No abstract available.
Epilepsies, Myoclonic* ; Neuroblastoma*

Hajdu-Cheney syndrome (HCS) is a rare skeletal dysplasia that is characterized by acroosteolysis of the distal phalanges, distinctive craniofacial and skull changes, dental abnormalities and generalized osteoporosis. The clinical and radiologic characteristics are variable and these characteristics progress with age. This syndrome shows autosomal dominant inheritance with sporadic cases. The genetic defects or molecular pathogenesis of HCS are still unknown. We experienced a case of Hajdu-Cheney syndrome in a 20-year-old man who had generalized osteoporosis with multiple non-traumatic spine compression fractures. He had acroosteolysis of the hands and feet, wormian bones in the skull, facial dysmorphism (mid-facial flattening, micrognathia and bushy eyebrows), a high arched palate, malocclusion and short dental alveolar processes. HCS was diagnosed based on the clinical and radiologic evidence. For the differential diagnosis, we excluded the other possible causes of the acroosteolysis and wormian bones, including hyperparathyroidism, osteogenesis imperfecta, hypophosphatemia and mandibuloacral dysplasia. The specific treatment of HCS is unknown, but case reports with bisphosphonate treatment have been reported.
Acro-Osteolysis ; Alveolar Process ; Diagnosis, Differential ; Foot ; Fractures, Compression ; Hajdu-Cheney Syndrome ; Hand ; Humans ; Hyperparathyroidism ; Hypophosphatemia ; Malocclusion ; Osteogenesis Imperfecta ; Osteoporosis ; Palate ; Skull ; Spine ; Wills ; Young Adult

Barrett's esophagus is a metaplasia of the esophageal epithelium of any length, such that normal squamous epithelium is replaced by specialized columnar epithelium with goblet cells. It is important to diagnose and survey Barrett's esophagus because it is believed to be the major risk factor for development of esophageal adenocarcinoma. However, the prevalence of Barrett's esophagus in Korea is low. Mixed connective tissue disease (MCTD) is a systemic disorder in which patients have combinations of the clinical features of systemic lupus erythematosus, systemic sclerosis, and polymyositis. Although gastroesophageal reflux disease is common in esophageal involvement in MCTD, long-segment Barrett's esophagus in MCTD has not been reported in Korea. We report here a 15 cm-long segment of Barrett's esophagus extending to the proximal esophagus in a female patient who has had MCTD for 2 years, and we review the literature.
Adenocarcinoma ; Barrett Esophagus ; Epithelium ; Esophagus ; Female ; Gastroesophageal Reflux ; Goblet Cells ; Humans ; Korea ; Lupus Erythematosus, Systemic ; Metaplasia ; Mixed Connective Tissue Disease ; Polymyositis ; Prevalence ; Risk Factors ; Scleroderma, Systemic

BACKGROUND: It was frequently noticed in the course of performing a two-needle sympathectomy, that satisfactory spread of contrast solution could be produced by injection through only one of the needles. This led to a closer examination of the merits of single-needle technique. METHODS: Forty patients were randomized into two groups. Patients in the single-needle group (n = 20) were injected at the second or third lumbar sympathetic ganglia, while patients in the two-needle group (n = 20) were injected at the second and third lumbar sympathetic ganglia. Lumbar sympathetic blocks were performed using 3 ml of alcohol and the spread of injectate was verified with C-arm fluoroscopy. The indicators of a successful sympathetic block are increasing skin temperature, decreasing pain, and anhidrosis in the distal extremity. RESULTS: The duration of anhidrosis was 12.2 2.6 months (mean SD) in the single-needle group versus 13.6 3.6 months (mean SD) in the two-needle group. CONCLUSIONS: As these results were not significantly different from those obtained in patients having a two-needle sympathectomy, blockade of the single-needle technique under C-arm fluoroscopy is considered to be effective for a neurolytic lumbar sympathectomy.
Extremities ; Fluoroscopy ; Ganglia, Sympathetic ; Humans ; Hypohidrosis ; Needles ; Skin Temperature ; Sympathectomy

BACKGROUND: It was frequently noticed in the course of performing a two-needle sympathectomy, that satisfactory spread of contrast solution could be produced by injection through only one of the needles. This led to a closer examination of the merits of single-needle technique. METHODS: Forty patients were randomized into two groups. Patients in the single-needle group (n = 20) were injected at the second or third lumbar sympathetic ganglia, while patients in the two-needle group (n = 20) were injected at the second and third lumbar sympathetic ganglia. Lumbar sympathetic blocks were performed using 3 ml of alcohol and the spread of injectate was verified with C-arm fluoroscopy. The indicators of a successful sympathetic block are increasing skin temperature, decreasing pain, and anhidrosis in the distal extremity. RESULTS: The duration of anhidrosis was 12.2 2.6 months (mean SD) in the single-needle group versus 13.6 3.6 months (mean SD) in the two-needle group. CONCLUSIONS: As these results were not significantly different from those obtained in patients having a two-needle sympathectomy, blockade of the single-needle technique under C-arm fluoroscopy is considered to be effective for a neurolytic lumbar sympathectomy.
Extremities ; Fluoroscopy ; Ganglia, Sympathetic ; Humans ; Hypohidrosis ; Needles ; Skin Temperature ; Sympathectomy

BACKGROUND: Local anesthetics combined with an opioid are frequently used as a postoperative epidural analgesia, to minimize individual doses and to reduce unwanted side effects. The aims of this study were to compare analgesic effectiveness, occurrence of motor block and other side effects of a 48 hr postoperative continuous epidural infusion of 0.1% bupivacaine/morphine or 0.1% ropivacaine/ morphine. METHODS: Forty female patients scheduled for an elective total abdominal hysterectomy under general anesthesia were randomized in a double-blind fashion to receive either 0.1% bupivacaine with 0.02 mg/ml morphine or 0.1% ropivacaine with 0.02 mg/ml morphine using a continuous epidural analgesia pump at a rate of 4 ml/hr during the postoperative 48 hours. After the operation, we assessed the blood pressure, heart rate, visual analog scale (VAS), side effects, additional analgesic requirement and time to ambulation for 48 hours in 12-hour intervals. RESULTS: There were no significant differences in VAS on rest and cough between the groups, but VAS on deep breathing and position change were significantly lower in the bupivacaine group compared to the ropivacaine group. Side effects were similar in both groups except paresthesia. Time to ambulation was earlier in the ropivacaine group compared to the bupivacaine group. CONCLUSIONS: Both the postoperative continuous epidural infusion of 0.1% bupivacaine with morphine and 0.1% ropivacaine with morphine provided similar pain relief and side effects. However, earlier recovery of ambulation in patients receiving ropivacaine/morphine will improve outcome after abdominal surgery.
Analgesia, Epidural* ; Anesthesia, General ; Anesthetics, Local ; Blood Pressure ; Bupivacaine ; Cough ; Female ; Heart Rate ; Humans ; Hysterectomy* ; Morphine ; Paresthesia ; Respiration ; Visual Analog Scale ; Walking

Barrett's esophagus is a metaplasia of the esophageal epithelium of any length, such that normal squamous epithelium is replaced by specialized columnar epithelium with goblet cells. It is important to diagnose and survey Barrett's esophagus because it is believed to be the major risk factor for development of esophageal adenocarcinoma. However, the prevalence of Barrett's esophagus in Korea is low. Mixed connective tissue disease (MCTD) is a systemic disorder in which patients have combinations of the clinical features of systemic lupus erythematosus, systemic sclerosis, and polymyositis. Although gastroesophageal reflux disease is common in esophageal involvement in MCTD, long-segment Barrett's esophagus in MCTD has not been reported in Korea. We report here a 15 cm-long segment of Barrett's esophagus extending to the proximal esophagus in a female patient who has had MCTD for 2 years, and we review the literature.
Adenocarcinoma ; Barrett Esophagus ; Epithelium ; Esophagus ; Female ; Gastroesophageal Reflux ; Goblet Cells ; Humans ; Korea ; Lupus Erythematosus, Systemic ; Metaplasia ; Mixed Connective Tissue Disease ; Polymyositis ; Prevalence ; Risk Factors ; Scleroderma, Systemic

We report the case of a 57-year-old woman with abdominal discomfort. The patient had anemia and hypoalbuminemia. Esophagogastroduodenoscopy and colonoscopy revealed diffuse infiltrative lesions at the stomach, duodenum, and ascending colon. Multiple biopsies were taken and histological examination of the biopsies from the stomach, duodenum and colon showed amyloid deposits stained positively with Congo red. Immunohistochemistry study showed positive sign of kappa and lambda chain at light chain stain. In addition, monoclonal components at serum electrophoresis confirmed the diagnosis of amyloid light-chain amyloidosis. The patient was treated with thalidomide and dexamethasone.
Amyloid ; Amyloidosis* ; Anemia ; Biopsy ; Colon* ; Colon, Ascending ; Colonoscopy ; Congo Red ; Dexamethasone ; Diagnosis ; Duodenum* ; Electrophoresis ; Endoscopy ; Endoscopy, Digestive System ; Female ; Humans ; Hypoalbuminemia ; Immunohistochemistry ; Middle Aged ; Plaque, Amyloid ; Stomach* ; Thalidomide

BACKGROUND/AIMS: Entecavie (ETV) has a potent antiviral effect and low rates of resistance in hepatitis B virus (HBV) and is the first-line monotherapy in patients with HBV-related decompensated cirrhosis. We evaluated the efficacy of 12 months treatment with ETV and tried to determine predictive factors of response. METHODS: Forty-five consecutive decompensated cirrhotic patients who received ETV (0.5 mg/day) for more than six months were included. All patients were positive for HBV DNA, and the Child-Turcotte-Pugh (CTP) scores were over 8 point. Seventeen patients were HBeAg-positive. CTP score, model for end-stage liver disease (MELD) score, serum markers of liver function and HBV DNA were assessed every 3 months. RESULTS: ETV treatment for 12 months resulted in improvement of CTP and MELD scores. Pre-treatment mean CTP and MELD score were decreased from 10.1 (+/-2.0) and 13.48 (+/-4.05) to 7.24 (+/-2.0) and 9.68 (+/-4.85) at 12 months, respectively. The 1-year cumulative rates of HBV DNA negativity and HBeAg loss were 88.9% and 52.9%, respectively, by intention-to-treat analysis. Thirty-two (71.1%) showed improvement in CTP score. Eleven patients did not show change, and 2 patients got worse. The AST/ALT, albumin, bilrubin, prothrombin time were significantly normalized within six months. The good responder group had high level of prothrombin time than the poor responder group (p=0.004). CONCLUSIONS: Our result shows that entecavir can improve liver function in about 70% of patients with HBV related decompensated liver cirrhosis. INR may be a predictive factor of good response with entecavir in these patients.
Adult ; Aged ; Alanine Transaminase/blood ; Antiviral Agents/*therapeutic use ; Bilirubin/blood ; DNA, Viral/analysis ; Drug Administration Schedule ; Female ; Guanine/*analogs & derivatives/therapeutic use ; Hepatitis B e Antigens/blood ; Hepatitis B virus/*genetics ; Hepatitis B, Chronic/complications/*drug therapy ; Humans ; Liver Cirrhosis/*etiology ; Male ; Middle Aged ; Prothrombin Time ; Serum Albumin/analysis ; Severity of Illness Index

BACKGROUND AND OBJECTIVES: Reliable studies about the impact of short-term intubation, particularly as part of general anesthesia, are scarce. That scarcity led to the following research objectives. First of all, we tried to find out how often and why voice change last more than 72 hours after intubation conducted for general anesthesia. SUBJECTS AND METHOD: The study enrolled 80 patients who were due to undergo general anesthesia at the Seoul Paik Hospital from Aug. 2009 to May 2010. The patients were examined through stroboscopic examination and voice analysis before surgery. Three days after the surgery, the same tests were performed again to single out patients whose results were abnormal; thus a proportion could be calculated. The other objective was to determine the factors involved with voice change. This was done according to the Mallampati classification, using the images from laryngoscopy and compiling records of cuff pressure, cuff volume, tube size, duration of intubations, and the number of intubation trials. RESULTS: 7.5% of the patients suffered from voice change longer than 3 days. Three factors, namely, cuff pressure, duration of anesthesia and patient age demonstrated statistically significant relationships among them. CONCLUSION: The results indicate that there is a need for patients scheduled to face general anesthesia to receive sufficient explanation about the possible postoperative voice change that could last longer than 3 days. Furthermore, surgeons and anesthesiologists need to cooperate closely by taking the patient age, duration of anesthesia and cuff pressure into account in order to limit postoperative voice change to the minimum extent.
Anesthesia ; Anesthesia, General ; Humans ; Intubation ; Intubation, Intratracheal ; Laryngoscopy ; Outpatients ; Voice