Here we report an autopsy case of diabetic ketoacidosis (DKA) with severe hypertriglyceridemia (12,900 mg/dl). A 29-year-old woman with a history of type 1 diabetes was found dead at a motel. There was no injury on external inspection, but a lump of cheese-like material was noted in the heart at autopsy and peripheral blood plasma had a creamy appearance. After postmortem biochemical analysis, we made a diagnosis of DKA with severe hypertriglyceridemia and concluded that these unusual autopsy findings were caused by DKA and postmortem change. Uncontrolled diabetes often causes DKA and hypertriglyceridemia. To the best of our knowledge, this is the first report in Korea of DKA with severe hypertriglyceridemia diagnosed by autopsy.
Adult ; Autopsy* ; Diabetic Ketoacidosis* ; Diagnosis ; Female ; Heart* ; Humans ; Hypertriglyceridemia* ; Korea ; Plasma ; Postmortem Changes

Patients with Beckwith-Wiedemann syndrome (BWS) are predisposed to developing embryonal tumors, with hepatoblastoma being the most common type. Our patient showed hemihypertrophy, macroglossia, and paternal uniparental disomy in chromosome 11 and was diagnosed with BWS. When the patient was 9 months old, a 2.5×1.5 cm oval hypoechoic exophytic mass was detected in the inferior tip of his right liver. Preoperative imaging identified it as hepatoblastoma; however, histologic, immunohistochemistry, and electron microscopic findings were compatible with adrenal cortical neoplasm with uncertain malignant potential. The origin of the adrenal tissue seemed to be heterotopic. Here, we describe for the first time an adrenal cortical neoplasm with uncertain malignant potential arising in the heterotopic adrenal cortex located in the liver of a patient with BWS.
Adrenal Cortex ; Adrenal Gland Neoplasms ; Beckwith-Wiedemann Syndrome ; Chromosomes, Human, Pair 11 ; Hepatoblastoma ; Humans ; Immunohistochemistry ; Liver ; Macroglossia ; Uniparental Disomy

Purpose: Recent treatments for pediatric acute lymphoblastic leukemia (ALL) are founded on risk stratification. We examined the survival rates and prognostic factors of patients over a 20-year period at a single institution. Materials and Methods: This study analyzed patients diagnosed with ALL and treated at the Pediatric Department of Samsung Medical Center (SMC). Patients were categorized into standard-risk (SR), high-risk (HR), and very high-risk (VHR) groups. The SMC protocol for the HR group underwent two changes during the study period: a modified Children’s Cancer Group (CCG)-1882 protocol was used from 2000 to 2005, the Korean multicenter HR ALL-0601 protocol from 2006 to 2014, and the Korean multicenter HR ALL-1501 protocol from 2015 to 2019. Results: Of the 460 patients, complete remission was achieved in 436 patients (94.8%). The 10-year overall survival rate (OS) was 83.8±1.9% for all patients. OS according to the SMC risk group was as follows: 95.9%±1.4% in the SR group, 83.8%±3.6% in the HR group, and 66.2%±6.9% in the VHR group. The 5-year OS within the HR group varied according to the treatment protocol: 73.9%±7.5%, in the modified CCG-1882 protocol, 83.0%±3.9%, in the 0601 protocol, and 96.2%±2.6%, in the 1501 protocol. For those aged 15 years and older, the OS was only 56.5%±13.1%. Relapse occurred in 71 patients (15.4%), and the OS after relapse was 37.7%±6.0%. Conclusion The treatment outcomes of patients with ALL improved markedly. However, there is a need to further characterize adolescents and young adult patients, as well as those who have experienced relapses.

Purpose: Recent treatments for pediatric acute lymphoblastic leukemia (ALL) are founded on risk stratification. We examined the survival rates and prognostic factors of patients over a 20-year period at a single institution. Materials and Methods: This study analyzed patients diagnosed with ALL and treated at the Pediatric Department of Samsung Medical Center (SMC). Patients were categorized into standard-risk (SR), high-risk (HR), and very high-risk (VHR) groups. The SMC protocol for the HR group underwent two changes during the study period: a modified Children’s Cancer Group (CCG)-1882 protocol was used from 2000 to 2005, the Korean multicenter HR ALL-0601 protocol from 2006 to 2014, and the Korean multicenter HR ALL-1501 protocol from 2015 to 2019. Results: Of the 460 patients, complete remission was achieved in 436 patients (94.8%). The 10-year overall survival rate (OS) was 83.8±1.9% for all patients. OS according to the SMC risk group was as follows: 95.9%±1.4% in the SR group, 83.8%±3.6% in the HR group, and 66.2%±6.9% in the VHR group. The 5-year OS within the HR group varied according to the treatment protocol: 73.9%±7.5%, in the modified CCG-1882 protocol, 83.0%±3.9%, in the 0601 protocol, and 96.2%±2.6%, in the 1501 protocol. For those aged 15 years and older, the OS was only 56.5%±13.1%. Relapse occurred in 71 patients (15.4%), and the OS after relapse was 37.7%±6.0%. Conclusion The treatment outcomes of patients with ALL improved markedly. However, there is a need to further characterize adolescents and young adult patients, as well as those who have experienced relapses.

Purpose: Recent treatments for pediatric acute lymphoblastic leukemia (ALL) are founded on risk stratification. We examined the survival rates and prognostic factors of patients over a 20-year period at a single institution. Materials and Methods: This study analyzed patients diagnosed with ALL and treated at the Pediatric Department of Samsung Medical Center (SMC). Patients were categorized into standard-risk (SR), high-risk (HR), and very high-risk (VHR) groups. The SMC protocol for the HR group underwent two changes during the study period: a modified Children’s Cancer Group (CCG)-1882 protocol was used from 2000 to 2005, the Korean multicenter HR ALL-0601 protocol from 2006 to 2014, and the Korean multicenter HR ALL-1501 protocol from 2015 to 2019. Results: Of the 460 patients, complete remission was achieved in 436 patients (94.8%). The 10-year overall survival rate (OS) was 83.8±1.9% for all patients. OS according to the SMC risk group was as follows: 95.9%±1.4% in the SR group, 83.8%±3.6% in the HR group, and 66.2%±6.9% in the VHR group. The 5-year OS within the HR group varied according to the treatment protocol: 73.9%±7.5%, in the modified CCG-1882 protocol, 83.0%±3.9%, in the 0601 protocol, and 96.2%±2.6%, in the 1501 protocol. For those aged 15 years and older, the OS was only 56.5%±13.1%. Relapse occurred in 71 patients (15.4%), and the OS after relapse was 37.7%±6.0%. Conclusion The treatment outcomes of patients with ALL improved markedly. However, there is a need to further characterize adolescents and young adult patients, as well as those who have experienced relapses.

PURPOSE: The aim of this study was to compare job satisfaction, quality of life (QOL), incident report rate and overtime hours for 12-hour shifts and for 8-hour shifts in a pediatric intensive care unit (PICU). METHODS: A descriptive survey was conducted with a convenience sample of 36 staff nurses from a PICU in a regional hospital in Korea. Data were collected using self-administrated questionnaires regarding job satisfaction and QOL at 6 months before and after the beginning of 12-hour shifts. Incident report rate and overtime hours for both 12-hour and 8-hour shifts were compared. Comparisons were made using chi2-test, paired t-test and Mann-Whitney U test. RESULTS: After 12-hour shifts were initiated, job satisfaction significantly increased (t=3.93, p<.001) and QOL was higher for nurses on 12-hour shifts compared to 8-hour (t=7.83, p<.001). There was no statistically significant change in incident report rate (chi2=0.15, p=.720). The overtimes decreased from 36.3+/-34.7 to 17.3+/-34.9 minutes (Z=-8.91, p<.001). CONCLUSION: These results provide evidence that 12-hour shifts can be an effective ways of scheduling for staff nurses to increase job satisfaction and quality of life without increasing patient safety incidents or prolonged overtime work hours.
Intensive Care Units* ; Job Satisfaction* ; Korea ; Patient Safety ; Quality of Life* ; Surveys and Questionnaires ; Risk Management*

A review of the literature on cancer pain revealed that many persons with cancer receive inadequate analgesia for pain control, due in part to a lack of knowledge of the control of cancer pain by both physicians and nurses. This study is composed of two parts : one is to train nurses to change their knowledge of and attitude toward the pain management of patients having cancer and to evaluate the effectiveness of this training in comparison with other non-trained group ; the other is to test the applicability of the pain management method knowledge and attitude in the levels of pain of oncology patients. General characteristics of nurses such as age, education, educational experiences of cancer pain management were not different in both groups except the clinical experience. General characteristics of cancer patients and pain-related variables such as pain, sleep, daily activities, treatment modalities, causes of pain were not different in both groups except the educational levels of patients. After an eight-hour educational program given to the experimental nurse group, the knowledge and attitude about assessment of cancer pain, pain medication, and pharmacological knowledge were significantly higher in the experimental group than in the control group, while knowledge about classification of analgesics was not significantly different. The amount of analgesics, measured by the morphine equivalent doses, used in the experimental group was significantly lower than in the control group in the first and the last days. The experimental group used more systematic ways of drug changes from non-narcotic analgesics to narcotic analgesics than the control group. This indicated that the control group used fentanyl patches more commonly than in the control group. Cancer pain scores of both group of patients were measured on an hourly bases for a week in both groups. The patients' pain scores of the first day of measurement in experimental group were not significantly higher than those of control group of patients, while those of the last day were significantly higher than those of the control group. This study supports the need for educational program for the management of cancer pain to the nurses and the doctors.
Analgesia ; Analgesics ; Analgesics, Non-Narcotic ; Classification ; Education ; Fentanyl ; Humans ; Morphine ; Narcotics ; Pain Management*

Malignant germ cell tumore occur in children and young women in reproductive age, of all the germ cell maligaanci orily pure dysgerminiomas had a high cure rate prior to 1970. This was due to the exquisite wdioseneitivity of these tumors. Multiple agent chemotherapy has dramatieally improved the pr nosis af patients with malignant ovarian germ cell tumors. The purpose of this study is to report the experience at Aaan Medical Center, department of Obstetrics and Gynecology, in 16 patients withmalignant ovarian germ cell tumors treated between July, l989 and June, l994. We analyzed the effect of age, histolagic subtype, FIGO stage, surgical pmcedurse and regimens of chemotherapy, on the prognosis of thwe tumors. The results obtained were as follows: 1. In histologic subtypes, dysgenninoma(38.0%), endodermal sinus tumor(25.0%), squamous cell carcinoma arising in mature cystic teratoma(19.0%), mixed cell tumor(6.0%), immature teratoma(6.0%), malignant ectodermal tumor in mature cystic teratoma(6.0%), were counted in order. 2. No site predilection was identifed. 3. Main initial symptoms were abdominal distension(31.0%), abdominal pain(31.0%), abdominal mass palpation(25.0%), amenorrhea(6.0%) in order. 4. Multiple tumor markers were considered to be useful in diagnosis and follow up of malignant germ cell tumors of ovary. 5. The mean age of malignant ovarian germ cell tumors was 29.5 years, and 11 cases(69.3%) of tumors under the age of 30.0 years. 6. The survival rate seemed to be decreased with advancing FIGO stage.
Carcinoma, Squamous Cell ; Child ; Diagnosis ; Drug Therapy ; Ectoderm ; Endoderm ; Female ; Follow-Up Studies ; Germ Cells* ; Gynecology ; Humans ; Neoplasms, Germ Cell and Embryonal* ; Obstetrics ; Ovary* ; Prognosis ; Survival Rate ; Biomarkers, Tumor

BACKGROUND: Microdeletion syndromes not detectable by conventional cytogenetic analysis have been reported to occur in approximately 5% of patients with unexplained mental retardation (MR). Therefore, it is essential to ensure that patients with MR are screened for these microdeletion syndromes. Mental retardation syndrome multiplex ligation-dependent probe amplification (MRS-MLPA) is a new technique for measuring sequence dosages that allows for the detection of copy number changes of several microdeletion syndromes (1p36 deletion syndrome, Williams syndrome, Smith-Magenis syndrome, Miller-Dieker syndrome, DiGeorge syndrome, Prader-Willi/Angelman syndrome, Alagille syndrome, Saethre-Chotzen syndrome, and Sotos syndrome) to be processed simultaneously, thus significantly reducing the amount of laboratory work. METHODS: We assessed the performance of MLPA (MRC-Holland, The Netherlands) for the detection of microdeletion syndromes by comparing the results with those generated using FISH assays. MLPA analysis was carried out on 12 patients with microdeletion confirmed by FISH (three DiGeorge syndrome, four Williams syndrome, four Prader-Willi syndrome, and one Miller-Dieker syndrome). RESULTS: The results of MLPA analysis showed a complete concordance with FISH in 12 patients with microdeletion syndromes. CONCLUSIONS: On the basis of these results, we conclude that MLPA is an accurate, reliable, and cost-effective alternative to FISH in the screening for microdeletion syndromes.
*Chromosome Deletion ; Classical Lissencephalies and Subcortical Band Heterotopias/genetics ; DiGeorge Syndrome/genetics ; Humans ; In Situ Hybridization, Fluorescence/*methods ; Laboratories, Hospital ; Mental Retardation/*diagnosis/genetics ; Nucleic Acid Amplification Techniques/*methods ; Prader-Willi Syndrome/genetics ; Williams Syndrome/genetics

OBJECTIVES: The present study was performed to evaluate whether naltrexone treatment are effectively lowering the urge of alcohol drinking, and to investigate the its mechanism of action. METHODS: 15 healthy male social drinkers were voluntarily participated. The experimental method was double-blind placebo-controlled cross over design. Subjects ingested a naltrexone (50mg)/day or placebo for 1 week. Plasma beta-endorphin, plasma ACTH and serum cortisol levels were measured before, at 20 minutes and at 60 minutes after alcohol exposure. Subjects completed self-report questionnaires such as the visual analog scales of drink urge and the alcohol sensation scales at regular intervals. RESULTS: 1) During naltrexone pretreatment period, subjects reported more headache, dizziness, nervousness, fatigue, day somnolence, nausea, and decreased appetite than placebo pretreatment period. But serum GOT/GPT levels were not significantly different between two pretreatment periods. 2) In case of naltrexone pretreatment, subjects reported significantly less urge to alcohol drink on the self-reporting urge scales, especially at post-drinking 20 minutes and 60 minutes than placebo pretreatment. 3) After alcohol challenge, subjects reported significantly more dizziness on the alcohol sensation scales in case of naltrexone pretreatment, and reported less mood elevation trend though it was not statistically significant. Other scores were not significantly different between two pretreatments. 4) Plasma beta-endorphin levels were significantly different when treated with naltrexone. In case of naltrexone-pretreatment, the increasing degree of plasma beta-endorphin during 20 minutes after alcohol challenge was significantly higher than placebo pretreatment. 5) Basal plasma ACTH level and basal serum cortisol level were not significantly different between two pretreatments. After alcohol challenge, only the decreasing degree of plasma ACTH levels during 20 minutes was significantly lowered in the naltrexone pretreatment than placebo pretreatment. CONCLUSIONS: Naltrexone reduced urge to alcohol drinking in social drinker. The action mechanism of naltrexone may be partially blocking opioid positive reward system and partially alcohol mimicking its property.
Adrenocorticotropic Hormone ; Alcohol Drinking ; Anxiety ; Appetite ; beta-Endorphin* ; Cross-Over Studies ; Dizziness ; Fatigue ; Headache ; Humans ; Hydrocortisone ; Male ; Naltrexone* ; Nausea ; Plasma* ; Surveys and Questionnaires ; Reward ; Sensation ; Visual Analog Scale ; Weights and Measures