Bilateral dystrophic ossification of the thyroid cartilage appearing as symmetrical laryngeal masses.

Primary cartilaginous lesions of the larynx are relatively uncommon. We present a case of bilaterally pseudocystic lesion of the thyroid cartilage that demonstrated progressive calcification. Pathologic analysis showed features suggesting a dystrophic lesion with no evidence of malignancy. We hypothesize that repetitive microtrauma related to muscular overuse probably led to inflammatory changes at tendinous insertions on the laryngeal cartilage and resulted in dystrophic ossification of the laryngeal cartilage.

Merkel cell carcinoma: a rare cause of hypervascular nasal tumor.

Cutaneous neuroendocrine carcinoma, first described in 1972, is an aggressive disease usually occurring in sun-exposed skin. Other sites have been described, however; such tumors occasionally occur within the nasal fossa. A high rate of metastasis (>30%) explains the poor prognosis. Descriptions of the imaging features of these tumors, mainly located in cutaneous region, are rare. We therefore present the imaging features of two cases of Merkel cell carcinoma involving the sinonasal region, suggestive of a hypervascular tumor.

[Morphology and vascularization of the varicose internal saphenous vein. Comparison with the normal structure]. / Morphologie et vascularisation de la veine saphne interne variqueuse. Comparaison avec la structure normale.

After reviewing the main characteristics of the normal long saphenous vein, the histological alterations observed in varicose veins are described. Samples were obtained from 14 patients undergoing venous surgery for varicose veins with long saphenous vein incompetence. These alterations mostly involve the connective tissue prolife-rating in the middle layer and causing the separation and interruption of the muscular bundles. In addition, the vasa vasorum network through the venous wall was investigated. These vessels were normally observed in the adventitial layer and the outer third of the media. In some samples from varicose veins, vasa vasorum were increased and capillaries close to the intimal layer.

[Comparison of the effects of orally or percutaneously administered estradiol on carbohydrates and lipids after the menopause]. / Comparaison des effets de l'estradiol administré par voie orale et per-cutanée sur les glucides et les lipides après la ménopause.

The metabolic changes in carbohydrate (fasting blood sugar and blood sugar levels two hours after taking 70 g of glucose orally as well as insulin and glycosylated A1c haemoglobin) and also lipid metabolism as total and fractional HDL, triglycerides and alphaprotein B, were compared in 43 patients of a mean age of 48.6 years who had hysterectomy with castration. These patients were divided into two random groups: the first group (23 women) received hormone replacement treatment in a dose of 1.5 mg of oestradiol percutaneously, the second group of 20 women received 2 mg of oestradiol specially prepared for oral administration. Biological control was carried out immediately after the treatment and 3 and 6 months later. It consisted of levels of carbohydrates and of lipids and of oestriol, oestradiol and FSH. Both treatments, whether percutaneous or oral, were similar in their beneficial action on the changes brought about by castration. When 17 Beta oestradiol was administered percutaneously a better control over the carbohydrate and lipid (triglyceride) metabolism was obtained. This confirms that when oestradiol is administered orally the same respect has to be given to the contrary indications to its administration and to controlling the metabolic effects as when any other form of oestrogen therapy is administered orally.

[Primary Gougerot-Sjogren syndrome. Spontaneous one-year course of clinical, biological and histological signs]. / Syndrome de Gougerot-Sjögren primaire. Evolution spontanée sur un an des signes cliniques, biologiques et histologiques.

OBJECTIVES: The natural clinical course of primary Sjögren's syndrome was followed in 8 patients to identify the concomitant functional, clinical, biological, scintigraphic and histological manifestations of the disease. METHODS: The diagnosis of primary Sjögren's syndrome was made on the basis of functional signs (ocular or salivary sicca syndrome) and 2 positive tests among the 3 objective ocular tests (Schirmer's test, break-up time, Rose Bengale). Work-up included recording of functional and clinical signs, ophthalmologic examination and laboratory tests at diagnosis and every 3 months for 12 months. Scintigraphy of the salivary glands was performed together with a biopsy at diagnosis and at 12 months. RESULTS: No one parameter varied significantly over a 1 year period demonstrating the lack of need for renewed examinations for diagnosis or regular follow-up. CONCLUSION: This is the first report providing a homogeneous series studied by one team over a determined period of time. It demonstrates that clinical, biological and anatomic criteria for primary Sjögren's syndrome do not show any correlation between functional signs and objective ocular tests.

[Use of a severity index in 8 multidisciplinary resuscitation centers]. / Utilisation d'un indice de gravité dans huit services de réanimation multidisciplinaire.

A universal severity index for acutely ill patients was applied to 794 acute patients treated in eight medical and surgical French intensive care units located in teaching or community hospitals either in Paris or in provincial towns. Measured on the first day in hospital from objective numerical data, the index proved reliable and correlated well with hospital mortality and the sustained therapeutic efforts of the first 24 hours. It could be used in multicentre studies to compare outcomes and evaluate new treatments.

PHA dose dependent defect in lymphocyte stimulation and E-rosette-forming cells in untreated Hodgkin's disease.

14 untreated patients with Hodgkin's disease were tested for lymphocte reactivity in vitro to optimal and suboptimal doses of PHA. A PHA dose-dependent defect in lymphocyte stimulation was demonstrated in patients when compared with a group of normal individuals. No correlation between the in vitro defect and decreased absolute number of E-rosette-forming cells (T-lymphocytes) in peripheral blood was found.

Usefulness of a registry of congenital malformations for genetic counseling and prenatal diagnosis.

During three years, 39,924 infants born consecutively in the area covered by our registry of congenital malformations were surveyed; 775 had major congenital malformations. Recurrence risks for the major malformation was estimated and classified as high (greater than 10%, 5.3% of the cases), low (1 to 10%, 85.3% of the cases) or occasional (less than 1%, 9.4% of the malformed). Feasibility of prenatal diagnosis was considered. On the basis of the recurrence risk of 1% or higher and the feasibility of prenatal diagnosis, such a procedure should be considered in future pregnancies in 64.1% of the mothers. Genetic counseling has to be given to couples at risk of having a malformed child. For this purpose, as is shown in our study, the best way is the possibility of using a registry of congenital malformations.

[Hodgkin's disease. T lymphocyte defect in patients in complete remission (author's transl)]. / Maladie de Hodgkin. Déficit lymphocytaire thymo-dépendant chez des patients en rémission complète.

11 patients with Hodgkin's disease in remission, including 7 patients with an initial stage III-IV, off treatment for at least 6 months, were tested for blood T lymphocyte functions. All were completely re-evaluated at the end of treatment to assess complete remission. The number of peripheral blood lymphocytes was below 1,200/cu mm 5/11 patients. Absolute numbers of E-rosette-forming T lymphocytes were decreased in 8 patients, whereas active rosettes were normal in 4/6. A slightly increased percentage of EAC rosettes, a marker for B lymphocytes, was found in only 2 patients. In vitro lymphocyte reactivity to a sub-optimal dose of PHA was studied in 9 patients. A statistically significant defect of lymphocyte transformation was observed in the patient group T lymphocytes. A membrane change is suggested rather than a true depletion. The persistance of such abnormalities long after treatment may raise the question of a complementary immunostimulating treatment in these patients.

Sister chromatid exchanges in balanced translocation carriers and in patients with unbalanced karyotypes.

Sister chromatid exchanges (SCEs) were studied in peripheral human leukocytes from 16 patients with balanced translocations or with unbalanced karyotypes, and from 4 controls. No difference was seen between these two groups of people in the mean number of SCEs per cell, or in the total number of SCEs observed for each pair of autosomes involved in the translocations studied. With this last number no difference from the expected number of SCEs, if one supposes that SCEs follow a random distribution, was seen.

[Lymphocyte transformation test in Gell's and Coombs' drug reactions]. / Der Lymphozytentransformationstest bei medikamentösen Zwischenfällen des Reaktionstypus I nach Gell und Coombs

A method of interpretation of the lymphocyte transformation test is described which results on the basis of statistical evaluation. With this method good results were received in cases of drug induced side effects of type I according to Gell and Coombs. The investigations on 61 patients have shown that differneces between the clinical findings and lymphocyte transformation tests are rare. They are mainly in relation to false-positive reactions of the lymphocyte transformation test in Penicillin allergy.

Discordance for skeletal and cardiac defect in monozygotic twins.

A case of monozygotic male twins discordant for skeletal and cardiac defect is reported. One twin had the hemifacial microsomia type of the oculo-auriculo-vertebral dysplasia. The cotwin had no asymmetry of the face and normal ears, but preaxial polydactyly and ventricular and auricular septal defects. The cotwins were concordant for craniostenosis with a ridge metopic suture. Karyotypes were normal.

[Immotile cilia syndrome]. / Syndrome d'immotilité ciliaire chez un enfant de 10 ans.

Immotile cilia syndrome was diagnosed in a 10 year-old boy presenting with Kartagener syndrome: chronic otitis, sinusitis, recurrent respiratory disease and situs inversus. Electronmicroscopic examination of biopsies of the nasal mucosa showed the lack of interne dynein arms, explaining the immotile cilia syndrome in that boy.

Cold lymphocytotoxin reactivity with lymphocytes from donors in various physiological conditions.

Sera containing cold lymphocytotoxins were studied in the presence of lymphocytes from neonates, pregnant women, blood donors and old people and the degree of positivity varied according to the lymphocyte population investigated: a strongly positive response to lymphocytes from neonates and pregnant women, a weaker response to lymphocytes from old people and a very weak response to chronic leukemic lymphocytes. Cold lymphocytotoxins seem to be a supplementary method of demonstrating a difference in lymphocytic behavior.

Normal active rosette-forming-cells in untreated patients with Hodgkin's disease.

The percentage of E-rosettes and active E-rosettes was determined in untreated patients with Hodgkin's disease. All patients had numbers of peripheral blood lymphocytes within the normal range (1,200-5,000 lymphocytes/cu mm). The mean percentage of E-rosettes was significantly lower in the patients (55 +/- 15.7) as compared to normal controls (63 +/- 6.7). No difference in the percentage of active E-rosettes was found (36.6 +/- 8.6 in controls versus 40.3 +/- 10.8 in patients).