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Biomedical subjects

C Piussan

Publications and source records attributed to C Piussan.

At least 73 records · Page 4Linked to original sources
PubMed1985

[Teratogenic risk during treatment of Wilson disease].

Untreated Wilson's disease usually causes infertility or abortion, as a result of increased intrauterine copper level. Therefore, a chelation treatment is necessary during the whole pregnancy. The most used is D-Penicillamine whose teratogenic risks such as cutis laxa, dermatopathy or complex mesenchyme abnormalities are paradoxically rare in the new borns of treated Wilson's disease mothers, perhaps owing to hypercupremia that protects the foetus from excessive copper deficiency. Yet, it's wise to reduce chelation treatment about a quarter fold and to add 50 mg vitamin B6 weekly as we did in our case whose child was born normal.

Adult↗
PubMed1984

[Reactive arthritis in children].

Reactive arthritis is arthritis in which, although the nature of the responsible infection is known or suspected upon serological grounds, attempts at recovering the pathogen from the synovial fluid have failed. One of the main pathogenetic problems is the multiplicity of etiologic agents. Some are exogenous and may be related to the articular tropism of certain microorganisms, to immunologic depression due to an antecedent or coincident infection, and to successive reinfections by the same pathogen or by others which may promote an exacerbation of the disease. Others are endogenous and attention should be given to the local or systemic presence of an antigen as well as, in some instances, to the persistence of residual forms of infecting agents, which are more readily demonstrated with current bacteriological and serological methods. Although reactive arthritis is to be distinguished from septic arthritis, it can no longer be clearly differentiated from the classical post-infectious rheumatism. Once it has been produced, the antigenic stimulation is responsible for an immunologic response which tends to check systemic extension but may also produce tissue damage in the host. Some patients have circulating immune complexes which may bind to the joint, thereby damaging it. In other patients, particularly those who are HLA B27 positive, host-pathogen cross-reactions are demonstrated. Actually, the most frequent pathogenetic sequence seems to be a combination of two or more of these mechanisms, as there are reasons to believe that presence of the pathogen in situ is not required for the persistence of the inflammatory process. Reactive arthritis was first reported in adults following either sexually transmitted urethritis due to chlamydiae, mycoplasma or gonococci, or hepatitis B or an intestinal infection due to Yersinia, Campylobacter, Shigella, Klebsiella or Salmonella. Later, it was described in pediatric patients, particularly in Scandinavia where, for genetic reasons, the HLA B27 group is prevailing. Reactive arthritis seems less frequent in caucasian ethnic groups and above all in Latin Americans among whom HLA B27 carriers are more uncommon; however, it must be pointed out that they have not been as extensively studied and that other etiologic factors may still remain to be discovered. The course and etiology of the different forms of arthritis share certain characteristics which have been determined through a better knowledge of these conditions: onset occurs one or several weeks after a respiratory, urinary or, most often in children, digestive infection. This episode is unremarkable or latent and often overlooked.(ABSTRACT TRUNCATED AT 400 WORDS)

Antigen-Antibody Complex↗
PubMed1984

[Prenatal ultrasonic diagnosis of malformation uropathies: 13 cases].

The systematic prenatal diagnosis of 5 cases of urinary tract malformations out of 2 500 pregnancies in 1982 in a special care maternity, or 2 per 1 000 is compared with the discovery of 10 cases in 28 months in 17 960 foetuses, or 0.55 per 1 000 whereas the overall frequency varies from 0.3 to 5.25 per 1 000 in infants. We studied 13 children whose prenatal abnormalities were 2 unilateral ureterohydronephrosis and 7 bilateral dilatations associated to 2 ureteroceles, 1 multicystic dysplasia and 1 ureteral duplicity. All of these except two were confirmed at birth. The exceptions consisted in one unilateral dilatation and one bilateral ureterohydronephrosis which turned out to be dysplastic kidneys unknown during pregnancy. Three ureteral duplicities were also ignored. The results are the following: Three cases were severe: one therapeutic abortion after 27 weeks, one intra-uterine decompression followed after few days by a preterm delivery and neonatal death and one provocated preterm delivery (36 weeks). All the others underwent decompression during the first hours of their life. Five cases can be considered unsuccessful: 2 abortions, the death of a 22 days old infant with an unilateral multicystic dysplasia and 2 bilateral malformations with renal failure, one of which was associated to an unilateral dysplastic kidney. Reliability of sonography as well as its interest in prognosis and prenatal evaluation of renal function are demonstrated.

Amniotic Fluid↗
PubMed1983

[Regular dominance of thumb ankylosis with mental retardation transmitted over 3 generations].

A young girl 12 old, sent to us for obesity, and coxa-epiphysiolysis showed signs of mental retardation and bilateral thumb ankylosis. The fact that the mother was also affected by both of these signs, led to a more detailed genetic research. The latter revealed that not only the daughter, the mother, but also their own mother and may be, the sister, the grand-mother and the great-aunt of the patient had a retardation, a slight dysmorphia, a type A brachydactylia, signs of obesity and an identical ankylosis of both thumbs. This vertical inheritance, affecting apparently females only, but not associated with a high rate of miscarriage, has, it seems, never been reported. The characteristics of this family are being considered and discussed.

Adult↗
PubMed1983

[Solitary upper incisor, hypopituitarism and monosomy 18p chromosome aberration].

A prepubertal boy with hypopituitarism, mental retardation, dysmorphia and solitary maxillary central incisor is described, karyotypic studies showed deletion of the short arm of chromosome 18 (46, XY, del (18) (p11). It is suggested that caryotypic studies is of interest among the patients with midline defects and/or hypopituitarism.

Child↗
PubMed1983

[Panhypopituitarism in one of 2 monozygotic twins].

Anterior panhypopituitarism has been observed in one of monozygous twin girls. Etiologic investigations were negative and only suggested an hypothetic perinatal insult. Genetic abnormalities could be excluded. Replacement therapy with human growth hormone was strikingly successful, with a remarkable catch-up growth and, however, a velocity of bone age comparable with the velocity of growth.

Age Determination by Skeleton↗