Background: Joubert syndrome and mitochondrial disease are rare congenital diseases in which a wide range of symptoms affects multiple organs. Patients with these diseases present characteristic symptoms related to the musculoskeletal, respiratory, and neurological systems, which make it difficult for anesthesiologists to manage the patient’s airway and choose appropriate anesthetic drugs. Case A 13-year-old male patient with Joubert syndrome and mitochondrial disease underwent elective surgery to insert a continuous ambulatory peritoneal dialysis catheter. Anesthesia was induced and maintained with propofol, remifentanil, and rocuronium. An I-gel was inserted to secure the airway; however, the fitting did not work properly, so the patient was intubated. The operation was completed without any major problems, and the intubated patient was transferred to the intensive care unit. Conclusions Anesthesiologists should determine the method of anesthesia and prepare for unintended complications based on a full understanding of these congenital diseases.

Background: Joubert syndrome and mitochondrial disease are rare congenital diseases in which a wide range of symptoms affects multiple organs. Patients with these diseases present characteristic symptoms related to the musculoskeletal, respiratory, and neurological systems, which make it difficult for anesthesiologists to manage the patient’s airway and choose appropriate anesthetic drugs. Case A 13-year-old male patient with Joubert syndrome and mitochondrial disease underwent elective surgery to insert a continuous ambulatory peritoneal dialysis catheter. Anesthesia was induced and maintained with propofol, remifentanil, and rocuronium. An I-gel was inserted to secure the airway; however, the fitting did not work properly, so the patient was intubated. The operation was completed without any major problems, and the intubated patient was transferred to the intensive care unit. Conclusions Anesthesiologists should determine the method of anesthesia and prepare for unintended complications based on a full understanding of these congenital diseases.

OBJECTIVE: To investigate the efficacy of systemic pulmonary rehabilitation (PR) after lung resection in patients with lung cancer. METHODS: Forty-one patients undergoing lung resection were enrolled and classified into the experimental (n=31) and control groups (n=10). The experimental group underwent post-operative systemic PR which was conducted 30 min/day on every hospitalization day by an expert physical therapist. The control group received the same education about the PR exercises and were encouraged to self-exercise without supervision of the physical therapist. The PR group was taught a self-PR program and feedback was provided regularly until 6 months after surgery. We conducted pulmonary function testing (PFT) and used a visual analog scale (VAS) to evaluate pain, and the modified Borg Dyspnea Scale (mBS) to measure perceived respiratory exertion shortly before and 2 weeks, 1, 3, and 6 months after surgery. RESULTS: A significant improvement on the VAS was observed in patients who received systemic PR >3 months. Significant improvements in forced vital capacity (FVC) and mBS score were observed in patients who received systemic PR >6 months (p<0.05). Other PFT results were not different compared with those in the control group. CONCLUSION: Patients who received lung resection suffered a significant decline in functional reserve and increases in pain and subjective dyspnea deteriorating quality of life (QoL). Systemic PR supervised by a therapist helped improve reduced pulmonary FVC and QoL and minimized discomfort during the postoperative periods in patients who underwent lung resection.
Dyspnea ; Education ; Exercise ; Hospitalization ; Humans ; Lung Neoplasms ; Lung* ; Organization and Administration ; Physical Therapists ; Postoperative Period ; Quality of Life ; Rehabilitation* ; Respiratory Function Tests ; Visual Analog Scale ; Vital Capacity

PURPOSE: DNA methylation is a major epigenetic mechanism for modification of genetic expression without a change in the DNA sequence. MBD2 (methyl-CpG-binding domain 2 protein) belongs to a family of enzymes concerning of DNA demethylation and suppresses the hypermethylation of the CpG island and DNA transcription. In this study, we investigated the change of MBD2 expression in the blood and tissue of colorectal cancer patients and compared the two expression levels. METHODS: The 68 patients included in this study were patients with colorectal cancer who had undergone surgery at our hospital, and 50 other patients with no malignant disease were recruited from normal populations. Total RNA samples were isolated from whole blood samples and cancer tissues of specimens using a TRI REAGENT BD kit. MBD 2 expression was measured by real-time quantitative reverse transcription-polymerase chain reaction assays. RESULTS: The mean age was older in the case group than in the control group. The mean expression level of MBD2 in blood was not different between the two groups. In the case group, the tissue MBD2 expression was lower than the blood MBD2 expression under all conditions, and that difference was statistically significant (P<0.01). The expression of MBD2 in cancer tissue showed a negative correlation with that in the blood of cancer patients, correlation coefficient of R=0.073, but that result was not statistically significant (P=0.611). CONCLUSIONS: The blood MBD2 expression was statistically the same in the cancer and the control groups. In the cancer group, blood MBD2 expression was significantly higher than tissue MBD2 expression. The reverse correlation between blood MBD2 expression and tissue MBD2 expression in cancer patients suggests that MBD2 may affect the mechanism of carcinogenesis.
Base Sequence ; Colorectal Neoplasms ; CpG Islands ; DNA ; DNA Methylation ; Epigenomics ; Humans ; RNA

The purpose of this study is to demonstrate whether the signal intensity (SI) of myocardial infarction (MI) on contrast enhanced (CE)-cine MRI is useful for differentiating recently infarcted myocardium from chronic scar. This study included 24 patients with acute MI (36-84 years, mean age: 57) and 19 patients with chronic MI (44-80 years, mean age: 64). The diagnosis of acute MI was based on the presence of typical symptoms, i.e. elevation of the cardiac enzymes and the absence of any remote infarction history. The diagnosis of chronic MI was based on a history of MI or coronary artery disease of more than one month duration and on the absence of any recent MI within the previous six months. Retrospectively, the ECG-gated breath-hold cine imaging was performed in the short axis plane using a segmented, balanced, turbo-field, echo-pulse sequence two minutes after the administration of Gd-DTPA at a dose of 0.2 mmol/kg body weight. Delayed contrast-enhanced MRI (DCE MRI) in the same plane was performed 10 to 15 minutes after contrast administration, and this was served as the gold standard of reference. The SI of the infarcted myocardium on the CE-cine MRI was compared with that of the normal myocardium on the same image. The area of abnormal SI on the CE-cine MRI was compared with the area of hyperenhancement on the DCE MRI. The area of high SI on the CE-cine MRI was detected in 23 of 24 patients with acute MI (10 with homogenous high SI, 13 high SI with subendocardial low SI, and one with iso SI). The area of high SI on the CE-cine MRI was larger than that seen on the DCE MRI (p < 0.05). In contrast, the areas of chronic MI were seen as iso-SI with thin subendocardial low SI on the CE-cine MR in all the chronic MI patients. The presence of high SI on both the CE-cine MRI and the DCE MRI is more sensitive (95.8%) for determining the age of a MI than the presence of myocardial thinning (66.7%). This study showed the different SI patterns between recently infarcted myocardium and chronic scar on the CE-cine MRI. CE-cine MRI is thought to be quite useful for determining the age of myocardial infarction, in addition to its utility for assessing myocardial contractility.
Signal Processing, Computer-Assisted ; Retrospective Studies ; Myocardium/*pathology ; Myocardial Infarction/*diagnosis ; Middle Aged ; Male ; *Magnetic Resonance Imaging, Cine ; Magnetic Resonance Imaging/methods ; Humans ; Female ; Contrast Media ; Cicatrix/*diagnosis ; Aged, 80 and over ; Aged ; Adult

Purpose: To determine the differences in the radiographic findings of miliary tuberculosis between patients with and without associated acute respiratory failure (ARF). MATERIALS AND METHODS: We retrospectively reviewed 32 patients in whom miliary tuberculosis had been diagnosed, and assigned them to one of two groups: with ARF (n=10), and without ARF (n=22). Chest radiographic findings such as the presence of miliary nodules, cosolidation, ground-glass opacity (GGO), pleural effusion, small calcified nodules and linear opacities were assessed, the size and profusion of nodules in each of four zones were analyzed and scored using the standard radiographs of the International Labor Organization, and the extent of consolidation and GGO were scored according to the percentage of involved lung. We compared the radiologic findings between the two groups. RESULTS: Ground-glass opacity, consolidation, and pleural effusion were seen more frequently in miliary tuberculosis patients with ARF than in those without ARF. Although the size and profusion of nodules were similar in both groups (p>0.05), consolidation and ground-glass opacity in cases of miliary tuberculosis with ARF were significantly more extensive than in those without ARF (p<0.005). CONCLUSION: GGO and consolidation were more extensive in miliary tuberculosis patients with ARF. A finding of ground-glass opacity in miliary tuberculosis patients might be an early indication of developing ARF.
Humans ; Lung ; Pleural Effusion ; Radiography, Thoracic ; Respiratory Insufficiency* ; Retrospective Studies ; Tuberculosis, Miliary* ; Tuberculosis, Pulmonary

Ground-glass opacity is a frequent but nonspecific finding seen on high-resolution CT scans of lung parenchyma. Histologically, this appearance is observed when thickening of the alveolar wall and septal interstitium is minimal or the alveolar lumen is partially filled with fluid, macrophage, neutrophils, or amorphous material. It has been shown that ground-glass opacity may be caused not only by an active inflammatory process but also by fibrotic processes. When a focal area of ground-glass opacity persists or increases in size, the possibility of neoplasm-bronchioloalveolar carcinoma or adenoma, or lymphoma, for example- should be considered. Diffuse nonsegmental ground-glass opacity in both lung fields was incidentally found on follow up abdominal CT in a stomach cancer patient and signet-ring cell-type metastatic lung cancer was confirmed by transbronchial lung biopsy. We report a case of diffuse ground-glass opacity seen in metastatic lung cancer from adenocarcinoma of the stomach.
Adenocarcinoma* ; Adenoma ; Biopsy ; Follow-Up Studies ; Humans ; Lung Neoplasms ; Lung* ; Lymphoma ; Macrophages ; Neoplasm Metastasis* ; Neutrophils ; Stomach Neoplasms ; Stomach* ; Tomography, X-Ray Computed

Doxylamine is common over-the-counter sleep preparations & frequently involved in overdoses. The clinical course is dominated by the anticholinergic effects, including central nervous system & autonomic effects. We report 4 cases of suicide attempts in adults where ingestion of the doxylamines were complicated by rhabdomyolysis. They ingested doxylamines variable amount & were carried to emergency department. They complained gastrointestinal or central nervous system symptoms. Gastric lavages & administrations of activated charcoal were done. Creatine phosphok inase levels were normal or markedly elevated on arrival, but peaked several days later. Serum creatinine levels were normal. 99mTc-MDP bone scans were showed increased muscle labelling at the regions of muscle injury. They were treated with hydration, urine alkalinization, & supportive measures in hospital. On considering cause of rhabdomyolysis, our patients did not show any evidence of viral illness or coingestion of other potential myopathic toxins to support a secondary cause of rhabdomyolysis. The mechanism of rhabdomyolysis in cases of doxylamine overdose seems to be a direct toxic effect of the drug on striated muscle, but the exact mechanism is not clear. In all cases where such overdoses are suspected, consideration should be given to obtaining a urinalysis & a creatine phosphokinase level on arrival & creatine phosphokinase levels are carefully followed. Primary detoxication included gastric lavage & administration of activated charcoal. The patient's urine output & renal function should be closely monitored.
Adult ; Autonomic Agents ; Central Nervous System ; Charcoal ; Creatine ; Creatine Kinase ; Creatinine ; Doxylamine* ; Eating ; Emergency Service, Hospital ; Gastric Lavage ; Humans ; Muscle, Striated ; Rhabdomyolysis* ; Suicide ; Technetium Tc 99m Medronate ; Urinalysis

Mongolian gerbil has been as an model animal for studing the neurological diseases such as stroke and epilepsy because of the congenital incompleteries in Willis circle, as well as the investigation of water metabolism because of the long time-survival in the condition of water-deprived desert condition, compared with other animal species. In order to accomplish this research, first of all another divided the laboratory animals 5 groups of which each group include the 5 animals. In this study of the long term water deprived condition author investigatied the vasopressinergic and oxytocinergic magnocellular neurons of the hypothalamus by using a quantitative immunohistochemistry, measured the plasma osmolalities at the time of sacrifice of indivisual animals, and the body weights every day during water-deprived. The results obtained in this study were summarized as followings: 1. The body weights and decreasing rates of the body weight in water-deprived animal groups were continuosly decreased. 2. The plasma osmolalities were increased from the 5th water-deprived day, after then the gradually increase reached nearly its equilibrium state at the 10th water-deprived day. 3. Vasopressin and oxytocin immunoreactive cells were mainly observed in PVN, SON and a few in the lateral magnocellular area of hypothalamus. 4. The number of VP immunoreactive cells in paraventricular and supraoptic nucleus were abruptly decreas-ed until the 5th day in the supraoptic nucleus in number and until the 10th day in the paraventricular nucleus of water-deprived. 5. The OT secreting cells were severely decreased on the 5th water deprived day in paraventricular and supraoptic nucleus, after than these cells were very slowly decreased until to the 38th water deprived day.
Animals ; Animals, Laboratory ; Body Weight ; Circle of Willis ; Epilepsy ; Gerbillinae* ; Hypothalamus* ; Immunohistochemistry ; Metabolism ; Neurons* ; Osmolar Concentration ; Oxytocin ; Paraventricular Hypothalamic Nucleus ; Plasma ; Stroke ; Supraoptic Nucleus ; Vasopressins

PURPOSE: To evaluate the effect of reduced volume of contrast media on vascular opacification and image quality in spiral CT of the chest. MATERIALS AND METHODS: Sixty patients referred for chest CT were examined withspiral CT with 60ml(n=30) or 90ml(n=30) of 30% ionic contrast media(Rayvist 300 , Schering, Germany) alternately.Injection rate of each group was as follows : 2.0 ml/sec for 20 seconds followed by 1.0 ml/sec for 20 seconds in 60ml group and 2.0ml/sec for 45 seconds in 90ml group. Twenty-five seconds scanning delay was employed. For the objective comparison of vascular opacification, CT numbers were measured at superior vena cava, ascending and descending aorta, right and left pulmonary artery, left atrium, and inferior vena cava. For the subjective comparison three radiologists scored the grade of vascular opacification and image quality blindly and independently. All data were analyzed statistically. RESULTS: The mean values of measured CT numbers in 90ml group were higher than those in 60ml group(p <.05) at the same level. The overall mean score of vascular opacification in 90ml group was 2.86, and 2.31 in 60ml group(p <.0001). In the overall mean score of imagequality, there was no statistically significant difference between 90ml group(2.46) and 60ml group(2.40). CONCLUSION: Althought there is some degradation of vascular opacification in 60ml group, overall image quality is not degraded. Therefore, 60 ml of contrast media can be used in spiral CT of the chest without degradation ofoverall image quality except in spiral CT angiography.
Angiography ; Aorta ; Contrast Media* ; Heart Atria ; Humans ; Pulmonary Artery ; Thorax* ; Tomography, Spiral Computed* ; Tomography, X-Ray Computed ; Vena Cava, Inferior ; Vena Cava, Superior