Faculty Bibliography

OBJECTIVE: To evaluate medical treatment for hemangiomas involving the parotid area with or without other areas of involvement. DESIGN: Retrospective analysis of pediatric patients treated medically for proliferative hemangiomas of the parotid region with or without hemangiomas in other regions. Indications for treatment included respiratory symptoms relating to hemangiomas of the upper airway, difficulty feeding, rapid rate of growth of the hemangioma, and deformity or obstruction of the ear canal. SETTING: New York University Multidisciplinary Vascular Anomaly Conference, New York, NY, and the Pediatric Oncology Department of Ospedale Pediatrico Bambino Gesu, Rome, Italy. PATIENTS: Thirteen patients with proliferative hemangiomas in the parotid area were treated medically to inhibit growth and enhance involution of the hemangioma. INTERVENTION: Six patients were treated with corticosteroids alone (2-4 mg/kg daily). Two patients were treated with corticosteroids (2-4 mg/kg daily) followed by interferon alfa-2a (3 million U/m2 daily) because of a failure to respond to corticosteroid therapy. One patient was treated with interferon alfa-2a alone (3 million U/m2 daily). Four patients were initially treated with interferon alfa-2a, then treated with corticosteroids. One of these patients required intralesional corticosteroid therapy for a massively enlarged lip and is therefore included in this group. The other patient was given oral corticosteroids for unknown reasons at another institution. In the remaining 2 patients, there was no response to the use of interferon alfa-2a. MAIN OUTCOME MEASURES: The size, bulk, and symptoms relating to the hemangiomas of the patients were assessed. RESULTS: None of the patients had a significant improvement of the lesions of the parotid hemangiomas. In contrast, for those patients with clinical symptoms due to hemangiomas elsewhere or with cutaneous involvement typical of hemangiomas, the symptoms improved with either of the above therapies, and the cutaneous areas demonstrated signs of involution. CONCLUSIONS: The results in the 13 patients in this article demonstrate that hemangiomas in certain anatomic sites, such as the parotid area, may be more resistant to therapy with corticosteroids or interferon alfa-2a. Differences in drug metabolism, caliber of blood vessels, and/or blood flow in the parotid gland may account for this observation

PURPOSE: Yersinia enterocolitica sepsis is rarely encountered in patients without an underlying susceptibility and is most frequently reported in iron-overloaded patients. This is thought to be related to the unusual utilization of iron by this microorganism. We report a case of Y. enterocolitica bacteremia in a chronically transfused adolescent with sickle cell anemia. This type of serious infection in sickle cell disease is previously unreported. A description of the case and the relationship between Y. enterocolitica and iron is discussed. A review of the literature is presented. RESULTS: Y. enterocolitica can cause a severe septicemia, and increased virulence of this organism has been shown to correlate with increased iron burden and/or use of the chelator deferoxamine. It may also occur as a consequence of a contaminated blood transfusion. CONCLUSIONS: We believe our case demonstrates that Y. enterocolitica should be considered a possible pathogen in febrile chronically transfused patients with sickle cell disease. Broad antibiotic coverage should be initiated and deferoxamine discontinued pending results of cultures

Recently, a novel class of angiostatic steroids which block angiogenesis in several systems has been described. Since the elaboration of proteases is believed to be an important component of angiogenesis, we tested whether these steroids blocked the fibrinolytic response of endothelial cells to the angiogenic protein, basic fibroblast growth factor [bFGF]). Cultured bovine aortic endothelial (BAE) cells were incubated with bFGF and/or medroxyprogesterone acetate (MPA), an angiostatic steroid which has been shown to inhibit vascularization, collagenolysis, and tumor growth. When bFGF (3 ng/ml) was added to confluent monolayers of BAE cells, plasminogen activator (PA) activity in the medium was increased threefold. In contrast, MPA at 10(-6) M, 10(-7) M, 10(-8) M, and 10(-9) M decreased PA levels in the medium by 83%, 83%, 75%, and 39%, respectively. The stimulation of PA levels in BAE cells by bFGF (3 ng/ml) was abrogated by the presence of 10(-6) M MPA. This decrease in PA activity was found to be mediated by a significant increase in plasminogen activator inhibitor type-1 (PAI-1) production. MPA, therefore, negated one of the important enzymatic activities associated with the angiogenic process. In contrast to the decreased levels of secreted PA in cultures exposed simultaneously to MPA and bFGF, cell-associated PA levels remained high, consistent with earlier observations indicating that PAI-1 does not inhibit cell-associated PA. Thus, angiostatic steroids may exert their inhibitory effects on angiogenesis by increasing the synthesis of PAI-1. This, in turn, inhibits PA activity and, therefore, plasmin generation, which is essential for the invasive aspect of angiogenesis