• Biomolecules & Therapeutics
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• 제30권4호
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• pp.309-319
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• 2022

Metabolic bone diseases are serious health issues worldwide, since several million individuals over the age of 50 are at risk of bone damage and should be worried about their bone health. One in every two women and one in every four men will break a bone during their lifetime due to a metabolic bone disease. Early detection, raising bone health awareness, and maintaining a balanced healthy diet may reduce the risk of skeletal fractures caused by metabolic bone diseases. This review compiles information on the most common metabolic bone diseases (osteoporosis, primary hyperparathyroidism, osteomalacia, and fluorosis disease) seen in the global population, including their symptoms, mechanisms, and causes, as well as discussing their prevention and the development of new drugs for treatment. A large amount of research literature suggests that balanced nutrition and balanced periodic supplementation of calcium, phosphate, and vitamin D can improve re-absorption and the regrowth of bones, and inhibit the formation of skeletal fractures, except in the case of hereditary bone diseases. Meanwhile, new and improved drug formulations, such as raloxifene, teriparatide, sclerostin, denosumab, and abaloparatide, have been successfully developed and administered as treatments for metabolic bone diseases, while others (romososumab and odanacatib) are in various stages of clinical trials.

• 한국임상수의학회지
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• 제18권3호
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• pp.265-268
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• 2001

Metabolic bone disease was diagnosed in 2 green iguanas hospitalized in Veterinary Medical Teaching Hospital of Seoul National University. Treatments were focused on initially correcting of husbandry with attention being paid to diet, increasing UV exposure time and increasing temperature of aquarium. Calcium complex was injected intramuscularly one time and administered calcium powder orally. Treatments responses were good with recovering body condition and increasing plasma Ca level.

• 대한유전성대사질환학회지
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• 제14권1호
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• pp.42-47
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• 2014

Gaucher disease is a multisystemic disorder arising from a deficient activity of the lysosomal enzyme glucocerebrosidase, which leads to accumulation of glycosylceraide and other glycolipids in the regiculoendothelial system. The characteristics of Gaucher disease are anemia, thrombocytopenia, hepatosplenomegaly, and skeletal disease. Enzyme replacement therapy (ERT) has been proven to prevent progressive manifestations of Gaucher disease and effective in improving anemia, thrombocytopenia, bone markers and biomarkers. However, some patient needs still remain unmet because of the inaccessibility of certain sites including brain, bone and various organs. ERT could not Improve the irreversible lesion such as liver fibrosis, hepatopulmonary syndrome, and necrosis or infarction of bone and other organs. Adult patients with Gaucher disease should be screened for longterm complication such as bone disease, pulmonary hypertension, gallstone, and cancer, especially in patients with splenectomy. Parkinsonism and polyneuropathy was also reported among patients with type 1 Gaucher disease, but ERT does not improve neurological function. We need to review the benefits and unmet needs of ERT in Gaucher disease.

• 대한유전성대사질환학회지
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• 제23권1호
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• pp.1-11
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• 2023

Inborn errors of metabolism encompass a wide variety of disorders, frequently affecting bone. This review presents a comprehensive retrospect on the primary involvement of bone in inborn errors of metabolism. Primary involvement of bone in inborn errors of metabolism includes entities that primarily affect the bone marrow, mineral component or cartilage. These include lysosomal storage disorders, hypophosphatasia, and hereditary hypophosphatemic rickets. In this review, we discuss the primary involvement of bone in inborn errors of metabolism (hypophosphatasia, X-linked hypophosphatemic rickets, Gaucher disease, and mucopolysaccharidoses) along with the therapeutic agents used in clinical settings, diagnostic strategies, and general management. With the development of disease-specific targeted therapies and supportive care, more number of patients with these disorders live longer and survive into adulthood. Moreover, skeletal symptoms have become a more prominent feature of these disorders. This makes the awareness of these skeletal symptoms more important.

• 한국임상수의학회지
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• 제9권2호
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• pp.467-472
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• 1992

Seven patients with signs of metabolic bone disease clinically seen at the Veterinary Teaching Hospital of Seoul National University from May 1990 to February 1992 were evaluated retrospectively. 1. Clinical signs include bowed leg, deformity of spine, digestive disturbance, constipation, retarded growth, lameness and hindlimb paralysis. 2. Radiographical sings include generalized skeletal demineralization, decreased radiological contrast between skeleton and soft tissues, thinning of bone cortex and pathological fractures. The diagnosis of nutritional secondary hyperparathyroidism resulting in metabolic bone disease caused by mineral imbalance was made based upon history, clinical signs, physical examination and radiographical signs.

• 한국생물공학회:학술대회논문집
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• 한국생물공학회 2005년도 생물공학의 동향(XVII)
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• pp.874-874
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• 2005

• Clinical and Experimental Pediatrics
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• 제49권11호
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• pp.1152-1157
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• 2006

Inherited metabolic disease is rare disorders that show symptoms mainly in pediatric age and early treatment is important for preventing complications of the disease. Recent development in molecular and biochemical techniques help clinicians with proper diagnosis of patients, however, many of the disease still remain lack of effective therapeutic strategies. Better understanding on biochemical and molecular basis of pathogenesis of the disease combined with advanced medical care would provide new sight on the disease that can also improve the quality of life and long-term prognosis of patients. Traditionally, there are several modalities in the treatment of metabolic diseases depend on the biochemical basis of the disease such as diet restriction, removing or blocking the production of toxic metabolites, and stimulating residual enzyme activity. The inherited metabolic disease is not familiar for many clinicians because the diagnosis is troublesome, treatment is complicated and prognosis may not as good as expected in other diseases. Recently, new therapeutic regimens have been introduced that can significantly improve the medical care of patients with metabolic disease. Enzyme replacement therapy has showed promising efficacy for lysosomal storage disease, bone marrow transplantation is effective in some disease and gene therapy has been trying for different diseases. The new trials for treatment of the disease will give us promising insight on the disease and most clinicians should have more interest in medical progress of the metabolic disease.

• 대한의생명과학회지
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• 제16권3호
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• pp.169-177
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• 2010

Osteoporosis is a disease that increases the fracture rates and a major cause of increased mortality and morbidity in the elderly people. This study is to determine which components of body composition and metabolic syndrome risk factors are important to bone health, we analysed the relationship among bone mineral density (BMD), body composition and metabolic syndrome risk factors in females. Totally 630 females participated in a medical check-up program (mean age 47 years) were selected for this study. Body composition analysis was performed by segmental bioelectrical impedance method, muscle mass, and percent body fat were measured. We also measured metabolic syndrome risk factors including abdominal obesity, HDL-cholesterol, triglyceride, blood pressure and fasting glucose level. Metabolic syndrome was defined by NCEP-ATP III criteria. The lumbar spine and femoral neck BMD were measured using the dual energy X-ray absorptiometry. Osteopenia and osteoporosis were observed in 180 and 51 persons, respectively. Muscle mass and HDL-cholesterol decreased in osteopenia and osteoporosis groups compared to the control group, and the grade was shown progressively by the symptoms. Significant positive correlation between BMD and muscle mass was observed. Multi variable regression analyses showed that % body fat and muscle mass were independent predictors of BMD after adjustment of age, height and weight. In conclusion, the BMD showed negative correlation with the metabolic and body composition was associated with BMD.

• 대한핵의학회지
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• 제16권2호
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• pp.97-102
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• 1982

Avascular necrosis of bone has become a well-recognized complication of renal transplantation. While preexisting metabolic bone disease, especially hyperparathyroidism, and metabolic disturbances induced by steroids have been implicated as etiological factors, the pathogenesis is controversial. The diagnosis of avascular necrosis of bone had been based on a history of joint pain and radiographic demonstration of bone necrosis. Recently the bone scan using 99mTc-methylene diphosphonate is helpful in determining the early stage of bone necrosis. We report two cases of avascular necrosis of femur head, of which diagnosis was made by the bone scan using 99mTc-methylene diphosphonate.

• International Journal of Oral Biology
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• 제44권4호
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• pp.130-143
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• 2019

Rheumatoid arthritis, osteoarthritis, and periodontal disease are bone destructive diseases mainly caused by inflammation. Various studies are being conducted to develop treatments for inflammatory bone destructive diseases. Many of these studies involve plant-derived natural compounds. In these studies, cell differentiation, signal transduction pathways, and bone resorption were measured at the cellular level. In disease-induced animal models, the amount of inflammatory mediators or matrix destructive enzymes and serum metabolic markers were measured. This study examined the effects of plant-derived natural compounds, such as flavonoids, on inflammatory bone destructive diseases. In addition, we structurally classified various substances used to maintain bone health and summarized the biological effects and related mechanisms of the components.