The reinforcement of regulation on of post-market safety management including adverse drug reactions (ADRs) has received significant emphasiszed significantly over the last several years in Korea. Not only has there been an increase in the number of spontaneous reports on ADRs, but an amendment of to the pharmaceutical law has been passed and notifications have noticeably been accelerated noticeably. However, compared with advanced countries, the efficiency of the system and people's satisfaction withon post-market safety management was has been as low as ever. This article focuses on the state of the regulations with regard to reporting of ADRs information. In addition, the status and kinds of drug utilization review informations offered by the Korea Food and Drug Administration were are illustrated in detail.
Drug Toxicity ; Drug Utilization Review ; Jurisprudence ; Korea ; Reinforcement (Psychology) ; Safety Management ; Social Control, Formal ; United States Food and Drug Administration

Henoch-Schonlein purpura (HSP) is the most common form of systemic vasculitis in children. Palpable purpura, arthralgia, arthritis, abdominal pain and renal involvement are the major clinical manifestations. Gastrointestinal involvement is related with abdominal pain and bleeding. We described a 71 year-old female experienced acute exacerbation of HSP presented with gastrointestinal bleeding. She was hospitalized for hematemesis and diagnosed duodenitis by esophagogastroduonenoscopy (EGD). Duodenitis was improved at EGD checked in 7 days. She still complained of melena and abdominal pain. There were no abnormal findings at sigmoidoscopy. Jejunal ulcer and purpura were diagnosed by capsule endoscopy. Symptoms were relieved after administration of systemic steroid. But she needed renal replacement therapy for 3 months. Small bowel ulcer diagnosed by capsule endoscopy in patients with HSP was rarely described in Korean literature. This case suggests that capsule endoscopy have a role in diagnosis of small bowel ulcer and its severity in HSP with gastrointestinal symptom.
Abdominal Pain ; Arthralgia ; Arthritis ; Capsule Endoscopy ; Child ; Duodenitis ; Female ; Gastrointestinal Hemorrhage ; Hematemesis ; Hemorrhage ; Humans ; Melena ; Purpura ; Purpura, Schoenlein-Henoch ; Renal Replacement Therapy ; Sigmoidoscopy ; Systemic Vasculitis ; Ulcer

Zinner's syndrome is a rare congenital abnormality of the mesonephric duct. Unilateral renal agenesis, ipsilateral seminal vesicle cyst, and ipsilateral ejaculatory duct obstruction are the triad of maldevelopment of the mesonephric duct which comprises Zinner's syndrome. It is an extremely rare case, in that approximately 100 cases only have been reported worldwide. We discovered a rare developmental anomaly with other mesonephric duct-associated abnormalities, Zinner's syndrome with a presumed ectopic prostate and triorchidism and do report here.

Humoral hypercalcemia of malignancy (HHM) is rarely associated with cholangiocarcinoma (CC), and represents dismal prognosis. A 63-year-old male was admitted for evaluation of an intrahepatic mass. He was diagnosed with HHM associated with locally advanced CC. As the tumor responded to the concurrent chemoradiotherapy with capecitabine and cisplatin, serum calcium level was normalized. However, according to the disease progression, he suffered recurrence of HHM and he expired approximately one year after initial diagnosis. A 68-year-old male who presented with abdominal pain was diagnosed with metastatic CC. After the eighth cycle of gemcitabine and cisplatin, progression of the disease was found with HHM. He was treated with the best supportive care, until his demise approximately one month after the diagnosis of HHM. We report on two cases of HHM associated with CC that demonstrate strong correlation between hypercalcemia and disease burden.
Abdominal Pain ; Calcium ; Chemoradiotherapy ; Cholangiocarcinoma ; Cisplatin ; Deoxycytidine ; Disease Progression ; Fluorouracil ; Humans ; Hypercalcemia ; Male ; Paraneoplastic Syndromes ; Parathyroid Hormone-Related Protein ; Prognosis ; Recurrence ; Capecitabine

Recent evidence supports a neuroprotective role of Src homology 2-containing protein tyrosine phosphatase 2 (SHP-2) against ischemic brain injury. However, the molecular mechanisms of SHP-2 activation and those governing how SHP-2 exerts its function under oxidative stress conditions are not well understood. Recently we have reported that reactive oxygen species (ROS)-mediated oxidative stress promotes the phosphorylation of endogenous SHP-2 through lipid rafts, and that this phosphorylation strongly occurs in astrocytes, but not in microglia. To investigate the molecules involved in events leading to phosphorylation of SHP-2, raft proteins were analyzed using astrocytes and microglia. Interestingly, caveolin-1 and -2 were detected only in astrocytes but not in microglia, whereas flotillin-1 was expressed in both cell types. To examine whether the H2O2-dependent phosphorylation of SHP-2 is mediated by caveolin-1, we used specific small interfering RNA (siRNA) to downregulate caveolin-1 expression. In the presence of caveolin-1 siRNA, the level of SHP-2 phosphorylation induced by H2O2 was significantly decreased, compared with in the presence of control siRNA. Overexpression of caveolin-1 effectively increased H2O2-induced SHP-2 phosphorylation in microglia. Lastly, H2O2 induced extracellular signal-regulated kinase (ERK) activation in astrocytes through caveolin-1. Our results suggest that caveolin-1 is involved in astrocyte-specific intracellular responses linked to the SHP-2-mediated signaling cascade following ROS-induced oxidative stress.
Animals ; Astrocytes/*metabolism ; Caveolin 1/*genetics/metabolism ; Caveolin 2/genetics ; Cell Line ; Cells, Cultured ; Extracellular Signal-Regulated MAP Kinases/metabolism ; Gene Expression ; Humans ; Microglia/metabolism ; Phosphoric Monoester Hydrolases/*metabolism ; Phosphorylation ; Protein Tyrosine Phosphatase, Non-Receptor Type 11/*metabolism ; Rats ; Reactive Oxygen Species/*metabolism

Neuropsychiatric events are common in patients with systemic lupus erythematosus (SLE). The estimated incidence of neuropsychiatric SLE (NPSLE) is 30 to 40%. However, NPSLE poses a difficult diagnostic challenge because a variety of conditions should be considered in the differential diagnosis, especially when patients present with uncommon or rare NPSLE features. We herein describe a 49-year-old man with SLE who initially presented with diplopia, ptosis, and gait disturbance that had developed 1 week after an upper respiratory tract infection. He was finally diagnosed with Miller Fisher syndrome (a variant of Guillain-Barre syndrome) according to clinical symptoms, anti-GQ1b antibody positivity, and neurological study results. The patient recovered without sequelae with intravenous immunoglobulin therapy. This is the first report to describe a case of Miller Fisher syndrome that developed in a patient with SLE in Korea and suggests that Miller Fisher syndrome should be included as a differential diagnosis of NPSLE.
Diagnosis, Differential ; Diplopia ; Gait ; Humans ; Immunization, Passive ; Incidence ; Korea ; Lupus Erythematosus, Systemic ; Middle Aged ; Miller Fisher Syndrome ; Respiratory Tract Infections

Epilepsy of infancy with migrating focal seizure (MFEI) is an early-onset epileptic encephalopathy characterized by randomly migrating focal seizures and psychomotor deterioration. It is associated with mutations in a variety of genes, with potassium sodium-activated channel subfamily T member 1 (KCNT1) being an example. Previously reported KCNT1 mutations in MFEI are gain-of-function mutations. Therefore, quinidine therapy targeted at reduction of pathologically increased KCNT1 channel-mediated potassium conductance has been proposed as a target treatment for MEFI with KCNT1 mutation. The authors report a case involving a patient with MFEI and a missense mutation in KCNT1 (c.7129G>A; p.Phe346Leu) treated with quinidine therapy. Seizure activity was poorly responsive to quinidine.
Brain Diseases ; Epilepsy* ; Humans ; Mutation, Missense ; Potassium ; Quinidine* ; Seizures*

OBJECTIVES: To identify the factors that are most significantly associated with the decline of Activities of Daily Living (ADL) status in subjects with Mild Cognitive Impairment (MCI) and early dementia of primary degenerative cause. METHODS: Medical records, results of neuropsychological tests, and Activities of Daily Living scale were retrospectively analyzed in 134 subjects meeting the diagnostic criteria of MCI or early dementia, respectively. Demographic factors, memory functions, depressive symptoms and executive functions of the participants were also analyzed. RESULTS: Correlations revealed that measures of executive functions were significantly associated with ADL status in participants with MCI, while both memory and executive functions were significantly associated in early demented elderly. Stroop test which measures the ability of inhibition showed the closest relation to executive function. Depressive symptoms did not have a significant influence on ADL status. CONCLUSION: Among participants with MCI, the early functional difficulties are associated mainly with executive function, which enables inhibition in particular. In participants with early dementia, on the contrary, the ADL status was associated with both executive function and memory. Further studies on the symptom pattern and related neuropathology in process of dementia progression are needed for Quality of Life (QoL) in elderly population.
Activities of Daily Living ; Aged ; Dementia ; Demography ; Depression ; Executive Function ; Humans ; Medical Records ; Memory ; Mild Cognitive Impairment ; Neuropsychological Tests ; Quality of Life ; Retrospective Studies ; Stroop Test

OBJECTIVES: This study aimed to evaluate the association between presence of depression symptoms and the exposure level to insecticides among aged population in rural area, determined via measured levels of urinary 3-phenoxybenzoic acid (3-PBA), after controlling for socioeconomic confounding factors. METHODS: Using a cross-sectional study design, we randomly recruited participants for our study (161 male and 239 female) from rural areas of Asan, Chungnam, Korea. Environmental risk factor exposure was assessed using a questionnaire, and gas chromatography- mass spectrometry was used to analyze urinary 3-PBA levels. We used a logistic regression analysis to assess the association of urinary 3-PBA levels with the presence of self-reported depression symptoms. RESULTS: After controlling for creatinine levels, the median (interquartile range) concentration of 3-PBA was approximately 1.5 times (p<0.05) higher among female (1.54 [0.90 to 2.35]) mug/g) than among male (1.06 [0.64 to 1.81] mug/g). Our study found that among female participants, the unit increase in 3-PBA levels exhibited a likely positive association (odds ratio, 1.12; 95% confidence interval, 1.00 to 1.25) with an increased risk of presence of self-reported depression symptoms, after adjusting for socioeconomic insurance type, daily physical condition, marital status, smoking status, and age. CONCLUSIONS: Given our finding of a potential association between the presence of selfreported depression symptoms and 3-PBA levels, precautions should be considered to minimize exposure to insecticides and thus protect the health of aged residents in rural areas.
Aged* ; Chungcheongnam-do* ; Creatinine ; Cross-Sectional Studies ; Depression* ; Female ; Humans ; Insecticides ; Insurance ; Korea* ; Logistic Models ; Male ; Marital Status ; Mass Spectrometry ; Risk Factors ; Smoke ; Smoking

OBJECTIVES: This study aimed to evaluate the association between presence of depression symptoms and the exposure level to insecticides among aged population in rural area, determined via measured levels of urinary 3-phenoxybenzoic acid (3-PBA), after controlling for socioeconomic confounding factors. METHODS: Using a cross-sectional study design, we randomly recruited participants for our study (161 male and 239 female) from rural areas of Asan, Chungnam, Korea. Environmental risk factor exposure was assessed using a questionnaire, and gas chromatography- mass spectrometry was used to analyze urinary 3-PBA levels. We used a logistic regression analysis to assess the association of urinary 3-PBA levels with the presence of self-reported depression symptoms. RESULTS: After controlling for creatinine levels, the median (interquartile range) concentration of 3-PBA was approximately 1.5 times (p<0.05) higher among female (1.54 [0.90 to 2.35]) mug/g) than among male (1.06 [0.64 to 1.81] mug/g). Our study found that among female participants, the unit increase in 3-PBA levels exhibited a likely positive association (odds ratio, 1.12; 95% confidence interval, 1.00 to 1.25) with an increased risk of presence of self-reported depression symptoms, after adjusting for socioeconomic insurance type, daily physical condition, marital status, smoking status, and age. CONCLUSIONS: Given our finding of a potential association between the presence of selfreported depression symptoms and 3-PBA levels, precautions should be considered to minimize exposure to insecticides and thus protect the health of aged residents in rural areas.
Aged* ; Chungcheongnam-do* ; Creatinine ; Cross-Sectional Studies ; Depression* ; Female ; Humans ; Insecticides ; Insurance ; Korea* ; Logistic Models ; Male ; Marital Status ; Mass Spectrometry ; Risk Factors ; Smoke ; Smoking