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| Year : 2022 | Volume
: 5
| Issue : 2 | Page : 95-96 |
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Steroid dependent nephrotic syndrome with vesicoureteral reflux
Suchi Acharya, Biju M John
Department of Pediatrics, AFMC, Pune, Maharashtra, India
| Date of Submission | 20-May-2022 |
| Date of Decision | 05-Oct-2022 |
| Date of Acceptance | 11-Oct-2022 |
| Date of Web Publication | 31-Dec-2022 |
Correspondence Address:
Suchi Acharya
Department of Pediatrics, AFMC, Wanowrie, Pune - 411 040, Maharashtra
India
 Source of Support: None, Conflict of Interest: None
How to cite this article:
Acharya S, John BM. Steroid dependent nephrotic syndrome with vesicoureteral reflux. Asian J Pediatr Nephrol 2022;5:95-6 |
Sir,
A 3-year-old girl, firstborn of a nonconsanguineous marriage, presented with generalized edema, pain in the abdomen, increased frequency of micturition, and dysuria for the past 7 days. She also had a history of urge incontinence and nocturnal enuresis for the last 6 months. Physical examination was unremarkable except for anasarca.
At the initial visit, laboratory studies revealed hypoalbuminemia (serum albumin 1.0 g/dl), hyperlipidemia (cholesterol 482 mg/dl), and proteinuria (24-h urinary protein 4 g); blood levels of creatinine and electrolytes were normal. Urine culture showed significant growth of Escherichia More Details coli. Ultrasonography abdomen showed hydronephrosis affecting the left kidney. She was diagnosed with the first episode of idiopathic nephrotic syndrome with urinary tract infection, and treated with oral antibiotics and, later prednisolone.[1] She was also evaluated for urge incontinence and left-sided hydronephrosis. Dimercaptosuccinic acid (DMSA) scintigraphy showed bilateral normal kidneys without any scars. Micturating cystourethrogram revealed unilateral left-sided grade IV vesicoureteral reflux (VUR) [Figure 1]. L,L,ethylenedicysteine (LLEC) scintigraphy showed a bilateral nonobstructive drainage pattern with a differential function of 45% and 55% for the right and left kidneys, respectively. | Figure 1: Micturating cystourethrogram depicting left-sided grade IV vesicoureteral reflux
Click here to view |
Later, the patient showed a steroid-dependent course of nephrotic syndrome. In view of persistent urge incontinence, she underwent urodynamic studies that showed reduced detrusor compliance and moderate detrusor overactivity. She was treated with tolterodine and cotrimoxazole prophylaxis, with improvement in urinary symptoms. Although there was left-sided grade IV VUR, renal scans did not reveal any scarring, and the diagnosis of reflux nephropathy was not considered. For steroid dependence, she also received therapy with mycophenolate mofetil at a dose of 800 mg/m2. The patient is currently in remission for the past 1 year.
Nephrotic syndrome and congenital anomalies of the kidneys and urinary tract (CAKUT) are two important causes of chronic kidney disease. There are limited reports on the association between nephrotic syndrome and CAKUT in children.[2],[3],[4],[5] The present patient had steroid-dependent nephrotic syndrome along with VUR. Although the etiology of CAKUT is multifactorial, studies suggest a genetic basis in a small proportion of cases.[4],[6] Very few studies have shown a genetic association of nephrotic syndrome with CAKUT.[4],[5] Further studies with detailed phenotyping and comprehensive genetic evaluation are required to examine the association between nephrotic syndrome and CAKUT.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient's parents have given their consent for patient's images and other clinical information to be reported in the journal. The patient's parents understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
| References | |  |
| 1. | Sinha A, Bagga A, Banerjee S, Mishra K, Mehta A, Agarwal I, et al. Steroid sensitive nephrotic syndrome: Revised guidelines. Indian Pediatr 2021;58:461-81.  |
| 2. | Demaria WJ, Krueger RP, Anderson EE. Urinary tract abnormalities in the nephrotic syndrome. Seven cases demonstrating such an association. Clin Pediatr (Phila) 1972;11:530-3. |
| 3. | Berger M, Travis LB, Lorentz WB Jr., Carvajal HF. Transient obstructive uropathy associated with the nephrotic syndrome: Case report. J Pediatr 1973;83:456-8. |
| 4. | Vats AN, Ishwad C, Vats KR, Moritz M, Ellis D, Mueller C, et al. Steroid-resistant nephrotic syndrome and congenital anomalies of kidneys: Evidence of locus on chromosome 13q. Kidney Int 2003;64:17-24. |
| 5. | Tohidi MR, Seyedzadeh MS, Hookari S, Amiri R, Seyedzadeh A. Bilateral Vesicoureteral Reflux in Congenital Nephrotic Syndrome. Nephro-Urol Mon 2020;12:e107531. |
| 6. | Gimelli S, Caridi G, Beri S, McCracken K, Bocciardi R, Zordan P, et al. Mutations in SOX17 are associated with congenital anomalies of the kidney and the urinary tract. Hum Mutat 2010;31:1352-9. |
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