Hypoglycemia due to an adult-onset nesidioblastosis, a diagnostic and management dilemma

“[4] He described nesidioblastosis as neoformation of Langerhans islets from the pancreatic ductal epithelium, and it is now known to be the primary cause of persistent hyperinsulinemic hypoglycemia in infants.[5] In Adults, hyperinsulinemic hypoglycemia is mainly caused by insulinomas, other causes are rare and usuall…”

“Histopathology descriptions of excised pancreatic tissue include islet cell hypertrophy with enlarged and hyperchromatic nuclei, increased periductular islets ,and β-cells budding off ductular epithelium.[12,13]”

“[2,8] Moreover, radiological localization studies, such as CT scan, endoscopic ultrasound, octreotide scan, and selective arterial calcium stimulation test (SACST) with hepatic venous sampling, can be performed to distinguish between a focal abnormality (insulinoma) and a diffuse process (nesidioblastosis).”

“Histopathology descriptions of excised pancreatic tissue include islet cell hypertrophy with enlarged and hyperchromatic nuclei, increased periductular islets ,and β-cells budding off ductular epithelium.[12,13]”

“[1] The majority of hyperinsulinemic hypoglycemia cases are caused by insulinoma and only less than 5% are caused by noninsulinoma pancreatogenous hypoglycaemia syndrome (NIPHS) or nesidioblastosis.[2,3] Here we report a case of uncontrolled symptomatic hypoglycemia due to nesidioblastosis in a patient with history of…”

“[10] On the other hand, recurrent hypoglycemia has also been reported that may require total pancreatectomy.[11]”

“Although there is limited data regarding the efficacy of pancreatectomy for nesidioblastosis, successful resolution of hypoglycemia has been reported after partial or subtotal pancreatectomy.[10] On the other hand, recurrent hypoglycemia has also been reported that may require total pancreatectomy.”

“[2,8] Moreover, radiological localization studies, such as CT scan, endoscopic ultrasound, octreotide scan, and selective arterial calcium stimulation test (SACST) with hepatic venous sampling, can be performed to distinguish between a focal abnormality (insulinoma) and a diffuse process (nesidioblastosis).”

“similar, and imaging studies can be equivocal.[1,9] In patient with nesidioblastosis symptoms occur mainly postprandially and only rarely while fasting.”

“[5] In Adults, hyperinsulinemic hypoglycemia is mainly caused by insulinomas, other causes are rare and usually due to adult-onset nesidioblastosis, which was first described in 1975.[6] Since then fewer than 100 cases have been reported but it seems to be increasing in frequency.”

“[6] Since then fewer than 100 cases have been reported but it seems to be increasing in frequency.[7]”

“(123) Case report N = 1 Distal pancreatectomy (80%) • Full remission initially after surgery but hypoglycemia”

“hepatic vein to test for insulin levels [37,84].”

“Subsequent total pancreatectomy Qintar et al., 2012 [15] 1 Distal (80”

“[17,18] In a retrospective analysis of 32 EHH, [19] five cases of nesidioblastosis were reported, each of which underwent 70% of distal pancreatectomy (removing all pancreatic tissue to the left of the superior mesenteric vessels).”

“With the increasing population undergoing RYGB surgery, the reported cases of RYGB-associated nesidioblastosis also seem to be increasing in frequency [2].”

“The only comparative pancreatic abnormality associated with profound islet cell hyperplasia is the extremely rare adult type of hyperinsulinemic hypoglycemia [6,7], the morphological patters of which is entirely different.”

“Reports in the literature recommend subcutaneous administration of 100-600 µg of octreotide per day [16,17].”