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Nesidioblastosis

Islet Cell Hyperplasia


Presentation

  • We present the clinical, laboratory, radiological and pathological findings in a 34 yr-old-man with recurrent hypoglycemia. The pertinent literature is reviewed. A 34- yr-old man presented in 1992 with symptoms of recurrent hypoglycemia.[ncbi.nlm.nih.gov]
  • Persistent hyperinsulinaemic hypoglycaemia (nesidioblastosis) presenting with hypoglycaemia is extremely rare in adults. The features are suggestive of an insulinoma with a vague presentation and delayed diagnosis.[ncbi.nlm.nih.gov]
  • We present two cases in which near total pancreatectomy was performed with favourable outcome. Both patients are normoglycemic, with one requiring pancreatic enzyme supplements.[ncbi.nlm.nih.gov]
  • We discuss the anaesthetic management of a patient with nesidioblastosis who presented for near total pancreatectomy and review the literature pertinent to the anaesthesiologist.[ncbi.nlm.nih.gov]
  • Postprandial symptoms of hypoglycemia are the clinical presentation of the disease. A female patient with diabetes mellitus who underwent a Roux-en-Y gastric bypass began to present postprandial symptoms of hypoglycemia.[ncbi.nlm.nih.gov]
Fatigue
  • We ask about general symptoms (anxious mood, depressed mood, fatigue, pain, and stress) regardless of condition. Last updated: May 13, 2019[patientslikeme.com]
  • We ask about general symptoms (anxious mood, depressed mood, fatigue, pain, and stress) regardless of condition. Last updated: January 31, 2019[patientslikeme.com]
  • Causes symptoms of fatigue, dizziness, increased heart rate, and other symptoms. See syncope. We thank you for using the Health Dictionary to search for Nesidioblastosis .[healthdictionary.info]
  • Fatigue can occur which can also be accompanied by headaches. Increasing hunger pangs and thirst with increased urination are some of the common symptoms of Nesidioblastosis of pancreas.[epainassist.com]
  • Nesidioblastosis may include the following: Symptoms of low glucose level in blood, such as sweating, dizziness/confusion, blurring of vision, and even loss of consciousness Nausea and vomiting Abdominal pain, back pain Loss of appetite, weight loss Fatigue[dovemed.com]
Sudden Infant Death Syndrome
  • Pathologists have found abnormal formation and placement of beta cells in people with cystic fibrosis, Zollinger-Ellison syndrome or sudden infant death syndrome and even in healthy infants.[wisegeek.com]
Aspiration
  • An octreotide scan revealed increased hepatic uptake and fine needle aspiration of a liver lesion confirmed metastatic insulinoma.[ncbi.nlm.nih.gov]
  • A fine‐needle aspiration puncture was executed and no hyperplasia or dysplasia was found.[shmabstracts.com]
  • Tomography Scan (PET) Percutaneous transhepatic cholangiography (PTC) Endoscopic retrograde cholangio-pancreatography (ERCP) Invasive diagnostic procedures include laparoscopy, laparotomy, and tissue biopsy Tissue diagnosis can be done through Fine needle aspiration[epainassist.com]
  • […] putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis The tissue for diagnosis can be procured in multiple different ways, and they include: Fine needle aspiration[dovemed.com]
Pleural Effusion
  • Here, we report a case of a 12-year-old male who presented with pleural effusion and was revealed to have a large anterior mediastinal mass. Biopsy of the mass revealed benign mature teratoma.[ncbi.nlm.nih.gov]
Melena
  • We describe a case of a young, white male who presented with complaints of hematemesis and melena 18 years following subtotal pancreatectomy for nesidioblastosis.[ncbi.nlm.nih.gov]
Hematemesis
  • We describe a case of a young, white male who presented with complaints of hematemesis and melena 18 years following subtotal pancreatectomy for nesidioblastosis.[ncbi.nlm.nih.gov]
Thrombosis
  • Splenic vein thrombosis resulting in gastric variceal hemorrhage has not been a previously reported complication from 95% pancreatectomy for this indication. A case is presented and the management of this unusual complication discussed.[ncbi.nlm.nih.gov]
Suggestibility
  • This case and other evidence from the literature suggest that hyperplasia and neoplasia may occur sequentially in the pancreas, and that endocrine and exocrine tumorigenesis may be linked in some individuals.[ncbi.nlm.nih.gov]
  • The features are suggestive of an insulinoma with a vague presentation and delayed diagnosis. We describe a report of adult nesidioblastosis in association with a pancreatic endocrine microadenoma.[ncbi.nlm.nih.gov]
  • Ectopic nesidioblastosis has not been previously reported, but our experience suggests the necessity of examination for ectopic pancreas in cases of SIDS or its near-miss.[ncbi.nlm.nih.gov]
  • We think that this is strongly suggestive of autosomal recessive inheritance. Seven other reports of presumed autosomal recessive hyperinsulinism are reviewed. To our knowledge, we report the first case in sibs whose parents were consanguineous.[ncbi.nlm.nih.gov]
  • We describe a case of familial nesidioblastosis in which an intrauterine diagnosis could be suggested by high levels of insulin and C-peptide and low values of glucose in the amniotic fluid.[ncbi.nlm.nih.gov]
Drop Attacks
  • One patient underwent an approximately 80% pancreatectomy with good results but subsequently experienced recurrent drop attacks and fainting from hyperinsulinism; a completion pancreatectomy via a pancreaticoduodenectomy was then required.[ncbi.nlm.nih.gov]
Dysarthria
  • In 1990, a 42-year-old woman had 1½ years of diaphoresis, shaking, palpitations, confusion, dysphagia, dysarthria, diplopia, lightheadedness, and a 9.07-kg weight gain.[jamanetwork.com]

Workup

  • Both cases underwent partial pancrectomy and splenectomy after workup. The histology of the first case was consistent with insulinoma, while the second case was that of nesidioblastosis.[ncbi.nlm.nih.gov]
  • On workup, she was found to be hypoglycemic, with serum glucose values between 32 and 50 mg/dL. Magnetic resonance imaging revealed a nodule arising from the corpus of the pancreas, which was slightly enhanced, measuring 1.0 cm in diameter.[enets.org]
  • The preoperative workup included triple-phase contrast islet cell protocol CT scan of abdomen, endoscopic ultrasound of pancreas, 72-hr fast, and calcium stimulated selective arteriography.[sages.org]
  • A 72-hour fast was performed, but the blood glucose level never fell below 2.8 mmol/L (50 mg/dL), and no further workup was attempted at that time.[jamanetwork.com]
Mediastinal Mass
  • Here, we report a case of a 12-year-old male who presented with pleural effusion and was revealed to have a large anterior mediastinal mass. Biopsy of the mass revealed benign mature teratoma.[ncbi.nlm.nih.gov]
Gastric Varices
  • Splenic vein thrombosis resulting in gastric variceal hemorrhage has not been a previously reported complication from 95% pancreatectomy for this indication. A case is presented and the management of this unusual complication discussed.[ncbi.nlm.nih.gov]
Anterior Mediastinal Mass
  • Here, we report a case of a 12-year-old male who presented with pleural effusion and was revealed to have a large anterior mediastinal mass. Biopsy of the mass revealed benign mature teratoma.[ncbi.nlm.nih.gov]
Ketonuria
  • […] test in adults and in neonates by determining the glucose requirements to maintain normoglycemia, inappropriately high insulin and c-peptide levels, low free fatty acid and ketone body concentrations, glycemic response to glucagons, and the absence of ketonuria[ncbi.nlm.nih.gov]
Base Deficit
  • ABG done at this time showed a pH of 7.43, O2 saturation 100% and base deficit - 0.3 meq/L. The serum K? was 4.2 meq/ L and serum Na? 134 meq/L.[jpgmonline.com]
Pleural Effusion
  • Here, we report a case of a 12-year-old male who presented with pleural effusion and was revealed to have a large anterior mediastinal mass. Biopsy of the mass revealed benign mature teratoma.[ncbi.nlm.nih.gov]

Treatment

  • Treatment involves maintaining normal blood glucose levels; treatment modalities include high glucose infusion rates, use of medications, and surgical intervention.[ncbi.nlm.nih.gov]
  • Pancreatectomy is an acceptable treatment for this condition. We present the video of a case of near-total distal robotic pancreactectomy for the treatment of nesidioblastosis after Roux-en-Y gastric bypass.[ncbi.nlm.nih.gov]
  • If appropriate treatment is delayed there is a high risk of the development of cerebral palsy, impaired mental development, epilepsy or other forms of irreversible brain damage, so early detection and early treatment to stabilize serum glucose levels[ncbi.nlm.nih.gov]
  • After unsuccessful treatment with corticosteroids and somatostatin analogs, treatment with pasireotide, a novel somatostatin analog with high affinity to somatostatin receptor 5 and a possible side effect of hyperglycemia, was initiated (0.6 mg BID).[ncbi.nlm.nih.gov]
  • Early diagnosis and treatment are important in young and very young patients because early treatment accounts for favourable mental outcomes.[ncbi.nlm.nih.gov]

Prognosis

  • CONCLUSION: These rare paediatric tumours appear to have a good prognosis, even when malignant, and respond well to radical surgical ablation. Adjuvant therapy appears to be unnecessary.[ncbi.nlm.nih.gov]
  • (Outcomes/Resolutions) The prognosis of Nesidioblastosis depends upon the severity of the signs and symptoms.[dovemed.com]
  • Thus, it is essential to determine the cause of OHS and to apply the surgical cure method during the preoperational period, thereby improving the long-term prognosis.[enets.org]
  • Most cases of nesidioblastosis have good prognosis. Causes And Risk Factors For Nesidioblastosis The exact etiology of nesidioblastosis of pancreas is obscure. This disease can occur since birth or may develop later in life.[tandurust.com]

Etiology

  • Severe, persistent hypoglycemia can have various etiologies; one of the most common causes is hyperinsulinism. Nesidioblastosis, although rare, is the most common cause of hyperinsulinism in the neonate.[ncbi.nlm.nih.gov]
  • This population may especially benefit from this novel assay to confirm complete resection and to differentiate multiple etiologies of hyperinsulinism. More About This Work Academic Units Surgery Published Here September 9, 2014[academiccommons.columbia.edu]
  • SLE may also present with hypoglycemia by production of anti‐insulin antibodies, but in this case nesidioblastosis was the confirmed etiology.[shmabstracts.com]
  • Causes And Risk Factors For Nesidioblastosis The exact etiology of nesidioblastosis of pancreas is obscure. This disease can occur since birth or may develop later in life.[tandurust.com]

Epidemiology

  • Most common cause of persistent hyperinsulinemic hypoglycemia in neonates Synonym(s): noninsulinoma pancreatogenous hypoglycemia (NIPHS); persistent hyperinsulinemic hypoglycemia of infancy (PHHI); congenital hyperinsulinism; islet cell hyperplasia Epidemiology[unboundmedicine.com]
Sex distribution
Age distribution

Pathophysiology

  • The article reviews the pathophysiology and treatment modalities for nesidioblastosis.[ncbi.nlm.nih.gov]
  • However, whether the presence of glomerulocystic disease in association with those other neurocristopathic pathologies is a coincidental finding or shares a common pathophysiological mechanism remains to be determined.[ncbi.nlm.nih.gov]
  • Abstract Although the endocrine pancreas appears to play an important role in the pathophysiology of sickle cell disease, very little is known about the morphologic changes in this tissue.[ncbi.nlm.nih.gov]
  • Although it is well-described in infants, the pathophysiology in adults is poorly understood. Aim(s): From our review of the literature, we believe that this is the second reported case of localized nesidioblastosis in an adult.[enets.org]

Prevention

  • In such a patient, diazoxide or, when it is of no avail, surgical intervention should promptly be instituted to prevent possible neurologic sequelae induced by hypoglycemia.[ncbi.nlm.nih.gov]
  • Subtotal pancreatectomy failed to prevent recurrent post-operative hypoglycaemia. Subcutaneous somatostatin analogue (SMS 201-995) was shown to increase the blood concentrations of glucose and B-hydroxybutyrate while lowering serum insulin levels.[ncbi.nlm.nih.gov]
  • Despite subtotal distal pancreatectomy both children required frequent daytime feedings and continuous nocturnal intragastric polycose administration to prevent symptomatic hypoglycemia.[ncbi.nlm.nih.gov]
  • The main aim is to prevent the severe episodes of hypoglycaemia which can cause damage to the brain and/or mental retardation.[ncbi.nlm.nih.gov]
  • Severe neonatal hypoglycemia due to nesidioblastosis demands prompt diagnosis and treatment to prevent mental retardation. Early central venous catheter placement is essential for a constant glucose infusion.[ncbi.nlm.nih.gov]

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