Differential Diagnosis Of Hypoglycemia Research Articles

Neuroglycopaenia as the first manifestation of MEN1 syndrome

Insulinoma is a neuroendocrine tumour of islet cells and a rare cause of hypoglycaemia in both adults and the paediatric population, with an incidence is 1–4 cases/million/year. Insulinoma may occur as an isolated tumour or as a component of multiple endocrine neoplasia syndrome 1. In the case presented below, a 15-year-old female patient with no significant medical history was brought to the emergency department by a medical rescue team, exhibiting symptoms of neuroglycopaenia for several hours. After excluding more common causes of hypoglycaemia, insulinoma was suspected. Given the patient’s very young age at onset and a significant family history, laboratory and imaging diagnostics were performed for multiple endocrine neoplasia syndrome 1. Once more common causes have been ruled out, insulinoma should be considered in the differential diagnosis of hypoglycaemia. If the diagnosis of insulinoma is confirmed, the possibility of multiple endocrine neoplasia syndrome 1 should be considered.

Insulin autoimmune syndrome

Insulin autoimmune syndrome (IAS), also known as Hirata's disease, is a rare endocrine disease. It is a form of autoimmune endogenous hyperinsulinemic hypoglycemia, which develops when genetically predisposed individuals get exposed to triggering factors such as viral infection or certain medications containing sulfhydryl groups. It is important to consider IAS in the differential diagnosis of hypoglycemia as its incidence seems to be increasing worldwide. In this article, we summarize a report of two cases of IAS and their clinical manifestations, biochemical characteristics, their step-by-step diagnosis, and also prompt utilization of continuous glucose monitoring system as a monitoring tool for appropriate management. The first patient who presented with hypoglycemic episodes had recently suffered from a viral pneumonia, which probably acted as a superantigen and triggered development of IAS. The second patient also had hypoglycemic episodes, which used to recover after intake of glucose-containing liquids. Alpha-lipoic acid containing multivitamin may have triggered the development of IAS in the second patient. The tests performed showed high serum insulin and C-peptide levels along with insulin autoantibody positivity confirming the diagnosis of Hirata's disease. Immunosuppression therapy was not required as both the patients responded well to diet modification and corticosteroid therapy. It is important to be mindful while prescribing alpha-lipoic acid-containing supplements though it has good antioxidant properties, as it is presumed to be associated with increasing number of IAS cases.

Diffuse, Adult-Onset Nesidioblastosis/Non-Insulinoma Pancreatogenous Hypoglycemia Syndrome (NIPHS): Review of the Literature of a Rare Cause of Hyperinsulinemic Hypoglycemia.

Differential diagnosis of hypoglycemia in the non-diabetic adult patient is complex and comprises various diseases, including endogenous hyperinsulinism caused by functional β-cell disorders. The latter is also designated as nesidioblastosis or non-insulinoma pancreatogenous hypoglycemia syndrome (NIPHS). Clinically, this rare disease presents with unspecific adrenergic and neuroglycopenic symptoms and is, therefore, often overlooked. A combination of careful clinical assessment, oral glucose tolerance testing, 72 h fasting, sectional and functional imaging, and invasive insulin measurements can lead to the correct diagnosis. Due to a lack of a pathophysiological understanding of the condition, conservative treatment options are limited and mostly ineffective. Therefore, nearly all patients currently undergo surgical resection of parts or the entire pancreas. Consequently, apart from faster diagnosis, more elaborate and less invasive treatment options are needed to relieve the patients from the dangerous and devastating symptoms. Based on a case of a 23-year-old man presenting with this disease in our department, we performed an extensive review of the medical literature dealing with this condition and herein presented a comprehensive discussion of this interesting disease, including all aspects from epidemiology to therapy.

Differential Diagnosis of Hypoglycemia

Hypoglycemia is common but can lead to life-threatening consequences. Accurate diagnosis is important to establish the appropriate treatment strategy. Most cases of hypoglycemia are caused by hypoglycemic agents, although it can occur in individuals without diabetes. A systemic and comprehensive diagnostic approach is required to diagnose hypoglycemia in patients without diabetes. It is important to perform appropriate blood testing during an episode of hypoglycemia. This review will focus on the definition, differential diagnosis, causes, and treatment of hypoglycemia, particularly in people without diabetes.

Clinical evaluation of 31 children with pituitary insufficiency in the course of the pituitary stalk interruption syndrome. The unexpected growth without growth hormone in 2 children.

The pituitary stalk interruption syndrome (PSIS) is one of the complex -forms of congenital pituitary insufficiency. Symptoms resulting from insufficiency of the pituitary gland, in spite of the inborn character of the disease, may appear at various stages of life. The aim of this paper was to present clinical presentation in 31 patients with PSIS confirmed radiologically. In the whole study population during first examination 25.8% children were diagnosed with combined pituitary hormone deficiency (CPHD). During the endocrinological observation (median follow-up 5.1 years, range 0.513.2) of the above-mentioned group 74.2% subjects were diagnosed with CPHD, while 25.8% patients with isolated growth hormone deficiency (GHD). Two children with initially short stature were confirmed with GHD. As aresult of the parents' decision, growth hormone therapy was either not started or discontinued. During further follow-up, however, the children achieved normal height. Children with PSIS present adiverse clinical picture and should be observed because of the risk of further pituitary disorders. In the differential diagnosis of hypoglycemia in the neonatal period and in infancy, hypopituitarism should be considered. The phenomenon of normal growth in patients with confirmed growth hormone deficiency has been observed, although is not fully understood.

Toxicity of agents used for opioid withdrawal: a case-based approach.

Toxicity of agents used for opioid withdrawal: a case-based approach.

Recurrent insulinoma in a 10-year-old boy with Down's syndrome.

An insulinoma is a rare tumour with an incidence of four cases per million per year in adults. The incidence in children is not established. There is limited literature available in children with insulinoma, and only one case is reported in association with Down’s syndrome in adults. Insulinoma diagnosis is frequently missed in adults as well as in children. The Whipple triad is the most striking feature although it has limited application in young children. Hypoglycaemia with elevated insulin, C-peptide and absent ketones is highly suggestive of hyperinsulinism. We present a case of 10-year-old boy with Down’s syndrome with recurrent insulinoma. He was initially misdiagnosed as having an adrenal insufficiency and developed cushingoid features and obesity secondary to hydrocortisone treatment and excessive sugar intake. The tumour was successfully localised in the head of the pancreas with an MRI and octreotide scan on first presentation. Medical treatment with diazoxide and octreotide could not achieve normal blood glucose levels. The insulinoma was laparoscopically enucleated and pathological examination confirmed a neuroendocrine tumour. Subsequently, he had complete resolution of symptoms. He had a recurrence after 2 years with frequent episodes of hypoglycaemia. The biochemical workup was suggestive of hyperinsulinism. MRI and PET scan confirmed the recurrence at the same site (head of the pancreas). He had an open laparotomy for insulinoma resection. The pathology was consistent with benign insulinoma, and subsequently, he had complete resolution of symptoms.Learning points:Insulinoma is a very rare tumour in children; it should be considered in the differential diagnosis of hypoglycaemia with absent ketones.Refractory neurological symptoms like seizure, migraine, mood changes and regression of learning abilities should suggest evaluation for hypoglycaemia.MRI with contrast and PET scan would localise the majority of pancreatic beta islet cell lesions.Medical treatment with diazoxide, octreotide and the addition of corn starch in feeds is not curative but can be supportive to maintain normoglycemia until the surgical resection.Surgical resection is the only curative treatment. The surgical procedure of choice (laparoscopic/open laparotomy) depends on local expertise, preoperative localisation, tumour size and number.Surgical treatment results in complete resolution of symptoms, but all cases should be closely followed up to monitor for recurrence. The recurrence rate is four times higher in MEN1 cases.

Clinical case of rare neuroendocrine tumor of pancreas - proinsulinoma

Proinsulinoma is very rare neuroendocrine tumor of pancreas with proinsulin activity. Today there is no data about its true prevalence and occurrence within the syndrome of Multiple Endocrine Neoplasia (MEN) type 1. We have found no one clinical cases of the disease in available domestic literature, but only few clinical cases in foreign literature. Actually the management of patients with proinsulinoma is unknown, because such clinical guidelines are absent. Therefore, it is essential to describe particularly every new case of this neuroendocrine tumor in order to obtain sufficient data about proinsulinoma, because it is unclear whether the following management of such patients is correct or not. The clinical case of patient with proinsulinoma is described in the article. Atypical clinical presentation of hypoglycemia is reported. All stages of diagnostic research with main errors, the disputed key issues of follow up are discussed in detail, especially differential diagnosis of hypoglycemia in real clinical practice, screening of syndrome of MEN type 1 and the protocol of further observation.

GLP1 and glucagon co-secreting pancreatic neuroendocrine tumor presenting as hypoglycemia after gastric bypass.

SummaryPost-prandial hypoglycemia is frequently found after bariatric surgery. Although rare, pancreatic neuroendocrine tumors (pNET), which occasionally are mixed hormone secreting, can lead to atypical clinical manifestations, including reactive hypoglycemia. Two years after gastric bypass surgery for the treatment of severe obesity, a 54-year-old female with previous type 2 diabetes, developed post-prandial sweating, fainting and hypoglycemic episodes, which eventually led to the finding by ultrasound of a 1.8-cm solid mass in the pancreatic head. The 72-h fast test and the plasma chromogranin A levels were normal but octreotide scintigraphy showed a single focus of abnormal radiotracer uptake at the site of the nodule. There were no other clinical signs of hormone secreting pNET and gastrointestinal hormone measurements were not performed. The patient underwent surgical enucleation with complete remission of the hypoglycemic episodes. Histopathology revealed a well-differentiated neuroendocrine carcinoma with low-grade malignancy with positive chromogranin A and glucagon immunostaining. An extract of the resected tumor contained a high concentration of glucagon (26.707 pmol/g tissue), in addition to traces of GLP1 (471 pmol/g), insulin (139 pmol/g) and somatostatin (23 pmol/g). This is the first report of a GLP1 and glucagon co-secreting pNET presenting as hypoglycemia after gastric bypass surgery. Although pNET are rare, they should be considered in the differential diagnosis of the clinical approach to the post-bariatric surgery hypoglycemia patient.Learning points pNETs can be multihormonal-secreting, leading to atypical clinical manifestations.Reactive hypoglycemic episodes are frequent after gastric bypass.pNETs should be considered in the differential diagnosis of hypoglycemia after bariatric surgery.

Commercial insulin immunoassays fail to detect commonly prescribed insulin analogues

ObjectivesBlood insulin and C-peptide are key investigations in the differential diagnosis of hypoglycaemia. Analogues of insulin have modified primary-sequences compared to native human insulin, as such may not cross react with insulin assays. This has important implications in detecting surreptitious or malicious insulin administration.The aim of this study is to assess the cross-reactivity of all insulins currently listed in the British National Formulary (BNF65, 2013) in clinical insulin assays currently used in UK clinical laboratories. Design and methodsSample sets were prepared for all 15 exogenous insulin classes listed in the BNF, at concentrations of 1000pmol/L and 300pmol/L, using pooled human serum. Samples were sent blinded to 5 participating analytical laboratories to cover analysis on the 10 major clinical insulin assays used in the UK. ResultsThe ability of insulin assays to detect exogenous insulin preparations was highly variable and ranged from 0% to >140% for a single exogenous insulin. Four assays were highly specific for the human insulin sequence and had no cross-reactivity with any synthetic analogue insulin. Two detected all insulin types (human sequence, animal and synthetic analogue), with the remaining having variable cross-reactivity. ConclusionThe cross-reactivity of the 15 exogenous insulin preparations is highly variable in the assays used in clinical laboratories around the UK. It is important that laboratories and clinicians are aware of the limitations of their local assays to avoid missing the important diagnosis of hypoglycaemia secondary to excessive exogenous insulin. Where necessary, samples should be referred to specialist centres for insulin analysis and ideally by a validated and fully-quantitative mass spectrometry-based method.

Congenital central hypoventilation syndrome with hyperinsulinemia in an infant

Congenital central hypoventilation syndrome (CCHS) is a rare disorder that typically presents in the newborn period and is characterized by alveolar hypoventilation and symptoms of autonomic nervous system dysregulation. We describe an infant with CCHS who developed hyperinsulinism, which is an uncommon association. She was born by semi-elective Caesarean section at 37 weeks of gestation after a pregnancy complicated by polyhydramnios. Apgar scores were 7and 9 (at 1 and 5 min) and birth weight was 3550 g. She was intubated and ventilated within 24 hours for frequent apnoea’s and desaturations. Multiple extubation attempts on the following days failed because of progressive respiratory acidosis. She underwent tracheostomy at 2 months of age. CCHS was diagnosed in the first month of life, after a polyalanine repeat expansion of the PHOX2B gene (20/26genotype) was detected. At 7 months of age, she was admitted to hospital with seizures associated with hypoglycaemia. A critical sample (glucose 2.5mmol/L) revealed an inappropriately raised plasma insulin of 3.0 mU/L, growth hormone 7mu/L, cortisol 470 mmol/L, and serum ketones <0.1g/L. Urine and serum metabolic screen was normal, as was an EEG. Diazoxide was commenced at 5 mg/kg/day along with hydrochlorothiazide at 1mg/kg/d. These relatively low doses were used in view of parental concerns of potential drug side effect of hirsutism. The dose had to be increased to 7.5mg/kg/day as hypoglycaemic episodes persisted. Capillary blood glucose testing and continuous glucose monitoring showed no further hypoglycaemia at this dose. Sequence analysis of the KCNJ11 and ABCC8 genes implicated in congenital hyperinsulinism was undertaken and no pathogenic mutation was found. The mechanisms underlying abnormal glycaemia in CCHS are not fully understood. Multiple hypotheses have been postulated including the co-expression of the dopamine beta hydroxylase gene mutation with PHOX 2B mutation. Further, disordered autonomic homeostasis as seen in PHOX2B mutations is thought to impact the regulation of insulin and glucagon secretion. Our case highlights the importance of considering hyperinsulinism in the differential diagnosis of hypoglycaemia in infants with CCHS. Written informed consent was obtained from the patient's parent or guardian for publication of this abstract and any accompanying images. A copy of the written consent is available for review by the Editor of this journal.

Index of Suspicion in the Nursery

A term male infant is transferred to the NICU for persistent hypoglycemia at age 3.5 hours. The infant had a blood sugar level of 21 mg/dL at age 3 hours and despite refeeding 20 mL of 20 calories per ounce of formula, his blood glucose level remained at 20 mg/dL at 3.5 hours. The infant was born via cesarean delivery for a nonreassuring heart rate tracing to a 19-year-old G1P 0 African-American mother. All prenatal laboratory results were negative, including an oral glucose tolerance test. Prenatal ultrasonography was performed during the second trimester and showed no gross fetal anomalies other than the presence of a two-vessel cord. The infant cried spontaneously at birth and had some transient respiratory distress. The infant weighs 2,960 g (50th percentile) with a length of 48 cm (50th percentile) and a head circumference of 32.5 cm (25th–50th percentile). Results of the physical examination are normal except for a small penis (stretched penile length of 1.5 cm, width of 0.6 cm) and an undescended left testicle. Blood glucose levels below 40 mg/dL persist despite two boluses of 10% dextrose and administration of continuous dextrose infusion with a glucose infusion rate of ∼5 mg/kg per minute. Glucose levels above 40 mg/dL are eventually achieved with a glucose infusion rate of 8 mg/kg per minute. The pediatric endocrinology service was consulted on the second day for persistent hypoglycemia. A 3,490-g term male infant is born to a 20-year-old primigravida mother. She received good prenatal care, and her pregnancy was uncomplicated. Antenatal screening for human immunodeficiency virus, hepatitis B, and syphilis were negative, and she had immunity against rubella. Results of the patient’s cervical culture were positive for group B streptococcus, but she received adequate intrapartum penicillin prophylaxis before delivery. There was no abnormality noted on the fetal cardiotocography. …

Recurrent Hypoglycemia from Insulin Autoimmune Syndrome

Insulin autoimmune syndrome (IAS) is an uncommon cause of hyperinsulinemic hypoglycemia characterized by autoantibodies to endogenous insulin in individuals without previous exposure to exogenous insulin. IAS is the third leading cause of spontaneous hypoglycemia in Japan, and is increasingly being recognized worldwide in non-Asian populations. We report a case of IAS in a Caucasian woman with recurrent complaints of hypoglycemia, with laboratory findings of serum glucose 2.5 mmol/L (45 mg/dL), insulin 54,930 pmol/L (7,909 μIU/mL), connecting peptide (C-peptide) 4,104 pmol/L (12.4 ng/mL), and a corresponding insulin to C-peptide molar ratio of 13.4 during a spontaneous hypoglycemic event. Autoantibodies to insulin were markedly elevated at > 50 kU/L (> 50 U/mL). IAS should be considered in the differential diagnosis of hypoglycemia in non-diabetic individuals. Distinction from insulinoma is especially crucial to prevent unwarranted invasive procedures and surgical interventions in hypoglycemic patients.

Profound Neonatal Hypoglycemia and Lactic Acidosis Caused by Pyridoxine-Dependent Epilepsy

Pyridoxine-dependent epilepsy (PDE) was first described in 1954. The ALDH7A1 gene mutations resulting in α-aminoadipic semialdehyde dehydrogenase deficiency as a cause of PDE was identified only in 2005. Neonatal epileptic encephalopathy is the presenting feature in >50% of patients with classic PDE. We report the case of a 13-month-old girl with profound neonatal hypoglycemia (0.6 mmol/L; reference range >2.4), lactic acidosis (11 mmol/L; reference range <2), and bilateral symmetrical temporal lobe hemorrhages and thalamic changes on cranial MRI. She developed multifocal and myoclonic seizures refractory to multiple antiepileptic drugs that responded to pyridoxine. The diagnosis of α-aminoadipic semialdehyde dehydrogenase deficiency was confirmed based on the elevated urinary α-aminoadipic semialdehyde excretion, compound heterozygosity for a known splice mutation c.834G>A (p.Val278Val), and a novel putative pathogenic missense mutation c.1192G>C (p.Gly398Arg) in the ALDH7A1 gene. She has been seizure-free since 1.5 months of age on treatment with pyridoxine alone. She has motor delay and central hypotonia but normal language and social development at the age of 13 months. This case is the first description of a patient with PDE due to mutations in the ALDH7A1 gene who presented with profound neonatal hypoglycemia and lactic acidosis masquerading as a neonatal-onset gluconeogenesis defect. PDE should be included in the differential diagnosis of hypoglycemia and lactic acidosis in addition to medically refractory neonatal seizures.

Testing for insulin antibodies is mandatory in the differential diagnosis of hypoglycaemia in nondiabetic subjects

Clinical EndocrinologyVolume 76, Issue 4 p. 603-604 LETTERS TO THE EDITOR Testing for insulin antibodies is mandatory in the differential diagnosis of hypoglycaemia in nondiabetic subjects Adel A. A. Ismail, Adel A. A. Ismail ‘Tanglewood’ Chevet Lane, Wakefield, West Yorkshire, UK E-mail: adelaaismail@aol.comSearch for more papers by this author Adel A. A. Ismail, Adel A. A. Ismail ‘Tanglewood’ Chevet Lane, Wakefield, West Yorkshire, UK E-mail: adelaaismail@aol.comSearch for more papers by this author First published: 13 October 2011 https://doi.org/10.1111/j.1365-2265.2011.04259.xCitations: 8Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinked InRedditWechat No abstract is available for this article.Citing Literature Volume76, Issue4April 2012Pages 603-604 RelatedInformation

Type B insulin resistance syndrome with systemic lupus erythematosus

Type B insulin resistance syndrome is a rare disease. Auto-antibodies to the insulin receptor frequently appear in the case of systemic lupus erythematosus (SLE). We report herein a case of a 56-year-old man who had presented discoid skin lesions since 1990. He was admitted to the hospital because of unconsciousness and severe hypoglycemia in 2006, and was diagnosed as having Type B insulin resistance syndrome with the presence of insulin receptor antibody. He had frequently repeated hypoglycemic and hyperglycemic episodes in spite of treatment with prednisolone (5 - 10 mg/day), and mild proteinuria of 1.5 g/day was observed. His laboratory findings on admission revealed pancytopenia and positive titer for antinuclear antibody (ANA). From these findings and his past history of skin lesions, we diagnosed him as SLE. We performed renal biopsy and his histological diagnosis was lupus nephritis Class 5 with the findings of podocytic shedding. Prednisolone dosage was increased from 10 to 60 mg/day. Thereafter, his glucose metabolism improved and proteinuria disappeared. The dose of prednisolone was tapered to 30 mg/day without recurrence of hypoglycemia and proteinuria. Early treatment with prednisolone might ameliorate proteinuria and insulin resistance. We experienced a rare case of Type B insulin resistance syndrome with increased activity of SLE, complicated with lupus nephritis. It appears that Type B insulin resistance syndrome should be suspected in differential diagnosis of hypoglycemia in SLE patients.

Fatal hypoglycemia in malignant pheochromocytoma: direct glucose consumption as suggested by 18F-2-fluoro-2-deoxy-d-glucose positron emission tomography/computed tomography imaging

We present a patient with metastatic pheochromocytoma, who developed progressive and fatal hypoglycemia most likely secondary to direct tumor glucose consumption that did not respond to high-dose glucose infusion, corticosteroids, or glucagon therapy. The pattern of glucose uptake on (18)F-2-fluoro-2-deoxy-D-glucose positron emission tomography, with preferential tumor glucose uptake in association with a marked reduction in normal uptake in the heart, muscles, and brain, is highly suggestive of direct consumption of glucose by the tumor rather than insulin-like growth factor-2 mediated hypoglycemia. In patients with large-volume metastatic malignancies, direct tumor glucose consumption should be considered in the differential diagnosis of hypoglycemia. Nuclear medicine imaging techniques can illustrate the pathophysiology of hypoglycemia in such cases.

Reversible Magnetic Resonance Imaging Changes Associated With Hypoglycemia -Case Report-

A 63-year-old man was found with confusion and right limb monoparesis. He was taken to the emergency center under suspicion of stroke. Head computed tomography and magnetic resonance (MR) imaging and MR angiography were immediately conducted, which revealed no abnormality, but diffusion-weighted imaging showed increased intensity areas in the splenium of the corpus callosum and the left posterior limb of the internal capsule with decreased apparent diffusion coefficient (ADC) in the same areas. Immediately after the head scan, blood sugar level was measured, which revealed hypoglycemia (23 mg/dl). He quickly became lucid after intravenous administration of 20 ml of 50% glucose solution, and the paresis disappeared. Follow-up brain MR imaging was conducted 3 days later, but no clearly abnormal findings were seen on T(2)-weighted, fluid-attenuated inversion recovery, diffusion-weighted, or ADC images. Reports of reversible high intensity area in the splenium of the corpus callosum on diffusion-weighted imaging due to transient hypoglycemia are rare. Hemiparesis is one of the manifestations of hypoglycemia, so verifying the blood sugar level is important. Since MR imaging can be conducted easily now, we may need to consider the imaging findings in the differential diagnosis of hypoglycemia.

Seltene Ursache für eine Hypoglykämie bei einem Patienten mit Typ-2-Diabetes mellitus

Insulinomas are rare neuroendocrine tumours with an incidence of four cases per million a year. Only few cases of insulinoma in patients with preexisting diabetes mellitus have been reported. We present a 50-year-old male with type 2 diabetes mellitus who suffered from recurring hypoglycemia. He had gained 20 kilograms of weight in five years. 72-hour fast revealed hypoglycaemia in the presence of inadequately high C-peptide and insulin levels. Magnetic resonance imaging and selective arterial calcium stimulation test confirmed a mass in the body of the pancreas. The tumor was removed surgically. Pathological examination demonstrated a benign insulinoma. Postoperatively, blood glucose levels were within the therapeutic range. The HbA (1c) value was 6.8 % three months after the intervention. Clinicians should be alert to insulinoma as a, though rare, differential diagnosis of hypoglycaemia in diabetes, in particular in patients with recurrent, otherwise unexplained hypoglycaemia.

Type B insulin resistance syndrome induced by increased activity of systemic lupus erythematosus in a hemodialysis patient

A-23-year-old woman with systemic lupus erythematosus (SLE) and on hemodialysis for 5 years was admitted to the hospital because of severe general fatigue. On admission, laboratory findings showed that fasting plasma glucose levels ranged from 25 â 45 mg/dl, a test for antinuclear antibody (ANA) was positive (1 : 320, with a discrete-speckled pattern), serum C3 and C4 complement and CH50 level were remarkably depressed (22.5 mg/dl, < 5.1 mg/dl, < 13 U/ml, respectively), and insulin receptor antibodies were present (89.3% inhibition of 125I-insulin binding to cultured human lymphocytes by a binding inhibition assay). She also showed acanthosis nigricans. The patient was diagnosed to suffer from a Type B insulin resistance syndrome. The patient's serum insulin and C-peptide levels were markedly elevated during hypoglycemia without insulinoma (2,313.8 microU/ml, 55 ng/ml, respectively). Interestingly, not only postprandial hyperglycemia but also fasting hypoglycemia was observed. Treatment with steroid pulse and subsequent high dose of prednisolone resulted in restoration of euglycemia associated with disappearance of insulin receptor antibodies and improvement of both serum hypocomplementemia and the high titer of ANA. Later, the patient's course was complicated by hemorrhagic shock due to duodenal ulcer and she died of subsequent pneumocystis carinii pneumonia. The presented patient developed a Type B insulin resistance syndrome induced by increased activity of SLE after she had been treated with hemodialysis for 5 years. This is the first reported case with such a history and constellation. It is recommended that SLE patients on dialysis are carefully followed-up by clinical and serological monitoring. Type B insulin resistance syndrome must be considered in the differential diagnosis of hypoglycemia in SLE patients on dialysis.