1. CHAPTER 4 Paediatric Renal Biopsies Lee Ming Lee Lim Yam Ngo Lynster Liaw Mirunalini a/p Appadurai Selva Kumar a/l Sivapuniam Susan Pee Wan Jazilah Wan Ismail Yap Yok Chin

2. Table 4.2.2: Number of patients from various hospitals HospitalsN% Hospital Kuala Lumpur40439.1 Other MOH Hospitals60658.7 University Hospital70.7 Army Hospital10.1 Private Hospital141.4 Total number of patients1032100.0 4 th MRRB report 2010, Malaysia

3. Table 4.2.3: Number of renal biopsies Yearn (%) 1999 44 (4.0) 2000 34 (3.1) 2001 31 (2.8) 2002 31(2.8) 2003 57 (5.2) 2004 40 (3.6) 2005 128 (11.6) 2006 137 (12.4) 2007 145 (13.1) 2008 165 (14.9) 2009 137 (12.4) 2010 157 (14.2) Total1106 (100.0) 4 th MRRB report 2010, Malaysia

4. Table 4.2.4: Number of biopsies done on each patients at different times Total no of biopsy/patientn% 1 st episode98088.6 2 nd 1029.2 3 rd 222.0 >4 th 20.2 Total number of Patient1106100.0 4 th MRRB report 2010, Malaysia

5. Table 4.3.1: Conclusive report 4 th MRRB report 2010, Malaysia Year Total number of biopsies Report conclusiveReport not conclusive n%n% 1999 -20101106105695.5504.5

6. Table 4.3.2: Number of glomeruli obtained at each biopsy *7 cases with missing number of glomeruli 4 th MRRB report 2010, Malaysia Year Total number of biopsies ≥ 10 Glomeruli< 10 Glomeruli n%n% 1999 -2010110685878.124121.9

7. Table 4.4.1: Gender and racial distribution n% Gender Male 49447.9 Female 53852.1 Total 1032100.0 Race Malay 64062.0 Chinese 20319.7 Indian 737.1 Others* 11611.2 Total 1032100.0 4 th MRRB report 2010, Malaysia

8. Table 4.4.2: Age distribution Age (years)1999-2010 n 1106 Mean 9.5 Standard deviation 4.0 Minimum 0.02 Maximum 15.0 4 th MRRB report 2010, Malaysia

9. Table 4.5.1: Clinical presentation at biopsy Clinical presentationn% Asymptomatic urine abnormalities17215.6 Nephritic syndrome16314.7 Nephrotic syndrome57852.3 Nephritic nephrotic syndrome1089.8 Not available766.9 Missing90.8 Total1106100.0 4 th MRRB report 2010, Malaysia

10. Table 4.5.2: Renal function at biopsy Renal function at biopsyn% Impaired34831.5 Normal69562.8 Not available or missing data635.7 Total944100.0 4 th MRRB report 2010, Malaysia

11. Table 4.5.3: Hypertension at biopsy Hypertension At biopsyn% Present37133.5 Absent70864.0 Not available or missing data272.4 Total1106100.0 Drug a ACEI10728.8 Alpha Blocker4111.1 ARB92.4 Beta Blocker5414.6 Calcium Channel Blocker15842.6 Others287.5 No drug available14639.4 4 th MRRB report 2010, Malaysia

12. Table 4.6.1: Diagnosis of paediatric renal biopsies *Patients may have more than 1 diagnosis classification 4 th MRRB report 2010, Malaysia

13. Table 4.6.2 Annual frequency of the main renal biopsy findings 1999-2010 HSP: Henoch Schonlein Purpura *Patients may have more than 1 diagnosis classification 4 th MRRB report 2010, Malaysia

14. Table 4.7.1: Renal histopathology diagnosis of children presenting with nephrotic syndrome Diagnosisn% FSGS22939.9 MCD16729.1 Lupus nephritis8715.2 IgA nephropathy132.3 Mesangial proliferative GN non-IgA142.4 Post-infectious GN71.2 Others**498.5 Unknown/Missing81.4 Total574100.0 *Patients may have more than 1 diagnosis classification ** Others – Henoch Schonlein Purpura, HUS/TTP, Systemic vasculitis, Malignancy, Membranous nephropathy, Membrano-proliferative, Idiopathic crescentic GN, Acute interstitial nephritis, Acute tubular necrosis, Chronic interstitial nephritis, Hereditary (others), Advance GN, Others 4 th MRRB report 2010, Malaysia

15. Table 4.7.2: The histopathological profile in different steroid response categories DiagnosisSteroid responsiveSteroid resistant n%n% FSGS2629.212151.1 MCD4044.95824.5 Lupus nephritis1011.2239.7 IgA nephropathy11.131.3 Mesangial proliferative GN non-IgA33.473.0 Post-infectious GN--10.4 Others*66.7208.4 Unknown33.441.7 Total89100.0237100.0 *Patients may have more than 1 diagnosis classification ** Others – Henoch Schonlein Purpura, Membranous nephropathy, Membrano-proliferative, Idiopathic crescentic GN, Acute interstitial nephritis, Acute tubular necrosis, Chronic interstitial nephritis, Advance GN, Others 4 th MRRB report 2010, Malaysia

16. Table 4.8: Renal histopathology diagnosis of children presenting with nephritic syndrome Diagnosisn% Post-infectious GN5432.5 Lupus nephritis4527.1 Acute tubular necrosis106.0 Henoch Schonlein Purpura95.4 FSGS95.4 IgA nephropathy84.8 MCD84.8 Messangial proliferative GN-non lgA42.4 Others**1911.4 Total166100.0 *Patients may have more than 1 diagnosis classification ** Others – HUS/TTP, Anti GBM disease, Advance GN, Systemic vasculitis, Membrano-proliferative, Idiopathic crescentic GN, Acute interstitial nephritis, Chronic interstitial nephritis, Alport’s syndrome 4 th MRRB report 2010, Malaysia

17. Table 4.9: Histology finding of children who had severe renal failure (needed dialysis therapy) who underwent renal biopsy Diagnosisn% Post-infectious GN2333.8 Lupus nephritis1522.1 Acute tubular necrosis68.8 FSGS57.4 Advanced glomerulosclerosis (advance GN)57.4 Acute interstitial nephritis34.4 HUS/TTP11.5 IgA nephropathy11.5 Others*913.2 Total68100.0 4 th MRRB report 2010, Malaysia * Membrano-proliferative, Messangial Proliferative GN-non lgA, Idiopathic Crescentic GN, Henoch scholein purpura, Systemic vasculitis, Malignancy, Chronic interstitial nephritis, Others

18. Table 4.10.1: Clinical characteristics of children with steroid resistant nephrotic syndrome a, c, j, k, l Performed Wilcoxon Rank Sum test b, d, f, h Performed Chi-square test e, g, i, m Performed Fisher’s exact test 4 th MRRB report 2010, Malaysia

19. Continue; a, c, j, k, l Performed Wilcoxon Rank Sum test b, d, f, h Performed Chi-square test e, g, i, m Performed Fisher’s exact test 4 th MRRB report 2010, Malaysia

20. Table 4.10.2: Patient survival for focal segmental glomerulosclerosis and minimal change disease Clinical characteristics Minimal change diseaseFSGS Interval (months)n% survivalSEn% survivalSE 0170100.0-229100.0- 1213698.20.01120498.70.008 2410996.60.015177 98.10.010 3686 96.6 0.015148 98.10.010 4852 96.6 0.015121 98.10.010 6036 96.6 0.01589 97.20.013 7223 96.6 0.01554 97.20.013 8418 96.6 0.01542 94.90.026 9613 96.6 0.01534 94.90.026 10813 96.6 0.01522 94.90.026 1207 96.6 0.01516 94.90.026 132596.60.015688.50.066 4 th MRRB report 2010, Malaysia

21. Figure 4.10.2: Patient survival by focal segmental glomerulosclerosis and minimal change disease 4 th MRRB report 2010, Malaysia

22. Table 4.10.3: Renal survival of patient with focal segmental glomerulosclerosis and minimal change disease Clinical characteristics Minimal change diseaseFSGS* Interval (days)n% survivalSEn% survivalSE 0 170100.0-228100.0- 12 13698.20.01119394.10.016 24 10895.70.01716691.90.019 36 8494.80.02013590.00.021 48 50 94.80.020 11090.00.021 60 34 94.80.020 7785.30.029 72 23 94.80.020 4684.00.031 84 18 94.80.020 3578.00.045 96 13 94.80.020 2675.00.052 108 13 94.80.020 1775.00.052 120 7 94.80.020 1475.00.052 132592.80.020569.20.073 4 th MRRB report 2010, Malaysia

23. Figure 4.10.3: Renal survival by focal segmental glomerulosclerosis and minimal change 4 th MRRB report 2010, Malaysia

24. Table 4.11.1: Total number of patient and biopsies (SLE) YearTotal number of PatientTotal number of biopsies 1999 -2010230270 4 th MRRB report 2010, Malaysia

25. Table 4.11.2: Distribution of renal biopsy in patients with lupus by numbers of biopsy Total number of biopsy /patientn% 1 st episode 23285.9 2 nd episode 3412.6 3 rd episode 41.5 Total Patient 270100.0 4 th MRRB report 2010, Malaysia

26. Table 4.11.3.1: Gender distribution for paediatric lupus nephritis Gendern% Male 3113.5 Female 19986.5 Total 230100.0 4 th MRRB report 2010, Malaysia

27. Table 4.11.3.2: Racial distribution for paediatric lupus nephritis Ethnicn% Malay 14462.6 Chinese 5925.7 Indian 73.0 Others* 208.7 Total 230100.0 4 th MRRB report 2010, Malaysia

28. Table 4.11.3.3 Age for paediatric lupus nephritis Age (years)1999-2010 n 270 Mean 11.4 Standard deviation 3.1 Minimum 0.1 Maximum 15.0 4 th MRRB report 2010, Malaysia

29. Table 4.11.3.4 Dialysis therapy for paediatric lupus nephritis at the time of biopsy Needed dialysis therapyn% Yes165.9 No20676.3 Missing/Not available4817.8 Total270100.0 4 th MRRB report 2010, Malaysia

30. Table 4.11.3.5: Patient with hypertension (SLE) Hypertension n% Yes11241.5 No14854.8 Missing/Not available103.7 Total270100.0 4 th MRRB report 2010, Malaysia

31. Table 4.11.3.6: Clinical presentation at biopsy (SLE) Clinical presentationn% Asymptomatic urine abnormalities7025.9 Nephritic syndrome4416.3 Nephrotic syndrome8431.1 Nephritic nephrotic syndrome4617.0 Unknown238.5 Missing31.1 Total270100.0 4 th MRRB report 2010, Malaysia

32. Table 4.11.4(a): Clinical presentation of paediatric lupus Clinical presentationN=270% Malar rash 14152.2 Photosensitivity 8531.5 Oral ulcers 7126.3 Discoid rash 103.7 Hematological 14854.8 Arthritis 7427.4 Serositis 3613.3 Cerebral 3412.6 Renal 23185.6 4 th MRRB report 2010, Malaysia

33. Table 4.11.4(b): ARA criteria at presentation Number of ARA criterian% <4 6122.6 ≥ 4 20977.4 Total 270100.0 4 th MRRB report 2010, Malaysia

34. Table 4.11.5: Classification of paediatric lupus nephritis WHO/ISN /RPS ClassNeeded dialysis therapy Not needed dialysis therapy n%n% Class I00.010.5 Class II16.3157.3 Class III or V+III00.04320.9 Class IV or V+IV1168.813666.0 Class V or V+II212.5104.9 Class VI212.500.0 Unknown00.010.5 Total16100.0206100.0 4 th MRRB report 2010, Malaysia

35. Table 4.11.6: Patients survival in lupus nephritis Lupus Nephritis patients Interval (months)n% survivalSE 0246100.0- 1219795.60.014 2416593.00.018 36130 91.8 0.019 48100 91.8 0.019 6072 88.4 0.027 7248 88.4 0.027 8439 88.4 0.027 9630 88.4 0.027 10823 84.7 0.044 12019 84.7 0.044 13212 78.7 0.071 4 th MRRB report 2010, Malaysia

36. Figure 4.11.6: Patient survival in lupus nephritis 4 th MRRB report 2010, Malaysia 0.00 0.25 0.50 0.75 1.00 Cumulative Survival 050100150 Duration (months)

37. Table 4.11.7: Renal survival of patients with lupus nephritis (ESRF&ESRF+died) Lupus Nephritis patients Interval (months)n% survivalSE 0246100.0- 1219197.30.011 2416096.70.012 36124 95.5 0.015 4895 95.5 0.015 6067 93.3 0.021 7245 93.3 0.021 8437 93.3 0.021 9626 86.9 0.048 10819 86.9 0.048 12015 81.4 0.069 1329 81.4 0.069 4 th MRRB report 2010, Malaysia

38. Figure 4.11.7: Renal survival of patient with lupus nephritis 4 th MRRB report 2010, Malaysia 0.00 0.25 0.50 0.75 1.00 Cumulative Survival 050100150 Duration (months)...

39. Table 4.12: Causes of end stage renal disease in children who underwent renal biopsy Causesn% FSGS3331.7 Lupus nephritis1615.4 Advance glomerulosclerosis (advance GN)1615.4 Post-infectious GN76.7 IgA nephropathy76.7 Chronic interstitial nephritis43.8 Acute interstitial nephritis32.9 Systemic vasculitis32.9 Membranoproliferative GN32.9 MCD32.9 Mesangial proliferative GN non-IgA21.9 Henoch Schonlein Purpura21.9 HUS/TTP21.9 Acute tubular necrosis11.0 Idiopathic crescentic GN11.0 Membranous nephropathy11.0 Total104100.0 4 th MRRB report 2010, Malaysia

40. Table 4.13.1: Frequency of complication 4 th MRRB report 2010, Malaysia n% Total Number of biopsies1106- Total Number of complication555 Type of complication Bleeding Gross haematuria3869.1 Haematoma23.6 Perirenal collection1018.2 Infection00 Arteriovenous malformation11.8 Hypotension11.8 Others35.5

41. Table 4.13.2: Risk factors for complication (ref*) Reference category CI-confidence interval ** Not able to do compute due to the small sample size a No information on renal failure needed dialysis for biopsy procedure data b No information on calculated gfr for biopsy procedure data c No information on haemoglobin (Hgb) level for biopsy procedure data d No information ultrasound biopsy for biopsy procedure data e No information on plug biopsy for biopsy procedure data f No information on needle size for biopsy procedure data g No information on number of passes for biopsy procedure data 4 th MRRB report 2010, Malaysia

42. Continue; (ref*) Reference category CI-confidence interval ** Not able to do compute due to the small sample size a No information on renal failure needed dialysis for biopsy procedure data b No information on calculated gfr for biopsy procedure data c No information on haemoglobin (Hgb) level for biopsy procedure data d No information ultrasound biopsy for biopsy procedure data e No information on plug biopsy for biopsy procedure data f No information on needle size for biopsy procedure data g No information on number of passes for biopsy procedure data 4 th MRRB report 2010, Malaysia