1 alpha(OH)D3 (ETALPHA) treatment and receptor studies in 16 patients with chronic and myeloproliferative disorders

10 patients with CLL and 2 with CML were treated with gradually increasing doses of 1 alpha(OH)D3, up to 4 micrograms daily during 6 wk. 3 patients with preleukemia and 1 with myelofibrosis were treated with 2 micrograms daily of 1 alpha(OH)D3 for a prolonged period up to 17 wk. The treatment with 1 alpha (OH)D3 did not result in changes of disease parameters in any of the patients under study. Receptor studies for 1,25(OH)2D3 were performed in 8 CLL patients and revealed only 1 patient with increased specific receptor binding capacity. The maximum tolerable dose of 1 alpha(OH)D3 varied individually, but was in the range of 2-4 micrograms daily.     

Comparison of immunohistochemical staining of a mitochondrial protein, lipoamide dehydrogenase, with Fab'-peroxidase conjugates prepared by maleimide or periodate

IgG-maleimide peroxidase, Fab'-maleimide peroxidase, polymer and monomer types of Fab'-periodate peroxidase were prepared from an antibody against rat lipoamide dehydrogenase, a component of the pyruvate dehydrogenase complex which is located in mitochondria. They were examined for immunohistochemical staining of the rat kidney. Fab'-maleimide peroxidase was the best for staining mitochondrial protein. IgG-maleimide peroxidase and the monomer type of Fab'-periodate peroxidase had the same intensity of staining. The polymer type of Fab'-periodate peroxidase could not stain the lipoamide dehydrogenase.     

Influence of bezafibrate and colestipol on LDL-cholesterol, LDL-apolipoprotein B and HDL-cholesterol in hyperlipoproteinaemia

A significant increase of LDL-apolipoprotein B by 13% and LDL-cholesterol by 19% was observed in a group of 9 patients with hyperlipoproteinaemia Type III after bezafibrate treatment. Additional administration of colestipol caused a significant decrease of both LDL-apolipoprotein B by 18% and LDL-cholesterol by 25%. In 10 patients of hyperlipoproteinaemia Type IIb a significant decrease of both LDL-apolipoprotein B by 28% and LDL-apolipoprotein B by 18% was observed after bezafibrate therapy. When bezafibrate was given together with colestipol a further decrease of both LDL-cholesterol by 17% and LDL-apolipoprotein B by 16% occurred. HDL-cholesterol concentration increased significantly in both groups of hyperlipaemic patients during therapy. This may be the effect of both bezafibrate and colestipol. It is concluded that bile acid resins may effectively prevent the LDL-cholesterol concentration increase observed sometimes after clofibrate analogues.     

Cutaneous nerves in patients with diabetic neuropathy--an electron microscopic study

Since the role of cutaneous nerves in the pathogenesis of cutaneous diseases associated with diabetes mellitus is not well defined, cutaneous nerves in ten patients with severe diabetic neuropathy were electron microscopically investigated as a preliminary study. The specimens were taken from normal-appearing skin of their lower extremities. Cutaneous nerves were seen as axon-Schwann cell complexes in which variously degenerated axons and Schwann cells coexisted with normal ones. The degenerative changes were not, however, specific for diabetes mellitus.     

Juvenile-onset acid maltase deficiency with unusual familial features

From early childhood, two brothers had mild gait difficulties due to acid maltase deficiency (AMD). Biochemical studies of family members were consistent with autosomal recessive inheritance, but the asymptomatic mother had AM activity in the homozygote range, and her parents had decreased AM activity. The asymptomatic mother may be homozygous for the adult-onset variant of AMD. Alternatively, either the mother or the children may be genetic compounds of the childhood and adult forms of AMD.