BACKGROUND Acute fulminant liver failure rarely occurs in the neonatal period.The etiologies include viral infection(15%),metabolic/genetic disease(10%),hematologic disorders(15%),and ischemic injury(5%).Gestational a...BACKGROUND Acute fulminant liver failure rarely occurs in the neonatal period.The etiologies include viral infection(15%),metabolic/genetic disease(10%),hematologic disorders(15%),and ischemic injury(5%).Gestational alloimmune liver disease usually manifests as severe neonatal liver failure,with extensive hepatic and extrahepatic iron overload,sparing the reticuloendothelial system.Empty liver failure is a rare cause of liver failure where a patient presents with liver failure in the neonatal period with no hepatocytes in liver biopsy.CASE SUMMARY A 5-week-old male presented with jaundice.Physical examination revealed an alert but deeply icteric infant.Laboratory data demonstrated direct hyperbilirubinemia,a severely deranged coagulation profile,normal transaminase,and normal ammonia.Magnetic resonance imaging of the abdomen was suggestive of perinatal hemochromatosis.Liver biopsy showed histiocytic infiltration with an absence of hepatocytes.No hemosiderin deposition was identified in a buccal mucosa biopsy.CONCLUSION Neonatal liver failure in the absence of hepatocellular regeneration potentially reflects an acquired or inborn defect in the regulation of hepatic regeneration.展开更多
When oral feeding cannot provide adequate nutritional support to children,enteral tube feeding becomes a necessity.The overall aim is to ultimately promote appropriate growth,improve the patient’s quality of life and...When oral feeding cannot provide adequate nutritional support to children,enteral tube feeding becomes a necessity.The overall aim is to ultimately promote appropriate growth,improve the patient’s quality of life and increase carer satisfaction.Nasogastric tube feeding is considered appropriate on a short-term basis.Alternatively,gastrostomy feeding offers a more convenient and safer feeding option especially as it does not require frequent replacements,and carries a lower risk of complications.Gastrostomy tube feeding should be considered when nasogastric tube feeding is required for more than 2-3 wk as per the ESPEN guidelines on artificial enteral nutrition.Several techniques can be used to insert gastrostomies in children including endoscopic,image guided and surgical gastrostomy insertion whether open or laparoscopic.Each technique has its own advantages and disadvantages.The timing of gastrostomy insertion,device choice and method of insertion is dependent on the local expertise,patient requirements and family preference,and should be individualized with a multidisciplinary team approach.We aim to review gastrostomy insertion in children including indications,contraindications,history of gastrostomy,insertion techniques and complications.展开更多
Coronavirus disease 2019(COVID-19)typically presents with fever and respiratory symptoms in children.Most children develop an asymptomatic and mild illness,with a minority requiring specialist medical care.Gastrointes...Coronavirus disease 2019(COVID-19)typically presents with fever and respiratory symptoms in children.Most children develop an asymptomatic and mild illness,with a minority requiring specialist medical care.Gastrointestinal manifestations and liver injury can also occur in children following infection.The mechanisms of liver injury may include infection following direct viral hepatic tissue invasion,immune response,or medication effects.Affected children might develop mild liver dysfunction which has a benign course in most children with no pre-existing liver disease.However,the presence of non-alcoholic fatty liver disease or other pre-existing chronic liver disorders is associated with a higher risk of developing severe COVID-19 illness with poor outcomes.On the other hand,the presence of liver manifestations is associated with the severity of COVID-19 disease and is considered an independent prognostic factor.Respiratory,hemodynamic,and nutritional supportive therapies are the mainstay of management.Vaccination of children at increased risk of developing severe COVID-19 disease is indicated.This review describes the liver manifestations in children with COVID-19,detailing its epidemiology,basic mechanisms,clinical expression,management,and prognosis in those with and without pre-existing liver disease and also children who have had earlier liver transplantation.展开更多
文摘BACKGROUND Acute fulminant liver failure rarely occurs in the neonatal period.The etiologies include viral infection(15%),metabolic/genetic disease(10%),hematologic disorders(15%),and ischemic injury(5%).Gestational alloimmune liver disease usually manifests as severe neonatal liver failure,with extensive hepatic and extrahepatic iron overload,sparing the reticuloendothelial system.Empty liver failure is a rare cause of liver failure where a patient presents with liver failure in the neonatal period with no hepatocytes in liver biopsy.CASE SUMMARY A 5-week-old male presented with jaundice.Physical examination revealed an alert but deeply icteric infant.Laboratory data demonstrated direct hyperbilirubinemia,a severely deranged coagulation profile,normal transaminase,and normal ammonia.Magnetic resonance imaging of the abdomen was suggestive of perinatal hemochromatosis.Liver biopsy showed histiocytic infiltration with an absence of hepatocytes.No hemosiderin deposition was identified in a buccal mucosa biopsy.CONCLUSION Neonatal liver failure in the absence of hepatocellular regeneration potentially reflects an acquired or inborn defect in the regulation of hepatic regeneration.
文摘When oral feeding cannot provide adequate nutritional support to children,enteral tube feeding becomes a necessity.The overall aim is to ultimately promote appropriate growth,improve the patient’s quality of life and increase carer satisfaction.Nasogastric tube feeding is considered appropriate on a short-term basis.Alternatively,gastrostomy feeding offers a more convenient and safer feeding option especially as it does not require frequent replacements,and carries a lower risk of complications.Gastrostomy tube feeding should be considered when nasogastric tube feeding is required for more than 2-3 wk as per the ESPEN guidelines on artificial enteral nutrition.Several techniques can be used to insert gastrostomies in children including endoscopic,image guided and surgical gastrostomy insertion whether open or laparoscopic.Each technique has its own advantages and disadvantages.The timing of gastrostomy insertion,device choice and method of insertion is dependent on the local expertise,patient requirements and family preference,and should be individualized with a multidisciplinary team approach.We aim to review gastrostomy insertion in children including indications,contraindications,history of gastrostomy,insertion techniques and complications.
文摘Coronavirus disease 2019(COVID-19)typically presents with fever and respiratory symptoms in children.Most children develop an asymptomatic and mild illness,with a minority requiring specialist medical care.Gastrointestinal manifestations and liver injury can also occur in children following infection.The mechanisms of liver injury may include infection following direct viral hepatic tissue invasion,immune response,or medication effects.Affected children might develop mild liver dysfunction which has a benign course in most children with no pre-existing liver disease.However,the presence of non-alcoholic fatty liver disease or other pre-existing chronic liver disorders is associated with a higher risk of developing severe COVID-19 illness with poor outcomes.On the other hand,the presence of liver manifestations is associated with the severity of COVID-19 disease and is considered an independent prognostic factor.Respiratory,hemodynamic,and nutritional supportive therapies are the mainstay of management.Vaccination of children at increased risk of developing severe COVID-19 disease is indicated.This review describes the liver manifestations in children with COVID-19,detailing its epidemiology,basic mechanisms,clinical expression,management,and prognosis in those with and without pre-existing liver disease and also children who have had earlier liver transplantation.