M. Cheikh

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.

Budd-Chiari Syndrome: An Unusual Presentation of Multisystemic Sarcoidosis

Sarcoidosis is a multisystem granulomatous disease of unknown origin. All organs may be affected. Liver involvement is common but it is rarely symptomatic. Only a few cases of Budd-Chiari syndrome (BCS) secondary to a hepatic sarcoidosis have been described so far. We describe a case of multisystemic sarcoidosis presenting with BCS. A 42-year old female was referred to our department for chronic and anicteric cholestasis. Laboratory and imaging investigations disclosed features of chronic BCS associated with multisystemic sarcoidosis. The positive diagnosis was based on microscopic features, which showed hepatic, gastric and cutaneous non-caseating granulomas. Screening for an underlying thrombophilic disorder was negative. The diagnosis of BCS complicating hepatic sarcoidosis was the most likely. She was put on corticosteroids and anticoagulation therapy. To our knowledge, few cases of sarcoidosis-related BCS have been reported in the literature. In addition to being an uncommon presentation of sarcoidosis, this case illustrates the importance of recognizing an unusual cause of BCS and its therapeutic difficulties.

Gastric schwannoma: a case report

Schwannomas are generally benign, slow growing tumors. They are rarely observed in the gastrointestinal tract with the most common site being the stomach. These tumors are usually asymptomatic. The preoperative diagnosis via endoscopy is a challenging issue due to the difficulty of differentiation from other submucosal tumors. A 54-year-old woman presented with epigastric pain persisting for the last 10 months. Upper endoscopy revealed an elevated submucosal mass of the gastric antrum. The overlying mucosa was normal. Biopsy specimens yielded only unspecific signs of mild inactive chronic inflammation. Endoscopic ultrasound examination noted a hypoechoic homogeneous mass lesion located in the gastric antrum. The mass appeared to arise from the muscularis propria, and there was no perigastric lymphadenopathy. A contrast-enhanced computed tomography scan identified a homogeneous round mass and arising from the antrum of the stomach. Submucosal tumor was suspected and surgical intervention was recommended. The patient underwent an elective laparoscopic partial gastrectomy. The histopathologic features and immunohistochemical-staining pattern were consistent with a benign gastric schwannoma. Our patient shows no recurrence with a follow-up of one year. The definitive diagnosis of gastric schwannomas requires immunohistochemical studies. Complete margin negative surgical resection, as in this case, is the curative treatment of choice. The clinical course is generally benign.

Nodular Hyperplasia of the Gastrointestinal Tract After Liver Transplantation: Role of Immunosuppressive Therapy? A Case Report

Nodular lymphoid hyperplasia (NLH) of the gastrointestinal tract is a rare disease usually reported in patients with congenital or acquired immunodeficiency and chronic gastrointestinal infections. However, no case of NLH in a patient receiving immunosuppressive therapy has been reported to date. We describe the case of a woman who developed chronic diarrhea related to NLH 9 years after liver transplantation. Other causes of diarrhea and NLH were excluded. Her immunosuppressive regimen consisted on mycophenolate mofetil (MMF) and tacrolimus. Reduction of MMF dose improved symptoms but led to a rising aminotransferase level. Given the risk of graft rejection, MMF at full dose was resumed and she was started on symptomatic treatment for diarrhea. The role of immunosuppressive drugs in the pathogenesis of NLH may be related to the reduction of T- and B-lymphocyte proliferation and decreasing antibody production. NLH will further develop to compensate functionally inadequate lymphoid tissue, as reported in congenital immunodeficiency states.

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.