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Congenital and Infantile Nephrotic Syndrome Single Center Study

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Document Type : Research Paper

10.52573/ipmj.2019.191043
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Abstract

ABSTRACT:
BACKGROUND:
congenital nephrotic syndrome occurring in the first 3 months of life and infantile presenting after 3 to12 months.It differs from the nephrotic syndrome in older children in terms of histopathological finding and carries a poor prognosis.
OBJECTIVE:
To study the diagnosis and treatment of nephrotic syndrome cases manifested in the first year of life and their outcome.
PATIENTS AND METHODS:
A single-center retrospective study was conducted in the nephrology unit in central child teaching hospital, Baghdad, Iraq. During the study period from the 1st of July to end of December 2017,                 the records of all patients who diagnosed as nephrotic syndrome below one year from January 2007 to December 2016 with minimal follow up of six months in the nephrology department reviewed and analyzed.
RESULTS:
Seventy cases were diagnosed with nephrotic syndrome below one year from a total number of nephrotic 620 cases (30 of them congenital and 40 were an infantile type), their mean age 5.242 months. Male was higher than female (67.1% versus 32.9%) with a male to female ratio 2.04:1.                 In this study, patient represented infantile nephrotic were the higher proportion (57.1%) than congenital type (42.9%)both types represented (11.29%)of all nephrotic cases .concerning family history was slightly higher than those with negative history (52. 9%).The higher proportion of parents without consanguinity (67.1%).
Study patient who suffered from sepsis were of the higher proportion (62. 9%).In regard to hypertension in this study (92.9%) had no hypertension with a significant association between hypertension and infantile type. The proportion of those with elevated serum creatinine was (11.4%), anemic patients (51.4%) in regard to thyroid function all patients with congenital nephrotic had hypothyroidism while only (6.2%)of 32 cases with infantile had hypothyroidism. According to biopsy finding in (60) patients, Finnish type representing (91.3%)of patient with congenital nephrotic while in infantile type focal segmental glomerulosclerosis represent the highest proportion (54. 1%). regarding the outcome of study cases (80%) of these cases were ended with no remission, while remission was noticed among only (20%) of cases.
CONCLUSION:
Nephrotic syndrome in the first year constitute a small percentage of nephrotic syndrome and it had a poor prognosis, Finnish type represent the highest proportion of biopsy in congenital nephrotic syndrome and focal segmental glomerulosclerosis highest proportion in infantile type.
 
 

Keywords

  • congenital nephrosis
  • Finnish type
  • outcome
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References
  1. Sahay M, Gowrishankar S, Narayen et al. Nephrotic syndrome in the first year of life.J Acad Med Sci. 2015;p1–22.
  2. Hebert LA, Parikh S, Prosek J, et al. Differential diagnosis of glomerular disease: A systematic and inclusive approach. American Journal of Nephrology. 2013. p. 253–256.
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  6. Zenker M, Aigner T, Wendler et al. Human laminin beta2 deficiency causes congenital nephrosis with mesangial sclerosis and distinct eye abnormalities. Hum Mol Genet. 2004; p 2625–32.
  7. Besbas N, Bayrakci US, Kale et al. Cytomegalovirus-related congenital nephrotic syndrome with diffuse mesangial sclerosis. Pediatr Nephrol. 2006; p 2–740.
  8. Basker M, Agarwal I, Bendon et al. Congenital nephrotic syndrome - a treatable cause. Ann Trop Paediatr. 2007; p 89–90.
  9. Kari JA, Montini G, Bockenhauer D, et al. Clinico-pathological correlations of congenital and infantile nephrotic syndrome over twenty years. Pediatr Nephrol. 2014;29(11): P 2173–80.
  10. Kerlin BA, Haworth K, Smoyer et al. Venous thromboembolism in pediatric nephrotic syndrome., Pediatric Nephrology. 2015, Vol. 29, p. 989–97.
  11. Mundat L. Chemical Analysis of Urine. Graff’s Textbook of Urinalysis and Body Fluids. 2017. p 35–45.
  12. Samuel S, Bitzan M, Zappitelli et al. Canadian Society of Nephrology Commentary on the 2012 KDIGO clinical practice guideline for glomerulonephritis: management of nephrotic syndrome in children. Am J Kidney Dis. 2014 Mar;63(3): p 354–62.
    1. Habib R. Nephrotic syndrome in the 1st year of life. Pediatr Nephrol. 1993;7(4):p 347–53.Fischbach M, Niaudet P, Schaefer, et al. Pediatric nephrology. Int J Pediatr Nephrol. 2012; p 416–749.
    2. Mattoo TK, Al-Sowailem AM, et al. Nephrotic syndrome in 1st year of life and the role of unilateral nephrectomy. Pediatr Nephrol. 2015 Jan;6(1):p 16–8.
    3. Kari JA, Montini G, Bockenhauer D, et al. Clinico-pathological correlations ofcongenital and infantile nephrotic syndrome over twenty years. Pediatr Nephrol. 2014;29(11): P 2173–80.
    4. Vachvanichsanong P, Mitarnun W, et al. Congenital and infantile nephrotic syndrome in thai infants., Clinical Pediatrics. 2005, 44, p. 169–74.
    5. Sibley RK, Mahan J, Al M et al. A clinicopathologic study of forty-eight infants with nephrotic syndrome. Kidney Int. 2000 Mar;27(3):p 544–52.
    6. Senguttuvan P, Prasad HK et al. Profile and outcome of infantile nephrotic syndrome treated in a tertiary care center. Saudi J Kidney Dis Transpl. 2016 Jan;24(1): p 139–40. 
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Iraqi Postgraduate Medical Journal
Volume 18, Issue 3
September 2019
Page 277-284
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APA

(2019). Congenital and Infantile Nephrotic Syndrome Single Center Study. Iraqi Postgraduate Medical Journal, 18(3), 277-284. doi: 10.52573/ipmj.2019.191043

MLA

. "Congenital and Infantile Nephrotic Syndrome Single Center Study". Iraqi Postgraduate Medical Journal, 18, 3, 2019, 277-284. doi: 10.52573/ipmj.2019.191043

HARVARD

(2019). 'Congenital and Infantile Nephrotic Syndrome Single Center Study', Iraqi Postgraduate Medical Journal, 18(3), pp. 277-284. doi: 10.52573/ipmj.2019.191043

VANCOUVER

Congenital and Infantile Nephrotic Syndrome Single Center Study. Iraqi Postgraduate Medical Journal, 2019; 18(3): 277-284. doi: 10.52573/ipmj.2019.191043

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