No abstract available.
Chromosome Aberrations*

BACKGROUND: Hemophagocytic lymphohistiocytosis(HLH) is a rare and fatal disorder in children. Recently its clinical characteristics and etiology of uncontrolled activation of cellular immune system in genetically predirected patients have keen elucidated. The authors analyzed the clinical characteristics and response to immunosuppressive agents of HLH patients in single institute. METHODS: The authors retrospectively analyzed various clinical data including CSF and bone marrow examination at diagnosis and follow up period in the 6 patients who were diagnosed as HLH at Asan Medical Center during last 2 years. Antithymocyte globulin(ATG : 10 mg/kg/day) and methylprednisolone(methyl-PD: 5 mg/kg/day) for 5 consecutive days as induction treatment and cyclosporin A(CsA) as maintenance treatment after induction with weekly intrathecal methotrexate for 5-6 weeks were given to the recently diagnosed 3 patients. RESULTS: All the patients except one were infants. Persistent fever, hepatosplenomegaly and pancytopenia were observed in all the patients. Family history of suspicious HLH was observed in 4 patients(67%). The characteristic elevated serum triglyceride(TG) level and/or decreased fibrinogen level were noted in all. Mild to moderate CSF pleocytosls with increased lymphocytes and monocytes was also observed in all during the disease course. Variable degree of nonqr-Langerhans cell histiocytic infiltration and hemophagocytosis were observed in all the cases. Of the 3 patients treated with ATG and methyl-PD, one achieved complete remission and the others achieved partial remission within 2 weeks of treatment, but all expired after 5 months, remission with CsA. Regardless of treatment regimen, all the 6 patients expired due to CNS sequelae of HLH. CONCLUSIONS: HLH mainly developed in infants. Persistant fever, hepatosplenomegaly and pancytopenia were observed in most cases with the characteristic change of serum TG and/or fibrinogen level. HLH should be included in the differential diagnosis in patients with these features especially when the family history of suspicious HLH is present, and histologic comfirmation of HLH could be easily accomplished with bone marrow study in most cases. Remission induction of HLH could be achieved with immunosuppressive treatment but it was difficult to maintain long term remission.
Bone Marrow ; Bone Marrow Examination ; Child ; Chungcheongnam-do ; Cyclosporine ; Diagnosis ; Diagnosis, Differential ; Fever ; Fibrinogen ; Follow-Up Studies ; Humans ; Immune System ; Immunosuppressive Agents ; Infant ; Lymphocytes ; Lymphohistiocytosis, Hemophagocytic* ; Methotrexate ; Monocytes ; Pancytopenia ; Remission Induction ; Retrospective Studies

No abstract available.
Adrenocortical Carcinoma* ; Female* ; Humans

BACKGROUND: Despite the development of empiric antibiotics in treatment of neutropenic fever(NF) in pediatric cancer patients, bacterial infection is still the most important cause of death in these patients. In this study the authors analyzed clinical characteristics and tried to find out the possible risk factors of the pediatric cancer patients who succumbed to the documented bacterial infection after episodes of neutropenic fever. METHODS: 17 pediatric cancer patients expired after episodes of neutropenic fever(NF) in the pediatric department of Asan Medical Center from Mar. 1990 to Feb. 1996 were grouped by the results of bacterial culture. 7 cases (Group A) had documented bacterial culture results and 10 cases (Group B) had negative culture results. The clinical characteristics of these two groups were analyzed retrospectively. RESULTS: There were no differences in the types of cancer between the two groups. All the documented bacteria were gram negative organisms and all cultured from the blood. There were no differences between the two groups in the treatment duration, known risk factors of infection before and at the onset of fever, antibiotics administered, and interval from the onset of NF to the administration of antibiotics. The response rate of initial empiric antibiotics was lower in group A(14%) compared to Group B(40%), even though all the cultured organisms except 2 cases in Group A were sensitive to initial empirical antibiotics. Septic shock was the cause of death in 86% of Group A patients, but only in 10% of Group B patients. The other causes of death were progression of cancer, bleeding, hepatic failure, adult respiratory distress syndrome and multiple organ failure, which showed no difference between the 2 groups. The interval from the onset of fever to death was significantly shorter in Group A(6.2+/-2.3 days) compared to Group B(24.9+/-18.6 days). CONCLUSIONS: Bacterial infection is still the most frequent cause of death after NF. Most patients succumbed to the documented bacterial infection showed rapid progression of bacteremia to septic shock despite administration of sensitive antibiotics. The known risk factors of infection before the onset of NF and other treatment factors shows no differences between the two groups in this study. These results suggest that the bacteremia is obvious risk factor of poor outcome after NF episode, and antibiotics alone may be insufficient to prevent the rapid progression of septic shock in these patients.
Anti-Bacterial Agents ; Bacteremia ; Bacteria ; Bacterial Infections ; Cause of Death* ; Chungcheongnam-do ; Fever* ; Hemorrhage ; Humans ; Liver Failure ; Multiple Organ Failure ; Respiratory Distress Syndrome, Adult ; Retrospective Studies ; Risk Factors ; Shock, Septic

BACKGROUND AND PURPOSE: We had previously demonstrated by immunohistochemical study (IHS) that nm23-H1 gene may play an important role in disease prognosis as well as its participation in metastasis of urothelial cancer. The purpose of present study was 1) to reexamine the role of nm23-H1 gene in urothelial cancers at molecular level, 2) to identify the molecular mechanism of decreased immunoreactivity for nm23-H1 protein in metastatic urothelial cancers, and 3) to identify whether IHS is reliable in studying the expression of nm23-H1. MATERIALS AND METHODS: We studied expression level and mutation profiles of nm23-H1 gene in 25 fresh surgical specimens of urothelial cancer by reverse transcription polymerase chain reaction(RT-PCR)-Southern blotting analysis, and PCR of genomic DNA followed by single strand conformation polymorphism and sequencing analysis. The results of RT-PCR-Southern blotting were comparatively analyzed with those of IHS. RESULTS: mRNA transcript levels of nm23-H1 gene were significantly decreased in tumor tissues with metastasis as compared with those without metastasis. The transcript levels of nm23-H1 gene were also significantly decreased in metastatic tumor tissues as compared with primary tumor tissues. Point mutation of nm23-H1 gene was detected in only 1 of 13 urothelial cancer tissues with metastasis, whereas, mutation was observed in none of those without metastasis. The results of IHS corresponded with those of RT-PCR-Southern blotting analysis in 23 of 25 specimens. CONCLUSIONS: The nm23-H1 gene may play an important role in metastasis of urothelial cancer. Decreased transcription at mRNA level may be a major molecular mechanism of loss of immunoreactivity for nm23-H1 protein in urothelial cancer. IHS used in the present study may be a clinically useful method to study the expression of nm23-H1 and to predict metastasis potential and prognosis of urothelial cancers.
DNA ; Neoplasm Metastasis ; Point Mutation ; Polymerase Chain Reaction ; Prognosis ; Reverse Transcription ; RNA, Messenger ; Urologic Neoplasms*

BACKGROUND: To investigate the possibie difference, if any, between office blood pressure(BP) and home BP may be important in the diagnosis and treatment of hypersensive patients. This report deails the difference between the two BP's and the usefulness of digital electronic sphygmomanometer(DES) for self-monitoring of home BP. METHODS: The BP's of 14 patients with essential hypertension were measured with mercury sphygmomanometers at outpatient department by physician and with DES at home(twice a day) by the patients. Patients were followed up every 2 weeks for 4 weeks and previous 2 weeks' average home BP's were compared with the office BP's of each 2 weeks' end. RESULTS: There was a significant difference between office and home BP(both systolic and diastolic) ; office average BP(151/95mmHg) was higher than home average BP(136/86mmHg). CONCLUSION: Caution may be needed in the interpretation of office BP unless it is measured several times after adequate rest.
Blood Pressure* ; Diagnosis ; Humans ; Hypertension ; Outpatients ; Sphygmomanometers*

No abstract available.

To elucidate the complex of insulin-like growth factor binding proteins (IGF-BPs) in short stature patients, we carried out a prospective study on three patients who were diagnosed as complete GH deficiency at the department of pediatrics from July 1992 to June 1993. The results were summarized as follows: 1) Two circulating IGFs complexed to specific binding protein existed in normal serum. Binding activity was found to be in the 150,000 molecular weight area (the large complex) and 50~60,000 molecular weight area (the small complex). 2) Binding activity for the large complex was seen to be dependent on advancing age, level of large IGF-BP3 complex peacked at the age of 15~16 years. 3) The binding activity for large complex diminished in three GH deficient patients and increased after hGH injection to near or above normal level. 4) Increased growth rate after GH treatment in GH deficient patient was closely related with increasing level of the large IGF-BP3 complex. Therefore we suggest that the large IGF-BP3 complex is regulated by GH. Estimating its serum level is useful for screening of GH deficiency and the monitoring of response to GH therapy.
Carrier Proteins ; Growth Hormone* ; Humans ; Insulin-Like Growth Factor Binding Proteins ; Insulin-Like Growth Factor I* ; Mass Screening ; Molecular Weight ; Pediatrics ; Prospective Studies

No abstract available.

OBJECTIVES: The aim of this study was to compare root canal volume change and canal transportation by Vortex Blue (VB; Dentsply Tulsa Dental Specialties), ProTaper Next (PTN; Dentsply Maillefer), and ProTaper Universal (PTU; Dentsply Maillefer) nickel-titanium rotary files in curved root canals. MATERIALS AND METHODS: Thirty canals with 20°–45° of curvature from extracted human molars were used. Root canal instrumentation was performed with VB, PTN, and PTU files up to #30.06, X3, and F3, respectively. Changes in root canal volume before and after the instrumentation, and the amount and direction of canal transportation at 1, 3, and 5 mm from the root apex were measured by using micro-computed tomography. Data of canal volume change were statistically analyzed using one-way analysis of variance and Tukey test, while data of amount and direction of transportation were analyzed using Kruskal-Wallis and Mann-Whitney U test. RESULTS: There were no significant differences among 3 groups in terms of canal volume change (p > 0.05). For the amount of transportation, PTN showed significantly less transportation than PTU at 3 mm level (p = 0.005). VB files showed no significant difference in canal transportation at all 3 levels with either PTN or PTU files. Also, VB files showed unique inward transportation tendency in the apical area. CONCLUSIONS: Other than PTN produced less amount of transportation than PTU at 3 mm level, all 3 file systems showed similar level of canal volume change and transportation, and VB file system could prepare the curved canals without significant shaping errors.
Dental Pulp Cavity ; Humans ; Molar ; Transportation ; X-Ray Microtomography