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Background: Early treatment is the only potential cure for periampullary cancer. The pathway to surgery is complex and involves multiple procedures across local and specialist hospitals. The aim of this study was to analyse variability within this pathway, and its impact on cost and outcomes. Methods: Patients undergoing surgery for periampullary cancer (2011-2016) were identified retrospectively and their pathway to surgery was analysed. Patients who had early surgery (shortest quartile, Q1) were compared with those having late surgery (longest quartile, Q4). Results: A total of 483 patients were included in the study, with 121 and 124 patients in Q1 and Q4 respectively. The median time from initial CT to surgery was 21 days for Q1 versus 112 days for Q4 (P < 0·001). Diagnostic delays were common in Q4; these patients required significantly more investigations than those in Q1 (endoscopic ultrasonography (EUS): 74·2 versus 18·2 per cent respectively, P < 0·001; MRI: 33·6 versus 20·6 per cent, P = 0·036). The median time to diagnostic EUS was 13 days in Q1 versus 59 days in Q4 (P < 0·001). Some 42·1 per cent of jaundiced patients in Q1 underwent preoperative biliary drainage, compared with all patients in Q4. There were significantly more unplanned admissions and associated longer duration of hospital stay per patient and costs in Q4 than in Q1 (median: 8 versus 3 days respectively; 5652 versus 2088; both P < 0·001). There was a higher likelihood of potentially curative surgery in Q1 (82·6 per cent versus 66·9 per cent in Q4; P = 0·005). Conclusion: There is wide variation across the entire pathway, suggesting that multiple strategies are required to enable early surgery. Defining an effective pathway by anticipating the need for investigations and avoiding biliary drainage reduces unplanned admissions and costs and increases resection rates.
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Neoplasias Pancreáticas , Tiempo de Tratamiento/estadística & datos numéricos , Anciano , Ampolla Hepatopancreática/cirugía , Femenino , Humanos , Masculino , Persona de Mediana Edad , Neoplasias Pancreáticas/diagnóstico , Neoplasias Pancreáticas/economía , Neoplasias Pancreáticas/epidemiología , Neoplasias Pancreáticas/cirugía , Pronóstico , Estudios Retrospectivos , Tomografía Computarizada por Rayos XRESUMEN
BACKGROUND: At the time of planned pancreatoduodenectomy patients frequently undergo exploratory laparotomy without resection, leading to delayed systemic therapy. This study aimed to develop and validate a prognostic model for the preoperative prediction of resectability of pancreatic head tumours. METHODS: This was a retrospective study of patients undergoing attempted resection for confirmed malignant tumours of the pancreatic head in a university hospital in Hannover, Germany. The prognostic value of patient and tumour characteristics was investigated in a multivariable logistic regression model. External validation was performed using data from two other centres. RESULTS: Some 109 patients were included in the development cohort, with 51 and 175 patients in the two validation cohorts. Eighty patients (73·4 per cent) in the development cohort underwent resection, and 37 (73 per cent) and 141 (80·6 per cent) in the validation cohorts. The main reasons for performing no resection in the development cohort were: local invasion of vasculature or arterial abutment (15 patients, 52 per cent), and liver (12, 41 per cent), peritoneal (8, 28 per cent) and aortocaval lymph node (6, 21 per cent) metastases. The final model contained the following variables: time to surgery (odds ratio (OR) 0·99, 95 per cent c.i. 0·98 to 0·99), carbohydrate antigen 19-9 concentration (OR 0·99, 0·99 to 0·99), jaundice (OR 4·45, 1·21 to 16·36) and back pain (OR 0·02, 0·00 to 0·22), with an area under the receiver operating characteristic (ROC) curve (AUROC) of 0·918 in the development cohort. AUROC values were 0·813 and 0·761 in the validation cohorts. The positive predictive value of the final model for prediction of resectability was 98·0 per cent in the development cohort, and 91·7 and 94·7 per cent in the two external validation cohorts. [Corrections added on 18 July 2018, after first online publication: The figures for OR of the variables time to surgery and CA19-9 in the abstract and in Table 3 and Table 4 were amended from 1·00 to 0·99]. CONCLUSION: For preoperative prediction of the likelihood of resectability of pancreatic head tumours, this validated model is a valuable addition to CT findings.
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AIM: The need for developing efficient and safe alternatives to parabens has been growing in the cosmetic and pharmaceutical industries. To this end, the antimicrobial activities and safety of methyl-ß-d-maltoside, methyl-ß-d-maltotrioside and their dodecyl homologues were investigated. METHODS AND RESULTS: Antimicrobial activities of methyl- and dodecyl-ß-d-oligomaltoside have been tested on European pharmacopoeia reference strains. When effective, minimal inhibitory concentrations ranged from 32 to 1024 mg l(-1) . Methyl derivatives exhibited greater antibacterial properties, while their dodecyl homologues were more active on fungal strains. To elucidate the mechanism of action of methyl compounds, enzymatic inhibition assays of key maltose metabolism enzymes have been conducted. Methyl-ß-maltotrioside (MeG3) was found to be effective in inhibiting microbial glucoamylase and α-amylase. MeG3 and dodecyl-ß-maltotrioside cytotoxicity were also checked on HepG2 cells and were found to be low, compared with benzalkonium chloride or parabens. CONCLUSION: Methyl- and dodecyl-ß-maltoside or maltotrioside were found to be active against reference strains used for preservatives efficacy testing. Methyl derivatives could act through an interesting inhibition of the microbial enzymatic metabolism. SIGNIFICANCE AND IMPACT OF THE STUDY: Given their very simple chemical structure, low toxicity and good antimicrobial activity, methyl-ß-d-oligomaltosides could represent a valuable alternative to currently used preservatives such as parabens.
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Antiinfecciosos/farmacología , Glucósidos/farmacología , Conservadores Farmacéuticos/farmacología , Antiinfecciosos/toxicidad , Cosméticos , Inhibidores Enzimáticos/farmacología , Glucósidos/toxicidad , Pruebas de Sensibilidad Microbiana , Parabenos/farmacología , Conservadores Farmacéuticos/toxicidad , alfa-Amilasas/antagonistas & inhibidoresRESUMEN
An electrophysiologic study (EPS) of children and teenagers with paroxysmal supraventricular tachycardia (SVT) and normal electrocardiography (ECG) in sinus rhythm was evaluated. Generally, EPS is performed only before paroxysmal SVT ablation in these patients. In this study, 140 patients (mean age, 15 ± 3 years) with normal ECG in sinus rhythm were studied for SVT by a transesophageal route in baseline state and after isoproterenol. Idiopathic left or right ventricular tachycardia was diagnosed in four patients (3 %). Anterograde conduction over an atrioventricular (AV) left lateral (n = 10) or septal (n = 9) accessory pathway (AP) was noted in 19 patients (13.5 %) at atrial pacing. Orthodromic AV reentrant tachycardia (AVRT) was induced in these children. Five of the patients had a high rate conducted over AP (>240 bpm in baseline state or >290 bpm after isoproterenol). Two of the patients (a 10-year-old girl with well-tolerated SVT and a 17-year-old with syncope-related SVT) had the criteria for a malignant form with the induction of atrial fibrillation conducted over AP at a rate exceeding 290 bpm in baseline state. Of the 140 patients, 74 (53 %) had typical AV node reentrant tachycardia (AVNRT), nine had atypical AVNRT (6 %), 1 had atrial tachycardia (0.7 %), and 33 (23.5 %) had AVRT related to a concealed AP with only retrograde conduction. Electrophysiologic study is recommended for children with paroxysmal SVT and normal ECG in sinus rhythm. The data are helpful for guiding the treatment. Ventricular tachycardia or atrial tachycardia can be misdiagnosed. Masked preexcitation syndrome with anterograde conduction through AP was present in 13.5 % of the patients, and 1.4 % had a malignant preexcitation syndrome.
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Técnicas Electrofisiológicas Cardíacas/métodos , Taquicardia Supraventricular/fisiopatología , Adolescente , Niño , Preescolar , Electrocardiografía , Esófago , Femenino , Estudios de Seguimiento , Humanos , Masculino , Síndromes de Preexcitación , Estudios Retrospectivos , Taquicardia Supraventricular/etiología , Adulto JovenRESUMEN
UNLABELLED: The electrophysiological evaluation of Wolff-Parkinson-White syndrome (WPW) is recommended in children aged more than five years to detect a risk of life-threatening arrhythmia. The purposes of the study were to determine the feasibility of transesophageal EPS in a child between six and 10 years in out-patient clinic. METHODS: Electrophysiological study (EPS) was indicated in 22 children, aged six to 10 years, with a manifest WPW either for no documented tachycardia (n=7), unexplained dizziness (n=2) or for a sportive authorization in 10 asymptomatic children. Two of the last children had a history of permanent tachycardia after the birth but were asymptomatic since the age of one year without drugs. RESULTS: EPS was performed in all children. The main difficulty lied in passing the catheter through the mouth. Programmed stimulation at cycle length of 380 ms was performed in all children to avoid high rates of pacing when the conduction through the accessory pathway (AP) and normal AV system was evaluated. Isoproterenol was not required in five children, because they developed a catecholaminergic sinus tachycardia. The AP refractory period was determined in all children between 200 and 270 ms. Orthodromic reentrant tachycardia (RT) was induced in 11 children, three asymptomatic children (27%), seven complaining of tachycardia and one with syncope. Rapid antidromic tachycardia was induced in this last child with dizziness. Atrial fibrillation was never induced. CONCLUSIONS: Esophageal EPS can be performed without sedation in a young child six to 10-year-old with a shortened protocol of stimulation, which was capable to clearly evaluate the WPW-related risks.
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Sedación Consciente , Técnicas Electrofisiológicas Cardíacas/métodos , Sistema de Conducción Cardíaco/fisiopatología , Síndrome de Wolff-Parkinson-White/diagnóstico , Síndrome de Wolff-Parkinson-White/fisiopatología , Niño , Preescolar , Estudios de Factibilidad , Humanos , Pacientes AmbulatoriosRESUMEN
AIMS: To determine the feasibility and the results of exercise testing (ET) and electrophysiological study (EPS) in outpatient asymptomatic children with a Wolff-Parkinson-White (WPW) syndrome. METHODS AND RESULTS: Exercise testing and transesophageal EPS were performed in 55 outpatient asymptomatic children aged 6 to 19 years old (14 +/- 3) with WPW. Wolff-Parkinson-White persisted during maximal exercise. Isoproterenol was not required in five children younger than 10 years old, because they developed a catecholaminergic sinus tachycardia. Maximal rate conducted through accessory pathway (AP) was higher in children younger than 16 years old than in teenagers (P < 0.05). Atrioventricular re-entrant tachycardia (AVRT) was induced in six children; atrial fibrillation (AF) in 12 children. The induction of tachycardias and the dangerous forms (18%) were not influenced by age. After 5 +/- 1 years, one child, 12 year old with inducible rapid AF, had a sudden cardiac arrest; two children became symptomatic after ablation. CONCLUSIONS: Transesophageal EPS was required to determine the prognosis of asymptomatic WPW in children. The maximal rate conducted in AP was higher in children younger than 16 years old than in teenagers; other data did not differ. AVRT was rare; 71% of children had no inducible arrhythmia and were authorized to resume physical activities.
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Cardiología/métodos , Técnicas Electrofisiológicas Cardíacas , Síndromes de Preexcitación/fisiopatología , Adolescente , Adulto , Fibrilación Atrial , Niño , Muerte Súbita Cardíaca/prevención & control , Electrofisiología/métodos , Estudios de Factibilidad , Femenino , Humanos , Isoproterenol/farmacología , Masculino , Síndromes de Preexcitación/diagnóstico , Pronóstico , Medición de Riesgo , Resultado del Tratamiento , Síndrome de Wolff-Parkinson-White/diagnóstico , Síndrome de Wolff-Parkinson-White/fisiopatologíaRESUMEN
DNA repair polymorphisms may represent susceptibility factors affecting DNA integrity, and possibly cancer risk, in human population. In order to elucidate the influence of a few widely studied DNA repair polymorphisms on individual levels of DNA damage and their possible interaction with lifestyle and environmental exposures, 171 subjects from a well-characterized human population enrolled in a previous study on genetic effects of air pollution were genotyped for the XRCC1 Arg280His and Arg399Glu, XRCC3 Thr241Met and ERCC2 Lys751Gln polymorphisms. The association between DNA repair genotype, alone or in combination with metabolic genotype, on the levels of SCE, micronuclei and tail moment values in peripheral lymphocytes was evaluated. A significant influence of the ERCC2 genotype on SCE frequency was observed. Subjects with ERCC2 751 Gln/Gln genotype had significantly higher risk of high (above the median) SCE/cell with respect to Lys/Lys referents (OR 4.55, 95% CI 1.48-13.99). A non-significantly elevated OR was also observed in Gln/Lys heterozygotes, suggesting a gene dosage effect. When subjects were categorized by smoking habits and professional exposure, the variant ERCC2 751 Gln/Gln genotype was associated with elevated SCE rates in non-smokers and in exposed subjects, but not in smokers. The results of this study support the hypothesis that some DNA repair polymorphisms exert a modifying effect on individual levels of DNA damage in healthy subjects, possibly also modulating cancer risk.
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Daño del ADN , Reparación del ADN , Polimorfismo Genético , Adulto , Contaminantes Ocupacionales del Aire/toxicidad , Femenino , Marcadores Genéticos , Genotipo , Humanos , Italia , Linfocitos/citología , Linfocitos/efectos de los fármacos , Masculino , Persona de Mediana Edad , Mutágenos/toxicidad , Exposición Profesional , Fumar , Proteína de la Xerodermia Pigmentosa del Grupo D/genética , Proteína de la Xerodermia Pigmentosa del Grupo D/metabolismoRESUMEN
UNLABELLED: Implantable defibrillator is the recognized treatment of sudden cardiac death. Miniaturization of the devices allows implantation in children. METHODS: This multicentric retrospective study analyzed data of 33 children aged 18 years and less who were implanted from 1990 to 2005. RESULTS: Age of patients are 10 to 18 years, 20 patients were implanted after a resuscitation of sudden death, 10 after a syncope, 2 after a ventricular tachycardia and 1 in a prophylactic way. Tachycardias on primary electrical disease are most frequent (46%). Hypertrophic cardiomyopathy accounts for 22%, DAVD for 14%, congenital cardiopathies for 12%. Seventeen patients received appropriate shocks and 14 patients had inappropriate shocks. There were two unexplained deaths. Five leads fractures and two device infections were noted. CONCLUSION: Implantable defibrillator is an effective treatment for children high-risk of sudden death. Occurrence of inappropriate shocks due to sinusal tachycardia, infections and leads fractures are frequent.
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Muerte Súbita Cardíaca/prevención & control , Desfibriladores Implantables , Adolescente , Cardiomiopatía Hipertrófica/terapia , Niño , Muerte Súbita Cardíaca/etiología , Femenino , Estudios de Seguimiento , Francia , Cardiopatías Congénitas/terapia , Humanos , Masculino , Registros Médicos , Estudios Retrospectivos , Taquicardia Ventricular/terapia , Resultado del TratamientoRESUMEN
OBJECTIVES: To investigate the clinical profile, natural history, and optimal management of persistent or permanent junctional reciprocating tachycardia (PJRT) in children. METHODS AND RESULTS: 85 patients meeting the ECG criteria for PJRT were enrolled in a retrospective multicentre study. Age at diagnosis varied from birth to 20 years (median 3 months). Follow up ranged from 0.1 to 26.0 (median 8.2) years. At the time of referral, 24 of 85 patients (28%) had congestive heart failure that was resolved with medical treatment in all patients. Eighty three patients received drug treatment initially. Amiodarone and verapamil were the most effective with a success rate of 84-94% alone or in association with digoxin. Radiofrequency ablation of the accessory pathway was performed in 18 patients. There was a trend for a relation between age at ablation and the result of the procedure, failures being more common in younger patients (three of six procedures in younger and 15 of 18 in older children were successful; p = 0.14). Two patients with persistent left ventricular dysfunction on echocardiography but with no symptoms of congestive heart failure died suddenly one month and three years after diagnosis. PJRT resolved spontaneously in 19 patients (22%). Age at diagnosis of PJRT was not a predictor of spontaneous resolution. CONCLUSIONS: PJRT is a potentially lethal arrhythmia in children with tachycardia induced cardiomyopathy. Spontaneous resolution of tachycardia is not uncommon. Antiarrhythmic treatment is often effective. Radiofrequency ablation should be performed in older children or when rate is not controlled, especially in patients with persistent left ventricular dysfunction.
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Taquicardia Paroxística/terapia , Adolescente , Adulto , Antiarrítmicos/uso terapéutico , Ablación por Catéter , Niño , Preescolar , Femenino , Estado de Salud , Insuficiencia Cardíaca/etiología , Humanos , Lactante , Recién Nacido , Masculino , Remisión Espontánea , Estudios Retrospectivos , Taquicardia Paroxística/etiologíaRESUMEN
The creation of a paediatric surgical unit requires autoevaluation in order to: assess the quality of the results with respect to recognised international standards, answer the family's questions about the results obtained and adhere to criteria of accreditation Between January 2003 and December 2004, 201 consecutive patients, children (N= 164) or operated for adult congenital heart disease (N= 37) were treated. No patient was excluded. The RACHS-1 risk score, the ARISTOTLE scores of complexity and performance and the CUSUM and VLAD graphic analyses were applied to the study of hospital mortality. An original "variable performance-adjusted display" (VPAD) graphic analysis was performed to show up any possible variations of performance. Paediatric hospital survival was 97.56% (95% CI: 93.9 - 99.1). The paediatric complexity and performance scores were 6.79 +/- 0.22 and 6.62 respectively. In the absence of statistical significance in this field of autoevaluation, graphic analyses indicated the performance of our unit with no "learning" curves. Graphic scores and analyses allow assessment of the function of a paediatric cardiac surgical unit and the variations of complexity with respect to time, before the appearance of statistical significance. The ARISTOTLE complexity and performance scores and their adaptation in VPAD seem to be more reliable and discriminating than the RACHS-1 score.
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Procedimientos Quirúrgicos Cardiovasculares/métodos , Procedimientos Quirúrgicos Cardiovasculares/estadística & datos numéricos , Cardiopatías Congénitas/clasificación , Cardiopatías Congénitas/cirugía , Pediatría/estadística & datos numéricos , Adolescente , Automatización , Niño , Preescolar , Gráficos por Computador , Femenino , Humanos , Lactante , Recién Nacido , Masculino , Evaluación de Resultado en la Atención de Salud/estadística & datos numéricos , Pronóstico , Valores de Referencia , Medición de Riesgo , SobrevidaRESUMEN
The authors undertook a retrospective study of the modes of prescription, the tolerance and efficacy of prostaglandin E1 in 62 consecutive neonates with congenital heart disease (average Age 1.6 days: 35 boys: weight: 3.1 +/- 0.6 Kg) admitted to the paediatric intensive care unit of Nancy University Hospital between 1998 and 2002. The infusion time and cumulative dosage were 134 +/- 112 (6-480) hours and 111 +/- 94 (4-396) microg/Kg respectively. The side effects that were observed were: Apnoea (19%), abdominal distension (16%), bradycardia (13%), enterocolitis (6.5%), hypotension (6.5%), vomiting (5%), fever (1.6%) and skin rash (1.6%). Gastrointestinal disturbances are associated with a low body weight (p<0.04), to prolonged treatment (p<0.02) with no influence of initial or cumulative dosages (P=NS), with respiratory assistance (p<0.03) and longer hospital stay (p<0.01). Hypotension was commoner in cases of poor neonatal adaptation. Mortality was correlated with severe initial acidosis (p<0.02), a low Apgar score, the initial prolonged use of high doses of prostaglandin (p<0.04), and the presence of severe valvular aortic stenosis or hypoplasia of the left heart (p<0.002). The authors conclude that treatment with prostaglandin is effective in the majority of cases despite the use of low maintenance doses (0.01 microg/Kg/min). Gastrointestinal disturbances favourised by the perinatal context, the cardiac disease, and prolonged treatment are significant factors for morbidity and mortality. The beneficial role of early neonatal enteral feeding was not demonstrated in this high risk population.
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Alprostadil/efectos adversos , Alprostadil/uso terapéutico , Cardiopatías Congénitas/tratamiento farmacológico , Vasodilatadores/efectos adversos , Vasodilatadores/uso terapéutico , Apnea/inducido químicamente , Peso Corporal , Enterocolitis/inducido químicamente , Exantema/inducido químicamente , Femenino , Francia , Humanos , Recién Nacido , Unidades de Cuidado Intensivo Pediátrico/estadística & datos numéricos , Masculino , Estudios Retrospectivos , Factores de Riesgo , Vómitos/inducido químicamenteRESUMEN
UNLABELLED: An electrophysiological investigation is the most reliable means of detecting malignant forms of Wolff-Parkinson-White syndrome (WPW). However, an endocavity investigation is an invasive procedure, especially in young subjects with few symptoms. The aim of this study was to examine the feasibility and results of an electrophysiological study performed by the transoesophageal route in children with WPW. The study was performed in 70 children aged between 11 and 19 years (mean 15 +/- 3) with an obvious ECG appearance of WPW: 13 had dizziness or syncope (group I), 25 had tachycardia (group II) and 32 were asymptomatic (group III). The ages were similar in all three groups. The transoesophageal electrophysiological investigation without premedication consisted of atrial stimulation at increasing frequencies and programmed atrial stimulation using one and two extra stimuli delivered in the basal state and after infusion of 2 to 5 microg of isoproterenol. RESULTS: The investigation was completed in all the children except one in group II. A paroxysmal junctional tachycardia was induced in 7 group I children (54%), 22 in group II (92%) and 4 in group III (12.5%). Atrial fibrillation lasting more than one minute was induced in 7 group I children (54%), 6 in group II (25%) and 6 in group III (19%). The percentage of malignant forms combining rapid conduction in the bundle of Kent at a rate of more than 240/min in the basal state or more than 300/min with isoproterenol, and atrial fibrillation was 54% in group I, 21% in group II, and 22% in group III. In conclusion, a transoesophageal electrophysiological investigation was possible as an outpatient procedure in children older than 10 years, and allowed the detection of potentially serious forms whatever the indication for the investigation, with nevertheless a significantly higher incidence in those presenting with dizziness or syncope. The incidence of 22% for potentially malignant forms in asymptomatic children provides an incentive to recommend an ECG in all children older than 10 years participating in an active sport in order to detect WPW and to propose oesophageal investigation.
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Técnicas Electrofisiológicas Cardíacas , Síndrome de Wolff-Parkinson-White/diagnóstico , Adolescente , Adulto , Factores de Edad , Niño , Mareo/etiología , Electrocardiografía , Femenino , Humanos , Masculino , Pacientes Ambulatorios , Sensibilidad y Especificidad , Síncope/etiología , Síndrome de Wolff-Parkinson-White/fisiopatologíaRESUMEN
Hypothyroidism is a well recognized complication of 22q11.2 deletion syndrome. Auto-immune hyperthyroidism is less common. We report three patients with a 22q11.2 deletion and Graves' disease diagnosed at age 17, 14 and 11 years, respectively. The clinical and biological presentation was typical for auto-immune hyperthyroidism. Graves' disease should be periodically sought during the follow-up program of patients with 22q11.2 deletion syndrome.
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Deleción Cromosómica , Cromosomas Humanos Par 22 , Enfermedad de Graves/genética , Adolescente , Adulto , Mapeo Cromosómico , Femenino , Humanos , MasculinoRESUMEN
UNLABELLED: We studied 52 consecutive patients with Kawasaki disease hospitalized (1984 -2003) during the acute phase (mean age 2.5 + 2.4 years; range 0.3 to 16 years, 34 males, 18 cases with coronary aneurysms, median follow-up 6.7 years), and identified a subgroup presenting a refractory subtype to immunoglobulin therapy. RESULTS: forty-nine infants benefited from a first regimen of immunoglobulins, 8.4 + 6 days following the onset of symptoms. Eleven infants (1.4 + 1.2 years, range 0.3 - 4.3 years, median 1.7 years) were non-responders, with coronary aneurysms in 8 cases (giant aneurysms (>8 mm) in 4 cases). These 11 infants were treated a second time by immunoglobulins, but 6 cases (1.8 + 1.6 years, with two cases of severe ventricular dysfunction and 2 cases of fatal myocardial infarction) required an additive therapy with (oral or IV route) corticosteroids (2) and cyclophosphamide bolus (4) with or without repetitive plasmapheresis (4). Non-responder patients had their treatment onset later (p<0.0003) using higher dosages (p<0.005), a longer delay for fever or biological signs correction (p<0.02), a worsening of coronary lesions (p<0.05) with more coronary secondary aneurysms (p<.005). The aneurysms, more frequent at the second phase of the disease (p<0.0001) are associated with: a younger age (p<0.03), a lower weight (p<0.02), a later onset of treatment (p<0.03), prolonged fever or inflammatory syndrome (p<0.05), higher level of fibrinogene (p<0.02). The overall mortality (5.7%) is correlated with giant aneurysms (p<0.001), myocardial ischemia (p<0.0001), heart failure (p<0.0001), and lack of early response to treatment (p<0.003). CONCLUSION: immunoglobin therapy can be repeated. In case of severe forms, the use of corticosteroids, cyclophosphamide and plasmapheresis may be proposed.
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Inmunoglobulinas/uso terapéutico , Síndrome Mucocutáneo Linfonodular/tratamiento farmacológico , Adolescente , Niño , Preescolar , Femenino , Estudios de Seguimiento , Humanos , Lactante , Masculino , Estudios RetrospectivosRESUMEN
We report the case of a 39 day old infant, hospitalised for congenital cardiopathy associated with type A blockage of the aortic arch with a large type I aortopulmonary window. The infant was in cardiogenic shock with pulmonary systemic hypertension and a tightly stenosed arterial canal (< 2 mm). With no possibility of re-opening the arterial canal under PGE1 at this stage, complete repair was performed as an emergency. After section of the aortopulmonary window, it was closed on the pulmonary side with a patch of autologous pericardium. Repair of the aortic arch was performed without prosthetic material, under selective cerebral perfusion to protect the brain parenchyma, after mobilisation of the descending thoracic aorta, which was anastomosed directly with the distal part of the window and aortic arch. Recovery was uncomplicated, with no residual lesion at 6 month post-operative follow up. The late clinical presentation of this patient shows the effect of medical management without prior catheterisation, with operative techniques minimising peri-operative tissular ischaemia and conserving aortic and pulmonary growth potential.
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Anomalías Múltiples , Aorta Torácica/anomalías , Arteria Pulmonar/anomalías , Anomalías Múltiples/diagnóstico por imagen , Aorta Torácica/diagnóstico por imagen , Humanos , Lactante , Masculino , Arteria Pulmonar/diagnóstico por imagen , Factores de Tiempo , UltrasonografíaRESUMEN
The human-Chinese hamster hybrid cell line XR-C1#8, containing human chromosome 8, was used as a model system to investigate the relative importance of cellular enzymatic environment and chromosomal structure for modulating the efficiency of repair of UV-induced DNA damage. The hybrid cells were irradiated with UVC light and the extent of cytogenetic damage, detected as frequencies of sister chromatid exchanges (SCEs), was compared between the human and the hamster chromosomes. The combination of immunofluorescent staining for SCEs and chromosome painting with fluorescence in situ hybridization allowed the simultaneous analysis of SCEs in the human and hamster chromosomes. The aim of the present study was to determine if the differences in biological response to comparable UV treatments observed between human and hamster cells were maintained in the hybrid cells in which human and hamster chromosomes are exposed in the same cellular environment. The analysis of replication time of human chromosome 8 indicated the active status of this chromosome in XR-C1#8 hybrid cells. The frequencies of SCEs for human chromosome 8 and a hamster chromosome of comparable size were 0.35 +/- 0.52, 0.80 +/- 0.73, 1.24 +/- 2.24 and 0.36 +/- 0.12, 0.71 +/- 0.2, 0.97 +/- 0.27, respectively, after irradiation with 0, 5, and 10 J/m2. The persistence of UV-induced SCEs after three cell cycles was also analyzed, both for the human and hamster chromosomes. The observed frequencies of SCEs were 0.40 +/- 0.57, 0.62 +/- 1.05, 0.58 +/- 0.83 and 0.26 +/- 0.08, 0.67 +/- 0.18, 0.69 +/- 0.24, in human and hamster chromosomes respectively, after treatment with 0, 10, and 20 J/m2 of UVC light. No significant differences could be observed between the human and hamster chromosomes. These results suggest that the enzymatic environment of human and hamster cells has the main role, in comparison to the structural organization of human and hamster chromosomes, for determining the different level of repair of UV-induced DNA damage observed in these two species.
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Cromosomas Humanos Par 8/efectos de la radiación , Cricetulus/genética , Proteínas de Unión al ADN , Células Híbridas/ultraestructura , Intercambio de Cromátides Hermanas/efectos de la radiación , Animales , Pintura Cromosómica , Cromosomas/efectos de la radiación , Cromosomas/ultraestructura , Cromosomas Humanos Par 8/ultraestructura , Cricetinae , Daño del ADN , Reparación del ADN , Replicación del ADN , Proteína Quinasa Activada por ADN , Colorantes Fluorescentes , Humanos , Células Híbridas/efectos de la radiación , Hibridación Fluorescente in Situ , Proteínas Nucleares , Proteínas Serina-Treonina Quinasas/genética , Proteínas Serina-Treonina Quinasas/metabolismo , Tolerancia a Radiación , Especificidad de la Especie , Factores de Tiempo , Rayos UltravioletaRESUMEN
We report the case of a 22-year-old primigravida patient presenting pulmonary atresia with intact ventricular septum, a rare congenital heart disease. Caesarean delivery was performed at 32 weeks of gestation because of moderate maternal function deterioration and foetal growth restriction. General anaesthesia was used because of the presence of lumbar Harrington rods. The administration of etomidate, celocurine and a continuous perfusion of remifentanil right from induction ensured haemodynamic stability and a rapid emergence and recovery.
Asunto(s)
Anestesia General , Cesárea , Atresia Pulmonar/fisiopatología , Adulto , Femenino , Retardo del Crecimiento Fetal/etiología , Hemodinámica/fisiología , Humanos , Monitoreo Intraoperatorio , EmbarazoRESUMEN
This study evaluates the problems and the evolution of cardiac stimulation in infants (aged < 3.5 years) by comparing the endocavity and epicardial routes in a retrospective series of 37 patients. Thirty seven patients aged 1.2 +/- 0.9 years treated with epicardial (n = 19) or endocavity (n = 18) stimulation were followed for 10.9 +/- 6.4 years (0.75-24). The 2 patient groups did not differ in age or weight. Four patients were lost to follow up, and 1 died. The functional duration of the first stimulator was not significantly different if the initial approach was epicardial or endocavity. The endocavity probes were introduced by venous denudation in 15 cases and by subclavian puncture in 3 cases. Fourteen of the 19 children fitted by the epicardial route went on to endocavity stimulation, of which 10 were at the first replacement. None of the 18 patients fitted by the endocavity route went on to epicardial stimulation. Out of 11 endocavity probe replacements and 9 atrialisations, the homolateral venous approach was always possible except in 2 cases. In conclusion, the results for the epicardial and endocavity routes are comparable. For technical reasons (calibre of the veins, size of the stimulator) it would appear reasonable if the endocavity route was used, making do initially with a mono chamber stimulation. The advances in the epicardial electrodes abolishes the major handicap (threshold elevation) of this approach which can be advocated when double chamber stimulation seems preferable.
Asunto(s)
Estimulación Cardíaca Artificial/métodos , Bloqueo Cardíaco/terapia , Marcapaso Artificial , Estimulación Cardíaca Artificial/efectos adversos , Preescolar , Femenino , Bloqueo Cardíaco/etiología , Cardiopatías Congénitas/complicaciones , Humanos , Lactante , Recién Nacido , Masculino , Marcapaso Artificial/efectos adversos , Estudios RetrospectivosRESUMEN
The objective of this work was to study the long term evolution of a retrospective series of 54 patients affected with congenital isolated complete atrio-ventricular block (CAVB) and to analyse the value of the different methods used for surveillance. Our series included 54 patients affected with isolated CAVB, without associated cardiopathy, diagnosed at an average age of 5.3 +/- 5.5 years, of which 9 were in utero. The average duration of follow up was 14.5 +/- 9.6 years. During the evolution, a cardiac stimulator was placed in 41 patients (76%) at a relatively late average age of 13.3 +/- 9 years, significantly lower for CAVB diagnosed before the age of 1 year (9.6 +/- 7.4 years) than for those diagnosed after the age of 1 year (16.2 +/- 9.2 years) (p < 0.02). The approach was endocavitary in 39 cases and epicardial in 2 cases. The only 2 deaths in our series (4%) concerned 2 patients of 18 and 26 years already fitted with a stimulator. Three patients progressed to severe dilated cardiomyopathy despite implantation of a stimulator. The decisive arguments for implantation of a PM were clinical (11 patients), Holter ECG (25 patients), stress test (17 patients), electrophysiological investigation (5 patients), echocardiography (3 patients) and surgical intervention (2 patients). In conclusion, our study confirms the good prognosis of isolated congenital complete atrio-ventricular block, but underlines the possible progression in rare cases in spite of stimulation towards dilated cardiomyopathy for which the aetiology remains uncertain. Three quarters of the patients required a stimulator at a somewhat late age.