Analysis Of Treatment For Inpatients With Osteogenesis Imperfecta

Background:Osteogenesis imperfecta(0I)is a rare genetically heterogeneous bone disease.Bone fragility at birth with malformation,repeated fractures,and short stature are signs of osteogenesis imperfecta.It is mainly caused by COL1A1 or COL1A2 gene mutation leading to type Ⅰ collagen lesions.Due to the presence of type I collagen in many tissues,patients with OI have a variety of clinical phenotypes and may present with extrasolar manifestations including blue sclera,dentine imperfecta,skin lesions,joint hyperactivity and/or vascular fragility.Due to OI’s repeated fractures and skeletal deformity,most patients have difficulty walking,and even affect their daily life and mental health.Therefore,the treatment of OI is very important.At present,drug treatment and surgical treatment of OI are the most commonly used clinical treatment methods.Bisphosphonate is the main drug therapy to improve bone density and prevent fracture.Surgical treatment is feasible for severe fractures.Objectives:This study evaluated the clinical efficacy of the three treatment methods by comparing various indicators of inpatients treated with surgery alone,drugs alone and surgery plus drugs.Methods:A total of 150 OI patients hospitalized in Provincial Hospital affiliated to Shandong First Medical University from 2009 to January 2023 were divided into three groups according to different treatment methods:operation group(n=30),bisphosphonate group(n=30)and bisphosphonate injection combined group(n=90).The prognosis of the three treatment methods was analyzed.Results:1.Motor function grading and functional independence score were improved in all three groups after treatment.2.There was no statistical difference in the functional independence scores of the three groups before treatment,and the motor function grading medication group was stronger than the other two groups.3.After treatment,the combined group had the best motor function grading and functional independence score with the greatest improvement,while the other two groups had no statistical difference.4.There were no significant differences in deformity and limb length among the three groups.5.The number of patients without surgical revision after treatment in the combined group was higher than that in the surgery group,and the difference was statistically significant.6.The fracture frequency of the combination group was higher than that of the medication group before treatment,but there was no difference between the two groups after treatment,and the reduced fracture frequency was stronger than that of the medication group,the difference was statistically significant.Conclusions:1.All three treatments improved motor ability and independence.2.Exercise ability and independence were the best and the improvement was the largest after operation with pamidronate sodium.3.There was no significant difference in the effect between operation alone and pamidronate disodium alone.4.There was no significant difference in the influence of the three treatments on patients’deformity and abnormal limb length5.The revision rate of surgical plus pamidronate was lower than that of surgical treatment alone.6.Patients treated with pamidronate disosodium alone had milder disease(greater exercise capacity and lower fracture frequency).7.Operation plus pamidronate disosodium is more beneficial to the prevention of fracture than simple pamidronate disosodium.Background:OI is a phenotypic and genetically heterogeneous bone dysplasia characterized by bone fragility,growth defects,and skeletal malformations.Previously thought to be caused by defects in type Ⅰ collagen,the major protein of the extracellular matrix,it is now also considered to be a collagenous disorder caused by defects in collagen folding,post-translational modification and processing,bone mineralization and osteoblast differentiation,with OI type inheritance spanning autosomal dominant and recessive as well as X-linked recessive.The main features are increased bone fragility,repeated fractures,bone deformity,and short stature.Due to OI’s repeated fractures and skeletal deformity,most patients have difficulty walking,and even affect their daily life and mental health.Therefore,the treatment of OI is very important.Bisphosphonates are currently the most commonly used treatment for patients with OI.Many studies have proved that bisphosphonates can increase bone mineral density,relieve bone pain,reduce the incidence of fractures in patients,improve limb mobility and improve the quality of life of patients.However,the current treatment of bisphosphonates varies,and once the use of bisphosphonates is stopped,the improvement effect of bisphosphonates will disappear,and it was found in the review that few patients can regularly receive bisphosphonate injection course.In this study,we compared the three common bisphosphonates in clinical practice(regular use,irregular use,no use)to evaluate the difference in the effects of the three treatment methods on patients.Objectives:Bisphosphonates are very important in the treatment of OI and are the first choice for OI patients.This study analyzed the effects of intravenous bisphosphonates on bone mineral density,exercise ability and independence in PATIENTS with OI.And observe the therapeutic effect of bisphosphonates on OI patients with different use conditions(regular use,irregular use,no use).Methods:Ninety patients aged 0-20 years who were clinically diagnosed with OI were divided into three groups(regular administration group,irregular administration group and non-administration group)according to whether they followed the recommended frequency and dose of bisphosphonates.Patients who followed the recommended intravenous administration of bisphosphonates(once every 4 months or three times a year(7.5mg/kg/year))were classified into regular administration group.Patients who had been treated with bisphosphonates but did not follow the recommendations(less than 7.5mg/kg/year of bisphosphonate injection)were divided into the irregular administration group;Conservative and surgical treatment of fractures alone were assigned to the untreated group of patients who had never used bisphosphonates.Bone mineral density(BMD),motor function grading and kinetic independent score were analyzed.Results:1.Bone mineral density,motor function grading and functional independence score of patients in three groups were improved after treatment.2.BMD,motor function grading and functional independence scores of the regular medication group were higher than those of the irregular medication group and the non-medication group after treatment.3.BMD and functional independence scores of regular medication group were stronger than those of irregular medication group and non-medication group after treatment;There was no statistical difference in motor function classification between the irregular drug use group and the non-drug use group.Conclusion:Bisphosphonates can increase bone mineral density and improve exercise and self-care.Regular and adequate use of bisphosphonates had the most beneficial effect on bone mineral density,exercise ability and self-care ability after treatment.But in terms of the degree of improvement in athletic performance,it was similar to the irregular use of bisphosphonates.Background:Osteogenesis imperfecta(OI)is an inherited bone disorder that causes brittle bones.It has a heterogeneous phenotype and typically presents with reduced bone mass,multiple fractures,malformations,and chronic disability.Sillence classification divides OI into four types according to the different clinical severity of OI.Type Ⅱ is the most serious and will lead to perinatal death due to intrauterine fracture or respiratory complications.Therefore,there is almost no chance to see patients with type Ⅳ in clinical diagnosis and treatment.TypeⅠ patients had the least clinical symptoms and almost no malformation.Type Ⅲ patients are the most severe of the surviving OI patients,with short stature,skeletal deformities and even the inability of many patients to walk unaided.Type Ⅳ OI is between type Ⅰ and type Ⅲ in severity.There is currently no cure for OI,and the current goal is to reduce the number of fractures and improve the quality of life of patients.Bisphosphonates are the most effective clinical treatment at present,but there is still no clear optimal dose and frequency.Based on the Ⅰ,Ⅲ and Ⅳ classification of OI,this study analyzed the difference in therapeutic effects of two kinds of bisphosphonates(regular and irregular)on patients.Objectives:The differences of bone mineral density and fracture frequency between regular and irregular use of bisphosphonates in patients with TYPE Ⅰ,Ⅲ and Ⅳ OI were analyzed.Methods:Patients hospitalized in Shandong First Medical University Provincial Hospital from January 2009 to February 2023 with definite Sillence classification were divided into regular group and irregular group according to the recommended course of bisphosphonate treatment.There were 10 patients with type Ⅰ OI,5 in the regular group and 5 in the irregular group.There were 14 patients in type Ⅲ and 7 in each group.Type Ⅳ 26,13 in each group.Bone mineral density(BMD)and fracture frequency of patients with OI were analyzed after treatment in the regular group and irregular group.Results:1.Bone mineral density and fracture frequency of type Ⅰ,Ⅲ and Ⅳ OI patients improved after treatment in both groups.2.There was no statistical difference in the therapeutic effect of type Ⅰ OI patients between the two groups.3.The BMD,fracture frequency and the increased BMD after treatment in the regular group of type Ⅲ OI patients were better than those in the irregular group,but the reduced fracture frequency was similar to that in the irregular group.4.The regular group of patients with type Ⅳ OI had higher BMD after treatment,but the effect on fracture risk was similar to that of the irregular group.Conclusion:Bisphosphonates increase bone mineral density and decrease fracture risk in patients with type Ⅰ,Ⅲ,and Ⅳ OI.Perhaps due to the mild condition of type Ⅰ OI patients,regular and irregular injection of bisphosphonates has no significant difference in the impact of their disease.Regular and adequate use of bisphosphonates results in higher bone mineral density in patients with type Ⅲ and Ⅳ OI and a lower risk of fracture in patients with type Ⅲ OI.