RESUMEN
The side-on end-on dinitrogen hydride complex [{Na(dme)}2{(O3)Nb}2(µ-η1:η2-N2)(µ-H)2] (3-Na, [O3]3- = [(3,5-tBu2-2-O-C6H2)3CH]3-) was observed to undergo facile elimination of H2 and cleavage of the N-N bond in the presence of 9-borabicyclo[3.3.1]nonane (9-BBN), AlMe3, and ZnMe2. Treatment of 3-Na with 9-BBN and ZnMe2 afforded the nitride complex [{K(dme)2}2{(O3)Nb}2(µ-N)2] (2-Na). The reaction of 3-Na with AlMe3 afforded [{Na(dme)}2{(O3)AlMe}2(NbMe2)2(µ-N)2] (5). The nitride complex 2-Na was treated with 9-BBN and AlMe3 to form [{Na(dme)}2{(O3)Nb}(µ-NH)(µ-NBC8H14){Nb(O3C)}] (4) and 5, respectively. Complex 2-Na, 4, and 5 were structurally characterized.
RESUMEN
Li-Fraumeni syndrome (LFS) is a rare inherited disease characterized by a high and early-onset cancer risk. A cancer surveillance program is important to reduce cancer-related morbidity and mortality in individuals with LFS. We report 2 pediatric cases with LFS-related malignancy in a family. Eight-year-old elder brother was diagnosed with adrenocortical carcinoma and was found to have a heterozygous missense germline mutation c.736A>G: p.Met246Val in the TP53 gene. Cancer screening led to the diagnosis of rhabdomyosarcoma at a curable stage in his 2-year-old younger brother. Comprehensive surveillance resulted in early tumor detection and improved survival.