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Polycystic Kidney Diseases

Important
It is possible that the main title of the report Polycystic Kidney Diseasesis not the name you expected.

Synonyms

  • PKD
  • Polycystic Renal Diseases

Disorder Subdivisions

  • Autosomal Recessive Polycystic Kidney Disease
  • ARPKD
  • PKD3
  • Neonatal Autosomal Recessive Polycystic Kidney Disease
  • Perinatal Polycystic Kidney Diseases
  • Infantile Autosomal Recessive Polycystic Kidney Disease
  • Juvenile Autosomal Recessive Polycystic Kidney Disease
  • Autosomal Dominant Polycystic Kidney Disease
  • ADPKD
  • PKD1
  • Adult Polycystic Kidney Disease
  • Potter Type III Polycystic Kidney Disease
  • PKD2
  • Potter Type I Infantile Polycystic Kidney Diseases

General Discussion

Polycystic Kidney Diseases are inherited renal disorders characterized by the presence of multiple cysts in both kidneys (bilateral renal cysts). Normal kidney tissue is replaced by fluid-filled sacs or cysts of varying sizes that become larger as the disease progresses. Findings associated with Polycystic Kidney Diseases include abnormally high blood pressure (hypertension) and the progressive loss of kidney function, leading to end-stage renal failure. Symptoms may include abdominal distention, vomiting, and/or failure to thrive. Some infants with Autosomal Recessive Polycystic Kidney Disease (ARPKD) have unusual facial features. This occurs when there is a severe reduction in the amount of amniotic fluid surrounding the developing fetus (oligohydramnios), resulting in what is known as the Potter deformation sequence.

There are two major subdivisions of Polycystic Kidney Disease (PKD): Autosomal Recessive Polycystic Kidney Disease (ARPKD) and Autosomal Dominant Polycystic Kidney Disease (ADPKD).

ARPKD was previously subdivided into perinatal, neonatal, infantile, and juvenile forms. The different forms of the disease were based on the age at onset, which frequently correlates with the rate of progression and severity of the symptoms. This classification is archaic and is no longer valid nor should it be used.

ADPKD typically affects adults although increasing numbers of children are identified because of the increased use of ultrasound. ADPKD typically progresses more slowly than ARPKD.
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Resources

March of Dimes Birth Defects Foundation
1275 Mamaroneck Avenue
White Plains
NY
10605
Tel: (914)428-7100
Fax: (914)997-4763
800: (888)663-4637
Askus@marchofdimes.com
http://www.marchofdimes.com

American Kidney Fund, Inc.
6110 Executive Boulevard
Suite 1010
Rockville
MD
20852
USA
Tel: (301)881-3052
Fax: (301)881-0898
800: (800)638-8299
helpline@kideyfund.org
http://www.kidneyfund.org

National Kidney Foundation
30 East 33rd Street
New York
NY
10016
Tel: (212)889-2210
Fax: (212)689-9261
800: (800)622-9010
info@kidney.org
http://www.kidney.org

PKD Foundation
9221 Ward Parkway
Suite 400
Kansas City
MO
64114-3367
USA
Tel: (816)931-2600
Fax: (816)931-8655
800: (800)753-2873
pkdcure@pkdcure.org
http://www.pkdcure.org

American Urological Association Foundation
1000 Corporate Blvd.
Linthicum
MD
21090
USA
Tel: (410)689-3700
Fax: (410)689-3800
800: (866)746-4282
auafoundation@auafoundation.org
http://www.auafoundation.org

Guay-Woodford, Lisa M., M.D.
Division of Nephrology
University of Alabama Medical Center
ZRB 624, 1530 Third Ave S.
Birmingham
AL
35294-0007
Tel: (205)934-7308
Fax: (205)975-5689NIH/National Kidney and Urologic Diseases Information Clearinghouse
3 Information Way
Bethesda
MD
20892-3580
800: (800)891-5390
nkudic@info.niddk.nih.gov
http://kidney.niddk.nih.gov/

International Patient Advocacy Association
800 Bellevue Way
MGM Building, Suite 400
Bellevue
WA
98004
Tel: (426)646-3768National Hypertension Association, Inc.
324 East 30th Street
New York
NY
10016
Tel: (212)889-3557
Fax: (212)447-7032
800: (800)575-9355
nathypertension@aol.com
http://www.nathypertension.org

ARPKD/CHF Alliance
PO Box 70
Kirkwood
PA
17536
USA
Tel: (717)529-5555
Fax: (800)807-9110
800: (800)708-8892
info@arpkdchf.org
http://www.arpkdchf.org

Birth Defect Research for Children
930 Woodcock Rd
Suite 225
Orlando
FL
32803
USA
Tel: (407)895-0802
Fax: (407)895-0824
staff@birthdefects.org
http://www.birthdefects.org

MUMS (Mothers United for Moral Support, Inc) National Parent-to-Parent Network
150 Custer Court
Green Bay
WI
54301-1243
USA
Tel: (920)336-5333
Fax: (920)339-0995
800: (877)336-5333
mums@netnet.net
http://www.netnet.net/mums/

Kidney & Urology Foundation of America, Inc.
152 Madison Ave.
Suite 201
New York
NY
10016
Tel: (212)629-9770
Fax: (212)629-5652
800: (800)633-6628
info@kidneyurology.org
http://www.kidneyurology.org

For a Complete Report:

This is an abstract of a report from the National Organization for Rare Disorders, Inc. ® (NORD). A copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see http://www.rarediseases.org/search/rdblist.html

The information provided in this report is not intended for diagnostic purposes. It is provided for informational purposes only. NORD recommends that affected individuals seek the advice or counsel of their own personal physicians.

It is possible that the title of this topic is not the name you selected. Please check the Synonyms listing to find the alternate name(s) and Disorder Subdivision(s) covered by this report

This disease entry is based upon medical information available through the date at the end of the topic. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder.

For additional information and assistance about rare disorders, please contact the National Organization for Rare Disorders at P.O. Box 1968, Danbury, CT 06813-1968; phone (203) 744-0100; web site www.rarediseases.org or email orphan@rarediseases.org

Last Updated:  5/1/2008
Copyright  1986, 1987, 1990, 1991, 1992, 1994, 1995, 1996, 1999, 2002, 2003 National Organization for Rare Disorders, Inc.

WebMD Medical Reference from the National Organization of Rare Disorders

Last Updated: May 01, 2008
This information is not intended to replace the advice of a doctor. Healthwise disclaims any liability for the decisions you make based on this information.
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