Wilms tumor in a patient with Prader-Willi syndrome. 1993

M J Coppes, and H Sohl, and I E Teshima, and A Mutirangura, and D H Ledbetter, and R Weksberg
Department of Cancer Biology, Cleveland Clinic Foundation, Ohio.

The development of Wilms tumor in a patient with Prader-Willi syndrome prompted us to determine the parental origin of the genes implicated in both disorders because of the sex-specific parent-of-origin effects previously demonstrated for both conditions. A paternal chromosome 15q11-q13 deletion was demonstrated, but no changes were demonstrated in a limited analysis of chromosome 11p, which harbors two Wilms tumor suppressor genes, WT1 and WT2.

UI MeSH Term Description Entries
D007223 Infant A child between 1 and 23 months of age. Infants
D007680 Kidney Neoplasms Tumors or cancers of the KIDNEY. Cancer of Kidney,Kidney Cancer,Renal Cancer,Cancer of the Kidney,Neoplasms, Kidney,Renal Neoplasms,Cancer, Kidney,Cancer, Renal,Cancers, Kidney,Cancers, Renal,Kidney Cancers,Kidney Neoplasm,Neoplasm, Kidney,Neoplasm, Renal,Neoplasms, Renal,Renal Cancers,Renal Neoplasm
D008297 Male Males
D009396 Wilms Tumor A malignant kidney tumor, caused by the uncontrolled multiplication of renal stem (blastemal), stromal (STROMAL CELLS), and epithelial (EPITHELIAL CELLS) elements. However, not all three are present in every case. Several genes or chromosomal areas have been associated with Wilms tumor which is usually found in childhood as a firm lump in a child's side or ABDOMEN. Bilateral Wilms Tumor,Nephroblastoma,Wilms Tumor 1,Wilms' Tumor,Nephroblastomas,Tumor, Bilateral Wilms,Tumor, Wilms,Tumor, Wilms',Wilm Tumor,Wilm's Tumor,Wilms Tumor, Bilateral
D011218 Prader-Willi Syndrome An autosomal dominant disorder caused by deletion of the proximal long arm of the paternal chromosome 15 (15q11-q13) or by inheritance of both of the pair of chromosomes 15 from the mother (UNIPARENTAL DISOMY) which are imprinted (GENETIC IMPRINTING) and hence silenced. Clinical manifestations include MENTAL RETARDATION; MUSCULAR HYPOTONIA; HYPERPHAGIA; OBESITY; short stature; HYPOGONADISM; STRABISMUS; and HYPERSOMNOLENCE. (Menkes, Textbook of Child Neurology, 5th ed, p229) Labhart-Willi Syndrome,Royer Syndrome,Labhart-Willi-Prader-Fanconi Syndrome,Prader Labhart Willi Syndrome,Prader-Labhart-Willi Syndrome,Royer's Syndrome,Willi-Prader Syndrome,Labhart Willi Prader Fanconi Syndrome,Labhart Willi Syndrome,Prader Willi Syndrome,Royers Syndrome,Syndrome, Labhart-Willi,Syndrome, Labhart-Willi-Prader-Fanconi,Syndrome, Prader-Labhart-Willi,Syndrome, Prader-Willi,Syndrome, Royer,Syndrome, Royer's,Syndrome, Willi-Prader,Willi Prader Syndrome
D002872 Chromosome Deletion Actual loss of portion of a chromosome. Monosomy, Partial,Partial Monosomy,Deletion, Chromosome,Deletions, Chromosome,Monosomies, Partial,Partial Monosomies
D002880 Chromosomes, Human, Pair 11 A specific pair of GROUP C CHROMOSOMES of the human chromosome classification. Chromosome 11
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man

Related Publications

M J Coppes, and H Sohl, and I E Teshima, and A Mutirangura, and D H Ledbetter, and R Weksberg
January 2017, Tijdschrift voor psychiatrie,
M J Coppes, and H Sohl, and I E Teshima, and A Mutirangura, and D H Ledbetter, and R Weksberg
January 2018, International journal of retina and vitreous,
M J Coppes, and H Sohl, and I E Teshima, and A Mutirangura, and D H Ledbetter, and R Weksberg
January 1995, Japanese journal of ophthalmology,
M J Coppes, and H Sohl, and I E Teshima, and A Mutirangura, and D H Ledbetter, and R Weksberg
January 2001, Annales de genetique,
M J Coppes, and H Sohl, and I E Teshima, and A Mutirangura, and D H Ledbetter, and R Weksberg
July 1981, Masui. The Japanese journal of anesthesiology,
M J Coppes, and H Sohl, and I E Teshima, and A Mutirangura, and D H Ledbetter, and R Weksberg
July 2006, Acta paediatrica (Oslo, Norway : 1992),
M J Coppes, and H Sohl, and I E Teshima, and A Mutirangura, and D H Ledbetter, and R Weksberg
June 2007, European journal of paediatric dentistry,
M J Coppes, and H Sohl, and I E Teshima, and A Mutirangura, and D H Ledbetter, and R Weksberg
December 1995, Masui. The Japanese journal of anesthesiology,
M J Coppes, and H Sohl, and I E Teshima, and A Mutirangura, and D H Ledbetter, and R Weksberg
March 2000, Clinical nephrology,
M J Coppes, and H Sohl, and I E Teshima, and A Mutirangura, and D H Ledbetter, and R Weksberg
March 1983, The British journal of surgery,
Copied contents to your clipboard!