Infantile hemangiomas (IHs) are common neoplasms composed of proliferating endothelial-like cells. Despite the relative frequency of IH and the potential severity of complications, there are currently no uniform guidelines for treatment. Although propranolol has rapidly been adopted, there is significant uncertainty and divergence of opinion regarding safety monitoring, dose escalation, and its use in PHACE syndrome (PHACE = posterior fossa, hemangioma, arterial lesions, cardiac abnormalities, eye abnormalities; a cutaneous neurovascular syndrome characterized by large, segmental hemangiomas of the head and neck along with congenital anomalies of the brain, heart, eyes and/or chest wall). A consensus conference was held on December 9, 2011. The multidisciplinary team reviewed existing data on the pharmacologic properties of propranolol and all published reports pertaining to the use of propranolol in pediatric patients. Workgroups were assigned specific topics to propose protocols on the following subjects: contraindications, special populations, pretreatment evaluation, dose escalation, and monitoring. Consensus protocols were recorded during the meeting and refined after the meeting. When appropriate, protocol clarifications and revision were made and agreed upon by the group via teleconference. Because of the absence of high-quality clinical research data, evidence-based recommendations are not possible at present. However, the team agreed on a number of recommendations that arose from a review of existing evidence, including when to treat complicated IH; contraindications and pretreatment evaluation protocols; propranolol use in PHACE syndrome; formulation, target dose, and frequency of propranolol; initiation of propranolol in infants; cardiovascular monitoring; ongoing monitoring; and prevention of hypoglycemia. Where there was considerable controversy, the more conservative approach was selected. We acknowledge that the recommendations are conservative in nature and anticipate that they will be revised as more data are made available.

Epidermolysis Bullosa (EB) is a group of rare genetic disorders resulting in skin fragility and other symptoms. Commissioned by DEBRA International and funded by DEBRA Norway, this evidence-bases guideline provides recommendations to optimise psychosocial wellbeing in EB. An international multidisciplinary panel of social and health care professionals (HCP) and people living with EB was formed. A systematic international literature review was conducted by the panel following the Scottish Intercollegiate Guidelines Network (SIGN) methodology. The resulting papers underwent systematic selection and critique processes. Included papers were allocated to 6 different outcome groups to allow data synthesis and exploration: quality of life, coping, family, wellbeing, access to HCP and pain. Based on the evidence in those papers, recommendations were made for individuals living with EB, family and caregivers and HCP working in the field. Few studies have investigated interventions and which factors lead to better outcomes, but general recommendations can be made. EB is a complex disease impacting enormously on every aspect of psychosocial life. People and families living with EB need access to multidisciplinary support, including psychological guidance, in order to improve quality of life and psychosocial wellbeing. Interventions should stimulate social participation to prevent isolation. People with EB and their families should be able to access a supportive network. HCP should be well supported and educated about the complexity of EB. They should work collaboratively with those around the individual with EB (e.g. schools, employers etc.) to provide psychosocial opportunity and care. Attention should be paid to the psychosocial impact of EB as well as physical needs. Directions for research are indicated. Electronic supplementary material The online version of this article (10.1186/s13023-019-1086-5) contains supplementary material, which is available to authorized users.

BackgroundDeficits in motor movement in children with autism spectrum disorder (ASD) have typically been characterized qualitatively by human observers. Although clinicians have noted the importance of atypical head positioning (e.g. social peering and repetitive head banging) when diagnosing children with ASD, a quantitative understanding of head movement in ASD is lacking. Here, we conduct a quantitative comparison of head movement dynamics in children with and without ASD using automated, person-independent computer-vision based head tracking (Zface). Because children with ASD often exhibit preferential attention to nonsocial versus social stimuli, we investigated whether children with and without ASD differed in their head movement dynamics depending on stimulus sociality.MethodsThe current study examined differences in head movement dynamics in children with (n = 21) and without ASD (n = 21). Children were video-recorded while watching a 16-min video of social and nonsocial stimuli. Three dimensions of rigid head movement—pitch (head nods), yaw (head turns), and roll (lateral head inclinations)—were tracked using Zface. The root mean square of pitch, yaw, and roll was calculated to index the magnitude of head angular displacement (quantity of head movement) and angular velocity (speed).ResultsCompared with children without ASD, children with ASD exhibited greater yaw displacement, indicating greater head turning, and greater velocity of yaw and roll, indicating faster head turning and inclination. Follow-up analyses indicated that differences in head movement dynamics were specific to the social rather than the nonsocial stimulus condition.ConclusionsHead movement dynamics (displacement and velocity) were greater in children with ASD than in children without ASD, providing a quantitative foundation for previous clinical reports. Head movement differences were evident in lateral (yaw and roll) but not vertical (pitch) movement and were specific to a social rather than nonsocial condition. When presented with social stimuli, children with ASD had higher levels of head movement and moved their heads more quickly than children without ASD. Children with ASD may use head movement to modulate their perception of social scenes.Electronic supplementary materialThe online version of this article (10.1186/s13229-018-0198-4) contains supplementary material, which is available to authorized users.

There were no external sources of funding for this study.The authors have no conflicts of interest to declare.

According to dominant theories of affect, humans innately and universally express a set of emotions using specific configurations of prototypical facial activity. Accordingly, thousands of studies have tested emotion recognition using sets of highly intense and stereotypical facial expressions, yet their incidence in real life is virtually unknown. In fact, a commonplace experience is that emotions are expressed in subtle and nonprototypical forms. Such facial expressions are at the focus of the current study. In Experiment 1, we present the development and validation of a novel stimulus set consisting of dynamic and subtle emotional facial displays conveyed without constraining expressers to using prototypical configurations. Although these subtle expressions were more challenging to recognize than prototypical dynamic expressions, they were still well recognized by human raters, and perhaps most importantly, they were rated as more ecological and naturalistic than the prototypical expressions. In Experiment 2, we examined the characteristics of subtle versus prototypical expressions by subjecting them to a software classifier, which used prototypical basic emotion criteria. Although the software was highly successful at classifying prototypical expressions, it performed very poorly at classifying the subtle expressions. Further validation was obtained from human expert face coders: Subtle stimuli did not contain many of the key facial movements present in prototypical expressions. Together, these findings suggest that emotions may be successfully conveyed to human viewers using subtle nonprototypical expressions. Although classic prototypical facial expressions are well recognized, they appear less naturalistic and may not capture the richness of everyday emotional communication. (PsycINFO Database Record

Human observations can only capture a portion of ongoing classroom social activity, and are not ideal for understanding how children’s interactions are spatially structured. Here we demonstrate how social interaction can be investigated by modeling automated continuous measurements of children’s location and movement using a commercial system based on radio frequency identification. Continuous location data were obtained from 16 five-year-olds observed during three 1-h classroom free play observations. Illustrative coordinate mapping indicated that boys and girls tended to cluster in different physical locations in the classroom, but there was no suggestion of gender differences in children’s velocity (i.e., speed of movement). To detect social interaction, we present the radial distribution function, an index of when children were in social contact at greater than chance levels. Rank-order plots indicated that children were in social contact tens to hundreds of times more with some peers than others. We illustrate the use of social ties (higher than average levels of social contact) to visualize the classroom network. Analysis of the network suggests that transitivity is a potential lens through which to examine male, female, and mixed-sex cliques. The illustrative findings suggest the validity of the new measurement approach by re-examining well-established gender segregation findings from a new perspective.

Infantile hemangiomas (IH) are benign tumors of endothelial-like cells. Occurring in 4.5% of children, they are the most common tumor of childhood. The great majority of patients with IH will not need treatment, but 10% require systemic treatment. Many treatments have been described for the treatment of IH, but the Food and Drug Administration has not approved any. Over the last decade, numerous reports of successful treatment of IH with propranolol have been published. Despite its widespread use, little is known regarding the proper dosing, safety monitoring, and during of treatment or long-term outcomes for propranolol treatment of IH. Given its potential side effects, detailed education regarding proper administration of the medication as well as warning signs to watch for is necessary for parents and caretakers. Herein, we provide a parental handout that practitioners can individually tailor for use in their clinics when educating parents and caretakers about the use of propranolol for IH. Updates will also need to be made as more is learned regarding the optimal dosing and safety monitoring when using propranolol for this indication. Forward This article is a little different from the usual Techniques for Tots which have typically focused on examination or surgical techniques. We hope the readers will find it useful as an educational tool for families whose children are receiving propranolol for infantile hemangiomas. Ilona J. Frieden M.D. Lawrence F. Eichenfield M.D. Editors, Pediatric Dermatology

Positive emotional engagement develops in the context of face-to-face interactions during the first six months of life. Deficits in emotional engagement are characteristic of autism spectrum disorder (ASD) and may characterize the younger siblings of children with ASD (high-risk siblings). High-risk siblings are likely to exhibit a broad range of positive emotional engagement that may or may not be associated with ASD outcomes. We examined positive emotional engagement (i.e., smiling rate and contingent responsiveness to the partner’s smile) during the infant-parent interaction episodes of the Face-to-Face/Still Face protocol at six months of age. The sample included 43 high-risk infant siblings, 11 of whom went on to an ASD diagnosis, and 25 low-risk siblings with no family history of ASD. Low-risk siblings and high-risk siblings without ASD showed the typical “still-face effect” (i.e., decreases in smiling rate after period of parental non-responsiveness), but high-risk siblings with later ASD outcomes did not show this decrease. Although high-risk siblings without an ASD diagnosis were less likely to respond to their parents’ smiles than low-risk siblings, the children with eventual ASD did not differ from the other groups in contingent responsiveness. Findings suggest that subtle differences in positive emotional engagement are present in the early development of high-risk siblings but are not consistently associated with ASD outcomes.