Phéochromocytome révélé par un syndrome inflammatoire isolé

Among the atypical forms of phaeochromocytoma the isolated inflammatory form is rare and difficult to diagnose clinically. The authors report such a case, where computerized tomography and magnetic resonance imaging contributed enormously to the diagnosis.     

Cholestérolémie et régression de l'athérosclérose

Clinical studies of primary prevention of atherosclerosis by medicinal treatment of hypocholesterolaemia have shown a very favourable effect on coronary mortality and morbidity. Experimental studies and incidental clinical data suggest that this benefit is due not only to slowing down in the progress of coronary atherosclerosis lesions but also to their regression. This has now been clearly demonstrated by prospective randomized coronary angiographic studies. Regression of atherosclerosis can be obtained either by medicinal treatment, as in the Cholesterol Lowering Atherosclerosis Study (CLAS) or the Familial Atherosclerosis Study (FATS) or by deep changes in feeding habits and life style, as in the Life Style Heart Trial. Regression, however, requires a substantial reduction of cholesterolaemia (−40% of LDL-cholesterol) for a prolonged period. This approach seems to be effective against advanced arterial lesions. These results are in favour of active treatment of hypercholesterolemia, even in patients with symptomatic coronary disease, but one must bear in mind the need for primary prevention based on early detection and treatment of hyperlipidaemia and correction of associated risk factors.     

Élimination biliaire du céfotaxime et du désacétylcéfotaxime

The biliary excretion of cefotaxime (CTX) and its metabolite desacetylcefotaxime (DSCTX) was measured by HPLC in 9 recently cholecystectomized-patients following the IV injection of 15 mg/kg body weight of CTX. The totality of bile was collected by an original procedure: the inflated balloon of a Fogarty catheter was introduced into the distal branch of a Kehr drain T-tube. Biliary clearance of CTX and DSCTX was measured for 8 h. Cefotaxime peaked at 90 min. after injection at 34.5 +/- 15.3 micrograms/ml; in the 7-8 h sample it was 2.7 +/- 1.7 micrograms/ml. DSCTX peaked at the same time at 49.3 +/- 17.0 micrograms/ml, and was 4.6 +/- 3.2 micrograms/ml at 8 h. The bile/serum ratio of CTX and DSCTX concentration was 1 from the 1st to the 8th hours (range: 1.35 +/- 1.08 to 11.0 +/- 3.1). The biliary clearance of CTX was 0.190 ml/min. The total amounts of CTX and DSCTX eliminated in bile were respectively 1050 +/- 472.8 micrograms and 1902.7 +/- 804.1 micrograms (0.093 +/- 0.041 p. 100 of the dose and 0.186 +/- 0.077 p. 100 of the dose). Considering the minimum inhibitory concentration of the pathogens currently encountered in the biliary sepsis, CTX should be a suitable antimicrobial agent for the treatment of biliary infections.     

Lymphome non hodgkinien Ki-1 positif révélé par des ulcérations cutanéomuqueuses et une glomérulonéphrite

Malignant lymphoma particularly of T phenotype can be associated with specific or non specific cutaneous lesions. These cutaneous manifestations can occur at the onset of the disease being sometimes the revealing sign or they can appear during the course of the lymphoreticular malignancies. Glomerulonephritis was also described in lymphoma. Ki- positive large cell lymphoma was recently identified. A new case is reported with lymphadenopathy and intestinal localisation revealed by cutaneous and mucosal ulcerations principally in the mouth and a focal segmental glomerulonephritis with endo- and extracapillary proliferation. The absence of lymphoma in cutaneous and renal lesions and the clinical presentation support the hypothesis of paraneoplastic manifestations, may be related to a vasculitis.     

Photosensibilité du lupus érythémateux

Photosensitivity is one of the ARA diagnostic criteria of systemic lupus erythematosus. Sun exposure can also induce extracutaneous manifestations of the disease. Photosensitivity may be difficult to prove by history taking in lupus patients, as the delay between sun exposure and the onset of specific skin lesions is rather long. Photo-induction of lupus can occur by ultraviolet A (UVA) radiation in the shadow or behind window glass, so that the relationship between radiation exposure and exacerbation of the disease may not seem obvious to the patient. Phototesting procedures for lupus erythematosus have been described, but they are not used in routine practice. Both UVB and UVA play a role in the pathogenesis of lupus erythematosus: in the epidermis they induce DNA damage, they expose nuclear antigens and photo-induced neo-antigens at the cell surface, they lead to an accumulation of apoptotic material, and they induce several pro-inflammatory cytokines. In the dermis, UV radiation triggers skin infiltration by inflammatory cells by modulation of microvascular flow rates and by upregulation of white blood cell migration from dermal capillaries to the skin. Photodistribution of skin lesions and a delay of their onset of more than 48 hours after sun exposure are clinical hallmarks of cutaneous lupus erythematosus that are usually completed by histological confirmation. Photoprotection is essential in the treatment of lupus patients: it comprises sun avoidance suitable for both UVB and UVA radiation, protective clothing, and topical broad-spectrum filters.     

La deuxième génération des antisécrétoires gastriques: l''oméprazole

Omeprazole is the first representative of a new class of gastric acid secretion inhibitors. It acts by specific and prolonged inhibition of H+K+ ATPase, or proton pump, which is the terminal and compulsory stage of acid secretion by the parietal cells. The drug therefore inhibits both basal and stimulated secretion. It appears from therapeutic trials that omeprazole administered in doses of 20 mg a.m. once a day is superior to the H2-receptor antagonists in the healing of active duodenal ulcers and ulcerated reflux oesophagitis. It is as active or slightly more active than histamine inhibitors in gastric ulcers. Finally, it is the treatment of choice to prevent ulcerations in Zollinger-Ellison syndrome. These are the 4 indications for omeprazole at the moment. The use of that drug for periods of a few weeks has not given rise to any significant side-effect.     

State of the art in muscle glycogenoses

The recognition of a series of metabolic/enzymatic dysfunctions in glycogenoses has allowed new therapeutic advances for their treatment due to the development of recombinant enzyme. A recent advance appears enzymatic replacement therapy (ERT) in glycogenosis type II in both infantile, juvenile and adult form. Targeted manipulation of diet has been tried both in glycogenosis type II (Pompe disease) and type V (Mc Ardle disease).Key words: Glycogenosis, Pompe disease, McArdle disease, enzyme replacement therapy     

Insuffisance hépatique aiguë révélatrice d’un carcinome pulmonaire avec métastases hépatiques

Liver failure as a result of neoplasia is a rare event before the terminal stage of the illness. We report a 66-year-old man who presented with clinical features of acute liver failure as the initial manifestation of a small-cell lung carcinoma. Liver was enlarged without ascitis. Abdominal CT-scan revealed a massive hepatomegaly with multiple low-density wedge-shaped lesions. The patient developed stage 3 hepatic encephalopathy and died on day 4. The diagnosis was obtained with post-mortem study. A Medline search of acute liver failure due to small-cell carcinoma identified only 17 cases already published, with a universally poor prognosis.     

Intérêt de l''exploration de l''auto-immunité biologique en pathologie gynéco-obstétricale

The relationship between autoimmunity and obstetrical pathology is reviewed in the light of data from the literature and of the authors' own experience. The link, already demonstrated, between recurrent spontaneous abortion and the risk factor represented by antibodies directed against phospholipids (circulating anticoagulant antibodies of the lupus type, anticordiolipin antibody) is described, and the pathogenetic theories concerning these antibodies are presented, together with the treatments used for routine prevention of the foetal and maternal risk (corticosteroids, anti-platelet drugs, heparin). The results of screening for antinuclear antibodies in 354 non-pregnant women are reported. Antinuclear antibodies were present in titers of 1/100 in 4.2 percent and 1/50 in 8.7 percent of these women. In the same category of women the frequency of antithyroid antibodies was as high as 12 percent. These data should encourage routine examination for auto-antibodies before and during pregnancy in order to evaluate and prevent the obstetrical risk to which these women are exposed.     

Réaction éosinophile leucémoïde au cours d'une adénopathie angio-immunoblastique

A case of eosinophilic leukemoid reaction is described. This picture is seldom encountered during the course of angio-immunoblastic adenopathy. After reviewing various diagnoses and pointing out similar cases of the literature, the authors discuss the possible pathophysiologic mechanisms of cosinophilia in this disorder.