Fatal Polyarteritis Nodosa With Gastrointestinal Involvement in a Child

• Published in: Pediatric emergency care Vol. 22; no. 12; pp. 810 - 812
• Main Authors: Kendirli, Tanl, Yüksel, Selçuk, Oral, Mehmet, Ünal, Necmettin, Tulunay, Melek, Dilek, Umman Şanl, Yalçnkaya, Fatoş
• Format: Journal Article
• Language: English
• Published: Hagerstown, MD Lippincott Williams & Wilkins, Inc 01-12-2006; Lippincott Williams & Wilkins
• Subjects: Adolescent; Anesthesia. Intensive care medicine. Transfusions. Cell therapy and gene therapy; Aneurysm - etiology; Aneurysm - surgery; Biological and medical sciences; Blood. Blood and plasma substitutes. Blood products. Blood cells. Blood typing. Plasmapheresis. Apheresis; Child; Fatal Outcome; Gastrointestinal Diseases - complications; Gastrointestinal Diseases - therapy; Hepatic Artery; Humans; Intensive care medicine; Male; Medical sciences; Mesenteric Artery, Superior; Polyarteritis Nodosa - complications; Polyarteritis Nodosa - therapy; Psychology. Psychoanalysis. Psychiatry; Psychopathology. Psychiatry; Sepsis - drug therapy; Sepsis - microbiology; Transfusions. Complications. Transfusion reactions. Cell and gene therapy; Victimology; Human; Intensive care; Aneurysm; gastrointestinal involvement; Cardiovascular disease; Periarteritis nodosa; Scintigraphy; Gastrointestinal; Vascular disease; Vasculitis; Gastrointestinal bleeding; Systemic disease; Digestive diseases; Intestinal disease; gastrointestinal bleeding scintigraphy; Child; Resuscitation; polyarteritis nodosa
• ISSN: 0749-5161; 1535-1815
• DOI: 10.1097/01.pec.0000245172.38967.d0

Polyarteritis nodosa (PAN) is a necrotizing angiitis that predominantly affects small- and medium-sized arteries. Polyarteritis nodosa occurs rarely during childhood. Boys and girls seem to be equally affected, with a peak at the age of 10 years. Rarely, severe and fatal gastrointestinal involvement is seen in PAN. Here, we report a 15-year-old boy with PAN, who had gastrointestinal involvement with multiple aneurysms of the hepatic and superior mesenteric arteries. This involvement could be demonstrated with conventional angiography and gastrointestinal bleeding scintigraphy. The progression of the symptoms and the decrease in the size of the aneurysms were noted after combination treatment with cyclophosphamide and prednisolone, but there was severe bleeding from small bowel, and it was taken under control by resection of jejunum. However, the patient died because of sepsis. In conclusion, severe gastrointestinal involvement in PAN is usually fatal despite aggressive therapy, as is the case in our patient.