Biomedical subjects
Publications and source records attributed to P Babin.
We report on four cases of trisomy 14 as the sole anomaly. Three cases were myelodysplastic syndromes and one was a non-Hodgkin's lymphoma. This anomaly is mainly in myeloid disorders and still remains to be well documented. On the other hand, we show this anomaly to be also a non-random anomaly in lymphoproliferative disorders.
We report a case of inflammatory polyp in a male patient, which was totally obstructing the left main bronchus but could be removed by simple endoscopic traction. The polyp was responsible for an absence of left lung ventilation which in turn produced by reflex an absence of left lung perfusion completely reversible after removal of the polyp. Inflammatory polyp is a rare benign tumour. Its physiopathology is controverted, but it seems to result from local irritation of the bronchial mucosa. Bronchial endoscopy is the essential examination which in most cases make it possible to diagnose and treat the tumour.
A 6-week-old girl presented with cutis laxa, emphysema, heart anomalies and a diaphragmatic hernia. She died at 22 weeks. A recurrent ctb(7)(q31.3) was found and the laminin gene was suspected to be involved in the disease. Anti-human laminin antiserum showed that this protein was absent from the skin. This case, together with 17 other similar cases, could represent a new type of connective tissue disease.
Various phytoestrogens such as formononetin, daidzein, genistein and equol were synthesized. Their purity was assessed by various analytical techniques including melting point determination, thin-layer chromatography (TLC), infra-red spectra (i.r. spectra), nuclear magnetic resonance (1H- and 13C-NMR) and gas chromatography coupled with mass spectrometry (GC-MS). The estrogenic activity of these compounds, as well as biochanin A and coumestrol, was biologically tested by the induction of vitellogenin secretion in yearling sturgeon and compared to the activity of estradiol-17 beta. Pure daidzein, biochanin A, genistein, equol and coumestrol all had estrogenic activity as assessed by their induction of hepatic synthesis of vitellogenin when administrated intraperitoneally to yearling Siberian sturgeon. Coumestrol seemed to be the most potent compound, inducing the most vitellogenin secretion with the lowest dose administered. Formononetin was inactive when administered by the intraperitoneal route. All the phytoestrogens tested were considerably less potent than estradiol-17 beta.
We report two cases of t(8;9) with probable breakpoints in 8q12 and 9p21 in malignant lymphoma. In a review of the literature, we found two cases of acute lymphocytic leukemia and one case of malignant lymphoma which probably share the same breakpoints.
Seventeen cases of Wegener's granulomatosis have been reviewed in search of articular involvement. Articular symptoms were present in 13 cases (76 p. cent), and were inaugural in 9 cases (53 p. cent). Six of these patients experienced arthralgias, which were most often migratory, and were inaugural in 3 cases. Seven patients had arthritides, which were inaugural in 6 cases; they were fixed and additive in 6 of these 7 cases, making up a distal polyarthritis in 3 patients, and an oligoarthritis in the 3 other ones; they were transient and migratory in 1 case. The 3 cases of distal polyarthritis were inaugural and fulfilled the ARA criteria for rheumatoid arthritis; two of them were accompanied by nodules which were quite identical to rheumatoid ones. There was no axial involvement. Joint involvement was not destructive and had a favourable course under disease treatment. Myalgias were present in 3 cases, one of which simulated Horton's disease. Biological manifestations chiefly consisted of marked inflammatory changes. Antineutrophil cytoplasm antibodies were present in 11 out of 16 patients in whom they were searched; among 6 of these patients who had active disease, they were present in 5. The antibody level decreased as treatment reduced disease activity and suppressed joint involvement. Joint involvement in Wegener's granulomatosis seems to be the inconstant hallmark of disease activity. It requires no specific treatment.
The authors report a case of metastasis from a breast cancer located in a frontal meningioma. A review of the literature revealed 43 cases of primary intracranial tumours containing a secondary from an unrelated extracranial primary malignant tumour; in 34 of these cases the intracranial tumour invaded was a meningioma.
We report 2 cases of intestinal perforation caused by tuberculosis and affecting the small intestine in one case and the colon in the other case. The patients were men aged 49 and 51 years respectively. Both were cachectic and presented with advanced open pulmonary tuberculosis. Perforation in free peritoneal cavity occurred 2 and 8 days respectively after an antituberculous treatment was initiated. The outcome was rapidly fatal in both cases. Tuberculous enteritis has become rare, but it can still be observed in patients with severe open pulmonary tuberculosis, where the gastro-intestinal tract is contaminated by the large number of virulent mycobacteria swallowed. In such patients clinicians must be alert to abdominal premonitory signs. Intestinal perforations in free peritoneal cavity are uncommon. Most perforation are small, single or multiple, and located on the antimesenteric side of the terminal ileum. They may occur at any time, and particularly just after an antituberculous therapy has been instituted. Clinical presentation is one of acute peritonitis requiring emergency laparotomy. Mortality has been reduced by technical improvements, notably temporary enterostomy, but perforation remains a serious and often fatal complication of tuberculosis in patients with severe malnutrition.
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The squamous cell carcinoma of the external auditory canal is extremely rare. The facial paralysis is usual, but pain seems to be the most common symptom. The mean age at diagnosis is 50 to 60 years, with chronic external otitis preceding. The CT-scan is the most important investigation before treatment: surgery and external radiotherapy. Surgical procedure must be large, with or without facial nerve dissection, following by irradiation of the temporal bone and cervical nodes. The generally poor prognosis is explained by the frequency of advanced disease at the time of diagnosis. This paper reports our 4 experiences, with a bilateral carcinoma case.
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A 77-year-old woman had variceal bleeding related to systemic mastocytosis. Physical examination revealed minimal ascites and mild hepatomegaly noted 11 years before. Liver function tests were nearly normal. Because of early recurrent bleeding, a mesocaval shunt was performed. Wedged liver biopsy showed a moderate fibrosis of portal tracts and massive mast cell infiltration within portal tracts and sinusoids. Perisinusoidal collagen deposition was demonstrated ultrastructurally. We suggest that systemic mastocytosis be added to the list of diseases related portal hypertension with perisinusoidal fibrosis. As there is currently no specific treatment, a portocaval shunt should be discussed.
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We report on 33 unpublished patients with clonal anomalies in chronic lymphocytic leukaemia. The literature was thoroughly reviewed in order 1) to quantify the frequency of anomalies found in chronic lymphocytic leukaemia and to give new status to the rarest, 2) to determine whether a given anomaly was an additional anomaly and/or a primary anomaly, and 3) to find out whether strong associations between different anomalies exist in this disease.
In two white adults born, raised, and living in central France and presenting with long-lasting malabsorption, massive and diffuse lymphoid infiltrate of the lamina propria associated with crypt scarcity was found along the whole small bowel. It was mostly composed of mature lymphocytes, focally mixed with plasma cells and reactive germinal centers. There was no evidence of celiac disease, systemic or intestinal immune deficiency or alpha-chain disease, overt lymphoid malignancy, or stagnant-loop syndrome. By immunofluorescence the infiltrate was constituted in 1 case of polyclonal B cells and, in the other, of a large majority of T11, T8, T10, and class II-positive T cells associated with a population of monotypic B cells. A gluten-free diet and parenteral nutrition proved ineffective. A dramatic and protracted clinical response was observed in both patients after the onset of oral tetracycline therapy, and still persists after 8 and 5.5 yr, respectively, together with morphologically unchanged small bowel infiltrate. These cases may be the equivalents, in people from Western developed countries, of the predominantly lymphocytic variety of the immunoproliferative small intestinal disease described in people from developing countries.
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The authors report the case of an isolated supratentorial hemangioblastoma occurring in a woman operated on for a cerebellar hemangioblastoma twenty years before. The data furnished by immunohistochemical technics don't seem to be deciding and don't work out the problem of hemangioblastoma histogenesis.