Nesidioblastosis
Calcium Gluconate
Hypoglycemia
Hyperinsulinism
Insulinoma
Adenoma, Islet Cell
Islets of Langerhans
Pancreatic Neoplasms
Pancreas
Hyperinsulinemic hypoglycemia with nesidioblastosis after gastric-bypass surgery. (1/16)
We describe six patients (five women and one man; median age, 47 years; range, 39 to 54) with postprandial symptoms of neuroglycopenia owing to endogenous hyperinsulinemic hypoglycemia after Roux-en-Y gastric bypass surgery. Except for equivocal evidence in one patient, there was no radiologic evidence of insulinoma. Selective arterial calcium-stimulation tests, positive in each patient, were used to guide partial pancreatectomy. Nesidioblastosis was identified in resected specimens from each patient, and multiple insulinomas were identified in one. Hypoglycemic symptoms diminished postoperatively. We speculate that hyperfunction of pancreatic islets did not lead to obesity but that beta-cell trophic factors may have increased as a result of gastric bypass. (+info)Noninsulinoma pancreatogenous hypoglycemia syndrome: a rare case of adult-onset nesidioblastosis. (2/16)
The most common cause of hyperinsulinemic hypoglycemia in adults is insulinoma. Nesidioblastosis is a rare, but well-recognized disorder of persistent hyperinsulinemic hypoglycemia in infancy, but adult-onset nesidioblastosis associated with hyperinsulinemic hypoglycemia, termed noninsulinoma pancreatogenous hypoglycemic syndrome (NIPHS), has been reported. Here, we describe an extremely rare case of NIPHS in an elderly man. A 78-year-old man was admitted to our hospital due to hypoglycemic coma. During the previous 3 months, he noticed excessive sweating at midafternoon. His low fasting plasma glucose level (27 mg/dl) and high immunoreactive insulin level (11.1 muU/ml) were consistent with the possible presence of insulinoma. Localizing studies including computed tomography of the abdomen and celiac arteriography were negative, but selective arterial calcium infusion (SACI) test suggested the presence of insulinoma in the body and tail of the pancreas. Surgical exploration by palpation and intraoperative ultrasonography failed to detect any mass in the pancreas, and 60% distal pancreatectomy was performed. Postoperatively, his hypoglycemic episodes completely disappeared. Histological examination of the resected pancreas revealed diffuse islet cell hyperplasia consistent with a pathological diagnosis of nesidioblastosis. Thus, our case is a very rare case of NIPHS, or adult-onset nesidioblastosis, in which SACI test was proven to be a useful diagnostic tool for localization of the pancreatic lesion. (+info)The triad of nesidioblastosis, congenital neuroblastoma and glomerulocystic disease of the newborn: a case report. (3/16)
Neuroblastoma is the most common malignant tumor of the newborn, comprising 20% of all malignancies encountered during the neonatal period. We herein report a newborn who was born after 29 weeks' gestation and died unexpectedly at the 12th hour of life with no response to vigorous cardiopulmonary resuscitation. Autopsy findings revealed a right pararenal mass; microscopic examination showed neuroblastoma. Although the pancreas was grossly normal, its microscopic sections revealed a reduced number of islets of Langerhans and dispersion of the islet cells throughout the exocrine cells of the pancreas, and immunocytochemistry for the pancreatic hormones confirmed the dispersion of the islet cells. Final pathologic interpretation thus concluded the presence of nesidioblastosis. Furthermore, microscopic examination of the kidney showed glomerulocystic disease. Although the association of congenital neuroblastoma and nesidioblastosis has recently been defined as a new complex, neurocristopathy, the triad of congenital neuroblastoma, nesidioblastosis and glomerulocystic disease of the newborn has not been reported previously. To our knowledge, our case is the first reported newborn presenting with this triad. In conclusion, the association of nesidioblastosis and/or renal glomerulocystic disease should be kept in mind when encountering a case of congenital neuroblastoma. 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. (+info)A rare case of adult-onset nesidioblastosis treated successfully with diazoxide. (4/16)
A 54-year-old man was admitted to our hospital for evaluation of hypoglycemia. He had frequent episodes of loss of concentration before dinner. The ratio of IRI to plasma glucose (PG) was 0.8-1.0. Abdominal CT revealed no pancreatic tumor, and angiography of splenic artery showed no definite tumor stain within the pancreas. Based on the results of selective arterial calcium stimulation and hepatic venous sampling (ASVS), the provisional diagnosis was a small insulinoma in the pancreatic body. The patient underwent subtotal distal pancreatectomy. However, histopathological and immunohistochemical examinations of the resected tissue showed hypertrophy of islets of Langerhans islands and beta cells around pancreatic ducts. The final diagnosis was adult-onset nesidioblastosis. Postoperatively, the patient continued to exhibit hyperinsulinemia and nighttime hypoglycemia. Octreotide, voglibose and diet therapies failed to improve the nocturnal hypoglycemia. However, treatment with diazoxide at a starting dose of 200 mg/day resulted in immediate amelioration of nocturnal hypoglycemia. This is the first Japanese adult case of nesidioblastosis treated successfully with diazoxide. This case report suggests that diazoxide may be effective for adult-onset nesidioblastosis in a manner similar to that described for pediatric cases. (+info)Insulin responses to selective arterial calcium infusion under hyperinsulinemic euglycemic glucose clamps: case studies in adult nesidioblastosis and childhood insulinoma. (5/16)
Selective arterial calcium stimulation and hepatic venous sampling (ASVS) for insulin secretion is used as a diagnostic procedure in patients with insulinomas or adult nesidioblastosis. In some of those patients, severe hypoglycemia requiring urgent glucose administration occurs during the procedure. Such glucose administration, however, may affect the results and damage the validity of the test. We report two cases of hyperinsulinemic hypoglycemia, in which ASVS tests were successfully performed under hyperinsulinemic euglycemic glucose clamps. A 40-year-old male with nesidioblastosis developed continual severe hypoglycemia several years after a Billroth II-Braun gastrectomy, and continuous glucose infusion could not be stopped even during ASVS tests. A 9-year-old girl with an insulinoma that showed atypical hypovascularity on imaging examinations had ASVS tests under a glucose clamp for safety. Hyperinsulinemic (approximately 100 microU/ml) euglycemic (approximately 90 mg/dl) clamps were achieved by an artificial endocrine pancreas. The insulin analogue lispro was utilized for clamps and endogenous insulin was measured with an assay that does not cross-react with the analogue. Diagnostically significant responses (more than twofold) of insulin secretion were observed under hyperinsulinemic clamps in both cases. The use of the hyperinsulinemic glucose clamp technique during the ASVS test should be considered for maintaining the safety of some hypoglycemic patients. (+info)Hyperinsulinemic hypoglycemia due to adult nesidioblastosis in insulin-dependent diabetes. (6/16)
In neonates, persistent hyperinsulinemic hypoglycemia (PHH) is associated with nesidioblastosis. In adults, PHH is usually caused by solitary benign insulinomas. We report on an adult patient who suffered from insulin-dependent diabetes mellitus, and subsequently developed PHH caused by diffuse nesidioblastosis. Mutations of the MEN1 and Mody (2/3) genes were ruled out. Preoperative diagnostic procedures, the histopathological criteria and the surgical treatment options of adult nesidioblastosis are discussed. So far only one similar case of adult nesidioblastosis subsequent to diabetes mellitus II has been reported in the literature. In case of conversion of diabetes into hyperinsulinemic hypoglycemia syndrome, nesidioblastosis in addition to insulinoma should be considered. (+info)Recurrent hypoglycaemia in HIV-positive narcotic addicts. (7/16)
QUESTIONS UNDER STUDY: We describe two narcotic addict women with recurrent hypoglycaemic episodes. In both patients, hyperinsulinaemic hypoglycaemia occurring in the fasting state was documented and computed tomography of the pancreas was normal. METHODS AND RESULTS: In patient 1, selective arterial calcium stimulation with hepatic venous sampling (ASVS) revealed pronounced insulin hypersecretion predominantly in the tail and, to a lesser extent, in the corpus and the head of the pancreas. On laparoscopic exploration, tumours could not be detected be it grossly or by intraoperative ultrasound. Distal pancreatectomy was performed laparoscopically, and histological examination of the resected tissue revealed nesidioblastosis. ASVS was also performed in patient 2 revealing less marked increases in insulin secretion, ie up to 2.3-fold in response to calcium stimulation of the superior mesenteric artery, consistent with the presence of pathological beta-cells located predominantly in the head of the pancreas. Surgical exploration was not performed in this patient. CONCLUSION: HIV infection had been known in both women for around ten years and both patients were not on antiretroviral therapy. Because symptomatic nesidioblastosis in adult patients is a very rare disorder, we speculate that nesidioblastosis may develop in the context of HIV infection and/or abuse of narcotic drugs. Our observations illustrate that neurocognitive impairment in HIV positive patients is not always due to toxic compounds or a cerebral disorder but may be caused by an apparently rare pancreatic disorder, nesidioblastosis. Thus, the patients should be checked for the presence of hyperinsulinaemic hypoglycaemia. (+info)Hyperinsulinemic hypoglycemia due to diffuse nesidioblastosis in adults: a case report. (8/16)
Persistent hyperinsulinemic hypoglycemia is caused most commonly by an insulinoma in adults or by nesidioblastosis in neonates. In adults, nesidioblastosis is a rare disorder characterized by diffuse or disseminated proliferation of islet cells. We recently encountered a case of nesidioblastosis in an adult. A 71-year-old man was admitted due to intermittent general weakness, abdominal pain, and mild dyspnea. The patient underwent a subtotal gastrectomy for a gastric adenocarcinoma two years ago. After 5 d of admission, the patient showed symptoms of cold sweating, chilling, and hypotension 30 min after eating. Thereafter, he frequently showed similar symptoms accounting for hypoglycemia regardless of food consumption. Laboratory findings revealed a low fasting blood glucose level (25 mg/dL), and a high insulin level (47 muIU/mL). Selective intra-arterial calcium stimulation with hepatic venous sampling (ASVS) was performed to localize a mass and revealed an increased insulin level about four-fold that of the normal fasting level at 60 s in the splenic artery, which suggested the presence of an insulinoma in the tail of pancreas. A distal pancreatectomy was performed. Neither intraoperative exploration nor a frozen biopsy specimen detected any mass-forming lesion. On the histological examination, many of the islets were enlarged and irregularly shaped in all specimens, the arrangement of which was a lobulated islet pattern. Cytologically, a considerable subpopulation of endocrine cells showed enlarged and hyperchromatic nuclei. By immunohistochemistry, the cells were identified as beta-cells. These clinical, radiological, microscopic and immuno-histochemical findings are consistent with diffuse nesidioblastosis in adults. (+info)Nesidioblastosis is a very rare condition that affects the pancreas, a gland located behind the stomach that produces hormones and enzymes to help with digestion. In nesidioblastosis, there is an abnormal increase in the number of cells called beta cells in the pancreas that produce insulin, a hormone that regulates blood sugar levels. This can lead to persistent hyperinsulinemia (high levels of insulin in the blood) and hypoglycemia (low blood sugar levels), even when the person has not eaten for several hours.
The term "nesidioblastosis" comes from the Greek words "nesis," meaning island, and "blastos," meaning germ or bud. It refers to the abnormal formation of islets of Langerhans, which are clusters of hormone-producing cells in the pancreas. In nesidioblastosis, there is an overgrowth of beta cells within these islets, leading to excessive insulin production and secretion.
Nesidioblastosis can be congenital (present at birth) or acquired later in life. It is often diagnosed in infants and young children but can also occur in adults. The symptoms of nesidioblastosis include sweating, tremors, irritability, seizures, and loss of consciousness due to low blood sugar levels. Treatment typically involves medication to control insulin secretion, dietary modifications, and, in some cases, surgery to remove part or all of the pancreas.
Pancreatic diseases refer to a group of medical conditions that affect the structure and function of the pancreas, a vital organ located in the abdomen. The pancreas has two main functions: an exocrine function, which involves the production of digestive enzymes that help break down food in the small intestine, and an endocrine function, which involves the production of hormones such as insulin and glucagon that regulate blood sugar levels.
Pancreatic diseases can be broadly classified into two categories: inflammatory and non-inflammatory. Inflammatory pancreatic diseases include conditions such as acute pancreatitis, which is characterized by sudden inflammation of the pancreas, and chronic pancreatitis, which is a long-term inflammation that can lead to scarring and loss of function.
Non-inflammatory pancreatic diseases include conditions such as pancreatic cancer, which is a malignant tumor that can arise from the cells of the pancreas, and benign tumors such as cysts or adenomas. Other non-inflammatory conditions include pancreatic insufficiency, which can occur when the pancreas does not produce enough digestive enzymes, and diabetes mellitus, which can result from impaired insulin production or action.
Overall, pancreatic diseases can have serious consequences on a person's health and quality of life, and early diagnosis and treatment are essential for optimal outcomes.
Calcium gluconate is a medical compound that is used primarily as a medication to treat conditions related to low calcium levels in the body (hypocalcemia) or to prevent calcium deficiency. It is also used as an antidote for treating poisoning from certain chemicals, such as beta-blockers and fluoride.
Calcium gluconate is a form of calcium salt, which is combined with gluconic acid, a natural organic acid found in various fruits and honey. This compound has a high concentration of calcium, making it an effective supplement for increasing calcium levels in the body.
In medical settings, calcium gluconate can be administered orally as a tablet or liquid solution, or it can be given intravenously (directly into a vein) by a healthcare professional. The intravenous route is typically used in emergency situations to quickly raise calcium levels and treat symptoms of hypocalcemia, such as muscle cramps, spasms, or seizures.
It's important to note that while calcium gluconate can be beneficial for treating low calcium levels, it should only be used under the guidance of a healthcare provider, as improper use or overdose can lead to serious side effects, including kidney damage and heart problems.
Hypoglycemia is a medical condition characterized by an abnormally low level of glucose (sugar) in the blood. Generally, hypoglycemia is defined as a blood glucose level below 70 mg/dL (3.9 mmol/L), although symptoms may not occur until the blood sugar level falls below 55 mg/dL (3.0 mmol/L).
Hypoglycemia can occur in people with diabetes who are taking insulin or medications that increase insulin production, as well as those with certain medical conditions such as hormone deficiencies, severe liver illnesses, or disorders of the adrenal glands. Symptoms of hypoglycemia include sweating, shaking, confusion, rapid heartbeat, and in severe cases, loss of consciousness or seizures.
Hypoglycemia is typically treated by consuming fast-acting carbohydrates such as fruit juice, candy, or glucose tablets to rapidly raise blood sugar levels. If left untreated, hypoglycemia can lead to serious complications, including brain damage and even death.
A pancreatectomy is a surgical procedure in which all or part of the pancreas is removed. There are several types of pancreatectomies, including:
* **Total pancreatectomy:** Removal of the entire pancreas, as well as the spleen and nearby lymph nodes. This type of pancreatectomy is usually done for patients with cancer that has spread throughout the pancreas or for those who have had multiple surgeries to remove pancreatic tumors.
* **Distal pancreatectomy:** Removal of the body and tail of the pancreas, as well as nearby lymph nodes. This type of pancreatectomy is often done for patients with tumors in the body or tail of the pancreas.
* **Partial (or segmental) pancreatectomy:** Removal of a portion of the head or body of the pancreas, as well as nearby lymph nodes. This type of pancreatectomy is often done for patients with tumors in the head or body of the pancreas that can be removed without removing the entire organ.
* **Pylorus-preserving pancreaticoduodenectomy (PPPD):** A type of surgery used to treat tumors in the head of the pancreas, as well as other conditions such as chronic pancreatitis. In this procedure, the head of the pancreas, duodenum, gallbladder, and bile duct are removed, but the stomach and lower portion of the esophagus (pylorus) are left in place.
After a pancreatectomy, patients may experience problems with digestion and blood sugar regulation, as the pancreas plays an important role in these functions. Patients may need to take enzyme supplements to help with digestion and may require insulin therapy to manage their blood sugar levels.
Hyperinsulinism is a medical condition characterized by an excess production and release of insulin from the pancreas. Insulin is a hormone that helps regulate blood sugar levels by allowing cells in the body to take in sugar (glucose) for energy or storage. In hyperinsulinism, the increased insulin levels can cause low blood sugar (hypoglycemia), which can lead to symptoms such as sweating, shaking, confusion, and in severe cases, seizures or loss of consciousness.
There are several types of hyperinsulinism, including congenital forms that are present at birth and acquired forms that develop later in life. Congenital hyperinsulinism is often caused by genetic mutations that affect the way insulin is produced or released from the pancreas. Acquired hyperinsulinism can be caused by factors such as certain medications, hormonal disorders, or tumors of the pancreas.
Treatment for hyperinsulinism depends on the underlying cause and severity of the condition. Treatment options may include dietary changes, medication to reduce insulin secretion, or surgery to remove part or all of the pancreas.
Insulinoma is a rare type of neuroendocrine tumor that originates from the beta cells of the pancreatic islets (islets of Langerhans). These tumors produce and secrete excessive amounts of insulin, leading to hypoglycemia (low blood sugar levels) even when the person hasn't eaten for a while. Insulinomas are typically slow-growing and benign (noncancerous), but about 10% of them can be malignant (cancerous) and may spread to other parts of the body. Common symptoms include sweating, confusion, dizziness, and weakness due to low blood sugar levels. The diagnosis is often confirmed through imaging tests like CT scans or MRI, and measuring insulin and C-peptide levels in the blood during a fasting test. Treatment usually involves surgical removal of the tumor.
An islet cell adenoma is a rare, typically benign tumor that develops in the islets of Langerhans, which are clusters of hormone-producing cells in the pancreas. The islets of Langerhans contain several types of cells, including beta cells that produce insulin, alpha cells that produce glucagon, and delta cells that produce somatostatin.
Islet cell adenomas can cause various endocrine disorders depending on the type of hormone-producing cells involved. For example, if the tumor consists mainly of beta cells, it may secrete excessive amounts of insulin, leading to hypoglycemia (low blood sugar). Conversely, if the tumor is composed primarily of alpha cells, it may produce too much glucagon, resulting in hyperglycemia (high blood sugar) and a condition known as glucagonoma.
Islet cell adenomas are usually slow-growing and small but can become quite large in some cases. They are typically diagnosed through imaging tests such as CT scans or MRI, and hormone levels may be measured to determine the type of cells involved. Treatment options include surgical removal of the tumor, medication to manage hormonal imbalances, and, in rare cases, radiofrequency ablation or embolization.
The Islets of Langerhans are clusters of specialized cells within the pancreas, an organ located behind the stomach. These islets are named after Paul Langerhans, who first identified them in 1869. They constitute around 1-2% of the total mass of the pancreas and are distributed throughout its substance.
The Islets of Langerhans contain several types of cells, including:
1. Alpha (α) cells: These produce and release glucagon, a hormone that helps to regulate blood sugar levels by promoting the conversion of glycogen to glucose in the liver when blood sugar levels are low.
2. Beta (β) cells: These produce and release insulin, a hormone that promotes the uptake and utilization of glucose by cells throughout the body, thereby lowering blood sugar levels.
3. Delta (δ) cells: These produce and release somatostatin, a hormone that inhibits the release of both insulin and glucagon and helps regulate their secretion in response to changing blood sugar levels.
4. PP cells (gamma or γ cells): These produce and release pancreatic polypeptide, which plays a role in regulating digestive enzyme secretion and gastrointestinal motility.
Dysfunction of the Islets of Langerhans can lead to various endocrine disorders, such as diabetes mellitus, where insulin-producing beta cells are damaged or destroyed, leading to impaired blood sugar regulation.
Pancreatic neoplasms refer to abnormal growths in the pancreas that can be benign or malignant. The pancreas is a gland located behind the stomach that produces hormones and digestive enzymes. Pancreatic neoplasms can interfere with the normal functioning of the pancreas, leading to various health complications.
Benign pancreatic neoplasms are non-cancerous growths that do not spread to other parts of the body. They are usually removed through surgery to prevent any potential complications, such as blocking the bile duct or causing pain.
Malignant pancreatic neoplasms, also known as pancreatic cancer, are cancerous growths that can invade and destroy surrounding tissues and organs. They can also spread (metastasize) to other parts of the body, such as the liver, lungs, or bones. Pancreatic cancer is often aggressive and difficult to treat, with a poor prognosis.
There are several types of pancreatic neoplasms, including adenocarcinomas, neuroendocrine tumors, solid pseudopapillary neoplasms, and cystic neoplasms. The specific type of neoplasm is determined through various diagnostic tests, such as imaging studies, biopsies, and blood tests. Treatment options depend on the type, stage, and location of the neoplasm, as well as the patient's overall health and preferences.
The pancreas is a glandular organ located in the abdomen, posterior to the stomach. It has both exocrine and endocrine functions. The exocrine portion of the pancreas consists of acinar cells that produce and secrete digestive enzymes into the duodenum via the pancreatic duct. These enzymes help in the breakdown of proteins, carbohydrates, and fats in food.
The endocrine portion of the pancreas consists of clusters of cells called islets of Langerhans, which include alpha, beta, delta, and F cells. These cells produce and secrete hormones directly into the bloodstream, including insulin, glucagon, somatostatin, and pancreatic polypeptide. Insulin and glucagon are critical regulators of blood sugar levels, with insulin promoting glucose uptake and storage in tissues and glucagon stimulating glycogenolysis and gluconeogenesis to raise blood glucose when it is low.