BACKGROUND AND PURPOSE: Charcot-Marie-Tooth disease (CMT) type 1A (CMT1A) is the demyelinating form of CMT that is significantly associated with PMP22 duplication. Some studies have found that the disease-related disabilities of these patients are correlated with their compound muscle action potentials (CMAPs), while others have suggested that they are related to the nerve conduction velocities. In the present study, we investigated the correlations between the disease-related disabilities and the electrophysiological values in a large cohort of Korean CMT1A patients. METHODS: We analyzed 167 CMT1A patients of Korean origin with PMP22 duplication using clinical and electrophysiological assessments, including the CMT neuropathy score and the functional disability scale. RESULTS: Clinical motor disabilities were significantly correlated with the CMAPs but not the motor nerve conduction velocities (MNCVs). Moreover, the observed sensory impairments matched the corresponding reductions in the sensory nerve action potentials (SNAPs) but not with slowing of the sensory nerve conduction velocities (SNCVs). In addition, CMAPs were strongly correlated with the disease duration but not with the age at onset. The terminal latency index did not differ between CMT1A patients and healthy controls. CONCLUSIONS: In CMT1A patients, disease-related disabilities such as muscle wasting and sensory impairment were strongly correlated with CMAPs and SNAPs but not with the MNCVs or SNCVs. Therefore, we suggest that the clinical disabilities of CMT patients are determined by the extent of axonal dysfunction.
Action Potentials ; Axons ; Charcot-Marie-Tooth Disease ; Cohort Studies ; Humans ; Muscles ; Neural Conduction

The publisher wishes to apologize for incorrectly displaying the author (Seok Beom Gwon) name. We correct his name from Seok Beom Gwon to Seok Beom Kwon.

PURPOSE: To report an epidemiologic study of keratoplasty in which we analyze basic data of corneal transplants and corneal donations in Korea. METHODS: From June 2004 through October 2004, questionnaires were distributed to 25 hospitals. The questionnaires were about the characteristics of patients registered in eye banks for keratoplasty from May 2001 to April 2003, and about the results of keratoplasties performed from May 2002 to April 2003. RESULTS: The leading corneal diseases in patients registered for keratoplasty were infectious keratitis (22.0%), trauma (21.0%), and pseudophakic and aphakic bullous keratopathy (13.8%), in that order. The leading indications in corneal recipients were: trauma (15.5%), pseudophakic and aphakic bullous keratopathy (13.7%), and infectious keratitis (10.7%), in that order. In 233 cases of keratoplasties, penetrating keratoplasty was performed in 229 cases (98.3%) and lamellar keratoplasty was performed in 4 cases (1.7%). The most common combined surgery was cataract extraction. Common causes of graft failure were rejection of donor cornea and infection. Imported donor corneas were used in sixty-one cases (26.2%) among the 233 total keratoplasties. CONCLUSIONS: We constructed an epidemiologic data base of patients registered for keratoplasty, corneal donation, and the results of keratoplasties throughout Korea.
Cataract Extraction ; Cornea ; Corneal Diseases ; Corneal Transplantation* ; Epidemiologic Studies* ; Eye Banks ; Humans ; Keratitis ; Keratoplasty, Penetrating ; Korea* ; Surveys and Questionnaires ; Tissue Donors ; Transplants

PURPOSE: To report an epidemiologic study of keratoplasty in which we analyze basic data of corneal transplants and corneal donations in Korea. METHODS: From June 2004 through October 2004, questionnaires were distributed to 25 hospitals. The questionnaires were about the characteristics of patients registered in eye banks for keratoplasty from May 2001 to April 2003, and about the results of keratoplasties performed from May 2002 to April 2003. RESULTS: The leading corneal diseases in patients registered for keratoplasty were infectious keratitis (22.0%), trauma (21.0%), and pseudophakic and aphakic bullous keratopathy (13.8%), in that order. The leading indications in corneal recipients were: trauma (15.5%), pseudophakic and aphakic bullous keratopathy (13.7%), and infectious keratitis (10.7%), in that order. In 233 cases of keratoplasties, penetrating keratoplasty was performed in 229 cases (98.3%) and lamellar keratoplasty was performed in 4 cases (1.7%). The most common combined surgery was cataract extraction. Common causes of graft failure were rejection of donor cornea and infection. Imported donor corneas were used in sixty-one cases (26.2%) among the 233 total keratoplasties. CONCLUSIONS: We constructed an epidemiologic data base of patients registered for keratoplasty, corneal donation, and the results of keratoplasties throughout Korea.
Cataract Extraction ; Cornea ; Corneal Diseases ; Corneal Transplantation* ; Epidemiologic Studies* ; Eye Banks ; Humans ; Keratitis ; Keratoplasty, Penetrating ; Korea* ; Surveys and Questionnaires ; Tissue Donors ; Transplants

OBJECTIVE: The purpose of this study was to evaluate the relationship between uterine size and surgical outcomes in women undergoing total vaginal hysterectomy. METHODS: We reviewed the medical records of the 170 patients who underwent total vaginal hysterectomy from February 2001 to November 2003. These patients were stratified into two groups; Group I, patients with uterine weight of between 300 gm to 1000 gm; Group II, patients with uterine weight of <300 gm. The two groups were compared for ages, parity, uterine weight, previous abdominal operations, concurrent surgical procedures, postoperative discharge day, bleeding amount, perioperative hemoglobin concentration change and operative time. RESULTS: 1. There were no significant difference between the two groups with respect to postoperative discharge day, concurrent surgical procedures, bleeding amount and perioperative hemoglobin concentration change. 2. The rate of surgical complications were similar in the two groups (Group I 12.5%; Group II 13.1%) (P=.660). 3. Operative time for vaginal hysterectomy was slightly longer for women in Group I than Group II (Group I 78.4 +/- 27.1 minutes; Group II 62.9 +/- 22.7 minutes) (P=.176), but the difference between the two groups in operative time was not statistically significant. CONCLUSION: The vaginal hysterectomy is as safe and effective for the woman with enlarged uterus (300-1000 gm) as for the woman with uterine weight of <300 gm. Uterine enlargement (uterine weight > 300 gm) is not an absolute contraindication to vaginal hysterectomy.
Female ; Hemorrhage ; Humans ; Hysterectomy, Vaginal* ; Medical Records ; Operative Time ; Parity ; Postoperative Care ; Uterus

OBJECTIVE: The purpose of our study is to find out the therapeutic effectiveness, reproductive outcome, and the diagnostic pathologic findings of the patients treated with resectoscopy. METHODS: We examined 110 patients who complained infertility, abnormal uterine bleeding and menorrhagia from May. 1995 to Dec. 2000 via office. Among the infertility and abnormal uterine bleeding patients with abnormal endometrial lesion, resectoscopy was performed and then the resected tissues was sended for pathologic examination, except IUA, uterine septum, and double uterus. After resectoscopy, we inserted Lippes loop and medicated premarin 2.5 mg, 54 days and medroxyprogesterone acetate 10 mg, 10 days to the IUA patients. In AUB patients, we only injected methergin for bleeding control. They visited office after 1 month, and the follow up for endometrial state was done through TVS, HSG, or hysteroscopy. RESULTS: Among the 44 infertility, 41 (93%) had normal endometrium findings. Of twenty pregnancy (48.8%), seven (35%) ended in a second trimester abortion, thirteen (65%) in a full-term infant. Among the 66 with abnormal uterine bleeding, the 62 (93%) had normal endometrial finding and normal menstruation pattern. But 2 patients recurred their symptom, then hysterectomy was done 3, 5 years later respectively. Most of the patients who performed histopathologic study were diagnosed as leiomyoma (59.4%), and then endometrial or endocervical polyp (25.3%) and residual placenta tissue (3.8%) respectively. CONCLUSION: The 101 patients (91.8%) improved their symptoms and intrauterine lesion. we suggest this technique which of diagnostic accuracy, cost safety, convenience, operation time, and patient's satisfaction.
Endometrium ; Estrogens, Conjugated (USP) ; Female ; Follow-Up Studies ; Gynecology* ; Hemorrhage ; Humans ; Hysterectomy ; Hysteroscopy ; Infant ; Infertility ; Leiomyoma ; Medroxyprogesterone Acetate ; Menorrhagia ; Menstruation ; Methylergonovine ; Placenta ; Polyps ; Pregnancy ; Pregnancy Trimester, Second ; Uterine Hemorrhage ; Uterus

Acute pancreatitis is one of the major complications of endoscopic retrograde cholangiopancreatography (ERCP) and endoscopic sphincterotomy (EST). Various etiology such as mechanical, chemical, hydrostatic, and thermal factor are thought to be involved for this procedure-related pancreatitis. However, acute pancreatitis can occur as a direct complication of endoscopic biliary drainage (EBD). Although the exact mechanism remains unclear, it is postulated that the stent compresses pancreatic ductal orifice and resultant pancreatic outflow obstruction actually provokes pancreatitis. Using the larger stent diameter over 10 Fr and a straight stent rather than curved one, proximal rather than distal bile duct obstruction are risk factors for stent-induced pancreatitis. We report on three cases of acute pancreatitis complicating the EBD with a plastic stent, nasobiliary catheter, and covered-metallic stent respectively.
Catheters ; Cholangiopancreatography, Endoscopic Retrograde ; Cholestasis ; Drainage ; Pancreatic Ducts ; Pancreatitis* ; Plastics ; Risk Factors ; Sphincterotomy, Endoscopic ; Stents*

PURPOSE: Malnutrition is common in CAPD patients and depends on many factors such as dialysis-related and nondialysis-related factors. The present study aimed to assess nutritional status, dialysis adequacy and their relationships with overall mortality and morbidity. METHODS: We studied 102 patients who had been receiving CAPD for at least 6 months. Dialysis adequacy was assessed by parameters derived from urea kinetic modeling(UKM) and nutritional status was assessed by serum biochemical measurement, normalized protein catabolic rate(nPCR), normalized protein equvalent of total nitrogen appearance(nPNA) and urea kinetic studies. Spearman's simple correlation and multiple linear stepwise regression analysis were used to assess correlation between dialysis adequacy and nutritional status in CAPD patients. We compared the differences between patients who suf fered morbid events, defined as either an infectious complication or hospitalization, and patients who remained well. RESULTS: The results showed that the total dialysis dose(total weekly Kt/Vurea) has statistically significant correlation with nPCR(r=0.234, p=0.028), nPNA (r=0.246, p=0.021), total weekly creatinine clearance (WCC)(r=0.479, p=0.0001), serum albumin levels(r= 0.233, p=0.029), serum cholesterol(r=0.266, p=0.013), serum BUN(r=-0.290, p=0.006) and serum creatinine levels(r=-0.408, p=0.0001). nPNA was positively correlated with serum cholesterol(r=0.217, p=0.045), serum transferrin(r=0.218, p=0.042) and serum ferritin levels(r=0.220, p=0.043). Patients who suffered morbid events had an old age(p=0.001), long duration of CAPD(p=0.0001), higher CRP(p=0.021), lower serum albumin level(p=0.020), lower hematocrit(p=0.049) and lower WCC(p=0.017). Conclusions : These results indicate that adequate dialysis is very important for the maintenance of adequant nutrition because nutritional status positively correlated with dialysis dose, which is best assessed by UKM. In addition, assessment of nutritional status and dialysis adequacy are important in predicting clinical outcomes in CAPD patients.
Creatinine ; Dialysis* ; Ferritins ; Hospitalization ; Humans ; Malnutrition ; Mortality ; Nitrogen ; Nutritional Status* ; Peritoneal Dialysis, Continuous Ambulatory ; Serum Albumin ; Urea

Silicone fluid(polydimethylsiloxane) is widely used in breast augmentation and other cosmetic procedures because of little incidence of complications and low mortality rate. However, local reaction following silicone injections can be occurred sometimes leading to serious complications. Especially, illicit silicone injections have resulted in severe reactions within the pulmonary area, and some have resulted in acute respiratory distress syndrome subsequently. We experienced a case of acute respiratory distress syndrome induced by subcutaneous injections of silicone at vaginal wall. The patients was 39-year-old, previously healthy woman who had complained of dyspnea related to silicone injection at vaginal wall. Chest X-ray and chest CT scan show diffuse air consolidation with ground glass opacities and perfusion lung scan revealed likelihood of pulmonary embolism as showing multiple perfusion defects. We report a case of acute respiratory distress syndrome after silicone injection with review of literature.
Adult ; Breast ; Dyspnea ; Female ; Glass ; Humans ; Incidence ; Injections, Subcutaneous ; Lung ; Mortality ; Perfusion ; Pulmonary Embolism* ; Respiratory Distress Syndrome, Adult* ; Silicones* ; Thorax ; Tomography, X-Ray Computed

The relationship between neoplastic disease and thromboembolic disorders has been recognized since 1865, when Armand Trousseau first reported a high incidence of venous thrombosis in a series of patients with gastric carcinoma. The overall incidence of thromboembolic disease in patients with cancer has been reported to vary 1% to 15%. In a prospective study, Ambrus and associates reported that thrombosis and/or bleeding was the second most common cause of death in hospitalized cancer patients. We report a case who presented as a thromboembolic disease and subsequently confirmed to have an underlying lung malignancy. This 45 years old male patient visited our hospital with abdominal pain and distention of 3 days duration. Abdominal CT scan revealed multiple splenic and renal infarctions. On 20th hospital day, drowsy mental status was developed and hemorrhagic cerebral infarction was noted in brain CT scan. Chest CT scan revealed a 4cm sized spiculated mass on left lung apex and multiple paratracheal lymph adenopathy. With surgical biopsy of left supraclavicular lymph nodes, this patient was confirmed to have adenocarcinoma.
Abdominal Pain ; Adenocarcinoma ; Biopsy ; Brain ; Cause of Death ; Cerebral Infarction ; Hemorrhage ; Humans ; Incidence ; Infarction* ; Lung* ; Lymph Nodes ; Male ; Middle Aged ; Prospective Studies ; Thrombosis ; Tomography, X-Ray Computed ; Venous Thrombosis