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Biomedical subjects

F Gottrand

Publications and source records attributed to F Gottrand.

At least 163 records · Page 9Linked to original sources
PubMed1990

Severe combined immunodeficiency syndrome associated with autosomal recessive familial multiple gastrointestinal atresias: study of a family.

Hereditary multiple atresias involving the gastrointestinal tract from pylorus to rectum are the most unusual form of intestinal atresia; the type of inheritance was suggested to be autosomal recessive. The inheritance of the severe combined immunodeficiency syndrome can be autosomal recessive or X-linked. We report on 3 sibs with multiple-level intestinal atresias. One sib had severe combined immunodeficiency syndrome and clinical histories of the other 2 sibs strongly suggested a congenital immunodeficiency syndrome. The parents of those children were healthy and nonconsanguineous. To our knowledge, this is the first report of the association of multiple gastrointestinal atresias and immunodeficiency which appears to have an autosomal recessive pattern of transmission. Our family report suggests that, in the presence of multiple gastrointestinal atresias, attention should be given to possible associated immunological disorders.

Abnormalities, Multiple↗
PubMed1990

Intraoperative endoscopic diagnosis of heterotopic gastric mucosa in the ileum causing recurrent acute intussusception.

Heterotopic gastric mucosa (HGM) may be found anywhere in the gastrointestinal tract, most often in a Meckel's diverticulum. Approximately 30 cases of HGM, located in the small bowel beyond the ligament of Treitz and not associated with a Meckel's diverticulum, have been reported. They were most often revealed by intestinal intussusception, occasionally by perforation of an intestinal ulcer or intestinal bleeding. We report a 4-year-old boy who had three attacks of acute intestinal intussusception over a 5-month period resulting in surgery. Both physical examination and barium examination of the small bowel and large intestine were found to be normal between attacks. Peroperative palpation of the small bowel was normal during the three laparotomies. During the third operation, he underwent an intraoperative endoscopy (IOE), which revealed a polypoid mass 2 cm in diameter and 0.5 cm in height, 40 cm proximal to the ileocaecal valve. Histologic examination showed HGM with fundic glands, and chief and parietal cells. This case emphasizes the interest of IOE, the main indications of which are the localization of unknown sites of gastrointestinal bleeding and the search for hamartomatous polyps of the Peutz-Jeghers syndrome for polypectomy and/or segmental resection.

Acute Disease↗
PubMed1990

[Neonatal pyloric obstruction. Diagnostic and therapeutic aspects. Apropos of 5 cases].

Five cases pyloric obstruction, revelated at the birth-period were summarized from 1964 to 1987; 1 pyloro-duodenal atresia, two cases of total pyloric obstruction by diaphragm, two cases of pyloric obstruction associated with several ileal and/or-colic atresia in the same family, and suspected at the antenatal echographic study. The treatment was either pyloro-duodenal anastomosis (1 case) or diaphragmatic resection with pyloroplasty (4 cases). In immediate results are obtained one immediate death, at the third day, and 4 initial good results; but two deaths came in a further period at 3 months and 8 months (the same family) with sepsis after ileus. A syndrome of immuno-deficiency was demonstrated in this two familial cases.

Duodenal Diseases↗
PubMed1990

[The bronze baby syndrome Apropos of 3 cases].

Bronze baby syndrome is an infrequent complication of phototherapy for neonatal jaundice which develops in infants with liver disease. The characteristic feature is a grayish-brown discoloration of the skin, plasma and urine. Three cases are reported. There was one case each of bile duct atresia, galactosemia, and parenteral nutrition with cholestasis. The pigment responsible for the discoloration has not been identified and pathogenesis remains unelucidated. The bronze color disappears spontaneously and the prognosis is dependent on the causative liver disease for which investigations should be performed routinely.

Biliary Atresia↗
PubMed1990

[Proctorrhagia secondary to lymphonodular hyperplasia revealing a food allergy].

A case of lymphonodular hyperplasia as a cause for lower gastrointestinal bleeding in a 6 year-old child is reported. Bleeding associated with peribuccal eczema and buccal itching after eating some foods, all regressing on restricted diets, suggest the possible role of a food allergy. Digestive and cutaneous signs improved with oral sodium cromoglycate associated with restricted diet.

Child↗
PubMed1989

Familial hyperinsulinism with nesidioblastosis of the pancreas: further evidence for autosomal recessive inheritance.

We report on a brother and sister with hyperinsulinism and nesidioblastosis of the pancreas. In addition, one brother and one sister who died in the neonatal period were probably affected. The parents of these children were healthy and consanguineous. We think that this is strongly suggestive of autosomal recessive inheritance. Seven other reports of presumed autosomal recessive hyperinsulinism are reviewed. To our knowledge, we report the first case in sibs whose parents were consanguineous. We think that early recognition of the condition is of obvious importance not only for therapy, but also for purposes of genetic counseling.

Consanguinity↗
PubMed1988

[Dumping syndrome: a case after Nissen's operation].

We report on a case of dumping syndrome 1 month after Nissen fundoplication in a 5 month-old infant presenting with symptoms of late hypoglycemia. Blood glucose levels in pre- and post-prandial periods, oral glucose tolerance test and isotopic gastric emptying study confirmed the diagnosis. All symptoms resolved with diet therapy which could be stopped 8 months later. Dumping syndrome is not a so rare complication of gastric surgery and should be considered in patients with non specific symptoms.

Dumping Syndrome↗
PubMed1988

[Ring chromosome 9. Case report and review of the literature].

We report on a girl with ring chromosome 9, and review the 9 other cases of the literature. The main signs of this de novo chromosomal anomaly are: severe microcephaly, growth and psychomotor retardations, and heart malformations. Infectious complications occurs often. We found a decreased level of leucocyte interferon.

Abnormalities, Multiple↗
PubMed1988

[Indications for liver transplantation in Wilson's disease].

We report the case of a 12 year-old boy with Wilson's disease, presenting with decompensated liver cirrhosis. Medical treatment failed to prevent fulminant liver failure in less than one month. Emergency liver transplantation was successfully performed. This report led us to review the prognosis of Wilson's disease with liver failure according to the present results of liver transplantation.

Adolescent↗
PubMed1988

[Intestinal biopsy: fiberscopy or Watson's capsule?].

We compared the results of 100 fiberendoscopic small bowel biopsies (F) with those of 100 Watson capsule biopsies (W). Three to 6 specimens were obtained during endoscopy. The F biopsy fragments were deeper than the W biopsies (P less than 0.001). The F biopsies were more often located in the duodenum than the W biopsies. The failure rate of the F biopsies (3%) was lower than for the W biopsies (12%): P less than 0.02. Multiple biopsies increased the diagnostic value of fiberendoscopy, which was 85% in our experience versus 77% for Watson capsule. The duration of the endoscopic procedure, available in 15 children, was 6.5 min for 4 to 6 samples, i.e. 1.5 min by specimen. Small bowel fiberendoscopy appears to be a safe and accurate method for performing small bowel biopsies in infants and children.

Adolescent↗
PubMed1987

[Study of congenital hemiplegia: value of x-ray computed tomography of the brain and of somatosensory evoked potentials].

21 cases of children with congenital hemiparesis have been studied by computerized tomography (CT) of the head and somatosensory evoked potentials (SEP). Computerized tomography is correlated with clinical features and may disclose cerebral lesions and their physiopathological mechanisms. Somatosensory evoked potentials allow a more functional approach of the handicap but are not perfectly correlated with the motor deficit. SEP are correlated with the topography of cerebral lesions showed by CT. These two investigations seem to be interesting and complementary for the exploration of congenital hemiparesis.

Adolescent↗