Congenital hyperinsulinism
Demetrius Pertsemlidis, William B. Inabnet III, Michel Gagner in Endocrine Surgery, 2017
The underlying pathological condition in a newborn whose hyperinsulinism persists is almost never a true insulinoma. Controversy has surrounded the actual underlying histopathology. The term nesidioblastosis, applied broadly to the congenitally abnormal pancreas producing excessive insulin and thought to be a diffuse abnormality in the pancreas, was introduced in 1939, curiously, in a paper actually about focal pancreatic lesions [5]. The author, Laidlaw, called the underlying causative lesions nesidioblastomas. Laidlaw speculated that if such lesions were multiple in a pancreas, the condition could be called nesidioblastosis. Laidlaw recognized islet cells budding from pancreatic ductal cells. The term nesidioblastosis, once thought to be the cause of CH, is no longer used because this histological finding described by Laidlaw has not consistently been associated with hyperinsulinism. Actually, the finding of beta cells budding from epithelium (ductuloinsular complexes) is characteristic of the fetal developmental process. It persists as a developmental stage postnatally [6]. It is reproduced in many states of injury, including pancreatic duct obstruction, and in recovery from these conditions. This histological condition has been recognized in the normal pancreas of newborns and infants. In the view of many pathologists, the finding of nesidioblastosis does not correlate with hypoglycemia [7–9].
The Metabolic Medicine Postoperative Bariatric Surgery Consultation
Michael M. Rothkopf, Jennifer C. Johnson in Optimizing Metabolic Status for the Hospitalized Patient, 2023
On the other hand, hypoglycemia can occur as part of the dumping syndrome. Further, a more severe form of hypoglycemia, with neuroglycopenic symptoms, has developed in some diabetic and pre-diabetic postbariatric patients. It occurs with the combination of excess insulin production in a patient who is becoming less insulin resistant (through weight loss). This syndrome has been labeled nesidioblastosis by Service et al. (2005). It can cause life-threatening hypoglycemic episodes, which require medical management or even partial pancreatectomy to resolve.
The adrenal glands and other abdominal endocrine disorders
Professor Sir Norman Williams, Professor P. Ronan O’Connell, Professor Andrew W. McCaskie in Bailey & Love's Short Practice of Surgery, 2018
The differential diagnosis of hypoglycaemia includes hormonal deficiencies, hepatic insufficiency, medication, drugs and enzyme defects. Occasionally, differentiating insulinoma from other causes of hypoglycaemia can be difficult. Nesidioblastosis is a rare disorder, mainly encountered in children, which is characterised by replacement of normal pancreatic islets by diffuse hyperplasia of islet cells.
Evidence of unrestrained beta-cell proliferation and neogenesis in a patient with hyperinsulinemic hypoglycemia after gastric bypass surgery
Published in Islets, 2018
Nidheesh Dadheech, Dominique Garrel, Jean Buteau
We herein describe a patient who developed HIHG after gastric bypass. Pancreatic sections presented both unrestrained beta-cell proliferation and histological evidence of beta-cell neogenesis from ductal progenitor cells, compared to tissue samples from weight-matched donors. This latter conclusion is born out by our detection of i) small cluster of insulin-positive cells abutting ducts, ii) bi-hormonal-positive cells in the duct epithelium and iii) the expression of nestin, an early endocrine progenitor marker expressed during pancreatic morphogenesis but generally absent in the adult pancreas.20 These findings are consistent with the histopathologic definition of “nesidioblastosis”.21 The term was first coined to describe persistent hyperinsulinemic hypoglycemia of infancy (PHHI), a pathology also characterized by abnormal islet mass expansion and islet neogenesis,22 but was more recently used to encompass HIHG after gastric bypass as well. A limitation to our study is that it is limited to a single patient but we expect that further advances will be made possible as more tissues become available from the increasing number of persons with extreme obesity undergoing bariatric surgical procedures.
Diazoxide during pregnancy and lactation: drug levels in maternal serum, cord blood, breast milk, and infant serum: a case report
Published in Gynecological Endocrinology, 2022
Jumpei Saito, Hiroyo Kawasaki, Natsuho Adachi, Aiko Sasaki, Naho Yakuwa, Tomo Suzuki, Haruhiko Sago, Akimasa Yamatani, Reiko Horikawa, Atsuko Murashima
Nesidioblastosis is a common cause of congenital hyperinsulinemic hypoglycemia [1]. It is defined as non-neoplastic proliferation of both islet and ductular cells and formation of ductuloinsular complexes, which are characterized by islet cell hyperplasia arising from proliferating ductal epithelium [2]. Women with severe hypoglycemia are at increased risk for postpartum hemorrhage and infection, as well as hemolysis, elevated liver enzymes, and low platelet (HELLP) syndrome [3]. Thus, close medical monitoring should be required for pregnant women with hypoglycemia. Maternal hypoglycemia during pregnancy is associated with intrauterine growth retardation and pre-eclampsia [4,5].
Related Knowledge Centers
- Beta Cell
- Congenital Hyperinsulinism
- Diabetes
- Microscope
- Pancreas
- Hyperplasia
- Pancreatic Duct
- Hyperinsulinemic Hypoglycemia
- Pancreatic Islets
- Bariatrics