Metaphyseal Chondrodysplasia is rare, hereditary disease characterized by defective enchondral bone formation with major manifestation at the metaphysis. Jansen originally used the term metaphyseal dysostosis in 1934 to describe a patient who has a short stature with irregular metaphysis of the lower extremity and hands. Schmid reported a milder form of Metaphyseal dysostosis in 1949, which is more common and is transmitted in autosomal dominant trait. Mukusick reported another form of Metaphyseal Chondrodysplasia which is associated with ectodermal abnormalities in 1964. The other different types were reported alos, but they are extremly rare. The basic defect in the disease may be the failure of hypertrophic cells to mature and degenerate, caused by a block in or deficiency of enzymes of glycolytic cycle. The skull and spine are spared. Serum chemistry and kidney function are normal. The only treatment necessary, once adequate diagnosis has been estabilished, is careful observation and properly timed corrective orthopaedic surgery. We experienced one case of Schmid Type Metaphyseal Chondrodysplasia. Corrective osteotomy was performed and satisfactory result was obtained.
Chemistry ; Diagnosis ; Dysostoses ; Ectoderm ; Genetic Diseases, Inborn ; Hand ; Humans ; Kidney ; Lower Extremity ; Osteogenesis ; Osteotomy ; Skull ; Spine

Acid-soluble collagen (ASC) and pepsin-soluble collagen (PSC) from the spine (ASC-SP and PSC-SP) and skull (ASC-SK and PSC-SK) of the skipjack tuna, Katsuwonus pelamis, were successfully isolated and characterized. The yields of ASC-SP, PSC-SP, ASC-SK and PSC-SK were (2.47 ± 0.39)%, (5.62 ± 0.82)%, (3.57 ± 0.40)%, and (6.71 ± 0.81)%, respectively, on the basis of dry weight. The four collagens contained Gly (330.2-339.1 residues/1 000 residues) as the major amino acid, and their imino acid contents were between 168.8 and 178.2 residues/1 000 residues. Amino acid composition, SDS-PAGE, and FTIR investigations confirmed that ASC-SP and ASC-SK were mainly composed of type I collagen, and had higher contents of high-molecular weight cross-links than those of PSC-SK and PSC-SP. The FTIR investigation also certified all the collagens had triple helical structure. The denaturation temperatures of ASC-SK, PSC-SK, ASC-SP, and PSC-SP were 17.8, 16.6, 17.6, and 16.5 °C, respectively. All isolated collagens were soluble at acidic pH (1-5) and lost their solubilities when the NaCl concentration was above 2% (W/V). The isolated collagens from the spines and skulls of skipjack tuna could serve as an alternative source of collagens for further application in food, cosmetic, biomedical, and pharmaceutical industries.
Acids ; chemistry ; Amino Acids ; analysis ; Animals ; Collagen ; chemistry ; isolation & purification ; Collagen Type I ; chemistry ; isolation & purification ; Hydrogen-Ion Concentration ; Molecular Structure ; Molecular Weight ; Pepsin A ; chemistry ; Skull ; chemistry ; Sodium Chloride ; Solubility ; Spine ; chemistry ; Temperature ; Tuna

OBJECTIVETo improve the histocompatibility of chicken calamus keratin (CCK) graft by collagen-gel coating or using of cyclosporine A (CsA).

METHODSThirty SD rats were equally randomized into 5 groups, and in 4 of them, CCK implantation into the bilateral erector spinae was performed on different treatment protocols. In group A, the rats received daily intraperitoneal injection of CsA (5 mg/kg) for two consecutive weeks after CCK implantation; in group B, CCK was soaked in CsA (2.5 mg/ml) solution at 4 degrees Celsius; for 48 h before grafting; in group C, CCK coated with collagen gel was grafted; and in group D, only CCK was implanted. Rats in the fifth group received only cutaneous incision as well as muscular dissection to serve as the blank control. CCK degradation and its effect on the surrounding tissues were observed at 2, 4 and 8 weeks after grafting. Immunohistochemistry was performed to identify T lymphocyte infiltration in the host tissues.

RESULTSAll the rats survived the operation. Numerous macrophages, especially multinucleated giant cells occurred on the peripheral of the CCK grafts, and small degraded CCK pieces were observed in their cytoplasm. Only a few inflammatory cells were seen in the host tissues. At 2, 4 and 8 weeks after CCK implantation, only a few CD3-positive cells were found in all the groups, and in group A and B, the density of T lymphocytes was significantly lower than that in group D, and there was no significant difference between group A and the blank control group.

CONCLUSIONSCsA significantly improves the histocompatibility of CCK material, and short-term systemic CsA administration achieves the best results. Macrophages, especially multinucleated giant cells participate in CCK degradation in vivo.

Animals ; CD3 Complex ; analysis ; Chickens ; Coated Materials, Biocompatible ; administration & dosage ; chemistry ; Collagen ; chemistry ; Cyclosporine ; administration & dosage ; chemistry ; Feathers ; chemistry ; Female ; Gels ; Histocompatibility ; drug effects ; Immunohistochemistry ; Immunosuppressive Agents ; administration & dosage ; chemistry ; Implants, Experimental ; Injections, Intraperitoneal ; Keratins ; chemistry ; Male ; Muscle, Skeletal ; chemistry ; drug effects ; surgery ; Random Allocation ; Rats ; Rats, Sprague-Dawley ; Spine ; T-Lymphocytes ; chemistry ; cytology ; Tissue Engineering ; methods

The cuttlebones, harvested from cuttles, undergo the chemical reaction in high temperature and high pressure for a certain time. The products are qualitatively analysed, and spacial structure observation and cytocompatibility are tested. The results show that the chemical component of the cuttlebone is CaCO3 and the crystal type is aragonite. Cuttlebones undergo a hydro-thermal reaction, and thus transform into hydroxyapatite-that is, the cuttlebone-transformed hydroxyapatite(CBHA). The CBHA materials have the interconnected microporous network structures. Under the high magnification, CBHAs appear to have many micro-spheres, thus construct a new self-assembled nano-material system. The marrow stromal osteoblasts can adhere to and proliferate well on the surface of the CBHAs. These results show that CBHAs have good biocompatibility. Therefore, it can be a potential candidate scaffold for bone tissue engineering.
Animals ; Bone Substitutes ; chemical synthesis ; chemistry ; toxicity ; Cells, Cultured ; Durapatite ; chemical synthesis ; chemistry ; toxicity ; Materials Testing ; Osteoblasts ; cytology ; drug effects ; Rabbits ; Sepia ; anatomy & histology ; Spine ; anatomy & histology ; chemistry ; Tissue Engineering

BACKGROUND: Osteopenia or osteoporosis is one of the most frequently encountered complications in patients receiving various immunosuppressants after kidney transplantation. The few available preventive strategies for these complications tend to result in various outcomes. In this study, we evaluated the effect of intermittent etidronate therapy for the prevention of bone loss after kidney transplantation. METHODS: Fifty patients who received kidney transplantation for various reasons were recruited and followed for one year. Thirty-eight of these patients commenced etidronate treatment 7 days after operation, the other 12 were followed without etidronate therapy. The treatment consisted of 400mg of etidronate administered orally for 14 days, then repeated four-times every three months. Blood chemistry, iPTH and aluminium levels were tested periodically in all patients. Also checked were bone mineral density of the lumbar spine(L2-4) and femur at baseline, 6 and 12 months after kidney transplantation, as well as D-L spine lateral x-ray at baseline and 12 months. Serum osteocalcin and urine deoxypyridinoline were measured at baseline, 7 days and then every 3 months. RESULTS: Both the etidronate-treated and control groups showed significant decreases in bone mineral densities of the lumbar spine, femur neck and total femur at 6 and 12 months after kidney transplantation(p<0.005). Bone loss was significantly lower in the etidronate-treated group than the control at 12 months after kidney transplantation; lumbar spine(-3.54% vs. -9.51%, p<0.0005), femur neck (-5.41% vs. -8.91%, p<0.0005), total femur (-7.59% vs. -9.07%, p<0.005). Osteocalcin was decreased and deoxypyridinoline increased in both groups. No significant differences in the level or pattern of osteocalcin and deoxypyridinoline were observed in either group. New radiologic compression fractures were found in two patients of the treated group who exhibited severe osteoporosis at baseline during follow-up. CONCLUSIONS: The intermittent administration of etidronate seems to be effective in preventing rapid bone loss after kidney transplantation. Furthermore, this method is safe and convenient for administration and follow-up. Further studies will be required to elucidate the most effective treatment course for the prevention of fractures after kidney transplantation, especially in patients with established severe osteoporosis.
Bone Density ; Bone Diseases, Metabolic ; Chemistry ; Etidronic Acid* ; Femur ; Femur Neck ; Follow-Up Studies ; Fractures, Compression ; Humans ; Immunosuppressive Agents ; Kidney Transplantation* ; Kidney* ; Osteocalcin ; Osteoporosis ; Spine

OBJECTIVES: Bee venom contains a potent antiinflammatory peptide 401 as well as mellitin. The purpose of this study was to see the efficacy and safety of purified bee venom injection therapy for knee or spinal osteoarthritis patients. METHODS: One hundred and one osteoarthritis patients were randomly assigned to bee venom injection therapy or oral nabumetone medication group. Bee venom injection group was subdivided into 3 groups according to different dosing schedule(group A: gradual increase up to 0.7mg, group B: up to 1.5mg and group C: up to 2.0mg). Control group patients(group D) were given 1000mg nabumetone daily for 6 weeks. There were 25, 26, 25, and 26 patients assigned to A, B, C, or D group. The efficacy of treatment was evaluated by measuring instruments developed by authors, and the safety of bee venom injection was evaluated by hematology and chemistry laboratory examination. RESULTS: Among 101 patients, eighty-one patients completed the study, but twenty patients were dropped out and two of these patients were dropped out due to adverse drug reaction. The efficacy in bee venom group showed better improvement than nabumetone group(p<0.01). Within bee venom group, group B and C showed better improvement than group A(p<0.01). Itching around injection site occurred in most patients, and bodyache occurred in 49 patients (81.7%). Hemoglobin was decreased(0.3g/dl) in group C, but no significant changes were observed in other laboratory values. CONCLUSION: The efficacy of bee venom injection in the control of knee or back pain in osteoarthritis patients was better than nabumetone medication. No severe allergic or adverse reaction was observed in bee venom treatment patients, but problems related with bee venom injection, such as pruritis, bodyache, and the possibility of anaphylaxis, should be considered for the use of bee venom injection.
Anaphylaxis ; Back Pain ; Bee Venoms* ; Bees* ; Chemistry ; Drug-Related Side Effects and Adverse Reactions ; Hematology ; Humans ; Knee ; Melitten ; Osteoarthritis* ; Osteoarthritis, Spine ; Pruritus

BACKGROUND: Steroid-induced osteoporosis(SIO) is one of the serious complications of long- term steroid therapy, especially in growing children. Recently bisphosphonates have been used to treat or prevent SIO in adult, which is rare in children with glomerular diseases. We studied the effect of pamidronate on SIO using dual energy X-ray absorptiometry and biochemical markers of bone turnover. METHODS: Forty four children receiving moderate-to-high doses of steroids were enrolled. They had no history of bone, liver, or endocrine disease. Patients were stratified by their baseline bone mineral density(BMD) findings. All patients received corticosteroids for 3 month and oral calcium supplementation(500 mg/day) daily. Among them, 28 patients were treated with placebo and 16 were treated with pamidronate(125 mg) for 3 months. Blood chemistry and bone mineral density(BMD) were measured at baseline, and 3months. In addition, parathyroid hormone(PTH), serum osteocalcin, and urinary dipyridinoline levels were evaluated. RESULTS:In overall population, the mean lumbar spine BMD decreased from 0.754+/-0.211 (g/cm2) to 0.728+/-0.208(g/cm2) in the placebo group(P<0.05) and increased from 0.652+/-0.194 (g/cm2) to 0.658+/-0.226(g/cm2) in the pamidronate group(P>0.05). CONCLUSION:Pamidronate appears to be effective in preventing SIO in children with glomerular diseases requiring long-term steroids therapy. Further careful observation and follow-up might be needed for children receiving bisphosphonates such as pamidronate.
Absorptiometry, Photon ; Adrenal Cortex Hormones ; Adult ; Biomarkers ; Calcium ; Chemistry ; Child* ; Diphosphonates ; Endocrine System Diseases ; Follow-Up Studies ; Humans ; Liver ; Osteocalcin ; Spine ; Steroids

In order to define osteoporosis on the basis of bone mineral measurements, one must define an acceptable normal range or fracture threshold. It is clear that the normal range cannot be compared between different ethnic groups. We have measured spinal bone mineral density (BMD) by dual photon absorptiometry in 277 women without spinal fracture, aged 30-91 years, and in 53 women with asymptomatic spinal fracture to provide such a database for normal Korean women. Peak bone mass at the 3rd decade was 1.24 g/cm2. BMD from age 40-69 was strongly correlated with age (r = -0.7) and the annual decrease averaged 0.018gm/cm2. The rate of annual loss slowed by 50% in women after 70% years of age. Fracture threshold was evaluated at the 90th percentile for spinal BMD in patients with vertebral fractures. The fracture threshold of the vertebra was 0.94 g/cm2. Approximately 50% of normal women over 50 years of age had values below this threshold. These findings suggest that the way of developing low bone mass in Korean women is to peak high and lose fast.
Adult ; Age Factors ; Aged ; Asian Continental Ancestry Group ; *Bone Density ; European Continental Ancestry Group ; Female ; Fractures, Bone/etiology ; Humans ; Korea ; Menopause/metabolism ; Middle Aged ; Osteoporosis/*metabolism ; Spine/*chemistry

The clinical effects of two different methods-high-viscosity cement percutaneous vertebroplasty (PVP) and low-viscosity cement percutaneous kyphoplasty (PKP) in the treatment of osteoporotic vertebral compression fractures (OVCFs) were investigated. From June 2010 to August 2013, 98 cases of OVCFs were included in our study. Forty-six patients underwent high-viscosity PVP and 52 patients underwent low-viscosity PKP. The occurrence of cement leakage was observed. Pain relief and functional activity were evaluated using the Visual Analog Scale (VAS) and Oswestry Disability Index (ODI), respectively. Restoration of the vertebral body height and angle of kyphosis were assessed by comparing preoperative and postoperative measurements of the anterior heights, middle heights and the kyphotic angle of the fractured vertebra. Nine out of the 54 vertebra bodies and 11 out of the 60 vertebra bodies were observed to have cement leakage in the high-viscosity PVP and low-viscosity PKP groups, respectively. The rate of cement leakage, correction of anterior vertebral height and kyphotic angles showed no significant differences between the two groups (P>0.05). Low-viscosity PKP had significant advantage in terms of the restoration of middle vertebral height as compared with the high-viscosity PVP (P<0.05). Both groups showed significant improvements in pain relief and functional capacity status after surgery (P<0.05). It was concluded that high-viscosity PVP and low-viscosity PKP have similar clinical effects in terms of the rate of cement leakage, restoration of the anterior vertebral body height, changes of kyphotic angles, functional activity, and pain relief. Low-viscosity PKP is better than high-viscosity PVP in restoring the height of the middle vertebra.
Administration, Cutaneous ; Aged ; Bone Cements ; chemistry ; therapeutic use ; Female ; Follow-Up Studies ; Fractures, Compression ; pathology ; rehabilitation ; surgery ; Humans ; Kyphoplasty ; instrumentation ; methods ; Male ; Middle Aged ; Osteoporosis ; pathology ; rehabilitation ; surgery ; Pain ; diagnosis ; physiopathology ; Pain Measurement ; Recovery of Function ; physiology ; Spinal Fractures ; pathology ; rehabilitation ; surgery ; Spine ; pathology ; surgery ; Treatment Outcome ; Viscosity ; Visual Analog Scale