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Saudi Journal of Kidney Diseases and Transplantation
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Year : 2009  |  Volume : 20  |  Issue : 5  |  Page : 854-857
Pattern of steroid resistant nephrotic syndrome in children living in the kingdom of Saudi Arabia: A single center study


1 Department of Pediatrics, King Abdul Aziz University Hospital, Jeddah, Saudi Arabia
2 Department of Histopathology, King Abdul Aziz University Hospital, Jeddah, Saudi Arabia

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Date of Web Publication2-Sep-2009
 

   Abstract 

Steroid resistant nephrotic syndrome (SRNS) remains a challenge facing pediatric nephrologists. The underlying histopathology usually affects the course of the disease and the response to treatment. We studied the pattern of histopathology in children with SRNS who presented to the King Abdul Aziz University Hospital (KAUH), Jeddah, Saudi Arabia. The records of all children with primary SRNS, who were seen between 2002 and 2007 were reviewed. Only patients who had undergone a renal biopsy were included in the study. The histopathology slides were reviewed by two renal pathologists independently. Patients with congenital nephrotic syndrome, lupus or sickle cell disease, were excluded from the study. Thirty­six children fulfilled the inclusion criteria, and included 25 girls and 11 boys with female to male ratio of 2.3:1. Fifty percent of the children (n=18) were Saudi and the remaining 50% were from various other racial backgrounds (9 Asians, 4 Arabs, 2 Africans and 3 from the Far East). Their mean age at presentation was 4.3 ± 3.0 years (range 1-12 years). The mean serum albumin at presentation was 15.6 ± 7.1 g/L and all of them had 4+ proteinuria on urinalysis. Five children had elevated serum creatinine at presentation while the mean serum creatinine was 50.4 ± 45.6 µmol/L. Three children had low serum complement levels at presentation and none were positive for hepatitis B surface antigen or antinuclear antibody (ANA). The renal histopathology was compatible with focal and segmental glomerulosclerosis (FSGS) in 39% (n=14), IgM nephro­pathy in 28% (n=10), mesengioproliferative glomerulonephritis (MesPGN) in 17% (n=6), mini­mal change disease (MCD) and C1q nephropathy (C1qNP) in 8% each (n=3 + 3) and IgA nephro­pathy in 3% (n=1). Our retrospective review shows that FSGS was the commonest underlying histopathology in children who presented with SRNS followed by IgM nephropathy and other variants of MCD such as MesPGN. C1qNP was the underlying cause in some children.

How to cite this article:
Kari JA, Halawani M, Mokhtar G, Jalalah SM, Anshasi W. Pattern of steroid resistant nephrotic syndrome in children living in the kingdom of Saudi Arabia: A single center study. Saudi J Kidney Dis Transpl 2009;20:854-7

How to cite this URL:
Kari JA, Halawani M, Mokhtar G, Jalalah SM, Anshasi W. Pattern of steroid resistant nephrotic syndrome in children living in the kingdom of Saudi Arabia: A single center study. Saudi J Kidney Dis Transpl [serial online] 2009 [cited 2020 Oct 26];20:854-7. Available from: https://www.sjkdt.org/text.asp?2009/20/5/854/55379

   Introduction Top


Steroid resistant nephrotic syndrome (SRNS) is believed to be associated with a high risk of developing chronic renal failure (CRF). The underlying histopathology usually affects the course of the disease as well as the response to treatment. [1],[2] Recent reports from Louisiana in the USA showed a higher incidence of initial and subsequent steroid resistance, which is not consistent with typical minimal change neph­rotic syndrome (NS), which carries a benign prognosis. [3]

Focal and segmental glomerulosclerosis (FS GS) has been reported as the main cause of SRNS in western countries, [4],[5] as well as many other parts of the world. [6],[7],[8],[9],[10],[11] However, in black South Africans, membranous nephropathy asso­ciated with hepatitis B infection was reported as a main cause of SRNS, [9] while minimal change disease (MCD) was reported as the main cause in Kuwaiti children.

In this study, we report on the pattern of the histopathology of SRNS in children who pre­sented to the King Abdul Aziz University Hos­pital (KAUH), Jeddah, Saudi Arabia.


   Patients and Methods Top


We reviewed the records of all children who presented with primary SRNS to the pediatric renal unit at KAUH, between 2002 and 2007. SRNS was defined as no response after four weeks of therapy with oral prednisolone (60 mg/m 2 /day). We recorded the age at presenta­tion and histopathology results. Patients with congenital nephrotic syndrome, lupus or sickle cell disease, were excluded from the study. Only patients who had undergone a renal biopsy were included in the study.

The histopathology slides were reviewed by two renal pathologists independently. They re­ported the same diagnosis for most of the slides but for the occasional slide where there was any confusion, the pathologists reviewed such slides together, and a final diagnosis was arrived at.


   Results Top


Thirty-six children fulfilled the inclusion cri­teria, 25 girls and 11 boys with female-male ratio of 2.3:1. Fifty percent of the study children (n=18) were Saudi and the other 50% were from various other racial backgrounds (9 Asians, 4 Arabs, 2 Africans and 3 from the Far East). Their mean age at presentation was 4.3 ± 3.0 years (range 1-12 years). The mean serum albumin at presentation was 15.6 ± 7.1 g/L and all patients had 4+ proteinuria on urinalysis. Five children had elevated serum creatinine at presentation, while the mean creatinine value was 50.4 ± 45.6 µmol/L. Three children had low serum complement at presentation and none were positive for hepatitis B surface anti­gen or antinuclear antibody (ANA). The renal histopathology was compatible with focal and segmental glomerulosclerosis (FSGS) in 39% (n=14), IgM nephropathy in 28% (n=10), me­sengioproliferative GN (MesPGN) in 17% (n=6), MCD and C1q nephropathy (C1qNP) in 8% each (n=3 +3) and IgA nephropathy in one pa­tient (3%) [Figure 1].


   Discussion Top


Our results are similar to previous reports, which indicate that FSGS was the main under­lying histopathology in children with SRNS. [4],[5],[6],[7],[8],[9],[10] Thirty-nine percent of our cohort had FSGS, while the reported prevalence of FSGS has varied from 59% in the study by Gulati et al from India [1] to 15% in the study of El Reshaid et al from Kuwait. [11] The report from Kuwait reported MCD as the main cause of SRNS (65%) in Kuwaiti children, [11] while it contri­buted only to 8% of SRNS in our study. No other reports exist about the histopathological pattern of SRNS in children from the Arab world. However, Mattoo et al, reported around two decades ago, that FSGS was the commonest histopathology (39%) in Saudi nephrotic chil­dren who had undergone a renal biopsy. [12] Similarly, we have reported increasing pre­valence of FSGS (35%) and membranoprolife­rative glomerulonephritis in nephrotic children over the years in the western area of the Kingdom of Saudi Arabia. [13] A small study from Qatar also reported that three of six children (50%) with SRNS had FSGS. [14] FSGS is a cause of nephrotic syndrome or proteinuria and is characterized by resistance to steroid treat­ment, frequent progression to end-stage renal disease, and recurrence in the transplanted kidney. [15],[16],[17]

IgM nephropathy was the underlying histo­pathology in 28% of our cohort, which is dif­ferent from earlier reports. [4],[5],[6],[7],[8],[9],[10] IgM nephro­pathy was described around three decades ago as a cause of nephrotic syndrome, with a variable response to treatment and frequent relapses. [18],[19] Recently, Myllymaki et al from Finland reported an eight years of follow up of 110 patients with IgM nephropathy. [20] Thirty six percent developed renal insufficiency, 29% were resistant to corticosteroids and the majo­rity who responded to prednisolone were ste­roid dependent. [20]

MesPGN was the cause of SRNS in 17% of our patients. This is similar to the report by Ejaz et al from Lahore who reported MesPGN as the underlying histopathology in 12% of their SRNS patients. [8] Mattoo et al, also reported that MesPGN was the finding in 21% of nephrotics who underwent renal biopsy. [12] MesPGN has been reported as a cause of SRNS as well as steroid sensitive nephrotic syndrome (SSNS) with a troublesome course but with no pro­gression of renal disease over the years. [21]

MCD was seen in 8% of our cohort. This frequency is lower than earlier reports in which MCD was seen more frequently as a cause of SRNS. However, MCD carries a good response to treatment and better prognosis than FSGS. [1],[2],[3],[4],[5]

C1qNP was the underlying histopathology in 8% of our patients. It has been reported before as a cause of steroid-dependent or steroid re­sistant nephrotic syndrome. [22],[23] The latter sho­wed a poor response to immunosuppressive therapy and carried a high risk for progressive renal insufficiency. [22]

IgA nephropathy was seen in one patient in our study. It has been diagnosed before as a rare cause of SSNS and SRNS with the usual presentation being macroscopic or microscopichematuria. [24],[25]


   Conclusion Top


FSGS was the commonest underlying histo­pathology in children who presented with SRNS to our institution. IgM nephropathy and other variants of MCD such as MesPGN as well as C1qNP were the underlying cause in a few other children.

 
   References Top

1.Gulati S, Sengupta D, Sharma RK, et al. Steroid resistant nephrotic syndrome: role of histopathology. Indian Pediatr 2006;43(1):55-60.  Back to cited text no. 1    
2.Kirpekar R, Yorgin PD, Tune BM, Kim MK, Sibley RK. Clinicopathologic correlates predict the outcome in children with steroid-resistant idiopathic nephrotic syndrome treated with pulse methylprednisolone therapy. Am J Kidney Dis 2002;39(6):1143-52.  Back to cited text no. 2    
3.Kim JS, Bellew CA, Silverstein DM, Aviles DH, Boineau FG, Vehaskari VM. High inci­dence of initial and late steroid resistance in childhood nephrotic syndrome. Kidney Int 2005; 68(3):1275-81.  Back to cited text no. 3    
4.Tune BM, Mendoza SA. Treatment of the idio­pathic nephrotic syndrome: regimens and out­comes in children and adults. J Am Soc Nephrol 1997;8(5):824-32.  Back to cited text no. 4    
5.Ehrich JH, Geerlings C, Zivicnjak M, Franke D, Geerlings H, Gellermann J. Steroid resistant idiopathic childhood nephrosis: overdiagnosed and undertreated. Nephrol Dial Transplant 2007; 22(8):2183-93.  Back to cited text no. 5    
6.Tufro-McReddie A, Alvarez E, Arrizurieta E, Repetto H. Focal glomerulosclerosis in children: an Argentinian experience. Pediatr Nephrol 1992;6(2):158-61.  Back to cited text no. 6    
7.Ali A, Ali D, Mehran H, Ali Z. Idiopathic nephrotic syndrome in Iranian children. Indian Pediatr 2008;45(1):52-3.  Back to cited text no. 7    
8.Ejaz I, Khan HI, Javaid BK, Rasool G, Bhatti MT. Histopathological diagnosis and outcome of paediatric nephrotic syndrome. J Coll Physicians Surg Pak 2004;14(4):229-33.  Back to cited text no. 8    
9.Bhimma R, Coovadia HM, Adhikari M. Nephrotic syndrome in South African children: changing perspectives over 20 years. Pediatr Nephrol 1997;11(4):429-34.  Back to cited text no. 9    
10.Doe JY, Funk M, Mengel M, Doehring E, Ehrich JH. Nephrotic syndrome in African children: lack of evidence for 'tropical neph­rotic syndrome'? Nephrol Dial Transplant 2006;21(3):672-6.  Back to cited text no. 10    
11.El-Reshaid K, Kapoor M, Nampoory N, Madda J, Jawad N, Johny K. Treatment of children with steroid refractory idiopathic nephrotic syndrome: the Kuwaiti experience. Ren Fail 1999;21(5):487-94.  Back to cited text no. 11    
12.Mattoo TK, Mahmood MA, al-Harbi MS. Nephrotic syndrome in Saudi children clinico­pathological study of 150 cases. Pediatr Nephrol 1990;4(5):517-9.  Back to cited text no. 12    
13.Kari JA. Changing trends of histopathology in childhood nephrotic syndrome in western Saudi Arabia. Saudi Med J 2002;23(3):317-21.  Back to cited text no. 13    
14.Eltohami EA, Akl KF. Primary nephrotic syn­drome in Qatar (Arabian Gulf). Br J Clin Pract 1989;43(10):366-8.  Back to cited text no. 14    
15.Velosa JA, Donadio JV, Jr., Holley KE. Focal sclerosing glomerulonephropathy: a clinico­pathologic study. Mayo Clin Proc 1975;50(3): 121-33.  Back to cited text no. 15    
16.Ramirez F, Travis LB, Cunningham RJ, et al. Focal segmental glomerulosclerosis, crescent, and rapidly progressive renal failure. Int J Pediatr Nephrol 1982;3(3):175-8.  Back to cited text no. 16    
17.Iyer VK, Chua C, Milford DV, Ramani P. Focal segmental glomerulosclerosis in childhood: histology, glomerular morphometry, electron microscopy and immunofluorescence findings in biopsies performed early in the course of the disease. Indian J Pathol Microbiol 2002;45(3): 233-9.  Back to cited text no. 17    
18.Mampaso F, Gonzalo A, Teruel J, et al. Mesangial deposits of IgM in patients with the nephrotic syndrome. Clin Nephrol 1981;16(5): 230-4.  Back to cited text no. 18    
19.Hsu HC, Chen WY, Lin GJ, et al. Clinical and immunopathologic study of mesangial IgM nephropathy: report of 41 cases. Histopathology 1984;8(3):435-46.  Back to cited text no. 19    
20.Myllymaki J, Saha H, Mustonen J, Helin H, Pasternack A. IgM nephropathy: clinical picture and long-term prognosis. Am J Kidney Dis 2003;41(2):343-50.  Back to cited text no. 20    
21.Uszycka-Karcz M, Stolarczyk J, Wrzolkowa T, et al. Mesangial proliferative glomerulonephritis in children. Int J Pediatr Nephrol 1982;3(4): 251-6.  Back to cited text no. 21    
22.Jennette JC, Hipp CG. C1q nephropathy: a dis­tinct pathologic entity usually causing nephrotic syndrome. Am J Kidney Dis 1985;6(2):103-10.  Back to cited text no. 22    
23.Kari JA, Jalalah SM. C1q nephropathy in two young sisters. Pediatr Nephrol 2008;23(3):487­-90.  Back to cited text no. 23    
24.Sieniawska M, Kozak-Bialasik D, Gura C, et al. Clinical course and treatment results of IgA nephropathy in children. Pediatr Pol 1996;71 (4):313-20.  Back to cited text no. 24    
25.Cengiz N, Baskin E, Agras PI, Bilezikci B, Saatci U. Unusual presentation of IgA neph­ropathy in childhood: a case report. Ren Fail 2005;27(6):795.  Back to cited text no. 25    

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Correspondence Address:
Jameela A Kari
Department of Pediatrics, King Abdul Aziz University Hospital, P.O. Box 80215, Jeddah 21589
Saudi Arabia
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    Abstract
    Introduction
    Patients and Methods
    Results
    Discussion
    Conclusion
    References
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