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N Orfan

Publications and source records attributed to N Orfan.

7 recordsLinked to original sources

Immunologic analysis of anaphylaxis to protamine component in neutral protamine Hagedorn human insulin.

We report the clinical and immunologic analysis of two patients with diabetes who had anaphylaxis to neutral protamine Hagedorn (NPH) human insulin in the absence of allergy to regular insulin. A 36-year-old woman without a recent history of local insulin reactions or interruption of insulin therapy experienced anaphylaxis within 15 minutes of her usual morning dose of subcutaneously administered NPH human insulin. A 62-year-old man with a history of generalized reactions to NPH human insulin and of anaphylaxis to intravenously administered protamine had generalized urticaria after injection of NPH human insulin. Both patients subsequently tolerated Lente human insulin. Skin test results in both patients were negative to regular and Lente insulin preparations but positive to NPH insulin and to protamine at concentrations tested. In vitro assays demonstrated that both patients had markedly elevated serum levels of IgE and IgG to protamine, but not to regular human insulin, and that their IgE antibodies to protamine recognized protamine antigenic determinants in NPH human insulin. We conclude that the anaphylactic reactions to NPH insulin in our patients were mediated by IgE to protamine, which should be a pathogenetic consideration in the evaluation of immediate-type reactions to protamine-containing insulins.

Adult↗

Malignant idiopathic anaphylaxis: three additional cases and extended evaluation.

Northwestern University's Division of Allergy and Immunology (NUAI) has evaluated and treated 225 patients with idiopathic anaphylaxis (IA) over a period of 16 years. Four patients have been identified with malignant IA. The term "malignant" is used to identify those patients with the most severe form of IA that is resistant to standard therapy. The diagnosis of malignant IA is made in patients with IA whose controlling oral corticosteroid dose was not able to be reduced below 60 mg of prednisone (or its equivalent) every other day or 20 mg of prednisone (or its equivalent) every day without an exacerbation of IA. Presented here is the long-term evaluation of one patient as well as three additional patients with malignant IA managed by our service. At the time of this report we have had no deaths due to IA in patients treated with pharmacologic regimen.

Adrenal Cortex Hormones↗

Systemic cold urticaria in a five-year-old boy.

A 5-year-old white boy had a history of generalized urticaria on total body exposure to a cold environment. Standard ice cube testing was negative. Plasma analysis revealed the presence of cryofibrinogen. Systemic cold challenge with serial plasma assays for complement, histamine, and prostaglandin D2 disclosed an elevation and peak of plasma histamine and prostaglandin D2 levels after the onset of generalized urticaria with no change in serum complement levels.

Child↗

Severe angioedema related to ACE inhibitors in patients with a history of idiopathic angioedema.

Angiotensin converting enzyme (ACE) inhibitors have been associated with the onset of angioedema in a small subset of treated patients. The angioedema commonly involves the face and oropharyngeal tissues and may result in life-threatening airway compromise. The mechanism by which ACE inhibitors precipitate angioedema has not been well-defined, and retrospective analysis of reported cases has failed to identify a group of patients at high risk. We report four cases of ACE inhibitor-related angioedema that required immediate medical intervention. All four cases occurred in patients with a prior history of idiopathic angioedema, an otherwise uncommon clinical entity. These observations suggest that patients with a history of idiopathic angioedema are at increased risk for the development of ACE inhibitor-related angioedema and should be treated cautiously with this class of drugs.

Adult↗

Emergency medical recognition and management of idiopathic anaphylaxis.

Idiopathic anaphylaxis (IA) is a diagnosis of exclusion that is made when no identifiable causative factors can be found for an episode of anaphylaxis. IA is a potentially life-threatening disease that is the result of a nonimmunologic mast cell activation syndrome. Acute presentation and treatment of these patients is most often in the emergency department and is clinically the same as anaphylaxis from allergens. Since these episodes are unpredictable and often recurrent, these patients are at risk of death if not identified on acute presentation and managed appropriately. As an increasing number of patients are being diagnosed with IA, they will be presenting to emergency departments with initial and recurrent episodes of IA. Therefore, increased awareness of IA and coordinated care is needed so that the morbidity and mortality of this potentially fatal disease can be kept at a minimum.

Acute Disease↗