Nationwide survey of endogenous hyperinsulinemic hypoglycemia in Japan (2017-2018): Congenital hyperinsulinism, insulinoma, non-insulinoma pancreatogenous hypoglycemia syndrome and insulin autoimmune syndrome (Hirata's disease).

AIMS/INTRODUCTION: We aimed to investigate the nationwide incidence, treatment details and outcomes of patients with endogenous hyperinsulinemic hypoglycemia (EHH), including those with transient/persistent congenital hyperinsulinism (CHI), insulinoma, non-insulinoma pancreatogenous hypoglycemia syndrome and insulin autoimmune syndrome (Hirata's disease) in Japan. MATERIALS AND METHODS: A nationwide, questionnaire-based survey was carried out to determine the number of patients with EHH who were treated for hypoglycemia or hypoglycemia-related complications in 2017-2018. The questionnaires were sent to all hospitals in Japan with >300 beds, and with pediatric and/or adult clinics likely managing EHH patients. The secondary questionnaires were sent to obtain the patients' date of birth, sex, age at onset, treatment details and post-treatment outcomes. RESULTS: A total of 447 patients with CHI (197 transient CHI, 225 persistent CHI and 25, unknown histology), 205 with insulinoma (118 benign, 18 malignant and 69 unknown subtype), 111 with non-insulinoma pancreatogenous hypoglycemia syndrome (33 post-gastric surgery HH, 57 postprandial HH, 10 nesidioblastosis and 11 unknown subtype) and 22 with insulin autoimmune syndrome were identified. Novel findings included: (i) marked improvement in the prognosis of persistent CHI over the past 10 years; (ii) male dominance in the incidence of transient CHI; (iii) non-insulinoma pancreatogenous hypoglycemia syndrome emerging as the second most common form of EHH in adults; (iv) frequent association of diabetes mellitus with insulin autoimmune syndrome; and (v) frequent post-treatment residual hypoglycemia and impaired quality of life. CONCLUSIONS: The first nationwide, all age group survey of EHH showed the current status of each type of EHH disorder and the unmet needs of the patients.

Hyperinsulinemic Hypoglycemia after Bariatric Surgery: Diagnosis and Management Experience from a Spanish Multicenter Registry.

BACKGROUND: Severe postprandial hypoglycemia after bariatric surgery is a rare but invalidating complication. Our aim was to describe the different tests performed for its diagnosis and their outcomes as well as the response to the prescribed pharmacological and surgical treatments. METHODS: Multicenter, retrospective systematic review of cases with recurrent severe postprandial hypoglycemia. RESULTS: Over 11 years of follow-up, 22 patients were identified. The test most used to provoke hypoglycemia was the oral glucose load test followed by the mixed meal test which was the least standardized test. With pharmacological treatment, 3 patients were symptom-free (with octreotide) and in 12 patients hypoglycemic episodes were attenuated. Seven patients had persistent hypoglycemic episodes and underwent surgery. Partial pancreatectomy was performed in 3 patients who had positive selective arterial calcium stimulation, and nesidioblastosis was confirmed in 2 patients. Reconversion to normal anatomy was performed in 3 patients, and 1 patient underwent a resection of the 'candy cane' roux limb, with resolution of hypoglycemia in all cases. CONCLUSIONS: There is high heterogeneity in the evaluation and treatment options for postoperative hypoglycemia. In patients that do not respond to pharmacological treatment, reconstruction of gastrojejunal continuity may be the safest and most successful procedure.

Postprandial hyperinsulinemic hypoglycemia after gastric bypass surgical treatment.

An association between post-Roux-en-Y gastric bypass (RYGB) hypoglycemia and nesidioblastosis was reported in 2005 and may cause serious neuroglycopenic symptoms. Most patients with postprandial hypoglycemia after RYGB respond to nutritional and medical treatment. A subset of patients, however, may not respond adequately and surgery may be considered. This review describes the current experience with surgical intervention for severe post-RYGB hypoglycemia. PubMed and MEDLINE searches were made for reports describing clinical outcome after such surgery. Fourteen papers including 75 patients were identified. Different surgical interventions were applied including gastric tube placement, reversal of the bypass with and without concomitant sleeve resection, gastric pouch restriction, and pancreatic resection and reresection. Pancreatic resection was performed in 51 (68%) patients, 17 (23%) had RYGB reversal and eleven (15%) had gastric pouch restriction alone. Eight (11%) patients received 2 or more consecutive procedures for hypoglycemia and combined interventions were made in several patients. Resolution of the symptoms occurred in 34/51 (67%) patients after pancreatic resection, 13/17 (76%) after reversal, and 9/11 (82%) after pouch restriction. Mean follow up, however, was short for most series and the methods applied for evaluation of hypoglycemia varied. Weight regain, diabetes and recurrent symptoms were late complications. The optimal therapy for hypoglycemia after RYGB is not defined. Long-term evaluations and knowledge about the physiology of post-RYGB hypoglycemia, may enable therapy with improved control of the glucose excursions.