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Renal Parenchymal Disease

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Pediatric Urogenital Radiology

Part of the book series: Medical Radiology ((Med Radiol Diagn Imaging))

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Abstract

Renal parenchymal disease (RPD) is classified into glomerular, tubular, interstitial, and vascular disease. These various diseases may be congenital (hereditary) or acquired. They exclusively may affect the kidneys or are systemic disorders with a wide range of renal contribution. This chapter focuses on the “classic entities” of RPD like glomerulonephritis (GN) or nephrotic syndrome (NS) showing clinical presentation, pathogenesis, renal histology if renal biopsy is indicated, treatment, and prognosis of the various diseases. References are restricted to important new publications where the interested reader can also find suggestions for further reading. The implications for imaging including ultrasound-guided renal biopsy are described in chapter “Imaging in Renal Parenchymal Disease”.

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References

  • Akchurin O, Reidy KJ (2015) Genetic causes of proteinuria and nephrotic syndrome: impact on podocyte pathobiology. Pediatr Nephrol 30:221–233

    Article  PubMed  Google Scholar 

  • Alchi B, Jayne D (2010) Membranoproliferative glomerulonephritis. Pediatr Nephrol 25:1409–1418

    Article  PubMed  Google Scholar 

  • Alchi B, Griffiths M, Jayne D (2010) What nephrologists need to know about antiphospholipid syndrome. Nephrol Dial Transplant 25:3147–3154

    Article  PubMed  Google Scholar 

  • Ayalon R, Beck LH Jr (2015) Membranous nephropathy: not just a disease for adults. Pediatr Nephrol 30:31–39

    Article  PubMed  Google Scholar 

  • Baker RJ, Pusey CD (2004) The changing profile of acute tubulointerstitial nephritis. Nephrol Dial Transplant 19:8–11

    Article  PubMed  Google Scholar 

  • Barbour TD, Pickering MC, Cook HT (2013) Dense deposit disease and C3 glomerulopathy. Semin Nephrol 33:493–507

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Beck LH Jr, Bonegio RG, Lambeau G et al (2009) M-type phospholipase A2 receptor as target antigen in idiopathic membranous nephropathy. N Engl J Med 361:11–12

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Becker AM (2011) Sickle cell nephropathy: challenging the conventional wisdom. Pediatr Nephrol 26:2099–2109

    Article  PubMed  Google Scholar 

  • Berger J, Hinglais N (1968) Les dépôts intercapillaires d’IgA-IgG. J Urol Neprol 74:694–695

    CAS  Google Scholar 

  • Berman H, Rodriguez-Pinto I, Cervera R et al (2014) Pediatric catastrophic antiphospholipid syndrome: descriptive analysis of 45 patients from the “CAPS Registry”. Autoimmun Rev 13:157–162

    Article  PubMed  Google Scholar 

  • Besbas N, Karpman D, Landau D et al (2006) A classification of hemolytic uremic syndrome and thrombotic thrombocytopenic purpura and related disorders. Kidney Int 70:423–431

    Article  CAS  PubMed  Google Scholar 

  • Bomback AS, Smith RJ, Barile GR et al (2012) Eculizumab for dense deposit disease and C3 glomerulonephritis. Clin J Am Soc Nephrol 7:748–756

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Brogan P, Eleftheriou D, Dillon M (2010) Small vessel vasculitis. Pediatr Nephrol 25:1025–1035

    Article  PubMed  Google Scholar 

  • Cakar N, Özcakar ZB, Soy D et al (2008) Renal involvement in childhood vasculitis. Nephron Clin Pract 108:c202

    Article  PubMed  Google Scholar 

  • Cara-Fuentes G, Wei C, Segarra A et al (2014) CD80 and suPAR in patients with minimal change disease and focal glomerulosclerosis: diagnostic and pathogenic significance. Pediatr Nephrol 29:1363–1371

    Google Scholar 

  • Cattran DC, Coppo R, Cook HT et al (2009) The Oxford classification of IgA nephropathy: rationale, clinicopathological correlations, and classification. Kidney Int 76:534–545

    Article  PubMed  Google Scholar 

  • Coppo R, Troyanov S, Camilla R et al (2010) The Oxford IgA nephropathy clinicopathological classification is valid for children as well as adults. Kidney Int 77:921–927

    Article  CAS  PubMed  Google Scholar 

  • Dallas MH, Triplett B, Shook DR et al (2013) Long-term outcome and evaluation of organ function in pediatric patients undergoing haploidentical and matched related hematopoietic cell transplantation for sickle cell disease. Biol Blood Marrow Transplant 19:820–830

    Article  PubMed  PubMed Central  Google Scholar 

  • Debiec H, Lefeu F, Kemper MJ et al (2011) Early-childhood membranous nephropathy due to cationic bovine serum albumin. N Engl J Med 364:2101–2110

    Article  CAS  PubMed  Google Scholar 

  • Delmas Y, Vendrely B, Clouzeau B et al (2014) Outbreak of Escherichia coli O104:H4 haemolytic uraemic syndrome in France: outcome with eculizumab. Nephrol Dial Transplant 29:565–572

    Article  CAS  PubMed  Google Scholar 

  • Dillon MJ, Eleftheriou D, Brogan PA (2010) Medium-size-vessel vasculitis. Pediatr Nephrol 25:1641–1652

    Article  PubMed  Google Scholar 

  • Edvardsson VO, Goldfarb DS, Lieske JC et al (2013) Hereditary causes of kidney stones and chronic kidney disease. Pediatr Nephrol 28:1923–1942

    Article  PubMed  PubMed Central  Google Scholar 

  • Eison TM, Ault BH, Jones DP et al (2011) Post-streptococcal glomerulonephritis in children: clinical features and pathogenesis. Pediatr Nephrol 26:165–180

    Article  PubMed  Google Scholar 

  • Eleftheriou D, Brogan PA (2009) Vasculitis in children. Best Pract Res Clin Rheumatol 23:309–323

    Article  PubMed  Google Scholar 

  • Emma F, Nesterova G, Langman C et al (2014) Nephropathic cystinosis: an international consensus document. Nephrol Dial Transplant 29:iv87–iv94

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Gauthier B, Trachtman H, Frank R et al (1989) Familial thin basement membrane nephropathy in children with asymptomatic microhematuria. Nephron 51:502–508

    Article  CAS  PubMed  Google Scholar 

  • Gbadegesin R, Winn M, Smoyer WE (2013) Genetic testing in nephrotic syndrome: challenges and opportunities. Nat Rev Nephrol 9:179–184

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Gellermann J, Weber L, Pape L et al (2013) Mycophenolate mofetil versus cyclosporin A in children with frequently relapsing nephrotic syndrome. J Am Soc Nephrol 24:1689–1697

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Gutierrez E, Zamora I, Ballarin JA et al (2012) Long-term outcomes of IgA nephropathy presenting with minimal or no proteinuria. J Am Soc Nephrol 23:1753–1760

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Hinkes BG, Mucha B, Vlangos CN et al (2007) Nephrotic syndrome in the first year of life: two thirds of cases are caused by mutations in 4 genes (NPHS1, NPHS2, WT1, and LAMB2). Pediatrics 119:e907–e919

    Article  Google Scholar 

  • Hochberg MC (1997) Updating the American College of Rheumatology revisited criteria for the classification of systemic lupus erythematosus. Arthritis Rheum 40:1725

    Article  CAS  PubMed  Google Scholar 

  • Houssiau FA, Vasconcelos D, D’Cruz D et al (2012) The 10-year follow-up data of the Euro-Lupus Nepritis Trial comparing low-dose and high-dose intravenous cyclophosphamide. Ann Rheum Dis 69:61–64

    Article  CAS  Google Scholar 

  • Huang E, Parke C, Mehrnia A et al (2013) Improved survival among sickle cell kidney transplant recipients in the recent era. Nephrol Dial Transplant 28:1039–1046

    Article  PubMed  Google Scholar 

  • Ishikura K, Hamasaki Y, Sakai T et al (2012) Posterior reversible encephalopathy syndrome in children with kidney diseases. Pediatr Nephrol 27:375–384

    Article  PubMed  Google Scholar 

  • Jahnukainen T, Ala-Houala M, Karikoski R et al (2011) Clinical outcome and occurrence of uveitis in children with idiopathic tubulointerstitial nephritis. Pediatr Nephrol 26:291–299

    Article  PubMed  Google Scholar 

  • Jais JP, Knebelmann B, Giatras I et al (2003) X-linked Alport syndrome: natural history and genotype-phenotype correlations in girls and women belonging to 195 families: A “European Community Alport Syndrome Concerted Action” study. J Am Soc Nephrol 14:2603–2610

    Article  PubMed  Google Scholar 

  • Jodele S, Fukuda T, Vinks A et al (2014) Eculizumab therapy in children with severe hematopoietic stem cell transplantation-assiciated thrombotic microangiopathy. Biol Blood Marrow Transplant 20:518–525

    Article  CAS  PubMed  Google Scholar 

  • Joss N, Morris S, Young B et al (2007) Granulomatous interstitial nephritis. Clin J Am Soc Nephrol 2:222–230

    Article  CAS  PubMed  Google Scholar 

  • Kashtan CE (2006) Renal transplantation in patients with Alport syndrome. Pediatr Transplant 10:651–657

    Article  PubMed  Google Scholar 

  • Kestilä M, Lenkkeri U, Männikkö M et al (1998) Positionally cloned gene for a novel glomerular protein-nephrin-is mutated in congenital nephrotic syndrome. Mol Cell 1:575–582

    Article  PubMed  Google Scholar 

  • Koehler H, Wandel E, Brunck N (1991) Acanthocyturia – A characteristic marker for glomerular bleeding. Kidney Int 40:115–120

    Article  Google Scholar 

  • Korkmaz C (2012) Therapeutic approach to patients with familial Mediterranean fever-related amyloidosis resistant to colchicine. Clin Exp Rheumatol 30(Suppl 72):S104–S107

    PubMed  CAS  Google Scholar 

  • Krug P, Boyer O, Balzamo E et al (2012) Nephrotic syndrome in Kawasaki disease: a report of three cases. Pediatr Nephrol 27:1547–1550

    Article  PubMed  Google Scholar 

  • Lameire N, Van Biesen W, Vanholder R (2005) Acute renal failure. Lancet 365:417–430

    Article  CAS  PubMed  Google Scholar 

  • Loirat C, Frémeaux-Bacci V (2011) Atypical hemolytic uremic syndrome. Orphanet J Rare Dis 6:60

    Article  PubMed  PubMed Central  Google Scholar 

  • Lombel RM, Gipson DS, Hodson EM (2013a) Treatment of steroid-sensitive nephrotic syndrome: new guidelines from KDIGO. Pediatr Nephrol 28:415–426

    Article  PubMed  Google Scholar 

  • Lombel RM, Hodson EM, Gipson DS (2013b) Treatment of steroid-resistant nephrotic syndrome in children: new guidelines from KDIGO. Pediatr Nephrol 28:409–414

    Article  PubMed  Google Scholar 

  • Lutalo PM, D’Cruz DP (2014) Diagnosis and classification of granulomatosis with polyangiitis (aka Wegener’s granulomatosis). J Autoimmun 48–49:94–98

    Article  CAS  PubMed  Google Scholar 

  • Mache CJ, Schwingshandl J, Riccabona M et al (1993) Ultrasound and MRI findings in a case of childhood amyloid goiter. Pediatr Radiol 23:565–566

    Article  CAS  PubMed  Google Scholar 

  • Mache CJ, Acham-Roschitz B, Frémeaux-Bacchi V et al (2009) Complement inhibitor Eculizumab in atypical hemolytic uremic syndrome. Clin J Am Soc Nephrol 4:1312.1316

    Article  CAS  PubMed Central  Google Scholar 

  • Malina M, Gulati A, Bagga A et al (2013) Peripheral gangrene in children with atypical hemolytic uremic syndrome. Pediatrics 131:e331–e335

    Article  PubMed  Google Scholar 

  • McKie KT, Hanevold CD, Hernandez C et al (2007) Prevalence, prevention, and treatment of microalbuminuria and proteinuria in children with sickle cell disease. J Pediatr Hematol Oncol 29:140–144

    Article  CAS  PubMed  Google Scholar 

  • Menon S, Valentini RP (2010) Membranous nephropathy in children: clinical presentation and therapeutic approach. Pediatr Nephrol 25:1419–1428

    Article  PubMed  Google Scholar 

  • Murakami M, Yamamoto H, Ueda Y et al (1991) Urinary screening of elementary and junior high-school children over a 13-year period in Tokyo. Pediatr Nephrol 5:50–53

    Article  CAS  PubMed  Google Scholar 

  • Nesterova G, Gahl WA (2013) Cystinosis: the evolution of a treatable disease. Pediatr Nephrol 28:51–59

    Article  PubMed  Google Scholar 

  • Noris M, Remuzzi G (2005) Hemolytic uremic syndrome. J Am Soc Nephrol 16:1035–1050

    Article  CAS  PubMed  Google Scholar 

  • Ozen S, Pistorio A, Iusan MS et al (2009) The EULAR/PRINTO/PRES criteria for childhood polyarteritis nodosa. Ankara 2008. Ann Rheum Dis 68(Suppl 3):713

    Google Scholar 

  • Park YH, Choi JY, Chung HS et al (2005) Hematuria and proteinuria in a mass school screening test. Pediatr Nephrol 20:1126–1130

    Article  PubMed  Google Scholar 

  • Pohl M (2015) Henoch-Schönlein purpura nephritis. Pediatr Nephrol 30:245–252

    Article  PubMed  Google Scholar 

  • Pozzi C, Andrulli S, Del Veccio L et al (2004) Corticosteroid effectiveness in IgA nephropathy: long-term results of a randomized, controlled trial. J Am Soc Nephrol 15:157–163

    Article  CAS  PubMed  Google Scholar 

  • Punaro MG (2013) The treatment of systemic lupus proliferative nephritis. Pediatr Nephrol 28:2069–2078

    Article  PubMed  Google Scholar 

  • Radhakrishnan S, Cattran DC (2012) The KDIGO practice guideline on glomerulonephritis: reading between the (guide)lines-application to the individual patient. Kidney Int 82:840–856

    Article  PubMed  Google Scholar 

  • Radhakrishnan S, Lunn A, Kirschfink M et al (2012) Eculizumab and refractory membranoproliferative glomerulonephritis. N Engl J Med 366:1165–1166

    Article  CAS  PubMed  Google Scholar 

  • Rana K, Wang YY, Buzza M et al (2005) The genetics of thin basement membrane nephropathy. Semin Nephrol 25:163–170

    Article  CAS  PubMed  Google Scholar 

  • Rosales A, Hofer J, Zimmerhackl LB et al (2012) Need for long-term follow-up in enterohemorrhagic Escherichia coli-associated hemolytic uremic syndrome due to late-emerging sequelae. Clin Inf Dis 54:1413–1421

    Article  Google Scholar 

  • Savige J, Gregory M, Gross O et al (2013) Expert guidelines for the management of Alport syndrome and thin basement membrane nephropathy. J Am Soc Nephrol 24:364–375

    Article  CAS  PubMed  Google Scholar 

  • Sethi S, Fervenza FC (2011) Membranoproliferative glomerulonephritis: pathogenetic heterogeneity and proposal for a new classification. Semin Nephrol 31:341–348

    Article  CAS  PubMed  Google Scholar 

  • Sethi S, Fervenza FC (2012) Membranoproliferative glomerulonephritis – a new look at an old entity. N Engl J Med 366:1119–1131

    Article  CAS  PubMed  Google Scholar 

  • Seyberth HW, Schlingmann KP (2011) Bartter- and Gitelman-like syndromes: salt-losing tubulopathies with loop or DCT defects. Pediatr Nephrol 26:1789–1802

    Article  PubMed  PubMed Central  Google Scholar 

  • Sharpe CC, Thein SL (2011) Sickle cell nephropathy – a practical approach. Br J Haematol 155:287–297

    Article  CAS  PubMed  Google Scholar 

  • Shenoy M, Plant ND, Lewis MA et al (2010) Intravenous methylprednisolone in idiopathic childhood nephrotic syndrome. Pediatr Nephrol 25:899–903

    Article  PubMed  Google Scholar 

  • Stone JH, Merkel PA, Spiera R et al (2010) Rituximab versus cyclophosphamide for ANCA-associated vasculitis. N Engl J Med 363:221–232

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Szugye HS, Zeft AS, Spalding SJ (2014) Takayasu arteritis in the pediatric population: a contemporary United States-based single center cohort. Pediatric Rheumatology 12:21

    Article  PubMed  Google Scholar 

  • Tan EM, Cohen AS, Fries JF et al (1982) The 1982 revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum 25:1271–1277

    Article  CAS  PubMed  Google Scholar 

  • Tarr PI, Gordon CA, Chandler WL (2005) Shiga-toxin-producing Escherichia coli and haemolytic uraemic syndrome. Lancet 365:1073–1086

    PubMed  CAS  Google Scholar 

  • Town M, Jean G, Cherqui S et al (1998) A novel gene encoding an integral membrane protein is mutated in nephropathic cystinosis. Nature Genet 18:319–324

    Article  CAS  PubMed  Google Scholar 

  • Tullus M (2012) New developments in the treatment of systemic lupus erythematosus. Pediatr Nephrol 27:727–732

    Article  PubMed  Google Scholar 

  • Van Husen M, Kemper MJ (2011) New therapies in steroid-sensitive and steroid-resistant idiopathic nephrotic syndrome. Pediatr Nephrol 26:881–892

    Article  PubMed  Google Scholar 

  • Vehaskari VM, Rapola J (1982) Isolated Proteinuria: analysis of a school-age population. J Pediatr 101:661–668

    Article  CAS  PubMed  Google Scholar 

  • Vehaskari VM, Rapola J, Koskimies O et al (1979) Microscopic hematuria in school children: epidemiology and clinicopathologic evaluation. J Pediatr 95:676–684

    Article  CAS  PubMed  Google Scholar 

  • Vester U, Mache CJ (2014) Pharmacologic treatment of atypical hemolytic-uremic syndrome. Exp Opin Orphan Drugs 2:123–135

    Article  CAS  Google Scholar 

  • Watanabe T (2013) Kidney and urinary tract involvement in Kawasaki disease. Int J Pediatr Article ID 831834

    Google Scholar 

  • Weening JJ, D’Agati VD, Schwartz MM et al (2004) The classification of glomerulonephritis in systemic lupus erythematosus revisited. J Am Soc Nephrol 15:241–250

    Article  Google Scholar 

  • Wyatt RJ, Julian BA (2013) IgA nephropathy. N Engl J Med 368:2402–2414

    Article  CAS  Google Scholar 

  • Zand L, Specks U, Sethi S et al (2014) Treatment of ANCA-associated vasculitis: new therapies and a look at old entities. Adv Chronic Kidney Dis 21:182–193

    Article  PubMed  Google Scholar 

  • Zuber J, Fakhouri F, Roumenina LT et al (2012) Use of eculizumab for atypical hemolytic uremic syndrome and C3 glomerulopathies. Nat Rev Nephrol 8:643–657

    Article  CAS  Google Scholar 

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Correspondence to Ekkehard Ring MD (Retired) .

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Ring, E., Acham-Roschitz, B. (2018). Renal Parenchymal Disease. In: Riccabona, M. (eds) Pediatric Urogenital Radiology. Medical Radiology(). Springer, Cham. https://doi.org/10.1007/978-3-319-39202-8_12

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