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Tertiary hyperparathyroidism is characterized by hypercalcemia resulting from autonomous parathyroid hormone production and usually occurs after a prolonged period of secondary hyperparathyroidism. This condition can be a complication of X-linked hypophosphatemia (XLH), a rare genetic disease characterized by renal phosphate loss and consequent hypophosphatemia. Parathyroidectomy is considered the first-line therapy but surgical intervention can be complicated by hungry bone syndrome. A male Caucasian patient presented with XLH, diagnosed at the age of 3 years. At the age of 21, tertiary hyperparathyroidism occurred. Neck ultrasonography, neck magnetic resonance imaging, and 99Tc-sestamibi parathyroid scintigraphy revealed two hyperplastic parathyroid glands. To minimize the risk of hypercalcemia, calcimimetic therapy was initiated. After 6 months and preparation with 1,25-dihydroxy vitamin D, the patient underwent total parathyroidectomy with autotransplantation of half of a parathyroid gland into the sternocleidomastoid muscle. Histopathological examination revealed diffuse microscopical hyperplasia of the parathyroid glands. Despite oral supplementation with calcium carbonate and calcitriol, severe hypocalcemia developed on the second postoperative day, attributable to hungry bone syndrome. This finding was confirmed by an increase in bone turnover markers and a reduction in urinary calcium excretion. Hypocalcemia correction required continuous infusion of calcium gluconate for over 2 months. After approval, the patient began burosumab therapy with significant benefits. This case illustrates the complexity of treating tertiary hyperparathyroidism and mineral metabolism in patients with XLH. The hungry bone syndrome can complicate parathyroidectomy, exposing the patients to life-threatening risks. Burosumab therapy may reduce the risk of tertiary hyperparathyroidism developing in these patients.

Abstract Background Recently, the development of advanced, noninvasive methods has allowed the study of respiratory function even in uncooperative infants. To date, there is still little data on the application of this technique in infants with suspected airway obstruction. The aims of our study were: - To evaluate the role of respiratory function testing (PFR) in the diagnosis and follow-up of infants with stridor - To evaluate the differences between patients with inspiratory stridor and expiratory stridor. - To evaluate the concordance between PFR and endoscopy. Methods We enrolled infants aged

Abstract Background Studies have indicated an association between cesarean section (CS), especially elective CS, and an increased risk of celiac disease (CD), but the conclusions of other studies are contradictory. The primary aim of this study (CD-deliver-IT) was to evaluate the rate of CS in a large population of CD patients throughout Italy. Methods This national multicenter retrospective study was conducted between December 2020 and November 2021. The coordinating center was the Pediatric Gastroenterology and Liver Unit of Policlinico Umberto I, Sapienza, University of Rome, Lazio, Italy. Eleven other referral centers for CD have participated to the study. Each center has collected data on mode of delivery and perinatal period of all CD patients referring to the center in the last 40 years. Results Out of 3,259 CD patients recruited in different Italian regions, data on the mode of delivery were obtained from 3,234. One thousand nine hundred forty-one (1,941) patients (60%) were born vaginally and 1,293 (40%) by CS (8.3% emergency CS, 30.1% planned CS, 1.5% undefined CS). A statistically significant difference was found comparing median age at time of CD diagnosis of patients who were born by emergency CS (4 years, CI 95% 3.40–4.59), planned CS (7 years, CI 95% 6.02–7.97) and vaginal delivery (6 years, CI 95% 5.62–6.37) (log rank p

Background The COVID-19 pandemic had an important psychological impact on children and their families. This study aims to explore paediatric health services users’ perceptions of quality of care (QOC) and COVID-19-related fears, and their evolution over time in relation to COVID-19 pandemic.Methods In a multicentre cross-sectional study involving 11 public hospitals providing paediatric care across the Italian territory, we collected data from services users through a validated questionnaire. We analysed four indicators—(1) QOC perceived in relation to COVID-19; (2) overall QOC perceived; (3) fear of accessing health services due to COVID-19; (4) fear of contracting COVID-19 in hospital—and calculated Spearman’s correlation indexes (ρ) with the number of COVID-19 new cases over time. Subgroup analyses were conducted by macroregions and single facility.Results Data from 956 services users were analysed. QOC indicators were stable over time at values close to the maximum (range 77–100 and median 100 for COVID-19 QOC, range 74–98 and median 80 for overall QOC), and no correlations were found with the COVID-19 new cases (ρ=−0.073 and −0.016, respectively). Fear of accessing care and fear of contracting the infection varied over time in between 0%–52% and 0%–53%, respectively, but did not correlate directly with number of COVID-19 new cases (ρ=0.101, 0.107 and 0.233, 0.046, respectively). At subgroup analyses, significantly higher frequencies of fear (p values

Coronavirus disease 2019 (COVID-19) has been chiefly linked with substantial respiratory complications. However, emerging studies have brought attention to the occurrence of severe muscle inflammation (myositis) related to COVID-19, potentially leading to multi-organ failure and increased mortality. Myositis is generally characterized by heightened serum creatine kinase (CK) levels. Acute myositis is characterized by an infiltration of viruses into calf muscle fibers, which may cause a subsequent inflammatory response leading to calf muscle pain. Symptomatic and supportive management, along with explanation and reassurance, is all that is required in managing this condition. While the association between myositis and severe outcomes has been recognized in adults, it remains less understood in the pediatric population. The current retrospective study, conducted at Policlinico San Marco University Hospital in Catania, aimed to analyze clinical and laboratory factors associated with myositis in pediatric patients with SARS-CoV-2 infection. Between January 2022 and January 2023, ten pediatric patients diagnosed with myositis and SARS-CoV-2 infection were evaluated. The study highlighted clinical manifestations such as fever, calf muscle pain, and abnormal gait. Lab results showed elevated CK levels among other findings. All patients underwent treatment, with the majority recovering without complications. A notable correlation was observed between CK levels, blood urea nitrogen (BUN), and the urea/creatinine ratio (UCR). The study also discusses potential pathophysiological mechanisms behind SARS-CoV-2’s impact on skeletal muscles, emphasizing an indirect inflammatory response. Our findings underscore that while myositis in children with SARS-CoV-2 infection appears to follow a benign and self-limiting trajectory, it is crucial to monitor specific markers for early intervention and management. Further research is warranted to elucidate the underlying mechanisms and improve clinical outcomes.

ObjectivesTo evaluate glomerular and tubular renal functions and analyze blood pressure in a cohort of pediatric patients with juvenile idiopathic arthritis (JIA).MethodsA total of 40 pediatric patients, 20 (50%) with JIA and 20 (50%) healthy control subjects, were studied, and performed the renal function on 24-h collection and the 24-h ambulatory blood pressure monitoring (ABPM). Moreover, we compared renal function and blood pressure trends between the groups of JIA patients with different disease activities.ResultsNo statistically significant differences were observed between patients with JIA and healthy children in terms of glomerular filtration rate (GFR), fractional excretion of sodium (FENa), tubular reabsorption of phosphate (TRP), and calcium-creatinine urine ratio (CaU/CrU). In contrast, we observed significantly higher values in JIA patients than in controls for the presence of hematuria (p

Cesarean section is considered a possible trigger of atopy and gut dysbiosis in newborns. Bifidobacteria, and specifically B. bifidum, are thought to play a central role in reducing the risk of atopy and in favoring gut eubiosis in children. Nonetheless, no trial has ever prospectively investigated the role played by this single bacterial species in preventing atopic manifestations in children born by cesarean section, and all the results published so far refer to mixtures of probiotics. We have therefore evaluated the impact of 6 months of supplementation with B. bifidum PRL2010 on the incidence, in the first year of life, of atopy, respiratory tract infections, and dyspeptic syndromes in 164 children born by cesarean (versus 249 untreated controls). The results of our multicenter, randomized, and controlled trial have shown that the probiotic supplementation significantly reduced the incidence of atopic dermatitis, upper and lower respiratory tract infections, and signs and symptoms of dyspeptic syndromes. Concerning the gut microbiota, B. bifidum supplementation significantly increased α-biodiversity and the relative values of the phyla Bacteroidota and Actinomycetota, of the genus Bacteroides, Bifidobacterium and of the species B. bifidum and reduced the relative content of Escherichia/Shigella and Haemophilus. A 6-month supplementation with B. bifidum in children born by cesarean section reduces the risk of gut dysbiosis and has a positive clinical impact that remains observable in the following 6 months of follow-up.

Abstract FIRES is defined as a disorder that requires a prior febrile infection starting between 2 weeks and 24 h before the onset of the refractory status epilepticus with or without fever at the onset of status epilepticus. The patients, previously normal, present in the acute phase recurrent seizures and status epilepticus followed by a severe course with usually persistent seizures and residual cognitive impairment. Boundary with “new onset refractory status epilepticus (NORSE) has not clearly established. Pathogenetic hypothesis includes inflammatory or autoimmune mechanism with a possible genetic predisposition for an immune response dysfunction. Various types of treatment have been proposed for the treatment of the acute phase of the disorder to block the rapid seizures evolution to status epilepticus and to treat status epilepticus itself. Prognosis is usually severe both for control of the seizures and for cognitive involvement. FIRES is an uncommon but severe disorder which must be carefully considered in the differential diagnosis with other epileptic encephalopathy.

Abstract Background SARS-CoV-2 infection in the pediatric age group has a milder course than in adults, but in some cases even children may present with severe forms or develop long-term consequences. The aim of this study was to analyze the clinical features, long-term effects, lifestyle changes and psychological effects of SARS-CoV-2 infection in a pediatric sample of the Italian population. Methods We conducted a telephone survey among 3075 children infected with SARS-CoV-2 in the Latina Local Health Authority. Outcomes included: clinical features of infection, long-term symptoms, lifestyle changes and emotional symptoms during the illness. The information obtained was automatically linked to a spreadsheet and analyzed. Results One thousand four hundred thirteen children agreed to participate in the study; the mean age was 112.8 ± 21.9 months. Children were infected mainly inside familial clusters (59.6%; n = 842); 99% (n = 1399) of children were asymptomatic or exhibited mild symptoms. 20% (n = 259) of children experienced long-term symptoms; risk factors were: older age, higher body mass index and longer duration of infection. Throughout the period of infection, children spent most of the time on devices like tv-video, social media and mobile phone for non-educational activities. 58.8% (n = 620) of parents expressed a negative opinion about distance learning. Finally, we observed that 49,6% (n = 532) of children experienced psychological symptoms during quarantine period. Conclusion Despite a lower susceptibility to COVID-19 in children, it is important to keep the focus high in children, both because of the possible long symptoms after infection and the impact on a children’s mental and physical health due to pandemic. We believe that the return to school or other extracurricular activities are important to correct some of the risk factors for the long COVID syndrome, as obesity, and to limit the cultural damage generated by distance learning and psychological effects related to restrictive measures.

ObjectiveTo evaluate the potential role of Streptococcus salivarius K12 (SSK12) in controlling febrile flares in patients with Periodic Fever, Aphthous stomatitis, Pharyngitis, and cervical Adenitis (PFAPA) syndrome. Further aims were to assess the impact of SSK12 on (i) flare duration, (ii) variation in the degree of the highest body temperature during flares, (iii) steroid-sparing effect, and (iv) change of PFAPA accompanying symptoms before and after SSK12 introduction.Patients and methodsThe medical charts from 85 pediatric patients with PFAPA syndrome (49 males and 36 females) enrolled in the AIDA registry and treated with SSK12 for a median period of 6.00 ± 7.00 months in the period between September 2017 and May 2022 were examined. Children recruited had a median time of disease duration of 19.00 ± 28.00 months.ResultsThe number of febrile flares significantly decreased comparing the 12 months before [median (IQR), 13.00 (6.00)] and after SSK12 initiation [median (IQR), 5.50 (8.00), p < 0.001]. The duration of fever was significantly reduced from 4.00 (2.00) days to 2.00 (2.00) days [p < 0.001]. Similarly, the highest temperature in°C was found significantly lower in the last follow-up assessment [median (IQR), 39.00 (1.00)] compared to the period prior to SSK12 start [median (IQR), 40.00 (1.00), p < 0.001]. Steroid load (mg/year) of betamethasone (or any equivalent steroid) significantly decreased between 12 months before treatment with SSK12 [median (IQR), 5.00 (8.00) mg/year] and the last follow-up visit [median (IQR), 2.00 (4.00) mg/year, p < 0.001]. The number of patients experiencing symptoms including pharyngitis/tonsillitis (p < 0.001), oral aphthae (p < 0.001) and cervical lymphadenopathy (p < 0.001) significantly decreased following SSK12.ConclusionSSK12 prophylaxis given for at least 6.00 months was found to reduce febrile flares of PFAPA syndrome: in particular, it halved the total number per year of fever flares, shortened the duration of the single febrile episode, lowered body temperature by 1°C in the febrile flare, provided a steroid-sparing effect, and significantly reduced the accompanying symptoms related to the syndrome.

Background: Pediatric rheumatic diseases (PRDs) are a group of chronic disorders that start in childhood and are characterized by periodic exacerbations and remissions of symptoms, with limitations in family, school, and social activities. The aim of this study was to detect differences in parents’ psychological adjustment and emotion regulation strategies, and parent-reported children’s adjustments in families of children with active and inactive PRDs. Methods: Fifty-four parents (38 mothers and 16 fathers) of children with PRD were recruited from a pediatric unit. Disease activity was evaluated by their pediatric rheumatologist, while parents’ depressive and anxiety symptoms, emotion regulation strategies, and children’s emotional difficulties and hyperactivity–inattention symptoms were assessed through a web-based survey. Results: Parents of children with active PRDs reported higher levels of their child’s emotional difficulties and hyperactivity–inattention symptoms. Linear regression analysis demonstrated that having a child in the active phases of PRD and lower use of cognitive reappraisal lead to higher children’s emotional symptoms, while active disease, low use of cognitive reappraisal, and greater expressive suppression were associated with higher hyperactivity–inattention symptoms. Our study highlights that children with PRDs and their parents may be at increased risk for psychological problems, especially during the active disease phase, highlighting the importance of a multidisciplinary approach.

Objectives To assess the frequency of loss of smell and taste in children during Covid-19 infection and their prevalence along with other symptoms, as well as the recovery of chemosensory function once healed. Methods To evaluate symptoms during infection, we adapted the Scandinavian adaptation of the Multi-Clinic Smell and Taste Questionnaire and the modified Monel-Jefferson questionnaire. For smell analysis we used Odor Identification (OI) and two variants of the Odor Discrimination (OD) test, and we compared the results with those of a control group. Results We enrolled nine patients in our experimental group and nine in our control group. Fever was the most frequent symptom (55% of cases), followed by anosmia and ageusia (44% of cases), muscle pain and asthenia (22% of cases) and diarrhea, abdominal pain, cough, and headache (11% of cases). In 11% of cases, olfactory symptoms were the only manifestation of the disease. There was no statistically significant difference in OI test and OD tests between the two groups (Children healed from Covid-19 and Control Group). Conclusion Loss of smell and taste are the second most common symptoms of pediatric Covid-19, and they should always be tested because they can be the only manifestations of infection. Olfactory function in Covid-19 children decreases with increasing age and improves with the passage of time after illness.

Abstract Background Since last year, COVID-19, the disease caused by the novel Sars-Cov-2 virus, has been globally spread to all the world. COVID-19 infection among pregnant women has been described. However, transplacental transmission of Sars-Cov-2 virus from infected mother to the newborn is not yet established. The appropriate management of infants born to mothers with confirmed or suspected COVID-19 and the start of early breastfeeding are being debated. Case presentation We report a case of the joint management of a healthy neonate with his mother tested positive for Covid-19 before the delivery and throughout neonatal follow-up. The infection transmission from the mother to her baby is not described, even after a long period of contact between them and breastfeeding. Conclusion It may consider an appropriate practice to keep mother and her newborn infant together in order to facilitate their contact and to encourage breastfeeding, although integration with infection prevention measures is needed.

Abstract Background In 2019, a multidisciplinary panel of experts from eight Italian scientific paediatric societies developed a consensus document for the use of inhaled corticosteroids in the management and prevention of the most common paediatric airways disorders. The aim is to provide healthcare providers with a multidisciplinary document including indications useful in the clinical practice. The consensus document was intended to be addressed to paediatricians who work in the Paediatric Divisions, the Primary Care Services and the Emergency Departments, as well as to Residents or PhD students, paediatric nurses and specialists or consultants in paediatric pulmonology, allergy, infectious diseases, and ear, nose, and throat medicine. Methods Clinical questions identifying Population, Intervention(s), Comparison and Outcome(s) were addressed by methodologists and a general agreement on the topics and the strength of the recommendations (according to the GRADE system) was obtained following the Delphi method. The literature selection included secondary sources such as evidence-based guidelines and systematic reviews and was integrated with primary studies subsequently published. Results The expert panel provided a number of recommendations on the use of inhaled corticosteroids in preschool wheezing, bronchial asthma, allergic and non-allergic rhinitis, acute and chronic rhinosinusitis, adenoid hypertrophy, laryngitis and laryngospasm. Conclusions We provided a multidisciplinary update on the current recommendations for the management and prevention of the most common paediatric airways disorders requiring inhaled corticosteroids, in order to share useful indications, identify gaps in knowledge and drive future research.

ObjectiveAim of this paper is to illustrate the methodology, design, and development of the AutoInflammatory Disease Alliance (AIDA) International Registry dedicated to patients with the Periodic Fever, Aphthous stomatitis, Pharyngitis, and cervical Adenitis (PFAPA) syndrome.MethodsThis is a physician-driven, non-population- and electronic-based registry proposed to gather real-world demographics, clinical, laboratory, instrumental and socioeconomic data from PFAPA patients. Data recruitment is realized through the on-line Research Electronic Data Capture (REDCap) tool. This registry is thought to collect standardized information for clinical research leading to solid real-life evidence. The international scope and the flexibility of the registry will facilitate the realization of cutting-edge study projects through the constant updating of variables and the possible merging and transfer of data between current and future PFAPA registries.ResultsA total of 112 centers have already been involved from 23 countries and 4 continents starting from August 24th, 2021, to April 6th, 2022. In total 56/112 have already obtained the formal approval from their local Ethics Committees. The platform counts 321 users (113 principal investigators, 203 site investigators, two lead investigators, and three data managers). The registry collects retrospective and prospective data using 3,856 fields organized into 25 instruments, including PFAPA patient's demographics, medical histories, symptoms, triggers/risk factors, therapies, and impact on the healthcare systems.ConclusionsThe development of the AIDA International Registry for PFAPA patients will enable the on-line collection of standardized data prompting real-life studies through the connection of worldwide groups of physicians and researchers. This project can be found on https://clinicaltrials.gov NCT 05200715.

Abstract Background Coronavirus Disease-19 (COVID-19) has rapidly become a pandemic emergency, distressing health systems in each affected country. Preparation strategies for managing this pandemic have been keys to face the COVID-19 surge all over the world and all levels of care. Materials and Methods During the epidemic, the Italian society of pediatric emergency-urgency (SIMEUP) promoted a national survey aiming to evaluate preparedness and response of pediatric emergency departments (PED) critical in ensuring optimal management of COVID-19 cases. Results Our results suggest that Italian PED have promptly set a proactive approach to the present emergency. 98.9% of the hospitals have defined special pathways and assistive protocols concerning the management of pediatric COVID-19 cases. The highest percentage of application of the measures for preventive and protective for COVID-19 concerned the use of personal protective equipments. Conclusions Results show that the following measures for pediatric patients, admitted in PED, have been promptly implemented throughout the whole country: eg. use of protective devices, pre-triage of patients accessing the hospital. Despite COVID-19 being a new threat, we have shown that by developing an easy-to-follow decision algorithm and clear plans for the interventional platform teams, we can ensure optimal health care workers and patients’ safety.

Abstract Background A national consensus document on inhaled corticosteroids (ICS) use in childhood, produced by the main Italian pediatric scientific societies, has been recently released. The aim of this study was to gather information on the current pediatricians’ ICS prescription habits in Italy for the management of the most common pediatric respiratory diseases, namely allergic rhinitis (AR), asthma, preschool wheezing and laryngitis. Methods From the 1st October 2018 to the 31st January 2019 a link to an online questionnaire was sent monthly through a newsletter to the members of the Italian Society of Pediatrics. The questionnaire included 18 items on ICS use in the most common pediatric respiratory diseases. Data collection and reporting was based on STROBE Statement Checklist for cross-sectional studies. Results One thousand-two questionnaires were returned from primary care pediatricians (39.1%), hospital pediatricians (38.7%), private practicers (16.4%), university pediatricians (3.1%) and Pediatrics residents (2.7%). We found a good adherence to the international guidelines on AR, with prevalent use of oral antihistamine (60.6%) in the secretive phenotype and nasal ICS in the obstructive phenotype (64.8%). In asthma exacerbations ICS are not used in 53.4% of cases, but they are used at high dose in 27.9% and at low dose in 18.7% of cases. In intermittent asthma, ICS are not chosen as a daily controller therapy in 54.1% of cases, while they are chosen as a low dose daily therapy in 44.5% of cases (high dose in 1.4%). In children with persistent asthma, ICS are chosen as a daily low dose therapy in 67.4% of cases and as a daily high dose therapy in 31%. In the management of preschool wheezing, when a long-term treatment is needed, ICS are chosen both alone and in association with antileukotrienes in 71.4% of cases. Children affected by recurrent asthma exacerbations and wheezing are closely followed up, in particular by their primary care pediatricians. The preference for certain molecules in the treatment of different respiratory diseases also emerged. Conclusions Pediatricians’ ICS prescription habits in Italy should be improved, especially in the management of asthma. Future surveys on a more numerous sample will be useful to analyze differences in prescription habits on the basis of pediatricians’ work settings and geographical distribution.

Abstract Background The coronavirus disease 2019 (COVID-19) is currently rare in children and they seem to have a milder disease course and better prognosis than adults. However, SARS-Cov-2 pandemic has indirectly caused problems in pediatric medical assistance. In view of this we wanted to draw a picture of what happened during health emergency and analyze future prospects for restarting. Methods We involved the Italian pediatric scientific societies institutionally collected in the Italian Federation of Associations and Scientific Societies of the Pediatric Area (FIARPED); We sent a questionnaire to all scientific societies about the pediatric care activity during the COVID-19 emergency and future perspectives for the phase of post-containment. Results The analysis of the questionnaires showed significant decrease of:admission, outpatient visits and specialist consultancy activities during the COVID-19 emergency, primarily linked to the fear of infection. Instead it was increased the serious degree of diseases admitted. Most of scientific societies maintained the relationship with chronic patients through some form of telemedicine, reporting a strong positive opinion about this modality. Finally showed the need to give life a new approach for hospitalizations and outpatient visits through a greater use of telemedicine, educational programs on families and a more decisive role of family pediatricians. Conclusions Our study highlighted many aspects that can be improved in pediatric care. We think that It will be necessary a new shared strategy to improve the management and continuity of care for pediatric patients, primarily developing a network of collaboration between families, family pediatrician and hospitals and by enhancing the use of new methods of telecommunications.

Point-of-care thoracic ultrasound at the patient’s bedside has increased significantly recently, especially in pediatric settings. Its low cost, rapidity, simplicity, and repeatability make it a practical examination to guide diagnosis and treatment choices, especially in pediatric emergency departments. The fields of application of this innovative imaging method are many and include primarily the study of lungs but also that of the heart, diaphragm, and vessels. This manuscript aims to describe the most important evidence for using thoracic ultrasound in the pediatric emergency setting.

Psoriatic disease is a chronic, relapsing inflammatory disorder, characterized mostly by cutaneous erythematous scaly plaques sometimes associated with arthritis. Hidradenitis suppurativa (HS) is a chronic relapsing inflammatory disease of the apocrine glands, characterized clinically by painful abscesses, sinus tracts and scars. It typically occurs after puberty, affecting mainly intertriginous areas of the body. There is a strong association between HS and psoriasis since they share the same pathogenic inflammatory pathway. The patient presented: low birthweight, microcephaly, facial dysmorphisms, lumbar hyperlordosis, walking difficulties, global psychomotor developmental delay and learning disabilities. A genetic evaluation revealed a 2.5 Mb de novo microduplication in the 17q21.31 chromosomal region. Dermatological examination revealed HS (Hurley stage II-HS) distributed in the genital area and inguinal folds, psoriatic plaques on the retroauricolar folds, on the elbows bilaterally and on the lateral aspect of the right ankle and psoriatic arthritis. The patient was treated with adalimumab, with a marked improvement of both conditions. To our best knowledge, we report the first case of coexisting Psoriatic Arthritis Disease and Hidradenitis Suppurativa in a child with chromosome 17q21.31 microduplication syndrome. We hypothesize that gene CRHR1 duplication included in the 17q21.31 chromosomal region might be involved in the pathogenesis of both diseases.

Objectives Evidence showed that, even in high-income countries, children and adolescents may not receive high quality of care (QOC). We describe the development and initial validation, in Italy, of two WHO standards-based questionnaires to conduct an assessment of QOC for children and young adolescents at inpatient level, based on the provider and user perspectives.Design Multiphase, mixed-methods study.Setting, participants and methods The two questionnaires were developed in four phases equally conducted for each tool. Phase 1 which included the prioritisation of the WHO Quality Measures according to predefined criteria and the development of the draft questionnaires. In phase 2 content face validation of the draft questionnaires was assessed among both experts and end-users. In phase 3 the optimised questionnaires were field tested to assess acceptability, perceived utility and comprehensiveness (N=163 end-users). In phase 4 intrarater reliability and internal consistency were evaluated (N=170 and N=301 end-users, respectively).Results The final questionnaires included 150 WHO Quality Measures. Observed face validity was excellent (kappa value of 1). The field test resulted in response rates of 98% and 76% for service users and health providers, respectively. Among respondents, 96.9% service users and 90.4% providers rated the questionnaires as useful, and 86.9% and 93.9%, respectively rated them as comprehensive. Intrarater reliability was good, with Cohen’s kappa values exceeding 0.70. Cronbach alpha values ranged from 0.83 to 0.95, indicating excellent internal consistency.Conclusions Study findings suggest these tools developed have good content and face validity, high acceptability and perceived utility, and good intrarater reliability and internal consistency, and therefore could be used in health facilities in Italy and similar contexts. Priority areas for future research include how tools measuring paediatric QOC can be more effectively used to help health professionals provide the best possible care.

Background: infectious mononucleosis is very common during childhood and neurological manifestations are extremely rare. However, when they occur, an appropriate treatment must be undertaken to reduce morbidity and mortality as well as to ensure appropriate management. Methods: we describe the clinical and neurological records of a female patient with post-EBV acute cerebellar ataxia, whose symptoms rapidly resolved with intravenous immunoglobulin therapy. Afterwards, we compared our results with published data. Results: we reported the case of an adolescent female with a 5-day history of sudden asthenia, vomiting, dizziness, and dehydration, with a positive monospot test and hypertransaminasemia. In the following days, she developed acute ataxia, drowsiness, vertigo, and nystagmus with a positive EBV IgM titer, confirming acute infectious mononucleosis. The patient was clinically diagnosed with EBV-associated acute cerebellitis. A brain MRI showed no acute changes and a CT scan showed hepatosplenomegaly. She started therapy with acyclovir and dexamethasone. After a few days, because of her condition’s deterioration, she received intravenous immunoglobulin and demonstrated a good clinical response. Conclusions: although there are no consensus guidelines for the treatment of post-infectious acute cerebellar ataxia, early intervention with intravenous immunoglobulin might prevent adverse outcomes, especially in cases that do not respond to high-dose steroid therapy.

Objectives: We compared the number of accesses, causes, and instrumental evaluations of chest pain in children between the pre-COVID-19 era and the COVID-19 period and analyzed the assessment performed in children with chest pain, highlighting unnecessary examinations. Methods: We enrolled children with chest pain admitted to our emergency department between January 2019 and May 2021. We collected demographic and clinical characteristics and findings on physical examinations, laboratory tests, and diagnostic evaluations. Then, we compared the number of accesses, causes, and instrumental assessments of chest pain between the pre-COVID-19 era and the COVID-19 era. Results: A total of 111 patients enrolled (mean age: 119.8 ± 40.48 months; 62 males). The most frequent cause of chest pain was idiopathic (58.55%); we showed a cardiac origin in 4.5% of the cases. Troponin determination was performed in 107 patients, and the value was high only in one case; chest X-rays in 55 cases and echocardiograms in 25 cases showed pathological findings, respectively, in 10 and 5 cases. Chest pain accesses increased during the COVID-19 era (p < 0.0001), with no differences in the causes of chest pain between the two periods. Conclusions: The increase in accesses for chest pain during the COVID-19 pandemic confirms that this symptom generates anxiety among parents. Furthermore, our findings demonstrate that the evaluation of chest pain is still extensive, and new chest pain assessment protocols in the pediatric age group are needed.

Background: Whether the diagnostic approach for celiac disease (CD) can really affect quality of life (QoL) and dietary compliance remains controversial.Aims: This study aimed to evaluate QoL and compliance to gluten-free diet (GFD) in adolescents/young adults diagnosed with CD through a screening strategy during childhood compared to age-matched CD patients diagnosed by case-finding and to assess whether follow-up at a referral center for CD influences compliance and QoL.Materials and Methods: Thirty-seven CD patients who were diagnosed by screening programs (SC-group) and 38 age-matched CD patients diagnosed due to symptoms (CF-group) were enrolled. Patients were asked to answer a questionnaire on QoL, dietary compliance, and follow-up care for CD.Results: Twenty-nine patients of the SC-group (median age 18.0 years, interquartile range [IQR] 16.0–19.0) and 31 patients of the CF-group (median age 17.0 years, IQR 15.5–18.0) completed the questionnaire. No significant difference relating adherence to the GFD and QoL was shown between the two groups. The majority (93.5%) of CF-group regularly had annual follow-up at a referral center compared to 37.9% of the SC-group (p < 0.001).Conclusion: The diagnostic strategy does not seem to impact QoL and dietary compliance. However, implementation of follow-up might still be necessary for patients identified through screening.

In response to the current COVID-19 pandemic, universal face masking represents one of the most important strategies to limit the spread of infection. However, their use in children is still highly debated (Esposito and Principi, 2020; Esposito et al., 2020) and there are few data (Lubrano et al., 2021a, 2021b) describing their possible effects on respiratory function in children.A dataset in this paper presents a comparison of the data related to the effects on respiratory function of children wearing a filtering facepiece 2 (N95 mask) with or without exhalation valve. 22 healthy children were randomly assigned to two groups, both groups wearing an N95 mask: one without an exhalation valve (group A), another with an exhalation valve (group B).Children were subjected to a 72 min test: the first 30 min without mask, then 30 min wearing face mask while practiced their usual play activity; finally, 12 min, with face mask in place, while they walked as in a walking test. They were monitored through to microstream capnography system (Rad-97TM with Nomo-Line Capnography, Masimo, Irvine, CA, USA) to log oxygen saturation (SpO2) and respiratory rate (RR).We use the Wilcoxon test to analyzed the differences between the parameters recorded during the study in group A and B. Data analysis was performed using JMP14.3.0 program for Mac by SAS Institute inc.

Objective To evaluate the best timing for ECG screening in order to diagnose long QT syndrome and lower, at the same time, the false positives.Design We retrospectively evaluated the corrected QT (QTc) interval in the clinical reports of the ECG screening performed, as per internal protocol.Setting An outpatient setting in our Unit of Neonatology and Pediatrics, Santa Maria Goretti Hospital in Latina, Italy.Patients We enrolled 3467 healthy neonates between 14 and 30 days of life.Interventions The newborns with abnormal QTc interval were invited to subsequent revaluation every 21 days, until normalisation or necessity to refer to a tertiary paediatric cardiology centre.Main outcome measures Difference in QTc according to patients’ characteristics and number of false positives at second ECG evaluation.Results At first evaluation, 249 (7.2%) newborns had prolonged QTc. We did not find any significant difference in the QTc length according to gestational age (p=0.40) and birth weight (p=0.81). As expected, girls had longer QTc than boys (p=0.01). Only 11 out of 240 (4.6%) and 1 out of 238 infants (0.4%) had persistently prolonged QTc at second and third ECG evaluation, respectively. The QTc decreased significantly at second (p

Abstract Background Vaccine coverage of health care professionals against influenza is still low in Italy, as well as in other European countries. Methods Between March and May 2018, this study was performed to collect the opinions of Pediatric health care professionals, working in emergency departments, regarding the efficacy and safety of the influenza vaccine. An anonymous online survey was employed to evaluate socio-demographic and professional characteristics, knowledges, beliefs and attitudes. Results Five hundred four health care professionals completed the survey: 331 physicians, 140 nurses and 33 other health are professionals. During the 2017–18 season, 55.8% of physicians, 19.3% of nurses and 12.1% of other health care professionals had vaccinated against the influenza virus. Not vaccinated physicians and nurses with less than 40 years of age were fewer than not vaccinated physicians and nurses with more than 40 years of age. Nurses and other health care professionals were less trustworthy of the influenza vaccination, less aware of the possibility of contracting and transmitting influenza and other vaccine-preventable diseases. Conclusions Insufficient adherence to the influenza vaccination in physicians, nurses and other health care professionals is a concern for those assisting high-risk patients, especially in emergency departments. Therefore, it is vital to promote education of health care professionals and students regarding vaccinations. High vaccine coverage should be embedded in the safe hospital paradigm and should become a goal for the hospital's directors.

Objective: To evaluate whether the use of a surgical and N95 mask for overweight and obese children was associated with respiratory distress. Methods: We enrolled 15 healthy and 14 overweight or obese children. We performed two sessions: one wearing a surgical, the other an N95 mask. We tracked changes in partial pressure of end-tidal carbon dioxide (PETCO2), oxygen saturation (SaO2), pulse rate (PR), and respiratory rate (RR) during a 72 min test: 30 min without a mask, 30 min wearing a mask, and then during a 12 min walking test. Results: In healthy children, there was no significant change in SaO2 and PETCO2 during the study; there was a significant increase in PR and RR after the walking test with both the masks. In overweight or obese children, there was no significant change in SaO2 during the study period; there was a significant increase in PETCO2 as fast as wearing the mask and an increase in PETCO2, PR, and RR after walking test. After the walking test, we showed a significant correlation between PETCO2 and body mass index. Conclusion: Overweight or Obese children who wear a mask are more prone to developing respiratory distress, which causes them to remove it frequently. In a crowded environment, they are at greater risk of infection. For this reason, it is desirable that they attend environments where everyone uses a mask.

Objective: Our aim was to evaluate the safety of COVID-19 vaccine in children resident in the Latina Local Health Authority. Methods: We conducted a telephone survey among children aged 5–11 years receiving BNT162b2 mRNA COVID-19 vaccine between December 15 and 21. The main outcomes included the presence of allergic reactions or anaphylaxis, adverse events after 24–48 h, 7 and 20 days of taking the first and second doses of medications, and documented SARS-CoV-2 infection after vaccination. The information obtained was automatically linked to a spreadsheet and analyzed. Results: 569 children were enrolled. The mean age was 114 ± 4.24 months; there were 251 males in the study. The vaccine showed a favorable safety profile; no anaphylaxis or serious adverse events were reported. The most common symptoms both after the first and second dose were injection site reactions, asthenia, and headache. Injection site reactions were more frequent after the first dose (p = 0.01), while systemic symptoms were more frequent after the second dose (p = 0.022). These symptoms were more frequent in patients with comorbidities (p = 0.0159). Conclusion: Our findings confirm the safety of COVID-19 vaccine in children younger 11 years and could be useful to promote its diffusion in pediatric ages in order to achieve “herd immunity” and prevent the virus’s circulation.

At present, the vaccine authorized in children aged 5 years and older is the BNT162b2 messenger RNA COVID-19 vaccine. Unlike adults, there is limited data available in the pediatric age describing adverse events after vaccine. We report a case of adenomesenteritis in a young girl following the first dose of vaccine.

Objective: To evaluate how the restrictive measures implemented during the SARS-CoV-2 pandemic have influenced the incidence of the most common children’s diseases and the consumption of medications in 2020 compared to 2019. Methods: We involved all family pediatricians of the local health authority of Latina, from which we requested data of monthly visits in 2019 and 2020 for six common diseases disseminated through droplets and contact, and the territorial and integrative pharmaceutical unit of the area, from which we requested data of the net expenditure regarding the most commonly used drugs at pediatric age. Results: There was significant reduction in the incidence of the evaluated diseases and in the consumption of investigated drugs between 2019 and 2020 in the months when the restrictive measures were in place, with an attenuation of this effect during the months of the gradual loosening of those measures. Conclusion: Nonpharmaceutical intervention measures have caused changes in the diffusion of common pediatric diseases. We believe that the implementation of a reasonable containment strategy, even outside of the pandemic, could positively influence the epidemiology of infectious and allergic diseases in children, and healthcare system spending.

Background: actual literature suggests that children of methylmalonic acidemia patients are mostly healthy, but data are only partial, especially regarding long-term outcome. Therefore, our aim was to evaluate the possible long-term neurological effects of fetal exposure to high levels of methylmalonic acid in a child of a renal transplant recipient. Methods: we retrospectively evaluated the clinical and neurological records of a girl whose mother is a kidney transplant recipient affected by methylmalonic acidemia. Subsequently, we compared our results with the ones already published. Results: the girl’s weight and stature were within the normal range in the first years of life but, starting from 4 years of age, she became progressively overweight. Regarding the neurodevelopment aspects, for the first time we performed a complete and seriated neuropsychological evaluation, highlighting a mild but significant weakness in the verbal domain, with a worsening trend at three-year revaluation. Conclusions: since children of MMA patients are exposed to methylmalonic acid, the efforts of the physicians caring for these children should be directed on careful evaluation of growth, prevention of obesity and regular neurological examination together with structured neuropsychological tests to achieve a better insight in possible complications of pregnancy in patients suffering from this condition.

Abstract Background It is not yet clear if blood pressure and renal function changes evolve differently in children with a congenital or acquired solitary functioning kidney. This study aims to assess if there are any differences between these two types of solitary kidney patients. Methods Current research is a retrospective study assessing the evolution of glomerular filtration rate, proteinuria, and blood pressure in clinical records of 55 children with a solitary functioning kidney (37 congenital and 18 acquired). We used the medical records of children who had been assisted, in our unit of pediatric nephrology, for a period of 14 years (168 months), from the time of diagnosis, between January/1997 and December/2015. Results During the study period, glomerular filtration rate (T0 128.89 ± 32.24 vs T14 118.51 ± 34.45 ml/min/1.73 m2, p NS) and proteinuria (T0 85.14 ± 83.13 vs T14 159.03 ± 234.66 mg/m2/die, p NS) demonstrated no significant change. However, after 14 years of follow-up 76.4% of patients had increased levels of arterial hypertension with values over the 90th percentile for gender, age, and height. Specifically, children with an acquired solitary functioning kidney mainly developed hypertension [T0 2/17 (12%) vs T14 9/17 (52.9%) p

Lung ultrasound has become increasingly used in both adult and pediatric populations, allowing the rapid evaluation of many lung and pleura diseases. This popularity is due to several advantages of the method such as the low cost, rapidity, lack of ionizing radiation, availability of bedside and repeatability of the method. These features are even more important after the outbreak of the SARS-CoV-2 pandemic, given the possibility of recognizing through ultrasound the signs of interstitial lung syndrome typical of pneumonia caused by the virus. The purpose of this paper is to review the available evidence of lung ultrasound (LUS) in children and its main applications in pediatric diseases.

Cerebral palsy (CP) is a frequent cause of childhood disability often associated with a complex group of disorders, including epilepsy, which is reported to impact approximately 40% of affected individuals. This retrospective study involved a group of children affected by CP, some of whom also had comorbid epilepsy. The aim of this study was to report our experience of analyzing, in particular, (a) some of the clinical aspects of the different type of CP, and (b) the relationship between the clinical data of children affected by CP plus epilepsy and each type of CP. Methods: This retrospective single-center study was performed with 93 children admitted to the Pediatric Department of the University of Catania, Italy, affected by CP and distinguished according to the type of motor clinical presentation, with 46 showing epileptic seizures, compared to a control group of 136 children affected by epilepsy without other neurologic disorders. Results: Among the 93 CP children, 25 (27%) had spastic quadriplegia (plus one patient with dystonic quadriplegia), 39 (42%) had spastic hemiplegia, 11 (12%) had spastic diplegia (plus two with ataxia and one with dyskinetic CP), and 14 (15%) did not have a well-defined type of CP. The frequency of epilepsy was higher in affected CP children who showed major motor dysfunction (GMFCS IV–V types). As regards the 46 children with CP plus epilepsy, compared to the group of the control, the age of epilepsy onset was found to be statistically significant: 21 ± 35.1 months vs. 67 ± 39.7. Conclusions: Epilepsy represents one of the most frequent comorbidities of cerebral palsy. In children with CP, particular attention should be paid to the early identification and treatment of comorbid epilepsy.

Background: Zoonoses are human infectious diseases caused by pathogens that primarily infect animals. Mediterranean Spotted Fever (MSF) represents one such example, affecting the Mediterranean region, in which household animals can be immune-carriers of infected ticks. Materials and methods: We retrospectively analysed the incidence and the clinical and laboratory features of MSF caused by R.Conorii in children admitted to the Paediatric Operative Unit from 1987 to 2010, for persistent fever and generalised macular-popular erythematous lesions. Clinical, immunological and serological parameters of 55 cases of Rickettsia infections observed in children between 2 and 11 years of age were collected. Results: We found an increasing incidence of MSF in childhood from 1987 to 2010. Diagnosis of MSF at the moment of hospital admission was done in 16 patients (29.09%). The presence of the typical Tache noire was observed in 16 cases out of 55 patients (29.09% of cases). We noticed a different representation of R. conorii antigens in serological testing over the time period of the study, corresponding to overall higher incidence rates for infection in the latter years. We also observed a higher incidence of infection in those years in which all four antigens were found positive at serum testing with respect to those years in which only two of the four antigens were observed (1987-1990: 0-16%; 2007-2010: 0.46%; P

Array-comparative genomic hybridization (Array-CGH) has been proposed as the first efficient approach to scan the entire genome for variations in DNA copy number. This diagnostic method is based on the study of total genomic DNA isolated from any sample and reference cell populations, differentially labelled and hybridized to DNA microarrays. The method has been initially applied in clinical genetics research and has recently also been used in cancer research, as tumor genomes have a wide variety of copy number phenotypes, indicating different types of genetic instability. In this field, array-CGH has been demonstrated to be an efficient diagnostic method to provide information on the location of important cancer genes. Recently the use of array-CGH has been expanded, including the analysis of constitutional abnormalities, to diagnose subtending mutations of neurologic diseases, with promising results in diagnosing genetic mutations otherwise not evident with other genetic tests. We performed a prospective study on the efficiency of array-CGH in the genetic-molecular diagnosis of pediatric patients affected by developmental delay and mental retardation associated with clinical signs of dimorphism and/or other relevant neurological symptoms. In our study, we had a detection rate of 22.71% by array-CGH analysis and we were able to take more precise genetic information for microdeletion and microduplication of our cohort of patients with developmental delay and/or idiopathic mental retardation and/or dimorphic face and/or epilepsy. In our opinion, we think that these findings would be helpful in early diagnosis and family genetic counseling, above all in those clinical neurologic cases in which other diagnostic tests have not succeed in performing a diagnosis.