Immune-complex glomerulonephritis with a membranoproliferative pattern in Frasier syndrome: a case report and review of the literature

Table 2 Summary of renal manifestations in Denys-Drash syndrome with Wilms tumor

Literature	Karyotype	Phenotype	WT onset (m)	NS or Proteinuria onset (m)	Renal histology	Tumor therapy	Renal outcomes after surgical excision
Drash A (1970) [12]	ND	Ambiguous genitalia	21	12	DMS or MPGN^a	Right nephrectomy	ESRD at age 7.5 yr
	46XY	Incomplete female genitalia		2	MPGN		ESRD at age 3.4 yr, incidental WT on autopsy
Spear GS (1971) [13]	46XY	Ambiguous genitalia	9	4	MPGN, IgG + C3		incidental WT on autopsy at age 4 m
Barratt TM (1978) [14]	46XY	Ambiguous genitalia	70	39	MPGN, Full-house	Right nephrectomy
McCoy FE (1983) [15]	46 XY	Ambiguous genitalia	35	35	MPGN	Right nephrectomy + V, A	ESRD
Thorner P (1984) [16]	ND	Female	22	22	MPGN	Right total & left partial nephrectomy	Proteinuria persisted Circulating IC vanished after nephrectomy
Scully RE (1998) [17]	46 XY^b	Aniridia	17	156	FSGS	V, A + radiation	Proteinuria persisted

WT Wilms tumor, NS nephrotic syndrome, ND not determined, DMS diffuse mesangial sclerosis, MPGN membranoproliferative glomerulonephritis, ESRD end-stage renal disease, V Vincristine, A actinomycin, IC immune complex, FSGS focal segmental glomerulosclerosis
^a Histology for this patient (case 1) was re-evaluated by Spear GS et al.; J Pediatr 1971
^b Suspected 11p13 deletion
WT mutations were not defined in the listed cases

ISSN: 1471-2369