title: Systemic sarcoidosis after cardiac transplantation in a 9-year-old child.
abstract: Sarcoidosis is a granulomatous disease of unknown etiology and is only rarely seen in infants and children. We present the case of a 9-year-old boy who developed sarcoidosis with multi-organ involvement 9 years after cardiac transplantation for Shone complex. The patient was on immunosuppressive therapy with tacrolimus and mycophenolate mofetil. He presented with severe respiratory distress due to marked mediastinal lymphadenopathy and bilateral pulmonary infiltrates in association with fatigue, low-grade fever, hepatosplenomegaly and generalized lymphadenopathy. Lymph node histology showed non-caseating epitheloid cell granulomas and giant cells. Initialization of therapy with prednisolone resulted in prompt clinical recovery and resolution of all symptoms except for the development of mild pulmonary fibrosis. Tapering of the steroids led to recurrence of mediastinal lymphadenopathy 5 months after the initial disease, which responded to an increase in steroid dose. The clinical course, the medical management, and the possible role of immunosuppression in the etiology of the disease are discussed.
authors.affiliation: Department of Pediatric Cardiology, University Children's Hospital Giessen, Giessen, Germany. ulrike.bartram@paediat.med.uni-giessen.de
authors.forename: U
authors.initials: U
authors.lastname: Bartram
authors.forename: J
authors.initials: J
authors.lastname: Thul
authors.forename: J
authors.initials: J
authors.lastname: Bauer
authors.forename: W
authors.initials: W
authors.lastname: Wössmann
authors.forename: D
authors.initials: D
authors.lastname: Schranz
mesh_terms: D002648:Child; D004305:Dose-Response Relationship, Drug; D005938:Glucocorticoids / Q000008:administration & dosage / Q000627:therapeutic use; D016027:Heart Transplantation; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008198:Lymph Nodes / Q000473:pathology; D008297:Male; D009173:Mycophenolic Acid / Q000031:analogs & derivatives / Q000627:therapeutic use; D011183:Postoperative Complications / Q000175:diagnosis* / Q000188:drug therapy; D011239:Prednisolone / Q000008:administration & dosage / Q000627:therapeutic use; D013902:Radiography, Thoracic; D012008:Recurrence; D019233:Retreatment; D012507:Sarcoidosis / Q000175:diagnosis* / Q000188:drug therapy; D016559:Tacrolimus / Q000627:therapeutic use; D014057:Tomography, X-Ray Computed
chemical_list: D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D011239:Prednisolone; D009173:Mycophenolic Acid; D016559:Tacrolimus