Wilson Disease (WD) usually presents in the first decades of life, although rare patients have a later presentation. We report the clinical features, diagnostic evaluation, and outcome with treatment of two septuagenarian siblings evaluated as part of a research trial for treatment of neurological WD. The index case was a 72-year-old woman who suffered progressive neurological disability, then ...

BACKGROUND Wilson's disease (WD) is a genetic disorder which can be controlled fairly well with decupuration therapy. However, symptoms, on rare occasions, can worsen even when WD is being treated. Herein, we report a case involving unusual neurological deterioration during decupuration therapy for WD. CASE PRESENTATION A 28-year-old man was diagnosed with WD 13 years prior to his clinical vi...

Background Wilson disease (WD) is an inherited neurometabolic disorder that results in excessive copper deposition in the liver and the brain, affecting children and young adults. Without treatment the disease is invariably fatal. Though treatments for WD have been available since the 1950s, the disease continues to be associated with considerable morbidity and mortality because of missed diagn...

The API triethylenetetramine dihydrochloride used as an alternative treatment of Wilson's disease is sensitive to water and it exhibits polymorphism. As this may become an issue for the drug formulation, the physical stability has been studied by differential scanning calorimetry, high-pressure thermal analysis, dynamic vapor sorption, and X-ray diffraction as a function of temperature. In addi...

OBJECTIVES Laribacter hongkongensis is a newly discovered bacterium associated with gastroenteritis and found in freshwater fish. Although isolates resistant to tetracycline have been described, their resistance mechanisms have not been studied. PATIENTS AND METHODS We describe the distribution and molecular characterization of tetracycline resistance in 48 L. hongkongensis isolates from huma...

Two new organic hybrid thiogermanates [Ni(dien)2](H2dien)Ge2S6 (1, dien = diethylenetriamine) and [Ni(teta)2]2Ge4S10 (2, teta = triethylenetetramine) were synthesized under solvothermal conditions. 1 contains a [Ni(dien)2] complex cation, a protonated dien molecule and a [Ge2S6]4− dimeric anion, while 2 consists of discrete [Ge4S10]4− adamantane-like cluster anions with [Ni(teta)2] complex cati...

Two tetracycline resistance genes of Streptomyces rimosus, an oxytetracycline producer, were cloned in Streptomyces griseus by using pOA15 as a vector plasmid. Expression of the cloned genes, designated as tetA and tetB was inducible in S. griseus as well as in the donor strain. The tetracycline resistance directed by tetA and tetB was characterized by examining the uptake of tetracycline and i...