Structural and functional abnormalities of large arteries in the Turner syndrome.

OBJECTIVE: To analyse the structural and functional abnormalities in the large arteries in women with the Turner syndrome. METHODS: Aortic stiffness (assessed by means of the carotid femoral pulse wave velocity), level of amplification of the carotid pressure wave (by applanation tonometry), and carotid remodelling (by high resolution ultrasound) were studied in women with the Turner syndrome. Clinical and ambulatory blood pressures were taken into account in the analysis. Thus, 24 patients with the Turner syndrome and 25 healthy female subjects matched for age were studied. RESULTS: Women with the Turner syndrome had a higher augmentation index than the controls (Turner, mean (SD) 0.04 (0.14) v controls, -0.14 (0.13), p < 0.001) but a lower peripheral pulse pressure (39 (8) mm Hg v 47 (11) mm Hg, p = 0.010 in the clinic; 44 (5) mm Hg v 47 (6) mm Hg, p = 0.036 during the 24 hour ambulatory recording). The luminal diameter of the common carotid artery and the carotid-femoral pulse wave velocity were similar in the two groups, whereas carotid intima-media thickness tended to be higher in women with the Turner syndrome (0.53 (0.06) mm v 0.50 (0.05) mm, p = 0.06). After correction for body surface area, carotid intima-media thickness and pulse wave velocity were higher in women with the Turner syndrome. CONCLUSIONS: Vascular abnormalities observed in the Turner syndrome are implicated in the origin of the cardiovascular complications that occur in this syndrome. These abnormalities are morphological but also functional. An increase in the augmentation index can be explained in part by the short height of these patients.

Transcatheter closure of patent foramen ovale in patients with platypnea-orthodeoxia: results of a multicentric French registry.

BACKGROUND: Dyspnea and the decrease in arterial saturation in the upright position in elderly subjects is described as platypnea-orthodeoxia syndrome (POS). POS is secondary to the occurrence of an atrial right-to-left shunt through a patent foramen ovale (PFO). METHODS: This French multicentric study reports on 78 patients (mean age 67 +/- 11.3 years) with POS who had transcatheter closure of the PFO; frequently associated diseases were pneumonectomy (n = 36) and an ascending aortic aneurysm (n = 11). In all patients, the diagnosis was confirmed by transthoracic or/and transesophageal echocardiography. Five different closure devices were used: Amplatz (n = 45), Cardioseal (n = 13), Sideris (n = 11), Das Angel Wings (n = 8) and Starflex (n = 1). Closure was successful in 76 patients (97%). RESULTS: Oxygen saturation increased immediately after occlusion from 84.6 +/- 10.7% to 95.1 +/- 6.4% (p < 0.001) and dyspnea improved from grade 2.7 +/- 0.7 to grade 1 +/- 1 (p < 0.001). A small residual shunt was immediately observed in 5 patients (3 with the Cardioseal device, 1 with the Sideris and 1 with the Amplatz) leading to the implantation of a second device in one case (Cardioseal). Two early deaths occurred unrelated to the procedure (one due to sepsis probably related to pneumonectomy, another due to respiratory insufficiency). Other complications were: a small shunt between the aorta and the left atrium, two atrial fibrillations and a left-sided thrombus which disappeared with anticoagulant therapy. At a mean follow-up of 15 +/- 12 months, there were 7 late deaths related to the underlying disease. CONCLUSION: Percutaneous occlusion of the foramen ovale is safe and gives excellent results thanks to continuing improvement in available devices. This technique enables some patients in an unstable condition to avoid a surgical closure.

[Congenital long QT syndrome in newborns]. / Syndrome du QT long congénital de révélation néonatale.

UNLABELLED: The perinatal manifestations of the long QT syndrome are rare, but early diagnosis and therapy are necessary to prevent sudden death. CASE REPORTS: A long QT syndrome was diagnosed in two neonates who presented with foetal bradycardia. In one case, a mutation in the gene KCNQ1 was identified, and a long QT syndrome was diagnosed in the mother and two brothers of the neonate. On beta-blocker therapy, one infant became free of long QT syndrome related symptoms, but a sudden death of the second infant occurred. CONCLUSION: The long QT syndrome should be considered in the differential diagnosis of foetal bradycardia. Early treatment of the neonate and his family may prevent ventricular arrhythmias and sudden death.

A dose reconstruction of 60Co-contaminated window frames in a Taiwanese school.

Since 1992, hundreds of buildings in Taiwan were discovered to have 60Co contamination in the structural rebar. The contamination resulted from improper handling of 60Co-contaminated scrap metal in 1982 and 1983, which subsequently was recycled and used throughout Taiwan. Hsin-hsin Kindergarten school enrolled about 600 students over the 10-y period before the contamination was discovered. Hsin-hsin Kindergarten had three 60Co-contaminated steel window frames with measured dose rates on contact up to 150 microSv h(-1). In this study, a range of potential doses received by the Hsin-hsin Kindergarten students were estimated using ISOSHLD dose modeling software. ISOSHLD is a rapid, inexpensive screening tool to reconstruct dose ranges. To assess the potential risks to habitants of the school for the first year after construction, calculated dose rate ranges of 0.08 microSv h(-1) to 75.38 microSv h(-1) were then applied to the International Commission [corrected] on Radiation Protection 60 nominal detriment coefficients for stochastic effects. Risk estimates ranged from 1.46 x 10(-4) to 7.42 x 10(-4) excess fatal cancers per lifetime.

[Angelman syndrome and severe vagal hypertonia. Three pediatric case reports]. / Syndrome d'Angelman et hypertonie vagale sévère. A propos de trois observations pédiatriques.

Angelman's syndrome is an association of severe mental retardation with absence of language, ataxia, convulsions and hyperactive, joyful behaviour with frequent bouts of laughing. Genetic diagnosis is possible in about 80% of cases. No cardiovascular abnormalities have been described in this syndrome to date. The authors report the cases of three children with Angelman's syndrome who presented with severe malaise due to increased vagal tone. The age of onset of symptoms was between 20 months and 8 years. One of the children had malaises triggered by bouts of laughing. The diagnosis was confirmed in all three cases by the results of Holter 24 hour ECG recording and oculo-cardiac reflex. The treatment chosen was Diphemanil (Prantal) in the two patients under 2 years of age (after failure of a trial of betablockers in one case) and Disopyramide for the oldest child with excellent results in all cases. However, one child died suddenly at the age of 6, two years after stopping diphemanil. Based on these observations, the authors suggest that all malaises in patients with Angelman's syndrome should be investigated by Holter ECG and oculo-cardiac reflex (or tilt test). In view of the potential gravity of the syncopal attacks, long-term medical treatment seems to be justified.

[Heart malformations and vascular complications associated with Turner's syndrome. Prospective study of 26 patients]. / Malformations cardiaques et complications vasculaires associées au syndrome de Turner. Etude prospective chez 26 patientes.

Turner's syndrome is associated with congenital heart disease in a third of cases. Several reports of aortic dilatation and of death by dissection or rupture of the aorta have been published. The authors undertook a prospective study to assess the incidence of cardiac malformations and aortic dilatation in genetically confirmed Turner's syndrome. Twenty-six out of 34 patients recalled (76%), aged 7 to 30 years (average 17 +/- 6 years) accepted their inclusion in this study and underwent clinical examination, ECG, chest X-ray and echocardiography. Thirteen patients had a monosomy 45X and 13 a mosaic or structural abnormality. Six had a history of cardiovascular disease (operated coarctation: 2 cases, kinking: 2 cases, Hypertension: 2 cases). Eight patients (30%) had one or several anatomical cardiovascular abnormalities: bicuspid aortic valve (19.2%), abnormalities of the aortic isthmus (kinking or coarctation) (15.4%), aortic regurgitation (7.7%), mitral stenosis (3.8%), partial anomalous venous drainage (3.8%), patent ductus arteriosus (3.8%) and left superior vena cava (11.5%). Systematic evaluation of the aorta resulted in the diagnosis of dilatation of the ascending aorta in 1 case and dilatation of the sinus of Valsalva in 2 other cases. The authors conclude that echocardiographic evaluation is essential after the diagnosis of Turner's syndrome. It should be repeated regularly to detect dilatation of the aorta which carries the risk of serious complications, such as rupture or dissection of the aorta.

Landmarks in burn prevention.

The objective of this paper is to highlight landmarks in burn prevention. Novel strategies in the areas of law and regulation, environmental and consumer product design, and educational programs are identified and discussed. Notwithstanding marked reductions in burn morbidity and mortality, especially in economically developed countries, burn injuries remain an important public health concern throughout the world. More, and more effective, burn prevention programs coupled with renewed efforts to reduce the social and environmental correlates of burn injuries (poverty, overcrowding, family stress, and educational deficits) are needed to further reduce burn incidence and its long-term sequelae.

[Hypertensive manifestation of an acute intestinal intussusception]. / Forme hypertensive d'une invagination intestinale aiguë.

BACKGROUND: Hypertension may be associated with intussusception. CASE REPORT: An 8-month-old infant showed the following symptoms: lethargy, vomiting and hypertension. Abdominal ultrasound suggested the diagnosis of intussusception, which was confirmed by barium enema. The hypertension resolved after the intussusception was reduced. CONCLUSION: Intussusception should be considered a diagnostic possibility in infants who show a history of vomiting and in whom lethargy and systematic hypertension are noted. This case re-affirms the diagnostic usefulness of abdominal ultrasonography.

A review and methodologic critique of the literature refuting whiplash syndrome.

The validity of whiplash syndrome has been a source of debate in the medical literature for many years. Some authors have published articles suggesting that whiplash injuries are impossible at certain collision speeds; others have stated that the problem is psychological, or is feigned as a means to obtain secondary financial gain. These articles contradict the majority of the literature, which shows that whiplash injuries and their sequelae are a highly prevalent problem that affects a significant proportion of the population. The authors of the current literature critique reviewed the biomedical and engineering literature relating to whiplash syndrome, searching for articles that refuted the validity of whiplash injuries. Twenty articles containing nine distinct statements refuting the validity of whiplash syndrome were found that fit the inclusion criteria. The methodology described in these articles was evaluated critically to determine if the authors' observations regarding the validity of whiplash syndrome were scientifically sound. The authors of the current critique found that all of the articles contained significant methodologic flaws with regard to their respective authors' statements refuting the validity of whiplash syndrome. The most frequently found flaws were inadequate study size, nonrepresentative study sample, nonrepresentative crash conditions (for crash tests), and inappropriate study design. As a result of the current literature review, it was determined that there is no epidemiologic or scientific basis in the literature for the following statements: whiplash injuries do not lead to chronic pain, rear impact collisions that do not result in vehicle damage are unlikely to cause injury, and whiplash trauma is biomechanically comparable with common movements of daily living.

International environmental health: priorities from Huairou.

Between August 30 and September 8, 1995, 38,000 women and several hundred men met in Huairou, China, to discuss the global status of women and to lobby for specific wording in the Agenda for Women that was being finalized in Beijing as part of the United Nations Fourth World Conference on Women. This article has two main objectives. The first objective is to summarize the priority concerns identified in Huairou that are related to international environmental health, especially concerns related to women. The second objective is to provide the rationale and documentation that support including these topics as part of professional education and practice in environmental health in the United States. It is hoped that this discussion will aid environmental health professionals in better appreciating the connection between global environmental health problems and local problems and the applicability of gender-relevant environmental health concerns to local programming, policies, and infrastructure.

[Aneurysm of the vein of Galen and cardiac insufficiency. Current therapeutic approach]. / Anévrisme de l'ampoule de Galien et insuffisance cardiaque. Attitude thérapeutique actuelle.

Aneurysm of the ampulla of Galien is a rare but serious cause of cardiac failure in the neonate or child. The diagnosis is suspected on finding a continuous murmur on auscultation of the skull. It is an intracranial arterio-venous malformation which is sometimes responsible for a very important shunt between the arterial (carotid or vertebral) and venous systems. The vital and neurological prognosis of these children is classically very poor. The authors report the cases of the last three consecutive children aneurysms of the ampulla of Galien followed up at Grenoble Hospital in the last five years. The clinical presentations were very different, corresponding to the usual three forms described in the literature: a reputedly very severe form with cardiac failure at the 3rd day of life requiring multiple percutaneous embolisations; a neonatal form revealed by an isolated macrocranium; and a form diagnosed late (4.5 years) in the investigation of effort dyspnoea which regressed after two sessions of embolisation. All three children are alive and have normal psychomotor development for their age. A multidisciplinary approach involving neonatal physicians, paediatric cardiologists and neurologists, with complementary non-invasive investigations such as transfontanellar ultrasonography, electroencephalography, MRI and echocardiography are necessary to optimise the management and limit the neurological sequellae in children with this type of malformation. Cerebral arteriography and percutaneous embolisation under general anaesthesia by a trained team gave very good medium-term results in two of these cases, lightening the usual pessimism surrounding this condition.

"Whiplash associated disorders: redefining whiplash and its management" by the Quebec Task Force. A critical evaluation.

STUDY DESIGN: The two publications of the Quebec Task Force on Whiplash-Associated Disorders were evaluated by the authors of this report for methodologic error and bias. OBJECTIVES: To determine whether the conclusions and recommendations of the Quebec Task Force on Whiplash-Associated Disorders regarding the natural history and epidemiology of whiplash injuries are valid. SUMMARY OF THE BACKGROUND DATA: In 1995, the Quebec Task Force authored a text (published by the Societe de l'Assurance Automobile du Quebec) and a pullout supplement in Spine entitled "Whiplash-Associated Disorders: Redefining Whiplash and its Management." The Quebec Task Force concluded that whiplash injuries result in "temporary discomfort," are "usually self-limited," and have a "favorable prognosis," and that the "pain [resulting from whiplash injuries] is not harmful." METHODS: The authors of the current report reviewed the text and the supplement for methodologic flaws that may have threatened the validity of the conclusions and recommendations of the Quebec Task Force. RESULTS: Five distinct and significant categories of methodologic error were found. They were: selection bias, information bias, confusing and unconventional use of terminology, unsupported conclusions and recommendations, and inappropriate generalizations from the Quebec Cohort Study. CONCLUSION: The validity of the conclusions and recommendations of the Quebec Task Force regarding the natural course and epidemiology of whiplash injuries is questionable. This lack of validity stems from the presence of bias, the use of unconventional terminology, and conclusions that are not concurrent with the literature the Task Force accepted for review. Although the Task Force set out to redefine whiplash and its management, striving for the desirable goal of clarification of the numerous contentious issues surrounding the injury, its publications instead have confused the subject further.

[An unusual site of cardiac myxoma. Apropos of a case in an 11-year old child]. / Localisation inhabituelle d'un myxome. A propos d'une observation chez un enfant de 11 ans.

Cardiac myxoma, a relatively common condition in adults, is exceptionally rare in childhood. It is usually observed in the left atrium. The condition may be latent and a chance echocardiographic finding or present with arrhythmias, intermittent mitral obstruction or embolic phenomena. The authors report the case of an 11-year old child with no previous medical history, admitted to hospital after sudden right hemiplegia due to an ischaemic cerebrovascular accident. The investigation of a cardiac embolic abnormality led to the finding of a very large left ventricular tumour inserted into the mitral annulus and prolapsing into the left ventricular outflow tract, the histological examination of which confirmed the diagnosis of myxoma. Cardiac myxomas in children seem to have a much more variable site of implantation than in adults, especially in the right heart in infancy. Treatment is always surgical. Echocardiography should be requested early in children presenting with a murmur of recent origin, malaises, or unexplained inflammatory syndromes, and would seem to be the only means of avoiding the severe embolic complications of these histologically benign tumours.

Enhancing the quality and usefulness of epidemiological studies of burn injuries.

The objectives of this manuscript are to highlight how certain features of epidemiological studies of burn injuries affect the quality of the investigation and the usefulness of the results, and to suggest a framework, in the form of a checklist, for future submissions of epidemiological studies to Burns.

[Cardiac output evaluation during exercise in children treated with atrial surgery for transposition of great vessels]. / Evaluation du débit cardiaque à l'exercice chez des enfants traités par chirurgie atriale d'une transposition des gros vaisseaux.

The long-term physiopathological consequences of atrial surgery (Senning or Mustard procedures) for transposition of the great vessels with respect to exercise capacity are not well known. We measured the cardiac index by the technique of CO2 rebreathing at two submaximal levels of exercise corresponding to a stable oxygen consumption of 20 (E20) and 30 (E30) ml/min/kg in 7 patients successfully operated for transposition of the great vessels and in 7 control children paired for age, gender and body surface area. Despite an identical chronotropic response to exercise in the two groups, the increase in cardiac index was not as great in the children operated for transposition (from 6.86 +/- 0.51 to 7.71 +/- 0.78 l/min/m2) as in the control population (from 7.71 +/- 0.78 to 10.2 +/- 0.51 l/min/m2; p < 0.02). The stroke volume index was therefore significantly lower in the transposition group at both levels of exercise (52 +/- 3.2 vs 63 +/- 4.1 ml/m2; p < 0.04 at E20; and 46.4 +/- 4.3 vs 66 +/- 5.1 ml/m2 at E30). The main cause of this reduction of the stroke volume index is probably a lack of adaptation of right ventricular systolic function on exercise but it is not possible to exclude diastolic dysfunction due to reduce compliance secondary to the intraatrial patch. The conditions of preload are in fact instrumental in increasing stroke volume index at submaximal exercise levels.

Cardiac output response to dynamic exercise after atrial switch repair for transposition of the great arteries.

Results from this study showed that patients who underwent successful operation for transposition of the great arteries had no appropriate increase in stroke volume in response to exercise of a nature similar to common recreational activities. The impairment, most likely due to disturbances in both venous return and ventricular systolic function, is compensated for by an increase in peripheral oxygen extraction; however, this increase may not be adequate with maturation or during prolonged exercise when cardiovascular constraints are more important.