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

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

STUDY DESIGN: A case report. OBJECTIVES: To report a rare case of subacute delayed ascending myelopathy. SUMMARY OF LITERATURE REVIEW: After low spinal cord injury, the cord injury may proceed to a proximal level and lead to subacute delayed ascending myelopathy. The patient suffered from orthostatic hypotension, weakness and sensory loss in the upper extremities and dyspnea. MRI showed more proximal progression of the spinal cord injury. There is no prevention or treatment for this condition. MATERIALS AND METHODS: A 62-year-old man fell from heights and had 11th thoracic spine flexion-distraction injury. Upon arrival at the hospital, he was found to suffer from lower extremity weakness and sensory loss, but showed no neurologic symptom in his upper extremities. Two days later, we performed posterior instrumentation with fusion, and no postoperative neurologic symptom change was detected. One week after the fall, he suffered from dyspnea, upper extremity weakness and sensory loss. MRI was taken and we discovered that his spinal cord injury had proceeded to the 2nd cervical spine level. RESULTS: Three months later, he showed little improvement in his upper extremity motor power, but not to the extent of the previous low spinal injury. CONCLUSION: Physicians should pay attention to the upper extremity and respiratory function of the patient with low spinal cord injury, because the level of spinal cord injury may proceed to a proximal level.
Dyspnea ; Humans ; Hypotension, Orthostatic ; Lower Extremity ; Middle Aged ; Neurologic Manifestations ; Spinal Cord ; Spinal Cord Diseases ; Spinal Cord Injuries ; Spine ; Upper Extremity

OBJECTIVES: The aims of this study were to determine the benefits of short-term empirical proton pump inhibitor (PPI) medication on laryngopharyngeal reflux (LPR) and to determine whether scores on the reflux symptom index (RSI) and the reflux finding score (RFS) could be combined to identify subgroups of patients that will more likely to improve with this medication. METHODS: Fifty-one Korean Otolaryngology Board-certified specialists joined this prospective, multi-center, and open-label observational study. A total of 1,142 adult patients with LPR was enrolled for 12 weeks of rabeprazol medication. According to pre-treatment scores on RSI and RFS, patients were divided into 4 subgroups. RFS and RSI were measured repeatedly with a month interval along the treatment period. Changes of RSI and RFS were analyzed in an overall study cohort as well as in each subgroup. RESULTS: Approximately 40% (n=455) of enrolled patients were followed up until 12 weeks of PPI treatment. Significant improvement in RSI was obtained in 29%, 58%, and 75% of patients after 4, 8, and 12 weeks of PPI medication. RFS was improved in 16%, 42%, and 57% of the patients with 4, 8, and 12 weeks of PPI medication. All subgroups showed improvement regardless of their pre-treatment scores on the RSI and RFS. CONCLUSION: Even though RSI and RFS may be used as a general guideline for LPR management, pre-treatment RSI and RFS are not useful in predicting the patients' response to short-term PPI medication in the usual pattern of practice for LPR, which is mostly based on the physical evaluation and history taking.
2-Pyridinylmethylsulfinylbenzimidazoles ; Adult ; Cohort Studies ; Humans ; Laryngopharyngeal Reflux ; Otolaryngology ; Prospective Studies ; Proton Pump Inhibitors ; Proton Pumps ; Specialization

Combination chemotherapy and radiation therapy have contributed to the successful treatment of various cancer patients. But the development of second malignancies is an inevitable complication of long-term cytotoxic treatment. The most serious and frequent of such complications is acute myelogenous leukemia (AML). Therapy-related leukemia is generally fatal. Since the number of patients exposed to chemotherapy is increasing each year, the clinical significance of this entity cannot be underestimated. There have been many investigations of therapy-related leukemia, but in Korea published reports are rare. We describe four such cases, involving one older female with lung cancer and three children with acute lymphoblastic leukemia (ALL) and malignant lymphoma. Alkylating agents were used for chemotherapy, and in one case, topoisomerase II inhibitor. Irrespective of the causative agents, the latency periods were relatively short, and despite induction chemotherapy in two, all survived for only a few months. During the follow-up of patients treated for primary malignancies, the possibility of therapy-related leukemia should always be borne in mind.
Adolescence ; Aged ; Antineoplastic Agents, Alkylating/therapeutic use* ; Antineoplastic Agents, Alkylating/adverse effects ; Carcinoma, Small Cell/radiotherapy ; Carcinoma, Small Cell/drug therapy ; Case Report ; Child ; DNA Topoisomerase (ATP-Hydrolysing)/antagonists & inhibitors ; Fatal Outcome ; Female ; Human ; Leukemia, Lymphocytic, Acute, L1/drug therapy ; Leukemia, Monocytic, Acute/etiology ; Leukemia, Myelocytic, Acute/etiology* ; Leukemia, Myelomonocytic, Acute/etiology* ; Lung Neoplasms/radiotherapy ; Lung Neoplasms/drug therapy ; Lymphoma, B-Cell/radiotherapy ; Lymphoma, B-Cell/drug therapy ; Male ; Neoplasms, Second Primary/etiology*

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