Purpose: Acute promyelocytic leukemia (APL) is a rare disease in children and there are some different characteristics between children and adult. We aimed to evaluate incidence, clinical characteristics and treatment outcomes of pediatric APL in Korea. Materials and Methods: Seventy-nine pediatric APL patients diagnosed from January 2009 to December 2016 in 16 tertiary medical centers in Korea were reviewed retrospectively. Results: Of 801 acute myeloid leukemia children, 79 (9.9%) were diagnosed with APL. The median age at diagnosis was 10.6 years (range, 1.3 to 18.0). Male and female ratio was 1:0.93. Thirty patients (38.0%) had white blood cell (WBC) count greater than 10×109/L at diagnosis. All patients received induction therapy consisting of all-trans retinoic acid and chemotherapy. Five patients (6.6%) died during induction chemotherapy and 66 patients (86.8%) achieved complete remission (CR) after induction chemotherapy. The causes of death were three intracranial hemorrhage, one cerebral infarction, and one sepsis. Five patients (7.1%) suffered a relapse during or after maintenance chemotherapy. The estimated 4-year event-free survival and overall survival (OS) rates were 82.1%±4.4%, 89.7%±5.1%, respectively. The 4-year OS was significantly higher in patients with initial WBC < 10×109/L than in those with initial WBC ≥ 10×109/L (p=0.020). Conclusion This study showed that the CR rates and survival outcomes in Korean pediatric APL patients were relatively good. The initial WBC count was the most important prognostic factor and most causes of death were related to serious bleeding in the early stage of treatment.

No abstract available.
Endocarditis*

Staphylococcus lugdunensis is a coagulase-negative staphylococcus. However, it causes various clinically important human infections and behaves similar to Staphylococcus aureus. S. lugdunensis reportedly causes infective endocarditis, skin and soft tissue infection, bone and joint infection, septicemia, endarteritis, urinary tract infection, ocular infection, and peritonitis. There are no reports of septic arthritis by this organism in Korea. We presented a case of septic arthritis due to S. lugdunensis in an elderly patient with diabetes mellitus after an intra-articular injection.
Aged ; Arthritis* ; Arthritis, Infectious ; Diabetes Mellitus ; Endarteritis ; Endocarditis ; Eye Infections ; Humans ; Injections, Intra-Articular* ; Joints ; Knee Joint ; Knee Prosthesis ; Knee* ; Korea ; Peritonitis ; Sepsis ; Skin ; Soft Tissue Infections ; Staphylococcus ; Staphylococcus aureus ; Staphylococcus lugdunensis* ; Urinary Tract Infections

BACKGROUND: Because of the heterogeneity of human mesenchymal stem cells (MSCs), methods for cell expansion in culture and the effects on gene expression are critical factors that need to be standardized for preparing MSCs. We investigated gene expression patterns of MSCs with different seeding densities and culture times. METHODS: Bone marrow-derived MSCs were plated at densities from 200 cells/cm2 to 5,000 cells/cm2, and the gene expression patterns were evaluated over time using a reverse-transcription polymerase chain reaction assay. RESULTS: The mRNA levels of factors that play a critical role in cell migration and tissue regeneration, such as podocalyxin-like protein (PODXL), alpha4-integrin, alpha6-integrin, and leukemia inhibitory factor (LIF), were higher in MSCs plated at 200 cells/cm2 than in MSCs plated at 5,000 cells/cm2. The mRNA levels of these factors gradually increased for 10 days and then decreased by day 15 in culture. MSCs seeded at 200 cells/cm2 that were cultured for 10 days expressed high levels of Oct-4 and Nanog. Indoleamine 2,3-dioxygenase, cyclooxygenase-1, and hepatocyte growth factor expression were upregulated in the presence of the proinflammatory cytokine interferon-gamma in these cells. CONCLUSION: We found differences in the gene expression patterns of MSCs under different culture conditions. MSCs from 10-day cultures seeded at a low density were efficiently expanded, expressed PODXL, alpha6-integrin, alpha4-integrin, and LIF, and maintained properties like stemness and immunomodulation. Therefore, ex vivo expansion of MSCs maintained for an adequate culture time after plating at low cell density can provide an effective regenerative medicinal strategy for cell therapies using MSCs.
Cell Count ; Cell Movement ; Cyclooxygenase 1 ; Gene Expression ; Hepatocyte Growth Factor ; Humans ; Immunomodulation ; Indoleamine-Pyrrole 2,3,-Dioxygenase ; Interferon-gamma ; Leukemia Inhibitory Factor ; Mesenchymal Stromal Cells ; Polymerase Chain Reaction ; Population Characteristics ; Regeneration ; RNA, Messenger ; Seeds ; Tissue Therapy

Extracranial carotid stenosis is a well-established, modifiable risk factor for stroke. Asymptomatic extracranial carotid stenosis is increasingly being detected due to the introduction of less-invasive and more-sensitive advanced diagnostic technologies. For severe asymptomatic stenosis, earlier pivotal clinical trials demonstrated the benefit of carotid endarterectomy over the best medical therapy. Since then, great advances have been made in interventional and medical therapies as well as surgical techniques. The first edition of the Korean Stroke Clinical Practice Guidelines for primary stroke prevention for the management of asymptomatic carotid stenosis reflected evidences published before June 2007. After the publication of the first edition, several major clinical trials and observational studies have been published, and major guidelines updated their recommendation. Accordingly, the writing group of Korean Stroke Clinical Practice Guidelines (CPG) decided to provide timely updated evidence-based recommendations. The Korean Stroke CPG writing committee has searched and reviewed literatures related to the management of asymptomatic carotid stenosis including published guidelines, meta-analyses, randomized clinical trials, and nonrandomized studies published between June 2007 and Feb 2011. We summarized the new evidences and revised our recommendations. Key changes in the updated guidelines are the benefit of intensive medical therapy and further evidence of carotid artery stenting as an alternative to carotid endarterectomy. The current updated guidelines underwent extensive peer review by experts from the Korean Stroke Society, Korean Society of Intravascular Neurosurgery, Korean Society of Interventional Neuroradiology, Korean Society of Cerebrovascular Surgery, and Korean Neurological Association. New evidences will be continuously reflected in future updated guidelines.
Carotid Arteries ; Carotid Stenosis ; Constriction, Pathologic ; Endarterectomy, Carotid ; Neurosurgery ; Peer Review ; Primary Prevention ; Publications ; Risk Factors ; Stents ; Stroke ; Writing

BACKGROUND: Urine/serum protein electrophoresis (PEP) and immunofixation electrophoresis (IEP) for monoclonal protein (M-protein) are used for initial evaluation in patients with multiple myeloma. We evaluated the prognostic significance of M-proteinuria status and its association with other prognostic factors. METHODS: Between December 2002 and December 2004, 64 de novo symptomatic multiple myeloma patients with intact immunoglobulin (Ig) type were divided into two groups according to their initial urine PEP/IEP findings. RESULTS: Twenty-seven patients with undetectable or free light-chains only were classified into F group, and 37 with whole Ig with or without light-chains were classified into W group. The two groups were similar in sex, age, performance, azotemia, beta2-microglobulin, stage and treatment, but M-protein concentration was significantly higher in the W than in F group (5.1 vs 1.3g/dL, P<0.01). The overall response rate was significantly higher in F group than in W group (80.8% vs 63.6%, P=0.02), whereas the 2-year OS rate did not differ significantly between the groups (81.0% vs 57.7%, P=0.15). CONCLUSION: Monoclonal proteinuria is helpful in identifying patients with advanced disease and poorer prognosis in multiple myeloma.
Azotemia ; Electrophoresis ; Humans ; Immunoglobulins* ; Multiple Myeloma* ; Prognosis ; Proteinuria*

BACKGROUND: Although several hospital-based stroke studies were published, there has not been any reliable data representing the clinical characteristics of stroke in Korea. We analyzed the clinical characteristics of patients with ischemic stroke registered in the Korean Stroke Registry (KSR), which is the largest prospective hospital-based nation-wide stroke registry in Korea. METHODS: The KSR provided standardized protocols for collecting data, which includes the data of demographics, subtypes of stroke, risk factors, and neurological outcome at discharge. The brain imaging studies, including CT or MRI, were performed in all cases. RESULTS: KSR registered 10,811 patients of acute ischemic stroke between Nov. 2002 and Jun. 2004. The large-artery atherosclerosis was the most common subtype (37.3%), followed by small vessel occlusion (30.8%). Hypertension (65.4%) was the most common risk factor, followed by smoking (34.5%) and diabetes (28.3%). Although most of the hypertensive and diabetic patients had been diagnosed before the stroke, less than 45.4% and 32.5% of them were under regular control. The steno-occlusive lesion of extracranial carotid artery was only 29.3% and the ratio of intra- to extracranial artery disease was more than 2 in KSR. Only 20.5% of patients were admitted within 3 hours after stroke onset and 2.1% were treated with intravenous thrombolysis. In-hospital case-fatality was 5.2%, which is relatively comparable to those of previous studies. CONCLUSIONS: The KSR provided informative data in understanding the clinical characteristics of ischemic stroke in Korea. Further analysis of KSR will facilitate clinical trials and development of guidelines for the management of stroke patients.
Arteries ; Atherosclerosis ; Carotid Arteries ; Cerebrovascular Disorders ; Demography ; Epidemiology ; Humans ; Hypertension ; Korea ; Magnetic Resonance Imaging ; Neuroimaging ; Prospective Studies* ; Registries ; Risk Factors ; Smoke ; Smoking ; Stroke*

A 73-year-old man was admitted for intermittent episodes of fever and chills for 3 months. He had been implanted with a permanent pacemaker to control tachy-bradycardia syndrome 7 months before admission. Blood cultures were positive for Actinobacillus actinomycetemcomitans and a 99mTc-hexamethylpropylene amine oxime (99mTc-HMPAO) WBC scan revealed inflammation on the pacemaker lead in extracardiac site. Oral examination revealed several dental caries. The patient was treated with intravenous ceftriaxone, followed by oral ciprofloxacin without removal of the infected pacemaker lead. He was doing well 10 months without febrile episodes after discontinuation of antibiotics. This report describes the first case of A. actinomycetemcomitans bacteremia associated with a pacemaker lead and localized by 99mTc-HMPAO WBC scan
Actinobacillus* ; Aged ; Aggregatibacter actinomycetemcomitans* ; Anti-Bacterial Agents ; Bacteremia* ; Ceftriaxone ; Chills ; Ciprofloxacin ; Dental Caries ; Diagnosis, Oral ; Fever ; Humans ; Inflammation ; Technetium Tc 99m Exametazime

Staphylococcus lugdunensis is a coagulase-negative staphylococcus (CNS) that is commonly found on the human perineal skin. Contrary to other CNS, S. lugdunensis is a rare contaminant in cultures and has the potential to cause clinically significant infections, including infective endocarditis. Infective endocarditis due to S. lugdunensis have been known to resemble endocarditis due to S. aureus because of its aggressive clinical course and high mortality. We report a case of infective endocarditis caused by S. lugdunensis in a 21-year-old woman. She was cured of the infection with surgical and antibiotic therapy.
Endocarditis* ; Female ; Humans ; Mortality ; Skin ; Staphylococcus lugdunensis* ; Staphylococcus* ; Young Adult

A 73-year-old man was admitted for intermittent episodes of fever and chills for 3 months. He had been implanted with a permanent pacemaker to control tachy-bradycardia syndrome 7 months before admission. Blood cultures were positive for Actinobacillus actinomycetemcomitans and a 99mTc-hexamethylpropylene amine oxime (99mTc-HMPAO) WBC scan revealed inflammation on the pacemaker lead in extracardiac site. Oral examination revealed several dental caries. The patient was treated with intravenous ceftriaxone, followed by oral ciprofloxacin without removal of the infected pacemaker lead. He was doing well 10 months without febrile episodes after discontinuation of antibiotics. This report describes the first case of A. actinomycetemcomitans bacteremia associated with a pacemaker lead and localized by 99mTc-HMPAO WBC scan
Actinobacillus* ; Aged ; Aggregatibacter actinomycetemcomitans* ; Anti-Bacterial Agents ; Bacteremia* ; Ceftriaxone ; Chills ; Ciprofloxacin ; Dental Caries ; Diagnosis, Oral ; Fever ; Humans ; Inflammation ; Technetium Tc 99m Exametazime