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Resume Introduction.- La survenue de tumeurs carcinoides gastriques au cours de la maladie de Biermer a deja ete rapportee dans la litterature. A propos d'un nouveau cas, nous discutons les mecanismes physiopathologiques de cette association. Cas cliniques.- Nous rapportons le cas d'une Pakistanaise de 47 ans chez laquelle, au cours du bilan diagnostique d'une maladie de Biermer, deux formations polypoides ont ete decouvertes; l'histologie a permis de porter le diagnostic de tumeurs carcinoides associees. Conclusion.- Ces tumeurs, le plus souvent latentes, sont developpees a partir des cellules enterochromaffine-like. Le diagnostic histologique est etaye par un immunomarquage positif a la chromogranine A, permettant d'affirmer l'existence d'une tumeur endocrine. L'etude des differents mecanismes physiopathologiques mis en jeu dans la genese de ces tumeurs est en faveur d'un role predominant de l'hypergastrinemie secondaire a l'achlorhydrie. Leur evolution est habituellement benigne et un traitement conservateur par exerese endoscopique ou chirurgicale est preconise.

Medical treatment of systemic idiopathic fibrosis is first based on steroid therapy, of which efficacy is well known for nearly 75% of cases of retroperitoneal fibrosis, but les certain for the other localisations of the disease. Studies on the immuno-pathological mechanism of systemic fibrosis have led to immunosuppressive drug use, such as azathioprin, cyclophosphamide and methotrexate. Tamoxifen has been tried because of its efficacy on desmoid tumors and seems to be of great interest. Attention is focused on fibrosing cervicitis which may also be treated with immunosuppressive drugs.

All the clinical, laboratory and electrocardiographic adverse events detected during 24 Phase I studies in the same unit over a 5 y period are reported here. 430 healthy male volunteers were involved, corresponding to 5488 days of follow-up. The overall incidence of adverse events was 13.5%, with a significant difference between active drug (15.3%) and placebo (7.4%) treatments. There were 69 distinct types of adverse events. Headache was the most frequent symptom (2%). There were severe adverse events in 20 cases (0.36%), with an incidence of 20/430 per subject (4.6%). There were no deaths or life-threatening events. Although the main objective of Phase I studies is to determine the maximum dose tolerated, cause-effect relationships with adverse events are hard to establish, because of the frequency of adverse events with placebo, and because of the limited number of subjects included such studies.

Resume La periarterite noueuse est une vascularite necrosante dont le diagnostic repose sur un ensemble de criteres cliniques, angiographiques et histologiques. Dans une serie de 27 malades hospitalises en Medecine Interne, nous avons fait une etude retrospective des diverses manifestations cliniques et paracliniques. Des micro-anevrysmes n'ont ete observes au cours des angiographies viscerales que dans 12,5 % des cas, et les indications de l'angiographie nous paraissent devoir etre limitees. Une vascularite necrosante segmentaire des moyennes et petites arteres n'a ete observee que sur 33 % des prelevements musculaires. La sensibilite de ce critere est faible et doit etre amelioree par la realisation systematique d'un electromyogramme, anormal chez 87 % des patients. La sensibilite insuffisante des criteres paracliniques conduit a souligner l'interet pratique des criteres cliniques proposes par Godeau et Guillevin. Cependant nous proposons trois modifications dans leur definition: remplacement des multinevrites par les neuropathies peripheriques; incorporation du livedo dans le critere cutane et adjonction d'une serologie virale positive pour le virus B ou C de l'hepatite parmi les criteres de positivite.

Resume Afin de preciser la signification d'une TSH basse en medecine interne et l'interet d'une determination systematique lors d'une hospitalisation, 732 mesures consecutives de la TSH ont ete realisees chez des malades admis pour la premiere fois. 33 d'entre eux, soit 4,5 %, avaient une TSH L et T4 L etaient normales. Dans ce groupe, un goitre a ete mis en evidence chez 7 malades; les 8 autres etaient atteints d'une affection chronique severe. Ces resultats montrent qu'une TSH L et T4 L doivent etre effectues en premier lieu ainsi que la recherche soigneuse d'un goitre. Une atteinte organique severe et plusieurs medicaments sont susceptibles d'abaisser la TSH. Au total, la prevalence d'environ 1 % des hyperthyroidies dans cette population ne justifie pas une determination systemtique de la TSH; cependant chez les femmes de plus de 60 ans, les manifestations cliniques des hyperthyroidies peuvent etre trompeuses et meconnues et la determination de la TSH doit etre largement pratiquee.

Resume Introduction La xanthogranulomatose necrobiotique est une affection cutanee rare, habituellement associee a une gammapathie monoclonale de type IgG. Les lesions cutanees atteignent le plus frequemment le visage (region periorbitaire) et le tronc. Elles se caracterisent par des nodules ou des plaques xanthomateuses indurees. Exegese Nous rapportons l'observation d'un patient presentant une xanthogranulomatose necrobiotique inhabituelle du fait de son association a une gammapathie monoclonale de signification indeterminee en raison de la presence d'IgM kappa et du fait de l'absence d'atteinte periorbitaire. Conclusion La Xanthogranulomatose necrobiotique doit etre distinguee des autres manifestations cutanees associees aux dyscrasies plasmocytaires, notamment du xanthome plan normolipemique. Elle peut etre associee a une gammapathie monoclonale de type IgM.

Resume Nous rapportons l'observation d'une thrombose de l'artere splenique, survenant chez une femme de 54 ans apres une prise prolongee de contraceptifs oraux. Le diagnostic de cette affection, difficile cliniquement, a ete confirme par le scanner et l'arteriographie selective. La presence d'anticorps antiethinylestradiol dans le serum est discutee comme un facteur de risque des thromboses liees a la contraception orale.

Two new cases of eosinophilic ascites and a brief review of 40 cases found in the literature are presented. In three quarters of the cases eosinophilic ascites affects women aged 40 years on average. Because the patients present with a history of allergy (55%), blood hypereosinophilia (69%), associated pleural effusion (11%), gastrointestinal disorders and, above all, eosinophilic infiltrations in the walls of the digestive tract or the serous membranes (63%), this pathology may be regarded as a clinical form or eosinophilic gastroenteritis. The outcome is favourable in 90% of the cases; relapses occur in 26%. Is eosinophilic gastroenteritis and independent pathological entity, or should it be considered a minor clinical form of Chusid's idiopathic hypereosinophilic syndrome? The lack of decisive arguments precludes a firm conclusion.

Necrobiotic xanthogranulomatosis is a rare cutaneous disorder usually associated with monoclonal gammopathy. Most frequently, cutaneous lesions involve the face (periorbital region) and the trunk, and are characterized by indurated xanthomatous plaques and nodules.We describe a patient presenting with necrobiotic xanthogranulomatosis that was unusual, as it was associated with IgM kappa monoclonal gammopathy of undetermined clinical significance and because no periorbital involvement was observed.Necrobiotic xanthogranulomatosis should be distinguished from other cutaneous manifestations associated with plasma cell dyscrasias, such as normolipemic plane xanthoma. It can be associated with IgM gammopathy.

The occurrence of gastric carcinoid tumors during the cure of pernicious anemia has been reported in the literature. Concerning a new case, we review physiopathological data of this infrequent association.We report the case of a 47-year-old women with pernicious anemia in which gastroscopy found two small tumors developed from enterochromaffin-like cells.These tumors are usually latent and developed from enterochromaffin-like cells. The pathologic and histologic diagnosis is based on positive chromogranin A immunomarking. The physiopathological mechanism studies are in favour of the predominant part of hypergastrinemia following achlorhydria. Usually, evolution is benign and surgical or endoscopic resection recommended.

Medical treatment of systemic idiopathic fibrosis is first based on steroid therapy, of which efficacy is well known for nearly 75% of cases of retroperitoneal fibrosis, but les certain for the other localisations of the disease. Studies on the immuno-pathological mechanism of systemic fibrosis have led to immunosuppressive drug use, such as azathioprin, cyclophosphamide and methotrexate. Tamoxifen has been tried because of its efficacy on desmoid tumors and seems to be of great interest. Attention is focused on fibrosing cervicitis which may also be treated with immunosuppressive drugs.

Porphyria cutanea tarda is a metabolic disorder caused by reduced hepatic uroporphyrinogen decarboxylase activity characterized by skin lesions and liver damage. The high frequency of liver histological damage in patients with porphyria cutanea tarda led us to study hepatitis B and C viral infection which has a striking prevalence in southern Europe. We attempt here to expose different pathophysiological hypotheses: is viral hepatitis a triggering factor precipiting latent decreased uroporphyrinogen decarboxylase activity or does it directly induce the enzyme deficiency?

We report a case of the uncommon association of dermatomyositis and primary biliary cirrhosis in a causasion male of 48-year-old. Diagnosis of dematomyositis was made because of muscle weakness, loss of weight, skin telangiectasia, elevated serum concentration of creatine kinase, polyphasic low amplitudes waves on electromyography and histologic confirmation on muscle biopsy. Diagnosis of primary biliary cirrhosis was made because of elevated values of alcaline phosphatase and gamma glutamyl transferase, elevated values of type II mitochondrial antibody and compatible histological lesions on liver biopsy. We found only ten case reports associating polymyositis and primary biliary cirrhosis. We hypothesize that hepatic and muscle mitochondrial dysfonction may be involved.

Two cases of lupus dementia presented many points of particular interest: 1) the progressive installation of intellectual deterioration, inaugural for the first observation; 2) the diagnostic difficulties of neurolupus with the ARA criteria; 3) the appearance of cerebral magnetic resonance imaging with confluent hypersignals of the periventricular white matter on T2-weighted images; 4) the patholophysiological hypotheses: vascular disease? immunologic disease?; 5) the clinical improvement and SPECT amelioration for the second patient with corticosteroids.

A prospective study of deep vein thrombosis was conducted to compare diagnosis with venous echo-Doppler and phlebography in 101 hospitalized patients with symptomatic lower limbs.Phlebography was used as the reference examination to evaluate the sensitivity and specificity of echo-Doppler for lesions at all levels of the limb.Overall sensitivity was 90% and specificity 72%. The prevalence of venous thrombosis was 50% symptomatic patients and the positive predictive value of echo-Doppler was 76%. The negative predictive value was 88%. For patients with proximal signs, there were no false negatives with echo-Doppler and for those with distal signs, particularly in patients who had recently undergone surgery, were obese or had a past history of deep vein thrombosis echo-Doppler gave less satisfactory results.Echo-Doppler can be proposed as a first intention examination for the search for deep vein thrombosis in patients with clinical signs in the proximal area of the leg, while phlebography is still required for more distal areas.

Simultaneous elevation of thyreostimulin and thyroid hormones values, when associated with clinical hyperthyroidism, raises a dual problem of diagnosis and treatment. We report a case of hyperthyroidism with elevated thyreostimulin in a young adult man. The values of free triiodothyronin and free thyroxin were elevated and the thyroxin binding globulin was normal. A normal pituitary tomodensitometry, the normal values of the alpha sub-unit of thyreostimulin and the dynamic tests of thyreostimulin secretion allowed us to rule out the hypothesis of a pituitary adenoma. Non-tumoral inappropriate secretions of thyreostimulin are at present regarded as syndromes of resistance to thyroid hormones. Their biological translation is a simultaneous elevation of thyreostimulin and thyroid hormones. Peripheral resistance, which is rarely complete, finds its expression in clinical hyperthyroidism or in normal clinical condition, whereas pituitary resistance, which causes the lack of feedback on the secretion of thyreostimulin expresses itself by clinical hyperthyroidism, sometimes with goitre owing to the trophic action of thyreostimulin on the thyroid. Generalized syndromes of resistance are the most common. Our patient had a selective pituitary resistance to thyroid hormones and less than thirty cases are reported in the literature. Treatment with a beta-blocker remains indicated when facing clinical evidence for hyperthyroidism and oral triiodothyronin seems to be able to slow down the inappropriate secretion of thyreostimulin. Regarding our patient who experienced a clinical and biological relapse after the stopping of the treatment, we join many authors who recommend a prolonged treatment.

10% of young male healthy volunteers have a total bilirubin value over 20 mumol/l; thus such a value appears not relevant as screening cut off point in clinical pharmacology. This study was intended to confirm if a 27 mumol/l cut off point previously defined by the authors does not support a risk. This study dealt with 487 subjects who had together measurements of total bilirubin value and lab. tests of liver cytolysis, cholestasis or hemolysis during the selection process. 48 subjects (9.8%) had a total bilirubin value over 20 mumol/l. Correlation tests do not provide arguments of cytolysis, cholestasis or hemolysis and there was no argument in favor of Gilbert's syndrome. Out of 48 hyperbilirubinemic subjects only 22 were included in clinical pharmacology studies. In more than 60%, the total bilirubin value returned to normal spontaneously and in no case appeared a significant clinical, biological, pharmacokinetic or dynamic abnormality. Except a possible increase of slow acetylor frequency, the medical literature analysis does not show any relevant modification in metabolism, pharmacokinetics or pharmacodynamics until a 40 mumol/l value of total bilirubin. Thus, the 27 mumol/l value of total bilirubin previously proposed is confirmed as a useful limit that does not lead to an additional risk.

In retrospective and prospective studies the sera of 442 patients recruited from an Internal Medicine department were examined by acetone indirect immunofluorescence and immunoblot in search of anti-neutrophil cytoplasm antibodies (ANCA). Twenty-three patients had Wegener's granulomatosis, and 419 had various control diseases including connective tissue diseases, vasculitis and granulomatosis. The sera from 100 healthy blood donors were used as controls. Among the 23 patients with generalized (n = 15) or localized (n = 8) Wegener's granulomatosis (active in 21/23), the indirect immunofluorescence test was positive in 92 percent of those with the generalized form and in 62 percent of those with the localized form. Nine of the 13 sera examined by immunoblot (69 percent) were positive (31 +/- 57 kDa bands). On the other hand, the sera from 38 (6.9 percent) patients with control diseases were positive at immunofluorescence: vasculitis (14 cases, 50 percent), connective tissue diseases (4 cases, 14.3 percent) and miscellaneous diseases including 3 neoplasms (10 cases, 35.7 percent). Only 6 of these 28 sera showed a 1/100th titer of ANCA. The sera of all healthy controls were negative. The sensitivity of ANCA for Wegener's granulomatosis at all stages was 72.2 percent, and its specificity 94.4 percent. This study confirms the value of ANCA as third diagnostic criterion of Wegener's granulomatosis, after the clinical and histological criteria.

We investigated clinical data, sweat electrolytes and cystic fibrosis (CF) mutations in twelve patients with congenital bilateral aplasia of vasa deferentia (CBAVD) to debate arguments for diagnosing CF. Sweat chloride concentration was definitely raised in four patients. Three patients are CF compound heterozygotes. Six patients are CF heterozygotes. This result reinforces the hypothesis that white males with CBAVD might have a mild form of CE.

We report a case of splenic artery thrombosis developed in a 54-year old woman after prolonged use of oral contraceptives. The diagnosis of the disease, difficult on clinical grounds, was confirmed by computerized tomography and selective arteriography. The presence of anti-ethinyloestradiol antibodies in the serum is suspected to be a risk factor for thrombosis associated with oral contraception.

Two new cases of eosinophilic ascites and a brief review of 40 cases found in the literature are presented. In three quarters of the cases eosinophilic ascites affects women aged 40 years on average. Because the patients present with a history of allergy (55%), blood hypereosinophilia (69%), associated pleural effusion (11%), gastrointestinal disorders and, above all, eosinophilic infiltrations in the walls of the digestive tract or the serous membranes (63%), this pathology may be regarded as a clinical form or eosinophilic gastroenteritis. The outcome is favourable in 90% of the cases; relapses occur in 26%. Is eosinophilic gastroenteritis and independent pathological entity, or should it be considered a minor clinical form of Chusid's idiopathic hypereosinophilic syndrome? The lack of decisive arguments precludes a firm conclusion.

Periarteritis nodosa is a necrotizing vasculitis diagnosed on clinical, angiographic and histological criteria. We conducted a retrospective study of the various clinical and paraclinical manifestations of the disease in a series of 27 patients hospitalized in an Internal Medicine department. Visceral angiography showed microaneurysms in only 12.5% of the cases, and we consider that the indications of this method are limited. Segmental necrotizing vasculitis of the medium- and small-caliber arteries was found in only 33% of muscle biopsies. This criterion has low sensitivity and must be improved by systematic electromyography which showed abnormal results in 87% of the patients. Due to the insufficient sensitivity of paraclinical criteria, the clinical criteria proposed by Godeau and Guillevin are of great practical value. However, we suggest that their definition should be modified on three points: multineuritis should be replaced by peripheral neuropathies; livedo should be included in the cutaneous criterion, and positive serology for hepatitis B or C virus should be added to the positivity criteria.

Ehlers-Danlos Syndrome is a hereditary dysplasia of connective tissue with an abnormality in collagen synthesis. The syndrome consists of increased elasticity and fragility of the skin, increased laxity of the ligaments of the joints and fragility of the blood vessels. It is rarely associated with pregnancy but when it is several problems arise which are demonstrated in our case. Our patient had a type III syndrome (hypermobility of the joints) or a type IV syndrome (echymoses, Sack-Barabas Syndrome). In the literature the following are reported: premature rupture of the membranes with premature delivery, tears, perineal haematomas and type IV ruptures of the great vessels of the uterus. The best way of delivering and anaesthetising the patient are discussed. The prognosis depends above all on the type of the disease (25% of mothers die in type IV disease). Strict rest and Pfannestiel lower segment Caesarean section operation under general anaesthetic allowed us to achieve a favorable outcome for the mother and for her child.

The authors report the observation of one patient in whom the first administration of chemotherapy with fluoro-5 uracil has induced a severe but reversible cardiogenic shock. The latter was preceded by a prolonged, constrictive thoracic pain without myocardial infarction constitution. The cardiac toxicity of fluoro-5 uracil has been well described in the form of anginal pains receding with the discontinuation of the treatment. However, the possible occurrence of arrhythmias, of collapse and even sudden deaths leads the authors to suggest the daily clinical and electrical observation of the patients treated with fluoro-5 uractil.

In an attempt to determine the significance of low plasma thyrotropin (TSH) concentrations in internal medicine and the usefulness of systematic TSH assays in hospitals, 732 consecutive TSH measurements were performed in first-admission patients. TSH concentrations below 0.15 mU/l were found in 33 patients (4.5%) divided into 4 groups: a) in 5 patients a second assay made within 10 days of the first one showed no fall in TSH levels; b) 5 patients had known endocrine disease; c) in 8 patients hyperthyroidism could be asserted; the diagnosis had not been suspected in 3 elderly women and 1 pregnant women; d) 15 patients remained with low TSH concentrations but had normal free T3 and free T4 levels; in this group a goitre was detected in 7 patients and 8 had a severe chronic disease. These results showed that a TSH concentration below 0.15 mU/l corresponded to hyperthyroidism in less than one out of three patients in this population and that the 0.07 to 0.15 mU/l range is particularly misleading. A second TSH assay, free T3 and free T4 measurements ant thorough investigations in search of a goitre must be made. Severe organic diseases and several drugs may induce a fall in TSH. All considered, the 1% prevalence of hyperthyroidism in this population does not justify systematic TSH assays, but in subjects over 60 years of age, the clinical manifestations of hyperthyroidism may be misleading or unrecognized, and TSH assays should be widely performed.

Peripheral vascular manifestations of pseudoxanthoma elasticum (pxe): report of five cases. PXE is a rare heritable discorder of connective tissue. The cardiovascular lesions are less clearly defined than the skin and eye lesions. They can manifest at an early age and can be the first sign of the disease (3 cases). They may remain silent (3 cases) but they may be severe and at times fatal (1 case).

Authors report ten cases of localized amyloidosis exposed by surgery. Three patients have developped later a systemic amyloidosis. The others patients have a local disease (2 larynx, 1 heart valves, 1 urinary bladder, 1 sciatic nerve, 1 eyelid, 1 Fallopian tube) with an excellent pronostic.

We investigated clinical data, sweat electrolytes and cystic fibrosis (CF) mutations in twelve patients with congenital bilateral aplasia of vasa deferentia (CBAVD) to debate arguments for diagnosing CF. Sweat chloride concentration was definitely raised in four patients. Three patients are CF compound heterozygotes. Six patients are CF heterozygotes. This result reinforces the hypothesis that white males with CBAVD might have a mild form of CE.