BACKGROUND Liver injury manifesting as hepatic enzyme abnormalities,has been occasionally identified to be a feature of primary or secondary Addison's disease,an uncommon endocrine disease characterized by adrenal...BACKGROUND Liver injury manifesting as hepatic enzyme abnormalities,has been occasionally identified to be a feature of primary or secondary Addison's disease,an uncommon endocrine disease characterized by adrenal insufficiency.There have been no more than 30 reported cases of liver injury explicitly attributed to Addison's disease.Liver injury resulting from adrenal insufficiency due to glucocorticoid withdrawal is exceptionally rarer.CASE SUMMARY A 42-year-old man presented with fatigue and mildly elevated transaminases.Laboratory investigations and imaging studies excluded common etiologies of liver injury.Based on the fact that the patient discontinued long-term therapy with prednisone approximately 2 weeks before he was found to have elevated transaminase levels and the observation that his cortisol was lower than the normal value,he was diagnosed as having hypertransaminasemia secondary to adrenal insufficiency caused by glucocorticoid withdrawal.The patient was infused intravenously with compound diisopropylamine dichloroacctate and compound glycyrrhizin,and his transaminase levels returned to normal after 1 week.Approximately 2 years later,the patient received hydroprednisone treatment for 2 days in an endoscopic sinus surgery.Eight days after he discontinued the hydroprednisone treatment,he developed symptoms reminiscent of glucocorticoid withdrawal syndrome.These symptoms resolved spontaneously after 1 week.Intriguingly,the patient did not develop hepatic dysfunction this time.CONCLUSION The present case,showing some unusual clinical features,highlights the importance of education of clinicians and patients to avoid improper discontinuation of glucocorticoid therapy and complete history taking for prompt recognition.展开更多
Type 1 diabetes(T1D) is an autoimmune disorder caused by inflammatory destruction of the pancreatic tissue. The etiopathogenesis and characteristics of the pathologic process of pancreatic destruction are well describ...Type 1 diabetes(T1D) is an autoimmune disorder caused by inflammatory destruction of the pancreatic tissue. The etiopathogenesis and characteristics of the pathologic process of pancreatic destruction are well described. In addition, the putative susceptibility genes for T1 D as a monoglandular disease and the relation to polyglandular autoimmune syndrome(PAS) have also been wellexplored. The incidence of T1 D has steadily increased in most parts of the world, especially in industrialized nations. T1 D is frequently associated with autoimmune endocrine and non-endocrine diseases and patients with T1 D are at a higher risk for developing several glandular autoimmune diseases. Familial clustering is observed, which suggests that there is a genetic predisposition. Various hypotheses pertaining to viral- and bacterialinduced pancreatic autoimmunity have been proposed, however a definitive delineation of the autoimmune pathomechanism is still lacking. In patients with PAS, pancreatic and endocrine autoantigens either colocalize on one antigen-presenting cell or are expressed on two/various target cells sharing a common amino acid, which facilitates binding to and activation of T cells. The most prevalent PAS phenotype is the adult type 3 variant or PAS type Ⅲ, which encompasses T1 D and autoimmune thyroid disease. This review discusses the findings of recent studies showing noticeable differences in the genetic background and clinical phenotype of T1 D either as an isolated autoimmune endocrinopathy or within the scope of polyglandular autoimmune syndrome.展开更多
文摘BACKGROUND Liver injury manifesting as hepatic enzyme abnormalities,has been occasionally identified to be a feature of primary or secondary Addison's disease,an uncommon endocrine disease characterized by adrenal insufficiency.There have been no more than 30 reported cases of liver injury explicitly attributed to Addison's disease.Liver injury resulting from adrenal insufficiency due to glucocorticoid withdrawal is exceptionally rarer.CASE SUMMARY A 42-year-old man presented with fatigue and mildly elevated transaminases.Laboratory investigations and imaging studies excluded common etiologies of liver injury.Based on the fact that the patient discontinued long-term therapy with prednisone approximately 2 weeks before he was found to have elevated transaminase levels and the observation that his cortisol was lower than the normal value,he was diagnosed as having hypertransaminasemia secondary to adrenal insufficiency caused by glucocorticoid withdrawal.The patient was infused intravenously with compound diisopropylamine dichloroacctate and compound glycyrrhizin,and his transaminase levels returned to normal after 1 week.Approximately 2 years later,the patient received hydroprednisone treatment for 2 days in an endoscopic sinus surgery.Eight days after he discontinued the hydroprednisone treatment,he developed symptoms reminiscent of glucocorticoid withdrawal syndrome.These symptoms resolved spontaneously after 1 week.Intriguingly,the patient did not develop hepatic dysfunction this time.CONCLUSION The present case,showing some unusual clinical features,highlights the importance of education of clinicians and patients to avoid improper discontinuation of glucocorticoid therapy and complete history taking for prompt recognition.
文摘Type 1 diabetes(T1D) is an autoimmune disorder caused by inflammatory destruction of the pancreatic tissue. The etiopathogenesis and characteristics of the pathologic process of pancreatic destruction are well described. In addition, the putative susceptibility genes for T1 D as a monoglandular disease and the relation to polyglandular autoimmune syndrome(PAS) have also been wellexplored. The incidence of T1 D has steadily increased in most parts of the world, especially in industrialized nations. T1 D is frequently associated with autoimmune endocrine and non-endocrine diseases and patients with T1 D are at a higher risk for developing several glandular autoimmune diseases. Familial clustering is observed, which suggests that there is a genetic predisposition. Various hypotheses pertaining to viral- and bacterialinduced pancreatic autoimmunity have been proposed, however a definitive delineation of the autoimmune pathomechanism is still lacking. In patients with PAS, pancreatic and endocrine autoantigens either colocalize on one antigen-presenting cell or are expressed on two/various target cells sharing a common amino acid, which facilitates binding to and activation of T cells. The most prevalent PAS phenotype is the adult type 3 variant or PAS type Ⅲ, which encompasses T1 D and autoimmune thyroid disease. This review discusses the findings of recent studies showing noticeable differences in the genetic background and clinical phenotype of T1 D either as an isolated autoimmune endocrinopathy or within the scope of polyglandular autoimmune syndrome.
