Risk of nephrogenic systemic fibrosis: evaluation of gadolinium chelate contrast agents at four American universities.

PURPOSE: To retrospectively determine the benchmark incidence of nephrogenic systemic fibrosis (NSF) related to the confirmed use of different gadolinium chelate contrast agents at four U.S. university tertiary care centers. MATERIALS AND METHODS: Institutional review board approval was obtained for this HIPAA-compliant multi-institutional study; the requirement for informed patient consent was waived. Patients who had a diagnosis of NSF between January 2000 and December 2006 were identified at four tertiary care centers with renal transplant and dialysis services. A standard checklist was used to acquire reliable data from the four centers. The diagnosis of NSF was confirmed histopathologically in all patients. The association of NSF development with gadolinium chelate contrast agent administration in each patient was assessed. The type and cumulative dose of contrast agent administered to each patient with NSF were determined at each center by using the standard checklist. The benchmark incidence of NSF was determined and expressed as the ratio of the number of patients with NSF who had undergone gadolinium chelate-enhanced magnetic resonance (MR) imaging, relative to the total number of patients who underwent gadolinium chelate-enhanced MR imaging at each tertiary care center. Benchmark incidences of NSF were compared among the four centers by using Fisher exact tests. RESULTS: Gadodiamide was used at University of North Carolina at Chapel Hill (center A) and Emory University (center B), and gadopentetate dimeglumine was used at Wake Forest University (center C) and Thomas Jefferson University (center D) during the study period. Twenty-three patients at center A, nine patients at center B, three patients at center C, and one patient at center D had NSF and had undergone gadolinium chelate-enhanced MR imaging. The incidence of NSF was one in 2913 patients who underwent gadodiamide-enhanced MR examinations and one in 44,224 patients who underwent gadopentetate dimeglumine-enhanced MR examinations. CONCLUSION: The benchmark incidence of NSF was much greater at the two centers where gadodiamide was used than at the two centers where gadopentetate dimeglumine was used.

Intense pruritus and failure to thrive in Alagille syndrome.

A 4-year-old girl with failure to thrive and intense pruritus associated with Alagille syndrome is described. The clinical features of this autosomal dominant disorder are reviewed. This case highlights the importance of recognizing the severe pruritus as a manifestation of the disease.

Recurrent syncope and anaphylaxis as presentation of systemic mastocytosis in a pediatric patient: case report and literature review.

Mastocytosis refers to a rare collection of disorders, both cutaneous and systemic, that are characterized by increased numbers of mast cells. Depending on the extent of the disease, these disorders may present with symptoms resulting from mast cell degranulation including flushing, diarrhea, vomiting, cramping, syncope, or anaphylaxis. In pediatric patients, cutaneous involvement is most prevalent in the form of urticaria pigmentosa, which is typically asymptomatic or minimally so with resolution by adolescence. In this case report and review of literature, we review a case of a 3-year-old child with uritcaria pigmentosa displaying recurrent syncope and anaphylaxis as the first presentation of systemic mastocytosis. We found data to be limited on this topic, and concluded that pediatric patients with prior diagnoses of cutaneous mastocytosis could benefit from either more aggressive screening for systemic disease or prophylactic treatment with antihistamines and rescue subcutaneous epinephrine.