Reduction of serum cholesterol in two patients with homozygous familial hypercholesterolemia by dichloroacetate.

Dichloroacetate is known to reduce plasma cholesterol and triglyceride in patients with Fredrickson Types IIb or IV hyperlipoproteinemia. We now report the effects of chronic, oral dichloroacetate administration (as the sodium salt) in two patients with severe homozygous familial hypercholesterolemia. Dichloroacetate markedly reduced serum total and low density lipoprotein cholesterol levels and lowered the low density lipoprotein to high density lipoprotein cholesterol ratio. One patient developed a polyneuropathy while receiving dichloroacetate which resolved following discontinuation of the drug. Because of its apparent toxicity, dichloroacetate cannot be recommended for chronic oral use. Investigation of the mechanism of its lipid-lowering effect, however, may provide insight into the pathogenesis and treatment of hypercholesterolemic disorders.

Acrodynia: exposure to mercury from fluorescent light bulbs.

Medical attention was sought for a 23-month-old toddler because of anorexia, weight loss, irritability, profuse sweating, peeling and redness of his fingers and toes, and a miliarial rash. The diagnosis was mercury poisoning, and an investigation of his environment disclosed that he had been exposed to mercury from broken fluorescent light bulbs. Acrodynia resulting from fluorescent bulbs has not been previously reported.

Human health effects from accidental release of tetrachlorodibenzo-p-dioxin (TCDD) at Seveso, Italy.

This paper is a progress report of the epidemiologic work carried out under the supervision of the Lombardy Regional Authority during the two years elapsed from the accident in a TCP-producing factory (ICMESA) in Meda (Italy), which resulted in the contamination of several towns of a large, densely populated area called the Brianza di Seveso with a total population of 220,000 inhabitants. A wide follow-up program is in progress in the Seveso area; it includes a clinical screening of the population living in the contaminated area and longitudinal and systematic health control of different groups at risk; a long-term morbidity cohort study has been also undertaken. TCDD exposure following the ICMESA accident resulted in an increased chloracne frequency. Neurologic examinations showed both signs of idiopathic subclinical neurologic damage and cases of clinically detectable idiopathic polyneuropathy in adults. A limited percentage of idiopathic hepatomegaly was reported to be present on clinical investigation; no information, however, is given on the criteria by which the hepatomegaly was investigated. Some alterations were observed in some exposed people in one or more liver tests (mainly transaminases and gamma-GT). So far, immunologic investigations, cytogenetic examination and embryomorphology analysis on cases of therapeutical or spontaneous abortions have not given abnormal results.

Cutaneous manifestations of acrodynia (pink disease).

A 14-month-old girl who presented with multiple systemic complaints was found to have gingivitis, peeling of her palms and soles, and a peculiar acral eruption. A diagnosis of acrodynia, or pink disease, was confirmed by elevated levels of mercury in the urine. The many cutaneous manifestations of this once common disease are discussed.

Nifedipine in the treatment of acrodynia.

A case of acrodynia in an eight-and-a-half-year-old girl is presented whose symptoms--profuse perspiration, swelling, desquamation, pain, itching of the extremities, pinkish color of the nose and cheeks and hypertension--responded sharply to nifedipine therapy.

[Morvan's fibrillary chorea and acrodynic syndrome following mercury treatment]. / Chorée fibrillaire de Morvan et syndrome acrodynique après un traitement mercuriel.

A 31 year-old inhabitant of French Guiana was prescribed mercuric iodide per os for two and a half months. Shortly before the end of the treatment he developed fasciculations in the trunk and particularly the lower limb muscles, distal painful paresthesias with vasomotor disorders, episodes of excessive perspiration and palmoplantar erythema, moderate fluctuating hypertension, progressive loss of weight and irritability with insomnia. Clinical and electrical signs of neuropathy were lacking. The clinical picture was that of Morvan's fibrillary chorea with acrodynia, the conditions of onset strongly suggesting a mercurial intoxication. Blood and particularly urine mercury levels were elevated. Administration of dimercaprol (BAL) considerably increased urinary excretion of mercury and there was progressive improvement and finally recovery after two months of BAL treatment. This case exemplifies the possible co-existence of fibrillary chorea and acrodynia. Whereas in many cases of fibrillary chorea a precise etiology cannot be determined, the affection can be induced by mercury as by gold administration. The fact that cases of fibrillary chorea due to mercury poisoning are rarely reported may be the result of individual patient hypersensitivity or particular metabolic absorption and excretion features of mercury. This case cannot be included within the continuous activity syndrome of muscle fibers described by Isaacs, since muscle contractures were absent and there was associated acrodynia. Moreover, there was no latent polyneuropathy, in spite of the intense fasciculations. It must be concluded, therefore, that in spite of its rarity fibrillary chorea should keep its semiologic autonomy.

Mercury as a health hazard.

Pink disease has virtually disappeared since teething powders were withdrawn. We describe a case in a boy who was exposed to metallic mercury vapour. We discuss the potential health hazard of spilled elemental mercury in the house and the difficulties of removing it from the environment.

[The toxicity of mercury in amalgam dental fillings]. / Toxizität von Quecksilber in Amalgam-Zahnfüllungen.

1. A survey on the literature concerning the toxicity of amalgam is presented. 2. Opinions from various sources are discussed. It is found that there are no conclusive experimental results of clinical importance. Existing results on adults cannot be applied to children, who are more sensitive to amalgam. 3. Research into the toxicity of amalgam in children has begun. Results are expected later in 1992.

[Feer's disease]. / Die Feersche Krankheit.

Two sisters and their brother who had been exposed to mercury vapours from a broken fever thermometer were treated for acrodynia. Their urinary Hg concentrations, as well as the relatively few data that can be found in previous publications, show that the brain of children below the age of 5 years is much more sensitive to chronic mercury intoxication than the cerebrum of adults. Hg concentrations of well below 50 micrograms/l in the urine can be associated with severe symptoms. One of our patients had a mercury concentration of 6.3 micrograms/l. In adults with occupational exposure, urinary mercury concentrations of up to 200 micrograms/l are tolerated and permitted. Acrodynia possibly is not always diagnosed when it is occurring. Normal values for urinary Hg excretion in children under basal conditions and after administration of a chelating agent should be established.