Macrophage activation syndrome during Crohn´s disease: a case report.

The macrophage activation syndrome (MAS) is a rare but potentially fatal disease. We report two cases of Crohn´s disease, one under 5-aminosalicylic acid and the other under corticosteroid, having developed MAS. The first was a male patient hospitalized for high fever, clinical examination found fever and left inguinal lymph node. The second case was a female patient hospitalized for asthenia, clinical examination found fever and oral mycosis. The biology tests revealed bi/pancytopenia, hyperferritinemia, hypofibrinogenemia. The myelogram showed hemophagocytosis. The identified infectious cause was Klebsiella pneumoniae in the urinary tract in one patient and oral Candida albicans in the other one. Thoraco-abdomino-pelvic computed tomography (CT) scan showed a thickening of the bladder in the male patient, and eliminated a deep infection and tumour cause in the female patient. The diagnosis of MAS was made, and both patients were placed on broad-spectrum antibiotic, in addition to local antifungal treatment and corticosteroid for the female patient, but the evolution was fatal for both. In conclusion, the management of MAS should be fast and multidisciplinary, based on treatment of the causal infectious agent, immunomodulatory treatment of haemophagocytosis, symptomatic treatment and replacement of organ failure.

Polysplenia syndrome revealed in adulthood by pancreatic and vascular malformations: a case report.

Polysplenia syndrome (PSS) is a rare congenital disease that associates multiple spleens to other malformations, most frequently cardiac, vascular, visceral, and biliary malformations. Most patients with PSS die in the early neonatal period because the disease is often accompanied by serve cardiac and biliary abnormalities. However, some patients have only mild cardiovascular malformations or anomalies in the abdominal organs, which are typically diagnosed incidentally in adulthood. We report the case of a 54-year-old woman who consulted for chronic atypical diffuse abdominal pain. The clinical examination was normal. Abdominal computed tomography showed a total of 5 spleens with vascular and pancreatic malformations as part of polysplenia syndrome. Symptomatic treatment was instituted with good evolution. No specific therapeutic indication was indicated in our case discovered incidentally in adulthood.

Comparative analysis of volatile organic compounds of breath and urine for distinguishing patients with liver cirrhosis from healthy controls by using electronic nose and voltammetric electronic tongue.

Advanced stage detection of liver cirrhosis (LCi) would lead to high mortality rates in patients. Therefore, accurate and non-invasive tools for its early detection are highly needed using human emanations that may reflect this disease. Human breath, along with urine and blood, has long been one of the three main biological media for assessing human health and environmental exposure. The primary objective of this study was to explore the potential of using volatile organic compounds (VOCs) assay of exhaled breath and urine samples for the diagnosis of patients with LCi and healthy controls (HC). For this purpose, we used a hybrid electronic nose (E-nose) combining two sensor families, consisting of an array of five commercial chemical gas sensors and six interdigitated chemical gas sensors based on pristine or metal-doped WO3 nanowires for sensing volatile gases in exhaled breath. A voltammetric electronic tongue (VE-tongue), composed of five working electrodes, was dedicated to the analysis of urinary VOCs using cyclic voltammetry as a measurement technique. 54 patients were recruited for this study, comprising 22 patients with LCi, and 32 HC. The two-sensing systems coupled with pattern recognition methods, namely Principal Component Analysis (PCA) and Discriminant Function Analysis (DFA), were trained to classify data clusters associated with the health status of the two groups. The diagnostic performances of the E-nose and VE-tongue systems were studied by using the receiver operating characteristic (ROC) method. The use of the E-nose or the VE-tongue separately, trained with these appropriate classifiers, showed a slight overlap indicating no clear discrimination between LCi patients and HC. To improve the performance of both electronic sensing devices, an emerging strategy, namely a multi-sensor data fusion technique, was proposed as a second aim to overcome this shortcoming. The data fusion approach of the two systems, at a medium level of abstraction, has demonstrated the ability to assess human health and disease status using non-invasive screening tools based on exhaled breath and urinary VOC analysis. This suggests that exhaled breath as well as urinary VOCs are specific to a disease state and could potentially be used as diagnostic methods.

Endoscopic retrograde cholangiopancreatography in ruptured liver hydatid cyst.

One of the most common and serious complications of hepatic hydatid cyst disease is communication between the cyst and the biliary tree. Surgical management of biliary fistula is associated with high morbidity and mortality. We retrospectively reviewed the effectiveness of endoscopic treatment of ruptured hydatid cyst into intrahepatic bile ducts. Diagnosis of intrabiliary rupture of hydatid cyst was mostly suspected by acute cholangitis, jaundice, pain, and/or persistent external biliary fistula after surgery. The diagnosis was confirmed by radiology and endoscopic retrograde cholangiopancreatography (ERCP) findings. We retrospectively reviewed clinical, laboratory, imagery, and ERCP findings for all patients. The therapeutic methods performed were endoscopic sphincterotomy, extraction by balloon or Dormia basket, stenting, or nasobiliary drainage. Sixteen patients with ruptured hepatic hydatid cyst into bile ducts were seen in 9 years. Nine of 16 patients had a surgical history of hepatic hydatid cyst and three patients had a percutanous treatment history. We carried out ERCP with sphincterotomy and extraction of hydatid materials (extraction balloon n = 11; Dormia basket n = 5) or biliary drainage (nasobiliary drainage n = 1; biliary stenting n = 1). The fistula healed in 80 % of patients with a median time of 6 weeks [range, 1-12] after endoscopic treatment. ERCP was an effective method of treatment for hepatic hydatid cyst with biliary fistula.

MICA∗078: A novel allele identified in a Moroccan individual affected by celiac disease.

A novel MICA allele, MICA(∗)078, has been identified during HLA/MICA high resolution typing of Moroccan patients with celiac disease. MICA(∗)078 shows an uncommon variation at a highly conserved nucleotide position (nt 493, G → A), resulting in one amino acid change at codon 142 (V → I) of MICA gene (compared to MICA(∗)002:01), located in the α2-domain, in which V142 is the common residue.

Predictors of Developing Hepatocellular Carcinoma in Treated HCV-Carriers in Morocco according to University Hospital Experience.

Introduction. Hepatitis C is the first major cause for HCC in Morocco. Antiviral treatment reduces the risk of developing HCC but few cases of HCC in HCV-treated patients were reported. We aimed to define this population's features and to identify predictive factors of developing HCC. Patients and Methods. We included all HCV carriers who developed HCC after antiviral treatment from January 2002 to April 2010. We compare HCV-treated patients with no developed HCC to HCC population using khi-2 and Fisher Exact analysis. Results. 369 HVC-treated patients were considered, and 20 HCC were reported. The risk of HCC was not significant according to gender and genotypes (resp., P = 0.63 and P = 0.87). Advanced age and severe fibrosis were significant risk factors (resp., P = 0.003 and P = 0.0001). HCC was reported in 2.6% of sustained virological responders versus 12.5% of nonresponders (P = 0.004). Conclusion. In our series, 5% of previously treated patients developed an HCC. Advanced age and severe fibrosis at HCV diagnosis are predictive factors of HCC occurrence. Sustained virological response reduces considerably the risk of HCC occurrence but screening is indicated even after SVR.

Adult colocolic intussusception diagnosed by ultrasonography: a case report.

INTRODUCTION: Intussusception is highly uncommon in adults and accounts for only 5% of all reported cases. It is more commonly secondary to an identifiable bowel lesion in 90% of cases, whereas 10% have no discernable cause. Diagnosis is difficult due to non-specific symptoms of the disease. Diagnostic imaging plays an important role in the diagnosis of the condition. Sonography and computed tomography are the most commonly used imaging techniques. In adults, intussusception usually requires treatment by surgical resection of the affected bowel. CASE PRESENTATION: A 35-year-old Moroccan woman presented with a five-month history of intermittent abdominal pain and one episode of bleeding from the rectum. At physical examination an abdominal mass was noted. Abdominal sonography revealed a 6.3 × 8.5 cm midline mass in her upper abdomen that was tender. In transverse section, the mass had the multiple concentric rings of hypoechoic and echogenic layers associated with the sonographic appearance of intussusception. In longitudinal section, the mass had the sonographic aspect of multiple parallel lines, giving the so-called "sandwich appearance".A corresponding contrast-enhanced abdominal computed tomography scan also demonstrated the intussusception. Surgery confirmed a colocolic intussusception with a large, firm, indurated mass as the lead point. A right hemicolectomy was undertaken because of concern about possible malignancy. The resected ascending colon was then opened up, to find a protruding tumor of the ascending colon that was acting as the lead point. It measured 7.6 × 6.9 × 2.4 cm. Pathology diagnosed an infiltrating, differentiated adenocarcinoma of the ascending colon invading through the muscularis propria. No lymphovascular invasion was seen. Our patient has recovered well. CONCLUSION: Intussusception is relatively rare in the adult population, and this, along with the vague clinical features, makes diagnosis difficult. Ultrasonography and computed tomography have been proven to be effective diagnostic modalities. Ultrasonography can be performed quickly and accurately, and is widely available. In adults, intussusception is usually associated with an underlying cause and requires treatment by surgical resection.

[Advances in the diagnosis and management of gastrointestinal tumours: 5 cases]. / Nouvelles acquisitions diagnostiques et thérapeutiques sur les tumeurs stromales digestives: à propos de cinq cas.

INTRODUCTION: Gastrointestinal stromal tumours (GIST) are the most frequent mesenchymatous tumours of the digestive tract. Options for diagnosis and treatment have developed rapidly in recent years. The authors report five cases of GIST. CASES: The study concerns 5 patients: 3 men and 2 women, with a mean age at diagnosis of 39.8 years. We describe the circumstances of discovery and the clinical and morphological characteristics of these tumours. They were located in the oesophagus (1 case), stomach (2 cases), small intestine (1 case) and mesentery (1 case). Tumour size ranged from 4-20 cm. Liver metastasis was identified at initial diagnosis for one patient. Immunohistochemical analysis identified expression of CD34, CD117 and S-100 proteins in all cases and smooth muscle actin in 2 cases. All the patients underwent surgical resection for GIST, and one received chemotherapy. No patient received imatinib treatment. After a mean follow-up of 40 months: one patient presented peritoneal carcinosis at one year, and 3 patients liver metastasis at 2 years; one of the latter died. DISCUSSION: The cases reported here are original by the rarity of their localization (especially the oesophagus and the mesentery). Diagnosis and treatment options for patients are described in a discussion of the recent advances in the field. CONCLUSION: GIST are the most frequently mesenchymatous tumours of the digestive tract. They must be recognized, especially in view of their frequency. Optimal management today involves complete tumour resection and imatinib.