All of the following statements about Ataxia-Telangiectasia are true, except:

The most frequent humoral immunologic abnormality is the selective absence of IgA, which occurs in 50-80% of these patients-Nelson Ataxia-Telangiectasia: (Autosomal Recessive genetic disorder) : Mutation of AT gene (Ataxia telangiectasia gene). Located on Chromosome 11) (11q 22-33). Defect in DNA repair. Increased number of chromosomal breaks. Ataxia Telangiectasia Immunodeficiency Malignancies Endocrinal Progressive cerebellar ataxia typically becomes evident soon after these children begin to walk to progresses until they are confined to a wheel chair, usually by the age of 10-12 yrs. Oculocutaneous Telangiectasia, characteristically present on the bulbar conjunctiva appears by the age of 3-6 years and may also be seen on the face or extremities Immunodeficiency clinically maifests as chronic recurrent & sinopulmonary infections leading to bronchiectasis Most frequent humoral immunological abnormality is selective absence of IgA. IgE and IgG may also be reduced IgM and IgD are normal Defect in DNA repair mechanisms renders cells highly susceptible to radiation induced chromosmal damage and results in malignancies Lymphoreticular malignancy is most common Hodgkin&;s Lymphomas acute Leukemia Insulin Resitance Insulin dependent diabetes mellitus Premature ageing REf: Nelson 20th edition Pgno: 1415