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2019, Number 6

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Acta Pediatr Mex 2019; 40 (6)

Urological malformations and nephrotic syndrome: A case report

Palacios-Legarda A, Gahona-Villegas J, Romero A, García-de la Puente S

Language: Spanish
References: 12
Page: 328-334
PDF size: 186.91 Kb.


ABSTRACT

Background: Nephrotic syndrome assumes an incidence of 1-3 per 100,000 children under 16 years. More than 90% of these cases manifest between the first and 10 years of age. Nephrotic syndrome secondary to reflux nephropathy is an alteration reported in adults; however, it has not been clearly established in children.
Clinical case: 2-year-old and 5-month-old girl, with a previous diagnosis of the left kidney with a double full pyeloureteral system, ureter from the ectopic upper pole and apparent reflux. He was admitted to the Pediatric Nephrology service for a 15-day course of evolution, characterized by bilateral eyelid edema, which radiated to the lower limbs, predominantly in the morning, hypertension and hematuria. Laboratory studies reported: dyslipidemia, proteinuria in the nephrotic limits, hematuria, hypoalbuminemia, normal complement and negative viral markers. Full dose corticosteroids (60 mg/m2/day) were prescribed, which reduced edema. However, proteinuria, hematuria and hypertension persisted. The diagnosis of corticorresistencia was established. Renal biopsy showed focal and segmental glomerulosclerosis cell type. The genetic study was negative to identify any gene related to the nephrotic syndrome. At the moment, the patient is being monitored by the Pediatric Nephrology service. With the histopathological report, treatment was initiated with calcineurin inhibitor and corticosteroids at low doses, with improvement in paraclinical studies. Today he suffers from nephrotic syndrome with partial remission.
Conclusions: It is important to continue reporting these types of cases, in order to establish the appropriate multidisciplinary and comprehensive treatment in children.


REFERENCES

  1. Cattran DC, et al. Kidney Disease: Improving Global Outcomes (KDIGO), Glomerulonephritis Work Group. KDIGO Clinical Practice Guideline for Glomerulonephritis. Kidney Inter Suppl 2012;2:139-274. https://doi.org/10.1038/ kisup.2012.9

  2. Eddy A, et al. Nephrotic syndrome in childhood. Lancet 2003;362:629-39. 10.1016/S0140-6736(03)14184-0.

  3. Mantan M, et al. Presence of CAKUT: A predictor of difficult-to-control nephrotic syndrome. Hong Kong J Nephrol 2013;15(1):44-47. https://doi.org/10.1016/j. hkjn.2013.03.006.

  4. D'Agati VD, et al. Focal segmental glomerulosclerosis. N Eng J Med 2011;365(25):2398-411. DOI: 10.1056/ NEJMra1106556

  5. Bakkaloglu SA, et al. Enfermedades del riñon y del tracto urinario en niños. En: Skorecki K, et al editors. Brener y Rector. El Riñón. 10ª ed. Madrid: Elsevier, 2018; 2308- 2364. https://books.google.com.mx/books?id=SqJTDw AAQBAJ&pg=PA2308&lpg=PA2308&dq=Sevcan+A.+Ba kkaloglu,Franz+Schaefer.&source=bl&ots=Xs5v2b-nJ6& sig=ACfU3U3PgoqwNQ7ThwyKpqYJYhI9sJy3IQ&hl=es- 419&sa=X&ved=2ahUKEwjJ1az-m7_lAhVSMawKHTU3BL kQ6AEwAHoECAkQAQ#v=onepage&q=Sevcan%20A.%20 Bakkaloglu%2CFranz%20Schaefer.&f=false

  6. Mora-Bautista VM. Anomalías congénitas del riñón y del tracto urinario en niños con síndrome de Down. Rev Colomb Nefrol 2018;5(1):54-60. https://revistanefrologia. org/index.php/rcn/article/view/268/html

  7. Palacios-Loro ML, et al. Anomalías nefrourológicas congénitas. Una visión para el pediatra. An Pediatr (Barc) 2015;83:442 DOI: 10.1016/j.anpedi.2015.07.035

  8. Román-Ortiz E. Síndrome nefrótico pediátrico. Protoc Diagn Ter Pediatr 2014;1:283-301. https://www.aeped. es/sites/default/files/documentos/18_sindrome_nefrotico. pdf

  9. Vats AN, et al. Steroid-resistant nephrotic syndrome and congenital anomalies of kidneys: evidence of locus on chromosome 13q. Kidney Int. 2003 Jul;64(1):17-24. DOI: 10.1046/j.1523-1755.2003.00066.x

  10. Melo-Tavares R, et al. Association between focal segmental glomerulosclerosis and cross-fused renalectopia: a new cause of secondary focal segmental glomerulosclerosis or a casual association?. World J Nephrol Urol 2015;4(1):183- 185. http://www.wjnu.org/index.php/wjnu/article/ view/207/172

  11. Rosenblum N. Enfermedades del riñon y del tracto urinario en niños.. En: Skorecki K, et al editors. Brener y Rector. El Riñón. 10ª ed. Madrid: Elsevier, 2018;2294-2307. https://books.google.com.mx/books?id =SqJTDwAAQBAJ&pg=PA2308&lpg=PA2308&dq=Sevcan+ A.+Bakkaloglu,Franz+Schaefer.&source=bl&ots=Xs5v2b-n J6&sig=ACfU3U3PgoqwNQ7ThwyKpqYJYhI9sJy3IQ&hl=es- 419&sa=X&ved=2ahUKEwjJ1az-m7_lAhVSMawKHTU3BL kQ6AEwAHoECAkQAQ#v=onepage&q=Sevcan%20A.%20 Bakkaloglu%2CFranz%20Schaefer.&f=false

  12. Adeyemo A, et al. HLA-DQA1 and APOL1 as Risk Loci for Childhood-Onset Steroid-Sensitie and Steroid-Resistant Nephrotic Syndrome. Am J Kidney Dis DOI: 10.1053/j. ajkd.2017.10.013




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