| Abstract|| |
To identify the variations in pediatric renal biopsy pathology and clinicopathological features in Guangxi, China, in the past ten years, we studied retrospectively the kidney biopsies performed to evaluate the primary nephrotic syndrome (PNS) in 218 children at two main medical centers in Guangxi from January 1999 to January 2009. The major pathological finding was mesangial proliferative glomerulonephritis (48.2%), focal segmental glomerulosclerosis (16.5%), immunoglobulin A nephropathy (13.3%) and minimal change disease (11.0%). Patients with different pathological types yielded different response rates to glucocorticoids (P <0.001). There were statistical significant differences between prognosis for the different pathological types (P <0.05). The pathological characteristics of PNS in children were diverse and significant for guiding the grade of glucocorticoid response and predicting the prognosis of the PNS disease.
|How to cite this article:|
Zhou TB, Lin N, Qin YH, Liu YG. Distribution of pathological finding in the children with nephrotic syndrome from Guangxi. Saudi J Kidney Dis Transpl 2014;25:684-8
|How to cite this URL:|
Zhou TB, Lin N, Qin YH, Liu YG. Distribution of pathological finding in the children with nephrotic syndrome from Guangxi. Saudi J Kidney Dis Transpl [serial online] 2014 [cited 2019 Nov 20];25:684-8. Available from: http://www.sjkdt.org/text.asp?2014/25/3/684/132240
Tian-Biao ZhouFNx01, Na LinFNx01, Yuan-Han QinFNx01
FNx01The authors wish it to be known that, in their opinion, the first three authors should be regarded as joint First Authors.
| Introduction|| |
Primary nephrotic syndrome (PNS) is the most common glomerular nephropathy in pediatrics, with great variation in patients' characteristics in different regions of the world.  Many investigators reported the pathological characteristics of PNS children in their countries or regions with variations of their findings. There are no reports investigating the pathological characteristics of PNS in children in the Guangxi Zhuang autonomous region, which is the most important province in south China.
In this study, we retrospectively analyzed the clinical and pathological characteristics of PNS in the children of our region.
| Patients and Methods|| |
All the children in our study underwent kidney biopsies in the Department of Pediatrics in two main medical centers in Guangxi, the First Affiliated Hospital of Guangxi Medical University and the Affiliated Hospital of Youjiang National Medical College. We excluded from the study the children with eight or less glomeruli in the obtained biopsies and those with incomplete clinical data.
Patients were diagnosed to suffer from PNS according to the criteria formulated by the International Study of Kidney Disease in Children (ISKDC). 
According to the clinical manifestations, the PNS was divided into simple nephrotic syndrome (simple NS) and nephritis nephrotic syndrome (nephritis NS), which has glomerular hematuria in addition to gross proteinuria, recurrent or persistent hypertension (6-14 years old ≥130/90 mm Hg, 1-5 years old ≥120/80 mm Hg), renal insufficiency and sustained hypocomplementemia.
The steroid responses were defined as follows:  Steroid-sensitive nephrotic syndrome (SSNS) was defined as the disappearance of proteinuria (i.e. urinary protein <4 mg/h/m 2 or negative protein on dipstick examination) for at least three consecutive days, after a course of prednisolone 2 mg/kg/day (maximum 60 mg/day) for four or six weeks; steroid-non-responsive nephrotic syndrome (SRNS) was defined as persistence, i.e., a minimum exposure of eight weeks of prenisolone 2 mg/kg/d or 60 mg/m 2 /d for four weeks followed by 1.5 mg/kg or 40 mg/m2 per dose alternate-day for four weeks; steroid-dependent nephrotic syndrome (SDNS) was defined as the tendency to relapse while on or during tapering of the steroid dose or within 14 days of steroid withdrawal.
The pathological types were classified according to the World Health Organization (WHO) recommendations, , including minimal change disease (MCD), mesanioproliferative glomerulonephritis (MsPGN), focal segmental glomerulosclerosis (FSGS), membranous nephropathy (MN), membranoproliferative glomerulonephritis (MPGN) and IgA nephropathy (IgAN).
Outcome of the PNS: (1) clinical cure: Complete remission: Without recurrence for more than three years; (2) complete remission: Routine blood test, blood biochemistry and urine examination were normal; (3) partial remission: Urine protein <+ + +; (4) no remission: Urinary protein ≥+ + +.
| Statistical Analysis|| |
Data were analyzed using statistical package for social sciences 13.0 (SPSS 13.0). Variables were compared using the χ2 test. P <0.05 was selected as the level of statistical significance.
| Results|| |
We studied 218 children with the PNS; 145 (66.51%) children were males and the age of the patients ranged from 1-14 years, with a mean of 8.2 ± 3.1 years. The detailed distributions of the PNS according to gender and age are shown in [Figure 1]. The duration of the disease was from 1 month to 6 years, with a mean of 38 ± 8.6 months.
|Figure 1: (a) Sex characteristics of the recruited PNS children; (b) age characteristics of the recruited PNS children.|
Click here to view
There were 84 (38.5%) children who presented with simple NS and 134 (61.5%) who presented with nephritis NS. The most common pathological findings included MsPGN (105 patients, 48.2%), FSGS (36 cases, 16.5%), IgAN (29 patients, 13.3%), MCD (25 patients, 11.5%), MPGN (10 cases, 4.6%) and MN (8 patients 3.67%) [Figure 2].
|Figure 2: Distributions of pathological characteristics. MsPGN: Mesangial proliferative glomerulonephritis, FSGS: Focal segmental glomeralosclerosis, IgAN: Immunoglobulin A nephropathy, MCD: Minimal change disease, MPGN: Membranoproliferative glomerulonephritis, MN: Membranous nephropathy.|
Click here to view
The principal manifestations of MsPGN, FSGS and IgAN were nephritis NS, and the principal manifestation of MCD was simple NS [Table 1]. There was a significantly statistical difference in the distributions of pathological characteristics between simple NS and nephritis NS (χ2 = 32.19, P <0.01).
|Table 1: Relationship between pathological characteristics and clinical manifestations.|
Click here to view
The correlation between the pathological patterns and steroid responses are shown in [Table 2]. A significantly statistical difference in the distributions of pathological findings was observed among the SSNS, SRNS and SDNS groups (χ2 = 62.59, P <0.01).
|Table 2: The correlation between the pathological characteristics and steroid responses in the study patients.|
Click here to view
One hundred and fifty-six patients were successfully followed-up by telephone or mail, and the data of the prognosis are presented in [Table 3]. There was a significantly statistical difference for the distributions of the pathological characteristics among the clinical cure, complete remission and no remission groups (χ2 = 104.66, P <0.05).
| Discussion|| |
In our study, we found that children who were six- to ten-year-old were the most susceptible population for the PNS, and the main pathological characteristics of PNS children were MsPGN, FSGS, IgAN and MCD. Interestingly, we found that all the IgAN children had accompanying nephritis NS and all the FSGS children were not steroid sensitive. Furthermore, the children with IgAN or MCD had a good prognosis.
MCD was the most common histopathological lesion reported in the children with PNS. ,,,, However, there are some reports that found FSGS and MsPGN as the most common histopathological pattern in their children with PNS. ,, In our study, MsPGN and FSGS presented a higher percentage than MCD.
In our study, we found that the differences of MsPGN, FSGS, MCD, MPGN and MN distributions between simple NS and nephritis NS were not notable. However, all the IgAN children were suffering from a clinical manifestation of nephritis NS. There was no study reporting the distribution characteristics of IgAN in simple NS and nephritis NS from children with PNS.
The correlation between the pathological characteristics and the steroid responses was also investigated. We found that all the FSGS children were not steroid sensitive; it was consistent with some other reports, which found that most of the FSGS patients were not sensitive to the steroid treatment. ,,
The follow-up of our study patients with IgAN or MCD disclosed a good prognosis. Most of the patients with IgAN were sensitive to the steroid administration as those with MCD.
This study provided data of the clinicopathological features of the PNS in children at two main medical centers in Guangxi. The sample size in our study was small, and larger studies should be performed to confirm our findings in the future.
Conflict of Interest: None.
Funding Source: None.
| References|| |
|1.||Kaddah A, Sabry S, Emil E, El-Refaey M. Epidemiology of primary nephrotic syndrome in Egyptian children. J Nephrol 2012;25:732-7. |
|2.||The primary nephrotic syndrome in children. Identification of patients with minimal change nephrotic syndrome from initial response to prednisone. A report of the International Study of Kidney Disease in Children. J Pediatr 1981;98:561-4. |
|3.||Zhou TB, Qin YH, Ou C, et al. A metaanalysis of the association between angiotensin-converting enzyme insertion/deletion gene polymorphism and steroid-sensitive nephrotic syndrome in children. J Renin Angiotensin Aldosterone Syst 2012;13:175-83. |
|4.||Churg J, Sobin L. Renal disease: Classification and atlas of glomerular disease. Tokyo: Igaku-Shoin; 1982. p. 425. |
|5.||Churg J, Bernstein J, Glassock R. Renal disease: Classification and atlas of glomerular disease. 2 nd ed. New York: Igaku-Shoin; 1995. p. 42. |
|6.||Moorani KN, Sherali AR. Histopathological pattern in childhood glomerulonephritis. J Pak Med Assoc 2010;60:1006-9. |
|7.||Mubarak M, Lanewala A, Kazi JI, et al. Histopathological spectrum of childhood nephrotic syndrome in Pakistan. Clin Exp Nephrol 2009;13:589-93. |
|8.||Khan AZ, Ahmad A. Minimal change disease, the leading cause of glomerulopathies in paediatric population at Peshawar. J Ayub Med Coll Abbottabad 2010;22:26-8. |
|9.||Bonilla-Felix M, Parra C, Dajani T, et al. Changing patterns in the histopathology of idiopathic nephrotic syndrome in children. Kidney Int 1999;55:1885-90. |
|10.||Hogg RJ, Silva FG, Berry PL, Wenz JE. Glomerular lesions in adolescents with gross hematuria or the nephrotic syndrome. Report of the Southwest Pediatric Nephrology Study Group. Pediatr Nephrol 1993;7:27-31. |
|11.||Gulati S, Sharma AP, Sharma RK, Gupta A. Changing trends of histopathology in childhood nephrotic syndrome. Am J Kidney Dis 1999;34:646-50. |
|12.||Lichtig C, Ben-Izhak O, On A, Levy J, Allon U. Childhood minimal change disease and focal segmental glomerulosclerosis: A continuous spectrum of disease? Pathologic study of 33 cases with long-term follow-up. Am J Nephrol 1991;11:325-31. |
|13.||Bircan Z, Yavuz Yilmaz A, Katar S, Vitrinel A, Yildirim M. Childhood idiopathic nephrotic syndrome in Turkey. Pediatr Int 2002;44:608-11. |
|14.||Sozeri B, Mir S, Mutlubas F, Sen S. The long-term results of pediatric patients with primary focal and segmental glomerulosclerosis. Saudi J Kidney Dis Transpl 2010;21:87-92. |
|15.||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. |
|16.||Shatat IF, Schoeneman M, Flynn JT, Woroniecki RP. Association of steroid and cyclosporin resistance in focal segmental glomerulosclerosis. Pediatr Nephrol 2007;22:834-9. |
Dr. Tian-Biao Zhou
Department of Pediatric Nephrology, The First Affiliated Hospital of Guangxi Medical University, Nanning 530021, Guangxi
[Figure 1], [Figure 2]
[Table 1], [Table 2], [Table 3]