Primary Hyperoxaluria Type 1 in 18 Children: Genotyping and Outcome

Table 2

Management and outcome.

Number	Supportive; therapy	Follow-up period (year)	RRT	Transplant	Outcome
Ia	C, F, P, ESWL	13	HD	—	Alive
Ib	C, F, P	7.5	HD	LUL	Alive
Ic	C, P	3.5	HD	—	Died
IIa	C, F, P, ESWL	13	HD	LRL and 2 LRK	Alive
IIb	C, P	2	PD	—	Died
IIc	C, F, P	9	HD	LRL and LRK	Alive
IId	C, F, P	3	—	—	Died
IIIa	C, P	4.5	HD	—	Died
IIIb	C, P	3.5	HD	—	Died
IIIc	C, F, P	4	—	LRL	Alive
IIId	C, P	2.5	HD	—	Alive
IVa	C, F, P, ESWL	9	HD	LUL	Died
IVb	C, P	1.5	—	—	Died
IVc	C, F, P	9	Supportive	—	^*47
IVd	C, F, P	1.5	Supportive	—	^*16
IVe	C, P	1	PD	—	Died
IVf	C, F, P	4	Supportive	—	^*64
IVg	C, P	2	PD	—	Died

Family number 1 (I), family number 2 (II), family number 3 (III), and family number 4 (IV). ESRD: end-stage renal disease, C: citrate, F: fluid, P: pyridoxine, ESWL: electric shock wave lithotripsy, CKD: chronic kidney disease, HD: hemodialysis, PD: peritoneal dialysis, LRL: living-related liver transplant, LRK: living-related kidney transplant, LUL: living-unrelated liver transplant, and ^*estimated glomerular filtration rate (eGFR mL/min/m²).