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At least 19 recordsLinked to original sources
PubMedDate not supplied

Serum and pancreatic immunoreactive insulin (IRI) and proinsulin-like component (PLC), serum IRI and PLC response to different stimuli in normal subjects and organic hyperinsulinism.

The serum levels of total immunoreactive insulin (IRI) and proinsulin-like component (PLC) in the fasting state and following the administration of insulin secretagogues in 5 patients with organic hyperinsulinism and age and sex matched normal subjects are reported. Diagnosis of organic hyperinsulinism could be established in all instances on the basis of the inappropriately high total serum IRI levels for the corresponding blood glucose values; such an abnormal relationship was not seen in normal subjects, and was further enhanced by insulin secretagogues. Unrestrained insulin secretion in organic hyperinsulinism was enhanced following the administration of glucose, tolbutamide, glucagon or amino acids; the last 2 stimuli are known to be ineffective in causing insulin secretion in the presence of hypoglycemia in normal subjects. Four patints had insulinomas and one probably had islet cell hyperplasia or abnormal function of islet cells. Chromatography of serum IRI to quantitate PLC is a useful adjunct to the diagnosis of organic hyperinsulinism as in the fasting state the proportion of PLC is always elevated, above the normal range of 5-22%. Following the administration of insulin secretagogues there was pronounced increase in total serum IRI in organic hyperinsulinism but the proportion of PLC generally decreased, suggesting thereby that mojor increase in IRI was due to release of stored granular IRI which is known to have a low proportion of PLC.

Adenoma, Islet Cell↗
PubMed1976

[Hyperinsulinism. Neurological and psychiatric aspects (author's transl)].

A case of a patient with hyperinsulinism due to insulinoma associated with neurological and psychiatric disturbances including EEG alterations is reported. The hunger test as well as the i.v. tolbutamid test proved to be of diagnostic importance. In addition, the electroencephalographic studies combined with blood sugar analyses before and after 50 g glucose, orally, showed a reversibility of the EEG alterations together with normalization of the blood surgar levels. These results point to the possibility of differentiating biochemical from structural cerebral lesions associated with hyperinsulinism.

Adult↗
PubMed1977

Insulin, proinsulin, glucagon and gastrin in pancreatic tumors and in plasma of patients with organic hyperinsulinism.

Insulin, proinsulin, glucagon and gastrin were determined in extracts of tumors of 27 patients with pancreatic islet cell neoplasia of pancreas, in one patient with nesidioblastosis, in extracts of uninvolved portions of the pancreas in 11 of the tumor patients and of 15 control pancreases. Mean insulin concentration in solitary adenomas and in adenomas of patients with adenomatosis was higher than in control pancreases; however, in all but 1 patient the insulin concentration in neoplastic islet tissue was lower than in islet tissue of control pancreas, assuming islet volume is 1% of pancreas. The percentage of proinsulin was elevated in 52% of tumors. Adenoma insulin content correlated with increments of plasma insulin after tolbutamide administration. Insulin and proinsulin concentrations in pancreas uninvolved by tumor were not suppressed. Fasting plasma glucagon was elevated in patients with islet cell adenomatosis and in patients with islet cell carcinoma some of whom had multiple endocrine adenomatosis. The mean concentration of glucagon in tumors was lower than in control pancreases. Elevated concentration of gastrin was found in some adenomas. The data indicate: 1) insulin-secreting islet cell tumors have decreased storage capacity for insulin, 2) elevated concentration of proinsulin in tumors may be due to decreased capacity to store insulin and in some to decreased conversion of proinsulin to insulin as well, 3) tolbutamide stimulates the exaggerated release of a relatively constant fraction of insulin stored in adenomas. 4) solitary adenomas may contain excess amounts of pancreatic hormones in addition to insulin, 5) elevated plasma glucagon in patients with organic hyperinsulinism may indicate malignancy, microadenomatosis or multiple endocrine adenoma syndrome, and 6) chronic hyperinsulinism and hypoglycemia due to adenoma do not suppress insulin and proinsulin content of uninvolved pancreas.

Adenoma↗
PubMed1976

Medical treatment of endogenous organic hyperinsulinism.

There are several situations in which medical therapy of hyperinsulinism induced by islet cell tumors or hyperplasia is necessary and at present we have at our disposal several drugs which are capable of reducing endogenous hyperinsulinism. They are: -Streptozotocin, which represents today the most useful therapeutic agent for beta cell carcinoma therapy; -Diazoxide, which represents the drug of first choice for the treatment of most hypoglycemic syndromes caused by islet cell adenoma or hyperplasia; -Propranolol, Chlorpromazine, Diphenylhydantoin, which may be regarded as a useful alternative to diazoxide, although they are capable of giving rather inconstant results. These drugs may today effectively substitute for corticosteroids and glucagon in the medical treatment of almost every chronic hyperinsulinemic hypoglycemic syndrome, including malignant beta cell carcinoma.

Adenoma, Islet Cell↗
PubMed1978

Treatment of hyperinsulinism after partial pancreatectomy: medical or surgical?

A 21 year old male patient with hypoglycemia secondary to hyperinsulinism had no palpable adenoma at surgery, and failed to respond to a partial (75%) pancreatectomy. Subsequently, he was begun on oral Diazoxide. Soon after initiation of Diazoxide he developed a viral pneumonitis, and because of hyperglycemia, the Diazoxide was temporarily discontinued. He has been treated since June of 1975 with Diazoxide, 100 mg, three times daily. He is asymptomatic, completely rehabilitated, and physically active at work for 10 hours daily. Diazoxide therapy may be an option worth considering even in benign states of hyperinsulinism when surgery has failed to correct the process.

Adult↗
PubMed1978

Fetal hyperinsulinism in rhesus isoimmunization.

Analyses of peritoneal ascitic fluid obtained prior to intrauterine transfusion show that some babies with severe rhesus isoimmunization develop raised insulin levels up to a month before delivery. The glucose content of fetal ascitic fluid is usually only about 10 mg. per 100 ml. less than the glucose content of maternal plasma and there is no evidence that this relation is influenced by the fetal or maternal insulin level. The electrolyte content of fetal ascitic fluid is very similar to that of maternal plasma, but fluid from babies with hyperinsulinism has an unusually high calcium content.

Ascitic Fluid↗
PubMed1977

Hyperinsulinism of hepatic cirrhosis: Diminished degradation or hypersecretion?

The breakdown of proinsulin in the pancreatic beta cell yields insulin and C-peptide which are secreted in equimolar amounts. Unlike insulin, C-peptide is not degraded significantly by the liver, so that its measurement should give a better assessment of insulin secretion than estimation of peripheral insulin levels alone; particularly in the presence of hepatic dysfunction. Plasma C-peptide and insulin response to an oral glucose load have therefore been assessed in 14 cirrhotic and 7 normal subjects. Cirrhotic patients were divided into hyperinsulinaemic and normoinsulinaemic groups based on fasting plasma-insulin concentrations. Fasting blood-blucose and plasma-C-peptide concentrations were the same in normal and cirrhotic subjects, suggesting that basal pancreatic insulin secretion was the same in all subjects. Thus the C-peptide/insulin ratio was significantly decreased in hyperinsulinaemic subjects (2-13 +/- 0-31, compared with 4-63 +/- 0-48 in controls). After oral glucose, the two groups of cirrhotic patients showed the same glucose intolerance. C-peptide concentrations were also the same but insulin concentrations were markedly increased in the hyperinsulinaemic group. It is suggested that pancreatic insulin secretion is not increased in cirrhosis and that the peripheral hyperinsulinism is due solely to decreased hepatic insulin degradation secondary to either spontaneous portal-systemic shunting or to parenchymal damage.

Administration, Oral↗
PubMedDate not supplied

Pancreatic venography and plasma ketone measurements in the diagnosis of hyperinsulinism.

Preoperative localization of an insulinoma was possible using a technique of transhepatic pancreatic venography, with measurement of insulin levels in six pancreatic veins. Surgical exploration confirmed the presence of an insulinoma at the site predicted by venography. Peripheral plasma insulin levels were only minimally elevated at the time of hypoglycemia. However, complete suppression of ketogenesis during a 74-hour fast was observed, and felt to be a biological marker for hyperinsulinism. These observations suggest that transhepatic pancreatic venography and the measurement of plasma ketones in addition to glucose and insulin during fasting, may be useful supplementary studies in the evaluation of patients with fasting hypoglycemia.

Adult↗
PubMed1978

Failure of somatostatin to diagnose organic hyperinsulinism.

In four patients with organic hyperinsulinism (two with surgically proven beta-cell adenomas of the body of the pancreas) a standard tolbutamide test during continuous somatostatin infusion (5 microgram/min) was carried out. Tolbutamide induced insulin release was completely inhibited by somatostatin as in normal subjects. These results suggest that the inhibition test with somatostatin does not seem to be a better or safer way of diagnosing insulin producing tumours.

Adult↗
PubMedDate not supplied

[Neonatal hypoglycemia due to hyperinsulinism secondary to Langerhans' polyadenomatosis. Recovery after pancreatectomy].

Report of a case of a neonatal hypoglycemia detected at the 11th hour of life, secondary to a Langherans polyadenomatosis in a girl with an hemihypertrophy. Pre- and post-operative tests are reported. In case of neonatal hypoglycemia, the criteria leading to the diagnosis of hyperinsulinism, i.e. the only neonatal hypoglycemia with surgical treatment are reviewed. Except in the case of adenoma, the pancreatectomy should be performed subtotally. The best time for surgery is as soon as the 3rd week, in order to preserve the cerebral development.

Adenoma, Islet Cell↗
PubMed1978

[Clinical aspects, diagnosis and treatment of organic hyperinsulinism. Experience with 46 operated patients (author's transl)].

The clinical picture of organic hyperinsulinism is presented with reference to experience in 46 operated patients. For the recognition of disease, a careful history is a decisive contribution. The hunger test showed a characteristic hypoglycemic reaction in 100%; the tolbutamide test gave a positive result in 92%. Insulinomas could be localized angiographically in 71%; in one patient this could only be done with an ERCT. The treatment of choice is operation as soon as possible. If possible, enucleation is to be given preference over pancreas resection because of the low complication rate. A search for ectopic (2%) and multiple (12%) adenomas is important. A cure was achieved in 76% of all those operated on. The hospital mortality was about 4%.

Adenoma↗
PubMed1975

The ultrastructure of focal islet cell adenomatosis in the newborn with hypoglycemia and hyperinsulinism.

In a newborn severe persistent hypoglycemia due to an insulin-producing tumorous proliferation of pancreatic islet cells (insulinoma) was observed. The insulinoma showed the histologic pattern of focal adenomatosis of islet cells. According to the present literature the focal proliferation of islet cell complexes seems to be a frequent and particular feature of insulinomas in the newborn. Differential islet cell staining identified 80%-90% of the proliferated islet cells as B cells. 10%-20% of the cells were found to be A or D cells. Ultrastructurally the majority of the proliferated islet cells were well differentiated B cells. The remaining cells represented either A or D cells or a fourth islet cell type with small spheric granules. Electronmicrscopic evidence of transitions between differentiated islet cells, particularly B cells, and the fourth islet cell type suggests that the fourth islet cell type might represent a precursor cell within the APUD-cell system.

Adenoma, Islet Cell↗
PubMed2026

A New Case of Lethal Congenital Contracture Syndrome Type 3 With Hyperinsulinism and Optic Atrophy.

Lethal congenital contracture syndrome 3 (LCCS3, MIM #611369) is a rare autosomal recessive neuromuscular disorder caused by biallelic loss-of-function (LOF) variants in PIP5K1C, reported in only two families to date. It typically presents with severe fetal akinesia, arthrogryposis multiplex congenita, and perinatal lethality due to respiratory insufficiency case. Herein, we report a new case with survival beyond birth. Prenatal findings included clubfeet with preserved amniotic fluid volume and fetal movements. The infant was delivered by cesarean section at 37 + 7 weeks following breech presentation and developed respiratory distress requiring 14 days of ventilatory support. Physical examination revealed bilateral talipes equinovarus, flexion contractures of the knees, restricted hip mobility, clenched hands with flexion contractures of the third and fourth fingers, and hyperextension of the second and fifth fingers. Neurologically, he had encephalopathy, profound hypotonia with a frog posture, and abnormal neonatal reflexes with a discontinuous background pattern on cerebral function monitoring. Additional observed features were bilateral optic atrophy and hyperinsulinemic hypoglycemia responsive to Diazoxide. Trio genome sequencing identified a homozygous pathogenic splice-site variant in PIP5K1C (c.1127+1G>A, NM_012398.3). The infant died at 6 months from multisystemic failure. Further studies are warranted to elucidate the pathomechanisms underlying the PIP5K1C defect and its phenotypic consequences.

LCCS3↗