyloid-A (SAA), homocysteine (Hcy), lipoprotein-a (Lp-a), and common carotid artery intima-media thickness (CCA-IMT) were 10.75 +/- 15.29 vs 4.03 +/- 1.20, 23.22 +/- 41.94 vs 3.53 +/- 1.04, 10.36 +/- 3.36 vs 8.64 +/- 3.15, 20.84 +/- 23.89 vs 8.56 +/- 7.48, and 0.038 +/- 0.007 vs 0.032 +/- 0.004, respectively, and significantly higher than the mean values of control group (p < 0.05). However, no correlation was found between CCA-IMT and CRP, SAA, Hcy, and Lp-a. Twenty-nine patients had M694V mutation, and 13 patients had other mutations. There was no correlation between CCA-IMT and MEFV mutation subgroups. In conclusion, because of the nature of the disease, FMF patients should be considered to have an increased risk of early vascular alteration and atherosclerosis. For this reason, CCA-IMT measurement can be recommended as a noninvasive and early diagnostic method.
Assuntos
Doenças das Artérias Carótidas/diagnóstico por imagem , Doenças das Artérias Carótidas/epidemiologia , Febre Familiar do Mediterrâneo/diagnóstico por imagem , Febre Familiar do Mediterrâneo/epidemiologia , Proteínas de Fase Aguda/metabolismo , Adolescente , Biomarcadores/sangue , Proteína C-Reativa/metabolismo , Doenças das Artérias Carótidas/imunologia , Artéria Carótida Primitiva/diagnóstico por imagem , Criança , Estudos Transversais , Febre Familiar do Mediterrâneo/imunologia , Feminino , Homocisteína/sangue , Humanos , Lipoproteína(a)/sangue , Masculino , Fatores de Risco , Proteína Amiloide A Sérica/metabolismo , Túnica Íntima/diagnóstico por imagem , Túnica Média/diagnóstico por imagem , UltrassonografiaRESUMO
Henoch Schonlein purpura (HSP) is the most common vasculitis of childhood. Susceptibility to HSP and associated clinical heterogeneity in HSP may be conferred by a number of genetic loci, including the major histocompatibility complex. We aimed to investigate the implications of the human leukocyte antigen (HLA) class 1 alleles in susceptibility to HSP and determine the possible associations with renal, gastrointestinal (GI), and joint manifestations of the disease. 110 children with HSP (66 boys, 44 girls) and 250 unrelated healthy controls were enrolled in the study. The mean age was 8.65 +/- 3.59 years. HSP was diagnosed on the basis of clinical and laboratory data according to the American College of Rheumatology classification. The diagnosis was supported with skin and/or kidney in most of the patients. Clinical and laboratory findings revealed: skin involvement in 110 (100%), joint manifestations in 82 (74.5%), GI symptoms in 58 (52.7%), and hematuria and/or proteinuria in 36 (32.7%) patients. HLA class 1 alleles were identified by DNA amplification, hybridized with specific primer sequences. Comparison of frequencies between patients and controls were made by using the Fisher's exact test. Odds ratio (OR) was used as the measure of association. HLA A2, A11, and B35 antigens showed an increased risk for predisposition to HSP (OR = 1.714, 95%CI = 1.088-2.700, p = 0.020; OR = 2.185, 95%CI = 1.289-3.703, p = 0.003; and OR = 2.292, 95%CI = 1.451-3.619, p = 0.000, respectively), while HLA A1, B49, and B50 antigens revealed decreased risk for predisposition to HSP (OR = 4.739, 95%CI = 1.828-12.345, p = 0.001; OR = 3.268, 95%CI = 0.955-11.236, p = 0.047; and OR = 7.462, 95%CI = 0.975-55.555, p = 0.024, respectively). Considering the renal involvement and severity of proteinuria, there was no association with HLA class 1 alleles. Our results suggest that the increased frequency of HLA A2, A11, and B35 alleles in unselected pediatric HSP patient population and miscarrying of HLA A1, B49, and B50 could be considered as a risk factor for susceptibility to HSP.
Assuntos
Frequência do Gene , Genes MHC Classe I , Predisposição Genética para Doença , Antígenos de Histocompatibilidade Classe I/genética , Vasculite por IgA/genética , Adolescente , Criança , Pré-Escolar , Feminino , Gastroenteropatias/etiologia , Gastroenteropatias/genética , Gastroenteropatias/imunologia , Antígenos HLA-B/sangue , Antígenos HLA-B/genética , Antígenos de Histocompatibilidade Classe I/sangue , Teste de Histocompatibilidade , Humanos , Vasculite por IgA/complicações , Vasculite por IgA/imunologia , Artropatias/etiologia , Artropatias/genética , Artropatias/imunologia , Nefropatias/etiologia , Nefropatias/genética , Nefropatias/imunologia , Masculino , Reação em Cadeia da Polimerase , Polimorfismo Genético , Proteinúria/etiologia , Proteinúria/genética , Proteinúria/imunologiaRESUMO
We aimed to evaluate the patients who were diagnosed as Henoch Schonlein purpura (HSP) for disease characteristics and prognosis of those with joint, gastrointestinal (GI), and renal involvement. Two hundred and fifty-four children who were followed up with the diagnosis of HSP in the Pediatric Nephrology Clinics of Meram Medical Faculty of Selcuk University and Medical Faculty of Gazi University between January 2003 and June 2006 were retrospectively evaluated. The clinical follow-up and treatment regimens of patients in whom renal biopsy was performed were evaluated in detail. The study group consisted of 254 children, 147 boys (57.8%) and 107 girls (42.2%), and the ratio of boys to girls was 1.37. The percentages of skin, joint, GI, and renal manifestations were 100%, 66%, 56%, and 30%, respectively. Eight patients had intussusception. Five of them recovered with steroid treatment only while three patients were operated on. Sixty-four patients (44%) with GI involvement had severe disease and were successfully treated with steroids. Renal biopsy was performed in 26 patients. Among those 26 patients, two of them recovered spontaneously within 3 and 4 weeks. Ten patients improved with only steroid treatment while 12 patients recovered with steroid and cyclophosphamide treatment. Two patients were resistant to steroid and cyclophosphamide treatment and were treated with cyclosporine A. We believe that steroid therapy given to the HSP patients with GI manifestations might be helpful to prevent probable complications such as GI bleeding and intussusception. In addition, combined therapy with steroid and cyclophosphamide can usually be an appropriate treatment for patients with nephrotic proteinuria.
Assuntos
Vasculite por IgA , Criança , Pré-Escolar , Ciclosporina/uso terapêutico , Feminino , Seguimentos , Gastroenteropatias/complicações , Humanos , Vasculite por IgA/complicações , Vasculite por IgA/terapia , Intussuscepção/complicações , Artropatias/complicações , Nefropatias/complicações , MasculinoRESUMO
OBJECTIVE: To report a case with the diagnosis of IgM nephropathy and familial Mediterranean fever (FMF). CLINICAL PRESENTATION AND INTERVENTION: A 9-year-old boy was admitted to our hospital with recurrent abdominal pain since the age of 4 years. Laboratory investigations revealed a sedimentation rate of 88 mm/h, C-reactive protein: 83.2 mg/l (0-10 mg/l), white blood cell count: 12,700/mm(3), fibrinogen: 622 mg/dl (200-400 mg/dl) and serum amyloid A: 186 mg/l (0-5.8 mg/l). Urinalysis revealed +2 proteinuria. A 24-hour urinary protein excretion was 12 mg/m(2)/h. M694V homozygous mutation was identified in exon 10. Percutaneous renal biopsy showed mesangial cell proliferation and increased mesangial matrix in the glomeruli, without amyloid accumulation. Immunofluorescence study showed IgM (+1) and C1q (+1) deposits. Treatment with 1 mg/day colchicine was started. Six weeks later, proteinuria had disappeared and the patient was asymptomatic. CONCLUSION: This case illustrates the unusual association of FMF with non-amyloid glomerulopathy. Glomerular diseases such as IgM nephropathy may be seen as a manifestation of FMF.
Assuntos
Febre Familiar do Mediterrâneo/complicações , Glomerulonefrite/complicações , Glomerulonefrite/imunologia , Imunoglobulina M/imunologia , Criança , Colchicina/uso terapêutico , Febre Familiar do Mediterrâneo/diagnóstico , Febre Familiar do Mediterrâneo/tratamento farmacológico , Febre Familiar do Mediterrâneo/fisiopatologia , Glomerulonefrite/diagnóstico , Glomerulonefrite/tratamento farmacológico , Humanos , Masculino , Moduladores de Tubulina/uso terapêuticoRESUMO
INTRODUCTION: The aim was to investigate the relationship between vesicoureteral reflux (VUR) and renal damage in non-febrile, febrile for the first time and recurrent urinary tract infection (UTI) patients. The secondary aim was to determine whether C-reactive protein (CRP) in febrile UTIs could be a predictor of renal scarring. POPULATION AND METHODS: This prospective study included non-febrile, febrile for the first time and recurrent pediatric UTI cases. The routine lab analyses comprised a complete blood count, urea, creatinine, fully automated urinalysis, urine culture and CRP analyses. All the participants were examined using urine ultrasonography subsequent to their UTI diagnosis, voiding cystourethrography (VCUG) after six weeks and Tc-99m dimercaptosuccinic acid (DMSA) static renal scintigraphy after six months. RESULTS: There were included 47 children with non-febrile UTIs, 48 with first febrile UTIs and 61 with recurrent UTIs. A statistically significant difference was found among the groups in terms of VUR and renal scarring (p= 0.001 and p= 0.011, respectively). A statistically significant difference was also found in terms of renal scarring between patients with and without VUR (p= 0.001). Moreover, a statistically significant difference was also present in relation to renal scarring (p <0.05) in patients with five-fold lower or higher CRP values than the accepted cut-off value (5mg/dl). CONCLUSION: The ratio of renal scars detected was found to be parallel to the VUR frequency. The higher the VUR grade, the more renal damage was found. A positive correlation between elevated CRP and renal scarring was determined, indicating the presence of scarring during the diagnosis of pyelonephritis.
Introducción. El objetivo del estudio fue investigar la relación entre reflujo vesicoureteral (RVU) y daño renal en pacientes con infección urinaria (IU) sin fiebre, primera IU febril e IU recurrente. El objetivo secundario, determinar si la proteína C-reactiva (PCR) actuaría como predictor de nefroesclerosis en las IU febriles. Población y métodos. Estudio prospectivo; pacientes pediátricos con IU sin fiebre, primera IU febril e IU recurrente. Los análisis de laboratorio de rutina incluyeron hemograma completo, urea, creatinina, análisis de orina completamente automatizado, urocultivo y PCR. Se realizó ecografía urológica luego del diagnóstico de IU, cistouretrografía miccional tras seis semanas y gammagrafía renal estática con ácido dimercaptosuccínico marcado con 99mTc tras seis meses a todos los participantes. Resultados. Participaron 47 niños con IU sin fiebre, 48 con primera IU febril y 61 con IU recurrente. Hubo una diferencia estadísticamente significativa entre los grupos respecto de RVU y nefroesclerosis (p= 0,001 y p= 0,011, respectivamente). También hubo una diferencia estadísticamente significativa respecto de nefroesclerosis entre los pacientes con y sin RVU (p= 0,001). Además, se estableció una diferencia estadísticamente significativa respecto de nefroesclerosis (p < 0,05) en los pacientes con PCR cinco veces mayor o menor que el valor de corte aceptado (5 mg/dl). Conclusión. La proporción de nefroesclerosis fue paralela a la frecuencia de RVU. Cuanto mayor era el grado de RVU, mayor era el daño renal. Se determinó una correlación positiva entre PCR elevada y nefroesclerosis, lo que señala esclerosis durante el diagnóstico de pielonefritis.
Assuntos
Cicatriz/epidemiologia , Rim/patologia , Infecções Urinárias/complicações , Refluxo Vesicoureteral/epidemiologia , Proteína C-Reativa/metabolismo , Criança , Pré-Escolar , Cicatriz/etiologia , Feminino , Febre/epidemiologia , Febre/etiologia , Humanos , Lactente , Rim/diagnóstico por imagem , Masculino , Estudos Prospectivos , Pielonefrite/complicações , Pielonefrite/diagnóstico , Recidiva , Ácido Dimercaptossuccínico Tecnécio Tc 99m/administração & dosagem , Ultrassonografia/métodos , Infecções Urinárias/epidemiologia , Refluxo Vesicoureteral/etiologiaRESUMO
Longitudinally extensive transverse myelitis (LETM) is defined as an inflammatory lesion of the spinal cord that extends to three or more segments. LETM is a commonly characteristic feature of neuromyelitis optica (NMO) or various autoimmune diseases. Manifestation of Mycobacterium tuberculosis (MTB) infection with LETM are rare and usually in the cervicothoracic spinal cord. Our patient presented with holocord LETM, so NMO was considered initially diagnosis. After in further research, MTB was diagnosed and treated successfully. The current case underscores that tuberculosis must be keep in mind when undertaking differential diagnosis of demyelinating diseases even in the absence of symptoms of infection, and especially in endemic regions.
RESUMO
BACKGROUND: In this study, we examined the efficacy and safety of a once-daily dosage schema of colchicine compared with a twice-daily dosage schema in pediatric patients with familial Mediterranean fever (FMF). METHODS: In this 24-week, multicenter, randomized controlled noninferiority trial, pediatric patients newly diagnosed with FMF carrying a homozygous or compound heterozygous mutation and not receiving any treatment were included. Patients were randomly assigned using a block randomization method to receive treatment with a once- or twice-daily dosage. Clinical and laboratory characteristics and medication side effects were recorded and compared between groups. The study was carried out in compliance with Good Clinical Practice and the Consolidated Standards for Reporting of Trials (CONSORT) statement. RESULTS: A total of 92 patients were selected, and 79 patients completed the study. There were 42 patients in the once-daily dosage group and 37 in the twice-daily dosage group. The results indicated that the once-daily dosage was not inferior to the twice-daily dosage regarding decrease in attack frequency and duration as well as improvement in clinical findings and Mor severity scores. Alterations in laboratory findings indicating inflammation, such as erythrocyte sedimentation rate, C-reactive protein, and serum amyloid A, were similar in both groups. The rates of drug side effects were similar between the once- and twice-daily dosage groups, implying comparable safety of colchicine, with the exception of diarrhea, which was slightly higher in the once-daily dosage group. CONCLUSIONS: Using colchicine with either a once- or twice-daily dosage provides similar clinical and laboratory improvements. Considering both efficacy and safety, colchicine can be prescribed with a once-daily dosage. TRIAL REGISTRATION ID: ClinicalTrials.gov identifier NCT02602028 . Registered 5 November 2015.
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Colchicina/administração & dosagem , Febre Familiar do Mediterrâneo/tratamento farmacológico , Moduladores de Tubulina/administração & dosagem , Criança , Esquema de Medicação , Feminino , Humanos , MasculinoRESUMO
Calcium pyrophosphate dehydrate (CPPD) crystal deposition disease (also known as chondrocalcinosis, CC) is a rare metabolic arthropathy mostly seen in elderly patients. Chondrocalcinosis may be associated with metabolic diseases such as hypomagnesemia when it occurs in young people. We report here a case with hypomagnesemia due to familial hypomagnesemia with hypercalciuria and nephrocalcinosis (FHHNC) who developed CC during clinical follow-up. To our best knowledge this is the first case of a young patient with CPPD associated with FHHNC.
Assuntos
Condrocalcinose/complicações , Hipercalciúria/complicações , Nefrocalcinose/complicações , Erros Inatos do Transporte Tubular Renal/complicações , Adolescente , Condrocalcinose/diagnóstico por imagem , Humanos , Hipercalciúria/diagnóstico por imagem , Masculino , Nefrocalcinose/diagnóstico por imagem , Radiografia , Erros Inatos do Transporte Tubular Renal/diagnóstico por imagemRESUMO
Association of Kocher-Debré-Semelaigne syndrome-a myopathy of hypothyroidism in childhood characterized by muscular hypertrophy, with rhabdomyolysis is very rare. We present a case of Kocher-Debré-Semelaigne syndrome with rhabdomyolysis secondary to Hashimoto's thyroiditis. He had muscular symptoms simulating poly/dermatomyositis, massively elevated creatine kinase (CK) levels and high creatinine levels. All of the findings reversed on treatment of hypothyroidism. The response to the therapy strongly suggested that Kocher-Debré-Semelaigne (KDS) syndrome was the underlying etiology. Serum thyroid- stimulating hormone levels should be routinely determined in all patients with muscular symptoms and/or elevation of CK and creatinine, keeping KDS syndrome in mind.
Assuntos
Hipotireoidismo Congênito/complicações , Creatinina/sangue , Doença de Hashimoto/complicações , Hipertrofia/complicações , Músculo Esquelético/patologia , Doenças Musculares/complicações , Rabdomiólise/etiologia , Adolescente , Hipotireoidismo Congênito/sangue , Creatina Quinase/sangue , Doença de Hashimoto/sangue , Doença de Hashimoto/tratamento farmacológico , Humanos , Hipertrofia/sangue , Masculino , Doenças Musculares/sangue , Rabdomiólise/sangue , Tiroxina/uso terapêutico , Resultado do TratamentoRESUMO
Introducción. El objetivo del estudio fue investigar la relación entre reflujo vesicoureteral (RVU) y daño renal en pacientes con infección urinaria (IU) sin fiebre, primera IU febril e IU recurrente. El objetivo secundario, determinar si la proteína C-reactiva (PCR) actuaría como predictor de nefroesclerosis en las IU febriles. Población y métodos. Estudio prospectivo; pacientes pediátricos con IU sin fiebre, primera IU febril e IU recurrente. Los análisis de laboratorio de rutina incluyeron hemograma completo, urea, creatinina, análisis de orina completamente automatizado, urocultivo y PCR. Se realizó ecografía urológica luego del diagnóstico de IU, cistouretrografía miccional tras seis semanas y gammagrafía renal estática con ácido dimercaptosuccínico marcado con 99mTc tras seis meses a todos los participantes. Resultados. Participaron 47 niños con IU sin fiebre, 48 con primera IU febril y 61 con IU recurrente. Hubo una diferencia estadísticamente significativa entre los grupos respecto de RVU y nefroesclerosis (p= 0,001 y p= 0,011, respectivamente). También hubo una diferencia estadísticamente significativa respecto de nefroesclerosis entre los pacientes con y sin RVU (p= 0,001). Además, se estableció una diferencia estadísticamente significativa respecto de nefroesclerosis (p < 0,05) en los pacientes con PCR cinco veces mayor o menor que el valor de corte aceptado (5 mg/dl). Conclusión. La proporción de nefroesclerosis fue paralela a la frecuencia de RVU. Cuanto mayor era el grado de RVU, mayor era el daño renal. Se determinó una correlación positiva entre PCR elevada y nefroesclerosis, lo que señala esclerosis durante el diagnóstico de pielonefritis.
Introduction. The aim was to investigate the relationship between vesicoureteral reflux (VUR) and renal damage in non-febrile, febrile for the first time and recurrent urinary tract infection (UTI) patients. The secondary aim was to determine whether C-reactive protein (CRP) in febrile UTIs could be a predictor of renal scarring. Population and methods. This prospective study included non-febrile, febrile for the first time and recurrent pediatric UTI cases. The routine lab analyses comprised a complete blood count, urea, creatinine, fully automated urinalysis, urine culture and CRP analyses. All the participants were examined using urine ultrasonography subsequent to their UTI diagnosis, voiding cystourethrography (VCUG) after six weeks and Tc-99m dimercaptosuccinic acid (DMSA) static renal scintigraphy after six months. Results. There were included 47 children with non-febrile UTIs, 48 with first febrile UTIs and 61 with recurrent UTIs. A statistically significant difference was found among the groups in terms of VUR and renal scarring (p= 0.001 and p= 0.011, respectively). A statistically significant difference was also found in terms of renal scarring between patients with and without VUR (p= 0.001). Moreover, a statistically significant difference was also present in relation to renal scarring (p <0.05) in patients with five-fold lower or higher CRP values than the accepted cut-off value (5mg/dl). Conclusion. The ratio of renal scars detected was found to be parallel to the VUR frequency. The higher the VUR grade, the more renal damage was found. A positive correlation between elevated CRP and renal scarring was determined, indicating the presence of scarring during the diagnosis of pyelonephritis.
Assuntos
Humanos , Masculino , Feminino , Recém-Nascido , Lactente , Pré-Escolar , Criança , Adolescente , Infecções Urinárias/complicações , Refluxo Vesicoureteral/epidemiologia , Cicatriz/epidemiologia , Rim/patologia , Pielonefrite/complicações , Pielonefrite/diagnóstico , Recidiva , Infecções Urinárias/epidemiologia , Refluxo Vesicoureteral/etiologia , Proteína C-Reativa/metabolismo , Estudos Prospectivos , Ultrassonografia/métodos , Cicatriz/etiologia , Ácido Dimercaptossuccínico Tecnécio Tc 99m/administração & dosagem , Febre/etiologia , Febre/epidemiologia , Rim/diagnóstico por imagemAssuntos
Enterococcus , Infecções por Bactérias Gram-Positivas , Peritonite/microbiologia , Criança , Feminino , Infecções por Bactérias Gram-Positivas/diagnóstico , Infecções por Bactérias Gram-Positivas/tratamento farmacológico , Humanos , Diálise Peritoneal Ambulatorial Contínua , Peritonite/diagnóstico , Peritonite/tratamento farmacológicoRESUMO
Early determination of renal scar development risk in children following first urinary tract infection (UTI) and early detection and treatment of vesicoureteral reflux (VUR) are important to prevent renal functional impairment. The aim of this study was to determine the prevalence of VUR and associated renal scar formation, in children who had acute pyelonephritis (APN), first afebrile UTI, and recurrent afebrile UTIs. Patient records of 642 children having UTI were scrutinized and 278 out of 642 were enrolled in this study. The patients were divided into three groups: Group 1, patients with APN (n = 73); Group 2, patients with the first afebrile UTI (n = 88); and group 3, patients with recurrent afebrile UTIs (n = 117). Among these groups, VUR prevalence did not differ significantly (group 1: 24.6%, group 2: 22.7%, and group 3: 26.4%. Renal scarring was detected in 19.3% of the patients and was remarkably lower in group 2 when compared with the other two groups (P < 0.05). Renal scarring was found in 37.1% of the patients with VUR, whereas it was found in 14.3% of patients without VUR. Renal scarring incidence was remarkably higher in patients with grades 4-5 VUR (61.5%) compared with those with grades 1-3 VUR (30.6%) (P = 0.055). In conclusion, since VUR was demonstrated in as many as one-quarter of patients with the first afebrile UTI and VUR incidence did not differ significantly among the groups, all UTIs, lower and upper ones, should be carefully evaluated.