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Biomedical subjects

B Puech

Publications and source records attributed to B Puech.

32 records · Page 2Linked to original sources
PubMed1989

Craniofacial dysmorphism in Mozart's skull.

Mozart's craniofacial dysmorphism shown in his portraits and in the skull held by the Mozarteum in Salzburg (Austria) helps to document the role of pathology in human identification. The specific syndrome is formed by a premature synostosis of the metopic suture (PSMS) in association with an abnormally shaped skull.

Austria↗
PubMed1989

[The course of fundus flavimaculatus in Stargardt disease].

From the study of twelve of Fundus Flavimaculatus and after recording the main characteristics of this disease the authors try to distinguish the "classical juvenile" form of Fundus Flavimaculatus form the "tardy" form of Fundus Flavimaculatus. This last one would affect the adult and have a better prognosis.

Age Factors↗
PubMed1977

[Isotopic criteria for the assessment of the effects of lumbar sympathectomy (author's transl)].

The authors present a work intended to assess the effects of lumbar sympathectomy using radioactive microspheres. These contain 99mTe and are injected into the femoral artery. The distribution of radioactivity is then measured at the lower limb with a gamma camera linked to a computer. Seventeen patients were tested before and after lumbar sympathectomy. It appears from this short series that lumbar sympathectomy causes a redistribution of the radioactive material directed towards the extremities, mainly the feet.

Aged↗
PubMed1975

[Stargardt's disease and fundus flavimaculatus].

From their sixty two personnal observations and a study of literature cases, the authors demonstrate that the ophthalmoscopic fluoroscopic and functionnal aspects of macular lesions are strictly identical in Stargardt disease and in Fundus Flavimaculatus. Their transmission is also identical, generally autosomal and recessive, more rarely dominant. Flavimaculate lesions situated in perimacular or inperipheric area may coexist in the same family, and certainly correspond to variable forms of expressivity of a unique gene. The authors discuss the nosologic problems brought by these two affections and other juvenile macular degenerations. Their conclusions are as follows: The same disease may present three different forms: -- Pure Stargardt disease; -- Stargardt disease with perimacular flavimaculate crown; -- Stargardt disease with peripheric Fundus Flavimaculatus.

Adolescent↗
PubMedDate not supplied

Identification of the cranium of W.A. Mozart.

In 1801 at the cemetery in Vienna, Austria, the skull of W.A. Mozart was exhumed (La Chronique Médicale, 13 (1906) 423), and now it has been examined for identification. The osteometrical and osteological findings correspond with the available data of W.A. Mozart. Superimposition gives evidence that craniofacial distinctiveness of the cranium is consistent with the portrait. Additional individual particularities caused by the premature synostosis of the metopic suture (PSMS) and a bone lesion are described.

Austria↗
PubMedDate not supplied

[Individualization of X-flavimaculated macular dystrophy in hereditary macular dystrophies].

X-shaped macular dystrophy with flavimaculatus flecks is individualized of other heredo-macular dystrophies. This aspect was showed in two families with a retinal pigment epithelial dystrophy characterised by an X-shaped yellowish macular lesion and numerus flavimaculatus retinal flecks. Nine members were variously affected. The condition was bilateral, had a dominant inheritance, started in middle age with a slow-developing macular lesion. Visual functions were often minimally disturbed for two or three decades. Relations with others here-domacular dystrophy are discussed particularly with pattern dystrophy.

Adult↗
PubMedDate not supplied

[Heredity in Stargardt disease and fundus flavimaculatus].

Many features allow to assert that Stargardt disease and fundus flavimaculatus are the one and same disease: clinically and functionally, macular and perimacular lesions present an absolute identity "Pure" fundus flavimaculatus does not exist the two affections can be found in a same pedigree (5 cases) a patient presenting one of these diseases can develop a more complete form (7 cases). The disease is usually and more often inherited as an autosomal recessive than an autosomal dominant fashion (5 cases out of 96 genealogies) with variable expressivity particularly for "flavimacular" lesions. The frequency of the disease is 1/6,670 inhabitants. There are two genes or more in charge of the disease.

Diagnosis, Differential↗
PubMedDate not supplied

[Electroretinographic problems in the diagnosis of diseases of the cones and macula].

From series of clinical cases, the authors expose how ERG is an essential element for the diagnosis of cone dysfunction with or without macular involvement. They show that to obtain an accurate diagnosis, precise proceedings must be chosen, using stimulation technics correctly selected. They study the advantage of focal or wide stimulations, of the level of the intensity of the stimulation and of the different colored filters. They propose a proceeding for adults examination. For young children, they discuss the advantages and limits of a particular proceeding.

Adult↗