Autosomal dominant hypocalcemia type 1 (ADH1) is caused by gain-of-function mutations in the gene encoding the calcium-sensing receptor (CASR),^1,2 a condition that results in hypocalcemia and seizures, hypomagnesemia, hyperphosphatemia, reduced parathyroid hormone (PTH) levels, and hypercalciuria. The receipt of calcium and vitamin D analogues for the treatment of ADH1 predisposes patients to nephrocalcinosis and renal impairment.^1,3 Calcium-sensing receptor antagonists, known as calcilytics, represent a possible treatment option² but are available only as a research tool and have not been approved for clinical use. However, recombinant PTH (1-34) (a peptide consisting of 34 N-terminal PTH amino acids) may increase the serum calcium level without causing hypercalciuria in patients with some forms of hypoparathyroidism.^4,5

Lifelong adherence to a gluten-free diet (GFD) is the cornerstone of management of celiac disease (CD), but adhering to a GFD can be hard. Although several factors are positively associated with adherence of pediatric CD patients to a GFD, it is unknown whether these are influenced by variability caused by the specific tool used to assess adherence to a GFD. Here, we aimed to evaluate how individual patient characteristics and dietary counselling by a trained dietitian influence adherence to a GFD in children with CD, as assessed by two validated questionnaires: the Biagi questionnaire and the Leffler short questionnaire adapted for pediatric patients. Some 139 children and adolescents were recruited in a cross-sectional, multicenter study. Concordance between the two questionnaires in defining adherence was fair (weighted Cohen’s kappa coefficient 0.39, 95%CI 0.19–0.60). Upon regression analysis, having a cohabiting family member with CD, being of Italian origin, and receiving specialized dietary counselling during follow-up were found to positively influence stricter adherence to a GFD for children with CD. Neither questionnaire detected a significant relationship between adherence to a GFD and the presence of symptoms after gluten ingestion. This study provides important new data on the factors influencing GFD adherence in the pediatric population, and highlights the importance of dietician input and overcoming language and cultural barriers when educating patients.

The presenting pattern of celiac disease (CD) at diagnosis in children has changed over time, with a reduction of malabsorption-related phenotypes and an increase in regular or even excessive growth patterns. We retrospectively reviewed the body mass index (BMI) distribution of all patients with a new diagnosis of CD made in a Pediatric Gastroenterology Outpatient Clinic in 1990–2011, compared to those diagnosed in 2012–2022, according to their clinical and serological characteristics. The 1990–2011 and 2012–2022 cohorts included 250 (M:F = 90:160, mean age 7.3 ± 6.1 years) and 243 children (M:F = 81:162, mean age 7.1 ± 3.7 years, NS), respectively. The prevalence of underweight (UW) was higher in the 1990–2011 cohort (61/250, 24.4% in 1990–2011 vs. 31/243, 12.7% in 2012–2022, p = 0.0001), whereas that of overweight (OW) and obese (OB) subjects was significantly higher in 2012–2022 (10/250, 4% in 1990–2011 vs. 24/243, 9.9% in 2012–2022, p = 0.012, and 1/250, 0.4% in 1990–2011 vs. 8/243, 3.3% in 2012–2022, p = 0.018, respectively). In both cohorts, gastrointestinal symptoms were more frequent in OW/OB than in UW children (6/11, 54.5% vs. 5/61, 8.2% in 1990–2011, p < 0.0001, and 24/32, 75% vs. 10/31, 32.3%, p < 0.0001 in 2012–2022), and the extent of anti-transglutaminase antibody increase was similar in OW/OB and UW subjects. The prevalence of children with a normal or even high BMI at CD diagnosis has increased in the past three decades; therefore, CD should be suspected regardless of BMI status.

Objectives There is little experience in implementing the WHO Standards for improving the quality of care (QOC) for children. We describe the use of 75 WHO-Standard based Quality Measures to assess paediatric QOC, using health workers (HWs) as data sources. Design Cross-sectional study. Setting 12 Italian hospitals. Participants The minimum target of 75% of HWs was reached in all facilities; answers from 598 HWs were analysed. Primary and secondary outcome measures 75 prioritised WHO Quality Measures were collected using a validated, and Italian-language questionnaire exploring views of HWs providing care to children. A QOC index was also calculated based on the assessed Quality Measures. Results In both the domain of resources and work organisation, most Quality Measures showed a high overall frequency of reported ‘need for improvement’, with high variability across hospitals. Key needs for improvement included: availability of clear and complete protocols (eg, on paediatric emergencies: 44.6%; range 10.6%–92.6%); clear hospitalisation criteria for diarrhoea (50.5%; range 30.3%–71.7%); number of hand-washing stations (13.2%; range 3.4%–37.0%); equipped working rooms with computers for HWs (66.1%; range: 32.1%–97.0%); training (eg, on pain management: 43.5%; range 17.9%–76.7%), periodic discussion of clinical cases (43.5%; range 8.1%–83.7%) audits (48.8%; range 29.7%–76.7%); and all indicators related to system to improve QOC. Factors significantly associated with a lower QOC Index included HWs working in facilities in Southern Italy (p=0.001) and absence of a paediatric emergency department (p=0.011). Conclusions The use of the 75 prioritised Quality Measures, specific to HWs provide valuable data on paediatric QOC, which can be used to drive a quality improvement process.

Background There is little experience on the use of the WHO Standards for improving the quality of care (QOC) for children. We describe the use of four prioritised WHO Standard-based Quality Measures to assess the provision of care for children with pain in emergency departments (EDs). Methods In a multicentre observational study in 10 EDs with different characteristics in Italy, we collected data on 3355 children accessing the EDs between January 2019 and December 2020. The association between children and facility characteristics and quality measures was analysed through multivariate analyses. Results The proportion of children whose pain was measured was 68.7% (n=2305), with extreme variations across different centres (from 0.0% to 99.8%, p<0.001). The proportion of children treated for pain was 28.9% (n=970) again with a wide range (5.3%–56.3%, p<0.001). The difference between the frequency of children with pain measured and pain treated varied widely between the facilities (ranging from −24.3 to 82). Children with moderate and severe pain were more frequently treated (48.9% and 62.9% of cases, respectively), although with large variations across centres (ranges: 0%–74.8% and 0%–100% respectively, p<0.001). After correction for children’s characteristics, the variable more strongly associated with analysed outcomes was the facility which the child accessed for care. Being a facility in Northern Italy was associated with a higher rate of pain measurement (67.3%–95% CI: 39.9% to 94.6%, p<0.001) compared with facilities in South Italy (−22.1% lower (95% CI: −41.7% to −2.50%, p=0.03). Conclusions The use of few WHO Standard-based measures related to pain can help identifying priority gaps in QOC for children and in monitoring it over time. There is a need for more implementation research to establish which are the most sustainable and effective interventions to improve the QOC for acute pain in children.

The presenting pattern of celiac disease (CD) at diagnosis in children has changed over time, with a reduction of malabsorption-related phenotypes and an increase in regular or even excessive growth patterns. We retrospectively reviewed the body mass index (BMI) distribution of all patients with a new diagnosis of CD made in a Pediatric Gastroenterology Outpatient Clinic in 1990-2011, compared to those diagnosed in 2012-2022, according to their clinical and serological characteristics. The 1990-2011 and 2012-2022 cohorts included 250 (M:F=90:160, mean age 7.3±6.1 years) and 243 children (M:F=81:162, mean age 7.1±3.7 years, NS), respectively. The prevalence of underweight (UW) was higher in the 1990-2011 cohort (61/250, 24.4% in 1990-2011 vs. 31/243, 12.7% in 2012-2022, p=0.0001), whereas that of overweight (OW) and obese (OB) subjects was significantly higher in 2012-2022 (10/250, 4% in 1990-2011 vs. 24/243, 9.9% in 2012-2022, p=0.012, and 1/250, 0.4% in 1990-2011 vs 8/243, 3.3% in 2012-2022, p=0.018, respectively). In both cohorts, gastrointestinal symptoms were more frequent in OW/OB than in UW children (6/11, 54.5% vs. 5/61, 8.2% in 1990-2011, p<0.0001, and 24/32, 75% vs. 10/31, 32.3%, p<0.0001 in 2012-2022) and the extent of anti-transglutaminase antibodies increase was similar in OW/OB and UW subjects. The prevalence of children with a normal or even high BMI at CD diagnosis has increased in the past three decades, therefore CD should be suspected regardless of the BMI status.