Glucagon therapy as a possible cause of erythema necrolyticum migrans in two neonates with persistent hyperinsulinaemic hypoglycaemia

Wald, Martin; Lawrenz, Karin; Luckner, Doris; Seimann, Richard; Mohnike, Klaus; Schober, Edith

doi:10.1007/s00431-002-1022-9

Cited by 25 publications

(14 citation statements)

References 25 publications

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“…Despite controversy in the past regarding the specific cause of MNE, recent studies show glucagon infusions can lead directly to MNE83-85. However, MNE is not specific for glucagonoma occurring also in celiac disease, cirrhosis or pancreatitis81, 85, 86.…”

Section: Clinical Features and Diagnosis Of Petsmentioning

confidence: 99%

Gastrointestinal Neuroendocrine Tumors: Pancreatic Endocrine Tumors

2008

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Pancreatic endocrine tumors (PETs) have long fascinated clinicians and investigators despite their relative rarity. Their clinical presentation varies depending upon whether the tumor is functional or not and also according to the specific hormonal syndrome produced. Tumors may be sporadic or inherited but little is known about their molecular pathology, especially the sporadic forms. Chromogranin A appears to be the most useful serum marker for diagnosis, staging and monitoring. Initially, therapy should be directed at the hormonal syndrome as this has the major initial impact on the patient's health. Most PETs are relatively indolent but ultimately malignant, except for insulinomas which are predominantly benign. Surgery is the only modality that offers the possibility of cure although it is generally noncurative in patients with Zollinger-Ellison syndrome or nonfunctional PETs with MEN1. Preoperative staging of disease extent is necessary to determine the likelihood of complete resection though debulking surgery is often felt to be useful in unresectable patients. Once metastatic, biotherapy is usually the first modality employed because it is generally well tolerated. Systemic or regional therapies are generally reserved until symptoms occur or tumor growth is rapid. Recently a number of newer agents, as well as receptor-directed radiotherapy, are being evalulated for patients with advanced disease. This review addresses a number of recent advances regarding the molecular pathology, diagnosis, localization and management of PETs including discussion of peptide receptor radionuclide therapy and other novel antitumor approaches. We conclude with a discussion of future directions and unsettled problems in the field.

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Section: Clinical Features and Diagnosis Of Petsmentioning

confidence: 99%

Gastrointestinal Neuroendocrine Tumors: Pancreatic Endocrine Tumors

2008

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“…Because glucagon has actions on a number of other tissues, side effects of therapy include nausea, vomiting, increased growth hormone concentrations, increased myocardial contractility, and decreased gastric acid/pancreatic enzyme secretions. In high doses glucagon causes tachyphylaxis and erythema necrolyticum migrans (8,325).…”

Section: A Glucagonmentioning

confidence: 99%

Hyperinsulinism in Infancy: From Basic Science to Clinical Disease

Dunne

Cosgrove²,

Shepherd³

et al. 2004

Physiological Reviews

262

277

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Dunne, Mark J., Karen E. Cosgrove, Ruth M. Shepherd, Albert Aynsley-Green, and Keith J. Lindley. Hyperinsulinism in Infancy: From Basic Science to Clinical Disease. Physiol Rev 84: 239–275, 2004; 10.1152/physrev.00022.2003.—Ion channelopathies have now been described in many well-characterized cell types including neurons, myocytes, epithelial cells, and endocrine cells. However, in only a few cases has the relationship between altered ion channel function, cell biology, and clinical disease been defined. Hyperinsulinism in infancy (HI) is a rare, potentially lethal condition of the newborn and early childhood. The causes of HI are varied and numerous, but in almost all cases they share a common target protein, the ATP-sensitive K+ channel. From gene defects in ion channel subunits to defects in β-cell metabolism and anaplerosis, this review describes the relationship between pathogenesis and clinical medicine. Until recently, HI was generally considered an orphan disease, but as parallel defects in ion channels, enzymes, and metabolic pathways also give rise to diabetes and impaired insulin release, the HI paradigm has wider implications for more common disorders of the endocrine pancreas and the molecular physiology of ion transport.

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“…As possible side effects of glucagon treatment, erythema necrolyticum migrans has been described before in 2 of our patients [25]. This occurred during both intravenous and subcutaneous glucagon administration, but disappeared after discontinuation of the drug.…”

Section: Discussionmentioning

confidence: 96%

“…In 2 children (patients 8 and 9) erythema necrolyticum occurred but resolved without scarring after glucagon was stopped [25]. …”

Section: Methods and Resultsmentioning

confidence: 99%

Long-Term Non-Surgical Therapy of Severe Persistent Congenital Hyperinsulinism with Glucagon

et al. 2008

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Background: Congenital hyperinsulinism (CHI) is characterized by severe hypoglycemia caused by dysregulated insulin secretion. The long-term outcome is dependent on prevention of hypoglycemic episodes to avoid the high risk of permanent brain damage. Severe cases are usually resistant to diazoxide or nifedipine. In addition, somatostatin analogues are ineffective in a subgroup of patients to achieve stable euglycemia. In these infants the only remaining long-term option has been subtotal pancreatectomy with high risk of diabetes mellitus. Intravenous infusions of glucagon are used as immediate treatment to stabilize euglycemia in affected newborns. The rationale of this treatment comes from the observation of an increased glycogen content of the liver when glycogenolysis is inhibited by insulin. Objective: To review the efficacy and safety of long-term subcutaneous glucagon infusion as a potential therapeutic option for blood glucose stabilization in infants with severe CHI without the need of additional intravenous glucose or immediate surgical intervention. Method: Retrospective review of 9 children with CHI who received continuous subcutaneous infusion of glucagon for weeks or months. Glucagon was added to octreotide to replace octreotide-induced suppression of endogenous glucagon secretion, thereby liberating glucose by stimulation of hepatic glycogenolysis. In 3 cases, a stabilized formulation of glucagon was used to prevent glucagon crystallization that frequently occurs in smaller volumes. Results: Introduction of glucagon allowed the reduction or discontinuation of central glucose infusion in all children studied. In 2 patients, glucagon was introduced due to recurrent hypoglycemia despite subtotal pancreatectomy. Six out of 9 children were discharged home on this treatment, which their parents were able to continue without further symptomatic hypoglycemia, convulsions or unconsciousness. In 3 children, subcutaneous glucagon was continuously administered for 1–4 years leading to stable euglycemia. However, 2 children with diffuse type still required subtotal pancreatectomy. As a possible side effect, 2 children developed erythema necrolyticum, which resolved after discontinuation of the glucagon infusion. This has been described before in glucagonoma. Conclusion: In this retrospective series, combination therapy of low-dose octreotide and subcutaneous glucagon infusion has been effective in preventing hypoglycemic episodes in severe CHI. We propose this may serve as a therapeutic option in place of high rates of glucose infusion through a central venous catheter and as an alternative to subtotal pancreatectomy in diffuse type of CHI.

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Glucagon therapy as a possible cause of erythema necrolyticum migrans in two neonates with persistent hyperinsulinaemic hypoglycaemia

Abstract: erythema necrolyticum migrans should be considered as a differential diagnosis in patients who develop erythematosquamous skin lesions under glucagon treatment.

Cited by 25 publications

References 25 publications

Gastrointestinal Neuroendocrine Tumors: Pancreatic Endocrine Tumors

Gastrointestinal Neuroendocrine Tumors: Pancreatic Endocrine Tumors

Hyperinsulinism in Infancy: From Basic Science to Clinical Disease

Long-Term Non-Surgical Therapy of Severe Persistent Congenital Hyperinsulinism with Glucagon

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