Single-center Experience of Histopathological Spectrum and Treatment Profile in Adolescent-onset Nephrotic Syndrome in India
Journal of Pediatric Nephrology,
Vol. 6 No. 1 (2018),
4 May 2018
,
Page 1-4
https://doi.org/10.22037/jpn.v6i1.20566
Abstract
Introduction: Guidelines suggest a biopsy-tailored treatment rather than glucocorticoids as initial therapy for adolescent-onset nephrotic syndrome (NS) as minimal change disease is more common in younger children. There is a paucity of data regarding the clinical course and renal histopathological findings in this population. This study analyzed the clinical course and histological spectrum of adolescent-onset NS.
Materials and Methods: In this retrospective study, we evaluated the medical records of patients with adolescent-onset NS who received treatment at the Department of Pediatrics, SVPPGIP & SCB Medical College, Cuttack, Orissa, India, between January 2010 and January 2017. Patients with congenital, infantile, childhood-onset, and secondary causes of NS were excluded. All children were treated as per the Indian Society of Pediatric Nephrology (ISPN) protocol.
Results: The data of 48 adolescents were analyzed. The median age at onset of disease was 12 years (range: 10-14 years), and 68.75% (33/48) of the patients were males. Steroid-dependent NS (SDNS, 43.7%, 21/48) was the most common clinical course followed by frequently relapsing NS (FRNS, 29.1%, 14/48). Prednisolone with tacrolimus (37.5%, 18/48) and mycophenolatemofetil (35.4%, 17/48) were the most commonly used treatments. Biopsy results showed that minimal change disease (MCD) was the most common histopathological subtype (37.5%, 18/48) closely followed by focal segmental glomerulosclerosis (FSGS, 31.2%, 15/48). Most of the cases responded to a combination of prednisolone with either MMF, cyclophosphamide, or tacrolimus (23%).
Conclusions: The most common underlying cause of adolescent-onset nephrotic syndrome as assessed histopathologically is MCD, closely followed by FSGS with most of the cases responding to a combination of prednisolone with either mycophenolatemofetil, cyclophosphamide or tacrolimus.
Keywords: Adolescent-onset nephrotic syndrome; Minimal change disease; Focal segmental glomerulosclerosis; Steroid; Histopathology.
How to Cite
References
Hunley T, Yared A, Fogo A, MacDonell R. Nephrotic syndrome in an adolescent: The cry of the wolf. American Journal of Kidney Diseases 1998;31:155-160.
Burgstein J, Nephrotic syndrome. In: Behrman RE, Kliegman RM, Jenson HB, editors. Nelson Textbook of Pediatrics. 18th ed. Philadelphia: Saunders WB; pp. 2430–42. 2008.
Safaei AASL, Maleknejad S. Clinical and laboratory findings and therapeutic responses in children with nephrotic syndrome. Indian Journal of Nephrology 2010;20:68-71.
Trompeter RS, Lloyd BW, Hicks J, White RH, Cameron JS. Long-term outcome for children with minimal-change nephrotic syndrome. Lancet 1985;1:368-70.
Hogg RJ. Adolescents with proteinuria and/or the nephrotic syndrome. Adolesc Med Clin 2005;16:163-72.
Hodson EM, Knight JF, Willis NS, Craig JC. Corticosteroid therapy for nephrotic syndrome in children. Cochrane Database Syst Rev 2003:Cd001533.
Gulati S, Sural S, Sharma RK, Gupta A, Gupta RK. Spectrum of adolescent-onset nephrotic syndrome in Indian children. Pediatr Nephrol 2001;16:1045-8.
Srivastava T, Simon SD, Alon US. High incidence of focal segmental glomerulosclerosis in nephrotic syndrome of childhood. Pediatr Nephrol 1999;13:13-8.
Gall Z, Duicu C, Kiss E, Egyed-Zsigmond I, Cucerea M, Simon M. Clinical Features and Histopathological Spectrum in Adolescent Onset Nephrotic Syndrome in a Romanian Children Population. Acta Medica Marisiensis 2014;60:146.
Shakeel S, Mubarak M, Kazi JI. Frequency and clinicopathological correlations of histopathological variants of pediatric idiopathic focal segmental glomerulosclerosis. Indian Journal of Nephrology 2014;24:148-153.
INDIAN PEDIATRIC NEPHROLOGY GROUP, INDIAN ACADEMY OF PEDIATRICS. Management of Steroid Sensitive Nephrotic Syndrome: Revised Guidelines. 2008;45:203-214.
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.
- Abstract Viewed: 316 times
- PDF Downloaded: 208 times