Prior studies suggested that women with CKD have higher risk for cardiovascular disease (CVD) and mortality than men, although putative mechanisms for this higher risk have not been identified. We ...assessed sex differences in (1) CVD risk factors and left ventricular hypertrophy (LVH), and (2) the relationship of left ventricular mass (LVM) with different measures of body size in children with CKD.
The study population comprised 681 children with CKD from the Chronic Kidney Disease in Children cohort, contributing 1330 visits. CVD risk factors were compared cross-sectionally by sex. LVH was defined as LVM/height
>95th percentile and LVM relative to estimated lean body mass (eLBM) >95th percentile for age and sex. Differences in LVM by sex were assessed by adjusting for age, weight, height, and eLBM using bivariate and multivariate regression models.
Girls were less likely to have uncontrolled hypertension (26% versus 38%, P=0.001), had lower diastolic BP z-scores (+0.3 versus +0.6, P=0.001), and had lower prevalence of high triglycerides (38% versus 47%, P=0.03) compared with boys. When LVH was defined by LVM indexed to height, girls had higher prevalence of LVH (16% versus 9%, P=0.01); when LVH was defined by LVM relative to eLBM, prevalence of LVH was similar between girls and boys (18% versus 17%, P=0.92). In regression models adjusting for eLBM, no sex differences in LVM were observed.
Despite lack of increased prevalence of CVD risk factors, indexing LVM to height showed a higher proportion of LVH among girls, while estimates of LVH based on eLBM showed no sex differences. Indexing LVM to eLBM may be an alternative to height indexing in children with CKD.
Adult solid organ transplant (SOT) recipients commonly develop advanced kidney disease; however, the burden of end-stage kidney disease (ESKD) in children after SOT is not well-described. The ...objectives of this study were to determine the incidence of ESKD after pediatric SOT and the relative risk by SOT type.
Retrospective multicenter cohort study of children, ages ≤ 18 years, who received SOTs from 1990 through 2010 using Scientific Registry of Transplant Recipients data linked to the US Renal Data System. We performed a competing risks analysis to determine cumulative incidence of ESKD (chronic dialysis or kidney transplant), treating death as a competing risk, and fit a multivariable Cox regression model to assess hazard of ESKD by organ type.
The cohort included 16,604 pediatric SOT recipients (54% liver, 34% heart, 6% lung, 6% intestine, and 1% heart-lung). During a median follow-up of 6.2 years (interquartile range 2.2-12.1), 426 (3%) children developed ESKD. Compared with liver transplant recipients, in whom the incidence of ESKD was 2.1 cases per 1000 person-years, in adjusted analyses the highest risk of ESKD was among intestinal (hazard ratio HR 7.37, P < .001), followed by lung (HR 5.79, P < .001) and heart transplant recipients (HR 1.79, P < .001).
In a 20-year national cohort of pediatric SOT recipients, the risk of ESKD was highest among intestinal and lung transplant recipients. The burden of earlier stages of chronic kidney disease is probably much higher; modifiable risk factors should be targeted to prevent progressive kidney damage in this high-risk population.
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.
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.
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.
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.
A 17-year-old girl recently diagnosed with systemic lupus erythematosus (SLE) presented to the Emergency Room with acute onset of psychosis. A variety of potential etiologies, including those related ...to SLE, to the treatment of her SLE, or to another psychiatric condition, are discussed. In addition, the work-up and management decisions for this patient are reviewed in detail.
Summary
Limited organ supply has led to greater use of liver allografts with higher donor risk indices (DRI) and/or donated after cardiac death (DCD). DCD status is associated with acute kidney ...injury after liver transplantation; however, less is known about the association between donor quality and end‐stage renal disease (ESRD). Using SRTR data, we assembled a cohort of liver transplant recipients from 2/2002 to 12/2010. We fit multivariable Cox regression models for ESRD. Model 1 included total DRI; model 2 included components of DRI, including DCD, as separate variables. Forty thousand four hundred and sixty‐three liver transplant recipients were included. Median DRI was 1.40 (IQR 1.14, 1.72); 1822 (5%) received DCD livers. During median follow‐up of 3.93 years, ESRD occurred in 2008 (5%) and death in 11 075 (27%) subjects. There was a stepwise increase in ESRD risk with higher DRI (DRI ≥1.14 and <1.40: HR 1.17, P = 0.06; DRI ≥1.40 and <1.72: HR 1.29, P = 0.003; DRI ≥1.72: HR 1.39, P < 0.001, compared with DRI <1.14). Adjusting for DRI components separately, DCD status was most strongly associated with ESRD (HR 1.40, P = 0.008). Higher DRI is associated with ESRD after liver transplantation, driven in part by DCD status. Donor quality is an important predictor of long‐term renal outcomes in liver transplant recipients.
Background
Tyrosine kinase (TK) inhibitors are increasingly being used to treat a variety of pediatric malignancies. Reports in adult patients describe a range of effects of TK inhibitors on the ...kidney, including hypertension, proteinuria, acute kidney injury, and thrombotic microangiopathy (TMA); however, there are only a few reports of TK-inhibitor-associated nephrotic syndrome.
Methods
We report four pediatric patients with various malignancies (chronic myelogenous leukemia, acute lymphoblastic leukemia, and glioma/renal cell carcinoma) who developed nephrotic syndrome during treatment with TK inhibitors (imatinib, sunitinib, dasatinib, and quizartinib). One of the four patients also had clinical features of TMA.
Results
Three of the four patients achieved complete remission of nephrotic syndrome with discontinuation of the TK inhibitor and have had no additional nephrotic syndrome relapses to date. The temporal relationship of nephrotic syndrome onset to TK-inhibitor therapy and resolution of nephrotic syndrome with cessation of therapy strongly imply an association in these patients.
Conclusions
TK inhibitors are important therapies in pediatric cancer, and their use is expanding. Nephrotic syndrome with or without features of TMA is a potential complication of these therapies in children.
The natural history and survival of children with fibrocystic liver–kidney disease undergoing solid organ transplantation have infrequently been described. We report outcomes in a cohort of US ...children with fibrocystic liver–kidney disease receiving solid organ transplants over 20 yr. Retrospective cohort study of pediatric transplant recipients with diagnoses of fibrocystic liver–kidney disease from 1/1990 to 3/2010, using data from the SRTR. Subjects were categorized by the first transplanted organ: LT, KT, or SLK. Primary outcomes were death, re‐transplant, transplant of the alternate organ, or initiation of dialysis. Seven hundred and sixteen subjects were transplanted in this period. Median age at first transplant was 9.7 yr. Of the LT, 14 (19%) required a second liver transplant at median of 0.2 yr, and five (7%) required kidney transplant or dialysis at a median of 9.0 yr. Of the KT, 188 (31%) required a second kidney transplant or dialysis at a median of 5.9 yr. Twenty‐nine (5%) subsequently received liver transplant at a median of 6.0 yr. Among patients in this registry, far more children underwent kidney than liver transplants. The risk of subsequently needing transplantation of an alternate organ was low.
Atypical hemolytic uremic syndrome (HUS) refers to the triad of microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury in the absence of Shiga toxin-producing
Escherichia coli
...exposure or
Streptococcus pneumoniae
infection. Currently, approximately 50 % of the atypical cases have demonstrable mutations in complement regulatory proteins. Historically, the diagnosis of atypical HUS portends a poor prognosis with a high rate of disease recurrence, progression to end-stage renal disease, and death. However, it is now evident that atypical HUS actually encompasses a heterogeneous group of disorders, and there are reports suggesting that some cases of atypical HUS have a favorable prognosis, similar to that of diarrhea-associated disease. We present three patients with the atypical HUS phenotype who had complete renal recovery and no disease recurrence. We believe it is important to distinguish those cases of atypical HUS associated with disorders of complement regulatory proteins from other idiopathic causes of nondiarrheal HUS given the implications for prognosis and treatment.
Conclusion
: Given the heterogeneous nature and variable prognosis of atypical HUS, treatment should be carefully considered prior to the use of long-term plasma therapy and/or eculizumab.
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