Multicenter Study of Long-Term Outcomes and Quality of Life in PHACE Syndrome after Age 10.

OBJECTIVE: To characterize long-term outcomes of PHACE syndrome. STUDY DESIGN: Multicenter study with cross-sectional interviews and chart review of individuals with definite PHACE syndrome ≥10 years of age. Data from charts were collected across multiple PHACE-related topics. Data not available in charts were collected from patients directly. Likert scales were used to assess the impact of specific findings. Patient-Reported Outcomes Measurement Information System (PROMIS) scales were used to assess quality of life domains. RESULTS: A total of 104/153 (68%) individuals contacted participated in the study at a median of 14 years of age (range 10-77 years). There were infantile hemangioma (IH) residua in 94.1%. Approximately one-half had received laser treatment for residual IH, and the majority (89.5%) of participants were satisfied or very satisfied with the appearance. Neurocognitive manifestations were common including headaches/migraines (72.1%), participant-reported learning differences (45.1%), and need for individualized education plans (39.4%). Cerebrovascular arteriopathy was present in 91.3%, with progression identified in 20/68 (29.4%) of those with available follow-up imaging reports. Among these, 6/68 (8.8%) developed moyamoya vasculopathy or progressive stenoocclusion, leading to isolated circulation at or above the level of the circle of Willis. Despite the prevalence of cerebrovascular arteriopathy, the proportion of those with ischemic stroke was low (2/104; 1.9%). PROMIS global health scores were lower than population norms by at least 1 SD. CONCLUSIONS: PHACE syndrome is associated with long-term, mild to severe morbidities including IH residua, headaches, learning differences, and progressive arteriopathy. Primary and specialty follow-up care is critical for PHACE patients into adulthood.

Congenital syphilis presenting with granulomatous scalp nodules.

We describe a case of congenital syphilis in an adopted infant with a unique dermatologic presentation of scalp granulomas, along with lymphadenopathy, anemia, and elevated liver transaminases. To our knowledge, this cutaneous morphology has not been previously reported in the literature. This case highlights the varied clinical presentation of congenital syphilis and the diagnostic challenge it poses for clinicians, especially in the context of unknown prenatal history/unknown risk factors, or if syphilis is acquired during pregnancy after routine screening is performed. As the incidence of congenital syphilis has more than tripled in recent years, this diagnosis should be considered when a neonate or infant presents with unexplained skin nodules.

Photo- and Thermocatalytic CO2 Methanation: A Comparison of Ni/Al2O3 and Ni-Ce Hydrotalcite-Derived Materials under UV and Visible Light.

Catalysts derived from Ni/Al/Mg/Ce hydrotalcite were prepared via a co-precipitation method, varying the Ce/Al atomic ratio. All of the catalytic systems thus prepared were tested for CO2 methanation under dark and photocatalytic conditions (visible and ultraviolet) under continuous flow with the light intensity set to 2.4 W cm-2. The substitution of Al by Ce formed a solid solution, generating oxygen vacancies and Ce3+/Ce4+ ions that helped shift the dissociation of CO2 towards the production of CH4, thus enhancing the activity of methanation, especially at lower temperatures (<523 K) and with visible light at temperatures where other catalysts were inactive. Additionally, for comparison purposes, Ni/Al2O3-based catalysts prepared via wetness impregnation were synthesized with different Ni loadings. Analytical techniques were used for the characterization of the systems. The best results in terms of activity were as follows: Hydrotalcite with Ce promoter > Hydrotalcite without Ce promoter > 25Ni/Al2O3 > 13Ni/Al2O3. Hydrotalcite, with a Ce/Al atomic ratio of 0.22 and a Ni content of 23 wt%, produced 7.74 mmol CH4 min-1·gcat at 473 K under visible light. Moreover, this catalyst exhibited stable photocatalytic activity during a 24 h reaction time with a CO2 conversion rate of 65% and CH4 selectivity of >98% at 523 K. This photocatalytic Sabatier enhancement achieved activity at lower temperatures than those reported in previous publications.

Eosinophilic cellulitis in response to BNT162b2 COVID-19 vaccination.

A 12-year-old boy presented with a 2-week history of persistent pruritic edematous plaques one day after he received the first dose of the BNT162b2 COVID-19 mRNA vaccine. A skin biopsy showed urticarial dermatitis with tissue eosinophilia consistent with a diagnosis of vaccine-associated eosinophilic cellulitis, with polyethylene glycol as a potential trigger.

Uso de la Teleterapia para el Tratamiento de los Trastornos de Ansiedad, TOC y TEPT, Durante la Pandemia por COVID-19 en España / Use of Teletherapy for the Treatment of Anxiety Disorders, OCD and PTSD, During the COVID-19 Pandemic in Spain

La proporción de teletratamiento llevada a cabo por psicólogos durante la pandemia por COVID-19 para el TAG, fobia específica, agorafobia, ansiedad social, pánico, TOC y TEPT, así como si la experiencia en teleterapia tiene un efecto significativo en la demanda no han sido estudiadas. Los resultados indican que el TAG con un 69.2%, es el trastorno que más teletratamiento ha recibido y la fobia con un 60.5%, el que menos. El pánico, la ansiedad social, TEPT, TOC y agorafobia tuvieron una demanda media del 67.2%, 66.8%, 64.0%, 63.5% y 62.2%, respectivamente. La experiencia en teleterapia resultó significativa. El teletratamiento para psicólogos con experiencia aumentó una media del 93.9% respecto al 35.7% de aquellos sin experiencia. Un 22.8% de psicólogos no realizó teletramiento de la ansiedad. Estos resultados puede ser útiles para desarrollar programas específicos de prevención e intervención telemática para los trastornos de ansiedad ante futuras pandemias por coronavirus.

The distribution of teletreatment carried out by psychologists during the COVID-19 pandemic for GAD, specific phobia, agoraphobia, social anxiety, panic, OCD and PTSD, and whether experience in teletherapy has an significant effect on demand have not been studied. The results indicate that GAD with 69.2% has been the disorder that has received the most teletherapy and phobia with 60.5%, the least. Panic, social anxiety, PTSD, OCD, and agoraphobia had a demand of 67.2%, 66.8%, 64.0%, 63.5%, and 62.2%, respectively. The experience in teletherapy was significant. Tele-treatment for experienced psychologists increased an average of 93.9% compared to 35.7% for those without experience. 22.8% of psychologists did not carry out anxiety teletherapy. These results may be useful to develop specific prevention and telematic intervention programs for anxiety disorders in the face of future coronavirus pandemics

Atypical keratosis pilaris-like lesions in a patient with Bethlem myopathy.

Bethlem myopathy is a collagen VI-related myopathy. Collagen VI is primarily not only associated with the extracellular matrix of skeletal muscle, but is also found in the skin, blood vessels, and other organs. Dermatologic findings described for Bethlem myopathy include follicular hyperkeratosis and abnormal scar formation, although clinical and histopathologic photographs remain elusive in the literature. We present a case of atypical keratosis pilaris-like follicular lesions in a patient with Bethlem myopathy and provide histopathologic correlation to better characterize the development of skin lesions in this rare neuromuscular disease.

Quality of Life in Hidradenitis Suppurativa: validation of the HSQoL-24.

To date, there are no disease-specific instruments in Spanish to assess quality of life of patients with hidradenitis suppurativa. A multicentre study was previously carried out in Spain between 2016 and 2017 to develop the Hidradenitis Suppurativa Quality of Life-24 (HSQoL-24), a disease-specific questionnaire to assess quality of life in patients with hidradenitis suppurativa. The objectiv-es of this study are to revalidate the HSQoL-24 in Spanish with a larger sample of patients, and to present the English version. In this multi-centre study in Spain, patients with hidradenitis suppurativa completed the HSQoL-24, the Dermatology Life Quality Index and the Skindex-29. The Hurley staging system was used to assess the severity of the disease. Validation of the questionnaire was carried out in 130 patients, of whom 75 (57.7%) were women. This study demonstrates adequate values of reliability and validity of the HSQoL-24, confirming the previous test re-test validation and making this questionnaire one of wide clinical validity in terms of results perceiv-ed by patients.

Association of Demographic Factors and Infantile Hemangioma Characteristics With Risk of PHACE Syndrome.

Importance: A 2010 prospective study of 108 infants estimated the incidence of PHACE (posterior fossa malformations, hemangioma, arterial anomalies, cardiac defects, eye anomalies) syndrome to be 31% in children with facial infantile hemangiomas (IHs) of at least 22 cm2. There is little evidence regarding the associations among IH characteristics, demographic characteristics, and risk of PHACE syndrome. Objectives: To evaluate demographic characteristics and comorbidities in a large cohort of patients at risk for PHACE syndrome and assess the clinical features of large head and neck IH that may be associated with a greater risk of a diagnosis of PHACE syndrome. Design, Setting, and Participants: This multicenter, retrospective cohort study assessed all patients with a facial, head, and/or neck IH who were evaluated for PHACE syndrome from August 1, 2009, to December 31, 2014, at 13 pediatric dermatology referral centers across North America. Data analysis was performed from June 15, 2017, to February 29, 2020. Main Outcomes and Measures: The main outcome was presence or absence of PHACE syndrome. Data included age at diagnosis, sex, patterns of IH presentation (including size, segment location, and depth), diagnostic procedures and results, and type and number of associated anomalies. Results: A total of 238 patients (mean [SD] age, 2.96 [4.71] months; 184 [77.3%] female) were included in the analysis; 106 (44.5%) met the criteria for definite (n = 98) or possible (n = 8) PHACE syndrome. A stepwise linear regression model found that a surface area of 25 cm2 or greater (odds ratio [OR] 2.99; 95% CI, 1.49-6.02) and involvement of 3 or more locations (OR, 17.96; 95% CI, 6.10-52.85) to be statistically significant risk factors for PHACE syndrome. Involvement of the parotid gland (OR, 0.39; 95% CI, 0.18-0.85) and segment S2 (OR, 0.38; 95% CI, 0.16-0.91) was associated with a lower risk. Race and ethnicity may also be associated with PHACE syndrome risk, although more studies are needed. Conclusions and Relevance: This cohort study further described factors associated with both a higher and lower risk of PHACE syndrome. The presence of multiple anatomical sites and large surface area were associated with greater risk, whereas S2 or parotid IHs were associated with lower, but still potential, risk. These findings can help in counseling families and decision-making regarding evaluation of infants with large head and neck IHs.

Allergic contact dermatitis to immobilization mask used in radiation therapy.

Thermoplastic masks made of polyester resin are used to immobilize patients receiving radiation therapy. We describe a case of allergic contact dermatitis (ACD) to a thermoplastic mask worn by a child receiving proton beam therapy for treatment of medulloblastoma. ACD to a thermoplastic mask in an adult was first reported in 2018. (J Med Case Rep. 2018;12:181) To our knowledge, ACD to a thermoplastic mask has not been reported previously in the pediatric population.

Primary cutaneous aspergillosis at the site of cyanoacrylate skin adhesive in a neonate.

Primary cutaneous aspergillosis is a rare but potentially life-threatening disease. We present the case of a premature infant who developed primary cutaneous aspergillosis with Aspergillus niger at the site of a skin abrasion that had been treated with a purple-colored cyanoacrylate product. The infection was treated successfully with gentle debridement of the cyanoacrylate product, followed by intravenous voriconazole and topical fluconazole. To our knowledge, this is the first reported case of primary cutaneous aspergillosis occurring at the site of cyanoacrylate-based skin adhesive.

Pediatric penile porokeratosis: A case report.

We present what we believe to be the second case of pediatric penile porokeratosis and the youngest case reported. A 6-year-old boy presented with a pruritic, verrucous growth at the urethral meatus that recurred after two meatotomies. The diagnosis of porokeratosis was confirmed by biopsy. Porokeratosis should be added to the differential diagnosis of chronic hyperkeratotic penile lesions in children.

Extensive orf infection in a toddler with associated id reaction.

Orf is a zoonotic parapoxvirus typically transmitted to humans by a bite from goats or sheep. We present an unusual case of multiple orf lesions on the fingers of a 13-month-old child who was bitten by a goat and subsequently developed progressive swelling, blistering, and necrotic papulonodules of the hand followed by an additional diffuse, pruritic, papular rash. A primary diagnosis of orf infection was confirmed using real-time polymerase chain reaction, and the diffuse eruption was clinically consistent with an id reaction. Extensive necrosis and papular id reaction associated with orf rarely have been described.

Uso de las nuevas tecnologías por parte de los psicólogos españoles y sus necesidades / Use of information and communication technologies by Spanish psychologists, and their needs

El objetivo de este trabajo ha sido conocer la situación del uso de las nuevas tecnologías entre los psicólogos de la Comunidad de Madrid (CM) y su actitud ante las mismas. El método para acceder a esta información ha sido construir una encuesta, enviada por e-mail, elaborada a partir de una revisión de la literatura sobre este tema, con poca investigación previa en nuestro entorno pero que ya se ha explorado en otros países. La muestra estaba constituida por la totalidad de los psicólogos inscritos en el COP de Madrid, facilitado por la dirección del mismo, obteniéndose un total de 486 respuestas. Hasta la fecha sólo un 26% utiliza teleterapia. Los principales inconvenientes encontrados son las limitaciones de la comunicación no verbal y la alianza terapéutica, seguidas de la confidencialidad de los datos y los problemas técnicos en el manejo de los mismos

The aim of this paper is to know the real situation in the use of information and communication technologies alongside the attitude of psychologists in Madrid Region. We conducted a quantitative research by means of a questionnaire based on previous literature reviews regarding the same topic in other countries. The sample consisted of all psychologists registered in the Spanish Psychology Association (COP), Madrid branch, with a total 486 responses. Only 26% use teletherapy along with other combined therapy modality involving face to face communication. Drawbacks in the study include primarily limitations of non-verbal communication and the therapeutic alliance, followed by confidentiality of data and technical problems with handling data

Concurrent and longitudinal effects of maternal and paternal warmth on depression symptoms in children and adolescents.

The main aim of this study is to examine the concurrent and longitudinal effects of perceived affection of mothers and fathers separately on the self-reported symptoms of children's depression. Data were obtained from a 3-wave study of 535 families with children (41.3% boys) aged 9-15 years of age. Structural equation models were performed to test different models. Significant effects of mothers' and fathers' affection on depression symptomatology over the three years were found. The longitudinal effects of parental warmth on the child's depression symptoms were mediated over time by the previous levels of the mother's and father's warmth. The presence of parental warmth can lessen the severity of depression symptoms, especially when paternal and maternal warmth are applied consistently over a long period of time. These results were invariant across the child's sex. Treatments for childhood depression should take place over extended periods of time including both fathers and mothers.

"EB, or Not EB?" Neonatal Desquamative Impetigo in a Degloving Pattern.

We present the case of a 7-day-old boy with significant, rapidly spreading blistering and desquamation in a "degloving" pattern on the hands that mimicked epidermolysis bullosa but was ultimately diagnosed as bullous impetigo caused by a clinically aggressive strain of Staphylococcus aureus. Bullous impetigo is a desquamating condition caused by local release of S. aureus exfoliative toxin A and is more commonly seen in children. This case highlights the fragility of newborn skin and reviews the major diagnoses that should be considered in an infant with significant blistering.

Applied Psychology. The Case of the Baer, Wolf and Risley Prescriptions for Applied Behavior Analysis / Psicologia aplicada. El caso de las prescripciones de Baer, Wolf y Riesley para el análisis aplicado de la conducta

Baer, Wolf and Risley, members of the group that promoted the creation of the Journal of Applied Behavior Analysis, offered in its first issue a detailed series of 'prescriptions' that characterized the way research and research articles would be conceived as adequate to the applied field by the journal editors. Their 'prescriptions' have been largely cited, becoming a sign of authors' identification with the journal policy, and widely influencing the structure and topics of this specialized literature.

El "Journal of Applied of Behavior Analysis" publicó en su primer número, en 1967, unas reglas o prescripciones , elaboradas por tres miembros del grupo editorial (Baer, Wolf y Riesley) , que han servido eficazmente para orientar a los futuros autores acerca del modo como construir los articulos que podrían ser publicados en la revista. Esas prescripciones reunen las principales características propias de la investigación aplicada en el campo del análisis conductual.