[Constitutional hyperammonemia with carbamoylphosphate synthetase deficiency. Course treatment during dietetic].
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Biomedical subjects
Publications and source records attributed to L Cathelineau.
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Seventeen children (ages ranging from 2 to 11 years at the onset of the study) were followed for 24.5 +/- 15 months (3 to 47 months) for a laryngeal papillomatosis requiring tracheotomy in 14. In 8 of the children evolution was low with a decrease or disappearance of relapses. On the contrary, 9 children, i.e. more than half the cases, presented with highly crippling disease, with frequent relapses of the laryngeal papillomas, extension of the lesions to the trachea, bronchi or even lungs, and local laryngeal complications (synechiae, sub-glottic stenosis). Medical anti-proliferative treatments had no effect. These children were submitted to iterative excisions. In 4 of them tracheotomy remained necessary after 3 years of evolution and their vital prognosis might become involved, either because of local complications or due to the extension of the lesions.
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