Anticipation in familial plasma cell dyscrasias

Familial myeloma was described as early as 1925; however, the causative factors are unknown. Studies of families with other familial haematological malignancies demonstrate anticipation. Five new families are described in which plasma cell dyscrasias occurred in parent and child generations (six such pairs), and data were pooled with those of 16 other families (with 20 parent–child pairs affected) recorded in the literature. Disease-free survival for parent and child generations were each estimated and differences in the disease-free survival between generations were tested by the log-rank and signed rank methods. In all six previously unreported parent–child pairs with plasma cell dyscrasia and in 18/20 such pairs found in the literature, the disease occurred at an earlier age in the child generation. The median age of onset of myeloma in the parent and child generations of all 26 pairs was 71 years (95% CI 67–78 years) and 50 years (95% CI 45–55 years), respectively ( P  < 0.0001). The ages of onset of malignant plasma cell dyscrasias in the parent and child generations of these families compared with patients in the general population were significantly different for the child generation ( P  < 0.005) but not for the parent generation. It would appear that anticipation occurs in familial myeloma.     

Muscle strength and fiber typing

The authors discuss fiber typing and morphologic changes resulting from training and how these affect athletic performance. Also considered is the concept of strength, its measurement and development, and how strength affects athletic performance, activity, and injury.     

Liver replacement with reduced size donor organs

End stage liver disease in children can be treated with orthotopic liver transplantation (OLT). Nevertheless, the expansion of this therapy in Europe has been limited because of the shortage of appropriate size-matched donors. One possible technical solution is the OLT of a liver graft previously reduced in size by in situ resection preceding the harvesting procedure. To study the impact of this technique we examined two different operative procedures performed on Landrace pigs. Group 1 consisted of 20 standard donor/recipient weight matched OLT. In group 2, 15 OLT were performed using right lateral and medial lobes (55% of the original donor liver). The donor/recipient weight ratio in the group was 2:1. Cold ischemia times were 90 +/- 16 min for group 1 and 98 +/- 9 min for group 2. It is emphasized that in contrast to all the other reports using resected liver grafts for OLT, the donor resection in our study was always performed in situ under normothermic conditions, preceding the harvesting procedure. This was designed to reduce the cold ischemia time. No significant technical problems were encountered. The biochemical results of group 2 pigs compared to group 1 demonstrate an analogous, postoperative course. This might be explained by regenerative stimuli acting on the resected liver tissues and enhancing their metabolic function. These data support the conclusion that resected adult donor liver grafts may be used for pediatric transplant recipients.