Focal liver hyperplasia in a patient with Alagille syndrome: Diagnostic difficulties. A case report.

INTRODUCTION: Alagille syndrome is a multisystem autosomal disorder. The main clinical features are chronic cholestasis due to paucity of intrahepatic bile ducts, which can progress to cirrhosis and liver failure. PRESENTATION OF CASE: A 15 year-old girl with Alagille syndrome was referred for liver transplantation. She developed severe cirrhosis with refractory ascites. In the pre-transplant evaluation, imaging studies disclosed liver atrophy with a high density pseudotumor in the segment 4, raising the possibility of a hepatocellular carcinoma. However, behavior of the lesion was highly suggestive of focal compensatory hyperplasia surrounded by an atrophic liver. The patient was registered on the waiting list. DISCUSSION: Hepatic lesions have been described in Alagille syndrome in isolated case reports, and most of these have been reported to be hepatocellular carcinoma. However, they can be related to an area of focal compensatory hyperplasia in severe cirrhosis. These findings may also explain why progression of liver disease occurs only in 15% of patients. CONCLUSION: The presence of a large hepatic nodule Alagille syndrome can be benign in these patients also predisposed to hepatocellular carcinoma. Therefore, cautious evaluation with magnetic resonance imaging study before liver transplantation is mandatory.

[Monoclonal gammopathy and primary colonic mantle cell lymphoma]. / Une gammapathie monoclonale associée à un lymphome colique primitif à cellules du manteau.

The association of a monoclonal gammopathy (MG) with a B cell non-Hodgkin's lymphoma (NHL) is a well-known phenomenon. It has been recognized in many subtypes of primary gastrointestinal lymphoma but its association with primary colonic mantle cell lymphoma has never been yet described. We report a 65-year-old man who presented with an exudative ascites and constipation. Serum electrophoresis showed a monoclonal peak in the gamma region of 45g/L and immunoelectrophoresis confirmed the presence of monoclonal gammopathy of IgM kappa type. Bone marrow aspirate was normal. Radiologic and endoscopic investigations evidenced a primary colonic mantle cell lymphoma. Although the association of an MG with an NHL and, in particular, to a primitive digestive location appears a rare phenomenon, endoscopic investigations in patients with MG appears legitimate in the presence of any digestive sign.

UV-induced modification of stress distribution in optical fibers and its contribution to Bragg grating birefringence.

This paper discusses the importance of stress-induced contributions to the photo-induced birefringence observed in fiber Bragg gratings. Optical tomography measurements are performed in exposed and unexposed fibers to extract the stress profiles induced by UV-writing of fiber Bragg gratings for various exposure levels. A photoelastic analysis and a high-order isoparametric finite elements method are then used to calculate the birefringence caused by stress profile modifications. The results are compared to the birefringence directly measured by spectral analysis of a chirped fiber grating with multiple phase-shifts. We can therefore estimate the fraction of the photo-induced birefringence due to stress-induced anisotropy following UV exposure.

[Spinal anaesthesia for Caesarean section: dose injection speed have an effect on the incidence of hypotension?]. / Rachianesthésie pour césarienne: la vitesse d'injection a-t-elle une influence sur l'incidence de l'hypotension?

OBJECTIVE: To study effect of injection rate on spinal blockade and haemodynamic of spinal bupivacaine for Caesarean section. STUDY DESIGN: Prospective and randomized. PATIENTS AND METHODS: Sixty ASA I-II patients scheduled for elective Caesarean section were randomized to receive either fast (20 seconds, group R, n=30) or slow (60 seconds, group L, n=30) spinal injection of 10 mg of hyperbaric bupivacaine 0.5% associated with 25 microg of fentanyl and 100 microg of morphine. Sensory and motor blockade and haemodynamic parameters were recorded. RESULTS: Profiles of sensory and motor block were similar in both groups. Variations of arterial blood pressure and total dose of ephedrine were not different between R and L groups. However, the number of patient with systolic blood pressure lower than 100 mmHg were significantly lower in L group than in R group (p=0.04). Incidences of adverse effects were similar in both groups. CONCLUSION: Result of the present study suggests that a slow rate of injection would induce lower incidence of hypotension induced by spinal bupivacaine for Caesarean section.

Form birefringence in UV-exposed photosensitive fibers computed using a higher order finite element method.

The effective index change and form birefringence are calculated in UV-exposed fibers using a high-order vectorial finite element method. The birefringence is compared in optical fibers with and without photosensitive inner cladding.

[Menetrier's disease: a case report]. / Maladie de Menetrier: a propos d'un cas.

Menetrier's disease is an uncommon reason of hypertrophic gastropathy. The diagnosis is suspected when clinic, endoscopic and radiologic criteria are associated and is confirmed by histologic features which are a cryptric hyperplasia atrophy of gland of the fundus and an hypertrophy of the mucosa. The main complications of this disease are neoplasia and cachexia. The treatment of Menetrier's disease is first medical. Chirurgical treatment is indicated when bleeding or neoplasia occurred. In this paper, we report the case of patient who is 41 years old which, in spite of some therapeutic problems is in conformity with literature.

[Duplicate principal bile ducts. Two case reports]. / Duplicité de la voie biliaire principale. A propos de deux cas.

The duplicity of the way biliaire main is a rare abnormality. We report two observations revealed by episodes of angiocholite. The degrades cholangiopancreatography endoscopy allowed to wear diagnosis and to practise a therapeutic gesture.

[Esophageal cancer with esophagotracheal fistula. Treatment by esophageal prosthesis. Report of two cases]. / Cancer de l'oesophage avec fistules oesotracheales. Traitement par protheses oesophagiennes. A propos de deux observations.

Esophageal cancer has a bad prognosis because of late diagnosis. The rate of Survival is very low. Palliative treatment is frequently made. The aim of the treatment is to allow feeding, and treat some complications likes breathing troubles. We report two cases of esotracheal fistulae secondary to esophageal cancer. The esophageal fistula was successfully treated by esotracheal prosthesis.

[Epidemiologic aspects of acute anterior optic neuropathies]. / Aspects épidémiologiques des neuropathies optiques ischémiques antérieures aiguës.

The authors report 34 cases of anterior ischemic optic neuropathy, in 31 patients with one complete etiological examination. The modes of revelation, the symptoms and the results of the etiological inquest are related.

[Left ventricular function in dilated cardiomyopathy. Noninvasive determination of end-systolic stress-diameter and shortening fraction-stress relations]. / Etude de la fonction ventriculaire gauche des cardiomyopathies dilatées. Détermination non invasive des relations contrainte-diamètre et fraction de raccourcissement-contrainte en télésystole.

The ejection fraction and fractional shortening are parameters of left ventricular function dependent on the conditions of load. They are not perfect indices of myocardial contractility. The study of the relationships between stress and diameter and fractional shortening and stress in end-systole provides a better means of assessing the contractile state of the myocardium. The relationships between end-systolic stress-diameter and end-systolic stress-fractional shortening were studied non-invasively in 10 normal subjects (Group I) and 7 patients with severe dilated cardiomyopathy (Group II). End-systolic longitudinal stress of the left ventricle was calculated from Grossman's formula by coupling automatic measurement of blood pressure (cuff method) with simultaneous M mode recordings guided by 2D echocardiography. The line of regression of end-systolic stress-end-systolic diameter was determined in all cases from a series of 14 points obtained after sublingual administration of 10 mg of isosorbide dinitrate. The line of regression of fractional shortening-end-systolic stress was established in both groups by using the values observed under basal conditions and at the peak of action of the isosorbide dinitrate. The following results obtained: Under basal conditions, patients in Group II had greater end-diastolic diameters (69 +/- 8 vs 49 +/- 4 cm, p less than 0.01), greater end-systolic diameters (61 +/- 8 vs 33 +/- 4 mm, p less than 0.001) and higher end-systolic stress (140 +/- 54 vs 67 +/- 13 10(3) dyn/cm2, p less than 0.001). Fractional shortening was lower in Group II than in Group I (12 +/- 5 vs 33 +/- 5%, p less than 0.001).(ABSTRACT TRUNCATED AT 250 WORDS)

[Study of the peptide hormones of the hypothalamo-postpituitary system of the dromedary, Camelus dromedarius, (Camelidae, Mammalia) of Tunisia]. / Contribution à l'étude des hormones péptidiques du système hypothalamo-post-hypophysaire du dromadaire, Camel us dromedarius, (Camelidae, Mammalia) de Tunisie.

Ocytocin and vasopressin are two nonapeptides of the hypo thalamo-Post-hypophysary system. They are isolated and purified from dromedary post-hyphophysis, freshly collected in the meat centers of the south of Tunisia. The use of three chromatographies steps were found to be essential in obtaining high Pure hormones. These steps cousist in a sephadex G25 gel filtration and two successive High performance liquid chromatographies: H.P.L.C. The analysis of the two hormones by thin layer chromatography and Radioimmunoassays, and the identification of the amino-acid composition demonstrate that camel vasopressin is a 8 arginin vasopressin but ocytocin is identic to the other hormones isolated from different animal species.