Search

There is growing evidence to support new modes of transmission for human monkeypox infection. As these methods are being explored, this report delineates the day-to-day clinical sequelae following the initial exposure in an HIV-positive man who had sexual intercourse with another man days preceding his infection. We describe atypical cutaneous manifestations involving widespread erythematous pustules with preceding anogenital ulcerations and concomitant bilateral inguinal lymphadenopathy. Clinicopathologic correlation is used to assist in the workup and establishing the diagnosis. Our case supports others reported in the literature that suggest sexual contact as a means of transmission. More research is needed that investigates the presence of infection in both men and women, including those who could act as carriers, to elucidate other pathways in this evolving yet evasive viral disease.

Syphilis is a sexually transmitted infection with manifestations that can mimic other diseases, leading to misdiagnosis. Annular syphilis is a rare atypical secondary syphilitic lesion that seldomly involves the face. Human immunodeficiency virus (HIV) coinfection can increasingly lead to atypical manifestations of syphilis and complicate the diagnosis. Herein, we describe a 29-year-old man with a diagnosis of annular secondary syphilis and HIV coinfection. He had clinical manifestations of annular erythematous papules and plaques with white scales at the edges, distributed and scattered on the face, neck, and upper back. The skin lesions went undetected on several visits and were misdiagnosed owing to similarities with other dermatoses. Serological examination showed positive Treponema pallidum particle agglutination assay and venereal disease research laboratory test titer 1/512, confirming syphilis infection. Results from the histopathological examination supported the diagnosis of secondary syphilis. Positive anti-HIV rapid test results indicated concurrent HIV infection. He was treated for syphilis and given antiretroviral therapy, and showed a good response as demonstrated by improvement of the lesions and serological titers. This case highlights the importance of recognizing the possibility of annular secondary syphilis and HIV coinfection which can have atypical manifestations.

La ricerca sul tema delle prove atipiche nasce dall’esigenza di confrontarsi con le distorsioni applicative del dettato codicistico, in particolar modo dell’art. 189 c.p.p. Tale disposizione traccia una cornice legislativa dai requisiti stringenti entro i quali ammette l’assunzione di prove non disciplinate dalla legge. Nel tentativo di offrire un’ordinata ricostruzione del tema oggetto di ricerca, si è dedicato, nel primo capitolo della tesi, ampio spazio alla genesi dell’art. 189 c.p.p., codificato all’esito di un intenso dibattito circa la scelta alternativa tra il principio di libertà e il principio di tassatività delle prove penali. A tale scopo, si è provveduto a una ricostruzione storica dei sistemi probatori del passato che hanno influenzato l’evoluzione normativa fino ai giorni nostri. In particolare, si è analizzato il sistema delle prove legali per evidenziare le criticità che hanno portato al sorgere del principio dell’intimo convincimento, evolutosi, poi, in libero convincimento, al quale presto si è ancorata anche una libertà nella fase assuntiva e acquisitiva delle prove, confondendo così il piano valutativo con altri segmenti del procedimento probatorio. Tale ricostruzione ha permesso di mettere in luce come, nei primi codici dell’Italia unita, sia stata compiuta un’indebita sovrapposizione fra libero convincimento e libertà probatoria, tanto da assegnare al giudice un potere illimitato di ricerca e di assunzione delle prove, con il correlato sacrifico delle garanzie processuali e, in particolar modo, di un effettivo diritto di difesa. Si è dedicato, poi, ampio spazio ai lavori preparatori del codice vigente ove si contrapponevano le posizioni di chi reputava necessaria la chiusura del catalogo probatorio a quelle di chi riteneva essenziale ammettere un’apertura per poter recepire eventuali novità scientifiche o tecniche. Il legislatore ha optato per una scelta intermedia fra libertà e tassatività, prevedendo un’attenta disciplina per l, The research on the topic of atypical evidence stems from the need to confront the applicative distortions of the code's dictate, especially of Article 189 c.p.p. This Article outlines a legislative framework with stringent requirements within which it admits the taking of evidence not regulated by law. In an attempt to offer an orderly reconstruction of the subject under research, ample space was devoted in the first chapter of the thesis to the genesis of Article 189 c.p.p. To this end, a historical reconstruction of past evidentiary systems that have influenced the normative evolution up to the present day was undertaken. In particular, the system of legal evidence was analyzed in order to highlight the critical issues that led to the emergence of the principle of intimate belief, which evolved, later, into free belief, to which freedom in the assumptive and acquisitive phase of evidence was also soon anchored, thus confusing the evaluative plane with other segments of the evidentiary process. This reconstruction made it possible to highlight how, in the first codes of united Italy, an undue overlap was made between free conviction and evidentiary freedom, so much so as to assign to the judge unlimited power to search for and take evidence, with the correlated sacrifice of procedural guarantees and, in particular, of an effective right of defense. Considerable space was then devoted to the preparatory work of the current code where the positions of those who considered it necessary to close the evidentiary catalog were contrasted with those who considered it essential to admit an opening in order to be able to incorporate any scientific or technical novelties. The legislature opted for an intermediate choice between freedom and taxativity, providing careful discipline for typical evidence, but admitting the possibility of taking atypical evidence as well, in a context marked by the "culture of the legality of evidentiary law." In the second chapter, space was dev

L’obiettivo della presente rassegna narrativa è duplice: esaminare lo sviluppo della comprensione sociale nel periodo 0-6 anni considerando congiuntamente i costrutti di teoria della mente (theory of mind, ToM) e comprensione delle emozioni (emotion understanding, EU), non di rado esaminati separatamente; approfondire il ruolo del linguaggio come correlato e predittore di comprensione sociale per entrambi i costrutti. Il lavoro si conclude con l’individuazione di due particolari aree per la ricerca futura, collegate agli argomenti trattati nella rassegna: lo sviluppo della comprensione sociale implicita, e la validazione di interventi per la promozione della comprensione sociale in soggetti con profilo tipico o atipico., The aim of this narrative review was to shed further light on children's development of social cognition in the 0-6 years period, in relation to both theory of mind and emotion understanding, and with a particular focus on language as crucial developmental factor. Two areas of interest were flagged for future research: social cognition in infancy and the implementation of validated intervention programs.

En la actualidad, no existe una indicación formal de profilaxis anticomicial en neurocirugía oncológica. Tampoco existen recomendaciones específicas sobre el uso de fármacos antiepilépticos (FAE) en pacientes portadores de meningiomas y libres de crisis que van a ser intervenidos. En general, se prescriben FAE de forma discrecional, teniendo en cuenta diversos factores de riesgo clínico-radiológicos. Presentamos una revisión sistemática y metaanálisis sobre la efectividad de la profilaxis anticomicial en meningiomas sin historia previa de crisis., No formal indication currently exists for seizure prophylaxis in neurosurgical oncology patients. Neither have specific recommendations been made on the use of antiepileptic drugs (AED) in seizure-free patients with meningiomas scheduled for surgery. AEDs are generally prescribed on a discretionary basis, taking into consideration a range of clinical and radiological risk factors. We present a systematic review and meta-analysis exploring the effectiveness of antiepileptic prophylaxis in patients with meningioma and no history of seizures.

Introduction: Functional data analysis has many applications and is very useful in different fields such as economics, health sciences, and the environment. It provides, in conjunction with other statistical methods, information to predict events, whether natural, social or health. Objective: Adjust functional regression models with functional response applied to the meteorological variables, Temperature, Humidity, Pressure and Radiation. Methods: Descriptively analyze the meteorological variables; adjust the functional regression models of the Radiation variable depending on the variables temperature, humidity and pressure; to evaluate the adjustment of the functional regression models in the meteorological variables. Results: The graphs of each functional meteorological variable were obtained, an analysis of atypical functional data for each variable, the smoothing of the curves that represent the meteorological variables using Fourier bases. The residual analysis of the linear models with functional response of the radiation variable explained by the variables temperature and pressure resulted in less variation with temperature than with pressure. Conclusions:The functional regression models with functional response of the radiation variable depending on the pressure variable presented greater variation in the analysis of their residues with respect to the radiation model depending on the temperature variable., Introducción: El análisis de datos funcionales tiene muchas aplicaciones y es muy útil en diferentes campos como la economía, ciencias de la salud, medio ambiente. Proporciona, en conjunto con otros métodos estadísticos, información para predecir eventos, ya sean naturales, sociales o de salud. Objetivo: Ajustar modelos de regresión funcional con respuesta funcional aplicados a las variables meteorológicas, Temperatura, Humedad, Presión y Radiación. Métodos: Analizar de forma descriptiva las variables meteorológicas; ajustar los modelos de regresión funcional de la variable Radiación en dependencia de las variables temperatura, humedad y presión; evaluar el ajuste de los modelos de regresión funcional en las variables meteorológicas. Resultados: Se obtuvieron las gráficas de cada variable meteorológica, un análisis de datos atípicos funcional, el suavizado de las curvas que representan las variables meteorológicas mediante Fourier. El análisis residual de los modelos lineales con respuesta funcional de la  radiación explicada mediante las variables, temperatura y presión dieron como resultado menor variación con la temperatura, que con la presión.  Conclusiones: Los modelos de regresión funcional con respuesta funcional de la variable radiación en dependencia de la variable presión presento mayor variación en el análisis de sus residuos con respecto al modelo de radiación en dependencia de  temperatura.

Atypical chronic lymphocytic leukemia (CLL) is still defined according to morphological criteria. However, deviance from the typical surface immunological profile suggests an atypical immunological-based CLL. A large cohort of patients with CLL was retrospectively evaluated aiming at assessing morphological (FAB criteria), immunophenotypical (two or more discordances from the typical profile), and clinical–biological features of atypical CLL. Compared to typical cases, morphologically atypical CLL showed a greater percentage of unmutated IgVH and CD38 positivity, and a higher expression of CD20. Immunophenotypically atypical CLL was characterized by more advanced clinical stages, higher expression of CD20, higher rate of FMC7, CD79b and CD49d positivity, and by an intermediate–high expression of membrane surface immunoglobulin, compared to typical cases. When patients were categorized based on immunophenotypic and morphologic concordance or discordance, no difference emerged. Finally, morphological features better discriminated patients’ prognosis in terms of time-to-first treatment, while concordant atypical cases showed overall a worse prognosis. Discordant cases by immunophenotype and/or morphology did not identify specific prognostic groups. Whether—in the era of molecular markers used as prognostic indicators—it does make sense to focus on morphology and immunophenotype features in CLL is still matter of debate needing further research.

The purpose of this essay is to examine the way the flouting of the Gricean maxims is used to portray a fictional character with Autism Spectrum Disorder (ASD). This study analyzes a character named Sam Gardner from the television series Atypical. The study is based on written transcripts from six episodes of the TV series and was carried out qualitatively and quantitatively. The methodology of the study involved transcribing, counting, and analyzing the flouts used by Sam in these six episodes. The results suggest that Sam flouts all the maxims but flouts the maxim of relation most, with a total of 22 flouts, which can cause a problem in communication. Sam does not follow the maxim of relation as he tends to abruptly change the conversational topic to something else. In many cases, he changes the topic to something he is interested in, which is Antarctica and penguins. The second most dominant maxim in all six episodes to be flouted was the maxim of quantity, with a total of 15 flouts, as he gave either too much or too little information to the listener. The results of Sam’s limited pragmatic abilities fall into agreement with Fein’s (2010) claim that ASD individuals have pragmatic language deficits as they struggle to stay on topic and incorporate irrelevant details in conversations. This study further discusses how analyzing neurodivergent characters’ speech patterns can benefit teachers and students in a pedagogical setting.

Se presenta el caso de un paciente masculino de 26 años con un cuadro de dolor abdominal, ausencia de deposiciones, intolerancia a la vía oral y picos febriles. Dado lo peculiar del cuadro clínico de ingreso, fue mal enfocado en los servicios de urgencias, siendo diagnosticado erróneamente como constipación e infección de vías urinarias. Esto culminó en una resolución quirúrgica de su cuadro herniario atípico., We present the case of a 26-year-old male patient, with symptoms of abdominal pain, absence of bowel movements, intolerance to the oral route and feverish spikes, who, given the bizarre clinical picture on admission, was poorly addressed in the emergency services as constipation and urinary tract infection, ending in surgical resolution of his atypical hernia picture.

At the start of this thesis there were several unanswered questions with regard to optimal patient selection for different treatments of bronchial carcinoid. Also, there were unanswered questions with regard to classification of bronchial carcinoid on small histological samples and with regard to prognostic value of several new tissue biomarkers. The aim of this thesis was to further optimize patient selection for parenchyma sparing techniques, and to explore the potential value of biomarkers in the treatment of bronchial carcinoid. Part one (chapter 2-5) of this thesis describes the classification of bronchial carcinoid on small histological samples and the use of diagnostic and prognostic biomarkers. In Chapter 2 we analyze patient selection for endobronchial treatment and underscore the importance of radiological assessment of bronchial carcinoids. Differentiation between typical and atypical carcinoid according to the current WHO classification is accomplished by obtaining the mitotic count and the presence of necrosis. As outlined above, TC is defined as a neuroendocrine tumour with less than 2 mitoses per 2 mm2 and absence of necrosis, while AC is defined by 2-10 mitoses per 2 mm2 and/or the presence of (dot-like) necrosis. However, it is known that small biopsies have limited diagnostic value for the distinction between TC and AC. In Chapter 3 and Chapter 4 we analyzed biopsy-resection paired specimens of patients referred for treatment for bronchial carcinoid to measure accuracy of pretreatment biopsies and analyze the value of tumor biopsy size. Furthermore, we describe the reliability of different immunohistochemical markers on different biopsies and resection specimens, and assessed the impact of specimen size on the accuracy of the diagnosis. Because the current classification of TC and AC only partially reflects their metastatic potential, there is a need for more accurate prognostic biomarkers for bronchial carcinoids. In Chapter 5 we investigated a co

The purpose of this essay is to examine the way the flouting of the Gricean maxims is used to portray a fictional character with Autism Spectrum Disorder (ASD). This study analyzes a character named Sam Gardner from the television series Atypical. The study is based on written transcripts from six episodes of the TV series and was carried out qualitatively and quantitatively. The methodology of the study involved transcribing, counting, and analyzing the flouts used by Sam in these six episodes. The results suggest that Sam flouts all the maxims but flouts the maxim of relation most, with a total of 22 flouts, which can cause a problem in communication. Sam does not follow the maxim of relation as he tends to abruptly change the conversational topic to something else. In many cases, he changes the topic to something he is interested in, which is Antarctica and penguins. The second most dominant maxim in all six episodes to be flouted was the maxim of quantity, with a total of 15 flouts, as he gave either too much or too little information to the listener. The results of Sam’s limited pragmatic abilities fall into agreement with Fein’s (2010) claim that ASD individuals have pragmatic language deficits as they struggle to stay on topic and incorporate irrelevant details in conversations. This study further discusses how analyzing neurodivergent characters’ speech patterns can benefit teachers and students in a pedagogical setting.

Pleomorphic dermal sarcoma (PDS) was recognized in the 2013 World Health Organization Classification of Tumors of Soft Tissue and Bone as a clinical entity with adverse histopathologic features compared to the more superficial and less aggressive atypical fibroxanthoma (AFX). Although the gold standard treatment of AFX is Mohs micrographic surgery (MMS), the optimal treatment for PDS has yet to be determined. We report the case of a 71-year-old man with a PDS with perineural invasion on the scalp, with no recurrence one-year post-treatment with MMS and adjuvant radiation therapy. In contrast to wide local excision, MMS offers complete margin control and tissue preservation, which helps minimize scarring and morbidity. The comparative efficacy of MMS versus wide local excision in the treatment of PDS with and without radiation remains unknown and warrants further investigation.

Pleomorphic dermal sarcoma (PDS) was recognized in the 2013 World Health Organization Classification of Tumors of Soft Tissue and Bone as a clinical entity with adverse histopathologic features compared to the more superficial and less aggressive atypical fibroxanthoma (AFX). Although the gold standard treatment of AFX is Mohs micrographic surgery (MMS), the optimal treatment for PDS has yet to be determined. We report the case of a 71-year-old man with a PDS with perineural invasion on the scalp, with no recurrence one-year post-treatment with MMS and adjuvant radiation therapy. In contrast to wide local excision, MMS offers complete margin control and tissue preservation, which helps minimize scarring and morbidity. The comparative efficacy of MMS versus wide local excision in the treatment of PDS with and without radiation remains unknown and warrants further investigation.

Atypical fibroxanthoma is a rare cutaneous malignancy that usually presents as a rapidly growing red papule on the head and neck in elderly white males. Several variants have been described. We report a patient who presented with a slowly enlarging pigmented lesion on his left ear that was clinically worrisome for malignant melanoma. Histopathologic evaluation with immunohistochemistry revealed an unusual case of hemosiderotic pigmented atypical fibroxanthoma. The tumor was successfully extirpated with Mohs micrographic surgery, with no recurrence at 6-month follow-up.

Memory B cells (MBCs) are an essential part of our immunological memory. They respond fast upon re-encountering pathogens and can differentiate into plasma cells that secrete protective antibodies. The focus of this review is on MBCs that lack, or express low levels of, CD21, hereafter referred to as CD21-/low. These cells are expanded in peripheral blood with age and during chronic inflammatory conditions such as viral infections, malaria, common variable immunodeficiency, and autoimmune diseases. CD21-/low MBCs have gained significant attention; they produce disease-specific antibodies/autoantibodies and associate with key disease manifestations in some conditions. These cells can be divided into subsets based on classical B-cell and other markers, e.g. CD11c, FcRL4, and Tbet which, over the years, have become hallmarks to identify these cells. This has resulted in different names including age-associated, autoimmune-associated, atypical, tissue-like, tissue-resident, tissue-restricted, exhausted, or simply CD21-/low B cells. It is however unclear whether the expanded 'CD21-/low' cells in one condition are equivalent to those in another, whether they express an identical gene signature and whether they have a similar function. Here, we will discuss these issues with the goal to understand whether the CD21-/low B cells are comparable in different conditions., CC BY 4.0Correspondence: Inga-Lill Mårtensson, Department of Rheumatology and Inflammation Research, Institute of Medicine, Sahlgrenska Academy, University of Gothenburg, Gothenburg 40530, Sweden. Email: lill.martensson@rheuma.gu.seReceived 19 July 2022; Revised 5 October 2022; Accepted for publication 14 November 2022Funding has been received from: the Swedish Research Council 2018-03128 and 2021-01150 (ILM), 2016-00288 (IG), the Patient Association for Rheumatic Diseases R-94129 (ILM), R-940945 (IG), the Swedish Cancer Foundation 19 0464 (ILM), the Childhood Cancer Foundation PR2018-0170 (ILM), and the ALF agreement between the Swedish government and the county councils ALFGBG-965435 (IG), ALFGBG-277797 (ILM).

Meningiomas are the most common primary central nervous system neoplasm. Despite promising recent progress in elucidating the genomic landscape and underlying biology of these histologically, molecularly, and clinically diverse tumors, the mainstays of meningioma treatment remain maximal safe resection and radiation therapy. The aim of this review of meningioma radiotherapy is to provide a concise summary of the history, current evidence, and future for application of radiotherapy in meningioma treatment.

Meningiomas are the most common primary intracranial neoplasm. While traditionally viewed as benign, meningiomas are associated with significant patient morbidity, and certain meningioma subgroups display more aggressive and malignant behavior with higher rates of recurrence. Historically, the risk stratification of meningioma recurrence has been primarily associated with the World Health Organization histopathological grade and surgical extent of resection. However, a growing body of literature has highlighted the value of utilizing molecular characteristics to assess meningioma aggressiveness and recurrence risk. In this review, we discuss preclinical and clinical evidence surrounding the use of molecular classification schemes for meningioma prognostication. We also highlight how molecular data may inform meningioma treatment strategies and future directions.

Memory B cells (MBCs) are an essential part of our immunological memory. They respond fast upon re-encountering pathogens and can differentiate into plasma cells that secrete protective antibodies. The focus of this review is on MBCs that lack, or express low levels of, CD21, hereafter referred to as CD21-/low. These cells are expanded in peripheral blood with age and during chronic inflammatory conditions such as viral infections, malaria, common variable immunodeficiency, and autoimmune diseases. CD21-/low MBCs have gained significant attention; they produce disease-specific antibodies/autoantibodies and associate with key disease manifestations in some conditions. These cells can be divided into subsets based on classical B-cell and other markers, e.g. CD11c, FcRL4, and Tbet which, over the years, have become hallmarks to identify these cells. This has resulted in different names including age-associated, autoimmune-associated, atypical, tissue-like, tissue-resident, tissue-restricted, exhausted, or simply CD21-/low B cells. It is however unclear whether the expanded 'CD21-/low' cells in one condition are equivalent to those in another, whether they express an identical gene signature and whether they have a similar function. Here, we will discuss these issues with the goal to understand whether the CD21-/low B cells are comparable in different conditions., CC BY 4.0Correspondence: Inga-Lill Mårtensson, Department of Rheumatology and Inflammation Research, Institute of Medicine, Sahlgrenska Academy, University of Gothenburg, Gothenburg 40530, Sweden. Email: lill.martensson@rheuma.gu.seReceived 19 July 2022; Revised 5 October 2022; Accepted for publication 14 November 2022Funding has been received from: the Swedish Research Council 2018-03128 and 2021-01150 (ILM), 2016-00288 (IG), the Patient Association for Rheumatic Diseases R-94129 (ILM), R-940945 (IG), the Swedish Cancer Foundation 19 0464 (ILM), the Childhood Cancer Foundation PR2018-0170 (ILM), and the ALF agreement between the Swedish government and the county councils ALFGBG-965435 (IG), ALFGBG-277797 (ILM).

Memory B cells (MBCs) are an essential part of our immunological memory. They respond fast upon re-encountering pathogens and can differentiate into plasma cells that secrete protective antibodies. The focus of this review is on MBCs that lack, or express low levels of, CD21, hereafter referred to as CD21-/low. These cells are expanded in peripheral blood with age and during chronic inflammatory conditions such as viral infections, malaria, common variable immunodeficiency, and autoimmune diseases. CD21-/low MBCs have gained significant attention; they produce disease-specific antibodies/autoantibodies and associate with key disease manifestations in some conditions. These cells can be divided into subsets based on classical B-cell and other markers, e.g. CD11c, FcRL4, and Tbet which, over the years, have become hallmarks to identify these cells. This has resulted in different names including age-associated, autoimmune-associated, atypical, tissue-like, tissue-resident, tissue-restricted, exhausted, or simply CD21-/low B cells. It is however unclear whether the expanded 'CD21-/low' cells in one condition are equivalent to those in another, whether they express an identical gene signature and whether they have a similar function. Here, we will discuss these issues with the goal to understand whether the CD21-/low B cells are comparable in different conditions., CC BY 4.0Correspondence: Inga-Lill Mårtensson, Department of Rheumatology and Inflammation Research, Institute of Medicine, Sahlgrenska Academy, University of Gothenburg, Gothenburg 40530, Sweden. Email: lill.martensson@rheuma.gu.seReceived 19 July 2022; Revised 5 October 2022; Accepted for publication 14 November 2022Funding has been received from: the Swedish Research Council 2018-03128 and 2021-01150 (ILM), 2016-00288 (IG), the Patient Association for Rheumatic Diseases R-94129 (ILM), R-940945 (IG), the Swedish Cancer Foundation 19 0464 (ILM), the Childhood Cancer Foundation PR2018-0170 (ILM), and the ALF agreement between the Swedish government and the county councils ALFGBG-965435 (IG), ALFGBG-277797 (ILM).

Atypical Scrapie, which is not linked to epidemics, is assumed to be an idiopathic spontaneous prion disease in small ruminants. Therefore, its occurrence is unlikely to be controlled through selective breeding or other strategies as it is done for classical scrapie outbreaks. Its spontaneous nature and its sporadic incidence worldwide is reminiscent of the incidence of idiopathic spontaneous prion diseases in humans, which account for more than 85% of the cases in humans. Hence, developing animal models that consistently reproduce this phenomenon of spontaneous PrP misfolding, is of importance to study the pathobiology of idiopathic spontaneous prion disorders. Transgenic mice overexpressing sheep PrPC with I112 polymorphism (TgShI112, 1-2 × PrP levels compared to sheep brain) manifest clinical signs of a spongiform encephalopathy spontaneously as early as 380 days of age. The brains of these animals show the neuropathological hallmarks of prion disease and biochemical analyses of the misfolded prion protein show a ladder-like PrPres pattern with a predominant 7-10 kDa band. Brain homogenates from spontaneously diseased transgenic mice were inoculated in several models to assess their transmissibility and characterize the prion strain generated: TgShI112 (ovine I112 ARQ PrPC), Tg338 (ovine VRQ PrPC), Tg501 (ovine ARQ PrPC), Tg340 (human M129 PrPC), Tg361 (human V129 PrPC), TgVole (bank vole I109 PrPC), bank vole (I109I PrPC), and sheep (AHQ/ARR and AHQ/AHQ churra-tensina breeds). Our analysis of the results of these bioassays concludes that the strain generated in this model is indistinguishable to that causing atypical scrapie (Nor98). Thus, we present the first faithful model for a bona fide, transmissible, ovine, atypical scrapie prion disease.

Memory B cells (MBCs) are an essential part of our immunological memory. They respond fast upon re-encountering pathogens and can differentiate into plasma cells that secrete protective antibodies. The focus of this review is on MBCs that lack, or express low levels of, CD21, hereafter referred to as CD21-/low. These cells are expanded in peripheral blood with age and during chronic inflammatory conditions such as viral infections, malaria, common variable immunodeficiency, and autoimmune diseases. CD21-/low MBCs have gained significant attention; they produce disease-specific antibodies/autoantibodies and associate with key disease manifestations in some conditions. These cells can be divided into subsets based on classical B-cell and other markers, e.g. CD11c, FcRL4, and Tbet which, over the years, have become hallmarks to identify these cells. This has resulted in different names including age-associated, autoimmune-associated, atypical, tissue-like, tissue-resident, tissue-restricted, exhausted, or simply CD21-/low B cells. It is however unclear whether the expanded 'CD21-/low' cells in one condition are equivalent to those in another, whether they express an identical gene signature and whether they have a similar function. Here, we will discuss these issues with the goal to understand whether the CD21-/low B cells are comparable in different conditions., CC BY 4.0Correspondence: Inga-Lill Mårtensson, Department of Rheumatology and Inflammation Research, Institute of Medicine, Sahlgrenska Academy, University of Gothenburg, Gothenburg 40530, Sweden. Email: lill.martensson@rheuma.gu.seReceived 19 July 2022; Revised 5 October 2022; Accepted for publication 14 November 2022Funding has been received from: the Swedish Research Council 2018-03128 and 2021-01150 (ILM), 2016-00288 (IG), the Patient Association for Rheumatic Diseases R-94129 (ILM), R-940945 (IG), the Swedish Cancer Foundation 19 0464 (ILM), the Childhood Cancer Foundation PR2018-0170 (ILM), and the ALF agreement between the Swedish government and the county councils ALFGBG-965435 (IG), ALFGBG-277797 (ILM).

Memory B cells (MBCs) are an essential part of our immunological memory. They respond fast upon re-encountering pathogens and can differentiate into plasma cells that secrete protective antibodies. The focus of this review is on MBCs that lack, or express low levels of, CD21, hereafter referred to as CD21-/low. These cells are expanded in peripheral blood with age and during chronic inflammatory conditions such as viral infections, malaria, common variable immunodeficiency, and autoimmune diseases. CD21-/low MBCs have gained significant attention; they produce disease-specific antibodies/autoantibodies and associate with key disease manifestations in some conditions. These cells can be divided into subsets based on classical B-cell and other markers, e.g. CD11c, FcRL4, and Tbet which, over the years, have become hallmarks to identify these cells. This has resulted in different names including age-associated, autoimmune-associated, atypical, tissue-like, tissue-resident, tissue-restricted, exhausted, or simply CD21-/low B cells. It is however unclear whether the expanded 'CD21-/low' cells in one condition are equivalent to those in another, whether they express an identical gene signature and whether they have a similar function. Here, we will discuss these issues with the goal to understand whether the CD21-/low B cells are comparable in different conditions., CC BY 4.0Correspondence: Inga-Lill Mårtensson, Department of Rheumatology and Inflammation Research, Institute of Medicine, Sahlgrenska Academy, University of Gothenburg, Gothenburg 40530, Sweden. Email: lill.martensson@rheuma.gu.seReceived 19 July 2022; Revised 5 October 2022; Accepted for publication 14 November 2022Funding has been received from: the Swedish Research Council 2018-03128 and 2021-01150 (ILM), 2016-00288 (IG), the Patient Association for Rheumatic Diseases R-94129 (ILM), R-940945 (IG), the Swedish Cancer Foundation 19 0464 (ILM), the Childhood Cancer Foundation PR2018-0170 (ILM), and the ALF agreement between the Swedish government and the county councils ALFGBG-965435 (IG), ALFGBG-277797 (ILM).

BackgroundThe classic phenotype of CLN2 disease (neuronal ceroid lipofuscinosis type 2) typically manifests between ages 2 and 4 years with a predictable clinical course marked by epilepsy, language developmental delay, and rapid psychomotor decline. Atypical phenotypes exhibit variable time of onset, symptomatology, and/or progression. Intracerebroventricular-administered cerliponase alfa (rhTPP1 enzyme) has been shown to stabilize motor and language function loss in patients with classic CLN2 disease, but its impact on individuals with atypical phenotypes has not been described.MethodsA chart review was conducted of 14 patients (8 male, 6 female) with atypical CLN2 phenotypes who received cerliponase alfa. Pre- and posttreatment CLN2 Clinical Rating Scale Motor and Language (ML) domain scores were compared.ResultsMedian age at first presenting symptom was 5.9 years. First reported symptoms were language abnormalities (6 [43%] patients), seizures (4 [29%]), ataxia/language abnormalities (3 [21%]), and ataxia alone (1 [7%]). Median age at diagnosis was 10.8 years. ML score declined before treatment in 13 (93%) patients. Median age at treatment initiation was 11.7 years; treatment duration ranged from 11 to 58 months. From treatment start, ML score remained stable in 11 patients (treatment duration 11-43 months), improved 1 point in 1 patient after 13 months, and declined 1 point in 2 patients after 15 and 58 months, respectively. There were 13 device-related infections in 8 patients (57%) and 10 hypersensitivity reactions in 6 (43%).ConclusionsCerliponase alfa is well tolerated and has the potential to stabilize motor and language function in patients with atypical phenotypes of CLN2 disease.

Pulmonary carcinoid tumors are uncommon neuroendocrine tumors that rarely metastasize to the skin. We report the case of a 71-year-old woman with a longstanding history of primary atypical pulmonary carcinoid tumor who presented with a new tender cutaneous nodule. Immunostaining of the nodule was consistent with metastatic atypical carcinoid tumor of the skin including positive staining for neuroendocrine markers chromogranin and synaptophysin. Dermatologists should consider cutaneous neuroendocrine metastasis when evaluating new nodules in patients with stable pulmonary carcinoid tumors or in those with concomitant concerning respiratory symptoms.

The significance of periodontal pathology is determined not only bythe prevalence and severity of the disease, the negative impact on the bodyas a whole, but also by the insufficient effectiveness of the treatment(Atrushkevich V. G., 2012; Orekhova L. Yu., 2014; Grudyanov A. I., 2015;Bulgakova A. I., 2015). In modern orthopedic dentistry, an urgent medicaland social problem is inflammatory periodontal diseases( VZP), specialattention should be paid to gingivitis and periodontitis, since these diseasesare more common than others and affect the general condition of the oralcavity, the body as a whole, and the quality of life of the patient, inaddition, this problem comes to the fore due to the high prevalence and loweffectiveness of the therapy. There is an obvious increase in the frequencyof prevalence of atypical forms of periodontitis, it should be noted thataggressive forms also belong to atypical forms of periodontitis

The significance of periodontal pathology is determined not only bythe prevalence and severity of the disease, the negative impact on the bodyas a whole, but also by the insufficient effectiveness of the treatment(Atrushkevich V. G., 2012; Orekhova L. Yu., 2014; Grudyanov A. I., 2015;Bulgakova A. I., 2015). In modern orthopedic dentistry, an urgent medicaland social problem is inflammatory periodontal diseases( VZP), specialattention should be paid to gingivitis and periodontitis, since these diseasesare more common than others and affect the general condition of the oralcavity, the body as a whole, and the quality of life of the patient, inaddition, this problem comes to the fore due to the high prevalence and loweffectiveness of the therapy. There is an obvious increase in the frequencyof prevalence of atypical forms of periodontitis, it should be noted thataggressive forms also belong to atypical forms of periodontitis

Atypical vascular lesion (AVL) is an uncommon, benign vascular proliferation seen in previously irradiated skin, most commonly after radiotherapy for breast cancer. Atypical vascular lesion and angiosarcoma may share overlapping clinical and histopathologic features. We report the first case of AVL occurring outside the field of radiation. This patient's clinical course and histopathology was overall consistent with AVL, including two biopsies with focal MYC positivity. However, due to variations in the interpretation of her histopathology, the management plans devised by two centers involved in her care were widely discordant and she was treated with chemotherapy and extensive surgery for angiosarcoma. Great care must be taken to distinguish between these entities, as treatment for angiosarcoma may be associated with significant morbidity.

Diet has been associated with the risk of depression, whereas different subtypes of depression have been linked with different cardiovascular risk factors (CVRFs). In this study, our aims were to (1) identify dietary patterns with exploratory factor analysis, (2) assess cross-sectional associations between dietary patterns and depression subtypes, and (3) examine the potentially mediating effect of dietary patterns in the associations between CVRFs and depression subtypes. In the first follow-up of the population-based CoLaus|PsyCoLaus study (2009–2013, 3554 participants, 45.6% men, mean age 57.5 years), a food frequency questionnaire assessed dietary intake and a semi-structured interview allowed to characterize major depressive disorder into current or remitted atypical, melancholic, and unspecified subtypes. Three dietary patterns were identified: Western, Mediterranean, and Sweet-Dairy. Western diet was positively associated with current atypical depression, but negatively associated with current and remitted melancholic depression. Sweet-Dairy was positively associated with current melancholic depression. However, these dietary patterns did not mediate the associations between CVRFs and depression subtypes. Hence, although we could show that people with different subtypes of depression make different choices regarding their diet, it is unlikely that these differential dietary choices account for the well-established associations between depression subtypes and CVRFs.

Atypical vascular lesion (AVL) is an uncommon, benign vascular proliferation seen in previously irradiated skin, most commonly after radiotherapy for breast cancer. Atypical vascular lesion and angiosarcoma may share overlapping clinical and histopathologic features. We report the first case of AVL occurring outside the field of radiation. This patient's clinical course and histopathology was overall consistent with AVL, including two biopsies with focal MYC positivity. However, due to variations in the interpretation of her histopathology, the management plans devised by two centers involved in her care were widely discordant and she was treated with chemotherapy and extensive surgery for angiosarcoma. Great care must be taken to distinguish between these entities, as treatment for angiosarcoma may be associated with significant morbidity.

The significance of periodontal pathology is determined not only bythe prevalence and severity of the disease, the negative impact on the bodyas a whole, but also by the insufficient effectiveness of the treatment(Atrushkevich V. G., 2012; Orekhova L. Yu., 2014; Grudyanov A. I., 2015;Bulgakova A. I., 2015). In modern orthopedic dentistry, an urgent medicaland social problem is inflammatory periodontal diseases( VZP), specialattention should be paid to gingivitis and periodontitis, since these diseasesare more common than others and affect the general condition of the oralcavity, the body as a whole, and the quality of life of the patient, inaddition, this problem comes to the fore due to the high prevalence and loweffectiveness of the therapy. There is an obvious increase in the frequencyof prevalence of atypical forms of periodontitis, it should be noted thataggressive forms also belong to atypical forms of periodontitis

Pulmonary carcinoid tumors are uncommon neuroendocrine tumors that rarely metastasize to the skin. We report the case of a 71-year-old woman with a longstanding history of primary atypical pulmonary carcinoid tumor who presented with a new tender cutaneous nodule. Immunostaining of the nodule was consistent with metastatic atypical carcinoid tumor of the skin including positive staining for neuroendocrine markers chromogranin and synaptophysin. Dermatologists should consider cutaneous neuroendocrine metastasis when evaluating new nodules in patients with stable pulmonary carcinoid tumors or in those with concomitant concerning respiratory symptoms.

BackgroundThe classic phenotype of CLN2 disease (neuronal ceroid lipofuscinosis type 2) typically manifests between ages 2 and 4 years with a predictable clinical course marked by epilepsy, language developmental delay, and rapid psychomotor decline. Atypical phenotypes exhibit variable time of onset, symptomatology, and/or progression. Intracerebroventricular-administered cerliponase alfa (rhTPP1 enzyme) has been shown to stabilize motor and language function loss in patients with classic CLN2 disease, but its impact on individuals with atypical phenotypes has not been described.MethodsA chart review was conducted of 14 patients (8 male, 6 female) with atypical CLN2 phenotypes who received cerliponase alfa. Pre- and posttreatment CLN2 Clinical Rating Scale Motor and Language (ML) domain scores were compared.ResultsMedian age at first presenting symptom was 5.9 years. First reported symptoms were language abnormalities (6 [43%] patients), seizures (4 [29%]), ataxia/language abnormalities (3 [21%]), and ataxia alone (1 [7%]). Median age at diagnosis was 10.8 years. ML score declined before treatment in 13 (93%) patients. Median age at treatment initiation was 11.7 years; treatment duration ranged from 11 to 58 months. From treatment start, ML score remained stable in 11 patients (treatment duration 11-43 months), improved 1 point in 1 patient after 13 months, and declined 1 point in 2 patients after 15 and 58 months, respectively. There were 13 device-related infections in 8 patients (57%) and 10 hypersensitivity reactions in 6 (43%).ConclusionsCerliponase alfa is well tolerated and has the potential to stabilize motor and language function in patients with atypical phenotypes of CLN2 disease.

Though distinct pathological entities, corticobasal degeneration (CBD) and progressive supranuclear palsy (PSP) share multiple biochemical and genetic features suggesting overlapping pathophysiology. We report the case of a patient with an 18-year clinical course consistent with behavioral variant frontotemporal dementia. The neuropathological assessment revealed unclassifiable frontotemporal lobar degeneration with tau-immunoreactive inclusions sharing features of both CBD and PSP. Whole-genome sequencing revealed a unique combination of pleiotropic genetic risk variants associated with both PSP and CBD. These findings support the observation that CBD and PSP share genetic co-expression networks that influence neurodegenerative pathogenesis common to 4R tauopathies.

Among the works of Cesa-Bianchi in the field of developmental and educational psychology, his contributions on the theme of atypical development, on the relationship between the development of thought and language in a child's growth and, finally, his latest reflections and indications on teacher and educator training are of particular interest. Mostly sensitive to the Piagetian and maturationist approach, Cesa-Bianchi did not fail to welcome in his theoretical reflection, as much as in scientific research, the stimuli that came from post-Piagetian culture since the first half of the twentieth century, driving his attention to the bio-psycho-social dimensions of human development. This work traces some key themes of the contribution of Cesa-Bianchi and his close collaborators highlighting the correlations with the national and international scientific scenario.

Background: Laryngopharyngeal reflux (LPR) is a common disease in otolaryngology characterized by an inflammatory reaction of the mucosa of the upper aerodigestive tract caused by digestive refluxate enzymes. LPR has been identified as the etiological or favoring factor of laryngeal, oral, sinonasal, or otological diseases. In this case series, we reported the atypical clinical presentation of LPR in patients presenting in our clinic with reflux. Methods: A retrospective medical chart review of 351 patients with LPR treated in the European Reflux Clinic in Brussels, Poitiers and Paris was performed. In order to be included, patients had to report an atypical clinical presentation of LPR, consisting of symptoms or findings that are not described in the reflux symptom score and reflux sign assessment. The LPR diagnosis was confirmed with a 24 h hypopharyngeal-esophageal impedance pH study, and patients were treated with a combination of diet, proton pump inhibitors, and alginates. The atypical symptoms or findings had to be resolved from pre-to posttreatment. Results: From 2017 to 2021, 21 patients with atypical LPR were treated in our center. The clinical presentation consisted of recurrent aphthosis or burning mouth (N = 9), recurrent burps and abdominal disorders (N = 2), posterior nasal obstruction (N = 2), recurrent acute suppurative otitis media (N = 2), severe vocal fold dysplasia (N = 2), and recurrent acute rhinopharyngitis (N = 1), tearing (N = 1), aspirations (N = 1), or tracheobronchitis (N = 1). Abnormal upper aerodigestive tract reflux events were identified in all of these patients. Atypical clinical findings resolved and did not recur after an adequate antireflux treatment. Conclusion: LPR may present with various clinical presentations, including mouth, eye, tracheobronchial, nasal, or laryngeal findings, which may all regress with adequate treatment. Future studies are needed to better specify the relationship between LPR and these atypical findings, SCOPUS: ar.j, info:eu-repo/semantics/published

Neuroendocrine neoplasms (NENs) of the lung encompass neuroendocrine tumors (NETs) composed of typical (TC) and atypical (AC) carcinoids and full-fledged carcinomas (NECs) inclusive of large cell neuroendocrine carcinoma (LCNEC) and small cell carcinoma (SCLC). NETs and NECs are thought to represent distinct and separate lesions with neither molecular overlap nor common developmental continuum. Two perspectives were addressed regarding the morphologic and molecular classification of lung NENs: (i) a supervised approach by browsing the traditional classification, the relevant gene alterations, and their clinical implications; and (ii) an unsupervised approach, by reappraising neoplasms according to risk factors and natural history of disease to construct an interpretation model relied on biological data. We herein emphasize lights and shadows of the current classification of lung NENs and provide an alternative outlook on these tumors focused on what we currently know about the biological determinants and the natural history of disease., SCOPUS: re.j, info:eu-repo/semantics/published

Lung abscess is a circumscribed collection of pus or necrotic material localized in the lung parenchyma as a result of a bacterial infection. Is considered a disease of low prevalence, but it has an important morbimortality. This article presents the case of a 49 years old masculine patient that presents with cough and expectoration of 1 month, in which a pulmonary abscess with atypical presentation was documented and describes the image obtained via computerized tomography (CT). Also, it describes the definition, epidemiology, risk factors and clinical manifestations of this disease., El absceso pulmonar corresponde a una colección circunscrita de pus o material necrótico localizado en el parénquima pulmonar como resultado de una infección bacteriana. Se considera una patología de baja prevalencia, pero con importante morbimortalidad. El presente artículo reporta el caso de un paciente masculino de 49 años, con un cuadro de tos y expectoración de un mes de evolución, en quien se documentaron hallazgos sugestivos de un absceso pulmonar de presentación atípica y se describe la imagen obtenida por tomografía axial computarizada. Además, se describe la definición, epidemiología, factores de riesgo y manifestaciones clínicas de esta patología.

Pleomorphic dermal sarcoma is a cutaneous soft tissue sarcoma that presents as a rapidly enlarging tumor, typically on a sun-exposed location of elderly individuals. The neoplasm shares many similar features — clinical, pathologic, immunohistochemical and genomic — with atypical fibroxanthoma. However, adverse histologic characteristics (deep subcutaneous invasion, tumor necrosis, lymphovascular invasion, and/or perineural invasion) differentiate pleomorphic dermal sarcoma from atypical fibroxanthoma and may account for the more aggressive biologic behavior of pleomorphic dermal sarcoma: local recurrence and metastases. The features of a woman with pleomorphic dermal sarcoma are described. Her sarcoma presented as a rapidly growing ulcerated red nodule on the left side of her face. Imaging studies were performed prior to surgery. The tumor was extirpated with a wide local excision and she received postoperative radiotherapy. There has been no recurrence or metastasis at one-year follow-up. Pleomorphic dermal sarcoma has previously been referred to as a malignant fibrous histiocytoma (until the term became antiquated) and an undifferentiated pleomorphic sarcoma. However, the latter term includes not only neoplasms from the skin but also sarcomas from internal organs, retroperitoneal and osteoid origin. Therefore, when classifying this undifferentiated soft tissue sarcomas of cutaneous origin, the term pleomorphic dermal sarcoma may be preferred.

A partir da reforma energética de 2013, o setor elétrico no México adquiriu uma nova fisionomia. Essa reforma reestruturou a participação do Estado e dos indivíduos, em sua estrutura normativa, técnica, econômica e social, com tendência a tornar seus processos mais eficientes e seguros, criar mercados, gerar riqueza e melhorar os preços ao consumidor. Seguiu-se um turbilhão legislativo para adaptar as dependências e entidades do Estado à nova realidade, porque tanto a reforma constitucional quanto suas leis secundárias criaram novas instituições e reconfiguraram as antigas, sem parar para verificar se sua natureza coincidia com a organização. Da administração pública, independentemente do que a doutrina nacional dissesse a respeito. Esta pesquisa está posicionada em um nível descritivo do tipo documentário. Ao abordar a linha legal, foram encontrados os andaimes constitucionais que governam a administração pública e sua organização legalmente classificada como centralizada e paraestatal. Tomando conceitos de juristas que lançaram as bases para as leituras atuais, a natureza das dependências ou entidades foi analisada para observar suas semelhanças e discrepâncias, proximidade e diferenças; para tentar colocá-los de acordo com essas estruturas. Nessa linha, destacam-se duas instituições que não coincidem em todos os seus termos com a classificação, por abrangerem características de várias categorias, portanto, foi considerado inadequado continuar colocando-as nos conceitos tradicionais. Com isso em mente, foi realizada a busca por uma nova categoria na classificação da organização da administração pública que incluía a nova realidade. E concluiu-se que, através da administração pública transversal, algumas das instituições atípicas do setor elétrico mexicano se encaixam., As of the energy reform of 2013, the electricity sector in Mexico acquired a new physiognomy. This reform restructured the participation of the State and individuals, in its normative, technical, economic and social framework, with a tendency to make its processes more efficient and safe, create markets, generate wealth and improve consumer prices. It was followed by a legislative vortex to adapt the dependencies and entities of the State to the new reality, because both the constitutional reform and its secondary laws created new institutions and reconfigured the old ones, without stopping to verify if their nature coincided with the organization of the public administration, regardless of what the national doctrine said about it. This research is positioned at a descriptive level of documentary type. When addressing the legal line, the constitutional scaffolding that governs the public administration and its organization legally classified as centralized and parastatal was found. Taking up concepts of jurists that laid the foundations for the current readings, the nature of the dependencies or entities was analyzed to notice their similarities and discrepancies, proximity and differences; to try to locate them according to those structures. In that line, two institutions stand out that did not coincide in all of their terms with the classification, since they cover characteristics of several categories, so it was considered inappropriate to continue placing them in traditional concepts. Taking this into account, the search for a new category within the classification of the public administration organization that included the new reality was undertaken. And it was concluded that through the transversal public administration some of the atypical institutions of the Mexican electricity sector fit., A partir de la reforma energética de 2013, el sector eléctrico en México adquirió una nueva fisonomía. Dicha reforma reestructuró la participación del Estado y de los particulares, en su trama normativa, técnica, económica y social, con tendencia a hacer más eficientes y seguros sus procesos, crear mercados, generar riqueza y mejorar los precios del consumidor. Le siguió una vorágine legislativa para adaptar a las dependencias y entidades del Estado a la nueva realidad, porque tanto en la reforma constitucional como en sus leyes secundarias se crearon nuevas instituciones y se reconfiguraron las antiguas, sin detenerse a verificar si su naturaleza coincidía con la organización de la administración pública, sin reparar en lo que la doctrina nacional decía al respecto. La presente investigación se posiciona en un nivel descriptivo de tipo documental. Al abordar el trazo jurídico, se encontró el andamiaje constitucional que rige la administración pública y su organización clasificada legalmente como centralizada y paraestatal. Retomando conceptos de juristas que sentaron las bases para las lecturas actuales, se analizó la naturaleza de las dependencias o entidades para advertir sus similitudes y discrepancias, proximidades y diferencias; para tratar de ubicarlas conforme a esas estructuras. En esa línea, destacan dos instituciones que no coincidieron en todos sus términos con la clasificación, pues abarcan características de varias categorías, por lo que se consideró inapropiado seguirlas ubicando en los conceptos tradicionales. Tomado esto en cuenta, se emprendió la búsqueda de una nueva categoría al interior de la clasificación de la organización de la administración pública que comprendiera la nueva realidad. Y se concluyó que mediante la administración pública transversal caben algunas de las instituciones atípicas del sector eléctrico mexicano.

Pleomorphic dermal sarcoma is a cutaneous soft tissue sarcoma that presents as a rapidly enlarging tumor, typically on a sun-exposed location of elderly individuals. The neoplasm shares many similar features — clinical, pathologic, immunohistochemical and genomic — with atypical fibroxanthoma. However, adverse histologic characteristics (deep subcutaneous invasion, tumor necrosis, lymphovascular invasion, and/or perineural invasion) differentiate pleomorphic dermal sarcoma from atypical fibroxanthoma and may account for the more aggressive biologic behavior of pleomorphic dermal sarcoma: local recurrence and metastases. The features of a woman with pleomorphic dermal sarcoma are described. Her sarcoma presented as a rapidly growing ulcerated red nodule on the left side of her face. Imaging studies were performed prior to surgery. The tumor was extirpated with a wide local excision and she received postoperative radiotherapy. There has been no recurrence or metastasis at one-year follow-up. Pleomorphic dermal sarcoma has previously been referred to as a malignant fibrous histiocytoma (until the term became antiquated) and an undifferentiated pleomorphic sarcoma. However, the latter term includes not only neoplasms from the skin but also sarcomas from internal organs, retroperitoneal and osteoid origin. Therefore, when classifying this undifferentiated soft tissue sarcomas of cutaneous origin, the term pleomorphic dermal sarcoma may be preferred.

A partir da reforma energética de 2013, o setor elétrico no México adquiriu uma nova fisionomia. Essa reforma reestruturou a participação do Estado e dos indivíduos, em sua estrutura normativa, técnica, econômica e social, com tendência a tornar seus processos mais eficientes e seguros, criar mercados, gerar riqueza e melhorar os preços ao consumidor. Seguiu-se um turbilhão legislativo para adaptar as dependências e entidades do Estado à nova realidade, porque tanto a reforma constitucional quanto suas leis secundárias criaram novas instituições e reconfiguraram as antigas, sem parar para verificar se sua natureza coincidia com a organização. Da administração pública, independentemente do que a doutrina nacional dissesse a respeito. Esta pesquisa está posicionada em um nível descritivo do tipo documentário. Ao abordar a linha legal, foram encontrados os andaimes constitucionais que governam a administração pública e sua organização legalmente classificada como centralizada e paraestatal. Tomando conceitos de juristas que lançaram as bases para as leituras atuais, a natureza das dependências ou entidades foi analisada para observar suas semelhanças e discrepâncias, proximidade e diferenças; para tentar colocá-los de acordo com essas estruturas. Nessa linha, destacam-se duas instituições que não coincidem em todos os seus termos com a classificação, por abrangerem características de várias categorias, portanto, foi considerado inadequado continuar colocando-as nos conceitos tradicionais. Com isso em mente, foi realizada a busca por uma nova categoria na classificação da organização da administração pública que incluía a nova realidade. E concluiu-se que, através da administração pública transversal, algumas das instituições atípicas do setor elétrico mexicano se encaixam., As of the energy reform of 2013, the electricity sector in Mexico acquired a new physiognomy. This reform restructured the participation of the State and individuals, in its normative, technical, economic and social framework, with a tendency to make its processes more efficient and safe, create markets, generate wealth and improve consumer prices. It was followed by a legislative vortex to adapt the dependencies and entities of the State to the new reality, because both the constitutional reform and its secondary laws created new institutions and reconfigured the old ones, without stopping to verify if their nature coincided with the organization of the public administration, regardless of what the national doctrine said about it. This research is positioned at a descriptive level of documentary type. When addressing the legal line, the constitutional scaffolding that governs the public administration and its organization legally classified as centralized and parastatal was found. Taking up concepts of jurists that laid the foundations for the current readings, the nature of the dependencies or entities was analyzed to notice their similarities and discrepancies, proximity and differences; to try to locate them according to those structures. In that line, two institutions stand out that did not coincide in all of their terms with the classification, since they cover characteristics of several categories, so it was considered inappropriate to continue placing them in traditional concepts. Taking this into account, the search for a new category within the classification of the public administration organization that included the new reality was undertaken. And it was concluded that through the transversal public administration some of the atypical institutions of the Mexican electricity sector fit., A partir de la reforma energética de 2013, el sector eléctrico en México adquirió una nueva fisonomía. Dicha reforma reestructuró la participación del Estado y de los particulares, en su trama normativa, técnica, económica y social, con tendencia a hacer más eficientes y seguros sus procesos, crear mercados, generar riqueza y mejorar los precios del consumidor. Le siguió una vorágine legislativa para adaptar a las dependencias y entidades del Estado a la nueva realidad, porque tanto en la reforma constitucional como en sus leyes secundarias se crearon nuevas instituciones y se reconfiguraron las antiguas, sin detenerse a verificar si su naturaleza coincidía con la organización de la administración pública, sin reparar en lo que la doctrina nacional decía al respecto. La presente investigación se posiciona en un nivel descriptivo de tipo documental. Al abordar el trazo jurídico, se encontró el andamiaje constitucional que rige la administración pública y su organización clasificada legalmente como centralizada y paraestatal. Retomando conceptos de juristas que sentaron las bases para las lecturas actuales, se analizó la naturaleza de las dependencias o entidades para advertir sus similitudes y discrepancias, proximidades y diferencias; para tratar de ubicarlas conforme a esas estructuras. En esa línea, destacan dos instituciones que no coincidieron en todos sus términos con la clasificación, pues abarcan características de varias categorías, por lo que se consideró inapropiado seguirlas ubicando en los conceptos tradicionales. Tomado esto en cuenta, se emprendió la búsqueda de una nueva categoría al interior de la clasificación de la organización de la administración pública que comprendiera la nueva realidad. Y se concluyó que mediante la administración pública transversal caben algunas de las instituciones atípicas del sector eléctrico mexicano.

A partir da reforma energética de 2013, o setor elétrico no México adquiriu uma nova fisionomia. Essa reforma reestruturou a participação do Estado e dos indivíduos, em sua estrutura normativa, técnica, econômica e social, com tendência a tornar seus processos mais eficientes e seguros, criar mercados, gerar riqueza e melhorar os preços ao consumidor. Seguiu-se um turbilhão legislativo para adaptar as dependências e entidades do Estado à nova realidade, porque tanto a reforma constitucional quanto suas leis secundárias criaram novas instituições e reconfiguraram as antigas, sem parar para verificar se sua natureza coincidia com a organização. Da administração pública, independentemente do que a doutrina nacional dissesse a respeito. Esta pesquisa está posicionada em um nível descritivo do tipo documentário. Ao abordar a linha legal, foram encontrados os andaimes constitucionais que governam a administração pública e sua organização legalmente classificada como centralizada e paraestatal. Tomando conceitos de juristas que lançaram as bases para as leituras atuais, a natureza das dependências ou entidades foi analisada para observar suas semelhanças e discrepâncias, proximidade e diferenças; para tentar colocá-los de acordo com essas estruturas. Nessa linha, destacam-se duas instituições que não coincidem em todos os seus termos com a classificação, por abrangerem características de várias categorias, portanto, foi considerado inadequado continuar colocando-as nos conceitos tradicionais. Com isso em mente, foi realizada a busca por uma nova categoria na classificação da organização da administração pública que incluía a nova realidade. E concluiu-se que, através da administração pública transversal, algumas das instituições atípicas do setor elétrico mexicano se encaixam., As of the energy reform of 2013, the electricity sector in Mexico acquired a new physiognomy. This reform restructured the participation of the State and individuals, in its normative, technical, economic and social framework, with a tendency to make its processes more efficient and safe, create markets, generate wealth and improve consumer prices. It was followed by a legislative vortex to adapt the dependencies and entities of the State to the new reality, because both the constitutional reform and its secondary laws created new institutions and reconfigured the old ones, without stopping to verify if their nature coincided with the organization of the public administration, regardless of what the national doctrine said about it. This research is positioned at a descriptive level of documentary type. When addressing the legal line, the constitutional scaffolding that governs the public administration and its organization legally classified as centralized and parastatal was found. Taking up concepts of jurists that laid the foundations for the current readings, the nature of the dependencies or entities was analyzed to notice their similarities and discrepancies, proximity and differences; to try to locate them according to those structures. In that line, two institutions stand out that did not coincide in all of their terms with the classification, since they cover characteristics of several categories, so it was considered inappropriate to continue placing them in traditional concepts. Taking this into account, the search for a new category within the classification of the public administration organization that included the new reality was undertaken. And it was concluded that through the transversal public administration some of the atypical institutions of the Mexican electricity sector fit., A partir de la reforma energética de 2013, el sector eléctrico en México adquirió una nueva fisonomía. Dicha reforma reestructuró la participación del Estado y de los particulares, en su trama normativa, técnica, económica y social, con tendencia a hacer más eficientes y seguros sus procesos, crear mercados, generar riqueza y mejorar los precios del consumidor. Le siguió una vorágine legislativa para adaptar a las dependencias y entidades del Estado a la nueva realidad, porque tanto en la reforma constitucional como en sus leyes secundarias se crearon nuevas instituciones y se reconfiguraron las antiguas, sin detenerse a verificar si su naturaleza coincidía con la organización de la administración pública, sin reparar en lo que la doctrina nacional decía al respecto. La presente investigación se posiciona en un nivel descriptivo de tipo documental. Al abordar el trazo jurídico, se encontró el andamiaje constitucional que rige la administración pública y su organización clasificada legalmente como centralizada y paraestatal. Retomando conceptos de juristas que sentaron las bases para las lecturas actuales, se analizó la naturaleza de las dependencias o entidades para advertir sus similitudes y discrepancias, proximidades y diferencias; para tratar de ubicarlas conforme a esas estructuras. En esa línea, destacan dos instituciones que no coincidieron en todos sus términos con la clasificación, pues abarcan características de varias categorías, por lo que se consideró inapropiado seguirlas ubicando en los conceptos tradicionales. Tomado esto en cuenta, se emprendió la búsqueda de una nueva categoría al interior de la clasificación de la organización de la administración pública que comprendiera la nueva realidad. Y se concluyó que mediante la administración pública transversal caben algunas de las instituciones atípicas del sector eléctrico mexicano.

A partir da reforma energética de 2013, o setor elétrico no México adquiriu uma nova fisionomia. Essa reforma reestruturou a participação do Estado e dos indivíduos, em sua estrutura normativa, técnica, econômica e social, com tendência a tornar seus processos mais eficientes e seguros, criar mercados, gerar riqueza e melhorar os preços ao consumidor. Seguiu-se um turbilhão legislativo para adaptar as dependências e entidades do Estado à nova realidade, porque tanto a reforma constitucional quanto suas leis secundárias criaram novas instituições e reconfiguraram as antigas, sem parar para verificar se sua natureza coincidia com a organização. Da administração pública, independentemente do que a doutrina nacional dissesse a respeito. Esta pesquisa está posicionada em um nível descritivo do tipo documentário. Ao abordar a linha legal, foram encontrados os andaimes constitucionais que governam a administração pública e sua organização legalmente classificada como centralizada e paraestatal. Tomando conceitos de juristas que lançaram as bases para as leituras atuais, a natureza das dependências ou entidades foi analisada para observar suas semelhanças e discrepâncias, proximidade e diferenças; para tentar colocá-los de acordo com essas estruturas. Nessa linha, destacam-se duas instituições que não coincidem em todos os seus termos com a classificação, por abrangerem características de várias categorias, portanto, foi considerado inadequado continuar colocando-as nos conceitos tradicionais. Com isso em mente, foi realizada a busca por uma nova categoria na classificação da organização da administração pública que incluía a nova realidade. E concluiu-se que, através da administração pública transversal, algumas das instituições atípicas do setor elétrico mexicano se encaixam., As of the energy reform of 2013, the electricity sector in Mexico acquired a new physiognomy. This reform restructured the participation of the State and individuals, in its normative, technical, economic and social framework, with a tendency to make its processes more efficient and safe, create markets, generate wealth and improve consumer prices. It was followed by a legislative vortex to adapt the dependencies and entities of the State to the new reality, because both the constitutional reform and its secondary laws created new institutions and reconfigured the old ones, without stopping to verify if their nature coincided with the organization of the public administration, regardless of what the national doctrine said about it. This research is positioned at a descriptive level of documentary type. When addressing the legal line, the constitutional scaffolding that governs the public administration and its organization legally classified as centralized and parastatal was found. Taking up concepts of jurists that laid the foundations for the current readings, the nature of the dependencies or entities was analyzed to notice their similarities and discrepancies, proximity and differences; to try to locate them according to those structures. In that line, two institutions stand out that did not coincide in all of their terms with the classification, since they cover characteristics of several categories, so it was considered inappropriate to continue placing them in traditional concepts. Taking this into account, the search for a new category within the classification of the public administration organization that included the new reality was undertaken. And it was concluded that through the transversal public administration some of the atypical institutions of the Mexican electricity sector fit., A partir de la reforma energética de 2013, el sector eléctrico en México adquirió una nueva fisonomía. Dicha reforma reestructuró la participación del Estado y de los particulares, en su trama normativa, técnica, económica y social, con tendencia a hacer más eficientes y seguros sus procesos, crear mercados, generar riqueza y mejorar los precios del consumidor. Le siguió una vorágine legislativa para adaptar a las dependencias y entidades del Estado a la nueva realidad, porque tanto en la reforma constitucional como en sus leyes secundarias se crearon nuevas instituciones y se reconfiguraron las antiguas, sin detenerse a verificar si su naturaleza coincidía con la organización de la administración pública, sin reparar en lo que la doctrina nacional decía al respecto. La presente investigación se posiciona en un nivel descriptivo de tipo documental. Al abordar el trazo jurídico, se encontró el andamiaje constitucional que rige la administración pública y su organización clasificada legalmente como centralizada y paraestatal. Retomando conceptos de juristas que sentaron las bases para las lecturas actuales, se analizó la naturaleza de las dependencias o entidades para advertir sus similitudes y discrepancias, proximidades y diferencias; para tratar de ubicarlas conforme a esas estructuras. En esa línea, destacan dos instituciones que no coincidieron en todos sus términos con la clasificación, pues abarcan características de varias categorías, por lo que se consideró inapropiado seguirlas ubicando en los conceptos tradicionales. Tomado esto en cuenta, se emprendió la búsqueda de una nueva categoría al interior de la clasificación de la organización de la administración pública que comprendiera la nueva realidad. Y se concluyó que mediante la administración pública transversal caben algunas de las instituciones atípicas del sector eléctrico mexicano.

Purpose: To report a rare case of non-tuberculous mycobacterial (NTM) choroiditis masquerading as choroidal tumour, where the initial diagnosis was masked by keratitis. Case description: A 57-year-old heroin chaser with a pre-existing left eye blindness due to past blunt trauma presented with diffuse bacterial keratitis on the same side. Systemic examination revealed multiple non-tender cervical lymphadenopathies. B-scan ultrasonography showed a hyperechoic choroidal mass with surrounding exudative retinal detachment, resembling a choroidal tumour. However, computed tomography (CT) and magnetic resonance imaging (MRI) scan findings were suggestive of inflammatory choroidal changes. Inflammatory markers were significantly raised and infective screening was positive for HIV and Hepatitis C. Tuberculosis workup was normal. In view of intractable pain, evisceration was done and his vitreous humour was sent for polymerase chain reaction (PCR). It was reported to be positive for Mycobacterium Fortuitum. Conclusion: NTM ocular infections are rare, challenging to diagnose, and potentially sight threatening. Early recognition and prompt treatment is life and vision saving.

Pemphigus during pregnancy has a more complicated course owing to the limitations in treatment options and alterations in the severity and presentation of the clinical features. We would like to present two pemphigus vulgaris (PV) cases associated with pregnancy with an unusual clinical appearance exhibiting polycyclic, annular, vesiculobullous plaques with marked eosinophil infiltration in histopathology. To the best of our knowledge pregnancy-associated pemphigus cases with this particular clinical presentation have not been reported in the literature. Changes in the immunologic and hormonal state during pregnancy may play a role in altering the classic clinical presentation and treatment response of PV.

Los personajes adolescentes tienen una notable presencia en las series de televisión actuales y, especialmente, ocupando roles principales. En su construcción intervienen una serie de estereotipos reconocibles, aunque presentan también ciertos rasgos de los problemas y situaciones del público adolescente al que se dirigen. De esta manera, facilitan el proceso de identificación con los espectadores. Con el objetivo de reflexio-nar sobre la estereotipada imagen de los adolescentes en la ficción seriada, poner en valor la Teen TV estadounidense actual y abordar el universo serial de Netflix, en este trabajo se analizará a dos protagonistas de famosas series de la plataforma de VOD: Hanna Baker, de Por Trece razones, víctima de bullying en su instituto, y Sam Gardner, de Atípico, un adolescente con trastorno del espectro autista. Para ello, se les aplicará una plantilla de análisis de personajes de carácter cualitativo como persona y como rol basada en las teorías de los autores Francesco Casetti y Federico Di Chio, con la finalidad de conocer las dimensiones iconográficas, psicológicas, sociológicas y sexuales, así como las motivaciones y acciones de los personajes., Teen characters have a relevant presence in current TV shows, and, especially, play-ing the leading roles. Their construction involves a number of recognizable stereotypes, but they also include some characteristics of the problems and situations which are familiar to the teenager audience. In that way, they facilitate the process of identification with the viewers. With the aim of reflecting on the stereotyped image of teenagers in serial fiction, highlighting the current American ‘Teen TV’ and addressing the ‘Netflix serial uiverse’, two main characters of two famous series from the VOD platform will be analyzed in this paper: Hannah Baker from 13 Reasons Why, a victim of bullying in her highschool, and Sam Gardner, from Atypical, a teenager with autism spectrum disorder. For that reason, a qualitative analysis template, based on Francesco Casetti and Federico Di Chio’s theories, will be applied as a person and as a role, with the aim of learning the iconographic, psychological, sociological and sexual dimensions, as well like the motivations and actions of the characters.

Pemphigus during pregnancy has a more complicated course owing to the limitations in treatment options and alterations in the severity and presentation of the clinical features. We would like to present two pemphigus vulgaris (PV) cases associated with pregnancy with an unusual clinical appearance exhibiting polycyclic, annular, vesiculobullous plaques with marked eosinophil infiltration in histopathology. To the best of our knowledge pregnancy-associated pemphigus cases with this particular clinical presentation have not been reported in the literature. Changes in the immunologic and hormonal state during pregnancy may play a role in altering the classic clinical presentation and treatment response of PV.

Los personajes adolescentes tienen una notable presencia en las series de televisión actuales y, especialmente, ocupando roles principales. En su construcción intervienen una serie de estereotipos reconocibles, aunque presentan también ciertos rasgos de los problemas y situaciones del público adolescente al que se dirigen. De esta manera, facilitan el proceso de identificación con los espectadores. Con el objetivo de reflexio-nar sobre la estereotipada imagen de los adolescentes en la ficción seriada, poner en valor la Teen TV estadounidense actual y abordar el universo serial de Netflix, en este trabajo se analizará a dos protagonistas de famosas series de la plataforma de VOD: Hanna Baker, de Por Trece razones, víctima de bullying en su instituto, y Sam Gardner, de Atípico, un adolescente con trastorno del espectro autista. Para ello, se les aplicará una plantilla de análisis de personajes de carácter cualitativo como persona y como rol basada en las teorías de los autores Francesco Casetti y Federico Di Chio, con la finalidad de conocer las dimensiones iconográficas, psicológicas, sociológicas y sexuales, así como las motivaciones y acciones de los personajes., Teen characters have a relevant presence in current TV shows, and, especially, play-ing the leading roles. Their construction involves a number of recognizable stereotypes, but they also include some characteristics of the problems and situations which are familiar to the teenager audience. In that way, they facilitate the process of identification with the viewers. With the aim of reflecting on the stereotyped image of teenagers in serial fiction, highlighting the current American ‘Teen TV’ and addressing the ‘Netflix serial uiverse’, two main characters of two famous series from the VOD platform will be analyzed in this paper: Hannah Baker from 13 Reasons Why, a victim of bullying in her highschool, and Sam Gardner, from Atypical, a teenager with autism spectrum disorder. For that reason, a qualitative analysis template, based on Francesco Casetti and Federico Di Chio’s theories, will be applied as a person and as a role, with the aim of learning the iconographic, psychological, sociological and sexual dimensions, as well like the motivations and actions of the characters.

A system can only be improved if it is measured. In order to adequately measure a system, that system needs to be mapped and all key inter-nodal linkages, constraints and pathways recorded. Commercial supply chains demonstrate similar characteristics to other systems. Much has been written about mapping supply systems, where typically, the product or service is tracked from the originating source such as a raw materials supplier to the end customer of the product such as the consumer. There is however, another classification of supply system, where the payment for the product or service is not undertaken by the end consumer. This supply system is more often associated with not-for-profit (NFP) and non-government organisation (NGO) activities and little has been written concerning the mapping of these atypical supply systems. This is unfortunate, as it is often these types of networks that are most assumed to be inefficient and lacking appropriate quality measures. This paper discusses the characteristics of atypical supply networks and also describes a method of mapping them by using an auditing approach based on tracking funding through the system and not the flow of products or services within it. We argue that this approach is robust, because it enables the actual flow patterns within the network to be identified and not confused with, often, conflicting demands placed on atypical supply networks by the multiple stakeholders often associated with them.

Los personajes adolescentes tienen una notable presencia en las series de televisión actuales y, especialmente, ocupando roles principales. En su construcción intervienen una serie de estereotipos reconocibles, aunque presentan también ciertos rasgos de los problemas y situaciones del público adolescente al que se dirigen. De esta manera, facilitan el proceso de identificación con los espectadores. Con el objetivo de reflexio-nar sobre la estereotipada imagen de los adolescentes en la ficción seriada, poner en valor la Teen TV estadounidense actual y abordar el universo serial de Netflix, en este trabajo se analizará a dos protagonistas de famosas series de la plataforma de VOD: Hanna Baker, de Por Trece razones, víctima de bullying en su instituto, y Sam Gardner, de Atípico, un adolescente con trastorno del espectro autista. Para ello, se les aplicará una plantilla de análisis de personajes de carácter cualitativo como persona y como rol basada en las teorías de los autores Francesco Casetti y Federico Di Chio, con la finalidad de conocer las dimensiones iconográficas, psicológicas, sociológicas y sexuales, así como las motivaciones y acciones de los personajes., Teen characters have a relevant presence in current TV shows, and, especially, play-ing the leading roles. Their construction involves a number of recognizable stereotypes, but they also include some characteristics of the problems and situations which are familiar to the teenager audience. In that way, they facilitate the process of identification with the viewers. With the aim of reflecting on the stereotyped image of teenagers in serial fiction, highlighting the current American ‘Teen TV’ and addressing the ‘Netflix serial uiverse’, two main characters of two famous series from the VOD platform will be analyzed in this paper: Hannah Baker from 13 Reasons Why, a victim of bullying in her highschool, and Sam Gardner, from Atypical, a teenager with autism spectrum disorder. For that reason, a qualitative analysis template, based on Francesco Casetti and Federico Di Chio’s theories, will be applied as a person and as a role, with the aim of learning the iconographic, psychological, sociological and sexual dimensions, as well like the motivations and actions of the characters.