A new case of arginase deficiency in a Spanish male

A new case of arginase deficiency is reported in a male newborn from Spain. In contrast with the majority of the earlier cases, this infant showed severe protein intolerance of early onset. The diagnosis was based on the assay of the urea cycle enzymes in a postmortem liver sample. Levels of erythrocyte arginase were also determined in the parents and in a sister of the patient, and were consistent with heterozygosity. From a study of the pedigree it appears that arginase deficiency in this family presents a dramatic course.     

Association between physical activity and patient-reported outcome measures in patients with lung cancer: a systematic review and meta-analysis

Quality of Life Research - The aim of this systematic review was to examine the association between physical activity (PA) and Health-Related Quality of Life (HRQoL) as well as other...     

Complicaciones durante la intubación traqueal repetida en la unidad de cuidados intensivos. Estudio prospectivo,observacional comparando primera intubación y reintubación

Background of the studyThe goal of the study was to compare the incidence of complications, technical difficulty of intubation and physiologic pre-intubation status between the first intubation and reintubation performed on the same patient in an ICU.Materials and methodsThe study was approved by the ethics committee of Galicia (Santiago-Lugo, code No. 2015-012). Due to the observational, noninterventional, and noninvasive design of this study, the need for written consent was waived by the ethics committee of Galicia. Patients requiring tracheal intubation and reintubation in the ICU were included in this prospective observational study. Main endpoint was to compare the incidence of complications, physiologic pre-intubation status, and the rate of technical difficulty of intubation between the first intubation and reintubation performed on the same patient in an ICU.Results and discussion504 patients were intubated in our ICU during the study period, and 82 (16%) required reintubation. There was no difference between the first intubation and reintubation regarding number of total complication (35% vs 33%; P = .86), hypotension (24% vs 24%; P = 1), hypoxia (26% vs 26%; P = 1), esophageal intubation (1% vs 1%; P = 1), and bronchoaspiration (2% vs 1%; P = .86). Physiologic pre-intubation status and technical difficulty of intubation did not differ between the first intubation and reintubation.ConclusionsIn our ICU patients requiring tracheal reintubation, incidence of complications, physiologic pre-intubation status, and technical difficulty of intubation did not differ between the first intubation and reintubation.     

Vía clínica de recuperación intensificada en cirugía cardiaca. Documento de consenso de la Sociedad Española de Anestesiología,Reanimación y Terapéutica del Dolor (SEDAR), la Sociedad Española de Cirugía Cardiovascular y Endovascular (SECCE) y la Asociación Española de Perfusionistas (AEP)

The ERAS guidelines are intended to identify, disseminate and promote the implementation of the best, scientific evidence-based actions to decrease variability in clinical practice. The implementation of these practices in the global clinical process will promote better outcomes and the shortening of hospital and critical care unit stays, thereby resulting in a reduction in costs and in greater efficiency. After completing a systematic review at each of the points of the perioperative process in cardiac surgery, recommendations have been developed based on the best scientific evidence currently available with the consensus of the scientific societies involved.     

Exposure to Landfill Leachates Affects the Embryonic Development of Zebrafish,Danio rerio: A Case Study in Yucatan,Mexico

Bulletin of Environmental Contamination and Toxicology - We report the chemical characterisation and toxic effects of municipal solid waste landfill leachates on the embryonic development of Danio...     

Changes in retinoblastoma gene expression during cervical cancer progression

The role of tumour suppressor genes in the development of human cancers has been studied extensively. In viral carcinogenesis, the inactivation of suppressor proteins such as retinoblastoma (pRb) and p53, and cellular oncogenes overexpression, such as c-myc, has been the subject of a number of investigations. In uterine-cervix carcinomas, where high-risk human papillomavirus (HPV) plays an important role, pRb and p53 are inactivated by E7 and E6 viral oncoproteins, respectively. However, little is known about the in situ expression of some of these proteins in pre-malignant and malignant cervical tissues. On the other hand, it has also been demonstrated that c-myc is involved in cervical carcinogenesis, and that pRb participates in the control of c-myc gene expression. By using immunostaining techniques, we investigated pRb immunodetection pattern in normal tissues, squamous intraepithelial lesions (SILs) and invasive carcinomas from the uterine cervix. Our data show low pRb detection in both normal cervical tissue and invasive lesions, but a higher expression in SILs. C-Myc protein was observed in most of the cellular nuclei of the invasive lesions, while in SILs was low. These findings indicate a heterogeneous pRb immunostaining during the different stages of cervical carcinogenesis, and suggest that this staining pattern could be a common feature implicated in the pathogenesis of uterine-cervix carcinoma.     

Management of anorectal malformations during the newborn period

Anorectal malformations comprise a spectrum of disease. Some of these defects are minor, with an excellent functional prognosis. Others are complex and may be associated with other defects in the urinary tract that may risk the patient's life. The decision-making process for the management of a newborn with anorectal malformations is crucial; the timing and establishment of priorities are the key to success. Because the management of affected males and females differs significantly, they are discussed separately.

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Resumen Las malformaciones anorrectales comprenden todo un espectro de enfermedad. Algunos de los defectos son de naturaleza menor, fáciles de tratar y tienen un excelente pronóstico funcional. Otros son complejos, su manejo es difícil, su pronóstico incierto y algunoas veces se ven asociados con otros defectos en el tracto urinario que significan riesgo para la vida del paciente. El proceso de toma de decisión en el manejo de un recién nacido con malformación anorrectal es crucial porque determina el futuro inmediato y lejano del niño. Las más importantes decisiones a tomar durante este período son las relativas a la necesidad de una colostomía y sí el paciente requiere una derivación urinaria o vaginal con el fin de prevenir sepsis o acidosis metabólica. La definición de prioridades y su programación son la clave del éxito. Puesto que el manejo de estos pacientes difiere significativamente en los niños y en las niñas, se discuten por separado en este informe.

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Résumé Il existe une grande variété de malformations anorectales de l'enfant. Les malformations dites mineures sont faciles à réparer et leur pronostic est excellent. Parmi les variétés complexes, le traitement est difficile, le pronostic incertain, et parfois, lorsqu'il s'y associe des malformations urinaires, la vie de l'enfant est en jeu. De la décision thérapeutique, dépend étroitement le pronostic immédiat et tardif. Les problèmes essentiels concernent la décision de réaliser ou non une colostomie ou encore la nécessité de pratiquer une dérivation urinaire ou vaginale afin d'éviter une infection ou une acidose métabolique. Le traitement des malformations diffèrent naturellement entre le garçon et la fille et sera traité séparemment.