Background: Nilotinib is a tyrosine kinase inhibitor approved by the Ministry of Food and Drug Safety for frontline and 2nd line treatment of Philadelphia chromosome-positive chronic myeloid leukemia (Ph+ CML). This study aimed to confirm the safety and efficacy of nilotinib in routine clinical practice within South Korea. Methods: An open-label, multicenter, single-arm, 12-week observational post-marketing surveillance (PMS) study was conducted on 669 Korean adult patients with Ph + CML from December 24, 2010, to December 23, 2016. The patients received nilotinib treatment in routine clinical practice settings. Safety was evaluated by all types of adverse events (AEs) during the study period, and efficacy was evaluated by the complete hematological response (CHR) and cytogenetic response. Results: During the study period, AEs occurred in 61.3% (410 patients, 973 events), adverse drug reactions (ADRs) in 40.5% (271/669 patients, 559 events), serious AEs in 4.5% (30 patients, 37 events), and serious ADRs in 0.7% (5 patients, 8 events). Furthermore, unexpected AEs occurred at a rate of 6.9% (46 patients, 55 events) and unexpected ADRs at 1.2% (8 patients, 8 events). As for the efficacy results, CHR was achieved in 89.5% (442/494 patients), and minor cytogenetic response or major cytogenetic response was achieved in 85.8% (139/162 patients). Conclusion This PMS study shows consistent results in terms of safety and efficacy compared with previous studies. Nilotinib was well tolerated and efficacious in adult Korean patients with Ph + CML in routine clinical practice settings.

Background/Aims: We evaluated the feasibility and long-term efficacy of the combination of cytarabine, idarubicin, and all-trans retinoic acid (ATRA) for treating patients with newly diagnosed acute promyelocytic leukemia (APL). Methods: We included 87 patients with newly diagnosed acute myeloid leukemia and a t(15;17) or promyelocytic leukemia/retinoic acid receptor alpha (PML-RARα) mutation. Patients received 12 mg/m2/day idarubicin intravenously for 3 days and 100 mg/m2/day cytarabine for 7 days, plus 45 mg/m2/day ATRA. Clinical outcomes included complete remission (CR), relapse-free survival (RFS), overall survival (OS), and the secondary malignancy incidence during a 20-year follow-up. Results: The CR, 10-year RFS, and 10-year OS rates were 89.7%, 94.1%, and 73.8%, respectively, for all patients. The 10-year OS rate was 100% for patients that achieved CR. Subjects were classified according to the white blood cell (WBC) count in peripheral blood at diagnosis (low-risk, WBC < 10,000/mm3; high-risk, WBC ≥ 10,000/mm3). The low-risk group had significantly higher RFS and OS rates than the high-risk group, but the outcomes were not superior to the current standard treatment (arsenic trioxide plus ATRA). Toxicities were similar to those observed with anthracycline plus ATRA, and higher than those observed with arsenic trioxide plus ATRA. The secondary malignancy incidence after APL treatment was 2.7%, among the 75 patients that achieved CR, and 5.0% among the 40 patients that survived more than 5 years after the APL diagnosis. Conclusions Adding cytarabine to anthracycline plus ATRA was not inferior to anthracycline plus ATRA alone, but it was not comparable to arsenic trioxide plus ATRA. The probability of secondary malignancy was low.

Deep infection of Achilles tendon is one of the serious complications that occur after open repair of the tendon. It sometimes leads to a very large tendon defect during the course of treatment. We report on a case of massive defect in Achilles tendon, which was successfully treated with Achilles tendon allograft and flexor hallucis longus tendon transfer.
Achilles Tendon* ; Allografts* ; Tendon Transfer ; Tendons*

BACKGROUND: Low levels of physical activity (PA) are strongly associated with the development of metabolic syndrome (MetS) and chronic diseases. However, few studies have examined this association in Koreans. The primary purpose of this study was to examine the associations between PA and MetS risks in Korean adults. METHODS: A total of 1,016 Korean adults (494 males and 522 females) participated in this study. PA levels were assessed using the International PA Questionnaire. MetS risk factors were determined using clinically established diagnostic criteria. RESULTS: Compared with the highest PA group, the group with the lowest level of PA was at greater risk of high triglyceride (TG) in males (odds ratio [OR], 1.87; 95% confidence interval [CI], 1.07 to 3.24) and of hemoglobin A1c > or =5.5% in females (OR, 1.75; 95% CI, 1.00 to 3.04) after adjusting for age and body mass index. Compared with subjects who met the PA guidelines, those who did not meet the guidelines were more likely to have low high density lipoprotein cholesterol in both males (OR, 1.69; 95% CI, 1.11 to 2.58), and females (OR, 1.82; 95% CI, 1.20 to 2.77). Furthermore, those who did not meet the PA guidelines were at increased risk of high TG levels in males (OR, 1.69; 95% CI, 1.23 to 2.86) and abnormal fasting glucose (OR, 1.93; 95% CI, 1.17 to 3.20) and MetS (OR, 2.10; 95% CI, 1.15 to 3.84) in females. CONCLUSION: Increased levels of PA are significantly associated with a decreased risk of abnormal MetS components.
Adult ; Body Mass Index ; Cholesterol ; Cholesterol, HDL ; Chronic Disease ; Fasting ; Female ; Glucose ; Hemoglobin A, Glycosylated ; Hemoglobins ; Humans ; Lipoproteins ; Male ; Motor Activity ; Risk Factors ; Surveys and Questionnaires

The mortality rate of patients with acute graft versus host disease (GVHD) after liver transplantation is very high. Autoimmune hepatitis and diabetes mellitus (DM) is the risk factors of GVHD. Human leukocyte antigen (HLA)-homozygote donor with one way donor-recipient HLA matching is on the risk of fatal GVHD following living donor liver transplantation (LDLT). If recipient has more than one risk factors of GVHD and is going to LDLT, HLA typing is needed to identify donor-dominant one-way HLA matching and helpful to diagnose and treat early and survival of patient will be improved. We report a case of GVHD after liver transplantation for hepatocellular carcinoma and DM who received an allograft from his HLA-homozygous son.
Carcinoma, Hepatocellular ; Diabetes Mellitus ; Graft vs Host Disease ; Hepatitis, Autoimmune ; Histocompatibility Testing ; HLA Antigens ; Humans ; Leukocytes ; Liver ; Liver Transplantation ; Living Donors ; Risk Factors ; Tissue Donors ; Transplantation, Homologous ; Transplants

To expand the indications for living liver allograft, an increasing number of clinical trials of ABO incompatible liver transplantation have been reported in the literature. Administration of immunosuppressive drugs has resulted in decreased rates of mortality and graft loss in patients undergoing in ABO-incompatible living donor liver transplantation (LDLT). Rh incompatibility in liver transplantation has not been reported as frequently as that of ABO-incompatible LDLT. Rh-antigens are said to be located only on red blood cells, and have not been demonstrated on leukocytes or tissue cells. For this reason, the incompatibility of Rh antigen between the donor and the recipient has received significantly less consideration in organ transplantations. Here, we report on a case of Rh incompatible living related liver transplantation from a group O, Rh-positive donor to a group O, Rh-negative recipient. The recipient did not develop Rh alloimmunization after administration of immunosupressive drugs and Rh immunoglobin (RhIG).
Erythrocytes ; Humans ; Isoantibodies ; Korea ; Leukocytes ; Liver ; Liver Transplantation ; Living Donors ; Organ Transplantation ; Tissue Donors ; Transplantation, Homologous ; Transplants

To expand the indications for living liver allograft, an increasing number of clinical trials of ABO incompatible liver transplantation have been reported in the literature. Administration of immunosuppressive drugs has resulted in decreased rates of mortality and graft loss in patients undergoing in ABO-incompatible living donor liver transplantation (LDLT). Rh incompatibility in liver transplantation has not been reported as frequently as that of ABO-incompatible LDLT. Rh-antigens are said to be located only on red blood cells, and have not been demonstrated on leukocytes or tissue cells. For this reason, the incompatibility of Rh antigen between the donor and the recipient has received significantly less consideration in organ transplantations. Here, we report on a case of Rh incompatible living related liver transplantation from a group O, Rh-positive donor to a group O, Rh-negative recipient. The recipient did not develop Rh alloimmunization after administration of immunosupressive drugs and Rh immunoglobin (RhIG).
Erythrocytes ; Humans ; Isoantibodies ; Korea ; Leukocytes ; Liver ; Liver Transplantation ; Living Donors ; Organ Transplantation ; Tissue Donors ; Transplantation, Homologous ; Transplants

PURPOSE: Active perioperative intervention and improvement on surgical technique has decreased the mortality rate of total anomalous pulmonary venous connection (TAPVC); however, when complicated with pulmonary venous obstruction, operative mortality is still high. The purpose of this study was to investigate the clinical course of TAPVC. METHODS: Twenty-seven patients who were diagnosed with TAPVC (without other complex heart anomalies) by echocardiogram at Kyungpook National University Hospital from January 1994 to February 2008 were included. RESULTS: Mean age at diagnosis was 28.1+/-33.4 days (1-126 days). Sites of drainage were supracardiac type (15), cardiac (6), infracardiac (5), and mixed (1). Seven patients had pulmonary venous obstruction: 5 with supracardiac type, 1 with cardiac, and 1 with infracardiac. Intraoperative trans-esophageal echocardiograms were performed in 14 patients (58.3%). The operative mortality was 16.7% (4 of 24) and overall hospital mortality (including deaths without operation) was 22.2% (6 of 27). There were 5 postoperative pulmonary venous obstructions. The sites of obstruction were anastomotic in 3 of 5 (60%) patients, and ostial pulmonary vein in the other 2 (40%) patients. Three patients who presented with anastomotic pulmonary venous obstruction underwent reoperation, but all the patients were found to have pulmonary venous anastomotic obstruction. The other 2 patients with ostial pulmonary vein obstruction who had no significant symptoms were diagnosed by routine echocardiographic examination during follow-up. CONCLUSION: In TAPVC patients, early diagnosis and aggressive surgical management will improve prognosis, and we must pay attention to early and late pulmonary vein restenosis through intraoperative trans-esophageal echocardiogram and peri- and post-operative echocardiographic follow-up examinations.
Drainage ; Early Diagnosis ; Follow-Up Studies ; Heart ; Hospital Mortality ; Humans ; Prognosis ; Pulmonary Veins ; Reoperation

Hepatic myelopathy is a rare complication of chronic liver disease that is associated with extensive portosystemic shunts. The main clinical feature of hepatic myelopathy is progressive spastic paraparesis in the absence of sensory or sphincter impairment. Early and accurate diagnosis of hepatic myelopathy is important because patients with early stages of the disease can fully recover following liver transplantation. Motor-evoked potential studies may be suitable for the early diagnosis of hepatic myelopathy, even in patients with preclinical stages of the disease. Here we describe two patients who presented with spastic paraparesis associated with a spontaneous splenorenal shunt and without any previous episode of hepatic encephalopathy. One patient experienced improved neurologic symptoms after liver transplantation, whereas the other patient only received medical treatment, which did not prevent the progression of spastic paraparesis.
Adult ; Disease Progression ; Evoked Potentials, Motor/physiology ; Hepatitis B, Chronic/complications/diagnosis ; Hepatitis C, Chronic/complications/diagnosis ; Humans ; Liver Cirrhosis/*complications/diagnosis ; Liver Transplantation ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Paraparesis, Spastic/etiology/pathology ; Renal Veins/*radiography ; Spinal Cord Diseases/*diagnosis/etiology/radiography ; Splenic Vein/*radiography ; Tomography, X-Ray Computed ; Vascular Fistula/*radiography

Dermatofibrosarcoma protuberans is a slow-growing, but locally aggressive fibrous tumor that has a high rate of local recurrence after surgical resection. This tumor most commonly occurs in the trunk and proximal extremities. In this report we present a case of a 47-year-old woman with dermatofirbosarcoma protuberans in her breast, which is a very unusual site. Complete and careful resection is recommended for this type of tumor to prevent its recurrence.
Breast* ; Dermatofibrosarcoma* ; Extremities ; Female ; Humans ; Middle Aged ; Recurrence