AIM To evaluate the risk profile of sulfur hexafluoride in voiding urosonography(VUS) based on a large cohort of children.METHODS Since 2011 sulfur hexafluoride(SH,SonoV ue?,Bracco,Italy) is the only ultrasound contra...AIM To evaluate the risk profile of sulfur hexafluoride in voiding urosonography(VUS) based on a large cohort of children.METHODS Since 2011 sulfur hexafluoride(SH,SonoV ue?,Bracco,Italy) is the only ultrasound contrast available in the European Union and its use in children has not been approved.Within a 4-year-period,531 children with suspected or proven vesicoureteral reflux(f/m = 478/53; mean age 4.9 years; 1 mo-25.2 years) following parental informed consent underwent VUS with administration of 2.6 ± 1.2 mL SH in a two-center study.A standardizedtelephone survey on adverse events was conducted three days later.RESULTS No acute adverse reactions were observed.The survey revealed subacute,mostly self-limited adverse events in 4.1%(22/531).The majority of observed adverse events(17/22) was not suspected to be caused by an allergic reaction: Five were related to catheter placement,three to reactivated urinary tract infections,five were associated with perineal disinfection before voiding urosonography or perineal dermatitis and four with a common cold.In five patients(0.9%) hints to a potential allergic cause were noted: Perineal urticaria was reported in three interviews and isolated,mild fever in two.These were minor self-limited adverse events with a subacute onset and no hospital admittance was necessary.Ninety-six point two percent of the parents would prefer future VUS examinations with use of SH.CONCLUSION No severe adverse events were observed and indications of self-limited minor allergic reactions related to intravesical administration of SH were reported in less than 1%.展开更多
Inflammatory bowel diseases(IBD) represent challenges, both from a diagnostic, and therapeutic point of view. Deep-seated anatomic structures are difficult to assess by ultrasound technique alone. As radiation-free al...Inflammatory bowel diseases(IBD) represent challenges, both from a diagnostic, and therapeutic point of view. Deep-seated anatomic structures are difficult to assess by ultrasound technique alone. As radiation-free alternative cross-sectional imaging method, magnetic resonance imaging of the intestinal structures is costly and time-consuming. Examination of pediatric patients imply additional considerations: reduction of body motions in younger children and consideration of the most appropriate preparation, and examination technique. The demanding Sellink technique is the only means for appropriately distending the lesser intestine in order to detect small bowel strictures. Oral intake of contrast medium(CM) alone shows its limitations regarding distensibility. The need for intravenous contrast media application needs to be considered, too. Active inflammation of both intestinal wall, and mesentery can be demonstrated accurately. Nevertheless, viable alternatives to CM application is desirable, considering non-negligible adverse reactions. Recent data suggest diffusion weighted imaging might fill this diagnostic gap. Irrespective of sequence technique chosen, bowel movement remains a major obstacle. Antispasmolytics in their function as smooth muscle relaxants help in improving image quality, however, their use in children might be off-label. Optimal preparation for the examination and appropriate imaging technique allow for diagnosing typical patterns of changes in IBD, such as bowel wall thickening, ulcers, mural stratification, strictures, creeping fat, and comb sign, and lymphadenopathy. The article gives a detailed overview of current significance of magnetic resonance imaging pediatric patients suffering from IBD, considering indications, limitations, and safety aspects.展开更多
Sialic acid storage disease (Salla disease) is an autosomal recessive disorder caused by mutations in a lysosomal sialic acid export protein, SLC17A5 (OMIM #604369). This disorder was initially described in Northern F...Sialic acid storage disease (Salla disease) is an autosomal recessive disorder caused by mutations in a lysosomal sialic acid export protein, SLC17A5 (OMIM #604369). This disorder was initially described in Northern Finland but more recently has been reported in patients of other ethnicities. We describe the clinical presentation and the neuroimaging findings of two non-Finnish children where a diagnosis of Salla disease was suspected on the basis of brain magnetic resonance imaging. The biochemical confirmation of this diagnosis posed a challenge as both patients had elevated percent free urine sialic acid but biochemical analyses in fibroblasts were not conclusive;therefore, molecular testing was necessary for confirmation of the diagnosis. The described encounters demonstrate the importance of pursuing confirmatory molecular diagnostic testing when a sialic acid storage disorder is suspected.展开更多
文摘AIM To evaluate the risk profile of sulfur hexafluoride in voiding urosonography(VUS) based on a large cohort of children.METHODS Since 2011 sulfur hexafluoride(SH,SonoV ue?,Bracco,Italy) is the only ultrasound contrast available in the European Union and its use in children has not been approved.Within a 4-year-period,531 children with suspected or proven vesicoureteral reflux(f/m = 478/53; mean age 4.9 years; 1 mo-25.2 years) following parental informed consent underwent VUS with administration of 2.6 ± 1.2 mL SH in a two-center study.A standardizedtelephone survey on adverse events was conducted three days later.RESULTS No acute adverse reactions were observed.The survey revealed subacute,mostly self-limited adverse events in 4.1%(22/531).The majority of observed adverse events(17/22) was not suspected to be caused by an allergic reaction: Five were related to catheter placement,three to reactivated urinary tract infections,five were associated with perineal disinfection before voiding urosonography or perineal dermatitis and four with a common cold.In five patients(0.9%) hints to a potential allergic cause were noted: Perineal urticaria was reported in three interviews and isolated,mild fever in two.These were minor self-limited adverse events with a subacute onset and no hospital admittance was necessary.Ninety-six point two percent of the parents would prefer future VUS examinations with use of SH.CONCLUSION No severe adverse events were observed and indications of self-limited minor allergic reactions related to intravesical administration of SH were reported in less than 1%.
文摘Inflammatory bowel diseases(IBD) represent challenges, both from a diagnostic, and therapeutic point of view. Deep-seated anatomic structures are difficult to assess by ultrasound technique alone. As radiation-free alternative cross-sectional imaging method, magnetic resonance imaging of the intestinal structures is costly and time-consuming. Examination of pediatric patients imply additional considerations: reduction of body motions in younger children and consideration of the most appropriate preparation, and examination technique. The demanding Sellink technique is the only means for appropriately distending the lesser intestine in order to detect small bowel strictures. Oral intake of contrast medium(CM) alone shows its limitations regarding distensibility. The need for intravenous contrast media application needs to be considered, too. Active inflammation of both intestinal wall, and mesentery can be demonstrated accurately. Nevertheless, viable alternatives to CM application is desirable, considering non-negligible adverse reactions. Recent data suggest diffusion weighted imaging might fill this diagnostic gap. Irrespective of sequence technique chosen, bowel movement remains a major obstacle. Antispasmolytics in their function as smooth muscle relaxants help in improving image quality, however, their use in children might be off-label. Optimal preparation for the examination and appropriate imaging technique allow for diagnosing typical patterns of changes in IBD, such as bowel wall thickening, ulcers, mural stratification, strictures, creeping fat, and comb sign, and lymphadenopathy. The article gives a detailed overview of current significance of magnetic resonance imaging pediatric patients suffering from IBD, considering indications, limitations, and safety aspects.
文摘Sialic acid storage disease (Salla disease) is an autosomal recessive disorder caused by mutations in a lysosomal sialic acid export protein, SLC17A5 (OMIM #604369). This disorder was initially described in Northern Finland but more recently has been reported in patients of other ethnicities. We describe the clinical presentation and the neuroimaging findings of two non-Finnish children where a diagnosis of Salla disease was suspected on the basis of brain magnetic resonance imaging. The biochemical confirmation of this diagnosis posed a challenge as both patients had elevated percent free urine sialic acid but biochemical analyses in fibroblasts were not conclusive;therefore, molecular testing was necessary for confirmation of the diagnosis. The described encounters demonstrate the importance of pursuing confirmatory molecular diagnostic testing when a sialic acid storage disorder is suspected.
