Systemic sclerosis (SSc) is a chronic systemic disease that affects the skin, lungs, heart, gastrointestinal tract, kidneys, and musculoskeletal system. Although up to 90% of patients with scleroderma have been estimated to have gastrointestinal involvement, liver disease has been reported only rarely. A 51-year-old woman was hospitalized due to esophageal variceal bleeding. Her serum was positive for anti-nuclear antibody and anti-centromere antibody. Sclerodactyly was noted on both hands, and she had recently developed Raynaud's syndrome. Punch biopsy of the hand showed hyperkeratosis, regular acanthosis, and increased basal pigmentation in the epidermis, and thick pale collagenous bundles in the dermis. Liver biopsy showed chronic active hepatitis with bridging fibrosis. Consequently, she was diagnosed with liver cirrhosis due to autoimmune hepatitis (AIH) combined with SSc. AIH had subsided after administration of prednisolone at 40 mg per day. She received 5-10 mg/day of prednisolone as an outpatient, and her condition has remained stable. Patients with either AIH or SSc should be monitored for further development of concurrent autoimmune diseases. The early diagnosis of AIH combined with SSc will be helpful in achieving optimal management.
Anti-Inflammatory Agents/therapeutic use ; Antibodies, Antinuclear/blood ; Esophageal and Gastric Varices ; Female ; Gastrointestinal Hemorrhage ; Hepatitis, Autoimmune/complications/*diagnosis/drug therapy ; Humans ; Liver Cirrhosis/*diagnosis/etiology/pathology ; Middle Aged ; Prednisolone/therapeutic use ; Raynaud Disease/diagnosis ; Scleroderma, Systemic/complications/*diagnosis ; Skin/pathology

BACKGROUND: Postopertative nausea and vomiting (PONV) are frequent and distressing side effects of surgery. Even though many drugs has been developed, PONV still remains unsolved problem. Ondansetron is a commonly used 5-HT3 receptor antagonist. It acts through specific binding to the 5-HT3A, 5-HT3B receptor complex. We hypothesized that patients with genetic variation in 5-HT3A receptor might have variable incidence of PONV and respond differently to ondansetron. METHODS: We included 204 patients undergoing gynecologic laparoscopic surgery. PONV were documented during 24 hours after operation. Ondansetron was injected to every patient who had PONV at PACU and PONV reassessed after 15 minutes. DNA was extracted from blood and 5-HT3A Pro16Ser missense mutation was analyzed by using real-time PCR. RESULTS: The incidence of PONV were 50% for wild type, 53% for heterozygote and 0% for homozygote. There were no significant differences between wild type and heterozygote in VAS of nausea and VAS change after ondansetron. CONCLUSIONS: 5-HT3A receptor Pro16Ser polymorphism is not associated with the incidence of PONV and the response to ondansetron in Korean patients.
DNA ; Genetic Variation ; Heterozygote ; Homozygote ; Humans ; Incidence ; Laparoscopy ; Mutation, Missense ; Nausea ; Ondansetron ; Postoperative Nausea and Vomiting ; Real-Time Polymerase Chain Reaction ; Receptors, Serotonin, 5-HT3 ; Vomiting

Dyke-Davidoff-Masson syndrome (DDMS) is a rare condition characterized by asymmetric cerebral hemispheric growth with unilateral atrophy, ipsilateral compensatory osseous hypertrophy, hyperpneumatization of the paranasal sinuses and mastoid cells, and contralateral paresis. Varying degrees of hemiparesis, hemiplegia, seizures, mental retardation, and facial asymmetry can be associated with DDMS. We report the case of a 26-year-old man with DDMS associated with hypopituitarism who complained of polydipsia and polyuria. After an oral glucose tolerance test, he was diagnosed with type 2 diabetes. There is no report of DDMS associated with other pituitary dysfunction or hyperglycemia. Clinicians should consider the possibility of coexisting pituitary dysfunction or type 2 diabetes in patients with DDMS, as it is obviously important for the patient's outcome.
Adult ; Amides ; Atrophy ; Diabetes Mellitus ; Facial Asymmetry ; Glucose Tolerance Test ; Hemiplegia ; Humans ; Hyperglycemia ; Hypertrophy ; Hypopituitarism ; Intellectual Disability ; Mastoid ; Paranasal Sinuses ; Paresis ; Polydipsia ; Polyuria ; Seizures ; Sulfones

BACKGROUND: This clinical study was designed to evaluate the effect of midazolam as a premedication on the onset of propofol and rocuronium during propofol target-controlled infusion (TCI). METHODS: Seventy four patients (ASA class I or II) were randomly allocated to receive either no premedication (control group) or premedication with 0.04 mg/kg intravenous midazolam (midazolam group). Anesthesia was induced and maintained with propofol TCI. Time from propofol injection to loss of consciousness (LOC) and estimated effect concentration at LOC were recorded. After LOC, rocuronium (0.6 mg/kg) was injected. We monitored the degree of neuromuscular blockade by acceleromyography. The following parameters were measured and compared between groups: Time from rocuronium injection to depression of twitch height below 25%, time to maximal depression of twitch height (defined as rocuronium onset time). RESULTS: Systolic blood pressure before induction was lower in midazolam group (125 +/- 15 vs 135 +/- 20 mmHg), however, there was no difference in blood pressure at LOC between groups (111 +/- 16 vs 106 +/- 21 mmHg). In midazolam group, time to LOC in propofol TCI was shorter (63 +/- 22 vs. 203 +/- 118 sec) and estimated effect site concentration of propofol was significantly lower than control group (0.9 +/- 0.3 vs. 2.2 +/- 0.4 microl/ml). The onset time of rocuronium was not different between groups (120 +/- 39 vs. 137 +/- 42 sec). CONCLUSIONS: Midazolam pretreatment fastens the onset time of propofol and decreases the propofol requirement for LOC. However, it does not influence the onset of rocuronium.
Androstanols ; Anesthesia ; Blood Pressure ; Depression ; Humans ; Midazolam ; Neuromuscular Blockade ; Premedication ; Propofol ; Unconsciousness

BACKGROUND: The criteria for the diagnosis of diabetes mellitus have been modified by the American Diabetes Association (ADA) in 1997. The ADA proposed that the diagnosis of diabetes be defined by a fasting plasma glucose (FPG) of 7.0 mmol/L. Disagreement has been reported between criteria based on FPG and postchallenge 2-h plasma glucose (2-h PG). The aim of the present study is to assess the FPG criteria as the diagnostic screening test for diabetes in Korean middle-aged adults in comparison to the 2-h PG criteria. METHODS: Randomly selected 1,731 subjects (679 men and 1,052 women) aged 40~70 years (mean age: 58.4 +/- 7.89 years) without previously diagnosed diabetes completed 75 g oral glucose tolerance test (OGTT). We assessed the prevalence of diabetes mellitus and the level of agreement (kappa statistics) according to the different diagnostic glucose categories. RESULTS: The frequency of newly diagnosed diabetes was 2.7% (n = 51) using the FPG criteria only; 6.4% (n = 120) using the 2-h PG criteria only; and 6.9% (n = 130) using concentrations of > or = 7.0 mmol/L for FPG or > or = 11.1 mmol/L for 2-h PG. Of the 120 subjects with diabetes by the 2-h PG criteria, 65.8% (n = 79) were not diagnosed with diabetes according to FPG concentration. The level of agreement between two diagnostic criteria was low (kappa = 0.268). The receiver operating characterstic (ROC) curve analysis determined FPG of 5.6 mmol/L to yield optimal sensitivity and specificity corresponding to 2-h PG 11.1 mmol/L. CONCLUSION: The findings in this study demonstrate that the discordance between the FPG and 2-h PG criteria in the diagnosis of diabetes in Korean middle-aged adults is large. We suggest that IFG group (FPG 5.6~6.9 mmol/L) were performed 75 g OGTT for diagnosing diabetes mellitus in Korean middle-aged adults.
Adult ; Aged ; Diabetes Mellitus ; Diabetes Mellitus, Type 2 ; Fasting ; Glucose ; Glucose Tolerance Test ; Humans ; Male ; Mass Screening ; Plasma ; Prevalence ; Sensitivity and Specificity

Insuliin secreting tumor is 70% prevalent disease in female and predoadnant in forth and sixth deeade. The incidence of insulinoma is one case per 250,000 patient-years. Insulinoma in pregnancy was extremely rare, and the prevalence was not reported. The diagnosis of an insulinoma is depend on demonstration of hypoglycemia with high insulin and C-peptide levels. Immunoreactive insulin/plasma glucose ratio0.3 in particular support the diagnosis of an insulinoma. Fetal complication would be developed because of hypoglycemia. In approximately half of the cases reported, surgical exploration was done during pregrancy, the remainder were treated after delivery. Insulinoma poses serious diagnostic and therapeutic problems when she is pregnant. We experienced a case of insulinoma in pregnancy that represented Whipples triad and was treated by surgical intervention.
C-Peptide ; Diagnosis ; Female ; Glucose ; Humans ; Hypoglycemia ; Incidence ; Insulin ; Insulinoma* ; Pregnancy* ; Prevalence

DREAM (downstream regulatory element antagonistic modulator) is a calcium-binding protein that regulates dynorphin expression, promotes potassium channel surface expression, and enhances presenilin processing in an expression level-dependent manner. However, no molecular mechanism has yet explained how protein levels of DREAM are regulated. Here we identified group I mGluR (mGluR1/5) as a positive regulator of DREAM protein expression. Overexpression of mGluR1/5 increased the cellular level of DREAM. Up-regulation of DREAM resulted in increased DREAM protein in both the nucleus and cytoplasm, where the protein acts as a transcriptional repressor and a modulator of its interacting proteins, respectively. DHPG (3,5-dihydroxyphenylglycine), a group I mGluR agonist, also up-regulated DREAM expression in cortical neurons. These results suggest that group I mGluR is the first identified receptor that may regulate DREAM activity in neurons.
Calcium ; Cytoplasm ; Dynorphins ; Methoxyhydroxyphenylglycol ; Neurons ; Potassium Channels ; Presenilins ; Proteins ; Receptors, Metabotropic Glutamate ; Up-Regulation

PURPOSE: The combination of intravenous cyclophosphamide (CYC) and prednisolone is effective for the treatment of severe lupus nephritis but has serious adverse effects. Mycophenolate mofetil (MMF) Is a new immunosuppressive agent that selectively inhibits activated lymphocytes. This study reports on the clinical experiences at our clinic with MMF and intravenous CYC for the initial induction treatment in patients with lupus nephritis. METHODS: 50 patients with lupus nephritis received induction therapy consisting of MMF and prednisolone (n=22) or intravenous CYC and prednisolone (n=28), and followed up for six months. Complete remission was defined as a value for urinary protein: urinary creatinine ratio (U(p/Cr)) that was less than 0.3, with normal urinary sediment, a normal serum albumin concentration and values for serum creatinine that were no more than 15 percent above the base-line values. Partial remission was defined as a value for U(p/Cr) that was between 0.3 and 2.9, with a serum albumin concentration of at least 3.0 g/dL. RESULTS: 22 patients treated with MMF and 28 patients with intravenous CYC resulted in complete remission (31.8% vs 39.3%), partial remission (45.5% vs 39.3%) and treatment failure (22.7% vs 21.4 %). Fewer severe infections occurred among patients treated with MMF and prednisolone. CONCLUSION: As for the induction therapy of lupus nephritis, the combination of MMF and prednisolone may be an effective regimen. However, further randomized, prospective studies are needed to prove the effectiveness of MMF therapy in lupus nephritis.
Creatinine ; Cyclophosphamide* ; Humans ; Lupus Nephritis* ; Lymphocytes ; Prednisolone ; Serum Albumin ; Treatment Failure

Crystalline nephropathy is a rare yet well-known condition associated with multiple myeloma and other light chain–secreting disorders. Paraproteins that are resistant to proteolysis crystallize within proximal tubular cells and cause light-chain proximal tubulopathy, which presents clinically as Fanconi syndrome. Podocytes are rarely affected, and the crystalline inclusions within podocytes are typically precipitated, yielding significant glomerular proteinuria. Here we report a case of extensive crystalline inclusions primarily within podocytes and proximal tubules that presented only with Fanconi syndrome and renal insufficiency. Despite the presence of extensive crystalline inclusions in podocytes and diffuse foot process effacement, the patient had no clinical evidence suggestive of podocyte injury.
Crystallins* ; Fanconi Syndrome ; Foot ; Humans ; Multiple Myeloma* ; Paraproteins ; Podocytes ; Proteinuria* ; Proteolysis ; Renal Insufficiency

BACKGROUND: It has been more than 15 years since infection control was first introduced in Korea, but there is little information available on the status of infection control program in the country. METHODS: Included in the study were 139 acute care hospitals with more than 300 inpatient beds. A questionnaire, modified from US SENIC (Study on the Efficacy of Nosocomial Infection Control) and Canadian RICH (Resources for Infection Control in Canadian Acute Care Hospitals) survey, was mailed to the hospitals in the winter of 2003. RESULTS: Ninety-eight (70.5%) of 139 hospitals responded. There was an average of 1.2 (SD, 0.7) Infection Control Practitioners (lCPs) in each hospital and 95.7% were nurses and only 56.5% of the ICPs worked as full-time. The 71.4% of the hospitals had a position for Infection Control Doctor. All hospitals had an Infection Control Committee, which met an average of 3.7 (SD, 1.7) times a year. The 85.7% of the hospitals performed surveillance, but only 31.6% were monitoring surgical site infections. Review of microbiology data was the most common method for case-finding. More than 90% of the hospitals had infection control policies and guidelines, but an adherence to the policies and guidelines was not monitored regularly. CONCLUSION: This study reports the first comparable profile of infection control program of general acute care hospitals in Korea. Although the foundation for infection control program appears to have been established, there is the need for a further increase in the number of ICPs, the standardization of the surveillance method, and the promotion of adherence to the infection control guidelines.
Cross Infection ; Hospitals, General* ; Humans ; Infection Control Practitioners ; Infection Control* ; Inpatients ; Korea ; Postal Service ; Surveys and Questionnaires