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RESUMEN El páncreas anular es una malformación congénita rara caracterizada por un anillo pancreático que rodea la porción descendente del duodeno. Se reporta en 1/20 000 recién nacidos vivos. La edad de presentación depende de la severidad de la obstrucción intestinal. El diagnóstico requiere estudios de imagenología y exploración quirúrgica. Presentamos el caso de un neonato de trece días, hospitalizado en la Unidad de Cuidados Intensivos Neonatales del Hospital Nacional Arzobispo Loayza. Cursa con residuo gástrico, vómitos y distensión abdominal intermitente. Dada la persistencia de una sintomatología gastrointestinal a pesar del manejo instaurado, se realiza el examen radiológico de las vías digestivas altas con medio de contraste, donde se evidencia signos de estenosis duodenal. Se realizó una laparotomía exploratoria, con diagnóstico posoperatorio de páncreas anular. El tratamiento definitivo es mediante la cirugía de Kimura. ABSTRACT Annular pancreas is a rare congenital malformation characterized by a pancreatic ring surrounding the descending part of the duodenum. It occurs in 1:20,000 live births. The age of presentation depends on the severity of the intestinal obstruction. The diagnosis requires imaging studies and surgical exploration. This is the case of a 13-day-old newborn admitted to the Neonatal Intensive Care Unit of Hospital Nacional Arzobispo Loayza. He presented with gastric residuum, vomiting and intermittent abdominal distension. Given the persistent gastrointestinal symptoms despite the treatment administered, a radiological examination of the upper digestive tract with contrast dye was conducted. Signs of duodenal stenosis were found. An exploratory laparotomy showed a postoperative diagnosis of annular pancreas. Kimura’s diamondshaped side-to-side duodenoduodenal anastomosis was performed as a definitive treatment.

This paper presents an analysis of the fault ride-through requirements established in grid codes around the world, that conventional power plants should comply, in order to be able to be connected to the transmission network. It describes the causes of this requirement, the way it is currently applied in most electric utilities, and the effect that power plant auxiliaries have in the assessment of this important characteristic. It was found that auxiliaries have a very important effect in the fault ride-through capability of the power plant, and should therefore be explicitly represented in those cases. In addition, it was observed that, in the way this requirement is currently verified, it can be contradictory to stability criteria, and this requires improving the methods of verifying the fault ride-through capability of the plant, so as to conciliate this aspect with stability criteria.

Background To compare the patients’ and physician’s global assessment of disease activity in Behçet’s syndrome (BS) and investigate the frequency, magnitude, and determinants of potential discordance. Methods A total of 226 adult BS patients with a median (IQR) age of 46.9 (35.6–55.2) years were enrolled across Italy, Greece, Portugal, and Spain. Demographic, clinical, and therapeutic variables, as well as the patient reported outcomes, were collected at the recruitment visit. The physical (PCS) and mental (MCS) component summary scores of the Short Form Questionnaire 36 (SF-36) and the Behçet’s syndrome Overall Damage Index (BODI) were calculated. Disease activity was assessed by the patients’ (PtGA) and physician’s global assessment (PGA) in a 10-cm visual analog scale, as well as the Behçet Disease Current Activity Form (BDCAF). Discordance (∆) was calculated by subtracting the PGA from the PtGA and defined as positive (PtGA>PGA) and negative (PtGA Results Median PtGA and PGA scores were 2.0 (0.3–5.0) and 1.0 (0.0–3.0) cm, respectively. The discordance prevalence varied (from 29.6 to 55.3%) according to the cutoff applied, and the majority (> 80%) of disagreements were due to patients rating higher their disease activity. Higher values of BDCAF were associated to increased rate of positive discordance. When BDCAF = 0, the median (IQR) values of PtGA and PGA were 0.2 (0–2) and 0 (0–1), respectively. PCS (adjusted odds ratio (adjOR) 0.96 per unit, 95% CI 0.93–0.98, p = 0.006) and MCS (adjOR 0.96 per unit, 95% CI 0.93–0.99, p = 0.003) were independently associated with positive discordance using both cutoffs. Active ocular involvement emerged as a potential determinant of negative discordance (adjOR 5.88, 95% CI 1.48–23.30, p = 0.012). Conclusions PtGA and PGA should be considered as complementary measures in BS, as patients and physicians may be influenced by different factors when assessing active disease manifestations. Particularly, PtGA may be a useful tool in the assessment of BS disease activity, as it carries a low risk to misclassify an inactive disease, and may allow to capture aspects of the patient’s health that negatively affect his well-being and the treatment.

Visual Abstract, The medial prefrontal cortex (mPFC) processes contextual information from the hippocampus to generate appropriate fear responses. In rodents, one path for sending contextual information to the mPFC is via the direct projections from the ventral hippocampus (vHC) to the infralimbic cortex (IL). Plasticity in the synaptic communication from the vHC to the IL could contribute to the behavioral changes produced by the acquisition and extinction of conditioned fear. To examine this possibility, we used optogenetic stimulation of vHC synapses in brain slices from trained rats. We found that fear acquisition reduced NMDA receptor (NMDAR) currents at vHC synapses onto IL pyramidal neurons. The depression of NMDAR currents reversed more efficiently after extinction in the conditioning context than extinction in a novel context. Moreover, a cohort of animals that exhibited poor extinction retrieval failed to reverse the plasticity induced by fear conditioning. In addition, ex vivo application of brain-derived neurotrophic factor (BDNF), which is known to simulate extinction in IL, reversed this conditioning-induced plasticity mimicking extinction. Therefore, we have identified a novel mechanism that modulates conditioned fear via changes in NMDAR current at vHC synapses onto IL pyramidal neurons. Disruption of this mechanism could contribute to the abnormal contextual modulation of fear seen in posttraumatic stress disorder (PTSD).

ADP-ribosyl transferases are enzymes involved in the post-translational modification of proteins; they participate in multiple physiological processes, pathogenesis and host-pathogen interactions. Several reports have characterized the functions of these enzymes in viruses, prokaryotes and higher eukaryotes, but few studies have reported ADP-ribosyl transferases in lower eukaryotes, such as parasites. The locus EHI_155600 from Entamoeba histolytica encodes a hypothetical protein that possesses a domain from the ADP-ribosylation superfamily; this protein belongs to the diphtheria toxin family according to a homology model using poly-ADP-ribosyl polymerase 12 (PARP12 or ARTD12) as a template. The recombinant protein expressed in Escherichia coli exhibited in vitro ADP-ribosylation activity that was dependent on the time and temperature. Unlabeled βNAD+, but not ADP-ribose, competed in the enzymatic reaction using biotin-βNAD+ as the ADP-ribose donor. The recombinant enzyme, denominated EhToxin-like, auto-ADP-ribosylated and modified an acceptor from E. coli that was identified by MS/MS as the elongation factor Tu (EF-Tu). To the best of our knowledge, this is the first report to identify an ADP-ribosyl transferase from the diphtheria toxin family in a protozoan parasite. The known toxins from this family (i.e., the diphtheria toxin, the Pseudomonas aeruginosa toxin Exo-A, and Cholix from Vibrio cholerae) modify eukaryotic elongation factor two (eEF-2), whereas the amoeba EhToxin-like modified EF-Tu, which is another elongation factor involved in protein synthesis in bacteria and mitochondria.

To assess the efficacy of monoclonal anti-tumour necrosis factor (TNF)-α agents in patients with anterior uveitis (AU) in terms of decrease of recurrences, variation of visual acuity and steroid sparing effect and to identify any demographic, clinical or therapeutic variables associated with a sustained response to monoclonal TNF-α inhibitors.Data from patients suffering from AU treated with adalimumab, infliximab, golimumab or certolizumab pegol were retrospectively collected and statistically analysed.Sixty-nine patients (22 males, 47 females), corresponding to 101 eyes, were enrolled. The mean follow-up period was 29.25±23.51 months. The rate of ocular flares decreased from 42.03 events/100 patients/year recorded during the 12 months preceding the start of TNF-α inhibitors to 2.9 flares/100 patients/year after the start of treatment (p0.0001). The overall decrease in ocular flares was 93.1%. No statistically significant changes were identified in the best corrected visual acuity during the follow-up period (p0.99). The number of patients treated with corticosteroids at baseline was significantly higher compared with that referred to the 12-month evaluation (p0.001) and to the last follow-up visit (p=0.006). Concomitant treatment with conventional disease-modifying anti-rheumatic drugs (cDMARDs) represented the sole clinical, demographic or therapeutic variable associated with long-term treatment duration (p=0.045, R2=0.87).Monoclonal TNF-α inhibitors induce a remarkable decrease in the recurrence of AU during a long-term follow-up period and lead to a significant steroid sparing effect along with stabilisation of visual acuity. Concomitant treatment with cDMARDs represented the sole variable associated with treatment duration in the long-term.

To compare the efficacy of adalimumab (ADA) and infliximab (IFX) in patients with non-infectious intermediate uveitis, posterior uveitis, and panuveitis. Demographic, clinical, instrumental, and therapeutic data from patients enrolled were collected at the start of treatment, at 12-month follow-up, and at the last follow-up assessment. One hundred seven patients (46 females, 187 eyes) were enrolled, 66 (61.7%) treated with ADA and 41 (38.3%) with IFX. Bilateral involvement was observed in 80 cases. The mean follow-up was 26.45 ± 21.71 months for ADA patients and 56.60 ± 56.04 months for IFX patients. The overall decrease of uveitis frequency during the first 12 months of treatment was 66.7% in the IFX group and 84.2% in the ADA group, compared to the previous 12 months (p = 0.09). A significantly higher corticosteroid dosage was found among patients treated with ADA at the last follow-up visit (p = 0.008). The percentage of patients co-administered with corticosteroids was significantly higher among ADA patients both at the 12-month visit (p = 0.03) and at the last visit (p = 0.0004). The frequency of uveitic macular edema (UME) was significantly higher among patients treated with ADA compared to those treated with IFX at the 12-month assessment (p = 0.015) and at the last follow-up visit (p = 0.011); central macular thickness was significantly higher in ADA group compared to the IFX group at the last follow-up assessment (p = 0.04). ADA and IFX have shown a similar efficacy in controlling uveitis relapses, but IFX showed a more pronounced corticosteroid sparing effect and a significantly higher capacity in resolving UME compared to ADA.

Clinical research is needed to identify patients with axial spondyloarthritis (axSpA) who are more likely to be responsive to interleukin (IL)-17 inhibition.To evaluate short-term efficacy of secukinumab in the management of axSpA.Twenty-one patients (7 males, 14 females) with axSpA were consecutively treated with secukinumab. Laboratory and clinical assessments were based on erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), Ankylosing Spondylitis Disease Activity Score (ASDAS)-CRP, and Bath Ankylosing Spondylitis Disease Activity Index (BASDAI). Data were recorded at baseline and at a 3 month follow-up visit.The study was comprised of 21 patients. Both BASDAI and ASDAS-CRP showed a statistically significant reduction between the baseline and the 3 month visit (P0.0001 and P = 0.0005, respectively). During the laboratory assessment, ESR showed a significant decrease (P = 0.008) while CRP improvement did not reach statistical significance (P = 0.213). No statistical significance was observed between patients treated with secukinumab 150 mg vs. 300 mg in BASDAI (P=0.99), ASDAS-CRP (P = 0.69), ESR (P = 0.54), and CRP (P = 0.56). No significant differences emerged between the BASDAI (P = 0.15), ASDAS-CRP (P = 0.09), and CRP (P = 0.15) rates in biologic-naïve patients and those previously failing tumor necrosis factor-α inhibition. Conversely, ESR decrease was significantly higher in the biologic-naïve subgroup (P = 0.01). No adverse events were reported.Secukinumab has proven remarkable short-term effectiveness, regardless of the biologic treatment line. A dosage of 150 mg proved to be appropriate in the clinical and laboratory management of axSpA.

A maximum power point tracking (MPPT) scheme based on a general pulse width modulation (PWM) modulator is presented. The purpose of the scheme is that switching converter behaves as a resistor seen by the photovoltaic module, it also regulates the input voltage to a desired value. The MPPT scheme is applied to a noncascading converter topology, which has the characteristic of processing a fraction of the total power generated.

Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenopathy (PFAPA) syndrome is a non-Mendelian autoinflammatory disorder until now considered to be specifically limited to paediatric age. Recently, an increasing number of reports seems to suggest that PFAPA syndrome, diagnosed by the Marshall criteria revised by Thomas et al., can also affect adults.The Marshall/Thomas criteria have been applied to 989 adult patients presenting for recurrent fever episodes: all patients enrolled were reviewed for demographic, clinical, and therapeutic data. Infectious, neoplastic, autoimmune and other autoinflammatory diseases were ruled out.We identified 30 adult patients (19 males, 11 females) with a suspected PFAPA syndrome: their mean age at disease onset was 33.75±14.01 years, mean age at diagnosis 39.1±14.39 years, and mean body temperature peak 39.5±0.7°C. In addition, the mean frequency of febrile episodes was 11.58±8.97 per year. More precisely, patients complained of pharyngitis (77%), cervical adenitis (73%), asthenia (63%), arthralgia (67%), oral aphthosis (50%), myalgia (54%), cephalalgia (43%), abdominal pain (27%), nausea/vomiting (17%), periorbital pain (17%), and arthritis (10%). Six out of 30 (20%) patients had suffered from PFAPA syndrome also during childhood, and the disease had reappeared in adulthood.We provide the largest monocentric cohort of patients diagnosed with a suspected PFAPA syndrome in adulthood confirming that this syndrome can occur also during adulthood; moreover, due to the medical history of our patients and based on our experience, PFAPA syndrome might relapse during adulthood after a temporary remission reached in the course of paediatric age.

Sir, A 62-year-old Caucasian man was referred to us with multiple erythematous arciform lesions with a wood-grain-like appearance of fifteen months' evolution. The dermatosis had initially affected the patient's legs, extending to his abdomen and arms. His medical history included rheumatoid arthritis, treated with systemic corticosteroids; DMARDs (methotrexate) with folic acid supplements; omeprazole; and cardioaspirin. Interestingly, the resolution of rheumatoid arthritis with various DMARDs was related to the resolution of the dermatosis. Physical examination disclosed an eruption on the legs characterized by irregular, multiple, gyrate bands of erythema bordered on one side by scale and on the other by normal-looking skin. The lesions migrated, leaving a thin scale at the edges and creating a bizarre pattern [Figure 1]. The dermatosis exhibited characteristic rapid movement over hours. Pruritus was moderate. The patient had previously taken oral corticosteroids which had been partially successful, but his condition had worsened rapidly when treatment was interrupted. Histopathological findings were non-specific, showing evidence of hyperkeratosis, orthokeratosis, and a superficial, perivascular, mononuclear infiltrate, which although non-diagnostic, were consistent with changes described for erythema gyratum repens (EGR) [Figure 2]. Several case reports have shown positive direct immunofluorescence (DIF) in the skin: DIF tests on the skin describe deposits of IgG and C3 along the basement membrane zone. [1] However, DIF of involved non-sun-exposed and uninvolved skin was negative in our patient. Figure 1: Clinical presentation on admission Click here to view Figure 2: Histological features of erythema gyratum repens (EGR) included hyperkeratosis, orthokeratosis, and a perivascular inflammatory infiltrate (H and E, ×200) Click here to view Clinical findings were consistent with EGR. The patient had a family history of esophageal cancer, so the search for an associated neoplasm began. Laboratory tumor markers were all normal. Thirty-two months after the onset of EGR, investigations (chest X-ray, gastroscopy, colonoscopy, abdominal ultrasound, computerized tomography of the chest, abdomen and pelvis, and whole body PET-CT) still proved normal. The presentation of the skin disorder was so distinctive that a differential diagnosis was difficult to generate. In our patient, the differential diagnoses included erythema annulare centrifugum (EAC), urticarial vasculitis, and systemic lupus erythematosus. Distinction between EGR and EAC is very difficult, since clinical findings are similar; the histopathologic pictures of both diseases are nonspecific and both can be associated with malignancy (EGR more often). However, one of the differences between them is in the rapidity of movement. EGR exhibits characteristic rapid movement over minutes and hours; EAC moves slowly over days and months. The gyrate erythema presented in this case had clinical features similar to both EGR and EAC, but the rapidity of movement was more consistent with EGR. The differential diagnosis between EGR and urticarial vasculitis was made histopathologically. Urticarial vasculitis typically shows leukocytoclastic vasculitis. Classic symptoms of urticarial vasculitis, such as painful burning sensation, residual purpura, and pigmentation, were not present. Scaling trailing the leading edge of the eruption, which is one of the typical findings of EGR, was present in our case. The patient was studied for tuberculosis; chest X-ray, CT, Mantoux test, and Quantiferon test were all negative. EGR is a rare specific dermatosis, believed to be an obligate paraneoplastic cutaneous syndrome. In more than 80% of cases, EGR is associated with an underlying malignancy and in 80% of cases, this distinctive migratory eruption appears prior to a malignancy diagnosis. EGR can precede 1 to 72 months before malignancy, though the average is 4 to 9 months. Approximately, one-third of patients will manifest lung cancer, 8% esophageal cancer, and 6% an unknown primary. Tumors of the breast, bladder, prostate, cervix, and uterus have also been reported. [2] Here, however, we report a case of EGR which is non-paraneoplastic. Case reports demonstrating an association with non-neoplastic diseases have also been reported, such as pulmonary tuberculosis, drug use, hypereosinophilic syndrome, ichthyosis, autoimmune conditions such as CREST syndrome [3] or disorders with an EGR-like presentation such as tinea imbricata, lupus erythematosus, bullous diseases, resolving pityriasis rubra pilaris, [4] psoriasis, and Sjφgren syndrome. [5] This association may either be an example of an extremely unusual clinical presentation of the primary dermatosis in question, or may be a case of "true" EGR as observed during the course of other non-neoplastic conditions. Healthy patients with this distinctive eruption have been reported in literature, which might cast doubt on the reliability of the diagnosis. To our knowledge, no case of EGR associated with rheumatoid arthritis has, to date, been described in literature, making our case the first reported description of its kind. Moreover, a parallel evolution of the two affections (EGR and rheumatoid arthritis) was observed in our patient. In conclusion, our experience shows that EGR does not necessarily indicate underlying malignancy, but it can be associated with a non-neoplastic disease such as rheumatoid arthritis. References 1. Caux F, Lebbe C, Thomine E, Benyahia B, Flageul B, Joly P, et al. Erythema gyratum repens: A case studied with immunofluorescence, immunoelectronmicroscopy and immunohistochemistry. Br J Dermatol 1994;131:102-7. 2. Hauschild A, Swensson O, Christophers E. Paraneoplastic bullous pemphigoid resembling erythema gyratum repens. Br J Dermatol 1999;140:550-2. 3. Demonchy E, Lacour JP, Ortonne JP, Passeron T. Erythema gyratum repens, not always a bad omen for patients. J Eur Acad Dermatol Venereol 2010;24:738-9. 4. Almaani N, Robson A, Sarkany R, Griffiths WA. Erythema gyratum repens associated with pityriasis rubra pilaris. Clin Exp Dermatol 2011;36:161-4. 5. Matsumura T, Kumakiri M, Sato-Matsumura KC, Ohkawara A. Erythema gyratum repens-like eruption in a patient with Sjögren syndrome. Acta Derm Venereol 1995;75:327.

Ante el evidente crecimiento de la población mayor de 60 años y la escasez de personal idóneo para atender sus múltiples demandas a corto y mediano plazo, un grupo de profesionales de diferentes disciplinas e instituciones, decidieron organizarse en Yucatán, México, en 1999, bajo los auspicios de la Facultad de Medicina de la Universidad Autónoma de Yucatán y de la Rectoría de la Universidad Mesoamericana de San Agustín, en una Comisión para la elaboración del plan de estudios de la Licenciatura en Gerontología. Se hace un bosquejo del panorama local gerontológico y se describe la constitución subsecuente, en el año 2000, del Comité Interinstitucional Para el Estudio Gerontológico en Yucatán (CIPEGY) que dio origen a la impartición de los primeros diplomados universitarios en gerontología y a la difusión de una cultura sobre el problema del envejecimiento y de propuestas para afrontarlo. Se mencionan los nombres de los promotores iniciales de la profesionalización de la gerontología en Yucatán.

Objectives: The U.L.I.S.S.E. study is aimed at describing older patients who are cared for in hospitals, home care or nursing homes in Italy. Design: The U.L.I.S.S.E. study is an observational multicenter prospective 1-year study. Setting: Overall, 23 acute geriatric or internal medicine hospital units, 11 home care services and 31 nursing homes participated in the study. Measurements: The patient’s evaluation was performed using comprehensive geriatric assessment instruments, i.e. the interRAI Minimum Data Set, while data on service characteristics were recorded using ad-hoc designed questionnaires. Results: The older subjects who are in need of acute and long term care in Italy have similar characteristics: their mean age is higher than 80 years, they have a high level of disability in ADL, an important multimorbidity, and are treated with several drugs. The prevalence of cognitive impairment is particularly high in nursing homes, where almost 70% of residents suffer from it and 40% have severe cognitive impairment. On the other hand, there is a shortage of health care services, which are heterogeneous and fragmented. Conclusions: Health care services for older people in Italy are currently inadequate to manage the complexity of the older patients. An important effort should be undertaken to create a more integrated health care system.

We report the successful use of high-frequency oscillatory ventilation without recruitment maneuvers in a patient with ARDS and severe hypoxemia who developed a pneumomediastinum during conventional mechanical ventilation. High-frequency ventilation is an attractive modality for the treatment of pneumomediastinum, especially when the airway pressures cannot be reduced by variation in the settings of the conventional mechanical ventilator.