The congenital long-QT syndrome (LQTS) is characterized by recurrent syncope, prolonged QT intervals, QT interval lability, polymorphic ventricular tachycardia, and sudden death. We report a case of congenital long QT syndrome in a 28-day-old male infant who presented with syncope, bradycardia with 2: 1 pseudo-atrioventricular block and a markedly prolonged QT inteval. One episode occured after crying and degenerated into ventricular fibrillation and terminated after cardioversion. A VVI type cardiac pacemaker was implanted. Subsequently, the infant's heart rate was over 110/min and 2: 1 AV block and any other arrhythmia were absent. The infant recovered from the accompanied pneumonia and sepsis and was discharged 47 days after adrnission. However, 13 days after discharge, the infant returned to our hospital ER with syncope. Ventricular fibrillation ceased after cardioversion. Despite medication with propranolol, ventricular tachycardia persisted. The infant expired the day after he was discharged against medical advice.
Arrhythmias, Cardiac ; Atrioventricular Block* ; Bradycardia ; Crying ; Death, Sudden ; Electric Countershock ; Heart Rate ; Hospitals ; Humans ; Infant ; Long QT Syndrome ; Male ; Pneumonia ; Propranolol ; Sepsis ; Syncope ; Tachycardia, Ventricular ; Ventricular Fibrillation

Gottron's acrogeria syndrome is a rare genetic disease of unknown etiology with some features suggestive of accelerated aging. The typical clinical presentation consists of premature aging of the skin with atrophy of the cutis and the subcutis. After a brief review of the literature available on this syndrome, we report a case of Gottron s acrogeria syndrome in a 19 year-old man whose right rectus femoris muscle had a fibrotic contracture.
Aging ; Aging, Premature ; Atrophy ; Contracture ; Humans ; Quadriceps Muscle ; Skin ; Young Adult

Spinal reflex activity that remains after insult to the spinal cord brings with it significant functional impairment. Our patient had suffered from general spasticity and hyperreflexic neurogenic bladder caused by sixth cervical cord injury. Unfortunately, the use of oral medication (baclofen) was limited by an inadequate effect. So we performed two times of subarachnoid block with 0.5% heavy bupivacaine, the patient experienced improvement in bladder and sphincter function. We concluded that subarachnoid block with 0.5% heavy bupivacaine is an effective and safe modality for spasticity control in patients who are refractory to oral medication before neurolytic block.
Bupivacaine ; Humans ; Muscle Spasticity ; Reflex ; Spinal Cord ; Urinary Bladder ; Urinary Bladder, Neurogenic*

BACKGROUND: The reduction is cerebral blood flow (CBF) caused by hypocapnia is an important element of anesthetic techniques for neurosurgery as well as for nonneurologic surgery in patients with reduced intracranial compliance. Accordingly, the impact of anesthetic agents on the CO2 responsiveness of the cerebral circulation has important implications with regard to anesthetic selection. The purpose of this study was to investigate the effects of isoflurane-N2O and propofol-N2O anesthesia on the CBF response to changes in end-tidal CO2 in healthy patients. METHODS: 19 healthy patients with nonneurological operation were selected. In group 1, anesthesia was induced with thiopental sodium 4 mg/kg, fentanyl 1 g/kg, succinylcholine 1~1.5 mg/kg and was maintained with isoflurane 0.5~1.5 vol%. In group 2, anesthesia was induced with propofol 2~2.5 mg/kg, fentanyl 1 g/kg, succinylcholine 1~1.5 mg/kg and was maintained with a propofol infusion of 10 mg/kg/h for 10 min and then 8 mg/kg/h for 10 min and then was reduced 3~6 mg/kg/h of the remainder of the study. All patients were ventilated with N2O in O2 (FIO2 0.5) and measured end-tidal CO2 (PETCO2). Mean blood flow velocity of middle cerebral artery was measured using transcranial Doppler in PETCO2 45, 40, 35, 30, 25, 20 mmHg. RESULT: CO2 reactivity of MCA flow velocity during isoflurane-N2O and propofol-N2O anesthesia was 5.1 +/- 1.8 %/mmHg, 4.4 +/- 1.0 %/mmHg respectively. CONCLUSION: The cerebral vasculature in healthy patients remains responsive to changes in PETCO2 during isoflurane-N2O and propofol-N2O anesthesia.
Anesthesia* ; Anesthetics ; Blood Flow Velocity* ; Carbon Dioxide* ; Carbon* ; Compliance ; Fentanyl ; Humans ; Hypocapnia ; Isoflurane ; Middle Cerebral Artery* ; Neurosurgery ; Propofol ; Succinylcholine ; Thiopental

No abstract available.
Polychondritis, Relapsing*

No abstract available.
Polychondritis, Relapsing*

The distribution and properties of branched chain amino acid aminotransferase(EC 2.6.1.42) was investigated in adult Fasciola hepatica. Fasciola hepatica was fractionated by differential centrifugation into nuclear, mitochondrial and cytosolic fractions. The activity of branched chain amino acid aminotransferase was measured by the method of Ichihara and Koyama (1966) . Isozyme patterns of this enzyme was also examined by DEAE-cellulose column chromatography. The results obtained were as follows: The activity in homogenate was found to be 12.69 units/g wet tissue. The activity of this enzyme was relatively high compared with those in rat tissues. The distribution of branched chain amino acid aminotransferase in the subcellular organelles showed that 87.8 percent of the activity was in cytosolic, 10.9 percent in mitochondrial and 1.3 percent was in nuclear fraction. Cytosolic fraction of Fasciola hepatica contained Enzyme I, but not Enzyme II and III, of branched chain amino acid aminotransferase. Enzyme I was eluted by 50 mM phosphate buffer from DEAE-cellulose column and catalyzed the transamination of all three branched chain amino acids. The Enzyme I was purified about 22-folds increase in specific activity after chromatography on DEAE-cellulose. The best substrate among three amino acids (leucine, isoleucine and valine) was L-isoleucine. The optimal temperature of Enzyme I was 45 C and the optimal pH was 8.2. The Km value for leucine of Enzyme I was 4.17 mM. The Km values for alpha-ketoglutarate and pyridoxal phosphate of Enzyme I were 0.41 mM and 4.76 x 10(-3) mM, respectively.
parasitology-helminth-trematoda ; Fasciola hepatica ; biochemistry ; enzyme ; aminotransferase

The activity and distribution of aspartate aminotransferase (EC 2.6.1.1) and alanine aminotransferase (EC 2.6.1.2) in adult Fasciola hepatica have been studied. Fasciola hepatica was fractionated by differential centrifugation into nuclear, mitochondrial and cytosolic fractions. The activity of GOT and GPT was measured by the method of Reitman and Frankel. Isozyme patterns of those enzyme were also examined by DEAE-cellulose column chromatography. The results obtained were as follows: The activity of aspartate and alanine aminotransferase was about 0.55 unit and 0.92 unit per 1 g of Fasciola hepatica, respectively. The activity of those enzymes was relatively low compared with those in mammalian tissues. The distribution of aspartate aminotransferase in the subcellular organelles showed that 71 percent of the activity was in cytosolic, 24 percent in mitochondrial and 5 percent was in nuclear fraction. About 22 percent of the total alanine aminotransferase activity was found in the mitochondrial fraction, about 66 percent in the cytosolic fraction. Aspartate aminotransferase from cytosolic fraction was separated into two types of isozymes, whereas alanine aminotransferase from cytosolic fraction gave only one active peak on DEAE-cellulose column chromatography.
parasitology-helminth-trematoda ; Fasciola hepatica ; biochemistry ; enzyme ; aspartate aminotransferase ; alanine ; aminotransferase ; alanine aminotransferase

PURPOSE: To analyze clinical, radiological and pathological features as well as clinical outcome after surgical treatment of patients with secondary chondrosarcoma arising from osteochondroma(tosis). MATERIALS AND METHODS: We retrospectively reviewed clinical records, radiographs, pathologic slides of 14 patients. Nine patients were male and five were female. The mean age was 34 years. The mean follow-up period was 54 months. RESULTS: All patients had a history of previous mass since childhood or puberty. Preexisted osteochondroma was single in 3 patients and multiple in 10. Remaining 1 patient had multiple osteochondromatosis with enchondromatosis. MRI clearly provided thickness of cartilage cap, which was over 2 cm except in 2 cases. Chondrosarcoma was grade 1 in all except 1 case, which was grade 2. Wide excision was performed in 10 patients, marginal excision in 3 and amputation in 1. Twelve patients were doing very well without evidence of disease. Among 3 patients with marginal excision, 1 patient had local recurrence and 1 patient died of disease. CONCLUSION: Comprehensive understanding of clinical, radiological and pathological features of secondary chondrosarcoma is warranted for accurate diagnosis. The best result can be expected with early recognition of malignant change of osteohcondroma(tosis) and wide excision.
Amputation ; Cartilage ; Chondrosarcoma ; Enchondromatosis ; Female ; Follow-Up Studies ; Humans ; Male ; Osteochondroma ; Osteochondromatosis ; Puberty ; Recurrence ; Retrospective Studies

PURPOSE: To investigate prognostic factors influencing on local recurrence, distant metastasis and event-free survival of liposarcomas. MATERIALS AND METHODS: Fifty-two patients managed for liposarcomas since 1993 were analyzed respectively in the view of prognostic influence of patient age, tumor size, location, histologic type, histologic grade, resection type, surgical margin, chemotherapy and radiation therapy on local recurrence, distant metastasis and event-free survival. The mean follow up period was 39 months. The univariate and multivariate regression analysis were performed for statistical evaluation. RESULTS: The local recurrences occurred in 11 patients (21.2%) and distant metastasis in 4 patients (8%), Event-free survival rate at 4 year follow up was 67%. In univariate analysis, histologic grade, surgical margin, chemotherapy and radiation therapy were significant prognostic factors on local recurrence (p<0.05). However, histologic grade lost its significance in muitivariate analysis. Trunk location revealed higher rate of distant metastasis than extremity location. In univariate analysis on event-free survival. histologic grade and chemotherapy were significant factors (p<0.05). No factor remained significant in multivariate analysis. CONCLUSION: Considering selection bias, positive surgical margin was negative prognostic factor on local recurrence. Liposarcomas arisen in trunk revealed higher rate of distant metastasis. There was no independent prognostic factor on event-free survival of patients with liposarcomas.
Disease-Free Survival ; Extremities ; Follow-Up Studies ; Humans ; Liposarcoma ; Multivariate Analysis ; Neoplasm Metastasis ; Recurrence ; Retrospective Studies ; Selection Bias