Prevention of hip dislocation in severe cerebral palsy (GMFCS III-IV-V): an interdisciplinary and multi-professional Care Pathway for clinical best practice implementation.

BACKGROUND: Hip displacement (HD) and dislocation in severe Cerebral Palsy (CP) (GMFCS III, IV, V) are important causes of worsening disability and quality of life. Prevention must be started from the first months of life through screening programs and early treatments, both conservative and surgical. Evidence from Clinical Practice Guidelines also suggests the development of Care Pathways for good clinical practice. At the beginning of 2020 an interdisciplinary, multi-professional working group, composed of 26 members (including Physiatrists, Physiotherapist, Neuro-psychomotor Therapists and Orthopedists representing the respective Italian Scientific Societies) with the involvement of the FightTheStroke Foundation families' association, was set up. AIM: The aim of the multi-professional panel was the production of evidence-based recommendations for the Care Pathway "Prevention of Hip Displacement in children and adolescents with severe CP" for best clinical practice implementation in our national context. DESIGN: Clinical Care Pathway (Clinical Practice Guideline). SETTING: Inpatient and outpatient. POPULATION: Children with severe CP (GMFCS III-IV-V). METHODS: The recommendations of this Care Pathway were developed using the American Academy for Cerebral Palsy and Developmental Medicine (AACPDM) guidelines for Care Pathways development and the Grading of Recommendations Assessment Development and Evaluation (GRADE ADOLOPMENT) working group for adoption or adaption or de novo development of recommendations from high-quality guidelines. In 2020 a multidisciplinary working group (WG) developed four research questions on the prevention of HD on the following topics: screening, botulinum toxin treatment, postural management and preventive soft tissue surgery. A comprehensive review of the biomedical literature was performed on each question. Guidelines, Systematic Reviews and Primary studies were retrieved through a top-down approach. References were screened according to inclusion criteria and quality was assessed by means of specific tools. A list of recommendations was then produced divided by intervention (screening programs, postural management, botulinum toxin, preventive surgery). In a series of meetings, the panel graduated recommendations using the GRADE evidence to decision frameworks. RESULTS: Fifteen recommendations were developed: seven on screening programs, four on postural management strategies, one on botulinum toxin, and three on preventive surgery. Evidence quality was variable (from very low to moderate) and only a few strong recommendations were made. CONCLUSIONS: In severe CP at high risk of hip dislocation, it is strongly recommended to start early hip surveillance programs. In our national context, there is a need to implement Screening programs and dedicated Network teams. We also strongly recommend a comprehensive approach shared with the families and goal-oriented by integrating the different therapeutic interventions, both conservative and not, within Screening programs. CLINICAL REHABILITATION IMPACT: Implementing a comprehensive multi-professional approach for the prevention of hip dislocation in severe CP.

Early Origins of Chronic Obstructive Pulmonary Disease: Prenatal and Early Life Risk Factors.

The main risk factor for chronic obstructive pulmonary disease (COPD) is active smoking. However, a considerable amount of people with COPD never smoked, and increasing evidence suggests that adult lung disease can have its origins in prenatal and early life. This article reviews some of the factors that can potentially affect lung development and lung function trajectories throughout the lifespan from genetics and prematurity to respiratory tract infections and childhood asthma. Maternal smoking and air pollution exposure were also analyzed among the environmental factors. The adoption of preventive strategies to avoid these risk factors since the prenatal period may be crucial to prevent, delay the onset or modify the progression of COPD lung disease throughout life.

Long COVID in Children and Adolescents.

Severe acute respiratory syndrome Coronavirus 2 (SARS-CoV-2) has caused significant mortality and morbidity worldwide. In children, the acute SARS-CoV-2 infection is often asymptomatic or paucisymptomatic, and life-threatening complications are rare. Nevertheless, there are two long-term consequences of SARS-CoV-2 infection in children that raise concern: multisystem inflammatory syndrome in children (MIS-C) and long COVID. While the understanding and the experience regarding the acute phase of SARS-CoV-2 infection have remarkably increased over time, scientific and clinical research is still exploring the long-term effects of COVID-19. In children, data on long COVID are scant. Reports are conflicting regarding its prevalence, duration and impact on daily life. This narrative review explored the latest literature regarding long COVID-19 in the pediatric population. We showed that long COVID in children might be a relevant clinical problem. In most cases, the prognosis is good, but some children may develop long-term symptoms with a significant impact on their daily life. The paucity of studies on long COVID, including a control group of children not infected by SARS-CoV-2, prevents us from drawing firm conclusions. Whether the neuropsychiatric symptoms widely observed in children and adolescents with long COVID are the consequence of SARS-CoV-2 infection or are due to the tremendous stress resulting from the restrictions and the pandemics is still not clear. In both cases, psychological support can play a fundamental role in managing COVID pandemics in children. More knowledge is needed to share a standardized definition of the syndrome and improve its management and treatment.

Improvement in glycaemic control in paediatric and young adult type 1 diabetes patients during COVID-19 pandemic: role of telemedicine and lifestyle changes.

BACKGROUND AND AIM: COVID-19 pandemic determined a profound impact in everyday life and in routine follow-up of patients with type 1 diabetes (T1D). In this context, telemedicine represented an important tool to guarantee a regular care for these patients. Aim of our work was to assess metabolic control before and after lockdown in the cohort of T1D patients followed-up by our Service, to evaluate the impact of restrictive measures and of disease management through telemedicine. METHODS: This is a retrospective observational study. Subjects were enrolled among children, adolescents and young adults affected by T1D and followed at the Regional Paediatric Diabetology Centre of the University-Hospital of Parma, Italy. We collected data about age, gender, ethnicity, anthropometric measurements, duration of disease, type of blood glucose monitoring used, type of insulin administration, daily insulin requirement and metabolic control, assessed using capillary HbA1c. RESULTS: We enrolled 139 patients, mean age 13.9 years. During lockdown, we reported significantly more contacts through telemedicine between patients and medical team. Global glycol-metabolic control significantly improved, without differences in daily insulin requirement. Patients with a previous poor-controlled diabetes showed a greater improvement. Finally, mean weekly hours of physical activity decreased significantly, without worsening in BMI z-score. CONCLUSIONS: Our results show a global improvement in mean HbA1c, with a stronger result for patients with a previous non satisfactory control. In our setting, despite regulatory rules and physical and logistic limitations related to pandemic, no worsening of metabolic control has been shown for patients with type 1 diabetes.

Diabetic ketoacidosis at type 1 diabetes onset: indirect impact of COVID-19 pandemic.

Reorganization of healthcare resources due to COVID-19 pandemic has led to an unintentional neglect of essential care, especially for paediatric emergencies. This phenomenon has been observed also for type 1 diabetes patients at onset, and surveys from different countries have shown an increased number of diabetic ketoacidosis during lock-down period. We report the case of two patients admitted late at our emergency care service for type-1 diabetes at onset with ketoacidosis, for reasons related to COVID-19 pandemic outbreak. Case report 1: A 5 years old boy, presented with a severe diabetic ketoacidosis, requiring admission in Intensive Care Unit, prolonged intravenous insulin infusion and enteral nutrion via nasogastric tube. Case report 2:  A 10 years old girl presented in the emergency department with a history respiratory distress, due to Kussmaul's breathing, and severe dehydration. Laboratory findings were consistent with a diagnosis of moderate diabetic ketoacidosis. We have further analyzed the experience of our Centre regarding new onset type 1 diabetes patients during lock-down period: we observed a reduction of admissions for type 1 diabetes onset during lock-down period compared to same period of 2019, with a higher prevalence of moderate and severe diabetic ketoacidosis. We conclude highlighting the upcoming necessity, due to the emerging of a 'second wave' of the pandemic, that public opinion and healthcare practitioners provide correct information regarding access to paediatric services, in particular for children with newly onset symptoms, in order to avoid late access to emergency department in critical situations and to prevent avoidable morbidity and mortality.

Spinal bracing and lung function in type-2 spinal muscular atrophy.

BACKGROUND: Respiratory muscle weakness associated with scoliosis in type-2 spinal muscular atrophy (SMA) leads to respiratory impairment. Spinal brace, generally utilized to slow scoliosis progression and support sitting, could worsen lung function and hamper cough maneuvers. CASE SERIES: Six home-treated type-2 SMA children (aged 6-15 years, subtype 2.1-2.5) were assessed to evaluate time-dependent influence of "static-balanced brace" on pulmonary function. Lung function tests, including peak expiratory flow (PEF), peak cough flow (PCF), maximal static inspiratory pressure (MIP), maximal static expiratory pressure (MEP), forced vital capacity (FVC), were performed. PEF, MEP, FVC parameters were higher in tests after wearing braces three-hours, PCF slightly higher and MIP slightly lower compared to upon awakening values. CLINICAL REHABILITATION IMPACT: "Static-balanced brace" did not impair lung function in our sample of six type-2 SMA children; in addition, it seemed to support cough maneuvers. Double assessment is determinant for decisions concerning use/non-use of brace differently from the usual one time evaluation procedure (base level compared to level wearing brace).

Longitudinal analysis of immune cell phenotypes in early stage multiple sclerosis: distinctive patterns characterize MRI-active patients.

To investigate whether peripheral immune abnormalities are associated with brain inflammation in multiple sclerosis, and whether differences in MRI activity are paralleled by changes in leukocyte composition, we conducted a prospective longitudinal study in patients at their clinical onset. Twenty patients presenting a first inflammatory event in the central nervous system suggestive of multiple sclerosis underwent, every 45 days for one year, immunophenotyping of 98 blood cell subsets together with brain MRI and clinical evaluation. Six patients showed intense MRI activity, six patients did not display MRI activity, while the remaining 8 patients had low (i.e. intermediate) MRI activity during the follow-up. Our results show that MRI-active and MRI-inactive patients display significant differences in ten lymphocyte subsets. Among these, there are both effector (CCR7-CD45RA-CD4+ alphabeta T cells, CCR5+ gammadelta T cells) and regulatory (DN CD28+ alphabeta T cells and CD25+CD8+ alphabeta T cells) lymphocytes pertaining to the innate and the acquired arms of the immune system. Moreover, these differences were, upon employment of a class prediction procedure based on "support vector machines" algorithm utilizing leave-one-out cross validation procedures, able to correctly assign patients to their respective MRI activity group. All 6 MRI-active and 6 MRI-inactive patients were correctly classified, and, upon application of a class prediction model in an unsupervised manner to the 8 patients with intermediate MRI activity, 6 were predicted as MRI-active and 2 as MRI-inactive patients. Also, when the mean values of the first three time points (T0, T1 and T2) were used for the prediction of all patients, the selected lymphocyte subsets correctly classified 90% of patients. Sensitivity was 91.7% and specificity was 87.5%. These results provide evidence showing that brain inflammation in multiple sclerosis is associated with distinct changes in peripheral lymphocyte subsets, and raise the possibility that the identified subsets may, after adequate validation, assist in the prediction of MRI activity in the early stages of multiple sclerosis.