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Pemphigus foliaceus (PF) is an autoimmune bullous disease endemic in Brazil. Since serum IL-12 is increased in patients with PF and Langerhans cells (LC) produce IL-12, we titrated serum autoantibodies by indirect immunofluorescence, and quantified epidermal dendritic cells, known as LC, and dermal dendritic cells (DC). Biopsies of blistering lesions were obtained from 22 patients, 13 of whom were submitted to biopsy of both injured and of apparently healthy skin. The control groups consisted of skin from 8 cadavers and from 12 women submitted to breast plastic surgery. LC and DC were identified with anti-CD1a antibody and quantified by morphometric analysis. LC number in the lesion and in apparently healthy skin from PF patients was similar to that of both control groups. DC number in the injured skin (median = 0.94 DC/mm basement membrane) was higher than that of the cadaver group (median = 0.13 DC/mm basement membrane). In the 13 patients with biopsies of both injured and apparently healthy skin, LC and DC were present in larger numbers in the lesion. There was a direct correlation between DC number in the lesion of the PF group and serum autoantibody titers. This correlation was not observed for LC number. The increased number of DC in the lesion, as well as its direct correlation with serum autoantibody titers suggest the participation of DC in the pathogenesis of PF. The relationship between increased DC number and IL-12 in PF needs to be clarified.

Background: Variability in presentation of children with coronavirus disease 2019 (COVID-19) is a challenge in emergency departments (EDs) in terms of early recognition, which has an effect on disease control and prevention. We describe a cohort of 170 children with COVID-19 and differences with the published cohorts., Methods: Retrospective chart reviews on children (0-18 years) evaluated in 17 Italian pediatric EDs., Results: In our cohort (median age of 45 months; interquartile range of 4 months-10.7 years), we found a high number of patients <1 year with COVID-19 disease. The exposure happened mainly (59%) outside family clusters; 22% had comorbidities. Children were more frequently asymptomatic (17%) or with mild diseases (63%). Common symptoms were cough (43%) and difficulty feeding (35%). Chest computed tomography, chest radiograph, and point-of-care lung ultrasound were used in 2%, 36%, and 8% of cases, respectively. Forty-three percent of patients were admitted because of their clinical conditions. The minimal use of computed tomography and chest radiograph may have led to a reduced identification of moderate cases, which may have been clinically classified as mild cases., Conclusions: Italian children evaluated in the ED infrequently have notable disease symptoms. For pediatrics, COVID-19 may have rare but serious and life-threatening presentations but, in the majority of cases, represents an organizational burden for the ED. These data should not lower the attention to and preparedness for COVID-19 disease because children may represent a source of viral transmission. A clinically driven classification, instead of a radiologic, could be more valuable in predicting patient needs and better allocating resources., Competing Interests: POTENTIAL CONFLICT OF INTEREST: The authors have indicated they have no potential conflicts of interest to disclose., (Copyright © 2020 by the American Academy of Pediatrics.)

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.

The application of a design methodology for the determination of the optimal prosthesis architecture for a given upper limb amputee is presented in this paper along with the discussion of its results. In particular, a novel procedure was used to provide the main guidelines for the design of an actuated shoulder articulation for externally powered prostheses. The topology and the geometry of the new articulation were determined as the optimal compromise between wearability (for the ease of use and the patient's comfort) and functionality of the device (in terms of mobility, velocity, payload, etc.). This choice was based on kinematic and kinetostatic analyses of different upper limb prosthesis models and on purpose-built indices that were set up to evaluate the models from different viewpoints. Only 12 of the 31 simulated prostheses proved a sufficient level of functionality: among these, the optimal solution was an articulation having two actuated revolute joints with orthogonal axes for the elevation of the upper arm in any vertical plane and a frictional joint for the passive adjustment of the humeral intra-extra rotation. A prototype of the mechanism is at the clinical test stage.

Mycoplasma pneumoniae is a common intracellular pathogen, which is responsible for infections of the respiratory tract, particularly in patients between 5 and 30 years of age. Nevertheless, there is increasing evidence that Mycoplasma pneumoniae plays a role in determining clinical presentations different from the respiratory ones. Among extra pulmonary complications skin eruptions are more frequent than others, even with severe clinical features such as Stevens Johnson syndrome. It is important to note that dermatological involvement can occur before, during or after the appearance of respiratory symptoms or without them. We report two patients whose onset of symptoms was not a respiratory tract disease, as usual in Mycoplasma pneumoniae infections, but prolonged and high grade fever with a relevant skin involvement pointing out the importance of researching Mycoplasma pneumoniae in the pathogenesis of peculiar clinical features. The first patient is a 4-year-old boy with signs of Stevens Johnson syndrome while the second patient is a 16-year-old girl with red-purple maculae on both legs and arms; in both cases we detected Mycoplasma IgM antibodies as a part of differential diagnosis. We discuss below the immunological mechanism by which Mycoplasma pneumoniae can determine the clinical features shown by our patients.

A 7-year-old boy with toxic epidermal necrolysis (TEN) is reported. The patient was treated with intravenous fluids, antibiotics, steroids, and immunoglobulins. Moreover, total parenteral nutrition was required for 4 days. He gradually improved and was discharged in a good clinical condition. Appropriate treatment for TEN is still controversial. Larger study are required to address this issue.

Acute toxic exposures in childhood are quite a frequent event in Paediatric Emergency Medicine. Despite that, there are few papers about clinical toxicology in children, at least in Italy. This paper is the first that takes into account both epidemiological aspects and clinical features of acute poisoning in children on a national basis. Collected data show the increased use of activated charcoal as a treatment and the use of the short stay observation unit as an appropriate answer to the real clinical situation, although some aspects are still controversial. It will be necessary to reconsider the whole matter widening the number of participating centres, so that the most controversial aspects may be clarified.

Unlabelled: Endemic Pemphigus Foliaceus (EPF) is a bullous autoimmune skin disease whose incidence used to be high in the State of São Paulo (SP), Brazil, during the forties, but has declined thereafter., Objectives: to report a series of EPF patients from the northeastern region of SP., Methods: a retrospective study concerning demographic and epidemiological data of patients seen from 1973 to 1998 was conducted at the University Hospital, Faculty of Medicine of Ribeirão Preto, SP., Results: bullous disease was diagnosed in 340 patients, 245 with EPF (72.1%), 9.4 cases per year, 60.4% females, and 70.2% white, 7 to 82 year-old (29.4% in their teens); 46.9% lived in the rural zone. Concerning profession, housewives predominated among women (67.6%) and agricultural workers among men (40.2%). The time of disease was less than 1 year in 62.0% of cases, followed by 1 and 5 years (27%), and more than 5 years for the remaining patients (11%). 36.7% of patients were referred by the Direção Regional de Saúde (DIR) XVIII of Ribeirão Preto, with the largest number of cases being from Ribeirão Preto and Batatais: 33.3% and 23.3%, respectively; 22% from DIR XIII (Franca); 13.5% from DIR VII (Araraquara); 2.9% from DIR IX (Barretos); 4.1% from other DIRs of SP, and 20.8% from other States (16.7% from Minas Gerais). Thirteen (5.3%) patients reported occurrence of the disease in some relative, and 4 (1.6%) in neighbors., Conclusions: the present data characterize the northeastern region of the state of São Paulo as a remaining endemic focus of EPF.

Pemphigus foliaceus (PF) is characterized by acantholysis determined by IgG4 binding to desmoglein I, a 160-kDa desmosomal glycoprotein. To investigate the immunopathological aspects of Brazilian PF, we determined levels of serum cytokines in patients with PF. Twenty-five patients with PF and a control group consisting of 10 healthy individuals were studied. Serum IL-2, IL-4, IL-5, IL-10, IL-12 and IFN-gamma were measured in the two groups by ELISA. The median concentration of IL-2 was lower in PF patients compared to the control group (0.45 and 9.50 pg/ml, respectively), as also was the concentration of IL-4 (0.26 and 10.16 pg/ml, respectively). The same was observed for IL-5 (7.94 and 15.74 pg/ml, respectively) and for IFN-gamma (5.90 and 8.58 pg/ml, respectively). For IL-10 and IL-12, higher concentrations were observed in PF compared to the control group (IL-10: 24.76 and 20.92; IL-12: 2.92 and 1.17 pg/ml, respectively). Considering the Th1/Th2 paradigm, it seems that a Th2 profile, mainly represented by IL-10, predominates in PF.

The risk associated with poisonings in children was assessed on 6175 subjects admitted to the pediatric hospitals of 3 regions of Northern Italy between 1975 and 1990. The frequency of exposures and the severity of sequelae allowed high- and low-risk substances to be identified. Risks were studied separately in the 0-4, 5-9 and 10-13 y age group to determine age-related differences. A decrease in drug-related poisonings and an increase of those caused by household products, particularly in the first 2 y of life, were observed in 1983-90 as against 1975-82 in association with the introduction of safety caps and containers for drugs. Our study points out the need to strengthen active primary prevention through health education and parental and adolescent information, and to improve passive prevention through the extension of safety closures to household products.

A perspective study was carried out at the Emergency Department of the Gaslini Institute on a sample of the children admitted from May to September 1990. The study aimed to describe types and modalities of admission. "G. Gaslini" Institute is a multidisciplinary children's hospital: 45% of patients admitted in the hospital come from the southern regions of Italy, while the admission at the Emergency Department were local in 82%. Among all children considered, 2080 (71.9% were admitted on request of their parents, who did not ask previously any physician; of those cases, 356 (17%) were hospitalized, 474 (25%) were either examined by specialist or treated and then discharged. In the remaining cases, no intervention was necessary. As a consequence, 58% of children were admitted without any clinically plausible reason. Parents' anxiety clearly played an important role in most cases. 840 children were addressed to the Emergency Department by a physician, 652 (22.3%) by the doctor in charge, 213 (32.7%) of which were sent back home by the doctor on duty, who did not think it necessary any treatment. Of the 120 (4.1%) children sent by different Emergency Departments, 95 (79.1%) were hospitalized. The reason why physicians addressed quite a large number of children to the Emergency Department, when they didn't need any intervention remain nucleo. Their diagnosis, in fact, agreed with those of the doctor on duty in 83% of cases. In conclusion, it is difficult to imagine a diagnostic or therapeutic problem which needs specific structures. Cooperative programs with physicians working in our region will be carried out in order to control, where possible, admission to Emergency Departments.

The authors reviewed records of admission at the Fist Ais-Emergency Service of "G. Gaslini" Children's Hospital, data referring both to in and outpatients. First of all we took into account epidemiological data analysing occurrence and types of diseases; at the same time a demographic study, which aimed to show a decrease in the child population in Genova, was performed. Secondly we compared these data with the real number of admitted patients: collected data showed that this service has been used excessively.

The Authors take into account the organization of the medical emergency service at Gaslini children's hospital. Emergency medicine has been developing as a pediatric subspecialty, involving medical surgical and intensive care units to meet the peculiar needs of the acutely ill child. Moreover epidemiological data regarding all kinds of activity have been collected; they show a decrease in admitted patients and an increase in outpatients. These data undertime how effectively the service can act as a filter as well.

The paper describes a case of Sotos syndrome and reviews the world literature on the subject. Inheritance may be dominant autosomal as well as recessive, although the latter is quite rare. The pathophysiology is not well known but a common underlying basis between various syndromes (Sotos; Beckwith-Wiedemann; Klippel-Trenaunay) is hypothesised.

In type I diabetes mellitus (DM) the presence of C peptide (Cp), whose determination is unaffected by exogenous insulin, is considered expression of a residual beta-cell activity, which allows a better metabolic control. In 35 children affected by type I DM the fasting Cp was measured: in 18 cases (1st group) a value greater than or equal to 1 ng/ml was observed, while in the remaining 17 (2nd group) the Cp value was less than 1 ng/ml. A statistical comparison between the two groups demonstrated that in the first one a better metabolic control was achieved with a daily lower insulin dosage. Moreover in the 1st group the onset of the disease was more recent, while there was no difference between the two groups with regard to the age at diagnosis. The Cp evaluation and follow-up is useful in the assessment of the individual case; furthermore, on the basis of these studies, an immunosuppressive treatment may be considered during the early phase of the disease, when a residual beta-cell activity is demonstrated.

The Authors describe two cases of theophylline poisoning and discuss advantages of activated charcoal and magnesium hydroxide therapy. This treatment seems to be better in patients for whom hemoperfusion could be delayed. In fact, activated charcoal can determine a real decrease in serum theophylline as experimental data show. Patients who overdose with slow release theophylline preparation should receive multiple oral doses of activated charcoal; they should also receive multiple oral doses of cathartic, both saline or sorbitol like. There is a lack of references about the activated charcoal plus magnesium hydroxide regimen particularly in childhood. The Authors discuss their own experience.