Nephrology

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Alport's Syndrome

(NK001)

Introduction

Nephritis is a general term for inflammation of the kidneys. There are several hereditary forms of nephritis. Alport's syndrome (AS) is one of these but is unique because of the additional symptom of hearing loss in the presence of kidney failure. The disorder causes chronic glomerulonephritis with destruction of the glomeruli. The eventual result is end stage renal disease (ESRD) requiring dialysis (or transplantation) develops between adolescence and age 40.

AS affects the basement membranes which are the supporting layers of epithelial and endothelial tissues that make up much of the body's surfaces. The cause is a mutation in a gene for collagen. The disorder is uncommon, affecting about 2 out of 10,000 people.

The cause of AS is a mutation in a gene for collagen. The disorder is uncommon, affecting about 2 out of 10,000 people. Treatment requires medication, and often, kidney transplant.

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The Medifocus Guide on Alport's Syndrome provides answers to the following important questions and medical issues:

What are the most common symptoms of Alport's Syndrome?

Are there any recognized risk factors for developing Alport's Syndrome?

What kinds of medical tests are used to establish the diagnosis of Alport's Syndrome?

What is the current standard of care for the treatment of Alport's Syndrome?

What treatment options are available for the management of Alport's Syndrome?

Are there any promising new developments or potential breakthroughs in treatment?

Who are the most notable medical authorities who specialize in Alport's Syndrome?

Where are the leading hospitals and centers of research for Alport's Syndrome?

What are the most important questions to ask my doctor about Alport's Syndrome?

What Your Doctor Reads:

This MediFocus Guide contains an extensive listing of citations and abstracts of recent journal articles that have been published about this condition in trustworthy medical journals. This is the same type of information that is available to physicians and other health care professionals. A partial selection of journal articles that are abstracted in this MediFocus Guide includes:

Alport syndrome and diffuse leiomyomatosis. Clinical aspects, pathology, molecular biology and extracellular matrix studies. A synthesis.

Nephrologie. 2000

Animal models of Alport syndrome: advancing the prospects for effective human gene therapy.

Experimental Nephrology. 2000

Alport syndrome. An inherited disorder of renal, ocular, and cochlear basement membranes.

Medicine. 1999

Alport syndrome. A review of the ocular manifestations.

Ophthalmic Genetics. 1997

Can Alport syndrome be treated by gene therapy?.

Kidney International. 1997

Alport's syndrome.

Journal of Medical Genetics. 1997

Alport syndrome.

Kidney International - Supplement. 1997

Alport syndrome.

Kidney International. 1996

Ocular clues to the nature of disease causing end-stage renal failure.

Nephrology, Dialysis, Transplantation. 2000

Long-term effects of cyclosporine A in Alport's syndrome.

Kidney International. 1999

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Hypertension

(NK007)

Introduction

High blood pressure, also called hypertension (HTN) occurs when the body's smaller blood vessels (arterioles) narrow, causing the blood to exert excessive pressure against the vessel walls. The heart must therefore work harder to maintain the higher pressure. Over time, damage to the brain, eye and kidney can result ("target organ damage").

Cardiovascular disease is the leading cause of mortality in the United States. The most significant risk factor for cardiovascular disease is hypertension. High blood pressure is the key risk factor for stroke, heart attack, heart failure, kidney disease, and mortality.

Essential or primary hypertension means that there is no underlying condition that is causing the blood pressure to increase. Up to 95% of hypertension is the essential type. When an underlying cause is detected, the elevated blood pressure is considered to be secondary hypertension.

Unless blood pressure readings are very high, initial efforts at controlling blood pressure in essential hypertension are usually based on lifestyle changes. These include weight loss (if appropriate), exercise, dietary changes, and stress reduction. The goal of hypertensive treatment is to reduce overall cardiovascular disease risk and thus its morbidity and mortality.

More aggressive control of blood pressure is recommended for persons with comorbid conditions such as diabetes or renal (kidney) insufficiency.

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The Medifocus Guide on Hypertension provides answers to the following important questions and medical issues:

What are the most common symptoms of hypertension?

Are there any recognized risk factors for developing hypertension?

What kinds of medical tests are used to establish the diagnosis of hypertension?

What is the current standard of care for the treatment of hypertension?

What treatment options are available for the management of hypertension?

Are there any promising new developments or potential breakthroughs in treatment?

Who are the most notable medical authorities who specialize in hypertension?

Where are the leading hospitals and centers of research for hypertension?

What are the most important questions to ask my doctor about hypertension?

What Your Doctor Reads:

Hypertensive emergencies.

Lancet. 2000

Angiotensin converting enzyme inhibition: from viper to patient.

Heart. 2000

Elevated systolic blood pressure as a cardiovascular risk factor.

American Journal of Cardiology. 2000

Choosing a first-line drug in the management of elevated blood pressure: what is the evidence? 3: Angiotensin-converting-enzyme inhibitors.

CMAJ. 2000

Choosing a first-line drug in the management of elevated blood pressure: what is the evidence? 2: Beta-blockers.

CMAJ. 2000

Choosing a first-line drug in the management of elevated blood pressure: what is the evidence? 1: Thiazide diuretics.

CMAJ. 2000

The diagnosis and management of hypertensive crises.

Chest. 2000

Angiotensin II receptor blockers: equal or preferred substitutes for ACE inhibitors?.

Archives of Internal Medicine. 2000

When antihypertensive monotherapy fails: fixed-dose combination therapy.

Southern Medical Journal. 2000

Insulin resistance and hypertension. Patients in double jeopardy for cardiovascular disease.

Geriatrics. 2000

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Polycystic Kidney Disease

(NK012)

Introduction

Polycystic Kidney Disease (PKD) is a hereditary disorder that causes fluid-filled cysts to form in the nephrons of the kidneys. These cysts can grow so large and so numerous that they crowd out the normal kidney tissue, rendering the kidneys unable to function normally. Damage to the kidneys is caused by structural deformities caused by the enlarging cysts as well as by elevations in blood pressure (hypertension). Cysts can also form in other organs such as the liver.

PKD affects approximately 600,000 persons in the United States. It is one of the most common hereditary diseases and the fourth leading cause of kidney failure. It seems to affect all races and both genders equally.

Persons with autosomal dominant PKD often exhibit no symptoms and the disease can not be diagnosed by routine blood work in its early stages. Therefore, persons may have the disease for years before it is diagnosed. Once cysts have formed, they can be seen with noninvasive ultrasound imaging.

There is no cure for PKD. Medications or surgery may be used for pain control, to reduce blood pressure and for the treatment of infection. Dialysis is usually eventually necessary to perform functions (removal of toxins from the blood) that the kidneys are no longer able to do. Kidney transplant may be an option for persons with advanced PKD.

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The Medifocus Guide on Polycystic Kidney Disease provides answers to the following important questions and medical issues:

What are the most common symptoms of polycystic kidney disease?

Are there any recognized risk factors for developing polycystic kidney disease?

What kinds of medical tests are used to establish the diagnosis of polycystic kidney disease?

What is the current standard of care for the treatment of polycystic kidney disease?

What treatment options are available for the management of polycystic kidney disease?

Are there any promising new developments or potential breakthroughs in treatment?

Who are the most notable medical authorities who specialize in polycystic kidney disease?

Where are the leading hospitals and centers of research for polycystic kidney disease?

What are the most important questions to ask my doctor about polycystic kidney disease?

What Your Doctor Reads:

Renal cell carcinoma in cases of adult polycystic kidney disease: changing diagnostic and therapeutic implications.

Urologia Internationalis. 2000

Complications of autosomal dominant polycystic kidney disease in hemodialysis patients.

Seminars in Dialysis. 2000

Perinatal differential diagnosis of cystic kidney disease and urinary tract obstruction: anatomic pathologic, ultrasonographic and genetic findings.

2000

Digital glomerular reconstruction in a patient with a sporadic adult form of glomerulocystic kidney disease.

American Journal of Kidney Diseases. 2000

Unilateral renal cystic disease.

Urology. 1999

Traumatic hematuria in patients with polycystic kidney disease.

American Surgeon. 1999

Pathophysiology and clinical management of polycystic kidney disease in women.

Seminars in Nephrology. 1999

Autosomal dominant polycystic kidney disease associated with familial sensorineural deafness.

Scandinavian Journal of Urology & Nephrology. 1999

Outcome and cost analysis of operative versus nonoperative management of neonatal multicystic dysplastic kidneys.

Journal of Urology. 1998

New insights into polycystic kidney disease and its treatment.

Current Opinion in Nephrology & Hypertension. 1998

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Renal Calculi (Kidney Stones)

(NK014)

Introduction

Nephrolithiasis (kidney stones, renal calculi) is the development of stones within the kidney that may then be passed through the ureters into the bladder and excreted with the urine. This process of stone passage may be painless if the stones are very small, or may cause significant pain (renal colic).

Nephrolithiasis is a common disorder affecting between 5-15% of Americans. Most stones seem to occur during the fourth and fifth decades of life in men and somewhat earlier in women

While kidney stones can cause significant pain during the acute episode, the risk of kidney failure is very small and occurs only with obstruction of both kidneys or the obstruction of a remaining kidney when one is not functioning or has been removed.

While the majority (80-90%) of stones eventually pass without intervention, the remaining stones require intervention known as lithotripsy or, rarely, surgical intervention to be removed.

Get the Facts... With your MediFocus Guide

The Medifocus Guide on Nephrolithiasis provides answers to the following important questions and medical issues:

What are the most common symptoms of kidney stones?

Are there any recognized risk factors for developing kidney stones?

What kinds of medical tests are used to establish the diagnosis of kidney stones?

What is the current standard of care for the treatment of kidney stones?

What treatment options are available for the management of kidney stones?

Are there any promising new developments or potential breakthroughs in treatment?

Who are the most notable medical authorities who specialize in kidney stones?

Where are the leading hospitals and centers of research for kidney stones?

What are the most important questions to ask my doctor about kidney stones?

What Your Doctor Reads:

Should dietary calcium and protein be restricted in patients with nephrolithiasis?.

Nutrition Reviews. 2000

Management of residual stones.

Urologic Clinics of North America. 2000

Kidney stones as a manifestation of hypercalcemic disorders. Hyperparathyroidism and sarcoidosis.

Urologic Clinics of North America. 2000

Nephrolithiasis with unusual initial symptoms.

Journal of Manipulative & Physiological Therapeutics. 2000

Kidney stones.

Harvard Mens Health Watch. 2000

New insights into causes and treatments of kidney stones.

Hospital Practice (Office Edition). 2000

Pathogenesis of Dent's disease and related syndromes of X-linked nephrolithiasis.

Kidney International. 2000

Update on dietary recommendations and medical treatment of renal stone disease.

Nephrology, Dialysis, Transplantation. 2000

Metabolic evaluation and medical therapy for stone formation.

Current Opinion in Urology. 1999

Outcomes of contemporary percutaneous nephrostolithotomy in morbidly obese patients.

Journal of Urology. 1998

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