HOME PRODUCTS SERVICES COMPANY CONTACT FAQ

Hereditary Nephritis (Alport Syndrome)

Subscribe to New Research on Hereditary Nephritis

A group of inherited conditions characterized initially by HEMATURIA and slowly progressing to RENAL INSUFFICIENCY. The most common form is the Alport syndrome (hereditary nephritis with HEARING LOSS) which is caused by mutations in genes for TYPE IV COLLAGEN and defective GLOMERULAR BASEMENT MEMBRANE.

Also Known As:

Alport Syndrome; Alport's Syndrome; Syndrome, Alport; Nephritis, Hereditary; Alport Syndrome, Autosomal Dominant; Alport Syndrome, Autosomal Recessive; Alport Syndrome, X-Linked; Alport Syndrome, X Linked; Alport Syndromes; Alports Syndrome; Familial Nephritides; Familial Nephritis; Hereditary Nephritides; Nephritides, Familial; Nephritides, Hereditary; Syndrome, Alport's; Syndrome, X-Linked Alport; Syndromes, Alport; X-Linked Alport Syndrome; Nephritis, Familial

Networked: 629 relevant articles (12 outcomes, 62 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Proteinuria
2.	Chronic Kidney Failure (Chronic Renal Failure)
3.	Glomerulonephritis
4.	IGA Glomerulonephritis (IGA Nephropathy)
5.	Membranous Glomerulonephritis (Membranous Nephropathy)

Experts

1.	Gross, Oliver: 20 articles (12/2015 - 02/2003)
2.	Kashtan, Clifford E: 17 articles (10/2014 - 02/2002)
3.	Ding, Jie: 12 articles (01/2016 - 04/2004)
4.	Heidet, Laurence: 12 articles (03/2015 - 01/2002)
5.	Sado, Yoshikazu: 12 articles (05/2013 - 01/2002)
6.	Cosgrove, Dominic: 10 articles (10/2013 - 05/2003)
7.	Borza, Dorin-Bogdan: 10 articles (05/2013 - 10/2002)
8.	Wang, Fang: 9 articles (01/2016 - 04/2004)
9.	Hudson, Billy G: 9 articles (05/2013 - 10/2002)
10.	Miner, Jeffrey H: 8 articles (10/2014 - 12/2005)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Hereditary Nephritis:

1.	Cyclosporine (Ciclosporin)FDA LinkGeneric 03/01/2003 - "These findings indicate that cyclosporine A is beneficial in slowing, but not stopping, the clinical and pathologic progression of Alport syndrome. " 06/01/2014 - "Cyclosporin A may cause injury to undifferentiated glomeruli persisting in patients with Alport syndrome." 07/01/2010 - "Cyclosporine A treatment in patients with Alport syndrome: a single-center experience." 01/01/2007 - "Cyclosporin therapy in patients with Alport syndrome." 03/01/1999 - "In 1991, our initial results of cyclosporine A (CsA) administration in eight patients with Alport's syndrome were published. "
2.	Peptidyl-Dipeptidase A (Angiotensin Converting Enzyme)IBA 12/04/2015 - "The efficiency of the inhibition of the angiotensin converting enzyme, the most widely used therapy for the Alport syndrome, depends on the onset of the therapy-the earlier the better. " 10/01/1997 - "Treatment of X-linked hereditary nephritis in Samoyed dogs with angiotensin converting enzyme (ACE) inhibitor." 10/01/2014 - "Although angiotensin-converting enzyme inhibition is effective in Alport syndrome patients for slowing progression to end-stage renal disease, it is neither a cure nor an adequate long-term protector. " 04/01/2014 - "Inhibition of angiotensin-converting enzyme slows progression to kidney failure in patients with Alport syndrome but is not a cure. " 03/01/2012 - "Thus, Alport syndrome is treatable with angiotensin-converting enzyme inhibition to delay renal failure and therapy improves life expectancy in a time-dependent manner. "
3.	Ramipril (Altace)FDA LinkGeneric 01/01/2012 - "Safety and Efficacy of the ACE-Inhibitor Ramipril in Alport Syndrome: The Double-Blind, Randomized, Placebo-Controlled, Multicenter Phase III EARLY PRO-TECT Alport Trial in Pediatric Patients." 02/01/2014 - "The study could pave the way for approval of ramipril as a drug to treat children with Alport syndrome. " 02/01/2003 - "The data in COL4A3 -/- mice as an animal-model for Alport syndrome suggest that ramipril might as well delay renal failure in humans with AS. " 02/01/2003 - "Preemptive ramipril therapy delays renal failure and reduces renal fibrosis in COL4A3-knockout mice with Alport syndrome."
4.	Idiopathic minimal change nephrotic syndromeIBA 05/01/1999 - "Eight patients had an initial diagnosis of minimal change disease, 4 were diagnosed as thin-membrane nephropathy, 2 had Alport syndrome, and the remaining 2 had hypertensive nephropathy. " 03/01/1998 - "These findings indicate that the detection of mature macrophages in urine is useful to estimate the prognosis of renal parenchymal diseases and may help to differentiate some glomerular diseases (e.g., thin basement membrane disease vs. Alport's syndrome, and minimal change nephrotic syndrome vs. primary focal segmental sclerosis)." 06/01/2007 - "The study includes 10 cases each of minimal change disease (MCD), idiopathic membranous glomerulonephritis (idiopathic MGN), thin basement membrane disease (TBMD) and Alport's syndrome. " 06/01/2007 - "Glomerular morphometry in biopsy evaluation of minimal change disease, membranous glomerulonephritis, thin basement membrane disease and Alport's syndrome." 09/01/2011 - "Electron microscopy was essential in the diagnosis of minimal change disease, hereditary nephritis, fibrillary glomerulonephritis, and certain classes of lupus nephritis. "
5.	AngiotensinsIBA 01/01/2016 - "The aim of this study was to analyze the long-term efficacy and safety of angiotensin-converting enzyme inhibitor (ACEi) and ACEi + angiotensin receptor blocker (ARB) treatments in a cohort of children with Alport syndrome (AS). " 01/01/2015 - "Combined ACE inhibitor, angiotensin-receptor-blocker, non-dihydropyridine calcium-channel-blocker, and statin therapy (Remission Clinic) reduced proteinuria and halted progression in non-diabetic nephropathies, but their efficacy in Alport syndrome (AS) nephropathy is unknown. "
6.	VitronectinIBA 04/01/1995 - "Urinary Vitronectin and the positive ratio in various renal diseases were as follows: IgA nephropathy: 0-59.22 (3.420 +/- 9.180) micrograms/g.Cre, 41%; Henoch-Schönlein purpura nephritis: 0-118.40 (19.619 +/- 31.713) micrograms/g.Cre, 53%; membranous nephropathy: 0-2.76 (0.595 +/- 0.885) micrograms/g.Cre, 18%; membrano-proliferative glomerulonephritis: 0-11.00 (1.888 +/- 3.078) micrograms/g.Cre, 33%; acute glomerulonephritis: 0.07-0.84 (0.456 +/- 0.317) microgram/g.Cre, 0%; Alport's syndrome: 0.81-112.12 (32.378 +/- 44.038) micrograms/g.Cre 67%; focal segmental glomerulosclerosis: 0.30-31.86 (12.070 +/- 17.241) micrograms/g.Cre, 67%; idiopathic nephrotic syndrome with proteinuria: 0-2.89 (0.551 +/- 1.064) micrograms/g.Cre 17%; idiopathic nephrotic syndrome in remission: 0-0.97 (0.258 +/- 0.372) microgram/g.Cre 0%, respectively. " 04/01/1995 - "In IgA nephropathy, Henoch-Schönlein purpura nephritis, membrano-proliferative glomerulonephritis, Alport's syndrome, focal segmental glomerulosclerosis and chronic renal failure, the positive ratio of urinary vitronectin was higher than in healthy children, but in membranous nephropathy, acute glomerulonephritis, idiopathic nephrotic syndrome with proteinuria and in remission, the positive ratio of urinary vitronectin was not significantly different from that of healthy children.(ABSTRACT TRUNCATED AT 250 WORDS)"
7.	Calcium Channels (Calcium Channel)IBA 01/01/2015 - "Combined ACE inhibitor, angiotensin-receptor-blocker, non-dihydropyridine calcium-channel-blocker, and statin therapy (Remission Clinic) reduced proteinuria and halted progression in non-diabetic nephropathies, but their efficacy in Alport syndrome (AS) nephropathy is unknown. "
8.	1,4-dihydropyridine (dihydropyridine)IBA 01/01/2015 - "Combined ACE inhibitor, angiotensin-receptor-blocker, non-dihydropyridine calcium-channel-blocker, and statin therapy (Remission Clinic) reduced proteinuria and halted progression in non-diabetic nephropathies, but their efficacy in Alport syndrome (AS) nephropathy is unknown. "
9.	Collagen Type IV (Type IV Collagen)IBA 09/01/2008 - "To study the hereditary mode and clinical characteristics and detect mutations of gene COL4A5 encoding type IV collagen a5 chain among family members of an X-linked dominant inherited Alport's syndrome (AS) family of China, we studied all of 38 family members of whom 2 volunteers underwent renal biopsy. " 01/01/2008 - "The differential diagnosis with Alport's syndrome may be difficult and requires accurate family investigations, immunohistochemical evaluation of type IV collagen alpha chains in renal tissue and, if appropriate, genetic studies. " 11/01/2005 - "X-linked Alport syndrome provides a system to study collagen type IV gene expression within a setting of disturbed protein synthesis. " 08/01/1997 - "Immunofluorescence study of type IV collagen alpha chains in epidermal basement membrane: application in diagnosis of X-linked Alport syndrome." 04/01/1995 - "Autosomal recessive Alport syndrome: immunohistochemical study of type IV collagen chain distribution."
10.	CollagenIBA 05/01/2013 - "Prognostic value of glomerular collagen IV immunofluorescence studies in male patients with X-linked Alport syndrome." 09/01/2005 - "On the basis of the results of our study and the existing national register of Alport syndrome patients, we demonstrated that non-invasive methods, such as the genetic analysis of collagen genes, particularly in the case of young patients with undefined clinical features, may be of great importance and could diminish the need for invasive skin and renal biopsy. " 10/01/2003 - "X-linked Alport syndrome is much less common than TBMN and can often be identified in family members by its typical clinical features (including retinopathy), a lamellated GBM without the collagen alpha3(IV), alpha4(IV), and alpha5(IV) chains, and by gene linkage studies or the demonstration of a COL4A5 mutation. " 03/01/2015 - "Alport syndrome is a clinically heterogeneous, progressive nephropathy caused by mutations in collagen IV genes, namely COL4A3 and COL4A4 on chromosome 2 and COL4A5 on chromosome X. The wide phenotypic variability and the presence of incomplete penetrance suggest that a simple Mendelian model cannot completely explain the genetic control of this disease. " 10/01/2014 - "Alport syndrome, estimated to affect 1 in 5000-10,000 individuals, is caused by mutations in any one of the three genes that encode the α chain components of the collagen α3α4α5(IV) heterotrimer: COL4A3, COL4A4, and COL4A5. "

Therapies and Procedures

1.	Phacoemulsification 07/01/2009 - "We recommend phacoemulsification with IOL implantation as a safe and effective procedure in patients with lenticonus secondary to Alport syndrome." 06/01/2007 - "We recommend phacoemulsification with IOL implantation as a safe and effective procedure in patients with anterior lenticonus secondary to Alport syndrome." 09/01/2015 - "Phacoemulsification with toric IOL implantation in Alport syndrome with anterior lenticonus having spontaneously ruptured anterior capsule." 07/01/2014 - "To report a rare case of Alport Syndrome (AS) highlighting the precautions and surgical complications during phacoemulsification. " 07/01/2014 - "Phacoemulsification in a Rare Case of Alport's Syndrome."
2.	Intraocular Lens Implantation 09/01/1998 - "One should be cautious in attempting intraocular lens implantation into the lens capsule of patients with Alport's syndrome, because the lens capsule may be fragile in this disease." 11/01/1994 - "Clear lens extraction and intraocular lens implantation in a patient with bilateral anterior lenticonus secondary to Alport's syndrome." 01/01/2008 - "We report two cases of anterior lenticonus due to Alport Syndrome and describe clear lens phacoemulsification and foldable intraocular lens implantation as an effective and safe refractive procedure in the four eyes of these two patients. " 06/01/2007 - "Phacoemulsification and intraocular lens implantation in Alport syndrome with anterior lenticonus." 09/01/2003 - "Phacoemulsification and intraocular lens implantation in an Alport's syndrome patient with bilateral anterior and posterior lenticonus."
3.	Capsulorhexis 11/01/2008 - "Clear lens phacoemulsification with continuous curvilinear capsulorhexis and foldable intraocular lens implantation was a safe and effective therapeutic choice in this patient for the management of anterior lenticonus due to Alport syndrome." 11/01/2008 - "Clear lens phacoemulsification with continuous curvilinear capsulorhexis and foldable intraocular lens implantation for the treatment of a patient with bilateral anterior lenticonus due to Alport syndrome."
4.	Kidney Transplantation 01/01/2007 - "The aim of this study was to evaluate the results of kidney transplantation in patients with Alport syndrome. " 01/01/1994 - "Previous electrophysiological studies on patients with Alport's syndrome have mostly been on adult patients undergoing haemodialysis, or after renal transplantation. " 07/01/1985 - "From this study, the authors conclude that renal transplantation is the preferred method of treatment for patients with Alport's Syndrome." 06/01/2015 - "Outcomes of Renal Transplantation in Patients With Alport Syndrome." 01/01/2012 - "Outcome of renal transplantation in Alport's syndrome: a single-center experience."
5.	Homologous Transplantation (Allograft) 04/01/2007 - "The renal allograft is prone to develop almost all forms of glomerular diseases either as recurrent or de novo disease, with the exception of Alport's syndrome. " 01/01/2007 - "Fifteen patients with Alport syndrome were compared with a control group including 212 kidney allograft recipients. " 06/01/2002 - "Diffuse glomerular basement membrane (GBM) lamellation, reminiscent of Alport's syndrome, has rarely, and exclusively, been reported in renal allografts from pediatric donors to adult recipients. " 04/01/1995 - "The absence of alpha 3(IV) chain in the GBM of patients with both these forms of Alport syndrome, due either to a failure of synthesis or a failure of assembly, presumably leads to a loss of immunologic tolerance for the alpha 3(IV) NC1 domain in transplanted allografts." 05/01/1971 - "Related donor renal allografts in hereditary nephritis Report of two siblings."