keyword
https://read.qxmd.com/read/18202467/oligomeganephronia-an-unexpected-cause-of-chronic-renal-failure
#21
JOURNAL ARTICLE
Mohamed Abdelraheem, Alan R Watson, Thomas A McCulloch
Oligomeganephronia represents a distinct subgroup of renal hypoplasia in which there is a marked reduction in the number of nephrons with hypertrophy of those that are present. It can only be recognised on renal biopsy. We describe a 14-year-old boy who presents with a history of dysuria and some blood in his pants. Urinalysis showed no hematuria but persistent heavy proteinuria. The size of the kidneys on ultrasound was between -1 to -2 SD for height (right 9.3; left 9.6cm) and both kidneys showed a diffuse increase in echogenicity...
January 2004: Saudi Journal of Kidney Diseases and Transplantation
https://read.qxmd.com/read/16572339/age-related-pathophysiological-changes-in-rat-oligomeganephronic-hypoplastic-kidney
#22
JOURNAL ARTICLE
Hiroetsu Suzuki, Tsuyoshi Tokuriki, Hideto Kamita, Chiharu Oota, Masaki Takasu, Kenichi Saito, Katsushi Suzuki
Rat male hypogonadism (hgn/hgn) is accompanied by oligomeganephronic hypoplastic kidney (HPK), in which each kidney contains one quarter the number of nephrons present in a normal kidney. The nephrons of the HPK are extremely hypertrophied. These characters were apparently common to human oligomeganephronia (OMN). To determine the age-related changes in renal pathophysiology in HPK rats, we measured several parameters of renal function at 70 days, 140 days, 210 days, and 280 days of age. At all time points, relative kidney weight was significantly smaller in HPK rats than in their normal litter mates...
May 2006: Pediatric Nephrology
https://read.qxmd.com/read/15041338/renal-outcome-of-children-exposed-to-cyclosporine-in-utero
#23
REVIEW
P Cochat, S Decramer, E Robert-Gnansia, L Dubourg, P Audra
The number of pregnancies in immunosuppressed women has increased during the recent years and this has become a major part of the rehabilitation and quality of life of treated patients. Most of them are organ transplant recipients and large series from the literature have shown that children born to such women may present with intrauterine growth retardation, a condition which may be associated with significant reduction in nephron number and oligomeganephronia. On the other hand, experimental data in animals have demonstrated that in utero exposure to CsA may alter nephrogenesis and further alter renal function...
March 2004: Transplantation Proceedings
https://read.qxmd.com/read/12911528/enlarged-nephrons-and-severe-nondiabetic-nephropathy-in-hepatocyte-nuclear-factor-1beta-hnf-1beta-mutation-carriers
#24
JOURNAL ARTICLE
Jørn V Sagen, Leif Bostad, Pål R Njølstad, Oddmund Søvik
BACKGROUND: Mutations in hepatocyte nuclear factor-1beta (HNF-1beta) lead to a syndrome with diabetes and urogenital malformations [maturity onset of diabetes of the young, type 5 (MODY5)]. The aim of this study was to perform a clinicopathologic investigation of the renal disease in members of a Norwegian family with the HNF-1beta mutation R137-K161del. METHODS: The study was based on long-term clinical observations of five mutation carriers, combined with renal biopsies from four of these...
September 2003: Kidney International
https://read.qxmd.com/read/12874457/distinct-molecular-and-morphogenetic-properties-of-mutations-in-the-human-hnf1beta-gene-that-lead-to-defective-kidney-development
#25
JOURNAL ARTICLE
Silvia Bohn, Heike Thomas, Gülüzar Turan, Sian Ellard, Coralie Bingham, Andrew T Hattersley, Gerhart U Ryffel
The homeobox transcription factor hepatocyte nuclear factor 1beta (HNF1beta) is a tissue-specific regulator that plays an essential role in early vertebrate development. In humans, heterozygous mutations in the HNF1beta gene are associated with young-onset diabetes as well as a variety of disorders of renal development with cysts as the most consistent feature. This report compares and classifies nine different HNF1beta mutations that lead in humans to distinct renal diseases, including solitary functioning kidney, renal dysplasia, glomerulocystic kidney disease, and oligomeganephronia...
August 2003: Journal of the American Society of Nephrology: JASN
https://read.qxmd.com/read/11908261/oligonephropathy-from-a-rare-childhood-disorder-to-a-possible-health-problem-in-the-adult
#26
REVIEW
Alfred Drukker
Recent data have shed significant new light on the structural and functional development of the kidneys, as well as on a rare congenital form of bilateral renal hypoplasia called congenital oligomeganephronia. In this renal disorder, few greatly enlarged and "hard-working" nephrons are found that will ultimately sclerose and lead to end-stage renal failure during early childhood. At the same time it has been recognized that the number of nephrons in the kidneys of various animal species and humans is correlated to renal mass...
March 2002: Israel Medical Association Journal: IMAJ
https://read.qxmd.com/read/11732768/muromonab-cd3-induced-neurotoxicity-report-of-two-siblings-one-of-whom-had-subsequent-cyclosporin-induced-neurotoxicity
#27
JOURNAL ARTICLE
P Thaisetthawatkul, A Weinstock, S L Kerr, M E Cohen
Muromonab-CD3 is widely used for immunosuppression in patients undergoing solid organ transplant. We report two siblings with oligomeganephronia and end-stage renal disease who developed encephalopathy and seizures from muromonab-CD3 following renal transplant. The first case is a 13-year-old girl who developed encephalopathy, seizure, and triparesis following renal transplant while muromonab-CD3 was used for immunosuppression. The second case was the 6-year-old sister of the first case, who also developed recurrent focal seizures while she was on muromonab-CD3 for renal transplant immunosuppression...
November 2001: Journal of Child Neurology
https://read.qxmd.com/read/11434182/-a-nineteen-year-observation-of-a-boy-with-branchio-oto-renal-syndrome-and-chronic-renal-failure
#28
JOURNAL ARTICLE
P Sikora, M Zajaczkowska, E Katska, G Niedzielska
Branchio-oto-renal syndrome is a rare autosomal dominant disorder of the first and second embryonic branchial arches and the urinary tract. It is characterized in its full expression by branchial fistulas or cysts, preauricular pits, outer, middle and inner ear defects, hearing loss, lachrymal duct stenosis, facial paralysis and mental retardation. Renal anomalies may range from mild hypoplasia to complete absence. Our report demonstrates the patient with classical BOR syndrome and severe renal insufficiency since infancy up to end stage renal failure at 18 years of age caused by bilateral renal hypoplasia...
April 2001: Polski Merkuriusz Lekarski: Organ Polskiego Towarzystwa Lekarskiego
https://read.qxmd.com/read/11168927/pax2-mutations-in-oligomeganephronia
#29
JOURNAL ARTICLE
R Salomon, A L Tellier, T Attie-Bitach, J Amiel, M Vekemans, S Lyonnet, P Dureau, P Niaudet, M C Gubler, M Broyer
BACKGROUND: Oligomeganephronia (OMN) is a rare congenital and usually sporadic anomaly. It is characterized by bilateral renal hypoplasia, with a reduced number of enlarged nephrons. The mechanisms involved in this deficient nephrogenesis are unknown. The paired box transcription factor PAX2 plays a fundamental role in renal development. Heterozygous Pax2 mutants in mice are characterized by renal hypoplasia and retinal defects, and in humans, PAX2 mutations have been described in the renal-coloboma syndrome...
February 2001: Kidney International
https://read.qxmd.com/read/11139890/-branchio-oto-renal-syndrome-bor-syndrome-a-dysplasia-syndrome-with-branchial-abnormalities-deafness-and-kidney-disease
#30
JOURNAL ARTICLE
M Holzmüller
The branchio-oto-renal syndrome (BOR syndrome) is characterized by auricular abnormalities, lateral cervical fistulas and preauricular tags. The hearing impairment may be a conductive, a sensorineural or a mixed hearing loss. The renal disease is oligomeganephronia, a bilateral, congenital renal abnormality with reduced numbers of nephrons. The BOR syndrome is an autosomal-dominant disease. An 8-year-old girl with preauricular tags, cervical fistulas and auricular abnormalities is reported upon. She has a mixed hearing loss and anomalies in the vestibular system...
November 2000: HNO
https://read.qxmd.com/read/10861677/distal-limb-deficiencies-oral-involvement-and-renal-defect-report-of-a-third-patient-and-confirmation-of-a-distinct-entity
#31
JOURNAL ARTICLE
K Keymolen, R Van Damme-Lombaerts, A Verloes, J P Fryns
In 1987 Buttiens and Fryns [1987: Am J Med Genet 27:651-660] reported on two sibs, brother and sister, with severe distal limb defects, micrognathia, and mild to moderate mental retardation. The male also showed severe myopia and oligomeganephronia. To the best of our knowledge, no other similar patients have been described since. We report on a boy with a similar phenotype. .
July 3, 2000: American Journal of Medical Genetics
https://read.qxmd.com/read/9332654/ring-chromosome-4-mosaicism-coincidence-of-oligomeganephronia-and-signs-of-seckel-syndrome
#32
JOURNAL ARTICLE
C E Anderson, R Wallerstein, S T Zamerowski, C Witzleben, J R Hoyer, L Gibas, L G Jackson
We present a patient with features suggestive of Seckel syndrome who was found to be mosaic for ring 4 chromosome. Seckel syndrome is a rare entity characterized by marked growth retardation, microcephaly, facies characterized by receding forehead and chin, large beaked nose, and severe retardation, usually thought to be inherited as an autosomal recessive condition. In addition, our patient had oligomeganephronia, a rare and usually sporadic renal malformation, previously reported in two other patients with abnormalities of chromosome 4...
October 31, 1997: American Journal of Medical Genetics
https://read.qxmd.com/read/9277763/-renal-agenesis-renal-dysplasia-renal-aplasia-renal-hypoplasia-oligomeganephronia
#33
REVIEW
T Kawamura
No abstract text is available yet for this article.
1997: Ryōikibetsu Shōkōgun Shirīzu
https://read.qxmd.com/read/8971900/acrorenal-syndrome-associated-with-visual-defect
#34
JOURNAL ARTICLE
A A al Salloum, S A al Rasheed, M A al Husain, M M al Mugeiren, A S al Rikabi, M O al Sohaibani
The clinical biochemical, radiological, and histological data of a 5-year-old boy with severe limb deformities and renal failure due to oligomeganephronia and renal hypoplasia are reported. This patient represents another example of acrorenal syndrome. This boy has a severe visual defect due to pigmentory retinopathy, which has not been reported previously.
December 1996: Pediatric Nephrology
https://read.qxmd.com/read/8196224/-a-case-of-oligomeganephronia-quantitative-measurement-of-the-glomerular-surface-area-and-the-number-of-glomeruli-with-a-color-image-analyzer
#35
JOURNAL ARTICLE
T Ohshima, S Inaba, M Arai, Y Sakai, K Kurose, S Ishihara, T Takahashi, R Takai, H Matukura, R Yoshida
We report here a nine-year-old boy with oligomeganephronia. The patient had proteinuria and morphometric analysis of the renal biopsy specimen confirmed the diagnosis of oligomeganephronia. We employed a color image analyzer (OLYMPUS Corp.) to determine the glomerular surface area and the number of glomeruli per mm2 of the renal cortex of the patient. All the scanned glomeruli showed marked hypertrophy; the mean maximal surface area for the 5 glomeruli was 46 x 10(3) microns 2, which is approximately 4 times larger than that of normal children of the same age...
March 1994: Nihon Jinzo Gakkai Shi
https://read.qxmd.com/read/8108293/oligomeganephronia-associated-with-4p-deletion-type-chromosomal-anomaly
#36
JOURNAL ARTICLE
S H Park, J G Chi
Oligomeganephronia is a rare congenital renal hypoplasia that is characterized histologically by a reduction in the number of reniculi with compensatory hypertrophy of the glomeruli and proximal renal tubules. Oligomeganephronia has generally been regarded as a congenital but not genetic disease. Kusuyama et al. first suggested that oligomeganephronia might be associated with a chromosomal anomaly, namely 4p monosomy syndrome. Their assumption originated from the fact that external anomalies of their cases of oligomeganephronia are very much like those described in 4p monosomy syndrome...
November 1993: Pediatric Pathology
https://read.qxmd.com/read/7855013/deficient-metanephric-blastema-a-cause-of-oligomeganephronia
#37
JOURNAL ARTICLE
S V Foster, E P Hawkins
The histopathology and morphometric findings in two cases of oligomeganephronia occurring in a fetus and a preterm infant are reported. Metanephric blastema was decreased in the kidney from an 18-week fetus and was absent in the kidney from a liveborn preterm infant of 33 weeks gestation. These findings support the hypothesis that a primary deficiency of metanephric blastema may be a pathogenetic mechanism of oligomeganephronia.
November 1994: Pediatric Pathology
https://read.qxmd.com/read/7120695/-oligomeganephronia-report-of-two-cases
#38
JOURNAL ARTICLE
S Taniguchi, Y Terai, H Murakawa, S Oriso, T Sakuma, R Satodate, N Tomisawa
No abstract text is available yet for this article.
March 1982: Nihon Jinzo Gakkai Shi
https://read.qxmd.com/read/7072713/congenital-oligomeganephronia-in-a-solitary-kidney-report-of-a-case
#39
JOURNAL ARTICLE
M Lam, D Halverstadt, G Altshuler, J E Wenzl
No abstract text is available yet for this article.
March 1982: American Journal of Kidney Diseases
https://read.qxmd.com/read/6714259/a-new-variant-of-the-acrorenal-syndrome-associated-with-bilateral-oligomeganephronic-hypoplasia
#40
JOURNAL ARTICLE
M Miltényi, L Balogh, K Schmidt, Z Detre, T Hernády, A Czeizel
The case of a 2-year-old boy with associated anomalies of the limbs and the urinary system is reported. Similar to cases reported earlier he presented a split hand and foot syndrome, but differed from previously reported cases by the presence of bilateral renal hypoplasia leading to end stage renal failure. Renal histology disclosed oligomeganephronia.
April 1984: European Journal of Pediatrics
keyword
keyword
211693
2
3
Fetch more papers »
Fetching more papers... Fetching...
Remove bar
Read by QxMD icon Read
×

Save your favorite articles in one place with a free QxMD account.

×

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"

We want to hear from doctors like you!

Take a second to answer a survey question.