Splenic infarction(SI)is a rare clinical phenomenon that occurs when the blood supply to the spleen is interrupted,resulting in tissue ischemia and necrosis[1].There are many causes of SI;in adult patients,cardioembol...Splenic infarction(SI)is a rare clinical phenomenon that occurs when the blood supply to the spleen is interrupted,resulting in tissue ischemia and necrosis[1].There are many causes of SI;in adult patients,cardioembolism,hematological malignancy,and infectious disease are the most common[2].Rheumatic diseases,especially systemic lupus erythematosus(SLE)and granulomatosis with polyangitis,can also lead to SI[3];however,only a few cases have been reported.There are currently no statistically meaningful data on the etiology of SI in children.Although most patients with SI can recover after conservative treatment,a small number of patients develop complications,such as splenic abscess,splenic rupture,splenic hemorrhage,and hemoperitoneum,and may even undergo splenectomy in severe cases.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
基金supported partly by the National Key R&D Program of China(Grant Number 2021YFC2702005)Beijing Hospitals Authority’s Ascent Plan(DFL20221001).
文摘Splenic infarction(SI)is a rare clinical phenomenon that occurs when the blood supply to the spleen is interrupted,resulting in tissue ischemia and necrosis[1].There are many causes of SI;in adult patients,cardioembolism,hematological malignancy,and infectious disease are the most common[2].Rheumatic diseases,especially systemic lupus erythematosus(SLE)and granulomatosis with polyangitis,can also lead to SI[3];however,only a few cases have been reported.There are currently no statistically meaningful data on the etiology of SI in children.Although most patients with SI can recover after conservative treatment,a small number of patients develop complications,such as splenic abscess,splenic rupture,splenic hemorrhage,and hemoperitoneum,and may even undergo splenectomy in severe cases.
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.
