Glossary
Last modification: 2024/01/15 https://kinderkrebsinfo.de/doi/e8939
This is a glossary of a number of special words and medical terms used by this information service.
WAGR syndrome
syn. chromosome 11p syndrome, WT1-associated syndrome
abbreviation for a clinical condition associated with a predisposition to Wilms tumours, complete or partial absence of the iris of the eye (aniridia), malformations of the urinary and genital organs (genitourinary system) and mental retardation; there is also an increased risk of developing kidney disease (e.g. renal insufficiency) with chronic renal failure. It usually manifests itself between the first and fourth year of life and is due to the loss of a piece of chromosome 11. This activates the so-called Wilms tumour gene WT1.
Example / Relevance Pediatrics: children and adolescents with WAGR syndrome have an approximately 30-60% increased risk of developing a Wilms tumour (nephroblastoma).
Werner`s syndrome
syn. Rabbiosi syndrome, Progeria adultorum
very rare, genetic disease associated with premature aging and an increased risk of cancer; the first symptoms appear in early puberty (short stature; weak, high-pitched voice), the full manifestation of the disease presets around the age of 30 years. Symptoms include, for example, characteristic skin changes, whitish and sparse scalp hair, bilateral cataracts, type II diabetes mellitus, arteriosclerosis, muscle breakdown and osteoporosis. In addition, patients with Werner syndrome have an increased risk of malignant tumours. The disease is inherited in an autosomal recessive manner and is one of the cancer predisposition syndromes.
Example / Relevance Pediatrics: cancers associated with Werner syndrome are mainly sarcomas, for example soft tissue sarcomas.
WHO
abbreviation for World Health Organization; international federation for cooperation in the field of public health
WHO classification
international guideline developed by the World Health Organization (WHO) for classification, diagnosis and differentiation/grading of (malignant) diseases
Wilms tumour
syn. Nephroblastoma
embryonic, malignant solid tumour of the kidney, most common in children between 1 and 5 years of age, especially in the presence of various syndromes or congenital abnormalities; Wilms tumour accounts for 6% of all malignancies in childhood and adolescence.
Wilms tumour gene
genes that play a decisive role in the development of the Wilms tumour (as a result of tumour-specific changes in certain chromosomes); so far, despite indications of the existence of various Wilms tumour genes, only the Wilms tumour suppressor gene WT1 on chromosome 11 (gene locus 11p13) has been identified and decoded.
Wiskott-Aldrich syndrome
congenital disease with coagulation disorder and immunodeficiency; typical features include skin bleeding, increased susceptibility to infections, eczema-like skin lesions and a tendency to allergic reactions as well as an increased risk of cancer. Wiskott-Aldrich syndrome is, therefore, also one of the cancer predisposition syndromes (CPS).
Example / Relevance Pediatrics: children and adolescents with this disease have a significantly increased risk of developing lymphoma (Hodgkin lymphoma; Non-Hodgkin lymphoma).