[Corticosensitive pseudoperiarteritis nodosa. Look for cholesterol embolism and aortic aneurysm].
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Biomedical subjects
Publications and source records attributed to M Le Bras.
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A 35-year-old man, sephardic jew, complains for the last eleven years of typical and frequent attacks of FMF. His liver is hypertrophic. Needle-biopsy reveals an extensive macrovacuolar triglyceride storage (60 per cent) and an active vascular congestion with erythrodiapedesis in the mild and centrolobular zone, without any necrosis, cellular infiltration nor fibrosis. Electron microscopy shows lipofuschin deposits and mild lesions of mitochondrias, endoplasmic reticulum. Blood triglycerides and apo B are rather low. After six weeks of colchicine treatment, needle biopsy shows no more active congestion nor erythrodiapedesis. Triglyceride storage lowers to 40 per cent. After seven months of colchicine treatment, triglyceride storage falls down to 12 per cent. FMF may be considered as a cause of fatty liver when there is not any cause else and only after deep decrease or disparition of triglyceride deposit by a long time colchicine treatment.
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A 21-year-old man presented with a 10-year history of a malabsorption syndrome of moderate severity, splenomegaly, and recurrent respiratory infections. Investigations revealed total atrophy of the villi and primary agammaglobulinemia. A gluten-free diet was ineffective. In spite of the absence of lambliasis, treatment with metronidazole produced objective clinical improvement and biological signs of healing of the malabsorption syndrome, but no alteration in the agammaglobulinemia. The authors discuss the relationship between total villous atrophy and primary agammaglobulinemias and the mode of action of metronidazole.
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A preliminary study involving 12 subjects suffering from carcinoma of the liver indicates the value of the estimation of alpha-1 antitrypsin and the determination of its phenotype by bidimensional electrophoresis. Alpha-1 antitrypsin was greater than 3.3 g/l in all cases, with a normal of 2.5 g/l. The MZ phenotype was found in 9 cases out of 12, the MM phenotype in the three recent cases and in particular in the only case of secondary carcinoma. In the Pi system, the Z trait would seem to be a genetic risk factor in primary carcinoma of the liver. Determination of the phenotype of alpha-1 antitrypsin may aid in its laboratory diagnosis.
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Constrictive endocardial fibrosis (CEF) or endomyocardial fibrosis (EMF) was studied on 14 files of this rather frequent disease in Ivory Coast. On the basis of the data derived from angiocardiography, catheterization and mechanograms, the clinical signs are interpreted better. Thus one is able to describe with accuracy some clinical forms, the commonest of which being a right heart restriction combined with left-sided signs. It seems important underline how useful the external pulse recordings might be useful for the diagnosis in view of their analogy with the pressure curves. In this disease with a severe course, bold surgical treatments provide some hope. On the contrary, its aetiology remains mysterious. Our best landmark being the link of CEP with tropical ecology, and in particular, in our cases, with the warm and damp forest ecology of some areas.
1. Study of a group of 50 patients suspected to have coronary artery disease. This is a complement to a previous study concerning "definite" coronary patients. 2. The method followed consisted in cross-examination of the files by three observers in order to separate the subjects who seemed really affected by coronary artery disease. This treble examination led to a rather restrictive selection. To facilitate the study, the patients were subdivided into 4 groups: patients with arterial hypertension, with diabetes mellitus, with cardiac failure, with a heart disease and miscellaneous patients. 3. The study of the 20 files which were discarded was peculiarly interesting as it provided the opportunity to underline the differential diagnosis either with common diseases (left ventricular overload, heart block, brain vascular accident), or with more specific diseases for Black Africa (endomyocardial fibrosis, aneurysm of the left ventricle, cardiomyopathy). In that respect, it is underlined that, in the absence of any anatomical or functional disease, the electrocardiogram of the healthy Black is identical to that of the White. 4. Study of the 30 patients considered as coronary made it possible to underline aetiological and epidemiological factors, although some are still lacking. However the facts observed could be compared with those reported in a previous work concerning 45 "definite" coronary patients. The overall documents thus gathered in 75 patients suggest that the African candidate to coronary artery diseases resembles his Occidental homologue, but that he might have kept a traditional diet.
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