Pulmonary hemosiderosis is defined as the clinical and functional consequence of iron overload of the lungs,which usually occurs due to recurrent intra-alveolar bleeding.It can manifest as miliary mottling and should ...Pulmonary hemosiderosis is defined as the clinical and functional consequence of iron overload of the lungs,which usually occurs due to recurrent intra-alveolar bleeding.It can manifest as miliary mottling and should be entertained in the differential diagnosis of patients presenting with miliary nodules on chest radiography,especially those with mitral stenosis.The management of secondary pulmonary hemosiderosis secondary to valvular heart disease includes valvuloplasty and/or valve replacement.The radiological opacities may disappear with successful treatment of the underlying valvular disease in many patients.However,they may persist with no physiological impairment to the patient.Here,we present a 32-year-old man with mitral stenosis who presented with fever and miliary shadows on chest radiography,which was ultimately diagnosed as secondary pulmonary hemosiderosis.展开更多
Focal nodular hyperplasia(FNH) is the second most common benign lesion of the liver.It is a solitary lesion and usually does not enlarge.We present the magnetic resonance imaging findings of multiple progressive FNH l...Focal nodular hyperplasia(FNH) is the second most common benign lesion of the liver.It is a solitary lesion and usually does not enlarge.We present the magnetic resonance imaging findings of multiple progressive FNH lesions in a patient with hemosiderosis using GadoliniumEOB-DTPA(Eovist) as a hepatobiliary contrast agent.The possible mechanisms underlying the occurrence and progression of FNH lesions and the potential value of Eovist in characterizing the lesions were discussed.展开更多
The congenital dyserythropoietic anemias comprise a group of rare hereditary disorders of erythropoiesis, characterized by ineffective erythropoiesis as the predominant mechanism of anemia and by characteristic morpho...The congenital dyserythropoietic anemias comprise a group of rare hereditary disorders of erythropoiesis, characterized by ineffective erythropoiesis as the predominant mechanism of anemia and by characteristic morphological aberrations of the majority of erythroblasts in the bone marrow. Congenital dyserythropoietic anemia type II is the most frequent type. All types of congenital dyserythropoietic anemias distinctly share a high incidence of iron loading. Iron accumulation occurs even in untransfused patients and can result in heart failure and liver cirrhosis. We have reported about a patient who presented with liver cirrhosis and intractable ascites caused by congenital dyserythropoietic anemia type II. Her clinical course was further complicated by the development of autoimmune hemolytic anemia. Splenectomy was eventually performed which achieved complete resolution of ascites, increase of hemoglobin concentration and abrogation of transfusion requirements.展开更多
Background and Aims: Hepatocellular iron accumulation in patients with chronic liver disease has been linked to adverse outcomes. The objective of this study was to identify clinical factors associated with hemosidero...Background and Aims: Hepatocellular iron accumulation in patients with chronic liver disease has been linked to adverse outcomes. The objective of this study was to identify clinical factors associated with hemosiderosis. Methods: A total of 103 consecutive liver transplant recipients were identified, in whom liver biopsy had been performed prior to transplantation. Laboratory and clinical data at biopsy and transplant were abstracted from the medical records and hepatocyte iron was graded in the biopsy and explant. The association of change in iron score from biopsy to transplant, with the time interval between these two events, was examined using linear mixed model analysis for repeated measures. Results: Most subjects had advanced fibrosis (F3-F4) at liver biopsy, which was performed on average about 2.5 years before transplant. Over 80% of patients had no or 1+ hepatocyte iron at biopsy;iron increased between biopsy and transplant in about 40%. The only demographic or clinical feature that correlated with increased iron was the presence of a transjugular intrahepatic portosystemic shunt. Increased iron at transplant was associated with higher serum iron and transferrin saturation at biopsy, and with lower hemoglobin level, greater mean corpuscular volume, mean corpuscular hemoglobin and mean corpuscular hemoglobin concentration, higher ferritin and model for end-stage liver disease score at transplant.Conclusions: The development of hemosiderosis in end-stage liver disease is associated with lower hemoglobin levels and alterations in red blood cell indices that are suggestive of hemolysis. These observations suggest that extravascular hemolysis may play a role in the development of secondary iron overload.展开更多
文摘Pulmonary hemosiderosis is defined as the clinical and functional consequence of iron overload of the lungs,which usually occurs due to recurrent intra-alveolar bleeding.It can manifest as miliary mottling and should be entertained in the differential diagnosis of patients presenting with miliary nodules on chest radiography,especially those with mitral stenosis.The management of secondary pulmonary hemosiderosis secondary to valvular heart disease includes valvuloplasty and/or valve replacement.The radiological opacities may disappear with successful treatment of the underlying valvular disease in many patients.However,they may persist with no physiological impairment to the patient.Here,we present a 32-year-old man with mitral stenosis who presented with fever and miliary shadows on chest radiography,which was ultimately diagnosed as secondary pulmonary hemosiderosis.
文摘Focal nodular hyperplasia(FNH) is the second most common benign lesion of the liver.It is a solitary lesion and usually does not enlarge.We present the magnetic resonance imaging findings of multiple progressive FNH lesions in a patient with hemosiderosis using GadoliniumEOB-DTPA(Eovist) as a hepatobiliary contrast agent.The possible mechanisms underlying the occurrence and progression of FNH lesions and the potential value of Eovist in characterizing the lesions were discussed.
文摘The congenital dyserythropoietic anemias comprise a group of rare hereditary disorders of erythropoiesis, characterized by ineffective erythropoiesis as the predominant mechanism of anemia and by characteristic morphological aberrations of the majority of erythroblasts in the bone marrow. Congenital dyserythropoietic anemia type II is the most frequent type. All types of congenital dyserythropoietic anemias distinctly share a high incidence of iron loading. Iron accumulation occurs even in untransfused patients and can result in heart failure and liver cirrhosis. We have reported about a patient who presented with liver cirrhosis and intractable ascites caused by congenital dyserythropoietic anemia type II. Her clinical course was further complicated by the development of autoimmune hemolytic anemia. Splenectomy was eventually performed which achieved complete resolution of ascites, increase of hemoglobin concentration and abrogation of transfusion requirements.
文摘Background and Aims: Hepatocellular iron accumulation in patients with chronic liver disease has been linked to adverse outcomes. The objective of this study was to identify clinical factors associated with hemosiderosis. Methods: A total of 103 consecutive liver transplant recipients were identified, in whom liver biopsy had been performed prior to transplantation. Laboratory and clinical data at biopsy and transplant were abstracted from the medical records and hepatocyte iron was graded in the biopsy and explant. The association of change in iron score from biopsy to transplant, with the time interval between these two events, was examined using linear mixed model analysis for repeated measures. Results: Most subjects had advanced fibrosis (F3-F4) at liver biopsy, which was performed on average about 2.5 years before transplant. Over 80% of patients had no or 1+ hepatocyte iron at biopsy;iron increased between biopsy and transplant in about 40%. The only demographic or clinical feature that correlated with increased iron was the presence of a transjugular intrahepatic portosystemic shunt. Increased iron at transplant was associated with higher serum iron and transferrin saturation at biopsy, and with lower hemoglobin level, greater mean corpuscular volume, mean corpuscular hemoglobin and mean corpuscular hemoglobin concentration, higher ferritin and model for end-stage liver disease score at transplant.Conclusions: The development of hemosiderosis in end-stage liver disease is associated with lower hemoglobin levels and alterations in red blood cell indices that are suggestive of hemolysis. These observations suggest that extravascular hemolysis may play a role in the development of secondary iron overload.