Dear Editor,Ciliary body masses are diagnostically challenging due to their hidden location,diverse pathologies,and limited examination methods[1].We report a case of a ciliary body inflammatory mass treated with tran...Dear Editor,Ciliary body masses are diagnostically challenging due to their hidden location,diverse pathologies,and limited examination methods[1].We report a case of a ciliary body inflammatory mass treated with trans-scleral excision and antiinflammatory therapy,preserving functional vision.展开更多
Growth and nutritional status are important issues in paediatric inflammatory bowel disease(IBD).While linear growth is easy to assess,nutritional status is more complicated,with reports often compromised by the use o...Growth and nutritional status are important issues in paediatric inflammatory bowel disease(IBD).While linear growth is easy to assess,nutritional status is more complicated,with reports often compromised by the use of simple measures,such as weight and the body mass index,to assess nutritional status rather than more appropriate and sophisticated techniques to measure body composition.This review is an update on what is currently known about nutritional status as determined by body composition in paediatric IBD.Further,this review will focus on the impact of biologics on growth in paediatric IBD.Significant lean mass deficits have been reported in children with IBD compared with controls,and there is evidence these deficits persist over time.Furthermore,data imply that gender differences exist in body composition,both at diagnosis and in response to treatment.With respect to growth improvements following treatment with biologics,there are conflicting data.While some studies report enhancement of growth,others do not.The relationship between disease severity,impaired growth and the requirement for biologics needs to be considered when interpreting these data.However,key features associated with improvements in growth appear to be successful clinical response to treatment,patients in early stages of puberty,and the presence of growth failure at the onset of treatment.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 by National Natural Science Foundation of China(No.82101113).
文摘Dear Editor,Ciliary body masses are diagnostically challenging due to their hidden location,diverse pathologies,and limited examination methods[1].We report a case of a ciliary body inflammatory mass treated with trans-scleral excision and antiinflammatory therapy,preserving functional vision.
基金Supported by Hill RJ in receipt of the Reginald Ferguson Research Fellowship in Gastroenterology,The University of Queensland
文摘Growth and nutritional status are important issues in paediatric inflammatory bowel disease(IBD).While linear growth is easy to assess,nutritional status is more complicated,with reports often compromised by the use of simple measures,such as weight and the body mass index,to assess nutritional status rather than more appropriate and sophisticated techniques to measure body composition.This review is an update on what is currently known about nutritional status as determined by body composition in paediatric IBD.Further,this review will focus on the impact of biologics on growth in paediatric IBD.Significant lean mass deficits have been reported in children with IBD compared with controls,and there is evidence these deficits persist over time.Furthermore,data imply that gender differences exist in body composition,both at diagnosis and in response to treatment.With respect to growth improvements following treatment with biologics,there are conflicting data.While some studies report enhancement of growth,others do not.The relationship between disease severity,impaired growth and the requirement for biologics needs to be considered when interpreting these data.However,key features associated with improvements in growth appear to be successful clinical response to treatment,patients in early stages of puberty,and the presence of growth failure at the onset of treatment.
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.
