Hemoglobin D-Los Angeles [beta 121(GH4)Glu----Gln] in the Province of Liège, Belgium.

Since 1975, more than 19,000 persons have been tested for hemoglobin abnormalities in Liège. Nine unrelated carriers of Hb D-Los Angeles were discovered. They were of Belgian origin. The family of the ninth index case was studied thoroughly. In six generations, 372 descendants of a couple married in 1818 were recorded. Of the 93 tested, 31 were carriers of the Hb D variant. The complete pedigree indicates that Hb D-Los Angeles was already present in Liege province in the 18th century. A connection with India is unlikely.

[Huntington's chorea. The Liège experience]. / Chorée de Huntington. L'expérience Liégeoise.

The hereditary choreas are studied in Liège since twenty-five years. The records of psychiatric hospitals (ruled by the belgian law on confinement, 1850-1873), the archives of the "Etat-Civil" (since 1806) and the parochial registers (XVIIIth century) were tapped. From hundreds of choreic records, unquestionable or dubious, genealogical trees, sometimes stretching along nine generations were built up. About fifty pedigrees gather the patients of four Belgian provinces (out of nine). The oldest and undeniable document was written in 1793. With this wealth in hand, we are able to corroborate or invalidate diagnosis of Huntington's chorea put forward by today psychiatrists and neurologists. The diagnostic blunders were not uncommon and, sometimes, funny. The geneticist comes often too late, post mortem, through archives. When he intervenes in due time, his advice is usually not successful with young adults, uninstructed and in love. The frequency of the disease is estimated around 1 in 10.000 births. The disappearance of old pedigrees and the dawn of new ones can be predicted. Our followers will observe it and, perhaps, be able to measure the frequency of mutation.

[40 years of toxicomania at the psychiatric hospital in Liège]. / Quarante ans de toxicomanie dans un hôpital psychiatrique liégeois.

Drug abuse was observed during 40 years (1939-1979) in a psychiatric hospital for women (sanatorium Sainte-Agathe, Liège, Belgium). Seventy patients were labelled with that diagnosis, -i.e. 2,9% of 2386 admissions. The frequency of drug addiction grew with years and between 1975 and 1979, outnumbered 10% of all admissions. 3/4 of those patients were previously inmates of other psychiatric clinics and well known in medical urgency services. Morphinomaniacs were the only patients between 1939 and 1949 (11 women). As a rule, they survived and did not come back. No haschich, L.S.D. or heroin addicts were seen: their psychiatric confinement was never required. Beginning in december 1959, barbituromaniacs (58 patients) invaded the wards. Other hypnotics were used as well (e.g. metaqualone). Forty-five per cent of the patients died and the survivors came back and back again, -en masse. The illness is very serious if it starts before 30 years: the risk of death goes to 75%. Barmaids and prostitutes were definitely doing hazardous jobs (5 deaths among 6 cases). Other psychiatric illnesses interfere with toxicomania, especially depression. Four patients committed suicide without hypnotics. To conclude: barbiturates are dangerous drugs and they ought not to be used for insomnia. Benzodiazepine abuse did not occur in the hospital and did not induce fatal issues. Combined with phenothiazines, benzodiazepines can solve nearly all sleep disturbances.

[Porphyria variegata: a study of a large family (author's transl)]. / Porphyria variegata: étude d'une grande famille.

Porphyria variegata (or South-African porphyria) is not a rare disease in Europe. Its transmission, in a Walloon family, is described. The pedigree extends over 7 generations and comprises 184 individuals. It was possible to carry out biological studies on 100 family members. The results of the analyses are presented and discussed. The levels of fecal porphyrines, the presence of cutaneous lesions and the genealogical relationships permitted the establishment of a coherent picture. Three other families suffering from the same disease are briefly reported. A systematic search for carriers of the gene is indispensable in porphyrias. All identified carriers must be warned of the risks they run and must be supplied with a list of medicaments to be avoided. The prevention of cutaneous lesions by beta-carotene is envisaged.

[A difficult genetic counsel and an unexpected form of porphyria]. / Un conseil génétique difficile et une variété inattendue de porphyrie

PIP: The case of a young woman who died of porphyria lead the authors to study this disease. 3 varieties of porphyria have been identified: Swedish porphyria, South African porphyria or porphyria variegata, and coproporphyria. Acute attacks of porphyria are lethal 30 to 40% of the time. It is a dominant autosomic disease which one of the parents and 50% of the children get. An examination of the whole family is necessary in order to discover the latent carriers of the pathological gene. The carriers must be informed of the risk and of the medicines which they must avoid. The search of potential subjects is more efficient after puberty. To identify Swedish porphyria, a dosage of the urosynthetasis of the red globules must be done. Latent South African porphyria may or may not be accompanied with cutaneous disorders. When exploring porphyria, one must check not only the urine, but also the stools.^ieng