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         Renal Agenesis:     more detail
  1. Renal agenesis: An entry from Thomson Gale's <i>Gale Encyclopedia of Genetic Disorders, 2nd ed.</i> by Maria, PhD Basile, 2005
  2. A case of renal agenesis and hypertension by Holly Lynne Zapf, 1997

1. Renal Agenesis Surveillance -- United States
renal agenesis Surveillance United States. Newborns with bilateral renal agenesishave lowset, floppy ears, a broad, flat nose, and underdeveloped lungs.
http://www.cdc.gov/epo/mmwr/preview/mmwrhtml/00001301.htm
November 10, 1988 / 37(44);679-680,685-686
Renal Agenesis Surveillance United States
Newborns with bilateral renal agenesis have low-set, floppy ears, a broad, flat nose, and underdeveloped lungs. These newborns often die of respiratory failure within a few hours of birth. Lungs of infants with at least one functional kidney usually develop normally; thus, unilateral renal agenesis is often not detected during the perinatal period. The cases in this evaluation included 1404 newborns with an ICD code of 753.0. Participating hospitals provided copies of the discharge summary, pathology reports, and x-ray reports. For 966 (69%) of the infants, hospital reports supported the diagnosis of renal agenesis or renal dysgenesis. Of these, 468 (48%) had bilateral renal agenesis, 105 (11%) had one agenic and one dysgenic kidney, 229 (24%) had bilateral renal dysgenesis, 136 (14%) had unilateral renal agenesis, and 28 (3%) had unilateral renal dysgenesis. For the 14-year period 1970-1983, clinical reports of anomalies coded to 753.0 showed the following trends: 1) the annual frequency of bilateral renal agenesis varied considerably but appeared to increase slowly; 2) the prevalence at birth of one agenic and one dysgenic kidney remained constant; 3) the annual frequency of unilateral renal agenesis varied, although the number of infants with this diagnosis was small, and most cases were found incidentally during autopsy; and 4) the incidence of bilateral renal dysgenesis increased 10-fold from 0.03 per 10,000 births in 1970 to 0.33 per 10,000 births in 1983.

2. Perinate.org
Click here to return to our home page, CAR Anomalies Renal renal agenesis. Renalagenesis anomaly may affect one or both kidneys and has an unknown aetiology.
http://www.wmpi.net/car/anomaly/renal/renalagenesis.htm
CAR: Anomalies - Renal
Renal Agenesis
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Abdominal
Cardiac Chromosome CNS ... Back to the CAR introduction page INTRODUCTION Renal agenesis anomaly may affect one or both kidneys and has an unknown aetiology. Bilateral renal agenesis (BRA) occurs sporadically but there is some evidence that genetic factors are involved and it is important to screen both parents and siblings. BRA can also occur as part of syndromes such as VATER and branchio-oto-renal syndrome but it is more commonly an isolated finding. BRA occurs more frequently in males (2:1) and is incompatible with life. Unilateral renal agenesis (URA) is much more common than BRA and is associated with some ear and ureteral anomalies. Top of the page Back to Renal ANTENATAL Renal agenesis may not be immediately apparent on ultrasound, but the bladder is never seen and from 16 weeks gestation it becomes apparent that there is reduced, or no liquor around the fetus. Doppler studies can be used to distinguish BRA from severe intrauterine growth retardation.

3. Renal Agenesis
renal agenesis and Hypoplastic Lung Syndrome Vol 2. No. 10 Ande L.Karimu MD PhD. Resident ObGyn Yale New Haven Hospital Renal
http://www.hygeia.org/poems22.htm
Renal Agenesis and Hypoplastic Lung Syndrome
Vol 2. No. 10

Ande L. Karimu MD PhD.
Resident Ob-Gyn Yale New Haven Hospital
Renal agenesis and hypoplastic lung syndrome are congenital malformations of the neonates involving the kidneys and the lungs respectively i.e. the newborns are born with these disorders. The etiology of these malformations is probably multifactorial i.e. there are both inherited and environmental factors in the causation of the malformations. Oftentime both conditions co-exist as part of multiple congenital malformations.
Renal Agenesis
Renal agenesis is the complete absence of the kidney(s). The kidneys are the organs that filter the blood of waste products, eliminating them as urine.
There are two kidneys in the human under normal circumstances. Absence of the kidney could be unilateral or bilateral. If it is unilateral, it means only one kidney is absent. However, if it is bilateral, it means both kidneys are absent. Unilateral absence of the kidneys is compatible with life whereas bilateral absence of the kidneys is incompatible with life.
Development of the Kidneys
The kidneys are parts of the urinary system. Other members of the system include the ureters, bladder and the urethra. The urinary system develops in close association with the genital organs.

4. Renal Agenesis
renal agenesis. DESCRIPTION. renal agenesis is the absence of one or both of the kidneys. Bilateral renal agenesis is invariably fatal. Unilateral renal agenesis may be
http://www.tdh.state.tx.us/tbdmd/risk/risk22-renal.htm
Get an Adobe Acrobat version of this document. Important information about this document. Return to Risk Factor Menu
BIRTH DEFECT RISK FACTOR SERIES:
RENAL AGENESIS
DESCRIPTION
Renal agenesis is the absence of one or both of the kidneys. Bilateral renal agenesis is invariably fatal. Unilateral renal agenesis may be asymptomatic and is often incidentally diagnosed by abdominal ultrasound or computed tomography (CT) scan secondary to another condition. In infants with unilateral renal agenesis, the remaining kidney may be enlarged, and there is increased risk of problems with the remaining kidney. Renal agenesis has been associated with such chromosomal abnormalities as trisomy 21, trisomy 22, trisomy 7, trisomy 10, 45,X mosaicism, and 22q11 microdeletion (Bianchi et al., 2000). Fetal deaths have been reported to account for 10-33% of cases of renal agenesis (Bianchi et al., 2000; Riley et al., 1998; Cunniff et al., 1994). Renal agenesis can be prenatally detected by ultrasound (Bianchi et al., 2000). Thus, in regions where elective termination is allowed, prenatal diagnosis and elective termination may reduce the birth prevalence of renal agenesis (Riley et al., 1998; Sipek et al., 1997; Chi et al., 1995; Papp et al., 1995; Stoll et al., 1995a; Stoll et al., 1995b; Cunniff et al., 1994; Julian-Reynier et al., 1994; Stoll et al., 1992). The metanephric buds begin to develop into kidneys in the fifth week of gestation. If the metanephric buds fail to develop, renal agenesis results.

5. TheFetus.net - Renal Agenesis, Bilateral-Philippe Jeanty, MD, PhD & Sandra R Sil
Technical explanation of bilateral renal agenesis.
http://www.thefetus.net/page.php?id=540

6. Potter's Syndrome (www.whonamedit.com)
Potter's syndrome The total absence or malformation of infant kidneys. Bilateral renal agenesis syndrome (BRA) syndrome, bilateral kidney agenesis syndrome, dysplasia renofacialis Edith L .
http://www.whonamedit.com/synd.cfm/2331.html

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Potter's syndrome Synonyms:
Bilateral renal agenesis syndrome (BRA) syndrome, bilateral kidney agenesis syndrome, dysplasia renofacialis, oligohydramnios sequence, oligohydramnios tetrad, renofacial dysplasia, renofacial syndrome.
Associated persons: Edith Louise Potter Description: Potters Syndrome is the term used to describe the total absence or malformation of infant kidneys in combination with other congenital abnormalities, including Potter's facies, pulmonary hypoplasia, oligohydramnios, failure of foetal urinary production and skeletomuscular anomalies such as clubbing of the hands and feet and contractures. Oligohydramnios (deficiency of the amount of amniotic fluid) is the cause of failure of lung development and of limb compression in the foetus. Males are more commonly affected than females. The affected children are usually stillborn or die shortly after birth. Authors disagree whether inheritance is autosomal dominant recessive. Bibliography:
  • H. Madisson:

7. "The Teaching File Case 1"
seminal vesicle cysts are associated with ipsilateral renal agenesis. When findings such as this case occur (renal agenesis, simple ipsilateral pelvic cyst), the
http://www.uab.edu/pedradpath/case1.html
Radiology-Pathology Teaching File Case# 1
Ten Month Old Boy with Pelvic Mass
Clinical Information: Ten-month-old white male with constipation. A nontender mass is palpated on rectal exam. A. B. IVU: Intravenous urogram (B) demonstrates non-visualization of the left kidney and conpensatory hypertrophy of the right kidney. A mass in the left pelvis indents the bladder. Pelvic ultrasound: A transverse US image (A) reveals a mid to left sided retrovesicular simple cyst with displacement of the urinary bladder anteriorly and to the right. No solid component is seen. C. D. Pathologic specimen: The gross specimen (D) reveals a cystic mass of the left seminal vesicle which was resected with the cyst. Microscopic evaluation (C) revealed a simple cyst with the cyst wall consisting of smooth muscle and fibrous connective tissue with a small amount of stratified squamous epithelium. These findings are typical for a seminal vesicle cyst. Discussion: Seminal Vesicle Cyst with Ipsilateral Renal Agenesis Ipsilateral renal agenesis and cyst of the seminal vesicle are associated due to the common origin of the ureteral bud and seminal vesicle from the mesonephric (Wolffian duct). If an insult occurs approximately at the 12th gestational week, the embryogenesis of the kidney, ureter, seminal vesicle, and vas deferens can be altered.

8. NORD - National Organization For Rare Disorders, Inc.
General Discussion. Bilateral renal agenesis is the absence of both kidneys at birth have proven that unilateral renal agenesis and bilateral renal agenesis are genetically related
http://www.rarediseases.org/search/rdbdetail_abstract.html?disname=Renal Agenesi

9. Renal Agenesis
, clinical features, and management.......
http://hygeia.org/poems22.htm
Renal Agenesis and Hypoplastic Lung Syndrome
Vol 2. No. 10

Ande L. Karimu MD PhD.
Resident Ob-Gyn Yale New Haven Hospital
Renal agenesis and hypoplastic lung syndrome are congenital malformations of the neonates involving the kidneys and the lungs respectively i.e. the newborns are born with these disorders. The etiology of these malformations is probably multifactorial i.e. there are both inherited and environmental factors in the causation of the malformations. Oftentime both conditions co-exist as part of multiple congenital malformations.
Renal Agenesis
Renal agenesis is the complete absence of the kidney(s). The kidneys are the organs that filter the blood of waste products, eliminating them as urine.
There are two kidneys in the human under normal circumstances. Absence of the kidney could be unilateral or bilateral. If it is unilateral, it means only one kidney is absent. However, if it is bilateral, it means both kidneys are absent. Unilateral absence of the kidneys is compatible with life whereas bilateral absence of the kidneys is incompatible with life.
Development of the Kidneys
The kidneys are parts of the urinary system. Other members of the system include the ureters, bladder and the urethra. The urinary system develops in close association with the genital organs.

10. Pediatric Pathology
Agenesis refers to the absence of formation of a body part in embryogenesis from the retroperitoneum, and this renal agenesis will result in oligohydramnios, because amniotic
http://www-medlib.med.utah.edu/WebPath/PEDHTML/PED202.html
Agenesis refers to the absence of formation of a body part in embryogenesis. Here the kidneys are absent from the retroperitoneum, and this renal agenesis will result in oligohydramnios, because amniotic fluid is mainly derived from fetal urine.

11. Renal Agenesis - Information / Diagnosis / Treatment / Prevention
home urological disorders congenital anomalies renal agenesis RenalAgenesis. Information Subtopics renal agenesis Personal Pages, Related
http://www.healthcyclopedia.com/urological-disorders/congenital-anomalies/renal-

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Subtopics: Renal Agenesis > Personal Pages Related Topics: Reproductive Health/Pregnancy and Birth/Complications/Oligohydramnios Medical Definition: University of Newcastle-upon-Tyne Medical Dictionary: "renal agenesis" Health News: Search millions of published articles for news on Renal Agenesis Modern Medicine Aging The Ardell Wellness Report HealthFacts Medical Post Medical Update Men's Health and the National Women's Health Report Note: Subscription required to access the full text of articles. Web Directory: Development Summary of a study titled "Renal agenesis and hypodysplasia in ret-k- mutant mice result from defects in ureteric bud development". Pediatric Pathology Picture of a baby with typical "Potter's facies".

12. Development -- Summaries: Schuchardt Et Al. 122 (6): 1919
Summary of a study titled renal agenesis and hypodysplasia in retk- mutant mice result from defects in ureteric bud development .
http://dev.biologists.org/cgi/content/abstract/122/6/1919
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JOURNAL ARTICLES
Renal agenesis and hypodysplasia in ret-k- mutant mice result from defects in ureteric bud development
A Schuchardt, V D'Agati, V Pachnis and F Costantini
Department of Genetics and Development, College of Physicians and Surgeons, Columbia University, NY 10032, USA. The c-ret gene encodes a receptor tyrosine kinase that is expressed in the Wolffian duct and ureteric bud of the developing excretory system. Newborn mice homozygous for a mutation in c-ret displayed renal agenesis or severe hypodysplasia, suggesting a critical role for this gene in metanephric kidney development. To investigate the embryological basis of these defects, we characterized the early development of the excretory system in mutant homozygotes, and observed a range of defects in the formation

13. Renal Agenesis
renal agenesis. associated with duplicated vagina and/or uterus (mullerianduct anomaly); seminal vesicle cysts. Charles E. Kahn, Jr
http://chorus.rad.mcw.edu/doc/00464.html
CHORUS Collaborative Hypertext of Radiology Kidney
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renal agenesis
associated with
  • duplicated vagina and/or uterus ( mullerian duct anomaly)
  • seminal vesicle cysts
Charles E. Kahn, Jr., MD - 2 February 1995
Last updated 26 May 2004

Medical College of Wisconsin

14. Renal Agenesis
renal agenesis. associated with duplicated vagina and/or uterus (mullerianduct anomaly); seminal vesicle cysts. Home Kidney Disclaimer
http://chorus.rad.mcw.edu/to-go/00464.html
renal agenesis
associated with
  • duplicated vagina and/or uterus ( mullerian duct anomaly)
  • seminal vesicle cysts
Home Kidney

15. Renal Agenesis Articles, Support Groups, And Resources
renal agenesis articles, support groups, and resources for patientsfrom Med Help International (www.medhelp.org). renal agenesis.
http://www.medhelp.org/HealthTopics/Renal_Agenesis.html
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16. Renal Agenesis, Bilateral
Bilateral renal agenesis is the absence of both kidneys at birth. It is a genetic disorder characterized by a failure of the kidneys to develop in a fetus. This absence of kidneys causes a agenesis). Studies have proven that unilateral renal agenesis and bilateral renal agenesis are genetically related.
http://hw.healthdialog.com/kbase/nord/nord691.htm
document.write(''); var hwPrint=1; var hwDocHWID="nord691"; var hwDocTitle="Renal Agenesis, Bilateral"; var hwRank="1"; var hwSectionHWID="nord691"; var hwSectionTitle=""; var hwSource="us6.0"; var hwProdCfgSerNo="wsh_html_003_s"; var hwDocType="NORD";
National Organization for Rare Disorders, Inc.
Renal Agenesis, Bilateral
Important
It is possible that the main title of the report is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.
Synonyms
  • Kidney Agenesis Renal Agenesis
Disorder Subdivisions
  • None
Related Disorders List
Information on the following diseases can be found in the Related Disorders section of this report:
  • Oligohydramnios Sequence (Potter Syndrome) Cat-Eye Syndrome Fraser Syndrome Melnick-Fraser Syndrome MURCS Association Rokitansky Sequence Sirenomelia Sequence Unilateral Renal Agenesis
General Discussion
Bilateral Renal Agenesis is the absence of both kidneys at birth. It is a genetic disorder characterized by a failure of the kidneys to develop in a fetus. This absence of kidneys causes a deficiency of amniotic fluid (Oligohydramnios) in a pregnant woman. Normally, the amniotic fluid acts as a cushion for the developing fetus. When there is an insufficient amount of this fluid, compression of the fetus may occur resulting in further malformations of the baby.
This disorder is more common in infants born of a parent who has a kidney malformation, particularly the absence of one kidney (unilateral renal agenesis). Studies have proven that unilateral renal agenesis and bilateral renal agenesis are genetically related.

17. Renal Agenesis
renal agenesis. Forum The Urology Forum Topic Kidney I ve just discovered (thruCT Scan) I have congenital unilateral renal agenesis (absent kidney).
http://www.medhelp.org/forums/urology/messages/C30954-1.html
Renal Agenesis
Forum: The Urology Forum
Topic: Kidney ID From To Post Q GI Joe
(08-Apr-04) I've just discovered (thru CT Scan) I have congenital unilateral renal agenesis (absent kidney). I'm a military member (U.S.)and generally in good health. Will this condition disqualify me from military service? A Forum-M.D.-KYP
(09-Apr-04) GI Joe I do not work for the US miltary so I do not know what the conditions are for disqualification. However, I can give information about the prognosis of the disorder.
Renal agenesis refers to congenital absence of the kidney and ureter, which may be either unilateral or bilateral. Unilateral renal agenesis has been noted in 1/1000 autopsies.
The prognosis for patients with one normal kidney is excellent, with a survival rate similar to that of age and sex-matched controls from United States life tables. However, proteinuria (i.e. protein in the urine), hypertension, and renal insufficiency appear to be more common later in life. Most pediatric urologists recommend that children with a solitary kidney avoid contact sports, although the risk of kidney loss resulting from trauma is less than 1 percent.
I am not sure if that last statement would be relevent in active duty. You may want to address this issue with your personal physician.

18. Renal Agenesis, Bilateral
Bilateral renal agenesis is the absence of both kidneys at birth. NationalOrganization for Rare Disorders, Inc. renal agenesis, Bilateral.
http://www.bchealthguide.org/kbase/nord/nord691.htm
document.write(''); var hwPrint=1; var hwDocHWID="nord691"; var hwDocTitle="Renal Agenesis, Bilateral"; var hwRank="1"; var hwSectionHWID="nord691"; var hwSectionTitle=""; var hwSource="cn6.0"; var hwProdCfgSerNo="wsh_html_031_s"; var hwDocType="NORD";
National Organization for Rare Disorders, Inc.
Renal Agenesis, Bilateral
Important
It is possible that the main title of the report is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.
Synonyms
  • Kidney Agenesis Renal Agenesis
Disorder Subdivisions
  • None
Related Disorders List
Information on the following diseases can be found in the Related Disorders section of this report:
  • Oligohydramnios Sequence (Potter Syndrome) Cat-Eye Syndrome Fraser Syndrome Melnick-Fraser Syndrome MURCS Association Rokitansky Sequence Sirenomelia Sequence Unilateral Renal Agenesis
General Discussion
Bilateral Renal Agenesis is the absence of both kidneys at birth. It is a genetic disorder characterized by a failure of the kidneys to develop in a fetus. This absence of kidneys causes a deficiency of amniotic fluid (Oligohydramnios) in a pregnant woman. Normally, the amniotic fluid acts as a cushion for the developing fetus. When there is an insufficient amount of this fluid, compression of the fetus may occur resulting in further malformations of the baby.
This disorder is more common in infants born of a parent who has a kidney malformation, particularly the absence of one kidney (unilateral renal agenesis). Studies have proven that unilateral renal agenesis and bilateral renal agenesis are genetically related.

19. Fraser Syndrome
may include improper development (dysplasia), underdevelopment (hypoplasia), orabsence of one or both kidneys (unilateral or bilateral renal agenesis).
http://www.bchealthguide.org/kbase/nord/nord739.htm
document.write(''); var hwPrint=1; var hwDocHWID="nord739"; var hwDocTitle="Fraser Syndrome"; var hwRank="1"; var hwSectionHWID="nord739"; var hwSectionTitle=""; var hwSource="cn6.0"; var hwProdCfgSerNo="wsh_html_031_s"; var hwDocType="NORD";
National Organization for Rare Disorders, Inc.
Fraser Syndrome
Important
It is possible that the main title of the report is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.
Synonyms
  • Cryptophthalmos-Syndactyly Syndrome
Disorder Subdivisions
  • None
Related Disorders List
Information on the following diseases can be found in the Related Disorders section of this report:
  • Renal Agenesis, Bilateral Cat-Eye Syndrome Melnick-Fraser Syndrome
General Discussion
Symptoms
Fraser Syndrome is characterized by multiple physical abnormalities. These may include eye defects with complete fusion of the eyelids (cryptophthalmos), malformed or missing kidneys (renal agenesis), partial fusion of the fingers and toes (syndactyly) and middle and outer ear deformities. The nose is usually broad with a flattened bridge and deep indentations on the side of each nostril. Other characteristics may include hair growth that extends from the forehead to the eyebrows, high or cleft palate, malformation of the eyelid ducts that convey tears, a displaced navel, malformed or missing larynx, widely spaced nipples, malformation of the pubic bones and mental deficiency.

20. Pediatric Pathology
Here the kidneys are absent from the retroperitoneum, and this renal agenesis willresult in oligohydramnios, because amniotic fluid is mainly derived from
http://medlib.med.utah.edu/WebPath/PEDHTML/PED202.html
Agenesis refers to the absence of formation of a body part in embryogenesis. Here the kidneys are absent from the retroperitoneum, and this renal agenesis will result in oligohydramnios, because amniotic fluid is mainly derived from fetal urine.

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