BACKGROUND: The combination of Philadelphia chromosome (Ph) and monosomy 7(-7) was rarely observed in acute lymphoblastic leukemia (ALL). With the results from immunophenotyplc and molecular analysis, Philadelphia chromosome positive ALL with monosomy 7[Ph(+)/-7] has been considered that it may be derived from neoplastic transformation at the pluripotent stem cell level. We compared the clini-cal, laboratory, and hematological findings between 5 cases of Ph(+)/-7 and 5 cases of Ph(+) without monosomy 7 [Ph (+) /N7]. METHODS: During the period from January, 1995 to December, 1996, total 72 cases of ALL were confirmed among 259 cases of hematologic malignancy with bone marrow cytogenetic analysis. Among 72 ALL cases, 5 cases of Ph(+)/-7(monosomy 7 or 7q abnormalities) were compared with Ph only or Ph without monosomy 7(ph(+)/N7] on the hematological, immunophenotypic, other laboratory, clinical findings and event ree survival (EFS) The karyotyping of the bone marrow specimens was analysed byshort-term unsynchronized culture methods such as overnight colcemid treatment and 24 hours incubation following ethidium bromide treatment. RESULTS: The mean age of Ph(+)/-7 was 30.6+/-12.8 years, and it was significantly different from that of Ph(+)/N7 (p=0.009), Four cases of Ph(+)/-7 were classified as ALL L2 subtype, and 2 cases revealed CNS involvements. Immunophenotyping was positive in CD10, CDl9, CD2O, CD22 and HLA-DR. But one case revealed e-B-lymphoid lineage with positivity in CD34, CDl3, and CD33. The response to chemotherapy and EFS was very poor in Ph(+)/-7 group, and the mean EFS was 3.2+/-1.9 months(p=0.014). All of cases showed induction on failure in chemotherapy, relapsed with bone marrow, CNS and extramedullary involvements, and expired due to sepsis. CONCLUSIONS: Ph(+)/-7 ALL had very Poor clinical course with being resistant to chemotherapy and unfavorable prognosis, revealed L2 subtype by FAB classification, and was slightly older in ages compared with Ph(+)/N7 ALL.
Bone Marrow ; Classification ; Cytogenetic Analysis ; Demecolcine ; Drug Therapy ; Ethidium ; Hematologic Neoplasms ; HLA-DR Antigens ; Hydrogen-Ion Concentration ; Immunophenotyping ; Karyotyping ; Monosomy* ; Philadelphia Chromosome* ; Pluripotent Stem Cells ; Precursor Cell Lymphoblastic Leukemia-Lymphoma* ; Prognosis ; Sepsis

In male reproducible health, fertility and IVF (in-vitro fertilization), semen analysis has been most important. Semen analysis can be divided into concentration, motional and morphological analysis of sperm. The existing method which was developed earlier to analyze semen concentrated on the sperm motility analysis. To provide more useful and precise solutions for clinical problems such as infertility, semen analysis must include sperm morphological analysis. But the traditional tools for semen analysis are subjective, imprecise, inaccurate, difficult to standardize, and difficult to reproduce. Therefore, with the help of development of microcomputers and image processing techniques, we developed a new sperm morphology analyzer to overcome these problems. In this study the agreement on percent normal morphology was studied between different observers and a computerized sperm morphology analyzer on a slide-by-slide basis using strict criteria. Slides from 30 different patients from the SNUH andrology laboratory were selected randomly. Microscopic fields and sperm cells were chosen randomly and percent normal morphology was recorded. The ability of sperm morphology analyzer to repeat the same reading for normal and abnormal cells was studied. The results showed that there was no significant bias between two experienced observers. The limits of agreement were 4.1%~-3.8%. The Pearson correlation coefficient between readers was 0.79. Between the manual and sperm morphology analyzer, the same findings were reported. In this experiments the slides were stained by two different methods, PAP and Diff-Quik staining methods. The limits of agreement were 7.2%~-5.7% and 6.0%~-6.3%, respectively. The Pearson correlation coefficients ware 0.76 and 0.91, respectively. The limits of agreement was tighter below 20% normal forms. In the experiments of repeatability, 52 cells stained by PAP and Diff-Quik staining methods were analyzed three times in succession. Estimating pairwise agreement, the kappa statistic for the pairs were 0.76, 0.81, 0.86, and 0.75, 0.88, 0.88 respectively. In this study it was shown that there was good agreement between manual and computerized assessment of normal and abnormal cells. The repeatability and agreement per slide of computerized sperm morphology analyzer was excellent. The computer's ability to classify normal morphology per slide is promising. Based on results obtained, this system can be of clinical value both in andrology laboratories and IVF units.
Andrology ; Bias (Epidemiology) ; Fertility ; Humans ; Infertility ; Male ; Microcomputers ; Semen ; Semen Analysis ; Sperm Motility ; Spermatozoa*

During the period of 4 years from May, 1981 to April, 1985, 71 cases of obstructing subinfundibular muscular bundles of the right ventricle in congential cardiovascular anomalies were diagnosed by cariac catheterization and angiography at Hanyang University. The finding of right ventricular angiogram and degree of the right ventricular outflow obstruction, clinical data, electrocardiographic data and hemodynamic data were correlated irrespectively. The summary of this article is as follows. 1) The age of patients was ranged from 1 to 22 years old with a mean age of 8 years old. There were 39 males and 32 females with M:F ratio of 1.2:1. The incidence of obstructing subinfundibular muscular bundles of the right ventricle in congenital cardiovascular anomalies was higher with increasing age. 2) The associated cardic anomalies were as follows: 40 cases (56%) of isolated ventricular septal defect(VSD), 13(18%) of tetralogy of Fallot physiology, 7(10%) of patent ductus arteriosus(PDA), 3(4%) of pulmonary stenosis, 1 aortic stenosis, 1 double outlet of right ventricle(DORV), 1 trilogy, 1 ostium secundum defect, etc. The incidence of VSD with or without other associated cardiovascular anomalies was 56 cases(79%) out of 71 cases. 3) Maximum systolic pressure gradient between proximal and distal chamber of the right ventricle were under 25 mmHg in 32 cases, between 25 and 50 mmHg in 13 and above 50 mmHg in 26. Pressure gradients of all 7 cases with PDA were under 25 mmHg. 4) Correlative assesment of angiographic manifestation(2 indicies:Diameter of right ventricular outflow tract(systolic phase)/diameter of tricuspid valvular annulus(diastolic phase)=OT/TV, Diameter of right ventricular outflow tract(systolic phase)/Length of right ventricular diaphragmatic surface(systolic phase)=OT/RV) according to pressure gradient, OT/Tv and OT/RV values were lower the increasing pressure gradient, between proximal and distal chamber of the right ventricle. These were reverse correlations but coefficients of correlation(r) were-0.49and -0.48. Therefore, the degree of right ventricular outflow obstruction could be predicted, using 2 indices of right ventricular angiogram in individual cases, but could not be calculated accurately. 5) This indicated that pressuer gradient was also affected by technical errors, variable cardic anomalies, development of sinusoid, age and the other factors. 6) We assumed that non-invasive Doppler echocardiography could be useful in making the diagnosis and follow up of the patient with obstructing subinfundibular muscle bundles in right ventricle.
Angiography ; Aortic Valve Stenosis ; Blood Pressure ; Catheterization ; Catheters ; Child ; Diagnosis ; Echocardiography, Doppler ; Electrocardiography ; Equidae ; Female ; Heart Ventricles* ; Hemodynamics ; Humans ; Incidence ; Male ; Physiology ; Pulmonary Valve Stenosis ; Tetralogy of Fallot ; Ventricular Outflow Obstruction ; Young Adult

No abstract available.
Cholestasis, Intrahepatic*

Osteochondromadevelop most commonly at distal femur, proximal humerus and proximal tibia, but the rib osteochondroma was reported less commonly. In this report, scapular snapping syndrome complicated by adventitious bursa and soft tissue pseudotumor surrounding the osteochondroma of the 6th rib body was treated successfully by surgical excision of them. We report this rare case with reviewing the relevant literature.
Femur ; Humerus ; Osteochondroma* ; Ribs* ; Shoulder* ; Tibia

Microsatellites are short nucleotide repeat sequences present throughout the human genome. Alterations of microsatellites, comprising extra or missing copies of these se quences, have been termed microsatellite instability(MSI, genetic instability, replication errors, RER(+) phenotype). To date, at least four genes involved in DNA mismatch repair, hMSH2, hMLH1, hPMS1 and hPMS2, are thought to account for the observation of microsatellite instability in tumor from Hereditary nonpolyposis colorectal cancer (HNPCC) patients. The genetic defect responsible for the MIN+ phenotype in sporadic colorectal cancer, however, has yet to be clearly delineated. The purpose of this study was to determine the presence of MSI in sporadic cancer and to correlate its occurrence with clinicopathological parameters, we have studied six microsatellite loci by use of polymerase chain reaction amplification and denaturing polyacrylamide gel electrophoresis. We found that 20%(9 of 46 cases) sporadic colorectal cancers showed RER at two or several loci(RER+). Microsatellite instability was associated with location of the tumor in the proximal colon 66%(6 of 9 cases) and with poorly differentiated tumor phenotype 56%(5 of 9 cases). In order to better understand the role of somatic alterations within hMSH2 in the process of colorectal tumorigenesis, we examined the most conserved regions(codon 598~789) of this gene in nine patients with MIN spotadic colorectal cancer. 6 patient of RER(+) colorectal ca. patients had a polymorphism which was a T to C base change in the intron sequence at -6 position of the splice acceptor site at the 5'end of exon 13. This particular sequence variation is a polymorphism rather than a mutation which increase cancer susceptability. These data suggest that the genetic instability is detect ed in some colorectal cancers and play an important role in the pathogenesis of sporadic colorectal cancer.
Carcinogenesis ; Colon ; Colorectal Neoplasms* ; Colorectal Neoplasms, Hereditary Nonpolyposis ; DNA Mismatch Repair ; Electrophoresis, Polyacrylamide Gel ; Exons ; Genome, Human ; Humans ; Introns ; Microsatellite Instability* ; Microsatellite Repeats* ; Phenotype ; Polymerase Chain Reaction ; RNA Splice Sites

No abstract available.
Hemolytic-Uremic Syndrome* ; Mycoplasma Infections* ; Mycoplasma*

No abstract available.

No abstract available.

Many methods of correcting secondary cleft lip nose deformity have been introduced, but a single best method that can be uniformly adjusted for all cases does not exist. Since it is difficult to make the nostril of the cleft side symmetric with that of normal side, the authors tried to formulate the basis for correction of unilateral secondary cleft lip nose deformity according to the varying amounts of deformed nostril. We then adopted 4 different methods of correcting the soft tissue and nostril deformity in 63 patients from 1996 to 1998 and surveyed the results. Our principles were as follows: 1) Alar rim incision was done in cases of different-shaped cleft-side nostrils with nearly the same measured area in comparison to the normal side. 2) When deformity of the alar-columellar web was severe, we performed modified Z-plasty. 3) When the nostril of the cleft-side nostril was smaller than the normal side with slight asymmetry, reverse W-plasty was done. 4) We adopted a reverse-U incision when there was a discrepancy in height with moderate asymmetry compared with the normal nostril. After dissection, repositioning of alar cartilage was done by same method in each case. A nasal stent was kept in position for at least 6 months postoperatively in almost all cases. After follow-up of 6 to 24 months, we concluded that our choice was correct in selecting the optimal operative method, considering the characteristics of deformity of the cleft side nostril in an effort to make it symmetric with that of the normal side.
Cartilage ; Cleft Lip* ; Congenital Abnormalities* ; Follow-Up Studies ; Humans ; Nose* ; Stents