Clinical presentation and treatment of Wilson's disease: a single-centre experience.

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.

Personality traits in treated Wilson's disease determined by means of the Karolinska Scales of Personality (KSP).

OBJECTIVE: The aim was to elucidate the personality traits of patients with treated Wilsons disease (WD) in comparison to healthy volunteers. METHOD: 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. RESULTS: 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. CONCLUSION: 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.

Evaluation of penicillic acid for toxicity in broiler chickens.

Penicillic acid is a mycotoxin produced by various fungi. It may occur in high concentrations in corn and can also be produced concomitantly with other mycotoxins in poultry feed. This mycotoxin was evaluated for its toxicity in broiler chickens by feeding graded concentrations (0, 100, 200, and 400 microgram/g of diet) to 4 groups of 10 birds per treatment. No significant (P greater than .05) effects were measured on growth rate, feed conversion, relative size of pancreas, spleen, liver, heart, bursa, or kidney or on hemoglobin, packed cell volume, liver lipid, plasma protein, or glucose. The only significant effects were a slight reduction in the size of the proventriculus and gizzard at dose levels of 200 and 400 microgram/g. Neither the salt nor lactone forms of penicillic acid had any detectable effect. The acute oral LD50 for the sodium salt form was 92 +/- 9 mg/kg. These data suggest that penicillic acid by itself has little toxicity (less than 1% of that of aflatoxin) in chickens.

[Post-traumatic extrahepatic arterioportal fistula. A case with a spontaneously favourable course]. / Fistule artério-portale extra-hépatique, post-traumatique. Un nouveau cas d'évolution spontanément favorable.

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.

[Carcinogenic fungi (author's transl)]. / I funghi tumorigeni.

An association of carcinogenicity of toxic substances with fungi is shortly documented and discussed. The actions of the aflatoxin B1 (AFB1) are particularly examined; but it is emphasized that other carcinogenic mycotoxins are more frequent and therefore more dangerous. The man is unable to live estranged from them, because they are a great part in ecological systems and in the work-place, resulting a potentially major threat. The range of food and feedstuffs potentially affected has been increased in recent years. In effect contamination is liable to occur during transport, storage and food processing. These studies demand organized cooperation among mycologists, chemists, pathologists, pharmacologists, physicians. In Japan such extensive collaboration has a long history.

Antibody reactivity in penicillin-sensitive patients determinated with different penicillin derivatives.

35 individuals showing reactions to penicillin of anaphylactic shock, angioedema or urticaria were investigated. Their skin sensitivity was analysed using 16 different penicillin derivatives. In addition, the content of circulating reagins against the penicilloyl structure in the patient's sera were analysed using RAST. 17 of the patients had negative skin reactions and RAST results to all substances tested. The other 18 were skin test-positive to at least one derivative but showed markedly heterogeneous patterns of skin reactivity. 14 had positive reactions against penicilloyl structures accompanied by anti-penicilloyl reagins. Four patients showed doubtful reactions only to penicillin or penicilloate and/or penilloate. These patients also had very low levels of reagins against penicilloyl in their sera. Positive skin test results using monovalent penicillin derivatives such as penicillin, penicilloate, penilloate, penicilloyl amide, penicilloyl-formyl-lysine, penicillamine, which cannot form a multivalent antigen with penicillyol specificity, indicated formation of other derivatives of importance in penicillin allergy, e.g., penicillamine protein conjugates. Three patients showed skin reactions to ampicillin polymer and two to benzyl-penicillin polymer. The skin tests performed with the penicillin derivatives used do not seem to give more information on the sensitivity of the patients than does the RAST using penicilloyl structures.