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Biomedical subjects

P Bigel

Publications and source records attributed to P Bigel.

At least 19 recordsLinked to original sources

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.

Aged↗

Rhinoscleroma with orbital extension: CT and MRI.

We describe the MRI features of a rhinoscleroma with orbital extension. This benign bacterial and granulomatous lesion of the paranasal sinuses gave homogeneous low intensity on T2-weighted images and enhanced with gadolinium. It could simulate a malignant sinonasal tumour or a fungal sinusitis; the diagnosis must be considered in patients from endemic areas.

Adult↗

[Morphology and vascularization of the varicose internal saphenous vein. Comparison with the normal structure].

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.

Capillaries↗

[Primary Gougerot-Sjogren syndrome. Spontaneous one-year course of clinical, biological and histological signs].

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.

Aged↗

Infantile myofibromatosis. Report of two cases in one family.

Two brothers with infantile myofibromatosis are reported. Both had cutaneous and skeletal myofibromas with spontaneous and complete healing of their cutaneous lesions. These cases suggest autosomal recessive inheritance of this rare disorder.

Bone Neoplasms↗

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.

Abnormalities, Multiple↗

[Specific antigens of the papillomavirus group in various intra-epithelial proliferations. Methods of detection].

An antiserum against viral particles extracted from bovine papilloma has been obtained in rabbits (New Zealand). The microscopic assay was performed by the peroxidase - anti-peroxidase method on two laryngeal papillomas, four cervical condylomas, three cutaneous papillomas, one oral condyloma, as well as on four ano-genital condylomas. A positive nuclear labelling was observed in 5 to 10% of the tumor cells localized exclusively in the superficial layers as well as in the koilocytes in these tumors. The use of this antibody in immunocytochemical studies will provide an additional tool for the investigation of mucocutaneous proliferations.

Animals↗

[Idiopathic myositis of the small intestine. An unusual cause of chronic intestinal pseudo-obstruction in children].

The case reported concerns a child with chronic intestinal pseudo-obstruction (CIPO) whose digestive manifestations (intestinal adynamia and distension) were present from the age of 6 months and lasted, despite medical and surgical treatments until 4 years of age, when death occurred. The multiple samplings showed important inflammatory reactions centred on the muscular layers of the small intestine, together with degenerative lesions of the muscular fibres, progressively leading to fibrosis and atrophy of the intestinal wall with secondary and final impairment of the myenteric plexuses. The diagnosis of myositis of the small intestine is extremely rare. It is not part of the usual causes of intestinal adynamia and CIPO, which were reviewed. Hollow visceral myopathy and systemic sclerosis of the GI tract were more especially discussed. For lack of etiopathogenic convincing data and of similar observation in the literature, this case may be temporarily considered as an idiopathic myositis of the small intestine, a potentially new cause of CIPO.

Appendix↗

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.

Diseases in Twins↗

[Comparison of the effects of orally or percutaneously administered estradiol on carbohydrates and lipids after the menopause].

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.

Administration, Oral↗

[Use of a severity index in 8 multidisciplinary resuscitation centers].

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.

Disease↗

Reexamination of paternal age effect in Down's syndrome.

The recent discovery that the extra chromosome in about 30% of cases of 47, trisomy 21 is of paternal origin has revived interest in the possibility of paternal age as a risk factor for a Down syndrome birth, independent of maternal age. Parental age distribution for 611 Down's syndrome 47, +21 cases was studied. The mean paternal age was 0.16 year greater than in the entire population of live births after controlling for maternal age. There was no evidence for a significant paternal age effect at the 0.05 level. For 242 of these Down's syndrome cases, control subjects were selected by rigidly matching in a systematic manner. Paternal age was the variable studied, with maternal age and time and place of birth controlled. There was no statistically significant association between paternal age and Down's syndrome. After adjustment for maternal age, these two studies were not consistent with an increase of paternal age in Down's syndrome.

Adolescent↗

[Malignant histiocytosis of the intestine. An anatomo-clinical case report].

Clinico-pathological case report of a 11 year-old girl who successively presented persistent abdominal pain, pneumoperitoneum, repeated intestinal hemorrhages, superficial intestinal ulcerations, first localized on the jejunum and later extended to the all gut. After a course of 13 months, the child died from diffuse and repeated hemorrhages. The morphologic studies revealed a diffuse and homogeneous cellular infiltrate made of large round "lymphomatous" cells scattered within the lamina propria and submucosa. Initially this superficial cellular infiltrate was overshadowed by accompanying inflammatory cells and was not recognized as a tumoral process. Later on the dissemination of abnormal cells to the entire ileon, mesenteric lymph nodes, spleen and liver and the cellular appearance confirmed its neoplastic nature and allowed to consider this process as a malignant histiocytosis of the intestine as described by Isaacson and Wright. This case seems to be the first case reported in childhood.

Child↗