Congenital adrenal hyperplasia is inherited disorder of adrenal steroidogenesis. 21-hydroxylase deficiency is the most commone enzymatic defect and is divided into classic and late-onset or nonclassic forms. Both classic non-classic 21-hydrozylase deficiencies are inherited in a recessive manner as allelic variants. But it is rare that happened in twin infants. Chief complaints of affected twins in our case were ambiguous genitalia, hyperpigmentation and dehydrations. They were revealed into hyponatremia, hyperkalemia and increased amount of serum progesterone, 17-hydroxyprogesterone and urinary 17-ketosteroid excretion and were administered with DOCA, 9alpha-fluorohydrocortisone, hydrocortisone to control the electrolyte imbalance. And now, both of them are going to normal ratio of weight gain and body growth.
17-alpha-Hydroxyprogesterone ; Adrenal Hyperplasia, Congenital* ; Desoxycorticosterone Acetate ; Disorders of Sex Development ; Humans ; Hydrocortisone ; Hyperkalemia ; Hyperpigmentation ; Hyponatremia ; Infant ; Progesterone ; Steroid 21-Hydroxylase* ; Twins* ; Weight Gain

No abstract available.
Tuberculosis, Pulmonary*

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

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*

The diagnosis of soft tissue osteochondroma should be considered when a well-defined osseous mass is located in the soft tissues. The differential diagnosis includes myositis ossificans, tumoral calcinosis, synovial chondromatosis, and soft tissue osteosarcoma, true osteochondroma which arises from bone. One case of soft tissue osteochondroma in the knee, a lesion of uncertain pathogenesis is reported.
Calcinosis ; Chondromatosis, Synovial ; Diagnosis ; Diagnosis, Differential ; Knee ; Myositis Ossificans ; Osteochondroma* ; Osteosarcoma

The chest X-ray findings of 20 cases of pulmonary opportunistic infections were analyzed according tocausative agents. The results we as follows; 1. Final diagnoses of 20 cases of opportunistic infections weretuberculosis in 6 cases, pneumocystis carinii pneumonia in 5 cases, bacterial infection in 7 cases, and fungalinfection in 2 cases. 2. The underlying diseases were leukemia in 6 cases, kidney transplantation in 6 cases,lymphoma in 3 cases, nephrotic syndrome in 1 case, nasopharyngeal cancer in 1 case, multiple myeloma in 1 case,agranulocytosis in 1 case, and hypogammaglobulinemia in 1 case. 3. In tuberculosis, all the 6 cases showed severemanifestations such as miliary tuberculosis, tuberculous pneumonia, moderately advanced tuberculosis andtuberculous pericarditis. 4. In pneumocystis carinii pneumonia, the most frequent findings were bilateral alveolardensities and peripheral field of the lung was saved in most cases. 5. In 2 cases of fungal infections bilatarealmultiple cavitary nodules were noted. 6. In cases of bacterial infection there were more cases of gram negativeinfection than gram positive and 2 cases of pseudomonas revealed bilateral multiple cavitary nodules.
Agammaglobulinemia ; Bacterial Infections ; Diagnosis ; Kidney Transplantation ; Leukemia ; Lung ; Multiple Myeloma ; Nasopharyngeal Neoplasms ; Nephrotic Syndrome ; Opportunistic Infections ; Pericarditis ; Pneumonia ; Pneumonia, Pneumocystis ; Pseudomonas ; Thorax ; Tuberculosis ; Tuberculosis, Miliary

No abstract available in English.

Successful repair of lacerated tendons must restore continuity of the tendon and should yield a strong tenorrhaphy. Mechanical strength of repair should be adequate to early postoperative motion and mobility, The optimal repair technique must be able to withstand the rigors of early motion and also must not interfere with tendon healing. The relative strength of three suture methods of lacerated tendon were measured by mechanical disruption in effort to determine the strength of suture technique. Fifty-four Calcaneal tendons of 27 the New Zealand white rabbit were transected at mid portion and repaired with the three suture technique: group 1, Kessler suture, group 2, Pennington’s modified-Kessler suture and group 3, augmented- Becker suture technique. Each group was composed of 18 calcaneal tendons. Nine rabbits were sacrified immediately after suture, nine in postoperative 2 weeks and nine in postoperative 4 weeks Six calcaneal tendons in each three experimental group were obtained immediately after suture, at postoperative 2 and 4 weeks respectively. Tensile strength, maximum strength and modulus of elasticity of all experimental specimens were measured with Instron-UTM-4-100(Toyo-Baldiwin, Japan). The results were evaluated statistically to compare the strength of the three suture technique at three different periods. The tensile strength was predominantly strongest in augmented-Becker method among three suture technique at immediate suture, postoperative 2 weeks and 4 weeks respectively. The augmented Becker repair was strongest in maximum stress among Kessler and modified Kessler repair at immediate operation, postoperative 2 weeks and 4 weeks respectively. The augmented Becker repair was highest in modulus of elasticity than Kessler method and modified-Kessler method at immediate operation, postoperative 2 weeks and postoperative 4 weeks respectively. Tensile strength, maximum stress and modulus of elasticity were gradually increased from immediate operation to postoperative 4 weeks, but there were not statistically significance between experimental three suture methods at postoperative 4 weeks.
Elastic Modulus ; Methods ; New Zealand ; Rabbits ; Suture Techniques ; Sutures ; Tendons ; Tensile Strength

BACKGROUND: The importance of extended-spectrum beta-lactamases (ESBL) produced in gramnegative bacilli is now well recognized, but most clinical laboratories have problems in detecting and interpreting ESBL and implicating the findings in nosocomial infections caused by ESBL producing gram-negative bacilli. The present study aims primarily to evaluate the distributions of these enzymes among Escherichia coli and Klebsiella pneumoniae, the most frequent isolates of Enterobacteriaceae producing ESBL, to differentiate the types of enzymes in theses isolates and finally to relate the clonality of specific types within a part of Daegu city. METHODS: The clinical isolates of 1, 242 E. coli and 859 K. pneumoniae were screened for ESBL production by the disk diffusion method of the National Committee of Clinical Laboratory Standard, and it was confirmed by the double-disk synergy test (DDS). Antimicrobial susceptibility test was performed by the agar dilution method. The presence of -lactamase was tested by polymerase chain reaction (PCR) and plasmid analysis. Isoelectric focusing and nucleotide sequence analysis were performed to evaluate ESBL types. Pulsed-field gel electrophoresis (PFGE) of XbaI-digested DNA fragments was carried out to determine the extend of clonality within the hospital. RESULTS: Of 34 isolates of E. coli and 31 isolates of K. pneumoniae ramdomly selected from those isolates screened for ESBL production were further tested by DDS to confirm its production: 30 (88.2%) E. coli and 29 (93.5%) K. pneumoniae were positive. TEM-52 and SHV-12 were present both in E. coli and K. pneumoniae, but SHV-2a was distributed only in K. pneumoniae. The resistance was transferable in 66.7% of E. coli and 68.9% of K. pneumoniae. Six and 5 PFGE patterns were shown by E. coli and K. pneumoniae, respectively. Among the 5 patterns of K. pneumoniae, type B was dominant, suggesting a clonal outbreak in the hospital. CONCLUSIONS: The ESBL specific enzyme types were TEM-52, SHV-2a and SHV-12. Despite many different PFGE patterns of the ESBL producing isolates, a few outbreak and edemic clones appear to be prevalent in Dongsan Medical Center.
Agar ; Base Sequence ; beta-Lactamases* ; Clone Cells ; Cross Infection ; Daegu ; Diffusion ; DNA ; Electrophoresis, Gel, Pulsed-Field ; Enterobacteriaceae ; Escherichia coli* ; Isoelectric Focusing ; Klebsiella pneumoniae* ; Plasmids ; Pneumonia ; Polymerase Chain Reaction

Although occasional patients with chronic myeloid leukemia (CML) have chromosomal changes other than Philadelphia chromosome early in the disease, in typical cases the 9;22 translocation remains the sole abnormality throughout the disease course in chronic phase. When disease progression occurs, however, 75-80% develop additional chromosome aberrations. These secondary changes sometimes precede the more aggressive manifestations hematologically and clinically and thus may serve as valuable prognostic indicators. ider (9) (q10)t (9;22) (q34;q11.2) is very rare and a recurrent chromosomal abnormality associated with acute lymphoblastic leukemias (ALL) and lymphoblastic crisis of CML. And ider (9) (q10)t (9;22) (q34;q11.2) is a lymphoid-specific rearrangement and the patients with this abnormality are of older age on average. They commonly show pre-B cell lineage immunophenotype and L2 morphology. We report a case of ider (9) (q10)t (9;22) (q34;q11.2) as secondary aberration in a patient with lymphoblastic crisis of CML.
Blast Crisis ; Chromosome Aberrations ; Disease Progression ; Humans ; Leukemia* ; Leukemia, Myelogenous, Chronic, BCR-ABL Positive ; Philadelphia Chromosome ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; Precursor Cells, B-Lymphoid