• Journal of Genetic Medicine
• /
• v.13 no.2
• /
• pp.72-77
• /
• 2016

Purpose: Gaucher disease (GD) is the most common lysosomal storage disease caused by beta-glucocerebrosidase (GBA) deficiency. Oral substrate reduction therapy with miglustat ($Zavesca^{(R)}$) was approved for the treatment of adults with GD type 1, for whom enzyme replacement therapy (ERT) is unsuitable or not a therapeutic option. In this study, we report the effect of miglustat ($Zavesca^{(R)}$) in three Korean GD patients. Materials and Methods: Clinical findings comprising age at diagnosis, presenting signs, laboratory findings at diagnosis, GBA activity and mutations, and clinical courses of the three patients were reviewed. Results: Miglustat was administered to three patients who reported allergic reactions during intravenous imiglucerase infusions. One patient withdrew after 15 months of miglustat administration owing to continuous elevation of disease biomarker levels (chitotriosidase, acid phosphatase, and angiotensin-converting enzyme). Poor adherence to medication was suspected but was denied by the patient. In the other two patients, platelet count and levels of hemoglobin and other biomarkers remained stable during miglustat administration. However, they suffered from severe diarrhea and weight loss, which led to miglustat discontinuation after 1 and 12 months of administration. Conclusion: Our study shows that although miglustat is suggested to GD patients as an alternative treatment to ERT, significant adverse reactions may lead to discontinuation of miglustat. In addition, it is difficult to monitor the drug adherence.

• Journal of Yeungnam Medical Science
• /
• v.37 no.4
• /
• pp.329-331
• /
• 2020

Negative myoclonus (NM) is a shock-like jerky involuntary movement caused by a sudden, brief interruption of tonic muscle contraction. NM is observed in patients diagnosed with epilepsy, metabolic encephalopathy, and drug toxicity and in patients with brain lesions. A 55-year-old man presented with NM in both his arms and neck. He has taken medications containing tramadol at a dose of 80-140 mg/day for 5 days due to common cold. He had no history of seizures. Acute lesions were not observed during magnetic resonance imaging, and abnormal findings in his laboratory tests were not noted. His NM resolved completely after the discontinuation of tramadol and the oral administration of clonazepam. Our case report suggests that tramadol can cause NM in patients without seizure history or metabolic disorders, even within its therapeutic dose.

• Korean Journal of Biological Psychiatry
• /
• v.14 no.1
• /
• pp.68-71
• /
• 2007

Quetiapine is an atypical antipsychotic drug with a benign side effect profile. However, recent studies have reported that thyroid dysfunction is associated with quetiapine treatment. The authors report a patient with DSM-IV bipolar I disorder who developed subclinical hypothyroidism during quetiapine treatment. The patient showed no significant clinical symptoms, but only abnormal thyroid function test findings including antithyroglobulin antibody. The abnormal thyroid function test findings were normalized after discontinuation of quetiapine. The subclinical hypothyroidism developed during quetiapine treatment may be associated with autoimmune process.

• Anxiety and mood
• /
• v.1 no.1
• /
• pp.31-36
• /
• 2005

Benzodiazepine (BDZ) has the possibilities of development of tolerance, withdrawal symptoms, and abuse/addiction, as well as chronically adverse effects. Although many guidelines have proposed the restricted prescription of them, their uses in many psychiatric areas as well as primary practice are still wide spread. So we tried to reappraise the clinical characteristics of BDZ and then to consider the appropriate use. Firstly, meta-analyses on long-term use of BDZ indicated the cognitive impairment, which could be improved after discontinuation of BDZ. Next, there have been some evidences that the long-term use of BDZ does not develop tolerance, contrary to our concern, and maintains good anxiolytic effects. Also, physiological dependence should be discriminated from abuse/addiction, assuming the reality that the risk of BDZ abuse/addiction is surely overestimated. These issues are discussed in detail.

• Pediatric Gastroenterology, Hepatology & Nutrition
• /
• v.22 no.4
• /
• pp.387-391
• /
• 2019

Tea is the most widely consumed beverage after water in the world. The consumption of iced tea has increased in Western countries and spiked among teenagers for enjoyment, freshening up and alertness. A teenager presented with symptoms of hepatitis. Liver ultrasound revealed sludge in the gallbladder. Laboratory investigations excluded all known causes of hepatotoxicity. Detail nutritional history revealed that the patient had been drinking 1.5-2 liters of black iced tea per day for the last three months. He was immediately advised to stop drinking any tea. Gradually all symptoms disappeared and two months after discontinuation of the tea, all liver enzymes returned to normal and the sludge in the gallbladder disappeared. This case report underlines the importance of a meticulous assessment of a child's dietary behavior when investigating a case of hepatotoxicity and raises awareness about the potential side effects of tea overconsumption.

• Journal of Yeungnam Medical Science
• /
• v.36 no.2
• /
• pp.155-158
• /
• 2019

Imatinib mesylate is currently used as the first-line treatment for metastatic gastrointestinal stromal tumors (GISTs). Imatinib-induced hepatotoxicity in patients with GIST is very rare. Its features vary from subclinical elevation of serum aminotransferase to clinically apparent acute hepatitis, which is associated with immunologic reactions. Imatinib-induced hepatotoxicity with autoimmune-like features can be treated by the discontinuation of imatinib mesylate and the administration of oral steroids. Here, we report a case of late-onset imatinib-induced hepatitis with autoimmune-like features in a patient with metastatic GIST, which was improved by oral corticosteroids.

• Korean Journal of Clinical Pharmacy
• /
• v.25 no.4
• /
• pp.246-253
• /
• 2015

Background & Object: Ankylosing spondylitis (AS) is a chronic inflammatory disease that causes ankylosis and deformation of axial joints. Since current medicine cannot cure the disease yet, alleviating pain and preventing deformation with medications are the main therapy for patients with AS. The key medications for these purposes include nonsteroidal anti-inflammatory drugs (NSAIDs), and tumor necrosis $factor-{\alpha}$ ($TNF-{\alpha}$) inhibitors. This study aims to analyze prescribing patterns of AS patients in South Korea. Method: National Patients Sample data compiled by the Health Insurance Review and Assessment Service from 2013 was analyzed. Patients with AS were identified with Korean Standard Classification of Diseases code-6, which was M45. The rates of prescription, discontinuation, and switching ingredients were calculated for each medication during 2013. Results: Total number of patients was 655, and most of them were male (n = 514, 78.5%). Of all age groups, the proportion of 30-40 year old patients was the greatest (35.1%). The most utilized drug class was NSAIDs (82.4%). Less than half of patients were prescribed $TNF-{\alpha}$ inhibitors (n = 212, 32.4%). Meloxicam, aceclofenac, and celecoxib were the most frequently prescribed NSAIDs. In case of $TNF-{\alpha}$ inhibitors, adalimumab, etanercept and infliximab were the top three most prescribed drugs. Although not recommended by the current practice guideline, significant proportions of patients were identified using disease modifying anti-rheumatic drugs (DMARDs). Conclusion: Considering the current practice guideline and previous studies about the efficacy, the use of DMARDs should be reduced and medical insurance term in South Korea should be re-examined.

• Journal of Yeungnam Medical Science
• /
• v.33 no.1
• /
• pp.59-63
• /
• 2016

Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome is a rare and severe drug-induced hypersensitivity syndrome characterized by hematological abnormalities and multiorgan involvement. Liver involvement is the most common visceral manifestation. However, renal failure has been rarely described. The common culprit drugs are anticonvulsants and allopurinol. We experienced a patient with DRESS syndrome with acute interstitial nephritis caused by concomitant administration of quinolone and non-steroidal anti-inflammatory drugs (NSAIDs). A 41-year-old man presented with a diffuse erythematous rash and fever which developed after administration of quinolone and NSAIDs for a month due to prostatitis. He was diagnosed with DRESS syndrome. Skin rash, fever, eosinophilia, and elevations of liver enzymes improved with conservative treatment and discontinuation of the causative drugs. However, deterioration of his renal function occurred on day 8 of admission. The levels of blood urea nitrogen and serum creatinine increased and oliguria, proteinuria and urinary eosinophils were observed. Ultrasonography showed diffuse renal enlargement. The clinical features were compatible with acute interstitial nephritis. Despite intravenous rehydration and diuretics, renal function did not improve. After hemodialysis, his renal function recovered completely within 2 weeks without administration of systemic corticosteroid.

• The Korean Journal of Pain
• /
• v.37 no.2
• /
• pp.119-131
• /
• 2024

There are growing concerns regarding the safety of long-term treatment with opioids of patients with chronic non-cancer pain. In 2017, the Korean Pain Society (KPS) developed guidelines for opioid prescriptions for chronic non-cancer pain to guide physicians to prescribe opioids effectively and safely. Since then, investigations have provided updated data regarding opioid therapy for chronic non-cancer pain and have focused on initial dosing schedules, reassessment follow-ups, recommended dosage thresholds considering the risk-benefit ratio, dose-reducing schedules for tapering and discontinuation, adverse effects, and inadvertent problems resulting from inappropriate application of the previous guidelines. Herein, we have updated the previous KPS guidelines based on a comprehensive literature review and consensus development following discussions among experts affiliated with the Committee on Hospice and Palliative Care in the KPS. These guidelines may assist physicians in prescribing opioids for chronic non-cancer pain in adult outpatient settings, but should not to be regarded as an inflexible standard. Clinical judgements by the attending physician and patient-centered decisions should always be prioritized.

• Clinical and Experimental Pediatrics
• /
• v.59 no.3
• /
• pp.145-148
• /
• 2016

Drug rash with eosinophilia and systemic symptoms (DRESS) syndrome is a rare and potentially fatal condition characterized by skin rash, fever, eosinophilia, and multiorgan involvement. Various drugs may be associated with this syndrome including carbamazepine, allopurinol, and sulfasalazine. Renal involvement in DRESS syndrome most commonly presents as acute kidney injury due to interstitial nephritis. An 11-year-old boy was referred to the Children's Hospital of Pusan National University because of persistent fever, rash, abdominal distension, generalized edema, lymphadenopathy, and eosinophilia. He previously received vancomycin and ceftriaxone for 10 days at another hospital. He developed acute kidney injury with nephrotic range proteinuria and hypocomplementemia. A subsequent renal biopsy indicated the presence of acute tubular necrosis (ATN) and late exudative phase of postinfectious glomerulonephritis (PIGN). Systemic symptoms and renal function improved with corticosteroid therapy after the discontinuation of vancomycin. Here, we describe a biopsy-proven case of severe ATN that manifested as a part of vancomycin-induced DRESS syndrome with coincident PIGN. It is important for clinicians to be aware of this syndrome due to its severity and potentially fatal nature.