RESULTS OF RENAL BIOPSY IN CHILDREN WITH NEPHROTIC SYNDROME AT TRIPOLI CHILDREN HOSPITAL Naziha R. Rhuma1, Mabruka Ahmed Zletni1, Mohamed Turki1, Omar Ahmed Fituri1, Awatif. El Boeshi2 1- Faculty of medicine, University of Tripoli, Libya. 2- Nephrology Unit, Children Hospital of Tripoli, Libya. ABSTRACT Nephrotic syndrome is an important renal disorder in children. The role of renal biopsy in children with nephrotic syndrome is controversial, especially in children with frequent relapses or steroid-dependent nephrotic syndrome. The aims of this study are to verify indications of renal biopsy in children with nephrotic syndrome, to identify pat- terns of glomerular disease and its corresponding outcomes. This is a descriptive study reviewed retrospectively a 25 renal biopsies from children with nephrotic syndrome who followed up in nephrology unit at Tripoli Children Hos- pital from Jun. 1995 to Jan. 2006. Children with either steroid resistant or steroid dependent who underwent renal biopsy were included. Twenty five of children (14 male and 11 female) with nephrotic syndrome were included. The mean age 5.2±4.6years (range was from 1-14 years). 14(56%) children were steroid resistant and 11(44%) children were steroid dependent. Minimal-change disease (MCD) accounted for 12(48%) children, focal and segmental scle- rosis (FSGS) was accounted for 10(40%) children and 3(12%) children accounted for other histopathological types. 7(87.5%) of children with FSGS had progressed to end stage renal disease. Steroid resistant was the main indication for renal biopsy in children with nephrotic syndrome. There was increased frequency of FSGS nephrotic syndrome among children with steroid resistant type with poor outcomes. KEY WORDS: Nephrotic syndrome, Renal biopsy, Minimal change disease, Focal segmental glomerulosclerosis. INTRODUCTION1 more than 50% of patients(8,9,10,11). Our objectives are Minimal change disease (MCD) is the most frequent to verify the indications of renal biopsy in children lesion associated with idiopathic nephrotic syndrome with nephrotic syndrome, identifying patterns of (1) in children . In 1970, the International Study of glomerular disease frequency as well as their out- Kidney Disease in Children (ISKDC) reported an comes. increased frequency of MCD than focal segmental glomerulosclerosis (FSGS) and other renal patholo- PATIENTS AND METHODS A retrospective review was conducted of children gy. On the basis of data from the ISKDC conducted with idiopathic nephrotic syndrome who underwent in 1978, it has been accepted that MCD is by far the percutaneous renal biopsy from Jun, 1995 to Jan, most frequent lesion associated with idiopathic ne- 2006. The study include children with steroid re- phrotic syndrome in children and adolescents, ac- sistant nephrotic syndrome and children with steroid counted for 77% of cases in all biopsies performed (2) dependent who presented with atypical presentation in children while FSGS represented nearly 8-10% . which include Age (less than 2 years and more than However, recently several reports have been noted 10 years), persistent hypertension, renal function that there is an increase trend of FSGS in both chil- impairment persistent hematuria and decrease serum dren and adults who were biopsed for their nephrotic (3,4) complement. syndrome to rates as high as 50% . The role of At presentation, they were evaluated for hyperten- the renal biopsy in children with nephrotic syndrome sion, hematuria, renal function status and serum is controversial, especially in patients with frequent (5) complement. After discussion with parents, kidney relapses or steroid dependent nephrotic syndrome . biopsy was performed in all patients. The procedure Steroid resistant still remain the main indication for (6) done in Tunisia or Jordan. The specimens had exam- renal biopsy in children with nephrotic syndrome . ined by light microscopy, Immunoflourenscnce Certain clinical features are consider to be atypical stained and electron microscopy. Minimal change in steroid sensitive nephrotic syndrome these in- disease was diagnosed by normal glomeruli on light clude, extreme of age (less than 1 years, or greater microscope, lack of immuno deposit on Immuno- than 12 years), persistent hypertension, persistent flourenscnce and foot process effacement by elec- renal impairment, gross hematuria and low plasma (7) tron microscope. complement concentration . It is well recognized In idiopathic FSGS, the glomeruli show mesangeal that FSGS in children with idiopathic nephrotic syn- proliferation and segmental scaring on light micro- drome carries poor prognosis. Although initially scope, Immunoflourenscnce show IgM and C3 stain- considered a resistant lesion but the data suggest that ing in area of segmental sclerosis. By electron mi- more aggressive therapies can induce a response in croscope show segmental scaring of glomerular tuft with obliteration of glomerular capillary lumen. Correspondence and reprint request : FSGS was diagnosed when at least one glomerulus Naziha R. Rhuma demonstrate segmental sclerosis on light micro- Tripoli Children Hospital. Nephrology unit Email: [email protected] scope. MMSJ Vol.2 Issue.2 (Winter 2015) www.misuratau.edu.ly 114 Rhuma N & et.al Results of renal biopsy in children with nephrotic syndrome at Tripoli Children Hospital A diagnosis of steroid resistant was made if there is no response to 4-week course of prednisone at a dose of 60 mg/m2/day. Steroid dependent was de- fined as development of 2 consecutive relapses dur- ing steroid therapy or within 2 wk of its cessation. Hypertension was defined as a blood pressure con- sistently above 95th centile for height, age and sex. Hematuria and proteinuria are assessed by dipstick urine analysis. STATISTICS ANALYSIS After data collected they were coded and transferred into the Statistical Package for the Social Sciences (Figure 1) age at presentation n versus type (SPSS), version 12. Results are expressed as either of histopathology types means ± SD or percentage. The statistical tools used like frequencies, ratio, and chi-square test. The level When compare MCD with FSGS and other histo- of significance was set at P value < 0.05 in all cases. pathology types, children MCD were younger at diagnosis than FSGS and other pathology (2.9yeares RESULTS versus 7 and 8.7 years), boys were more likely to be This study provided the histopathological classifica- MCD than FSGS (64.3% versus 28.6%) and steroid tion of nephrotic syndrome in children followed up resistant children were more likely to be FSGS than in Tripoli Children Hospital over a period of 10 MCD while steroid dependent were more likely to years (1995-2006) on a total 25 renal biopsies, be MCD than FSGS as shown in (table 2). 14(56%) were males and 11(44%) were females. The mean age 5.2 ± 4.6years (range was from 1-14 (Table 2) MCD versus FSGS and other histopathology years). The mean period before biopsy was 15.8 mo types (range 1-84 mo) and mean follow up duration (6.5 ± Characteristic MCD FSGS Other pathology 3.1) years range (2-11) years. No. of children 12 10 3 The indications of renal biopsy were as following Mean age at 2.9 ± 2.5 7 ± 4.8 8.7± 6.9 yrs 14(56%) were steroid resistant and 11(44%) were presentation yrs yrs steroid dependent as shown in (table 1). Male: female 3 0.66 0.5 ratio (Table 1) Indications of renal biopsies Steroid resistant 23% 61.50% 15.50% MCD FSGS Other Type Total (%) Steroid depend- (%) (%) pathology 75% 16.70% 8.30% ent Steroid 4(28.6) 8(57.1) 2(14.3) 14(56) MC minimal change disease, FSGS: focal segmental glo- resistant merulonephrosis Steroid 8(72.1) 2(18.2) 1(9.1) 11(44) dependent Regarding the clinical outcomes of these children a Total 12(48) 10(40) 3(12) 25(100) chi-square was done between histopathology types and development of end stage renal failure, no chil- MCD minimal change disease, FSGS focal segmental dren with MCD advanced to end stage renal failure glomerulo-sclerosis SR steroid resistant, SD steroid de- however 7(87.5%) of children with FSGS progressed pendent. to end stage renal failure as shown in (table 3). Other histopathology accounted for 3(12%) which (Table 3) Long term follow up include lupus nephritis in one case, IgA nephropathy Type No RF (%) RF (%) Total (%) P value in one child and mesangio proliferative glomerulo- MCD 12(48) 0(0) 12(48) 0.002 sclerosis (MPGN) in one child. FSGS 3(17.6) 7(87.5) 10(40) 0.001 At presentation 12(48%) of these children had pre- Others 2(8) 1(4) 3(12) 0.001 sented with typical presentation and 13(52%) had Total 17(68) 8(32) 25(100) presented with atypical presentation. The atypical MCD minimal change disease, FSGS: focal segmental presentation include extreme of age, hypertension, glomerulonephrosis, RF: renal failure hematuria, renal impairment, low serum comple- ment. DISCUSSIONS When compare age at presentation versus histo- Idiopathic nephrotic syndrome in children has con- pathology types of nephrotic syndrome 9(36%) chil- ventionally been associated with minimal change dren were less than 2 years of age, 10(40%) children disease. White et al reported that MCNS in up to are between 2-10 years of age and 6(24%) are more 90% among all children with idiopathic nephrotic than 10 years of age as shown in (figure 1). syndrome, FSGS has generally been considered a MMSJ Vol.2 Issue.2 (Winter 2015) www.misuratau.edu.ly 115 Rhuma N & et.al Results of renal biopsy in children with nephrotic syndrome at Tripoli Children Hospital relatively infrequent cause(1). In first ISKDC report presentation and steroid resistant. Children with (1970) 127 children, MCD was diagnosed in 77%, MCD were more likely to be boys, younger age at FSGS in 9% and MPGN in 5% of children who were presentation and steroid dependent. Renal biopsy biopsy prior to treatment(2).