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]

  Some symptoms of NS include: Swelling in parts of the body, most noticeable around the eyes, hands, feet, and abdomen (edema) Weight gain due to extra fluid building up in the body High blood pressure (hypertension) High fat levels in the blood (high [nephcure.org]

  As edema worsens there is a gradual weight gain, which parents may mistake for healthy growth. [medical-dictionary.thefreedictionary.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]

• 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]

  On exam, patients may demonstrate anasarca, pericardial or pleural effusion, ascites, and abdominal pain. [ncbi.nlm.nih.gov]

• 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. [kumc.edu]

  On exam, patients may demonstrate anasarca, pericardial or pleural effusion, ascites, and abdominal pain. [ncbi.nlm.nih.gov]

  Background A 52-year-old man presented to hospital having experienced abdominal pain, abdominal distention and oliguria for 1 week. [lymphedemapeople.com]

  Anasarca may develop with ascites and pleural and pericardial effusion, leading to abdominal pain because of hypoperfusion and/or thrombosis, dyspnea (rarely), and cold extremities with low BP ( 11 ). [doi.org]

• 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]

  […] muscle wasting stomach pain dizziness when standing up from a lying or sitting position (orthostatic hypotension). loss of appetite fatigue Causes of nephrotic syndrome Some of the causes of nephrotic syndrome include: changes to the immune system (minimal [betterhealth.vic.gov.au]

• Hypertension

  Additional manifestations, although less common, include hematuria, hypertension, and renal insufficiency, and they are more frequently encountered among adults. [symptoma.com]

  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]

  In people of similar age, the prevalence of hypertension amounts to 5 to 10% ( 22 ). [doi.org]

• 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]

• Swelling of Hand

  The usual symptom is ankle swelling, or swelling of the face and hands. This may come on quite suddenly, and may vary with the time of day. [kidney.org.uk]

• Facial Edema

  = 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]

  = reduced glomerular B.M. thickness Acute (Post-Streptococcal) Glomerulonephritis Acute nephritis Abrupt oliguria, hematuria, facial edema, hypertension. [kumc.edu]

  Intravascular volume depletion and oliguria are also present, and concomitant factors (sepsis, diarrhea, diuretics) can lead to AKI, which is more frequently seen in adults ( 12 ), although it occurs in about half of children hospitalized for nephrotic [doi.org]

  Background A 52-year-old man presented to hospital having experienced abdominal pain, abdominal distention and oliguria for 1 week. [lymphedemapeople.com]

• Frothy Urine

  Diagnosis of nephrotic syndrome Diagnosing nephrotic syndrome involves a number of tests, including: urine tests – excessive protein makes the urine appear frothy and foamy. [betterhealth.vic.gov.au]

  Other clinical symptoms may include frothy urine, anoxrexia, malaise, retinal sheen, abdominal pain and wasting of muscles. [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]

• Seizure

  Growth and Development, Neurology, Oncology, Connective Tissue Diseases, and Immunization have been revised completely; and topics like Nephrotic Syndrome, Diabetes Mellitus, Newer Vaccines, Infectious Diseases, Genetic Disorders, Neurometabolic and Seizure [books.google.de]

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].

• 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]

  On exam, patients may demonstrate anasarca, pericardial or pleural effusion, ascites, and abdominal pain. [ncbi.nlm.nih.gov]

• 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]

The relapse rate after initial treatment, however, seems to be the same in both groups. [ncbi.nlm.nih.gov]

Treatment guidelines for adults is fairly limited, largely based on studies done on children. [en.wikipedia.org]

The treatment of MCD in adults will be reviewed here. [uptodate.com]

These patients require immunosuppressive treatment during several decades of their life. [doi.org]

GN Good Prognosis Bad Prognosis [slideplayer.com]

MCD, FSGS, and MN vary by renal prognosis and known secondary causes. Renal biopsy is the gold standard for the proper diagnosis of MCD, FSGS, and MN. [mayoclinic.pure.elsevier.com]

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]

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]

What is the prognosis? Even in adults, Minimal Change Disease typically has a favorable prognosis. Over 90% of patients will respond to oral steroids, with most of these having a complete remission. [unckidneycenter.org]

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]

(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]

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.medscape.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]

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]

Reidy K, Kaskel FJ (2007) Pathophysiology of focal segmental glomerulosclerosis. Pediatr Nephrol 22:350–354 PubMed CrossRef Google Scholar 26. [link.springer.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]

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]

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]

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.