Faculty Bibliography

Juvenile Xanthogranuloma (JXG) is a dendritic cell related histiocytic disorder which usually presents in the first year of life as a solitary cutaneous granuloma. Isolated presentation in the upper airway is very rare but can result in severe respiratory distress, especially in young children. We present the case of a 5-month-old male with an isolated subglottic JXG lesion. Endoscopic excision provided symptomatic relief and avoided the need for tracheostomy. The lesion has completely resolved 17 months later. Surgical excision without tracheostomy was the treatment of choice in two of the four additional cases of upper airway JXG presented in the literature. JXG has an excellent prognosis with spontaneous regression over time. Histology alone is frequently inadequate to differentiate JXG from the more common Langerhans Cell Histiocytosis (LCH), which carries a much less favorable prognosis. The evolving field of immunohistochemistry provides an essential tool to establish the correct diagnosis. The typical phenotype of JXG is Factor XIIIa+/Fascin+/CD68+/CD163+/CD14+/CD1a-/S100-

Congenital tracheal web is a rare entity often misdiagnosed as refractory asthma. Clinical suspicion based on patient history, examination, and pulmonary function tests should lead to its consideration. Bronchoscopy combined with CT imaging and multiplanar reconstruction is an accepted, highly sensitive means of diagnosis

Two adolescents presented with a history of dyspnea upon exertion and cough. In both cases, the chest X-ray and pulmonary function testing, including flow-volume loop, were normal. A bronchial tumor was diagnosed by CT scan, which was ordered after each patient had an episode of hemoptysis. The sedimentation rate was the only abnormal laboratory test in both cases. Mucoepidermoid carcinoma of the bronchus, a rare tumor in childhood, was found at pathology in both cases. There was no evidence of metastases to local lymph nodes or distal sites. There were 47 previously reported cases in children. Recurrent pneumonia and persistent cough were the most common presenting findings. These tumors are of low-grade malignant potential but they can become locally invasive, extending into cartilage and surrounding soft tissue. Prognosis is good with complete resection

BACKGROUND: Although cystic fibrosis is the most common genetic disorder of children, its heterogeneous spectrum of severity lends itself to underdiagnosis in the older adult patient population where the index of suspicion is not high. METHODS: We report a 60-year-old Hispanic man with asthma who presented with progressive dyspnea and wheezing unresponsive to inhaled corticosteroid treatment. Additionally, he had clinical findings and a past history suggestive of cystic fibrosis. Skin testing, radiography and laboratory studies were completed to evaluate for allergic bronchopulmonary aspergillosis (ABPA) and cystic fibrosis. RESULTS: Test results revealed peripheral eosinophilia and hyper IgE. Skin testing to Aspergillus fumigatus (Af) was positive. IgG, IgM, and Af specific antibodies were present. High resolution CT scan showed central bronchiectasis. Sweat tests were positive on two separate occasions and gene analysis showed our patient to have a positive gene mutation at D127ON/D127ON. CONCLUSION: Cystic fibrosis should be suspected in the older adult patient with a compatible clinical presentation.

Continuous arteriovenous hemofiltration (CAVH) has been used infrequently in the treatment of infants and children with acute renal failure. In this report we describe the use of CAVH in a critically ill neonate who was unable to be managed with either peritoneal dialysis or hemodialysis. The procedure was safe and effective in the management of fluid overload and multiple electrolyte disturbances including life-threatening hyperkalemia. The technical aspects of this procedure are described, and its potential benefits and complications are assessed with reference to the ten previously reported cases