The history of a 38-year-old, asymptomatic man with primary sclerosing cholangitis is reported. The diagnosis has been suggested by eosinophilia (66%, 27 X 10(9)/l leucocytes) and markedly elevated serum alkaline phosphatase levels (1860 U/l) and has been established by endoscopic retrograde cholangiography. Report of a therapeutic approach with D-Penicillamin. Documentation ...

Scleroderma in a two-year-old boy with gross enlargement of the right inguinal lymph nodes, as an early sign, is reported. Repeated lymph node biopsies revealed non-characteristic reactive changes and hyperplasia, but eventually histological examination of skin and muscle from the right leg were diagnostic. The effect of one year of ...

The effect of pregnancy has been studied in 10 mothers with Wilson's disease. Three were presymptomatic but had the typical biochemical lesion, two of these were receiving penicillamine treatment at the time of conception, the third had yet to be diagnosed. The remaining seven mothers had had symptoms of Wilson's ...

1. (+)-Penicillamine in a dose of 193 mumoles/kg given subcutaneously twice a day on the sixth and seventh days after the administration of 100 mug mercury increased the urinary excretion of rats more than the equimolar dose of N-acetyl-(+)-penicillamine but less than 2,3-dimercaptopropanol 48.3 mumoles/kg.2. Sodium maleate in a dose ...

The antiarthritic activity of a novel thiazole derivative, SM-8849, was compared with that of indomethacin and D-penicillamine, in mice with collagen-induced arthritis. SM-8849 reduced the incidence and severity of disease in collagen-immunized mice, as assessed by clinical observation. This efficacy was also confirmed by radiographic and histologic studies. Indomethacin produced ...

Therapy of acute heavy metal poisoning is currently limited to a group of moderately toxic drugs containing sulfhydryl groups. N-Acetylcysteine (NAC) was used in these studies to determine if this sulfhydryl containing amino acid would reduce the overall mortality of a group of heavy metal compounds. D-Penicillamine and dimercaprol (BAL) ...

Wilson's disease associated with hepatic failure is not common and the underlying mechanism triggering the event is not known at present. We treated a 28-year-old Japanese woman with Wilson's disease who developed hepatic failure associated with hemolytic crisis just after delivery. She was diagnosed as having Wilson's disease at 12 ...

Drug-induced lung disease presents several diagnostic and therapeutic problems to the clinician. This is especially true in the case of lung disease associated with antirheumatic agents in which pulmonary disease may be due to the underlying disorder. Unfortunately, no specific markers exist to differentiate drug-induced lung disease from other pathologic ...

The relative efficacy of 14 chelating agents in alleviating acute nickel(II) acetate (ip) intoxication has been determined. The LD50 for ip nickel(II) acetate in mice was found to be 45.7 mg/kg with a 95% confidence limit of 39.2--53.3 mg/kg. For a level of 62 mg/kg ip of nickel(II) acetate (i.e. ...

We describe here two patients with rheumatoid arthritis who developed nephrotic syndrome after administration of bucillamine, a novel antirheumatic drug developed in Japan. The nephrotic syndrome occurred after six months' and five months' treatment of bucillamine, respectively. The renal biopsy showed early phase of membranous glomerulonephritis (stage 1) in both ...

Chelating agent, such as CaEDTA, CaDTPA, D-penicillamine, DMSA, desferoxamine, NTA, cysteine ethyl ester, BAL, alpha-MPG, phthalein complexone, were tested as a possible contrast enhancing agent for tumor imaging with 67Ga-citrate. The intravenous administration of a chelating agent to Ehrlich's tumor bearing mice, one hour after the injection of 67Ga-citrate, accelerated ...

A 77-year-old patient is described who developed pemphigus vulgaris temporally with the administration of cefadroxil. The disease improved when the drug was discontinued but was exacerbated with the administration of ampicillin. This may be the first case of possible cefadroxil-induced and only the second case of ampicillin-induced pemphigus vulgaris reported. ...

The effect of 'second-line' drugs on radiological progression in rheumatoid arthritis is not clear, and previous studies have yielded contradictory results. Sixty-seven patients with rheumatoid arthritis have been followed up clinically and radiologically for approximately 2 years (26 patients were receiving intramuscular gold, 21 penicillamine, 10 levamisole, and there were ...

In a prospective study 88 patients, with rheumatoid arthritis who had stopped taking gold, penicillamine, or levamisole were randomly allocated to one of the alternative drugs and followed up for a minimum of one year. Concurrent studies of the effects of gold, penicillamine, and levamisole prescribed in 123 patients as ...

Meralluride, mercaptomerin, ethacrynic acid, and penicillamine inhibited urease activity of Proteus mirabilis. The activity of the organic mercurials and ethacrynic acid was markedly inhibited by human and dog urine. Antiurease activity could not be detected in the urine of a human and a dog given meralluride by injection. Urine from ...

We investigated the effect of antiarthritic drugs containing thiol groups, such as D-penicillamine, tiopronin (N-[2-mercaptopropionyl]glycine), sodium aurothiomalate, and aurothioglucose, on the chlorinating activity of myeloperoxidase purified from human leukocytes. Hypochlorite, the reactive product of the reaction catalyzed by myeloperoxidase, was effectively scavenged by these antiarthritic drugs, and in addition, D-penicillamine ...

The role of nitric oxide (NO) in the ocular surface remains unknown. We investigated the conditions leading to an increase of NO generation in tear and the main sources of NO in ocular surface tissue. We evaluated the dual action (cell survival or cell death) of NO depending on its ...

Diagnosis of Wilson's disease with hepatic presentation in childhood using clinical and common laboratory parameters is still challenging and is often missed or delayed. The aim of the study was to document the clinical and laboratory parameters of hepatic presentation of Wilson's disease in children. The study was conducted at ...

A comparison in mice has been made of the effectiveness of five chelating agents used clinically for acute mercuric chloride poisoning, or recommended for such use. The compounds examined were N-Acetyl-D,L-penicillamine (NAPA), D-penicillamine (DPA), 2,3-dimercaptosuccinic acid (DMSA), sodium 2,3-dimercaptopropanesulfonate (DMPS), and 2,3-dimercaptopropanol-1 (BAL). The test of effectiveness was their ability ...

OBJECTIVES: The response of serum transaminase levels to penicillamine and zinc treatment in Wilson's disease is poorly understood. The aim of this multicenter retrospective study was to evaluate transaminase levels after penicillamine and zinc treatment in children with Wilson's disease. PATIENTS AND METHODS: One hundred and nine patients with Wilson's ...

Spontaneous volume-induced contractions of the urinary bladder were recorded isometrically in urethane-anesthetized rats. Contractions were inhibited by alternate submaximal but equieffective doses of the selective mu and delta-opioid ligands [D-Ala2-Me-Phe4,Gly(ol)5] enkephaline (DAGO) and [2-D-penicillamine, 5-D-penicillamine] enkephalin (DPDPE), respectively, administered by the intracerebroventricular (i.c.v.) or spinal intrathecal (i.t.) route. Naloxonazine, postulated ...

Peroxynitrite-induced relaxation of isolated vessels may involve the formation of S-nitrosothiols. This study characterized the hemodynamic effects of systemically injected peroxynitrite in penotobarbital sodium-anesthetized rats and determined whether these effects were due to the formation of S-nitrosothiols. We utilized L-penicillamine, which attenuates the hemodynamic effects of systemically injected S-nitrosothiols. The ...

BACKGROUND: To study the clinical features of Wilson's disease in childhood. METHODS: Retrospective review of the clinical, laboratory, and histologic features and prognosis of Wilson's disease in 26 Spanish children. RESULTS: The first medical visit, at age 9.8+/-3.4 years (range, 4-16 years), was prompted by liver dysfunction detected accidentally (61%), ...

Wilson's disease is an autosomal recessive disorder of copper metabolism. Since the introduction of penicillamine treatment successful pregnancies have been reported. However little is known about the risks of breast feeding in patients on this medication. We describe the case of a patient suffering from Wilson's disease, who had two ...

One hundred sixty-two consecutive patients with rheumatoid arthritis (RA) were studied for possible association between HLA antigens, particularly DR antigens, and disease characteristics and adverse reactions to gold or D-penicillamine treatment. The frequency of HLA-DR4 was significantly increased: 62% in RA compared to 23% in controls. An association of HLA-DR4 ...

To compare the sensitivity of different mammalian cell types towards the cytotoxic action of nitric oxide, freshly isolated rat pancreatic islet cells, hepatocytes, resident and activated macrophages, cultured aortic endothelial cells and two murine tumor cell lines were tested for susceptibility towards exogenous nitric oxide. As sources for nitric oxide ...

In the present collaborative study, popliteal lymph node (PLN) responses to penicillin G (an allergenic chemical), D-penicillamine (an autoimmunity-inducing chemical), and barbital (a negative reference chemical) were investigated in three different mouse strains by ten pharmaceutical companies. Two inbred mouse strains (BALB/c and A/J) and one outbred strain (ICR) were ...

The ability of the antirheumatic drugs D-penicillamine, chloroquine and levamisole to modify macrophage-mediated inhibition of tumour cell growth in vitro was investigated. Increasing numbers of rat peritoneal macrophages were cocultured with a fixed number of ascites hepatoma AH-13 rat tumour cells. Tumour cell growth was assessed as the uptake of ...

Wilson's disease is an inherited copper toxicosis caused by defective putative copper transporting ATPase in the liver. Because of impaired biliary secretion, copper remains in the liver, resulting in chronic hepatic lesions including fatty metamorphosis, chronic hepatitis and cirrhosis. In the latter stage, extrapyramidal syndromes may develop with and without ...

Children with fully recessive (Type I/I) cystinuria have a high risk of stone formation in the first decade of life. To assess the tendency for cystine to precipitate in individual urine samples, we developed an in vitro assay in which radiolabelled cystine (4mM) was dissolved in urine at 37 degrees ...

Wilson's disease is an autosomal recessive disorder related to the copper metabolism. The clinical symptoms are due to copper deposition in various tissues, including liver, brain, kidney, cornea and others. The key strategy of treatment is to reduce the amount of copper in the liver and other tissues by administering ...

There are certain areas of uncertainty regarding the best therapeutic approach in patients diagnosed with Wilson Disease (WD). Our aim was to assess treatment response to different therapies in a cohort of WD patients followed in a single center. This is an observational, descriptive study in which clinical, laboratory and ...

On the basis of biochemical indices the anthors assessed the efficacy of a 6-month treatment with D-penicillin in 49 patients with ankylosing arthritis of the spine who did not respond to nonsteroid antiinflammatory drugs. Improvement in the movements of the spine was observed in patients with less marked roentgenological changes.

A comparison of two series of patients with rhe,matic polyarthritis, the one (n = 140) treated with dosage equal to or higher than 900 mg, the other (n = 60) with dosage between 600 and 750 mg, did not reveal significant difference in clinical and biological efficacy. The number of ...

This paper describes the regressive course over one year of hypervascular nodules in a patient with Wilson's disease. CT revealed multiple, enhancing nodules (up to 3 cm in diameter) detected in the liver in the early arterial phase after the administration of intravenous contrast material. Most of these nodules became ...

L 929 mouse fibroblasts were cultured as monolayers. Proliferation and glycosaminoglycan content in cells and medium were measured, the different glycosaminoglycans fractionated. It was shown that logarithmic cell growth is followed by a stationary phase. Glycosaminoglycan synthesis started later than did cell growth and continued during stationary phase. The glycosaminoglycan ...

Acantholysis is considered the initial and the main pathogenetic event of pemphigus. The first step in drug-induced acantholysis (biochemical and/or immunological) involves binding of the drug to the cell membrane and the formation of 'drug-cysteine' instead of 'cysteine-cysteine' bondings. We suggest that the reaction of D-penicillamine with cystine disulfides that ...

We determined whether pretreatment with (1) the μ-/δ-opioid receptor (μ-/δ-OR) antagonist, naloxone, (2) the δ1,2-OR antagonist, naltrindole, or (3) the peroxynitrite scavenger, D-penicillamine, affects the development of tolerance to the ventilatory depressant effects of morphine in rats. The injection of morphine in vehicle-pretreated rats decreased minute ventilation predominantly via decreases ...

Myasthenia gravis is uncommon in patients with scleroderma, and when diagnosed is usually associated with previous use of d-penicillamine. Clinically, both myasthenia and scleroderma may present with fatigue, weakness and bulbar symptoms, so one of diagnoses may be delayed. We report two new cases and review clinical features of 12 ...

N-Acetyl-D-(-)-penicillamine as a stable second-generation biospecific affinity ligand has previously been suggested for purification of Bacillus cereus 569/H beta-lactamase I. A complex spacer arm is coupled with the matrix by using epichlorohydrin and phloroglucinol doubly activated with divinyl sulphone in the meta position. Coupling of D-(-)-penicillamine ligand resulted in an ...

In the report by H. Vasken Aposhian on "Protection by d-penicillamine against the lethal effects of mercuric chloride" [Science 128, 93 (11 July 1958)], the heading of the last column of Table 1 should have been "Percent," and not "Survival (%)." Mortality, and not survival, is given in this column.

Ethylcysteine showed a prophylactic effect on collagen-induced arthritis in rats at 100 mg/kg, p.o., and the effect continued even after stopping the administration. However, it was not dose-dependent. D-penicillamine showed no effect under the same condition. Ethylcysteine tended to inhibit collagen-induced arthritis when it was administered therapeutically at 300 mg/kg, ...

Over a 5-year period 325 patients with rheumatoid arthritis received treatment with gold (162) or D-penicillamine (163). Thirty-eight (11.7%) developed proteinuria, which persisted in 10 patients; renal biopsies in these 10 patients revealed 6 cases of amyloid disease. These 6 cases illustrate the importance of investigation of rheumatoid patients developing ...

An anion-exchange high-pressure liquid chromatographic system capable of separating penicillin G potassium from five of its degradation products was developed. The retention times were: penicillin G potassium, 17.5 min; DL-penicillamine, 4.5 min; benzylpenilloic acid, 7.0 and 8.0 min; benzylpenamaldic acid, 13.0 min; benzylpenicilloic acid, 19.5 min; and benzylpenillic acid, 22.0 ...

From chromosome breaks observed in lymphocyte culture of patients with auto-immune diseases, the authors study the factors favouring the onset of these breaks, (clastogenic factor and superoxide radicle) and those which appear able to reduce them (D-penicillamine and dismutase superoxide). The therapeutic implications of these findings will be the object ...