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BACKGROUND: Focal segmental glomerulosclerosis (FSGS) in pediatric patients is typically difficult to treat and will progress to end-stage renal disease (ESRD) in about 10% of cases. Following kidney transplantation, FSGS can recur in up to 56% of renal allografts-with a near 100% recurrence in subsequent transplants. METHODS: Four different pediatric centers across the USA and the UK employed a protocol using LDL-apheresis (LDL-A) and pulse solumedrol to treat recurrent FSGS after transplantation in seven patients. All the patients included in this series demonstrated immediate, or early, recurrence of FSGS, which clinically presented as nephrotic-range proteinuria within hours to days after implantation of the kidney. RESULTS: All patients experienced reductions in urinary protein to creatinine ratios resulting in partial or complete remission. All patients demonstrated improvements in their estimated GFRs at their most recent follow-up since LDL-A discontinuation. CONCLUSIONS: This case series describes the successful treatment, across four different pediatric centers, of seven pediatric patients with recurrent post-transplant FSGS using the Liposorber® LA-15 in combination with pulse solumedrol.
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Eliminación de Componentes Sanguíneos/métodos , Glomeruloesclerosis Focal y Segmentaria/terapia , Trasplante de Riñón , Lipoproteínas LDL/sangre , Hemisuccinato de Metilprednisolona/administración & dosificación , Proteinuria/terapia , Aloinjertos/patología , Niño , Preescolar , Terapia Combinada/métodos , Creatinina/orina , Progresión de la Enfermedad , Femenino , Estudios de Seguimiento , Tasa de Filtración Glomerular , Glomeruloesclerosis Focal y Segmentaria/sangre , Glomeruloesclerosis Focal y Segmentaria/diagnóstico , Glomeruloesclerosis Focal y Segmentaria/patología , Humanos , Lactante , Fallo Renal Crónico/cirugía , Glomérulos Renales/patología , Masculino , Proteinuria/sangre , Proteinuria/diagnóstico , Proteinuria/patología , Quimioterapia por Pulso , Recurrencia , Inducción de Remisión/métodos , Estudios Retrospectivos , Resultado del TratamientoRESUMEN
Cystic kidney disease includes a wide range of hereditary, developmental, and acquired conditions of the kidneys. Some of the inherited causes of cystic kidney disease include autosomal dominant polycystic kidney diseases (caused by mutations in PKD1 or PKD2), autosomal recessive polycystic kidney disease, tuberous sclerosis complex, von Hippel-Lindau disease, oral-facial-digital syndrome type I, and Hadju-Cheney syndrome. Acquired cystic kidney disease has been reported in patients receiving long-term hemodialysis or peritoneal dialysis and in children after liver transplantation. Acute kidney injury can occur in patients with neuroblastoma, usually as a result of thrombotic microangiopathy associated with bone marrow transplantation. End-stage renal disease is described in long-term survivors. However, in this case report, we provide what is to our knowledge the first description of multiple kidney cysts in long-term survivors of stage IV neuroblastoma. None of the 7 patients we describe with neuroblastoma and multiple kidney cysts had a family history of autosomal dominant polycystic kidney disease. Also, all lacked stigmata of tuberous sclerosis complex, von Hippel-Lindau disease, or Hadju-Cheney syndrome. Two patients progressed to end-stage renal disease; in addition, one of them developed an oncocytoid renal cell carcinoma.
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Enfermedades Renales Quísticas/etiología , Neoplasias Renales , Neuroblastoma , Adolescente , Niño , Preescolar , Femenino , Estudios de Seguimiento , Humanos , Neoplasias Renales/terapia , Masculino , Neuroblastoma/terapia , Estudios Retrospectivos , Sobrevivientes , Adulto JovenRESUMEN
BACKGROUND: Chronic kidney disease is strongly linked to neurocognitive deficits in adults and children, but the pathophysiologic processes leading to these deficits remain poorly understood. The NiCK study (Neurocognitive Assessment and Magnetic Resonance Imaging Analysis of Children and Young Adults with Chronic Kidney Disease) seeks to address critical gaps in our understanding of the biological basis for neurologic abnormalities in chronic kidney disease. In this report, we describe the objectives, design, and methods of the NiCK study. DESIGN/METHODS: The NiCK Study is a cross-sectional cohort study in which neurocognitive and neuroimaging phenotyping is performed in children and young adults, aged 8 to 25 years, with chronic kidney disease compared to healthy controls. Assessments include (1) comprehensive neurocognitive testing (using traditional and computerized methods); (2) detailed clinical phenotyping; and (3) multimodal magnetic resonance imaging (MRI) to assess brain structure (using T1-weighted MRI, T2-weighted MRI, and diffusion tensor imaging), functional connectivity (using functional MRI), and blood flow (using arterial spin labeled MRI). Primary analyses will examine group differences in neurocognitive testing and neuroimaging between subjects with chronic kidney disease and healthy controls. Mechanisms responsible for neurocognitive dysfunction resulting from kidney disease will be explored by examining associations between neurocognitive testing and regional changes in brain structure, functional connectivity, or blood flow. In addition, the neurologic impact of kidney disease comorbidities such as anemia and hypertension will be explored. We highlight aspects of our analytical approach that illustrate the challenges and opportunities posed by data of this scope. DISCUSSION: The NiCK study provides a unique opportunity to address key questions about the biological basis of neurocognitive deficits in chronic kidney disease. Understanding these mechanisms could have great public health impact by guiding screening strategies, delivery of health information, and targeted treatment strategies for chronic kidney disease and its related comorbidities.
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Encefalopatías/psicología , Encéfalo/patología , Trastornos Cerebrovasculares/psicología , Trastornos del Conocimiento/psicología , Insuficiencia Renal Crónica/psicología , Adolescente , Adulto , Encefalopatías/complicaciones , Encefalopatías/patología , Estudios de Casos y Controles , Circulación Cerebrovascular , Trastornos Cerebrovasculares/complicaciones , Trastornos Cerebrovasculares/patología , Niño , Trastornos del Conocimiento/complicaciones , Trastornos del Conocimiento/patología , Estudios de Cohortes , Estudios Transversales , Imagen de Difusión por Resonancia Magnética , Femenino , Neuroimagen Funcional , Humanos , Imagen por Resonancia Magnética , Masculino , Pruebas Neuropsicológicas , Insuficiencia Renal Crónica/complicaciones , Insuficiencia Renal Crónica/fisiopatología , Adulto JovenRESUMEN
Hypertension is an established risk factor for subsequent cardiovascular and renal disease in children as well as adults. Sickle cell disease (SCD) is a genetic disorder associated with chronic hemolytic anemia with the major manifestation of vaso-occlusive crises. Although this disease entity involves most organ systems causing vascular and pulmonary injury, little is known about blood pressure (BP) levels or prevalence of hypertension in children with SCD. A cross-sectional study was conducted on 56 children with SCD (54 with hemoglobin SS disease; 2 with hemoglobin Sß0 thalassemia; 29 females). Study participants underwent 24-hour ambulatory BP monitoring (ABPM). Serum creatinine and cystatin C were obtained to assess estimated glomerular filtration rate with age-based formulas. A random urine sample was obtained to estimate urine osmolality and urine albumin to creatinine ratio. Mean age range was 11.9 (±4.5) years. Seventeen participants (30%) met criteria for hypertension based on ABPM. Of the 17 participants classified with hypertension, three had office hypertension with ambulatory hypertension, and 14 had masked hypertension detected on ABPM. Another 28 participants (50%) had some abnormal ABPM parameters in the form of either prehypertension and/or lack of normal nocturnal dipping status. The prevalence of confirmed hypertension, largely manifest by masked hypertension, is high in children, as young as 6 years of age with SCD. Early identification of hypertension in SCD children can confer benefit as it is an important modifiable risk factor for progression of cardiovascular and renal disease.
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OBJECTIVE: To compare behavior ratings of executive functioning in individuals with chronic kidney disease (CKD), using the Behavior Rating Inventory for Executive Functions (BRIEF), with a typically developing comparison group and to examine the correlation between disease severity and ratings of executive functioning. METHODS: Participants included 92 individuals with CKD (eGFR < 90 mL/min per 1.73 m), aged 8 to 25 years, recruited from nephrology clinics in both hospital and community settings. The disease severity ranged from CKD Stage II to V. The BRIEF was completed by parents for individuals younger than 18 years of age and the BRIEF-Adult was completed by individuals who were older than 18. RESULTS: For individuals with CKD younger than 18 years of age, the parent-reported BRIEF revealed significant group differences when compared with controls on the Metacognition Index and the individual scales of Initiate, Working Memory, and Plan/Organize. A large proportion of individuals with CKD were rated as being at-risk for executive dysfunction. For the individuals of 18 years of age and older, there were no significant group differences. The relationship between BRIEF ratings and disease severity was limited to a few scales across both versions of the BRIEF. CONCLUSION: This study supported the presence of executive dysfunction through a parent report, although the level of impairment was mild and its association with disease severity was related to select executive functions. Few difficulties were reported by older adolescents and young adults with CKD. It will be important for developmental-behavioral pediatricians to be cognizant of the level and pattern of executive function capabilities in their patients with CKD, and possible discrepancies with parent reports, so as to facilitate their management and transition planning.
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Función Ejecutiva , Insuficiencia Renal Crónica/psicología , Adolescente , Adulto , Estudios de Casos y Controles , Niño , Femenino , Tasa de Filtración Glomerular , Humanos , Masculino , Pruebas Neuropsicológicas , Índice de Severidad de la Enfermedad , Adulto JovenRESUMEN
CKD has been linked with cognitive deficits and affective disorders in multiple studies. Analysis of structural and functional neuroimaging in adults and children with kidney disease may provide additional important insights into the pathobiology of this relationship. This paper comprehensively reviews neuroimaging studies in both children and adults. Major databases (PsychLit, MEDLINE, WorldCat, ArticleFirst, PubMed, Ovid MEDLINE) were searched using consistent search terms, and studies published between 1975 and 2012 were included if their samples focused on CKD as the primary disease process. Exclusion criteria included case reports, chapters, and review articles. This systematic process yielded 43 studies for inclusion (30 in adults, 13 in children). Findings from this review identified several clear trends: (1) presence of cerebral atrophy and cerebral density changes in patients with CKD; (2) cerebral vascular disease, including deep white matter hyperintensities, white matter lesions, cerebral microbleeds, silent cerebral infarction, and cortical infarction, in patients with CKD; and (3) similarities in regional cerebral blood flow between patients with CKD and those with affective disorders. These findings document the importance of neuroimaging procedures in understanding the effect of CKD on brain structure, function, and associated behaviors. Results provide a developmental linkage between childhood and adulthood, with respect to the effect of CKD on brain functioning across the lifespan, with strong implications for a cerebrovascular mechanism contributing to this developmental linkage. Use of neuroimaging methods to corroborate manifest neuropsychological deficits or perhaps to indicate preventive actions may prove useful to individuals with CKD.