Background: Describe the efficacy of a galenic glycopyrrolate formulation and its impact on patients with sialorrhea Quality of Life (QoL), including costs analysis. Methods: We performed a retrospective observational study on 21 patients who received a custom-formulated galenic glycopyrrolate syrup for sialorrhea for an average period of 14.3 months. We analyzed the telephone interviews with elaborated and validated questionnaires and the therapy costs comparing the brand marketed drug with the galenic formulation. Results: Overall, 16 out of 21 patients (76.2%) reported a significant improvement in sialorrhea and QoL. In 14 subjects (66.7%), there was a remarkable decrease in the drooling severity; 10 individuals (47.6%) reported a reduction in drooling frequency. Nine patients experienced at least one adverse effect of glycopyrrolate therapy, and three of them stopped the treatment. No severe side effects were observed. The galenic drug significantly reduced costs for patients. Conclusions: An oral glycopyrrolate solution easily administered to children with brain injuries is not commercially available in many European countries. This study demonstrates the efficacy of a compounded glycopyrrolate syrup on drooling severity, frequency and ensures a better QoL in patients and their caregivers.

Background Different definitions of family-centred care (FCC) exist in the newborn setting, and many FCC interventions have been tested, while a comprehensive review synthesising characteristics of existing intervention studies is still lacking. Objective This review aims at summarising the characteristics of randomised controlled trials (RCTs) on FCC interventions in neonatal intensive care units. Methods We searched PubMed, Embase, Web of Science and the Cochrane Library up to 31 January 2022, and reference lists of included studies and other reviews. Interventions were grouped into five categories according to a previous Cochrane review: (1) family support, (2) educational, (3) communication, (4) environmental interventions and (5) family-centred policies. Subgroup analyses by time period (RCTs published before vs after 2016) and by country income (based on the World Bank Classification) were conducted. Results Out of 6583 retrieved studies, 146 RCTs met the eligibility criteria, with 53 (36.3%) RCTs published after 2016. Overall, 118 (80.8%) RCTs were conducted in high-income countries, 28 (19.1%) in middle-income countries and none in low-income countries. Only two RCTs were multicountry. Although mothers were the most frequent caregiver involved, fathers were included in 41 RCTs (28.1%). Very few studies were conducted in at-term babies (nine RCTs); siblings (two RCTs) and other family members (two RCTs), maternity care units (two RCTs). The role of health professionals was unclear in 65 (44.5%) RCTs. A large variety of intervention combinations was tested, with 52 (35.6%) RCTs testing more than 1 category of interventions, and 24 (16.4%) RCTs including all 5 categories. Conclusion There is a large and rising number of RCTs on FCC interventions in neonatal intensive care units, with specific research gaps. The large variety of FCC interventions, their high complexity, the need to tailor them to the local context and major gaps in implementation suggest that implementation research is the current priority.

Background: Erythropoietic protoporphyria is a rare disorder which represents an important health problem in children, causing painful photosensitivity. Little is known on the correlation between genetic profile and clinical manifestations. The standard of care for Erythropoietic protoporphyria is based on avoiding sun and using sun protections, but recent literature has suggested that cimetidine may have a role in improving sun sensitivity. Herein we report our case series describing the successful use of cimetidine and analyzing potential phenotype-genotype correlations. Case presentation: This case series describes five patients presented to our Rheumatology Service complaining sun sensitivity. Blood exams and genetic analysis were consistent with the diagnosis of erythropoietic protoporphyria. Four of 5 patients received cimetidine in addition to standard therapies and the effect of treatment was evaluated by Erythropoietic Protoporphyria - Quality of Life questionnaire. Conclusions: Erythropoietic protoporphyria usually manifests in early childhood after a short sun exposure. Skin manifestations are the main reason for investigations, although sometimes they can be more subtle, leading to a significant diagnostic delay. Skin diseases in children can have profound effects on their family and social relationships. A treatment with cimetidine appears to be an excellent therapeutic option in children with Erythropoietic protoporphyria.

Background: Limping is a common clinical symptom in childhood; different clinical conditions may lead to limping and the diagnosis of the underlying cause may often be a challenge for the pediatrician. Case presentation: We describe the clinical manifestations, radiological pictures and disease course of other causes of limping in childhood, through a case series of seven cases and a brief discussion of each disease. Conclusions: although trauma is the most common cause of acute limping, when there is no history of traumatic events and the limping has a chronic course, Juvenile Idiopathic Arthritis is usually the most likely clinical diagnosis. However, other some rare conditions should be taken into account if JIA is not confirmed or if it presents with atypical clinical picture.

Vengono analizzati sette casi di zoppia in età pediatrica, che si discostano dalle manifestazioni più comuni e pongono sfide significative nella diagnosi. Spesso, questi casi si trovano in diagnosi differenziale in primis con l'artrite idiopatica giovanile, ma anche con altre cause di zoppia. Si esplorano le caratteristiche cliniche, le indagini diagnostiche e le strategie di gestione, offrendo spunti preziosi per il loro riconoscimento, che parte dalla loro conoscenza.

Background:The definition of psoriatic arthritis in pediatrics (JPsA) is controversial. The study purpose to describe clinical and laboratory characteristics and responses to therapies in children with psoriatic arthropathies. Methods: A retrospective multicentre observational study enrolling subjects meeting the ILAR criteria for juvenile psoriatic arthritis. We also included subjects meeting the enthesitis-related arthritis criteria, except for the presence of psoriasis and/or family history of psoriasis or psoriatic arthritis in a first-degree relative. Disease remission was defined according to the Wallace criteria. Results: 71 patients (44 females, 27 males). Disease onset was before 10 years in 32 patients (median 5.5 years) and after 10 years (median 13 years) in 39. Inflammation indexes and antinuclear antibodies were raised, respectively, in 61% and 47% of the tested patients. Twenty patients (28.2%) got clinical remission with intra-articular glucocorticoids. Eighteen patients (25.3%) with synthetic disease-modifying anti-rheumatic drugs got better, and 33 patients (46.5%) with biological DMARDs. Two patients presented with intractable uveitis. Fifty-five patients (77.5%) met the ILAR criteria for JPsA; eight were HLA B27+ males with disease onset over 6 years of age, and eight had arthritis and enthesitis. Four clinical patterns were found using unsupervised principal component analysis. One of them was mostly made up of early-onset oligoarticular arthiritis, dactylitis, tenosynovitis, and uveitis. The two groups occurring after 10 years of age were more similar to adult-onset psoriatic arthritis or undifferentiated spondyloarthropaties. Conclusions: Post-pubertal clusters of JPsA are similar to typical adult PsA, while peculiar clusters are identified in younger children.