Metabolic disorders are frequently observed in pediatric patients with renal lithiasis. Objectives: Study the metabolic and anatomical alterations and perform the chemical analysis of stones found in children with nephrolithiasis in our region. Methods: A retrospective study on 158 children with evidence of recent renal stone formation was performed. One hundred and nine children concluded the metabolic study. Laboratory investigation consisted in two samples of 24-hour urine for calcium, uric acid, citrate, oxalate, sodium and creatinine; qualitative cystinuria, urinary pH following 12-hour fasting and water restriction, urine culture and chemical analysis when the stones were available. Renal imaging techniques included, at least, renal ultrasound and excretory urogram. Results: A cause for nephrolithiasis was identified in 96.3% of children. The main metabolic alteration was hypercalciuria (73.4%). Chemical analysis of stones showed calcium oxalate in 90.9% of the cases. Anatomical alterations were found in 18.0% of the investigated cases and the most frequently found alteration was pyelo-ureteral duplication (28.6%). Conclusions: Hypercalciuria was the most frequently found disorder and pyelo-ureteral duplication was the most common anatomical alteration; moreover, calcium oxalate was the most frequent chemical constituent. The present study showed the characteristics of pediatric patients with nephrolithiasis in our region.
Introduction: Prevention of attacks is a major goal in management of patients with hereditary angioedema (HAE). We aimed to investigate the effects of a systematic intervention for HAE patients. Methods: Thirty-three patients with HAE with C1-inhibitor deficiency, belonging to a single family, participated in a management program coordinated by an allergist/immunologist. Angioedema attacks before intervention were ascertained by interviews and emergency room charts and recorded prospectively by patients or caregivers after enrollment. Mean number of attacks/month was compared at 12 months preintervention and 8 and 14 months within intervention. Patient-reported outcome instruments were used to assess quality of life, including HAE Quality of Life (HAE-QoL) questionnaire, psychological conditions, and work impairment, at baseline and 8 and 14 months within intervention. Data were stored in REDCap platform and analyzed by adjusted Bayesian models of double Poisson regression. Results: Mean number of attacks/month significantly decreased (95% credible interval [CrI] excluding 0) from 1.15 preintervention to 0.25 and 0.23, 8 and 14 months within intervention, with mean decreases of −0.89 (95% CrI: −1.21 to −0.58) and −0.92 (95% CrI: −1.22 to −0.60), respectively. HAE-QoL scores showed mean total increases of 15.2 (95% CrI: 1.23–29.77) and 26 (95% CrI: 14.56–39.02) at 8 and 14 months within the study, as compared to baseline, revealing marked improvement in quality of life. Significant increase in role-emotional and reduction of depression, stress, and anxiety were observed at 14 months. Conclusion: A systematic approach integrating HAE-specific care with effective handling of psychological issues decreased the number of attacks and improved quality of life, targets for best practice in HAE.
the word ''peanut.'' These were manually reviewed for EPI documentation and other practice pattern characteristics. RESULTS: Of a total of 18,149 individuals, 152 (0.8%, mean age 6.4 months) had clinical documentation of EPI guidance (72% clinical note, 20% patient instructions, 5% both, 3% telephone note). EPI guidance was performed by 61 providers among 6 specialties (43% pediatrics, 40% family medicine, 15% allergy/immunology, 2% other) in 24 unique practices. Of those who received EPI guidance, the following comorbid diagnoses and/or characteristics were documented: 35% eczema, 11% egg allergy, 21% family history of food allergy, 31% topical corticosteroid use. Twenty percent were referred to an allergist for peanut allergy risk assessment and 12% had a peanut IgE obtained. CONCLUSIONS: Clinical documentation of EPI counseling is low. Further dissemination and implementation efforts are needed to encourage providers to prioritize EPI and increase standard utilization of consensus guidelines.
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