PURPOSE: Rufinamide (RFM) is known to be effective for children with Lennox-Gastaut syndrome (LGS). The aim of this study is to evaluate its efficacy and tolerability of Korean children with LGS. METHODS: This is a single center, open label, retrospective study. Patients with LGS who received rufinamide as adjunctive therapy were enrolled in this study. Their baseline clinical characteristics, the percent change in the seizure frequency per 4 weeks, and adverse events were evaluated. RESULTS: Among 32 children, 20 were males and the mean age was 11.3±6.6 years. After 1 month of rufinamide medication, the frequency of seizures was reduced by more than 50% in 31.3% of patients and 6.3% of patients had no seizures. After 6 months of rufinamide administration, patients with a 50% or less decrease in seizure frequency remained in a state of reduced seizure frequency and 3.1% of patients had no seizures. Side effects such as nausea, vomiting, anorexia, less active, somnolence, aggression, drooling were noted in 28.1% of patients. CONCLUSION: This study suggests that rufinamide can be considered as an effective and safe treatment option for intractable epileptic children such as LGS.
Aggression ; Anorexia ; Child* ; Epilepsy ; Humans ; Male ; Nausea ; Retrospective Studies ; Seizures ; Sialorrhea ; Vomiting

Most of the patients with hemorrhagic fever with renal syndrome (HFRS) by Hantaviruses recover completely. However, the prevalence of hypopituitarism as sequel of HFRS may be approximately 10%. However, it is rare for hyponatremia to present as a manifestation of hypopituitarism due to HFRS. A 42-year-old Asian woman presented with anorexia, nausea, and vomiting. She had a history of HFRS 7 years ago. During her first hospital admission, her serum sodium was 103 mmol/L. The rapid adrenocorticotropic hormone (ACTH) stimulation test showed no abnormal findings suggesting the possibility of adrenal insufficiency. After she underwent fluid therapy for 5 days, her serum sodium level was slightly increased to 131 mmol/L, and her clinical symptoms were improving. The next day after she was discharged, she suffered from the same symptoms and was admitted to our hospital again. On the day of her second admission, her serum sodium was 117 mmol/L. A combined pituitary function test showed diminished function of the anterior pituitary gland, which led to secondary adrenal insufficiency. Magnetic resonance imaging findings of her sella were compatible with empty sella turcica. After the replacement of prednisolone, her serum sodium levels returned to normal. Pituitary dysfunction should be included in the differential diagnosis of severe hyponatremia, especially in patients who suffered HFRS. This case also suggests that the rapid ACTH stimulation test has limitations with regard to the differential diagnosis of primary or secondary adrenal insufficiency.
Adrenal Insufficiency ; Adrenocorticotropic Hormone ; Adult ; Anorexia ; Asian Continental Ancestry Group ; Diagnosis, Differential ; Empty Sella Syndrome ; Female ; Fluid Therapy ; Hantavirus ; Hemorrhagic Fever with Renal Syndrome ; Humans ; Hyponatremia ; Hypopituitarism ; Magnetic Resonance Imaging ; Nausea ; Pituitary Function Tests ; Pituitary Gland, Anterior ; Prednisolone ; Prevalence ; Sodium ; Vomiting

BACKGROUND/AIMS: Loss of transforming growth factor beta1 (TGF-beta1) exhibits a similar pathology to that seen in a subset of individuals infected with Helicobacter pylori, including propagated gastric inflammation, oxidative stress, and autoimmune features. We thus hypothesized that gastric mucosal TGF-beta1 levels could be used to determine the outcome after H. pylori infection. METHODS: Northern blot for the TGF-beta1 transcript, staining of TGF-beta1 expression, luciferase reporter assay, and enzyme-linked immunosorbent assay for TGF-beta1 levels were performed at different times after H. pylori infection. RESULTS: The TGF-beta1 level was markedly lower in patients with H. pylori-induced gastritis than in patients with a similar degree of gastritis induced by nonsteroidal anti-inflammatory drugs. There was a significant negative correlation between the severity of inflammation and gastric mucosal TGF-beta1 levels. SNU-16 cells showing intact TGF-beta signaling exhibited a marked decrease in TGF-beta1 expression, whereas SNU-638 cells defective in TGF-beta signaling exhibited no such decrease after H. pylori infection. The decreased expressions of TGF-beta1 in SNU-16 cells recovered to normal after 24 hr of H. pylori infection, but lasted very spatial times, suggesting that attenuated expression of TGF-beta1 is a host defense mechanism to avoid attachment of H. pylori. CONCLUSIONS: H. pylori infection was associated with depressed gastric mucosal TGF-beta1 for up to 24 hr, but this apparent strategy for rescuing cells from H. pylori attachment exacerbated the gastric inflammation.
Blotting, Northern ; Enzyme-Linked Immunosorbent Assay ; Gastritis ; Helicobacter pylori ; Humans ; Inflammation ; Luciferases ; Oxidative Stress ; Transforming Growth Factor beta ; Transforming Growth Factor beta1 ; Ulcer

We report a case of prolonged extreme reactive thrombocytosis in a post-splenectomy patient with hereditary spherocytosis. A 29-year-old female patient presented with gall stones detected incidentally by abdominal ultrasonography. Her laboratory findings showed hemolytic anemia with spherocytosis on the peripheral blood smear and increased osmotic fragility. She was diagnosed with hereditary spherocytosis and underwent a laparoscopic cholecystectomy and splenectomy. After undergoing surgery, the hemolytic anemia was resolved but thrombocytosis was newly detected. Nineteen months after the splenectomy, the thrombocytosis was still persistent and extremely high. To our knowledge, this is the first report of a prolonged extreme reactive thrombocytosis after a splenectomy in Korea.
Adult ; Anemia, Hemolytic ; Cholecystectomy, Laparoscopic ; Female ; Gallstones ; Humans ; Korea ; Osmotic Fragility ; Spherocytosis, Hereditary ; Splenectomy ; Thrombocytosis

BACKGROUND/AIMS: There is increasing need for third-line therapy of Helicobacter pylori due to increasing level of antibiotics resistance. The aim of this study was to compare rifabutin and levofloxacin rescue regimens in patients with first- and second-line Helicobacter pylori eradication failures. METHODS: Patients, in whom a first treatment with proton pump inhibitor-clarithromycin-amoxicillin and a second trial with proton pump inhibitor-bismuth-tetracycline-metronidazole had failed, received treatment with either rifabutin or levofloxacin, plus amoxicillin (1 g twice daily) and standard dose proton pump inhibitor. Eradication rates were confirmed with 13C-urea breath test or rapid urease test 4 weeks after the cessation of therapy. RESULTS: Eradication rates were 71.4% in the rifabutin group, and 57.1% in the levofloxacin group, respectively. Although there was no significant difference in Helicobacter pylori eradication rates between two groups (p=0.656), rifabutin based regimen showed relatively higher eradication rate. CONCLUSIONS: Helicobacter pylori eradication rates of rifabutin- or levofloxacin-based triple therapy could not achieve enough eradication rate. Further studies would be needed on combination of levofloxacin and rifabutin-based regimen or culture based treatment.
Adult ; Aged ; Aged, 80 and over ; Amoxicillin/therapeutic use ; Anti-Bacterial Agents/*therapeutic use ; Breath Tests ; Drug Resistance, Bacterial/drug effects ; Drug Therapy, Combination ; Female ; Helicobacter Infections/*drug therapy ; Helicobacter pylori ; Humans ; Male ; Middle Aged ; Ofloxacin/*therapeutic use ; Proton Pump Inhibitors/therapeutic use ; Rifabutin/*therapeutic use ; Salvage Therapy