Hereditary quadriceps myopathy

A familial myopathy affecting a man, his three daughters, and one of his brothers is reported. The quadriceps muscle was predominantly involved, with aching pain as an early feature, and later prominent areas of hypertrophy projecting from patches of atrophy gave the quadriceps a most striking and unusual appearance. Presentation was in adult life, and the course was relatively benign, pelvic girdle and hand muscles becoming involved later. The evidence suggests a hereditary selective muscular dystrophy rather than polymyositis, although a hereditary form of spinal muscular atrophy could not be excluded entirely.     

Coexistent multiple myeloma and primary hyperparathyroidism

A patient with multiple myeloma and hypercalcemia responded to cytotoxic chemotherapy. However, hypercalcemia persisted. Because of the absence of lytic bone lesions, the presence of a low serum phosphate level, and a family history of possible primary hyperparathyroidism, the patient was evaluated for this disorder. Serum parathyroid hormone and urinary cyclic adenosine monophosphate levels were elevated. Exploration of the neck disclosed two enlarged parathyroid glands (1,850 mg and 210 mg), which were excised. After surgery, the patient's serum calcium levels remained normal for one year. Progressive myeloma bone disease developed that eventually resulted in recurrent hypercalcemia and death. Autopsy revealed only evidence of myeloma.     

The metabolism and disposition of tris(1,3-dichloro-2-propyl) phosphate (fyrol FR-2) in the rat

Fyrol FR-2, a flame retardant, was readily absorbed from the skin and gastrointestinal tract and rapidly distributed throughout the body, with the highest concentration being observed in kidney, liver, and lung. The route of administration had little effect on Fyrol FR-2 distribution. Its absorption and distribution from the gastrointestinal tract was unaffected over a dose range of two orders of magnitude. Fyrol FR-2 was subject to rapid and extensive metabolic degradation. Within the first 24 hr, greater than 80% of the radio-activity was excreted in urine, feces, or expired as CO₂. The major metabolite eliminated in the urine was bis(1,3-dichloro-2-propyl) phosphate. Fyrol FR-2 was rapidly metabolized in vitro by a NADPH-dependent microsomal mixed-function oxidase system as well as a glutathione dependent transferase system present in the soluble fraction. The microsomal enzymes metabolized Fyrol Fr-2 to 1,3-dichloro-2-propanol, 3-chloro-1,2-propanediol, and bis(1,3-dichloro-2-propyl) phosphate as well as at least one unidentified metabolite. The soluble fraction metabolized Fyrol FR-2 to one metabolite which is probably a glutathione conjugate formed with the intact Fyrol FR-2 molecule.     

Comparative anti-herpesvirus activities of 9-(1,3-dihydroxy-2-propoxymethyl)guanine, acyclovir, and two 2'-fluoropyrimidine nucleosides

9-(1,3-Dihydroxy-2-propoxymethyl)guanine (DHPG), was evaluated in cell culture and in animals for its inhibitory effect on herpes simplex viruses. Compounds run for comparison included acyclovir, 2'-fluoro-2'-deoxy-5-iodo-arabinofuranosylcytosine (FIAC), and 2'-fluoro-2'-deoxy-5-methyl-arabinofuranosyluracil (FMAU). In plaque reduction assays DHPG, acyclovir, FIAC, and FMAU were inhibitory to six herpes types 1 and 2 virus strains at concentrations of 0.2-2.4 microM. These concentrations were much lower than those required to inhibit Vero cell proliferation. In guinea pig vaginal infections, DHPG provided significantly greater inhibition of herpetic lesions than did acyclovir. In a herpes type 2 infection model in mice, DHPG, and FMAU were active at 5 mg/kg, whereas acyclovir and FIAC showed no statistically significant effect at 80 mg/kg. In a herpes type 1 encephalitis model, DHPG and FMAU were active at doses less than 10 mg/kg, with FMAU being about 4 times more potent than DHPG in that model.