Pain is a distinctive burden in atopic dermatitis and recognized as an important and highly prevalent symptom. It is unknown if the presence of atopic disease may sensitize children to adverse pain profiles in the long term. We aimed to assess the impact of early-life atopic dermatitis-like symptoms on pain at 10 years of age. We used data from 1302 and 874 participants of the Generation XXI birth cohort evaluated at 6 and 15 months, respectively, and 10 years. Atopy-like symptoms since birth, including atopic dermatitis, were collected at ages 6 and 15 months by interviewing parents. Pain history in the last 3 months at age 10 was collected from parents and children using structured questionnaires. We computed relative risks (RR) and respective 95% confidence intervals of pain features at age 10 according to each atopic-like symptom at 6 and 15 months. Children whose parents reported atopic dermatitis-like symptoms at 6 months and at 15 months had higher risk of reporting any pain (RR 1.75 [1.15–2.66]) and multisite pain, respectively (RR 1.67 [1.18–2.37]) at 10 years of age. Conclusion : Atopic dermatitis symptoms in early life were associated with a higher risk of pain at age 10, suggesting that potential for sensitization during the first decade of life and highlighting the importance of improving the health care of children with atopic dermatitis is worth investigating. What is Known: • Atopic disorders have been associated with many non-atopic comorbidities, including chronic pain. • Pain and atopic dermatitis share common inflammatory pathways. Inflammation, injury to the skin from scratching, fissures, and intolerance to irritants related to atopic dermatitis can cause pain. What is New: • Atopic dermatitis in early life is linked to an increased likelihood of experiencing pain at the age of 10, which suggests that exploring the potential for sensitization is a worthwhile area of investigation. • Our proof-of-concept study highlights the potential benefit of studying management targets and improving itching and relieving skin pain as quickly as possible, avoiding potential long-term consequences of the sensitization process.

The prevalence of hypertension in the pediatric age range is estimated at 1–5% worldwide, with higher rates in adolescence. Although primary hypertension is more common, due to the increasing prevalence of obesity and metabolic syndrome among adolescents, secondary hypertension should be always considered and excluded. We present the case of an adolescent with secondary hypertension and a challenging diagnosis associated with coarctation of aorta and Turner Mosaicism.

Herpetic esophagitis (HE) is an uncommon condition in immunocompetent, and even less common in young children. We present the case of a 6-year-old-child with HE and food allergy. Recently the association between atopic conditions, eosinophilic, and herpetic esophagitis has been suggested. Clinical suspicion is the key to diagnosis, especially with persistent symptoms.

Extubation failure (EF) is characterized by the need for reintubation after removal of the endotracheal tube. The prevalence in pediatric age is estimated at 2-20%. Although the causes are almost always multifactorial, EF has been associated with several risk factors and is generally associated with a worse prognosis and higher morbidity. We aimed to characterize the patients with EF admitted to the Pediatric Intensive Care Unit (PICU) of a Tertiary Hospital. It was performed a retrospective descriptive study of EF cases, considering all children admitted to the PICU between January 2017 and June 2020. Our study showed that patients with EF are mostly male and under the age of 2 years, with surgical admission, congenital heart disease, a higher LOS in PICU (12.5 days vs 5.6 days) and mortality (9.0% vs 2.67%). The presence of stridor was more prevalent in EF patients (45.4% vs 8.9%), probably increasing the risk of EF. Peri-extubation corticosteroids used in 72.7% of the EF patients seem to justify in part the existence of stridor after extubation (10.3% of all extubated patients vs 45.4% in EF patients). The higher use of corticosteroids in EF patients (72.7% vs 57.5%) is in line with higher stridor incidence among EF patients. Patients with EF have a higher length of stay, more comorbidities, more sequelae and generally worse prognosis. The adequate characterization of these patients makes it possible to plan and develop multidisciplinary strategies to prevent and minimize the risk factors associated with EF.

Aim To estimate agreement in the point prevalence of any pain, high-intensity pain and pain in two or more sites according to parental and child report. Methods We conducted a prospective study of 5639 children from a Portuguese birth cohort - Generation XXI, where parents and 7-year-old children answered the same questions at the same time. We assessed the accuracy of parental report, considering children's self-report as the gold standard. Results At 7 years of age, 499 children (8.8% (95% confidence interval (CI) 8.1-9.6)) reported having pain at the time of the interview. Of those, 44.1% had high-intensity pain (3.9% (95% CI 3.4-4.4) of the whole sample) and 12.4% reported pain in two or more sites (1.1% (95% CI 0.8-1.4) of the whole sample). In this community setting, pain prevalence and intensity were lower when collected from parents. Parental report had sensitivity below 20% and specificity above 95% but its positive predictive value was at most 25%. Conclusion Our findings support that, outside acute care, parents have a specific but not sensitive report of children's pain at the age of 7 years. Their report seemed useful to exclude major complaints but limited to screen children's pain. This limitation was higher for more severe pain, that is two or more sites or high-intensity pain. Children should be asked directly about pain to avoid under-estimating paediatric pain.

Introduction: Orofacial clefts (OFCs) are a common congenital craniofacial malformation that derive from a complexity of genetic and environmental factors. Although OFCs are usually an isolated malformations, over 400 syndromes have been reported in association. Purpose: This study aims to characterize patients with syndromic OFCs that attend Cleft Lip/Palate multidisciplinary group clinic at Centro Hospitalar Universitario de Sao Joao in Porto-Portugal. Methods: A retrospective analysis of medical records from a cohort of patients that were observed between January 1992 and December 2018 was performed. The OFCs types were listed according to the Spina classification modified by Silva-Filho et al. OFCs laterality, family history, associated syndromes and identified gene anomalies were collected. Results: The group included 617 patients, of which 57.3% were males. From the total, 18.6% had cleft lip, 38.1% had cleft lip and palate and 42% isolated cleft palate, which was the most frequent. The least frequent was the group of atypical clefts (1.3%). One hundred and fifty-seven patients (25.4%) had a family history of clefting. Syndromes were identified in 149 of the patients: Pierre Robin sequence was the most common (n=48; 32.2%), followed by 22q11.2 deletion syndrome, Van der Woude, Goldenhar and oral-facial-digital syndrome. Pierre Robin sequence occurred with additional syndromes in 18 cases (12.1%). Discussion: This cohort has a 24.1% of an identifiable syndrome, number higher than described in the literature. The importance of monitoring the OFCs and associated syndromes is a key element not only to tailor the approach and management of the OFCs, the possible complications and to provide accurate and insightful genetic counseling.

INTRODUCTION:Wolfram syndrome (WFS) is a neurological and endocrinological degenerative disorder, also known as DIDMOAD (Diabetes Insipidus, early-onset Diabetes Mellitus, progressive Optic Atrophy, and Deafness) syndrome. It is an autosomal recessive disorder, mostly involving the Wolfram syndrome 1 gene (WFS1). The phenotypic pleiomorphism, rarity, and molecular complexity complicate the follow-up of these patients.MATERIAL AND METHODS:We aimed to describe the clinical characteristics and the follow-up of 11 patients with this disorder. We retrospectively analysed all WFS patients diagnosed between 1990 and 2020 in the Centro Hospitalar São João, a tertiary hospital in Northern Portugal.RESULTS:Eleven patients were included. Four patients had all 4 components of DIDMOAD. The presentation was diabetes mellitus (DM) in 9 patients, optic atrophy (OA) in another patient, and diabetes insipidus (DI) in another one. The median age of DM and OA diagnosis was 6 and 14 years, respectively. Nine patients had diabetes mellitus, and the other 2 patients had impaired glucose tolerance. All patients had OA. Four patients presented DI, all of them diagnosed in adolescence. Four patients had hearing impairment, 5 had urological abnormalities, 5 had neurological disorders, and 8 had psychiatry disorders. Eight patients had a broad spectrum of recessive mutations in WFS1.CONCLUSION:The information obtained in this study can facilitate further research in an attempt to improve prevention strategies for this devastating disease.

Introduction: Pediatric pulmonary embolism (PE) is rare but associated with adverse outcomes. We aimed to characterize PE cases admitted in a tertiary hospital and to evaluate sensitivity of PE diagnostic prediction tools. Methods: Retrospective, descriptive study of PE cases admitted from 2008 to 2020. Data was collected from hospital records. Patients were grouped according to PE severity and setting (outpatients, inpatients). Associations with demographic characteristics, risk factors, clinical presentation, management and outcomes were analyzed. PE diagnostic prediction tools were applied. Results: 29 PE episodes occurred in 27 patients, 62.9% female, mean age 14.1 years. Most PE were central and massive or submassive. One was diagnosed in autopsy. Outpatients (n=20), admitted for classic PE symptoms, were adolescents; in half the diagnosis had been missed previously. Risk factors included contraceptives (65%), thrombophilia (35%), obesity (20%) and auto-immunity (20%). Inpatients´ PE (n=8), diagnosed during cardiorespiratory deterioration (n=5) or through incidental radiological findings (n=3), were younger and had immobilization (87.5%), complex chronic diseases (75%), infections (75%) and central venous catheter (62.5%) as risk factors. Retrospectively, D-dimer testing and adults’ scores performed better than pediatrics’ scores (sensitivity 92.9-96% vs 85.7- 92.9%). Both pediatrics’ scores missed a case with a positive family history. Discussion: Pediatric PE diagnosis is often delayed or missed. To improve it, the development of pediatric prediction tools as from validated adult scores merits to be explored. We propose that clinical presentation and risk factors may be different in inpatients and outpatients. Family history should be included.

P357 Figure 1 Conclusions Treatment with LAB is cheaper in comparison with Meglumine antimoniate. Owing to lesser hospitalization days the former decreases the risk to get nosocomial infections which in its turn prevents possible cost increase. P358 HOW CAN WE PROMOTE BETTER CARE FOR CHILDREN WITH CANCER ? DISCUSSION OF A CHILDAND FAMILY-CENTRED CARE APPROACH FOR EACH STAGE OF THE CHILD AND FAMILIES TREATMENT JOURNEY Imelda Coyne*. Trinity College Dublin , Dublin, Ireland 10.1136/archdischild-2019-epa.705 Family-centred care is viewed as a way of caring for children and their families within health services which ensures that care is planned around the whole family, not just the individual child/person. It is a popular and widely used model in children’s healthcare around the world. However research indicates that the practice of family-centred care is inconsistent and problematic with no solid evidence that FCC works or makes a difference to health outcomes. This lack of evidence coupled with continual reporting of problems with FCC has led to calls for a re-examination of the suitability of the model and suggestion that child-centred care may be a more appropriate model for children’s nursing. Family-centred care is seen as offering ways to facilitate parents’ involvement and active participation in their child’s care. However, current descriptions of the model does not offer clear guidance on how to involve and support children’s participation in their own care. Therefore a new childand family-centred care framework is required for parents and children with cancer. In this paper I will outline the core principles of familycentred care and consider the mixed evidence about the benefits of this model for children, parents, nurses and the health system. Parents and families have an essential role in caring for their children throughout the cancer treatment journey. But FCC needs to incorporate the rights of the child to participate in all aspects of health care delivery in conjunction with the need of their family. We need to ensure a child-centred perspective, which entails seeing each child as an active agent, the child’s needs are the starting point for care planning and provision, and where each child’s preferences, values, family situation, social circumstances are considered. Taking a child-centred approach requires all health professionals to include the child’s perspective in their actions and care delivery. It is important that the practice of child and familycentred care is flexible as needs will alter with each stage of inpatient cancer care and transition to home setting. The paper will conclude with guidance on how professionals can use a more child and family-centred care approach in their daily practice with children and families. P360 PROLONGED ADMISSIONS IN PAEDIATRICS – THE REALITY OF A PORTUGUESE CENTRAL HOSPITAL Marta Pinheiro*, Vanessa Gorito, Tiago Magalhães, Margarida Ferreira, Ana Maia,

We present a male newborn referred to pediatric cardiology due to a heart murmur, whose echocardiographic evaluation showed an intrahepatic inferior vena cava interruption with azygos continuation and two small mesoseptal ventricular septal defects. Although mainly incidental, the early diagnosis of interrupted inferior vena cava can be crucial to avoid complications of invasive vascular procedures.

Background. Type 1 diabetes (T1D) is the most common chronic metabolic disease among children and the autoimmune process can induce the development of additional autoimmune diseases. We performed a retrospective study of the clinical records and analytical determinations of pediatric patients with T1 D during a five-year-period. Methods. We identify 102 patients, similar gender distribution. In 98% was performed, at least, one tracking of other autoimmune disease. We found positive autoantibodies in 31%: thyroid antibodies (n = 16), antibodies for celiac disease (n = 10), and antinuclear antibodies (n = 5). Antibodies positivity occurred in mean 3.6 years after T1 D diagnosis. Other conditions associated were pulmonary hemosiderosis, vitiligo, IgA deficit, uveitis, and positive Anti-Saccharomyces cerevisiae antibodies. Conclusions. Despite the antibodies' positivity we highlight the possibility of other less common autoimmune-related conditions. The screening of comorbidities is fundamental to perform an earlier diagnosis and prevent complications.

BACKGROUND:We evaluated different pain profiles as prospective predictors of multisite pain in 13-year-old adolescents (1300 girls and 1457 boys) enrolled in Generation XXI, a birth cohort study in Portugal.METHODS:Pain history was queried using the Luebeck Pain Questionnaire through parent proxy- (ages 7 and 10) and adolescent (age 13) self-reports. We estimated the risk of multisite pain (2 or more pain sites) at age 13, according to previous pain experiences, including accumulation and timing. We defined five profiles that combined adverse features at ages 7 and 10 (recurrence, multisite, frequency, duration, intensity, triggers, activity restrictions, passive coping, and family history) and estimated their relative risks (RR) and likelihood ratios (LR) for adolescent multisite pain.RESULTS:At age 13, 39.2% of girls and 27.2% of boys reported multisite pain in the previous three months. The risk was higher among girls with multisite and recurrent pain at ages 7 and 10 than in girls without those adverse features, especially if psychosocial triggers were also present (RR 1.87; 95% confidence interval 1.36, 2.36 and LR 3.49; 1.53, 7.96). Boys with recurrent pain of higher frequency and causing activity restrictions at ages 7 and 10 had a higher risk of multisite pain at 13 (RR 2.05; 1.03, 3.05 and LR 3.06; 1.12, 8.39). Earlier adverse experiences were more predictive of future pain in girls than in boys.CONCLUSIONS:Different profiles were useful to rule in future multisite pain in boys and girls. This provides clues for early stratification of chronic pain risk.SIGNIFICANCE:We identified sex-specific pain features that can be collected by practitioners in the first decade of life to improve the stratification of children in terms of their future risk of a maladaptive pain experience in adolescence. Using a prospective population-based cohort design, we show that early multisite pain and psychosocial triggers are relevant predictors of future multisite pain in girls, whereas repeated reports of high-frequency pain leading to activity restrictions are predictive of adolescent multisite pain in boys.

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Lupus erythematosus (LE) is a group of autoimmune disorders with a clinical spectrum ranging from localized discoid lesions (DLE) to life-threatening systemic manifestations (1). Lupus erythematosus panniculitis is a rare variant that may occur as a separate disease or coexist with DLE or SLE (2). The incidence is estimated between 1-3% of LE cases, more prevalent (2:1) among females (5), especially at a young age. It is characterized by persistent, tender, and hard nodules localized on the face, arms, shoulders, breasts, and buttocks. Healing of lesions is associated with scarring, lipoatrophy, and rarely ulceration (7). The diagnosis is based on the clinical-pathological correlation. Histopathological features are lymphocytic panniculitis, hyaline degeneration and calcification, allowing the diagnosis (6). There is non consensus on the therapeutic approach. Local treatment with corticosteroids (topical or injectable) is used (4). Antimalarial drugs seem to be beneficial in mild cases of isolated LP (8).

INTRODUÇÃO: As fendas lábio-palatinas são um grupo heterogêneo de defeitos congênitos que ocorrem em cerca de 1,7 / 1000 recém-nascidos. Eles podem ocorrer com outras anomalias congênitas, incluindo defeitos cardíacos. O nosso objetivo é descrever uma população com fendas lábio-palatinas e anomalias cardíacas associadas. MÉTODOS: Estudo retrospectivo de doentes seguidos pelo Grupo Multidisciplinar de Fendas Lábio-Palatinas no Hospital Universitário São João, Porto-Portugal. Foram analisados os prontuários médicos de janeiro de 1992 a dezembro de 2018. Os doentes foram divididos em quatro grupos, de acordo com a classificação de Spina: fenda labial (CL), fenda labial e palatina (CLP), fenda palatina isolada (PC) e fenda atípica (CA). Outras categorizações incluíram sexo, parentes afetados, anomalias e síndromes congênitas associadas RESULTADOS: Dos 588 pacientes incluídos, 77 (13%) apresentaram anomalias cardíacas. Daqueles com fenda e anomalias cardíacas, 53% eram do sexo masculino e 17% tinham parentes afetados. A PC foi a fenda mais comum entre os doentes com anomalia cardíaca (aproximadamente 56%). Anomalias congénitas adicionais, como defeitos faciais, malformações do sistema nervoso central, renais e esqueléticas foram encontradas em 89,7%. Síndromes foram identificadas em 61,5%, sendo Pierre-Robin a mais comum (n = 22), seguida pela microdeleção 22q11. 2 (n = 9). Anomalias congénitas adicionais e a presença de uma síndrome genética foram significativamente mais prevalentes em doentes com doença cardíaca associada (p chr(38)lt;0,05). Os principais grupos de anomalias cardíacas foram shunt da esquerda para a direita (n = 47) e obstrução da via de saída do ventrículo direito (n = 14). Destes, 26 apresentaram comunicação interventricular, 15 comunicação interauricular e sete pacientes apresentaram tetralogia de Fallot. Cinco pacientes apresentaram disritmias. CONCLUSÕES: Devido à elevada prevalência de anomalias cardíacas na população de doentes com Fenda Lábio-Palatina, aconselhamos uma avaliação cardíaca de rotina em todos

AIMS: Orofacial clefts (OFC) are a heterogeneous group of birth defects arising in about 1.7/1000 newborns. They can occur with other congenital anomalies, including heart defects. We aim to describe a population with orofacial clefts and associated cardiac anomalies.METHODS: Retrospective study of patients attended in the Cleft Lip and Palate Multidisciplinary Group outpatient clinic at Hospital Universitario São João, Porto-Portugal. Medical records from January 1992 through December 2018 were reviewed. Patients were divided into four groups according to the Spina classification: cleft lip (CL), cleft lip and palate (CLP), isolated cleft palate (CP) and atypical cleft (AC). Further categorization included gender, affected relatives, associated congenital anomalies and syndromes.RESULTS: From the 588 patients included, 77 (13%) presented cardiac anomalies. Of those with orofacial cleft and cardiac anomalies, 53% were males and 17% had known affected relatives. CP was the most common cleft among patients with cardiac anomaly (~56%). Additional congenital anomalies were found in 89.7% of patients, namely facial defects, central nervous system, renal and skeletal malformations. A recognizable syndrome was identified in 61.5%, being Pierre-Robin the most common (n=22), followed by 22q11.2 microdeletion (n=9). Both additional congenital anomalies and recognizable syndromes were significantly more prevalent in patients with heart disease (p<0.05). The main groups of cardiac anomalies were left-to-right shunt (n=47) and right ventricular outflow tract obstruction (n=14). From these, 26 had a ventricular septal defect, 15 atrial septal defect and seven patients had tetralogy of Fallot. Five patients had dysrhythmias.CONCLUSIONS: Due to the high prevalence of cardiac anomalies in the cleft population, a routine cardiac evaluation should be performed in all these patients.

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Abstract Background: Impaired sensitivity to thyroid hormone refers to any process that negatively affects its action, including defects in its transport, metabolism and action on the receptor. Resistance to thyroid hormone due to beta-receptor mutations (RTH-beta) is the most common form of this entity and is characterized by reduced response of peripheral tissues to the action of thyroid hormone. The genetic variability of cofactors involved in the action of thyroid hormone explains the heterogeneity of resistance among affected individuals. Generally, patients with this disorder, have increased levels of free T4 and free T3 in association with normal or high TSH. Clinical case: 11-year-old boy, with personal history of Attention-deficit/hyperactivity disorder (ADHD). A pediatric endocrinology consultation was requested to evaluate abnormalities in his thyroid function tests. A few months earlier, his father was referred to endocrinology consultation because of thyroid function tests abnormalities: TSH - 3.01 μIU / mL (N: 0.35 - 4.94); Free T4 1.7 ng / dL (N: 0.7-1.48); Free T3 4.77 pg / mL (N: 1.71-3.71). Initially, two diagnostic hypotheses were considered: central hyperthyroidism or impaired sensitivity to thyroid hormone. The adult underwent pituitary magnetic resonance, which raised the hypothesis of a pituitary microadenoma, and TRH stimulation test, whose result was strongly suggestive of the second diagnostic possibility. A genetic study was requested and the presence of the c700 G> A variant (p. Ala 324 trh) in the THRB gene was identified, which confirmed the most likely hypothesis. At the time of the pediatric endocrinology consultation, the 11-year-old boy had the results of his lab tests: TSH - 6.67 μIU / mL (N: 0.35 - 5); T4L 2.27 ng / dL (N: 0.88-1.58); T3L 7.79 pg / mL (N: 2-4.20). Given his perfect height and weight evolution and the absence of symptoms suggestive of hypo or hyperthyroidism, it was decided not to start any medication, keeping only periodic surveillance. Conclusion: This case exemplifies unusual thyroid function tests. This discordance between serum thyroid hormone and TSH concentrations should raise the possibility of impaired sensitivity to thyroid hormone. In this condition, patients may present with symptoms of hypo or hyperthyroidism and the etiology of thyroid function tests abnormalities are not easily recognized. This can lead to misdiagnosis and consequently unnecessary treatment.

Tuberculosis meningitis is the most severe extrapulmonary complication of tuberculosis. The authors present a case of an unvaccinated female infant with the initial suspicion of bacterial meningitis. However, due to the absence of therapeutical response, clinical worsening and given the imaging and laboratory findings, the diagnosis of pulmonary and meningeal tuberculosis was confirmed.