[Translated article] Changes in the Location of Cutaneous Melanoma Over the Past 30 Years. A Retrospective Observational Study.

BACKGROUND: The location of cutaneous melanoma is associated with photoexposure. OBJECTIVES: To retrospectively analyze changes in the location of cutaneous melanoma over the past 30 years. PATIENTS AND METHODS: All patients treated at our hospital for cutaneous melanoma from 1988 through 2017 were prospectively collected. Data obtained in cases diagnosed from 1988 through June 2006 were compared to those diagnosed from July 2006 through 2017. RESULTS: A total of 1,937 patients (876 men and 1061 women; median age, 57 years; interquartile range 27) were diagnosed with primary cutaneous melanoma. The location of melanoma was head and neck (470 cases), trunk (745 cases), upper limbs (239 cases), and lower limbs (483 cases). From July 2006 through 2017 we detected an increase in the incidence of head and neck melanomas (19.9% vs 28.6%, p <0.001). A drop in the incidence of melanomas located in the lower extremities was also seen in women (39.8% vs 30.4%, p <0.001), and in the trunk men (57.5% vs 47.3%, p=0.003). In the multivariate analyses, only the decrease in melanomas located in lower extremities in women remained significant. CONCLUSION: The increased incidence of head and neck melanomas in both sexes and the decrease in trunk melanomas in men can be attributed to the aging of our population. The reduction in the incidence of melanomas in the lower extremities in women could be associated with changes in photoexposure patterns. Analyzing the factors possibly associated with these changes would contribute to better understanding the pathogenesis of cutaneous melanoma for prevention purposes.

Changes in the Location of Cutaneous Melanoma Over the Past 30 Years. A Retrospective Observational Study. / Cambios en la localización del melanoma cutáneo en los últimos 30 años. Estudio observacional retrospectivo.

BACKGROUND: The location of cutaneous melanoma is associated with photoexposure. OBJECTIVES: To retrospectively analyze changes in the location of cutaneous melanoma over the past 30 years. PATIENTS AND METHODS: All patients treated at our hospital for cutaneous melanoma from 1988 through 2017 were prospectively collected. Data obtained in cases diagnosed from 1988 through June 2006 were compared to those diagnosed from July 2006 through 2017. RESULTS: A total of 1,937 patients (876 men and 1061 women; median age, 57 years; interquartile range 27) were diagnosed with primary cutaneous melanoma. The location of melanoma was head and neck (470 cases), trunk (745 cases), upper limbs (239 cases), and lower limbs (483 cases). From July 2006 through 2017 we detected an increase in the incidence of head and neck melanomas (19.9% vs 28.6%, p <0.001). A drop in the incidence of melanomas located in the lower extremities was also seen in women (39.8% vs 30.4%, p <0.001), and in the trunk men (57.5% vs 47.3%, p=0.003). In the multivariate analyses, only the decrease in melanomas located in lower extremities in women remained significant. CONCLUSION: The increased incidence of head and neck melanomas in both sexes and the decrease in trunk melanomas in men can be attributed to the aging of our population. The reduction in the incidence of melanomas in the lower extremities in women could be associated with changes in photoexposure patterns. Analyzing the factors possibly associated with these changes would contribute to better understanding the pathogenesis of cutaneous melanoma for prevention purposes.

[Our experience with sirolimus for the treatment of complicated vascular anomalies]. / Nuestra experiencia con la utilización de sirolimus en el tratamiento de anomalías vasculares complejas.

AIM OF THE STUDY: mTOR inhibitors are being used to treat complex vascular anomalies (VA) without response to conventional treatments. We report our results in pediatric patients treated with sirolimus. METHODS: Retrospective review of patients treated with sirolimus between 2014 and 2017, analyzing vascular anomaly type, treatment response and complications. Treatment protocol included patients with complex vascular anomalies, after signing the informed consent. The initial dose was 0.8 mg/m2/12 h, verifying plasmatic levels. Favorable response was defined both in clinical and radiological terms. MAIN RESULTS: Sirolimus was employed in nine patients, median age 14 months old (1 month-14 years), 66% girls. Five complex micro-cystic lymphatic malformations (LM), one multifocal lynphangioendotheliomatosis with thrombocytopenia, one kaposiform lymphangiomatosis, one lymphatic-venous malformation and one kaposiform hemangioendothelioma (KHE) were treated. Median treatment was 4 months (IQR 2-18 months). Resolution or improvement was objectified in four patients (44%). KHE patient presented complete resolution after two months of treatment. Two patients with micro-cystic LM and the one with lymphatic-venous malformation improved after a median treatment of three months. Two patients presented rebound effect after discontinuing treatment. Three patients had hypertransaminasemia and hypercholesterolemia without requiring medical treatment. CONCLUSION: Sirolimus presented mild effects for treatment of complex VA in our study, but was highly resolutive at KHE.

OBJETIVOS: Los inhibidores mTOR se están utilizando para el tratamiento de anomalías vasculares (AV) complejas sin respuesta a tratamientos convencionales. Presentamos nuestros resultados en pacientes pediátricos tratados con sirolimus. METODOS: Análisis retrospectivo de pacientes tratados con Sirolimus, entre 2014 y 2017, describiendo el tipo de anomalía vascular, respuesta al tratamiento y complicaciones derivadas de su empleo. Se incluyeron pacientes con anomalías vasculares complejas, tras firma del consentimiento informado y con dosis inicial de 0,8 mg/m2/12 horas, monitorizando niveles plasmáticos. Se definió respuesta favorable tanto en términos clínicos como radiológicos. RESULTADOS: Recibieron sirolimus 9 pacientes, mediana de edad de 14 meses (RIQ: 1 mes-14 años). El 66% fueron niñas. Se trataron 5 malformaciones linfáticas (ML) microquísticas complejas (en algunas como coadyuvante a otros tratamientos), 1 linfangioendoteliomatosis multifocal con trombopenia, 1 linfangiomatosis kaposiforme, 1 malformación mixta veno-linfática, 1 hemangioendotelioma kaposiforme (HEK). Mediana de tratamiento: 4 meses (2-18 meses). En 6 pacientes (66%) se objetivó resolución o mejoría de las lesiones. El paciente con HEK mostró resolución completa tras 2 meses de tratamiento. En dos pacientes con ML microquística y en el paciente con malformación mixta, se apreció mejoría clínica y disminución del tamaño de las lesiones tras una mediana de tratamiento de 3 meses. Se apreció efecto rebote en dos ML al suspender el tratamiento. Tres pacientes presentaron hipertransaminasemia e hipercolesterolemia, sin precisar tratamiento médico. CONCLUSIONES: En nuestro estudio, objetivamos que el sirolimus tuvo una eficacia moderada en el tratamiento de AV complejas, pero fue resolutivo en el HEK.

Non-osteogenic muscle hypertrophy in children with McArdle disease.

INTRODUCTION: McArdle disease is an inborn disorder of muscle glycogen metabolism that produces exercise intolerance, and has been recently associated with low values ​​of lean mass (LM) and bone mineral content (BMC) and density (BMD) in affected adults. Here we aimed to study whether this bone health problem begins in childhood. METHODS: Forty children and adolescents were evaluated: 10 McArdle disease and 30 control children (mean age of both groups, 13 ± 2y). Body composition was evaluated by dual-energy X-ray absorptiometry and creatine kinase (CK) levels were determined in the patients as an estimate of muscle damage. RESULTS: Legs bone mass was significantly lower in patients than in controls (-36% for BMC and -22% for BMD). Moreover, patients had significantly higher LM values in the legs than controls, whereas no difference was found for fat mass. CK levels were positively associated with LM in McArdle patients. A correlation was found between LM and BMD variables in the control group but not in McArdle patients. CONCLUSION: We have identified a 'non-osteogenic muscle hypertrophy' in children with McArdle disease. This phenomenon warrants special attention since low osteogenesis at an early age predicts a high risk for osteoporosis later in life.

Genetic diversity and patterns of population structure in Creole goats from the Americas.

Biodiversity studies are more efficient when large numbers of breeds belonging to several countries are involved, as they allow for an in-depth analysis of the within- and between-breed components of genetic diversity. A set of 21 microsatellites was used to investigate the genetic composition of 24 Creole goat breeds (910 animals) from 10 countries to estimate levels of genetic variability, infer population structure and understand genetic relationships among populations across the American continent. Three commercial transboundary breeds were included in the analyses to investigate admixture with Creole goats. Overall, the genetic diversity of Creole populations (mean number of alleles = 5.82 ± 1.14, observed heterozygosity = 0.585 ± 0.074) was moderate and slightly lower than what was detected in other studies with breeds from other regions. The Bayesian clustering analysis without prior information on source populations identified 22 breed clusters. Three groups comprised more than one population, namely from Brazil (Azul and Graúna; Moxotó and Repartida) and Argentina (Long and shorthair Chilluda, Pampeana Colorada and Angora-type goat). Substructure was found in Criolla Paraguaya. When prior information on sample origin was considered, 92% of the individuals were assigned to the source population (threshold q ≥ 0.700). Creole breeds are well-differentiated entities (mean coefficient of genetic differentiation = 0.111 ± 0.048, with the exception of isolated island populations). Dilution from admixture with commercial transboundary breeds appears to be negligible. Significant levels of inbreeding were detected (inbreeding coefficient > 0 in most Creole goat populations, P < 0.05). Our results provide a broad perspective on the extant genetic diversity of Creole goats, however further studies are needed to understand whether the observed geographical patterns of population structure may reflect the mode of goat colonization in the Americas.

Clinical assessment of enamel wear caused by monolithic zirconia crowns.

The purpose of this study was to measure enamel wear caused by antagonistic monolithic zirconia crowns and to compare this with enamel wear caused by contralateral natural antagonists. Twenty monolithic zirconia full molar crowns were placed in 20 patients. Patients with high activity of the masseter muscle at night (bruxism) were excluded. For analysis of wear, vinylpolysiloxane impressions were prepared after crown incorporation and at 6-, 12-, and 24-month follow-up. Wear of the occlusal contact areas of the crowns, of their natural antagonists, and of two contralateral natural antagonists (control teeth) was measured by use of plaster replicas and a 3D laser-scanning device. Differences of wear between the zirconia crown antagonists and the control teeth were investigated by means of two-sided paired Student's t-tests and linear regression analysis. After 2 years, mean vertical loss was 46 µm for enamel opposed to zirconia, 19-26 µm for contralateral control teeth and 14 µm for zirconia crowns. Maximum vertical loss was 151 µm for enamel opposed to zirconia, 75-115 µm for control teeth and 60 µm for zirconia crowns. Statistical analysis revealed significant differences between wear of enamel by zirconia-opposed teeth and by control teeth. Gender, which significantly affected wear, was identified as a possible confounder. Monolithic zirconia crowns generated more wear of opposed enamel than did natural teeth. Because of the greater wear caused by other dental ceramics, the use of monolithic zirconia crowns may be justified.

Factors Associated to a Reactive Result of Rapid-HIV Test in Socio-culturally Adapted Services in Primary Care in Spain.

The Services of Prevention and Early diagnosis of HIV in Madrid (Spain) are set in selected primary care centers. Cultural mediators targeted to vulnerable groups (economic immigrants, MSM, sex workers…) perform risk assessment and counselling. Between 2010 and 2014 they performed 6 039 rapid-HIV test, 27.8 % in MSM, 41.2 % in men who have sex exclusively with women (MSW) and 31.0 % in women; 35.7 % in immigrants, mainly from Latin America. A reactive result was more common among MSM (6.0 %) compared to women (0.6 %) and MSW (0.5 %). In MSM it was associated to being immigrant and to antecedents of sexually transmitted infections (STI). Among MSW the factors associated to a reactive result were: seropositivity of sexual partner and heroine consumption, and in women: infrequent use of condoms, seropositivity of sexual partner and antecedents of STI. Preventive interventions to reduce risk of HIV transmission and for early detection should be adapted and targeted to high risk population.

Gender differences in right ventricular function in patients with non-ischaemic cardiomyopathy.

AIM: To evaluate sex-related differences in right ventricular (RV) function, assessed with cardiac magnetic resonance imaging, in patients with stable non-ischaemic dilated cardiomyopathy. METHODS: Prospective multicentre study. We included 71 patients (38 men) and 14 healthy volunteers. RESULTS: Mean age was 60.9 ± 12.2 years. Men presented higher levels of haemoglobin and white blood cell counts than women, and performed better in cardiopulmonary stress testing. A total of 24 patients (12 women) presented severe left ventricular (LV) systolic dysfunction, 32 (13 female) moderate and 15 (8 women) mild LV systolic dysfunction. In the group with severe LV systolic dysfunction, average right ventricular ejection fraction (RVEF) was normal in women (52 ± 4 %), whereas it was reduced in men (39 ± 3 %) p = 0.035. Only one woman (8 %) had severe RV systolic dysfunction (RVEF < 35 %) compared with 6 men (50 %) p < 0.001. In patients with moderate and mild LV dysfunction , the mean RVEF was normal in both men and women. In the 14 healthy volunteers, the lowest value of RVEF was 48 % and mean RVEF was normal in women (56 ± 2 %) and in men (51 ± 1 %), p = 0.08. CONCLUSIONS: In patients with dilated cardiomyopathy, RV systolic dysfunction is found mainly in male patients with severe LV systolic dysfunction.

Enamel wear caused by monolithic zirconia crowns after 6 months of clinical use.

The purpose of this study was to evaluate enamel wear caused by monolithic zirconia crowns and to compare this with enamel wear caused by contralateral natural antagonists. Twenty monolithic zirconia crowns were placed in 20 patients requiring full molar crowns. For measurement of wear, impressions of both jaws were made at baseline after crown cementation and at 6-month follow-up. Mean and maximum wear of the occlusal contact areas of the crowns, of their natural antagonists and of the two contralateral natural antagonists were measured by the use of plaster replicas and 3D laser scanning methods. Wear differences were investigated by the use of two-sided paired Student's t-tests and by linear regression analysis. Mean vertical loss (maximum vertical loss in parentheses) was 10 (43) µm for the zirconia crowns, 33 (112) µm for the opposing enamel, 10 (58) µm for the contralateral teeth and 10 (46) µm for the contralateral antagonists. Both mean and maximum enamel wear were significantly different between the antagonists of the zirconia crowns and the contralateral antagonists. Gender and activity of the masseter muscle at night (bruxism) were identified as possible confounders which significantly affected wear. Under clinical conditions, monolithic zirconia crowns seem to be associated with more wear of opposed enamel than are natural teeth. With regard to wear behaviour, clinical application of monolithic zirconia crowns is justifiable because the amount of antagonistic enamel wear after 6 months is comparable with, or even lower than, that caused by other ceramic materials in previous studies.

Hemorrhagic cytitis after bone marrow transplantation.

OBJECTIVES: Hemorrhagic cystitis (HC) presenting with gross hematuria, bladder pain and urinary frequency develops in 13-38% of patients following bone marrow transplantation (BMT). The objective of the study was to study the characteristics of patients suffering hemorrhagic cystitis after hematopoietic stem cell transplantation in our center. METHODS: We conducted a retrospective chart review of all patients who underwent BMT at our institution between January 1996 and August 2012. We recorded the age, sex, diagnosis, conditioning regimen, interval between BMT and development of symptoms of cystitis and treatment instituted. RESULTS: Five hundred patients underwent BMT in the period of time studied. 52 of them developed hemorrhagic cystitis. The mean age of the affected patients was 39 years; there were 34 males and 18 females. The diagnoses include AML (n=11), ALL (n=8), CML (n=6), MDS (n=11), CLL (n=5), NHL (n=1), HD (n=5), MM (n=2), Medular aplasia((n=3). HC appeared 59.48 days after BMT. There were no differences between sexes. Mortality among the 52 patients was 51.14% but HC was not the cause of death in any patient. Polyomaviruses were detected in the urine of 78.94 % of survivors. CONCLUSIONS: Polyomavirus infection with BK and JC types is usually acquired in infancy and the virus remains latent in renal tissue. Immunosuppression facilitates reactivation of the renal infection and replication of the virus responsible for the clinical manifestations of HC. The differential diagnoses include other urinary infections, lithiasis, thrombocytopenia and adverse effects of pharmacological agents. The urologist plays a limited role in the management of this disease.

[Quality of life and long term results in Hirschsprung's disease]. / Calidad de vida y resultados a largo plazo en pacientes con enfermedad de Hirschsprung.

INTRODUCTION: The most frequent long term side effects of Hirschsprung's disease (HD) surgery are obstructive problems and fecal incontinence. In this study, we analyse long term functional results of the two most used surgical techniques (Duhamel-D- and De la Torre-dlT-) from the patient's point of view, through quality of life and functionality questionnaires. METHODS: We selected short segment HD patients ≥ 4 years) that were operated in our unit from 1996 until 2011. We employed two validated questionnaires: Bowel Function Score (BFS) and Gastrointestinal Quality of Life Index (GIQLI). Both questionnaires were also tested in two control age matched groups, each for every arm (controlD or control dlT). RESULTS: Among 28 preselected patients, 22 (78.6%) answered both questionnaires. Group D (10 patients) showed more constipation: 60% vs. 16.7% ( p = 0.01). Patients in group dlT (12 patients) showed more leaks: 58.3 vs. 10% (p = 0.03). Results of both questionnaires were higher (better) in Group D: 16 vs. 12.8 points (BFS, p = 0.007) and 74.1 vs. 69.8 (GIQLI, p = 0.17). The control group showed an overall better scores than HD patients: 17.2 vs. 14.3 (BFS p = 0.001) and 75.9 vs. 71.8 (GIQLI, p = 0.04). Separately, both groups of patients showed worse scores when compared with each control group. DISCUSSION: Our results are similar to other studies, where global scores of functional results and quality of life are worse in operated HD patients than in age matched controls. Fecal incontinence has more impact on social scores than constipation. Due to the fact that our dlT patients have more frequently fecal leaks, their scores are worse than in the D group. Nevertheless, patients in dlT group are younger and may improve their results as they get older, as it is often the case in operated HD patients.

Influence of lymphopenia on long-term mortality in septic shock, a retrospective observational study.

BACKGROUND AND OBJECTIVE: The diagnosis of infection, to diagnose septic shock, has been qualified by leukocyte counts and protein biomarkers. Septic shock mortality is persistently high (20%-50%), and rising in the long term. The definition of sepsis does not include leukocyte count, and lymphopenia has been associated with its mortality in the short term. Immunosuppression and increased mortality in the long term due to sepsis have not been demonstrated. The aim is to relate the occurrence of lymphopenia and its lack of recovery during septic shock with mortality at 2 years. PATIENTS AND METHODS: Cohort of 332 elderly patients diagnosed with septic shock. Mortality at 28 days and 2 years was analysed according to leukocyte, neutrophil, and lymphocyte counts, and the ability to recover from lymphopenia (LRec). RESULTS: A total of 74.1% of patients showed lymphopenia, and 73.5% did not improve during ICU stay. Mortality was 31.0% and 50.3% at 28 days and 2 years, respectively. Lymphopenia was a predictor of early mortality (OR 2.96) and LRec of late mortality (OR 3.98). Long-term mortality was associated with LRec (HR 1.69). CONCLUSIONS: In elderly patients with septic shock, 28-day mortality is associated with lymphopenia and neutrophilia, and LRec with 2-year mortality; this may represent 2 distinct phenotypes of behaviour after septic shock.

Effectiveness of smoking cessation intervention based on the ABC Approach in patients with TB.

SETTING: Urban setting in the Philippines. OBJECTIVE: To assess the effectiveness of the ABC Approach developed by The Union as a tobacco-smoking cessation intervention for TB patients at a primary healthcare level in an urban setting in the Philippines. DESIGN: We set up an intervention group whose patients with TB received the ABC approach and a control group of patients with TB receiving only routine health education in Manila, The Philippines. We collected smoking status and the domestic secondhand-smoking (SHS) status data from patients with TB at months 0, 2, 4, 6, 8, and 12. TB treatment outcome data were also collected. RESULTS: Patients with TB (n = 2,174) were enrolled upon TB registration. Smoking rates were consistently low in the intervention group (3.9% vs. 8.7% at Month 6). The odds ratios of both tobacco-smoking status and domestic SHS status in the intervention group were significantly lower than those in the control group (tobacco-smoking status: P < 0.001, domestic SHS status: P < 0.01). TB treatment success rates were similar between the groups (85.0% vs. 87.3%; P = 0.201). CONCLUSION: The ABC approach successfully reduced tobacco-smoking rates, maintained low domestic SHS rates and TB treatment success rates in the Philippines.

CADRE: Cadre urbain aux Philippines. OBJECTIF: Évaluer l'efficacité de l'approche ABC développée par L'Union en tant qu'intervention de sevrage tabagique pour les patients atteints de TB au niveau des soins de santé primaires en milieu urbain aux Philippines. MÉTHODE: Nous avons mis en place un groupe d'intervention dont les patients atteints de TB ont reçu l'approche ABC et un groupe témoin de patients atteints de TB ne recevant qu'une éducation sanitaire de routine à Manille, aux Philippines. Nous avons recueilli des données sur le statut tabagique et le statut de tabagisme passif (SHS, pour l'anglais « secondhand-smoking ¼) domestique des patients atteints de TB aux mois 0, 2, 4, 6, 8 et 12. Des données sur les résultats du traitement de la TB ont également été recueillies. RÉSULTATS: Les patients atteints de TB (n = 2 174) ont été recrutés lors de l'enregistrement de la TB. Les taux de tabagisme étaient constamment faibles dans le groupe d'intervention (3,9% contre 8,7% au 6e mois). Les rapports de cotes du tabagisme et du statut SHS canadien dans le groupe d'intervention étaient significativement inférieurs à ceux du groupe témoin (tabagisme : P < 0,001, statut SHS national : P < 0,01). Les taux de réussite du traitement de la TB étaient similaires entre les groupes (85,0% contre 87,3% ; P = 0,201). CONCLUSION: L'approche ABC a permis de réduire les taux de tabagisme, de maintenir de faibles taux nationaux de SHS et de taux de réussite du traitement de la TB aux Philippines.

[Proposals from a French expert panel for respiratory care in ALS patients]. / Propositions formulées par un groupe d'experts français concernant la prise en charge respiratoire chez les patients atteints de sclérose latérale amyotrophique.

BACKGROUND: Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by progressive diaphragm weakness and deteriorating lung function. Bulbar involvement and cough weakness contribute to respiratory morbidity and mortality. ALS-related respiratory failure significantly affects quality of life and is the leading cause of death. Non-invasive ventilation (NIV), which is the main recognized treatment for alleviating the symptoms of respiratory failure, prolongs survival and improves quality of life. However, the optimal timing for the initiation of NIV is still a matter of debate. NIV is a complex intervention. Multiple factors influence the efficacy of NIV and patient adherence. The aim of this work was to develop practical evidence-based advices to standardize the respiratory care of ALS patients in French tertiary care centres. METHODS: For each proposal, a French expert panel systematically searched an indexed bibliography and prepared a written literature review that was then shared and discussed. A combined draft was prepared by the chairman for further discussion. All of the proposals were unanimously approved by the expert panel. RESULTS: The French expert panel updated the criteria for initiating NIV in ALS patients. The most recent criteria were established in 2005. Practical advice for NIV initiation were included and the value of each tool available for NIV monitoring was reviewed. A strategy to optimize NIV parameters was suggested. Revisions were also suggested for the use of mechanically assisted cough devices in ALS patients. CONCLUSION: Our French expert panel proposes an evidence-based review to update the respiratory care recommendations for ALS patients in daily practice.

[Functional results of Hirschsprung's disease patients after Duhamel and De la Torre procedures]. / Resultados funcionales en pacientes con enfermedad de Hirschsprung intervenidos mediante las técnicas de Duhamel y De la Torre.

INTRODUCTION: Long term results of different surgical techniques in Hirschsprung's Disease (HD) are contradictory. There are still no long term large or multicentric reports about functional results of De la Torre technique. We have studied the mid term functional results of the patients operated on Duhamel (D) and De la Torre (dlT) pull-through procedures. PATIENTS AND METHODS: We collected data from medical records and telephone interviews of the HD patients operated in our unit in the last 16 years. RESULTS: 38 patients were found. Ages ranged from 1.5 to 21 years. Mean age was 7.7 years. Median follow up was 5.9 years. 33 (86.8%) had rectosigmoid disease and 5 (13.2%) had long segment disease. D procedure was performed in 17 (44.7%), Soave in 1 (2.6%), Duhamel-Lester-Martin in 4 (10.5%) and dlT pull-through in 16(42%). In the last visit record, 12 (31.6%), had constipation, and fecal leaks were noted in 11 (33.3%) of the 33 patients > or = 4 years old.. 10 patients (29.4% of the > or = 4 years old group) referred encopresis along the follow-up. Patients from the D group referred higher rates of constipation than those in the dlT group (53.3% vs 20% p=0.048). dlT patients referred more frequency of leaks (46,1% vs 13,3%, p=0,05) Children with very short resections (< or = 10 cm) were more prone to constipation than children with longer resections (66,6% vs 17.4% p=0.007), and less prone to present leaks (12.5% vs 47.3% p=0.08). Encopresis was similar in all groups. DISCUSSION: Both techniques show similar functional results in the mid term, although children in the D group were more prone to constipation and those in the dlT group presented more fecal leaks. All patients with EH need long follow-ups.

Efficient multi-fidelity computation of blood coagulation under flow.

Clot formation is a crucial process that prevents bleeding, but can lead to severe disorders when imbalanced. This process is regulated by the coagulation cascade, a biochemical network that controls the enzyme thrombin, which converts soluble fibrinogen into the fibrin fibers that constitute clots. Coagulation cascade models are typically complex and involve dozens of partial differential equations (PDEs) representing various chemical species' transport, reaction kinetics, and diffusion. Solving these PDE systems computationally is challenging, due to their large size and multi-scale nature. We propose a multi-fidelity strategy to increase the efficiency of coagulation cascade simulations. Leveraging the slower dynamics of molecular diffusion, we transform the governing PDEs into ordinary differential equations (ODEs) representing the evolution of species concentrations versus blood residence time. We then Taylor-expand the ODE solution around the zero-diffusivity limit to obtain spatiotemporal maps of species concentrations in terms of the statistical moments of residence time, , and provide the governing PDEs for . This strategy replaces a high-fidelity system of N PDEs representing the coagulation cascade of N chemical species by N ODEs and p PDEs governing the residence time statistical moments. The multi-fidelity order( p ) allows balancing accuracy and computational cost, providing a speedup of over N/p compared to high-fidelity models. Using a simplified coagulation network and an idealized aneurysm geometry with a pulsatile flow as a benchmark, we demonstrate favorable accuracy for low-order models of p = 1 and p = 2. These models depart from the high-fidelity solution by under 16% ( p = 1) and 5% ( p = 2) after 20 cardiac cycles. The favorable accuracy and low computational cost of multi-fidelity models could enable unprecedented coagulation analyses in complex flow scenarios and extensive reaction networks. Furthermore, it can be generalized to advance our understanding of other systems biology networks affected by blood flow.

Proposal for a systematic analysis of polygraphy or polysomnography for identifying and scoring abnormal events occurring during non-invasive ventilation.

Non-invasive ventilation (NIV) is recognised as an effective treatment for chronic hypercapnic respiratory failure. Monitoring NIV during sleep may be preferable to daytime assessment. This paper reports the findings of an international consensus group which systematically analysed nocturnal polygraphic or polysomnographic tracings recorded with either volume-cycled or pressure-cycled ventilators. A systematic description of nocturnal respiratory events which occur during NIV is proposed: leaks, obstruction at different levels of the upper airway (glottis and/or pharynx), with or without decrease of respiratory drive and asynchrony.

Traditional sexing methods and external egg characteristics combination allow highly accurate early sex determination in an endangered native turkey breed.

Early sex determination methods are not only crucial in the worldwide massive poultry industry, but also for small-holder producers. The profitability of sexing techniques must be accounted for when aiming to boost management, nutrition, and conservation practices in endangered poultry breeds. This becomes pivotal when the local breed dealt with belongs to an understudied species, such as the turkey. So, the main objective of this study is to identify which method combination may report a higher likelihood of successful sex determination in poults across the three-pattern varieties of the Andalusian turkey breed. A total of 84 one to two days old Andalusian turkey poults (42 black, 28 black-roan, and 14 bronze-roan) were evaluated in this study. Sex determination was performed using 15 methods, which included testing external egg metrics and eggshell color, poult morphological appraisal and phaneroptics, and behavioral traits. Possible differences across plumage varieties and the interaction between sex and plumage were observed when external egg quality was measured. Sex determination through behavioral methods in black base feathered (black and black-roan) male sex individuals showed seven times higher sensitivity when compared to the rest of the studied individuals (χ2 = 7.14, df = 1, P < 0.01). In contrast, for the black-roan plumage females, the method based on the color of down feathers was approximately four times more sensitive (χ2 = 3.95, df = 1, P ≤ 0.05). For the bronze-roan pattern, none of the sexing techniques was reported to efficiently predict sex itself. However, the most proper method combination to determine sex, independent of plumage color, was physical external egg characteristics, the color of down feathers, and behavioral approaches ("English method" and "slap technique"). The specificity values were found to be 49.12, 93.33, and 100%, while the sensitivity values were observed to be 74.64, 91.03, and 100%, which translated into accuracy of 63.10, 92.26, and 100% in black, black-roan, and bronze-roan poults, respectively. Our results suggest that the method combination tested in this study could be considered a highly accurate, simple, and affordable alternative for sex determination in turkeys. This could mean a pivotal advance for small producers of turkeys, as early sex detection can help to plan timely conservational management strategies, which is of prominent importance in the context of endangered poultry breeds.

Proposals from a French expert panel for respiratory care in ALS patients.

BACKGROUND: Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by progressive diaphragm weakness and deteriorating lung function. Bulbar involvement and cough weakness contribute to respiratory morbidity and mortality. ALS-related respiratory failure significantly affects quality of life and is the leading cause of death. Non-invasive ventilation (NIV), which is the main recognized treatment for alleviating the symptoms of respiratory failure, prolongs survival and improves quality of life. However, the optimal timing for the initiation of NIV is still a matter of debate. NIV is a complex intervention. Multiple factors influence the efficacy of NIV and patient adherence. The aim of this work was to develop practical evidence-based advices to standardize the respiratory care of ALS patients in French tertiary care centres. METHODS: For each proposal, a French expert panel systematically searched an indexed bibliography and prepared a written literature review that was then shared and discussed. A combined draft was prepared by the chairman for further discussion. All of the proposals were unanimously approved by the expert panel. RESULTS: The French expert panel updated the criteria for initiating NIV in ALS patients. The most recent criteria were established in 2005. Practical advice for NIV initiation were included and the value of each tool available for NIV monitoring was reviewed. A strategy to optimize NIV parameters was suggested. Revisions were also suggested for the use of mechanically assisted cough devices in ALS patients. CONCLUSION: Our French expert panel proposes an evidence-based review to update the respiratory care recommendations for ALS patients in daily practice.

[Take-home messages from the COPD 2021 biennial of the French Society of Respiratory Diseases. Understanding to so as to better innovate]. / Principaux messages de la première Biennale BPCO 2021 de la SPLF. Mieux comprendre pour innover.

INTRODUCTION: The first COPD biennial organized by the French Society of Respiratory Diseases (SPLF) took place on 17 December 2021. STATE OF THE ART: The objective of the biennial was to discuss current knowledge regarding COPD pathophysiology, current treatments, research development, and future therapeutic approaches. PERSPECTIVES: The different lecturers laid emphasis on the complexity of pathophysiologic mechanisms including bronchial, bronchiolar and parenchymal alterations, and also dwelt on the role of microbiota composition in COPD pathenogenesis. They pointed out that addition to inhaled treatments, ventilatory support and endoscopic approaches have been increasingly optimized. The development of new therapeutic pathways such as biotherapy and cell therapy (stem cells…) call for further exploration. CONCLUSIONS: The dynamism of COPD research was repeatedly underlined, and needs to be further reinforced, the objective being to "understand so as to better innovate" so as to develop effective new strategies for treatment and management of COPD.