eMedicine Specialties > Nephrology > Hereditary Kidney Disorders
Alport Syndrome: Treatment & Medication
Updated: Sep 9, 2008
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
- Multimedia
Treatment
Medical Care
No definite treatment exists for Alport syndrome. Data from animal studies suggest benefits from angiotensin-converting enzyme (ACE) inhibitors in the reduction of proteinuria and progression of renal disease; thus, the use of ACE inhibitors is reasonable in patients with Alport syndrome who have proteinuria with or without hypertension. Some reports suggest that cyclosporine may reduce proteinuria and stabilize renal functions in patients with Alport syndrome; however, the studies were small and uncontrolled. Larger and controlled studies are needed to define the role of cyclosporine in Alport syndrome.
Gene therapy for Alport syndrome is being studied. Animal studies are underway to evaluate the delivery of human alpha-5 (IV) chain of GBM in a canine model of X-linked Alport syndrome.
Surgical Care
Renal transplantation is usually offered to patients with Alport syndrome who develop ESRD. The allograft survival rate in these patients is similar to patients with other renal diseases. Recurrent disease does not occur in the transplant; however, approximately 3-5% of patients with Alport syndrome who undergo transplant develop anti-GBM nephritis. In view of excellent graft survival rates and a very low incidence of anti-GBM disease, renal transplantation is not contraindicated in patients with Alport syndrome.
Consultations
- Ophthalmologist
- Otorhinolaryngologist
- Transplant surgeon
- Surgeon
- Dialysis specialist
Diet
Patients require a renal failure diet once ESRD ensues.
Medication
ACE inhibitors or angiotensin-receptor blockers (ARBs) should be administered to patients with Alport syndrome who have proteinuria with or without hypertension.
ACE inhibitors
Help to reduce proteinuria by decreasing intraglomerular pressure; moreover, angiotensin II is a growth factor that is implicated in glomerular sclerosis. By inhibiting angiotensin II, these drugs have a potential role in slowing down glomerular sclerosis.
Enalapril (Vasotec)
Competitive inhibitor of ACE. Reduces angiotensin II levels, decreasing aldosterone secretion.
Adult
5 mg/d PO initial; not to exceed 40 mg/d
Pediatric
Not established
NSAIDs may reduce hypotensive effects of enalapril; ACE inhibitors may increase digoxin, lithium, and allopurinol levels; rifampin decreases enalapril levels; probenecid may increase enalapril levels; the hypotensive effects of ACE inhibitors may be enhanced when administered concurrently with diuretics
Documented hypersensitivity; angioedema
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Category D in second and third trimesters of pregnancy; caution in renal impairment (serum creatinine >3.5), valvular stenosis, or severe congestive heart failure; monitor serum potassium
Fosinopril (Monopril)
Competitive inhibitor of ACE. Reduces angiotensin II levels, decreasing aldosterone secretion.
Adult
10 mg/d PO initial; not to exceed 80 mg/d
Pediatric
Not established
NSAIDs may reduce hypotensive effects of fosinopril; ACE inhibitors may increase digoxin, lithium, and allopurinol levels; rifampin decreases fosinopril levels; probenecid may increase fosinopril levels; the hypotensive effects of ACE inhibitors may be enhanced when administered concurrently with diuretics
Documented hypersensitivity; collagen vascular disease; angioedema
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Category D in second and third trimester of pregnancy; caution in renal impairment (serum creatinine >3.5), valvular stenosis, or severe congestive heart failure; monitor serum potassium
Lisinopril (Zestril, Prinivil)
Competitive inhibitor of ACE. Reduces angiotensin II levels, decreasing aldosterone secretion.
Adult
10 mg/d PO initial; not to exceed 80 mg/d
Pediatric
Not established
NSAIDs may reduce hypotensive effects of lisinopril; ACE inhibitors may increase digoxin, lithium, and allopurinol levels; rifampin decreases lisinopril levels; probenecid may increase lisinopril levels; the hypotensive effects of ACE inhibitors may be enhanced when administered concurrently with diuretics
Documented hypersensitivity; angioedema
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Category D in second and third trimester of pregnancy; caution in renal impairment (serum creatinine >3.5), valvular stenosis, or severe congestive heart failure; monitor serum potassium
Quinapril (Accupril)
Competitive inhibitor of ACE. Reduces angiotensin II levels, decreasing aldosterone secretion.
Adult
10 mg/d PO initial; not to exceed 80 mg/d
Pediatric
Not established
NSAIDs may reduce hypotensive effects of enalapril; ACE inhibitors may increase digoxin, lithium, and allopurinol levels; rifampin decreases enalapril levels; probenecid may increase enalapril levels; the hypotensive effects of ACE inhibitors may be enhanced when administered concurrently with diuretics
Documented hypersensitivity; angioedema
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Category D in second and third trimester of pregnancy; caution in renal impairment (serum creatinine >3.5), valvular stenosis, or severe congestive heart failure; monitor serum potassium
Angiotensin-receptor blockers
Help reduce proteinuria by decreasing the intraglomerular pressure. By inhibiting angiotensin II, these drugs have a potential role in slowing down glomerular sclerosis, as with ACE inhibitors. Unlike ACE inhibitors, ARBs do not activate bradykinin and are not associated with cough and angioedema.
Losartan (Cozaar)
Nonpeptide angiotensin II receptor antagonist that blocks the vasoconstrictor and aldosterone-secreting effects of angiotensin II. May induce a more complete inhibition of the renin-angiotensin system than ACE inhibitors and do not affect the response to bradykinin and are less likely to be associated with cough and angioedema. For patients unable to tolerate ACE inhibitors.
Adult
50 mg/d PO initial; not to exceed 100 mg/d
Pediatric
Not established
Ketoconazole, sulfaphenazole, and phenobarbital may decrease effects; cimetidine may increase effects of losartan
Documented hypersensitivity
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Category D in second and third trimester of pregnancy; caution in renal impairment (serum creatinine >3.5), valvular stenosis, or severe congestive heart failure; monitor serum potassium
Candesartan (Atacand)
Nonpeptide angiotensin II receptor antagonist that blocks the vasoconstrictor and aldosterone-secreting effects of angiotensin II. May induce a more complete inhibition of the renin-angiotensin system than ACE inhibitors and do not affect the response to bradykinin and are less likely to be associated with cough and angioedema. For patients unable to tolerate ACE inhibitors.
Adult
16 mg/d PO initial; not to exceed 32 mg/d
Pediatric
Not established
Ketoconazole, sulfaphenazole, and phenobarbital may decrease effects; cimetidine may increase effects of candesartan
Documented hypersensitivity
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Category D in second and third trimester of pregnancy; caution in renal impairment (serum creatinine >3.5), valvular stenosis, or severe congestive heart failure; watch for serum potassium
More on Alport Syndrome |
| Overview: Alport Syndrome |
| Differential Diagnoses & Workup: Alport Syndrome |
 Treatment & Medication: Alport Syndrome |
| Follow-up: Alport Syndrome |
| Multimedia: Alport Syndrome |
| References |
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References
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Further Reading
Keywords
Alport syndrome, kidney failure, renal failure, AS, hereditary nephritis, deafness, hematuria, type IV collagen, end-stage renal disease, ESRD, glomerular basement membrane, GBM, tubular basement membrane, TBM, autosomal dominant Alport syndrome, ADAS, autosomal recessive Alport syndrome, ARAS, X-linked Alport syndrome, XLAS, leiomyomatosis, anterior lenticonus, dot-and-fleck retinopathy, proteinuria
