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1.
Pediatr Pulmonol ; 53(3): 358-365, 2018 03.
Artículo en Inglés | MEDLINE | ID: mdl-29327810

RESUMEN

INTRODUCTION: The efficacy of nebulized hypertonic saline (HS) therapy for shortening hospital length of stay (LOS) or improving bronchiolitic symptoms remains controversial. Most studies enrolled small numbers of subjects and did not consider the role of respiratory syncytial virus (RSV), the most common cause of acute bronchiolitis. Our aim was to evaluate the efficacy and safety of nebulized HS therapy for acute bronchiolitis due to RSV in moderately ill hospitalized infants. MATERIALS AND METHODS: This was an open-label, multicenter, randomized controlled trial comparing a nebulized HS treatment group with a normal saline (NS) group. The subjects, 128 infants with bronchiolitis due to RSV, were admitted to five hospitals in Tokyo, Japan. Three-percent HS or NS was administered via bronchodilator four times daily post-admission. The primary outcome was LOS, defined as the time until the patients fulfilled the discharge criteria, namely, absence of fever, no need for supplemental oxygen, and adequate feeding. Survival analysis was conducted in accordance with the intention-to-treat principle. RESULTS: The baseline characteristics were similar between the two groups. There was no significant overall difference in LOS between the groups (4.81 ± 2.14 days in HS vs 4.61 ± 2.18 days in NS; P = 0.60). Survival analysis by log-rank test also showed no significance (P = 0.62). Multivariate adjustment did not significantly alter the results. The treatment was well-tolerated, with no adverse effects attributable to the use of HS. CONCLUSIONS: Nebulized HS therapy did not significantly reduce LOS among infants with bronchiolitis due to RSV.


Asunto(s)
Bronquiolitis/tratamiento farmacológico , Infecciones por Virus Sincitial Respiratorio/tratamiento farmacológico , Solución Salina Hipertónica/administración & dosificación , Administración por Inhalación , Método Doble Ciego , Femenino , Humanos , Lactante , Tiempo de Internación , Masculino , Nebulizadores y Vaporizadores , Alta del Paciente
2.
Pediatr Nephrol ; 32(7): 1275-1278, 2017 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-28435991

RESUMEN

BACKGROUND: Low birthweight infants have a reduced number of nephrons and are at high risk of chronic kidney disease. Preterm birth and/or intrauterine growth restriction (IUGR) may also affect peritubular capillary development, as has been shown in other organs. CASE-DIAGNOSIS/TREATMENT: We report two patients with a history of preterm birth and extremely low birthweight who showed polycythemia and renal capillary rarefaction. Patient 1 and 2, born at 25 weeks of gestation with a birthweight of 728 and 466 g, showed mild proteinuria at age 8 and 6 years, respectively. In addition to increasing proteinuria, hemoglobin levels became elevated towards adolescence and their serum erythropoietin (EPO) was high despite polycythemia. Light microscopic examination of renal biopsy specimens showed glomerular hypertrophy, focal segmental glomerulosclerosis, and only mild tubulointerstitial fibrosis. A decrease in the immunohistochemical staining of CD31 and CD34 endothelial cells in renal biopsy specimens was consistent with peritubular capillary rarefaction. CONCLUSIONS: Since kidney function was almost normal and fibrosis was not severe, we consider that the capillary rarefaction and polycythemia associated with elevated EPO levels were largely attributable to preterm birth and/or IUGR.


Asunto(s)
Glomeruloesclerosis Focal y Segmentaria/patología , Enfermedades del Prematuro/patología , Glomérulos Renales/patología , Túbulos Renales/patología , Rarefacción Microvascular/patología , Nefronas/patología , Policitemia/patología , Nacimiento Prematuro/patología , Adolescente , Bloqueadores del Receptor Tipo 1 de Angiotensina II/uso terapéutico , Antígenos CD34 , Puntaje de Apgar , Biopsia , Niño , Células Endoteliales/metabolismo , Eritropoyetina/sangre , Femenino , Fibrosis , Glomeruloesclerosis Focal y Segmentaria/diagnóstico , Glomeruloesclerosis Focal y Segmentaria/terapia , Glomeruloesclerosis Focal y Segmentaria/orina , Hemoglobinas/análisis , Humanos , Recien Nacido Extremadamente Prematuro , Recién Nacido , Enfermedades del Prematuro/diagnóstico , Enfermedades del Prematuro/terapia , Enfermedades del Prematuro/orina , Recién Nacido de muy Bajo Peso , Masculino , Rarefacción Microvascular/sangre , Rarefacción Microvascular/diagnóstico , Rarefacción Microvascular/terapia , Molécula-1 de Adhesión Celular Endotelial de Plaqueta/metabolismo , Policitemia/sangre , Policitemia/diagnóstico , Policitemia/orina , Embarazo , Proteinuria/orina , Valsartán/uso terapéutico
3.
Am J Med Genet A ; 167A(3): 592-601, 2015 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-25691411

RESUMEN

6p duplication syndrome is a rare chromosomal disorder that frequently manifests renal complications, including proteinuria, hypoplastic kidney, and hydronephrosis. We report a girl with the syndrome, manifesting left hydronephrosis, proteinuria/hematuria, and focal segmental glomerular sclerosis (FSGS) resulting in chronic end-stage renal failure, successfully treated with renal transplantation. Microarray comparative genomic hybridization showed the derivative chromosome 6 to have a 6.4-Mb duplication at 6p25.3-p25.1 with 32 protein-coding genes and a 220-Kb deletion at 6p25.3 with two genes of no possible relation to the renal pathology. Review of the literature shows that variation of renal complications in the syndrome is compatible with congenital anomalies of the kidney and urinary tract (CAKUT). FSGS, observed in another patient with 6p duplication syndrome, could be a non-coincidental complication. FOXC1, located within the 6.4-Mb duplicated region at 6p25.3-p25.2, could be a candidate gene for CAKUT, but its single gene duplication effect would not be sufficient. FSGS would be a primary defect associated with duplicated gene(s) albeit no candidate could be proposed, or might occur in association with CAKUT.


Asunto(s)
Glomeruloesclerosis Focal y Segmentaria/genética , Hidronefrosis/genética , Proteinuria/genética , Trisomía , Anomalías Múltiples/diagnóstico , Anomalías Múltiples/genética , Biopsia , Niño , Bandeo Cromosómico , Cromosomas Humanos Par 6 , Hibridación Genómica Comparativa , Facies , Femenino , Estudio de Asociación del Genoma Completo , Glomeruloesclerosis Focal y Segmentaria/diagnóstico , Humanos , Hidronefrosis/diagnóstico , Riñón/anomalías , Riñón/patología , Proteinuria/diagnóstico , Síndrome , Ultrasonografía , Sistema Urinario/anomalías
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