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.
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

No abstract available.
Blood Cell Count* ; Blood Cells* ; Precursor Cell Lymphoblastic Leukemia-Lymphoma* ; Prognosis* ; Remission Induction*

Background:Ghrelin is a new endogenous ligand for the growth hormone secretagogue receptor. It activates the release of growth hormone from the pituitary and it also participates in the regualtion of energy homeostasis. The aims of the study were to characterize the changes in plasma ghrelin levels in obese subjects compared with lean control or overweight subjects, and their relationship to various parameters in obese subjects. METHODS:In this study, 121 elementary school children were divided into 3 groups according to their body mass index (BMI). The lean control subjects consisted of 28 children who had less than 85 percentile of BMI. The overweight subjects consisted of 22 children who had 85-95 percentile of BMI. The obese subjects consisted of 71 children who had over 95 percentile of BMI. All subjects in 3 groups were evaluated according to their age, height, weight, obesity index, plasma ghrelin, serum lipid, glucose and insulin levels. Leu72Met mutation of prepro-ghrelin gene was directly detected by digesting the PCR fragments with Bsrl. RESULTS:Among antropometric data, body weight, BMI and obesity index were higher in obesity and overweight subjects than those of lean control subjects (P<0.05). The plasma ghrelin levels were significantly lower in overweight and obese subjects (P<0.05). In addition, serum triglyceride and LDL cholesterol levels were significantly higher in these groups compared to the control subjects (P<0.05). The concentrations of plasma ghrelin were significantly negatively correlated with BMI, obesity index, serum triglyceride, LDL cholesterol and insulin in all the children. However, there was no significant relationship between plasma ghrelin levels and any various parameters in obese subjects. Leu72Met mutation was detected in about 30% of obese children. However, we could not find any differences between lean control and obese children. CONCLUSION: We proved a significantly lower plasma ghrelin levels in overweight and obese subjects. Further studies are now needed to establish the role of ghrelin in the pathogenesis of human obesity.
Body Mass Index ; Body Weight ; Child* ; Cholesterol, LDL ; Ghrelin* ; Glucose ; Growth Hormone ; Homeostasis ; Humans ; Insulin ; Obesity* ; Overweight ; Plasma* ; Polymerase Chain Reaction ; Receptors, Ghrelin ; Triglycerides

BACKGROUND: Several recent studies have suggested the context-sensitive half-time (CSHT) as a clinically more relevant measure of decreasing drug concentrations after a constant infusion of a given duration. The purpose of this study was to simulate the CSHT using a PK-SIM computer simulation program and compare this with duration of propofol infusion and real awakening time from anesthesia for the evaluation of CSHT as a useful tool of prediction of recovery from anesthesia. METHODS: Ninety-five ASA class I or II adult patients (18-55 yrs) scheduled for orthopedic elective surgery were randomly allocated into 5 groups according to duration of propofol infusion. Five groups were Group 1: less than 60 min, Group 2: 61-120 min, Group 3: 121-180 min, Group 4: 181-240 min, and Group 5: 241-300 min. Anesthesia was induced and maintained with propofol-nitrous oxide (67%)-oxygen (33%) according to Prys-Roberts' method. Propofol was discontinued immediately after skin closure and duration of infusion was checked. Duration of infusion, CSHT, and awakening time from anesthesia were compared and evaluated correlationship among them using a correlation coefficient. RESULTS: Plasma and effect site concentration after 5 hrs infusion of propofol derived from computer simulation was 3.3 microgram/ml. Awakening time for each group was 8.5-11.8 min and plasma CSHT for that was 6.4-9.5 min. The correlation coefficient (r) between duration of propofol infusion and awakening time was 0.98 (p<0.01) and that between CSHT and awakening time was 0.95 (p<0.01). CONCLUSIONS: There were strong relationship among duration of infusion of propofol, awakening time and CSHT. It seems that CSHT could be a good predictor of awakening from propofol-N20-02 general anesthesia.
Adult ; Anesthesia* ; Anesthesia, General ; Computer Simulation* ; Humans ; Orthopedics ; Plasma ; Propofol* ; Skin

No abstract available.
Gaucher Disease* ; Humans ; Siblings*

PURPOSE: Gelatin-hydroxyapatite nanocomposite is similar to inorganic nanostructure of bone. To make a scaffold with osteoinductivity, bone marrow derived stem cells from rabbit femur were impinged into the nanocomposite. This vitro study was to test osteogenic differentiation of the stem cells in the nanocomposite, which was made by authors. MATERIAL & METHODS: Gel-HA nanocomposite with 10g of HA, 3 g of Gel has been made by co-precipitation process. Bone marrow was obtained from femur of New Zealand White rabbits and osteogenic differentiation was induced by culturing of the BMSCs in an osteogenic medium. The BMSCs were seeded into the Gel-HA nanocomposite scaffold using a stirring seeding method. The scaffolds with the cells were examined by scanning electron microscopy (SEM), colorimetry assay, biochemical assay with alkaline phosphatase (ALP) diagnostic kit, osteocalcin ELISA kit. RESULTS: Gel-HA nanocomposite scaffolds were fabricated with relatively homogenous microscale pores (20-40 micrometer). The BMSCs were obtained from bone marrow of rabbit femurs and confirmed with flow cytometry, Alizarin red staining. Attachment and proliferation of BMSCs in Gel-HA nanocomposite scaffold could be identified by SEM, ALP activity and osteocalcin content of BMSCs. CONCLUSION: The Gel-HA nanocomposite scaffold with micropores could be fabricated and could support BMSCs seeding, osteogenic differentiation.
Alkaline Phosphatase ; Anthraquinones ; Bone Marrow ; Colorimetry ; Durapatite ; Enzyme-Linked Immunosorbent Assay ; Femur ; Flow Cytometry ; Microscopy, Electron, Scanning ; Nanocomposites ; Nanostructures ; Osteocalcin ; Rabbits ; Seeds ; Stem Cells

PURPOSE: Gelatin-hydroxyapatite nanocomposite is similar to inorganic nanostructure of bone. To make a scaffold with osteoinductivity, bone marrow derived stem cells from rabbit femur were impinged into the nanocomposite. This vitro study was to test osteogenic differentiation of the stem cells in the nanocomposite, which was made by authors. MATERIAL & METHODS: Gel-HA nanocomposite with 10g of HA, 3 g of Gel has been made by co-precipitation process. Bone marrow was obtained from femur of New Zealand White rabbits and osteogenic differentiation was induced by culturing of the BMSCs in an osteogenic medium. The BMSCs were seeded into the Gel-HA nanocomposite scaffold using a stirring seeding method. The scaffolds with the cells were examined by scanning electron microscopy (SEM), colorimetry assay, biochemical assay with alkaline phosphatase (ALP) diagnostic kit, osteocalcin ELISA kit. RESULTS: Gel-HA nanocomposite scaffolds were fabricated with relatively homogenous microscale pores (20-40 micrometer). The BMSCs were obtained from bone marrow of rabbit femurs and confirmed with flow cytometry, Alizarin red staining. Attachment and proliferation of BMSCs in Gel-HA nanocomposite scaffold could be identified by SEM, ALP activity and osteocalcin content of BMSCs. CONCLUSION: The Gel-HA nanocomposite scaffold with micropores could be fabricated and could support BMSCs seeding, osteogenic differentiation.
Alkaline Phosphatase ; Anthraquinones ; Bone Marrow ; Colorimetry ; Durapatite ; Enzyme-Linked Immunosorbent Assay ; Femur ; Flow Cytometry ; Microscopy, Electron, Scanning ; Nanocomposites ; Nanostructures ; Osteocalcin ; Rabbits ; Seeds ; Stem Cells

Although liver metastasis is commonly found in cancer patients, fulminant hepatic failure secondary to diffuse cancer infiltration into the liver is rare. Liver metastasis-induced fulminant hepatic failure has been reported in patients with primary cancer of the gastrointestinal tract, breast and uroepithelium, and in patients with melanoma and hematologic malignancy. Small cell lung cancer is so highly invasive that hepatic metastasis is common, but rapid progression to fulminant hepatic failure is extremely rare. We report here on a case of a patient who died because of rapid progression to fulminant hepatic failure as a result of hepatic metastasis of small cell lung carcinoma.
Aged ; Carcinoma, Small Cell/complications/pathology/*secondary ; Female ; Humans ; Liver Failure, Acute/diagnosis/*etiology ; Liver Neoplasms/complications/pathology/*secondary ; Lung Neoplasms/complications/*pathology ; Neoplasm Invasiveness ; Tomography, X-Ray Computed