A new ring nematode, Xenocriconemella costaricense sp. nov., (Nematoda: Criconematidae) from Costa Rica.

During nematode surveys of natural vegetation in forests of La Cima de Copey de Dota, San José, San José province, Costa Rica, a Xenocriconemella species closely resembling X. macrodora and related species was found. Integrative taxonomical approaches demonstrated that it is a new species described herein as X. costaricense sp. nov. The new species is parthenogenetic (only females have been detected) and characterised by a short body (276-404 µm); lip region with two annuli, not offset, not separated from body contour; first lip annulus partially covering the second lip annulus. Stylet thin, very long (113-133 µm) and flexible, occupying 30.5-47.8% of body length. Excretory pore located from one or two annuli anterior to one or two annuli posterior to level of stylet knobs, at 42 (37-45) µm from anterior end. Female genital tract monodelphic, prodelphic, outstretched, and occupying 35-45% of body length, with vagina slightly ventrally curved (14-18 µm long). Anus located 6-11 annuli from the tail terminus. Tail conoid and bluntly rounded terminus, the last 2-3 annuli oriented dorsally. Results of molecular characterisation and phylogenetic analyses of D2-D3 expansion segments of 28S rRNA, ITS, and partial 18S rRNA, as well as cytochrome oxidase c subunit 1 gene sequences further characterised the new species and clearly separated it from X. macrodora and other related species (X. iberica, X. paraiberica, and X. pradense).

The more we search, the more we find: discovering and expanding the biodiversity in the ring nematode genus Xenocriconemella De Grisse and Loof, 1965 (Nematoda: Criconematidae).

The ring nematode genus Xenocriconemella De Grisse and Loof, 1965 comprises only one nominal species, Xenocriconemella macrodora (Taylor, 1936) De Grisse and Loof, 1965. The initial objective of the present study was to investigate the morphological-morphometric and molecular diversity of 28 X. macrodora populations in the Iberian Peninsula associated with tree forests (mainly Quercus spp.). However, a detailed integrative taxonomic analysis (morphological-morphometric and molecular data) from each population and analysis of this data using principal component analysis (PCA) for morphometric data (including these 28 populations and other 25 X. macrodora populations around the world) and molecular and phylogenetic species delimitation methods revealed that X. macrodora forms a species complex. This species complex is composed by species that are morphometricly and morphologically similar, but clearly different at the molecular level. Three new species are described applying integrative taxonomy, namely as Xenocriconemella iberica sp. nov., Xenocriconemella paraiberica sp. nov. and Xenocriconemella pradense sp. nov. However, the molecular diversity of this species in USA and Italy confirmed that additional species are likely present in this species complex, and the diversity of this group may be higher than expected. The study of X. macrodora topotypes can clarify the position of this species using molecular markers under an integrative approach.

Anatomical and molecular characterization of some rhigonematid parasites of millipedes in Nigeria, with new insights into their phylogeny.

Parasitic nematodes of millipedes from Nigeria are molecularly characterized for the first time. During nematode surveys on live giant African millipedes from several localities in Nigeria, 4 species of rhigonematids were identified by application of integrative taxonomical approaches (morpho-anatomy and molecular markers), including Brumptaemilius sp., Gilsonema gabonensis, Obainia pachnephorus, and Rhigonema disparovis. The results of morphometric and molecular analyses of D2-D3 28S, ITS, partial 18S rRNA, and cytochrome oxidase c subunit 1 (COI) gene sequences further characterized the rhigonematid species, and clearly separated them from other related species. Phylogenetic relationships based on 28S and 18S rRNA genes suggest that genera within Ransomnematoidea (Ransomnema, Heth, Carnoya, Brumptaemilius, Cattiena, Insulanema, Gilsonema) and Rhigonematoidea (Rhigonema, Obainia, Xystrognathus, Trachyglossoides, Ichthyocephaloides) clustered rather closer than could be expected in view of their morphological differences. Phylogenetic relationships based on ITS and COI are congruent with those of other ribosomal genes; however, they are not conclusive due to the scarcity of available sequences of these genes for these genera in NCBI.

A new needle nematode, Longidorus maginicus n. sp. (Nematoda: Longidoridae) from southern Spain.

During nematode surveys in natural vegetation in Sierra Mágina, Jaén province, southern Spain, a Longidorus species closely resembling Longidorus carpetanensis was found, but application of integrative taxonomic approaches clearly demonstrated that it is a new species described herein as Longidorus maginicus n. sp. The new species is amphimictic, characterized by a moderately long body (4.2-5.2 mm); lip region anteriorly flattened, slightly separated from the rest of body by a depression, 9.0-11.0 µm wide and 3.5-6.0 µm high; amphidial fovea not lobed; relatively short odontostyle (61.0-70.5 µm); guiding ring located 23.5-27.0 µm from anterior end; vulva located at 42.0%-51.3% of body length; female tail 39.0-61.0 µm long, conoid, dorsally convex with rounded terminus (c' = 1.3-2.1), with two or three pairs of caudal pores; and males common (1:2 ratio males:females), with moderately long spicules (39.0-48.5 µm) and 1 + 6-9 ventromedian supplements and three juvenile developmental stages. According to the polytomous key, codes for the new species are (codes in parentheses are exceptions): A2-B1-C2-D2-E1-F2(3)-G2-H5(4)-I2-J1-K6. The results of molecular analysis of D2-D3 28S, internal transcribed spacer region, partial 18S rDNA, and cytochrome oxidase c subunit 1 (coxI) gene sequences further characterized the new species status, and separated it from L. carpetanensis and other related species.

Secuestro pulmonar: a propósito de un caso / Pulmonary sequestration: a case report

Resumen Introducción El secuestro pulmonar es un segmento anormal de tejido pulmonar sin comunicación con el árbol traqueobronquial e irrigado por una arteria aberrante de origen sistémico. El secuestro pulmonar puede ser intralobar o extralobar. Caso Clínico Presentamos el caso de un neonato con dificultad respiratoria debido a secuestro pulmonar. El tratamiento quirúrgico consiste en la resección del segmento pulmonar con la ligadura y corte de su arteria. Se describe el manejo realizado y la revisión de la literatura indexada.

Introduction Pulmonary sequestration is an abnormal segment of lung tissue without communication with the tracheobronchial tree and irrigated by an aberrant artery of systemic origin. Can be intralobar or extralobar. Case report We present the case of a neonate with respiratory distress due to pulmonary sequestration. Surgical treatment consists in the resection of the lung segment with the ligature and cut of its artery. Is describe the management carried out and the review of the indexed literature.

Litiasis urinaria y su relación con el consumo de aguas duras en la comuna de Maipú / Urinary lithiasis and its relation to the consumption of hard water in the Maipu Borough

INTRODUCCIÓN: La litiasis urinaria es una patología frecuente, que afecta principalmente a adultos en edad productiva, y que en el último tiempo ha presentado un aumento en la incidencia global. Cerca del 80 % de los cálculos se componen mayoritariamente de calcio. Existe controversia en la literatura sobre el rol que podría tener el consumo de aguas con altos índices de dureza y el riesgo de padecer litiasis. El objetivo de este estudio es identificar una posible asociación entre dureza de agua para consumos e incidencia de litiasis urinaria en la poblacion de Maipú. MATERIALES Y METODOS: Se incluyen en el estudio todos los pacientes atendidos por urólogos en forma ambulatoria electiva en el Hospital El Carmen de Maipú, por diagnóstico de litiasis urinaria, en los años 2015 y 2016, que residen en la comuna. Se georreferenciaron de acuerdo a sus direcciones en los distintos sectores de distribución de agua potable.Se realizó análisis fisicoquímico de muestras de agua extraídas de cada sector para determinar su dureza. Se estudiaron a través del índice de correlación de Pearson las posibles asociaciones entre dureza del agua e incidencia de litiasis. RESULTADOS: La dureza promedio del agua de Maipú es de 484 mg/L de CaCO3, valor que se encuentra por sobre el promedio regional y nacional. La incidencia anual de litiasis en la poblacion estudiada fue de 4,4 casos/10.000 habitantes, valor similar al promedio nacional. El índice de correlación de Pearson entre dureza de agua e incidencia anual fue de -0,51. CONCLUSIONES: No fue posible establecer una relación causal entre dureza de agua e incidencia de litiasis en la población estudiada. Se sugiere avanzar en esta misma línea con estudios futuros de casos y controles.AU

INTRODUCTION: Urinary lithiasis is a frequent pathology, which mainly affects adults in a productive age, and which-in recent times- has increased in terms of global incidence. About 80 % of the stones are composed mainly of calcium.There is controversy in literature addressing the possible relation amongst water consumption with high hardnessindexes and the risk of lithiasis. The objective of this study is to identify a possible association between water hardnessfor consumption and urinary lithiasis incidence in the Maipu population. MATERIALS AND METHODS: All patients assisted by urologists on an elective ambulatory basis, at the El Carmen Hospital in Maipú, diagnosed with urinary lithiasis, in the years 2015 and 2016, and who reside in the Borough have been included in the study. They were georeferenced according to their address in the different drinking water distribution areas. Physiochemical analysis of water samples extracted from each sector was carried out to determine its hardness. The possible associations between hardness of water and incidence of lithiasis were studied through the Pearson correlation index. RESULTS: The average water hardness in Maipu is 484 mg / L of CaCO3, a value that is above the regional and national average. The annual incidence of lithiasis in the studied population was 4.4 cases / 10,000 inhabitants, a value that is similar to the national average. The Pearson correlation index between water hardness and annual incidence was -0.51. CONCLUSIONS: It was not possible to establish a causal relation between water hardness and incidence of lithiasis in the studied population. It is suggested to advance in this same line with future case and control studies.

The utility of mtDNA and rDNA for barcoding and phylogeny of plant-parasitic nematodes from Longidoridae (Nematoda, Enoplea).

The traditional identification of plant-parasitic nematode species by morphology and morphometric studies is very difficult because of high morphological variability that can lead to considerable overlap of many characteristics and their ambiguous interpretation. For this reason, it is essential to implement approaches to ensure accurate species identification. DNA barcoding aids in identification and advances species discovery. This study sought to unravel the use of the mitochondrial marker cytochrome c oxidase subunit 1 (coxI) as barcode for Longidoridae species identification, and as a phylogenetic marker. The results showed that mitochondrial and ribosomal markers could be used as barcoding markers, except for some species from the Xiphinema americanum group. The ITS1 region showed a promising role in barcoding for species identification because of the clear molecular variability among species. Some species presented important molecular variability in coxI. The analysis of the newly provided sequences and the sequences deposited in GenBank showed plausible misidentifications, and the use of voucher species and topotype specimens is a priority for this group of nematodes. The use of coxI and D2 and D3 expansion segments of the 28S rRNA gene did not clarify the phylogeny at the genus level.

Mitochondrial genome diversity in dagger and needle nematodes (Nematoda: Longidoridae).

Dagger and needle nematodes included in the family Longidoridae (viz. Longidorus, Paralongidorus, and Xiphinema) are highly polyphagous plant-parasitic nematodes in wild and cultivated plants and some of them are plant-virus vectors (nepovirus). The mitochondrial (mt) genomes of the dagger and needle nematodes, Xiphinema rivesi, Xiphinema pachtaicum, Longidorus vineacola and Paralongidorus litoralis were sequenced in this study. The four circular mt genomes have an estimated size of 12.6, 12.5, 13.5 and 12.7 kb, respectively. Up to date, the mt genome of X. pachtaicum is the smallest genome found in Nematoda. The four mt genomes contain 12 protein-coding genes (viz. cox1-3, nad1-6, nad4L, atp6 and cob) and two ribosomal RNA genes (rrnL and rrnS), but the atp8 gene was not detected. These mt genomes showed a gene arrangement very different within the Longidoridae species sequenced, with the exception of very closely related species (X. americanum and X. rivesi). The sizes of non-coding regions in the Longidoridae nematodes were very small and were present in a few places in the mt genome. Phylogenetic analysis of all coding genes showed a closer relationship between Longidorus and Paralongidorus and different phylogenetic possibilities for the three Xiphinema species.

Tratamiento del asma severo con omalizumab: experiencia pediátrica en Chile y revisión de la literatura / Omalizumab treatment on severe asthma patients: pediatric experience in Chile and literature review

This paper presents clinical experience with Omalizumab treatment in 8 pediatric patients in Chile. All children presented difficult to control asthma despite receiving high intensity treatment, with low quality of life. All patients were studied in order to discard errors in asthma diagnosis and to evaluate asthma treatment adherence and inhalation technique. After evaluation, patients proven to have severe therapy resistant asthma were indicated treatment with Omalizumab. Significant clinical improvement was observed, with reduced asthma symptoms and number of exacerbations, as well as an improved quality of life. Omalizumab showed a good safety profile with mild and transient adverse reactions in 6 administrations of a total of 122.

Se presenta la experiencia clínica con el uso de Omalizumab en 8 pacientes pediátricos en nuestro país. Todos los pacientes presentaban asma sin control a pesar de recibir terapia de alta intensidad, asociado a una muy deficiente calidad de vida. La totalidad de los pacientes fueron sometidos en cada centro a un estudio exhaustivo para poder descartar error en el diagnóstico y se evaluó la adherencia y la técnica inhalada. Al comprobarse que estos pacientes tenían asma severo resistente a tratamiento se indicó Omalizumab, el cual produjo una mejoría clínica significativa. Se observó una reducción de las exacerbaciones y de los síntomas de asma acompañado de una mejoría de la calidad de vida, asociado a un buen perfil de seguridad. Se observaron reacciones adversas leves y transitorias en 6 administraciones de un total de 122.

Biological characterization of PM226, a chromenoisoxazole, as a selective CB2 receptor agonist with neuroprotective profile.

Cannabinoids have emerged as promising neuroprotective agents due to their capability to activate specific targets, which are involved in the control of neuronal homeostasis and survival. Specifically, those ligands that selectively target and activate the CB2 receptor may be useful for their anti-inflammatory and neuroprotective properties in various neurological disorders, with the advantage of being devoid of psychotropic effects associated with the activation of CB1 receptors. The aim of this work has been to investigate the neuroprotective properties of 7-(1,1-dimethylheptyl)-4,4-dimethyl-9-methoxychromeno[3,4-d]isoxazole (PM226), a compound derived from a series of chromeno-isoxazoles and -pyrazoles, which seems to have a promising profile related to the CB2 receptor. The compound binds selectively to this receptor with an affinity in the nanomolar range (Ki=12.8±2.4nM). It has negligible affinity for the CB1 receptor (Ki>40000nM) and no activity at the GPR55. PM226 was also evaluated in GTPγS binding assays specific to the CB2 receptor showing agonist activity (EC50=38.67±6.70nM). In silico analysis of PM226 indicated that it has a good pharmacokinetic profile and a predicted ability to cross the blood-brain barrier. Next, PM226 was investigated in an in vitro model to explore its anti-inflammatory/neuroprotective properties. Conditioned media were collected from LPS-stimulated cultures of BV2 microglial cell line in the absence or presence of different doses of PM226, and then media were added to cultured M213-2O neuronal cells to record their influence on cell viability evaluated using MTT assays. As expected, cell viability was significantly reduced by the exposure to these conditioned media, while the addition of PM226 attenuated this reduction in a dose-dependent manner. This effect was reversed by co-incubating with the CB2 antagonist SR144528, thus confirming the involvement of CB2 receptors, whereas the addition of PM226 to neuronal cultures instead cultured BV2 cells was not effective. PM226 has also been studied in an in vivo model of mitochondrial damage generated by intrastriatal application of malonate in rats. MRI analysis showed that PM226 administration decreased the volume of the striatal lesion caused by malonate, effect that was confirmed after the histopathological evaluation (Nissl staining, Iba-1 immunostaining and TUNEL assay) of striatal sections derived from malonate-lesioned rats in the absence or presence of PM226. Again, the beneficial effects of PM226 were dependent on the activation of CB2 receptors as they were reversed by blocking these receptors with AM630. Overall, PM226 has shown to have a promising neuroprotective profile derived from its ability to selectively activate CB2 receptor, so that it could be a useful disease-modifying agent in those neurodegenerative pathologies in which the activation of these receptors may have therapeutic value.

Consideration of noninherited maternal Ags as permissible HLA mismatches in cord blood donor selection.

In cord blood (CB) transplantation, virtual 6/6 HLA matches, whereby the donor-recipient mismatch is identical to the CB noninherited maternal Ag (NIMA), have similar outcomes to inherited 6/6 matches. In the UK-British Bone Marrow Registry (BBMR), 4707 of the total 21 020 CB donors have the NIMA defined. Retrospective searches of these donors, for 1-3 NIMA matches, identified a virtual 6/6 match for 31.4% of 274 European Caucasoid (EC) and 25.4% of 67 other ethnicity (OE) patients. Patients weighing ⩽50 kg were also evaluated for a single graft with adequate cell dose. In 125 EC patients, 6/6 HLA matches were identified for 24.0% and virtual 6/6 matches were identified for a further 21.6%. The remaining EC patients had a 5/6 (30.4%) or a 4/6 (22.4%) match. In OE patients, 6/6 HLA matches were identified for 9.3% and virtual 6/6 matches were identified for a further 18.7%. The remaining OE patients had a 5/6 (30.2%) or a 4/6 (37.2%) match. Searches were also performed using the 26 735 Bone Marrow Donors Worldwide CB with defined NIMA and yielded comparable increases. Considering NIMA as permissible mismatches in donor selection therefore increased the availability of a 6/6 match in this cohort.

A new stem nematode, Ditylenchus oncogenus n. sp. (Nematoda: Tylenchida), parasitizing sowthistle from Adriatic coast dunes in southern Italy.

Morphological and molecular analyses of a stem nematode causing a severe disease on infected sowthistle (Sonchus bulbosus) plants, involving the formation of gall-like structures on infected leaves and stems, have led to the description of a new species named Ditylenchus oncogenus n. sp. Morphologically, the new species is characterized by a medium to large body size (all adults more than 1 mm in length); a delicate stylet (9.0-11.0 µm long) with minute, rounded knobs; a long post-vulval uterine sac (c. 65% of the vulva-anus distance); six incisures at the lateral fields and characteristic D. destructor-pattern of spicules (with pronounced ventral tumulus and anteriorly pointed, less sclerotized, cuticle parts present within the lamina). The results of molecular analysis of rRNA gene sequences, including the D2-D3 expansion regions of 28S rRNA, internal transcribed spacer (ITS) rRNA, partial 18S rRNA gene, the protein-coding mitochondrial gene, cytochrome oxidase c subunit I (COI), and the heat-shock protein 90 (hsp90) gene, support the new species status. The results of a host-suitability test indicated that the new species does not parasitize potato (Solanum tuberosum) tubers and broad bean (Vicia faba) seedlings. Histopathological observations on naturally infected sowthistle tissues revealed that D. oncogenus n. sp. causes floral stem neoplasia and midrib leaf gall formation on the type, and to date only known, host. The galls were characterized by extensive hyperplasia, where several necrotic cells in the neoplasic area were directly damaged by feeding of the nematode, whereas a number of adjacent cells showed typical cytological changes, such as granulated cytoplasm with hypertrophied nuclei and nucleoli.

Caracterización epidemiológica del tipo de urgencias urológicas en el hospital barros luco trudeau (HBLT) período 2008-2012 / Epidemiological characterization of types of urological emergencies at barros luco trudeau hospital, analysis of the 2008-2012 period

INTRODUCCIÓN: En la mayoría de servicios de urgencia estatales no cuentan con un Urólogo de llamado, por lo que la mayoría de los pacientes que acuden al Servicio de Urgencia (SU) son evaluados por cirujanos generales. MATERIALES Y MÉTODO: Estudio observacional retrospectivo de corte transversal de la totalidad de ingresos al SU de nuestro centro entre enero de 2008 y diciembre de 2012, de los cuales se analizaron los pacientes con diagnósticos urológicos. Las variables estudiadas fueron: edad, sexo, diagnóstico determinado según la CIE-10 y si se realizó cirugía de urgencia. RESULTADOS: Media de edad: 48,5 años (D.E.:27,07), Sexo: Masculino 74 por ciento, Media de Consultas totales en SU: 142.138pacientes anuales, Media de consultas Urológicas en SU: 4.837 pacientes anuales, correspondientes al 3,4 por ciento del total. Las consultas más frecuentes fueron: ITU baja: 33,5 por ciento, Cólico Renal: 33,1 por ciento , ITU alta 15,6 por ciento, de los cuales un 0,6 por ciento correspondena Pionefrosis, Patología Genital Infecciosa: 4,7 por ciento, de los cuales un 76 por ciento, corresponden a Orquiepididimitis, Retención Aguda de Orina: 3,7 por ciento, Hematuria Persistente: 2,4 por ciento,, Trauma Urogenital: 1,86 por ciento,, Urgencias Genitales: 0,37 por ciento,. Pacientes que requirieron Cirugía de Urgencia: 1.02 por ciento, CONCLUSIONES: Si bien, el porcentaje de pacientes a los que se realizó una intervención quirúrgica de urgencia es bajo,sería importante implementar en los servicios de urgencia estatales Urólogos de llamado, ya que patologías altamente prevalentes, como la litiásica, podrían tener una mejor resolución. (AU)

Most of the public emergency services do not count with urologist and these patients are evaluated by general surgeons. MATERIALS AND METHODS: cross-section, retrospective study of all admissions in Emergency Services (ES) at our center between January 2008 and December 2012, of patients with urological diagnoses were analyzed. The variables studied were: age, sex, diagnosis determined according to the International Classification Diseases, 10th version and if emergency surgery was performed. RESULTS: Average age: 48.5 years (SD: 27.07), Gender: Male 74 pertcent Average Total consultations in ES: 142,138 patients annually, Media of urological evaluations at ES: 4,837 patients annually, corresponding to 3.4 pertcent of the total. The most frequent diagnosis were: low UTI: 33.5 pertcent,Renal pain: 33.1 pertcent, 15.6 pertcent high UTI, of which 0.6 pertcent are to Pyonephrosis, Infectious Genital Pathology 4.7 pertcent, of which 76 pertcent are Epydidimo- Orchitis, Acute Urinary Retention: 3.7 pertcent, Persistent Hematuria 2.4 pertcent, Genitourinary Trauma: 1.86 pertcent Genital Emergencies: 0.37 pertcent. Patients requiring urgent surgery: 1.02 pertcent CONCLUSIONS: While the percentage of patients who performed an emergency surgical intervention is low, it would be important to implement Urologists in public Emergency Services as highly prevalent diseases such as urolithiasis, could have a better resolution.(AU)

Transitional B lymphocytes are associated with protection from kidney allograft rejection: a prospective study.

Recent cross-sectional studies suggest an important role for transitional B lymphocytes (CD19 + CD24hiCD38hi) in promoting transplant tolerance, and protecting from late antibody-mediated rejection (ABMR). However, prospective studies are lacking. This study enrolled 73 de novo transplant recipients, and collected serial clinical, immunological and biochemical information over 48 ± 6 months. Cell phenotyping was conducted immediately prior to transplantation, and then on five occasions during the first year posttransplantation. When modeled as a time-dependent covariate, transitional B cell frequencies (but not total B cells or "regulatory" T cells) were associated with protection from acute rejection (any Banff grade; HR: 0.60; 95% CI: 0.37-0.95; p = 0.03). No association between transitional B cell proportions and either de novo donor-specific or nondonor-specific antibody (dnDSA; dnNDSA) formation was evident, although preserved transitional B cell proportions were associated with reduced rejection rates in those patients developing dnDSA. Three episodes of ABMR occurred, all in the context of nonadherence, and all associated with in vitro anti-HLA T cell responses in an ELISPOT assay (p = 0.008 versus antibody-positive patients not experiencing ABMR). This prospective study supports the potential relevance of transitional ("regulatory") B cells as a biomarker and therapeutic intervention in transplantation, and highlights relationships between humoral immunity, cellular immunity and nonadherence.

Porfiria cutánea tarda: revisión de la literatura a propósito de un caso / Porphyria cutanea tarda: a case report and literature review

La porfiria cutánea tarda (PCT) es el tipo más frecuente de porfiria, conjunto de enfermedades metabólicas, sistémicas, caracterizadas por una alteración en la síntesis del grupo hemo. La PCT se considera parte de las fotodermatosis, y puede subdividirse en variedad familiar o esporádica. El principal factor precipitante es el consumo excesivo de alcohol, y se manifiesta clínicamente como fragilidad cutánea con formación de vesículas en zonas fotoexpuestas, principalmente dorso de manos y cara, con prurito ocasional en zonas afectadas. En algunos casos se puede ver hipertricosis facial e hiperpigmentación. No presenta síntomas sistémicos. La cuantificación de porfirinas en orina confirma el diagnóstico. Se puede tratar con medidas generales, sangrías y/o antimaláricos en dosis bajas, con buena respuesta. Puede recurrir después de meses o años, por lo que requiere control de por vida. Se presenta el reporte de un caso con características clínicas clásicas y revisión bibliográfica actualizada de esta entidad.

Porphyria cutanea tarda (PCT) is the most common type of porphyria, a group of systemic metabolic diseases, characterized by defects in the heme production. PCT is considered amongst the photodermatoses, and may be classified as familial or sporadic. The main precipitating factor is excessive alcohol consumption, and it presents clinically with skin fragility and vesicle formation on photoexposed areas, mainly the back of the hands and face. Some patients may show hypertrichosis, hyperpigmentation, and pruritus. There are no systemic symptoms associated with this condition. Quantification of porphyrins in urine confirms the diagnosis. Treatment options include general measures, blood letting, and/or low dose antimalarials, usually with good results. This disease may recur months or years after treatment, determining the need for lifelong follow-up. We present a case with a classical clinical presentation, along with an updated review of the literature.

Síndrome de PHACES / PHACES syndrome

El síndrome de PHACE consiste en una entidad neurocutánea, cuyo nombre deriva del acrónimo en inglés para la constelación de alteraciones asociadas a algunos hemangiomas segmentarios de localización cefálica-cervical: Malformaciones de fosa Posterior, hemangiomas, anormalidades arteriales, coartación de la aorta y defectos cardíacos, anomalías oculares. Cuando se encuentran defectos de la línea media (Sternal defects) se denomina síndrome de PHACES. Su etiología es desconocida y se presenta con mayor frecuencia en mujeres. Requiere de un estudio exhaustivo de malformaciones asociadas, dado que pueden ser causa importante de morbimortalidad. Presentamos nuestra experiencia en dos casos clínicos, con excelente respuesta a tratamiento con propranolol, junto con una revisión de la literatura.

PHACE syndrome is a neurocutaneous entity, derived from an acronym that describes the constellation of alterations associated with some segmental hemangiomas in the cephaliccervical region: Posterior fossa malformations, hemangiomas, arterial anomalies, aorta coarctation, cardiac defects, and eye abnormalities. When midline anomalies are found (Sternal defects) the term PHACES syndrome is used. Its etiology is unknown, and is more frequent in females. It requires a thorough study of associated malformation, because is an important source or morbimortality. We present our experience with two cases, with excelent response to treatment with propranolol, along with a review of the literature.