Progressive Muscular Dystrophy is a hereditary disorder characterized by progressive weakness and wasting of muscules. The etiology of muscular dystrophy is unknown, and no from of pharmacological treatment is considered effective. We report 2 cases of progressive muscular dystrophy occuring in a family, which were diagnosed by clinical findings, serum enzyme study and electromyography.
Electromyography ; Humans ; Muscular Dystrophies

No abstract available.
Brain* ; Central Nervous System Diseases* ; Central Nervous System* ; Child* ; Humans

No abstract available.
Brain* ; Central Nervous System Diseases* ; Central Nervous System* ; Child* ; Humans

Total anomalous pulmonary venous return(connection) is a congenital heart disease. Especially, the reports of the patients with double connections of all pulmonary veins were rare. An infant who underwent operation for total anomalous pulmonary venous return had double drainge of all pulmonary veins to both left vertical vein and anomalous descending vein. This second lesion was not diagnosed preoperatively, but in operative field. Operative treatments were performed by ligation of left vertical vein and central pulmonary vein-to-left atrium anastomosis without ligation of the descending anomalous vein. For these less common variants fo total anomalous pulmonary venous return,a more detailed and precise understanding of morphology can facilitate accurate surgical repair. And also, early detection of the disease is an another issue for a good prognosis.
Heart Defects, Congenital ; Humans ; Infant ; Ligation ; Prognosis ; Pulmonary Veins ; Scimitar Syndrome* ; Veins

No abstract available.
Tuberous Sclerosis*

No abstract available.
Animals ; Inositol* ; Kidney* ; Liver* ; Phospholipases* ; Rats* ; Type C Phospholipases*

The Clufoot is still controversial in the etiology, the pathology, and the method of the treatment. Turco issued one stage posteromedial soft tissue release in 1971, which is still widely used. But the Turco's procedure is not satisfactory results in forefoot adduction and ankle motion. McKay and Simon were performed one stage complete subtalar soft tissue release and reported better results compared with those who had posteromedial release. So we performed Turco's posteromedial release with lateral release by Cincinnati incision in 12 patients (20 feet) and analysis the results from Oct. 1987 to Jul. 1989 at the department of orthopaedic surgery of CNUH. The results were as followings ; 1. The mean age of the patient at first examination was 1 year 9 months (range from at birth to 5 year 10 months) and the mean age at operation was 2 year 1 month (range from 4 months to 5 year 10 months). 2. The clinical results were excellent in 2 feet, good in 14 feet, fair in 3 feet and unsatisfactory in 1 foot. 3. The radiological results were satisfactory in 18 feet and unsatisfactory in 2 feet. 4. Forefoot adduction was corrected in 14 feet (70%) as normal range, 3 feet as acceptable, and 3 feet as unacceptable 10' over the normal range. 5. Two feet of hind foot overcorrection result was operation as McKay's method. 6. The Clincinnati incision was favorable to visulization and release of the posteromedial, anteromedial and posterolateral structure of the foot.
Ankle ; Clubfoot ; Foot ; Humans ; Methods ; Parturition ; Pathology ; Reference Values

BACKGROUNDS: The Rh antigens are important m clinical practice. The classification of Rh phenotype is usually based on the antigen detection done by conventional serologic method, but it has many limitation such as delicate grading of antigen expression. Recently, Flowcytometry has been introduced in immunohematology to detect and quantitate cell bound immunoglobulins to assess blood cell antigens and related antibodies. So, we tried these method to detect Rh(D) antigen and measure its density and evaluated the possibility of clinical usage. MATERIALS AND METHODS: We performed a flowcytometric analysis for the expression of D antigen in D-positive, negative and weak D group in indirect immune fluorescence assay by using polyclonal antibodies. We measured the intensity of immunofluorescence as a degree of antigen density and analysed the difference of mean channel fluorescence value(MCF) among these groups. RESULTS: Weak D groups had the lower fluorescence than D-positive group, while D-negative sample showed the same fluorecence as negative ntrol. The difference of fluorescence intensity of D antigen between that of C antigen were not observed and were statistically insignificant. CONCLUSION: Flowcytometry appear to be a good tool for antigen density measurement in immunohematologic areas and shows the possibility of application to other aspect.
Antibodies ; Blood Cells ; Classification ; Fluorescence ; Fluorescent Antibody Technique ; Immunoglobulins ; Phenotype

Widely used tests for the detection of platelet antibodies in Korea include platelet suspension immunofluorescence test(PSIFT), enzyme immunoassay and mixed passive hemagglutination(MPHA). In these tests, removal of HLA antigens from platelet are required to detect platelet-specific antibodies. Modified antigen capture ELISA(MACE) is known to be very sensitive for the detection of platelet-specific antibodies, in which specific platelet glycoprotein, captured by the monoclonal antibody is used as a target antigen. MACE is very useful for the detection of platelet-specific alloantibodies in neonatal alloimmune thrombocytopenia(NAIT) and posttransfusion purpura(PTP). We employed MACE in our laboratory, using AP2(anti-GPIIb/IIIa, monoclonal), #30 sera(anti-PlA1), 90-545 sera(anti-HLA-B51+52) and LYS sera(multispecific HLA antibodies). LYS sera had been used as our positive control( 1:120) in MPHA. Platelet from PIA1(+), HLA-B5 I, blood group O healthy male donor, gave positive result with #30 sera(1:40) and negative result with 90-545 sera in MACE. With LYS sera, MACE showed negative in 1:120, but positive in 1:20. So LYS sera was thought to contain strong multispecific HLA antibodies and relatively weak antibody(-ies) reacting with GPllb/Illa. Further studies employing different monoclonal antibodies, such as anti-GPIb/IX, -GPIa/Ila and -GPIV are under way.
Antibodies* ; Antibodies, Monoclonal ; Blood Platelets ; Fluorescent Antibody Technique ; Glycoproteins ; HLA Antigens ; Humans ; Immunoenzyme Techniques ; Isoantibodies ; Korea ; Male ; Tissue Donors

The renal abscess is a rare disease in neonate. The clinical findings consist of non- specific symptoms such as fever, lethargy, flank mass and laboratory abnormalities including leukocytosis, elevated CRP, ESR and pyuria. The diagnosis can be made with ultrasonography, CT scan and DMSA. The renal abscess involving corticomedullary region is known to be frequently associated with urologic abnormality and can progress into perirenal abscess which has been reported to have a poor prognosis. The treatments include antibiotic therapy, drainage of abscess and surgical intervention. We report a neonate with bilateral renal abscesses with underlying vesicoureteral reflux grade V which was successfully treated with antibiotic therapy and drainage of abscess.
Abscess* ; Diagnosis ; Drainage ; Fever ; Humans ; Infant, Newborn* ; Lethargy ; Leukocytosis ; Prognosis ; Pyuria ; Rare Diseases ; Succimer ; Tomography, X-Ray Computed ; Ultrasonography ; Vesico-Ureteral Reflux