RESUMEN
OBJECTIVE: We report a case of laparoscopic nephrectomy of a cross-fused ectopic kidney in a 4-year-old girl with renal hypertension and Fanconi anemia. MATERIALS AND METHODS: We performed a transperitoneal laparoscopy. Ectopic kidney resection was done after dissection of the pathological kidney and after clamping vessels, using an ultrasonic device. Hospitalization time was 4 days. RESULTS: At 6 months, blood pressure was normalized and the patient showed an adequate growth curve. CONCLUSION: The transperitoneal route is very effective when a nephrectomy is necessary. It offers perfect exposure with limited risk of complications.
Asunto(s)
Anemia de Fanconi/complicaciones , Hipertensión Renal/etiología , Riñón/anomalías , Laparoscopía/métodos , Nefrectomía/métodos , Preescolar , Femenino , Humanos , Hipertensión Renal/cirugía , Riñón/irrigación sanguínea , Riñón/cirugía , Imagen por Resonancia MagnéticaRESUMEN
The aim of this study was to develop a population pharmacokinetic model of tacrolimus in pediatric kidney transplant patients, identify factors that explain variability, and determine dosage regimens. Pharmacokinetic samples were collected from 50 de novo pediatric kidney transplant patients (age 2-18 years) who were on tacrolimus treatment. Population pharmacokinetic analysis of tacrolimus was performed using NONMEM, and the impact of variables (demographic and clinical factors, and CYP3A4-A5, ABCB1, and ABCC2 polymorphisms) was tested. The pharmacokinetic data were described by a two-compartment model that incorporated first-order absorption and lag time. The apparent oral clearance (CL/F) was significantly related to body weight (allometric scaling); in addition, it was higher in patients with low hematocrit levels and lower in patients with CYP3A5*3/*3. The population pharmacokinetic-pharmacogenetic model developed in de novo pediatric kidney transplant patients demonstrated that, in children, tacrolimus dosage should be based on weight, hematocrit levels, and CYP3A5 polymorphism. Individualization of therapy will enable the optimization of tacrolimus exposure, with resultant beneficial effects on kidney function in the initial post-transplantation period.
Asunto(s)
Citocromo P-450 CYP3A/genética , Inmunosupresores/farmacocinética , Trasplante de Riñón , Modelos Biológicos , Polimorfismo Genético , Tacrolimus/farmacocinética , Subfamilia B de Transportador de Casetes de Unión a ATP , Miembro 1 de la Subfamilia B de Casetes de Unión a ATP/genética , Miembro 1 de la Subfamilia B de Casetes de Unión a ATP/metabolismo , Adolescente , Factores de Edad , Peso Corporal , Niño , Preescolar , Citocromo P-450 CYP3A/metabolismo , Cálculo de Dosificación de Drogas , Monitoreo de Drogas , Quimioterapia Combinada , Femenino , Francia , Hematócrito , Humanos , Inmunosupresores/administración & dosificación , Masculino , Proteína 2 Asociada a Resistencia a Múltiples Medicamentos , Proteínas Asociadas a Resistencia a Múltiples Medicamentos/genética , Proteínas Asociadas a Resistencia a Múltiples Medicamentos/metabolismo , Reproducibilidad de los Resultados , Tacrolimus/administración & dosificación , Resultado del TratamientoRESUMEN
Hyper-IgD and periodic fever syndrome (HIDS) is a hereditary autoinflammatory syndrome, characterized by recurrent inflammatory attacks. Treatment of HIDS is difficult. Recently, the IL-1ra analogue anakinra was reported to be successful in aborting the IgD inflammatory attacks in a vaccination model. We report a clinical case of spectacular reduction of febrile attacks in a severe HIDS patient.