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Papers of the Week

2022 Jan

Transl Pediatr



Successful treatment of polyarteritis nodosa with intestinal necrosis in a Chinese boy: a case report.


Guo R, Liu H, Liu X, Liu W, Zhang S
Transl Pediatr. 2022 Jan; 11(1):157-162.
PMID: 35242662.


Polyarteritis nodosa (PAN) is a systemic necrotizing inflammatory disease of the medium and small arteries which has variable clinical manifestations, course, and organ involvement. Intestinal necrosis resulting from PAN is rare, and successful treatment of such cases is even more uncommon. Here, we report the first successful treatment of PAN with intestinal necrosis in a young Chinese child. A 5-year-old boy was admitted to our hospital with a 5-day history of abdominal pain. The patient underwent an emergency exploratory laparotomy, which showed skipping necrosis of the intestinal wall. Intestinal resection and anastomosis failed to improve his abdominal pain. The patient's blood pressure remained high (140/120 mmHg), despite captopril treatment. The erythrocyte sedimentation rate (ESR) and D-dimer level were also elevated at (106.00 mm/h and 11.16 mg/L, respectively), as was the 24-hour urine protein (197.6 mg/24 h). Echocardiography revealed that the diameters of the left and right coronary arteries were increased (0.45 and 0.49 cm, respectively). Ultrasound showed polyarteritis in the anterior tibial, radial, iliac, and renal arteries. Histopathologic examination revealed elastic fiber rupture and partial mesenteric arteriolar stenosis with occlusion. After 2 months of treatment with systemic methylprednisolone, cyclophosphamide, and prednisone, the patient's abdominal pain was relieved. Furthermore, the patient's ESR and D-dimer levels had reduced to 5 mm/h and 0.63 mg/L, respectively; his 24-hour urine protein was normal (60.0 mg/24 h); and his blood pressure had dropped to 101/46 mmHg. The left and right coronary arteries had reduced to 0.35 and 0.38 cm in diameter, respectively; however, no significant improvement was observed in the other vessels involved. Unfortunately, the child's parents did not continue to seek medical attention; therefore, his long-term outcome is unknown. In this case, the patient was operated on immediately after symptom onset. The postoperative infection was also quickly controlled, avoiding the occurrence of septic shock. Vascular B-ultrasound and pathology aided in establishing a clear and timely diagnosis, which allowed systematic medical treatment to be delivered, achieving good short-term results.