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Lipoid Nephrosis

Minimal Change Disease

Lipoid nephrosis, more commonly known as minimal change disease, is a term describing a form of glomerulonephritis that can result in nephrotic syndrome and progress to end-stage renal disease in rare cases. The disorder is usually seen in children, when proteinuria, and less commonly hypertension and edema, present as main clinical features. The diagnosis rests on clinical criteria and histological findings after performing a biopsy.


Presentation

Lipoid nephrosis, frequently termed minimal change disease (MCD), is by far the most common cause of nephrotic syndrome in children. More than 90% of pediatric cases arise on the grounds of MCD, while 10-15% of adult cases, in whom the diagnosis is most frequently made in the third and fourth decades of life, occurs due to MCD [1] [2] [3]. The principal feature of lipoid nephrosis is the sudden onset of proteinuria accompanied by edema in a previously healthy individual, with urine protein levels reaching as high as 10 g/24h [3] [4] [5]. Additional manifestations, although less common, include hematuria, hypertension, and renal insufficiency, and they are more frequently encountered among adults [4] [5] [6]. Spontaneous remission of proteinuria, usually after 8-12 weeks [7], is seen in approximately 30% of pediatric cases [5], which may be one of the distinguishing features of lipoid nephrosis. However, relapses are quite frequently observed, even after appropriate therapy with corticosteroids, and are reported to occur even 10 years after the initial presentation [3] [7]. Moreover, many patients become steroid-dependent in order to prevent further deterioration of kidney function. End-stage renal disease is the most feared complication of lipoid nephrosis.

Pleural Effusion
  • The fluid shift progresses and eventually causes abdominal swelling from ascites, respiratory difficulty from pleural effusion, and generalized edema. anasarca (severe generalized swelling) sometimes occurs in association with an acute infection.[medical-dictionary.thefreedictionary.com]
Weight Gain
  • As edema worsens there is a gradual weight gain, which parents may mistake for healthy growth.[medical-dictionary.thefreedictionary.com]
  • gain (from fluid retention) Minimal change disease does not reduce the amount of urine produced.[medlineplus.gov]
  • Symptoms Signs and symptoms of nephrotic syndrome include: Severe swelling (edema), particularly around your eyes and in your ankles and feet Foamy urine, which may be caused by excess protein in your urine Weight gain due to excess fluid retention Fatigue[mayoclinic.org]
  • These include appetite increase and weight gain, change in the shape of the face and a tendency to be more prone to diabetes and infection. When the steroid dosage is reduced, the condition sometimes relapses (comes back).[kidney.org.uk]
  • Symptoms Swelling ( edema ) general around the eyes in the extremities, especially the feet and ankles Swollen abdomen Facial swelling Foamy appearance of the urine Weight gain (unintentional) from fluid retention Poor appetite High blood pressure Signs[lymphedemapeople.com]
Malaise
  • Symptoms of nephrotic syndrome The symptoms of nephrotic syndrome include: foamy and frothy urine unexplained weight loss general malaise (feeling unwell) oedema (fluid retention or swelling), particularly around the abdomen (belly area), legs and eyes[betterhealth.vic.gov.au]
  • Other clinical symptoms may include frothy urine, anoxrexia, malaise, retinal sheen, abdominal pain and wasting of muscles.[lymphedemapeople.com]
Loss of Appetite
  • […] loss general malaise (feeling unwell) oedema (fluid retention or swelling), particularly around the abdomen (belly area), legs and eyes muscle wasting stomach pain dizziness when standing up from a lying or sitting position (orthostatic hypotension). loss[betterhealth.vic.gov.au]
  • […] of appetite When to see a doctor Make an appointment with your doctor if you have any signs or symptoms that worry you.[mayoclinic.org]
Abdominal Pain
  • Mesangial deposits Young men 15-30 Henoch-Schonlein Purpura Same as above, plus systemic disease: purpura of extremities, arthritis, colicky abdominal pain. Mesangial cell proliferation, more serious than above. F.M. Granular appearance, IgG C3 E.M.[kumc.edu]
  • Other clinical symptoms may include frothy urine, anoxrexia, malaise, retinal sheen, abdominal pain and wasting of muscles.[lymphedemapeople.com]
Hypertension
  • Additional manifestations, although less common, include hematuria, hypertension, and renal insufficiency, and they are more frequently encountered among adults.[symptoma.com]
  • […] on other servers: Lipoid nephrosis Clinical features Nephrotic syndrome, proteinuria selective for albumin, causing hypoalbuminemia leading to severe edema Elevated serum cholesterol, but with minimal microscopic glomerular alterations and usually no hypertension[pathologyoutlines.com]
  • Hypertension , somewhat paradoxically, also may occur in approximately 9-14% of children. Hypertension occurs in approximately 30% of adults, with a greater incidence in older patients ( 60 y).[emedicine.medscape.com]
  • Adults, particularly if older or hypertensive, are more prone to adverse effects from these cytotoxic drugs.[merckmanuals.com]
  • A Atchley and others studied the effects of hypertension, endocarditis, and circulatory diseases on the kidney and few years ago we never heard of kidney disease and now you are speaking of patients in the hundreds of thou spawned successive generations[books.google.com]
Orthostatic Hypotension
  • hypotension). loss of appetite fatigue Causes of nephrotic syndrome Some of the causes of nephrotic syndrome include: changes to the immune system (minimal change disease or lipoid nephrosis) – nephrotic syndrome due to changes to the immune system is[betterhealth.vic.gov.au]
Facial Edema
  • Looks normal F.M. negative E.M. reduced glomerular B.M. thickness Acute (Post-Streptococcal) Glomerulonephritis Acute nephritis Abrupt oliguria, hematuria, facial edema, hypertension.[kumc.edu]
Oliguria
  • Oliguria and azotemia were corrected following initiation of glucocorticoid therapy. This case suggests that lipoid nephrosis can appear as acute oliguric renal failure without historical or physical evidence of preexisting nephrotic syndrome.[ncbi.nlm.nih.gov]
  • Looks normal F.M. negative E.M. reduced glomerular B.M. thickness Acute (Post-Streptococcal) Glomerulonephritis Acute nephritis Abrupt oliguria, hematuria, facial edema, hypertension.[kumc.edu]
  • Background A 52-year-old man presented to hospital having experienced abdominal pain, abdominal distention and oliguria for 1 week.[lymphedemapeople.com]
Headache
  • Call your health care provider if nephrotic syndrome persists or if new symptoms develop, including severe headache , fever , sores on the skin, cough , discomfort with urination , or decreased urine output .[lymphedemapeople.com]

Workup

Clinical suspicion toward lipoid nephrosis can only be made if sufficient information is obtained through a detailed patient history and a thorough physical examination. Data regarding the onset of symptoms and their recurrence and the potential presence of edema and/or hypertension must be further assessed through laboratory testing. Blood urea nitrogen (BUN) and creatinine levels, as well as urinalysis, with a particular emphasis on the extent of protein levels in urine, are mandatory steps in the workup. Serum electrolyte levels, particularly sodium and potassium, should be evaluated as well. A 24-hour collection of urine is often recommended to determine the exact loss of protein, and the term "selective proteinuria" is used to describe the selective urinary loss of albumin [5], one of the main features of lipoid nephrosis. Despite the fact that clinical criteria and findings from urinalysis may provide enough clues to make the diagnosis, a definite confirmation is achieved after biopsy, which may not be always indicated due to its risks. The absence of immunoglobulin deposits (characteristic of other types of glomerulonephritis that have an underlying immunologic mechanism of disease) and preserved glomeruli are main features on light microscopy, whereas diffuse effacement of foot processes of podocytes are typical findings on electron microscopy [1] [3] [8].

HLA-DR7
  • Increased risk in those with possession of HLA-DR7 allele. Minimal change disease is highly responsive to corticosteroids and with treatment displays a good prognosis.[pathwaymedicine.org]
  • •Increased risk of MCD in those with possession of HLA-DR7 allele 32. Synaptopodin & Response to Steroids • Synaptopodin is a proline-rich protein intimately associated with actin microfilaments present in the foot processes of podocytes.[slideshare.net]
  • Associated with atopy in children, especially those who are HLA-DR7-positive. May also be related to underlying Hodgkin's disease in adults. Usually responds to a course of high-dose prednisolone but relapse is frequent.[patient.info]
Pleural Effusion
  • The fluid shift progresses and eventually causes abdominal swelling from ascites, respiratory difficulty from pleural effusion, and generalized edema. anasarca (severe generalized swelling) sometimes occurs in association with an acute infection.[medical-dictionary.thefreedictionary.com]

Treatment

  • The relapse rate after initial treatment, however, seems to be the same in both groups.[ncbi.nlm.nih.gov]
  • The majority of children with minimal change disease will respond to this treatment.[en.wikipedia.org]
  • The treatment of MCD in adults will be reviewed here.[uptodate.com]
  • Complete remission occurs in 80% of patients treated with corticosteroids, and treatment is usually continued for 1 to 2 yr. However, half or more relapse, requiring treatment with the same or a different regimen.[merckmanuals.com]
  • Ultimately, the prognosis for patients with MCD or FSGS is defined by the response to treatment.[sjkdt.org]

Prognosis

  • GN Good Prognosis Bad Prognosis[slideplayer.com]
  • Prognosis is excellent. Treatment is with corticosteroids or, in patients who do not respond, cyclophosphamide or cyclosporine .[msdmanuals.com]
  • The presence of focal glomerulosclerosis in epithelial cell disease therefore warrants a guarded prognosis with respect to response to treatment and deterioration of renal function.[annals.org]
  • The clinical presentation is very similar to that of MCD, but it seems that there is minor response to steroids and the prognosis can be worse ( Myllymaki J, et al, IgM nephropathy: clinical picture and long-term prognosis.[kidneypathology.com]
  • Decoding the significance of proteinuria as an indicator of severity or prognosis in kidney disease is a stimulating challenge to students and practitioners of nephrology.[books.google.com]

Etiology

  • Such cases demonstrate the necessity of an etiological investigation of particularly the atopic features in lipoid nephrosis. Results of these investigations can lead to specific therapy in selected cases.[ncbi.nlm.nih.gov]
  • During the last decade only little progress has been made in the understanding of the etiology and pathogenesis of MCNS.[ncbi.nlm.nih.gov]
  • (See "Etiology, clinical manifestations, and diagnosis of nephrotic syndrome in children" and "Treatment of idiopathic nephrotic syndrome in children" .)[uptodate.com]
  • Because the disease responds to corticosteroids and is often associated with pediatric allergies, an autoimmune etiology is suspected.[pathwaymedicine.org]
  • Zech P, Colon PH, Deteix P, et al: The nephrotic syndrome in adults aged over 60: Etiology, evaluation and treatment of 76 cases. Clin Nephrol 18:232–236, 1982 Google Scholar 4.[link.springer.com]

Epidemiology

  • Lipoid Nephrosis-Epidemiology • Among teenagers who develop NS, it is caused by minimal change disease about halfthe time. 8.[slideshare.net]
  • Epidemiology [ edit ] Minimal change disease is most common in very young children but can occur in older children and adults.[en.wikipedia.org]
  • Minimal-change disease in adolescents and adults: epidemiology and therapeutic response. Clin Kidney J . 2013 Oct. 6 (5):469-72. [Medline] . Day CJ, Cockwell P, Lipkin GW, Savage CO, Howie AJ, Adu D.[emedicine.medscape.com]
  • In this special pediatric nephrology series, we have combined contributions from experts in childhood NS working in a variety of fields, including basic science, clinical medicine, and epidemiology to provide the reader with a comprehensive and the most[journal.frontiersin.org]
  • Epidemiology and natural course of idiopathic nephrotic syndrome. Clin Nephrol 1991;35(Suppl 1):S3-7. [ PUBMED ] 38. Ponticelli C, Rizzoni G, Edefonti A, et al. A randomized trial of cyclosporine in steroid resistant idiopathic nephrotic syndrome.[sjkdt.org]
Sex distribution
Age distribution

Pathophysiology

  • Brenner/Rector remains the go-to resource for practicing and training nephrologists and internists who wish to master basic science, pathophysiology, and clinical best practices.[books.google.com]
  • Definition / general Also called minimal change disease, nil disease, lipoid nephrosis and foot process disease Causes 80% of cases of nephrotic syndrome in children (usually ages 2 - 6), 20% in adults Pathophysiology Extensive foot process "fusion" appears[pathologyoutlines.com]
  • Pathophysiology [ edit ] For years, pathologists found no changes when viewing specimens under light microscopy, hence the name "minimal change disease."[en.wikipedia.org]
  • Pathology (Pathophysiology) A circulating T-cell factor/permeability factor (Hemopexin) causes podocyte cytoskeleton disorganization leading to increased glomerular capillary permeability and/or changes (neutralize negative charges of heparan sulfate)[slideshare.net]
  • Ellis described current pathophysiology of edema formation in NS ( Ellis ).[journal.frontiersin.org]

Prevention

  • Moreover, many patients become steroid-dependent in order to prevent further deterioration of kidney function. End-stage renal disease is the most feared complication of lipoid nephrosis.[symptoma.com]
  • While on bed rest the child will need diligent skin care to prevent breakdown of the skin over edematous tissues. Measures are taken to avoid respiratory infections to which these children are especially susceptible.[medical-dictionary.thefreedictionary.com]
  • Your doctor may recommend medications and dietary changes to prevent these and other complications of nephrotic syndrome.[mayoclinic.org]
  • If you are in hospital, doctors will generally give heparin injections to prevent this, and some patients require a course of warfarin tablets as well.[kidney.org.uk]
  • Patients with large amounts of proteinuria are refractory to such treatments and often require immunosuppressive medications to promote a remission of the proteinuria and the nephrotic syndrome and to prevent progressive renal failure.[clinicaltrials.gov]

References

Article

  1. Lee H, Yoo KD, Oh YK, et al. Predictors of Relapse in Adult-Onset Nephrotic Minimal Change Disease. Nadasdy. T, ed. Medicine (Baltimore). 2016;95(12):e3179.
  2. Keskar V, Jamale TE, Kulkarni MJ, Kiggal Jagadish P, Fernandes G, Hase N. Minimal-change disease in adolescents and adults: epidemiology and therapeutic response. Clin Kidney J. 2013;6(5):469-472.
  3. Dias CB, Pinheiro CC, dos Santos Silva V, Hagemann R, Barros RT, Woronik V. Proteinuria predicts relapse in adolescent and adult minimal change disease. Clinics (Sao Paulo). 2012;67(11):1271-1274.
  4. Waldman M, Crew RJ, Valeri A, et al. Adult minimal-change disease: clinical characteristics, treatment, and outcomes. Clin J Am Soc Nephrol. 2007;2(3):445-453.
  5. Longo DL, Fauci AS, Kasper DL, Hauser SL, Jameson J, Loscalzo J. eds. Harrison's Principles of Internal Medicine, 18e. New York, NY: McGraw-Hill; 2012.
  6. Szeto CC, Lai FM, Chow KM, et al. Long-term outcome of biopsy-proven minimal change nephropathy in Chinese adults. Am J Kidney Dis. 2015;65(5):710-718.
  7. Tse KC, Lam MF, Yip PS, et al. Idiopathic minimal change nephrotic syndrome in older adults: steroid responsiveness and pattern of relapses. Nephrol Dial Transplant. 2003;18(7):1316-1320.
  8. Aster, JC, Abbas, AK, Robbins, SL, Kumar, V. Robbins basic pathology. Ninth edition. Philadelphia, PA: Elsevier Saunders; 2013.

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Last updated: 2018-06-22 08:05