BACKGROUND: The aim of this study was to investigate the prevalence, clinical and laboratory findings of hereditary hemolytic anemia (HHA) in Korea from 1997 to 2006 and to develop the appropriate diagnostic approach for HHA. METHODS: By the use of questionnaires, information on the clinical and laboratory findings ofHHA diagnosed from 1997 to 2006 in Korea was collected and analyzed retrospectively. A total of 431 cases were enrolled in this study from 46 departments of 35 hospitals. RESULTS: The overall frequency of HHA did not change through the 10-year period for pediatrics but did show an increasing tendency for internal medicine. The overall male to female sex ratio did not show sex predominance (1.17:1), but a significant male predominance with a ratio of 1.49:1 was seen for pediatrics while a significant female predominance with a ratio of 1:1.97 was seen forinternal medicine. Of the total cases, 74.2% (282/431) were diagnosed before the age of 15 years. The etiologies of HHA were classified as red cell membrane defects, hemoglobinopathies, red cell enzyme deficiencies and unknown causes. There were 382 cases (88.6%) of red cell membrane defects with 376 cases (87.2%) of hereditary spherocytosis and 6 cases (1.4%) of hereditary elliptocytosis, 20 cases (4.6%) of hemoglobinopathies with 18 cases (4.2%) of beta-thalassemia, a case (0.2%) of alpha-thalassemia and a case (0.2%) of Hemoglobin Madrid, 7 cases (1.6%) of red cell enzyme deficiencies with 5 cases (1.2%) of glucose-6- phosphate dehydrogenase (G-6-PD) deficiency, a case (0.2%) of pyruvate kinase (PK) deficiency and a case (0.2%) of enolase deficiency, and 22 cases (5.1%) of unknown causes. The most common chief complaint in pediatric patients was pallor and that in adult patients was jaundice. In the red cell membrane defect group of patients, the level of hemoglobin was significantly higher than in adult patients. The mean corpuscular volume, mean corpuscular hemoglobin, corrected reticulocyte count, total and indirect bilirubin level and lactate dehydrogenase levels in the hemoglobinopathy group of patients were significantly lower than the values in the red cell membrane defect group of patients. The mean concentration of G-6-PD was 0.8+/-0.7U/1012RBC in the G-6-PD deficient patients, PK was 1.7U/1010 RBC in the PK deficient patient, and the level of enolase was 0.04U/g of Hb in the enolase deficient patient. CONCLUSION: The most prevalent cause of HHA in Korea during 1997 to 2006 was hereditary spherocytosis, but HHA by other causes such as hemoglobinopathy and red cell enzyme deficiency gradually increased with the development of molecular diagnostic methods and increasing general interest. However, the etiologies of HHA need to be pursued further in 5.1% of the patients. An systematic standard diagnostic approach is needed in a nationwide prospective study for correct diagnoses and appropriate management of HHA.
Adult ; alpha-Thalassemia ; Anemia, Hemolytic, Congenital* ; beta-Thalassemia ; Bilirubin ; Cell Membrane ; Diagnosis ; Elliptocytosis, Hereditary ; Erythrocyte Indices ; Female ; Hemoglobinopathies ; Humans ; Internal Medicine ; Jaundice ; Korea* ; L-Lactate Dehydrogenase ; Male ; Oxidoreductases ; Pallor ; Pathology, Molecular ; Pediatrics ; Phosphopyruvate Hydratase ; Prevalence ; Pyruvate Kinase ; Reticulocyte Count ; Retrospective Studies* ; Sex Ratio ; Surveys and Questionnaires

PURPOSE: Perforated gastric cancer (PGC) is rare and occurs in 1~4% of all gastric cancers. Possible dissemination of tumor cells at the time of perforation of the gastric carcinoma has been a matter of concern. The intraoperative determination of what kind of operation should be done and how extensive the lymphnode dissection should be still remains controversial. The purpose of this study is to evaluate the factors influencing the survival and to determine the optimal treatment for PGC. MATENRIALS AND METHODS: A total of 42 patients were operated on for a perforated gastric carcinoma at Soonchunhyang University Chunan Hospital from 1983 to 2000. the age and the sexes of the patients, the location of perforation, the diameter of perforation, the histologic type of the tumor, the depth of wall invasion, the absence or presence of lymph node metastasis / distant metastasis, the stage of disease, the type of operation, and the outcomes were examined. Statistically significant differences were analyzed by using Fisher's exact test. RESULTS: The stage distributions according to the UICC classification were 1 case of stage I, 6 cases of stage II, 17 cases of stage III, and 11 cases of stage IV. An emergency gastrectomy was done in 26 patients (61.9%), with a 5-yr survival rate of 44%. The survival of patients was significantly influenced by the depth of wall invasion, the lymphnode metastasis, distant metastasis, the stage of disease, and the type of operation. CONCLUSION: an emergency gastrectomy is the treatment of choice for most patients with resectable PGC. Choosing more a optimistic surgical approach for potentially curative cases of PGC should be one way to increase the patient's survival rate.
Chungcheongnam-do ; Classification ; Emergencies ; Gastrectomy ; Humans ; Lymph Nodes ; Neoplasm Metastasis ; Stomach Neoplasms* ; Survival Rate

The neonatal lupus syndrome is characterized by skin lesions, hepatic and hematologic abnormalities and congenital heart block. Congenital heart block which is believed to be caused by transplacental passage of the anti-Ro (SSA)/La (SSB) antibodies from mother to infant, is known to occur in 1 in 20,000 live births. In contrast to other manifestation of neonatal lupus syndrome, which usually subside within 6 months after birth, congenital heart block is a permanent and potentially fatal complication. We experienced a case of neonatal lupus syndorme with congenital complete heart block in a newborn of asyptomatic mother with anti-Ro (SSA)/ La (SSB) antibodies.
Antibodies ; Heart Block* ; Heart* ; Humans ; Infant ; Infant, Newborn ; Live Birth ; Mothers ; Parturition ; Skin

BACKGROUND: Although estrogen is known to retard atherosclerosis, the mechanisms of which remain unclear. To examine the effect of estrogen on blood nitric oxide production and aortic atherosclerosis, we did an experiment with rabbits. MATERIAL AND METHOD: Forty male New Zealand White rabbits aging 5 weeks were randomly divided as 10 normal diet(ND), 10 normal diet(ND) + transdermal estradiol-2 patches(END; 0.02 microgram/d), 10 hypercholesterol diet(CD ; 0.5% of diet; w/w), and 10 CD + transdermal estradiol-2 patches(ECD) group, respectively. After 12 weeks, we measured the serum estradiol-2, nitric oxide and cholesterol and examined the atherosclerotic lesion of thoracic and abdominal aorta with light and scanning electron microscopy. RESULT: The concentration of estradiol-2 was increased in both ED and ECD groups(p<0.001). The serum nitric oxide production of END, CD, and ECD groups decreased significantly than that of ND group(p<0.001). The total, HDL and LDL cholesterol concentrations were increase in both CD and ECD groups(p<0.001). Aortic lesion was observed only in CD and ECD groups, without any difference. On light microscopic examination, the lesions were consists mainly of accumulation of lipid-laden macrophages (foam cell). On scanning electron microscopic examinations, CD group showed scattered areas of endothelial damage. However, estradiol-2 administration to the cholesterol-fed animals did not significantly change these pathologic findings. CONCLUSION: The results shows hyperlipidemia brings a decreased production of nitric oxide, probably due to endothelial dysfunction. Estradiol-2 patch administration decreased nitric oxide level in male cholesterol-fed rabbits with unknown mechanism. The transdermal estradiol-2 patch did not show any effect on blood cholesterol level. The estrogen did not cause any change on atherogenesis on both thoracic and abdominal aorta. The gender difference and administration method may offset the beneficial effect of estrogen on atherogenesis. To gain a complete understanding for the action of estrogen on atherogenesis, further detailed studies for both genders are needed.
Aging ; Animals ; Aorta, Abdominal ; Atherosclerosis* ; Cholesterol ; Cholesterol, LDL ; Diet ; Estrogens* ; Humans ; Hyperlipidemias ; Macrophages ; Male ; Microscopy, Electron, Scanning ; Nitric Oxide* ; Rabbits*

Bone metastasis of hepatocellular carcinoma appears to be peculiar when clinical manifestation of liver disease is not apparent, and initial diagnosis of metastatic hepatocellular carcinoma by fine needle aspiration cytology is rarely obtained. We experienced a case of 45-year-old man with metastatic hepatocellular carcinoma in the sacrum, which was diagnosed by fine needle aspiration cytology. The intrahepatic mass, measuring 1.2cm in diameter and kept unchanged in size for two years, was never proved to be hepatocellular carcinoma histopathologically. The aspirated neoplastic cells were mostly in sheets, showing abundant acidophilic cytoplasm and large, round, centrally located nuclei with single, prominent acidophilic mucleoli. In the cell block section, diagnosis of metastatic well-differentiated hepatocellular carcinoma was made without difficulty, and definite trabecular fashion with sinusoidal endothelial cell lining was found.
Biopsy, Fine-Needle ; Carcinoma, Hepatocellular ; Cytoplasm ; Diagnosis ; Endothelial Cells ; Humans ; Korea* ; Liver Diseases ; Middle Aged ; Neoplasm Metastasis ; Prevalence* ; Sacrum

Pressure controlled ventilation has been proposed to recruit closed alveolar units and improve oxygenation through changing the inspiratory flow pattern from a square wave as used with volume controlled ventilation to a rapidly exponentially decaying curve and through maintaining airway pressure at a constant level throughout the inspiratory phase. The purpose of this study was to evaluate the cardiorespiratory efficacy of pressure controlled ventilation in severe respiratory failure. Cardiorespiratory values were measured in ten patients with severe respiratory failure on volume controlled and pressure controlled ventilation. Tidal volume, ventilatory rate, PEEP, inspiratory:expiratory ratio and FIO2 were maintained at the same level for both ventilatory modalities. Changing from volume controlled ventilation to pressure controlled ventilation was associated with significant improvement in PaO2 and decrease in peak inspiratory pressure. There were no significant changes in other cardiorespiratory values, such as arterial blood pressure, heart rate, inspiratory pause pressure and static compliance. These results suggest that pressure controlled ventilation may be a beneficial ventilatory modality in the treatment of severe respiratory failure compared to volume controlled ventilation.
Arterial Pressure ; Compliance ; Heart Rate ; Humans ; Oxygen ; Respiratory Insufficiency* ; Tidal Volume ; Ventilation*

Problems related to agitation in the ICU patients include cardiorespiratory instability, ina bility to cooperate with nursing care, failure to maintain op timal positioning in bed, dis- ruption of life sustaining tubes and catheters, and injuries to patients and hospital person- nel. Thus, the ability to provide safe, controllable, and reversible sedation can be important in the care of critically ill patients. Midazolam is a water soluble imidazobenzodiazepine with a rapid onset of ac tion and short elimination half life compared with diazepam or lorazepam. We evaluated the use of midazolam by continuous infusion for prolonged sedation of critically ill adult patients. The results were as follows ; 1) Midazolam infusion effectively controlled severe agitation in all patients. 2) No episodes of cardiovascular depression due to midazolam occur red during the study period. 3) In one patient, tolerance was developed 6 days after infusion. 4) Mean time to alertness was 2.23 hours. 5) In a renal failure patient, there was no significant prolongation of time to alertness. These results suggest that midazolam infusion provides safe, controllable, and reversible sedation in the care of critically ill patients.
Adult ; Catheters ; Critical Illness ; Depression ; Diazepam ; Dihydroergotamine ; Half-Life ; Humans ; Lorazepam ; Midazolam* ; Nursing Care ; Renal Insufficiency

A total of 394 cases of minimal lesion were reviewed and reassessed clinically and by laboratory investiga- tion, for 4 years from 1979 to 1982. Association with nephrotic syndrome is significantly higher in the cases with histologically normal-appearing mesangium than in the cases with mesangial proliferation. In 43% of the cases of minimal lesion, a minimal but prominent mesangial deposit of Immunoglobulin M with or without C3 deposit was found, and frequently accompanied with nephrotic syndrome, which is not sufficient to accept the condition as a specific disease entity such as "IgM Nephropathy" in our present study. Minimal lesion with a minimal but unmistakable deposit of lgA on the mesangium was noted less frequently and was accompanied or unaccompanied by nephrotic syndrome, a condition which call for an investigation clarify the characteristics and the extent of lgA(Berger's) nephropathy. Response to steroids in minimal lesion nephrotic syndrome was better in children and in the cases without mesangial proliferation, but was unrelated to either hematuria or immunoglobulin deposit. However, the cases with mesangial proliferation are significantly lesser in therapeutic response. Transformation to another morphological type of original glomerular change during follow-up was not observed in 4 available cases of minimal lesion nephrotic syndrome. Henoch-Sch nlein purpura was seen more commonly in children, and lgA(Berger's) nephropathy more commonly in adults.
Adolescent ; Adult ; Cell Division* ; Child ; Complement/analysis* ; Female ; Fluorescent Antibody Technique ; Glomerular Mesangium/pathology* ; Human ; Immunoglobulins/analysis* ; Male ; Nephrosis, Lipoid/pathology* ; Nephrotic Syndrome/pathology*

No abstract available.
Adenocarcinoma, Papillary* ; Thyroid Gland*

Of 7 cases of Guillain-Barre syndrome reviewed, which was admitted to the Department of Pediatrics Yonsei University, College of Medicine during 3 months period from July through September 1980, serologic surveys for an infectious agent were found to be without demonstrable causes such as cytomegalovirus and measles virus. We reviewed 87 acses of Guillain-Barre syndrome, which was admitted to our hospital during 10 years from January 1970 through December 1979. In age incidence, 57% of patients were under 5 years of age. Males were affected more often than females; the ratio was 2:1. In seasonal incidence, 59% of patients occured in summer time.
Cytomegalovirus ; Female ; Guillain-Barre Syndrome* ; Humans ; Incidence ; Male ; Measles virus ; Pediatrics ; Seasons