Patterns of idiopathic nephrotic syndrome in Upper Egyptian children
abdelreheam, G., Ahmed, A., Fadda, S., Ali, S., Ahmed, N. (2017). Patterns of idiopathic nephrotic syndrome in Upper Egyptian children. EKB Journal Management System, 21(1), 135-160. doi: 10.21608/smj.2017.40106
Ghada abdelreheam; Ali Abo Elmagd Ahmed; Swsan Abd Al-Monium Fadda; Safaa Ali; Naglaa Ahmed. "Patterns of idiopathic nephrotic syndrome in Upper Egyptian children". EKB Journal Management System, 21, 1, 2017, 135-160. doi: 10.21608/smj.2017.40106
abdelreheam, G., Ahmed, A., Fadda, S., Ali, S., Ahmed, N. (2017). 'Patterns of idiopathic nephrotic syndrome in Upper Egyptian children', EKB Journal Management System, 21(1), pp. 135-160. doi: 10.21608/smj.2017.40106
abdelreheam, G., Ahmed, A., Fadda, S., Ali, S., Ahmed, N. Patterns of idiopathic nephrotic syndrome in Upper Egyptian children. EKB Journal Management System, 2017; 21(1): 135-160. doi: 10.21608/smj.2017.40106

Patterns of idiopathic nephrotic syndrome in Upper Egyptian children

Sohag Medical Journal
Article 14, Volume 21, Issue 1, January 2017, Page 135-160  XML PDF (95.12 K)
Document Type: Original Article
DOI: 10.21608/smj.2017.40106
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Authors
Ghada abdelreheam1; Ali Abo Elmagd Ahmed1; Swsan Abd Al-Monium Fadda2; Safaa Ali1; Naglaa Ahmed1
1Department,Faculty of Medicine, Sohag University, Sohag, Egypt.
2Pathology Department, Qasr El Eyni Hospital, Cairo University, Cairo, Egypt
Abstract
Background and aim of work: Idiopathic nephrotic syndrome (INS) is a common and important chronic kidney disease in childhood. Steroid response pattern and the underlying histopathological spectrum of INS show variabilities in the different geographical regions. This work aimed to highlight the clinical profile, steroid response pattern and histopathological spectrum of INS in our locality in Sohag, Upper Egypt.
Patients and Methods: A prospective study included 77 children with INS aged from 1-15 years followed for at least one year, diagnosed and treated at our pediatric nephrology unit in the period from January 2010 to December 2014 was done.The patients' demographic features, clinical profile, steroid response pattern and the underlying histopathological spectrum were studied.
Results: Total included patients were 77 children. The mean age at disease onset was 4.87±2.94 (range 1.5-15) years. Male/female ratio was 1.6/1. Steroid was given to all patients, 79.2% of patients had steroid sensitive nephrotic syndrome (SSNS), while 20.8% had steroid resistant nephrotic syndrome (SRNS). Out of 61 steroid sensitive patients, about 54% developed either steroid dependent nephrotic syndrome (SDNS) or frequently relapsing nephrotic syndrome (FRNS). Biopsy was done in 33.8% of patients and revealed focal segmental glomerulosclerosis (FSGS) in 42%, minimal change disease (MCD) in 39% and mesangio-proliferative glomerulonephritis (MesPGN) in 19% of patients. End stage renal disease (ESRD) developed in 1 patient with SRNS and FSGS.
Conclusion: About eighty percent of our patients were steroid sensitive but more than half of them had SDNS or FRNS. The main histopathological pattern seen in renal biopsies was FSGS
Keywords
Idiopathic nephrotic syndrome; steroid response and focal segmental glomerulosclerosis
Supplementary Files
References
  1. McCaffrey J, Lennon R, Webb NJ (2016) The non-immunosuppressive management of childhood nephrotic syndrome. Pediatr Nephrol 31:1383–1402
  2. Chanchlani R, Parekh RS (2016) Ethnic Differences in Childhood Nephrotic Syndrome. Frontiers in Pediatrics 4:39
  3. Pais P, Avner ED: Nephrotic syndrome. In: Kliegman RM, Stanton BF, Geme JW, Schor NF editors: Nelson Textbook of pediatrics, 20th ed, Philadelphia, Elsevier Inc, 2016; 527:2521-2527.
  4. Larkins N, Kim S, Craig J, Hodson E (2016) Steroid-sensitive nephrotic syndrome: an evidence-based update of immuno-suppressive treatment in children. Archives of Disease in Childhood 101(4):404-440
  5. Sureshkumar P, Hodson EM, Willis NS, Barzi F, Craig JC (2014) Predictors of remission and relapse in idiopathic nephrotic syndrome: a prospective cohort study. Pediatr Nephrol 29:1039–1046
  6. ISKDC (International study of Kidney Disease in Children) 1981 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 98(4):561–564  
  7. Ishikura K, Matsumoto S, Sako M, Tsuruga K, et al (2015) Clinical practice guideline for pediatric idiopathic nephrotic syndrome 2013: medical therapy. Clin Exp Nephrol 19:6–33
  8. Kaddah A, Sabry S, Emil E, El-Refaey M (2012) Epidemiology of primary nephrotic syndrome in Egyptian children. J Nephrol25 (05):732-737
  9. Bakr A, Eid R, Sarhan A, Hammad A,  El-Refaey AM, et al (2014) Pathological profile of biopsied Egyptian children with primary nephrotic syndrome: 15-year single center experience. J Nephrol 27(4):419-423
  10. Alharthi AA (2016) Patterns of Childhood Steroid-Sensitive and Steroid-Resistant Nephrotic Syndrome in Saudi Children: Clinicopathological Study of 87 Cases. Clinical Pediatrics 1-7. DOI: 10.1177/0009922816645521
  11. Ali SH, Ali AM, Najim AH (2016) The Predictive Factors for Relapses in Children with Steroid-Sensitive Nephrotic Syndrome. Saudi J Kidney Dis Transpl 27(1):67-72
  12. Bin-Mohanna M, Bin Alzoa A (2014) Pediatric Idiopathic Nephrotic Syndrome in Al-Sabeen Hospital Sana'a City-Yemen. Hadhramout Journal ofMedical Sciences (HJMS) 3(1,2):239-242
  13. Mortazavi F, Khiavi YS (2011) Steroid response pattern and outcome of pediatric idiopathic nephrotic syndrome: a single-center experience in northwest Iran. Therapeutics and Clinical Risk Management 7:167–171
  14. Hacıhamdioğlu DÖ, Kalman S, Gök F (2015) Long-term results of children diagnosed with idiopathic nephrotic syndrome; single center experience. Türk Ped Arş 50:37-44
  15. Wong W (2007) Idiopathic nephrotic syndrome in New Zealand children, demographic, clinical features, initial management and outcome after twelve-month follow-up: Results of a three-year national surveillance study. Journal of Paediatrics and Child Health 43:337–341
  16. Zhou TB, Lin N, Qin YH, Liu YG (2014)  Distribution of Pathological Finding in the Children with Nephrotic Syndrome from Guangxi. Saudi J Kidney Dis Transpl 25(3):684-688
  17. Niaudet P, Boyer O: Idiopathic nephrotic syndrome in childhood: clinical aspects. In: Avner ED, Harmon WE, Niaudet P, Yoshikawa N editors: Pediatric Nephrology. 6th ed. Germany: Springer-Verlag, 2009; 667-702.
  18. Pais, Ellis, Avner ED: Nephrotic syndrome. In: Kliegman RM, Stanton BF, Geme JW, Schor NF, Behrman RE, editors: Nelson Textbook of pediatrics 19th ed, Philadelphia, Elsevier Saunders, 2011; 521:1801-1807.
  19. Bagga A (2008) Revised guidelines for management of steroid-sensitive nephrotic syndrome. Indian J Nephrol 18(1):31–39
  20. KDIGO (Kidney Disease Improving Global Outcomes) Glomerulonephritis Work Group 2012: KDIGO clinical practice guideline for glomerulonephritis. Kidney Int Suppl 2:139-274
  21. Gipson DS, Massengill SF, Yao L, Nagaraj S, Smoyer WE, Mahan JD, et al (2009) Management of childhood onset nephrotic syndrome. Pediatrics 124(2):747–757
  22. Valentini RP, Smoyer WE: Nephrotic syndrome. In: Kher KK, Schnaper HW, Makker SP, editors: Clinical Pediatric Nephrology. Second edition, Informa Healthcare, 2007; 11:155-194.
  23. Mendonça AC, Oliveira EA, Fróe BP, Faria LD, et al (2015) A predictive model of progressive chronic kidney disease in idiopathic nephrotic syndrome. Pediatr Nephrol 30:2011–2020
  24. Inaba A, Hamasaki Y, Ishikura K, Hamada R, Sakai T, et al (2016) Long-term outcome of idiopathic steroid-resistant nephrotic syndrome in children. Pediatr Nephrol 31:425–434
  25. Gbadegesin R, Gibson KL, Smoyer WE: Steroid resistant Nephrotic Syndrome. In: Geary DF, Schaefer F Editors: Pediatric Kidney Disease, Second Edition, Springer-Verlag Berlin Heidelberg 2016; 16:455-478.
  26. Andersen RF, Thrane N, Noergaard K, Rytter L, Jespersen B, Rittig S (2010) Early age at debut is a predictor of steroid-dependent and frequent relapsing nephrotic syndrome. Pediatr Nephrol 25(7):1299–1304
  27. Letavernier B, Letavernier E, Leroy S, Baudet-Bonneville V, Bensman A, Ulinski T (2008) Prediction of high-degree steroid dependency in pediatric idiopathic nephrotic syndrome. Pediatr Nephrol 23(12):2221–2226
  28. Arif MK, Arif M, Amjad N (2016) A histopathological outlook on nephrotic syndrome: A pediatric perspective. Indian J Nephrol 26(3):188–191
  29. Kari JA (2002) Changing trends of histopathology in childhood nephrotic syndrome in western Saudia Arabia. Saudia Med J 23:317–321
  30. Asinobi AO, Ademola AD, Okolo CA, Yaria JO (2015) Trends in the histopathology of childhood nephrotic syndrome in Ibadan Nigeria: preponderance of idiopathic focal segmental glomerulosclerosis. BMC Nephrology 16:213
  31. Mubarak M, Lanewala A, Kazi JI, Akhter F, Sher A, Fayyaz A, Bhatti S (2009) Histopathological spectrum of childhood nephrotic syndrome in Pakistan. Clin Exp Nephrol 13(6):589–593
 

 

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