Nesidioblastosis is the histological equivalent of noninsulinoma pancreatogenous hypoglycemia syndrome and refers to an increase in the size and number of. Nesidioblastosis seen in newborns is now called persistent hyperinsulinemic hypoglycemia of infancy phhi while the condition in adults is called noninsulinoma pancreatogenous hypoglycemia syndrome niphs as a separate entity. Pancreatogenous hypoglycemic syndrome insulinoma or noninsulinoma origin niphs, hypoglycemia causes and occurrences, everlon cid rigobelo, intechopen, doi. An update in 10 surgically treated patients, surgery on deepdyve, the largest online rental service for scholarly research with thousands of academic publications available at your fingertips. Noninsulinoma pancreatogenous hypoglycemic syndrome niphs is a rare disorder among adults and, to our knowledge, only about 40 cases have been reported in the literature 1, 3, 4, 5. Hypoglycemia due to primary adult onset nesidioblastosis. Physiological levels of glucose in fasting state are 70110mgdl glycogenolysis helps brain in fasting state 8hr gluconeogenesis plays its role after glycogen is depleted.
Help us write another book on this subject and reach those readers. Noninsulinoma pancreatogenous hypoglycemia syndrome niphs. Congenital hyperinsulinism, insulinoma, noninsulinoma pancreatogenous hypoglycemia syndrome and insulin. T1 outcomes and quality of life after partial pancreatectomy for noninsulinoma pancreatogenous hypoglycemia from diffuse islet cell disease au vanderveen, kimberly a. Clinical features of niphs are similar to those of insulinoma. In nondiabetic adult patients, hypoglycaemia may be related to drugs, critical illness, cortisol or glucagon insufficiency, nonislet cell tumour, insulinoma, or it may be surreptitious. Noninsulinoma pancreatogenous hypoglycemia syndrome. We describe our surgical experience for the management of this rare condition. The possibility of noninsulinoma pancreatogenous hypoglycemia syndrome is considered. In the hereditary form of hyperinsulinemic hypoglycemia of in. Nationwide survey of endogenous hyperinsulinemic hypoglycemia in japan 20172018.
The topic noninsulinoma pancreatogenous hypoglycemia syndrome niphs you are seeking is a synonym, or alternative name, or is closely related to the. This definition appears somewhat frequently and is found in the following acronym finder categories. Noninsulinoma pancreatogenous hypoglycaemia in adults a. Noninsulinoma pancreatogenous hypoglycemic syndrome niphs is a rare disorder among adults and, to our knowledge, only about 40 cases have been reported in the literature 1, 35.
Normative values for symptomatic or asymptomatic postbariatric patients are not. It is impossible to clinically differentiate insulinomas from niphs. Postprandial hypoglycemia alimentary noninsulinoma pancreatogenous hypoglycemia syndrome functional occult diabetes mellitus. Noninsulinoma pancreatogenous hypoglycemia syndrome diagnosis of insulinoma inappropriately high serum insulin level during a spontaneous or induced episode of hypoglycemia 72 hour fast serum glucose concentration 3 serum cpeptide 200 serum. Management of refractory noninsulinoma pancreatogenous. This paper presents a novel case as an example of how such patients may present and how they may be investigated. This increased level is caused by hyperplasia and dysplasia of beta cells of pancreas. Niph stands for noninsulinoma, pancreatogenous hypoglycemia. Non insulinoma pancreatogenous hypoglycemia syndrome was first described by service et al.
The term is not necessarily a diagnosis since it requires an evaluation to determine the cause of the hypoglycemia. Post gastric bypass hyperinsulinemic hypoglycemia defines a group of patients with postprandial neuroglycopenic symptoms similar to insulinoma but in many cases more severe. Noninsulinoma pancreatogenous hypoglycemia syndrome article pdf available in the journal of the association of physicians of india 594. In adults, nesidioblastosis also termed noninsulinoma pancreatogenous hypoglycemia syndrome, niphs, is a rare cause of hypoglycemia in adults who have not undergone gastrointestinal surgery. Hypoglycaemia related to inherited metabolic diseases in.
We report our experience with gradientguided partial pancreatectomy in a newly recognized clinical hypoglycemic disorder, noninsulinoma pancreatogenous hypoglycemia syndrome niphs. Hyperinsulinemic hypoglycemia due to adult nesidioblastosis in insulindependent diabetes. Hyperinsulinemic hypoglycemia is relatively recently recognized in persons undergoing bariatric surgery although knowledge and experience with this condition may not be commensurate with the number of such procedures being performed globally. Pancreatogenous hypoglycemic syndrome insulinoma or non ins ulinoma origin niphs 175 sulfonylureas and glinides, if available, to differentiate the factitious hypoglycemia. The noninsulinoma pancreatogenous hypoglycemia syndrome niphs identifies a group of hyperinsulinemic hypoglycemic patients with unique clinical, diagnostic, surgical, and pathologic features. Journal of clinical endocrinology and metabolism, 84 5, 15821589. Noninsulinoma pancreatogenous hypoglycemic syndrome a. Hyperinsulinaemic hypoglycaemia hh is also classically referred to as nesidioblastosis. There are few reports of patients with this condition. Hyperinsulinemic hypoglycemia describes the condition and effects of low blood glucose caused by excessive insulin. Adultonset noninsulinoma pancreatogenous hypoglycemia syndrome niphs associated with hyperinsulinemic hypoglycemia is a very rare entity.
Noninsulinoma pancreatogenous hypoglycemia syndrome as a. Rouxeny gastric bypass rygb is a commonly performed, effective bariatric procedure. Diffuse nesidioblastosis with hypoglycemia mimicking an. Nevertheless, some hypoglycaemic episodes remain unexplained, and inborn errors of metabolism iem should be considered, particularly in cases of multisystemic involvement. Case report management of refractory noninsulinoma pancreatogenous hypoglycemia syndrome with gastric bypass reversal. A rare case of noninsulinoma pancreatogenous hypoglycemia. Read noninsulinoma pancreatogenous hypoglycemia syndrome. Uptodate, electronic clinical resource tool for physicians and patients that provides information on adult primary care and internal medicine, allergy and immunology, cardiovascular medicine, emergency medicine, endocrinology and diabetes, family medicine, gastroenterology and hepatology, hematology, infectious diseases, nephrology and hypertension, neurology, obstetrics, gynecology, and women. For full access to this pdf, sign in to an existing account, or purchase an annual subscription. We report a case that was successfully treated with rygb reversal. Persistent islet hyperplasia noninsulinoma pancreatogenous hypoglycemia syndrome jul 10, 2007 viewed. Hypoglycaemia following upper gastrointestinal surgery.
Pdf noninsulinoma pancreatogenous hypoglycemia syndrome. Management of post gastric bypass noninsulinoma pancreatogenous hypoglycemia nesidioblastosis introduction. Outcomes and quality of life after partial pancreatectomy for noninsulinoma pancreatogenous hypoglycemia from diffuse islet cell disease. Noninsulinoma pancreatogenous hypoglycemia in adults. Successful medical treatment of adult nesidioblastosis. We present a patient with longstanding symptoms of postprandial hypoglycemia occurring within 15 minutes of meals in the absence of fasting hypoglycemic symptoms. Adrenal insufficiency is excluded based on the normal acth and cortisol levels. This case report adds new information, as we performed an electrophysiologic characterization of isolated islet cells. In infants and children, it can lead to serious and permanent damage to the central nervous system, which leads to the manifesting mental retardation. Insulinoma is the most common cause of persistent hyperinsulinemic hypoglycemia. These patients experience predominantly postprandial hypoglycemia and have nesidioblastosis with islet cell hypertrophy, findings different from those in patients with insulinomas. Hypoglycemia with neuroglycopenic symptoms presents the main diagnostic condition.
The hypoglycemic states current medical diagnosis and. Hypothesis nesidioblastosis is an important cause of adult hyperinsulinemic hypoglycemia, and control of this disorder can often be obtained with a 70% distal pancreatectomy. Pancreatogenous hypoglycemic syndrome insulinoma or non. In certain instances, patients who have undergone the procedure experience postprandial symptoms of neuroglycopenia caused by noninsulinoma pancreatogenous hypoglycemia syndrome niphs.
This is a rare cause of hypoglycemia in adults and is presently designated as noninsulinoma pancreatogenous hypoglycemia syndrome niphs. Heterogeneous clinical manifestation of the disease causes risk of late diagnosis or even misdiagnosis. Nesidioblastosis is a condition with diffuse hyperplasia of the pancreatic islets, leading to hyperinsulinemic hypoglycemia. Niphs is a recently described syndrome and is also very rare, and therapeutic approaches are still under debate. Hypoglycemia due to excess insulin is the most common type of serious hypoglycemia. Postgastric bypass noninsulinoma hyperinsulinemic pancreatogenous hypoglycemia defines a group of patients with postprandial neuroglycopenic symptoms similar to insulinoma but in many cases more severe. Immunopathologic hypoglycemia idiopathic antiinsulin antibodies which release their bound insulin antibodies to insulin receptors which act as agonists druginduced hypoglycemia.
Diffuse nesidioblastosis as a cause of hyperinsulinemic hypoglycemia in adults. Outcomes and quality of life after partial pancreatectomy. It is the most important differential diagnosis to insulinoma in the adult, but only 0. Counterregulatory mechanism starts when glucose level goes just below the physiological range. Insulin responses during selective arterial calciumstimulation testing, often performed with arteriography, have been used in the past to exclude insulinoma and to diagnose noninsulinoma pancreatogenous hypoglycemia syndrome in nonbariatric individuals 16, 17. Organic hyperinsulinism causing hypoglycemia in adults is caused by insulinoma, islet hyperplasia, or a combination of adenomata and hyperplasia. The typical patient develops hypoglycemia hours after ingestion of a carbohydratecontaining meal and hypoglycemia is absent in the fasting state. Noninsulinoma pancreatogenous hypoglycemia syndrome niphs nesidioblastosis is a pancreatic condition caused by increased levels of insulinlike growth factor ii a vital growth protein, due to abnormal function of the pancreas.
Other causes of postprandial hypoglycemia include insulinoma and noninsulinoma pancreatogenous hypoglycemia syndrome niphs. Read management of postgastric bypass noninsulinoma pancreatogenous hypoglycemia, surgical endoscopy on deepdyve, the largest online rental service for scholarly research with thousands of academic publications available at your fingertips. Although both diagnoses are rare, they should be considered if no improvement in hypoglycemic symptoms occurs after dietary modification. Reactive hypoglycemia, postprandial hypoglycemia, or sugar crash is a term describing recurrent episodes of symptomatic hypoglycemia occurring within four hours after a high carbohydrate meal in people with and without diabetes. A novel syndrome of hyperinsulinemic hypoglycemia in adults independent of mutations in kir6. Whereas nesidioblastosis is the primary cause of persistent hyperinsulinemic hypoglycemia in infants, noninsulinoma pancreatogenous hypoglycemia syndrome niphs is very infrequent in adults.
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