Biomedical subjects
Publications and source records attributed to C Bourgain.
A patient with myelofibrosis who developed a progressive paraparesis caused by spinal cord compression due to thoracic extramedullary hematopoietic tissue is reported. He recovered well after local radiotherapy alone.
In human ovarian cancer, multiple specific chromosomal deletions can be found by cytogenetic analysis or molecular techniques such as restriction fragment length polymorphism probing. This work confirms the loss of HRAS alleles in 1 out of 2 cases of invasive ovarian cancer as determined in 32 samples or cell lines derived from 19 patients. Results with other polymorphic probes indicate that a consensus deletion probably includes 11p15.5-11p13. Tumor suppressor genes might be located in the deleted area, and deletion of the gene might then play a role in disease progression. Examination of DNA from distinct tumor sites of individual patients indicates clonal heterogeneity in the malignant cell population, indicating that loss of 11p sequences is a late event in the disease progression. Loss of alternate 11p alleles at different disease sites in one patient is inconsistent with the current model of tumor suppressor gene inactivation. The 11p deletion seems to be limited to ovarian cancers in younger patients. Eight novel permanent ovarian cancer cell lines, previously not described in the literature and derived from tumor sites from five patients, were included in the current analysis.
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A luteal phase defect has been demonstrated in cycles stimulated using a protocol including a gonadotrophin releasing hormone agonist (GnRHa). We have conducted a randomized prospective study of luteal and early pregnancy supplementation in 262 women selected for in-vitro fertilization (IVF), gamete intra-Fallopian transfer (GIFT) or zygote intra-Fallopian transfer (ZIFT). Either intramuscular progesterone in oil (50 mg/day) or intravaginal micronized progesterone (600 mg/day) was used as luteal supplement. In association with oestradiol valerate, progesterone administration was initiated from the day before oocyte retrieval until the 12th week of pregnancy. The implantation rate just failed to reach statistical significance (P = 0.07) in favour of the group receiving intravaginal progesterone. In the latter group, we observed a higher clinical pregnancy rate (33.6 versus 26.7%, not significant). Despite lower plasma progesterone levels, a lower first trimester abortion rate (P less than 0.05) was found in the intravaginally treated group. Intravaginal micronized progesterone was well tolerated by all patients and appeared more effective than intramuscular progesterone in improving the implantation rate, and in decreasing the incidence of abortions in stimulated cycles including GnRHa.
Deficiency in the luteal phase has been shown during stimulated cycles using a protocol involving a GnRH agonist. The authors undertook a randomised prospective trial of supplementation by progesterone of the luteal phase and of early pregnancy in two hundred and seventy two patients requiring fertilisation in vitro (FIV), gamete inter-fallopian transfer (GIFT) or zygote inter-fallopian transfer (ZIFT). Either progesterone in solution in oil (50 mg/day) administered by intramuscular injection or micronized progesterone administered intra-vaginally (600 mg/d) were used as support for the luteal phase. Administration of progesterone in association with estradiol valerate was started on the day prior to oocyte puncture and was continued until the 12th week of pregnancy. The implantation rate was very close to the threshold of significance (P = 0.07) in favour of the patients given vaginal progesterone. There was a higher rate of clinical pregnancies (33.6 versus 26.7 p. cent) in the latter group, though this was not significant. While plasma progesterone (Pg) levels were lower in patients using vaginal progesterone, the abortion rate during the first three months was lower in this group (P < 0.05). Micronized progesterone administered vaginally was well tolerated by all patients. During stimulated cycles, notably by GnRHa, it thus proved to be more effective than Pg administered by intramuscular injection with regard to implantation and abortion rates.
An 80-year-old patient presented with multiple biliary papillomatosis of both intra- and extrahepatic bile ducts, with focal epithelial dysplasia. After repeated attempts at drainage the patient died of a massive hemorrhage. The histology of this very unusual tumor is discussed, and a complete review of the literature is presented.
We report a case of ossifying fibroma of the long bones (OFLB). This lesion, occurring mainly during the first decade of life, typically affects the tibial diaphysis and clinically presents as a painless bony enlargement. Radiographic features include an osteolytic cortical expansion, containing diffuse calcifications, and separated from the medullary by a thick cortical rim. CT appears to be the examination of choice to define these signs and permits differentiation from monostotic fibrous dysplasia and from adamantinoma (by demonstrating the presence or absence of diffuse high attenuation values). The importance of making the differential diagnosis is stressed, as prognosis and management of OFLB, fibrous dysplasia and adamantinoma may vary considerably.
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In 43 patients without ovaries, endometrial biopsies at day 21 of 75 substituted cycles were studied by light and electron microscopy. The morphology of the endometrium was compared after oral, vaginal or intramuscular administration of progesterone, and correlated with the serum levels of 17-beta oestradiol and progesterone and the pregnancies obtained after oocyte donation. After vaginal application of micronized progesterone, endometrial morphology closely matched that of a natural cycle. This therapy was able to support two ongoing pregnancies. No adequate endometrial response was noted after oral ingestion of progesterone. The maturation of the endometrium after intramuscular injections of progesterone in oil was heterogeneous. It was concluded that the vaginal route for administering micronized progesterone can be advised as the treatment of choice in patients without ovarian function.
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To evaluate any beneficial effect of progesterone supplementation during the luteal phase of GIFT or IVF cycles stimulated by clomiphene citrate and HMG, two random prospective studies were performed. In the first study, a group of patients received a luteal phase supplement of 50 mg natural progesterone i.m. daily from the day of oocyte retrieval onwards. Initial results on 168 patients indicated that the pregnancy rate was similar in patients with or without progesterone supplements. No differences were found between the two groups in an analysis of pregnant and failed cycles. In a second study two different protocols of luteal phase supplementation after Buserelin-HMG stimulation were compared: natural progesterone in combination with oestradiol valerate (50 patients) or HCG supplements (41 patients). A 32% pregnancy rate per cycle was encountered in both groups. Endometrial biopsies, taken during the luteal phase from patients who did not undergo embryo replacement, revealed retarded endometrial development in most of the biopsies.
The clinical, hormonal and cytogenetic findings in 36 women with primary ovarian failure, referred for oocyte or embryo donations are reported. Fifteen women were suffering from ovarian dysgenesis and 11 from premature menopause. Six of these 26 patients showed X-chromosome abnormalities. One patient had a Noonan syndrome. The remaining 10 had surgical menopause. The mean duration of their infertility was 6.5 +/- 3.2 years (+/- SD). All patients had elevated serum gonadotrophins within the menopausal range. Hypothalamic, pituitary and thyroid function were found to be intact. In one of the 15 ovarian biopsies on the patients with chromosomal competent ovarian failure, primordial follicles were found. Hysterosalpingograms revealed a normal uterine cavity in all patients. In view of oocyte donation, careful evaluation of the obstetric risk was mandatory in the six patients with X-chromosome aberrations and in the patient with the Noonan syndrome, because of their short stature and possible concomitant cardiovascular and renal disease. After substitution therapy with oestradiol valerate and natural progesterone, 13 pregnancies were established, seven patients delivered (one set of twins), eight healthy children were born, three pregnancies aborted and three pregnancies are progressing normally.
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Granulomatous lobular mastitis is a rare chronic inflammatory disease of the breast. The differential diagnosis with malign breast disease is often not easy. In most cases a surgical biopsy is needed for correct diagnosis. Idiopathic granulomatous mastitis is an exclusion diagnosis, based on the demonstration of a characteristic histological pattern, combined with the exclusion of other possible causes of granulomatous breast lesions. There is still no generally accepted optimal treatment. If surgery forms part of the treatment, a conservative approach seems to be adequate in most cases. Another option is a long-term steroid treatment. It is mandatory to exclude infectious causes of granulomatous mastitis before corticoid therapy is started.
Different routes of natural progesterone supplementation have been tried as luteal phase support in infertility treatments. Orally administered progesterone is rapidly metabolized in the gastrointestinal tract and its use has proved to be inferior to i.m. and vaginal routes. Progesterone i.m. achieves serum progesterone values that are within the range of luteal phase and results in sufficient secretory transformation of the endometrium and satisfactory pregnancy rates. The comparison between i.m. and vaginal progesterone has led to controversial results as regards the superiority of one or the other in inducing secretory endometrial transformation. However, there is increasing evidence in the literature to favour the use of vaginal progesterone. Vaginally administered progesterone achieves adequate endometrial secretory transformation but its pharmacokinetic properties are greatly dependent on the formulation used. After vaginal progesterone application, discrepancies have been detected between serum progesterone values and histological endometrial features. Vaginally administered progesterone results in adequate secretory endometrial transformation, despite serum progesterone values lower than those observed after i.m. administration, even if they are lower than those observed during the luteal phase of the natural cycle. This discrepancy is indicative of the first uterine pass effect and therefore of a better bioavailability of progesterone in the uterus, with minimal systematic undesirable effects.
Mediastinal lymphadenopathy and goiter have been associated with primary amyloidosis, although not in the same patient. One previous case report described the association of an amyloid goiter and hyperthyroidism (due to Graves' disease) with primary amyloidosis. Till now no case reports of patients presenting simultaneously with mediastinal lymphadenopathy, intrathoracic amyloid goiter and hyperthyroidism as the first manifestation of systemic primary (idiopathic) amyloidosis have been described. The present case report describes the clinical, biological radiological and histological features in such a male patient.
Forty-four women with absent ovaries who were referred to our centre for oocyte donation were treated with oestradiol valerate and natural progesterone. Serum concentrations and endometrial changes were evaluated on day 21 of stimulated luteal phases after the daily administration of 100 mg of natural progesterone in oil injected intramuscularly or after the ingestion of 300 mg of micronized progesterone or after vaginal administration of 300 mg or 600 mg micronized progesterone, respectively. Endometrial tissue did not show evidence of an adequate secretory pattern after orally administered progesterone. However, after intramuscular injection of 100 mg of natural progesterone and after the vaginal administration of 300 to 600 mg of micronized progesterone, the histologic and electron microscopic pictures were similar in all treatment groups and were close to the expected day 21 of the cycle. Serum levels of progesterone after intramuscular injection were five times higher than after vaginal administration.