BACKGROUND Insulin autoimmune syndrome(IAS)is a severe manifestation of spontaneous hypoglycemia.It is characterized by elevated levels of immune-reactive insulin and highly potent insulin autoantibodies(IAAs),which a...BACKGROUND Insulin autoimmune syndrome(IAS)is a severe manifestation of spontaneous hypoglycemia.It is characterized by elevated levels of immune-reactive insulin and highly potent insulin autoantibodies(IAAs),which are induced by endogenous insulin circulating in the bloodstream.It is distinguished by recurring instances of spontaneous hypoglycemia,the presence of IAA within the body,a substantial elevation in serum insulin levels,and an absence of prior exogenous insulin administration.Nevertheless,recent studies show that both conventional insulin and its analogs can induce IAS episodes,giving rise to the notion of nonclassical IAS.Therefore,more attention should be paid to these diseases.CASE SUMMARY In this case report,we present a rare case of non-classical IAS in an 83-year-old male patient who present with symptoms of a psychiatric disorder.Upon symptom onset,the patient exhibited Whipple's triad(including hypoglycemia,blood glucose level less than 2.8 mmol/L during onset,and rapid relief of hypoglycemic symptoms after glucose administration).Concurrently,his serum insulin level was significantly elevated,which contradicted his C-peptide levels.After a comprehensive examination,the patient was diagnosed with exogenous insulin autoimmune syndrome.Considering that the patient had type 2 diabetes mellitus and a history of exogenous insulin use before disease onset,it was presumed that non classical IAS was induced by this condition.The PubMed database was used to search for previous cases of IAS and non-classical IAS to analyze their characteristics and treatment approaches.CONCLUSION The occurrence of non-classical IAS is associated with exogenous insulin or its analogs,as well as with sulfhydryl drugs.Symptoms can be effectively alleviated through the discontinuation of relevant medications,administration of hormones or immunosuppressants,plasma exchange,and lifestyle adjustments.展开更多
BACKGROUND Survival in patients with autoimmune liver disease overlap syndromes(AILDOS)compared to those with single autoimmune liver disease is unclear.AIM To investigate the survival of patients with AILDOS and asse...BACKGROUND Survival in patients with autoimmune liver disease overlap syndromes(AILDOS)compared to those with single autoimmune liver disease is unclear.AIM To investigate the survival of patients with AILDOS and assess the accuracy of non-invasive serum models for predicting liver-related death.METHODS Patients with AILDOS were defined as either autoimmune hepatitis and primary biliary cholangitis overlap(AIH-PBC)or autoimmune hepatitis and primary sclerosing cholangitis overlap(AIH-PSC)and were identified from three tertiary centres for this cohort study.Liver-related death or transplantation(liver-related mortality)was determined using a population-based data linkage system.Prognostic scores for liver-related death were compared for accuracy[including liver outcome score(LOS),Hepascore,Mayo Score,model for end-stage liver disease(MELD)score and MELD incorporated with serum sodium(MELD-Na)score].RESULTS Twenty-two AILDOS patients were followed for a median of 3.1 years(range,0.35-7.7).Fourteen were female,the median age was 46.7 years(range,17.8 to 82.1)and median Hepascore was 1(range,0.07-1).At five years post enrolment,57%of patients remained free from liver-related mortality(74%AIH-PBC,27%AIH-PSC).There was no significant difference in survival between AIH-PBC and AIH-PSC.LOS was a significant predictor of liver-related mortality(P<0.05)in patients with AIH-PBC(n=14)but not AIH-PSC(n=8).A LOS cut-point of 6 discriminated liver-related mortality in AIH-PBC patients(P=0.012,log-rank test,100%sensitivity,77.8%specificity)(Harrell's C-statistic 0.867).The MELD score,MELD-Na score and Mayo Score were not predictive of liver-related mortality in any group.CONCLUSION Survival in the rare,AILDOS is unclear.The current study supports the LOS as a predictor of liver-related mortality in AIH-PBC patients.Further trials investigating predictors of survival in AILDOS are required.展开更多
BACKGROUND Latent autoimmune diabetes in adults(LADA)is a special type of type 1 diabetes mellitus.During the early stages,patients with LADA are treated with oral antidiabetics.However,insulin treatment is still requ...BACKGROUND Latent autoimmune diabetes in adults(LADA)is a special type of type 1 diabetes mellitus.During the early stages,patients with LADA are treated with oral antidiabetics.However,insulin treatment is still required as islet function gradually declines.Once patients have developed insulin allergy,clinical treatment and nursing care become very challenging.CASE SUMMARY Here,we report a case of LADA with insulin-related lipodystrophy,allergy,and exogenous insulin autoimmune syndrome during insulin treatment,thus making it very difficult to effectively control glucose levels with insulin.We attempted subcutaneous injection and an insulin pump to desensitize the patient’s response to insulin,and finally assisted the doctor to select the appropriate insulin treatment for the patient.We describe the management of this patient from a nursing viewpoint.CONCLUSION We summarize the nursing experience of a case with complex insulin allergy requiring desensitization treatment.Our approach is very practical and can be applied to similar patients needing insulin desensitization.展开更多
BACKGROUND The development of immune checkpoint inhibitors(ICIs)has heralded a new era in cancer treatment,enabling the possibility of long-term survival in patients with metastatic disease.Unfortunately,ICIs are incr...BACKGROUND The development of immune checkpoint inhibitors(ICIs)has heralded a new era in cancer treatment,enabling the possibility of long-term survival in patients with metastatic disease.Unfortunately,ICIs are increasingly implicated in the development of autoimmune diseases.CASE SUMMARY We present a man with squamous cell carcinoma of the oropharynx on a combination of teriprizumab,docetaxel,and cisplatin therapy who developed autoimmune polyendocrine syndrome typeⅡ(APS-2)including thyroiditis and type 1 diabetes mellitus and Crohn’s disease(CD).He developed thirst,abdominal pain,and fatigue after two-week treatment with the protein 1 ligand inhibitor teriprizumab.Biochemistry confirmed APS-2 and thyrotoxicosis.He was commenced on an insulin infusion.However,his abdominal pain persisted.Follow-up surgery confirmed CD and his abdominal pain was relieved by mesalazine.He was continued on insulin and mesalazine therapy.CONCLUSION Immunotherapy can affect all kinds of organs.When clinical symptoms cannot be explained by a single disease,clinicians should consider the possibility of multisystem damage.展开更多
Autoimmune hypoglycemia, also called insulin autoimmune syndrome (IAS), was first reported by Hirata and Ishizu in 1972 in Japan. IAS is characterized by the diagnostic criteria of spontaneous hypoglycemia without e...Autoimmune hypoglycemia, also called insulin autoimmune syndrome (IAS), was first reported by Hirata and Ishizu in 1972 in Japan. IAS is characterized by the diagnostic criteria of spontaneous hypoglycemia without evidence of exogenous insulin administration, high levels of total immunoreactive insulin, and the presence of a high titer of insulin autoantibodies.展开更多
Some patients present with overlapping features between disorders within the spectrum of autoimmune liver diseases, i.e., autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC), and primary sclerosing cholang...Some patients present with overlapping features between disorders within the spectrum of autoimmune liver diseases, i.e., autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC), and primary sclerosing cholangitis (PSC), and are commonly classified as having an "overlap syndrome". The pathophysiological mechanisms underlying AIH-PBC overlap remain unclear.^(1) Due to the lack of standardization and variations in the populations under study,展开更多
Objective To evaluate the efficacy of ursodeoxycholic acid(UDCA)alone or combined with immunosuppressant in primary biliary cirrhosis-autoimmune hepatitis overlap syndrome(PBC-AIH).Methods From January2001 to December...Objective To evaluate the efficacy of ursodeoxycholic acid(UDCA)alone or combined with immunosuppressant in primary biliary cirrhosis-autoimmune hepatitis overlap syndrome(PBC-AIH).Methods From January2001 to December 2014,41 patients with PBC-AIH were screened out from patients with autoimmune hepatitis(AIH)and retrospectively analyzed.According to展开更多
The identification of the first molecular defect leading to Mendelian Susceptibility to Mycobacterial Disease ;MD),1 a rare syndrome conferring predisposition to(MSMD),1 a rare syndrome conferring predisposition to...The identification of the first molecular defect leading to Mendelian Susceptibility to Mycobacterial Disease ;MD),1 a rare syndrome conferring predisposition to(MSMD),1 a rare syndrome conferring predisposition to disease caused by weakly virulent mycobacteria (such as Mycobacterium bovis, Bacille Calmette Gu6rin vaccines and environmental mycobacteria), has led to a paradigm shift in the field of primary immunodeficiencies in the last two decades. The "classic" patient with multiple immunologic abnormalities, conferring a broad susceptibility to multiple and recurrent infectious diseases caused by both weakly pathogenic and more virulent microorganisms, was the main Primary Immunodeficiency (PID) phenotype identified. Since 1996, mutations causing MSMD have been found in at least 6 genes related to interferon (IFN)-7-mediated immunity.2展开更多
文摘BACKGROUND Insulin autoimmune syndrome(IAS)is a severe manifestation of spontaneous hypoglycemia.It is characterized by elevated levels of immune-reactive insulin and highly potent insulin autoantibodies(IAAs),which are induced by endogenous insulin circulating in the bloodstream.It is distinguished by recurring instances of spontaneous hypoglycemia,the presence of IAA within the body,a substantial elevation in serum insulin levels,and an absence of prior exogenous insulin administration.Nevertheless,recent studies show that both conventional insulin and its analogs can induce IAS episodes,giving rise to the notion of nonclassical IAS.Therefore,more attention should be paid to these diseases.CASE SUMMARY In this case report,we present a rare case of non-classical IAS in an 83-year-old male patient who present with symptoms of a psychiatric disorder.Upon symptom onset,the patient exhibited Whipple's triad(including hypoglycemia,blood glucose level less than 2.8 mmol/L during onset,and rapid relief of hypoglycemic symptoms after glucose administration).Concurrently,his serum insulin level was significantly elevated,which contradicted his C-peptide levels.After a comprehensive examination,the patient was diagnosed with exogenous insulin autoimmune syndrome.Considering that the patient had type 2 diabetes mellitus and a history of exogenous insulin use before disease onset,it was presumed that non classical IAS was induced by this condition.The PubMed database was used to search for previous cases of IAS and non-classical IAS to analyze their characteristics and treatment approaches.CONCLUSION The occurrence of non-classical IAS is associated with exogenous insulin or its analogs,as well as with sulfhydryl drugs.Symptoms can be effectively alleviated through the discontinuation of relevant medications,administration of hormones or immunosuppressants,plasma exchange,and lifestyle adjustments.
文摘BACKGROUND Survival in patients with autoimmune liver disease overlap syndromes(AILDOS)compared to those with single autoimmune liver disease is unclear.AIM To investigate the survival of patients with AILDOS and assess the accuracy of non-invasive serum models for predicting liver-related death.METHODS Patients with AILDOS were defined as either autoimmune hepatitis and primary biliary cholangitis overlap(AIH-PBC)or autoimmune hepatitis and primary sclerosing cholangitis overlap(AIH-PSC)and were identified from three tertiary centres for this cohort study.Liver-related death or transplantation(liver-related mortality)was determined using a population-based data linkage system.Prognostic scores for liver-related death were compared for accuracy[including liver outcome score(LOS),Hepascore,Mayo Score,model for end-stage liver disease(MELD)score and MELD incorporated with serum sodium(MELD-Na)score].RESULTS Twenty-two AILDOS patients were followed for a median of 3.1 years(range,0.35-7.7).Fourteen were female,the median age was 46.7 years(range,17.8 to 82.1)and median Hepascore was 1(range,0.07-1).At five years post enrolment,57%of patients remained free from liver-related mortality(74%AIH-PBC,27%AIH-PSC).There was no significant difference in survival between AIH-PBC and AIH-PSC.LOS was a significant predictor of liver-related mortality(P<0.05)in patients with AIH-PBC(n=14)but not AIH-PSC(n=8).A LOS cut-point of 6 discriminated liver-related mortality in AIH-PBC patients(P=0.012,log-rank test,100%sensitivity,77.8%specificity)(Harrell's C-statistic 0.867).The MELD score,MELD-Na score and Mayo Score were not predictive of liver-related mortality in any group.CONCLUSION Survival in the rare,AILDOS is unclear.The current study supports the LOS as a predictor of liver-related mortality in AIH-PBC patients.Further trials investigating predictors of survival in AILDOS are required.
文摘BACKGROUND Latent autoimmune diabetes in adults(LADA)is a special type of type 1 diabetes mellitus.During the early stages,patients with LADA are treated with oral antidiabetics.However,insulin treatment is still required as islet function gradually declines.Once patients have developed insulin allergy,clinical treatment and nursing care become very challenging.CASE SUMMARY Here,we report a case of LADA with insulin-related lipodystrophy,allergy,and exogenous insulin autoimmune syndrome during insulin treatment,thus making it very difficult to effectively control glucose levels with insulin.We attempted subcutaneous injection and an insulin pump to desensitize the patient’s response to insulin,and finally assisted the doctor to select the appropriate insulin treatment for the patient.We describe the management of this patient from a nursing viewpoint.CONCLUSION We summarize the nursing experience of a case with complex insulin allergy requiring desensitization treatment.Our approach is very practical and can be applied to similar patients needing insulin desensitization.
文摘BACKGROUND The development of immune checkpoint inhibitors(ICIs)has heralded a new era in cancer treatment,enabling the possibility of long-term survival in patients with metastatic disease.Unfortunately,ICIs are increasingly implicated in the development of autoimmune diseases.CASE SUMMARY We present a man with squamous cell carcinoma of the oropharynx on a combination of teriprizumab,docetaxel,and cisplatin therapy who developed autoimmune polyendocrine syndrome typeⅡ(APS-2)including thyroiditis and type 1 diabetes mellitus and Crohn’s disease(CD).He developed thirst,abdominal pain,and fatigue after two-week treatment with the protein 1 ligand inhibitor teriprizumab.Biochemistry confirmed APS-2 and thyrotoxicosis.He was commenced on an insulin infusion.However,his abdominal pain persisted.Follow-up surgery confirmed CD and his abdominal pain was relieved by mesalazine.He was continued on insulin and mesalazine therapy.CONCLUSION Immunotherapy can affect all kinds of organs.When clinical symptoms cannot be explained by a single disease,clinicians should consider the possibility of multisystem damage.
文摘Autoimmune hypoglycemia, also called insulin autoimmune syndrome (IAS), was first reported by Hirata and Ishizu in 1972 in Japan. IAS is characterized by the diagnostic criteria of spontaneous hypoglycemia without evidence of exogenous insulin administration, high levels of total immunoreactive insulin, and the presence of a high titer of insulin autoantibodies.
文摘Some patients present with overlapping features between disorders within the spectrum of autoimmune liver diseases, i.e., autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC), and primary sclerosing cholangitis (PSC), and are commonly classified as having an "overlap syndrome". The pathophysiological mechanisms underlying AIH-PBC overlap remain unclear.^(1) Due to the lack of standardization and variations in the populations under study,
文摘Objective To evaluate the efficacy of ursodeoxycholic acid(UDCA)alone or combined with immunosuppressant in primary biliary cirrhosis-autoimmune hepatitis overlap syndrome(PBC-AIH).Methods From January2001 to December 2014,41 patients with PBC-AIH were screened out from patients with autoimmune hepatitis(AIH)and retrospectively analyzed.According to
文摘The identification of the first molecular defect leading to Mendelian Susceptibility to Mycobacterial Disease ;MD),1 a rare syndrome conferring predisposition to(MSMD),1 a rare syndrome conferring predisposition to disease caused by weakly virulent mycobacteria (such as Mycobacterium bovis, Bacille Calmette Gu6rin vaccines and environmental mycobacteria), has led to a paradigm shift in the field of primary immunodeficiencies in the last two decades. The "classic" patient with multiple immunologic abnormalities, conferring a broad susceptibility to multiple and recurrent infectious diseases caused by both weakly pathogenic and more virulent microorganisms, was the main Primary Immunodeficiency (PID) phenotype identified. Since 1996, mutations causing MSMD have been found in at least 6 genes related to interferon (IFN)-7-mediated immunity.2