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.

Impact of empirical treatment with antifungal agents on survival of patients with candidemia.

OBJECTIVE: The objective of this study was to evaluate the impact of echinocandins and fluconazole) on mortality 7 and 30 days after candidemia onset and overall in-hospital mortality), in patients with candidemia at a Spanish tertiary hospital. METHODS: A retrospective study was conducted that enrolled all non-neutropenic adult patients diagnosed with candidemia at Hospital Clínico Universitario de Valladolid between 2007 and 2016. A total of 179 patients were evaluated, they were divided into two sub-groups: surviving patients (n = 92) and non-surviving patients (n = 87). RESULTS: The 7-day mortality was 25,1% (45), 30-day mortality was 46,9% (84), and overall in-hospital mortality was 48,6% (87). 40.8% of patients received no antifungal treatment (43.8% of surviving patients and 37.8% of non-surviving patients; p=0.15). A total of 106 (59.2%) patients were treated, of which 90 patients (50.3%) received empiric treatment. 19.6% and 47.8% of surviving patients were treated with echinocandins and fluconazole, respectively. By contrast, of non-surviving patients, 31.0% were treated with echinocandins and 47.1% received fluconazole. Survival for the first 7 days was significantly higher in treated with antifungal agents (log-rank = 0.029), however, there were not significant differences in 30-day survival. Factors linked to a significant increase in overall in-hospital mortality were age (OR 1.040), septic shock (OR 2.694) and need for mechanical ventilation > 48 h (OR 2.812). CONCLUSIONS: Patients who received antifungal treatment, regardless of whether they received fluconazole or echinocandins, had a significantly lower mortality rate after 7 days than untreated patients, although no significant differences in 30-day mortality were seen.

Imágenes ultrasonográficas de vía aérea superior tras la inserción de mascarilla laríngea: estudio descriptivo y utilidad clínica / Descriptive study of ultrasound images of the upper airway obtained after insertion of laryngeal mask

Objetivo: Evaluar la utilidad clínica de la ecografía de vía aérea superior en la visualización de la mascarilla laríngea correctamente insertada. Material y métodos: Estudio observacional prospectivo en pacientes programados para cirugía abdominal bajo anestesia general, a quienes se aseguraba la permeabilidad de la vía aérea mediante mascarilla Ambu(R)AuraGainTM. Se llevó a cabo un escaneo ecográfico de la vía aérea superior, mediante cortes trasversales en sentido cráneo-caudal y cortes longitudinales en línea media cervical anterior y parasagital, en tres momentos: antes, tras insertar y tras retirar la mascarilla. Las imágenes registradas eran evaluadas en un segundo tiempo por un radiólogo experto en ecografía de la vía aérea. Posteriormente, se relacionaban los datos ecográficos con los clínicos de dificultad de inserción y presencia de fuga aérea. Resultados: Recogimos datos de 30 pacientes (20 mujeres y 10 varones) intervenidos de histerectomía abdominal (15), eventroplastia (6), miomectomía uterina (3) y hernioplastia umbilical (4) e inguinal (2). La inserción a ciegas del dispositivo no presentó dificultades en 24 pacientes (80%). Detectamos fuga aérea en 8 pacientes (26,7%): moderada en 7 casos y grave en uno. Los hallazgos ecográficos confirmaban buena colocación de la mascarilla en 22 pacientes (73,3%). Se objetivaron cambios anatómicos en la vía aérea tras extraer la mascarilla laríngea en 3 pacientes (12%), todos leves. Hubo asociación estadísticamente significativa (p<0,05) entre el grado de dificultad de inserción del dispositivo y el grado de fuga aérea detectado. Conclusiones: La ultrasonografía de la vía aérea superior podría confirmar la colocación correcta de la mascarilla laríngea. No se objetivó edema laríngeo tras la extracción del dispositivo

Objective: To evaluate clinical usefulness of ultrasound images of the upper airway in order to check correct laryngeal mask placement. Material and methods: A prospective observational study was conducted on patients scheduled for abdominal surgery under general anaesthesia, in whom the patency of the upper airway was ensured using an Ambu(R)AuraGainTM laryngeal mask. An ultrasound scan was performed of the upper-airway in the cranio-caudal direction and with longitudinal scans in the anterior midline and parasagittal axis, in three moments: before, after inserting and after removing the mask. All recorded images were evaluated in a second time by a radiologist-expert in upper airway ultrasound. Subsequently, the ultrasound data were related to the clinical difficulty of the insertion and presence of air leaks. Results: Data was collected from 30 patients (20 females and 10 males) being operated on for abdominal hysterectomy (15), eventroplasty (6), uterine myomectomy (3), and umbilical (4) and inguinal herniorrhaphy (2). The blind insertion of the masks did not present difficulties in 24 (80%) patients. Air leakage was detected in 8 (26.7%) patients, which was moderate in 7 cases and severe in one of them. The ultrasound findings confirmed good mask placement in 22 (73.3%) patients. Anatomical airway changes after laryngeal mask extraction were only observed in 3 (12%) patients, all of them minor. There was a statistically significant association (P<.05) between difficulty in inserting the device and the level of air leakage. Conclusions: Upper airway ultrasound is a useful diagnostic method to evaluate laryngeal mask placement. Laryngeal oedema was not observed after removal of the device

Tratamiento anestésico en una paciente pediátrica con miopatía congénita por desproporción del tipo de fibras / Anaesthetic management of a paediatric patient with congenital fibre type disproportion myopathy

La desproporción congénita del tipo de fibras (DCTF) es un raro tipo de miopatía caracterizado por debilidad muscular e hipotonía durante la infancia. Las características clínicas incluyen retraso motor, dificultades en la alimentación, debilidad de las extremidades, contracturas articulares y escoliosis. Se describe el tratamiento anestésico de una paciente de 3 años con miopatía DCTF asociada a mutación del gen TPM3, programada para realización de adenoamigdalectomía por presentar un síndrome de apnea-hipopnea obstructiva del sueño (SAHOS). Nuestras principales preocupaciones fueron la posible susceptibilidad a la hipertermia maligna, el riesgo de rabdomiólisis inducida por anestesia, una mayor sensibilidad a los relajantes musculares no despolarizantes y la presencia de SAHOS. La anestesia total intravenosa con propofol y el empleo de rocuronio/sugammadex parecen ser opciones seguras. Dado el alto riesgo de compromiso respiratorio y otras complicaciones, los pacientes deben controlarse estrechamente en el periodo postoperatorio

Congenital fibre type disproportion (CFTD) is a rare type of myopathy that is characterised by muscle weakness and hypotonia during childhood. Clinical features include motor delay, feeding difficulties, limb weakness, joint contractures, and scoliosis. A report is presented of the anaesthetic management of a 3-year-old girl with CFTD myopathy associated with a mutation of the TPM3 gene, scheduled for adenotonsillectomy because of obstructive sleep apnoea hypopnoea syndrome (OSAHS). The main concerns were the possible susceptibility to malignant hyperthermia, the risk of anaesthesia-induced rhabdomyolysis, a greater sensitivity to non-depolarising muscle relaxants, and the presence of OSAHS. Total intravenous anaesthesia with propofol and the use of rocuronium/sugammadex appear to be safe options. Given the high risk of respiratory compromise and other complications, patients should be closely monitored in the post-operative period

Descriptive study of ultrasound images of the upper airway obtained after insertion of laryngeal mask. / Imágenes ultrasonográficas de vía aérea superior tras la inserción de mascarilla laríngea: estudio descriptivo y utilidad clínica.

OBJECTIVE: To evaluate clinical usefulness of ultrasound images of the upper airway in order to check correct laryngeal mask placement. MATERIAL AND METHODS: A prospective observational study was conducted on patients scheduled for abdominal surgery under general anaesthesia, in whom the patency of the upper airway was ensured using an Ambu®AuraGainTM laryngeal mask. An ultrasound scan was performed of the upper-airway in the cranio-caudal direction and with longitudinal scans in the anterior midline and parasagittal axis, in three moments: before, after inserting and after removing the mask. All recorded images were evaluated in a second time by a radiologist-expert in upper airway ultrasound. Subsequently, the ultrasound data were related to the clinical difficulty of the insertion and presence of air leaks. RESULTS: Data was collected from 30 patients (20 females and 10 males) being operated on for abdominal hysterectomy (15), eventroplasty (6), uterine myomectomy (3), and umbilical (4) and inguinal herniorrhaphy (2). The blind insertion of the masks did not present difficulties in 24 (80%) patients. Air leakage was detected in 8 (26.7%) patients, which was moderate in 7 cases and severe in one of them. The ultrasound findings confirmed good mask placement in 22 (73.3%) patients. Anatomical airway changes after laryngeal mask extraction were only observed in 3 (12%) patients, all of them minor. There was a statistically significant association (P<.05) between difficulty in inserting the device and the level of air leakage. CONCLUSIONS: Upper airway ultrasound is a useful diagnostic method to evaluate laryngeal mask placement. Laryngeal oedema was not observed after removal of the device.

Anaesthetic management of a paediatric patient with congenital fibre type disproportion myopathy. / Tratamiento anestésico en una paciente pediátrica con miopatía congénita por desproporción del tipo de fibras.

Congenital fibre type disproportion (CFTD) is a rare type of myopathy that is characterised by muscle weakness and hypotonia during childhood. Clinical features include motor delay, feeding difficulties, limb weakness, joint contractures, and scoliosis. A report is presented of the anaesthetic management of a 3-year-old girl with CFTD myopathy associated with a mutation of the TPM3 gene, scheduled for adenotonsillectomy because of obstructive sleep apnoea hypopnoea syndrome (OSAHS). The main concerns were the possible susceptibility to malignant hyperthermia, the risk of anaesthesia-induced rhabdomyolysis, a greater sensitivity to non-depolarising muscle relaxants, and the presence of OSAHS. Total intravenous anaesthesia with propofol and the use of rocuronium/sugammadex appear to be safe options. Given the high risk of respiratory compromise and other complications, patients should be closely monitored in the post-operative period.

Estimating the predictive validity of diabetic animal models in rosiglitazone studies.

For therapeutic studies, predictive validity of animal models - arguably the most important feature of animal models in terms of human relevance - can be calculated retrospectively by obtaining data on treatment efficacy from human and animal trials. Using rosiglitazone as a case study, we aim to determine the predictive validity of animal models of diabetes, by analysing which models perform most similarly to humans during rosiglitazone treatment in terms of changes in standard diabetes diagnosis parameters (glycosylated haemoglobin [HbA1c] and fasting glucose levels). A further objective of this paper was to explore the impact of four covariates on the predictive capacity: (i) diabetes induction method; (ii) drug administration route; (iii) sex of animals and (iv) diet during the experiments. Despite the variable consistency of animal species-based models with the human reference for glucose and HbA1c treatment effects, our results show that glucose and HbA1c treatment effects in rats agreed better with the expected values based on human data than in other species. Induction method was also found to be a substantial factor affecting animal model performance. The study concluded that regular reassessment of animal models can help to identify human relevance of each model and adapt research design for actual research goals.

Contribution of animal models to the understanding of the metabolic syndrome: a systematic overview.

The metabolic syndrome (MetS) is one of the most important challenges to public health and biomedical research. Animal models of MetS, such as leptin-deficient obese mice, obese spontaneously hypertensive rats, JCR: LA-cp rats and the Ossabaw and Göttingen minipigs, have contributed to our understanding of the pathophysiological basis and the development of novel therapies. For a complex disease syndrome, no animal model can be expected to serve all needs of research. Although each animal model has limitations and strengths, used together in a complementary fashion, they are essential for research on the MetS and for rapid progress in understanding the aetiology and pathogenesis towards a cure. The purpose of this review is to assess how current animal models contributed to our knowledge of the human MetS, and to systematically evaluate the strengths and weaknesses of the currently available 78 animal models from 11 species.

[Osteogenon in therapy of distal radial bone fractures in patients with secondary osteoporosis].

AIM: To assess efficacy of osteogenon in consolidation of solitary fractures of distal part of the radial bone (RB) in patients with secondary osteoporosis (SO) due to rheumatoid arthritis (RA) and chronic renal failure (CRF). MATERIAL AND METHODS: The study group consisted of 7 patients with documented RA and 8 patients with CRF caused by chronic glomerulonephritis or chronic pyelonephritis in the predialysis period. The control group comprised 13 patients (6 and 7 patients, respectively). The patients were matched by clinico-demographic and therapy characteristics. Patients of the study group received osteogen from the fracture diagnosis to its consolidation. RESULTS: Osteogenon has an analgetic effect, improves well being, raises physical activity, reduces duration of fracture consolidation in patients with SO.

[Activity of genetically programmed cell death of lymphocytes in rheumatoid arthritis].

AIM: To study activity of genetically programmed cell death (PCD) of lymphocytes in patients with rheumatoid arthritis (RA). MATERIAL AND METHODS: Lymphocytes from 30 RA patients including 14 patients with RA history up to 2 years, and 12 healthy donors were studied for activity of caspase 4, 6, 8 usingfluorescence and caspase substrates 30, 60, 120, 150 and 180 min after start of the reaction and number of 1-2-thread serration of DNA with fluorescence of two DNA-tropic stains--EtBr and 4,6-DAPY Correlation was also studied between lymphocyte activity and RA activity, x-ray stage, duration, level of TNF-alpha. The trend in PCD activity of lymphocytes in immunosuppressive therapy was analysed. RESULTS: The activity of lymphocytic caspases in RA patients was low compared to healthy donors. A negative correlation was found between RA activity and activity of caspase 8 and 6. In RA therapy with methotrexate, sulphasalasine, glucocorticosteroids the activity of caspases and number of 1-thread serrations of DNA were subnormal. CONCLUSION: RA is associated with lymphocyte PCD disorders which may be involved in RA pathogenesis, in formation of clones of potentially autoaggressive lymphocytes, in particular. This process is not normalized by current methods of RA treatment causing unsatisfactory outcomes of RA therapy.

[Radiation-modifying effect of oxygen barotherapy in complex treatment of patients with rheumatoid arthritis and osteoarthrosis]. / Radiomodifitsiruiushchii éffekt oksigenobaroterapii v kompleksnom lechenii bol'nykh revmatoidnym artritom i osteoartrozom.

AIM: To evaluate the efficiency of radiotherapy (RT) in combination with oxygen barotherapy (OBT) versus PT used in the complex therapy for rheumatoid arthritis (RA) and osteoarthrosis (OA). MATERIALS AND METHODS: 46 patients with RA and 18 patients with OA were examined, of them 24 patients with RA and 10 with OA received complex therapy involving a course of OBT. The groups of patients had no statistically significant differences in the characteristics of their disease and in the nature of the therapy performed. All the patients received RT as radiation therapy. The patients were followed up for 2 years by assessing basic clinical (the Richi articular index, total pain index, local articular index, pain index for knee and hand joints, circumference of knee and wrist joints) and ultrasound (the magnitude of exudate, an erosive process, osteophytes, articular fissure stenosis, the thickness of the synovial membrane and cartilage) indices. RESULTS: Use of OBT in patients with RA and OA just before articular radiation therapy brought about a more pronounced positive effect of complex therapy. In addition to significant positive changes in clinical parameters, there was a slow progression of a pathological process, as evidenced by ultrasound study. CONCLUSION: By reducing needs for drug therapy, for nonsteroidal antiflammatory drugs in particular, OBT produces a pharmacoeconomic effect.