Table 1.

Main features of the reported double-positive pediatric patients

Patient [ref]	Age (yr, sex)	Initial symptoms	Hb, g/dl	S-Cr, mg/dl		Anti-GBM Abs	ANCA type	Crescents, %	Treatment	F/U	Outcome
				onset F/U
1 [14]	17, f	URTI	~ 6.0	12.5	-	+	p/MPO	90	supportive	-	Death at presentation
2 [15]	12, f	-	7.4	0.5	0.3	+	p/MPO	13	MP, PE, CY	few weeks	Normal GFR
3 [16]	12, m	fever,rash, arthritis	10.6	7.4	2.1	+	p/MPO	>60	PE, MP, CY Pdn, MMF	18 mos	GFR 58.5 ml/min
4 [17]	4, f	epistaxis	n.a.	2.7	0.8	+	p/MPO	62	PE, MP, Pdn, CY	9 mos	Normal GFR
5 [18]	8, f	sore throat	2.8	7.7	-	-	p/MPO	83	supportive	-	Death at presentation
6 [6]	13, m	rash, sinusitis	n.a.	D	D	+	c/PR3	+	PE, Pdn, CY	n.a.	ESRD
7 [6]	17, f		n.a.	D	D	+	p/MPO	20	PE, CY, Pdn, Aza	n.a.	ESRD
8 [this report]	10, f	fever, malaise, legs pain	4.4	0.53	0.66	±	p/MPO	16	MP, CY, Pdn, MMF	10 mos	Normal GFR

URTI, upper respiratory tract infection; F/U, follow-up; Abs, antibodies; p, perinuclear; MPO, myeloperoxidase; PR3, proteinase-3; MP, methyl-prednisolone; PE, plasma exchange; CY, cyclophosphamide; GFR, glomerular filtration rate; Pdn, prednisone; MMF, mycophenolate mofetil; Aza, azathioprine; ESRD, end-stage renal disease; D, dialysis; S-Cr, serum creatinine concentration; GBM, glomerular basement membrane;

ANCA, anti-neutrophil cytoplasmic antibodies; n.a., not available; +, positive/present; -, negative/absent.