Arthrogryposis accompanying congenital spinal-type muscular atrophy.
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Biomedical subjects
Publications and source records attributed to A Behar.
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It is still too soon to make a full assessment of the Chernobyl disaster, but one should be begun, and practical inferences, with particular reference to the prevention of radiation-induced cancers, may be drawn.
On April 26, 1986, after partial fusion and confining loss by explosion of a nuclear reactor, 5 x 10(7) Ci of radionuclides escaped from Chernobyl. Three years later, maps show contamination by radioactive isotopes (formed during that period) of 21,000 km2 of Soviet soil, mainly in Byelorussia and part of the Ukraine. Decontamination measures have not been effective to date and 135,000 persons are being followed medically, taking into account the radioactive doses they received. An initial excess of morbidity from solid tumors has been noted much sooner than in the case of Hiroshima and Nagasaki, but its significance is in dispute. Three years later, only the extent of the ecologic disaster caused by the radioactive contamination can be confirmed. It is too early to draw conclusions about radiation-induced carcinogenesis for the contaminated population.
Primary Malignant Fibrous Histiocytoma (M.F.H.) of the breast proper is a very rare tumour. From the limited number of cases reported so far (22 including the present one), it seems that a rather high rate of local recurrence and lung metastasis might be expected, and that there is still uncertainty as to the most adequate treatment of the tumour. Therefore, the need for regional node dissection appears justified and we describe herein a case treated by breast quadrantectomy and axillary lymph node dissection.
Microcirculatory disorders are often a complication of diabetes. They are associated with an increased capillary permeability which can be assessed by the Landis test using 99mTc-labelled albumin. Labelled albumin retention is measured by external detection (residual radioactivity after removal of venous tourniquet). The Fast Fourier Transform of the radioactivity disappearance graph reflects the lymphatic albumin resorption. Thirteen out-patients with diabetes from 1 to 19 years suffering from evident microcirculatory disorders were treated with a micronized flavonoid fraction: Daflon 500 mg (2 tablets per day) during a month. Antihypertensive and/or antidiabetic treatment were continued during the study if they were given before starting. A test was performed before, and at the end of the treatment. In 10 of the 13 patients, the test was carried out a third time 5.5 +/- 1.0 months later, after treatment withdrawal. Results were as follows: 1) labelled albumin retention was abnormal (greater than or equal to 8%) at the start and became normal in the patients and improved in 1 patient. After treatment withdrawal, the results of the third test were again abnormal, in the patients who had been normalized beforehand; 2) the initially abnormal lymphatic resorption became normal in six of the 13 patients at the end of the treatment and became abnormal again in all patients after treatment withdrawal. In a parallel group of 15 patients with abnormal test lymphatic fluctuations and not being treated with a vasculo-protector agent, the initially abnormal test remained abnormal 5 to 48 months later in all patients.(ABSTRACT TRUNCATED AT 250 WORDS)
The Tc99m albumin test can be used to confirm idiopathic cyclic oedema (ICO) syndrome by a pathological retention of albumin of more than 8% of the maximum level, following release of the tourniquet (results obtained in 420 patients compared with 100 healthy women). This test was completed by a study involving double labelling using I131-albumin and Tc99m red cells in ten healthy subjects and ten female patients with untreated ICO syndrome. The tests were repeated at a six week interval. Evaluation of retention levels after tourniquet release showed nil retention of red cells in both populations and nil albumin retention in the healthy subjects whilst it was pathological in the patients with ICO. The curve of decreasing radioactivity was often marked by irregular oscillations after tourniquet removal. Study of these oscillations by the Fast Fourier Transform revealed reproducible abnormalities in the low frequency zone only, between 37 and 28 mHz in the ICO patients. These abnormalities took the form of more frequent peaks with higher amplitudes than in the healthy subjects and were linked--in the case of excessive leak of albumin from capillaries--to lymphatic resorption. In the high frequency zone between 630 and 39 mHz, results corresponding to variations related to arteriovenous compliance were similar in both populations and by both tests. A double blind placebo-controlled trial in 30 patients with ICO was carried out to study the pharmacodynamic activity of a flavonoid, Daflon 500 mg (2 tabs daily for 6 weeks), which revealed a decrease in the degree of retention--initially high--of labelled albumin (p = 0.01).(ABSTRACT TRUNCATED AT 250 WORDS)
The purpose of this trial was to evaluate the clinical effect of Veinotonyl 75 in 42 women, 42 +/- 11 years old, with orthostatic cyclic edema. The main evaluation criterion was the result of a capillary filtration test with 99 mTc albumin which was performed at day 0 and at day 30 after treatment. At day 0, all the women had abnormal results ie an albumin retention > or = 8% (mean: 14.3% +/- 4.8%). At day 30, 75% of the patients were normalized in the Veinotonyl 75 group versus 38% in the placebo group (p < 0.02). The albumin retention in Veinotonyl 75 group was under the pathological level (mean: 5.2% +/- 4.8%) while it was over in the placebo group (mean: 9.4% +/- 7%). This study confirms the activity of Veinotonyl 75 on capillar protein hyperfiltration and on return venous circulation.
Microangiopathic disorders, characterized by capillary vasodilation and increases in capillary blood flow and permeability, are common in diabetes and can occur before the development of microangiopathic complications. In a study of 163 diabetic patients, capillary permeability, measured by albumin retention (AR), was increased in 39% of patients. AR was increased more frequently in women than in men, and in patients without microangiopathic complications than in patients with complications. Increased AR was significantly associated with insulin-dependent diabetes in male patients. Lymphatic function was abnormal in 72% of patients; this abnormality was often present before AR increased. The pathophysiology of microangiopathy is complex and involves metabolic, haemodynamic, neurological and hormonal factors. Improved control of glycaemia and blood pressure can reduce capillary permeability. In addition, studies with a flavonoid fraction and n-3 polyunsaturated fatty acids suggest that these agents may also be beneficial.