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Biomedical subjects

L Boccon-Gibod

Publications and source records attributed to L Boccon-Gibod.

At least 307 records · Page 17Linked to original sources
PubMed1982

An original hypercalcemic infantile renal tum or without bone metastasis: heterotransplantation to nude mice: report of two cases.

Two cases of hypercalcemic infantile renal tumor without macroscopic bone metastases are reported. Optical and ultrastructural characteristics as well as biological and clinical data are sufficiently different from those of other infantile renal tumors to justify the individualization of an original tumor entity. The hypercalcemia is not clearly understood but may be related, in one case, with a high N terminal PTH serum level. Ultrastructural elements (secretory granules) and polypeptide hormone products (glucagon) are discussed in relation with APUD cells characteristics.

Animals↗
PubMed1982

[Segmental dilatation of the ileum in a neonate. Study of the myenteric plexus with a silver staining preparation (author's transl)].

A case of segmental dilatation of the ileum in a neonate is reported. Conventional staining methods failed to show any abnormality, but a study of the myenteric plexus with a silver preparation demonstrated reduction in the number of agyrophil neurons and irregular and altered neuron cell bodies. Occurring in a neonate, therefore without previous clinical history, this case, together with various published data suggests that in some cases segmental dilatation of the ileum is due to congenital damage to the myenteric plexus.

Dilatation, Pathologic↗
PubMed1981

[A study of immunoglobulin-producing cells in the colonic mucosa of children with Crohn's disease (author's transl)].

The immunoglobulin-producing cells of the rectal and colonic mucosae were studied on 12 biopsies obtained from 6 children, aged from 13 to 18 years, suffering from Crohn's disease involving the ileum and colon or the colon only. After incubation with immune sera specifically directed against alpha, gamma and mu chains, the fluorescent plasmocytes present in a predetermined surface area of the chorion were counted in the patients and in a control group of 5 children. Compared with controls, children with Crohn's disease had less IgA-producing plasmocytes per surface area (mean: 32.3 and 44.3 respectively) and a lower ratio of these plasmocytes to total immunoglobulin-producing cells (patients: 79%, controls: 85%). The decrease in IgA-producing cells was more pronounced in the colon but was also observed in the rectum, despite normal histology.

Adolescent↗
PubMed1981

[Diffuse cystic angiomatosis of bone. A report on one case followed-up over five years (author's transl)].

The authors report a case of diffuse cystic angiomatosis of bone. The follow-up over five years showed a dramatic worsening which has seldom been observed. The roentgenographic aspect of this rare disease seems always similar, but non specific. The pathologic diagnosis is difficult. The bony involvement is due to a widespread vascular dysplasia, and the final diagnosis relies upon the association with other arteriovenous or lymphatic disorders, or with an abnormal lymphogram.

Angiomatosis↗
PubMed1980

[Urinary incontinence following prostatectomy: hopes and disappointments. 42 cases (author's transl)].

Between 0,5 and 1% of prostatectomies are complicated by urinary incontinence due to the destruction of both proximal and distal urethral sphincters. Any incontinence persisting more than 12 months after surgery should be considered as permanent. Among all treatments proposed (medical treatment, electrical stimulation of the perineum, various plastic operations and prostheses with varied and numerous materials), only inflatable prostheses seem to give "better than nothing results. The best treatment of post-operative incontinence, however, is preventive: extreme care should be exercised during the enucleation or trans-urethral resection of the adenoma.

Aged↗
PubMed1980

Clinical, cytogenetical, histological, immunological and hormonal studies in a case of true hermaphroditism.

A true hermaphrodite with ambiguous genitalia and 46XX karyotype was studied from 18 months of age to 17 years. At 13 years, he developed an ambiguous puberty, with marked bilateral gynaecomastia and public hair score three. A relatively high testosterone level (21 nmol/l), not increased by hCG stimulation, was associated with a high LH level (6 UI/l). In the venous blood of the right ovotestis, the steroid concentrations were in the adult male range, especially testosterone (1400 nmol/l). After removal of the right gonad, large fluctuations of oestradiol levels were observed (150-810 pmol/l). The testosterone secretion of the left ovotestis was low, concentrations begin 4.2 and 130 nmol/l in peripheral and gonadal blood respectively at 16 years. A significant LH surge was induced by oral ethinyl-oestradiol before removal of the left gonad. The 5 alpha-reductase activity was normal in pubic skin. In the left gonad the concentrations of cytosol receptors for testosterone and 5 alpha-dihydrotestosterone (DHT) were significant: 14.5 and 65.5 fmol/mg protein respectively. However the plasma DHT level was not increased by hCG. Finally, the presence of H-Y antigen was demonstrated on lymphocytes. This accords with the presence of testicular tissue in an XX subject, and with significant testosterone production. The high testosterone production did not prevent the appearance of a positive oestrogen-LH feedback. The relative peripheral insensitivity to testosterone, is, in some ways, inconsistent with the presence of receptors for androgens.

3-Oxo-5-alpha-Steroid 4-Dehydrogenase↗
PubMed1980

Staghorn calculus: the place of nephrolithotomy.

Out of 190 staghorn calculi submitted to conservative surgical treatment, 20 were selected for deliberate nephrolithotomy on the basis of previous stone surgery, shape of stone or of kidney pelvis. Stone clearance was achieved in 80% of the cases. 1 patient died of pulmonary embolus; a massive secondary hemorrhage was successfully treated by transcatheter arterial embolization. Marked loss of renal function occurred three times on previously heavily scarred kidneys. Nephrolithotomy should be considered only when local conditions preclude extended pyelocalicotomy.

Adolescent↗
PubMed1980

[Surgical excision of a hepatoma complicating chronic tyrosinemia (author's transl)].

A girl with chronic hereditary tyrosinemia is described in whom the diet caused an immediate resolution of tubular defect and rapid healing of the rickets. However cirrhosis was not prevented and at the age of 8 she developed a hepatoma. Complete surgical excision was possible and she remained well 15 months later. The value of blood alpha-fetoprotein levels and liver echotomography to monitor the course of the disease is emphasized. The apparent ineffectiveness of the low phenylalanine and tyrosine diet in preventing hepatic complications is discussed.

Amino Acid Metabolism, Inborn Errors↗