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Biomedical subjects

R Bouvier

Publications and source records attributed to R Bouvier.

186 records · Page 11Linked to original sources
PubMedDate not supplied

[Renal involvement in the Laurence-Moon-Bardet-Biedl syndrome. Apropos of 3 cases].

Three cases of Laurence-Moon-Bardet-Biedl (LMBB) syndrome with renal involvement characterized by tubulo-interstitial lesions are reported. Caliceal deformations were noted on urography. From 55 additional cases found in the literature the main characteristics of the renal disease in LMBB are defined : impairment of renal concentration is present in 36 % of the cases, and urographic abnormalities, mainly dysplasic features and multiple cystic formations on the calices, in 90 %; tubulo interstitial lesions are the most frequent histological finding. The renal involvement lead to chronic renal insufficiency responsible for the death of 60 % of the patients. Renal disease appears therefore as the sixth cardinal features of the LMBB syndrome and makes this syndrome as an entity close to other hereditary disorders with renal abnormalities as Alström syndrome.

Child↗
PubMedDate not supplied

[Pseudotumoral cystitis in the newborn infant and children. Problems of the differential diagnosis with bladder botryosarcoma. Apropos of 3 cases].

The authors report three cases of pseudo-tumoral cystitis with one prenatally diagnosed by ultra-sound investigation. They point out the difficulties of such a diagnosis. Etiopathogenesis is discussed: though in most of the cases local irritation (due to urinary infection or physical causes) is involved, in several cases no origin is determined.

Biopsy↗
PubMedDate not supplied

Meconium ileus and intestinal atresia in fetuses and neonates.

A collaborative study was performed to determine the different types and mechanisms of intestinal abnormalities during gestation. Cases had to fulfill one or more of the following three criteria: (1) meconium ileus, (2) intestinal stenosis or atresia, and (3) meconium peritonitis. Esophageal atresia, anorectal atresia, and abdominal wall defects were excluded. One hundred two cases were reviewed from the autopsies of 42 induced abortions, 22 stillborns, and the surgical findings in 38 neonates. Meconium ileus was detected mainly during the second trimester (28/38), and was associated with cystic fibrosis (15), fetal blood deglutition (4), infection (6), or multiple-abnormalities (10), in which three chromosomal aberrations were found. Intestinal stenosis or atresia was more commonly detected during the third trimester of gestation (46/56). Sixteen of the 30 duodenal malformations were associated with trisomy 21, whereas in the 26 small intestinal atresias, signs of distress or ischemia were most frequently detected. Only 8 of 25 meconium peritonitis cases were isolated. A total of 20 cystic fibrosis cases could be proved. In this series, functional abnormalities were observed predominantly in the second trimester and associated mainly with cystic fibrosis or amniotic fluid abnormalities. Anatomic lesions were commonly detected later on and associated with ischemic conditions, chromosomal aberrations, and even cystic fibrosis.

Constriction, Pathologic↗