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Phenotypic plasticity and rapid evolution are fundamental processes by which organisms can maintain their function and fitness in the face of environmental changes. Here we quantified the plasticity and evolutionary potential of an alpine herb Wahlenbergia ceracea. Utilising its mixed-mating system, we generated outcrossed and self-pollinated families that were grown in either cool or warm environments, and that had parents that had also been grown in either cool or warm environments. We then analysed the contribution of environmental and genetic factors to variation in a range of phenotypic traits including phenology, leaf mass per area, photosynthetic function, thermal tolerance, and reproductive fitness. The strongest effect was that of current growth temperature, indicating strong phenotypic plasticity. All traits except thermal tolerance were plastic, whereby warm-grown plants flowered earlier, grew larger, produced more reproductive stems compared to cool-grown plants. Flowering onset and biomass were heritable and under selection, with early flowering and larger plants having higher relative fitness. There was little evidence for transgenerational plasticity, maternal effects, or genotype-by-environment interactions. Inbreeding delayed flowering and reduced reproductive fitness and biomass. Overall, we found that W. ceracea has the capacity to respond rapidly to climate warming via plasticity, and the potential for evolutionary change.
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BACKGROUND: COVID-19 pandemic is thought to have changed the epidemiology of some pediatric neurosurgical disease: among them are the intracranial complications of sinusitis and otitis (ICSO). According to some studies on a limited number of cases, both streptococci-related sinusitis and ICSO would have increased immediately after the pandemic, although the reason is not clear yet (seasonal changes versus pandemic-related effects). The goal of the present survey of the European Society for Pediatric Neurosurgery (ESPN) was to collect a large number of cases from different European countries encompassing the pre-COVID (2017-2019), COVID (2020-2021), and post-COVID period (2022-June 2023) looking for possible epidemiological and/or clinical changes. MATERIAL AND METHODS: An English language questionnaire was sent to ESPN members about year of the event, patient's age and gender, presence of immune-deficit or other favoring risk factors, COVID infection, signs and symptoms at onset, site of primary infection, type of intracranial complication, identified germ, type and number of surgical operations, type and duration of medical treatment, clinical and radiological outcome, duration of the follow-up. RESULTS: Two hundred fifty-four cases were collected by 30 centers coming from 14 different European countries. There was a statistically significant difference between the post-COVID period (129 children, 86 cases/year, 50.7% of the whole series) and the COVID (40 children, 20 cases/year, 15.7%) or the pre-COVID period (85 children, 28.3 cases/year, 33.5%). Other significant differences concerned the presence of predisposing factors/concurrent diseases (higher in the pre-COVID period) and previous COVID infection (higher in the post-COVID period). No relevant differences occurred as far as demographic, microbiological, clinical, radiological, outcome, morbidity, and mortality data were concerned. Paranasal sinuses and middle ear/mastoid were the most involved primary site of infection (71% and 27%, respectively), while extradural or subdural empyema and brain abscess were the most common ICSO (73% and 17%, respectively). Surgery was required in 95% of cases (neurosurgical and ENT procedure in 71% and 62% of cases, respectively) while antibiotics in 99% of cases. After a 12.4-month follow-up, a full clinical and radiological recovery was obtained in 85% and 84% of cases, respectively. The mortality rate was 2.7%. CONCLUSIONS: These results suggest that the occurrence of ICSO was significantly increased after the pandemic. Such an increase seems to be related to the indirect effects of the pandemic (e.g., immunity debt) rather than to a direct effect of COVID infection or to seasonal fluctuations. ICSO remain challenging diseases but the pandemic did not affect the management strategies nor their prognosis. The epidemiological change of sinusitis/otitis and ICSO should alert about the appropriate follow-up of children with sinusitis/otitis.
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Absceso Encefálico , COVID-19 , Empiema Subdural , Otitis , Sinusitis , Niño , Humanos , Pandemias , COVID-19/complicaciones , Absceso Encefálico/epidemiología , Empiema Subdural/etiología , Sinusitis/complicaciones , Otitis/complicaciones , Otitis/epidemiología , Estudios RetrospectivosRESUMEN
BACKGROUND: Long-term changes in exercise capacity and cardiopulmonary hemodynamics after pulmonary endarterectomy (PEA) for chronic thromboembolic pulmonary hypertension (CTEPH) have been poorly described. METHODS: We analyzed the data from 2 prospective surgical CTEPH cohorts in Hammersmith Hospital, London, and Amsterdam UMC. A structured multimodal follow-up was adopted, consisting of right heart catheterization, cardiac magnetic resonance imaging, and cardiopulmonary exercise testing before and after PEA. Preoperative predictors of residual pulmonary hypertension (PH; mean pulmonary artery pressure >20 mm Hg and pulmonary vascular resistance ≥2 WU) and long-term exercise intolerance (VO2max <80%) at 18 months were analyzed. RESULTS: A total of 118 patients (61 from London and 57 from Amsterdam) were included in the analysis. Both cohorts displayed a significant improvement of pulmonary hemodynamics, right ventricular (RV) function, and exercise capacity 6 months after PEA. Between 6 and 18 months after PEA, there were no further improvements in hemodynamics and RV function, but the proportion of patients with impaired exercise capacity was high and slightly increased over time (52%-59% from 6 to 18 months). Long-term exercise intolerance was common and associated with preoperative diffusion capacity for carbon monoxide (DLCO), preoperative mixed venous oxygen saturation, and postoperative PH and right ventricular ejection fraction (RVEF). Clinically significant RV deterioration (RVEF decline >3%; 5 [9%] of 57 patients) and recurrent PH (5 [14%] of 36 patients) rarely occurred beyond 6 months after PEA. Age and preoperative DLCO were predictors of residual PH post-PEA. CONCLUSIONS: Restoration in exercise tolerance, cardiopulmonary hemodynamics, and RV function occurs within 6 months. No substantial changes occurred between 6 and 18 months after PEA in the Amsterdam cohort. Nevertheless, long-term exercise intolerance is common and associated with postoperative RV function.
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Hipertensión Pulmonar , Embolia Pulmonar , Humanos , Tolerancia al Ejercicio , Embolia Pulmonar/complicaciones , Embolia Pulmonar/cirugía , Volumen Sistólico , Estudios Prospectivos , Función Ventricular Derecha , Hemodinámica , Endarterectomía/métodos , Arteria Pulmonar/cirugía , Enfermedad CrónicaRESUMEN
INTRODUCTION: Ependymomas in the posterior fossa have poor prognosis. This study reports a single-center pediatric series, focusing on the value of surgical resection. MATERIAL AND METHODS: A single-center retrospective study included all patients operated on by the senior author (CM) for posterior fossa ependymoma from 2002 to 2018. Medical and surgical data were extracted from the hospital's medical database. RESULTS: Thirty-four patients were included. Age ranged from 6 months to 18 years, with a median of 4.7 years. Fourteen patients underwent initial endoscopic third ventriculocisternostomy before the direct surgical resection. Surgical removal was complete in 27 patients. There were 32 surgeries for second-look, local recurrence or metastasis despite complementary chemotherapy and/or radiotherapy. Twenty patients were WHO grade 2 and 14 grade 3. Sixteen patients showed recurrence (47%). Overall survival was 61.8% at a mean 10.1 years' follow-up. Morbidities comprised facial nerve palsy, swallowing disorder, and transient cerebellar syndrome. Fifteen patients had normal schooling, 6 had special assistance; 4 patients reached university, 3 of whom experienced difficulties. Three patients had a job. CONCLUSION: Posterior fossa ependymomas are aggressive tumors. Complete surgical removal is the most important prognostic factor, despite risk of sequelae. Complementary treatment is mandatory, but no targeted therapy has so far proved effective. It is important to continue the search for molecular markers in order to improve outcomes.
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Ependimoma , Niño , Humanos , Lactante , Estudios Retrospectivos , Ependimoma/diagnóstico , Ependimoma/cirugía , Terapia Combinada , Progresión de la EnfermedadRESUMEN
INTRODUCTION: Treatment with biologics has significantly reduced the social and economic burden of severe asthma. However, some patients may still feature a suboptimal control of their symptoms while on therapy. In this subset of asthmatic patients, a benefit from a dual biologic therapy has sporadically been reported in literature. Our aim is to add our experience to the limited body of evidence supporting combination biologic therapies. CASE STUDY: Here we present the case of a 68-year-old nonsmoker female, with an allergic and eosinophilic corticosteroid-dependent severe asthma. She displayed well controlled comorbidities and good adherence to the inhaled therapy. Omalizumab was started in 2008 with an initial remarkable clinical improvement. After nine years of biologic therapy, she reported a gradual worsening of her symptoms and exacerbations. Mepolizumab was then added in 2019. RESULTS: The addition of Mepolizumab resulted in a meaningful amelioration of her quality of life, asthma control, number of exacerbations and 6-minute-walking-distance at 3-year follow-up. The average Prednisone dosage was tapered from 25 mg to 20 mg daily. No adverse events were observed since the introduction of the second biologic. CONCLUSION: Our experience indicates that Mepolizumab may be beneficial and safe as an add-on biologic in a patient whose allergic and eosinophilic asthma remains uncontrolled despite treatment with an anti-IgE strategy. Further studies on a larger number of patients are required to demonstrate whether the positive outcomes published so far are replicable on a larger scale.
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Antiasmáticos , Asma , Humanos , Femenino , Anciano , Asma/tratamiento farmacológico , Asma/inducido químicamente , Calidad de Vida , Omalizumab/uso terapéutico , Terapia BiológicaRESUMEN
BACKGROUND: Vitamin D (VitD) deficiency has been reported to be associated with respiratory tract infection. In this work we evaluated the concentration of VitD in COVID-19 patients experiencing acute respiratory infections of different levels of severity excluding those who underwent invasive respiratory support. METHODS: The levels of serum VitD and C-reactive protein (CRP) were analyzed in 118 consecutive hospitalized COVID-19 patients (74 male, 44 female), confirmed with rRT-PCR. Of these patients with ventilation support 52 (44.1%) received oxygen via nasal cannula, oxygen mask or an oxygen mask with a reservoir, 48 (40.7%) were on a continuous positive airway pressure device (CPAP) and 18 (15,3%) on non-invasive mechanical ventilation (NIMV). RESULTS: The median values (range) of VitD and of CRP were 15.1 ng/mL (1.3-73.3) and 14.2 mg/L (5.0-151.2), respectively. A negative correlation from VitD levels and those of CRP (correlation coefficient: 0.259: P=0.005) was observed. VitD levels in O
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COVID-19 , Ventilación no Invasiva , Deficiencia de Vitamina D , Humanos , Masculino , Femenino , SARS-CoV-2 , Respiración Artificial , Vitamina D , Vitaminas , Oxígeno , Deficiencia de Vitamina D/diagnósticoRESUMEN
The Agouti gene (ASIP) is one of the most important genes for coat color determination in mammals. It has a complex structure with several promoters and alternative non-coding first exons that are transcribed into mRNAs with different 5'UTR. These mRNA isoforms regulate the temporal and spatial expression of the gene, producing diverse pigmentation patterns. Here, we studied ASIP transcriptional variants and their expression in the skin of llamas with different coat color phenotypes. We also described the ASIP locus, including promoter usage and the splicing events that originate each transcript variant. Using 5'RACE-PCR we isolated seven ASIP transcripts with alternative 5'UTR, where exons 1A, 1A', 1C, 1D, and a novel non-coding exon 1A" were identified. Additionally, new alternative spliced forms were found. The diversity of ASIP 5'UTRs is originated by a complex pattern of alternative promoter usage, multiple transcription start sites and splicing events that include exon skipping and alternative 3' splicing site selection. We found that ASIP was highly expressed in llamas with white and brown phenotypes while black animals presented very low expression. The main responsible for this difference was a fusion transcript between ASIP and NCOA6 genes, which was present in the skin of white and brown llamas but not in the black ones. The rest of ASIP transcripts presented very low expression in the skin, indicating that the main regulation point for ASIP gene expression is at the transcriptional level. Nevertheless, the characteristics of the 5'UTRs sequences suggest that alternative transcripts could be regulated differently at the protein synthesis level.
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Regiones no Traducidas 5' , Proteína de Señalización Agouti/genética , Camélidos del Nuevo Mundo/genética , Pigmentación/genética , Empalme Alternativo , Animales , Camélidos del Nuevo Mundo/fisiología , Exones , Expresión Génica , Fenotipo , Regiones Promotoras Genéticas , Pigmentación de la Piel/genéticaRESUMEN
Trigonocephaly is a craniosynostosis characterized by a premature fusion of the metopic suture associating a characteristic triangular head shape, with a frontal medial crest and hypotelorism. Various techniques have been described for its surgical treatment which is usually performed during the first year of life. However, there might be cases with a late referral, after the age of one year. One of the technical issues with a fronto-orbital advancement surgery in a child over one year of age is that there is a significant risk of persistent residual bone defects. This article describes a surgical technique of crenellated fronto-orbital advancement for correcting trigonocephaly in children over one year of age, allowing to reduce residual bone defects.
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Craneosinostosis , Niño , Suturas Craneales , Craneosinostosis/cirugía , Hueso Frontal/cirugía , Humanos , Lactante , Órbita/diagnóstico por imagen , Órbita/cirugíaRESUMEN
OBJECTIVES: Gastrointestinal manifestations of coronavirus disease 19 (COVID-19) have been well established, but pancreatic involvement is under debate. Our aims were to evaluate the presence of acute pancreatitis in COVID-19 patients and to assess the frequency of pancreatic hyperenzymemia. METHODS: From April 1, 2020, to April 30, 2020, 110 consecutive patients (69 males, 41 females; mean age, 63.0 years; range, 24-93 years) met these criteria and were enrolled in the study. The clinical data and serum activity of pancreatic amylase and lipase were assayed in all patients using commercially available kits. RESULTS: None of the patients studied developed clinical signs or morphological alterations compatible with acute pancreatitis. However, it was found that 24.5% of the patients had amylase values above 53 IU/L and 16.4% had lipase values above 300 IU/L. Only 1 patient (0.9%) had both amylase and lipase values in excess of 3-fold the upper normal limit without clinical signs of pancreatitis. CONCLUSIONS: The presence of pancreatic hyperenzymemia in a patient with COVID-19 requires the management of these patients be guided by clinical evaluation and not merely by evaluation of the biochemical results.
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Amilasas/sangre , COVID-19/complicaciones , Pruebas Enzimáticas Clínicas , Lipasa/sangre , Enfermedades Pancreáticas/diagnóstico , Pancreatitis/diagnóstico , Adulto , Anciano , Anciano de 80 o más Años , Biomarcadores/sangre , COVID-19/diagnóstico , Diagnóstico Diferencial , Femenino , Humanos , Masculino , Persona de Mediana Edad , Enfermedades Pancreáticas/sangre , Enfermedades Pancreáticas/etiología , Pancreatitis/sangre , Pancreatitis/etiología , Valor Predictivo de las Pruebas , Pronóstico , Regulación hacia Arriba , Adulto JovenRESUMEN
Reasons why significant prostate cancer is still missed in early stage were investigated at the 22nd National SIEUN (Italian Society of integrated diagnostic in Urology, Andrology, Nephrology) congress took place from 30th November to 1st December 2020, in virtual modality. Even if multiparametric magnetic resonance (MR) has been introduced in the clinical practice several, limitations are emerging in patient with regular digital rectal examination (DRE) and serum prostate specific antigen (PSA) levels approaching the normal limits. The present paper summarizes highlights observed in those cases where significant prostate cancer may be missed by PSA or imaging and DRE. The issue of multidisciplinary interest had been subdivided and deepened under four main topics: biochemical, clinical, pathological and radiological point of view with a focus on PI-RADS 3 lesions.
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Tacto Rectal , Imágenes de Resonancia Magnética Multiparamétrica , Antígeno Prostático Específico/sangre , Neoplasias de la Próstata/sangre , Neoplasias de la Próstata/diagnóstico , Humanos , Italia , Masculino , Guías de Práctica Clínica como Asunto , Neoplasias de la Próstata/diagnóstico por imagen , Sociedades Médicas , UrologíaRESUMEN
INTRODUCTION: Modern approach for the treatment of posterior fossa medulloblastomas remains a challenge for pediatric neurosurgeons and pediatric oncologists and requires a multidisciplinary approach to optimize survival and clinical results. MATERIAL AND METHODS: We report the surgical principles of the treatment of posterior fossa medulloblastomas in children and how to avoid technical mistakes especially in very young patients. We also report our experience in a series of 64 patients operated from a medulloblastoma between 2000 and 2018 in Lyon. RESULTS: All patients had a craniospinal MRI. Eighty-one percent of the patients (n=50) had strictly midline tumor while 19% (n=14) had lateralized one. Eleven percent (n=7) had metastasis at diagnosis on the initial MRI. Forty-one percent (n=29) had an emergency ETV to treat hydrocephaly and the intracranial hypertension. All patient underwent a direct approach and a complete removal was achieved in 78% (n=58) of the cases on the postoperative MRI realized within 48h postsurgery. Histological findings revealed classical medulloblastoma in 73% (n=46), desmoplastic medulloblastoma in 17% (n=11) and anaplastic/large cell medulloblastoma in 10% (n=7). Patients were classified as low risk in 7 cases, standard risk in 30 cases and high risk in 27 cases. Ninety-six percent (n=61) of the patient received radiotherapy. Seventy-six percent (n=48) received pre-irradiation or adjuvant chemotherapy. At last follow-up in December 2018, 65% (n=41) of the patient were in complete remission, 12% (n=8) were in relapse and 27% (n=15) had died from their disease. The overall survival at five , ten and fifteen years for all the series was of 76%, 73% and 65.7% respectively. CONCLUSIONS: Medulloblastomas remain a chimiosensible and radiosensible disease and the complete surgical removal represents a favorable prognostic factor. The extension of surgery has also to be weighted in consideration of the new biomolecular and genetic knowledge that have to be integrated by surgeons to improve quality of life of patients.
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Neoplasias Cerebelosas/epidemiología , Neoplasias Cerebelosas/cirugía , Fosa Craneal Posterior/cirugía , Meduloblastoma/epidemiología , Meduloblastoma/cirugía , Adolescente , Neoplasias Cerebelosas/diagnóstico por imagen , Quimioterapia Adyuvante/métodos , Niño , Preescolar , Fosa Craneal Posterior/diagnóstico por imagen , Femenino , Francia/epidemiología , Humanos , Lactante , Masculino , Meduloblastoma/diagnóstico por imagen , Estadificación de Neoplasias/métodos , Calidad de VidaRESUMEN
INTRODUCTION: The premature fusion of the metopic suture may be associated with the presence of emissary veins (EV) and abnormally large pericerebral cerebrospinal fluid (CSF) spaces which suggest an associated focal disturbance in CSF dynamics. The incidence of such findings and their potential significance in terms of management of the disease have not been fully elucidated. The aim of this study is to investigate whether these phenomena identify specific subtypes of trigonocephaly. In such a direction, we evaluated the volume of the pericerebral CSF spaces and their relationship to the morphology ("Ω," "V," or flat type) of the prematurely fused metopic suture and to the value of the interfrontal angle value on the grounds of computed tomographic (CT) scan examinations. METHOD: The preoperative brain CT scans of 74 children (52 boys, 22 girls) with trigonocephaly who had undergone fronto-orbital remodeling were evaluated. The volume of the pericerebral CSF spaces and the value of the interfrontal angle were calculated. The type of intracranial notch was studied and classified according to its shape on the preoperative CT scan: a groove "Ω," a ridge/"V" ridge or absent when flat and evidence of emissary veins related to the abnormally fused suture. RESULTS: Preoperatively, an endocranial metopic groove or ridge was seen in 70% of the children. Emissary veins were identified in 34 of 74 patients (45%), at a mean distance of 2.04 cm (1.18-2.94 cm) from the nasion. The presence of large pericerebral CSF spaces significantly correlated with the presence of EV (p < 0.05), with the "Ω" type (p < 0.05) and with interfrontal angles under 134° (p < 0.005). CONCLUSIONS: Metopic suture early fusion shows an association between EV, pericerebral CSF spaces, and the "Ω" groove appearance of the suture. This association identifies a specific subgroup in which the presence of emissary veins and large pericerebral CSF spaces is an indicator of local venous hypertension due to the sagittal sinus constriction within an osseous groove created by the abnormal suture fusion process. The implications for the surgical management and long-term results as compared to trigonocephalic children with small or absent normal peripheral spaces and EV are still to be determined.
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Craneosinostosis , Niño , Suturas Craneales/diagnóstico por imagen , Femenino , Humanos , Lactante , Masculino , Cráneo , Suturas , Tomografía Computarizada por Rayos XRESUMEN
Understanding plant thermal tolerance is fundamental to predicting impacts of extreme temperature events that are increasing in frequency and intensity across the globe. Extremes, not averages, drive species evolution, determine survival and increase crop performance. To better prioritize agricultural and natural systems research, it is crucial to evaluate how researchers are assessing the capacity of plants to tolerate extreme events. We conducted a systematic review to determine how plant thermal tolerance research is distributed across wild and domesticated plants, growth forms and biomes, and to identify crucial knowledge gaps. Our review shows that most thermal tolerance research examines cold tolerance of cultivated species; c. 5% of articles consider both heat and cold tolerance. Plants of extreme environments are understudied, and techniques widely applied in cultivated systems are largely unused in natural systems. Lastly, we find that lack of standardized methods and metrics compromises the potential for mechanistic insight. Our review provides an entry point for those new to the methods used in plant thermal tolerance research and bridges often disparate ecological and agricultural perspectives for the more experienced. We present a considered agenda of thermal tolerance research priorities to stimulate efficient, reliable and repeatable research across the spectrum of plant thermal tolerance.
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Frío , Calor , Cambio Climático , Ecosistema , Fotosíntesis , TemperaturaAsunto(s)
Antipsicóticos/uso terapéutico , Trastorno de Personalidad Limítrofe/tratamiento farmacológico , Clozapina/uso terapéutico , Narcóticos/administración & dosificación , Oxicodona/administración & dosificación , Trastornos Psicóticos/tratamiento farmacológico , Ideación Suicida , Prevención del Suicidio , Administración Sublingual , Adulto , Trastorno de Personalidad Limítrofe/diagnóstico , Trastorno de Personalidad Limítrofe/psicología , Femenino , Humanos , Trastornos Psicóticos/diagnóstico , Trastornos Psicóticos/psicología , Suicidio/psicología , Resultado del TratamientoAsunto(s)
COVID-19 , Neurocirugia/tendencias , Pandemias , Pediatría/tendencias , Niño , Francia , HumanosRESUMEN
BACKGROUND: Debilitating symptoms of schizophrenia often persist after sustained treatment with atypical antipsychotics. To date, clozapine has been the most effective of the atypical antipsychotics; however, negative symptoms may persist, indicating a critical need to develop augmenting treatment approaches. METHODS: A retrospective chart review evaluated outcomes for 5 young adult inpatients with treatment-resistant schizophrenia who were prescribed off-label oxytocin (OT; 10 IU/sublingual, 1 time per day, to 20 IU/sublingual, 3 times per day) after their therapeutic response to clozapine plateaued (dose range: 200 to 600 mg). The augmented treatment was well tolerated and continued for at least 1 year after discharge from the hospital, with continued outpatient follow-up by the treating psychiatrist. Evaluation included the Positive and Negative Syndrome Scale and clinical review based on both self and parent/guardian reports. RESULTS: The augmentation of clozapine with sublingual OT in young adults with treatment-resistant schizophrenia appeared to reduce negative symptoms, maintain lowered positive symptoms, and increase occupational and social functioning (eg, return to work or school), as noted by family members. CONCLUSIONS: Future controlled, prospective studies should investigate the possibility that OT can significantly reduce negative symptoms of chronic psychotic illnesses that are inadequately responsive to clozapine or other antipsychotic medications alone.
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Antipsicóticos/uso terapéutico , Clozapina/uso terapéutico , Oxitocina/uso terapéutico , Esquizofrenia/tratamiento farmacológico , Adulto , Escalas de Valoración Psiquiátrica Breve , Clozapina/sangre , Femenino , Humanos , Masculino , Uso Fuera de lo Indicado , Estudios Retrospectivos , Adulto JovenRESUMEN
Background: The idea of phenotype in chronic obstructive pulmonary disease (COPD) has evolved in the last decades, and the importance of peculiar treatment strategies has now been acknowledged. Although dyspnea and exercise limitation are hallmarks of COPD, this aspect has never been fully explored in literature in terms of disease phenotype. The aim of the present study was to explore the relevance of clinical COPD phenotypes on exercise ventilation and maximal capacity. Methods: In this observational cohort retrospective study we analyzed the data of 50 COPD patients who underwent cardiopulmonary exercise test, categorized as emphysematous (n=29), and non-emphysematous (n=21) according to a previously validated model. Results: We found a significant difference in terms of VE/VCO2 slope (median values 32.4 vs 28.0, p=0.015) and VE/VCO2 ratio at nadir (median values 37 vs. 33, p=0.004), which resulted higher in emphysematous patients, who also presented lower PETCO2 values (median values 32.6 vs 35.6, p=0.008). In a subgroup of 31 tests which met the maximality criteria, emphysematous patients presented a significantly lower work rate at peak (median value 51 vs 72% predicted, p=0.016), and showed a lower peak oxygen consumption, although at the limit of significance (median values of 63 vs 85 % predicted, p=0.051). Conclusions: This study extends our knowledge about the characterization of the COPD phenotypical expression of disease, showing that patients affected by emphysema are more prone to ventilatory inefficiency during exercise, and that this is likely to be an important cause of their overall reduced exercise capacity.
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INTRODUCTION: Surgical treatment of craniosynostosis in children may generate secondary cranial vault defects by incomplete ossification process. The incidence of cranial defects after craniosynostosis surgery is poorly reported in the literature with a variable incidence of 0.5% to 18.2%. To better understand the impact and management of the cranial defects after craniosynostosis surgery, we selected and reviewed al cranioplasties that met this criterion between 2002 and 2019. PATIENTS AND METHOD: We found 41 patients (28 M and 13 F) operated between January 2002 to January 2019 who underwent cranioplasty for cranial defects secondary to craniosynostosis surgery in the infancy. All patient were at least 5 years old (range 5 to 17 years, median 11). The indication was aesthetic in 28 patients and for headaches, learning difficulties or changes in the cerebral blood flow in 13 patients. The material used in cranioplasty was the poly-methyl-methacrylate (PMMA) in 40 patients and customized porous hydroxyapatite (CPHA) in 1 patient. RESULTS: The mean follow-up period after cranioplasty was of 37 months (range 2 to 125 months, median of 27 months). In the postoperative period there were no mortality nor neurological complications. There were 3 cases (7%) of superficial local postoperative wound infection but with no evidence of long-term infection. The long-term CT scan follow-up showed no material migration, dislocation or fractures. CONCLUSION: Considering the characteristics of the cranial vault after craniosynostosis surgery the treatment by cranioplasty with heterologous materials appears to be feasible thus avoiding donor site morbidity. For selected cases with small defects, our experience shows that the use of PMMA may be a safe alternative for the cranial vault repair in children older than 5 years.
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Craneosinostosis/cirugía , Procedimientos de Cirugía Plástica/métodos , Cráneo/cirugía , Adolescente , Materiales Biocompatibles , Niño , Preescolar , Durapatita , Femenino , Humanos , Lactante , Masculino , Polimetil Metacrilato , Estudios Retrospectivos , Infección de la Herida Quirúrgica/epidemiología , Infección de la Herida Quirúrgica/terapia , Resultado del Tratamiento , Adulto JovenRESUMEN
BACKGROUND: Cranial deformations have historically aroused the interest of people worldwide. One of the more debated points about positional plagiocephaly is the choice of the treatment. In this article, the senior author (CM) shares his experience on almost 30 years of use of the helmet molding therapy in children with deformation plagiocephaly. METHODS AND POPULATION: We retrospectively and systematically reviewed the cases of 2188 patients (75% males and 25% females) presenting positional head deformity and treated between 1991 and 2013 with a cranial helmet. To assess the effectiveness of the helmet, we compared the cranial index in bilateral plagiocephaly and the Cranial Diagonals Difference (CDD) in unilateral plagiocephaly at the beginning and at the end of the treatment. RESULTS: The cranial indexes ranged between 94.4% and 124.2% before the treatment and decreased significantly between 86.8% and 121.4% after the treatment (P<0.01). The CDD ranged between 0.3 cm and 4.5 cm with an average of 1.50±0.54 cm before the treatment and decreased significantly between 0.1 cm and 2.5 cm with an average of 0.72±0.37 cm after the treatment (P<0.01). For unilateral plagiocephaly, at the beginning of the treatment, 2.5% children presented a mild plagiocephaly, 19.6% a moderate plagiocephaly and 77.9% a severe plagiocephaly. At the end of the treatment, the deformation was classified as mild in 40.2% children, moderate in 44.3% children and severe in 15.5% children with significantly less children in the most severe subgroups (P<0.01) Facial symmetry pre-existed before the treatment in 13.7% of children. This rate was significantly increased at the end of the treatment to 66.7% (P<0.01). In only 8 cases (0.2%), the helmet therapy did not allow to obtain correct clinical results and a surgical posterior cranial remodeling was performed. CONCLUSIONS: The results observed in this series confirms that cranial helmet is a simple and well tolerated alternative which bring satisfying results. Its success implies a good collaboration with parents and a management both by orthoptist, physiotherapist and doctor. Nevertheless, it remains many controversies in the literature concerning in particular long-term cosmetic and functional outcomes. A long-term multicentric prospective study could enable to remove doubts.