A 20-month-old male infant with multiorgan dysfunction after Epstein-Barr virus(EBV)infection developed Reye’s syndrome.He also suffered from acute liver failure,life-threatening cerebral edema,severe disseminated in...A 20-month-old male infant with multiorgan dysfunction after Epstein-Barr virus(EBV)infection developed Reye’s syndrome.He also suffered from acute liver failure,life-threatening cerebral edema,severe disseminated intravascular coagulation(DIC),and myocardial involvement.EBV infection aggravated the progress of Reye’s syndrome,leading to death despite full supportive and symptomatic therapy.This critical case suggested that pediatricians should pay attention to multiorgan involvement of severe EBV infection.展开更多
We report two cases of rapidly fatal hemophagocytic lymphohistiocytosis (HLH) that presented as Reye’s syndrome (RS). The patients were referred to our hospital because of altered level of consciousness with hyperamm...We report two cases of rapidly fatal hemophagocytic lymphohistiocytosis (HLH) that presented as Reye’s syndrome (RS). The patients were referred to our hospital because of altered level of consciousness with hyperammonemia and hypoglycemia. The first patient, a 24-month-old girl (Case 1), died soon after arrival and was clinically diagnosed as having RS, but a diagnosis of HLH was established on the basis of autopsy findings. The other patient, a 4-month-old boy (Case 2), was diagnosed as having HLH in view of the bone marrow findings on admission, but immunosuppressive therapy failed to prevent a rapidly fatal course. Marked hypercytokinemia was found in both patients, and liver pathology demonstrated panlobular microvesicular steatosis of hepatocytes, resembling that in RS. Mononuclear cell infiltration with hemophagocytosis in the liver was evident in case 2, but not in case 1. In both cases, hypercytokinemia was thought to have caused mitochondrial dysfunction, resulting in RS-like microvesicular steatosis of hepatocytes. HLH is an important differential diagnosis of children presenting with RS-like picture.展开更多
文摘A 20-month-old male infant with multiorgan dysfunction after Epstein-Barr virus(EBV)infection developed Reye’s syndrome.He also suffered from acute liver failure,life-threatening cerebral edema,severe disseminated intravascular coagulation(DIC),and myocardial involvement.EBV infection aggravated the progress of Reye’s syndrome,leading to death despite full supportive and symptomatic therapy.This critical case suggested that pediatricians should pay attention to multiorgan involvement of severe EBV infection.
文摘We report two cases of rapidly fatal hemophagocytic lymphohistiocytosis (HLH) that presented as Reye’s syndrome (RS). The patients were referred to our hospital because of altered level of consciousness with hyperammonemia and hypoglycemia. The first patient, a 24-month-old girl (Case 1), died soon after arrival and was clinically diagnosed as having RS, but a diagnosis of HLH was established on the basis of autopsy findings. The other patient, a 4-month-old boy (Case 2), was diagnosed as having HLH in view of the bone marrow findings on admission, but immunosuppressive therapy failed to prevent a rapidly fatal course. Marked hypercytokinemia was found in both patients, and liver pathology demonstrated panlobular microvesicular steatosis of hepatocytes, resembling that in RS. Mononuclear cell infiltration with hemophagocytosis in the liver was evident in case 2, but not in case 1. In both cases, hypercytokinemia was thought to have caused mitochondrial dysfunction, resulting in RS-like microvesicular steatosis of hepatocytes. HLH is an important differential diagnosis of children presenting with RS-like picture.
