This paper aims to propose that the psychiatrist George Frankl had more than a marginal role in the early history of autism. Frankl's conception of autism as characterized by a lack of affective language has influenced both Asperger and Kanner. First, this proposal is historically supported; second it is corroborated by Frankl's unpublished manuscript on Autism. We found that Frankl's perspective about autism was, and still can be, considered innovative for multiple reasons. Specifically, Frankl proposed that autism could cover a spectrum of conditions; that it is a state of mind that is not necessarily abnormal; and that it is a neurobiological condition, which primarily needs to be understood by others. Finally, Frankl's concepts of affective contact and affective language are reconsidered with reference to contemporary neuropsychology from which autism emerges not as a higher-order cognitive deficit, but as a result of an impairment of primordial ability to process low level sensory, motor and perceptual information gained through experiencing other persons.Screen media multitasking (SMM), the act of exposure ≥ 2 screen media simultaneously, is increasing nowadays. Single media use was associated with child's behavioural problems in previous studies. However, the associations between SMM since early childhood and behavioural problems in school-age children had not been elucidated. This study aimed to investigate the association between SMM from age 6 months to 4 years and behavioural problems in 6-year-old children. SAN Two hundred and ninety-one (52.6% girls) healthy children were enrolled since age 6 months and 259 participants were followed-up until age 6 years. SMM was obtained at age 6, 12, 18, 24 months, 3 and 4 years. Behavioural concerns were assessed at age 4 years by the Child Behaviour Checklist and by both the Strengths and Difficulties Questionnaire with the Conners Kiddie Continuous Performance Test at age 6 years. Parenting style, cognitive ability, and baseline characteristics were also ascertained. Partial least squares path modelling was performed to determine both direct and indirect relationships among variables. Results showed that children who had been exposed to SMM for longer duration, specifically at age 18 months, showed more behavioural problems at age 4 and 6 years. Positive parenting during preschool and behavioural concerns at age 4 years were main mediators of those associations. Moreover, SMM in minutes at age 18 months was related to decreased preschool cognition. To minimise behavioural problems in school-age children, SMM should be avoided before 2 years of age so that positive parenting may be successfully implemented since early childhood.Pediatric patients undergoing mechanical thrombectomy may be challenging for the anesthesiologists as regards the best anesthetic choice, especially if concomitant to severe comorbidities such as heart failure. A 16-year-old patient affected by arrhythmogenic right ventricle dysplasia/cardiomyopathy underwent mechanical thrombectomy. He was not eligible for deep sedation or general anesthesia since he has been suffering from severe heart failure. The patient stillness was obtained by intra-arterial injection of propofol from the contralateral internal carotid artery. The procedure has been well tolerated, without cardiorespiratory impairment. The case stresses the growing importance to tailor a proper anesthesiologic plan during mechanical thrombectomy, especially in extreme conditions.
Calcinosis is a common complication of systemic sclerosis with no known effective pharmacologic therapy. We reviewed the literature regarding systemic sclerosis-related calcinosis as well as other disorders of biomineralization in order to identify targets of future study for calcinosis.
Patients with systemic sclerosis-related calcinosis demonstrate systemic abnormalities in mineralization pathways, including decreased levels of the mineralization inhibitor inorganic pyrophosphate. Insights from other mineralization disorders suggest that local and systemic phosphate metabolism pathways involving the ABCC6, ENPP1, and NT5E genes play a critical role in regulation of ectopic calcification. Knockout models of these genes may lead to an appropriate murine model for study of calcinosis. Poly(ADP-ribose) polymerase (PARP) enzymes may also play a critical role in hydroxyapatite nucleation and warrant future study in systemic sclerosis. Study of local and systemic mineralization pathways, particularly phosphate metabolism pathways and PARP enzymes, should provide greater insight into the pathogenesis of systemic sclerosis-related calcinosis.
Patients with systemic sclerosis-related calcinosis demonstrate systemic abnormalities in mineralization pathways, including decreased levels of the mineralization inhibitor inorganic pyrophosphate. Insights from other mineralization disorders suggest that local and systemic phosphate metabolism pathways involving the ABCC6, ENPP1, and NT5E genes play a critical role in regulation of ectopic calcification. Knockout models of these genes may lead to an appropriate murine model for study of calcinosis. Poly(ADP-ribose) polymerase (PARP) enzymes may also play a critical role in hydroxyapatite nucleation and warrant future study in systemic sclerosis. Study of local and systemic mineralization pathways, particularly phosphate metabolism pathways and PARP enzymes, should provide greater insight into the pathogenesis of systemic sclerosis-related calcinosis.The original version of this article, published on 22 July 2020, unfortunately contained a mistake.Though ventricular assist devices (VADs) are an important treatment option for acute heart failure, an extracorporeal membrane oxygenator (ECMO) is usually used in pediatric patients for several reasons. However, a temporary centrifugal pump-based Bi-VAD might have clinical advantages versus ECMO or implantable VADs. From January 2000 to July 2018, we retrospectively reviewed 36 pediatric patients who required mechanical circulatory support (MCS) for acute heart failure. Cases with postoperative MCS were excluded. Since 2016, we have tried to immediately add a right VAD rather than ECMO, when the patients begin to present features of right heart failure after left VAD support started in cases that the patients' respiratory function did not require an oxygenator. Original diagnoses included dilated cardiomyopathy (n = 18), myocarditis (n = 11), and others (n = 7). Eleven patients were supported by Bi-VAD, and 25 patients were supported by ECMO; of these. Four patients were successfully weaned from VAD, and 10 patients were weaned from ECMO.SAN
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