Outcomes of children with idiopathic steroid resistant nephrotic syndrome: a single centre observational study

Mishra, Om P.; Sidar, Minketan; Batra, Vineeta V.; Prasad, Rajniti; Singh, Ankur; Abhinay, Abhishek; Mishra, Akash; Yadav, Ashish K.

doi:10.1590/2175-8239-JBN-2022-0073en

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Brazilian Journal of Nephrology

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Original Article • Braz. J. Nephrol. 45 (2) • Apr-Jun 2023 • https://doi.org/10.1590/2175-8239-JBN-2022-0073en copy

Outcomes of children with idiopathic steroid resistant nephrotic syndrome: a single centre observational study

Authorship SCIMAGO INSTITUTIONS RANKINGS

ABSTRACT

Introduction:

Idiopathic steroid resistant nephrotic syndrome (SRNS) has variable outcomes in children. The primary objective of the present study was to assess the cumulative remission rate and the secondary objectives were to assess factors affecting the remission status, kidney function survival, and adverse effects of medications.

Methods:

One hundred fourteen patients with SRNS were included. Calcineurin inhibitor-based treatment protocol along with prednisolone and angiotensin-converting enzyme inhibitor were used, and patients were followed over 5 years.

Results:

Median age was 4.5 years; 53.5% of cases were between 1 to 5 years of age. Sixty-two patients (54.4%) were at initial stage and 52 (45.6%) were at a late SRNS stage. Median eGFRcr was 83.5 mL/min/1.73m² at presentation. Of the 110 patients, 63 (57.3%) achieved remission [complete remission 30 (27.3%), partial remission 33 (30%)], and 47 (42.7%) had no remission. Kidney function survival was 87.3% and 14 cases (12.7%) had progression to CKD (G3-8, G4-3, G5-1, and G5D-2). Median duration of follow up was 36 months (IQR 24, 60). Age of onset, cyclosporine/tacrolimus, eGFRcr, and histopathology (MCD/FSGS) did not affect remission. Similarly, remission status in addition to age of onset, drug protocol, and histopathology did not significantly affect kidney function during a period of 5 years. Hypertension, cushingoid facies, short stature, cataract, and obesity were observed in 37.7, 29.8, 25.5, 17.5, and 0.7% of cases, respectively.

Conclusion:

About half of the cases achieved remission. Age of onset of disease, cyclosporine/tacrolimus use, and histopathological lesion neither affected remission status nor short-term kidney function survival in SRNS.

Keywords:
Nephrotic Syndrome; Steroid resistant; Remission; Kidney Function survival

Parameters	Findings
Age groups 6 m–1y 1–5 y 6–12 y 13–18 y	3 (2.6%) 61 (53.5%) 44 (38.6%) 6 (5.3%)
Gender Male Female	74 (64.9%) 40 (35.1%)
Category Initial Late	62 (54.4%) 52 (45.6%)
Height (cm)	106.3 ± 24.3
Weight (kg)	19.2(14, 27.6)^a
Body mass index (kg/m²)	18.2 ± 3.6
Systolic BP (mm Hg)	105.1 + 14.9
Diastolic BP (mm Hg)	68.6 + 11.7
Estimated glomerular filtration rate (eGFRcr) (mL/min/1.73 m²)	83.5 (65.6,102)^a
Edema	111 (97.4%)
Hypertension	43 (37.7%)
Hematuria	43 (37.7%)
Hypothyroidism	11 (9.6%)
History of consanguinity	2 (1.7%)
Genetic mutations (n = 39) Present Absent	4 (10.3%) 35 (89.7%)
Histopathology Minimal change disease Focal segmental glomerulosclerosis Diffuse mesangial proliferation	63 (55.3%) 48 (42.1%) 03 (2.6%)
Hemoglobin (g/dL)	11.1 ± 2.1
Total leucocyte counts (ṡ10³/mm³)	12.4 (9087,16167)^a
Absolute neutrophil counts (ṡ10³/mm³)	6.0 ± 1.5
Absolute lymphocyte counts (ṡ10³/mm³)	3.2 ± 1.3
Absolute platelets count (lakh per mm³)	4.4 ± 1.8
Serum urea (mg/dL)	29.6 (20.9,43.3)^a
Serum creatinine (mg/dL)	0.5 (0.4,0.5)^a
Serum total protein (g/dL)	4.3 + 0.9
Serum albumin (g/dL)	1.7 ± 0.4
Serum cholesterol (mg/dL)	410 ± 145
Urine protein/creatinine ratio (mg/mg)	15.1 (9.1, 23.2)^a

Parameters	Remission (n = 63)	No remission (n = 47)	P
Age (months) <60 >60	37 (58.7%) 26 (41.3%)	25 (53.2%) 22 (46.8%)	0.681^a
Total serum protein (g/dL)	4.3 ± 0.9	3.0 ± 0.6	0.702^b
Serum albumin (g/dL)	2.9 (2.6, 3.3)	2.1 (2.0, 2.5)	0.003^c
Upr/cr (mg/mg)	14.8 (9.6, 22.4)	16.7 (8.9, 25.7)	0.370^c
Histopathology MCD FSGS	36 (57.1%) 27 (57.4%)	27 (42.9%) 20 (42.6%)	0.596^a
eGFRcr (mL/min/1.73 m²)	89.2 ± 27.6	88.5 ± 32.3	0.538^b
Time to remission (months)	3.2 ± 0.5	3.5 ± 0.4	0.550^b

Parameters	eGFRcr ≥ 60 mL/min/1.73 m² (n = 96)	eGFRcr < 60 mL/min/1.73 m² (n = 14)	P
Age at onset <5 years >5 years	52 (54.2%) 44 (45.8%)	10 (71.4%) 04 (28.6%)	0.262^a
Gender Male Female	63 (65.6%) 33 (34.4%)	10 (71.4%) 04 (28.6%)	0.770^a
Remission (n = 110) Complete remission No remission	58 (60.4%) 38 (39.6%)	05 (35.7%) 09 (64.3%)	0.116^a
Histopathology MCD FSFS Mesangial proliferation	54 (56.3%) 39 (40.6%) 03 (3.1%)	06 (42.8%) 08 (57.2%) 00 (0.0%)	0.375^a
Treatment (n = 110) Cyclosporine Tacrolimus Cyclophosphamide	41 (42.7%) 52 (54.2%) 03 (3.1%)	05 (35.7%) 09 (64.3%) 00 (0.0%)	0.554^a
Hypertension Yes No	35 (36.5%) 61 (63.5%)	06 (42.9%) 08 (57.1%)	0.427^a
SRNS Initial SRNS Late SRNS	50 (52%) 46 (48%)	08 (57.1%) 06 (42.9%)	0.475^a
Upr/cr (mg/mg)	9.0 (2,12.6)	11.9 (7.5,18.5)	0.465^b
Serum albumin (g/dL)	3.0 ± 1.9	2.2 ± 0.59	0.212^c

Parameters	Hazard ratio	95% CI	P
Age of onset >60 months (ref. ≤ 60 months)	1.4	0.7–2.6	0.35
Drug Tacrolimus (ref. cyclosporine)	0.5	0.3–1.0	0.06
Histopathology FSGS (ref. MCD)	1.2	0.6–2.1	0.64
eGFRcr < 60 mL/min/1.73 m² (ref. ≥ 60 mL/min/1.73 m²	0.7	0.3–1.5	0.32

Parameters	Hazard ratio	95% CI	P
Age of onset >60 months (ref. ≤ 60 months)	0.7	0.2–1.9	0.40
Drug Tacrolimus (ref. cyclosporine)	0.4	0.1–1.3	0.12
Histopathology FSGS (ref. MCD)	1.9	0.6–6.0	0.30
Remission status No remission (ref. remission)	0.7	0.2–2.3	0.54

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[1] Correspondence to:
Om P. Mishra. E-mail: opmpedia@yahoo.co.uk