BACKGROUND Myositis ossificans(MO)is a rare disease involving the formation of bone outside the musculoskeletal system.While surgical intervention is the main treatment approach,preventing recurrence and standardized ...BACKGROUND Myositis ossificans(MO)is a rare disease involving the formation of bone outside the musculoskeletal system.While surgical intervention is the main treatment approach,preventing recurrence and standardized rehabilitation are also crucial.Here,we present a surgical strategy to prevent the recurrence of MO.CASE SUMMARY A 28-year-old female patient was admitted for the first time for a comminuted fracture of the left olecranon.However,incorrect postoperative rehabilitation resulted in the development of elbow joint stiffness with ectopic ossification,causing a loss of normal range of motion.The patient was diagnosed with MO based on physical examination,X-ray findings,and clinical presentation.We devised a surgical strategy to remove MO,followed by fixation with an Ilizarov frame,and implemented a scientifically reasonable rehabilitation plan.The surgery lasted for 3 h with an estimated blood loss of 45 mL.A drainage tube was placed after surgery,and fluid was aspirated through ultrasound-guided puncture.The patient experienced a significant reduction in joint stiffness after surgery.In the final follow-up at 9 mouths,there was evident improvement in the range of motion of the elbow joint,and no other symptoms were reported.CONCLUSION The Ilizarov frame is an advantageous surgical technique for facilitating rehabilitation after MO removal.It offers benefits such as passive recovery,individualized treatment,and prompt recovery.展开更多
BACKGROUND Myositis ossificans(MO)is an uncommon disorder characterized by heterotopic ossification within soft tissues.Only a few cases of intra-abdominal MO(IMO)have been described in the literature.Histology could ...BACKGROUND Myositis ossificans(MO)is an uncommon disorder characterized by heterotopic ossification within soft tissues.Only a few cases of intra-abdominal MO(IMO)have been described in the literature.Histology could be difficult to understand and a wrong diagnosis could lead to an improper cure.CASE SUMMARY We herein report the case of IMO in a healthy 69-year-old man.The patient presented with an abdominal mass in the left lower quadrant.A computed tomography scan showed an inhomogeneous mass with multiple calcifications.The patient underwent radical excision of the mass.Histopathological findings were compatible with MO.Five months later the patient showed a recurrence causing hemorrhagic shock due to intractable intralesional bleeding.The patients eventually died within three months since recurrence.CONCLUSION The case described could be classified as post-traumatic MO that developed close to the previously fractured iliac bone.The subsequent surgical procedure was ineffective and the disease rapidly recurred.The misleading intraoperative diagnosis led to improper surgical treatment with a dramatic evolution.展开更多
BACKGROUND Nontraumatic myositis ossificans is a rare disease whose specific pathogenesis is unclear.Early diagnosis of this disease is very difficult in children because of difficulties in determining medical history...BACKGROUND Nontraumatic myositis ossificans is a rare disease whose specific pathogenesis is unclear.Early diagnosis of this disease is very difficult in children because of difficulties in determining medical history and nonspecific early clinical manifestations,which may lead to the failure of timely and effective diagnosis and treatment in some patients.We report the diagnosis and treatment of a child with nontraumatic myositis ossificans and summarize the clinical characteristics and diagnosis and treatment of the disease.CASE SUMMARY An 8-year-old girl first came to our hospital for more than a week with pain in the right lower limb.There was no history of trauma or strenuous activities.On physical examination,no mass on the right thigh was found,and the movement of the right lower extremity was limited.Ultrasonography showed synovitis of the hip,and bed rest was recommended.Three days later,the child’s pain persisted and worsened,accompanied by fever and other discomforts.She came to our hospital again and a mass was found on the right thigh with redness and swelling on the surface.The images showed a soft tissue tumor on the right thigh with calcification.Routine blood tests revealed that the inflammation index was significantly increased.In case of infection,the patient was given antibiotics,and the pain was relieved soon after,without fever.However,the right thigh mass persisted and hardened.The patient underwent incision biopsy more than 1 mo later,and the postoperative pathology showed nontraumatic myositis ossificans.After approximately 9 mo of observation,the tumor still persisted,which affected the life of the child,and then resection was performed.Since follow-up,there has been no recurrence.CONCLUSION Due to the difficulty in discerning a child’s medical history and the diverse early manifestations,it is difficult to diagnose nonossifying muscle disease in children in its early stage.Measures such as timely follow-up and periodic image monitoring are conducive to early diagnosis of the disease.The disease has a certain degree of self-limitation,and it can be observed and treated first.If the tumor persists in the later stage or affects functioning,then surgery is considered.展开更多
A case of florid reactive periostitis ossificans(RPO) arising in a long bone is presented. This is a rare bone proliferation with a pronounced periosteal reaction. Less than 100 cases have been described in the litera...A case of florid reactive periostitis ossificans(RPO) arising in a long bone is presented. This is a rare bone proliferation with a pronounced periosteal reaction. Less than 100 cases have been described in the literature with far fewer outside the bones of the hand, feet, fingers, and toes. Although the etiology is unknown, a relationship to preceding trauma is suggested. The imaging and histologic features show an overlap with other bone lesions including bizarre parosteal osteochondromatous proliferation, subungual exostosis, and malignant surface tumors of bone and cartilage which include, periosteal and parosteal osteosarcoma. It is important to recognize the clinical presentation and diagnostic features of RPO as a benign entity so that it is not mistaken for a more aggressive neoplasm. We present a case of a right distal humeral lesion that on histopathological review revealed florid RPO. This diagnosis was not suspected on imaging studies, but was made on open biopsy of the mass. The patient remains disease free, years postoperatively. In addition to presenting this unique case report, we review the pertinent literature, and offer a differential diagnosis and treatment strategy for its management.展开更多
Myositis ossificans is a benign self limiting condition that usually related to trauma. Despite a clinically and histologically distinct entity, myositis ossificans still causes considerable difficulties in diagnosis....Myositis ossificans is a benign self limiting condition that usually related to trauma. Despite a clinically and histologically distinct entity, myositis ossificans still causes considerable difficulties in diagnosis. A 33-year-old Moroccan woman presented with a 2-month history of left inguinal inflammatory pain with limping gait, MRI examination suggested a malignant neoplasm such as soft tissue osteosarcoma. A diagnosis of myositis ossificans was made by incisional biopsy. Conservative management with clinical and radiological follow up of 19 months confirm the diagnosis. The symptoms resolved within seven months. Myositis ossificans should be considered by clinicians as a possible diagnosis for a soft tissue lesion.展开更多
ARACHNOIDITIS ossificans (AO) is a rare type of chronic arachnoiditis presence of calcification spinal arachnoid. Small characterized by the in, or ossification of the plaques of calcificationof the dura mater are f...ARACHNOIDITIS ossificans (AO) is a rare type of chronic arachnoiditis presence of calcification spinal arachnoid. Small characterized by the in, or ossification of the plaques of calcificationof the dura mater are frequently observed in radiography or during surgery, which are often asymptomatic and have no apparent clinical significance. AO and dura calcification should not be confused.展开更多
Introduction: Fibrodysplasia ossificans progressiva (FOP) is a rare autosomal dominant disorder of skeletal malformations and progressive extraskeletal ossification. Patients and Methods: In the present study, we pres...Introduction: Fibrodysplasia ossificans progressiva (FOP) is a rare autosomal dominant disorder of skeletal malformations and progressive extraskeletal ossification. Patients and Methods: In the present study, we present a case of FOP with marked progressive ossification of extraskeletal tissues. We investigated whether soluble factors in serum would affect the osteoblast phenotype by examining the effects of serum from this patient and control subjects on mouse osteoblastic MC3T3-E1 cells. Results: The clinical findings of this patient were compatible with FOP, and direct sequence analysis of genomic DNA demonstrated the presence of a heterozygous 617G> A (R206H) mutation of activin type 1 receptor (ACVR1). Serum from the FOP patient enhanced the level of alkaline phosphatase (ALP) in Western blotting, compared with serum from the control, in MC3T3-E1 cells. Moreover, serum from the FOP patient enhanced the levels of ALP, osteocalcin and bone morphogenetic protein-2 mRNA in these cells. Conclusion: We presented a case of FOP with progressive ossification in extra-skeletal tissues with ACVR1 mutation. The present data suggest that the serum from this patient includes some soluble factors, which might enhance the osteoblast differentiation and BMP-2 expression in mouse osteoblastic cells.展开更多
Myositis ossificans (MO) is an extraskeletal tumor-like lesion with bone formation, and is relatively rare in the head and neck region. We report herein a clinicopathological analysis of MO associated with a muscle of...Myositis ossificans (MO) is an extraskeletal tumor-like lesion with bone formation, and is relatively rare in the head and neck region. We report herein a clinicopathological analysis of MO associated with a muscle of mastication. The patient was a 70-year-old Japanese woman who presented with a tumorous mass in the right parotid area and no history of trauma. The surgically excised tumorous lesion consisted of proliferating fibroblastic cells with no atypia in the central portion, and a formation of trabecular osteoid and/or bony tissue in the peripheral portion as the so-called “zonal phenomenon”. The final histological diagnosis was MO, and the lesion was located in the posterior belly of the digastric muscle on computer tomography. A review of the literature showed that many cases of MO predominantly affected the medial pterygoid and masseter muscle and showed a history of previous trauma. The present case represents a rare case of nontraumatic MO in the uncommon location of the posterior belly of the digastric muscle.展开更多
Arachnoiditis ossificans is an intradural extramedullary lesion resulting from an unusual chronic meningeal inflammatory process and it is thought to be the sequela of end-stage adhesive arachnoiditis secondary to sur...Arachnoiditis ossificans is an intradural extramedullary lesion resulting from an unusual chronic meningeal inflammatory process and it is thought to be the sequela of end-stage adhesive arachnoiditis secondary to surgery, trauma, arachnoid hemorrhage, meningeal irritation, myelography (particularly oil-based contrast agents), and spinal anesthesia. The spinal arachnoiditis ossificans may be silent or cause a variety of symptoms depending on its location and uncommonly happen to cause spinal cord compression. Very little attention has been paid to the management and outcome of this rare condition in the neurosurgical reem. We report the case of a 45 years old man admitted with L1 - L2 arachnoiditis ossificans revealed by polyradiculopathy on incomplete cauda equina syndrome mimicking spinal canal tumors. The patient underwent surgery and we performed the laminectomy of L1 - L2, total resection of the lesion, followed by a complete remission of the hemiparesis after three months of kinesitherapy nursing.展开更多
Background Fibrodysplasia ossificans progressiva(FOP)is a rare and disabling heritable connective tissue disease that is difficult to treat.This study seeks to explore the clinical characteristics,clinical manifestati...Background Fibrodysplasia ossificans progressiva(FOP)is a rare and disabling heritable connective tissue disease that is difficult to treat.This study seeks to explore the clinical characteristics,clinical manifestations,treatment and prognosis of FOP to provide a clinical basis for its early diagnosis and treatment.Methods Twenty-six children with FOP were retrospectively analyzed in terms of their onset,clinical manifestations,auxiliary examinations and treatment.Results Among the 26 cases,the youngest age of manifestation of mass was 8 days after birth,and the average age was 3 years and 2 months.The peak age was 2-5 years old.Inflammatory mass and toe-finger deformity are the main early clinical manifestations of the disease.These inflammatory masses often lead to hard osteogenic deposits that initially mainly involve the central axis,such as the neck(22/26,84.6%),back(20/26,76.9%),and head(13/26,50%).Toe-finger deformity mainly manifests as symmetrical great toe deformity,or short and deformed thumb and little finger.The diagnosis of FOP requires typical clinical manifestations or ACVR1 gene detection.The main therapeutic drugs for FOP include glucocorticoids and non-steroidal anti-inflammatory drugs.Although not compliant with the recommended medical management of FOP,in our clinical practice children with uncontrollable illness could be treated using a variety of immunosuppressive agents in combination.Conclusions FOP is a rare autosomal dominant heritable disease.The main clinical manifestations observed in this study were recurrent inflammatory mass and toe-finger deformity.If the diagnosis and treatment are not performed in a timely manner,serious complications are likely to affect the prognosis.Therefore,early diagnosis and active treatment should be performed.展开更多
Myositis ossificans circumscripta (MOC) is a kind of self-localized, benign and tumor-like lesions often seen in adults, with approximately 75% of cases caused by trauma. We reported a case of non-traumatic MOC occu...Myositis ossificans circumscripta (MOC) is a kind of self-localized, benign and tumor-like lesions often seen in adults, with approximately 75% of cases caused by trauma. We reported a case of non-traumatic MOC occurred at the elbowjoint in a 9-year old child and it has been excised by surgery. After 18 months follow-up, a favorable outcome has been achieved with the Broberg-Morrey score of 100. We suggest that surgical resection should be done as soon as the diagnosis is confirmed.展开更多
ObjectiveTo describe the clinical manifestations and radiographic characteristics of fibrodysplasia ossificans progressiva (FOP). MethodsThe radiographs and lesion photographs of five patients aged between 23 months a...ObjectiveTo describe the clinical manifestations and radiographic characteristics of fibrodysplasia ossificans progressiva (FOP). MethodsThe radiographs and lesion photographs of five patients aged between 23 months and 17 years diagnosed as FOP were presented and the literatures were reviewed. ResultsClinical features were shortened great toes at birth, episodic soft tissue swellings, pain in the subcutaneous tissues of neck, back, shoulders, and extremities, hard lumps at back and spine, and restricted mobility of neck and arms. Radiological findings included short digit with hallus valgus, scoliosis, exostoses on many bones, and ossification of ligament. The characteristic subcutaneous soft tissues included masses or ossifications of neck, back, extremities, and shoulders. ConclusionThe short terminal phalanges of the thumbs and intermittently progressive heterotopic ossifications of the striated muscles and soft tissues suggest that FOP should promptly get early genetic consultation.展开更多
Fibrodysplasia ossificans progressiva(FOP)is a rare autosomal dominant congenital disorder characterized by progressive heterotopic ossification in muscle tissues.A constitutively activated mutation of a bone morphoge...Fibrodysplasia ossificans progressiva(FOP)is a rare autosomal dominant congenital disorder characterized by progressive heterotopic ossification in muscle tissues.A constitutively activated mutation of a bone morphogenetic protein(BMP)receptor,ALK2,has been identified in patients with FOP.We report here that four structurally related compounds,lucilactaene,hydroxylucilactaene,NG-391 and NG-393,produced by fungal strain Fusarium sp.B88,inhibit BMP signaling in vitro.Alkaline phosphatase activity,a marker enzyme of osteoblastic differentiation,was decreased in C2C12 myoblasts stably expressing mutant ALK2 by treatment with those compounds with IC_(50) values of 5.7,6.8,6.9 and 6.1 mM,respectively.Furthermore,NG-391 and NG-393 inhibited BMP-specific luciferase reporter activity,which is directly regulated by transcription factor Smads,with IC50 values of 1.4 and 2.1 mM,respectively.These findings suggest that these fungal metabolites may provide a new direction in the development of FOP therapeutics.展开更多
基金Supported by Gansu Provincial Science and Technology Program Project,No.008108491108。
文摘BACKGROUND Myositis ossificans(MO)is a rare disease involving the formation of bone outside the musculoskeletal system.While surgical intervention is the main treatment approach,preventing recurrence and standardized rehabilitation are also crucial.Here,we present a surgical strategy to prevent the recurrence of MO.CASE SUMMARY A 28-year-old female patient was admitted for the first time for a comminuted fracture of the left olecranon.However,incorrect postoperative rehabilitation resulted in the development of elbow joint stiffness with ectopic ossification,causing a loss of normal range of motion.The patient was diagnosed with MO based on physical examination,X-ray findings,and clinical presentation.We devised a surgical strategy to remove MO,followed by fixation with an Ilizarov frame,and implemented a scientifically reasonable rehabilitation plan.The surgery lasted for 3 h with an estimated blood loss of 45 mL.A drainage tube was placed after surgery,and fluid was aspirated through ultrasound-guided puncture.The patient experienced a significant reduction in joint stiffness after surgery.In the final follow-up at 9 mouths,there was evident improvement in the range of motion of the elbow joint,and no other symptoms were reported.CONCLUSION The Ilizarov frame is an advantageous surgical technique for facilitating rehabilitation after MO removal.It offers benefits such as passive recovery,individualized treatment,and prompt recovery.
文摘BACKGROUND Myositis ossificans(MO)is an uncommon disorder characterized by heterotopic ossification within soft tissues.Only a few cases of intra-abdominal MO(IMO)have been described in the literature.Histology could be difficult to understand and a wrong diagnosis could lead to an improper cure.CASE SUMMARY We herein report the case of IMO in a healthy 69-year-old man.The patient presented with an abdominal mass in the left lower quadrant.A computed tomography scan showed an inhomogeneous mass with multiple calcifications.The patient underwent radical excision of the mass.Histopathological findings were compatible with MO.Five months later the patient showed a recurrence causing hemorrhagic shock due to intractable intralesional bleeding.The patients eventually died within three months since recurrence.CONCLUSION The case described could be classified as post-traumatic MO that developed close to the previously fractured iliac bone.The subsequent surgical procedure was ineffective and the disease rapidly recurred.The misleading intraoperative diagnosis led to improper surgical treatment with a dramatic evolution.
文摘BACKGROUND Nontraumatic myositis ossificans is a rare disease whose specific pathogenesis is unclear.Early diagnosis of this disease is very difficult in children because of difficulties in determining medical history and nonspecific early clinical manifestations,which may lead to the failure of timely and effective diagnosis and treatment in some patients.We report the diagnosis and treatment of a child with nontraumatic myositis ossificans and summarize the clinical characteristics and diagnosis and treatment of the disease.CASE SUMMARY An 8-year-old girl first came to our hospital for more than a week with pain in the right lower limb.There was no history of trauma or strenuous activities.On physical examination,no mass on the right thigh was found,and the movement of the right lower extremity was limited.Ultrasonography showed synovitis of the hip,and bed rest was recommended.Three days later,the child’s pain persisted and worsened,accompanied by fever and other discomforts.She came to our hospital again and a mass was found on the right thigh with redness and swelling on the surface.The images showed a soft tissue tumor on the right thigh with calcification.Routine blood tests revealed that the inflammation index was significantly increased.In case of infection,the patient was given antibiotics,and the pain was relieved soon after,without fever.However,the right thigh mass persisted and hardened.The patient underwent incision biopsy more than 1 mo later,and the postoperative pathology showed nontraumatic myositis ossificans.After approximately 9 mo of observation,the tumor still persisted,which affected the life of the child,and then resection was performed.Since follow-up,there has been no recurrence.CONCLUSION Due to the difficulty in discerning a child’s medical history and the diverse early manifestations,it is difficult to diagnose nonossifying muscle disease in children in its early stage.Measures such as timely follow-up and periodic image monitoring are conducive to early diagnosis of the disease.The disease has a certain degree of self-limitation,and it can be observed and treated first.If the tumor persists in the later stage or affects functioning,then surgery is considered.
基金Supported by The University of Alabama at Birmingham,Alabama and The Orthopaedic Center,Birmingham,AL,United States
文摘A case of florid reactive periostitis ossificans(RPO) arising in a long bone is presented. This is a rare bone proliferation with a pronounced periosteal reaction. Less than 100 cases have been described in the literature with far fewer outside the bones of the hand, feet, fingers, and toes. Although the etiology is unknown, a relationship to preceding trauma is suggested. The imaging and histologic features show an overlap with other bone lesions including bizarre parosteal osteochondromatous proliferation, subungual exostosis, and malignant surface tumors of bone and cartilage which include, periosteal and parosteal osteosarcoma. It is important to recognize the clinical presentation and diagnostic features of RPO as a benign entity so that it is not mistaken for a more aggressive neoplasm. We present a case of a right distal humeral lesion that on histopathological review revealed florid RPO. This diagnosis was not suspected on imaging studies, but was made on open biopsy of the mass. The patient remains disease free, years postoperatively. In addition to presenting this unique case report, we review the pertinent literature, and offer a differential diagnosis and treatment strategy for its management.
文摘Myositis ossificans is a benign self limiting condition that usually related to trauma. Despite a clinically and histologically distinct entity, myositis ossificans still causes considerable difficulties in diagnosis. A 33-year-old Moroccan woman presented with a 2-month history of left inguinal inflammatory pain with limping gait, MRI examination suggested a malignant neoplasm such as soft tissue osteosarcoma. A diagnosis of myositis ossificans was made by incisional biopsy. Conservative management with clinical and radiological follow up of 19 months confirm the diagnosis. The symptoms resolved within seven months. Myositis ossificans should be considered by clinicians as a possible diagnosis for a soft tissue lesion.
文摘ARACHNOIDITIS ossificans (AO) is a rare type of chronic arachnoiditis presence of calcification spinal arachnoid. Small characterized by the in, or ossification of the plaques of calcificationof the dura mater are frequently observed in radiography or during surgery, which are often asymptomatic and have no apparent clinical significance. AO and dura calcification should not be confused.
文摘Introduction: Fibrodysplasia ossificans progressiva (FOP) is a rare autosomal dominant disorder of skeletal malformations and progressive extraskeletal ossification. Patients and Methods: In the present study, we present a case of FOP with marked progressive ossification of extraskeletal tissues. We investigated whether soluble factors in serum would affect the osteoblast phenotype by examining the effects of serum from this patient and control subjects on mouse osteoblastic MC3T3-E1 cells. Results: The clinical findings of this patient were compatible with FOP, and direct sequence analysis of genomic DNA demonstrated the presence of a heterozygous 617G> A (R206H) mutation of activin type 1 receptor (ACVR1). Serum from the FOP patient enhanced the level of alkaline phosphatase (ALP) in Western blotting, compared with serum from the control, in MC3T3-E1 cells. Moreover, serum from the FOP patient enhanced the levels of ALP, osteocalcin and bone morphogenetic protein-2 mRNA in these cells. Conclusion: We presented a case of FOP with progressive ossification in extra-skeletal tissues with ACVR1 mutation. The present data suggest that the serum from this patient includes some soluble factors, which might enhance the osteoblast differentiation and BMP-2 expression in mouse osteoblastic cells.
文摘Myositis ossificans (MO) is an extraskeletal tumor-like lesion with bone formation, and is relatively rare in the head and neck region. We report herein a clinicopathological analysis of MO associated with a muscle of mastication. The patient was a 70-year-old Japanese woman who presented with a tumorous mass in the right parotid area and no history of trauma. The surgically excised tumorous lesion consisted of proliferating fibroblastic cells with no atypia in the central portion, and a formation of trabecular osteoid and/or bony tissue in the peripheral portion as the so-called “zonal phenomenon”. The final histological diagnosis was MO, and the lesion was located in the posterior belly of the digastric muscle on computer tomography. A review of the literature showed that many cases of MO predominantly affected the medial pterygoid and masseter muscle and showed a history of previous trauma. The present case represents a rare case of nontraumatic MO in the uncommon location of the posterior belly of the digastric muscle.
文摘Arachnoiditis ossificans is an intradural extramedullary lesion resulting from an unusual chronic meningeal inflammatory process and it is thought to be the sequela of end-stage adhesive arachnoiditis secondary to surgery, trauma, arachnoid hemorrhage, meningeal irritation, myelography (particularly oil-based contrast agents), and spinal anesthesia. The spinal arachnoiditis ossificans may be silent or cause a variety of symptoms depending on its location and uncommonly happen to cause spinal cord compression. Very little attention has been paid to the management and outcome of this rare condition in the neurosurgical reem. We report the case of a 45 years old man admitted with L1 - L2 arachnoiditis ossificans revealed by polyradiculopathy on incomplete cauda equina syndrome mimicking spinal canal tumors. The patient underwent surgery and we performed the laminectomy of L1 - L2, total resection of the lesion, followed by a complete remission of the hemiparesis after three months of kinesitherapy nursing.
文摘Background Fibrodysplasia ossificans progressiva(FOP)is a rare and disabling heritable connective tissue disease that is difficult to treat.This study seeks to explore the clinical characteristics,clinical manifestations,treatment and prognosis of FOP to provide a clinical basis for its early diagnosis and treatment.Methods Twenty-six children with FOP were retrospectively analyzed in terms of their onset,clinical manifestations,auxiliary examinations and treatment.Results Among the 26 cases,the youngest age of manifestation of mass was 8 days after birth,and the average age was 3 years and 2 months.The peak age was 2-5 years old.Inflammatory mass and toe-finger deformity are the main early clinical manifestations of the disease.These inflammatory masses often lead to hard osteogenic deposits that initially mainly involve the central axis,such as the neck(22/26,84.6%),back(20/26,76.9%),and head(13/26,50%).Toe-finger deformity mainly manifests as symmetrical great toe deformity,or short and deformed thumb and little finger.The diagnosis of FOP requires typical clinical manifestations or ACVR1 gene detection.The main therapeutic drugs for FOP include glucocorticoids and non-steroidal anti-inflammatory drugs.Although not compliant with the recommended medical management of FOP,in our clinical practice children with uncontrollable illness could be treated using a variety of immunosuppressive agents in combination.Conclusions FOP is a rare autosomal dominant heritable disease.The main clinical manifestations observed in this study were recurrent inflammatory mass and toe-finger deformity.If the diagnosis and treatment are not performed in a timely manner,serious complications are likely to affect the prognosis.Therefore,early diagnosis and active treatment should be performed.
文摘Myositis ossificans circumscripta (MOC) is a kind of self-localized, benign and tumor-like lesions often seen in adults, with approximately 75% of cases caused by trauma. We reported a case of non-traumatic MOC occurred at the elbowjoint in a 9-year old child and it has been excised by surgery. After 18 months follow-up, a favorable outcome has been achieved with the Broberg-Morrey score of 100. We suggest that surgical resection should be done as soon as the diagnosis is confirmed.
文摘ObjectiveTo describe the clinical manifestations and radiographic characteristics of fibrodysplasia ossificans progressiva (FOP). MethodsThe radiographs and lesion photographs of five patients aged between 23 months and 17 years diagnosed as FOP were presented and the literatures were reviewed. ResultsClinical features were shortened great toes at birth, episodic soft tissue swellings, pain in the subcutaneous tissues of neck, back, shoulders, and extremities, hard lumps at back and spine, and restricted mobility of neck and arms. Radiological findings included short digit with hallus valgus, scoliosis, exostoses on many bones, and ossification of ligament. The characteristic subcutaneous soft tissues included masses or ossifications of neck, back, extremities, and shoulders. ConclusionThe short terminal phalanges of the thumbs and intermittently progressive heterotopic ossifications of the striated muscles and soft tissues suggest that FOP should promptly get early genetic consultation.
文摘Fibrodysplasia ossificans progressiva(FOP)is a rare autosomal dominant congenital disorder characterized by progressive heterotopic ossification in muscle tissues.A constitutively activated mutation of a bone morphogenetic protein(BMP)receptor,ALK2,has been identified in patients with FOP.We report here that four structurally related compounds,lucilactaene,hydroxylucilactaene,NG-391 and NG-393,produced by fungal strain Fusarium sp.B88,inhibit BMP signaling in vitro.Alkaline phosphatase activity,a marker enzyme of osteoblastic differentiation,was decreased in C2C12 myoblasts stably expressing mutant ALK2 by treatment with those compounds with IC_(50) values of 5.7,6.8,6.9 and 6.1 mM,respectively.Furthermore,NG-391 and NG-393 inhibited BMP-specific luciferase reporter activity,which is directly regulated by transcription factor Smads,with IC50 values of 1.4 and 2.1 mM,respectively.These findings suggest that these fungal metabolites may provide a new direction in the development of FOP therapeutics.