Biomedical subjects
Publications and source records attributed to B Goichot.
We report here a case of primary hepatic anaplastic large cell Ki-1 non-Hodgkin's lymphoma in a 60 year old patient followed for hereditary hemochromatosis.
The effects of smoking on the thyroid gland have been studied for years. The consequences of smoking on thyroid function and size are however still controversial. It is accepted that an increase in serum thyiocyanate, a potent inhibitor of iodine transport, may contribute to the development of thyroid dysfunction, particularly in geographical areas with borderline iodine deficiency. In the general population, smoking is associated with normal thyroid hormone levels, with a tendency to lower TSH levels and enlargement of thyroid size. There is an increased risk of developing over thyroid disease. The relationship between smoking and relapse of Graves' disease and the incidence and severity of Graves' ophthalmopathy are constantly reported. Smoking also has a potent nodular goitrigenic effect in low iodine areas. Despite the fact that smoking decrease both thyroid secretion and thyroid hormone action, hypothyroidism does not appear to be more frequent. Smoking does however increase the metabolic effects of hypothyroidism. Active and passive smoking have proven deleterious effects on the fetal thyroid. Paradoxically, there is evidence of reduced risk for thyroid cancer in smokers.
PURPOSE: To clarify the physiological function of dehydroepiandrosterone (DHEA), the most abundant steroid in human plasma, which remains poorly understood. To analyse the beneficial effects of a supplementation in order to alleviate its decrease in ageing and improve well-being. CURRENT KNOWLEDGE AND KEY POINTS: DHEA (and its sulfate) acts on peripheral tissues as an androgenic and estrogenic precursor. It is also considered as a neurosteroid. DHEA administration in several pathological animal models is promising, especially in metabolic diseases such as obesity and insulin resistance. It appears like a factor of immunomodulation and facilitates cognitive acquisition. In humans there is little evidence that DHEA may be useful in characterized pathologies apart from adrenal insufficiency. An interesting effect was also noted in severe systemic lupus erythematosus. The effects on cognitive and neuropsychiatric diseases such as midlife dysthymia are not yet convincing. Prospective studies of supplementation versus placebo indicate inconstant improvement in well-being in the post-menopausal state. DHEA is not a panacea against ageing despite there being a well-established aging-related decrease of DHEA. Contrary to some assertions there are no proven relations between cardiovascular or cancer risk. FUTURE PROSPECTS AND PROJECTS: Until now adrenal insufficiency has been the only well-documented indication of an oral DHEA supplementation. However, DHEA may be a good way for androgen supplementation in menopausal men. Further investigations are needed to better know the anti-inflammatory and immunomodulation properties of DHEA. At the least, prospective studies on large populations are necessary to assess the true benefits and dangers of DHEA in prevention of ageing.
PURPOSE: Unexplained inflammatory syndrome is a frequent and worrying condition in Internal Medicine. However, the long-term clinical outcome of these patients cannot be inferred from the literature. The aim of this study is to describe the long-term follow-up and the prognosis of a group of patients hospitalised for an inflammatory syndrome and discharged without causal diagnosis. METHODS: This retrospective study was carried out on 46 patients, 15 men and 31 women, aged 21 to 90 years, hospitalised between 1992 and 1999. Data concerning the hospital stay were obtained from the patients' medical record. Follow-up was performed by consulting the treating physician. RESULTS: The prognosis of these patients is fairly good. In one third of the cases, the inflammatory syndrome resolved spontaneously (n = 13). In the second third, a definite diagnosis was established after discharge (n = 14) and consisted mainly of chronic inflammatory diseases (n = 9), cured with a specific treatment. In the remaining third (n = 12), the inflammatory syndrome persisted, in clinically asymptomatic patients. CONCLUSION: These results suggest that the persistence of an inflammatory syndrome is not a poor prognostic factor. Thus we propose for patients discharged with an undiagnosed persistent inflammatory syndrome despite thorough investigations, a simple clinical and biological follow-up instead of repeated etiological investigations.
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BACKGROUND: The standard treatment for cobalamin (vitamin B(12)) deficiency involves regular intramuscular cobalamin injection. It has been suggested that oral cobalamin therapy may be effective for treating patients who have food-cobalamin malabsorption. SUBJECTS AND METHODS: We prospectively studied 10 patients with cobalamin deficiency and well-established food-cobalamin malabsorption who received 3000 microg or 5000 microg of oral crystalline cyanocobalamin once a week for at least 3 months. Complete blood counts and serum cobalamin, homocysteine, and folate levels were determined at baseline and after 3 months of treatment. Patients were reexamined after 6 months. RESULTS: After 3 months of treatment, all patients had increased hemoglobin levels (mean increase, 1.9 g/dL; 95% confidence interval: 0.9 to 3.9 g/dL;P <0.01 compared with baseline) and decreased erythrocyte cell volume (mean decrease, 7.8 fL; 95% confidence interval: 0.9 to 16.5 fL;P<0.001). However, 2 patients had only minor, if any, responses. Serum cobalamin levels were increased in all 8 patients in whom it was measured. CONCLUSION: Our findings suggest that moderate doses of crystalline cyanocobalamin given orally may be an effective treatment for food-cobalamin malabsorption.
INTRODUCTION: Hypokalemia is the most frequent electrolytic disturbance in hospitalized patients. It is sometimes familial. Careful clinical and biological evaluation may guide further genetic analysis. CURRENT KNOWLEDGE AND KEY POINTS: Genetic hypokalemia is linked to disorders of mineralocorticoid hormone synthesis or action (glucocorticoid-remediable hyperaldosteronism, congenital adrenal hyperplasia, apparent excess of mineralocorticoids), to renal tubular disorders (Liddle's syndrome, Bartter's and Gitelmann's syndrome, tubular acidosis) or to disorders of cellular transfer of potassium (hypokalemic periodic paralysis). FUTURE PROSPECTS AND PROJECTS: Molecular mechanisms of adult Bartter's syndrome are probably different from pediatric syndromes. A better clinical and biological evaluation with longitudinal follow-up could allow significant progress in the knowledge of the natural history and prognosis of these syndromes.
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OBJECTIVE: The aim of this study was to report our experience with autoimmune neuropathies associated with hematological disorders and to describe their etiological and clinical polymorphism. PATIENTS AND METHODS: A retrospective study was conducted in five patients with autoimmune peripheral neuropathies with anti-MAG (myelin-associated glycoprotein) antibodies. RESULTS: Autoimmune neuropathies were associated with Waldenström's macroglobulinemia (n=2), Hodgkin disease (n=1), chronic lymphocytic leukemia (n=1) and idiopathic polyclonal B lymphoproliferation (n=1). Most of the patients had a sensorial polyneuropathy, predominant in the legs, exhibiting slow progress. Our patients showed a disappointing response to chemotherapy with stabilization or short response.
OBJECTIVE: Certain liver test abnormalities have been described in adult Still's disease. The objective of the present study was to analyze their type and frequency. PATIENTS: In a 10 year retrospective study, patients were included if they fulfilled Kahn's and/or Yamaguchi's diagnostic criteria (median follow-up: 6.5 years). RESULTS: Twelve patients were selected. The median age was 25 years old and the sex ratio H/F was 2.7. Fever was present in 100% of patients and hepatomegaly in 41%. Liver test abnormalities were identified in 92% of patients: moderate cytolysis (level of transaminases between 2 and 5 N) (83%), severe cytolysis (level of transaminases > 5 N) (17%), cholestasis (elevated levels of GGT and/or alkaline phosphatase) (75%), and an increase in the LDH level (41%). All these liver abnormalities resolved spontaneously or during treatment (83%), within a median of 18 days. CONCLUSION: Our study confirms the high frequency of liver test abnormalities (> 2/3 of the patients) in adult Still's disease. These abnormalities are generally moderate and asymptomatic (3/4 of the cases), but severe cytolysis may exist. This emphasizes the need to consider a diagnosis of adult Still's disease in the presence of fever and elevated transaminase activity.