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Saudi Journal of Kidney Diseases and Transplantation
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LETTER TO THE EDITOR  
Year : 2013  |  Volume : 24  |  Issue : 1  |  Page : 139-140
Profile and outcome of infantile nephrotic syndrome treated in a tertiary care center


1 Pediatric Nephrology, Sri Ramachandra Medical College and University, Porur, Chennai, India
2 Pediatric Nephrology, Bharathi Vidyapeeth Deemed University, Pune, India

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Date of Web Publication22-Jan-2013
 

How to cite this article:
Senguttuvan P, Prasad HK. Profile and outcome of infantile nephrotic syndrome treated in a tertiary care center. Saudi J Kidney Dis Transpl 2013;24:139-40

How to cite this URL:
Senguttuvan P, Prasad HK. Profile and outcome of infantile nephrotic syndrome treated in a tertiary care center. Saudi J Kidney Dis Transpl [serial online] 2013 [cited 2020 Jun 5];24:139-40. Available from: http://www.sjkdt.org/text.asp?2013/24/1/139/106312
To the Editor,

Nephrotic syndrome is known to occur even in the first year of life. Inherited causes of nephrotic syndrome during infancy include congenital nephrotic syndrome of Finnish type and diffuse mesangial sclerosis. [1] Nephrotic syndrome with onset before three months is considered as congenital, and between three and 12 months is considered infantile. [2]

We retrospectively reviewed the medical records of 20 Indian children with infantile nephrotic syndrome (INS) admitted in the Institute of Child Health and Hospital for Children, Chennai, India, from the period of 1 st January 2008 to 1 st December 2010. A diagnosis of INS was entertained in children with clinical and laboratory features as per the International Study of Kidney Disease in Children (ISKDC) [3] criteria, with onset of symptoms between three and 12 months. [2] Hypertension was defined as blood pressure measurements ≥90 th percentile for age, sex and height. [4] Anthropometric measurements were converted into Z-scores. [5] Renal biopsy was performed by a Trucut needle under ultrasound guidance. The case records were reviewed for clinical features, investigations, management modalities and outcome.

Twenty children who fulfilled the criteria for INS were included in the study. Of the 20 subjects, 13 (65%) were males and seven (35%) were females, with a male to female ratio of 1.85:1 and a mean age of 7.2 ± 2.2 months (range: 3.5-11.4). Four and three infants were products of second and third degree consanguinity each. Edema, abdominal distension and oliguria were the predominant presenting features in all the 20 (100%) subjects, followed by fever in five (25%) and hypertension in three (15%). The mean height Z-score was -1.23 and weight Z-score was -1.11 compared to the population as per the WHO standards. No obvious congenital anomalies were noted in any infant.

Investigations revealed microscopic hematuria in four (20%), gross hematuria in one (5%) and pyuria in seven (35%) subjects. Mean serum cholesterol was 406.78 ± 21.1 mg/dL (range 220-760 mg/dL), mean serum albumin was 1.2 g/dL ± 0.45 (range: 0.82-2 gm/dL) and mean 24-h urinary protein excretion was 2.5 ± 0.54 g/day (range: 1.86-2.9 g/day). Renal parameters were abnormal in three children, with a mean serum creatinine of 3.5 mg/dL in them, renal parameters were normal in 17 infants. Uropathogens were cultured in five subjects - E. coli in four and Klebsiella in one subject. Serology for syphilis, toxoplasma, cytomegalovirus, rubella and HIV performed in 18 infants were negative. Karyotype done in ten infants was normal. Thyroid functions assessed in all infants were normal. Enlarged kidneys with increased echogenicity of parenchyma and exaggerated corticomedullary differentiation were the predominant ultrasound findings.

Biopsy after parental consent was done in ten children; one infant had membranoproliferative glomerulonephritis type I, seven infants had diffuse mesangial sclerosis and the remaining two infants showed diffuse mesangial proliferation. Biopsy was not done in the other infants as either parental consent was denied or they were too sick for the procedure.

The subjects were treated with 20% human albumin at a dose of 1 g/kg and frusemide 2 mg/kg/day, and all were started on 2 mg/kg of prednisolone. If they did not respond to treatment for eight weeks, they were termed as steroid resistant and they were treated with enalapril 0.3 mg/kg. Infants presenting with acute renal failure underwent peritoneal dialysis.

Of the 20 cases, three (15%) expired due to sepsis, two (10%) expired due to respiratory failure and five (25%) were lost to follow-up. Among the remaining subjects on follow-up, eight (40%) became steroid resistant and two (10%) were steroid dependant.

INS with diffuse mesangial sclerosis is characterized by onset of proteinuria during infancy and poor prognosis with early onset of uremia, and most children die due to sepsis. [6] Aggressive medical management including nutritional supplementation with high calories, high protein and low sodium formula should be given. Daily infusions of 20% salt-poor albumin and diuretics are useful. [2] In our study also, male predominance, development of steroid resistance and diffuse mesangial sclerosis as a predominant biopsy finding, which is in concurrence with the other studies. [6],[7]

Persistent severe proteinuria and refractory edema, in spite of aggressive medical management, constitute indications for unilateral or bilateral nephrectomy and maintenance dialysis until the child undergoes renal transplant. [8] Based on the anti-proteinuric action of ACE inhibitors and NSAIDS in nephrotic syndrome, these drugs can be considered before unilateral or bilateral nephrectomy is performed. [8],[9]

An important limitation in our study is the lack of genetic studies owing to the lack of specialized laboratories and financial constraints of the parents. Mutations in NPHS1, NPHS2, PLACE1 and WT1 genes have been reported earlier in INS. [10] Children with INS have a poor outcome. Forty percent of the children become steroid resistant. As sepsis is the main cause of mortality, intravenous immunoglobulin and antibiotic prophylaxis may be considered.

 
   References Top

1.Niaudet P. Genetic forms of nephrotic syndrome. Pediatr Nephrol 2004;19:1313-8.  Back to cited text no. 1
[PUBMED]    
2.Bagga A, Srivastava RN. Nephrotic syndrome. In: Srivastava RN, Bagga A, editors. Pediatric Nephrology. 4 th ed. New Delhi: Jaypee Publishers; 2005. p. 159-200.  Back to cited text no. 2
    
3.International Study of Kidney Disease in Children. The primary nephrotic syndrome in children: identification of patients with minimal change nephrotic syndrome from initial response to prednisone. J Pediatr 1981;98:561-4.  Back to cited text no. 3
[PUBMED]    
4.National Heart Lung, and Blood Institute. The Fourth Report on the Diagnosis, Evaluation, and Treatment of High Blood Pressure in Children and Adolescents. Bethesda, Maryland: US Department of Health and Human Services, Public Health Service, National Institutes of Health, National Heart Lung and Blood Institute. 2004; NIH Publication No. 05-5267.   Back to cited text no. 4
    
5.Agarwal DK, Agarwal KN. Physical growth in Indian affluent children (Birth - 6 years). Indian Pediatr 1994;31:377-413.   Back to cited text no. 5
[PUBMED]    
6.Vachvanichsanong P, Mitarnun W, Tungsinmunkong K, Dissaneewate P. Congenital and infantile nephrotic syndrome in Thai infants. Clin Pediatr 2005;44:169-74.  Back to cited text no. 6
[PUBMED]    
7.Mattoo TK, Al Sowailem AM, Al Harbi MS, Mahmood MA. Nephrotic syndrome in first year of life and role of unilateral nephrectomy. J Pediatr Nephrol 1992;6:(1):16-8??.  Back to cited text no. 7
    
8.Heaton PA, Smlaes O, Wong W. Congenital nephrotic syndrome responsive to captopril and indomethacin. Arch Dis Child 1999;81:174-5.  Back to cited text no. 8
    
9.Pomeranz A, Wolach B, Benheim J, Korzets Z. Succesful treatment of Finnish Congenital nephrotic syndrome with Captopril and Indomethacin. J Pediatr 1995;126:140-2.   Back to cited text no. 9
    
10.Ismaili K, Pawtowski A, Boyer O, Wissing KM, Janssen F, Hall M. Genetic forms of nephrotic syndrome: A single-center experience in Brussels. Pediatr Nephrol 2009;24:287-94.  Back to cited text no. 10
[PUBMED]    

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Correspondence Address:
Hemchand Krishna Prasad
Pediatric Nephrology, Bharathi Vidyapeeth Deemed University, Pune
India
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DOI: 10.4103/1319-2442.106312

PMID: 23354212

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