Table 1 Clinical characteristics and efficacy of RTX treatment in children with SDNS/FRNS

Steroid response [sensitive/dependent/resistant]

Dependent

Sensitive

Dependent

Sensitive

Sensitive

Secondary resistance

Sensitive

Relapse status [relapse/frequent relapse]

Frequent relapse

Frequent relapse

Frequent relapse

Frequent relapse

Frequent relapse

Frequent relapse

Frequent relapse

Duration of disease before the first dose (years)

1

8

6

1

1

4

2

The time of last relapse after diagnosis(months)

7

103

72

7

46

57

33

The time of last relapse before start treatment(days)

31

45

37

29

62

76

24

Renal pathophysiology

Not performed

FSGS

MCD

Not performed

Not performed

Not performed

Not performed

Genetic testing

Not performed

Not performed

FN1 c.2984 C > T, PTPRO c.1314 C > G

Not performed

Not performed

NPHS1 heterozygous mutation

Not performed

Other immunosuppressive drugs

No

CTX

MMF, FK506

No

No

FK506

Levamisole

Steroids before treatment

2 mg/kg qd

2 mg/kg qod

0.75 mg/kg qd

1 mg/kg qd

1 mg/kg qd

0.75 mg/kg qod

2 mg/kg qd

Tapering off steroids [months after the first dose]

6

5

5

4

5

5

6

MMF [months after the first dose]

7

12

8

5

6

10

6

Edema [yes/no]

No

No

No

No

No

No

No

Proteinuria [-~4+]

all(-)

all(-)

once(1+)

all(-)

all(-)

once(1+)

all(-)

24-hour urine protein (mg/kg.24 h)

2.67

1.59

2.08

2.43

3.90

2.68

2.06

PCR(mg/mg)

0.007

0.009

0.008

0.008

0.01

0.005

0.005

Serum albumin [M(P₂₅ ~ P₇₅)]/(g/L)

45.50(41.15 ~ 46.80)^ab

45.70(44.50 ~ 48.90) ^ac

46.00(41.40 ~ 48.20) ^ab

40.7(31.75~

44.80) ^bd

45.7(33.45 ~ 49.30) ^ab

43.30(41.60 ~ 44.80) ^ab

42.40(40.55 ~ 44.55) ^ab

Serum cholesterol [M(P₂₅ ~ P₇₅)]/(mmol/L)

3.86(3.79 ~ 4.97) ^a

3.99(3.42 ~ 4.59) ^a

5.79(4.33 ~ 6.07) ^a

4.12(3.62 ~ 7.55) ^a

4.45(3.57 ~ 5.89) ^a

4.88(4.08 ~ 5.48) ^a

4.67(3.63 ~ 5.77) ^a

Relapse[yes/no]

None

None

None

None

None

None

None

RTX allergy [yes/no]

None

None

None

None

None

None

None

Infections, all upper respiratory [0/1/2/3/4 times]

1 (COVID-19)

1 (Undetermined pathogen)

1 (COVID-19)

2 (Undetermined pathogen and COVID-19)

3 (Once undetermined pathogen and twice COVID-19)

1 (Un-determined pathogen)

4 (Un-determined pathogen)