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

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

Table 4.2.3: Number of renal biopsies Yearn (%) (4.0) (3.1) (2.8) (2.8) (5.2) (3.6) (11.6) (12.4) (13.1) (14.9) (12.4) (14.2) Total1106 (100.0) 4 th MRRB report 2010, Malaysia

Table 4.2.4: Number of biopsies done on each patients at different times Total no of biopsy/patientn% 1 st episode nd rd >4 th 20.2 Total number of Patient th MRRB report 2010, Malaysia

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

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%

Table 4.4.1: Gender and racial distribution n% Gender Male Female Total Race Malay Chinese Indian Others* Total th MRRB report 2010, Malaysia

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

Table 4.5.1: Clinical presentation at biopsy Clinical presentationn% Asymptomatic urine abnormalities Nephritic syndrome Nephrotic syndrome Nephritic nephrotic syndrome Not available766.9 Missing90.8 Total th MRRB report 2010, Malaysia

Table 4.5.2: Renal function at biopsy Renal function at biopsyn% Impaired Normal Not available or missing data635.7 Total th MRRB report 2010, Malaysia

Table 4.5.3: Hypertension at biopsy Hypertension At biopsyn% Present Absent Not available or missing data272.4 Total Drug a ACEI Alpha Blocker ARB92.4 Beta Blocker Calcium Channel Blocker Others287.5 No drug available th MRRB report 2010, Malaysia

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

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

Table 4.7.1: Renal histopathology diagnosis of children presenting with nephrotic syndrome Diagnosisn% FSGS MCD Lupus nephritis IgA nephropathy132.3 Mesangial proliferative GN non-IgA142.4 Post-infectious GN71.2 Others**498.5 Unknown/Missing81.4 Total *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

Table 4.7.2: The histopathological profile in different steroid response categories DiagnosisSteroid responsiveSteroid resistant n%n% FSGS MCD Lupus nephritis IgA nephropathy Mesangial proliferative GN non-IgA Post-infectious GN Others* Unknown Total *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

Table 4.8: Renal histopathology diagnosis of children presenting with nephritic syndrome Diagnosisn% Post-infectious GN Lupus nephritis Acute tubular necrosis106.0 Henoch Schonlein Purpura95.4 FSGS95.4 IgA nephropathy84.8 MCD84.8 Messangial proliferative GN-non lgA42.4 Others** Total *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

Table 4.9: Histology finding of children who had severe renal failure (needed dialysis therapy) who underwent renal biopsy Diagnosisn% Post-infectious GN Lupus nephritis 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 Total 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

Table : 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

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

Table : Patient survival for focal segmental glomerulosclerosis and minimal change disease Clinical characteristics Minimal change diseaseFSGS Interval (months)n% survivalSEn% survivalSE th MRRB report 2010, Malaysia

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

Table : Renal survival of patient with focal segmental glomerulosclerosis and minimal change disease Clinical characteristics Minimal change diseaseFSGS* Interval (days)n% survivalSEn% survivalSE th MRRB report 2010, Malaysia

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

Table : Total number of patient and biopsies (SLE) YearTotal number of PatientTotal number of biopsies th MRRB report 2010, Malaysia

Table : Distribution of renal biopsy in patients with lupus by numbers of biopsy Total number of biopsy /patientn% 1 st episode nd episode rd episode 41.5 Total Patient th MRRB report 2010, Malaysia

Table : Gender distribution for paediatric lupus nephritis Gendern% Male Female Total th MRRB report 2010, Malaysia

Table : Racial distribution for paediatric lupus nephritis Ethnicn% Malay Chinese Indian 73.0 Others* Total th MRRB report 2010, Malaysia

Table Age for paediatric lupus nephritis Age (years) n 270 Mean 11.4 Standard deviation 3.1 Minimum 0.1 Maximum th MRRB report 2010, Malaysia

Table Dialysis therapy for paediatric lupus nephritis at the time of biopsy Needed dialysis therapyn% Yes165.9 No Missing/Not available Total th MRRB report 2010, Malaysia

Table : Patient with hypertension (SLE) Hypertension n% Yes No Missing/Not available103.7 Total th MRRB report 2010, Malaysia

Table : Clinical presentation at biopsy (SLE) Clinical presentationn% Asymptomatic urine abnormalities Nephritic syndrome Nephrotic syndrome Nephritic nephrotic syndrome Unknown238.5 Missing31.1 Total th MRRB report 2010, Malaysia

Table (a): Clinical presentation of paediatric lupus Clinical presentationN=270% Malar rash Photosensitivity Oral ulcers Discoid rash Hematological Arthritis Serositis Cerebral Renal th MRRB report 2010, Malaysia

Table (b): ARA criteria at presentation Number of ARA criterian% < ≥ Total th MRRB report 2010, Malaysia

Table : Classification of paediatric lupus nephritis WHO/ISN /RPS ClassNeeded dialysis therapy Not needed dialysis therapy n%n% Class I Class II Class III or V+III Class IV or V+IV Class V or V+II Class VI Unknown Total th MRRB report 2010, Malaysia

Table : Patients survival in lupus nephritis Lupus Nephritis patients Interval (months)n% survivalSE th MRRB report 2010, Malaysia

Figure : Patient survival in lupus nephritis 4 th MRRB report 2010, Malaysia Cumulative Survival Duration (months)

Table : Renal survival of patients with lupus nephritis (ESRF&ESRF+died) Lupus Nephritis patients Interval (months)n% survivalSE th MRRB report 2010, Malaysia

Figure : Renal survival of patient with lupus nephritis 4 th MRRB report 2010, Malaysia Cumulative Survival Duration (months)...

Table 4.12: Causes of end stage renal disease in children who underwent renal biopsy Causesn% FSGS Lupus nephritis Advance glomerulosclerosis (advance GN) 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 Total th MRRB report 2010, Malaysia

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

Table : 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

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