Najet Bel Hadj

Encéphalopathie hépatique minime : un diagnostic précoce pour améliorer le pronostic

BACKGROUND AND AIMS Minimal hepatic encephalopathy (MHE) is the mildest form of the spectrum of hepatic encephalopathy that impairs health-related quality of life. The aim of this study is to evaluate the prevalence of MHE in patients with liver cirrhosis and analyze risk factors. METHODS Between September 2011 and December 2012, consecutive cirrhotic patients seen in our department were evaluated. Patients included were screened by the psychometric hepatic encephalopathy score (PHES) battery comprising 5 tests: number connection test A and B, line tracing test, serial dotting test and digit symbol test. Patients included were regularly followed-up for the development of overt hepatic encephalopathy, driving accidents, falls and death. RESULTS We included 45 cirrhotic patients. Etiology of cirrhosis was viral in half of cases. Child-Pugh score was A in 55.6 %, B in 26.7 % and C in 17.8 %. Median Meld score was 14. According to the PHES score, MHE was detected in 44.4 % of patients. Univariate analysis identified 4 variables significantly associated with the presence of MHE: age ≥ 55 years (P=0.031), poor educational status with years of study< 9 years (P=0.007), MELD score ≥ 15 (P=0.002) and Child-Pugh ≥ 7 (P=0.001). At multivariate analysis, the only independent risk factor of MHE was a MELD score≥15 (OR=15.4; P<0.001). During the follow-up, patients with MHE developed more often overt encephalopathy, falls and driving accidents, and had a lower survival, although the difference was not statically significant. CONCLUSION In this preliminary small series, prevalence of MHE in Tunisian cirrhotic patient was 44.4 %. A MELD score ≥ 15 was the only independent risk factor. MHE had a negative impact on the outcome, justifying an early diagnostic. Adequate therapy may improve cognitive function.

Hepatic sarcoidosis: a case series

Sarcoidosis is a systemic non caseous granulomas disease. Liver is a common location but usually asymptomatic. Evidence based guidelines for this location treatment is lacking and the effect of corticosteroids may be inadequate. The aim of our study was to describe the clinical, biochemical, radiological and therapeutic features of seven patients with systemic sarcoidosis and liver involvement. A retrospective and descriptive monocentric study, over 3 years, including seven patients with systemic sarcoidosis and liver involvement. We included 5 women and 2 men with an average age of 43 years. Hepatic localization revealed sarcoidosis in 5 cases. Hepatomegaly was observed in all patients as well as abnormal serum liver function test reflected by anicteric cholestasis. Liver biopsy, showed in all granulomatous lesions consistent with sarcoidosis and severe fibrosis in 2 cases. Extra-hepatic manifestations were present in all patients represented mainly by pulmonary location. All patients were treated, five by corticosteroid and two with ursodeoxycholic acid (UDCA). Complete response was observed in one case, partial response in another case and corticosteroid refractoriness in one case. In two cases, corticosteroid therapy was introduced for less than 1 month, not allowing assessment of response. Antimalarials in combination with UDCA were used successfully in a patient with steroid-resistant liver disease. Liver involvement can reveal systemic sarcoidois. Given the risk of progression to severe liver disease, it must be screened in all patients with systemic sarcoidosis. Treatment is not systematic, and still based on corticosteroid therapy. In the absence of prospective randomized controlled trials, the efficacy of UDCA need to be proven.

De novo autoimmune hepatitis following liver transplantation for primary biliary cirrhosis: an unusual cause of late grafts dysfunction.

De novo autoimmune hepatitis (AIH) is a rare disorder first described in 1998. It occurs in patients who underwent liver transplantation for a different etiology. We present the case of a 56-year-old woman who was diagnosed with primary biliary cirrhosis and had liver transplantation for refractory pruritis. Seven years after transplantation, she presented alterations in the hepatic profile with hypertransaminasemia, elevated alkaline phosphatase and gamma-glutamyl-transferase. Her liver functions test also showed elevated IgG levels. Serum autoantibodies were negative except for antimitochondrial antibodies. Histological findings indicated features of AIH without bile duct damage or loss. She had a pretreatment AIH score of 13 points and a post treatment score of 15 points according to the International AIH Group. The patient was treated effectively with prednisolone and her liver function and globulin levels rapidly returned to normal.

Downhill oesophageal variceal bleeding: A rare complication in Behçet’s disease-related superior vena cava syndrome

Behçets disease (BD) is a multisystemic disorder that involves vessels of all sizes. Superior vena cava (SVC) thrombosis is a rare complication that can lead to the development of various collateral pathways. A 31-year-old man presented with SVC syndrome. He had a history of recurrent genital aphthosis. Computed tomography revealed extensive thrombosis of the right internal jugular, axillary, and subclavian veins with collateral circulation. The patient was diagnosed with BD, and he was started on anticoagulation and immunosuppressive therapy. One week later, he presented with haematemesis. Upper gastrointestinal endoscopy disclosed varices in the upper third of the oesophagus with stigmata of recent bleeding. Portal hypertension was ruled out. Anticoagulation therapy was discontinued. He was discharged on immunosuppressive therapy. Bleeding from downhill oesophageal varices should be suspected in any patient presenting with upper gastrointestinal bleeding and a history of SVC syndrome due to BD.

Toxic megacolon complicating a first course of Crohn’s disease: about two cases

Toxic megacolon is a rare and serious complication of Crohn’s disease. Because of the associated high morbidity and mortality, early recognition and management of toxic megacolon is important. Through two cases of toxic megacolon complicating Crohn’s disease, we assessed the clinical, radiologic and therapeutic characteristics of this complication. A 35-year-old man presented a first course of Crohn’s disease treated with corticosteroid. He exhibited sudden severe abdominal pain and distension with shock. A plain abdominal radiography revealed toxic megacolon. He underwent medical therapy, but symptoms not relieved. The patient underwent subtotal colectomy with ileostomy. The resected specimen confirmed the diagnosis. Recovery of digestive continuity was performed. Endoscopic evaluation six months later did not shown recurrence. A 57-year-old man presented with severe acute colitis inaugurating Crohn’s disease, was treated with corticosteroid and antibiotics. He exhibited signs of general peritonitis. Computed tomographic examination revealed toxic megacolon with free perforation, showing prominent dilation of the transverse colon and linear pneumatosis. The patient underwent emergent subtotal colectomy and ileostomy. The final histological patterns were consisting with diagnosis of Crohn’s disease associated with cytomegalovirus infection. The patient underwent antiviral therapy during 15 days. Because of the high risk of postoperative recurrence, he underwent immunosuppressive therapy. Recovery of digestive continuity was performed successfully. Toxic megacolon in Crohn’s disease is a serious turning of this disease. We underscore the importance of early diagnosis of toxic megacolon and rapid surgical intervention if improvement is not observed on medical therapy.

Multiples ileal adenomas in Crohn's disease: Sporadic or ileitis associated dysplasia?

La Presse Medicale - In Press.Proof corrected by the author Available online since jeudi 29 decembre 2016

Refractory Ascites in Cirrhosis: Prevalence and Predictive Factors

Introduction: Ascitic decompensation is a common major complication of cirrhosis and is associated with a poor outcome. In 5-10% of patients, ascites become resistant to treatment (either do not respond to a high dose of diuretics or because these drugs induce complications), which is called Refractory Ascites (RA). RA is associated with poor survival: 20-50% at 1 year. Different treatments have been proposed, however, only liver transplantation can improve survival. The aims of this study were to determine prevalence and predictors of RA development in patients with cirrhosis. Methods: Retrospective study including consecutive cirrhotic patients admitted for controlling ascites between January 2010 and April 2013. Patients and cirrhosis characteristics were studied. Development of RA during followup was investigated. Predictive factors for RA development were evaluated. Results: We included 124 cirrhotic patients: 59 females (47.6%) and 65 males (52.4%) with a mean age of 58 years. Ascites was grade 3 in 38.5% and was the first episode in 45.1% of patients. Etiology of cirrhosis was mainly viral (57.3%). Child-Pugh score was B in 39.5% and C in 28.2%. Mean MELD score was 16 (6-40). During follow-up, 27 patients developed RA, meaning a prevalence of 21.8%. RA type was diuretic intractable in all cases. Predictive factors of RA development in univariate analysis were: ascites grade 3 (OR=4.17; p=0.004), Child-Pugh score C (OR=3.9; p=0.02), MELD score ≥ 15 (OR=4.99; p= 16 (OR=4.13; p=0.005), spontaneous bacterial peritonitis at the first admission (OR= 8,14; p=0.002), prothrombin time ≤ 64.5% (OR=3.36; p=0.013) and sodium urinary output ≤ 42 mmol/24 h (OR=5.13; p=0.03). In multivariate analysis, only urine sodium output was an independent predictive factor of RA development (OR= 4.74; p=0.015). Conclusion: In this present study, prevalence of RA was 21.8%. Urinary sodium output at the first admission for controlling ascite could allow early identification of patients who will develop RA.

Colectomy for porto-systemic encephalopathy: is it still topical?

Hepatic encephalopathy (HE) is a common long term complication of porto-systemic shunt. We report herein the case of a 59-year-old man with Child-Pugh A cirrhosis treated successfully 9 years earlier with distal splenorenal shunt for uncontrolled variceal bleeding. In the last year, he developed a severe and persistent hepatic encephalopathy secondary to the shunt, which was resistant to medical therapy. As liver transplantation was not available and obliteration of the shunt was hazardous, we performed subtotal colectomy in order to reduce ammonia production. This therapeutic option proved successful, as the grade of encephalopathy decreased and the patient improved. Our experience indicates that colonic exclusion should be considered as an option in the management of HE refractory to medical treatment in highly selected patients when liver transplantation is not available or even as a bridge given the long waiting time on lists.