Your browser doesn't support javascript.
loading
Mostrar: 20 | 50 | 100
Resultados 1 - 20 de 54
Filtrar
Más filtros

Banco de datos
País/Región como asunto
Tipo del documento
País de afiliación
Intervalo de año de publicación
1.
N Engl J Med ; 389(11): 987-997, 2023 Sep 14.
Artículo en Inglés | MEDLINE | ID: mdl-37702442

RESUMEN

BACKGROUND: The efficacy of continuous antibiotic prophylaxis in preventing urinary tract infection (UTI) in infants with grade III, IV, or V vesicoureteral reflux is controversial. METHODS: In this investigator-initiated, randomized, open-label trial performed in 39 European centers, we randomly assigned infants 1 to 5 months of age with grade III, IV, or V vesicoureteral reflux and no previous UTIs to receive continuous antibiotic prophylaxis (prophylaxis group) or no treatment (untreated group) for 24 months. The primary outcome was the occurrence of the first UTI during the trial period. Secondary outcomes included new kidney scarring and the estimated glomerular filtration rate (GFR) at 24 months. RESULTS: A total of 292 participants underwent randomization (146 per group). Approximately 75% of the participants were male; the median age was 3 months, and 235 participants (80.5%) had grade IV or V vesicoureteral reflux. In the intention-to-treat analysis, a first UTI occurred in 31 participants (21.2%) in the prophylaxis group and in 52 participants (35.6%) in the untreated group (hazard ratio, 0.55; 95% confidence interval [CI], 0.35 to 0.86; P = 0.008); the number needed to treat for 2 years to prevent one UTI was 7 children (95% CI, 4 to 29). Among untreated participants, 64.4% had no UTI during the trial. The incidence of new kidney scars and the estimated GFR at 24 months did not differ substantially between the two groups. Pseudomonas species, other non-Escherichia coli organisms, and antibiotic resistance were more common in UTI isolates obtained from participants in the prophylaxis group than in isolates obtained from those in the untreated group. Serious adverse events were similar in the two groups. CONCLUSIONS: In infants with grade III, IV, or V vesicoureteral reflux and no previous UTIs, continuous antibiotic prophylaxis provided a small but significant benefit in preventing a first UTI despite an increased occurrence of non-E. coli organisms and antibiotic resistance. (Funded by the Italian Ministry of Health and others; PREDICT ClinicalTrials.gov number, NCT02021006; EudraCT number, 2013-000309-21.).


Asunto(s)
Antibacterianos , Profilaxis Antibiótica , Infecciones Urinarias , Reflujo Vesicoureteral , Femenino , Humanos , Lactante , Masculino , Profilaxis Antibiótica/efectos adversos , Profilaxis Antibiótica/métodos , Glomerulonefritis , Análisis de Intención de Tratar , Reflujo Vesicoureteral/complicaciones , Reflujo Vesicoureteral/tratamiento farmacológico , Antibacterianos/administración & dosificación , Antibacterianos/efectos adversos , Antibacterianos/uso terapéutico , Infecciones Urinarias/etiología , Infecciones Urinarias/microbiología , Infecciones Urinarias/prevención & control , Farmacorresistencia Bacteriana/efectos de los fármacos
2.
Artículo en Inglés | MEDLINE | ID: mdl-38327222

RESUMEN

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.

3.
Pediatr Nephrol ; 39(5): 1509-1519, 2024 May.
Artículo en Inglés | MEDLINE | ID: mdl-38040872

RESUMEN

BACKGROUND: Myelomeningocele (MMC) is highly prevalent in developing countries, and MMC-related neurogenic bladder is an important cause of childhood chronic kidney disease (CKD). This nationwide study aimed to evaluate demographic and clinical features of pediatric patients with MMC in Turkey and risk factors associated with CKD stage 5. METHODS: Data from children aged 0-19 years old, living with MMC in 2022, were retrospectively collected from 27 pediatric nephrology centers. Patients > 1 year of age without pre-existing kidney abnormalities were divided into five groups according to eGFR; CKD stages 1-5. Patients on dialysis, kidney transplant recipients, and those with eGFR < 15 ml/min/1.73 m2 but not on kidney replacement therapy at time of study constituted the CKD stage 5 group. RESULTS: A total of 911 (57.8% female) patients were enrolled, most of whom were expectantly managed. Stages 1-4 CKD were found in 34.3%, 4.2%, 4.1%, and 2.4%, respectively. CKD stage 5 was observed in 5.3% of patients at median 13 years old (range 2-18 years). Current age, age at first abnormal DMSA scan, moderate-to-severe trabeculated bladder on US and/or VCUG, and VUR history were independent risk factors for development of CKD stage 5 (OR 0.752; 95%; CI 0.658-0.859; p < 0.001; OR 1.187; 95% CI 1.031-1.367; p = 0.017; OR 10.031; 95% CI 2.210-45.544; p = 0.003; OR 2.722; 95% CI 1.215-6.102; p = 0.015, respectively). Only eight CKD stage 5 patients underwent surgery related to a hostile bladder between 1 and 15 years old. CONCLUSION: MMC-related CKD is common in childhood in Turkey. A proactive approach to neurogenic bladder management and early protective surgery in selected cases where conservative treatment has failed should be implemented to prevent progressive kidney failure in the pediatric MMC population in our country.


Asunto(s)
Fallo Renal Crónico , Meningomielocele , Insuficiencia Renal Crónica , Vejiga Urinaria Neurogénica , Humanos , Niño , Femenino , Recién Nacido , Lactante , Preescolar , Adolescente , Adulto Joven , Adulto , Masculino , Meningomielocele/complicaciones , Meningomielocele/epidemiología , Estudios de Cohortes , Vejiga Urinaria Neurogénica/epidemiología , Vejiga Urinaria Neurogénica/etiología , Vejiga Urinaria Neurogénica/terapia , Estudios Retrospectivos , Diálisis Renal/efectos adversos , Insuficiencia Renal Crónica/diagnóstico , Insuficiencia Renal Crónica/epidemiología , Insuficiencia Renal Crónica/etiología , Fallo Renal Crónico/complicaciones
4.
Pediatr Transplant ; 27(2): e14444, 2023 03.
Artículo en Inglés | MEDLINE | ID: mdl-36447352

RESUMEN

BACKGROUND: This study aims to translate the Pediatric Transplant Rating Instrument (P-TRI) to conduct a validity and reliability study on Turkish children and define a cutoff value of this scale. METHOD: A total of 151 pediatric kidney transplant patients were included in the study. The files of the patients were reviewed by two clinicians, and the scale was filled for inter-rater reliability. One of the clinicians filled the scale again after one month for intra-rater reliability. Glomerular filtration rate (GFR) and creatinine values were used for predictive validity. A GFR below <60 ml/min/1.73 m2 and creatinine up to 3.0 mg/dl was defined as risk factors. RESULTS: Correlation of P-TRI with GFR (r = .252, p = .003) and creatinine (r = -.249, p = .002) was performed, and the internal consistency of the scale items as measured by Cronbach's alpha coefficient was found to be 0.825. When the test was performed again, the intra-class correlation coefficient was found as .922 for intra-rater reliability and as .798 for inter-rater reliability. For both creatinine and GFR, the best cutoff point for the total score was found to be 66.5. CONCLUSIONS: Patients who received P-TRI above 66.5 could be at risk in the post-transplant period. Identification of these patients before transplantation and following these young people more closely will aid in the prevention of serious consequences. The reliability and validity scores are satisfactory for use in transplantation clinics for psychosocial evaluation and compliance in Turkish pediatric renal transplantation patients.


Asunto(s)
Trasplante de Riñón , Trasplante de Órganos , Humanos , Niño , Adolescente , Creatinina , Reproducibilidad de los Resultados , Trasplante de Órganos/psicología , Tasa de Filtración Glomerular
5.
Pediatr Transplant ; 27(6): e14557, 2023 09.
Artículo en Inglés | MEDLINE | ID: mdl-37283242

RESUMEN

BACKGROUND: Literature supports the protective role of mineralocorticoid antagonist (MRA) against the renal injury induced by aldosterone in kidney transplant recipients. However, there is limited data available regarding the safety and efficacy of MRAs in pediatric renal transplant patients. Therefore, we aimed to investigate the effect of long-term eplerenone administration in children with chronic allograft nephropathy (CAN). METHODS: Twenty-six renal transplant children with biopsy-proven CAN, an estimated glomerular filtration rate (eGFR ) > 40 mL/min per 1.73 m2 and with a significant proteinuria were included. Selected patients were randomly divided into two groups as follows; Group 1 (n = 10) patients received 25 mg/day eplerenone and Group 2 (n = 16) patients did not receive eplerenone for 36 months. Patients were examined in the renal transplant outpatient clinic biweekly for the first month and once a month thereafter. The primary outcome of the patients was compared. RESULTS: Mean eGFR stayed stable in group 1 patients, but significantly decreased in group 2 at 36 months (57.53 ± 7.53 vs. 44.94 ± 8.04 mL/min per 1.73 m2 , p = .001). Similarly, spot protein-creatinine ratio was significantly lower in group 1 compared to group 2 patients at 36 months (1.02 ± 7.53 vs. 3.61 ± 0.53, p < .001). Eplerenone associated hyperkalemia was not observed in group 1 patients (4.6 ± 0.2 vs. 4.56 ± 0.3, p = .713). CONCLUSION: The long-term eplerenone administration blunted the chronic allograft nephropathy by maintaining a stable eGFR levels and decreasing urine protein-creatinine ratio. Eplerenone associated hyperkalemia was not observed in our study.


Asunto(s)
Hiperpotasemia , Espironolactona , Humanos , Niño , Eplerenona/uso terapéutico , Espironolactona/uso terapéutico , Espironolactona/farmacología , Creatinina , Antagonistas de Receptores de Mineralocorticoides/uso terapéutico , Antagonistas de Receptores de Mineralocorticoides/farmacología , Tasa de Filtración Glomerular , Aloinjertos
6.
Eur J Pediatr ; 181(9): 3339-3343, 2022 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-35789292

RESUMEN

Nutcracker syndrome related to the left kidney vein compression is a cause of orthostatic proteinuria during childhood. Some studies have shown that the ratios between maximum velocities and anterior-posterior diameters of hilar and aortomesenteric segments of the left kidney vein between upright and supine positions must be more than 4 in order to make a Nutcracker syndrome diagnosis. Our aim was to investigate whether the use of a decrease in aortomesenteric angle between upright and supine positions in the presence of isolated orthostatic proteinuria can be a criterion for the diagnosis of Nutcracker syndrome. Relevant patient information, which included demographic data, clinical examination findings, laboratory data, urinary system ultrasound, and kidney color flow Doppler ultrasound results, were prospectively collected. Thirty-nine pediatric patients with orthostatic proteinuria were included in the study. Left kidney vein compression findings were demonstrated in 31 patients. The ratio of maximum velocities of hilar and aortomesenteric segments of the left kidney vein between upright and supine positions was above 4 in only 7 of our patients. Ratio of aortomesenteric angle between upright and supine positions was significantly decreased for patients with left kidney vein compression findings.  Conclusion: The use of a decrease in the ratio of aortomesenteric angle between upright and supine positions in the presence of orthostatic proteinuria, instead of the ratios for maximum velocities and anterior-posterior diameters of hilar and aortomesenteric segments, can be more helpful for the diagnosis of Nutcracker syndrome in the differential diagnosis of orthostatic proteinuria. What is Known: • Proteinuria may be a sign of an impending kidney disease • Nutcracker syndrome is a cause of orthostatic proteinuria. What is New: • Ratio of aortomesenteric angle between upright and supine positions > 0.6 can be used for Nutcracker syndrome diagnosis.


Asunto(s)
Síndrome de Cascanueces Renal , Venas Renales , Niño , Humanos , Postura , Proteinuria/diagnóstico , Proteinuria/etiología , Síndrome de Cascanueces Renal/diagnóstico , Síndrome de Cascanueces Renal/diagnóstico por imagen , Venas Renales/diagnóstico por imagen , Ultrasonografía
7.
Eur J Pediatr ; 181(4): 1661-1667, 2022 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-35006379

RESUMEN

Familial Mediterranean fever (FMF) is an autoinflammatory disease characterized by recurrent attacks of fever and serositis. Diagnosis is made according to clinical findings and supported by genetic analysis. The most commonly used adult diagnostic criteria are the Tel-Hashomer criteria. Pediatric criteria for FMF diagnosis were described in 2009, but their reliability should be supported by additional reports. In this study, we aimed to compare the pediatric criteria and the Tel-Hashomer and 2019 Eurofever/PRINTO classification criteria using our FMF cohort. A total of 113 patients diagnosed with FMF were included. Demographic features and laboratory findings were retrospectively collected from the patients' files. The patients were evaluated with the Tel-Hashomer, pediatric and Eurofever/PRINTO classification criteria. At least two of five new pediatric criteria were as sensitive (89%) and specific (85%) as the Tel-Hashomer criteria (sensitivity 70%, specificity 96%). We also evaluated the Eurofever/PRINTO classification criteria using our cohort and found a sensitivity of 94% and specificity of 91%.   Conclusion: Using pediatric criteria for the diagnosis of FMF in children is a feasible and simple approach that can diagnose the disease based on at least two criteria. Therefore, our study supports the use of pediatric criteria in FMF diagnosis of children. Our results also confirm that the Eurofever/PRINTO classification criteria can be successfully applied for the diagnosis of FMF due to their high sensitivity (94%) and specificity (91%). What is Known: • The FMF diagnosis is made according clinical findings and supported by genetic analysis. • The use of adult diagnostic criteria in pediatric FMF patients is controversial since classical clinical presentation is often absent in children. What is New: • Our study supports both the use of pediatric criteria and Eurofever/PRINTO classification criteria in clinical practice.


Asunto(s)
Fiebre Mediterránea Familiar , Niño , Estudios de Cohortes , Fiebre Mediterránea Familiar/diagnóstico , Fiebre Mediterránea Familiar/genética , Fiebre , Humanos , Reproducibilidad de los Resultados , Estudios Retrospectivos
8.
Turk J Med Sci ; 52(6): 1762-1770, 2022 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-36945974

RESUMEN

BACKGROUND: There is limited data on COVID-19 disease in children with kidney disease. We aimed to investigate the characteristics and prognosis of COVID-19 in pediatric nephrology patients in Turkey. METHODS: This was a national, multicenter, retrospective cohort study based on an online survey evaluating the data between 11th March 2020 and 11th March 2021 as an initial step of a detailed pediatric nephrology COVID-19 registry. RESULTS: Two hundred and three patients (89 girls and 114 boys) were diagnosed with COVID-19. One-third of these patients (36.9%) were between 10-15 years old. Half of the patients were on kidney replacement therapy: kidney transplant (KTx) recipients (n = 56, 27.5%), patients receiving chronic hemodialysis (n = 33, 16.3%) and those on peritoneal dialysis (PD) (n = 18, 8.9%). Fifty-four (26.6%) children were asymptomatic. Eighty-two (40.3%) patients were hospitalized and 23 (28%) needed intensive care unit admission. Fifty-five percent of the patients were not treated, while the remaining was given favipiravir (20.7%), steroid (16.3%), and hydroxychloroquine (11.3%). Acute kidney injury developed in 19.5% of hospitalized patients. Five (2.4%) had MIS-C. Eighty-three percent of the patients were discharged without any apparent sequelae, while 7 (3.4%) died. One hundred and eight health care staff were infected during the study period. DISCUSSION: COVID-19 was most commonly seen in patients who underwent KTx and received HD. The combined immunosuppressive therapy and frequent exposure to the hospital setting may increase these patients' susceptibility. Staff infections before vaccination era were alarming, various precautions should be taken for infection control, particularly optimal vaccination coverage.


Asunto(s)
COVID-19 , Nefrología , Masculino , Niño , Femenino , Humanos , Adolescente , COVID-19/epidemiología , COVID-19/terapia , Turquía/epidemiología , Estudios Retrospectivos
10.
Pediatr Nephrol ; 33(8): 1395-1403, 2018 08.
Artículo en Inglés | MEDLINE | ID: mdl-29610995

RESUMEN

BACKGROUND: Atypical hemolytic uremic syndrome (aHUS) is a chronic disease characterized by thrombotic microangiopathy and a high risk of end-stage kidney disease. Dysregulation and/or excessive activation of the complement system results in thrombotic microangiopathy. Interest in extrarenal manifestations of aHUS is increasing. This study aimed to determine the clinical characteristics of patients with extrarenal manifestations of aHUS in childhood. METHODS: This study included 70 children with extrarenal manifestations of HUS from the national Turkish aHUS Registry. The demographics, clinical characteristics, genetic test results, all treatments, and renal/hematologic status of aHUS patients with extrarenal involvement were recorded. RESULTS: The most common extrarenal manifestation was neurological system involvement (n = 46 [27.2%]), followed by gastrointestinal (n = 20 [11.8%]), cardiovascular (n = 12 [7%]), and respiratory (n = 12 [7%]) involvement. The patients with neurological involvement had a higher mortality rate and a lower estimated glomerular filtration rate (eGFR) than the other patients at last follow-up. Eculizumab (with or without plasma exchange/plasma infusion) treatment increased the renal and hematologic recovery rates. CONCLUSIONS: The most common and serious extrarenal manifestation of aHUS is neurological involvement and treatment outcome findings presented herein are important to all relevant clinicians.


Asunto(s)
Síndrome Hemolítico Urémico Atípico/complicaciones , Enfermedades Cardiovasculares/epidemiología , Enfermedades del Sistema Nervioso Central/epidemiología , Enfermedades Gastrointestinales/epidemiología , Enfermedades Respiratorias/epidemiología , Adolescente , Anticuerpos Monoclonales Humanizados/uso terapéutico , Síndrome Hemolítico Urémico Atípico/genética , Síndrome Hemolítico Urémico Atípico/terapia , Enfermedades Cardiovasculares/diagnóstico , Enfermedades Cardiovasculares/etiología , Enfermedades Cardiovasculares/prevención & control , Enfermedades del Sistema Nervioso Central/diagnóstico , Enfermedades del Sistema Nervioso Central/etiología , Enfermedades del Sistema Nervioso Central/prevención & control , Niño , Preescolar , Femenino , Estudios de Seguimiento , Enfermedades Gastrointestinales/diagnóstico , Enfermedades Gastrointestinales/etiología , Enfermedades Gastrointestinales/prevención & control , Tasa de Filtración Glomerular , Humanos , Lactante , Masculino , Intercambio Plasmático , Pronóstico , Sistema de Registros/estadística & datos numéricos , Enfermedades Respiratorias/diagnóstico , Enfermedades Respiratorias/etiología , Enfermedades Respiratorias/prevención & control , Estudios Retrospectivos , Tasa de Supervivencia , Resultado del Tratamiento , Turquía/epidemiología
11.
Pediatr Transplant ; 21(2)2017 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-27900821

RESUMEN

DUSG is a useful diagnostic tool for the follow-up of renal transplant recipients. The measurement of intrarenal arterial RI by DUSG has been proven to predict short-term AF. The aim of the study was to evaluate the predictive value of DUSG performed during the early after RTx on long-term AF. Seventy patients were enrolled into study. DUSG was performed at third and seventh days after RTx. Patients were divided into two groups according to rate of recovery of graft function as patients with normal graft function and abnormal graft function. Although the RI values were correlated with the AF early after transplantation, they were not correlated with long-term AF. However, the rate of recovery of graft function at early period after RTx was correlated with creatinine level at first year and with glomerular filtration rate at first year and last visit. Although the RI has no predictive value for long-term AF, the rate of recovery of graft function at early post-transplantation period has predictive value for long-term AF; patients with higher RI values early after RTx should be followed carefully for the development of chronic allograft injury.


Asunto(s)
Trasplante de Riñón , Insuficiencia Renal/diagnóstico por imagen , Insuficiencia Renal/cirugía , Ultrasonografía Doppler , Adolescente , Adulto , Aloinjertos , Niño , Preescolar , Creatinina/análisis , Femenino , Estudios de Seguimiento , Tasa de Filtración Glomerular , Supervivencia de Injerto , Humanos , Inmunosupresores/uso terapéutico , Riñón/fisiopatología , Masculino , Valor Predictivo de las Pruebas , Insuficiencia Renal/fisiopatología , Resultado del Tratamiento , Resistencia Vascular , Adulto Joven
12.
Ren Fail ; 38(6): 899-905, 2016 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-27056252

RESUMEN

INTRODUCTION: To investigate autonomic nervous system function in enuretic children by performing ambulatory blood pressure monitor (ABPM) for 24 h. METHODS: Twenty-eight children ranging in age from 6 to 15 years with primary nocturnal enuresis and 27 age-matched healthy controls were enrolled and they get 24 h ABPM. Hypertension was defined as standard deviation score (SDS) > 1.64 (i.e., >95th percentile) adjusted for gender and height. Urinalysis, urine electrolyte levels, urinary culture, and urinary system ultrasound were carried out in all children. They have also requested to have a diary about daily fluid intake and urine volume. RESULTS: Although the mean 24-h and daytime diastolic blood pressure (BP) did not differ between the groups, systolic BP (SBP) was significantly higher in enuretic children (p < 0.05). The mean night-time SBP, DBP values, SDS and BP loads were found to be significantly higher than those in the controls (p < 0.01). A lack of nocturnal decrease was more prevalent in the enuretic children compared with the control subjects, the difference was statistically significant for DBP but not for SBP. Patients with elevated night-time BP load was found to have higher frequency of urinary incontinence per week as well as per night when compared with enuretic children with normal night-time BP load (r = 0.72, r = 0.69, p < 0.01, respectively). CONCLUSION: Subtle abnormalities of circadian BP regulation in enuretic children indicated by a selective elevation of nocturnal SBP, DBP, and MAP, and attenuated nocturnal dipping may reflect sympathetic hyper activation and its possible role in pathogenesis of enuresis.


Asunto(s)
Monitoreo Ambulatorio de la Presión Arterial , Ritmo Circadiano , Hipertensión/epidemiología , Enuresis Nocturna/epidemiología , Adolescente , Estudios de Casos y Controles , Niño , Femenino , Humanos , Masculino , Factores de Riesgo
13.
Pediatr Nephrol ; 30(5): 783-9, 2015 May.
Artículo en Inglés | MEDLINE | ID: mdl-25384530

RESUMEN

BACKGROUND: Disorders of complement regulation are the most important etiology of atypical hemolytic uremic syndrome (aHUS). Recent studies demonstrate that eculizumab is beneficial in long-term aHUS treatment. We present a series of children with aHUS resistant to/dependent on plasma exchange (PE) who were treated with eculizumab. METHODS: This was a retrospective study in which data were retrieved from the medical files of children who had received PE as treatment for aHUS. The data retrieved included age, sex, presenting symptoms, presence of diarrhea/vomiting, hospitalization duration, laboratory data on admission and follow-up, need for transfusion or dialysis, response to PE, response to eculizumab and outcome. RESULTS: Of the 15 children diagnosed with aHUS in 2011 and 2012 in our departments, ten were resistant to, or dependent on, plasma therapy and treated with eculizumab; these children were enrolled in the study. Three patients had relapses, and seven had a new diagnosis. Nine children had oliguria or anuria, and eight required dialysis. Hypertension was observed in six patients. Neurologic involvement developed in six patients, with the symptoms including seizures, loss of balance, vision loss and severe confusion. Five and five patients were resistant to and dependent on plasma therapy, respectively. Following the start of eculizumab treatment, all patients achieved full recovery of renal function and hematologic parameters. CONCLUSIONS: In our ten pediatric patients with aHUS who did not respond to PE, eculizumab was a lifesaving therapy and improved their quality of life. Early eculizumab use was a rescue therapy for renal function. Our results show that eculizumab is an effective treatment for aHUS. However, more studies are needed on the long-term efficacy and safety of eculizumab in children with aHUS and to determine the optimal duration of treatment.


Asunto(s)
Anticuerpos Monoclonales Humanizados/uso terapéutico , Síndrome Hemolítico Urémico Atípico/tratamiento farmacológico , Niño , Preescolar , Femenino , Humanos , Masculino , Estudios Retrospectivos
14.
Ren Fail ; 36(5): 663-5, 2014 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-24512186

RESUMEN

AIM: The importance of changes about platelet emphasized in most chronically diseases in recent years. Mean platelet volume (MPV) and platelet count can be used as a prognostic biomarker. In this study, clinical importance of the changes of MPV during active and remission phases in children with nephrotic syndrome was investigated. PATIENTS AND METHODS: Fifty-five children with nephrotic syndrome (30 females, 25 males) and 29 healthy children (18 females, 11 males) were included to the study. Patients were divided in two groups (steroid sensitive nephrotic syndrome and focal segmental glomerulosclerosis). Demographic characteristics of the patients, type of nephrotic syndrome were recorded and laboratory parameters in active and remission phases were evaluated. RESULTS: Mean platelet count in the patient group was significantly higher than control group. Mean platelet count of FSGS group was significantly higher than SSNS group. Mean MPV value was significantly lower in active period of nephrotic syndrome when compared with control group. A significant negative relation between mean MPV value and mean platelet count was found. Significant positive correlations between mean platelet count and mean total cholesterol and mean triglyceride levels were demonstrated. CONCLUSION: MPV in nephrotic syndrome patients can be an easy, cheap and simple method for determine the prognosis of the disease and steroid resistance.


Asunto(s)
Biomarcadores , Volúmen Plaquetario Medio , Síndrome Nefrótico/sangre , Adolescente , Estudios de Casos y Controles , Niño , Preescolar , Femenino , Humanos , Masculino , Síndrome Nefrótico/diagnóstico , Pronóstico
15.
Exp Clin Transplant ; 22(Suppl 1): 275-280, 2024 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-38385412

RESUMEN

OBJECTIVES: Solid-organ transplant recipients are at an increased risk of severe infections due to their immunosuppressed state. Despite the recommendation of routine screening and vaccination before transplant to mitigate this danger, vaccination rates in these patients are still below desirable levels. We aimed to investigate the prevalence of positive antibody rates for measles, mumps, rubella, and varicella among children who are candidates for renal transplant. MATERIALS AND METHODS: This retrospective study was conducted at a single center and included 144 pediatric kidney transplant patients for the past 7 years. We reviewed the medical records of all participants to evaluate their serologic status for measles, mumps, rubella, and varicella viruses before kidney transplant. RESULTS: In this study, 144 pediatric kidney transplant candidates (mean age 11.5 years, 56.9% male) were enrolled, and the most frequent causes of the chronic renal disease were congenital anomalies of the kidney and urinary tract and glomerular diseases (32.6%). Seropositivity rates for measles, mumps, rubella, and varicella were 59.0%, 31.9%, 46.5%, and 43.6%, respectively, and all patients who tested negative for antibodies were vaccinated before transplant. Younger age at transplant (OR = 0.909, 95% CI = 0.840-0.923; P = .017) and congenital anomalies of the kidney and urinary tract (OR = 3.46, 95% CI = 1.1548-7.735; P = .002) were significantly associated with increased measles seropositivity, although no significant associations were observed for the other viruses. CONCLUSIONS: We observed lower seropositivity rates for measles, mumps, rubella, and varicella in pediatric kidney transplant patients versus healthy children and other previous studies. It is essential to address these suboptimal rates to protect the health of these vulnerable patients. Future research should focus on targeted interventions to improve vaccination rates and outcomes in this population.


Asunto(s)
Varicela , Trasplante de Riñón , Sarampión , Paperas , Rubéola (Sarampión Alemán) , Vacunas Virales , Niño , Femenino , Humanos , Masculino , Anticuerpos Antivirales , Varicela/prevención & control , Herpesvirus Humano 3 , Sarampión/prevención & control , Vacuna contra el Sarampión-Parotiditis-Rubéola/administración & dosificación , Paperas/prevención & control , Estudios Retrospectivos , Rubéola (Sarampión Alemán)/prevención & control , Vacunas Atenuadas , Vacunas Virales/administración & dosificación
16.
Pediatr Nephrol ; 28(5): 827-30, 2013 May.
Artículo en Inglés | MEDLINE | ID: mdl-23389237

RESUMEN

BACKGROUND: Atypical hemolytic uremic syndrome (aHUS) is associated with defective regulation of the complement pathway. Neurological involvement is the most common extrarenal complication and represents a major cause of mortality and morbidity. CASE-DIAGNOSIS/TREATMENT: Two girls aged 11 and 6 years, respectively, developed aHUS and were treated immediately with plasma exchange (PE) and fresh frozen plasma infusion (PI). Although initial improvement in renal function was seen in both cases, the first patient showed progressing thrombotic microangiopathy (TMA) despite daily PE, and neurological manifestations (seizures, vision loss, loss of balance, and confusion) developed after 1 month. The second patient developed cerebral TMA (seizures, vision loss, and nystagmus) 6 days after initial presentation and remained unresponsive to PE/PI. Neurological symptoms were similar in both patients, even though they had different complement protein mutations. Treatment with eculizumab achieved complete control of neurological symptoms within 24 h and gradually normalized hematological and renal parameters in both children. CONCLUSIONS: Based on our two cases, we conclude that eculizumab is a rapid-acting, effective, and life-saving treatment for pediatric patients with aHUS and severe neurological involvement, which works by inhibiting complement-mediated TMA in the kidney and other organs, such as the brain.


Asunto(s)
Anticuerpos Monoclonales Humanizados/uso terapéutico , Síndrome Hemolítico-Urémico/tratamiento farmacológico , Enfermedades del Sistema Nervioso/tratamiento farmacológico , Síndrome Hemolítico Urémico Atípico , Niño , Activación de Complemento/efectos de los fármacos , Activación de Complemento/genética , Confusión/tratamiento farmacológico , Confusión/etiología , Femenino , Síndrome Hemolítico-Urémico/complicaciones , Síndrome Hemolítico-Urémico/diagnóstico , Síndrome Hemolítico-Urémico/genética , Síndrome Hemolítico-Urémico/inmunología , Humanos , Enfermedades del Sistema Nervioso/diagnóstico , Enfermedades del Sistema Nervioso/etiología , Enfermedades del Sistema Nervioso/inmunología , Examen Neurológico , Nistagmo Patológico/tratamiento farmacológico , Nistagmo Patológico/etiología , Equilibrio Postural/efectos de los fármacos , Convulsiones/tratamiento farmacológico , Convulsiones/etiología , Trastornos de la Sensación/tratamiento farmacológico , Trastornos de la Sensación/etiología , Resultado del Tratamiento , Trastornos de la Visión/tratamiento farmacológico , Trastornos de la Visión/etiología
17.
Exp Clin Transplant ; 21(4): 333-337, 2023 04.
Artículo en Inglés | MEDLINE | ID: mdl-32799785

RESUMEN

OBJECTIVES: Acute and chronic allograft rejection have been continuously an important obstacle in the follow-up of renal transplant recipients. During clinical management, several factors acting simultaneously result in acute rejection and chronic allograft nephropathy. Matrix metalloproteinases and tissue inhibitors of metalloproteinases are responsible for the organization of the extracellular matrix and play roles in cell proliferation and cellular invasion. Changes in matrix metalloproteinase expression levels have been reported to be associated with renal allograft rejection and interstitial fibrosis. In this study, we aimed to investigate functional polymorphisms of MMP2, MMP9, and TIMP2 genes in pediatric renal transplant recipients. MATERIALS AND METHODS: Our study included 68 kidney transplant recipients and 58 control patients. The kidney transplant recipient group was further divided into 2 subgroups: no graft rejection (n = 47) and graft rejection (n =21). MMP2 -735C >T (rs2285053), MMP2 -1306C >T (rs243865), MMP2 -1575G >A (rs243866), MMP9 c.-1562C >T (rs3918242), TIMP2 -418G >C (rs8179090), and TIMP2 303C > T (rs2277698) polymorphisms were analyzed with the use of polymerase chain reaction and restriction fragment-length polymorphism methods. Allele prevalence was compared with reference values of the control group, and Hardy-Weinberg equilibrium was tested. RESULTS: Mean ages were 16.7 ± 3.9 years for the study group and 14.8 ± 5.6 years for the control group. The mean follow-up time after transplant was 37.7 ± 7.9 months. We compared allele frequencies in the 2 groups and calculated a statistically significant difference in rs2285053, rs243865, rs243866, rs3918242, rs8179090, and rs2277698 polymorphism frequencies between the transplant recipients and control patients. When the transplant recipient group was compared in itself with regard to allograft rejection, all investigated polymorphisms except TIMP2 -418G >C (rs8179090) revealed a statistically significant difference between those with and without rejection (P < .05). CONCLUSIONS: Matrix metalloproteinases and their tissue inhibitors could be important predictive biological markers for the follow-up of kidney transplant recipients.


Asunto(s)
Trasplante de Riñón , Inhibidor Tisular de Metaloproteinasa-2 , Humanos , Niño , Adolescente , Adulto Joven , Adulto , Inhibidor Tisular de Metaloproteinasa-2/genética , Inhibidor Tisular de Metaloproteinasa-2/metabolismo , Trasplante de Riñón/efectos adversos , Metaloproteinasa 9 de la Matriz/genética , Receptores de Trasplantes , Metaloproteinasa 2 de la Matriz/genética , Metaloproteinasa 2 de la Matriz/metabolismo , Polimorfismo Genético , Aloinjertos , Polimorfismo de Nucleótido Simple , Genotipo
18.
Pediatr Transplant ; 16(2): 160-4, 2012 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-22321218

RESUMEN

HT is a frequent cardiovascular risk factor in liver transplant recipients. However, there are only few studies in the literature regarding the risk of HT in liver transplanted children. The aim of this study was to assess the 24 h BP profiles of liver transplanted patients and to compare the results with healthy children. ABPM was performed on 20 liver transplanted patients and 27 healthy children aged 7.1 ± 4.8 and 8.5 ± 2.9 yr, respectively. HT was defined as SDS > 1.64 (i.e., >95th percentile) adjusted for gender and height. The mean duration of post-transplant follow-up was 32 ± 19 months. Six (30%) patients were found to be hypertensive. The physiological nocturnal BP fall was attenuated significantly in the study group for diastolic BP (11.5 ± 6.1 mmHg vs. 17.7 ± 7.1 mmHg, p = 0.006). Specifically, the number of patients with high nighttime systolic and diastolic BP SDS (p = 0.02 and p = 0.004, respectively) as well as elevated nighttime systolic (p = 0.03) and diastolic (p = 0.003) BPLs was found to be significantly higher than those in the controls. Alteration of the "normal" circadian rhythm is very frequent in liver transplant recipients. Thus, it is recommended to perform ABPM on all liver transplanted children not to underdiagnose HT.


Asunto(s)
Trastornos Cronobiológicos/etiología , Hipertensión/etiología , Trasplante de Hígado , Complicaciones Posoperatorias , Adolescente , Monitoreo Ambulatorio de la Presión Arterial , Niño , Preescolar , Trastornos Cronobiológicos/diagnóstico , Femenino , Estudios de Seguimiento , Humanos , Hipertensión/diagnóstico , Hipertensión/epidemiología , Modelos Lineales , Masculino , Complicaciones Posoperatorias/diagnóstico , Complicaciones Posoperatorias/epidemiología
19.
Ren Fail ; 34(6): 792-4, 2012.
Artículo en Inglés | MEDLINE | ID: mdl-22462393

RESUMEN

Chronic renal failure (CRF) is associated with a high risk for hypertension. An individualized treatment should be initiated after the diagnosis of hypertension and underlying etiology. Many metabolic and endocrinal abnormalities are encountered in CRF. We present an 11-year-old boy with CRF developing galactorrhea and hyperprolactinemia associated with α-methyldopa, defective dopaminergic control, and resistance to multi-antihypertensive therapy. Cabergoline, a dopamine receptor agonist, was effectively used in the treatment of hypertension. It is important to remember that sometimes treatment of an illness becomes the cause of this illness.


Asunto(s)
Galactorrea/etiología , Hiperprolactinemia/complicaciones , Hipertensión/etiología , Fallo Renal Crónico/complicaciones , Diálisis Renal/efectos adversos , Niño , Galactorrea/tratamiento farmacológico , Humanos , Hiperprolactinemia/tratamiento farmacológico , Hipertensión/tratamiento farmacológico , Fallo Renal Crónico/terapia , Masculino
20.
Exp Clin Transplant ; 20(Suppl 3): 39-44, 2022 05.
Artículo en Inglés | MEDLINE | ID: mdl-35570598

RESUMEN

OBJECTIVES: The association between vitamin D deficiency and anemia is known. Vitamin D deficiency and anemia are common in kidney transplant recipients. We examined the relationship between vitamin D levels and anemia in pediatric kidney transplant recipients. MATERIALS AND METHODS: We reviewed retrospectively the data of 75 pediatric kidney transplant recipients (0-18 years of age). Patients were evaluated in 3 groups according to serum 25-hydroxyvitamin D levels (<20, 20-30, and >30 ng/mL) in the first year posttransplant: group 1 was the vitamin D deficiency group, group 2 was the vitamin D insufficiency group, and group 3 was normal vitamin D level group, respectively. Groups were compared in terms of anemia parameters, calcium, phosphorus, alkaline phosphatase, and parathyroid hormone levels, as well as infection, rejection, and graft loss status. All patients included in the study were grouped as those with anemia and without anemia, and the 2 groups were compared in terms of vitamin D levels, serum parathyroid hormone values, estimated glomerular filtration rate, and infection, rejection, and graft loss status. RESULTS: There were 41 patients (54.7%) in group 1, 24 patients (32%) in group 2, and 10 patients (13%) in group 3. There were 65 patients (86.7%) with vitamin D deficiency/insufficiency. When groups were compared, the hematocrit level was found to be lower in groups 1 and 2 (P < .05) and ferritin level was found to be lower in group 1 (P < .05). Anemia was present in 20 patients (26.6%): 61% of patients with anemia had vitamin D deficiency and 33% had vitamin D insufficiency (P > .05). In total, 94% of patients with anemia had vitamin D deficiency/insufficiency. CONCLUSIONS: Vitamin D deficiency/insufficiency is common in pediatric kidney transplant recipients. Vitamin D levels should be measured, especially in all kidney transplant recipients with persistent anemia. Thus, risk factors associated anemia can be reduced by treating the deficiency/insufficiency.


Asunto(s)
Anemia , Trasplante de Riñón , Deficiencia de Vitamina D , Adolescente , Anemia/diagnóstico , Anemia/epidemiología , Anemia/etiología , Niño , Preescolar , Humanos , Lactante , Recién Nacido , Trasplante de Riñón/efectos adversos , Hormona Paratiroidea , Estudios Retrospectivos , Receptores de Trasplantes , Resultado del Tratamiento , Vitamina D , Deficiencia de Vitamina D/complicaciones , Deficiencia de Vitamina D/diagnóstico , Deficiencia de Vitamina D/epidemiología
SELECCIÓN DE REFERENCIAS
DETALLE DE LA BÚSQUEDA