Osteogenesis Imperfecta (OI) Also Known

Osteogenesis imperfecta (OI) also known as Brittle Bone Disease, or Lobstein syndrome is a genetic bone disorder. People with OI are born with imperfect connective tissue, or devoid of the ability to make it, usually because of a lack of Type-I collagen. This deficiency arises from an amino acid replacement of glycine to bulkier amino acids in the collagen triple helix structure. The superior amino acid side-chains create steric hindrance that creates a bulge in the collagen complex, which in turn influences both the molecular nanomechanics as well as the interaction flanked by molecules, which are both compromised. As a result, the body may respond by hydrolyzing the improper collagen structure. If the body does not obliterate the improper collagen, the association between the collagen fibrils and hydroxyapatite crystals to form bone is altered, causing brittleness. There are different medications used in the treatment of Osteogenesis imperfecta namely; Calcitonin, Miacalcin, Calcimar, Caltine, Miacalcin NS, Miacalcic, Oseum and Tonocalcin.

CALCITONIN

Calcitonin-salmon is a man-made version of the hormone, calcitonin, found in salmon. In humans, it is produced by the thyroid gland and acts primarily on bone; however, the mechanism of its action is not well understood. Bone is always in a constant state of remodeling, old bone is removed by osteoclasts cells, and new bone laid down osteoblasts cells. Calcitonin inhibits bone removal by osteoclasts, and promotes bone formation by osteoblasts. It has no generic form.

It is used for treatment of osteogenesis imperfect, postmenopausal osteoporosis Paget's disease of bone, and hypercalcaemia. After menopause, the rate of bone loss increases in women with osteoporosis, calcitonin has been shown to boost bone density and strength. Paget's disease is characterized by a disorderly and accelerated remodeling of the bone, leading to weak and painful bones. Hence calcitonin has been found to reduce the turnover of bone and pain in Paget's disease patients.

It is found as an injection: 200 units/ml administered intramuscularly or subcutaneously. Also available is intranasal spray: 200 units/activation (0.09 ml/puff) which is administered into the nostril. There are no adequate studies of calcitonin in pregnant women and nursing mothers. Calcitonin salmon helps treat osteoporosis and Paget's disease of bone but does not cure them. Patients should continue using calcitonin salmon even if they feel well and should only be stopped by the doctor. Using calcitonin salmon for osteoporosis, it is also essential to get enough calcium and vitamin D

Calcitonin salmon may cause side effects such as stomach upset, vomiting, redness, swelling, or irritation at the site of injection, flushing of the face or hands, increased urination at night, itching of the ear lobes, feverish feeling, eye pain, decreased appetite, stomach pain, swelling of the feet, salty taste, rhinorrhea, nose bleeding, bone pain and headache. Some side effects can be serious. Though uncommon, but if experienced a doctor should immediately be notified, such as; hives, skin rash, itching, difficulty breathing or swallowing, swelling of the tongue or throat.