Bilateral renal agenesis in three consecutive siblings

Morse, Richard P.; Rawnsley, Eileen; Crowe, Harte C.; Marín-Padilla, Miguel; Graham, John M.

doi:10.1002/pd.1970070808

Cited by 13 publications

(6 citation statements)

References 23 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…First, the marked oligohydramnios which occurs in all fetuses with BRA does not manifest until after 14 weeks of gestation, when fetal urine production makes an appreciable contribution to amniotic fluid volume. However, this case illustrates, as have previous reports [Curry et al, 1984, Morse et al, 1987 that oligohydramnios will be seen by 16-18 weeks gestation in the absence of fetal kidneys. Second, oligohydramnios and/or BRA may be associated with an elevated maternal serum alphafetoprotein [Freling et al, 19831.…”

Section: Discussionsupporting

confidence: 49%

Bilateral renal agenesis and Mullerian anomalies in a 47,XXX fetus

et al. 1989

View full text Add to dashboard Cite

Congenital absence of the kidneys (bilateral renal agenesis, BRA) is a genetically heterogeneous condition, and all modes of inheritance have been suggested as causes. The finding of a 47,XXX chromosome constitution in a fetus with BRA and developmental arrest of the mesonephric and paramesonephric systems raises the possibility that X chromosome trisomy also may cause the urogenital adysplasia sequence.

show abstract

Section: Discussionsupporting

confidence: 49%

Bilateral renal agenesis and Mullerian anomalies in a 47,XXX fetus

et al. 1989

View full text Add to dashboard Cite

show abstract

“…Many authors recommend an ultrasound study of the kidney in all family members with a history of unilateral or bilateral RA in the family to detect the presence of asymptomatic anomalies of the genitourinary system. Morse et al (1987) and McPherson et al (1987) suggest that the risk of recurrence of severe renal adysplasia is very high. Multicystic dysplastic kidney can be familial but is most commonly a sporadic anomaly, so formal screening of relatives is not recommended (Belk et al 2002).…”

Section: Discussionmentioning

confidence: 99%

“…Both malformations are more commonly diagnosed in boys (Cascio et al 1999;John et al 1998) and usually found on the left side (Kaneyama et al 2004;Ranke et al 2001), but they may also be bilateral (Feldenberg and Siegiel 2000;Parikh et al 2002). Cases of familial occurrence of MCDK and RA suggest that both anomalies are genetically conditioned (Belk et al 2002;Doray et al 1999;Morse et al 1987). Unilateral RA/MCDK occurs as an isolated defect or is accompanied by other urological anomalies.…”

Section: Introductionmentioning

confidence: 99%

Urological anomalies in children with renal agenesis or multicystic dysplastic kidney

et al. 2006

View full text Add to dashboard Cite

This study aimed to determine the frequency of associated urological abnormalities in children with unilateral renal agenesis (RA) or multicystic dysplastic kidney (MCDK). In total, 38 children (10 girls, 28 boys) were studied: 21 with RA and 17 with MCDK. In 14 children (37%) anomalies of the urinary tract were suspected prenatally in ultrasound studies. In the remaining 24 children the diagnosis of RA/MCDK was made postnatally: in 13 (34%) in the first 7 days of life, in 11 (29%) at the age of 8 days to 34 months, mean 10.6+/-8.05 months. Voiding cystourethrography was done in 36 (95%) children, the isotopic 99mTc-EC/DMSA scan of the kidney in 29 (67%), and urography in 8. Urological anomalies were present in 11 (29%) children: in 7 (33%) with RA and in 4 (24%) with MCDK. Vesicoureteral reflux was diagnosed in 8 children: grade II in 4, III in 3, and IV in 1 (in 1 child to duplicated, in 1 to ectopic kidney); ureterovesical junction obstruction in 2 (9.5%); and ureteropelvic junction obstruction in 1 (4.8%). Among them, 2 children demanded surgery on the contralateral urinary tract: pyeloplasty in 1, antireflux procedure in 1; while 9 children were treated conservatively. Compensatory hypertrophy of the contralateral kidney was found in 90% of children. Thus due to an increased risk of pathological changes in the single functioning kidney, lifelong nephrological care is recommended in patients with unilateral RA/MCDK.

show abstract

“…With chronic leakage of fluid, the risk is generally very low. Defects such as renal agenesis or obstructive uropathy have a higher risk of recurrence and renal anomalies in first-degree relatives [74]. The risk may be as high as 25% with infantile polycystic kidney disease due to autosomal recessive inheritance [61].…”

Section: Oligohydramniosmentioning

confidence: 99%

Extrinsic Factors Influencing Fetal Deformations and Intrauterine Growth Restriction

Moh

Graham

Wadhawan

et al. 2012

Journal of Pregnancy

Self Cite

View full text Add to dashboard Cite

The causes of intrauterine growth restriction (IUGR) are multifactorial with both intrinsic and extrinsic influences. While many studies focus on the intrinsic pathological causes, the possible long-term consequences resulting from extrinsic intrauterine physiological constraints merit additional consideration and further investigation. Infants with IUGR can exhibit early symmetric or late asymmetric growth abnormality patterns depending on the fetal stage of development, of which the latter is most common occurring in 70–80% of growth-restricted infants. Deformation is the consequence of extrinsic biomechanical factors interfering with normal growth, functioning, or positioning of the fetus in utero, typically arising during late gestation. Biomechanical forces play a critical role in the normal morphogenesis of most tissues. The magnitude and direction of force impact the form of the developing fetus, with a specific tissue response depending on its pliability and stage of development. Major uterine constraining factors include primigravida, small maternal size, uterine malformation, uterine fibromata, early pelvic engagement of the fetal head, aberrant fetal position, oligohydramnios, and multifetal gestation. Corrective mechanical forces similar to those that gave rise to the deformation to reshape the deformed structures are often used and should take advantage of the rapid postnatal growth to correct form.

show abstract

Bilateral renal agenesis in three consecutive siblings

Cited by 13 publications

References 23 publications

Bilateral renal agenesis and Mullerian anomalies in a 47,XXX fetus

Bilateral renal agenesis and Mullerian anomalies in a 47,XXX fetus

Urological anomalies in children with renal agenesis or multicystic dysplastic kidney

Extrinsic Factors Influencing Fetal Deformations and Intrauterine Growth Restriction

Contact Info

Product

Resources

About