Pheochromocytoma in a Twelve-Year-Old Girl with SDHB-Related Hereditary Paraganglioma-Pheochromocytoma Syndrome

A twelve-year-old girl presented with a history of several weeks of worsening headaches accompanied by flushing and diaphoresis. The discovery of markedly elevated blood pressure and tachycardia led the child\u27s pediatrician to consider the diagnosis of a catecholamine-secreting tumor, and an abdominal CT scan confirmed the presence of a pheochromocytoma. The patient was found to have a mutation in the succinyl dehydrogenase B (SDHB) gene, which is causative for SDHB-related hereditary paraganglioma-pheochromocytoma syndrome. Herein, we describe her presentation and medical management and discuss the clinical implications of SDHB deficiency

Impact of Pediatric Obesity on Diurnal Blood Pressure Assessment and Cardiovascular Risk Markers

Background: The prevalence of hypertension is increasing particularly among obese children and adolescents. Obese children and adolescents with hypertension are likely to remain hypertensive as they reach adulthood and hypertension is linked to an increased risk for cardiovascular disease. Twenty-four-hour ambulatory blood pressure monitoring (ABPM) has become one of the most important tools in diagnosing hypertension in children and adolescents and circadian patterns of blood pressure may be important disease-risk predictors. Methods: A retrospective chart review was conducted in patients aged 6–21 years who underwent 24-h ABPM at Kentucky Children\u27s Hospital (KCH) from August 2012 through June 2017. Exclusion criteria included conditions that could affect blood pressure including chronic kidney disease and other renal abnormalities, congenital heart disease, cancer, and thyroid disease. Subjects were categorized by body mass index into normal (below 85th percentile), overweight (85th−95th percentile), stage I obesity (95th−119th percentile), stage II obesity (120th−139th) and stage III obesity (\u3e 140th). Non-dipping was defined as a nocturnal BP reduction of \u3c 10%. Results: Two hundred and sixty-three patients (156 male patients) were included in the analysis, of whom 70 were normal weight, 33 overweight, 55 stage I obesity, 53 stage II, and 52 stage III obesity. Although there was no significant difference between normal weight and obese groups for prevalence of hypertension, there was a greater prevalence of SBP non-dipping in obese patients as BMI increased (p = 0.008). Furthermore, non-dippers had a significantly elevated LVMI as well as abnormal lab values for uric acid, blood lipid panel, creatinine, and TSH (p \u3c 0.05). Conclusions: These findings demonstrate that obese children and adolescents constitute a large proportion of hypertensive children and adolescents and the severity of pediatric obesity is associated with nocturnal BP non-dipping. Additionally, obesity in children is linked to several cardiovascular risk factors including left ventricular hypertrophy, dyslipidemia, and elevated uric acid levels. Further studies utilizing ABPM measures on risk stratification in this very high-risk population are warranted

Ambulatory Systolic Blood Pressure and Obesity are Independently Associated with Left Ventricular Hypertrophic Remodeling in Children

Background: Children with obesity have hypertrophic cardiac remodeling. Hypertension is common in pediatric obesity, and may independently contribute to hypertrophy. We hypothesized that both the degree of obesity and ambulatory blood pressure (ABP) would independently associate with measures of hypertrophic cardiac remodeling in children. Methods: Children, aged 8–17 years, prospectively underwent cardiovascular magnetic resonance (CMR) and ABP monitoring. Left ventricular (LV) mass indexed to height2.7(LVMI), myocardial thickness and end-diastolic volume were quantified from a 3D LV model reconstructed from cine balanced steady state free precession images. Categories of remodeling were determined based on cutoff values for LVMI and mass/volume. Principal component analysis was used to define a “hypertrophy score” to study the continuous relationship between concentric hypertrophy and ABP. Results: Seventy-two children were recruited, and 68 of those (37 healthy weight and 31 obese/overweight) completed both CMR and ABP monitoring. Obese/overweight children had increased LVMI (27 ± 4 vs 22 ± 3 g/m2.7, p \u3c 0.001), myocardial thickness (5.6 ± 0.9 vs 4.9 ± 0.7 mm, p \u3c 0.001), mass/volume (0.69 ± 0.1 vs 0.61 ± 0.06, p \u3c 0.001), and hypertrophy score (1.1 ± 2.2 vs −0.96 ± 1.1, p \u3c 0.001). Thirty-five percent of obese/overweight children had concentric hypertrophy. Ambulatory hypertension was observed in 26% of the obese/overweight children and none of the controls while masked hypertension was observed in 32% of the obese/overweight children and 16% of the controls. Univariate linear regression showed that BMI z-score, systolic BP (24 h, day and night), and systolic load correlated with LVMI, thickness, mass/volume and hypertrophy score, while 24 h and nighttime diastolic BP and load also correlated with thickness and mass/volume. Multivariate analysis showed body mass index z-score and systolic blood pressure were both independently associated with left ventricular mass index (β=0.54 [p \u3c 0.001] and 0.22 [p = 0.03]), thickness (β=0.34 [p \u3c 0.001] and 0.26 [p = 0.001]) and hypertrophy score (β=0.47 and 0.36, both p \u3c 0.001). Conclusions: In children, both the degree of obesity and ambulatory blood pressures are independently associated with measures of cardiac hypertrophic remodeling, however the correlations were generally stronger for the degree of obesity. This suggests that interventions targeted at weight loss or obesity-associated co-morbidities including hypertension may be effective in reversing or preventing cardiac remodeling in obese children

Incidence and outcomes of neonatal acute kidney injury (AWAKEN): a multicentre, multinational, observational cohort study

Background: Single-center studies suggest that neonatal acute kidney injury (AKI) is associated with poor outcomes. However, inferences regarding the association between AKI, mortality, and hospital length of stay are limited due to the small sample size of those studies. In order to determine whether neonatal AKI is independently associated with increased mortality and longer hospital stay, we analyzed the Assessment of Worldwide Acute Kidney Epidemiology in Neonates (AWAKEN) database. Methods: All neonates admitted to 24 participating neonatal intensive care units from four countries (Australia, Canada, India, United States) between January 1 and March 31, 2014, were screened. Of 4273 neonates screened, 2022 (47·3%) met study criteria. Exclusion criteria included: no intravenous fluids ≥48 hours, admission ≥14 days of life, congenital heart disease requiring surgical repair at <7 days of life, lethal chromosomal anomaly, death within 48 hours, inability to determine AKI status or severe congenital kidney abnormalities. AKI was defined using a standardized definition -i.e., serum creatinine rise of ≥0.3 mg/dL (26.5 mcmol/L) or ≥50% from previous lowest value, and/or if urine output was <1 mL/kg/h on postnatal days 2 to 7. Findings: Incidence of AKI was 605/2022 (29·9%). Rates varied by gestational age groups (i.e., ≥22 to <29 weeks =47·9%; ≥29 to <36 weeks =18·3%; and ≥36 weeks =36·7%). Even after adjusting for multiple potential confounding factors, infants with AKI had higher mortality compared to those without AKI [(59/605 (9·7%) vs. 20/1417 (1·4%); p< 0.001; adjusted OR=4·6 (95% CI=2·5-8·3); p=<0·0001], and longer hospital stay [adjusted parameter estimate 8·8 days (95% CI=6·1-11·5); p<0·0001]. Interpretation: Neonatal AKI is a common and independent risk factor for mortality and longer hospital stay. These data suggest that neonates may be impacted by AKI in a manner similar to pediatric and adult patients

Clinical Characteristics and Treatment Patterns of Children and Adults With IgA Nephropathy or IgA Vasculitis: Findings From the CureGN Study

Introduction: The Cure Glomerulonephropathy Network (CureGN) is a 66-center longitudinal observational study of patients with biopsy-confirmed minimal change disease, focal segmental glomerulosclerosis, membranous nephropathy, or IgA nephropathy (IgAN), including IgA vasculitis (IgAV). This study describes the clinical characteristics and treatment patterns in the IgA cohort, including comparisons between IgAN versus IgAV and adult versus pediatric patients. Methods: Patients with a diagnostic kidney biopsy within 5 years of screening were eligible to join CureGN. This is a descriptive analysis of clinical and treatment data collected at the time of enrollment. Results: A total of 667 patients (506 IgAN, 161 IgAV) constitute the IgAN/IgAV cohort (382 adults, 285 children). At biopsy, those with IgAV were younger (13.0 years vs. 29.6 years, P < 0.001), more frequently white (89.7% vs. 78.9%, P = 0.003), had a higher estimated glomerular filtration rate (103.5 vs. 70.6 ml/min per 1.73 m2, P < 0.001), and lower serum albumin (3.4 vs. 3.8 g/dl, P < 0.001) than those with IgAN. Adult and pediatric individuals with IgAV were more likely than those with IgAN to have been treated with immunosuppressive therapy at or prior to enrollment (79.5% vs. 54.0%, P < 0.001). Conclusion: This report highlights clinical differences between IgAV and IgAN and between children and adults with these diagnoses. We identified differences in treatment with immunosuppressive therapies by disease type. This description of baseline characteristics will serve as a foundation for future CureGN studies

Acute Kidney Injury, Fluid Balance and Risks of Intraventricular Hemorrhage in Premature Infants

Objective: Evaluate association between fluid balance and intraventricular hemorrhage (IVH). Study design: Retrospective review of infants \u3c30 weeks gestation admitted to Kentucky Children’s Hospital Neonatal Intensive Care Unit. Results: Infants with acute kidney injury (AKI) had a 2.4-fold increased risk of IVH (OR 2.38, 95% CI 1.46–3.87) and a 3.5-fold increased risk of severe IVH (OR 3.45, 95% CI 1.98–6.04). Infants above birthweight on day 4 had a 1.9-fold increased risk of IVH (OR 1.86, 95% CI 1.05–3.27) and a 2.0-fold increased risk of severe IVH (OR 1.96, 95% CI 1.03–3.74). When controlling for confounding factors, infants with AKI or above birthweight on day 4 had a 4.6-fold (aOR 4.60, 95% CI 1.80–11.78) and 3.0-fold (aOR 2.96, 95% CI 1.01–8.65) increased risk of severe IVH, respectively. Conclusion: Infants with AKI during the first week of life had a higher association of severe IVH even after controlling for confounding factors

Tobacco Exposure in Children and Adolescents with Chronic Kidney Disease: Parental Behavior and Knowledge. A Study from the Midwest Pediatric Nephrology Consortium

The incidence of cardiovascular disease (CVD) in children with chronic kidney disease (CKD) is high. Exposure to second hand smoke (SHS) is a known risk factor for CVD. Due to a recent report of high incidence of SHS in children with CKD, we sought to investigate via questionnaire the smoking behaviors of caregivers of children with CKD. Material and methods: A cross sectional study was conducted in which caregivers of children and adolescents with CKD were asked to complete a single anonymous self-administered survey. Results: Almost 40% of children and adolescents lived with one or more smokers. Over half of smokers smoked in the presence of their children and in the car. Smokers were significantly less aware of the detrimental effect of SHS exposure on the renal health of their children. Among smokers, almost 70% reported they had not been advised by their child’s nephrologist to quit tobacco use. Conclusion: There is a high prevalence of SHS exposure among children and adolescents with CKD, which may contribute to CVD. Caregivers are not fully aware of the detrimental effects of SHS exposure on the renal health of their children

Tobacco Exposure in Children and Adolescents with Chronic Kidney Disease: Parental Behavior and Knowledge. A Study from the Midwest Pediatric Nephrology Consortium

The incidence of cardiovascular disease (CVD) in children with chronic kidney disease (CKD) is high. Exposure to second hand smoke (SHS) is a known risk factor for CVD. Due to a recent report of high incidence of SHS in children with CKD, we sought to investigate via questionnaire the smoking behaviors of caregivers of children with CKD. Material and methods: A cross sectional study was conducted in which caregivers of children and adolescents with CKD were asked to complete a single anonymous self-administered survey. Results: Almost 40% of children and adolescents lived with one or more smokers. Over half of smokers smoked in the presence of their children and in the car. Smokers were significantly less aware of the detrimental effect of SHS exposure on the renal health of their children. Among smokers, almost 70% reported they had not been advised by their child’s nephrologist to quit tobacco use. Conclusion: There is a high prevalence of SHS exposure among children and adolescents with CKD, which may contribute to CVD. Caregivers are not fully aware of the detrimental effects of SHS exposure on the renal health of their children

Proyecciones educativas y sociales del pensamiento filosófico de Paulo Freire

En este texto se pretende, en primer lugar, recuperar dos nociones de carácter filosófico de Paulo Freire presentes, en especial, en sus primeras obras, y que son aplicadas en su propuesta educativa, muy vinculada con propósitos sociales y políticos. Posteriormente y de manera breve, se plantean algunos de los primeros antecedentes de la corriente pedagógica llamada Educación Popular y sus características más relevantes, con el fin de mostrar que dicha corriente se sostiene en el pensamiento freirano y que es retomada e influye en los orígenes de dos instancias diametralmente opuestas: las escuelas autónomas zapatistas y las escuelas del movimiento “Fe y alegría”. A partir de ello, se busca señalar y resaltar la importancia educativa y social que tienen las ideas freiranas en la actualidad.El pensamiento filosófico-educativo de Paulo Freire pretende cambiar la sociedad a través de la alfabetización, de modo tal que los oprimidos no sólo se conozcan a sí mismos, sino también se desarrollen socialmente. Se busca mostrar que estos lineamientos están presentes en los orígenes de la llamada educación popular, en especial, en las escuelas autónomas zapatistas y en las instituciones educativas pertenecientes al Movimiento “Fe y Alegría” y que deben ser recuperados y aplicados en la actualidad con el fin de lograr una transformación de la sociedad en la que vivimos

Incidence and outcomes of neonatal acute kidney injury (AWAKEN): a multicentre, multinational, observational cohort study.

Background: Single-center studies suggest that neonatal acute kidney injury (AKI) is associated with poor outcomes. However, inferences regarding the association between AKI, mortality, and hospital length of stay are limited due to the small sample size of those studies. In order to determine whether neonatal AKI is independently associated with increased mortality and longer hospital stay, we analyzed the Assessment of Worldwide Acute Kidney Epidemiology in Neonates (AWAKEN) database. Methods: All neonates admitted to 24 participating neonatal intensive care units from four countries (Australia, Canada, India, United States) between January 1 and March 31, 2014, were screened. Of 4273 neonates screened, 2022 (47·3%) met study criteria. Exclusion criteria included: no intravenous fluids ≥48 hours, admission ≥14 days of life, congenital heart disease requiring surgical repair atlife, lethal chromosomal anomaly, death within 48 hours, inability to determine AKI status or severe congenital kidney abnormalities. AKI was defined using a standardized definition -i.e., serum creatinine rise of ≥0.3 mg/dL (26.5 mcmol/L) or ≥50% from previous lowest value, and/or if urine output was/kg/h on postnatal days 2 to 7. Findings: Incidence of AKI was 605/2022 (29·9%). Rates varied by gestational age groups (i.e., ≥22 to=47·9%; ≥29 to=18·3%; and ≥36 weeks =36·7%). Even after adjusting for multiple potential confounding factors, infants with AKI had higher mortality compared to those without AKI [(59/605 (9·7%) vs. 20/1417 (1·4%); p\u3c 0.001; adjusted OR=4·6 (95% CI=2·5-8·3); p= Interpretation: Neonatal AKI is a common and independent risk factor for mortality and longer hospital stay. These data suggest that neonates may be impacted by AKI in a manner similar to pediatric and adult patients. Funding: US National Institutes of Health, University of Alabama at Birmingham, Cincinnati Children\u27s, University of New Mexico