Overcoming limits: First ABO incompatible living donor paired kidney transplant in an hypersensitized pediatric recipient in Spain.

INTRODUCTION: HLA sensitization is a growing problem in children awaiting kidney transplantation. In some cases, finding an immunologically compatible donor entails contemplating the option of an ABO incompatible transplant or paired transplant. METHODS: Patient with genetic nephrotic syndrome and progressive chronic kidney disease, with a previous thrombosis of a first kidney transplant, resulting hypersensitized and remaining for a long-time on hemodialysis. Despite a desensitization strategy, family members were incompatible and deceased donation options must be ruled out due to the presentation of donor-specific antibodies (DSA). After 4 years, the possibility arises to perform a kidney paired transplant with a 62-year-old woman with an incompatible blood group. Although the current cytotoxicity- and cell-based crossmatches were negative, history of DSA were recorded. RESULTS: An intensive ABO and HLA desensitization protocol was performed in order to combat the isohemagglutinin antibodies and on the memory-HLA, based on rituximab, apheresis sessions, and immunoglobulins. Despite the donor being older in terms of pediatric transplantation, the donor-recipient weight difference, and immunological risk, the transplant was completed successfully. Maintenance of titration of up to 1/2 was confirmed after 3 weeks post-transplant (IgM and IgG). Kidney biopsy at 2 weeks and 6 months without signs of rejection. The patient is currently 12 months post-transplant and has not presented any signs of transplant rejection and has proper renal function. CONCLUSIONS: Kidney paired transplantation is an excellent solution for hypersensitized children, and ABO incompatibility can be considered to increase their options to find a good donor, without thereby obtaining worse results.

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.

Clinical, biochemical and microbiological factors associated with the prognosis of pneumococcal meningitis in children.

BACKGROUND: Pneumococcal meningitis (PM) has a high morbidity and mortality. The aim of the study was to evaluate what factors are related to a poor PM prognosis. METHODS: Prospective observational study conducted on patients admitted to the Pediatric Intensive Care Unit in a tertiary hospital with a diagnosis of PM (January 2000 to December 2013). Clinical, biochemical and microbiological data were recorded. Variable outcome was classified into good or poor (neurological handicap or death). A multivariate logistic regression was performed based on the univariate analysis of significant data. RESULTS: A total of 88 patients were included. Clinical variables statistically significant for a poor outcome were younger age (p=.008), lengthy fever (p=.016), sepsis (p=.010), lower Glasgow Score (p<.001), higher score on Pediatric Risk Mortality Score (p=0.010) and Sequential Organ Failure Assessment (SOFA) (p<.001), longer mechanical ventilation (p=.004), and inotropic support (p=.008) requirements. Statistically significant biochemical variables were higher level of C-reactive protein (p<.001) and procalcitonin (p=.014) at admission, low cerebrospinal (CSF) pleocytosis (p=.003), higher level of protein in CSF (p=.031), and severe hypoglycorrhachia (p=.002). In multivariate analysis, independent indicators of poor outcome were age less than 2 years (p=.011), high score on SOFA (p=.030), low Glasgow Score (p=.042), and severe hypoglycorrhachia (p=.009). CONCLUSIONS: Patients younger than 2 years of age, with depressed consciousness at admission, especially when longer mechanical ventilation is required, are at high risk of a poor outcome.

Association Between VDR and CYP24A1 Polymorphisms, Atopic Dermatitis, and Biochemical Lipid and Vitamin D Profiles in Spanish Population: Case-Control Study.

BACKGROUND: Atopic dermatitis (AD) is the most prevalent inflammatory skin disorder, characterized by impaired epidermal barrier function and an altered immune response, both of which are influenced by vitamin D deficiency. Single-nucleotide polymorphisms (SNPs) in VDR and CYP24A1 have been previously associated with AD. OBJECTIVE: We sought to characterize the associations between the VDR and CYP24A1 polymorphisms and the vitamin D and lipid biochemical profile in children diagnosed with AD. METHODS: A total of 246 participants (143 patients with AD and 103 healthy controls) were enrolled in this study. Genotyping for polymorphisms in VDR (rs2239185, rs1544410, rs7975232, rs2238136, rs3782905, rs2239179, rs1540339, rs2107301, rs2239182, and rs731236) and CYP24A1 (rs2248359 and rs2296241) was performed by allele-specific polymerase chain reaction using integrated fluidic circuit technology. Serum levels of calcium, phosphorus, and vitamin D were measured, and the biochemical lipid profile was determined. RESULTS: Among VDR SNPs, rs2239182 exerted a protective effect against the development of AD, whereas rs2238136 was identified as a risk factor for AD. The GCC haplotype (rs2239185-G, rs1540339-C, and rs2238136-C) appeared to protect against the development of AD. rs2239182-CC was associated with higher 25(OH)D concentrations, whereas rs2238136-TT, rs2239185-GA, and rs2248359-TT were present in a large proportion of patients with serum vitamin D deficiency. rs2239185-AA, rs2239182-CC, and rs1540339-CC were associated with higher serum total cholesterol; rs2239182-TT was associated with lower low-density lipoprotein cholesterol; and rs2239182-TC with lower high-density lipoprotein cholesterol. Both CYP24A1 SNPs (rs2296241-AA and rs2248359-TT) were associated with higher high-density lipoprotein cholesterol levels. CONCLUSIONS: The VDR SNP rs2238136 is a risk factor for AD and other SNPs in VDR and CYP24A1, which may lead to alterations in biochemical parameters that influence the risk of AD. Our findings highlight the complex genetic basis to AD and indicate that interrelationships between different genetic factors can lead to alterations in vitamin D metabolism or lipid profiles, which in turn may influence the development of AD.

Self-Reported Hidradenitis Suppurativa Severity: Is It Useful for Clinical Practice?

INTRODUCTION: Hidradenitis suppurativa (HS) is considered to be the most burdensome dermatosis, with a well-documented negative influence on quality of life (QoL). The patient's perception of the disorder, assessed as the self-reported severity, has been used in other dermatoses but not in HS. The aim of this study was to evaluate the usefulness of self-reported HS severity in clinical practice. METHODS: The study was performed on a group of 130 Spanish HS patients. HS severity was assessed for all the subjects. Hurley staging and patient self-reported severity were used. Moreover, QoL impairment was evaluated using the Dermatology Life Quality Index (DLQI) and the Hidradenitis Suppurativa Quality of Life 24 (HSQoL-24) questionnaire. RESULTS: The severity of HS according to the Hurley staging was most commonly assessed as Hurley II (47.7%), indicating moderate disease, followed by severe disease (Hurley III, 26.9%) and mild disease (Hurley I, 25.4%). According to the patient self-reported HS severity, most of the patients reported having mild disease (76 patients, 58.5%), followed by moderate disease (31 patients, 23.8%). Only 23 patients (17.7%) assessed their disease as severe. Moreover, men reported mild disease significantly more frequently than women (70.9% and 49.3%, respectively; p = 0.014). The self-reported HS severity correlated positively with the effect of the disease on patient QoL assessed with DLQI (r = 0.288, p < 0.001). Likewise, a strong positive correlation was found between self-reported HS severity and QoL impairment assessed with HSQoL-24 (r = 0.404, p = 0.001). No statistically significant correlation between Hurley severity stage and DLQI or HSQoL-24 was found. Moreover, there were significant differences in both DLQI and HSQoL-24 total score between different self-reported HS severities. This was not seen for any of the QoL instruments or for Hurley severity staging. CONCLUSION: The results show that self-assessment severity may reflect patients' subjective feelings more adequately than popular objective instruments, and there should be a place for its use in daily clinical practice.

Quantifying Physician Preferences for Systemic Atopic Dermatitis Treatments Using a Discrete-Choice Experiment.

INTRODUCTION: As research continues, new drugs will no doubt be added to the current pool of treatments for moderate-to-severe atopic dermatitis (AD). This raises the need for studies to determine prescriber preferences for different pharmacological options and the factors that influence their choice of treatment. Here we aim to explore physician preferences in the systemic treatment of moderate-to-severe AD, identify the sociodemographic characteristics that can influence physician preferences, and evaluate their satisfaction with current AD therapies. METHODS: A discrete-choice experiment (DCE) survey was administered to physicians treating patients with AD in Spain. Results were analyzed using a conditional logit model to estimate the relative importance of each attribute and the maximum risk accepted to achieve therapeutic benefit. RESULTS: A total of 28 respondents completed the DCE survey (67.9% female, mean age 45.9 years). Participants identified objective clinical efficacy and risk of severe adverse events (AEs) as the most important attributes, followed by improvement in sleep and pruritus and faster onset of action from the start of the treatment. Respondents gave less importance to mode of administration and therapeutic benefit in other atopic conditions. Respondents were willing to accept an increased risk of severe AEs and mild-to-moderate AEs leading to treatment discontinuation due to intolerance in order to obtain improvements in efficacy, sleep, and pruritus, and long-term clinical benefit. CONCLUSION: Our findings can help prescribers choose the most appropriate systemic AD therapy.

The Density of Renal Lymphatics Correlates With Clinical Outcomes in IgA Nephropathy.

Introduction: IgA nephropathy (IgAN) is the most common primary glomerulonephritis (GN) worldwide. The disease course fluctuates, and the most important challenge is the considerable variation in the time lag between diagnosis and the development of a hard clinical end point, such as end-stage kidney disease (ESKD). The reaction of renal tissue to damage resembles the common wound-healing response. One part of this repair in IgAN is the expansion of lymphatic vessels known as lymphangiogenesis. The aim of this work was to establish the prognostic value of the density of lymphatic vessels in the renal biopsy at the time of diagnosis, for predicting the risk of ESKD in a Spanish cohort of patients with IgAN. Methods: We performed a retrospective multicenter study of 76 patients with IgAN. The end point of the study was progression to ESKD. The morphometric analysis of lymphatic vessels was performed on tissue sections stained with antipodoplanin antibody. Results: Density of lymphatic vessels was significantly higher in patients with IgAN with mesangial hypercellularity >50%, segmental sclerosis, higher degrees of interstitial fibrosis, and tubular atrophy. Patients with more lymphatic vessels had significantly higher values of proteinuria and lower estimated glomerular filtration rate (eGFR). A density of lymphatic vessels ≥8 per mm2 was associated with a significantly higher rate of progression to ESKD at 3 years from biopsy. After adjustment for the International IgAN prediction score, at the multivariate logistic regression, high density of lymphatic vessels (≥8 per mm2) remained significantly associated with a higher rate of early progression to ESKD. Conclusion: This study contributes to the understanding of the natural history of the progression to ESKD in patients with IgAN revealing the density of lymphatics vessels may optimize the prognostic value of the International IgA predicting tool to calculate the risk of ESKD, favoring the evaluation of new targeted therapies.

The Use of HSQoL-24 in an Assessment of Quality-of-Life Impairment among Hidradenitis Suppurativa Patients: First Look at Real-Life Data.

Hidradenitis suppurativa (HS) is a chronic inflammatory skin disorder with well-documented effects on patients' quality of life (QoL). The aim of this study was to evaluate the QoL of patients with HS via the use of a newly developed questionnaire: Hidradenitis Suppurativa Quality of Life-24 (HSQoL-24). This study was performed on a population of 342 HS patients. Their QoL was assessed via the HSQoL-24 questionnaire. The perceived impairment of QoL due to HS in the studied group was considered to be serious (mean HSQoL-24 score: 58.3 ± 21.0 points). Women tended to experience a significantly higher impact from the disease than men (61.6 ± 19.2 points vs. 51.1 ± 23.1 points, p < 0.001). The HS severity had an effect on the perceived QoL, with statistically significant differences being evident between the self-assessed HS severity groups. The level of QoL impairment correlated positively with the number of affected body areas (r = 0.285, p < 0.001) and the duration of the disease (r = 0.173, p = 0.001), while the patients' age at disease onset correlated negatively with the HSQoL-24 global score (r = -0.182, p = 0.001). Patients living in their family house scored higher than other groups. The least affected were patients who lived alone. The study shows that the HSQoL-24 questionnaire is a reliable, HS-specific tool for measuring the QoL among patients with HS in real-life clinical settings.

Low-level light-assisted photodynamic therapy using a wearable cap-like device for the treatment of actinic keratosis of the scalp.

BACKGROUND: Daylight photodynamic therapy (dlPDT) is a painless and increasingly cost-effective treatment for actinic keratosis (AK). New protocols avoid incubation, minimizing pain and adverse events. However, it is time-consuming and dependent on specific weather conditions. In patients with AK of the scalp, we evaluated the efficacy of indoor photodynamic therapy (PDT) using a wearable low-level light therapy (LLLT) device, without pre-incubation with a photosensitizing agent. METHODS: In this pilot study, 27 patients with thin and moderately thick AK (Olsen Grades I-II) underwent a single 15-minute session of LLLT using a wearable cap-like device immediately after application of methyl-aminolevulinate (MAL) cream, with no prior preparation of the affected area. Treatment efficacy was quantified by measuring the reduction in AK lesion number and the AK quality of life (AKQoL) score. All AK lesions were mapped at baseline for follow-up 2 months later. Paired pre/post scalp biopsies from 5 patients were analysed using histological and immunohistochemical techniques (p53, p27, cyclin D1, p63, and Ki67 expression). Data were analysed using the Wilcoxon signed-rank test. RESULTS: In all patients we observed a global reduction in the number of AK lesions (71%; p < 0.0001) and AKQoL score (from 5.6 to 4.4; p = 0.034) 2 months after treatment. Histology and immunohistochemistry of skin biopsies from 5 patients also revealed marked improvements after LLLT. No patients reported any pain during treatment. CONCLUSION: PDT using LLLT is a rapid, painless, and efficacious modality for the treatment of AK.

The latest on skin photoprotection.

UV radiation is the main etiological agent of most types of skin cancer and a key factor responsible for photoaging. Photoprotection is thus critical to avoid these undesired effects. Sunscreens rank among the best photoprotective measures. Sunscreens are the main components of lotions and creams used to prevent UV-induced damage or to ameliorate its harmful effects. There are 3 types of sunscreens: physical photon blockers, antioxidants, and stimulators of repairing mechanisms. This review summarizes current topics in the development of sunscreens, with special emphasis on substances of natural origin bearing photoscreening, antioxidant, or repairing properties. The characterization of different parameters to evaluate the effects of sunscreens, such as the sunscreen protection factor, is discussed. Finally, the effect of public awareness and public health campaigns are also reviewed.

In vivo reflectance-mode confocal scanning laser microscopy in dermatology.

RCM offers tremendous potential for the advancement of medical research and clinical care. In research, it offers benefits both ex vivo and in vivo. Ex vivo, it can allow us to sample tissue and evaluate it noninvasively to determine what further testing--on the same exact tissue--may be helpful. In vivo RCM can be used to study normal or pathophysiologic processes in real-time noninvasively and by the same technique sequentially over time. Immunologic events previously only studied ex vivo or by static images can be traced from their inception to completion (Table 1). The potential of RCM in vivo is tremendous. How would our world change if we could noninvasively diagnose skin lesions and, with the advent of new minimally invasive therapies, administer treatment and noninvasively monitor that treatment? The potential to allow better medical care based on actual visualization of therapeutic response and healing is obvious. Much like early X-ray and ultrasound imaging, RCM is in its infancy. It is only a matter of time and continued persistent research that will lead to similar success and utility for RCM.

Clinical, biochemical and microbiological factors associated with the prognosis of pneumococcal meningitis in children / Factores clínicos, bioquímicos y microbiológicos relacionados con el pronóstico de la meningitis neumocócica en niños

BACKGROUND: Pneumococcal meningitis (PM) has a high morbidity and mortality. The aim of the study was to evaluate what factors are related to a poor PM prognosis. METHODS: Prospective observational study conducted on patients admitted to the Pediatric Intensive Care Unit in a tertiary hospital with a diagnosis of PM (January 2000 to December 2013). Clinical, biochemical and microbiological data were recorded. Variable outcome was classified into good or poor (neurological handicap or death). A multivariate logistic regression was performed based on the univariate analysis of significant data. RESULTS: A total of 88 patients were included. Clinical variables statistically significant for a poor outcome were younger age (p = .008), lengthy fever (p = .016), sepsis (p = .010), lower Glasgow Score (p < .001), higher score on Pediatric Risk Mortality Score (p = 0.010) and Sequential Organ Failure Assessment (SOFA) (p < .001), longer mechanical ventilation (p = .004), and inotropic support (p = .008) requirements. Statistically significant biochemical variables were higher level of C-reactive protein (p < .001) and procalcitonin (p = .014) at admission, low cerebrospinal (CSF) pleocytosis (p = .003), higher level of protein in CSF (p = .031), and severe hypoglycorrhachia (p = .002). In multivariate analysis, independent indicators of poor outcome were age less than 2 years (p = .011), high score on SOFA (p = .030), low Glasgow Score (p = .042), and severe hypoglycorrhachia (p = .009). CONCLUSIONS: Patients younger than 2 years of age, with depressed consciousness at admission, especially when longer mechanical ventilation is required, are at high risk of a poor outcome

INTRODUCCIÓN: Las meningitis neumocócicas (MN) se relacionan con una elevada morbimortalidad. El objetivo del estudio es evaluar qué factores se relacionan con un peor pronóstico. MÉTODOS: Estudio prospectivo observacional con pacientes diagnosticados de MN ingresados en la Unidad de Cuidados Intensivos Pediátricos de un hospital de tercer nivel (enero 2000-diciembre 2013). El pronóstico fue clasificado en buena o mala evolución (secuelas neurológicas o muerte). Se realizó un análisis multivariante de los resultados significativos obtenidos en el análisis univariante. RESULTADOS: Se reclutaron 88 pacientes. Las variables clínicas relacionadas de forma estadísticamente significativa con una peor evolución fueron: menor edad (p = 0,008), mayor duración de la fiebre (p = 0,016), sepsis (p = 0,010), menor puntuación en la Escala de Glasgow (p < 0,001), mayor puntuación en Pediatric Risk Mortality Score (p = 0,010) y Sequential Organ Failure Assessment(SOFA) (p < 0,001), ventilación mecánica (p = 0,004) y soporte inotrópico (p = 0,008) prolongados. Las bioquímicas fueron: mayor elevación de proteína C reactiva (p < 0,001) y de procalcitonina (p = 0,014) al ingreso, menor pleocitosis en líquido cefalorraquídeo (p = 0,003), intensas proteinorraquia (p = 0,013) e hipoglucorraquia (p = 0,002). En el análisis multivariante, los factores independientes relacionados con una peor evolución fueron: edad inferior a 2 años (p = 0,011), elevada puntuación en SOFA (p = 0,030), menor puntuación en la Escala de Glasgow (p = 0,042) e hipoglucorraquia intensa (p = 0,009). CONCLUSIONES: Los menores de 2 años con mayor depresión del sensorio al ingreso, especialmente cuando requieren soporte ventilatorio prolongado, tienen un mayor riesgo de mala evolución

La prevención del cáncer de piel: una asignatura pendiente en atención primaria / Prevention of skin cancer: A task still to be addressed in primary care

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Pioderma gangrenoso bilateral asociado a colitis ulcerosa y proctocolectomía restauradora / Bilateral pyoderma gangrenosum associated with ulcerative colitis and restorative proctocolectomy

No disponible

Factores asociados a la fotoprotección de los niños: una encuesta realizada a los padres / Predictors of sun protection in children: a parental survey

Fundamento: la exposición solar en la infancia parece ser importante para el desarrollo del cáncer de piel. Objetivo: conocer qué saben los padres sobre los efectos del sol en la piel y cuáles son sus hábitos de fotoprotección, los de sus hijos y los factores relacionados con ello. Métodos: estudio transversal en el que se encuestaron 460 padres de niños de 1.°, 3.° y 5.° cursos de Educación Primaria pertenecientes a cinco colegios de Huesca. Resultados: cuatrocientos cuarenta y dos padres respondieron la encuesta. Todos ellos (100%) opinaron que la fotoprotección es necesaria en los niños y el 96,7% de los padres afirman que sus hijos utilizan al menos un método de fotoprotección. El más usado fue la crema tanto por padres como por hijos, siendo ésta de factor de protección solar (FPS) 15 en el 78,05% de los hijos y en el 50% de los padres. El uso de dicho FPS por los hijos se asociaba principalmente con que sus padres también lo utilizaran y, además, con que los hijos tuvieron una edad 7 años, su piel fuera clara y sus padres conocieran la relación entre exposición solar en la infancia y cáncer de piel. Conclusiones: los hábitos de fotoprotección de los padres parecen ser el factor más determinante en la fotoprotección de sus hijos (AU)

Efectividad del programa educativo escolar 'SolSano' para la prevención del cáncer de piel / Efectivity of the skin cancer prevention programme for children 'SolSano'

Fundamento: enseñar a los niños a fotoprotegerse adecuadamente puede ayudar de forma importante a la prevención del cáncer cutáneo en el futuro. Objetivo: evaluar la efectividad del programa educativo escolar para la prevención del cáncer cutáneo Sol-Sano y proporcionar a los niños unos correctos conocimientos y hábitos de fotoprotección. Métodos: estudio experimental realizado en dos grupos de niños de entre 6 y 8 años de edad: 222 alumnos recibieron el programa y 190 alumnos no. Resultados: comparados con los controles los niños que recibieron el programa tenían mejores conocimientos y comportamientos de fotoprotección: 66,22% de estos niños empleaban fotoprotector con un factor de protección solar (FPS) igual o mayor a 15, mientras que en el grupo control el 62,35% desconocían el FPS que utilizaban. El porcentaje de niños que había sufrido alguna quemadura solar durante el verano y el porcentaje que mostraba un gusto por el bronceado fue significativamente menor en el grupo con intervención que en el control (12,79% y 13,52% menos, respectivamente [p < 0,05]). Además todos los métodos de fotoprotección fueron más representados en los dibujos de los alumnos que habían recibido la intervención que en los de los controles (p < 0,05). Conclusiones: el programa Sol-Sano proporciona unos adecuados conocimientos y hábitos de fotoprotección a los niños (AU)