11/30. Target-controlled infusion of propofol for a patient with myotonic dystrophy.We present a patient with myotonic dystrophy (MD) who was anesthetized with propofol using a target-controlled technique for electrophysiologic examination and cardiac catheter ablation. The patient became apneic unexpectedly at the same time when he fell asleep, with effect-site propofol concentration of 1.6 microg ml(-1). We had to insert a laryngeal mask airway (LMA), and mechanical ventilation was performed. The patient opened his eyes on verbal command at an effect-site concentration of 1.2 microg ml(-1) after the procedure. This concentration (1.2 microg ml(-1)) was slightly lower than our institutional average for adult male patients (1.5 /- 0.2 microg ml(-1)). However, the time from the end of anesthesia to the patient's awakening was about 10 min. We considered that emergence from anesthesia was not delayed in this case. Careful titration of propofol by target-controlled infusion (TCI) enabled to evaluate the patient's sensitivity to propofol. We conclude that TCI of propofol was a useful anesthetic technique in the MD patient. Respiratory depression might occur in MD patients at low propofol concentrations. Precise control and titration over target propofol concentration is important in anesthetic management for MD patients.- - - - - - - - - - ranking = 1keywords = cardiac (Clic here for more details about this article) |
12/30. Cyanotic episodes in a male child with fragile x syndrome.A 9-year-old male with a diagnosis of fragile x syndrome (FXS) was evaluated for cyanotic episodes of unknown etiology. Clinical observation revealed frequent episodes of hyperventilation lasting several minutes, only while the patient was awake. This was followed by apnea associated with cyanosis and oxygen desaturation. Polysomnogram confirmed episodic central apnea temporally associated with hypocapnia, only during the awake state. Extensive evaluation failed to reveal other neurological, cardiac, gastrointestinal, or pulmonary etiologies for the events. The clinical observations and investigations allowed us to conclude that the patient's cyanotic episodes were caused by primary behavioral hyperventilation in the awake state. Similar behaviors have been reported in children with a variety of diagnoses but to our knowledge have not been previously reported in children with FXS. Treatment for this unusual behavior in FXS consists of reassurance and behavior modification to decrease the frequency and severity of the cyanotic episodes.- - - - - - - - - - ranking = 1keywords = cardiac (Clic here for more details about this article) |
13/30. mebendazole poisoning in infancy.Accidental mebendazole poisoning in an 8-week-old infant and respiratory arrest with tachyarrhythmia associated with continuous seizures is reported. Exchange transfusion was undertaken as a life-saving measure. mebendazole, like piperazine citrate, has considerable neurotoxicity, especially in infancy, and we propose the use of exchange transfusion as a means of mebendazole elimination in infants.- - - - - - - - - - ranking = 5.0407987891695keywords = arrhythmia (Clic here for more details about this article) |
14/30. Hypersomnia-sleep apnea due to micrognathia. Reversal by tracheoplasty.A 67-year-old woman with acquired micrognathia developed severe daytime hypersomnia, loud snoring, nocturnal enuresis, encopresis, and hypertension. A polysomnogram demonstrated 564 sleep apneas, primarily obstructive, recurrent hypoxia, a bradytachycardia, and absent stages III, IV, and REM sleep. endoscopy during sleep revealed recurrent active closure of the upper pharynx associated with loud snoring. A tracheoplasty was done because of severity of symptoms and failure of conservative therapy. Dramatic improvement in sleepiness and hypertension occurred within 48 hours. On postoperative night 15 a repeated polysomnogram showed only 23 apneas, no hypoxia or bradytachycardia, and long periods of stage II, IV, and REM sleep. patients with the hypersomnia-sleep apnea syndrome should be provided with a tracheal opening during sleep when severe daytime somnolence, cardiac arrhythmias, and hypertension are present.- - - - - - - - - - ranking = 6.0407987891695keywords = arrhythmia, cardiac (Clic here for more details about this article) |
15/30. chloroquine poisoning in a child.chloroquine poisoning in children, although infrequent, is extremely dangerous because of the narrow margin between therapeutic and toxic doses. Children clinically present with apnea, seizures, and cardiac arrhythmias. We present the case of a 12-month-old infant, the second-youngest patient reported in the US literature to die from chloroquine poisoning. A serum level of 4.4 mg/L (13.64 mumol/L) was obtained after the infant ingested only one tablet (300 mg). This establishes a new minimal lethal dose/blood level for children. Although some pediatric and adult pharmacokinetic and clinical similarities exist, the outcome is different. Pediatric mortality is 80%, whereas adult mortality is only 10%. Pediatric ingestion cases are primarily unintentional, and most adult cases are suicide attempts. Current treatment in adults includes a protocol of diazepam and epinephrine. Further studies involving children and these medications and other modalities are needed to improve survival.- - - - - - - - - - ranking = 6.0407987891695keywords = arrhythmia, cardiac (Clic here for more details about this article) |
16/30. lightning injury: management of a case with ten-day survival.A boy, injured by lightning, had cardiopulmonary arrest. Following resuscitation and during the course of hospitalization, he developed further cardiac arrhythmia, cerebral edema, generalized seizures, and upper gastrointestinal bleeding. Despite efforts to combat these complications, the patient's condition deteriorated, and he died on the tenth day of hospitalization. The pathologic findings are described and the treatment discussed.- - - - - - - - - - ranking = 6.0407987891695keywords = arrhythmia, cardiac (Clic here for more details about this article) |
17/30. airway obstruction by vascular anomalies. Importance of telescopic bronchoscopy.This review of 28 cases of airway obstruction by vascular anomalies in the past 6 years emphasizes the importance of these anomalies as causes of obstruction, stridor, and apnea in infants as well as the important contribution of telescopic bronchoscopy to the recognition of these lesions, especially compression of the trachea by the innominate artery. There were 20 patients with compression by the innominate artery; four were surgically corrected. Seven vascular ring anomalies were all corrected by operation as was an enlarged left atrium and malformed mitral valve in one patient. Vascular anomalies caused 26% of the obstructive airway lesions in a series of infants who had bronchoscopy for obstruction, stridor, or apnea. Failure to diagnose and treat these entities may result in progressive respiratory embarrassment and even death. barium esophagogram and aortic arch arteriogram, the traditional modalities for diagnosing vascular rings, may fail to identify tracheal compression by enlarged cardiac chambers or the more common "anomalous" innominate artery. Telescopic bronchoscopy will identify tracheal compression by the innominate artery; furthermore, it will identify the area of compression by the vascular ring. observation of the compressed area during corrective surgery ensures that the operative manipulations are appropriate and successful in relieving the obstruction. This observation can be facilitated by televised monitoring and videotaping.- - - - - - - - - - ranking = 1keywords = cardiac (Clic here for more details about this article) |
18/30. Sudden and unexpected death between 1 and 5 years.Of a population of 9856 children followed up from birth, 9251 of whom underwent 24 hour tape recordings of electrocardiograms and abdominal wall breathing movements during early infancy, five died suddenly and unexpectedly at home at ages ranging from 16 months to 4 years. Postmortem examination, including full histological and microbiological investigations. failed to identify abnormalities ordinarily associated with death in all five cases. Two of the children were known to have had frequent cyanotic episodes and died during these events. In the three remaining cases there was no previous history of cyanotic or apnoeic episodes. The death of one of these three children was seen by his parents and the clinical features suggested that apnoea rather than a cardiac arrhythmia was the primary mechanism for his death. As in infancy, sudden and unexpected death for which no adequate cause is found at necropsy seems to constitute a major component of mortality between 1 and 5 years.- - - - - - - - - - ranking = 6.0407987891695keywords = arrhythmia, cardiac (Clic here for more details about this article) |
19/30. Intoxication with ajmaline in an infant.We present a case of overdosage of ajmaline in an infant. The appearance of atactic gait and clonic tonic seizures were followed by loss of consciousness, apnea, supraventricular tachycardia, left bundle-branch block, and a prolonged Q-T interval. cardiopulmonary resuscitation, gastric lavage, and forced diuresis were followed by complete recovery. Continuous electrocardiographic monitoring is mandatory in these cases, and the use of a cardiac pacemaker, respirator, and therapy with antiarrhythmic agents should be considered.- - - - - - - - - - ranking = 1keywords = cardiac (Clic here for more details about this article) |
20/30. sleep apnea syndrome in a patient with shy-drager syndrome.A patient with autonomic insufficiency and extrapyramidal signs (shy-drager syndrome) and sleep apnea syndrome (SAS) underwent hemodynamic studies. In comparison to patients with SAS and intact autonomic reflexes, systemic hypertension was absent and marked sinus arrhythmia during sleep was blunted. Cyclical pulmonary hypertension associated with frequent apneic episodes during sleep persisted, reflecting a minor role of autonomic reflexes in the generation of this abnormality. autopsy confirmed the shy-drager syndrome and multiple areas of degeneration were observed in areas of the CNS outside the medullary respiratory centers, suggesting their importance in the origin of the respiratory abnormalities in SAS.- - - - - - - - - - ranking = 5.0407987891695keywords = arrhythmia (Clic here for more details about this article) |
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