scholarly journals Liver Transplantation in an Adult with Citrullinaemia Type 2

2011 ◽  
Vol 2011 ◽  
pp. 1-4 ◽  
Author(s):  
Hui-Hui Tan ◽  
Wan-Cheng Chow ◽  
Kiat-Hon Lim ◽  
Wei-Keat Wan ◽  
Alexander Y. F. Chung ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Liver Transplant ◽  
Urea Cycle ◽  
Adult Onset ◽  
Urea Cycle Defect ◽  
Arginosuccinate Synthetase

Citrullinaemia is a urea cycle defect that results from a deficiency of the enzyme arginosuccinate synthetase. Type 1 disease is diagnosed in childhood, whereas Type 2 disease is adult onset. We report the outcome of a patient with citrullinemia Type 2 who received a liver transplant at our center and the implications of this diagnosis in liver transplantation.

Type 1 Diabetes-related Autoantibodies in Different Forms of Diabetes

2019 ◽  
Vol 15 (3) ◽  
pp. 199-204 ◽  
Author(s):  
Elin Pettersen Sørgjerd
Keyword(s):  
Type 2 Diabetes ◽  
Type 1 Diabetes ◽  
Autoimmune Diabetes ◽  
Acid Decarboxylase ◽  
Adult Onset ◽  
Latent Autoimmune Diabetes ◽  
Childhood Type 1 Diabetes ◽  
Childhood Type

Autoantibodies against Glutamic Acid Decarboxylase (GADA), insulinoma antigen-2 (IA- 2A), insulin (IAA) and the most recently Zinc Transporter 8 (ZnT8A) are one of the most reliable biomarkers for autoimmune diabetes in both children and adults. They are today the only biomarkers that can distinguish Latent Autoimmune Diabetes in Adults (LADA) from phenotypically type 2 diabetes. As the frequency of autoantibodies at diagnosis in childhood type 1 diabetes depends on age, GADA is by far the most common in adult onset autoimmune diabetes, especially LADA. Being multiple autoantibody positive have also shown to be more common in childhood diabetes compared to adult onset diabetes, and multiple autoantibody positivity have a high predictive value of childhood type 1 diabetes. Autoantibodies have shown inconsistent results to predict diabetes in adults. Levels of autoantibodies are reported to cause heterogeneity in LADA. Reports indicate that individuals with high levels of autoantibodies have a more type 1 diabetes like phenotype and individuals with low levels of autoantibody positivity have a more type 2 diabetes like phenotype. It is also well known that autoantibody levels can fluctuate and transient autoantibody positivity in adult onset autoimmune diabetes have been reported to affect the phenotype.

scholarly journals Relative contribution of type 1 and type 2 diabetes loci to the genetic etiology of adult-onset, non-insulin-requiring autoimmune diabetes

BMC Medicine ◽  
2017 ◽  
Vol 15 (1) ◽  
Author(s):  
Rajashree Mishra ◽  
◽  
Alessandra Chesi ◽  
Diana L. Cousminer ◽  
Mohammad I. Hawa ◽  
...  
Keyword(s):  
Type 2 Diabetes ◽  
Autoimmune Diabetes ◽  
Adult Onset ◽  
Genetic Etiology ◽  
Relative Contribution

scholarly journals Standard mortality rates and years of life lost for serologically defined adult-onset type 1 and type 2 diabetes – A fifteen year follow-up

2020 ◽  
Vol 160 ◽  
pp. 107943
Author(s):  
Maria Thunander ◽  
Anna Lindgren ◽  
Christer Petersson ◽  
Mona Landin-Olsson ◽  
Sara Holmberg
Keyword(s):  
Type 2 Diabetes ◽  
Mortality Rates ◽  
Years Of Life Lost ◽  
Adult Onset ◽  
Onset Type

Subtle cognitive dysfunction in adult onset myotonic dystrophy type 1 (DM1) and type 2 (DM2)

Neurology ◽  
2006 ◽  
Vol 67 (2) ◽  
pp. 350-352 ◽  
Author(s):  
C. Gaul ◽  
T. Schmidt ◽  
G. Windisch ◽  
T. Wieser ◽  
T. Muller ◽  
...  
Keyword(s):  
Myotonic Dystrophy ◽  
Cognitive Dysfunction ◽  
Myotonic Dystrophy Type 1 ◽  
Myotonic Dystrophy Type ◽  
Adult Onset

scholarly journals Tumor Necrosis Factor Microsatellite Polymorphism Influences the Development of Insulin Dependency in Adult-Onset Diabetes Patients with the DRB1∗1502-DQB1∗0601 Allele and Anti-Glutamic Acid Decarboxylase Antibodies

2000 ◽  
Vol 85 (9) ◽  
pp. 3348-3351
Author(s):  
Hiroshi Obayashi ◽  
Goji Hasegawa ◽  
Michiaki Fukui ◽  
Kenji Kamiuchi ◽  
Akane Kitamura ◽  
...  
Keyword(s):  
Type 2 Diabetes ◽  
Tumor Necrosis ◽  
Acid Decarboxylase ◽  
Diabetic Patients ◽  
Adult Onset ◽  
Group A ◽  
Group B ◽  
Necrosis Factor

Abstract Recently, several studies have demonstrated that tumor necrosis factor microsatellite polymorphism (TNFa) contributes to the susceptibility of type 1 diabetes. This study investigates the influence of TNFa on the predisposition to insulin dependency in adult-onset diabetic patients with type 1 diabetes-protective human leukocyte antigen haplotypes. The TNFa of three groups of DRB1∗1502-DQB1∗0601-positive diabetic patients who had initially been nonketotic and noninsulin dependent for more than 1 yr was analyzed. Group A included 11 antibodies to glutamic acid decarboxylase (GADab)-positive patients who developed insulin dependency within 4 yr of diabetes onset. Group B included 11 GADab-positive patients who remained noninsulin dependent for more than 12 yr. Group C included 12 GADab-negative type 2 diabetes, and a control group included 18 nondiabetic subjects. In the group C and control subjects, DRB1∗1502-DQB1∗0601 was strongly associated with the TNFa13 allele. DRB1∗1502-DQB1∗0601 was strongly associated with the TNFa12 allele among the group A patients, but not among the group B patients. Interestingly, sera from all patients with non-TNFa12 and non-TNFa13 in group B reacted with GAD65 protein by Western blot. These results suggest that TNFa is associated with a predisposition to progression to insulin dependency in GADab/DRB1∗1502-DQB1∗0601-positive diabetic patients initially diagnosed with type 2 diabetes and that determination of these patients’ TNFa genotype may allow for better prediction of their clinical course.

scholarly journals SUN-685 Approach to a Potential Liver Transplant Candidate with Insulin Antibody-Mediated Severe Insulin Resistance

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Danielle C Brooks ◽  
Emily Japp ◽  
Nirali Shah
Keyword(s):  
Insulin Resistance ◽  
Type 2 Diabetes ◽  
Liver Transplantation ◽  
Glycemic Control ◽  
Liver Transplant ◽  
Insulin Antibodies ◽  
Insulin Antibody ◽  
First Episode ◽  
Severe Insulin Resistance

Abstract Introduction: Insulin antibody (IA)-mediated insulin resistance is a rare autoimmune condition resulting in uncontrolled hyperglycemia. High titers of IA are associated with increased mortality secondary to severe insulin resistance and labile blood sugars. There is a paucity of standardized treatment for these patients. Although there have been reported cases of success with immunosuppressants, none of these cases involved patients with liver cirrhosis. We present a case of IA-mediated severe insulin resistance which resulted in uncontrolled hyperglycemia and ultimately delayed liver transplantation. Clinical Case: A 61-year-old male with IA-positive type 2 diabetes, decompensated hepatitis B and NASH cirrhosis presented with several episodes of diabetic ketoacidosis (DKA) and worsening insulin resistance. His liver transplant listing had been placed on hold until glycemic control is achieved. The patient was diagnosed with type 2 diabetes mellitus in 1998. He has no prior autoimmune history. His disease was controlled on Levemir 100 units daily until March 2019 when he presented with his first episode of DKA. He subsequently required 200 units of insulin degludec daily and U-500 insulin 200 units with meals. The patient was readmitted to our hospital in August 2019 for a variceal bleed. His hospital course was complicated by a second occurrence of DKA requiring 100-150 units/hour on an insulin drip for resolution. Labs were significant for HbA1c 8.7% and IA >625 uU/mL (negative if <5.0 uU/mL). He required increasing amounts of basal and prandial insulin after discharge. The patient was again admitted within two months for abdominal pain concerning for spontaneous bacterial peritonitis, which was complicated by his third episode of DKA. Glucoses remained uncontrolled in the range of 170 to 300 mg/dL despite high insulin doses upon discharge. Metformin was contraindicated due to episode of lactic acidosis in the setting of his cirrhosis and concern for repeated episodes of DKA prevented use of SGLT-2 inhibitors. Extensive multidisciplinary discussions led to the decision for an upcoming trial of mycophenolate mofetil followed by plasmapheresis. The goal is to improve glycemic control while also minimizing infection risk to ultimately list him for a liver transplant. Conclusion: This patient highlights a major therapeutic challenge related to uncontrolled hyperglycemia and insulin resistance from anti-insulin antibodies in a cirrhotic patient. This can place patients at high risk for infection, poor wound healing and most importantly prohibit liver transplantation. Immunosuppressant therapy and plasmapheresis may drastically lower insulin antibodies and improve glycemic control, however, it will increase the risk of infection.

scholarly journals Improving clinical utility of GAD65 autoantibodies by electrochemiluminescence assay and clinical phenotype when identifying autoimmune adult-onset diabetes

Diabetologia ◽  
2021 ◽  
Author(s):  
Yong Gu ◽  
Xiaofan Jia ◽  
Tanwi Vartak ◽  
Dongmei Miao ◽  
Fran Dong ◽  
...  
Keyword(s):  
Type 2 Diabetes ◽  
Type 1 Diabetes ◽  
Clinical Utility ◽  
Insulin Treatment ◽  
Clinical Phenotype ◽  
Adult Onset ◽  
Onset Diabetes ◽  
Adult Onset Diabetes

Abstract Aims/hypothesis It is important to differentiate the two major phenotypes of adult-onset diabetes, autoimmune type 1 diabetes and non-autoimmune type 2 diabetes, especially as type 1 diabetes presents in adulthood. Serum GAD65 autoantibodies (GADA) are the most sensitive biomarker for adult-onset autoimmune type 1 diabetes, but the clinical value of GADA by current standard radiobinding assays (RBA) remains questionable. The present study focused on the clinical utility of GADA differentiated by a new electrochemiluminescence (ECL) assay in patients with adult-onset diabetes. Methods Two cohorts were analysed including 771 diabetic participants, 30–70 years old, from the Action LADA study (n = 6156), and 2063 diabetic participants, 20–45 years old, from the Diabetes in Young Adults (DiYA) study. Clinical characteristics of participants, including requirement of early insulin treatment, BMI and development of multiple islet autoantibodies, were analysed according to the status of RBA-GADA and ECL-GADA, respectively, and compared between these two assays. Results GADA was the most prevalent and predominant autoantibody, >90% in both cohorts. GADA positivity by either RBA or ECL assay significantly discriminated clinical type 1 from type 2 diabetes. However, in both cohorts, participants with ECL-GADA positivity were more likely to require early insulin treatment, have multiple islet autoantibodies, and be less overweight (for all p < 0.0001). However, clinical phenotype, age at diagnosis and BMI independently improved positive predictive value (PPV) for the requirement of insulin treatment, even augmenting ECL-GADA. Participants with GADA detectable by RBA, but not confirmed by ECL, had a phenotype more similar to type 2 diabetes. These RBA-GADA positive individuals had lower affinity GADA compared with participants in which GADA was confirmed by ECL assay. Conclusions/interpretation Detection of GADA by ECL assay, given technical advantages over RBA-GADA, identified adult-onset diabetes patients at higher risk of requiring early insulin treatment, as did clinical phenotype, together allowing for more accurate clinical diagnosis and management. Graphical abstract

Perinatal Risk Factors in Young Adult-Onset Type 1 and Type 2 Diabetes—A Population-Based Case-Control Study

2009 ◽  
Vol 64 (8) ◽  
pp. 511-512
Author(s):  
Niina Lammi ◽  
Paul A. Blomstedt ◽  
Elena Moltchanova ◽  
Johan G. Eriksson ◽  
Jaakko Tuomilehto ◽  
...  
Keyword(s):  
Risk Factors ◽  
Type 2 Diabetes ◽  
Case Control Study ◽  
Population Based ◽  
Case Control ◽  
Adult Onset ◽  
Perinatal Risk Factors ◽  
Control Study

Young adult onset type 2 diabetes versus type 1 diabetes: Progression to and survival on renal replacement therapy

2021 ◽  
pp. 108023
Author(s):  
Timothy L. Middleton ◽  
Steven Chadban ◽  
Lynda Molyneaux ◽  
Mario D'Souza ◽  
Maria I. Constantino ◽  
...  
Keyword(s):  
Type 2 Diabetes ◽  
Type 1 Diabetes ◽  
Renal Replacement Therapy ◽  
Young Adult ◽  
Replacement Therapy ◽  
Adult Onset ◽  
Onset Type ◽  
Diabetes Progression
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