1/479. hypercalciuria and urolithiasis in a case of costello syndrome.costello syndrome is characterized by postnatal growth deficiency, mental retardation, curly hair, coarse characteristic face, and loose skin of hands and feet. patients with this syndrome have a high incidence of cardiac involvement, including arrhythmia, atrial septal defect, and hypertrophic cardiomyopathy. We report a 16-year-old adolescent female with costello syndrome who presents with hypercalciuria and urolithiasis.- - - - - - - - - - ranking = 1keywords = rhythm (Clic here for more details about this article) |
2/479. Fat embolism syndrome in a case of abdominal lipectomy with liposuction.Fat embolism syndrome is reported in a patient who underwent abdominoplasty and suction lipectomy for body contouring. Within 48 hours after surgery, she experienced adult respiratory distress syndrome, secondary to fat embolism syndrome. This was proven on bronchoscopy by evidence of fat laden macrophages. Aggressive respiratory support over 12 days resulted in patient survival.- - - - - - - - - - ranking = 0.0084142009881701keywords = hour (Clic here for more details about this article) |
3/479. status epilepticus-induced brain damage and opercular syndrome in childhood.This study reports on a girl with a permanent cerebral lesion and opercular syndrome after status epilepticus (SE). She had previously been healthy and had her first focal motor seizure at 5 years of age, which was controlled with intravenous phenytoin and rectal diazepam. Twenty-four hours later, she developed partial SE consisting of right facial twitching and right-hand clonic movements. These uncontrollable seizures lasted for 5 days, after which the partial SE changed to generalized SE, and the seizures continued for another 5 days. CT performed the day before onset of SE revealed no brain abnormality. Another CT performed a year later disclosed bilateral brain lesions, more severe in the left hemisphere. Follow up at 16 years of age revealed moderate motor sequelae of the right-hand side of the body, anarthria, difficulty chewing, dysphagia, bilateral facial weakness, and drooling, all of which clinically characterize opercular syndrome. An MRI study performed at 14 years of age showed a cerebral parenchymatous lesion which extended between the parietal cortices of both hemispheres, more severe on the left side, and which crossed the corpus callosum, destroying the posterior-middle zone. Evidence from the CT indicates that the lesion was not present before onset of SE. It seems likely that the focal SE caused the focal brain damage, but the possibility that the subsequent generalized SE played a role cannot be excluded.- - - - - - - - - - ranking = 0.0084142009881701keywords = hour (Clic here for more details about this article) |
4/479. Acute confusion and unreal experiences in intensive care patients in relation to the ICU syndrome. Part II.The intensive care unit syndrome (ICU syndrome) is defined as an altered emotional state occurring in a highly stressful environment, which may manifest itself in various forms such as delirium, confusion, crazy dreams or unreal experiences. The purpose of this part of a study of patients' experiences is to describe and illuminate patients' experiences of acute confusion, disorientation, wakefulness, dreams and nightmares during and after their stay in the ICU. The data were obtained from 19 ventilated patients, who were interviewed twice and had stayed at least 36 hours in the ICU, the first interview being about one week after discharge from the ICU, and the second 4-8 weeks later. The hermeneutic approach used when interpreting and analysing the text from the interviews revealed that patients' experiences of unreal experiences were often associated with intense fear. Intense or continuous unbearable fear seems to result in frightening unreal experiences, which further increase the level of fear. Care actions or caring relationships with relatives or nurses can reduce this fear, which can help to prevent the occurrence and/or duration and intensity of the unreal experiences. trust and confidence in nurses or significant others and feelings of self-control or trust in self-control seemed to reduce the risk of unreal experiences so that adverse stimuli might only trigger a mild confusion.- - - - - - - - - - ranking = 0.0084142009881701keywords = hour (Clic here for more details about this article) |
5/479. andersen syndrome autosomal dominant in three generations.andersen syndrome is a rare entity and comprises potassium sensitive periodic paralysis, ventricular arrhythmia, and an unusual facial appearance; syncope and sudden death have also been reported. The recognition of the characteristic face permits an early diagnosis in order to detect the severe systemic manifestations that are associated with this syndrome. The genetic defect is not linked to any other form of potassium sensitive periodic paralysis nor is it related to that of the long qt syndrome; nevertheless, a prolonged QT interval can be detected in a significant proportion of the cases. Sixteen cases of this syndrome have been described. We report on a three-generation family with 10 affected members. To our knowledge, this is the largest number of cases reported in one family. We noted some additional minor anomalies such as broad forehead and malar hypoplasia. Our patients had variable expression in the classical triad and of the severity of the systemic manifestations. Five of 8 affected studied members did not have a long QTc, which has been suggested as a constant finding in this syndrome.- - - - - - - - - - ranking = 1keywords = rhythm (Clic here for more details about this article) |
6/479. Transient local changes in right ventricular monophasic action potentials due to ajmaline in a patient with brugada syndrome.A 48-year-old patient with recurrent episodes of palpitations and syncope presented with transient ST segment elevation in the right precordial ECG leads. Structural heart disease was excluded. No arrhythmias were inducible by programmed ventricular stimulation. Parallel to ST elevation after intravenous ajmaline, a gradual and reversible delay in the upstroke of right ventricular (RV) monophasic action potentials (maps) occurred that was most marked in the RV outflow tract and nearly absent at right free-wall recordings. ajmaline led to a cycle length-dependent increase in RV dispersion of repolarization. Thus, right endocardial maps may demonstrate regionally different action potential changes that may contribute to the ECG changes in brugada syndrome.- - - - - - - - - - ranking = 1keywords = rhythm (Clic here for more details about this article) |
7/479. Asymptomatic form of brugada syndrome.We describe a patient with the asymptomatic form of brugada syndrome. His electrographical, electropharmacological, and electrophysiological characteristics were similar to those reported in patients with the symptomatic form of brugada syndrome. We believe that he has the same arrhythmogenic substrate as that of patients with brugada syndrome. The fact that he had no episode of spontaneous ventricular fibrillation might be explained by his absence of the triggering factors.- - - - - - - - - - ranking = 1keywords = rhythm (Clic here for more details about this article) |
8/479. Ballooning posterior leaflet syndrome: syncope and sudden death.Two of four patients with ballooning posterior leaflet syndrome died suddenly and the remaining two developed a near-fatal arrhythmia. These four patients demonstrate the potentially fatal nature of the ballooning posterior leaflet syndrome.- - - - - - - - - - ranking = 1keywords = rhythm (Clic here for more details about this article) |
9/479. A young man with recurrent syncopes, right bundle branch block and ST segment elevation.We report on the case of a 33-year-old man with recurrent syncopes appearing suddenly due to sustained monomorphic ventricular tachycardias. The electrocardiogram (ECG) showed a right bundle branch block pattern and ST segment elevation in the precordial leads V1 to V2, not explained by ischemia, electrolyte disturbances, toxic ingestion, or structural heart disease (coronary and right ventricle angiograms as well as biopsies of the right ventricle were normal). ECG image was compatible with the so-called brugada syndrome, first described in 1992. This entity is very rare. Missed diagnosis can be disastrous because life-threatening ventricular arrhythmias often develop in patients.- - - - - - - - - - ranking = 1keywords = rhythm (Clic here for more details about this article) |
10/479. Treatment of the bradycardia-tachycardia syndrome with permanent demand pacing.The bradycardia-tachycardia syndrome (paroxysmal supraventricular tachycardia alternating with sinus bradycardia and episodes of sinus node arrest) has previously presented a complicated therapeutic dilemma when excitatory and suppressive drugs have been utilized. A patient with this syndrome successfully treated with a permanent ventricular transvenous demand pacemaker is presented. Various aspects of this syndrome as well as facets of diagnosis and treatment have been reviewed and discussed. Significant underlying cardiac disease was ruled out in this patient by the usual diagnostic methods including left heart catheterization and coronary angiography. An interesting possibility of the relationship of vagal stimulation secondary to hiatus hernia as an etiologic factor in this syndrome has been discussed. The opinion is expressed that the currently preferred method of treatment is the insertion of a permanent transvenous pacemaker alone or in conjunction with antiarrhythmic drugs, preferably digitalis and propranolol.- - - - - - - - - - ranking = 1keywords = rhythm (Clic here for more details about this article) |
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