Biomedical subjects
Publications and source records attributed to H Rousset.
The authors report a case of right ventricular insufficiency by constrictive pericarditis revealing aortic and retroperitoneal fibrosis. Only one similar case has been published. We discuss the etiology of this affection, the possible analogies with idiopathic systemic fibrosis and Takayasu's disease and the possible participation of thyroiditis disease and therapy (particularly propranolol).
The authors report two cases of hypercalcemia which occurred in hypoparathyroid women treated with vitamin D and calcium. A recent literature review suggests a major role for parathyroid hormone-related protein (PTH-rP), secreted by the mammary tissue, in this phenomenon with obvious implications for clinical practice.
Explore the source record for details and available documents.
Multiple Endocrine Neoplasia type 1 (MEN 1) is an autosomal dominant familial syndrome characterized by involvement of several endocrine glands, including parathyroid, pancreatic islet cells, anterior pituitary and diffuse neuroendocrine tissues (carcinoids). The gene causing this syndrome has been localized to chromosome 11 but was not cloned up-to-date. Pre-clinical diagnosis in predisposed MEN 1 families was based on the use of genetic linkage analysis with polymorphic DNA probes flanking the disease locus. The set-up collaborative multi-disciplinar medical and surgical network facilitates further clinical and genetic studies on MEN 1 families. Semiological course of the disease is complex and the main objective in clinical follow-up of patients and related is to limit the probability of misdiagnosis. The present report describe the clinical and genetic analysis in a MEN 1 family and the difficulties related to diagnose the disease. An interesting observation on two cases of hyperprolactinemia by two individuals further excluded by genetic analysis assess the potential risk of bias in genetic linkage studies in non-well documented families. Concerted analysis of genetic and bio-clinical data permitted the evaluation of each patient and to exclude the risk of MEN 1 in all children tested. This example demonstrates the need of a complete clinical information previously to genetic analysis and a multi-disciplinar and collaborative approach in follow-up of patients in each family.
We report a case of arthritis, hypercalcemia, and lytic bone lesions that occurred shortly after repeated administration of recombinant hepatitis B virus (HBV) vaccine in a 44-year-old man who had had myasthenia gravis 20 years earlier. He presented with ankle and knee arthritis and hypercalcemia. Radiographs revealed small lytic lesions and densitometry showed severe osteopenia. Quantimetric bone biopsy confirmed major bone loss and showed dramatic increase in bone turnover, as well as an unusual periosteal apposition of woven bone. Short term treatment with prednisone and furosemide and longterm treatment with clodronate allowed rapid improvement. After one year, the patient remains clinically asymptomatic. Despite negative immunologic investigations to sustain the hypothesis of HBV vaccination as a causal factor, we believe the bone lesions could be attributed to unusual bone "hyperremodeling" triggered by an immune process in a predisposed individual.
Malignant angioendotheliomatosis is an intravascular proliferation of tumour cells in the small arteries, veins and capillaries. Its lymphomatous origin has been proved recently by immunohistochemistry. It is a rare, generally rapidly fatal condition. Less than 150 cases have been reported in the literature. We report three cases. Long course fever with delayed neurologic disturbances were observed in the first case and long course fever, hyponatraemia, corticotropic and gonadotropic insufficiency in the second. The third case began with febrile meningo-encephalitis. Diagnosis was made at autopsy in the two first cases and on evidence from kidney needle-biopsy in the third. Clinical manifestations are polymorphic and frequently simulate vasculitis. Neurologic signs are the most common. Histologically, kidneys, skin, endocrine glands, lungs, muscles and most of the organs may be involved. Surprisingly lymph nodes, bone marrow and spleen are often free from disease. Current treatment is based on polychemotherapy. Earlier diagnosis of the disease should improve the prognosis as observed in our third patient.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
The authors report eleven cases of multiple auto-immune syndrome, concerning a total of 15 different auto-immune diseases. This study suggests that auto-immune thyroid disorders (Graves' disease or hypothyroidism resulting from Hashimoto's thyroiditis) are a common feature of multiple auto-immune syndromes, while antithyroid antibodies are constant among our patients. Sjögren's syndrome is also very prevalent, and seems to be non-randomly associated with auto-immune thyroid disorders, a fact which suggests common aetiological factors. Among other auto-immune disorders, the antiphospholipid syndrome has been diagnosed twice. A genetic predisposition to multiple auto-immune syndrome is obvious, as shown by a familial history of auto-immune disorders (found in more than half of the cases), and a predominant HLA phenotype, B8 and/or DR3. The authors plead for a systematic enquiry for multiple auto-immune syndrome in patients with auto-immune thyroid disorders and a family history of auto-immune disorders.
Explore the source record for details and available documents.
Researchers in the field of chronic fatigue in tertiary care found that patients' somatic (e.g. viral) explanations for their condition may lead to chronicity of symptoms. We studied the influence of a somatic attributional bias on outcome and reported symptoms in primary care patients with fatigue. We compared fatigue scores on a specific scale, and number of presented symptoms, in two groups of primary care patients with 'functional' fatigue: 75 with a high score on the somatic subscale of the Fatigue Attribution Scale (S-FAS), and 95 with a low score on the S-FAS. At the index visit, patients with low and high scores on the S-FAS were not different for age, sex, fatigue scores, and levels of depressive symptoms. Patients with high scores on the S-FAS presented significantly more somatic and psychological symptoms-a total of 36 symptoms for 24 patients (25.3%) in the low-score group, and a total of 52 symptoms for 31 patients (41.3%) in the high-score group. Forty-two days later, at the follow-up visit, the fatigue scores were similar in both groups. In primary care patients with fatigue not due to somatic illness or major depression, the tendency to attribute fatigue to somatic causes is not associated with a worse outcome, but with a higher number of reported symptoms.
We report a case of granulomatous hepatitis in a patient with hyperthyroidism resulting from Graves' disease. A 30-year-old man presented with massive weight loss, jaundice, tachyarrhythmia and goitre. Liver function tests showed mild cytolysis and cholestasis and massive hyperbilirubinaemia. The echogram of liver and bile ducts was normal and no infection was found. A liver biopsy revealed a mixed cytolytic and cholestatic hepatitis with intralobular epithelioid granulomas. No specific cause was identified, and sarcoidosis and primary biliary cirrhosis were ruled out. The outcome was favourable with antithyroid therapy and short-term glucocorticoid therapy, and the patient was totally free of symptoms after 2 years. To our knowledge, this is the first case of granulomatous hepatitis to be reported in association with Graves' disease. The clinical evolution of the liver disease paralleled the evolution of hyperthyroidism.
The authors report a case of giant-cell arteritis involving the digestive tract, revealed by a subocclusive syndrome. It appeared to be an unusual pattern according to the location, the tumour aspect and the histologic appearance of a periarteritis nodosa type. The digestive involvement and the histologic appearance are discussed in a review of the literature, as well as the link with the periarteritis nodosa.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
OBJECTIVE: Recent studies have suggested that androgen secretion by ovarian virilizing tumours may be gonadotrophin dependent. The aim of this study was to investigate the suppressive effect of GnRH agonist administration on androgen secretion in women with such tumours. DESIGN AND PATIENTS: A single i.m. injection of D-Trp-6-GnRH (GnRHa), 3.75 mg, was given to five unrelated patients referred for clinical symptoms of virilization with plasma testosterone (T) levels greater than 7 nmol/l but with normal dehydroepiandrosterone sulphate (DHEAS) levels. Diagnoses of adrenal tumour or a non-classical 21-hydroxylase deficiency were screened for by the dexamethasone suppression test, ACTH stimulation test and adrenal CT scanning, and were ruled out in all patients. The one premenopausal patient received cyproterone acetate in a dose of 50 mg twice daily for 3 weeks, starting 1 week before GnRHa administration. MEASUREMENT: Testosterone, androstenedione (A), DHEAS, 17-hydroxyprogesterone (OHP), LH and FSH plasma concentrations were measured by radioimmunoassay of blood samples taken before and 3 weeks after GnRHa. RESULTS: In each patient, GnRHa suppressed gonadotrophin levels and reduced T and A to the range for normal control women. With these results, and because accurate localization of an ovarian androgen secreting tumour could not be achieved by pelvic ultrasonography and CT scanning, exploratory laparotomy was undertaken. A Sertoli-Leydig cell tumour was found in the premenopausal patient, and granulosa cell tumour, hilus cell tumour and two hyperthecoses in the four post-menopausal patients. After bilateral ovariectomy and hysterectomy in the post-menopausal woman and after unilateral ovariectomy in the premenopausal women, androgen levels were normalized. CONCLUSIONS: In virilized women, the findings of increased serum testosterone with normal gonadotrophin levels and GnRHa suppression of gonadotrophins leading to normalization of testosterone levels, suggest that various ovarian androgen-secreting tumours, as well as hyperthecosis, are not autonomous but apparently depend upon continuous gonadotrophin stimulation.