Recovery of renal function after long-term dialysis in hemolytic uremic syndrome

Long-lasting recovery of renal function of the native kidneys after prolonged renal replacement therapy is rare. An 8-year-old girl and a 3-year-old boy had suffered from acute atypical and diarrhea-associated hemolytic uremic syndrome (HUS), respectively, with subsequent apparent end-stage renal failure. Both recovered renal function after long-lasting anuria and dialysis of 8 and 16months, respectively. After prolonged follow-up, i.e., 7 and 5years after cessation of dialysis, they attained normal or slightly reduced renal function (plasma creatinine 84 and 90µmol/l, respectively). In addition, growth and cognitive development were normal. We conclude that caution is appropriate before offering early renal transplantation to pediatric patients with presumed end-stage kidney disease secondary to HU

Psychosocial health and psychological adjustment in adolescents and young adults with congenital melanocytic nevi: Analysis of self-reports

This study assessed self-reported health-related quality of life and psychological adjustment in 43 adolescents and young adults (ages in years: 14–24, M = 17.6, SD = 2.2) with congenital melanocytic nevi (CMN) and examined associations with sociodemographic variables, characteristics of the CMN, perceived social reactions, and cognitive emotion regulation strategies. Outcome measures included the Pediatric Quality of Life Inventory$^{™}$ 4.0 and the Strengths and Difficulties Questionnaire. Findings suggest impaired psychosocial health and psychological adjustment in youth with CMN compared to community norms. Impairments were associated with higher age of participants, lower socioeconomic status, visibility of the skin lesion, perceived stigmatization, poorer perceived social support, and maladaptive cognitive emotion regulation strategies (self-blame, rumination, and catastrophizing), but not with sex of participants, extent of the skin lesion, and surgical removal of the nevus. Implications for clinical practice and future research are discussed

As time goes by - Overlooking 40 years of inpatient burn treatment at a national pediatric burn center in Switzerland

BACKGROUND Despite extensive prevention programs, burns remain a frequent cause of injury in Switzerland with a known age peak in children. Pediatric burns may cause substantial morbidity, a psyochological burden and therapy related high economic costs. To improve preventive measures, precise knowledge of etiology and treatment of pediatric burns in Switzerland as well as their temporal evolution is indispensable. METHODS The present retrospective analysis included pediatric burn patients admitted for acute treatment to the Pediatric Burn Center of the University Children`s Hospital Zurich over the last four decades. Sociodemographic, injury related, and treatment related data were extracted from medical records. Linear regression analysis was applied to determine temporal changes during the past four decades and chi-square and t-tests were applied wherever applicable. RESULTS A total of 3425 acute burn patients were included in the study between 1977 and 2020, corresponding to a mean of 89 patients/year. Mean age was 3.60 ± 4.12 years, three quarters of all patients were preschool children (0-5 years) and mean total body surface area (TBSA) burned was 8.01% ± 9.57%, however only around one fifth had severe burns (>10% TBSA). Scald burns (65.31%) and flame burns (32.99%) were most commonly seen. Linear regression analysis showed the total number of thermal injuries treated at our center to have increased significantly as of 2004 (p < 0.001). Separate analysis showed the same for small and medium (<10% TBSA) burns (p < 0.001), whereas the number of severe burns did not increase significantly. Length of stay (LOS) was highly associated with %TBSA burned. The percentage of female patients amongst all patients increased over time (p = 0.012). LOS per TBSA burned decreased significantly (p < 0.001). CONCLUSION The present data show pediatric burns to remain a major health burden in Switzerland, especially small and medium burns in preschool children. Prevention programs should focus on this age population as well as on scald and flame burns as most common etiologies. The observed decrease in length of stay suggests a major improvement in overall quality of care in pediatric burns and supports centralization of care

Revealing four decades of snow cover dynamics in the Hindu Kush Himalaya

Knowledge about the distribution and dynamics of seasonal snow cover (SSC) is of high importance for climate studies, hydrology or hazards assessment. SSC varies considerably across the Hindu Kush Himalaya both in space and time. Previous studies focused on regional investigations or the influence of snow melt on the local hydrological system. Here, we present a systematic assessment of metrics to evaluate SSC dynamics for the entire HKH at regional and basin scale based on AVHRR GAC data at a 0.05° spatial and daily temporal resolution. Our findings are based on a unique four-decade satellite-based time series of snow cover information. We reveal strong variability of SSC at all time scales. We find significantly decreasing SSC trends in individual summer and winter months and a declining tendency from mid-spring to mid-fall, indicating a shift in seasonality. Thanks to this uniquely spatio-temporally resolved long-term data basis, we can particularly highlight the unique temporally variable character of seasonal snow cover and its cross-disciplinary importance for mountain ecosystems and downstream regions

Efficacy and safety of propranolol as first-line treatment for infantile hemangiomas

Beta-blockers are a highly promising treatment modality for complicated infantile hemangiomas (IH). However, data on propranolol as first-line treatment, objective outcome measures and impact on hemodynamics in young infants is limited. We retrospectively evaluated a homogenous group of infants with proliferating complicated IH treated with propranolol (2mg/kg/day). Outcome was assessed by blinded evaluation of clinical photographs by visual analogue scale (VAS), ultrasound examination and ophthalmological review (if appropriate). Tolerance and hemodynamic variables were recorded over time, including a 2-day in-patient observation at the initiation of therapy. Twenty-five infants (median age 3.6 (1.5-9.1) months) were included in the study. The median follow-up-time was 14 (9-20) months and 14 patients completed treatment at a median age of 14.3 (11.4-22.1) months, after a duration of 10.5 (7.5-16) months. In all patients, there was significant fading of colour (with a VAS of −9 (−6 to −9) after 7months) and significant decrease in size of the IH (with a VAS of −8 (−3 to −10) after 7months). Median thickness of the lesions assessed by ultrasound at baseline and after 1month was 14 (7-28) mm and 10 (5-23) mm, respectively (p < 0.01). In children with periocular involvement, astigmatism and amblyopia resolved rapidly within 8weeks. The overall tolerance of propranolol was good, and no relevant hemodynamic changes were noted. Conclusion: Our report supports the excellent effect and good tolerance of this novel therapy, and we propose the use of propranolol as first-line treatment for I

Stigmatization Predicts Psychological Adjustment and Quality of Life in Children and Adolescents With a Facial Difference

Objectives This cross-sectional study assessed psychological adjustment and health-related quality of life (HRQOL) in children and adolescents with congenital or acquired facial differences and identified potential predictors of adjustment. Methods Data were obtained from 88 children, ages 9 months to 16 years, by means of parent questionnaires (n = 86) and standardized interviews with children ≥7 years old (n = 31). Evaluation measures included the Child Behavior Checklist (CBCL), KIDSCREEN-27, TNO-AZL Preschool Quality of Life Questionnaire (TAPQOL), and Perceived Stigmatization Questionnaire. Results Psychological adjustment, as measured by the CBCL, was within norms. Parent-reported HRQOL was good in preschool children. Parent- and self-reported HRQOL of participants 7-16 years old was impaired in several dimensions, including psychological well-being. Psychological adjustment (especially internalizing behavior problems) and HRQOL were predicted primarily by perceived stigmatization. Conclusions Identification of stigma experiences and appropriate support may be crucial to enhancing psychological adjustment and quality of life in children with facial disfiguremen

Parathyroid hormone and phosphate homeostasis in patients with Bartter and Gitelman syndrome:an international cross-sectional study

Background:Small cohort studies have reported high parathyroid hormone (PTH) levels in patients with Bartter syndrome and lower serum phosphate levels have anecdotally been reported in patients with Gitelman syndrome. In this cross-sectional study, we assessed PTH and phosphate homeostasis in a large cohort of patients with salt-losing tubulopathies. Methods:Clinical and laboratory data of 589 patients with Bartter and Gitelman syndrome were provided by members of the European Rare Kidney Diseases Reference Network (ERKNet) and the European Society for Paediatric Nephrology (ESPN). Results:A total of 285 patients with Bartter syndrome and 304 patients with Gitelman syndrome were included for analysis. Patients with Bartter syndrome type I and II had the highest median PTH level (7.5 pmol/L) and 56% had hyperparathyroidism (PTH &gt;7.0 pmol/L). Serum calcium was slightly lower in Bartter syndrome type I and II patients with hyperparathyroidism (2.42 versus 2.49 mmol/L; P = .038) compared to those with normal PTH levels and correlated inversely with PTH (rs −0.253; P = .009). Serum phosphate and urinary phosphate excretion did not correlate with PTH. Overall, 22% of patients had low serum phosphate levels (phosphate—standard deviation score &lt; −2), with the highest prevalence in patients with Bartter syndrome type III (32%). Serum phosphate correlated with tubular maximum reabsorption of phosphate/glomerular filtration rate (TmP/GFR) (rs 0.699; P &lt; .001), suggesting renal phosphate wasting. Conclusions:Hyperparathyroidism is frequent in patients with Bartter syndrome type I and II. Low serum phosphate is observed in a significant number of patients with Bartter and Gitelman syndrome and appears associated with renal phosphate wasting.</p

Parathyroid hormone and phosphate homeostasis in patients with Bartter and Gitelman syndrome:an international cross-sectional study

Background:Small cohort studies have reported high parathyroid hormone (PTH) levels in patients with Bartter syndrome and lower serum phosphate levels have anecdotally been reported in patients with Gitelman syndrome. In this cross-sectional study, we assessed PTH and phosphate homeostasis in a large cohort of patients with salt-losing tubulopathies. Methods:Clinical and laboratory data of 589 patients with Bartter and Gitelman syndrome were provided by members of the European Rare Kidney Diseases Reference Network (ERKNet) and the European Society for Paediatric Nephrology (ESPN). Results:A total of 285 patients with Bartter syndrome and 304 patients with Gitelman syndrome were included for analysis. Patients with Bartter syndrome type I and II had the highest median PTH level (7.5 pmol/L) and 56% had hyperparathyroidism (PTH &gt;7.0 pmol/L). Serum calcium was slightly lower in Bartter syndrome type I and II patients with hyperparathyroidism (2.42 versus 2.49 mmol/L; P = .038) compared to those with normal PTH levels and correlated inversely with PTH (rs −0.253; P = .009). Serum phosphate and urinary phosphate excretion did not correlate with PTH. Overall, 22% of patients had low serum phosphate levels (phosphate—standard deviation score &lt; −2), with the highest prevalence in patients with Bartter syndrome type III (32%). Serum phosphate correlated with tubular maximum reabsorption of phosphate/glomerular filtration rate (TmP/GFR) (rs 0.699; P &lt; .001), suggesting renal phosphate wasting. Conclusions:Hyperparathyroidism is frequent in patients with Bartter syndrome type I and II. Low serum phosphate is observed in a significant number of patients with Bartter and Gitelman syndrome and appears associated with renal phosphate wasting.</p