Congenital and Infantile Nephrotic Syndrome Single Center Study

doi:10.52573/ipmj.2019.191043

	Congenital and Infantile Nephrotic Syndrome Single Center Study
Iraqi Postgraduate Medical Journal
Volume 18, Issue 3, 2019, Pages 277-284 PDF (122.35 K)
Document Type: Research Paper
DOI: 10.52573/ipmj.2019.191043
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 1^st 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

References
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