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Glucagonoma

A glucagonoma is a rare tumor of the alpha cells of the pancreas that results in up to a 1000-fold overproduction of the hormone glucagon. Alpha cell tumors are commonly associated with glucagonoma syndrome, though similar symptoms are present in cases of pseudoglucagonoma syndrome in the absence of a glucagon-secreting tumor. more...

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Medicines

History

Fewer than 250 cases of glucagonoma have been described in the literature since their first description by Becker in 1942. Because of its rarity (fewer than one in 20 million worldwide), long-term survival rates are as yet unknown.

Symptoms

The primary physiological effect of glucagonoma is an overproduction of the peptide hormone glucagon, which enhances blood glucose levels through the activation of catabolic processes including gluconeogenesis and lipolysis. Gluconeogenesis produces glucose from protein and amino acid materials; lipolysis is the breakdown of fat. The net result is hyperglucagonemia, decreased blood levels of amino acids (hypoaminoacidemia), anemia, diarrhea, and weight loss of 5-15 kg.

Necrolytic migratory erythema (NME) is a classical symptom observed in patients with glucagonoma and is present in 80% of cases. Associated NME is characterized by the spread of erythematous blisters and swelling across areas subject to greater friction and pressure, including the lower abdomen, buttocks, perineum, and groin.

Diabetes mellitus also frequently results from the insulin and glucagon imbalance that occurs in glucagonoma. Diabetes mellitus is present in 80-90% of cases of glucagonoma, and is exacerbated by preexisting insulin resistance.

Diagnosis

A blood serum glucagon concentration of 1000 pg/mL or greater is indicative of glucagonoma (the normal range is 50-200 pg/mL).

Blood tests may also reveal abnormally low concentrations of amino acids, zinc, and essential fatty acids, which are thought to play a role in the development of NME. Skin biopsies may also be taken to confirm the presence of NME.

A CBC can uncover anemia, which is an abnormally low level of hemoglobin.

The tumor itself may be localized by any number of radiographic modalities, including angiography, CT, MRI, PET, and endoscopic ultrasound. Laparotomy is useful for obtaining histologic samples for analysis and confirmation of the glucagonoma.

Treatment

Heightened glucagon secretion can be treated with the administration of octreotide, a somatostatin analog, which inhibits the release of glucagon. Doxorubicin and streptozotocin have also been used successfully to selectively damage alpha cells of the pancreatic islets. These do not destroy the tumor, but help to minimize progression of symptoms.

The only curative therapy for glucagonoma is surgical resection, where the tumor is removed. Resection has been known to reverse symptoms in some patients.

Read more at Wikipedia.org


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GlucaGen® HypoKit : an important consideration for all patients treated with insulin - glucagon rys
From Diabetes and Primary Care, 6/22/02

Many patients treated with insulin will experience severe hypoglycaemia at some time; yet relatively few have glucagon on hand. This is despite the fact that severe hypoglycaemia is considered to be a major cause of anxiety amongst insulin-treated patients. (1)

Causes of hypoglycaemia include skipping meals, stress, taking too much exercise or drinking too much alcohol. However, perhaps a more troubling cause is linked to intensive insulin therapy.

The risk of a severe hypoglycaemic event is 2-3 times higher in patients aiming to achieve optimal glucose control with intensive insulin therapy. (2) The fear of hypoglycaemia may inhibit patients from maintaining tight control of their blood sugar, thus depriving them of the key benefits intensive control offers, i.e. significant delay or prevention of long-term complications.

Healthcare professionals dealing with diabetes patients on a regular basis will be well attuned to the fear their patients have about hypoglycaemia. It is therefore important that glucagon administration is included as part of patient education programmes. Glucagon should be considered as a way of helping patients overcome their anxiety surrounding hypoglycaemia and become as expert as possible in achieving optimal control over their diabetes. Healthcare professionals can play a key role in ensuring that all patients and their relatives receive adequate training on hypoglycaemia and the use of glucagon.

The Novo Nordisk GlucaGen HypoKit provides a user friendly means of administering glucagon. For further information please call the Novo Nordisk Customer Care Line 0845 600 5055. (calls are charged at local rates and may be monitored for training purposes)

(1.) Pramming S. Thorsteinsson B. Bendtson I. Binder C. Diab Med 1991, 8: 217-222

(2.) DCCT Research Group. N Engl J Med 1993, 329: 977-86

(Prescribing information for GlucaGen HypoKit is on p. 3)

RELATED ARTICLE: ABBREVIATED PRESCRIBING INFORMATION FOR GLUCAGEN HYPOKIT 1MG

GlucaGen HypoKit 1mg Glucagon (rys)

Presentation: A vial containing 1mg (1 iu) glucagon (rys), as the hydrochloride, and lactose 107mg, together with a pre-filled syringe containing 1 ml Water for injections.

Uses: The treatment of severe hypoglycaemic reactions which may occur in the management of diabetic patients receiving insulin. As a motility inhibitor in examinations of the gastrointestinal tract. As a motility inhibitor in CT, NMR and DSA.

Dosage: The glucagon is dissolved in the accompanying diluent before use.

Treatment of severe hypoglycaemic reactions: 1mg (adults, children above 25kg or 6 - 8 years) or 0.5mg (children below 25kg or 6 - 8 years) by subcutaneous, intramuscular, or intravenous injection. When the patient responds, administer oral carbohydrate. If no response within 10 minutes, give intravenous glucose.

Diagnostic indications: Doses range from 0.1-2mg depending on the diagnostic technique used and the rank of administration. Usual dose for relaxation of stomach. duodenal bulb, duodenum and small bowel is 0.2-0.5mg i.v. or 1mg i.m. To relax the colon 0.5-0.75mg iv. or 2mg i.m. In CT, NMR and OSA doses up to 1mg i.v. are used.

Contra-indications: Phaeochromocytoma. Hypersensitivity to glucagon or excipients. Do not use if solution contains particles, or has viscous appearance.

Precautions: Glucagon reacts antagonistically towards insulin. Observe caution in patients with insulinoma or glucagonoma, and in diabetics or elderly patients with known cardiovascular disease.

Use in pregnancy: Glucagon does not cross the placenta. Glucagon has been used in pregnant diabetics; no harmful effects known with respect to course of pregnancy or health of foetus and neonate.

Side effects: Occasionally nausea and vomiting, which tends to be dose related. Positive inotropic and chronotropic effects (tachycardia). Rarely hypersensitivity.

PL numbers: GlucaGen 1mg PL 4668/0027

Diluent for GlucaGen 1mg fsyhnge) PL 4666/0026

Legal category: POM

Basic NHS price: GlucaGen HypoKit 1mg [pounds sterling]19.95 Full prescribing information can be obtained from: Novo Nordisk Limited, Broadfield Park, Brighton Road, Crawley, West Sussex RH 11 9RT. Tel: (01293) 613555

Date of Preparation: May 2002

HOW TO ADMINISTER GLUCAGEN: A STEP-BY-STEP GUIDE

1. Insert the needle through the rubber disk of the GlucaGen bottle and inject all of the contents of the syringe into the bottle.

2. Without withdrawing the syringe, gently shake the bottle until the GlucGen is completely dissolved.

3. Ensure that the plunger is first fully depressed. Then draw up the entire solution into the syringe.

4. Ensure there is no air remaining in the syringe before giving the injection. When the patient responds, give them a sweet drink or snack to prevent a further hypo.

COPYRIGHT 2002 S.B. Communications
COPYRIGHT 2003 Gale Group

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