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.

Background/Aims: Previous studies have reported the protective effects of tauroursodeoxycholic acid (TUDCA) on gastric epithelial cells in some animal models, but the precise mechanisms are unclear. This study examined the effects of TUDCA on NF-κB signaling in gastric epithelial cells. Moreover, the protective effects of TUDCA in experimental gastritis models induced by ethanol and NSAID were evaluated and compared with ursodeoxycholic acid (UDCA). Methods: After a pretreatment with TUDCA or UDCA, human gastric epithelial MKN-45 cells were stimulated with tumor necrosis factor (TNF)-α to activate NF-κB signaling. A real-time PCR (RT-PCR) for human interleukin (IL)-1 mRNA was performed. An electrophoretic mobility shift assay (EMSA) and immunoblot analyses were carried out. In murine models, after a pretreatment with TUDCA or UDCA, ethanol and indomethacin were administered via oral gavage. Macroscopic and microscopic assessments were performed to evaluate the preventive effects of TUDCA and UDCA on murine gastritis. Results: A pretreatment with TUDCA downregulated the IL-1α mRNA levels in MKN-45 cells stimulated with TNF-α, as assessed by RT-PCR. As determined using EMSA, a pretreatment with TUDCA reduced the TNF-α-induced NF-κB DNA binding activity. A pretreatment with TUDCA inhibited IκBα phosphorylation induced by TNF-α, as assessed by immunoblot analysis. TUDCA attenuated the ethanol-induced and NSAID-induced gastritis in murine models, as determined macroscopically and microscopically. Conclusions TUDCA inhibited NF-κB signaling in gastric epithelial cells and ameliorated ethanol- and NSAID-induced gastritis in murine models. These results support the potential of TUDCA for the prevention of gastritis in humans.

Background: Hodgkin's lymphoma (HL) constitutes 10%–20% of all malignant lymphomas and has a high cure rate (5-year survival, around 90%). Recently, interest has increased concerning preventing secondary complications (secondary cancer, endocrine disorders) in long-term survivors. We aimed to study the epidemiologic features and therapeutic outcomes of HL in children, adolescents, and young adults in Korea. Methods: We performed a multicenter, retrospective study of 224 patients aged < 25 years diagnosed with HL at 22 participating institutes in Korea from January 2007 to August 2016. Results: A higher percentage of males was diagnosed at a younger age. Nodular sclerosis histopathological HL subtype was most common, followed by mixed cellularity subtype.Eighty-one (36.2%), 101 (45.1%), and 42 (18.8%) patients were classified into low, intermediate, and high-risk groups, respectively. Doxorubicin, bleomycin, vinblastine, dacarbazine was the most common protocol (n = 102, 45.5%). Event-free survival rate was 86.0% ± 2.4%, while five-year overall survival (OS) rate was 96.1% ± 1.4%: 98.7% ± 1.3%, 97.7% ± 1.6%, and 86.5% ± 5.6% in the low, intermediate, and high-risk groups, respectively (P = 0.021). Five-year OS was worse in patients with B-symptoms, stage IV disease, highrisk, splenic involvement, extra-nodal lymphoma, and elevated lactate dehydrogenase level.In multivariate analysis, B-symptoms and extra-nodal involvement were prognostic factors for poor OS. Late complications of endocrine disorders and secondary malignancy were observed in 17 and 6 patients, respectively. Conclusion This is the first study on the epidemiology and treatment outcomes of HL in children, adolescents, and young adults in Korea. Future prospective studies are indicated to develop therapies that minimize treatment toxicity while maximizing cure rates in children, adolescents, and young adults with HL.

BACKGROUND AND OBJECTIVES: This trial evaluated the safety and efficacy of the Genoss drug-eluting coronary stent. METHODS: This study was a prospective, multicenter, randomized trial with a 1:1 ratio of Genoss drug-eluting stent (DES)™ and Promus Element™. Inclusion criteria were the presence of stable angina, unstable angina, or silent ischemia. Angiographic inclusion criteria were de novo coronary stenotic lesion with diameter stenosis >50%, reference vessel diameter of 2.5–4.0 mm, and lesion length ≤40 mm. The primary endpoint was in-stent late lumen loss at 9-month quantitative coronary angiography follow-up. Secondary endpoints were in-segment late lumen loss, binary restenosis rate, death, myocardial infarction (MI), target lesion revascularization (TLR), target vessel revascularization (TVR), and stent thrombosis during 9 months of follow-up. RESULTS: We enrolled 38 patients for the Genoss DES™ group and 39 patients for the Promus Element™ group. In-stent late lumen loss at 9 months was not significantly different between the 2 groups (0.11±0.25 vs. 0.16±0.43 mm, p=0.567). There was no MI or stent thrombosis in either group. The rates of death (2.6% vs. 0%, p=0.494), TLR (2.6% vs. 2.6%, p=1.000), and TVR (7.9% vs. 2.6%, p=0.358) at 9 months were not significantly different. CONCLUSION: This first-in-patient study of the Genoss DES™ stent showed excellent angiographic outcomes for in-stent late lumen loss and major adverse cardiac events over a 9-month follow-up.
Angina, Stable ; Angina, Unstable ; Constriction, Pathologic ; Coronary Angiography ; Coronary Artery Disease ; Drug-Eluting Stents ; Follow-Up Studies ; Humans ; Ischemia ; Mortality ; Myocardial Infarction ; Polymers* ; Prospective Studies ; Sirolimus ; Stents* ; Thrombosis

Macrophage activation syndrome (MAS) is a severe complication in patients with autoimmune disease. We should consider MAS in patients with autoimmune disease, who present with newly developed fever, and MAS needs proper management due to grave outcome. We report a case of MAS in a 15-year-old adolescent girl, who was newly diagnosed with systemic lupus erythematosus 1 month before the diagnosis of MAS. Her MAS was improved by intensive treatment, including etoposide.
Adolescent* ; Autoimmune Diseases ; Diagnosis ; Etoposide ; Female ; Fever ; Humans ; Lupus Erythematosus, Systemic* ; Macrophage Activation Syndrome*

PURPOSE: Varicella-zoster virus (VZV) infection is a common childhood disease. However, old and immune compromised patients are also at risk. Necrotizing fasciitis is a life threatening infection of the subcutaneous tissues, rapidly extending along the fascial planes. It is associated with a significant mortality rate, reported between 20% and 50%, and is therefore regarded as a surgical emergency. The authors treated a patient, who developed skin necrosis of her nose and left hemifacial area, following VZV infection. There are few literatures concerning this case; therefore, we present a rare case with review of literature. METHODS: A 39-year-old woman had shown a localized, painful, multiple bullae and eschar formation in her nose and left hemifacial area for several days. Her skin lesion had rapidly worsened in size and morphology. RESULTS: We diagnosed her as a necrotizing fasciitis, following herpes zoster, and then we performed a debridement of necrotic tissue and took a full thickness skin graft on her nose and left hemifacial area. Now, she was followed up with acceptable aesthetic result after 6 months. CONCLUSION: Secondary bacterial skin infection following VZV, can cause a result in a higher risk of complications. Among the complication, a necrotizing fasciitis of the head and neck is uncommon, and involvement of the nose is even more rare. Through this uncommon case report, we intend to emphasize the fact that early diagnosis of necrotizing fasciitis is very important, since it frequently necessitates surgical treatment which improves morbidity and leads to good recovery.
Adult ; Blister ; Debridement ; Early Diagnosis ; Emergencies ; Fasciitis, Necrotizing ; Female ; Head ; Herpes Zoster ; Herpesvirus 3, Human ; Humans ; Neck ; Necrosis ; Nose ; Skin ; Subcutaneous Tissue ; Transplants

BACKGROUND: Fingertip injuries involving subtotal or total loss of the digital pulp are common types of hand injuries and require reconstruction that is able to provide stable padding and sensory recovery. There are various techniques used for reconstruction of fingertip injuries, but the most effective method is functionally and aesthetically controversial. Despite some disadvantages, cross-finger pulp flap is a relatively simple procedure without significant complications or requiring special techniques. METHODS: This study included 90 patients with fingertip defects who underwent cross-finger pulp flap between September 1998 and March 2010. In 69 cases, neurorrhaphy was performed between the pulp branch from the proper digital nerve and the recipient's sensory nerve for good sensibility of the injured fingertip. In order to evaluate the outcome of our surgical method, we observed two-point discrimination in the early (3 months) and late (12 to 40 months) postoperative periods. RESULTS: Most of the cases had cosmetically and functionally acceptable outcomes. The average defect size was 1.7x1.5 cm. Sensory return began 3 months after flap application. The two-point discrimination was measured at 4.6 mm (range, 3 to 6 mm) in our method and 7.2 mm (range, 4 to 9 mm) in non-innervated cross-finger pulp flaps. CONCLUSIONS: The innervated cross-finger pulp flap is a safe and reliable procedure for lateral oblique, volar oblique, and transverse fingertip amputations. Our procedure is simple to perform under local anesthesia, and is able to provide both mechanical stability and sensory recovery. We recommend this method for reconstruction of fingertip injuries.
Amputation ; Anesthesia, Local ; Discrimination (Psychology) ; Finger Injuries ; Hand Injuries ; Humans ; Microsurgery ; Nerve Transfer ; Subcutaneous Tissue

Software for GafChromic EBT2 film dosimetry was developed in this study. The software provides film calibration functions based on color channels, which are categorized depending on the colors red, green, blue, and gray. Evaluations of the correction effects for light scattering of a flat-bed scanner and thickness differences of the active layer are available. Dosimetric results from EBT2 films can be compared with those from the treatment planning system ECLIPSE or the two-dimensional ionization chamber array MatriXX. Dose verification using EBT2 films is implemented by carrying out the following procedures: file import, noise filtering, background correction and active layer correction, dose calculation, and evaluation. The relative and absolute background corrections are selectively applied. The calibration results and fitting equation for the sensitometric curve are exported to files. After two different types of dose matrixes are aligned through the interpolation of spatial pixel spacing, interactive translation, and rotation, profiles and isodose curves are compared. In addition, the gamma index and gamma histogram are analyzed according to the determined criteria of distance-to-agreement and dose difference. The performance evaluations were achieved by dose verification in the 60o-enhanced dynamic wedged field and intensity-modulated (IM) beams for prostate cancer. All pass ratios for the two types of tests showed more than 99% in the evaluation, and a gamma histogram with 3 mm and 3% criteria was used. The software was developed for use in routine periodic quality assurance and complex IM beam verification. It can also be used as a dedicated radiochromic film software tool for analyzing dose distribution.
Calibration ; Film Dosimetry ; Lifting ; Light ; Noise ; Prostatic Neoplasms ; Software

PURPOSE: The early diagnosis and detection of prosthetic infection after TKA is very important and difficult for deter- mination of the plan in management and reduction of complications. In these reasons, this study was performed to evaluate the usefulness and limitation of (99m)Tc-HMPAO(Technetium (99m)-hexamethyl- propylene amine oxime)-labeled WBC scan in the patients with clinically suspicious prosthetic infection. MATERIALS AND METHODS: The study subjects were 25 patients(3 men and 19 women, mean age: 66.5 years) performed (99m)Tc-HMPAO WBC scan in the patients with clinically suspicious prosthesis infection after TKA from January, 2005 to May, 2007. And in 6 patients who had undergone bilateral arthroplasty, we regarded one patient as two cases. Thus, total 31 prostheses were included in this study. Final diagnosis of infection was based on bacteriological result by intrao- perative cultures, surgical findings, and histological evidence intraoperatively obtained in the suspicious site and clinical follow-up. RESULTS: In the final diagnosis, we confirmed that total 16 prosthetic joints were infected. Of these infected prostheses, increased (99m)Tc-HMPAO WBC scan uptake waspositive in 15, negative in 1. Finally, 15 cases were confirmed as nonin- fected prosthesis, 6 showed positive by (99m)Tc-HMPAO WBC scan uptake, 9 showed negative. Over all sensitivity, speci- ficity, and positive predictive value for diagnosisof infected TKA were 93.75, 60%, and 71.43%. CONCLUSION: (99m)Tc-HMPAO WBC scan was a highly sensitive method for the diagnosis of prosthetic infection after TKA. But low specificity and high false positive of (99m)Tc-HMPAO WBC scan demand more clinical follow-up for confirmed diagnosis. To increase specificity, additionally scan will be needed.
Arthroplasty* ; Diagnosis* ; Early Diagnosis ; Female ; Follow-Up Studies ; Humans ; Joints ; Knee* ; Male ; Prostheses and Implants ; Sensitivity and Specificity