Nesidioblastosis in Children

Knight, James A.

doi:10.1001/archsurg.1980.01380070068015

Cited by 14 publications

(2 citation statements)

References 5 publications

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“…Nesidioblastosis is a condition of diffuse islet hyperplasia and/or microadenomatous formation occurring usually in infants, with a high incidence of mental damage due to neuroglycopenia [39]. Distal resection of up to 90% of the pancreas has given good results in these young children [40]. Hyperplasia has been described in adults, but it is not very well documented, and it is not clear whether this is a persistence of the infantile condition.…”

Section: Islet-cell Hyperplasia and Nesidioblastosismentioning

confidence: 99%

The management of the hypoglycemic patient

Kennedy

1982

World j. surg.

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Organic hypoglycemia may be caused by an insulinoma. This is a rare tumor, multiple in about 10% and malignant in about 10% of cases. Multiplicity is particularly likely when insulinoma occurs as part of the MEN 1 syndrome. When a patient exhibits Whipple's triad, synchronous blood sugar and insulin estimations must be made, and repeated during fasting. Diagnosis depends on the finding of inappropriately high insulin levels in the presence of low blood sugars. Many stimulatory tests are available but none are as reliable as prolonged starvation, and probably should be abandoned.Measurement of proinsulin and C‐peptide are of particular value in the diagnosis and in elimination of factitious hypoglycemia.Preoperative localization is most useful and can be achieved in up to 90% of patients by skillful selective angiography. Percutaneous portal venous sampling has been used and is particularly indicated in patients after a fruitless first exploration.Tumors of the body and tail are usually treated by distal pancreatectomy, those of the head by enucleation. There is a risk of pancreatic fistula, and duct damage can be unmasked by secretin injection. Occult tumors and hyperplasia may require “blind” progressive distal resection with blood sugar monitoring until the hypoglycemia is controlled, but this is something of a desperation measure. In malignant tumors, hypoglycemia may be controlled by diazoxide or somatostatin. Streptozotocin may control the syndrome and lead to prolongation of life. Like other malignant apudomas, these tumors are usually slow growing and relatively benign.

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Section: Islet-cell Hyperplasia and Nesidioblastosismentioning

confidence: 99%

The management of the hypoglycemic patient

Kennedy

1982

World j. surg.

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“…The pancreatic lesions responsible for the hyperinsulinism are various: hyperplasia of the islets of Langherans, adenomatosis, nesidioblastosis and true adenomas [8][9][10][11][12][13][14].…”

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confidence: 99%

Pancreatic venous samplings in infants and children with primary hyperinsulinism

et al. 1989

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The authors present 19 cases of hyperinsulinism in children worked up with selective pancreatic venous samplings (PVS). Focal lesions were found in 7, diffuse secretion in 8 and normal insulin levels in 4. In three patients with focal hypersecretion less extensive surgery could be performed and confirmed the presence of focal lesions in two. These preliminary results are encouraging and PVS seems to be a valuable technic for detection of focal lesions in the pancreas of children with hyperinsulinism.

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