NEPHRITIC SYNDROME:

Nephritic is an older term used to clinically denote a child with hypertension, decreased renal function, hematuria, and edema, according to Fathallah-Shaykh (2011), nephritis suggest a noninfectious inflammatory process that involves the nephron; glomerulonephritis (GN) generally is a more precise term.

      Diseases that produce glomerulonephritis are usually classified as primary, where the kidney is the primarily affected organ, or secondary, in which systemic disorder involve the kidney in addition to other organs. (Fathallah-Shaykh, 2011).

      Essential of diagnosis. (Watnick & Morrison, 2005):

      Edema.

      Hypertension.

      Hematuria (with or without dysmorphic red cells, red blood cell casts).

      When, the inflammatory process involves glomeruli, it leads to hematuria and red blood cells casts in urine, associated with azotemia, oliguria, hypertension, and proteinuria < 3.5g/day.

      Medical care that Fathallah-Shaykh (2011) recommends for glomerulonephritis (GN) is usually divided into 2 major components: treatment of primary pathology and supportive care. In renal diseases, supportive care involves managing hypertension and fluid and electrolyte abnormalities and managing decreased renal function.

      The treatment of primary pathology ranges from watchful waiting, as in postinfectious GN, to treatment with immunosuppressive medication, such as steroids or cyclophosphamide in lupus; in the case of IgM nephropathy, no definitive therapy is known.

      Hypertension can be managed with antihypertensives, such as calcium channel–blocking agents, angiotensin-converting enzyme (ACE) inhibitors, angiotensin receptor–blocking agents, peripheral vasodilators, and diuretics. The most common fluid abnormality, hypervolemia, is managed with fluid restriction and diuretics or with dialysis if renal function is too poor to respond to diuretics. Hyponatremia is usually dilutional and responds, at least partially, to removal of excess fluid. Hypocalcemia may respond to oral or IV calcium, depending on severity. Mild metabolic acidosis may be present but rarely requires primary treatment.

      Some recommend a short course of steroids or cyclophosphamide for tubulointerstitial nephritis (TIN). However, these drugs are usually not necessary. Most often, stopping the offending agent leads to recovery. (Fathallah-Shaykh, 2011).

      Acute poststreptococcal glomerulonephritis:

      Is a disorder most frequently seen in children, that can resolves spontaneously (Le, Bhushan, & Grimm, First AID fro the USMLE Step 1, 2011) characterized by sudden appearance of edema, peripheral and periorbital edema, hematuria, proteinuria, and hypertension; it is a representative disease of acute nephritic syndrome in which inflammation of the glomerulus is manifested by proliferation of cellular elements secondary to an immunologic mechanisms. (Bhimma, 2011) (Rodríguez-Iturbe B, Jun 2007).

      Rapidly progressive glomerulonephritis (RPGN).

      RPGN have a poor prognosis with rapidly deteriorating renal function, days to weeks. The glomerular filtration rate (GFR) decreases at least 50% over a short period, from a few days to 3 months. The main pathologic finding is extensive glomerular crescent formation. ( Lohr, 2011).

      Rapidly progressive glomerulonephritis is classified pathologically into 3 categories (Lohr, 2011), as follows: (1) anti-GBM antibody disease (approximately 3% of cases), (2) immune complex disease (45% of cases), and (3) pauci-immune disease (50% of cases). Immunologic classification is based on the presence or absence of circulating antineutrophil cytoplasmic antibodies (ANCAs). The disorders are also classified based on their clinical presentation.

       Several disease processes may result in this pattern, including (Le, Bhushan, & Grimm, First AID for the USMLE step 1, 2011):

1)      Goodpasture syndrome: It is type II hypersensitivity; antibodies to GBM and alveolar basement membrane are presents.

2)      Wegener’s granulomatosis. (c-ANCA).

3)      Microscopie polyangiitis. (p-ANCA).

      Therapy for ANCA-associated disease consists of a combination of corticosteroids and cyclophosphamide. Treatment with steroids alone results in a 3-fold increase in the risk of relapse compared to combination therapy.

       Diffuse proliferative glomerulonephritis (DPGN).

       It the most common cause of death in SLE and MPGN, can also present as Nephrotic syndrome as explained Tao & at (2011), DPGN describe a distinct histologic form of GN common to various types of systemic inflammatory diseases, including autoimmune disorders, vasculitis syndromes, and infectious process. More than 50% of glomeruli show an increase in mesangial, epithelial, endothelial and inflammatory cells. The diagnosis is often suspected in a patient presenting with systemic inflammatory disease who manifests hematuria, proteinuria, and active urinary sediment or azotemia; histologic findings from kidney biopsy tissue are used to confirm the diagnosis. (Salifu, 2009).

      Early, aggressive therapy is indicated as regards Salifu (2009) because of the high risk of early-stage of renal diseases. Initiate the following as induction therapy: pulse methylprednisolone of 1 g daily for 3 days, followed by 1 mg/kg for 4-6 weeks and then tapered to 5-10 mg/d for maintenance therapy by 6 months. Alternatively, prednisolone 1 mg/kg (not to exceed 80 mg/d) can be started and tapered as above. Additional induction and maintenance therapy may be indicated, depending on the type of DPGN. Evidence suggests that mycophenolate mofetil (MMF) treatment benefits problematic patients who are refractory to conventional therapies for glomerulopathies (Sahin, Sahin, Kantarci, & Ergin, 2007).

      Berger’s diseases:

      Also know as Immunoglobulin A nephropathy is characterized by predominant IgA deposition in the glomerular mesangium, it is the most common cause of glomerulonephritis in the world. (Brake, 2010).

      Two common presentations of patients with IgA nephropathy are episodic gross hematuria and persistent microscopic hematuria. Recurrent macroscopic hematuria, usually associated with an upper respiratory tract infection, or, less often, gastroenteritis is the most frequent clinical presentation and is observed in 40-50% of presenting patients. In 30-40% of patients, the disease is asymptomatic, with erythrocytes (RBCs), RBC casts, and proteinuria discovered on urinalysis. Patients with IgA nephropathy can also present with acute or chronic renal failure, under what explains Brake (2010) currently, no cure exists for IgA nephropathy, but therapies that can delay the onset of need for dialysis and transplantation are available.

      Alport’s syndrome:

Due to a variety of gene defects resulting in abnormal type IV collagen, the most common form is X-linked recessive. Type IV collagen is an important structural component of the basement membrane of the kidney, ears, and eyes. The Alport’s syndrome presents as a progressive hereditary nephritic and deafness, may be associated with ocular disturbances (Le & at, 2011).

      No definite treatment exists for Alport’s syndrome. (Saxena, 2008).

Bibliography

Lohr, J. (2011, June 8). Medscape references. Retrieved from Rapidly Progressive Glomerulonephritis : http://emedicine.medscape.com/article/240457-overview

Bhimma, R. (2011, september 21). Medscape. Retrieved from Acute Poststreptococcal Glomeruloneph: http://emedicine.medscape.com/article/980685-overview

Brake, M. (2010, Mar 23). Medscape references. Retrieved from IgA Nephropathy: http://emedicine.medscape.com/article/239927-overview

Fathallah-Shaykh, S. (2011, june 29). Medscape references. Retrieved from http://emedicine.medscape.com/article/982811-overview

Le, T., Bhushan, V., & Grimm, L. (2011). First AID for the USMLE step 1. USA: McGraw Hill.

Le, T., Bhushan, V., & Grimm, L. (2011). First AID fro the USMLE Step 1. USA: McGraw Hill.

Rodríguez-Iturbe B, B. S. (Jun 2007). Pathogenesis of poststreptococcal glomerulonephritis a century after Clemens von Pirquet. In Kidney Int (pp. 71(11):1094-104.).

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Salifu, M. O. (2009, may 11). Medscape references. Retrieved from Glomerulonephritis, Diffuse Proliferative : http://emedicine.medscape.com/article/239646-overview

Saxena, R. (2008, september 8). Medscape references. Retrieved from Alport Syndrome Treatment & Management: http://emedicine.medscape.com/article/238260-treatment

Watnick, S., & Morrison, G. (2005). Kidney. Glomerulonephropathies. In L. M. Tierney, S. J. McPhee, & M. A. Papadakis, Current Medical Diagnosis & Treatment (p. 885). USA: McGraw Hill.