儿童与成人原发性IgA肾病患者临床及病理特征比较及其临床意义

doi:10.13481/j.1671-587X.20230322

Abstract

Abstract:

Objective To discuss the clinical and pathological characteristics of IgA nephropathy （IgAN） in the children and adults， and to clarify their clinical significances. Methods The clinical and pathological data of the patients diagnosed with primary IgAN were collected. According to their ages， the patients were divided into children group （n=160） and adult group （n=240）. The ages，genders，incidences of hypertension，time from onset to renal biopsy，estimated glomerular filtration rates，initial onset manifestations，clinical types，Lee’s grades， MEST-C scores，immunofluorescence types and ultrastructures of glomeruli of the patients in two groups were compared. Results The ratio of male to female in children group was 1.5∶1 and it was 1.1∶1 in adults group； compared with adult group， the incidence of hypertension and the time from onset to renal biopsy of the patients in child group were decreased （P<0.05）， while the eGFR was increased（P<0.05）. Compared with adult group， the percentages of gross hematuria and edema of the patients in child group were increased（P<0.01）， and the percentages of abnormal urine test， foam urine， and other manifestations were decreased（P<0.01）.Compared with adult group， the percentages of the patients with isolated hematuria and nephrotic syndrome in child group were increased （P<0.01）， while the percentages of the patients with isolated proteinuria and chronic nephritis were decreased （P<0.01）；compared with adult group， the percentage of the patients with grade Ⅱ in the Lee’s grades in child group was increased（P<0.01） and the percentage of the patients with grade Ⅴ in the Lee’s grades was decreased （P<0.01）.The light microscope obervation results showed that there were only focal mesangial cells and mesangial matrix hyperplasia in grade Ⅱ renal tissue of the patients in child group， rarely accompanied by glomerulosclerosis. The renal tubular and interstitial lesions were not significant； there were significant proliferations of the mesangial cells and matrix in grade Ⅴ renal tissue of the patients in adult group， with more glomerulosclerosis， and relatively severe renal tubular and interstitial lesions. Compared with adult group， the percentages of the patients with M1 and E1 in the MEST-C scores of the patients in child group were increased （P<0.05 or P<0.01）， and the percentages of the patients with S1 and T1/T2 were decreased （P<0.01）.The urinary protein grade of the patients in child group was positively correlated with M（r_s=0.462），E（r_s=0.342），and C（r_s=0.250）scores （P<0.01）；the urinary protein grade of the patients in adult group was positively correlated with M（r_s=0.217），E（r_s=0.145），S（r_s=0.187），T（r_s=0.269），and C（r_s=0.256）scores （P<0.01）；compared with adult group， the IgA+IgG+IgM deposition of the patients in child group was increased（P<0.05）， deposition rate of C3 was increased（P<0.01）， and the deposition rate of fibrinogen （Fib） was increased（P<0.01）. Conclusion There are significant differences in the clinical and pathological characteristics between the children and the adults with primary IgAN， which should be treated differently in the clinical diagnosis， treatment， and research.

Key words: Primary IgA nephropathy, Child, Adult, Clinical type, Lee’s grade

CLC Number:

R692.3

Siyu DAI,Dan SONG,Chundong SONG,Fengyang DUAN,Ningli WANG,Ting GUO,Bo ZHANG,Xiaoqing YANG,Hong XU. Comparisons of clinical and pathological characteristics between children and adults with primary IgA nephropathy and their clinical significances[J].Journal of Jilin University(Medicine Edition), 2023, 49(3): 722-732.

Figures/Tables 11

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References 34

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Group	n	Gross hematuria	Abnormalurine tset	Foam urine	Edema	Other manifestations
Child	160	88(55.0)^*	7(4.4)^*	5(3.1)^*	52(32.5)^*	8(5.0)^*
Adult	240	32(13.3)	80(33.3)	38(15.8)	49(20.4)	41(17.1)
χ²			123.985
P			<0.01
^*P<0.01 compared with adult group.

Group	n	Clinical type
Group	n	Isolated hematuria	Isolatedproteinuria	Hematuria and proteinuria	Acute nephritis	Nephrotic syndrome	Rapidly progressive nephritis	Chronic nephritis
Child	160	19(11.9)^*	2(1.3)^*	76(47.5)	10(6.3)	48(30.0)^*	2(1.3)	3(1.9)^*
Adult	240	4(1.7)	30(12.5)	111(46.3)	10(4.2)	43(17.9)	3(1.3)	39(16.3)
χ²				58.505
P				<0.01
^*P<0.01 compared with adult group.

Group	n	Lee’s grade
Group	n	Ⅰ	Ⅱ	Ⅲ	Ⅳ	Ⅴ
Child	160	3(1.9)	35(21.8)^*	89(55.6)	31(19.4)	2(1.3)^*
Adult	240	4(1.7)	29(12.1)	119(49.6)	65(27.1)	23(9.6)
χ²			19.494
P			0.001
^*P<0.05 compared with adult group.

Group	n	M score				E score				S score
Group	n	M0		M1		E0		E1		S0		S1
Child	160	41(25.6)		119(74.4)^*		90(56.3)		70(43.7)^**		104(65.0)		56(35.0)^**
Adult	240	87(36.3)		153(63.7)		198(82.5)		42(17.5)		83(34.6)		157(65.4)
χ²		4.980				32.813				35.677
P		0.026				<0.01				<0.01
Group	n		T score				C score
			T0		T1/T2		C0		C1		C2
Child	160		148(92.5)		12(7.5)^**		53(33.1)		83(51.9)		24(15.0)
Adult	240		178(74.2)		62(25.8)		97(40.4)		111(46.3)		32(13.3)
χ²			21.401				2.178
P			<0.01				0.337
^P<0.05, ^*P<0.01 compared with adult group.

MEST-C	n	Unine protein grade				r_s	P
MEST-C	n	No proteinuria (n=20)	Mild proteinuria (n=35)	Moderate proteinuria (n=33)	Massive proteinuria (n=72)	r_s	P
M
M0	41	14(34.1)	15(36.6)	6(14.6)	6(14.6)	0.462	<0.01
M1	119	6(5.0)	20(16.8)	27(22.7)	66(55.5)	0.462	<0.01
E
E0	90	18(20.0)	26(28.9)	16(17.8)	30(33.3)	0.342	<0.01
E1	70	2(2.9)	9(12.9)	17(24.3)	42(60.0)	0.342	<0.01
S
S0	104	14(13.5)	23(22.1)	23(22.1)	44(42.3)	0.065	0.412
S1	56	6(10.7)	12(21.4)	10(17.9)	28(50.0)	0.065	0.412
T
T0	148	20(13.5)	34(23.0)	30(20.3)	64(43.2)	0.155	0.051
T1/T2	12	0	1(8.3)	3(25.0)	8(66.7)	0.155	0.051
C
C0	53	11(20.8)	15(28.3)	10(18.9)	17(32.1)	0.250	0.001
C1	83	9(10.8)	15(18.1)	19(22.9)	40(48.2)
C2	24	0	5(20.8)	4(16.7)	15(62.5)

Comparisons of clinical and pathological characteristics between children and adults with primary IgA nephropathy and their clinical significances

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MEST-C	n	Urine protein grade						r_s	P
MEST-C	n	No proteinuria (n=9)	Mild proteinuria (n=63)	Moderate proteinuria (n=119)		Massive proteinuria (n=49)		r_s	P
M
M0	87	7(8.0)	29(33.3)		40(46.0) 79(51.6)		11(12.6)	0.217	0.001
M1	153	2(1.3)	34(22.2)		40(46.0) 79(51.6)		38(24.8)	0.217	0.001
E
E0	198	9(4.5)	55(27.8)		98(49.5)		36(18.2)	0.145	0.024
E1	42	0	8(19.0)		21(50.0)		13(31.0)	0.145	0.024
S
S0	83	4(4.8)	31(37.3)		36(43.4)		12(14.5)	0.187	0.004
S1	157	5(3.2)	32(20.4)		83(52.9)		37(23.6)	0.187	0.004
T
T0	178	8(4.5)	57(32.0)		85(47.8)		28(15.7)	0.269	<0.01
T1/T2	62	1(1.6)	6(9.7)		34(54.8)		21(33.9)	0.269	<0.01
C
C0	97	6(6.2)	31(32.0)		48(49.5)		12(12.4)	0.256	<0.01
C1	111	3(2.7)	29(26.1)		57(51.4)		22(19.8)
C2	32	0	3(9.4)		14(43.8)		15(46.9)

Group	n	Type of immune deposition				C3 deposition intensity			Fib deposition intensity
Group	n	IgA	IgA+IgM	IgA+IgG	IgA+IgG+IgM	-	+	?/?	-	+	?/?
Child	160	112(70.0)	37(23.1)	6(3.8)	5(3.1)^*	38(23.8)	80(50.0)	42(26.2)	93(58.1)	48(30.0)	19(11.9)
Adult	240	168(70.0)	68(28.3)	3(1.3)	1(0.4)	25(10.4)	138(57.5)	77(32.1)	207(86.3)	19(7.9)	14(5.8)
χ²		7.952				12.925			42.323
P		0.039				0.002			<0.01
^*P<0.05 compared with adult group.

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