No abstract

To describe the psychological profile of renal transplant adolescents compared to healthy peers and to adolescents with CKD, three groups of adolescents aged 12-18 yr were selected: TX, CX, and adolescents with CKD. Psychiatric symptoms and disorders were evaluated through direct interviews (K-SADS-PL) and self-report questionnaires (YSR and CBCL). Forty TX (14 LRD and 26 DD transplant recipients), 40 CX and 20 CKD were included. Twelve of 40 (30%) TX, three of 20 (15%) CKD, and three of 40 (7.5%) CX had a history of learning difficulties (p = 0.03). Compared to CX, TX had lower total YSR competencies score (p = 0.028) and lower total CBCL competencies score (p = 0.003). Twenty-six of 40 (65%) TX, 12 of 20 (60%) CKD and 15 of 40 (37.5%) CX (p = 0.038) met DSM-IV diagnostic criteria for lifetime psychiatric disorder, with rates of depressive disorder of 35% among TX and CKD compared to 15.2% among CX (p = 0.043). Eight of 40 (20%) TX had a history of simple phobia. Nine of 40 (22.5%) TX met diagnostic criteria for ADHD as compared to one of 20 (5%) CKD and three of 40 (7.5%) CX. In the TX group, we found no significant differences in educational and psychiatric variables between LRD and DD. In conclusion, we found a high prevalence of psychiatric morbidity (depression, phobia, ADHD), educational impairment and social competence problems in the TX group. CKD scored in between TX and CX on most measures.

No abstract

Maple syrup urine disease (MSUD, MIM 248600) can be complicated by metabolic crises necessitating extracorporeal removal therapy (ECRT). Since leucine levels are usually not immediately available during therapy, an accurate kinetic model of leucine plasma levels during removal would be useful to establish the duration of ECRT. Such a kinetic model is available for neonates undergoing continuous ECRT (CECRT) with a leucine clearance>or=35 ml min-1 1.73 m-2. The current study tests the validity of this model in older children. Plasma leucine levels were obtained from eleven ECRT sessions [seven CECRT and four intermittent hemodialysis (HDi) sessions] in seven children aged 1-14 years. No hemodynamic instability or neurological complications were observed during treatment. HDi provided a higher leucine clearance and required shorter sessions than CECRT (5.4+/-0.6 vs. 17.1+/-6.0 h). All patients regained precrisis neurological status except for one patient who had severe neurological damage (severe cerebral edema) at the time of dialysis and subsequently died despite efficient leucine removal. A leucine clearance>or=50 ml min-1 1.73 m-2 is required to obtain a kinetic model similar to that reported in neonates, both with CECRT and HDi. This model should be helpful in predicting the duration of therapy needed to attain desired leucine levels.

Based on the variables identified above, further studies are needed to determine whether the inflammatory response of the host represents only a marker of the severity of gastrointestinal infection or whether, alternatively, it is a pathophysiologic factor that leads to HUS.