Bishop Flap and Sigma Flap to Repair Surgical Defects in High-Tension Anatomical Regions. / Colgajo obispo y colgajo sigma para defectos quirúrgicos en zonas anatómicas de elevada tensión.

BACKGROUND: The reconstruction of surgical defects in high-tension anatomical regions is challenging due to the ischemia and subsequent necrosis associated with tension closure. Research on new flaps capable of closing these defects exerting less tension would be a tremendous advancement in dermatological surgery. PATIENTS AND METHODS: We conducted a multicenter, retrospective study that used 2 new flaps-the bishop and the sigma ones-to repair surgical defects in high-tension regions such as the scalp, lower extremities, and the nasal pyramid. The bishop flap was used in 9 patients, 5 of whom exhibited their lesion in the nasal pyramid, 2 in the legs and another 2 in the scalp. The sigma flap was used in 6 patients, 5 of whom exhibited scalp lesions and 1 leg lesion. RESULTS: Uneventful and excellent results were obtained in all 15 patients due to infection, dehiscence, or necrosis. CONCLUSIONS: Both the bishop and the sigma flaps are a good alternative to repair surgical defects in high-tension regions such as the scalp, lower extremities, or the nasal pyramid.

Mpox - Formerly Monkey Pox - in Dermatology: A Review of Epidemiologic Features, Clinical Presentation, Diagnosis, and Treatment. / MPOX (antes viruela del mono): revisión de los aspectos clínicos, epidemiológicos, diagnósticos y terapéuticos más relevantes para el dermatólogo.

Mpox is an emerging zoonotic disease that has spread rapidly around the world. It has been declared a public health emergency of international concern by the World Health Organization. This review is an update for dermatologists on the epidemiology, clinical presentation, diagnosis, and treatment of Mpox. The primary mode of transmission in the current outbreak is close physical contact during sexual activity. Although most of the initial cases were reported in men who have sex with men, anyone who has close contact with an infected person or contaminated fomites is at risk. Classic prodromal features of Mpox include subclinical manifestations and a mild rash. Complications are common but rarely require hospitalization. Polymerase chain reaction analysis of mucocutaneous lesions is the test of choice for a definitive diagnosis. In the absence of specific treatments, management focuses on symptomatic relief.

Pain and Late-Onset of Hidradenitis Suppurativa Can Have a Negative Influence on Occupational Status and Educational Level. A Cross-Sectional Study.

BACKGROUND AND OBJECTIVES: Hidradenitis suppurativa is a chronic and painful condition with negative impact on daily activity. Little information on the impact of disease-specific factors on educational level and occupational status in hidradenitis suppurativa patients has been reported. We sought to identify how disease-specific factors could influence occupational status and educational level in patients with hidradenitis suppurativa. METHODS: Cross-sectional study of patients with hidradenitis suppurativa seen between September 2017 and September 2018. Disease-specific variables were analyzed to find associations in patients with different educational levels and occupational status. RESULTS: Ninety-eight patients were included. Patients with non-university studies had more frequently≥3 affected areas (22.5% [16/73] vs 4.8% [1/22], p=0.049), a higher number of painful days (8.5 [SD 8.8] vs 4.6 [SD 4.8], p=0.048) and a higher score on the VAS scale (6.7 [SD 2.8] vs 5.0 [3.3], p=0.031). Patients from the inactive group had a significantly increased number of painful days (11.2 [SD 10.4] vs 5.7 [SD 6.2], p=0.004). This group had a greater number of patients with a history of depression (61.3% [19/31] vs 27.4% [17/62], p=0.002) and a higher mean BMI (32.3 [9.1] vs 28.4 [6.4], p=0.016). Late disease onset was significantly associated with being "inactive" (26.7% [8/31] vs 6.5% [4/62], p=0.026). No significant differences between severity scales of hidradenitis suppurativa and educational level or occupational status were found. LIMITATIONS: cross-sectional and single center study. CONCLUSIONS: Pain, ≥3 affected areas, history of depression, higher mean BMI, and late onset of hidradenitis suppurativa, are associated with low education level and inactive occupational status.

Pain and late-onset of hidradenitis suppurativa can have a negative influence on occupational status and educational level. A cross-sectional study. / [Artículo traducido] El dolor y la aparición tardía de la hidrosadenitis supurativa pueden tener una influencia negativa en el estatus ocupacional y el nivel educativo. Estudio transversal.

BACKGROUND AND OBJECTIVES: Hidradenitis suppurativa (HS) is a chronic and painful condition with negative impact on daily activity. Little information on the impact of disease-specific factors on educational level and occupational status in hidradenitis suppurativa patients has been reported. We sought to identify how disease-specific factors could influence occupational status and educational level in patients with HS. METHODS: Cross-sectional study of patients with HS seen between September 2017 and September 2018. Disease-specific variables were analyzed to find associations in patients with different educational levels and occupational status. RESULTS: Ninety-eight patients were included. Patients with non-university studies had more frequently ≥ 3 affected areas (22.5% [16/73] vs. 4.8% [1/22], p = 0.049), a higher number of painful days (8.5 [SD 8.8] vs. 4.6 [SD 4.8], p = 0.048) and a higher score on the VAS scale (6.7 [SD 2.8] vs. 5.0 [3.3], p = 0.031). Patients from the inactive group had a significantly increased number of painful days (11.2 [SD 10.4] vs. 5.7 [SD 6.2], p = 0.004). This group had a greater number of patients with a history of depression (61.3% [19/31] vs. 27.4% [17/62], p = 0.002) and a higher mean BMI (32.3 [9.1] vs. 28.4 [6.4], p = 0.016). Late disease onset was significantly associated with being «inactive¼ (26.7% [8/31] vs. 6.5% [4/62], p = 0.026). No significant differences between severity scales of HS and educational level or occupational status were found. LIMITATIONS: cross-sectional and single center study. CONCLUSIONS: Pain, ≥ 3 affected areas, history of depression, higher mean BMI, and late onset of HS, are associated with low education level and inactive occupational status.

Cutaneous leishmaniasis: 20 years' experience in a Spanish tertiary care hospital.

INTRODUCTION AND OBJECTIVES: Cutaneous leishmaniasis is the most common form of leishmaniasis, which is endemic in Spain. The aim of this study was to evaluate the epidemiological and clinical characteristics of cutaneous leishmaniasis seen in our hospital over a period of 20 years, with a particular focus on clinical differences according to immune status and origin of infection MATERIALS AND METHODS: We performed a chart review of 67 cases of cutaneous leishmaniasis diagnosed between 1992 and 2012. Follow-up data were available for 54 patients. RESULTS: Fifty-four patients with cutaneous leishmaniasis were included in the study. Of these, 26 had been diagnosed between 1992 and 2002 and 28 between 2003 and 2012. The mean age at diagnosis was 49 years, there was a predominance of male patients, and the mean time from onset of symptoms to consultation was 3 months. The most common clinical manifestations were plaques and ulcers. Most of the immunodepressed patients and patients with imported leishmaniasis had skin ulcers and/or multiple lesions. During the first decade of the study, diagnosis was based on clinical and histologic findings. These were supported by molecular techniques in the second decade. Pentavalent antimonials were the treatment of choice, producing good results and very few adverse effects CONCLUSION: The number of patients with cutaneous leishmaniasis and with compromised immune status was similar in the periods 1992-2002 and 2003-2013, but more cases of imported leishmaniasis were diagnosed in the second period. Patients with ulcers and/or multiple lesions should be evaluated to rule out immunosuppression or infection by Leishmania species from other parts of the world. Both systemic and intralesional meglumine antimonate was effective and safe.

Desmoplastic melanoma on the nose: electrochemotherapy as an alternative treatment to local advanced disease.

BACKGROUND: Desmoplastic malignant melanoma (DMM) is a rare and usually misdiagnosed type of melanoma. Delayed detection at complicated anatomical locations can lead to the necessity of alternative therapies. OBJECTIVE: Characterization of DMM on the nose, which is the second more frequent type of MM. METHODS: Review of case series of eight pathologically proven DMM on the nose from two referral centres with a mean follow-up of 69 ± 40.5 months. RESULTS: According to a single centre experience, there is a more than 70-fold increased risk of having a DMM on the nose compared with a non-DMM (P < 0.0005, CI99% 16.3-317.3). Clinical and pathological misdiagnoses were frequent, only three of the eight cases were properly diagnosed and treated and indeed they did not experience relapses. Due to non-clinical suspicion and superficial biopsies, three cases were initially pathologically misdiagnosed as basal cell carcinomas and a nevus respectively. Atypical vessels and remnants of pigment on dermoscopy are indicative findings even in non-pigmented cases. Although not significant, the mean disease-free survival differed between cases with a correct initial management (four cases, 66.7 ± 57.3 months) in contrast to improper (four cases, 16.25 ± 18.9 months). Electrochemotherapy achieved a complete local control of disease in two cases unsuitable for surgery. CONCLUSIONS: Use of dermoscopy and correctly selected biopsy of lesions on the face is mandatory to improve early diagnosis of DMM. Improper management of challenging cases implies a more complicated therapy and loco-regional invasion risk. Electrochemotherapy could be a promising therapy in local advanced tumours.

Three-dimensional geometries representing the retinal nerve fiber layer in multiple sclerosis, optic neuritis, and healthy eyes.

BACKGROUND: To represent and interpret the three-dimensional (3D) geometry and the distribution of the axonal damage to the retinal nerve fiber layer (RNFL) in patients with multiple sclerosis (MS) compared with healthy subjects. To analyze alterations in RNFL morphology in eyes of MS patients with or without previous episodes of optic neuritis (ON). METHODS: MS patients (n = 122) and age-matched healthy subjects (n = 108) were enrolled. The Spectralis optical coherence tomography system was used to determine the circumpapillary RNFL thickness. The 768 RNFL thickness measurements were used to evaluate thickness measurements in patients with or without antecedent ON and to design a 3D reconstruction of the RNFL thickness representing the mechanobiologic tissue response to neurodegeneration caused by MS and ON episodes. RESULTS: RNFL thickness was decreased in MS patients, and was higher in the MS group with previous ON. Statistical analysis and 3D RNFL reconstruction revealed greater damage to the ganglionar cells in the superonasal RNFL area (101.77 µm in MS vs. 125.47 µm in healthy subjects) and in the inferotemporal RNFL (119.05 µm in MS eyes and 149.26 µm in healthy eyes). CONCLUSIONS: The 3D representation of RNFL thickness based on measurements allows physicians to better observe damage in the temporal areas, especially in patients with previous ON.

[A retrospective study of clinical, histological, and immunological characteristics in patients with dermatitis herpetiformis. The experience of Hospital Clinic de Barcelona, Spain, between 1995 and 2010 and a review of the literature]. / Estudio retrospectivo de las características clínicas, histológicas e inmunológicas de los pacientes con dermatitis herpetiforme. Experiencia del Hospital Clínic de Barcelona entre los años 1995 y 2010 y revisión de la literatura.

BACKGROUND AND OBJECTIVES: Dermatitis herpetiformis is a chronic bullous disease that is currently considered a cutaneous expression of gluten hypersensitivity. The aim of this study was to analyze and describe the clinical, histological, and immunopathological characteristics of patients with dermatitis herpetiformis assessed at Hospital Clinic de Barcelona, Spain between 1995 and 2010. MATERIAL AND METHODS: Demographic, clinical, serologic, and histopathological data were reviewed for 33 patients with dermatitis herpetiformis. RESULTS: The median age of the patients at the time of disease onset was 30 years and the majority were men. Associated autoimmune disease was present in 49% of patients. In 6 patients, celiac disease was diagnosed before dermatitis herpetiformis. Although excoriations were the most predominant lesions, 9 patients had blisters. Histological findings in skin lesions were compatible with dermatitis herpetiformis in 46% of cases. The most frequently observed staining pattern by indirect immunofluorescence was the presence of granular immunoglobulin A deposits in the basement membrane (62%). More than 80% of intestinal biopsies were compatible with celiac disease. Antibodies linked to gluten sensitivity were observed in 79% of patients. Only 1 malignant tumor was detected. CONCLUSIONS: Notable findings were the frequent presence of bullous lesions, the high prevalence of celiac disease, and the positive findings on intestinal biopsy, all of which are suggestive of late diagnosis. Our findings confirm the lack of specificity of conventional histology in dermatitis herpetiformis and the association of the disease with other immunological disorders.

MPOX (antes viruela del mono): revisión de los aspectos clínicos, epidemiológicos, diagnósticos y terapéuticos más relevantes para el dermatólogo / Mpox — Formerly Monkey Pox — in Dermatology: A Review of Epidemiologic Features, Clinical Presentation, Diagnosis, and Treatment

MPOX es una enfermedad zoonótica emergente que se ha propagado rápidamente por todo el mundo y que ha sido declarada por la OMS como una emergencia de salud pública de interés internacional. Esta es una revisión no sistemática de los aspectos clínicos, epidemiológicos, diagnósticos y terapéuticos más relevantes para el dermatólogo. En el brote actual, la transmisión durante las relaciones sexuales es la principal forma de contagio como resultado del contacto físico cercano. Si bien los casos iniciales se informaron en hombres que tienen sexo con hombres, cualquier persona en contacto cercano con personas o fómites infectados está en riesgo. El pródromo clásico puede ser subclínico y la erupción puede ser sutil. Las complicaciones son frecuentes, pero el requerimiento de hospitalización es infrecuente. El diagnóstico definitivo se realiza mediante PCR de las lesiones mucocutáneas. Actualmente no existen tratamientos específicos, y el tratamiento sintomático es el pilar terapéutico (AU)

Mpox is an emerging zoonotic disease that has spread rapidly around the world. It has been declared a public health emergency of international concern by the World Health Organization. This review is an update for dermatologists on the epidemiology, clinical presentation, diagnosis, and treatment of Mpox. The primary mode of transmission in the current outbreak is close physical contact during sexual activity. Although most of the initial cases were reported in men who have sex with men, anyone who has close contact with an infected person or contaminated fomites is at risk. Classic prodromal features of Mpox include subclinical manifestations and a mild rash. Complications are common but rarely require hospitalization. Polymerase chain reaction analysis of mucocutaneous lesions is the test of choice for a definitive diagnosis. In the absence of specific treatments, management focuses on symptomatic relief (AU)

[Translated article] Mpox – Formerly Monkey Pox – in Dermatology: A Review of Epidemiologic Features, Clinical Presentation, Diagnosis, and Treatment / MPOX (antes viruela del mono): revisión de los aspectos clínicos, epidemiológicos, diagnósticos y terapéuticos más relevantes para el dermatólogo

Mpox is an emerging zoonotic disease that has spread rapidly around the world. It has been declared a public health emergency of international concern by the World Health Organization. This review is an update for dermatologists on the epidemiology, clinical presentation, diagnosis, and treatment of Mpox. The primary mode of transmission in the current outbreak is close physical contact during sexual activity. Although most of the initial cases were reported in men who have sex with men, anyone who has close contact with an infected person or contaminated fomites is at risk. Classic prodromal features of Mpox include subclinical manifestations and a mild rash. Complications are common but rarely require hospitalization. Polymerase chain reaction analysis of mucocutaneous lesions is the test of choice for a definitive diagnosis. In the absence of specific treatments, management focuses on symptomatic relief (AU)

MPOX es una enfermedad zoonótica emergente que se ha propagado rápidamente por todo el mundo y que ha sido declarada por la OMS como una emergencia de salud pública de interés internacional. Esta es una revisión no sistemática de los aspectos clínicos, epidemiológicos, diagnósticos y terapéuticos más relevantes para el dermatólogo. En el brote actual, la transmisión durante las relaciones sexuales es la principal forma de contagio como resultado del contacto físico cercano. Si bien los casos iniciales se informaron en hombres que tienen sexo con hombres, cualquier persona en contacto cercano con personas o fómites infectados está en riesgo. El pródromo clásico puede ser subclínico y la erupción puede ser sutil. Las complicaciones son frecuentes, pero el requerimiento de hospitalización es infrecuente. El diagnóstico definitivo se realiza mediante PCR de las lesiones mucocutáneas. Actualmente no existen tratamientos específicos, y el tratamiento sintomático es el pilar terapéutico (AU)

Pain and Late-Onset of Hidradenitis Suppurativa Can Have a Negative Influence on Occupational Status and Educational Level. A Cross-Sectional Study / El dolor y la aparición tardía de la hidrosadenitis supurativa pueden tener una influencia negativa en el estatus ocupacional y el nivel educativo. Estudio transversal

Background and objectives Hidradenitis suppurativa is a chronic and painful condition with negative impact on daily activity. Little information on the impact of disease-specific factors on educational level and occupational status in hidradenitis suppurativa patients has been reported. We sought to identify how disease-specific factors could influence occupational status and educational level in patients with hidradenitis suppurativa. Methods Cross-sectional study of patients with hidradenitis suppurativa seen between September 2017 and September 2018. Disease-specific variables were analyzed to find associations in patients with different educational levels and occupational status. Results Ninety-eight patients were included. Patients with non-university studies had more frequently ≥ 3 affected areas (22.5% [16/73] vs 4.8% [1/22], p = 0.049), a higher number of painful days (8.5 [SD 8.8] vs 4.6 [SD 4.8], p = 0.048) and a higher score on the VAS scale (6.7 [SD 2.8] vs 5.0 [3.3], p = 0.031). Patients from the inactive group had a significantly increased number of painful days (11.2 [SD 10.4] vs 5.7 [SD 6.2], p = 0.004). This group had a greater number of patients with a history of depression (61.3% [19/31] vs 27.4% [17/62], p = 0.002) and a higher mean BMI (32.3 [9.1] vs 28.4 [6.4], p = 0.016). Late disease onset was significantly associated with being “inactive” (26.7% [8/31] vs 6.5% [4/62], p = 0.026). No significant differences between severity scales of hidradenitis suppurativa and educational level or occupational status were found. Limitations: cross-sectional and single center study. Conclusions Pain, ≥3 affected areas, history of depression, higher mean BMI, and late onset of hidradenitis suppurativa, are associated with low education level and inactive occupational status (AU)

Antecedentes y objetivos La hidrosadenitis supurativa es una condición crónica y dolorosa, con impacto negativo en la actividad diaria. Se ha reportado poca información sobre el impacto que tienen los factores específicos de la enfermedad en el nivel educativo y el estatus ocupacional de los pacientes con esta situación. Nuestro objetivo fue identificar el modo en que dichos factores específicos de la enfermedad podrían influir en el nivel educativo y el estatus ocupacional de los pacientes con hidrosadenitis supurativa. Métodos Estudio transversal de pacientes con hidrosadenitis supurativa examinados entre septiembre de 2017 y septiembre de 2018. Se analizaron las variables específicas de la enfermedad para encontrar una asociación en los pacientes con diferentes niveles educativos y estatus ocupacionales. Resultados Se incluyó a 98 pacientes. Aquellos con estudios no universitarios tenían afectadas más frecuentemente ≥ 3 zonas (22,5% [16/73] vs. 4,8% [1/22], p = 0,049), pasaban un elevado número de días con dolor (8,5 [DE 8,8] vs. 4,6 [DE 4,8], p = 0,048) y su puntuación en la escala EVA era alta (6,7 [DE 2,8] vs. 5 [3,3], p = 0,031). Los pacientes pertenecientes al grupo inactivo tenían un número considerablemente incrementado de días con dolor (11,2 [DE 10,4] vs. 5,7 [DE 6,2], p = 0,004). En este grupo había un mayor número de pacientes con historia de depresión (61,3% [19/31] vs. 27,4% [17/62], p = 0,002) y un mayor IMC medio (32,3 [9,1] vs. 28,4 [6,4], p = 0,016). El inicio tardío de la enfermedad se asoció significativamente a la situación de «inactivo» (26,7% [8/31] vs. 6,5% [4/62], p = 0,026). No se encontraron diferencias significativas entre las escalas de gravedad de la hidrosadenitis supurativa y el nivel educativo y el estatus ocupacional. Limitaciones: estudio transversal y unicéntrico (AU)

[Artículo traducido] El dolor y la aparición tardía de la hidrosadenitis supurativa pueden tener una influencia negativa en el estatus ocupacional y el nivel educativo. Estudio transversal / Pain and late-onset of hidradenitis suppurativa can have a negative influence on occupational status and educational level. A cross-sectional study

Antecedentes y objetivos La hidrosadenitis supurativa es una condición crónica y dolorosa, con impacto negativo en la actividad diaria. Se ha reportado poca información sobre el impacto que tienen los factores específicos de la enfermedad en el nivel educativo y el estatus ocupacional de los pacientes con esta situación. Nuestro objetivo fue identificar el modo en que dichos factores específicos de la enfermedad podrían influir en el nivel educativo y el estatus ocupacional de los pacientes con hidrosadenitis supurativa. Métodos Estudio transversal de pacientes con hidrosadenitis supurativa examinados entre septiembre de 2017 y septiembre de 2018. Se analizaron las variables específicas de la enfermedad para encontrar una asociación en los pacientes con diferentes niveles educativos y estatus ocupacionales. Resultados Se incluyó a 98 pacientes. Aquellos con estudios no universitarios tenían afectadas más frecuentemente ≥ 3 zonas (22,5% [16/73] vs. 4,8% [1/22], p = 0,049), pasaban un elevado número de días con dolor (8,5 [DE 8,8] vs. 4,6 [DE 4,8], p = 0,048) y su puntuación en la escala EVA era alta (6,7 [DE 2,8] vs. 5 [3,3], p = 0,031). Los pacientes pertenecientes al grupo inactivo tenían un número considerablemente incrementado de días con dolor (11,2 [DE 10,4] vs. 5,7 [DE 6,2], p = 0,004). En este grupo había un mayor número de pacientes con historia de depresión (61,3% [19/31] vs. 27,4% [17/62], p = 0,002) y un mayor IMC medio (32,3 [9,1] vs. 28,4 [6,4], p = 0,016). El inicio tardío de la enfermedad se asoció significativamente a la situación de «inactivo» (26,7% [8/31] vs. 6,5% [4/62], p = 0,026). No se encontraron diferencias significativas entre las escalas de gravedad de la hidrosadenitis supurativa y el nivel educativo y el estatus ocupacional. Limitaciones: estudio transversal y unicéntrico (AU)

Background and objectives Hidradenitis suppurativa is a chronic and painful condition with negative impact on daily activity. Little information on the impact of disease-specific factors on educational level and occupational status in hidradenitis suppurativa patients has been reported. We sought to identify how disease-specific factors could influence occupational status and educational level in patients with hidradenitis suppurativa. Methods Cross-sectional study of patients with hidradenitis suppurativa seen between September 2017 and September 2018. Disease-specific variables were analyzed to find associations in patients with different educational levels and occupational status. Results Ninety-eight patients were included. Patients with non-university studies had more frequently ≥ 3 affected areas (22.5% [16/73] vs 4.8% [1/22], p = 0.049), a higher number of painful days (8.5 [SD 8.8] vs 4.6 [SD 4.8], p = 0.048) and a higher score on the VAS scale (6.7 [SD 2.8] vs 5.0 [3.3], p = 0.031). Patients from the inactive group had a significantly increased number of painful days (11.2 [SD 10.4] vs 5.7 [SD 6.2], p = 0.004). This group had a greater number of patients with a history of depression (61.3% [19/31] vs 27.4% [17/62], p = 0.002) and a higher mean BMI (32.3 [9.1] vs 28.4 [6.4], p = 0.016). Late disease onset was significantly associated with being “inactive” (26.7% [8/31] vs 6.5% [4/62], p = 0.026). No significant differences between severity scales of hidradenitis suppurativa and educational level or occupational status were found. Limitations: cross-sectional and single center study. Conclusions Pain, ≥3 affected areas, history of depression, higher mean BMI, and late onset of hidradenitis suppurativa, are associated with low education level and inactive occupational status (AU)

Perfluorinated alkyl substances (PFASs) in northern Spain municipal solid waste landfill leachates.

Landfill leachates have been recognized as significant secondary sources of poly- and perfluoroalkyl substances (PFASs). This study presents data on the occurrence and concentration of 11 perfluoroalkyl carboxylates (PFCAs) and 5 perfluoroalkyl sulfonates (PFSAs) in leachates from 4 municipal solid waste landfill sites located across northern Spain. To the best of our knowledge, this is the first report of the presence of PFASs in Spanish landfill leachates. Two of the landfill sites applied on-site treatment using membrane bioreactors (MBR), and its effect on PFASs occurrence is also reported. Total PFASs (∑PFASs) in raw leachates reached 1378.9 ng/L, while in treated samples ∑PFASs was approximately two-fold (3162.3 ng/L). PFCAs accounted for the majority of the detected PFASs and perfluorooctanoic acid (PFOA) was the dominant compound in raw leachates (42.6%), followed by shorter chain PFHxA (30.1%), PFPeA and PFBA. The age of the sites might explain the PFASs pattern found in raw leachates as all of them were stabilized leachates. However, PFASs profile was different in treated samples where the most abundant compound was PFHxA (26.5%), followed by linear perfluorobutane sulfonate (L-PFBS) (18.7%) and PFOA (17.7%). The overall increase of the PFASs content as well as the change in the PFASs profile after the MBR treatment, could be explained by the possible degradation of PFASs precursors such as fluorotelomer alcohols or fluorotelomer sulfonates. Using the volume of leachates generated in the landfill sites, that served 1.8 million people, the discharge of 16 ∑PFASs contained in the landfill leachates was estimated as 1209 g/year.

[Clinical usefulness and psychometric properties of the Cambridge Behavioural Inventory]. / Utilidad clinica y propiedades psicometricas del inventario conductual de Cambridge.

INTRODUCTION: An important area in neuropsychological assessment is that of psychological and behavioural symptoms. The Cambridge Behavioural Inventory (CBI) is a self-report measure aimed at relatives which takes account of a wide range of behavioural symptoms that may occur during the course of neurological diseases. The main objective of the study is to test the clinical usefulness of its Spanish adaptation. SUBJECTS AND METHODS: The CBI was completed by 215 members of kin of patients referred from neurology and psychiatry services. The CBI profiles of four groups of patients were compared, these being grouped according to their main clinical characteristics, psychometric data, imaging tests and the clinical judgement of the professional requesting the neuropsychological study. RESULTS: Most of the scales (10 out of 13) of the CBI yielded acceptable internal consistency values, and the memory and attention/orientation scales showed high correlations with objective measures of memory and time orientation. The CBI profiles of the groups of patients with different conditions (organic memory disorder, functional memory disorder, behavioural variant of frontotemporal dementia and Alzheimer's disease) were consistent with their main features. CONCLUSIONS: The CBI is a psychometrically reliable instrument with adequate convergent and discriminant validity that can be useful in the process of neuropsychological assessment. It can provide relevant information not only about cognitive functioning and the functional capabilities, but also about the behavioural and psychological symptoms of patients with cognitive disorders.

TITLE: Utilidad clinica y propiedades psicometricas del inventario conductual de Cambridge.Introduccion. Un area importante de la evaluacion neuropsicologica son los sintomas psicologicos y conductuales. El inventario conductual de Cambridge ­Cambridge Behavioural Inventory (CBI)­ es una medida de autoinforme dirigida a allegados que recoge una amplia variedad de sintomas conductuales que pueden darse en el curso de las enfermedades neurologicas. El principal objetivo del estudio es comprobar la utilidad clinica de su adaptacion al castellano. Sujetos y metodos. El CBI fue cumplimentado por 215 allegados de pacientes remitidos desde los servicios de neurologia y psiquiatria. Se compararon los perfiles del CBI de cuatro grupos de pacientes formados sobre la base de sus principales caracteristicas clinicas, datos psicometricos, pruebas de imagen y juicio clinico del profesional solicitante del estudio neuropsicologico. Resultados. La mayoria de las escalas (10 de 13) del CBI tuvo valores de consistencia interna aceptables, y las escalas de memoria y atencion/orientacion, correlaciones elevadas con medidas objetivas de memoria y orientacion temporal. Los perfiles del CBI de los grupos de pacientes con distintas condiciones (trastorno organico de la memoria, trastorno funcional de la memoria, variante conductual de demencia frontotemporal y enfermedad de Alzheimer) fueron consistentes con sus principales caracteristicas. Conclusiones. El CBI es un instrumento psicometricamente fiable y con adecuada validez convergente y discriminante que puede ser util en el proceso de evaluacion neuropsicologica, aportando informacion relevante no solo sobre el funcionamiento cognitivo y las capacidades funcionales, sino tambien sobre los sintomas conductuales y psicologicos de los pacientes con trastornos cognitivos.

[A case-control study of risk factors of acute cerebrovascular disease]. / Estudio de casos y controles de factores de riesgo de accidente cerebrovascular agudo.

BACKGROUND: Stroke is the cardiovascular disease which causes the greatest number of deaths in Galicia, mortality, particularly in women, being higher than in the rest of Spain. The aim of this study was to investigate the risk factors of stroke and its importance in Galicia. METHODS: A hospitalary study of cases and controls was performed including 76 patients with stroke and 76 controls individually paired for age, sex, population habitat and date of admission with anamnesis, weight, height and blood pressure, analysis and electrocardiogram being carried out in all. RESULTS: An association was found between the disease and family history of stroke (odds ratio = 3.6, confidence interval 95% = 1.2-13.3), personal history of stroke (17.9; 4.0-79.1), personal history of atrial fibrillation (15.0; 3.3-68.3), high blood pressure (4.5; 1.9-11.6) and ingestion of alcohol greater than or equal to 80 g/day in comparison with abstemious patients adjusted for the effect of high blood pressure (2.5; 1.1-5.7). An association was also observed with serum cholesterol levels greater than or equal to 250 mg/dl (6.46 mmol/l) (3.3; 1.2-8.8, in comparison with cholesterol less than 200 mg/dl). No association was found with the cigarette smoking (1.2; 0.7-2.3). CONCLUSIONS: The results observed for high blood pressure and the ingestion of alcohol regardless of the same are of importance in primary prevention due to being modifiable risk factors. The association with family history of stroke and auricular fibrillation reinforce the needs for primary prevention measures in these subpopulations while having had a stroke or a transitory ischemic attack is the characteristic with the most risk reinforcing the need for secondary prevention measures which have found to be effective. The controls presented abnormally low cholesterol levels that do not reflect those of the population from which they originate, thus existing the possibility that the association observed is spurious.

[Von Willebrand disease: characteristics and response to desmopressin. Study of 103 cases]. / Enfermedad de Von Willebrand: características y respuesta a desmopresina. Estudio de 103 casos.

BACKGROUND: To describe the main characteristics and response to desmopressin infusion in 103 patients suffering from von Willebrand disease (vWD). PATIENTS AND METHODS: The criteria for diagnosis were (except for type 2N) the coexistence of von Willebrand factor ristocetin cofactor (vWF:RCo) activity < 50 U/dl with bleeding disease or one of the following data: von Willebrand factor antigen (vWF:Ag) activity < 50 U/dl, factor VIII (FVIII) activity < 50 U/dl or the existence of a increased bleeding time (BT). Multimeric studies of vWF were performed in 51 cases and ristocetin induced platelet aggregation (RIPA) was also performed. RESULTS: Spontaneous bleeding was found in 36 patients, while in 18 cases the diagnosis was done after surgical bleeding. Thirteen patients (6 presenting with mild bleeding) were studied for abnormalities in the routine preanestesic tests. Other 22 patients were diagnosed with vWD by familial studies. There were 3 patients with type 2B, 1 case with type 2N and other patient with type 3. BT was found increased in 26 out of 58 patients. The activities of vWF:CoR and vWF:Ag were 38.4 (9.4) U/dl and 45.8 (23.2) U/dl, respectively, while the activity of FVIII was 49.9 (20.8) U/dl. Prophylactic DDAVP (desmopressin) was infused in 32 patients. After 1 h, basal activities of vWF:CoR and vWF:Ag were increased by 3.1 (3.2) and 3.4 (3.1) times, respectively, and maintained for 3 h. FVIII activity increased 3.6 (2.3) times the basal levels decreasing after 3 h (2.9 [2.1]; p < 0.01). The BT was corrected in 8 out of ten patients. CONCLUSIONS: vWD is a major cause of surgical bleeding. Preanestesic anamnesis and coagulation tests can be useful to identify vWD. Many patients with vWD have normal BT. A failure in the response to desmopressin infusion is unusual.

Use of Fourier-domain OCT to detect retinal nerve fiber layer degeneration in Parkinson's disease patients.

PURPOSE: To demonstrate axonal loss in the retinal nerve fiber layer (RNFL) of patients with Parkinson's disease (PD) and to evaluate the ability of Fourier-domain optical coherence tomography (OCT) to detect RNFL degeneration and retinal thinning in these patients. METHODS: PD patients (n=100) and healthy subjects (n=100) were included in the study and underwent visual acuity, color vision, and OCT examinations using two next-generation Fourier-domain devices (Spectralis and Cirrus). Differences in the RNFL thicknesses were compared between patients and controls. RESULTS: RNFL thicknesses were significantly reduced in PD patients compared with healthy subjects, especially those obtained using the Spectralis OCT, in the inferotemporal quadrant (155.6±16.5 µm in healthy eyes vs 142.1±24.9 µm in patients, P=0.040) and in the superotemporal quadrant (142.6±20.9 µm in healthy eyes vs 132.77±18.6 µm in PD patients, P=0.046). Significant differences were observed between controls and patients in relation to mean macular thickness (P=0.031), foveal thickness (P=0.030), and inferior outer thickness (P=0.019). CONCLUSION: PD is associated with RNFL loss and retinal thinning, which is detectable by Fourier-domain OCT measurements.

[Birt-Hogg-Dubé syndrome in a patient with cutaneous symptoms and a c.1429 C > T;p.R477X mutation in exon 12 of the folliculin gene]. / Síndrome de Birt-Hogg-Dubé, en un paciente con clínica cutánea y mutación en el exón 12 del gen BHD: c.1429 C > T;p.R477X.

Birt-Hogg-Dubé syndrome is an autosomal dominant genodermatosis characterized by the presence of fibrofolliculomas, renal cancer, pulmonary cysts, and spontaneous pneumothorax. Recently, the folliculin gene responsible for this process has been identified, located on the short arm of chromosome 17. We present the case of a 49-year-old man with multiple whitish papules on the face, neck, and retroauricular area. Histology was compatible with fibrofolliculoma and genetic study showed a pathogenic mutation of the folliculin gene.

Leishmaniasis cutánea. Experiencia de 20 años en un hospital español de tercer nivel / Cutaneous Leishmaniasis: 20 years’ experience in a Spanish tertiary care hospital

INTRODUCCIÓN Y OBJETIVOS: La leishmaniasis es endémica en España, siendo la leishmaniasis cutánea la forma más habitual de presentación. El objetivo del estudio fue valorar las características epidemiológicas y clínicas de la leishmaniasis cutánea en las últimas 2 décadas, haciendo hincapié en las diferencias clínicas según el estado inmunitario del paciente y el origen de la infección. MATERIALES Y MÉTODOS: Se revisaron retrospectivamente 67 historias clínicas de pacientes diagnosticados de leishmaniasis entre 1992 y 2012, de ellas 54 eran cutáneas, y con datos de seguimiento. RESULTADOS: Se incluyeron 54 pacientes: 26 diagnosticados entre 1992-2002 y 28 entre 2003-2012. La edad media fue de 49 años, con un predominio en varones y un tiempo medio de evolución previo a la consulta de 3 meses. Las manifestaciones clínicas más frecuentes fueron placas y úlceras. La mayoría de pacientes inmunodeprimidos y con leishmaniasis importada presentaron lesiones ulceradas y/o múltiples. El diagnóstico se basó en los hallazgos clínico-patológicos en la primera década, asociándose el diagnóstico por métodos moleculares en la segunda. El tratamiento de elección fue los antimoniales pentavalentes, con buenos resultados y escasos efectos adversos. CONCLUSIÓN: El número de casos y de pacientes inmunodeprimidos fueron similares en ambas décadas, diagnosticándose un mayor número de leishmaniasis importada en la segunda. En los pacientes con lesiones múltiples y/o ulceradas deberían descartarse la inmunosupresión del huésped y la infección por cepas importadas. El tratamiento con antimoniato de meglumina fue eficaz y seguro, tanto por vía sistémica como intralesional

INTRODUCTION AND OBJECTIVES: Cutaneous leishmaniasis is the most common form of leishmaniasis, which is endemic in Spain. The aim of this study was to evaluate the epidemiological and clinical characteristics of cutaneous leishmaniasis seen in our hospital over a period of 20 years, with a particular focus on clinical differences according to immune status and origin of infection MATERIALS AND METHODS: We performed a chart review of 67 cases of cutaneous leishmaniasis diagnosed between 1992 and 2012. Follow-up data were available for 54 patients. RESULTS: Fifty-four patients with cutaneous leishmaniasis were included in the study. Of these, 26 had been diagnosed between 1992 and 2002 and 28 between 2003 and 2012. The mean age at diagnosis was 49 years, there was a predominance of male patients, and the mean time from onset of symptoms to consultation was 3 months. The most common clinical manifestations were plaques and ulcers. Most of the immunodepressed patients and patients with imported leishmaniasis had skin ulcers and/or multiple lesions. During the first decade of the study, diagnosis was based on clinical and histologic findings. These were supported by molecular techniques in the second decade. Pentavalent antimonials were the treatment of choice, producing good results and very few adverse effects CONCLUSION: The number of patients with cutaneous leishmaniasis and with compromised immune status was similar in the periods 1992-2002 and 2003-2013, but more cases of imported leishmaniasis were diagnosed in the second period. Patients with ulcers and/or multiple lesions should be evaluated to rule out immunosuppression or infection by Leishmania species from other parts of the world. Both systemic and intralesional meglumine antimonate was effective and safe