The Study of the Epidemiology of Pediatric Hypertension Registry (SUPERHERO): Rationale and Methods.

Despite increasing prevalence of hypertension in youth and high adult cardiovascular mortality rates, the long-term consequences of youth-onset hypertension remain unknown. This is due to limitations of prior research such as small sample sizes, reliance on manual record review, and limited analytic methods that did not address major biases. The Study of the Epidemiology of Pediatric Hypertension (SUPERHERO) is a multisite retrospective Registry of youth evaluated by subspecialists for hypertension disorders. Sites obtain harmonized electronic health record data using standardized biomedical informatics scripts validated with randomized manual record review. Inclusion criteria are index visit for International Classification of Diseases Diagnostic Codes, 10th Revision (ICD-10 code)-defined hypertension disorder ≥January 1, 2015 and age <19 years. We exclude patients with ICD-10 code-defined pregnancy, kidney failure on dialysis, or kidney transplantation. Data include demographics, anthropomorphics, U.S. Census Bureau tract, histories, blood pressure, ICD-10 codes, medications, laboratory and imaging results, and ambulatory blood pressure. SUPERHERO leverages expertise in epidemiology, statistics, clinical care, and biomedical informatics to create the largest and most diverse registry of youth with newly diagnosed hypertension disorders. SUPERHERO's goals are to (i) reduce CVD burden across the life course and (ii) establish gold-standard biomedical informatics methods for youth with hypertension disorders.

Kidney transplantation in children with decreased left ventricular systolic function: a Midwest Pediatric Nephrology Consortium study.

BACKGROUND: Left ventricular (LV) systolic dysfunction is a relatively uncommon but serious complication of pediatric chronic kidney disease, and may be related to uremia and uncontrolled hypertension. There is limited information on the strategy for managing these children. In some cases, combined heart-kidney transplant may be considered or kidney transplant delayed until cardiac function improves. It is unknown whether these patients are at increased risk for poor outcomes after kidney transplantation. METHODS: We conducted a retrospective, multicenter study on the outcomes of children with severe and symptomatic cardiomyopathy who underwent kidney transplantation. RESULTS: Eleven patients receiving maintenance dialysis with systolic dysfunction underwent kidney transplantation without simultaneous heart transplant. Nine patients had congestive heart failure in the pre-transplant period. There were no identified complications post-transplant related to the underlying cardiac dysfunction. LV systolic function normalized in all patients and the mean shortening fraction increased from 19.0 ± 4.6 % to 32.0 ± 4.4 % (p < 0.0001). CONCLUSIONS: Kidney transplantation should be considered for children receiving maintenance dialysis with severe LV dysfunction.

Restless legs syndrome in children with chronic kidney disease.

BACKGROUND: Restless legs syndrome (RLS) is considerably more common among adults with chronic kidney disease (CKD) than in the general population and is associated with increased morbidity and mortality. There is limited information on RLS in children with CKD. Failure to account for conditions that might mimic RLS can lead to overdiagnosis of this syndrome. METHODS: In a prospective, cross-sectional study, RLS prevalence was compared between pediatric CKD patients and healthy children. RLS was assessed via a questionnaire that included exclusion of mimics. Sleep characteristics and health-related quality of life (HRQoL) were also assessed. RESULTS: Restless legs syndrome was more prevalent in CKD patients (n = 124) than in 85 normal children (15.3 vs. 5.9 %; p = 0.04). There was no significant association between RLS and CKD stage, CKD etiology, CKD duration, and dialysis or transplant status. Children with RLS were more likely to rate their sleep quality as fairly bad or very bad (41.2 vs. 8.8 %; p = 0.003) and report using sleep medications (42.1 vs. 14.7 %; p = 0.01). RLS was associated with lower HRQoL by parent report (p = 0.03). Only five of the 19 patients (26.3 %) with CKD and RLS had discussed RLS symptoms with a healthcare provider, and only one of these patients had been diagnosed with RLS prior to this study. CONCLUSIONS: The prevalence of RLS is increased in children with CKD and appears to be underdiagnosed. Systematic screening for RLS and sleep problems would therefore appear to be warranted in children with CKD.

Transition of Care in Children with Chronic Kidney Disease, Dialysis, and Transplantation.

Improvement in management of pediatric renal disorders has led to patient survival rates of 85-90%, increasing the number of adolescent and young adult (AYA) patients with childhood onset chronic kidney disease (CKD) transitioning to adult care settings. Pediatric CKD patients differ from adults with CKD in view of early onset of disease (sometimes with fetal onset), different disease spectrum, the potential effect of CKD on neurodevelopment, and substantial involvement of parents in medical decision making. In addition to the usual challenges of emerging adulthood (graduation from school to work, independent living, peak in impulsivity and risk-taking behaviors), young adults with pediatric CKD need to learn to manage a serious medical condition independently. In kidney transplant patients, regardless of the age at transplantation, graft failure rates are higher during adolescence and young adulthood than at any other age. All pediatric CKD patients must move from a pediatric to adult-focused settings and this transition is a longitudinal process requiring collaboration and interactions of AYA patients, their families, providers, health care environment and agencies. Consensus guidelines have provided recommendations to pediatric and adult renal teams to enable successful transition. Suboptimal transition is a risk factor for poor adherence to treatment and unfavorable health outcomes. The authors discuss the process of transition as it applies to pediatric CKD patients and review challenges faced by patients/families, pediatric and adult nephrology teams. They provide some suggestions and available tools to optimize the transition of pediatric CKD patients to adult-oriented care.

Chylous ascites complicating pediatric renal transplantation.

BACKGROUND: Chylous ascites (CA) is an unusual complication of pediatric abdominal surgery which can be difficult to manage. Little data are available to guide therapy. CASE-DIAGNOSIS/TREATMENT: We describe the presentation, clinical course and management of two patients, a 7-year-old boy and 2.5-year-old girl, who experienced CA following intraperitoneal renal transplantation. Both cases failed medical management, and the patients required surgical intervention. One required a peritoneovenous shunt while the other required an external abdominal drain. Both patients eventually experienced resolution of the CA and excellent long-term renal function. CONCLUSIONS: These case descriptions highlight the challenges of optimizing nutrition while controlling chylous output. We also review some of the risk factors associated with CA and draw attention to the consideration of this diagnosis in any renal transplant recipient with post-operative abdominal distention and ascites.

Hepatic hydrothorax without any evidence of ascites.

Hepatic hydrothorax usually presents in association with ascites, but there are rare cases when it does not. This case helps to support the differential of hepatic hydrothorax in patients who have a history of liver cirrhosis, portal hypertension, and recurrent pleural effusions without ascites. We hope to support the conclusion that a patient with recurrent pleural effusions, without ascites, does not exclude gastrointestinal involvement in its etiology.

More Than Dysuria: Corynebacterial Encrusted Cystitis.

Corynebacterium species, typically considered contaminants in urine culture, can cause encrusted cystitis (EC), a form of chronic urinary tract infection causing pain, bladder necrosis, renal failure, and death. Delayed diagnosis is common due to its rarity and the fastidious nature of Corynebacterium urealyticum. Reported mostly in elderly and immunocompromised patients, EC is rare in pediatric patients. A female adolescent on high dose steroids developed persistent dysuria after urinary catheterization. Abnormal bladder ultrasound and characteristic cystoscopy led to the diagnosis of EC. Appropriate treatment instituted 4 months from onset of dysuria led to an excellent response.

Mounier-Kuhn syndrome: a case report.

Mounier-Kuhn syndrome is a rare clinical entity. It was described in 1932 to refer to the presence of tracheobronchomegaly. The clinical presentation is variable and it can occur in several different age groups. We present the case of a young man who was admitted to the hospital for an asthma exacerbation and was found to have tracheobronchomegaly on his CT scan. A review of the literature for this rare, but interesting, phenomenon is included.

Restless Legs Syndrome in Chronic Kidney Disease: Is Iron or Inflammatory Status To Blame?

STUDY OBJECTIVES: Restless legs syndrome (RLS) is increased in pediatric chronic kidney disease (CKD). In adults without CKD, central nervous system iron deficiency is involved in RLS pathogenesis and a low serum ferritin levels is consequently an indication for initiation of iron therapy. However, children with CKD are at risk for iron deficiency and inflammation, which raises serum ferritin. We examined the role of iron deficiency and inflammation in RLS in pediatric CKD. METHODS: This cross-sectional study examined RLS prevalence in three groups of pediatric patients with CKD: nontransplant, nondialysis CKD (estimated GFR < 60 mL/min/1.73 m²) (n = 27); renal transplant recipients (n = 65); and dialysis (n = 32). RLS was diagnosed using a validated questionnaire. Serum ferritin < 100 ng/mL or transferrin saturation < 20% defined iron deficiency. Serum high sensitivity C-reactive protein ≥ 1 mg/L defined inflammation. RESULTS: Among 124 patients, RLS prevalence was 15.3%; this did not differ across groups. There was no significant difference in RLS prevalence between those with and without iron deficiency, defined by either reduced ferritin or transferrin. Median ferritin levels in patients with RLS tended to be higher than in those without RLS (51.2 versus 40.1 ng/mL; P = .08). Inflammation (elevated CRP) also did not differ significantly by RLS status (57.9% [with RLS] versus 41.2% [without RLS], P = .18). CONCLUSIONS: Neither ferritin nor inflammation differentiated pediatric patients with CKD with and without RLS. This study suggests that the factors mediating the pathogenesis and, potentially, treatment, of RLS in pediatric CKD may be different from non-CKD populations.

The efficacy of a chinstrap in treating sleep disordered breathing and snoring.

STUDY OBJECTIVES: A previously published case report suggested that a chinstrap alone might improve obstructive sleep apnea (OSA). We conducted this study to determine whether a chinstrap was a feasible alternative to continuous positive airway pressure (CPAP) in patients with OSA. METHODS: 26 adult patients with OSA (apnea-hypopnea index [AHI] > 5/h on diagnostic polysomnogram [PSG]) underwent a modified split-night PSG, using only a chinstrap for the first 2 hours of sleep, followed by CPAP titration for the remainder of the night. Improvements in AHI, arterial oxygen saturation (SpO2), and snoring with chinstrap use were compared to results with optimal CPAP pressures. RESULTS: There was no significant difference between the diagnostic PSG and the chinstrap portion of the split-night PSG in the following parameters: general AHI (median [IQR] 16.0/h [9.7-26.0] vs. 25.9/h [10.7-42.7]), SpO2 nadir (84.0% [80.5-87.5] vs. 87.0 [84.0-88.5]), AHI in REM sleep (26.7/h [16.8-43.7] vs. 42.4/h [21.3-57.7]), AHI in supine sleep (24.9/h [11.9-51.5] vs. 29.8/h [11.7-55.5]), snoring index (253.2/h [147.5-353.1] vs. 180.0/h [9.8-393.3]) or subjective snoring scale (3.0 [0.8-3.0] vs. 2.5 [0.4-3.0]). The AHI and SpO2 nadir in the 13 patients with mild OSA also did not improve with chinstrap use (9.6/h [8.1-12.2] vs. 10.6/h [6.8-35.4] and 87.0% [83.0-90.0] vs. 88.0% [87.0-89.0]). All these parameters showed significant improvement with optimal CPAP titration (p < 0.05). CONCLUSIONS: A chinstrap alone is not an effective treatment for OSA. It does not improve sleep disordered breathing, even in mild OSA, nor does it improve the AHI in REM sleep or supine sleep. It is also ineffective in improving snoring.

Association of hypophosphatemia with failure-to-wean from mechanical ventilation.

Severe hypophosphatemia is known to be associated with respiratory failure, but there are few studies that specifically examine the relationship between serum phosphorus concentration and failure to wean patients from mechanical ventilation. This study investigated the association between hypophosphatemia and weaning failure in patients in two medical intensive care units (ICU). The study was conducted in a prospectively developed cohort of 66 patients being treated with ventilatory support and in whom 193 weaning trials were attempted. Ultimately, all 66 subjects were successfully weaned. A cross-sectional analysis was conducted on serum phosphorus levels and success or failure to wean the patients from ventilators. At the time of the successful weaning attempts (n = 66), the subjects' serum phosphorus concentrations (mean +/- SD) were 1.18 +/- 0.27 mmol/L, whereas at all failed weaning attempts (n = 127) serum phosphorus concentrations averaged 1.06 +/- 0.31 mmol/L (p = 0.008). Subjects with phosphorus concentrations below the reference interval (RI) in our laboratory (<0.80 mmol/L) had greater risk for weaning failure compared to subjects with phosphorus concentrations at or above the RI (relative risk = 1.18; 95% confidence interval = 1.06 to 1.32; p = 0.01). Serum calcium concentrations were not significantly different at the time of successful weaning compared to those at failed weaning attempts. This study indicates that there is an association between hypophosphatemia and failure-to-wean from mechanical ventilation in ICU patients on ventilatory support.

Obesity among pre-adolescent and adolescents of a developing country (India).

There are very few reports from the developing world on the prevalence of obesity among children even though in developed countries it has reached epidemic proportions. The objective of this study was to determine the prevalence of obesity in pre-adolescent and adolescent children in a developing country (India) using WHO guidelines for defining obesity and overweight. This cross-sectional study was carried out on 2008 school-children aged 9-15 years. Approximately half the subjects belonged to a school attended by children of well to do families while the rest belonged to two schools from middle and lower socio-economic background. Weight and height were taken for all children and the body mass index (BMI) calculated. Children whose BMI was >85th percentile for age and sex were defined as overweight. Triceps skin fold thickness (TSFT) was measured for all overweight children and those with TSFT >90th percentile for age and sex were defined as obese. The overall prevalence of obesity and overweight was 11.1% and 14.2% respectively. The prevalence of obesity as well as overweight was higher in boys as compared to girls (12.4% vs 9.9%, 15.7% vs 12.9%). Prevalence of obesity decreased significantly with age, from 18.5% at 9 years to 7.6% at 14 years, rising at 15 years to 12.1%. Significantly more children from higher socio-economic status were obese and overweight than those from lower socio-economic status groups. No significant gender difference for obesity prevalence was seen among children from a less privileged background, however, amongst children from affluent families, significantly more boys were obese as compared to girls. Pediatric obesity is an emerging problem in developing countries, especially among higher socio-economic status groups. Significant gender disparity is seen, with boys of affluent background having a higher prevalence.