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Background Although children with special health care needs (CSHCN) represent a minority of the population, they go through more hospitalizations, more admissions to the Emergency Department (ED), and receive a major number of medical prescriptions, in comparison to general pediatric population. Objectives of the study were to determine the reasons for admission to the ED in Italian CSHCN, and to describe the association between patient’s demographic data, clinical history, and health services requirements. Methods Ad hoc web site was created to collect retrospective data of 3479 visits of CSHCN to the ED in 58 Italian Hospitals. Results Seventy-two percent of patients admitted to ED were affected by a previously defined medical condition. Most of the ED admissions were children with syndromic conditions (54%). 44.2% of the ED admissions were registered during the night-time and/or at the weekends. The hospitalization rate was of 45.6% among patients admitted to the ED. The most common reason for admission to the ED was the presence of respiratory symptoms (26.6%), followed by gastrointestinal problems (21.3%) and neurological disorders (18.2%). 51.4% of the access were classified as ‘urgent’, with a red/yellow triage code. Considering the type of ED, 61.9% of the visits were conducted at the Pediatric EDs (PedEDs), 33.5% at the Functional EDs (FunEDs) and 4.6% at the Dedicated EDs (DedEDs). Patients with more complex clinical presentation were more likely to be evaluated at the PedEDs. CSHCN underwent to a higher number of medical procedures at the PedEDs, more in comparison to other EDs. Children with medical devices were directed to a PedED quite exclusively when in need for medical attention. Subjects under multiple anti-epileptic drug therapy attended to PedEDs or FunEDs generally. Patients affected by metabolic diseases were more likely to look for medical attention at FunEDs. Syndromic patients mostly required medical attention at the DedEDs. Conclusions Access of CSHCN to an ED is not infrequent. For this reason, it is fundamental for pediatricians working in any kind of ED to increase their general knowledge about CHSCN and to gain expertise in the management of such patients and their related medical complexity.

A three-month-old infant was referred to the emergency department with fever and a right cervical abscess. Nasopharyngeal swab was positive for SARSCoV- 2 infection. Main causes of infectious abscess and immunodeficiency disorders were ruled out. After a surgical drainage and an antibiotic therapy, clinical conditions of the infant improved, and Staphylococcus Aureus was found in the wound drainage culture. This is a possible, not yet described in infant patients, concurrent clinical manifestation of SARS-CoV-2 infection.

Aim Italian national guidelines on pain management were published in 2010, but there is little information on how effective pain management is in paediatric emergency care, with other countries reporting poor levels. Using headache as an indicator, we described pain assessment in Italian emergency departments and identified predictors of algometric scale use. Methods All Italian paediatric and maternal and child hospitals participated, plus four general hospitals. Data on all children aged 4–14 years admitted during a one-month period with headache as their chief complaint were abstracted from clinical records. Multivariable analyses identified predictors of algometric assessment, taking into account the cluster study design. Results We studied 470 admissions. During triage, pain was assessed using a standardised scale (41.5%), informally (15.5%) or was not recorded (42.9%). Only 32.1% of the children received analgesia in the emergency department. The odds ratios for predictors of algometric assessment were non-Italian nationality (3.6), prehospital medication (1.8), admission to a research hospital (7.3) and a more favourable nurses-to-admissions ratio of 10.8 for the highest versus lowest tertile. Conclusion Despite national guidelines, paediatric pain assessment in Italian emergency care was suboptimal. Hospital variables appeared to be stronger predictors of adequate assessment than patient characteristics.

Detailed data on clinical presentations and outcomes of children with COVID-19 in Europe are still lacking. In this descriptive study, we report on 130 children with confirmed COVID-19 diagnosed by 28 centers (mostly hospitals), in 10 regions in Italy, during the first months of the pandemic. Among these, 67 (51.5%) had a relative with COVID-19 while 34 (26.2%) had comorbidities, with the most frequent being respiratory, cardiac, or neuromuscular chronic diseases. Overall, 98 (75.4%) had an asymptomatic or mild disease, 11 (8.5%) had moderate disease, 11 (8.5%) had a severe disease, and 9 (6.9%) had a critical presentation with infants below 6 months having significantly increased risk of critical disease severity (OR 5.6, 95% CI 1.3 to 29.1). Seventy-five (57.7%) children were hospitalized, 15 (11.5%) needed some respiratory support, and nine (6.9%) were treated in an intensive care unit. All recovered.Conclusion:This descriptive case series of children with COVID-19, mostly encompassing of cases enrolled at hospital level, suggest that COVID-19 may have a non-negligible rate of severe presentations in selected pediatric populations with a relatively high rates of comorbidities. More studies are needed to further understand the presentation and outcomes of children with COVID-19 in children with special needs. What is Known:• There is limited evidence on the clinical presentation and outcomes of children with COVID-19 in Europe, and almost no evidence on characteristics and risk factors of severe cases. What is New:• Among a case series of 130 children, mostly diagnosed at hospital level, and with a relatively high rate (26.2%) of comorbidities, about three-quarter had an asymptomatic or mild disease.• However, 57.7% were hospitalized, 11.5% needed some respiratory support, and 6.9% were treated in an intensive care unit.

Chest pain is a relatively common cause of admission to the Emergency Department, being related in most of the cases to benign conditions with only a minority of the cases affected by heart disease. Limited data are available about the follow up of these patients in terms of risk of recurrence of symptoms, Emergency Department re-admissions, level of impairment, and school absenteeism. We identified 761 children who visited our ED with the chief complaint of chest pain, equal to 0.68% of all admissions. Twenty-four patients were excluded for a previous history of cardiac disease. Eight (1%) patients were determined to have chest pain of cardiac origin. Ninety-seven percent of patients were successfully contacted by telephone: 69% agreed to answer the questionnaire. Of these, 33% experienced recurrent chest pain, up to 41% was forced to be absent from school, about 20% was limited in its daily activities, and about 20% repeated a cardiologic evaluation. Conclusion: Chest pain is mainly due to benign causes and is a recurrent symptom in a high percentage of patients, associated with re-admission and school absenteeism.What is Known:• Chest pain is a relatively common cause of admission to the Emergency Department.• It is mainly due to benign causes and is among the most common reasons for referral to the pediatric cardiologist.What is New:• Chest pain is a recurrent symptom associated with re-admission and school absenteeism.• As a family history of cardiac disease or exertional symptoms are a well-known red flag for chest pain of cardiac origin, school absenteeism should be considered a red flag for symptoms related to psychological distress in patients with non-cardiac chest pain.

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Introduction Early introduction oral immunotherapy (E‐OIT) in the first year of life can be a safe treatment for infants with cow's milk allergy (CMA). Once the protocol is completed, doubts remain whether children achieve tolerance or remain desensitized. According to current guidelines, this is determined by an avoidance period followed by a re‐exposure to the food allergen during an in‐hospital oral food challenge (OFC). In real life, this approach can be complicated, time‐consuming, and anxiety‐provoking for parents. We assessed the long‐term safety of E‐OIT for CMA in a cohort of children who switched to an unrestricted diet without testing the achievement of tolerance at the end of the OIT protocol. Materials and Methods We performed a descriptive analysis of the clinical follow‐up of a cohort of children diagnosed with IgE‐mediated CMA and undergoing E‐OIT protocol in their first year of life. In a previous publication, the same cohort of patients had been studied to assess the feasibility of E‐OIT for CMA. In the present study, we reported the results of a telephone survey, carried out through a questionnaire to their families enquiring about milk consumption and other ongoing atopic conditions of children. Results After an average of 4 years from the start of E‐OIT, 62/73 patients (85% of the historical cohort) participated in the survey. Among them, all 56 patients who had previously successfully completed the protocol reported an unrestricted cow's milk intake. Ninety–three percent of these children did not experience any further allergic reactions, while the remaining 7% described only mild and transitory reactions until the 6‐month period after the end of the protocol. Conclusions This study confirmed the long‐term safety of E‐OIT for CMA and challenged the paradigm of the need for allergen food withdrawal to discern between desensitization and tolerance. It could be a starting point for planning future trials on this issue.

Clubfoot is the most frequent congenital malformation of the foot, affecting more than 1–2 subjects per 1.000 newborns. Without appropriate treatment, a child with congenital clubfoot will never be able to walk physiologically with a dramatic impact on the quality of life. In the last decades, different corrective solutions have been proposed, and there is rising scientific evidence that the Ponseti non-invasive method is safe and effective in the treatment of the clubfoot. So, what should a general paediatrician know about this condition and what should he concretely do in the suspect of a congenital clubfoot?

Summary The multidisciplinary International Committee for the Advancement of Procedural Sedation presents the first fasting and aspiration prevention recommendations specific to procedural sedation, based on an extensive review of the literature. These were developed using Delphi methodology and assessment of the robustness of the available evidence. The literature evidence is clear that fasting, as currently practiced, often substantially exceeds recommended time thresholds and has known adverse consequences, for example, irritability, dehydration and hypoglycaemia. Fasting does not guarantee an empty stomach, and there is no observed association between aspiration and compliance with common fasting guidelines. The probability of clinically important aspiration during procedural sedation is negligible. In the post‐1984 literature there are no published reports of aspiration‐associated mortality in children, no reports of death in healthy adults (ASA physical status 1 or 2) and just nine reported deaths in adults of ASA physical status 3 or above. Current concerns about aspiration are out of proportion to the actual risk. Given the lower observed frequency of aspiration and mortality than during general anaesthesia, and the theoretical basis for assuming a lesser risk, fasting strategies in procedural sedation can reasonably be less restrictive. We present a consensus‐derived algorithm in which each patient is first risk‐stratified during their pre‐sedation assessment, using evidence‐based factors relating to patient characteristics, comorbidities, the nature of the procedure and the nature of the anticipated sedation technique. Graded fasting precautions for liquids and solids are then recommended for elective procedures based upon this categorisation of negligible, mild or moderate aspiration risk. This consensus statement can serve as a resource to practitioners and policymakers who perform and oversee procedural sedation in patients of all ages, worldwide.

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Case presentationA 10-month-old boy was admitted to the emergency department due to a sudden onset of left unilateral mydriasis (figure 1). His medical history was unremarkable. A minor head trauma 2 days before was reported, without alarming signs or symptoms. His mother was putting him to sleep, after coming back from work, when she noticed a different pupil size and promptly went to the ED with her husband. The parents denied any use of medications, including nebulised therapy or direct contact with plants. The child was well appearing and his vital signs were within the standard age limits. His extraocular motility was normal as well as the rest of his neurological and physical examination. Parents’ behaviour was somehow remarkable. Even though the child was not suffering, the mother seemed very worried while the father was nervous and aggressive, repeatedly asking for a discharge.Figure 1Left unilateral mydriasis.­QuestionsWhat is the most likely diagnosis based on this clinical presentation?Local contact with a mydriatic substanceIntracerebral haemorrhageBrain tumourThird nerve palsyWhat would be the next step in the investigation to confirm this diagnosis?Brain CTBrain MRIFundus oculi examinationToxicological screening of urineHow is this condition managed, and what is the prognosis?Answers can be found on page XX

A 15-year-old girl presented with a 3-month history of non-pruritic hyperpigmented macules on her back (figure 1). Her history included severe obesity and hyperinsulinism. The lesions were previously diagnosed as acanthosis nigricans. Figure 1 Well-demarcated, coalescing, hyperpigmented macules on the patient’s back. Physical examination showed well-demarcated, coalescing and finely scaling macules on the back. Examination with a Wood lamp revealed a yellow-green fluorescence. A diagnosis of pityriasis versicolor (PV) was made. Oral treatment with 2 weeks of fluconazole was …

Opioid-related mortality in adolescents is spreading in the US, with prescription opioids playing a crucial role in the development of addiction. We traced back to the process leading to the so called “opioid overflow”, trying to identify any modifiable attitude.Since the late 1990s, pain was labelled as the “fifth vital sign” and its proper management was prompted, encouraging the use of opioids for any pain scored at a Numerical Rating Scale (NRS) of 7 or higher. This assumption has some remarkable limitations. NRS is a proxy of pain severity in children, and pain measurement should be strengthened by a more comprehensive pain evaluation. Moreover, while remaining a fundamental therapeutic right of patients suffering postoperative or chronic severe pain, opioids show no evidence of superiority respect to non-opioid regimens in the management of pain from several acute conditions.Italy, as other European countries, is often reluctant to the use of opioids, even when highly recommendable, missing the opportunity of properly treating those selected patients with severe pain. Both attitudes can be viewed as the result of an extreme simplification of the complex process of pain evaluation and treatment, by means of a ‘one-size-fits-all’ approach.This highlights the need for a systematic and patient-tailored attitude to children in pain, avoiding applying guidelines without question. Good clinical practice must rely on guidelines, which, however, as often based on partial and insufficient data, can be questioned by emerging new evidence, and should not substitute our rational thinking, and capability to understand each patient, avoiding excessive conformism.

A healthy 5-year-old boy presented with a 10-day history of painless, swollen and hyperaemic eyelids without conjunctival injection or fever. Baseline blood tests including inflammatory markers were unremarkable except for mild raised levels of erytrocyte sedimentation rate (38 mm/hour). Physical examination was totally negative with no sign of sinusitis. During the recovery, 3 days after the admission, an orthoptic evaluation showed bilateral upper eyelid ptosis (figure 1) and painful extrinsic ocular movements, with a restriction of upgaze in both eyes. Biomicroscopy showed conjunctival hyperaemia and chemosis; fundus oculi was normal. Figure 1 Bilateral upper painless swollen and hyperaemic eyelids with associated ptosis. Which of the following do you think is the most likely diagnosis in this patients? 1. Orbital cellulitis . 2. Orbital myositis. 3. Thyroid orbitopathy. 4. Myasthenia gravis. Which is the next diagnostic step to confirm the clinical hypothesis? 1. Orbital biopsy. 2. CT. 3. MRI. 4. Visual …

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Background We reported the case of a two-old-year boy with a painful acute hemorrhagic edema. This is a self-limited benign condition: usually, affected children are well appearing and this strongly support the diagnosis. In the opposite, in our case, we observed a painful presentation of the edema. Therefore, we demonstrated that rarely, this condition could have also a painful presentation. Conclusions This case report helps clinician to know that also acute hemorrhagic edema could have a painful presentation, so we must considered it in the differential diagnosis with sepsis, sickle cell crisis and child abuse. We believe that these findings will be of interest to pediatricians.

A 6-year-old boy was evaluated for a 6-week history of low back pain. Initially, the pain was exacerbated by movements, eventually showing a milder and fluctuating trend. History was unremarkable for previous traumatic events, fever or nocturnal pain. Physical examination revealed localised pain at palpation of the spinous processes at the lumbosacral level. Blood tests showed a normal blood count, negative C reactive protein (CRP) and erythrocyte sedimentation rate, normal lactic acid dehydrogenase (LDH) and creatine phosphokinase.A posterior–anterior radiograph of the lumbar spine resulted normal. An MRI scan revealed a lumbosacral transitional vertebra with bone oedema of the posterior arch until the spinous process.For better bone definition, a CT scan was performed (figure 1).Figure 1CT scan of the transitional lumbosacral (L5) vertebra.QuestionsWhich causes of persistent low back pain should be ruled out in children under 10 years of age?OsteochondrosisNeoplasmFunctional painInfectionsWhat is the diagnosis in this patient?How is the diagnosis performed?How is this condition managed?Answers can be found on page 2.

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Background Plexiform neurofibromas (PNs) are congenital tumors that affect around 50 % of the subjects with neurofibromatosis type 1. Despite being histologically benign, PNs can grow rapidly, especially in the pediatric age, and cause severe morbidities. In the past, various therapeutic approaches have been proposed to treat these masses, none of which obtained valuable results. Selumetinib, an inhibitor of mitogen-activated protein kinase (MEK) 1 and 2, has been the first molecule to demonstrate the ability of tackling the growth of PNs. The drug’s most common side effects, which usually are mild or moderate, include gastrointestinal symptoms (diarrhea, abdominal pain), dermatologic manifestations (maculo-papular and acneiform rash, paronychia, mucositis), and various laboratory test abnormalities (elevation of creatine kinase and aminotransferase). Cases presentation We report two previously undescribed adverse events in pediatric patients: peripheral edema and hair color change. The first case of peripheral edema occurred in a 7-year-old boy affected by a severe form of NF1, after two years of treatment with selumetinib at the standard dose (25 mg/m2twice a day). The edema involved the right leg, and the patient did not complain of pain. The second case of peripheral edema occurred in a 12-year-old girl after six months of therapy with selumetinib at the standard dose, involving her lower left leg. The patient initially complained of pain in that area, but it gradually and spontaneously resolved. In both patients, all the radiological exams, including lymphoscintigraphy, pelvic and abdominal ultrasound, and doppler ultrasound of the affected limb, as well as blood tests, revealed no abnormalities. Hair color change appeared in a 4-year-old boy after six months of therapy at the standard dose. The boy’s hair, whose natural color was dark blonde, became lighter in some areas. Despite the appearance of these side effects, all the patients and their families decided to continue the treatment with selumetinib, in considerations of its clinical benefits. Conclusions Since the use of selumetinib to treat plexiform neurofibromas is increasing in the pediatric population, clinicians should be aware of its side effects, so to decide whether continuing the treatment, reducing the dose or even interrupting it, when appropriate.

A 33+1-week female baby was born by caesarean section for worsening hydrops fetalis (HF) detected in the third trimester of pregnancy. Prenatal ultrasound showed a moderate enlargement of the right heart sections with a mild tricuspid insufficiency, without tachycardia, polyhydramnios, placentamegaly, renal agenesis and oesophageal atresia. At delivery, she developed transient hypotonia and apnoea and received nasal continuous positive airway pressure. Physical examination showed tachypnoea (70 breaths/min) and a moderate generalised subcutaneous oedema. Chest X-ray showed a cardiothoracic index of 0.82. At 90 min of life, her heart rate was 200–250 per minute. An ECG showed a paroxysmal supraventricular tachycardia (PSVT) with narrow complexes (figure 1). Vagal manoeuvres and five boluses of adenosine (250 μg/kg/dose) were ineffective, but she responded to amiodarone. Echocardiography showed severe tricuspid insufficiency and dilated right cardiac chambers. The baby presented further transient PSVT episodes, but these gradually improved on amiodarone, hydrochlorothiazide, spironolactone and furosemide. A primitive tricuspid valvulopathy with heart failure secondary to PSVT was diagnosed. …

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MED13-related disorder is a new neurodevelopmental disorder recently described in literature, which belongs to the group of CDK8-kinase module genes-associated conditions. It is characterized by variable intellectual disability and/or developmental delays, especially in language. Autism spectrum disorder (ASD), attention deficit hyperactivity disorder (ADHD), eye or vision problems, hypotonia, mild congenital hearth abnormalities and dysmorphisms have been described among individuals with MED13 mutations. We report the case of a 13-year-old girl who received a previous clinical diagnosis of Kabuki syndrome (KS) without mutations in classic KS genes. After a whole exome sequencing (WES) analysis a de novo missense mutation in MED13 (c.C979T; p.Pro327Ser) was found. This variant has been once described in literature as accountable for a novel neurodevelopmental disorder. The aim of this report is to improve clinical delineation of MED13-related condition and to explore differences and similarities between KS spectrum and MED13-related disorders.

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A male preterm infant was born at 36 weeks of gestational age by vaginal delivery after an uncomplicated monochorionic diamniotic twin pregnancy. He was the fourth child, and his neonatal weight was 2420 g. At birth, a downward right deviation of the mouth was noted during crying (figure 1), with no asymmetry at rest. No other asymmetric features were observed. His twin brother had an unremarkable physical examination. Figure 1 A newborn with asymmetric face due to congenital hypoplasia of the depressor anguli oris muscle. A full-term female infant was born to a primigravida mother by emergency caesarean section after unsuccessful attempts of vaginal delivery by means of vacuum extractor and forceps for mechanical dystocia. Neonatal weight was 3335 g. At birth, an asymmetric crying face was noted with downward deviation of the mouth on the right side (figure 2). The left eye remained persistently open, and the left side of the forehead and the …

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Background: This study investigated whether performing kidney ultrasound (KUS) only in children presenting either a pathogen other than E. coli at their first febrile urinary tract infection (fUTI) or experiencing fUTI recurrence would increase missed diagnoses of kidney anomalies. Methods: Patients aged 2–36 months with fUTI who underwent KUS evaluation from 2 January 2013 to 31 June 2018 were enrolled. Cystourethrography was performed after pathological KUS or recurring fUTIs. Thereafter, we retrospectively assessed the detection rate of kidney anomalies through performing KUS only in patients with atypical pathogen at first fUTI or with recurring fUTIs. Results: In 263 patients included, the isolated pathogen was E. coli in 223 cases (84.8%) and atypical in 40 cases (15.2%). KUS detected kidney anomalies in 14/223 (6.3%) of fUTIs caused by E. coli and in 11/40 (27.5%) of fUTIs caused by an atypical pathogen (OR 5.5, 95%CI 2.5–14.5). Cystourethrography was performed in 40 patients and vesicoureteral reflux (VUR) found in 20 cases. None of the high grade VUR diagnoses or other kidney anomalies would have been lost through a different diagnostic protocol that required the presence of an atypical pathogen at the first fUTI or a fUTI recurrence to perform the KUS. Conclusions: A diagnostic protocol that requires presence of an atypical pathogen at the first fUTI or a second episode of fUTI to perform the KUS would allow a reduction in the number of negative ultrasounds with a negligible risk of missed diagnoses of kidney anomalies.

BackgroundHome chemotherapy programs for children with cancer are safe and feasible, and their impact on the quality of life has been reported in different countries. A home chemotherapy program was implemented between 2011 and 2019 in an Italian region. This pilot study investigates its safety and feasibility, along with parental satisfaction.MethodsPatients between 0 and 18 years diagnosed with malignancy were included. Deceased patients and patients whose families moved abroad or interrupted contact with the service were excluded. Adverse events comprised immediate deterioration of the patient’s condition, equipment failure, errors in drug storage, dose or patient identification and personnel safety issues. Parental satisfaction was explored through an email survey of 32 Likert-type and short open questions.ResultsThirty-five patients received 419 doses of intravenous chemotherapy at home (cytarabine, vincristine, vinblastine). No adverse events were reported. Twenty-three families out of 25 eligible completed the survey. Most reported being “very satisfied” with the possibility of maintaining a work/domestic routine and reducing time and financial burden of hospital access. Most were “very satisfied” with the opportunity for their child of being less troubled by the treatment. Besides, most reported being “very satisfied” with the chance for healthy siblings of maintaining their routine and coping with their brother/sister’s disease. Most perceived the program as safe. All families recommended extending the program to all children in the region.ConclusionsThis first Italian study supports home chemotherapy as safe and effective, positively influencing the quality of life for children and their families.

Background: Plexiform neurofibromas (PN) are congenital tumors that affect up to 50% of individuals with neurofibromatosis type 1. Despite their benign nature, they can grow rapidly and cause severe morbidities. Selumetinib, an inhibitor of mitogen-activated protein kinase (MEK) 1 and 2, was reported to induce a clinical response in pediatric subjects with inoperable PN. Objective: The aim of this paper is to describe a prospective case series of patients treated with selumetinib with emphasis on drug adverse events. Patients and methods: All the subjects who received selumetinib at the Pediatric Department of Scientific Research Institute and Hospital “Burlo Garofolo”, from November 2017 to January 2020, were progressively included. We monitored the patients with a follow-up visit every 3 months. MRI or CT scans to monitor the growth of the tumor were performed after 3 months of treatment, and then every 6–9 months. Results: Selumetinib was prescribed to nine children, with a total of 17 inoperable PN. The mean follow-up period was 12 months. During the follow-up, one patient experienced an ischemic stroke, unrelated to the treatment. Only minor adverse events were observed: six individuals developed gastrointestinal side effects, seven patients presented a mild form of acne, six had paronychia, four developed irritability, and two showed a mild increase in creatine kinase. None of the patients stopped the treatment. Tumor reduction > 20% was recorded in 16 out of 17 PN (94%). One PN remained stable. No tumor growth was recorded during the treatment. Conclusions: In this case series, selumetinib appears to be effective and safe for the pediatric population.

Background:Congenital dacryostenosis is one of the most common ophthalmological disorders in infants, with a high spontaneous resolution rate. In patients unresponsive to conservative treatment, the first-line approach is lacrimal drainage system probing, thought there is no clear consensus on optimal timing of surgery. The optimal treatment of patients unresponsive to primary probing is also controversial.Objectives:The aim of this study is to assess the optimal timing of probing in children with congenital dacryostenosis. Other purposes are to evaluate the efficacy of repeated probing and dacryointubation in patients unresponsive to the initial surgery without evident lacrimal outflow dysgenesis, and to determine the epidemiology of these maldevelopments.Methods:A retrospective consecutive cohort study was conducted in 625 eyes of 457 patients aged 7–48 months who underwent surgery for dacryostenosis. Patients were divided into 4 cohorts according to the timing of surgery. Data were analyzed using Fisher's test.Results:The success rate of primary probing was high, without significant differences between cohorts. One-third of recurrences were related to maldevelopments, the other two-thirds were treated with a second probing or dacryointubation, with high success rates, that did not significantly differ between the procedures. All cases unresponsive to the second surgery were resolved with dacryointubation.Conclusions:Probing is highly effective and its outcome is not affected by timing of surgery. Nevertheless, we advocate for early intervention, in order to identify possible maldevelopments, which require more invasive management. In patients unresponsive to primary probing, without evident maldevelopments, repeated probing should still be considered as the first-line approach, since it's less invasive but similarly effective to dacryointubation.

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BackgroundThe study aimed to investigate if the behaviours suggestive of ADHD were more frequent in a population of children attending the Emergency Department (ED) for injuries, rather than for other causes.MethodsA cross-sectional study was carried out. Patients, aged 6 to 17 years, attending the ED for acute injuries and other causes were considered cases and controls, respectively. We used a questionnaire, which investigates the presence in the child of inattention, hyperactivity, and impulsivity. The primary outcome was the number of children with behaviours suggestive of ADHD in cases and controls.ResultsFive hundred forty-five children were enrolled, 251 with injuries and 294 with other complains. Twenty two out of two hundred fifty one (9%) children visited for injuries, and 30 out of 294 (10%) visited for other causes had behaviours suggestive of ADHD (p = 0.661). Among these cases, children with evocative ADHD scores had a higher probability (OR 4.52; 95% CI 1.45–14.04;p = 0.009) of having had more than five previous ED accesses due to injury, compared to the others.ConclusionsThis study did non shown a difference in behaviours suggestive of ADHD between cases and controls, but identified a population of children with behaviours suggestive of ADHD who more frequently access the ED for injuries.

A 13-year-old boy was admitted for a 4-day history of fever, malaise, sore throat and cough, treated with amoxicillin for 2 days. Physical examination revealed sparse targetoid cutaneous lesions, stomatitis with vesicles, blisters and mild conjunctival hyperaemia (figure 1). Blood tests showed an increase in erythrocyte sedimentation rate (92 mm/hour; normal value

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Background Eosinophilic gastrointestinal disorders (EGID) are characterized by eosinophilic inflammation and are subclassified according to the affected site(s) as eosinophilic esophagitis, eosinophilic gastritis, eosinophilic enteritis and eosinophilic colitis. Clinical presentation includes dyspeptic symptoms, vomiting, abdominal pain, diarrhoea and gastrointestinal bleeding. Peripheral eosinophilia is usually found but is not required for the diagnosis. The treatment is based on dietary elimination therapy, consisting of removal of common food triggers, most frequently cow’s milk in infants. Corticosteroids are used as first line drug therapy in EG if dietary therapy fails to achieve an adequate clinical response or is impractical. Case presentation A four month old infant was admitted for an episode of melena and hematemesis. An esophagogastroduodenoscopy showed haemorrhagic gastritis with ulcerative lesions and fibrin. A significant gastric bleeding was noted after the procedure. The gastric mucosa biopsies showed an eosinophilic infiltration. Conclusions A clinically relevant anaemia is a quite rare complication in infants with eosinophilic gastritis and a biopsy may worsen bleeding, to a potentially severe level of low haemoglobin. In infants with low haemoglobin levels and suspect eosinophilic gastritis a watchful follow up after the biopsy should be considered, as well as the possibility of postponing the biopsy to reduce the bleeding risk.

Background Ogilvie’s syndrome is described in the adult population, but rarely seen in children. Case presentation We present a case of a girl who suffered acute colonic pseudo-obstruction after laparoscopic appendectomy. Conclusions Ogilvie’s syndrome, although rare in the pediatric population, should be considered as possible diagnosis after a surgical procedure in presence of persisting subocclusive symptoms and radiological signs of massive colonic dilatation without mechanical obstruction.

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The COVID-19 pandemic challenged health care systems to balance high standards of care with patients' needs and health care workers' safety.1 Pediatric sedation poses multiple potential contagion risks: aerosol generating procedures such as bag-valve mask ventilation and oro-pharyngeal suctioning2 , close proximity of the sedationist to the patient's airway, the need for airway manipulation or insertion of airway devices and the risk of patient vomiting and coughing. Although COVID risk mitigation to providers is achieved for elective procedures that allow for pre-sedation COVID-19 testing, it is important to recognize that there are still false negatives, with sensitivity cited to be as low as 73.3%3 . For this reason, thoughtful consideration should be directed to means of lowering the healthcare providers' risk of COVID-19 exposure during pediatric procedural sedation.

Background Monosomy 1p36 is the most common terminal deletion syndrome with an autosomal dominant pattern of inheritance. This syndrome is defined by an extremely wide spectrum of characteristics; however, developmental delay and intellectual disability of various degree are present in all patients and about the 90% of patients have a severe intellectual disability. Dental agenesis or other dental anomalies have not been described in previous reports. Case presentation We report the case of two little sisters born from healthy and non-consanguineous parents, presenting with dental anomalies and one of them with epilepsy, dilated cardiomyopathy with left-ventricular non-compaction, strabismus, history of poor growth, hypotonia and mild language delay. Patients were evaluated in several departments (genetic, child neuropsychiatric, cardiology, odontostomatology, ophthalmology, otorhinolaryngology) of Institute for Maternal and Child Health, IRCCS Burlo Garofolo, Trieste, Italy. They underwent investigations such as electrocardiogram, echocardiogram, dental orthopantomography X-Ray and Computed Tomography, electroencephalograms, abdomen ultrasound, blood tests, IQ tests, genetic analysis. They both have an Intelligence Quotient greater than 70 and a negative neurologic exam. Each sister carries the same 1p36 deletion of about 2.3 Mb. Genetic analysis of the parents’ blood samples (Single Nucleotide Polymorphism- array, karyotype and Fluorescent In Situ Hybridization) did not reveal any deletion, translocation or inversion and confirmed the paternity. A third sib of the probands does not carry the 1p36 deletion or other quantitative alterations. Conclusion This report describes a new trait linked to monosomy 1p36, namely a mild intellectual outcome associated with significant dental anomalies. Our finding suggests that 1p36 deletion syndrome may present with a mild cognitive impairment or even with a normal intellectual development: this is very important for the genetic counselling, especially in a prenatal setting. Moreover, we report the third study with recurrent 1p36 deletion syndrome in two siblings, likely due to germline mosaicism. Finally, we believe that the dental anomalies should be investigated in 1p36 deletion syndrome and that the spectrum of the condition could be broader than we assume.

Background Italian laws allow the protection of a child who is suspected to be a victim of abuse through a procedure that can be put in motion by the attending physician in any Emergency Department (article nr. 403 Civil Code) with a temporarily suspension of parental authority. This study aims at evaluating both the appropriateness of the activation of the protection procedure by ED doctors in cases of suspected child abuse in terms of judicial confirmation and how it impacts children in the long-term. Methods We selected cases in which the procedure was activated due to suspected child abuse. The children were admitted to the ED of a tertiary children hospital from 2006 to 2018. We then reviewed the medical charts and collected data from the social services through a questionnaire concerning the long-term outcomes . Results Twenty-eight patients were included (75% females, mean age 13.8 years). In 90% of cases the activation of the procedure in the ED was followed by a Court confirmation. Evaluation of long term outcome was possible in 22 cases. Among them, a positive social outcome was achieved in 15 cases (68.2%). The remaining abandoned the program or had critical reintegration in the family. Eighteen percent of patients developed major issues such as aberrant behaviours, substance abuse or psychiatric disorders. Conclusions This report identifies a good ability of ED doctors in the activation of an emergency procedure to protect the child. Overall, the social outcome was good for nearly 70% of the patients, highlighting the importance of activation of social support programs for child abuse.

A 14-year-old girl was admitted to our institute with a history of intermittent bilateral ankle swelling, and moderate but progressively worsening pain which has lasted for 2 years. The patient’s history was unremarkable. She did not take medications and was not involved in any sports activity. She reported no fever, gastrointestinal symptoms, fatigue, weight loss, travels abroad or previous infections. She reported moderate pain at night, associated with a sense of heaviness, tightness and general discomfort, and with no response to ibuprofen. Physical examination was remarkable only for bilateral ankle non-pitting oedema, more evident on the left leg, with a thickened skinfold at the base of the second toe, and without redness, swelling or skin warming. The patient had been previously examined, and her foot and ankle X-rays, ultrasound (US) and MRI were all negative. Blood tests (white cell count, C reactive protein, erythrocyte sedimentation rate, albumin, antinuclear antibodies, creatinine, transaminase, creatine kinase, lactate dehydrogenase, thyroid function and glucose) and urinalysis were in the normal range. Her ocular assessment and echocardiogram were also normal. Question 1 Based on the clinical picture and laboratory tests, what is the most likely diagnosis? Deep venous thrombosis. Osteochondritis. Lymphoedema. Juvenile idiopathic arthritis. Question 2 Based on what you see in figure 1, what is the underlying cause? Recurrent bacterial lymphangitis. Primary lymphoedema. Tumour. Filariasis. Figure 1 Lymphoscintigraphy of the lower extremities showing insufficient deep lymphatic circulation in the left leg (red arrow, A) replaced by superficial drainage (B). Question 3 Which is the best diagnostic test to confirm the diagnosis? US scan. MRI. Lymphoscintigraphy. Reassurance and clinical follow-up. Question 4 What is the mainstay of management of this condition? Wait and see. Antibiotic course. Supportive therapy (ie, physical activity, elevation of extremities, pneumatic compression). Surgical intervention. Answers can be found on page 2.

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ObjectiveThis study aims to explore the attitude of adolescents with chronic diseases toward social media exposure, focusing in particular on Facebook.DesignCross-sectional study.SettingAn anonymous semistructured survey was distributed to an Italian hospital-based cohort of adolescents with chronic disease to explore the role of Facebook in their daily life.PatientsWe recruited 212 adolescents (aged between 13 and 24 years) with a diagnosis of inflammatory bowel disease, coeliac disease, diabetes mellitus type 1 and cystic fibrosis.ResultsTwo hundred and seven of the 212 (97.6%) expressed the need of sharing their illness experience with friends, 201 out of 212 (94.8%) usually searched information on the internet to find new therapies and to discover their prognosis. One hundred and forty-nine out of 212 adolescents (70.3%) perceived dependence on their parents as the most negative aspect of having a chronic disease, and 200 out of 212 (94.3%) were looking for friends with the same disease on Facebook. Two hundred and ten out of 212 (99.1%) did not want their doctors or nurse on their social media platforms. During the active disease periods, the time spent with social media increased from an average of 5 to 11 hours.ConclusionsThis descriptive analysis focused on the Facebook impact on chronic disease perception among affected adolescents. It showed that they used to spend an increased amount of time on this platform during disease flare-up and highlighted their wish of keeping doctors and nurses away from their social dimension.

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Background Food Protein-Induced Enterocolitis Syndrome (FPIES) is a non IgE-mediated food allergy that generally affects children in the first year of life. Usually symptoms break out when formula milk or solid foods are introduced for the first time but they might also appear in exclusively breastfed infants, since the trigger elements, especially cow’s milk proteins, can be conveyed by maternal milk as well. FPIES in exclusively breastfed babies is a very rare clinical condition and only few cases have been reported in the medical literature. Case presentation We describe two cases of FPIES in exclusively breastfed babies. The first one is a two-month-old infant with a brief history of vomit and diarrhea that presented to the Emergency Department in septic-like conditions. The main laboratory finding was a significant increase in methemoglobin (13%). Clinically, we noted that, when breastfeeding was suspended, diarrhea drastically improved, and vice versa when maternal milk was reintroduced. An amino acid-based formula allowed a complete normalization of the symptoms. The second one is a three-month-old infant admitted for a 3 days history of persistent vomit and diarrhea. Blood tests showed a raised level of methemoglobin (7%). An esophagogastroduodenoscopy was performed and biopsies showed an eosinophilic infiltration of the duodenal mucosa. A maternal exclusion diet and an amino acid-based formula allowed a rapid regularization of the bowel function. Conclusions We searched all the cases of FPIES in exclusively breastfed babies reported in the medical literature, identifying eight patients, with an average age of 3 months (range 15 days – 6 months). The majority of the cases were initially diagnosed as gastroenteritis or sepsis, five cases were characterized by an acute on chronic scenario and cow’s milk was the most frequently involved food. Methemoglobin was never tested. An oral food challenge test was performed in two patients. FPIES in exclusively breastfed infants is a rare condition that, in the presence of compatible history and symptoms, should be considered also in exclusively breastfed babies. The evaluation of methemoglobin can simplify the diagnostic process.