Characteristics of intervention studies on family-centred care in neonatal intensive care units: a scoping review of randomised controlled trials.

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.

Chronic limping in childhood, what else other than juvenile idiopathic arthritis: a case series.

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.

Erythropoietic protoporphyria: case reports for clinical and therapeutic hints.

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.

Subcutaneous tocilizumab in the management of non-infectious uveitis in children: a brief report.

BACKGROUND: Tocilizumab is a humanized monoclonal antibody that acts as an IL-6 receptor antagonist. Intravenous tocilizumab is considered an option for children with anti-TNF refractory juvenile idiopathic arthritis-associated uveitis. In contrast, the potential of subcutaneous drug use with this indication is more controversial. Due to the decreased availability of intravenous tocilizumab during the COVID-19 pandemic, we started using the subcutaneous formulation of the drug in children with anti-TNF refractory uveitis. The study analyzes the serum concentration of tocilizumab and its clinical response in patients with anti-TNF refractory uveitis who started or switched to subcutaneous administration from intravenous use. METHODS: Five patients with non-infectious uveitis were treated with subcutaneous tocilizumab. Ocular inflammation was evaluated on slit lamp examination during clinical control. Serum tocilizumab concentrations were determined by ELISA. RESULTS: The mean blood concentration of tocilizumab was 61.4 µg/mL (range 2.7-137.0.), with higher values than levels recorded in adult patients with rheumatoid arthritis treated with intravenous tocilizumab. Three patients entered clinical remission. One patient developed a mild relapse and was treated with topical steroids. Only one patient did not respond to therapy. The medication was well tolerated without severe infection or other adverse events. CONCLUSION: Our results support a possible role of subcutaneous tocilizumab in anti-TNF refractory uveitis.

Compounded glycopyrrolate is a compelling choice for drooling children: five years of facility experience.

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.