scholarly journals A novel approach to a rare case of non-islet cell hypoglycaemia

2021 ◽  
Vol 2021 ◽  
Author(s):  
R K Dharmaputra ◽  
K L Wan ◽  
N Samad ◽  
M Herath ◽  
J Wong ◽  
...  
Keyword(s):  
Plasma Exchange ◽  
Islet Cell ◽  
B Lymphocyte ◽  
Serum Insulin ◽  
Insulin Antibody ◽  
Lymphocyte Depletion ◽  
Insulin Levels ◽  
Insulin Autoimmune Syndrome ◽  
Autoimmune Syndrome ◽  
High Insulin

Summary Insulin autoimmune syndrome (IAS) is a rare cause of non-islet cell hypoglycaemia. Treatment of this condition is complex and typically involves long-term use of glucocorticoids. Immunotherapy may provide an alternative in the management of this autoimmune condition through the suppression of antibodies production by B-lymphocyte depletion. We present a case of a 62-year-old male, with refractory hypoglycaemia initially presenting with hypoglycaemic seizure during an admission for acute psychosis. Biochemical testing revealed hypoglycaemia with an inappropriately elevated insulin and C-peptide level and no evidence of exogenous use of insulin or sulphonylurea. Polyethylene glycol precipitation demonstrated persistently elevated free insulin levels. This was accompanied by markedly elevated anti-insulin antibody (IA) titres. Imaging included CT with contrast, MRI, pancreatic endoscopic ultrasound and Ga 68-DOTATATE position emission tomography (DOTATATE PET) scan did not reveal islet cell aetiology for hyperinsulinaemia. Maintenance of euglycaemia was dependent on oral steroids and dextrose infusion. Complete resolution of hypoglycaemia and dependence on glucose and steroids was only achieved following treatment with plasma exchange and rituximab. Learning points Insulin autoimmune syndrome (IAS) should be considered in patients with recurrent hyperinsulinaemic hypoglycaemia in whom exogenous insulin administration and islet cell pathologies have been excluded. Biochemical techniques play an essential role in establishing high insulin concentration, insulin antibody titres, and eliminating biochemical interference. High insulin antibody concentration can lead to inappropriately elevated serum insulin levels leading to hypoglycaemia. Plasma exchange and B-lymphocyte depletion with rituximab and immunosuppression with high dose glucocorticoids are effective in reducing serum insulin levels and hypoglycaemia in insulin autoimmune syndrome (IAS). Based on our observation, the reduction in serum insulin level may be a better indicator of treatment efficacy compared to anti-insulin antibody (IA) titre as it demonstrated greater correlation to the frequency of hypoglycaemia and to hypoglycaemia resolution.

scholarly journals Insulin Autoimmune Syndrome Treated with Plasmapharesis

2020 ◽  
pp. 25-26
Author(s):  
Dr. Kavya Jonnalagadda ◽  
Dr. Praveen. V. Pavithran
Keyword(s):  
Low Dose ◽  
Serum Insulin ◽  
Oral Prednisolone ◽  
Symptomatic Improvement ◽  
Insulin Antibody ◽  
Autoimmune Syndrome ◽  
Dose Oral ◽  
High Insulin ◽  
History Of ◽  
Antibody Titers

A 66-year male with a history of Central Serous Retinopathy presented with recurrent episodes of hypoglycemia. On evaluation, he was found to have insulin-mediated hypoglycemia with serum insulin of 300uIU/ml, C peptide 27.51ng/ml, when the blood glucose was 46mg/dl. High insulin levels above 100uIU/ml, led to suspicion of Autoimmune hypoglycemia and were confirmed by a high anti-insulin antibody titer of 300U/ml. Imaging was negative for Insulinoma. The patient was started on low dose oral prednisolone under ophthalmological monitoring, but as there was no symptomatic improvement, the dose was increased following which there was a flare-up of CSR. The patient was initiated on plasmapheresis following which his hypoglycemia improved with drop in anti-insulin antibody titers to 29U/ml. The patient was maintained on low dose steroids, which were tapered and stopped over the next six months with complete resolution of hypoglycemia and normalization of anti-insulin antibody titers.

scholarly journals Insulin autoimmune syndrome: case report

2004 ◽  
Vol 122 (4) ◽  
pp. 178-180 ◽  
Author(s):  
Rodrigo Oliveira Moreira ◽  
Giovanna Aparecida Balarini Lima ◽  
Patrícia Carla Batista Peixoto ◽  
Maria Lucia Fleiuss Farias ◽  
Mario Vaisman
Keyword(s):  
Case Report ◽  
Pancreatic Tumor ◽  
Histopathological Examination ◽  
Severe Trauma ◽  
Insulin Antibodies ◽  
Insulin Levels ◽  
Insulin Autoimmune Syndrome ◽  
Autoimmune Syndrome ◽  
High Insulin ◽  
Biochemical Evaluation

CONTEXT: Insulin autoimmune syndrome (IAS, Hirata disease) is a rare cause of hypoglycemia in Western countries. It is characterized by hypoglycemic episodes, elevated insulin levels, and positive insulin antibodies. Our objective is to report a case of IAS identified in South America. CASE REPORT: A 56-year-old Caucasian male patient started presenting neuroglycopenic symptoms during hospitalization due to severe trauma. Biochemical evaluation confirmed hypoglycemia and abnormally high levels of insulin. Conventional imaging examinations were negative for pancreatic tumor. Insulin antibodies were above the normal range. Clinical remission of the episodes was not achieved with verapamil and steroids. Thus, a subtotal pancreatectomy was performed due to the lack of response to conservative treatment and because immunosuppressants were contraindicated due to bacteremia. Histopathological examination revealed diffuse hypertrophy of beta cells. The patient continues to have high insulin levels but is almost free of hypoglycemic episodes.

scholarly journals A Curious Case of Hypoglycemia: A Rare Occurrence of Insulin Autoimmune Syndrome

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A360-A361
Author(s):  
Rithikaa Ellangovan ◽  
Rachana Mundada ◽  
Ajinkya Kulkarni ◽  
Robert A Ries ◽  
Sudheer Konduru ◽  
...  
Keyword(s):  
African American ◽  
African American Male ◽  
Serum Glucose ◽  
C Peptide ◽  
Insulin Levels ◽  
Insulin Autoimmune Syndrome ◽  
Endogenous Insulin ◽  
Autoimmune Syndrome ◽  
High Insulin ◽  
Recurrent Hypoglycemia

Abstract Background: Hypoglycemia can be challenging, requiring close monitoring and evaluation. Although treating diabetes can cause hypoglycemia, the coexistence of autoimmune syndromes contributes to rare etiologies. They are characterized by elevated insulin levels with either insulin autoantibodies (IAA) or insulin receptor antibodies (IRA). It has been observed commonly in Japan but is scarce among non-Asian groups. We present a unique case of insulin autoimmune syndrome (IAS) that posed a diagnostic challenge in an African American male. Case: A 73-year-old African American male was admitted with altered mental status. Medical history included type 2 diabetes, hypertension, and hyperlipidemia. Home medications were carvedilol and simvastatin. On arrival, vital signs were normal. A fingerstick glucose was 52 mg/dL with a serum level of 68 mg/dL (70–110). Other labs were normal. Given symptomatic hypoglycemia, an IV dextrose infusion was initiated. Once his mentation improved, a diet was started. Despite this, he had recurrent hypoglycemia with glucose levels as low as 22 mg/dL, predominantly in fasting state with sporadic hyperglycemia. On rare occasions, he received correctional insulin for the same. An HbA1c was <4% (4–6). Thyroid function test and AM cortisol were normal. A cosyntropin stimulation test was negative for adrenal insufficiency. A hypoglycemia panel showed inappropriately high levels of insulin, highest at 77.4 μIU/mL(<=29.1), proinsulin of 19.7 pmol/L (<=8), and C-peptide of 5.6 (0.8–3.69 ng/mL) when serum glucose was 25 mg/dL. An MRI abdomen was normal. Octreotide study was negative for insulinoma. He had a normal response to IM glucagon, inferring normal glycogen stores. He was started on Diazoxide 160 mg thrice a day for recurrent hypoglycemia. An endoscopic ultrasound and DOTATATE scan were negative. He had no hypoglycemia for a few days, attributable to lingering effects of diazoxide. Eventually, his serum glucose was 52 mg/dL. Labs prior to glucose correction included an insulin level elevated at 1,000 (normal <3 mcIU/mL), c-peptide at 0.90 ng/mL, and proinsulin of 5.6 pmol/L. Given exceedingly high insulin levels, we measured an IAA level. This was >50 u/mL (normal <0.4 u/mL). With negative imaging and high IAAs, a diagnosis of IAS was made. Discussion: IAS or Hirata disease is a rare condition with hyperinsulinemic hypoglycemia and high titers of antibodies to endogenous insulin. The binding kinetics of endogenous insulin to these antibodies causes physiologically inappropriate levels of bioavailable insulin, causing either hyper- or hypoglycemia. IAA should be measured in patients with high insulin levels that are inconsistent with C peptide levels. We believe this to be the first African American patient to have been diagnosed with Hirata disease. Making a correct diagnosis may spare a hypoglycemic patient from unnecessary pancreatic surgical intervention.

scholarly journals A case of insulin autoimmune syndrome with transient islet cell surface antibodies accompanied by liver cirrhosis.

1986 ◽  
Vol 75 (9) ◽  
pp. 1250-1255
Author(s):  
Yasuo HARIGAYA ◽  
Yasumasa KUWABARA ◽  
Tokio TAKEUCHI ◽  
Sadao SATOH ◽  
Yasunori KANAZAWA
Keyword(s):  
Liver Cirrhosis ◽  
Cell Surface ◽  
Islet Cell ◽  
Insulin Autoimmune Syndrome ◽  
Autoimmune Syndrome ◽  
Islet Cell Surface Antibodies ◽  
Surface Antibodies

Pitfalls in the diagnosis of insulin autoimmune syndrome (Hirata’s disease) in a hypoglycemic child: a case report and review of the literature

2019 ◽  
Vol 32 (4) ◽  
pp. 421-428 ◽  
Author(s):  
Tiago Jeronimo Dos Santos ◽  
Caroline Gouvêa Buff Passone ◽  
Marina Ybarra ◽  
Simone Sakura Ito ◽  
Milena Gurgel Teles ◽  
...  
Keyword(s):  
Differential Diagnosis ◽  
Previous History ◽  
Human Leukocyte ◽  
Hyperinsulinemic Hypoglycemia ◽  
Insulin Levels ◽  
Insulin Autoimmune Syndrome ◽  
Endogenous Insulin ◽  
Autoimmune Syndrome ◽  
Insulin Autoantibody ◽  
Control Food Intake

Abstract Background Insulin autoimmune syndrome (IAS) is a rare cause of hyperinsulinemic hypoglycemia (HH) not addressed as a potential differential diagnosis in current pediatric guidelines. We present a case of IAS in a child with no previous history of autoimmune disease, no previous intake of triggering medications and absence of genetic predisposition. Case presentation A 6-year-old boy presented with recurrent HH (blood glucose of 26 mg/dL [1.4 mmol/L] and insulin of 686 μU/mL). Abdominal imaging was normal. After multiple therapeutic failures, we hypothesized misuse of exogenous insulin and factitious hypoglycemia. Council of Guardianship had the child separated from his mother, but insulin levels remained high. A chromatography test was then performed which showed high titers of endogenous insulin autoantibody (IAA) with early dissociation from the insulin molecule. The human leukocyte antigen (HLA) test showed a DRB1 *13:01/*08:02 genotype. The patient was advised to control food intake and physical activity routines. During a 5-year follow-up, hypoglycemic episodes were sparse, despite high insulin levels. Conclusions Misdiagnosis of IAS with factitious hypoglycemia may happen if IAS is not considered as a differential diagnosis, leading to potential traumatic consequences. Further efforts should be made to increase awareness of IAS as a differential diagnosis of hypoglycemia and to include it in pediatric guidelines.

Comparison of Two Autoimmune Dysglycemia Syndromes: Insulin Autoimmune Syndrome (IAS) and Type B Insulin Resistance Syndrome (B-IRS)

2019 ◽  
Vol 51 (11) ◽  
pp. 723-728 ◽  
Author(s):  
Sui Yu ◽  
Guoqing Yang ◽  
Jingtao Dou ◽  
Baoan Wang ◽  
Weijun Gu ◽  
...  
Keyword(s):  
Insulin Resistance ◽  
Serum Insulin ◽  
Insulin Resistance Syndrome ◽  
Tolerance Test ◽  
White Blood Count ◽  
Oral Glucose ◽  
Type B ◽  
Insulin Autoimmune Syndrome ◽  
Autoimmune Syndrome ◽  
Clinical Differential Diagnosis

AbstractInsulin autoimmune syndrome (IAS) and type B insulin resistance syndrome (B-IRS) are rare autoimmune dysglycemia syndromes, but their treatment and prognosis are different. This study aimed to provide a basis for the clinical differential diagnosis of IAS and B-IRS. This was a retrospective study of the medical records of all patients diagnosed with IAS or B-IRS between January 2006 and March 2018 at the Chinese PLA General Hospital. Demographic, clinical, biochemistry, treatment, and follow-up data were examined. There were several different biochemical parameters between IAS (n=13) and B-IRS (n=6): white blood count (WBC, 7.05±3.06 vs. 2.70±0.73×109/l, p=0.004), platelet (249±56.6 vs. 111±68.0×109/l, p<0.001), serum creatine (59.0±17.8 vs. 43.1±7.05 μmol/l, p=0.013), serum albumin (42.3±5.17 vs. 33.6±3.40 g/l, p=0.002), triglyceride (median, 1.33 (1.01, 1.93) vs. 0.56 (0.50, 0.79) mmol/l, p=0.002), plasma IgG (1183±201 vs. 1832±469 mg/ml, p=0.018), IgA (328±140 vs. 469±150 mg/ml, p=0.018), and C3 (128±23.4 vs. 45.3±13.5 mg/l, p<0.001). Fasting insulin in the IAS and B-IRS patients was high (299–4708 vs. 118–851 mU/l, p=0.106), and there was a difference in 2 h oral glucose tolerance test insulin (4217–8343 mU/l vs. 274–1143 mU/l, p=0.012). Glycated hemoglobin (HbA1c) in the B-IRS patients was higher than in IAS patients (114±14.4. vs. 40.6±8.89 mmol/mol, p<0.001). Serum insulin-like growth factor-1 (IGF-1) was lower in all B-IRS patients (25±0.00 vs. 132±52.7 ng/ml, p<0.001). Although IAS and B-IRS are autoimmune hyperinsulinemic dysglycemic syndromes, several clinical parameters (body mass index, HbA1c, WBC, platelet, albumin, triglyceride, IgG, C3, and IGF-1) are different between these two syndromes.

Therapeutic plasma exchange normalizes insulin-mediated response in a child with type 1 diabetes and insulin autoimmune syndrome

2017 ◽  
Vol 19 (1) ◽  
pp. 171-179 ◽  
Author(s):  
Erin F Sharwood ◽  
Ian P Hughes ◽  
Carel J Pretorius ◽  
Peter Trnka ◽  
Jane Peake ◽  
...  
Keyword(s):  
Type 1 Diabetes ◽  
Plasma Exchange ◽  
Therapeutic Plasma Exchange ◽  
Insulin Autoimmune Syndrome ◽  
Autoimmune Syndrome

scholarly journals Analysis of the clinical characteristics of insulin autoimmune syndrome induced by exogenous insulin in diabetic patients

2021 ◽  
Vol 13 (1) ◽  
Author(s):  
Zuojun Li ◽  
Dan Yi ◽  
Lijuan Zheng ◽  
Shiran Li ◽  
Weijin Fang ◽  
...  
Keyword(s):  
Clinical Characteristics ◽  
Serum Insulin ◽  
Insulin Antibodies ◽  
Hypoglycemic Agents ◽  
Oral Glucose ◽  
Diabetic Patients ◽  
Exogenous Insulin ◽  
Oral Hypoglycemic Agents ◽  
Insulin Autoimmune Syndrome ◽  
Autoimmune Syndrome

Abstract Background The exact incidence, clinical features and uniform diagnostic criteria of exogenous insulin autoimmune syndrome (EIAS) are still unclear. The purpose of this study is to explore the clinical characteristics of EIAS and to provide a structural approach for clinical diagnosis, treatment and prevention. Methods The literature on EIAS in Chinese and English from 1970 to 2020 was collected for retrospective analysis. Results A total of 122 patients (33 males and 73 females) were included in the study with a median age of 67 years (range 14–86) and a median HbA1c of 7.7%. EIAS mainly occurred in type 2 diabetes mellitus patients using premixed insulin. Symptoms manifested were hypoglycemia in 86.54%, recurrent episodes of symptomatic hypoglycemia in 35.58%, nocturnal hypoglycemia along with daytime hyperglycemia in 21.15% and recurrent hypoglycemia after discontinued insulin in 64.43%. The onset of symptoms occurred at night, in the early morning or during fasting, ranging from a few days to 78 months after the administration of insulin. The mean blood glucose level during the hypoglycemic phase was 2.21 mmol/L (range 1–3.4), and the serum insulin levels were mainly ≥ 100 U/mL and were associated with low C-peptide levels (≤ 10 ng/ml). Insulin autoantibodies (IAAs) were positive in all EIAS patients. The 75-g extended oral glucose tolerance test (OGTT) mainly showed a diabetic curve. Pancreatic imaging was unremarkable. Withdrawal of insulin alone or combination of oral hypoglycemic agents or replacement of insulin formulations or with corticosteroid treatment eliminated hypoglycemia in a few days to 3 months. IAA turned negative in 6 months (median, range 1–12). No hypoglycemia episodes were observed at a median follow-up of 6 months (range 0.5–60). Conclusions EIAS is an autoimmune disease caused by insulin-binding antibodies in susceptible subjects. Insulin antibodies change glucose dynamics and could increase the incidence of hypoglycemic episodes. Detection of insulin antibodies is the diagnostic test. Changing therapeutic modalities reduced the incidence of hypoglycemic episodes.

Sign in / Sign up

Export Citation Format

Share Document