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Journal: European journal of pediatrics

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It has been reported that asymptomatic people can transmit the new coronavirus disease 2019 (COVID-19) and become important sources of COVID-19. To reduce the role of asymptomatic or poorly symptomatic people in COVID-19, universal use of face masks in addition to hand hygiene and safety distance seems extremely useful. Consequently, preparing the healthy child to use face masks is strongly needed. To obtain maximal compliance, reasons for mask wearing without attempts of removing must be clearly explained. Moreover, child’s will must not be forced.Conclusion: On the basis of clinical findings, we think that the universal use of facial masks seems necessary when people have to go out in their everyday lives. In addition to the availability of masks of different sizes capable of adapting perfectly to the face, it is necessary that the use of masks in children is preceded by a strong parental work and school lessons on this issue and other hygiene topics with the main aim to obtain child cooperation.What is Known:• Asymptomatic people can transmit and become important sources of COVID-19.• Asymptomatic cases are common also in pediatrics.What is New:• Universal use of face masks for success against COVID-19 seems necessary also in pediatric age when people have to go out in their everyday lives.• In addition to the availability of masks of different sizes capable of adapting perfectly to the face, it is necessary that the use of masks in children is preceded by a strong parental work and school lessons with the main aim to obtain child cooperation.

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An association between a novel pediatric hyperinflammatory condition and SARS-CoV-2 was recently published and termed pediatric inflammatory multisystem syndrome, temporally associated with SARS-CoV-2 (PIMS-TS) or multisystem inflammatory syndrome (in children) (MIS(-C)). We performed a systematic review and describe the epidemiological, clinical, and prognostic characteristics of 953 PIMS-TS/MIS(-C) cases in 68 records. Additionally, we studied the sensitivity of different case definitions that are currently applied. PIMS-TS/MIS(-C) presents at a median age of 8 years. Epidemiological enrichment for males (58.9%) and ethnic minorities (37.0% Black) is present. Apart from obesity (25.3%), comorbidities are rare. PIMS-TS/MIS(-C) is characterized by fever (99.4%), gastrointestinal (85.6%) and cardiocirculatory manifestations (79.3%), and increased inflammatory biomarkers. Nevertheless, 50.3% present respiratory symptoms as well. Over half of patients (56.3%) present with shock. The majority of the patients (73.3%) need intensive care treatment, including extracorporal membrane oxygenation (ECMO) in 3.8%. Despite severe disease, mortality is rather low (1.9%). Of the currently used case definitions, the WHO definition is preferred, as it is more precise, while encompassing most cases.Conclusion: PIMS-TS/MIS(-C) is a severe, heterogeneous disease with epidemiological enrichment for males, adolescents, and racial and ethnic minorities. However, mortality rate is low and short-term outcome favorable. Long-term follow-up of chronic complications and additional clinical research to elucidate the underlying pathogenesis is crucial. What is Known: • A novel pediatric inflammatory syndrome with multisystem involvement has been described in association with SARS-CoV-2. • To date, the scattered reporting of cases and use of different case definitions provides insufficient insight in the full clinical spectrum, epidemiological and immunological features, and prognosis. What is New: • This systematic review illustrates the heterogeneous spectrum of PIMS-TS/MIS(-C) and its epidemiological enrichment for males, adolescents, and racial and ethnic minorities. • Despite its severe presentation, overall short-term outcome is good. • The WHO MIS definition is preferred, as it is more precise, while encompassing most cases.

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Primary ciliary dyskinesia (PCD) is a rare autosomal recessive disease, caused by specific primary structural and/or functional abnormalities of the motile cilia, in contrast with the transitory abnormalities seen in secondary ciliary dyskinesia. Disease-causing mutations in at least 16 genes have already been identified. The true incidence of PCD may be higher than currently reported, because the diagnosis is challenging and often missed. For the confirmation of PCD, both ciliary motility as well as ciliary ultrastructure must be evaluated. An early and adequate diagnosis and therapy can theoretically prevent bronchiectasis. Measurement of nasal nitric oxide has some value as a screening test but cannot be performed in young children. In the respiratory tract epithelium, impaired mucociliary clearance leads to chronic and/or recurrent upper and lower respiratory tract infections. In up to 75 % of the patients, respiratory manifestations start in the newborn period, although the diagnosis is often missed at that time. During embryogenesis, nodal cilia, which are motile cilia, determine the correct lateralization of the organs. Dysfunction of these cilia leads to random lateralization and thus situs inversus in approximately 50 % of the patients with PCD. The tail of a spermatozoon has a structure similar to that of a motile cilium. Consequently, male infertility due to immotile spermatozoa is often part of the characteristics of PCD. Given the heterogeneity and the rarity of the disorder, therapy is not evidence-based. Many treatment schedules are proposed in analogy with the treatment for cystic fibrosis. CONCLUSION: Respiratory infections, situs inversus and male infertility are typical manifestations of PCD, a rare autosomal recessive disorder.

Concepts: Sperm, Cystic fibrosis, Respiratory system, Upper respiratory tract, Cilium, Situs inversus, Respiratory tract, Primary ciliary dyskinesia

28

Venipuncture and intravenous cannulation are the most common painful procedures performed on children. The most widely used topical anesthetic is eutectic mixture of local anesthetics (EMLA). EMLA use is associated with a transient cutaneous vasoconstriction which can make it difficult to identify veins. We assessed with a prospective, multicenter, observational study whether EMLA interferes with venipuncture and intravenous cannulation. The primary study outcome was a success at first attempt in the course of venipuncture or venous cannulation. The study enrolled 388 children; 255 of them received EMLA and 133 did not. Eighty-six percent of procedures were successful at the first attempt in the EMLA group and 76.7 % in the no EMLA group. Conclusion: In this study, EMLA use did not interfere with the success of venipuncture or venous cannulation in children.

Concepts: Anesthesia, Topical anesthetic, Local anesthetic, Lidocaine, Local anesthetics, Prilocaine, Lidocaine/prilocaine

28

Fusobacterium necrophorum causes various clinical syndromes, ranging from otitis media to life-threatening Lemierre’s syndrome. The purpose of this study was to review our experience with pediatric Fusobacterium infections. The medical records of all children aged 0 to 18 years who were diagnosed between 1999 and 2011 with Fusobacterium infection were reviewed. Fusobacterium was isolated from clinical samples of 27 children: blood cultures (n = 16), abscesses (n = 8), joint fluids (n = 2), and cerebrospinal fluid (n = 1). The median age at admission was 3.5 years (range, 7 months to 17 years). Eight children (30 %) had seizures at presentation. Ten children (37 %) underwent lumbar puncture. Fifteen children (56 %) underwent brain imaging, and in seven of these children, a thrombus was identified either in a sinus vein or in an internal jugular vein. The most common source of infection was otogenic in 19 (70 %) of the children. Six of the children presented in 2011. All patients recovered. Conclusions: Neurologic manifestations are common at presentation of children with Fusobacterium infections. In young children, the most common source of infection is otogenic. Thrombotic complications are common, and imaging should be considered in all children with Fusobacterium infections arising from the head or neck region. There was a recent increase in the isolation of this bacterium, either because of better culturing techniques and increased awareness to this entity or a true increase in infections due to this organism.

Concepts: Brain, Bacteria, Internal jugular vein, Jugular vein, Otitis media, Lumbar puncture, Fusobacteria, Fusobacterium necrophorum

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Traumatic eye injuries are associated with significant visual impairment and are a major cause of monocular blindness. Rapid assessment and examination following trauma to the eye is crucial. A thorough knowledge of potential injuries is imperative to ensure rapid diagnosis, to prevent further damage to the eye, and to preserve visual capacity. One consequence of ocular trauma is cataract formation. We report a case and image of a 5-year-old boy who presented after sustaining a blunt head trauma. CT scan of the head revealed a markedly hypodense left eye lens.

Concepts: Eye, Visual perception, English-language films, Ophthalmology, Lens, Blindness, Cataract, Eye injury

28

Acne vulgaris is a chronic inflammatory disease with a complex pathogenesis that affects predominantly adolescents. The aim of the study was to investigate the interrelations between the presence of acne and several variables associated with somatic growth, pubertal maturation, and environmental conditions (altitude and regions of residence). A population sample of 6,200 clinically healthy boys (0-19 years) was examined and the presence of acne was determined. Height, weight, testicular volumes, penile length and circumference, as well as pubic hair were also measured. The prevalence of moderate and severe acne in the whole group was 7.74 %, while in the age group 12-19 years, it was 19.31 %. Twelve-15-year-old boys with acne were taller and heavier than the ones without. They also had increased penile length and circumference as well as larger testicular volumes. Somatometric and pubertal characteristics of 17-19-year-old boys with and without acne were similar. The prevalence of the disease did not differ between the rural and urban inhabitants. However, the acne frequency decreased with the increasing of the altitude where the boys lived. Conclusions: Our results showed that the development of acne vulgaris in male adolescents was associated with an intensive growth and pubertal maturation, while obesity per se did not play an important role. Of particular interest was the association between the prevalence of acne and the altitude of residence.

Concepts: Inflammation, Puberty, Penis, Acne vulgaris, Height, Length, Sebaceous gland, Pubic hair

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The main objectives of this qualitative study were to describe the perceptions and needs of pediatric health care professionals (HCPs) taking care of children with palliative care needs and to develop a concept for the first Center of Competence for Pediatric Palliative Care (PPC) in Switzerland. Within two parts of the study, 76 HCPs were interviewed. The main interview topics were: (1) definition of and attitude toward PPC; (2) current provision of PPC; (3) the support needs of HCPs in the provision of PPC; and (4) the role of specialized PPC teams. HCPs expressed openness to PPC and reported distinctive needs for support in the care of these patients. The main tasks of specialized PPC teams in Switzerland would encompass the coaching of attending teams, coordination of care, symptom control, and direct support of affected families during and beyond the illness of their child. Conclusion: This study indicates the need for specialized PPC in Switzerland both inside and outside of centers providing top quality medical care (Spitzenmedizin). Specialized PPC teams could have a significant impact on the care of children and families with PPC needs. Whether hospices are an option in Switzerland remains unanswered; however, a place to meet other families with similar destinies was emphasized.

Concepts: Health care, Health care provider, Medicine, Healthcare, Health, Illness, Hospice, Curative care

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Acute bronchiolitis has been associated with an increasing hospitalization rate over the past decades. The aim of this paper was to estimate the impact of home oxygen therapy (HOT) on hospital stay for infants with acute bronchiolitis. A retrospective cohort study was done including all children aged ≤12 months discharged from a pediatric tertiary-care center with a diagnosis of bronchiolitis, between November 2007 and March 2008. Oxygen was administered according to a standardized protocol. We assumed children with the following criteria could have been sent home with O(2), instead of being kept in hospital: age ≥2 months, distance between home and hospital <50 km, in-hospital observation ≥24 h, O(2) requirement ≤1.0 L/min, stable clinical condition, no enteral tube feeding, and intravenous fluids <50 mL/kg/day. Children with significant underlying disease were excluded. A total of 177 children were included. Median age was 2.0 months (range 0-11), and median length of stay was 3.0 days (range 0-18). Forty-eight percent of patients (85/177) received oxygen during their hospital stay. Criteria for discharge with HOT were met in 7.1 % of patients, a mean of 1.8 days (SD 1.8) prior to real discharge. The number of patient-days of hospitalization which would have been saved had HOT been available was 21, representing 3.0 % of total patient-days of hospitalization for bronchiolitis over the study period (21/701). Conclusions: In this study setting, few children were eligible for an early discharge with HOT. Home oxygen therapy would not significantly decrease the overall burden of hospitalization for bronchiolitis.

Concepts: Oxygen, Cohort study, Patient, Hospital, Physician, Feeding tube, Pediatrics, Oxygen concentrator

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Genetic mosaicism is defined as the existence of at least two genetically distinct cell populations within one individual. Mosaic presentation of genetic disorders is common and is often particularly obvious in the skin, because there it will generate recognizable patterns. Recognizing those can frequently assist in establishing a diagnosis. In this review, we discuss the mechanisms that give rise to genetic mosaicism. We describe its most frequent cutaneous manifestations that are relevant to paediatric practice. While most mosaic genetic diseases are rare, it is important to recognize them so that patients and parents may receive appropriate genetic counselling. Moreover, recent developments are now resulting in novel, targeted treatments for such disorders that promise to considerably improve patients' lives.

Concepts: Genetics, Disease, Genetic disorder, Mutation, Skin, Klinefelter's syndrome, Medical genetics, Chimera