Clinical Features of Isolated Noncompaction of the Ventricular Myocardium

PURPOSE: Isolated noncompaction of the ventricular myocardium(INVM) is one of the unclassified cardiomyopathies that is characterized by numerous, excessively prominent trabeculations, and deep intertrabecular recesses. We performed this study to evaluate the clinical features of INVM in children. METHODS: The medical records of 10 patients with INVM were reviewed. We analyzed the clinical manifestations, hemodynamics, pattern of inheritance, and long-term prognosis of INVM in children. RESULTS: Age at diagnosis was 45+/-53 months(1 day-14 years) with follow-up lasting as long as 78 months. Most INVM was asymptomatic on diagnosis. Associated cardiac anomalies were noted in six patients(ventricualr or atrial septal defect, patent ductus arteriosus with mitral valve prolapse, or mitral valve cleft). Depressed or flat changes of T wave in lead II, III and aVF were observed on electrocardiography. Various arrhythmia including WPW syndrome with paroxysmal, supraventricular tachycardia, third-degree atrioventricular block, and familial sick sinus node dysfuction were observed. The degree of trabeculation in INVM was significantly prominent from level of mitral valve to apex compared to age-matched control. Familial recurrences were noted in two patients. The systolic function of the left ventricle was decreased in 20% of patients during the follow-up period, but systemic embolism or ventricular tachycardia was not observed. CONCLUSIONS: INVM is not a rare disorder. The cardiac function may be deteriorated in children as well as adults during long-term follow up. Thus early diagnosis and long-term follow-up must be done. So, the nation-wide multicenter clinical study would be mandatory to evaluate the incidence, long-term prognosis, and establishment of objective diagnostic criteria of INVM.

Partial Airway Obstruction with an Armored Tube by Swelling of the Inner Layer / 대한마취과학회지

An Armored tube is known to be the most effective in maintaining of airway patency during anesthesia in any position. Unfortunately, the tube itself may become the cause of airway obstruction. One of the known complications of the armored tube is a separation of the individual latex layers. This can be caused by herniation into the lumen. Diffusion of nitrous oxide into the inner hernia considerably intensifies the obstruction. The bubbles in the tube wall arise during manufacturing as well as during resterilization. A 62-year-old man with herniation of lumbar disc herniation was intubated with a 8.0 mm armored tube for general anesthesia. 30 minutes later, we experienced signs of partial endotracheal tube obstruction including high arterial PCO2 and inspiratory pressure in prone position. Then, tube suction with catheter was done and signs was slightly improved. But, 90 minutes later, passage of suction catheter was impossible. When operation was finished, patient was turned to supine position. We exchanged the tube with another tube and found inner wall herniation into the armored tube lumen caused by layer separation.

One-lung Anesthetic Experience for Video-assisted Thoracoscopic Surgery in Two Children / 대한마취과학회지

Video-assisted thoracic surgery (VATS) has been increasingly used because of it is a less invasive procedure than the open thoracotomy. Neither commercially available double-lumen tubes nor the univent tube can be used in small children. An ordinary uncuffed tracheal tube was introduced into the main bronchus of the right lung. This technique proved to be a simple and effective method of isolating and ventilating the other lung. We describe our experience providing one-lung ventilation with ordinary endotracheal tube during VATS in two young children. (Korean J Anesthesiol 2001; 40: 824 ~ 828)

IGF-I on the Expression of Gene Associated with Hypoxic Ischemic Brain Injury in the Neonatal Rats

PURPOSE: To investigate the effect of intraperitoneal injection of IGF-I after hypoxic ischemic brain injury on neuronal cell necrosis, apoptosis and expression of proapoptotic and antiapoptotic proteins bax and bcl-2, respectively. METHODS: The right carotid artery was cut between the double ligation. Then allowed to recover for 30 minutes followed by exposure to 8% oxygen at 37degree C for 2 hours. Devided 2 groups, control group(N=30) and IGF-I treated group(N=30). IGF-I treated group received IGF-I 20 microg 2 hours after hypoxic ischemic injury intraperitoneally. Rates were decapitated at 24 hours and 72 hours following hypoxic ischemic brain injury. After then, right hippocampal CA1 and CA3 neuronsof rat brains were examined. RESULTS: The apoptosis and necrosis was significantly less in IGF-I treated group than control group and necrosis was more prominent in CA1 neurons than CA3 neurons. Necrosis was slightly decreased at 72 hours in both groups(P<0.05). The apoptosis was more prominent at 24 hours than 72 hours after hypoxic ischemic injury(P<0.05). Bax protein expression was prominent in control group, especially at 72 hours(P<0.05) and less in the IGF-I treated group than control group. Bcl-2 protein expression was not detected in both group. CONCLUSION: The results from this study suggest that exogenous systemic IGF-I had a neuroprotective effect by inhibition of up-regulation of bax protein expression after hypoxic ischemic brain injury.

The Combined Effect of Epidural Tramadol and Clonidine for Postoperative Analgesia / 대한마취과학회지

BACKGROUND: The efficacy of epidurally administered tramadol hydrochloride, a weak centrally acting analgesic, was studied for the relief of postoperative pain. Clonidine, an alpha2 adrenergic agonist, has nonopiate antinociceptive properties which might be an alternative for postoperative analgesia free of undesirable effects from opioids. The aim of this study was to evaluate the postoperative analgesic effects of an epidural administration with a combination of tramadol and clonidine. METHODS: Sixty patients undergoing lower abdominal surgery were randomly allocated to three treatment groups to be given the following agents by the epidural route: group 1, 10 ml of bupivacaine 0.125%; group 2, 10 ml of bupivacaine 0.125% with tramadol 50 mg; group 3, 10 ml bupivacaine 0.125% with tramadol 50 mg and clonidine 100 microgram. In the recovery room, postoperative analgesia was assessed by the visual analogue scale (VAS) at 30 min, 1, 2, 3, 4, 5 and 6 hour. Vital signs, sedation score and side effects were also checked. RESULTS: VAS scores were significantly lower in group 3 than group 1. In addition, VAS scores were significantly lower in group 3 than group 2 at 4 and 5 hours. Blood pressure, heart rate and sedation scores were not significantly different among the three groups. CONCLUSIONS: The combination of epidural 0.125 % bupivacaine, tramadol 50 mg and clonidine 100 microgram produces more profound and longer postoperative analgesic effects than 0.125% bupivacaine and tramadol 50 mg or only 0.125% bupivacaine for the lower abdominal surgery.

22q11 Microdeletion and Clinico-Genetic Correlation in CATCH 22 Syndrome

PURPOSE: Deletion of chromosome 22q11 is associated with DiGeorge syndrome, velocardiofacial syndrome, and conotruncal anomaly face syndrome. This study was performed to determine the criteria of clinical phenotype as recognizable syndrome and to research the loss of heterozygosity in CATCH 22 patients and their family. METHODS: An evaluation of the clinical and genetic profiles of 30 persons of CATCH 22 syndrome or their family referred with a diagnosis of either congenital heart disease or cleft palate was undertaken. The deletions of 22q11 were analyzed using the fluorescences in situ hybridization(N25, Oncor) and short tandem-repeat polymorphic makers(STRP, D22S941). RESULTS: The dysmorphic features of CATCH 22 showed considerable overlap and intrafamilial difference was common. The familial cases of CATCH 22 were transmitted maternally as autosomal dominant. The target gene study using the STRP maker(D22S941) in these series showed good clinico-genetic correlation but some heterogeneity. CONCLUSION: Although 22q11 deletion was large in size and high variable in polymorphic markers, extensive evaluation clinically as well as genetically will be necessary for subgrouping of CATCH 22 syndrome due to good clinicogenetic correlation. Furthermore, we also suggest the development of new polymorphic markers to research the unknown characteristics of polymorphic markers in Korean patients with CATCH 22 syndrome.

The Effect of Long-term Steroid Therapy on Cardiac Function in Nephrotic Syndrome

PURPOSE: The aim of this study is to assess the cardiac effect of long-term steroid therapy in nephrotic syndrome and the validity of LV functional parameters as an early predictor of subclinical cardiac dysfunction. METHODS: The study group was composed of 21 patients diagnosed as minimal change nephrotic syndrome(NS), being managed with prednisone over 6 months or within 6 weeks after stopping medication. The control group was composed of 23 healthy children without cardiopulmonary dysfunction. The functional parameters of the left ventricle, including systolic and diastolic indices were measured using 2D-doppler echocardiography. RESULTS: There was a significant increase of left ventricular mass index in the study group(P value<0.05). Isovolumetric relaxation time of the left ventricle was prolonged significantly in the study group(P value<0.05). The systolic phase area and the ratio of systolic and diastolic phase area of pulmonary vein flow profile were increased significantly in the study group(P value<0.05). CONCLUSION: In NS patients managed with long-term steroid therapy, left ventricular diastolic functional abnormality may be present even though functional limitation is not yet evident. In the future, the longitudinal study will be needed conderning the cardiac effect of long-term steroid treatment.

The Effect of Long-term Steroid Therapy on Cardiac Function in Nephrotic Syndrome

PURPOSE: The aim of this study is to assess the cardiac effect of long-term steroid therapy in nephrotic syndrome and the validity of LV functional parameters as an early predictor of subclinical cardiac dysfunction. METHODS: The study group was composed of 21 patients diagnosed as minimal change nephrotic syndrome(NS), being managed with prednisone over 6 months or within 6 weeks after stopping medication. The control group was composed of 23 healthy children without cardiopulmonary dysfunction. The functional parameters of the left ventricle, including systolic and diastolic indices were measured using 2D-doppler echocardiography. RESULTS: There was a significant increase of left ventricular mass index in the study group(P value<0.05). Isovolumetric relaxation time of the left ventricle was prolonged significantly in the study group(P value<0.05). The systolic phase area and the ratio of systolic and diastolic phase area of pulmonary vein flow profile were increased significantly in the study group(P value<0.05). CONCLUSION: In NS patients managed with long-term steroid therapy, left ventricular diastolic functional abnormality may be present even though functional limitation is not yet evident. In the future, the longitudinal study will be needed conderning the cardiac effect of long-term steroid treatment.

A case of miller fisher syndrome / 대한소아신경학회지

No abstract available.

One case of hereditary spherocytosis with aplastic crisis

No abstract available.

Clinical & Animal experimental observation on the Botulism Poisonig in a Family

No abstract available.

A Case of Chylous Ascites with Chylothorax

No abstract available.