Pancreatitis in patients with organic acidemias.

摘要: Abstract Study objective : The discovery of pancreatitis in two children with methylmalonic acidemia led us to review the experience a large number patients organic acidemias determine whether is an important complication these disorders. Design Case series. Setting Pediatric metabolism services at five tertiary care centers. Patients Records all followed institutions during past 10 years were reviewed. Pancreatitis was recognized by symptoms and laboratory findings confirmed imaging studies, surgery, or autopsy. At three cases younger than Measurements results Nine (seven acute chronic cases) identified among 108 branched-chain acidemias. They ranged age from 13 months 9 years. Five had acidemia, isovaleric one maple syrup urine disease. There deaths; hemorrhagic occurred children, found autopsy third. All after occurrence pancreatitis. survey 57 other (none inborn error) addition errors included this study. Conclusions Acute may complicate must be considered assessment disorders who have clinical deterioration vomiting, abdominal pain, encephalopathy shock, milder symptoms. Conversely, error acid should unknown origin. (J P EDIATR 1994;124:239-43)