No abstract available.
Humans ; Risk Factors*

No abstract available.
Child* ; Hemagglutination* ; Humans ; Mycoplasma pneumoniae* ; Mycoplasma* ; Pneumonia* ; Pneumonia, Mycoplasma*

Transient pancytopenia preceding acute lymphoblastic leukemia is an infrequent but well-known event, especially in childhood. We report a case of childhood acute leukemia preceded by transient pancytopenia and hepatitis. A seven-year-old boy presented with pancytopenia and hepatomegaly. He had suffered from fever, generalized rash, and jaundice for one week. His clinical symptoms and laboratory data normalized within two weeks. One and a half months later, however, he developed multiple lymphadenopathy and hepatomegaly, and follow-up bone marrow study revealed acute leukemia. The blasts were counted up to 92% of all nucleated cells in bone marrow, and showed lymphoid feature confirmed by the cytochemical reactions. Immunophenotypically, it was acute biphenotypic leukemia with positivity of CD13, CD33, CD19, CD10, CD34, HLA-DR, and terminal deoxynucleotidyl transferase. In conclusion, this is the first report of childhood leukemia preceded by pancytopenia in Korea, characteristically with the biphenotypic feature and the rare association with hepatitis.
Bone Marrow ; DNA Nucleotidylexotransferase ; Exanthema ; Fever ; Follow-Up Studies ; Hepatitis ; Hepatomegaly ; HLA-DR Antigens ; Humans ; Jaundice ; Korea ; Leukemia* ; Leukemia, Biphenotypic, Acute ; Lymphatic Diseases ; Male ; Pancytopenia* ; Precursor Cell Lymphoblastic Leukemia-Lymphoma

The use of a pneumatic torniquet to obtain a blood less field in the extremities is essential in the precise operations of present-day plastic and orthopedic surgery. There have been studies conducted regarding systemic and metabolic change in response to torniquet ischemia and complication, associated with torniquets have been reported such as injuries to nerves, muscles and blood vessel. The purpose of this investigation was to examine changes in the systemic circulation, blood gases serum potassium and base status on release of torniquet ischemia in adult. The results were as follows: 1) The mean blood pressure and heart rate did not change significantly after torniquet release. 2) The pH decreased significantly after the torniquet release. 3) The PaO2, and PaCO2, did not charge significantly after torniquet release. 4) The HCO3, and BE decreased significantly after torniquet release (P<0.05). 5) The serum potassium levels tended to increase after torniquet release but the changes was not significant. There-fore to minimize the predictable complications, the ventilatory control and rapid volume replacement are needed during the time immediately preceeding and following torniquet release.
Adult* ; Blood Circulation ; Blood Pressure ; Blood Vessels ; Extremities ; Gases ; Heart Rate ; Humans ; Hydrogen-Ion Concentration ; Ischemia ; Lower Extremity* ; Muscles ; Orthopedics ; Plastics ; Potassium

This study evaluated the methodological quality of CPGs using the Korean AGREE II scoring guide and a web-based appraisal system and was conducted by qualified appraisers. A total of 27 Korean CPGs were assessed under 6 domains and 23 items on the AGREE II instrument using the Korean scoring guide. The domain scores of the 27 guidelines were as following: the mean domain score was 82.7% (median 84.7%, ranging from 55.6% to 97.2%) for domain 1 (scope and purpose); 53.4% (median 56.9%, ranging from 11.1% to 95.8%) for domain 2 (stakeholder involvement); 63.0% (median 71.4%, ranging from 13.5% to 90.6%) for domain 3 (rigor of development); 88.9% (median 91.7%, ranging from 58.3% to 100.0%) for domain 4 (clarity of presentation); 30.1% (median 27.1%, ranging from 3.1% to 67.7%) for domain 5 (applicability); and 50.2% (median 58.3%, ranging from 0.0% to 93.8%) for domain 6 (editorial independence). Three domains including scope and purpose, rigor of development, and clarity of presentation were rated at more than 60% of the scaled domain score. Three domains including stakeholder involvement, applicability, and editorial independence were rated at less than 60% of the scaled domain score. Finally, of the 27 guidelines, 18 (66.7%) were rated at more than 60% of the scaled domain score for rigor of development and were categorized as high-quality guidelines.
Humans ; Internet ; *Practice Guidelines as Topic ; *Program Evaluation ; Quality Assurance, Health Care ; Republic of Korea

A 32-year-old female patient and her sister show high levels of high density lipoprotein (HDL) cholesterol in regular health checkups, since female patient was 11 years old. The patient's serum total cholesterol was 285 mg/dL and HDL cholesterol was 113 mg/dL. Her sister's total cholesterol was 240 mg/dL and the HDL cholesterol measured to be 90 mg/dL. Lipoprotein pattern and cholesteryl ester transfer activity gene analysis were examined in these patients. We found c.1321+1G>A (IVS14+1G/A) hetero mutation in cholesteryl ester transfer protein (CETP) genes. Generally, CETP mediates transfer and exchange of triglycerides and cholesteryl ester between plasma lipoproteins. Also we investigated a key role of HDL-CE and Apo A-1 metabolism. Patients with low levels of CETP have increased serum HDL levels. We hereby report two Korean cases of CETP deficiency in a family. Brief literature review ensues with the cases.
Adult ; Apolipoprotein A-I ; Cholesterol ; Cholesterol Ester Transfer Proteins ; Cholesterol, HDL ; Female ; Humans ; Hypercholesterolemia ; Lipid Metabolism, Inborn Errors ; Lipoproteins ; Plasma ; Protein Deficiency ; Siblings ; Triglycerides

This study introduces the Clinical practice guidelines (CPGs) appraisal system by the Korean Academy of Medical Sciences (KAMS). Quality management policies for CPGs vary among different countries, which have their own cultures and health care systems. However, supporting developers in guideline development and appraisals using standardized tools are common practices. KAMS, an organization representing the various medical societies of Korea, has been striving to establish a quality management system for CPGs, and has established a CPGs quality management system that reflects the characteristics of the Korean healthcare environment and the needs of its users. KAMS created a foundation for the development of CPGs, set up an independent appraisal organization, enacted regulations related to the appraisals, and trained appraisers. These efforts could enhance the ability of each individual medical society to develop CPGs, to increase the quality of the CPGs, and to ultimately improve the quality of the information available to decision-makers.
Guideline Adherence/*standards ; *Models, Organizational ; Practice Guidelines as Topic/*standards ; Quality Assurance, Health Care/*standards ; Republic of Korea

PURPOSE: The purpose of this cross-sectional study was to examine the relationship between characteristics of severe ALS patient-caregiver couples and health related quality of life (HRQoL) in family caregivers. METHODS: The participants in this study were 89 pairs of ALS patients using ventilators and a family caregiver. The characteristics of the ALS patients and caregivers, Korean-Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised, Zarit Burden Interview and SF-36 were measured in this study. The data were collected from August 2008 to April 2009. Descriptive statistics, Pearson correlation coefficients, and canonical correlation were used for data analysis. RESULTS: The physical component summary and mental component summary of the HRQoL score for family caregivers were 147.49+/-31.63 and 129.09+/-35.83, respectively. HRQoL for caregivers was related to characteristics of the ALS patient-caregiver couples, such as patient's gender, caregiver's age, gender, marital status, daily time spent in caregiving and burden with one significant canonical variable. The significant variate showed that the lower the age, the time spent in caregiving and the burden of caregivers, the higher the HRQoL of caregivers. CONCLUSION: The support systems for caregivers considering caregiver characteristics such as demographics and burden should be implemented to improve the HRQoL of caregivers.
Adult ; Aged ; Amyotrophic Lateral Sclerosis/*psychology ; Caregivers/*psychology ; Cross-Sectional Studies ; Dependency (Psychology) ; Female ; Humans ; Interviews as Topic ; Male ; Middle Aged ; *Quality of Life ; Questionnaires ; Ventilators, Mechanical

Trisomy 9 syndrome was first reported by Retheore in 1970 and has been rarely reported. This syndrome consists of partial and complete trisomy 9. It is characterized by growth and mental retardation, craniofacial abnormalities including microcephaly, hypertelorism, prominent nose, deep-set ears and down-slanting palpebral fissures. Congenital heart diseases and congenital dislocations of knee are also common in trisomy 9 syndrome. Here, we report a very rare case of partial trisomy 9 due to maternal balanced translocation t(9;21). He showed craniofacial abnormalities, brain malformation, cardiac defect, hydronephrosis and congenital dislocations of hip and knee joints.
Brain ; Chromosomes, Human, Pair 9 ; Craniofacial Abnormalities ; Dislocations ; Ear ; Heart Diseases ; Hip ; Hydronephrosis ; Hypertelorism ; Intellectual Disability ; Knee ; Knee Joint ; Microcephaly ; Nose ; Trisomy

Trisomy 9 syndrome was first reported by Retheore in 1970 and has been rarely reported. This syndrome consists of partial and complete trisomy 9. It is characterized by growth and mental retardation, craniofacial abnormalities including microcephaly, hypertelorism, prominent nose, deep-set ears and down-slanting palpebral fissures. Congenital heart diseases and congenital dislocations of knee are also common in trisomy 9 syndrome. Here, we report a very rare case of partial trisomy 9 due to maternal balanced translocation t(9;21). He showed craniofacial abnormalities, brain malformation, cardiac defect, hydronephrosis and congenital dislocations of hip and knee joints.
Brain ; Chromosomes, Human, Pair 9 ; Craniofacial Abnormalities ; Dislocations ; Ear ; Heart Diseases ; Hip ; Hydronephrosis ; Hypertelorism ; Intellectual Disability ; Knee ; Knee Joint ; Microcephaly ; Nose ; Trisomy