Minimal change disease natural history, complications and prognosis
|
Minimal Change Disease Microchapters |
|
Diagnosis |
|---|
|
Treatment |
|
Case Studies |
|
Minimal change disease natural history, complications and prognosis On the Web |
|
American Roentgen Ray Society Images of Minimal change disease natural history, complications and prognosis |
|
FDA on Minimal change disease natural history, complications and prognosis |
|
CDC on Minimal change disease natural history, complications and prognosis |
|
Minimal change disease natural history, complications and prognosis in the news |
|
Blogs on Minimal change disease natural history, complications and prognosis |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Yazan Daaboul, Serge Korjian ; Vamsikrishna Gunnam M.B.B.S [2]
Overview
Complications associated with the pathogenesis of the disease as a nephrotic syndrome include thromboembolic events and disorders of hemostasis, hyperlipidemia, vulnerability to infections, and hypertension. Before the steroid era, patients died of renal failure and from infections. Nowadays, patients have excellent renal outcomes when they are still steroid-responsive and virtually all patients survive with a normal creatinine clearance. Although renal outcomes are considered excellent with appropriate therapy, the risk of chronic renal disease cannot be completely ruled out, especially among patients receiving nephrotoxic medications for prolonged periods of time.
Natural History
- The hallmark of minimal change disease in children is acute-onset proteinuria that progresses into nephrotic syndrome.
- Fatigue and subsequent edema develops with symptoms of periorbital edema and weight gain.[1]
- Children are less likely to present with other clinical features, such as hypertension, renal failure, or hematuria.[1][2]
- In contrast, adults are more likely to present with hypertension in approximately 40% of cases, and hematuria in approximately 30% of cases.
- A reduced estimated glomerular filtration rate (eGFR) at presentation is also not uncommon.
- Finally, infections, such as pneumonia in an otherwise healthy individual, may be the first sign of nephrotic syndrome in minimal change disease.[1]
Complications
Prognosis
Outcomes in minimal change disease have been mostly studied in the pediatric population, with very little knowledge in in adults. Before the steroid era, patients died of renal failure and from infections.[3] Nowadays, patients have excellent renal outcomes when they are still steroid-responsive and virtually all patients survive with a normal creatinine clearance.[3][4] Up to 80% of children with nephrotic syndrome respond to corticosteroids and become in full remission within 1 month of steroid therapy. Of those, 30% require only 1 course of steroids, 20% require several courses, and 30-50% suffer from frequently relapsing nephrotic syndrome (FRNS) when steroids are discontinued.[5][4] Age at disease onset is strongly associated with the frequency of relapses in children.
It was once believed that only 10% of children with minimal change disease persist into adulthood.[6][7] More recent data shows that the rate is in fact higher, reaching approximately 25-42%. Risk factors for persistence of minimal change disease into adulthood are as follows:[4][8][3]
- Young age at diagnosis
- Increased number of relapses
- Use of alkylating agents and cyclosporine
Although renal outcomes are considered excellent with appropriate therapy, the risk of chronic renal disease cannot be completely ruled out, especially among patients receiving nephrotoxic medications for prolonged periods of time.[4]
There are much less knowledge of outcomes and prognosis of adult-onset minimal change disease. In adults, 90% achieve remission with corticosteroids, but the rate of relapse of the first episode of nephrotic syndrome is as high as 70%, where approximately 30% have frequent relapses.[1] Young patients < 40 years at onset of disease are found in some studies to experience recurrence of disease than their older peers; these findings, however, have not been consistent in the literature.[9][10][11][1] The number of relapses is ultimately associated with long-term renal outcomes and steroid dependence.[1]
References
- ↑ 1.0 1.1 1.2 1.3 1.4 1.5 Waldman M, Crew RJ, Valeri A, Busch J, Stokes B, Markowitz G; et al. (2007). "Adult minimal-change disease: clinical characteristics, treatment, and outcomes". Clin J Am Soc Nephrol. 2 (3): 445–53. doi:10.2215/CJN.03531006. PMID 17699450.
- ↑ Saha TC, Singh H (2006). "Minimal change disease: a review". South Med J. 99 (11): 1264–70. PMID 17195422.
- ↑ 3.0 3.1 3.2 Kyrieleis HA, Löwik MM, Pronk I, Cruysberg HR, Kremer JA, Oyen WJ; et al. (2009). "Long-term outcome of biopsy-proven, frequently relapsing minimal-change nephrotic syndrome in children". Clin J Am Soc Nephrol. 4 (10): 1593–600. doi:10.2215/CJN.05691108. PMC 2758253. PMID 19808243.
- ↑ 4.0 4.1 4.2 4.3 Niaudet P (2009). "Long-term outcome of children with steroid-sensitive idiopathic nephrotic syndrome". Clin J Am Soc Nephrol. 4 (10): 1547–8. doi:10.2215/CJN.05950809. PMID 19808239.
- ↑ Tarshish P, Tobin JN, Bernstein J, Edelmann CM (1997). "Prognostic significance of the early course of minimal change nephrotic syndrome: report of the International Study of Kidney Disease in Children". J Am Soc Nephrol. 8 (5): 769–76. PMID 9176846.
- ↑ Koskimies O, Vilska J, Rapola J, Hallman N (1982). "Long-term outcome of primary nephrotic syndrome". Arch Dis Child. 57 (7): 544–8. PMC 1627702. PMID 7103547.
- ↑ Trompeter RS, Lloyd BW, Hicks J, White RH, Cameron JS (1985). "Long-term outcome for children with minimal-change nephrotic syndrome". Lancet. 1 (8425): 368–70. PMID 2857421.
- ↑ Rüth EM, Kemper MJ, Leumann EP, Laube GF, Neuhaus TJ (2005). "Children with steroid-sensitive nephrotic syndrome come of age: long-term outcome". J Pediatr. 147 (2): 202–7. doi:10.1016/j.jpeds.2005.03.050. PMID 16126050.
- ↑ Nakayama M, Katafuchi R, Yanase T, Ikeda K, Tanaka H, Fujimi S (2002). "Steroid responsiveness and frequency of relapse in adult-onset minimal change nephrotic syndrome". Am J Kidney Dis. 39 (3): 503–12. doi:10.1053/ajkd.2002.31400. PMID 11877569.
- ↑ Korbet SM, Schwartz MM, Lewis EJ (1988). "Minimal-change glomerulopathy of adulthood". Am J Nephrol. 8 (4): 291–7. PMID 3189423.
- ↑ Huang JJ, Hsu SC, Chen FF, Sung JM, Tseng CC, Wang MC (2001). "Adult-onset minimal change disease among Taiwanese: clinical features, therapeutic response, and prognosis". Am J Nephrol. 21 (1): 28–34. doi:46215 Check
|doi=value (help). PMID 11275629.