The transition from pediatric to adult medical services has a greater impact on the care of adolescents or young adults with chronic diseases such as cystinosis. This transition period is a time of psychosocial development and new responsibilities placing these patients at increased risk of non-adherence. This can lead to serious adverse effects such as graft loss and progression of the disease...

J. B. Egan and M. L. Morse (Biochim. Biophys. Acta 97:310, 1965) reported a defined medium which would support the growth of Staphylococcus aureus NCTC8511 for several generations if a peptone medium culture was diluted into it. In our hands, cultures of S. aureus NCTC8511 or S. aureus HS1159 (obtained from R. Z. Korman, Department of Physical Biology, Cornell University, Ithaca, N.Y.) maintain...

Published evidence indicates that cystine-containing proteins have their disulphide bonds reduced during proteolysis in lysosomes. However, the intralysosomal accumulation of cystine in the cells of patients with cystinosis has been seen as evidence that protein cystine residues are not reduced. The data are reconcilable and fully in harmony if it is postulated that cysteine from the cytoplasm ...

The frequency of FMF-related amyloidosis has been decreased by colchicine use over the past few decades. However, the beneficial effect of colchicine may differ in accordance with nephropathic stages. When used in proper doses and with compliance, colchicine is very effective in preclinical and proteinuric stages of FMF-related amyloidosis. Even so, a large number of patients with nephrotic ran...