Disturbance of consciousness in patients with multiple myeloma is usually related with uremia, hypercalcemia or hyperviscosity syndrome. Currently, hyperammonemic encephalopathy which accompanies characteristic change of amino acid levels in pheripheral blood has been reported as a rare cause of mental change in multiple myeloma. We experienced a case of hyperammonemic encephalopathy in a 60-year-old man with multiple myeloma who had shown transient response to the combination chemotherapy with melphalan and prednisolone. Measurement of amino acid concentrations in peripheral blood revealed high glycine/tyrosine ratio and low Fisher ratio. In spite of conventional treatment on hyperammonemic encephalopathy, he died of disease progression without improvement of consciousness level.
Male ; Humans

Autografts ; Bone Remodeling ; Cleidocranial Dysplasia ; Denture, Overlay ; Dentures ; Humans ; Orthodontic Extrusion ; Osseointegration ; Osteoblasts ; Osteogenesis ; Palate ; Prostheses and Implants ; Tooth ; Tooth, Deciduous ; Tooth, Impacted ; Tooth, Supernumerary

BACKGROUND: Patients with paroxysmal nocturnal hemoglobinuria (PNH) often have concurrent aplastic anemia (AA). This study aimed to determine whether eculizumab-treated patients show clinical benefit regardless of concurrent AA. METHODS: We analyzed 46 PNH patients ≥18 years of age who were diagnosed by flow cytometry and treated with eculizumab for more than 6 months in the prospective Korean PNH registry. Patients were categorized into two groups: PNH patients with concurrent AA (PNH/AA, N=27) and without AA (classic PNH, N=19). Biochemical indicators of intravascular hemolysis, hematological laboratory values, transfusion requirement, and PNH-associated complications were assessed at baseline and every 6 months after initiation of eculizumab treatment. RESULTS: The median patient age was 46 years and median duration of eculizumab treatment was 34 months. Treatment with eculizumab induced rapid inhibition of hemolysis. At 6-month follow-up, LDH decreased to near normal levels in all patients; this effect was maintained until the 36-month follow-up regardless of concurrent AA. Transfusion independence was achieved by 53.3% of patients within the first 6 months of treatment and by 90.9% after 36 months of treatment. The mean number of RBC units transfused was significantly reduced, from 8.5 units during the 6 months prior to initiation of eculizumab to 1.6 units in the first 6 months of treatment, for the total study population; this effect was similar in both PNH/AA and classic PNH. CONCLUSION: This study demonstrated that eculizumab is beneficial in the management of patients with PNH/AA, similar to classic PNH.
Anemia, Aplastic* ; Cohort Studies* ; Flow Cytometry ; Follow-Up Studies ; Hemoglobinuria, Paroxysmal* ; Hemolysis ; Humans ; Prospective Studies*

Although lisdexamfetamine is used as a recreational drug, little research exists regarding its potential for dependence or its precise mechanisms of action. This study aims to evaluate the psychoactivity and dependence profile of lisdexamfetamine using conditioned place preference and self-administration paradigms in rodents. Additionally, biochemical techniques are used to assess alterations in the dopamine levels in striatal synaptosomes following administration of lisdexamfetamine. Lisdexamfetamine increased both conditioned place preference and self-administration. Moreover, after administration of the lisdexamfetamine, dopamine levels in the striatal synaptosomes were significantly increased. Although some modifications should be made to the analytical methods, performing high performance liquid chromatography studies on synaptosomes can aid in predicting dependence liability when studying new psychoactive substances in the future. Collectively, lisdexamfetamine has potential for dependence possible via dopaminergic pathway.
Chromatography, Liquid ; Dopamine ; In Vitro Techniques* ; Lisdexamfetamine Dimesylate* ; Rodentia ; Synaptosomes

Although lisdexamfetamine is used as a recreational drug, little research exists regarding its potential for dependence or its precise mechanisms of action. This study aims to evaluate the psychoactivity and dependence profile of lisdexamfetamine using conditioned place preference and self-administration paradigms in rodents. Additionally, biochemical techniques are used to assess alterations in the dopamine levels in striatal synaptosomes following administration of lisdexamfetamine. Lisdexamfetamine increased both conditioned place preference and self-administration. Moreover, after administration of the lisdexamfetamine, dopamine levels in the striatal synaptosomes were significantly increased. Although some modifications should be made to the analytical methods, performing high performance liquid chromatography studies on synaptosomes can aid in predicting dependence liability when studying new psychoactive substances in the future. Collectively, lisdexamfetamine has potential for dependence possible via dopaminergic pathway.
Chromatography, Liquid ; Dopamine ; In Vitro Techniques* ; Lisdexamfetamine Dimesylate* ; Rodentia ; Synaptosomes

No abstract available.
Gastrectomy* ; Hyperhomocysteinemia ; Obesity, Morbid* ; Thrombophilia*

BACKGROUND: Nosocomial candidemia is one of the most common blood-stream infection and associated with a high fatality rate in burn patients. To determine the clinical characteristics, strains of Candida species and to identify contributing factors to death, we analyzed severely burned patients with candidemia. METHODS: 60 cases with candidemia were reviewed retrospectively from January 1999 to December 2003 at a tertiary burn center in Korea. Candidemia was defined as at least one blood culture that grew Candida organisms. RESULTS: Burn size of all patients were 46+/-20.9% of total body surface area burn and overall mortality was 41.7%. 97 strains of Candida species from 60 cases were isolated. Candida albicans was the most frequently isolated species (53.6%), followed by C. tropicalis (20.6%), C. glabrata (15.5%) and C. parapsilosis (10.3%). Among various predisposing factors for candidemia, significant factors associated with death were endotracheal tube insertion or tracheostomy, mechanical ventilation, the use of vasoactive agents, arterial catheterization and nasogastric tube insertion in univariate analysis, but significant independent factors for mortality were mechanical ventilation (OR=26.63, 95% CI; 1.60, 444.18, p=0.022) and the use of vasoactive agents (OR=23.18, 95% CI; 2.80, 192.35, p=0.004) in multivariate analysis. Among 59 patients indwelling central venous catheters, only 24 patients (40.7%) received antifungal therapy with catheter removal while 19 patients (32.2%) had antifungal therapy without catheter removal. CONCLUSIONS: Clinical severity, such as mechanical ventilation or vasoactive agents dependencies, may contribute to high fatality in severely burned patients with candidemia.
Body Surface Area ; Burn Units* ; Burns* ; Candida ; Candida albicans ; Candidemia* ; Catheterization ; Catheters ; Causality ; Central Venous Catheters ; Humans ; Korea ; Mortality* ; Multivariate Analysis ; Respiration, Artificial ; Retrospective Studies ; Tracheostomy

Background: Gaucher disease (GD) is an autosomal recessive disorder characterized by excessive accumulation of glucosylceramide in multiple organs. This study was performed to determine the detection rate of GD in a selected patient population with unexplained splenomegaly in Korea. Methods: This was a multicenter, observational study conducted at 18 sites in Korea between December 2016 and February 2020. Adult patients with unexplained splenomegaly were enrolled and tested for β-glucosidase enzyme activity on dried blood spots (DBS) and in peripheral blood leukocytes. Mutation analysis was performed if the test was positive or indeterminate for the enzyme assay. The primary endpoint was the percentage of patients with GD in patients with unexplained splenomegaly. Results: A total of 352 patients were enrolled in this study (male patients, 199; mean age, 48.42 yr). Amongst them, 14.77% of patients had concomitant hepatomegaly. The most common sign related to GD was splenomegaly (100%), followed by thrombocytopenia (44.32%) and, anemia (40.91%). The β-glucosidase activity assay on DBS and peripheral leukocytes showed abnormal results in sixteen and six patients, respectively. Eight patients were tested for the mutation, seven of whom were negative and one patient showed a positive mutation analysis result. One female patient who presented with splenomegaly and thrombocytopenia was diagnosed with type 1 GD. The detection rate of GD was 0.2841% (exact 95% CI, 0.0072‒). Conclusion The detection rate of GD in probable high-risk patients in Korea was lower than expected.However, the role of hemato-oncologists is still important in the diagnosis of GD.

PURPOSE: Childhood asthma has a considerable social impact and economic burden, especially in severe asthma. This study aimed to identify the proportion of childhood asthma severity and to evaluate associated factors for greater asthma severity. METHODS: This study was performed on 667 children aged 5–15 years with asthma from the nationwide 19 hospitals in the Korean childhood Asthma Study (KAS). Asthma was classified as mild intermittent, mild persistent, and moderate/severe persistent groups according to the National Asthma Education and Prevention Program recommendations. Multinomial logistic regression models were used to identify the associated factors for greater asthma severity. RESULTS: Mild persistent asthma was most prevalent (39.0%), followed by mild intermittent (37.6%), moderate persistent (22.8%), and severe persistent asthma (0.6%). Onset later than 6 years of age (adjusted odds ratio [aOR], 1.69 for mild persistent asthma; aOR, 1.92 for moderate/severe persistent asthma) tended to increase asthma severity. Exposure to environmental tobacco smoke (aOR, 1.53 for mild persistent asthma; aOR, 1.85 for moderate/severe persistent asthma), and current dog ownership with sensitization to dog dander (aOR, 5.86 for mild persistent asthma; aOR, 6.90 for moderate/severe persistent asthma) showed increasing trends with greater asthma severity. Lower maternal education levels (aOR, 2.32) and no usage of an air purifier in exposure to high levels of outdoor air pollution (aOR, 1.76) were associated with moderate/severe persistent asthma. CONCLUSIONS: Modification of identified environmental factors associated with greater asthma severity might help better control childhood asthma, thereby reducing the disease burden due to childhood asthma.
Air Filters ; Air Pollution ; Animals ; Asthma ; Child ; Dander ; Dogs ; Education ; Environmental Exposure ; Humans ; Logistic Models ; Odds Ratio ; Ownership ; Risk Factors ; Smoke ; Social Change ; Tobacco