Hepatitis B reactivation during ruxolitinib treatment

Annals of Hematology -     

Potter's reno-facial syndrome

Potter's reno-facial syndrome is a rare innate abnormality. We bring 4 observations repertoried at the maternity of military hospital of Tunis over a period of 6 years (1997 - 2002). The purpose of our work is to determine after a review of the literature the echographic and foetopathologic characteristics, and the forecast of this syndrome. The frequency of the bilateral renale agenesis is of 0.27 per thousand. Positive diagnosis bases essentially on the ultrasound of the 2th, or the 3-th trimester. The signs of appeal are essentially the oligoamnios associated to an hypotrophy. The caryotype is systematic to eliminate an associeted chromosomic abnormality. Foetopathologic exam is usefull for the diagnosis. Main abnormality except the urinary pathology is the lung hypoplasia. Therapeutic interruption of the pregnancy in this situation not compatible with the extra-uterine life., only type IV authorize the development of the pregnancy according to echographic data and of foetal urinaire biochemistry. We insist on the early practice of the morphological ultrasound between 20 - 22 weeks for the diagnosis of foetal abnormalities and the place of the genetic advice in association with the geneticist in the coverage of the couple.     

Cellular immunity in healthy volunteers treated with an octavalent conjugate Pseudomonas aeruginosa vaccine

Humoral immunity in response to an octavalent O-polysaccharide-toxin A conjugate Pseudomonas aeruginosa vaccine is well studied, and a phase III clinical study in cystic fibrosis (CF) patients is currently ongoing. In contrast, little is known about cellular immunity induced by this vaccine. Fifteen healthy volunteers were immunized on days 1 and 60. Parameters of cellular immunity were studied before vaccination on day 1, and on day 74. Analyses included flow cytometry of whole blood and antigen-induced proliferation of and cytokine production by lymphocyte cultures. The effects of immunization on the composition of peripheral blood lymphocytes as determined by flow cytometry were minor. In contrast, after immunization a highly significant increase of proliferation in response to stimulation with detoxified toxin A was noted: the stimulation index rose from 1.4 on day 1 to 42.2 on day 74 (restimulation with 0.4 microg/ml; P = 0.003). Immunization led to significant production of interferon (IFN)-gamma and tumour necrosis factor (TNF)-alpha by antigen-stimulated lymphocytes. In contrast, no significant induction of interleukin (IL)-4 or IL-10 was observed. In conclusion, immunization of healthy volunteers led to activation of cellular immunity including strong antigen-specific proliferation and cytokine production. In CF patients priming of the cellular immune system towards a Th1-like pattern would be of potential advantage. Therefore, confirmatory analyses in immunized CF patients with and without chronic infection with P. aeruginosa are foreseen.     

Expression and activity of the cytochrome P450 2E1 in patients with nonalcoholic steatosis and steatohepatitis.

BACKGROUND/AIMS: Nonalcoholic steatohepatitis (NASH) and nonalcoholic fatty liver (NAFL) have a different prognosis and should be dealt with differently. The pathogenesis of NASH implicates the overexpression of cytochrome P450 2E1 (CYP2E1). We investigated whether the noninvasive determination of CYP2E1 activity could replace a liver biopsy in order to differentiate NASH from NAFL. METHOD: Forty patients referred for suspicion of NASH underwent liver biopsy. In these patients, CYP2E1 activity was determined noninvasively by the 6-hydroxychlorzoxazone/chlorzoxazone (CHZ) ratio (CHZ test). Expression of CYP2E1 on liver slides was assessed by immunohistochemistry, and immunostaining for smooth muscle actin was used to assess the activation of hepatic stellate cells (HSC). RESULTS: Thirty patients with NASH were compared with 10 subjects with NAFL. No statistically significant difference could be identified for the clinical and biochemical parameters between the two groups. In the histology, steatosis was more important in NASH than in NAFL (P<0.0001). There was no difference either in the activity (CHZ test) or in the expression of CYP2E1 (immunohistochemistry) between patients with NASH and patients with NAFL. The degree of HSC activation was also comparable between the two groups. A positive and significant correlation was found between the activity of CYP2E1 and body mass index (P<0.001) as well as with the degree of steatosis (P=0.008). CONCLUSION: For patients suspected to have NASH, noninvasive tests including the determination of the CYP2E1 activity are unable to distinguish them from patients with steatosis.     

Cellular immunity in healthy volunteers treated with an octavalent conjugate Pseudomonas aeruginosa vaccine

Humoral immunity in response to an octavalent O-polysaccharide-toxin A conjugate Pseudomonas aeruginosa vaccine is well studied, and a Phase III clinical study in cystic fibrosis (CF) patients is currently ongoing. In contrast, little is known about cellular immunity induced by this vaccine. Fifteen healthy volunteers were immunized on days 1 and 60. Parameters of cellular immunity were studied before vaccination on day 1, and on day 74. Analyses included flow cytometry of whole blood, and antigen-induced proliferation of and cytokine production by lymphocyte cultures. The effects of immunization on the composition of peripheral blood lymphocytes as determined by flow cytometry were minor. In contrast, after immunization a highly significant increase of proliferation in response to stimulation with detoxified toxin A was noted: the stimulation index rose from 1.4 on day 1 to 42.2 on day 74 (restimulation with 0.4 microg/ml; P = 0.003). Immunization led to significant production of interferon (IFN)-gamma and tumour necrosis factor (TNF)-alpha by antigen-stimulated lymphocytes. In contrast, no significant induction of interleukin (IL)-4 or IL-10 was observed. In conclusion, immunization of healthy volunteers led to activation of cellular immunity including strong antigen-specific proliferation and cytokine production. In CF patients priming of the cellular immune system towards a Th1-like pattern would be of potential advantage. Therefore, confirmatory analyses in immunized CF patients with and without chronic infection with P. aeruginosa are foreseen.     

Intracranial and orbital hydatid cysts

Summary Parasitic diseases involving brain and orbit are relatively rare in Tunisia; the most frequently encountered is the hydatid cyst, 55 examples of which were seen since CT scanning was introduced into Tunisia 2 years ago. Various aspects of the intracerebral and orbital hydatid cysts are reported and a tentative classification is made based on characteristics revealed by CT.     

Fluindione-induced acute generalised exanthematous pustulosis confirmed by patch testing

BACKGROUND: Fluindione is an oral anticoagulant belonging to the vitamin K antagonist class. Although fluindione is very widely prescribed in France, few cases of cutaneous drug reactions have been reported. Below we describe a case of acute generalised exanthematous pustulosis due to fluindione as confirmed by patch-testing. PATIENTS AND METHODS: A 70-year-old woman was hospitalised for diffuse erythematous and pustular rash 48hours after initiation of fluindione treatment for cardiac arrhythmia. A diagnosis of fluindione-induced acute generalised exanthematous pustulosis was made. After withdrawal of fluindione, the eruption cleared up within eight days. Warfarin was then used without skin reaction. Subsequent patch-tests were positive for fluindione. DISCUSSION: These signs were consistent with fluindione-induced acute generalised exanthematous pustulosis. A causative role of fluindione is very likely in view of the rapid onset after initiation, improvement after withdrawal and positive patch tests. Skin patch-testing, which is easily performed, can be extremely helpful in determining a causal relationship with medication.