Manejo anestésico perioperatorio de glosoplastia reductora en paciente con síndrome de Beckwith-Wiedemann / Perioperative anesthetic management of reductive glossoplasty in a patient with Beckwith-Wiedemann syndrome

Introducción: El síndrome de Beckwith-Wiedemann (SBW) es un trastorno de crecimiento congénito. El manejo perioperatorio puede ser a veces complicado. Sus principales manifestaciones clínicas son macroglosia, que puede causar dificultades en el manejo de la vía aérea, prematuridad, hemihipertrofia, onfalocele, tumores embrionarios y episodios de hipoglucemia neonatal. Objetivo: Nuestro objetivo principal es describir el manejo perioperatorio de pacientes pediátricos con SBW sometidos a glosectomía y sus posibles complicaciones anestésicas. Métodos: Reporte de caso y revisión del tema. Resultados: Describimos el caso de una paciente de 11meses diagnosticada con SBW que se sometió a cirugía reductora de macroglosia. Se realizó una evaluación preoperatoria exhaustiva con la consideración de las posibles complicaciones anestésicas derivadas tanto de la macroglosia como de la prematuridad, y los posibles episodios de hipoglucemia. El procedimiento se realizó bajo anestesia general, siguiendo los algoritmos de intubación difícil de la vía aérea, sin incidencias, y se realizó una extubación segura en el quirófano. Durante el postoperatorio la paciente permaneció estable, presentó buena dinámica respiratoria, SatO2>96% y buen control glucémico, comenzando la dieta oral a las 4h después de la cirugía. Fue dada de alta a planta 24h después de la intervención. Conclusión: El manejo exitoso de pacientes con SBW requiere un enfoque multimodal, con planificación preoperatoria completa y conocimiento sobre posibles complicaciones en relación tanto con las vías respiratorias como con las sistémicas.(AU)

Introduction: Postoperative management of patients with the congenital growth disorder Beckwith-Wiedemann syndrome (BWS) can be complicated. The main clinical manifestations of the syndrome are macroglossia — which may hamper airway management —, prematurity, hemihypertrophy, omphalocele, embryonal tumours and episodes of neonatal hypoglycaemia. Objective: Our main objective is to describe the perioperative management and potential anaesthetic complications in paediatric patients with BWS undergoing glossectomy. Methods: Case report and literature review. Results: We describe the case of an 11-month-old patient diagnosed with BWS who underwent reduction glossoplasty. We performed a comprehensive preoperative evaluation, taking into account potential anaesthetic complications derived from both macroglossia and prematurity, and the risk of hypoglycaemia. The procedure was performed under general anaesthesia. Intubation — performed according to difficult airway management algorithms — was uneventful and the patient was successfully extubated in the operating room. The patient remained stable during the postoperative period, with good respiratory dynamics, SatO2>96% and good glycaemic control. Oral intake was started 4hours after surgery, and she was discharged to the ward at 24hours. Conclusion: BWS patients require a multimodal approach that includes detailed preoperative planning and knowledge of potential airway-related and systemic complications.(AU)

Perioperative anesthetic management of reductive glossoplasty in a patient with Beckwith-Wiedemann syndrome. / Manejo anestésico perioperatorio de glosoplastia reductora en paciente con síndrome de Beckwith-Wiedemann.

INTRODUCTION: Postoperative management of patients with the congenital growth disorder Beckwith-Wiedemann syndrome (BWS) can be complicated. The main clinical manifestations of the syndrome are macroglossia - which may hamper airway management -, prematurity, hemihypertrophy, omphalocele, embryonal tumours and episodes of neonatal hypoglycaemia. OBJECTIVE: Our main objective is to describe the perioperative management and potential anaesthetic complications in paediatric patients with BWS undergoing glossectomy. METHODS: Case report and literature review. RESULTS: We describe the case of an 11-month-old patient diagnosed with BWS who underwent reduction glossoplasty. We performed a comprehensive preoperative evaluation, taking into account potential anaesthetic complications derived from both macroglossia and prematurity, and the risk of hypoglycaemia. The procedure was performed under general anaesthesia. Intubation - performed according to difficult airway management algorithms - was uneventful and the patient was successfully extubated in the operating room. The patient remained stable during the postoperative period, with good respiratory dynamics, SatO2>96% and good glycaemic control. Oral intake was started 4hours after surgery, and she was discharged to the ward at 24hours. CONCLUSION: BWS patients require a multimodal approach that includes detailed preoperative planning and knowledge of potential airway-related and systemic complications.

Effects of marine noise pollution on Mediterranean fishes and invertebrates: A review.

Marine noise pollution (MNP) can cause a multitude of impacts on many organisms, but information is often scattered and general outcomes difficult to assess. We have reviewed the literature on MNP impacts on Mediterranean fish and invertebrates. Both chronic and acute MNP produced by various human activities - e.g. maritime traffic, pile driving, air guns - were found to cause detectable effects on intra-specific communication, vital processes, physiology, behavioral patterns, health status and survival. These effects on individuals can extend to inducing population- and ecosystem-wide alterations, especially when MNP impacts functionally important species, such as keystone predators and habitat forming species. Curbing the threats of MNP in the Mediterranean Sea is a challenging task, but a variety of measures could be adopted to mitigate MNP impacts. Successful measures will require more accurate information on impacts and that effective management of MNP really becomes a priority in the policy makers' agenda.

Joint analysis of additive, dominant and first-order epistatic effects of four genes (IGF2, MC4R, PRKAG3 and LEPR) with known effects on fat content and fat distribution in pigs.

LEPR, MC4R, IGF2 and PRKAG3 are genes with known effects on fat content and distribution in pig carcass and pork. In a study performed with Duroc × Landrace/Large White pigs, we have found that IGF2 has strong additive effects on several carcass conformational traits and on fatty acid composition in several anatomical locations. MC4R shows additive effects on saturated fatty acid content in several muscles. On the other side, almost no additive effect has been found for PRKAG3 and very few for LEPR. In this work, no dominant effect has been found for any of the four genes. Using a Bayesian Lasso approach, we have been able now to find first-order epistatic (mainly dominant-additive) effects between LEPR and PRKAG3 for intramuscular fat content and for saturated fatty acid content in L. dorsii, B. femoralis, Ps. major and whole ham. The presence of interactions between genes in the shaping of traits of such importance as intramuscular fat content and composition highlights the complexity of heritable traits and the difficulty of gene-assisted selection for such traits.

Allelic frequencies of PRKAG3 in several pig breeds and its technological consequences on a Duroc × Landrace-Large White cross.

The allelic frequencies of PRKAG3 gene (the RN gene) have been investigated in several pig breeds. R200Q mutation appear only in Hampshire pigs, whereas V199I mutation is most abundant in Iberian, Porco Celta or Bizaro, and less in breeds selected for muscularity as Duroc, Landrace and Pietrain. A thorough study of phenotypic effects of V1991 has been performed in a Duroc × Landrace-Large White cross. 199I homozygous pigs show increased pH24 values in ham homogenates and loin (0.14 and 0.16 pH units, respectively) compared to 199V homozygous ones. Meat of 199I homozygous pigs exudates 42.6% less fluid and is darker (2.46 'L'-value units). 199I homozygous pigs are fatter (4.2 mm more backfat thickness) and contain less muscle mass in ham (1.0 percentage points) and shoulder (2.7 percentage points), than 199V homozygous ones. 199I homozygous pigs contain 7.3% less protein in the belly and 8.5% more fat in shoulder muscle mass than 199V homozygous pigs. 199I homozygous pigs have also superior functional properties: better gelling (22.8% larger G' value) and emulsion capacities (14 percentage points less of total exuded fluid), and higher curing yield in the belly (6 percentage points more). These data support the adipogenic character of the V199I mutation. The advantages and disadvantages of selecting any of the two PRKAG3 alleles for position 199 are discussed.

Early postmortem gene expression and its relationship to composition and quality traits in pig Longissimus dorsi muscle.

An mRNA expression study has been performed in 20- to 25-min postmortem-obtained samples from Longissimus dorsi muscle of 59 Duroc × Landrace/ Large White (LD/LW) pigs to search for gene sequences related to meat quality (pH(24), pH(45), "Lab" color coordinates, curing yield, and exudation at 3 different times) or meat composition [intramuscular fat, content of several fatty acids (C16:0, C18:0, C18:1, and C18:2), ratio of saturated, monounsaturated, and polyunsaturated fatty acids, and protein and humidity contents] traits to find targets for selection. Gene ontology analysis, biological pathway, and gene network studies all show that many more differentially expressed genes (506 vs. 279) are related to meat quality (Group P or perimortem characters) than to meat composition traits (Group L or whole life traits). The difference between the number of gene ontology terms annotated, biological pathways, and gene networks in groups P and L is notable due to the differences in the complexity of the "generation process" of P-traits and involvement of other tissues or organs in the generation of variability of L-traits. Also, interactions between differentially expressed genes were found in extracellular matrix (ECM)-receptor interaction, tumor growth factor (TGF)-beta signaling pathway, fatty acid elongation in mitochondria, and adipocytokine signaling pathway, indicating that a substantial fraction of the gene networks could be associated with interactions between differentially expressed genes related to traits under study. A large number of the most overexpressed genes are related to muscle development and functionality and repair mechanisms; they could be good candidates for breeding programs in which the main goal is to enhance meat quality.

Distrofia simpático refleja en el post-parto / Algodystrophie in the post-partum

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Cuerpo extraño intraocular como accidente de trabajo: a propósito de dos casos / Intraocular foreign body as occupational accident: report of two cases

Los traumatismos oculares perforantes con cuerpo extraño intraocular representan patologías muy graves debido a su importante repercusión funcional y anatómica. La causa más frecuente son accidentes ocupacionales. Se describen dos casos de accidentes ocupacionales con cuerpo extraño intraocular, ambos en trabajadores agrícolas, y se exponen su manejo y su curso evolutivo (AU)

[Epidemiological observations on the evolutive tendency of asthma mortality in Spain]. / Observaciones epidemiológicas sobre la tendencia evolutiva de la mortalidad por asma en España.

We have studied asthma mortality in Spain from 1951 to 1985, applying epidemiological descriptive methodology. A global decrease in the tendency is observed when comparing it to other countries. We have observed, however, an increased mortality in the four age groups proposed (5-19, 5-39, 40-64 and 5-64), based on previous international publications which avoid interferences with other causes of death. We have also observed that when applying the method of mortality related to natality rate, there was a decrease in mortality, specially in older generations. Finally, several hypothesis related to risk factors which would explain this tendency are presented.

[Trends in kidney cancer mortality in Spain (1951-1985) and review of hospital case histories]. / Tendencias en la mortalidad por cáncer de riñón en España (1951-1985) e investigación de una casuística hospitalaria.

The present study is a descriptive epidemiological study of the mortality trends for renal cancer based on the data available for the period spanning 1951 to 1985. The specific death rates show a rising trend that is more marked in males than in females. These have been observed to increase constantly for the group aged 60-64 years. The mortality rates by birth cohorts show the cohort effect to be less evident in the younger male and female population. A review of 81 clinical records (1984-1988) of La Fe Hospital in Valencia based on a protocol that focussed on selected risk factors showed a higher frequency (33.75%) for the group aged 60-69 followed by the 50-59 age group (26.75%) and a higher incidence in men (66.67%).

[Comparison of mortality in Spain and France, following the method of "potential years of life lost"]. / Datos comparativos de la mortalidad en Espana y Francia, segun el metodo del "nivel potencial de anos de vida perdidos".

PIP: Mortality trends in Spain from 13 major causes of death are analyzed for the period 1972-1982 and compared with trends for the same period in France. Increases in mortality in Spain are noted for three causes--malignant tumors, cardiac diseases, and suicides and homicides--whereas significant declines in mortality are noted for pneumonia, influenza, chronic bronchitis, and asthma. Excess mortality for males is common to both countries. (SUMMARY IN ENG)^ieng

Successful thrombolysis on a mechanical tricuspid prosthesis.

Recombinant tissue-type plasminogen activator (rt-PA) is presently used for the treatment of different clinical entities, mostly myocardial infarction. Its use for treatment of thrombotic dysfunction of prosthetic cardiac valves is more recent and has been only rarely reported. A 33-year-old woman with a St Jude Medical prosthesis in the tricuspid position, had suffered from thrombotic dysfunction of her prosthesis for more than 2 months. She was treated with rt-PA, and after infusion of 70 mg, the prosthesis functioned normally. She showed a moderate systemic fibrinolytic state associated with mild bleeding complications.