penicillic-acid and penicillamide

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 other reported cases of co-existing scleroderma and myasthenia gravis, unrelated to previous d-penicillamine therapy. Co-occurrence of myasthenia and scleroderma was reported almost exclusively (13/14) in women with a mean latency of 7.03 years. Most patients (10/11) had seropositive generalized myasthenia, and there were no cases with exclusively ocular symptoms. Three patients with pre-existing myasthenia were safely treated with d-penicillamine. Myasthenia and scleroderma occur in the context of an underlying autoimmune diathesis, but their co-occurrence could be underreported as the recognition of either disorder may be delayed by overlapping clinical symptoms. Our findings also suggest that d-penicillamine may be cautiously used in selected patients with pre-existing scleroderma and myasthenia, when potential benefits outweigh the risk of possible myasthenia exacerbation.

Topics: Adult; Antibodies, Antinuclear; Autoantibodies; Comorbidity; Follow-Up Studies; Hashimoto Disease; Humans; Middle Aged; Myasthenia Gravis; Neurologic Examination; Penicillic Acid; Raynaud Disease; Scleroderma, Systemic; Sjogren's Syndrome; Thymectomy; Thymus Hyperplasia

In this paper we present the long-term follow-up of two patients, after injection of metallic mercury. Case 1. In 1997, 29-years-old man injected himself to left elbow about 20 ml of metallic mercury by mistake (he was heroin abuser for short time). Mercury concentration in the blood was 400 microg/L. X-ray of the chest, abdomen and affected elbow area showed radiopaque foreign material (depots of mercury). Depots of mercury were also visible on the tricuspid valve in echocardiography. Mercury from the soft tissue left elbow pit was partially surgically removed. During 15 years follow-up two times chelating therapy was performed with d-penicyllamine and DMPS. In 2012, he was admitted to hospital next time. The blood and urine mercury concentration was still elevated (55.2 microg/L and 197 microg/L), mercury depots in the lung and abdomen were present. The signs and symptoms of CNS damage, like peripheral polyneuropathy and ataxia, were diagnosed. CT of brain did not revealed any changes, despite head trauma before 6 years. However neurological findings are typical for chronic mercury poisoning, it is not possible to determine whether these changes are directly related to mercury, because head trauma history, Case 2. In 2003, 16-years-old woman injected herself one month before, in suicidal attempts to both elbows several millilitres of metallic mercury. Mercury concentration in the blood was 56.2 microg/L, in urine 906 microg/L and in the hair 1.12 microg/g. Chest Xray showed depots of mercury in the lung. Mercury from the soft tissue was two times surgically removed. During 9 years two times chelating therapy was performed with d-penicyllamine and DMPS. After 9 years there is no symptoms of mercury poisoning. Mercury depots in the lung are still present. The blood and urine mercury concentration is low (13.7 microg/L and 2.53 microg/L). In mean time she gave birth two healthy children. Further patients evaluation is necessary.

Topics: Adolescent; Adult; Chelating Agents; Female; Follow-Up Studies; Humans; Injections, Intravenous; Lung; Male; Mercury; Mercury Poisoning; Penicillic Acid; Pregnancy; Pregnancy Outcome; Suicide, Attempted; Treatment Outcome; Unithiol; Young Adult

Thirty patients with Wilson's disease (WD) were observed at a movement disorder clinic between 1970 and 2000. Disease onset was at the mean age (SD) of 14.5 (+/-5.9) years. Presentation with hepatic disease occurred in 12 of 30 patients and with neurologic disease in 15. Three patients were asymptomatic at the time of diagnosis. The mean (SD) delay to diagnosis was 5.9 (+/-5.7) years. Five patients diagnosed in an advanced stage of disease died before initiating treatment. Eighteen patients were followed and treated with D-penicillamine alone or in combination with zinc sulphate. Treatment improved most of neurological symptoms. Dystonic postures, behavioural disturbances and dysarthria were the most resistant neurological signs. 'Pseudo-sclerotic' neurologic involvement predicted a good outcome, whereas hepatic onset and 'classic' neurologic involvement were associated with a poorer prognosis. Two of the 18 treated patients died of hepatic failure due to voluntary discontinuation of therapy. Both D-penicillamine and zinc sulphate were well tolerated. No teratogenic effect of D-penicillamine was observed throughout 5 pregnancies. Our results suggest that D-penicillamine or a combination of D-penicillamine and zinc sulphate is a safe and effective long-term treatment in patients with WD.

Topics: Adolescent; Adult; Brain; Child; Child, Preschool; Drug Therapy, Combination; Dysarthria; Dystonia; Female; Follow-Up Studies; Hepatolenticular Degeneration; Humans; Long-Term Care; Magnetic Resonance Imaging; Male; Mental Disorders; Neurologic Examination; Penicillic Acid; Pregnancy; Retrospective Studies; Survival Rate; Treatment Outcome; Zinc Sulfate

In addition to hepatic and extrapyramidal motor clinical symptoms, Wilson's disease patients also exhibit subclinical disorders of other central nervous pathways. In this study, an impairment profile is described by means of eight electrophysiological tests (EAEP, MSEP, TSEP, T-VEP, MEP, EEG, heart frequency variability, and SSR) for 37 patients (28 with neurological, nine with tnon-neurological form) undergoing long-term drug therapy. The occurrence in 64.3% of a delayed wave III and/or IPL III-V prolongation in patients with the neurological form makes pathological FAEP the most common form of the disorder, followed by disorders in MSEP, TSEP, MEP, and T-VEP. Patients with the non-neurological form usually have normal values, although latency prolongations occur in isolated cases. The range of evoked potential findings is characterised primarily by latency prolongations, i.e. a demyelinising impairment type, and significant losses of potential hardly occur (except in the MEP). The electrophysiological impairment profile does not include EEG changes or vegetative disorders.

Topics: Autonomic Nervous System Diseases; Basal Ganglia Diseases; Cerebral Cortex; Chelating Agents; Diagnosis, Differential; Electric Stimulation; Extrapyramidal Tracts; Galvanic Skin Response; Hepatolenticular Degeneration; Humans; Nervous System Diseases; Neurologic Examination; Penicillic Acid; Peripheral Nerves; Reaction Time; Trientine

The aim was to elucidate the personality traits of patients with treated Wilsons disease (WD) in comparison to healthy volunteers.. Twenty-five WD patients, ten females and 15 males, with a mean age of 35.2 +/- 8.3 years completed the Karolinska Scales of Personality (KSP), a self-report inventory comprising 15 separate scales. The results were compared to a control series comprising 200 men and 200 women drawn from the general population.. The patients with treated WD scored significantly lower than the healthy controls on aggressivity-hostility-related scales and the scale measuring Psychic Anxiety. Patients with predominantly hepatic symptoms had the lowest aggressivity-related scores and patients with predominantly neurological symptoms had the lowest Irritability, Guilt and Detachment scores and the highest Impulsiveness and Muscular Tension scores. Both groups scored low on the Somatic Anxiety scale.. The present results illustrate that patients with treated WD have significant deviations in personality traits, especially in aggressivity-hostility-related scales and Psychic Anxiety, compared to healthy controls when investigated by means of a self-report inventory, the KSP. The deviations were not related to age, age at onset or duration of the disease.

Topics: Adult; Aggression; Arousal; Female; Follow-Up Studies; Hepatolenticular Degeneration; Hostility; Humans; Male; Middle Aged; Penicillic Acid; Personality Inventory; Psychometrics; Reproducibility of Results; Trientine; Zinc Acetate

Several thiol-containing molecules (TCM) are currently used as antidotes for nickel, and vicinal TCM seem to be more effective in mobilizing tissue nickel than are mono TCM. Using single cell alkaline electrophoresis, we have shown that the vicinal TCM, meso-2, 3-dimercaptosuccinic acid (DMSA), 2,3-dimercaptopropane-1-sulfonate, and 2,3-dimercaptopropanol markedly enhanced, whereas the mono TCM, D-penicillamide, glutathione, beta-mercaptoethanol, and diethyl dithiocarbomate, reduced nickel chloride (Ni)-induced DNA breaks in a human leukemia cell line, NB4 cells. Ni or TCM alone did not induce plasmid DNA breaks in test tubes and neither did Ni plus mono TCM; however, Ni plus vicinal TCM did. Vicinal TCM did, but mono TCM did not generate H(2)O(2) in solution. H(2)O(2) alone did not, but H(2)O(2) plus Ni induced plasmid DNA breaks. Although Ni plus glutathione did not break DNA, Ni plus glutathione plus H(2)O(2) did. The Ni-DMSA-induced DNA breaks in NB4 cells, as well as in plasmids, were completely prevented by d-mannitol or partially prevented by several antioxidants. Therefore, the DNA breaks induced by Ni plus vicinal TCM seem to be due to the complex of Ni with TCM in concert with the H(2)O(2) produced by the vicinal TCM. The results that DMSA at a concentration as low as 5 microM enhanced the Ni-induced DNA breaks suggest a further evaluation of the TCM as nickel chelators is needed.

Topics: Cell Line; Comet Assay; Dimercaprol; Ditiocarb; DNA Damage; Dose-Response Relationship, Drug; Drug Synergism; Glutathione; Humans; Mercaptoethanol; Nickel; Oxidation-Reduction; Penicillic Acid; Plasmids; Succimer; Sulfhydryl Compounds

Topics: Adult; Arthritis, Rheumatoid; Caproates; Female; Glomerulonephritis; Humans; Penicillic Acid; Pulmonary Embolism; Radiography; Renal Veins; Thrombosis

The authors describe a case of post-traumatic arterio-portal fistula (APF), discovered on the 12th day after coeliomesenteric arteriography performed because of a postoperative complication, and emphasize the rare nature of this lesion, as shown by a review of the published literature. They stress the value of coeliomesenteric arteriography after urgent laparatomy for hepatic trauma for investigation of any possible lesions.

Topics: Angiography; Arteriovenous Fistula; Caproates; Duodenum; Humans; Liver; Male; Middle Aged; Penicillic Acid; Portal Vein; Stomach; Wounds, Gunshot