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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.

Weight Gain
  • Prolonged use of corticosteroids can lead to immunosuppression (leading to infection), growth compications, weight gain.[en.wikipedia.org]
  • gain (from fluid retention) Minimal change disease does not reduce the amount of urine produced.[nlm.nih.gov]
  • As edema worsens there is a gradual weight gain, which parents may mistake for healthy growth.[medical-dictionary.thefreedictionary.com]
  • 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]
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]
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]
Loss of Appetite
  • […] 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]
  • […] 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]
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]
Hypertension
  • Additional manifestations, although less common, include hematuria, hypertension, and renal insufficiency, and they are more frequently encountered among adults.[symptoma.com]
  • In people of similar age, the prevalence of hypertension amounts to 5 to 10% ( 22 ).[doi.org]
  • ARF occurred in 24 patients; they tended to be older and hypertensive with lower serum albumin and more proteinuria than those without ARF. At follow up, patients with an episode of ARF had higher serum creatinine than those without ARF.[cjasn.asnjournals.org]
  • -- Primary hypertension -- Nonpharmacologic prevention and treatment of hypertension -- Pharmacologic treatment of hypertension -- Evaluation and treatment of hypertensive emergencies and urgencies -- Endocrine causes of hypertension : aldosterone --[worldcat.org]
  • 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]
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]

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 mainstay of treatment for adult MCD, oral glucocorticoids, is based on two randomized controlled trials and extensive observational data in adults, and this treatment leads to remission in over 80% of cases.[ncbi.nlm.nih.gov]
  • The mainstay of treatment for adult MCD, oral gucocorticoids, is based on two randomized controlled trials and extensive observational data in adults, and this treatment leads to remission in over 80% of cases.[jasn.asnjournals.org]
  • Treatment guidelines for adults is fairly limited, largely based on studies done on children.[en.wikipedia.org]

Prognosis

  • GN Good Prognosis Bad Prognosis[slideplayer.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]
  • 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]
  • Early age of onset (before 16) and intermittent asthma were associated and had a more favourable prognosis, while the childhood bronchitic had a better outlook than the adult bronchitic. Intermittent and continuous asthma have been compared.[books.google.com]
  • Minimal change disease is highly responsive to corticosteroids and with treatment displays a good prognosis.[pathwaymedicine.org]

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]
  • Minimal change disease (MCD) is the etiology of 10%-25% of cases of nephrotic syndrome in adults.[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]

Epidemiology

  • Lipoid Nephrosis-Epidemiology • Among teenagers who develop NS, it is caused by minimal change disease about halfthe time. 8.[slideshare.net]
  • […] nephropathies -- Myeloma and the kidney -- Onconephrology : kidney disease in cancer patients -- Pathophysiology and etiology of acute kidney injury -- Acute kidney injury in the tropics -- Diagnosis and clinical evaluation of acute kidney injury -- Epidemiology[worldcat.org]
  • Epidemiology Frequency United States In preadolescents, minimal-change nephrotic syndrome (MCNS) makes up 85-95% of all cases of nephrotic syndrome.[emedicine.com]
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]
  • Johnson, and Marcello Tonelli, provides current information on clinical procedures and conditions as well as the scientific facts and pathophysiology that are foundational to nephrology practice.[worldcat.org]
  • Ellis described current pathophysiology of edema formation in NS ( Ellis ).[journal.frontiersin.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]

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]
  • […] and treatment of diabetic kidney disease -- Management of the diabetic patient with chronic kidney disease -- Normal blood pressure control and the evaluation of hypertension -- Primary hypertension -- Nonpharmacologic prevention and treatment of hypertension[worldcat.org]
  • 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]
  • Prevention of Recurrence Pretransplant plasmapheresis appears to prevent or delay recurrence in high-risk patients ( 28 ).[doi.org]

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