Humanistic and Economic Burden of Atopic Dermatitis in Pediatric Patients in Spain: A Systematic Review.

Atopic dermatitis (AD) is a chronic, inflammatory skin disease affecting all age groups, particularly children. This systematic review provides an overview of the humanistic and economic disease burden in the pediatric population with AD in Spain. The evidence, collected from 11 observational studies published over the past 10 years, exhibits the most common characteristics of the patients, disease burden, patient-reported outcomes, use of resources, and treatment patterns. The burden of AD extends beyond physical symptoms, with associated comorbidities such as asthma and impaired health-related quality of life and mental health disorders, particularly in severe cases. Traditional therapies, primarily topical corticosteroids, face adherence and efficacy challenges. Despite promising innovative treatments and available biological therapies, their use is still limited in the pediatric population. The findings of the present review highlight the scarce scientific evidence on the economic burden of pediatric AD, as well as the most updated humanistic evidence on this disease. At the same time, the need for individualized care and innovative therapeutic interventions to address the multifaceted challenges of pediatric AD in Spain is evident.

Accessible interferometric autocorrelator for noise-like pulses based on a Fabry-Perot cavity.

In this work, we develop experimentally a Fabry-Perot fiber optic interferometer applied to the measurement of autocorrelation of complex dynamic pulses generated by a figure-eight fiber laser. The principle is based in the superposition of multiple pulses, which requires two partially reflecting flat surfaces in parallel, resulting in a simple and compact autocorrelator design. The autocorrelation trace obtained exhibits a typical double-scaled structure for noise-like pulses (NLPs), with an ultrashort coherence spur on the order of 100 fs riding upon a broad pedestal of 120 ps. Finally, we show experimentally that the developed Fabry-Perot device is able to measure accurately the autocorrelation of NLPs, as confirmed by comparing the measurement with that of a conventional autocorrelator scheme based on a Michelson interferometer, with the additional advantages of a more compact setup and a much easier alignment procedure compared to the latter.

Epidermólisis bullosa en España: Estudio observacional de una cohorte de pacientes atendidos en un centro de referencia nacional / Epidermolysis Bullosa in Spain: Observational Study of a Cohort of Patients Treated in a National Referral Center

Antecedentes y objetivo La epidermólisis bullosa (EB) es un grupo heterogéneo de trastornos hereditarios caracterizado por un aumento de la fragilidad mucocutánea. El objetivo del presente estudio es describir las características clínicas y epidemiológicas de los pacientes con EB atendidos en el Hospital Universitario La Paz, centro de referencia nacional para EB hereditaria. Material y método Estudio observacional, retrospectivo y unicéntrico. Se incluyeron todos los pacientes con diagnóstico clínico y molecular de EB atendidos en el Servicio de Dermatología del Hospital Universitario La Paz desde el 1 de enero de 2000 hasta el 28 de febrero de 2021. Resultados Se registraron 214 pacientes, con una edad mediana de 17 años (RIQ: 8-32); el 54,2% fueron mujeres. Las formas clínicas correspondieron a EB distrófica con 135 (63,1%) casos, EB simple con 67 (31,3%) casos, EB juntural con ocho (3,7%), EB Kindler con tres (1,4%) casos y EB adquirida con un (0,5%) caso. El 35,5% de los pacientes procedían de Madrid. Las complicaciones clínicas más frecuentes en nuestra serie fueron el prurito (63,1%), las infecciones locales (56,5%) y el dolor (54,7%). Las complicaciones más graves fueron las cardíacas (5,6%) y la aparición de CCE (10,3%). Fallecieron 22 pacientes (10,3%). Conclusiones La forma clínica predominante fue la EBDR. Las complicaciones más prevalentes fueron el prurito, el dolor y las infecciones, y las más graves, la miocardiopatía y el CCE. Es un estudio pionero realizado en nuestro país que permitirá implementar estrategias para mejorar la situación sociosanitaria de los pacientes con EB (AU)

Background and objective Epidermolysis bullosa (EB) is a heterogeneous group of inherited disorders characterized by a high degree of mucocutaneous fragility. This study aimed to describe the clinical and epidemiologic characteristics of patients with EB treated in Hospital Universitario La Paz, a national referral center for inherited EB. Material and methods Observational, retrospective, single-center study. We included all cases with a clinical and molecular diagnosis of EB managed in the hospital's dermatology department from January 2, 2000, to February 28, 2021. Results A total of 214 cases were studied. The median (interquartile range) age was 17 (8–32) years; 54.2% were women. One hundred thirty-five (63.1%) patients had dystrophic EB, 67 (31.3%) had EB simplex, 8 (3.7%) had junctional EB, and 3 (1.4%) had Kindler syndrome. One (0.5%) had EB acquisita. Over a third (35.5%) of the patients resided in Madrid. The most common clinical complications were pruritus (63.1%), local infections (56.5%), and pain (54.7%). The most serious ones were cardiomyopathy (in 5.6%) and squamous cell carcinoma (10.3%). Twenty-two patients (10.3%) died. Conclusions Dystrophic EB was the most prevalent clinical form. The most prevalent complications were pruritus, pain, and infections. The most serious ones were cardiomyopathy and squamous cell carcinoma. This study is the first in Spain that explores strategies for improving the health status and quality of life of patients with EB (AU)

Epidermolysis Bullosa in Spain: Observational Study of a Cohort of Patients Treated in a National Referral Center. / Epidermólisis bullosa en España: Estudio observacional de una cohorte de pacientes atendidos en un centro de referencia nacional.

BACKGROUND AND OBJECTIVE: Epidermolysis bullosa (EB) is a heterogeneous group of inherited disorders characterized by a high degree of mucocutaneous fragility. This study aimed to describe the clinical and epidemiologic characteristics of patients with EB treated in Hospital Universitario La Paz, a national referral center for inherited EB. MATERIAL AND METHODS: Observational, retrospective, single-center study. We included all cases with a clinical and molecular diagnosis of EB managed in the hospital's dermatology department from January 2, 2000, to February 28, 2021. RESULTS: A total of 214 cases were studied. The median (interquartile range) age was 17 (8-32) years; 54.2% were women. One hundred thirty-five (63.1%) patients had dystrophic EB, 67 (31.3%) had EB simplex, 8 (3.7%) had junctional EB, and 3 (1.4%) had Kindler syndrome. One (0.5%) had EB acquisita. Over a third (35.5%) of the patients resided in Madrid. The most common clinical complications were pruritus (63.1%), local infections (56.5%), and pain (54.7%). The most serious ones were cardiomyopathy (in 5.6%) and squamous cell carcinoma (10.3%). Twenty-two patients (10.3%) died. CONCLUSIONS: Dystrophic EB was the most prevalent clinical form. The most prevalent complications were pruritus, pain, and infections. The most serious ones were cardiomyopathy and squamous cell carcinoma. This study is the first in Spain that explores strategies for improving the health status and quality of life of patients with EB.

Low-pressure and liquid level fiber-optic sensor based on polymeric Fabry-Perot cavity.

An experimental study of the interaction between a Mylar® polymer film and a multimode fiber-optic is presented for the simultaneous fiber-optic detection of low-pressure and liquid levels. The junction between the polymer and optical fiber produces an interference spectrum with maximal visibility and free spectral range around 9 dB and 31 nm, respectively. Water pressure, which is controlled by the liquid level, stresses the polymer. As a result, the spectrum wavelength shifts to the blue region, achieving high sensitivities around 2.49 nm/kPa and 24.5 nm/m. The polymeric membrane was analyzed using a finite element model; according to the results, the polymer shows linear stress response. Furthermore, the membrane material is operated below the yielding point. Moreover, the finite analysis provides information about the stress effect over the thickness and the birefringence changes. This sensor exhibits a quadratic polynomial fitting with an adjusted R-squared of 0.9539. The proposed sensing setup offers a cost-effective alternative for liquid level and low-pressure detection.

Clinical presentation, sonographic features and treatment options of segmental stiff skin syndrome.

Segmental stiff skin syndrome is a rare genetic connective tissue disease, which is often misdiagnosed. High-frequency ultrasonography can represent a useful clinical adjunct in the differential diagnosis of this condition, in conjunction with the clinical and histopathological findings. Treatment options are limited and evidence is scarce. We present the clinical, sonographic and histological features of five paediatric patients diagnosed at our institution and discuss their response to treatment.

Neutropenia inducida por adalimumab en un paciente con hidradenitis supurativa / Adalimumab-Induced Neutropenia in a Man With Hidradenitis Suppurativa

No disponible

Morfea con distribución isomórfica en una niña motociclista / Isomorphic Morphea in a Girl Motorcyclist

No disponible

Imagen clínica y ecográfica de un hamartoma fibrolipomatoso cutáneo / Clinical and Ultrasound Image of a Cutaneous Fibrolipomatous Hamartoma

No disponible

Single-mode Bragg gratings in tapered few-mode and multimode fibers.

We propose the implementation of fiber Bragg gratings in tapered few-mode and multimode fibers to accomplish single-mode operation by reducing the core diameter, while preserving the core-cladding structure. The gratings present a single reflection band, and the device shows low insertion losses after the taper fabrication and the fiber Bragg gratings inscription. The excitation of high-order odd modes in the core of the fiber has been identified as the main loss mechanism; it can be prevented by means of symmetric illumination of the fibers. We also demonstrate the excitation of high-order cladding modes (cladding-air modes) along the taper transitions; these modes can be removed without a significant increment of the insertion loss.