Hypoglycemia Syndrome Research Articles

Low sensitivity of chromogranin A in the diagnosis of insulinoma: a single-center study

Background: Insulinoma is a neuroendocrine tumor (NET), with its main clinical manifestation being the hypoglycemic syndrome. The symptoms of hypoglycemia are nonspecific, and therefore, in most cases, the diagnosis is made untimely. The Russian clinical guidelines for the diagnosis and treatment of NET suggest as a diagnostic test that the universal circulating marker of all NET, chromogranin A (CgA) be determined. However, the literature data on the sensitivity of CgA in the diagnosis of insulinoma are contradictory. Aim: To evaluate the diagnostic effectiveness of the CgA test in the diagnosis of insulinoma. Materials and methods: This was a hospital-based single-center, cross-sectional comparative (first step) and prospective non-comparative (second step) study conducted from 2016 to 2022. During the first part of the study, we determined serum CgA in 120 patients with suspected non-diabetic hypoglycemia and compared its levels in the patients with and without confirmed insulinoma (n = 87 and n = 33, respectively). During the second study step, CgA was measured in the insulinoma patients at 6 [4.0; 7.0] months after surgery. The CgA levels at baseline and post-surgery were analyzed in 74 patients (those with recurring non-diabetic hypoglycemia were excluded from the analysis). Results: In the study subjects without insulinoma, the median CgA level was 0.7 [0.5; 1.1] (range, 0.1 to 2.0) nmol/l and the difference (with Bonferroni adjustment) from its levels in the patients with insulinoma before surgery was non-significant (1.0 [0.7; 1.4], range, 0.1 to 8.5 nmol/l, р = 0.045). The CgA concentration in the insulinoma patients after surgery was 0.9 [0.7; 1.2], range, 0 to 1.9 nmol/l and significantly differed from that at baseline (1.0 [0.7; 1.4], range, 0.1 to 8.5 nmol/l, p = 0.012, Wilcoxon test). In the patients with insulinoma before surgery the CgA levels exceeding the generally accepted reference range ( 2 nmol/l) was found in 11.5% (n = 10), with its median level of 2.5 [2.3; 4.1], range 2.3 to 8.5 nmol/l. There were no significant associations between the CgA levels and localization, tumor numbers, their size and malignization grade, insulin and proinsulin values, and duration of fasting. The sensitivity and specificity of the CgA test were 12% [95% confidence interval [CI]: 6%–20%] and 100% [95% CI: 97%–100%], respectively. The prognostic value of a positive result (PVPR) was 100% [95% CI: 69%–100%] and the prognostic value of a negative result (PVNR) 30% [95% CI: 28%–32%]. Conclusion: As a diagnostic test, CgA has high specificity and prognostic value of a positive result. However, the uncertainty of the prognostic value of a negative result is unacceptably high. A special study is required to clarify the operational characteristics of CgA in insulinoma.

6712 Intractable Hypoglycemia: A Clue to Tumor Diagnosis

Abstract Disclosure: N. Kharkongor Chengappa: None. E.I. Krug: None. Background: Non-Islet Cell Tumor Hypoglycemia (NICTH) known as Doege-Potter syndrome, is a rare paraneoplastic syndrome of hypo-insulinemic hypoglycemia caused by excessive production of partially processed IGF-II from a solitary fibrous tumor (SFT).We present a case of a patient admitted with intractable hypoglycemia leading to the diagnosis and treatment of a large pelvic SFT. Clinical Case: A 59-year-old male with type 2 Diabetes Mellitus on metformin mono therapy, was admitted after he collapsed at work and was found to have blood glucose (BG) of 22 mg/dl. He was previously in his usual state of health except for an increase in his shoe size over the past year. Physical examination revealed coarse thick skin, large coarse bullous nose, enlarged fingers and a non-tender, firm abdomen with no obvious organomegaly. Laboratory results including CBC, CMP and thyroid function were normal. ACTH stimulation test revealed normal adrenal function. Hypoglycemic drug screen and insulin antibodies were negative. During a hypoglycemic event (BG of 45mg/dl) he had C-peptide level < 0.1ng/ml, beta-hydroxybutyrate levels of <0.05 and insulin level <0.4uIU/ml, indicating non-insulin mediated hypoglycemia. IGF-I and IGF-II levels were ordered. Due to a concern for paraneoplastic etiology of hypoglycemia. CT scan revealed a 20 cm solid mass, filling most of the pelvis and extending into the lower abdomen. Further investigations revealed normal PSA, CEA, CA19-9 and undetectable hCG and AFP levels. MRI confirmed a presence of 23 cm pelvic mass with few areas of central necrosis, extending into the lower abdomen. CT-guided biopsy revealed spindle cell neoplasm consistent with SFT. The patient underwent pelvic mass resection. Postoperatively, hypoglycemia resolved with BG readings of 138mg/dl to 178mg/dl. Surgical pathology results confirmed malignant SFT with positive STAT 6 and CD34 markers. IGF-II level obtained prior to surgery was markedly elevated at 2029 ng/ml (38-267 ng/mL). Conclusion: NICTH was described in 1930 by KW Doege and RP Potter. Less than 5% of patients with SFTs develop NICTH. Diagnosis requires manifestation of Whipple's triad, low insulin, pro-insulin and C-peptide levels, elevated IGF-II levels, or an IGF-I/IGF-II ratio>2, and the identification of the tumor. Positive immunohistochemical stain of the tumor for STAT6 confirms the diagnosis. Management involves resection of the tumor when feasible or debulking. For higher-risk tumors, radiation therapy may be considered. In inoperable cases, glucocorticoids, recombinant hGH and glucagon are recommended for palliation of hypoglycemia. Octreotide and Diazoxide are ineffective in NICTH.

8262 Non-insulinoma pancreatogenous hypoglycemia syndrome (NIPHS) after gastric sleeve surgery

Abstract Disclosure: R.E. Thomas: None. A. Charales: None. B. Bangash: None. S. Haider: None. S. Ouais: None. Introduction: Non-insulinoma pancreatogenous hypoglycemia syndrome (NIPHS) is a rare condition characterized by endogenous hyperinsulinemic hypoglycemia after meals. This article explores NIPHS, emphasizing its occurrence post-gastric sleeve surgery. We delve into clinical features, diagnosis, treatment, and challenges, using a case presentation to illustrate complexities.Clinical Features and Classification:NIPHS entails endogenous hyperinsulinemic hypoglycemia without islet cell neoplasms, featuring islet cell hypertrophy. Formerly "Nesidioblastosis," post-RYGB cases constitute a distinct diagnosis. Common features include postprandial hyperinsulinemic hypoglycemia, negative radiologic localization, and a positive calcium stimulation test.Patients present postprandial hypoglycemia 2-4 hours after meals, with symptoms like tachycardia, tremors, confusion, and occasional seizures. Lab findings include elevated insulin, C peptide, and pro-insulin, low beta-hydroxybutyrate, and negative sulfonylurea/meglitinide screen.Diagnostic Workup:Evaluate glucose, insulin, pro-insulin, C peptide, beta-hydroxybutyrate, and sulfonylurea/meglitinide. Ultrasound differentiates insulinoma from diffuse processes. If negative, conduct SACST for localization.Case Presentation:A 37-year-old female post-gastric sleeve surgery presented with severe hypoglycemia. Symptoms included confusion, nausea, and palpitations, occurring postprandially, relieved by food or juice. Glucagon was administered, and consultations sought. Diazoxide stabilized blood glucose.Treatment Modalities:Dietary modifications reduce free carbohydrate intake, spaced meals minimize hyperinsulinemic episodes. Medical management (octreotide, verapamil, diazoxide, acarbose) proves effective, especially post-gastric bypass. Sub-total pancreatectomy is considered in severe refractory cases.Multidisciplinary Approach:Management involves endocrinologists, surgeons, radiologists, and dietitians. Regular monitoring assesses efficacy, adjusts medications, and addresses complications. Challenges and Future Perspectives: NIPHS complexities include variable presentation and diagnostic challenges. Research should refine criteria, explore interventions, and understand pathophysiology. Conclusion: Non-insulinoma pancreatogenous hypoglycemia syndrome, especially post-gastric sleeve surgery, presents complexities. This exploration highlights clinical aspects, diagnostics, and management challenges, emphasizing the need for a multidisciplinary approach. Continued research aims to enhance diagnostic precision and improve outcomes for those affected by this intriguing syndrome. Presentation: 6/3/2024

6666 Asymptomatic Fasting Hypoglycemia After Sleeve Gastrectomy

Abstract Disclosure: K. Mitrollari: None. H.K. Deveaux: None. A. Gundeti: None. A.P. Calimag: None. T. Yasmeen: None. Introduction: Post-bariatric surgery hypoglycemia (PBSH), though previously considered rare, is increasingly recognized, occurring not only after Roux-en-Y gastric bypass (RYGB,) but also following various bariatric procedures. PBSH can manifest as postprandial or fasting hypoglycemia, requiring thorough investigation and prevention due to potential asymptomatic presentations that may lead to severe consequences, including coma and death. Case Report A 45-year-old female with a history of type 2 DM, ESRD on HD, gastric sleeve surgery, bilateral trans-metatarsal amputations, coronary artery disease, and congestive heart failure presented with bilateral lower extremity wounds. On admission her venous blood glucose was 71 mg/dL. Glycohemoglobin was 3.9% and fructosamine was 225 (205-285 umol/L). Her glycohemoglobin levels consistently remained below 6.4% status post sleeve gastrectomy off diabetes medications. Throughout admission, she experienced almost daily episodes of fasting asymptomatic hypoglycemia correlated with venous sample with lowest blood glucose reading 38 mg/dL. Initially, these episodes were attributed to NPO status for procedures or decreased PO intake. Her diet consisted of fast food brought by her family, which she reported was similar to her diet at home. Workup during a hypoglycemic episode (glucose 51 mg/dL,) revealed insulin 4 mUnits/L, proinsulin 7.1 pmol/L (<=7.2 pmol/L), C-peptide 25.7 ng/mL (0.8-3.9 ng/mL), and beta-hydroxybutyrate 0.5 mmol/L (0.0-0.3 mmol/L). Negative insulin antibodies and sulfonylurea panel, normal cortisol, and ACTH levels were noted. The patient was placed on a 10% dextrose infusion to avoid further episodes of hypoglycemia. However, she continued to experience hypoglycemia and was started on oral diazoxide. A CT pancreas with and without contrast was unremarkable, and endoscopic ultrasonography only revealed a 10 mm accessory spleen. The patient was discharged with counseling on glucose monitoring, dietary changes, and follow-up with endocrinology. Conclusion: PBSH should be considered as a differential diagnosis in patients with unexplained hypoglycemia and a history of bariatric surgery. PBSH mimics insulinoma or non-insulinoma pancreatogenous hypoglycemia syndrome (NIPHS) with elevated insulin, proinsulin, C-peptide, and low beta-hydroxybutyrate during hypoglycemia, however NIPHS is exclusive to non-bariatric patients. Imaging is crucial to rule out insulinoma, and invasive tests such as endoscopic ultrasound aid diagnosis. PBSH is a diagnosis of exclusion; therefore, workup involves ruling out other causes of hypoglycemia. First-line treatment involves diet modification, with medications like diazoxide or acarbose considered if dietary changes prove insufficient. This case underscores the complexity of hypoglycemia, implicating bariatric surgery as a contributing factor. Presentation: 6/3/2024

Trends in Presentation and Management of Endogenous Hyperinsulinaemic Hypoglycaemia Over the Last Three Decades at a Tertiary Care Centre (1992-2022).

Endogenous hyperinsulinaemic hypoglycaemia (EHH) is characterized by inappropriate insulin secretion from pancreatic beta cells despite low blood glucose concentrations. We aimed to evaluate the secular changes in presentation and management of EHH due to insulinoma/non-insulinoma pancreatogenous hypoglycaemia syndrome (NIPHS) at our centre. This was a single-centre ambispective study (2014-2022). The clinical, biochemical, hormonal and radiological parameters (n = 63) collected as part of this study were compared with our earlier studies (1992-2005, n = 31; and 2006-2013, n = 35) and with other centres across the world. A total of 63 patients (39 males) with a preoperative diagnosis of EHH (insulinoma, n = 58; and NIPHS, n = 5) and a mean age of 40.7 years were studied. The mean lag time from the onset of symptoms to diagnosis decreased from 4.6 years during the first study period to 1.9 years during this study period. However, the majority presented with fasting hypoglycaemia of 98.4%, and both fasting and postprandial hypoglycaemia of 32%. Exclusive postprandial hypoglycaemia was present in 1.7% of insulinoma. A histopathological diagnosis of insulinoma was made in 52 patients and nesidioblastosis in two patients. Intraoperative ultrasonography (IOUS) and intraoperative palpation (IOP) yielded 100% sensitivity, while endoscopic ultrasonography (EUS) and 68Ga-DOTA-Exendin-4 positron emission tomography/computed tomography (PET/CT) yielded sensitivity of 86% and 85%, respectively, for localizing insulinoma. Resolution of hypoglycaemia was noted in 53 of 57 (93%) patients who underwent surgery with a preoperative diagnosis of insulinoma. We observed a trend towards earlier diagnosis of EHH, increased patient numbers and availability of nuclear imaging techniques for preoperative localization in the last decade compared to earlier.

Genotype-histotype-phenotype correlations in hyperinsulinemic hypoglycemia.

Hyperinsulinemic hypoglycemia (HH) of pancreatic origin includes congenital hyperinsulinism (CHI), insulinoma, insulinomatosis, and adult-onset non-insulinoma persistent hyperinsulinemic hypoglycemia syndrome (NI-PHHS). In this review, we describe the genotype-histotype-phenotype correlations in HH and their therapeutic implications. CHI can occur from birth or later on in life. Histologically, diffuse CHI shows diffuse beta cell hypertrophy with a few giant nuclei per islet of Langerhans, most frequently caused by loss-of-function mutations in ABCC8 or KCNJ11. Focal CHI is histologically characterized by focal adenomatous hyperplasia consisting of confluent hyperplastic islets, caused by a paternal ABCC8/KCNJ11 mutation combined with paternal uniparental disomy of 11p15. CHI in Beckwith-Wiedemann syndrome is caused by mosaic changes in the imprinting region 11p15.4-11p15.5, leading to segmental or diffuse overgrowth of endocrine tissue in the pancreas. Morphological mosaicism of pancreatic islets is characterized by occurence of hyperplastic (type 1) islets in one or a few lobules and small (type 2) islets in the entire pancreas. Other rare genetic causes of CHI show less characteristic or unspecific histology. HH with a predominant adult onset includes insulinomas, which are pancreatic insulin-producing endocrine neoplasms, in some cases with metastatic potential. Insulinomas occur sporadically or as part of multiple endocrine neoplasia type 1 due to MEN1 mutations. MAFA mutations may histologically lead to insulinomatosis with insulin-producing neuroendocrine microadenomas or neuroendocrine neoplasms. NI-PHHS is mainly seen in adults and shows slight histological changes in some patients, which have been defined as major and minor criteria. The genetic cause is unknown in most cases. The diagnosis of HH, as defined by genetic, histological, and phenotypic features, has important implications for patient management and outcome.

Neonatal outcomes in women with diabetes mellitus — analysis of DAPSY data

BACKGROUND: Hyperglycemia in pregnancy is associated with short- and long-term implications for children. Nevertheless, the effects of different types of diabetes mellitus and treatment methods on the neonatal outcomes are to be investigated. AIM: The aim of this study was to evaluate the contribution of different types of maternal diabetes mellitus to the risk of adverse neonatal outcomes. MATERIALS AND METHODS: This retrospective cohort study included women (n = 3261) who delivered at the Research Institute of Obstetrics, Gynecology and Reproductology named after D.O. Ott, Saint Petersburg, Russia in 2008–2017. The following comparison groups were used: type 1 diabetes mellitus (n = 506; 1a, continuous subcutaneous insulin injections; 1b, multiple daily insulin injections), type 2 diabetes mellitus (n = 229; 2a, diet; 2b, insulin therapy), gestational diabetes mellitus (n = 2387; 3a, diet; 3b, insulin therapy), and control (n = 139). The main birth outcomes assessed included weight and length of newborns, Apgar score at birth and at 5 minutes after birth, and pathological conditions such as fetal macrosomia, syndrome of infant of mother with gestational diabetes mellitus or diabetic mother (P70.0–P70.1), light (P05.0) or small for gestational age (P05.1), intrauterine growth restriction, prematurity (P07), neonatal hypoglycemia (P70.4), and neonatal respiratory distress syndrome (P22). The secondary birth outcomes assessed included birth trauma (P10–P15), stillbirth (P95), disturbances of cerebral status of newborn (P91), and congenital malformations, deformations and chromosomal abnormalities (Q00–Q99). RESULTS: Pregestational diabetes mellitus is strongly associated with adverse neonatal outcomes. Type 1 diabetes mellitus women had the highest risks for fetal macrosomia and diabetic fetopathy, neonatal hypoglycemia, prematurity, and congenital malformations (odds ratio 3.54, 20.2, 5.59, 4.24 and 3.92 respectively). In type 2 diabetes mellitus patients, the risks of low birth weight, intrauterine growth restriction and birth trauma (odds 9.14, 5.42 and 6.3 respectively) were higher. For women with gestational diabetes mellitus, these risks were lower. CONCLUSIONS: This study provides a comprehensive analysis of the major neonatal pathology in patients with different types of diabetes mellitus, taking into account the treatment method. Preconception care and optimal glycemic control are necessary to reduce the risk of obstetric and perinatal complications in women with pregestational types of diabetes mellitus. Further research into the health status of this cohort of children at a later age is required.

Hypoglycemic syndrome: etiological factors, diagnosis and treatment algorithm

Hypoglycemic syndrome: etiological factors, diagnosis and treatment algorithm

Management of non-malignant hyperinsulinemic hypoglycaemia in resource limited setting: a case report

A 37 y.o. Asian male presented with frequent hypoglycaemia in both fasting and post-prandial state. He had elevated blood insulin levels during the hypoglycaemic episodes with normal pancreatic morphology and no extra-pancreatic tumor. Through systematic symptom assessment and the use of simple laboratory examination, non-insulinoma pancreatogenous hypoglycaemia syndrome presented as the most likely diagnosis. Conclusive aetiological diagnosis could not be reached due to the limited availability of diagnostic modalities. Nevertheless, modest symptom control and frequency reduction of hypoglycaemia were achieved with empirical dietary modification and α-glucosidase inhibitor treatment.

Peculiar case of post-gastrectomy noninsulinoma pancreatogenous hypoglycemic syndrome

Peculiar case of post-gastrectomy noninsulinoma pancreatogenous hypoglycemic syndrome

Histopathological features of non-insulinoma pancreatogenous hypoglycemia syndrome: A rare cause of hypoglycemia in non-diabetic adults

Histopathological features of non-insulinoma pancreatogenous hypoglycemia syndrome: A rare cause of hypoglycemia in non-diabetic adults

Exposure of Pregnancy to Pregestational Diabetes, Gestational Diabetes, and Antidiabetic Medications With Especial Focus on Major Congenital and Cardiac Malformations in Offspring.

The global prevalence of type 2 diabetes mellitus (T2DM) is increasing. T2DM is more common in patients with psychiatric disorders and those who take certain psychotropic drugs. T2DM occurs in 2%-7% of women of reproductive age. The prevalence of pregestational diabetes is 0.5%-2.4%, and that of gestational diabetes is 7%-28%, depending on geographical region. About 20%-50% of pregnancies, across the world, are unplanned; this figure is higher, at about 65%, in women with psychiatric disorders. As a result, many women of reproductive age who have diabetes, including women who do not know that they have diabetes, may unintentionally become pregnant, thus unknowingly exposing their pregnancy to diabetes and its treatment. Exposure of pregnancy to pregestational and gestational diabetes is associated with risks to the mother as well as risks to the child. Risks to the mother include obesity, hypertension, and preeclampsia. Risks to the child include spontaneous abortion, fetal death, macrosomia, major congenital malformations (MCMs), preterm delivery, neonatal hypoglycemia, neonatal hyperbilirubinemia, and neonatal respiratory distress syndrome. A recent large retrospective cohort study with data from 6 countries in Europe, Asia, and North America found that, in about 51,000 women with pregestational T2DM, neither MCMs nor cardiac malformations were more prevalent in offspring of children periconceptionally exposed to second-line antidiabetic treatments relative to exposure to insulin. These findings are reassuring but have limitations that are discussed. A reasonable conclusion from a reading of the reviewed literature is that pregestational and gestational diabetes are best treated during pregnancy, that insulin is a first-line treatment, that metformin is an increasingly accepted alternative, and that safety data on second-line antidiabetic treatments are, so far, reassuring.

Инсулинома: обзор литературы и описание клинического наблюдения

Insulinoma is a neuroendocrine tumor arising from β-cells of the islets of Langerhans, causing hypoglycemic attacks due to endogenous hyperinsulinism. The worldwide prevalence is approximately 1 case per 250,000–1,000,000 population, the incidence is 1–4 cases per million population per year. In most cases, the formation of insulinoma is sporadic (90%), less often it can be detected as part of the multiple endocrine neoplasia syndrome type 1 (10%). This review discusses the pathogenetic mechanisms of hypoglycemic syndrome, criteria for differential diagnosis, modern laboratory and imaging methods, and approaches to the treatment of insulinoma. A clinical observation is presented that demonstrates the difficulties of timely diagnosis of insulinoma due to nonspecific manifestations of hypoglycemia. The time that elapses from the first manifestations of hypoglycemic syndrome to diagnosis directly affects the prognosis and quality of life of a patient with insulinoma. It requires vigilance from doctors of all specialties regarding the clinical features of this disease.

A Critical Review of Diagnostic Strategies and Maternal Offspring Complications in Gestational Diabetes Mellitus.

Gestational diabetes mellitus (GDM) is a complex and significant health concern affecting pregnant individuals and their offspring. This review provides a comprehensive examination of GDM, focusing on diagnostic strategies and the associated maternal and offspring complications. We delve into the challenges and controversies surrounding GDM diagnosis, including the variability in diagnostic criteria, diagnostic accuracy and reproducibility issues, ethical considerations, and the influence of ethnicity and genetics. Maternal complications, such as preeclampsia, cesarean sections, long-term health implications, and neonatal complications like macrosomia, hypoglycemia, and respiratory distress syndrome, are explored in detail. Additionally, we investigate the long-term risks of childhood obesity and type 2 diabetes in offspring and potential cognitive and developmental outcomes. This review underscores the critical importance of early detection and effective management of GDM, the need for standardized diagnostic criteria, personalized care plans, and the ongoing pursuit of research to enhance our understanding of this complex condition. GDM remains a dynamic field where ongoing innovation and research promise to improve the health outcomes of pregnant individuals and their children.

The effectiveness of booklets on family knowledge of diabetes mellitus patients about the management of hypoglycaemia

The three main acute complications of diabetes mellitus related to an imbalance in glucose levels that lasted in the short term were hypoglycemia, diabetic ketoacidosis (DKA), and hyperglycemic hyperosmolar nonketotic syndrome. The family, as the people closest to and always interacting with the patient, played a significant role in preventing complications. A booklet served as one medium to increase knowledge for families. This study aimed to determine the effectiveness of the booklet on the knowledge of families of DM patients regarding the management of hypoglycemia. A quantitative method with a quasi-experimental design approach involving pre-test and post-test designs was employed. The population in this study comprised families of Diabetes Mellitus patients who were at Puskesmas (Public Health Centre) Batu X and Puskesmas Mekarbaru, Indonesia. The research sample consisted of 70 respondents selected through purposive sampling. The research variables included demographic factors, family knowledge before and after the intervention in the intervention and control groups, and bivariate analysis. The research instrument employed a questionnaire and a booklet regarding the management of hypoglycemia. Independent t-test analysis was conducted (α = 0.05). The booklet proved effective in improving the knowledge of families of diabetes mellitus patients regarding the management of hypoglycemia, with a p-value of 0.028. The booklet can enhance family knowledge about the management of hypoglycemia. Therefore, every internal medicine clinic or health centre should provide booklets as an educational medium.

Gestational Diabetes Mellitus: Association with Maternal and Neonatal Complications.

Background and objectives: Gestational diabetes mellitus (GDM) is known to be associated with pregnancy complications but there is limited evidence about the strength of these associations in recent clinical practice, especially after the introduction of strict guidelines for the management of pregnancies with GDM in a multidisciplinary team setting. The objectives of our study were to first compare the rates of complications in pregnancies with GDM with those that had pre-existing diabetes mellitus and those without diabetes; and second, to derive measures of effect size expressed as odds ratios after adjustment for confounding factors to assess the independent association of GDM in prediction of these pregnancy complications. Materials and Methods: This was a prospective cohort study undertaken at a large maternity unit in the United Kingdom between January 2010 and June 2022. We included singleton pregnancies that were booked at our unit at 11-13 weeks' gestation. Multivariate regression analysis was carried out to determine the risks of complications in pregnancies with GDM after adjusting for pregnancy characteristics. Risks were expressed as odds ratio (OR) (95% confidence intervals [CI]) and expressed graphically in forest plots. Results: The study population included 53,649 singleton pregnancies including 509 (1%) with pre-existing DM, 2089 (4%) with GDM and 49,122 (95%) pregnancies without diabetes. Multivariate regression analysis demonstrated that there was a significant independent contribution from GDM in the prediction of adverse outcomes, including maternal complications such as preterm delivery, polyhydramnios, preeclampsia and delivery of large for gestational age neonates and elective caesarean section (CS); and neonatal complications including admission to neonatal intensive care unit, hypoglycaemia, jaundice and respiratory distress syndrome. Conclusions: GDM is associated with an increased rate of pregnancy complications compared to those without diabetes, even after adjustment for maternal and pregnancy characteristics. GDM does not increase the risk of stillbirth, hypoxic ischaemic encephalopathy or neonatal death.

FRI665 Hirata's Syndrome: Autoimmune Hypoglycemia Diagnostic Pathway, A Mexican Case Report

Abstract Disclosure: G. Gonzalez De La Cruz: None. D. Porras Farret: None. Introduction: Autoimmune hypoglycemia or Hirata syndrome is a very rare cause of hypoglycemia in western populations, as an example, in Mexico until 2015, there was only a single case reported with these characteristics. Diagnosis is based on episodes of spontaneous, fasting or postprandial hypoglycemia, with endogenous hyperinsulinism, without pancreatic lesions on imaging and positive tests. Objective: To describe the approach to a patient with hypoglycemia, specifically to those with characteristics of autoimmune hypoglycemia syndrome, as well as the general approach to hypoglycemia of endogenous origin and the differential approach in those with a history of type 2 diabetes or drug use with secondary effects. Case description: An 86-year-old woman with a history of high blood pressure, prediabetes, and osteoporosis, presented episodes of dizziness, palpitations, diaphoresis, and syncope, documenting plasma glucose of 40 mg/dl. She was attended by paramedics responding adequately to oral glucose. She was evaluated by Cardiology, Holter was performed with predominant sinus rhythm, frequent atrial extrasystoles (2%) and infrequent ventricular extrasystoles. Next, she was referred to Endocrinology for evaluation and treatment, she was taken empagliflozin as prediabetes medication, and it was suspender. Laboratory studies are requested to address hypoglycemia, with serum insulin levels greater than 600 mU/l, c-peptide 3.9 ng/ml, TSH 6.1, T4T 7.5, T3T 67.8, T4L 1.1, ALT 26 U/L, AST 18 U/L, DHL 247 U/L, GGT 26 U/L, Cortisol 13.8 mcg/dl. An MRI of the upper abdomen was performed where there is no evidence of pancreatic injury. A profile of serum hypoglycemic agents (sulfonylureas in blood) was requested, which were not detected. Subsequently, anti-insulin studies were requested, which were reported in amounts greater than 100 U/ml. The diagnosis of autoimmune hypoglycemia syndrome was made, starting management with prednisone 70 mg every 24 hours, in addition to dietary changes. Conclusion: The approach to hypoglycemia in patients without clear evidence of exogenous administration of hypoglycemic agents must be ordered, taking into account differential diagnoses such as autoimmune causes. In the case of autoimmune hypoglycemia, remission is expected at 6 months, with constant monitoring and adjustment of drugs for underlying pathologies. Presentation: Friday, June 16, 2023

SAT661 Bypass-oma Or Medication-oma: A Clinical Dilemma About Hypoglycemia

Abstract Disclosure: C.A. Perez Hernandez: None. M.J. Pesantez Borja: None. Introduction With the ongoing worldwide obesity epidemic, the number of bariatric procedures rises every year. Although complications have decreased in the last 20 years, they can result in significant morbidity and mortality. Hypoglycemia is a complication that usually occurs 1-3 years after a Roux-en-Y gastric bypass, and is caused by increased postprandial incretin secretion. Although reported incidence is 0.1%, it is likely underdiagnosed and underestimated cause of hyper insulinemic hypoglycemia. Clinical Case A 33-year-old male with history of pancreatic cancer status post partial pancreatectomy and Roux-en-Y gastric bypass performed 10 years ago, and type 2 diabetes on insulin was admitted for severe persistent hypoglycemia refractory to treatment. He stopped using insulin 6 months ago after passing out while driving 1-2 hours postprandially. Only 2 hours after starting a 72-hour fasting test, the patient had a venous blood glucose of 39 mg/dl. Laboratory results showed: C-peptide:3.3, Insulin:9.3, cortisol:2.6, BHB: 0.5 and proinsulin:3.7; and negative sulfonylurea screening. Dotatate scan was unremarkable. He has started diazoxide, acarbose, octreotide and Everolimus with no improvement to his symptoms. Plan was to repeat the work up, however, the patient refused to cooperate. He also declined a Calcium stimulation test, endoscopic US and bariatric surgery reversal. Review of his imaging studies which, showed no signs of pancreatic surgery. When re-interrogated, medical history inconsistencies were noticed. Hypoglycemia episodes occurred exclusively after being transferred out of ICU, patient was placed in a one-to-one monitoring. During the night shift, he was found injecting himself an unknown medication via PICC line and several medications were found in his room. After being confronted by staff, the Patient left the AMA only to be readmitted the same day for hypoglycemia at another institution. Conclusion Our patient was diagnosed with Munchausen's syndrome, substance abuse and depressive disorder upon evaluation by psychiatry. It was found that 4 years prior to current admission, he completed a 72-hour fasting test in another hospital, without hypoglycemia episodes. However, due to concerns for insulinoma, his oncology team was contacted who reported never having seen the patient or diagnosing with pancreatic cancer. Also, patient was found self-injecting with an unknown medication, and when confronted, he left AMA just like he did while he was under our care. History of self-harm behavior and inconsistencies in medical history may call for atypical presentations of Post Bariatric Surgery hypoglycemia and should prompt a detailed anamnesis and psychiatric assessment. This case represents a clinical dilemma and illustrates the therapeutic challenges encountered with a patient diagnosed with post bariatric surgery hypoglycemia and Munchausen syndrome. Presentation: Saturday, June 17, 2023

SAT139 Non-insulinoma Pancreatogenous Hypoglycemia Syndrome In A Patient With 1p36 Deletion Syndrome

Abstract Disclosure: E.D. Auckley: None. M.A. Arosemena: None. L. Philipson: None. Background: 1p36 deletion syndrome comprises a phenotypic presentation that includes central nervous system, cardiac, and craniofacial anomalies. There has been no report of associated hypoglycemia with this syndrome. Nesidioblastosis, also known as non-insulinoma pancreatogenous hypoglycemia syndrome (NIPHS), is understood as a rare condition defined by diffuse pancreatic hypertrophy with subsequent hyperinsulinemic hypoglycemia. Diagnosis requires serologic workup consistent with endogenous hyperinsulinemic hypoglycemia, intraarterial calcium stimulation positive for inappropriately elevated insulin secretion, and imaging studies without identified masses. Clinical Case: A 21-year-old female with 1p36 deletion syndrome (seizure disorder, cerebral palsy, vision loss and heart failure) was admitted to the hospital due to persistent falls. On admission vital signs were within normal limits, and physical exam revealed a thin non-verbal patient without significant other findings. During hospitalization and prolonged fasting periods of approximately 14-18 hours related to poor appetite she developed several episodes of hypoglycemia with serum glucose as low as 57 mg/dl. As patient was non-verbal at baseline, symptoms of hypoglycemia such as sweating, lightheadedness or shakiness were not confirmed. Even when she started to eat, hypoglycemic events continued to occur. Dextrose containing fluids (Dextrose 5% and later 10%) were added to try to maintain blood sugars within the normal range. Nevertheless, she continued to develop hypoglycemia. Endocrinology service was consulted and recommended to obtain a critical sample. A critical sample revealed serum glucose of 59 mg/dl, insulin level of 20.6 mcU/mL, proinsulin of 33 pmol/L, and beta-hydroxybutyrate of <0.10 mmol/L. Insulin antibodies was negative. Adrenal insufficiency was unlikely due to a normal 9 am cortisol level of 13.7 ug/dl. Due to the presence of hyperinsulinemic hypoglycemia further workup was conducted. A CT of the abdomen with a pancreatic protocol and 68Ga-DOTATATE PET/CT scan was negative for a pancreatic mass. Endoscopic ultrasound did not reveal a clear pancreatic mass either. This was followed by a calcium stimulation test which showed a two-fold increase in insulin levels in all 3 catheterized pancreatic territories. In the absence of a discrete mass, non-surgical management was recommended. For hypoglycemia management patient was started on octreotide injections with resolution of hypoglycemia. Prior to discharge she was switched to lanreotide and was discharged to continue monthly lanreotide injections. Conclusion: To our knowledge, this is the first case reported of NIPHS in a patient with a mutation in 1p36 deletion. Hyperinsulinism in the setting of diabetes has been reported in this syndrome1 but to our knowledge no cases of hypoglycemia with NIPHS have been reported. Presentation: Saturday, June 17, 2023

Anaesthesia management of a patient with non-insulinoma pancreatogenous hypoglycaemia syndrome (NIPHS) - A case study.

Anaesthesia management of a patient with non-insulinoma pancreatogenous hypoglycaemia syndrome (NIPHS) - A case study.