[Hepatocellular carcinoma and hyperestrogenia in a male]. / Carcinome hépatocellulaire et hyperoestrogénie chez un homme.

The case of a 38 year-old man with hepatocellular carcinoma in a non-cirrhotic liver is reported. Hyperestrogenosis with gynecomastia, polycythemia and proteinuria were present. After complete removal of the tumor, estrogen levels, red blood-cell volume and urine analysis did not return to normal and these pathologic findings were probably not paraneoplastic syndromes. Outcome was good, the patient remaining completely well without evidence of recurrence during the ensuing 30 months. The likely explanation for the persistent hyperestrogenosis was an excessive conversion of sexual hormones. The authors suggest that this abnormal hyperestrogenic state could promote hepatic neoplasia as it has been established in animals.

[Peripheral neuropathy due to N-hexane in a drug addict (author's transl)]. / Neuropathie périphérique au N-hexane chez une toxicomane.

After voluntary inhalation of a domestic solvent containing N-Hexane and N-Heptane for three months, a 23-year-old woman developed motor deficit of the lower limbs, sensory symptoms and areflexia. Clinical disorders continued to progress after discontinuation of the intoxication, with a parallel aggravation of the E.M.G. disturbances. A nerve biopsy with ultrastructural study showed axon dilatation with accumulation of neurofilaments. The clinical, electrophysiological and pathological features of neuropathies induced by hexacarbon solvents are reviewed and their pathogenesis is discussed.

[Amyloid pseudotumor of the sciatic nerve]. / Pseudo-tumeur amyloïde du nerf sciatique.

A 60 year-old man complained of numbness and pain in the right lower limb, suggesting lesions of the fifth lumbar and first sacral roots. Sixteen months later, CT showed a tumor of 3.5 cm at the emergence of the first right sacral root. Microscopic examination disclosed an infiltration of the fibers of the nerve by numerous masses of hyaline eosinophilic material which stained with Congo red and produced green birefringence under polarized light. The persistence of congophilic properties of the amyloid deposits after permanganate pretreatment suggested an immunoglobulin origin (AL). A research of amyloid deposit in others viscera: heart, kidneys, digestive tract, was negative. We believe that this is the first reported case of amyloid pseudo-tumor involving a peripheral nerve.

[Progressive lupus dementia. 2 cases with or without antiphospholipid antibodies]. / Démence lupique progressive. Deux cas avec anticorps antiphospholipides ou non.

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.

[Critical value of bilirubin in the selection of healthy volunteers in for phase I]. / Valeur seuil de la bilirubine pour la sélection des voloñtaires sains en phase I.

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.

[Association of Biermer's anemia and gastric carcinoid tumors]. / Association d'une anémie de Biermer et de tumeurs carcinoïdes gastriques.

BACKGROUND: 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. CASE REPORT: 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. CONCLUSION: 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.

[Necrobiotic xanthogranulomatosis: a cutaneous manifestation of a monoclonal IgM gammopathy]. / Xanthogranulomatose nécrobiotique: manifestation cutanée d'une IgM monoclonale.

INTRODUCTION: 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. EXEGESIS: 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. CONCLUSIONS: 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.

[Primary biliary cirrhosis and idiopathic thrombopenic purpura. A new association]. / Cirrhose biliaire primitive et purpura thrombopénique idiopathique. Une association inédite.

A case of primary biliary cirrhosis with stage III histological changes associated with an asymptomatic thrombocytopenic purpura with raised antiplatelet antibody levels is described. This new association of two conditions in which an autoimmune participation is generally accepted suggests a predisposition to this form of disease and/or the intervention of common trigger factors; however, an analysis of known etiological mechanisms does not exclude the possibility of a fortuitous association.

[Clinical study and diagnostic criteria of periarteritis nodosa. Apropos of 27 cases]. / Etude clinique et critères diagnostiques de la périartérite noueuse. A partir de 27 observations.

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.

[Is male infertility caused by congenital bilateral atrophy of the vas deferens a clinical form of mucoviscidosis?]. / La stérilité masculine par atrophie congénitale bilatérale des canaux déférents est-elle une forme clinique de mucoviscidose?

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.

[Diagnostic significance of low thyrotropin in internal medicine]. / Signification diagnostique d'une hormone thyréotrope abaissée en médecine interne.

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.

[Splenic artery thrombosis with Adepal. Pathogenic role of anti-ethinylestradiol antibody?]. / Thrombose de l'artère splénique sous Adépal. Rôle pathogène d'anticorps anti-éthinylestradiol?

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.

[Eosinophilic ascites. 2 new case reports]. / Ascites à éosinophiles. A propos de deux nouvelles observations.

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.

[New medical treatments of systemic idiopathic fibrosis]. / Les nouveaux traitements médicaux des fibroses systémiques idiopathiques.

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.

[Cardiogenic shock caused by 5-fluorouracil]. / Choc cardiogénique dû au 5-fluorouracile.

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.

[Ehlers-Danlos syndrome and pregnancy. Apropos of a case]. / Syndrome d'Ehlers-Danlos et grossesse. A propos d'un cas.

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.

[Dermatopolymyositis and primary biliary cirrhosis. A rare association]. / Dermatopolymyosite et cirrhose biliaire primitive. Une association rare.

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.

[Porphyria cutanea tarda and hepatitis B and C virus infection]. / Porphyrie cutanée tardive et infection par les virus de l'hépatite B et C.

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?

[Hyperthyroidism at elevated thyreostimulin. A case with pituitary resistance to thyroid hormones]. / Hyperthyroïdie à thyréostimuline haute. Une observation avec résistance hypophysaire aux hormones thyroïdiennes.

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.

[Venous ultrasonography coupled with continuous Doppler in the diagnosis of deep venous thrombosis of the lower limbs. Evaluation in symptomatic patients]. / Echographie veineuse couplée au Doppler continu dans le diagnostic des thromboses veineuses profondes des membres inférieurs. Evaluation chez des malades symptomatiques.

OBJECTIVE: 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. METHODS: Phlebography was used as the reference examination to evaluate the sensitivity and specificity of echo-Doppler for lesions at all levels of the limb. RESULTS: 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. CONCLUSION: 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.