1/5. Normal pregnancy in a woman with nesidioblastosis treated with somatostatin analog octreotide.OBJECTIVE: We report the case of a 36-yr-old woman with nesidioblastosis treated throughout pregnancy with high doses of octreotide. We studied the course of blood glucose, foetal growth and development. methods: Blood samples were obtained every month throughout pregnancy and taken at birth from the umbilical cord. Sonography was performed repeatedly to monitor foetal growth. RESULTS: The daily dose of octreotide was adapted to blood glucose levels: a dose of 1000 microg was infused during the first part of pregnancy, then it was decreased step by step during the last trimester of gestation. An elective cesarean section was performed at 32 weeks of gestation. High octreotide concentrations were obtained during the first part of gestation (range 2888-5021 pg/ml). During the third trimester of pregnancy blood glucose increased despite high insulin levels attesting physiological insulin-resistance. plasma levels of placental GH and IGF-1 levels were similar to those observed in a normal pregnancy. Despite the presence of octreotide in the umbilical cord, TSH, free T4, PRL and pituitary GH concentrations were normal at birth. The female newborn (weight 3520 g, length 52 cm) had no malformation, and presented with normal postnatal development. CONCLUSION: Our study demonstrates that: 1) octreotide treatment can be effective in controlling endogenous hyperinsulinism during pregnancy; 2) octreotide does not affect physiological changes during pregnancy such as insulin-resistance or placental GH level; 3) exposure of the foetus to octreotide throughout pregnancy does not induce any malformation and does not affect foetal development.- - - - - - - - - - ranking = 1keywords = hyperinsulinism (Clic here for more details about this article) |
2/5. Persistent hyperinsulinemic hypoglycemia of infancy - nesidioblastosis.Persistent hyperinsulinemic hypoglycemia of infancy (PHHI) or nesidioblastosis is a rare condition presenting with severe hypoglycemia. Prompt diagnosis and early pancreatectomy can save many of them, in spite of the magnitude of surgery. We present two cases in which near total pancreatectomy was performed with favourable outcome. Both patients are normoglycemic, with one requiring pancreatic enzyme supplements.- - - - - - - - - - ranking = 0.00012098312610427keywords = infancy (Clic here for more details about this article) |
3/5. nesidioblastosis--a rare cause of hypoglycaemia in adults.A case of suspected clinically hormonally active insulinoma in a 48-year-old woman is presented. Despite the lack of features, which might correspond to the insulinoma in radiological examinations, the patient was qualified for a distal subtotal pancreatectomy and then, due to persistent hyperinsulinism, for total pancreatectomy. The insulinoma was found neither in a palpable examination of the pancreas nor in the intraoperative ultrasonic examination. In a histopathological examination supplemented with immunohistochemical tests, nesidioblastosis - a rare cause of hypoglycaemia in adults - was diagnosed.- - - - - - - - - - ranking = 1.6649722465787keywords = hyperinsulinism, hypoglycaemia (Clic here for more details about this article) |
4/5. Noninsulinoma pancreatogenous hypoglycemia syndrome: a rare case of adult-onset nesidioblastosis.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.- - - - - - - - - - ranking = 2.4196625220855E-5keywords = infancy (Clic here for more details about this article) |
5/5. Post-gastric bypass hyperinsulinism with nesidioblastosis: subtotal or total pancreatectomy may be needed to prevent recurrent hypoglycemia.Symptomatic hyperinsulinemic hypoglycemia and pancreatic nesidioblastosis have recently been described in a small series of patients after gastric bypass surgery for morbid obesity. In the limited published reports of patients with this condition, hyperinsulinism and nesidioblastosis have been managed with distal or subtotal pancreatectomy, with the extent of resection guided by calcium angiography. However, nesidioblastosis may involve the pancreas diffusely, and limited pancreatic resections may predispose patients to further hypoglycemic episodes. We have treated two patients with refractory hyperinsulinism and symptomatic hypoglycemia after successful gastric bypass surgery. 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. A second patient had profound hyperinsulinemic hypoglycemia and was treated successfully with a subtotal (95%) pancreatectomy. Our experience, the third published report of post-gastric bypass nesidioblastosis, suggests that the risk of recurrent symptomatic hyperinsulinism after limited pancreatectomy is significant and relative euglycemia may be achieved with subtotal or total pancreatectomy.- - - - - - - - - - ranking = 8keywords = hyperinsulinism (Clic here for more details about this article) |