Generation and characterization of dromedary Tenascin-C and Tenascin-W specific antibodies.

Tenascin-C (TNC) and tenascin-W (TNW), large hexameric glycoproteins overexpressed in the tumor microenvironment, are useful tumor biomarkers for theranostic applications. For now, polyclonal and monoclonal antibodies, as well as aptamers targeting TNC and TNW have been developed. However, the immunostaining sensitivity of antibodies is very heterogenous. The main aim of this study was to generate antibodies in dromedary that detect TNC and TNW, respectively. We show that immune sera from immunized dromedaries are able to specifically bind native TNC and TNW by ELISA and also to detect TNC and TNW in matrix tracks of mammary tumors by immunostaining. Furthermore, we demonstrate that purified IgG subtypes are able to interact specifically with TNC or TNW by ELISA and immunostaining. These camelid antibodies are a good basis to develop tools for the detection of TNC and TNW in the tumor microenvironment and could potentially have a broader application for early diagnosis of solid cancers.

Tenascin-W Is a Novel Stromal Marker in Biliary Tract Cancers.

Extrahepatic cancers of the biliary system are typically asymptomatic until after metastasis, which contributes to their poor prognosis. Here we examined intrahepatic cholangiocarcinomas (n = 8), carcinomas of perihilar bile ducts (n = 7), carcinomas of the gallbladder (n = 11) and hepatic metastasis from carcinomas of the gallbladder (n = 4) for the expression of the extracellular matrix glycoproteins tenascin-C and tenascin-W. Anti-tenascin-C and anti-tenascin-W immunoreactivity was found in all biliary tract tumors examined. Unlike tenascin-C, tenascin-W was not detected in normal hepatobiliary tissue. Tenascin-W was also expressed by the cholangiocarcinoma-derived cell line Huh-28. However, co-culture of Huh-28 cells with immortalized bone marrow-derived stromal cells was necessary for the formation and organization of tenascin-W fibrils in vitro. Our results indicate that tenascin-W may be a novel marker of hepatobiliary tumor stroma, and its absence from many normal tissues suggests that it may be a potential target for biotherapies.

Role of Computed tomography in predicting prognosis of Hepatic portal venous gas.

BACKGROUND: The aim of this study was to report through 13 cases the particularities of abdominal computed tomography (CT) aspects of hepatic portal venous gas (HPVG) and its correlation with patient prognosis. METHODS: We analyzed abundance of HPVG and its association with pneumatosis intestinalis (PI) in correlation with fatal outcome using chi-square tests. RESULTS: Etiologies were mesenteric infarction (n=5), sigmoid diverticulitis (n= 1), septic shock (n=1), postoperative peritonitis (n=1), acute pancreatitis (n=1), iatrogenic cause (n=3) and idiopathic after a laparotomy (n=1). The outcome was fatal in for 6 patients. Abundance of HPV was expressed in total number of hepatic segments involved. The involvement of 3 or more segments was a sensitive sign for lethal outcome with high sensitivity (100%) but it was not specific (50%). Negative predictive value of this sign was 100% (p≤0.005). Positive predictive value of PI for death was 100% (p≤0.001). DISCUSSION: Abundance of HPVG is correlated with prognosis. The presence of PI announces poor outcome Negative predictive value of presence of HPVG in 3 or more segments is interesting. Predicting prognosis with CT can help surgeons to assess the most adequate treatment. Iatrogenic causes are increasingly described after interventional radiology procedures with favorable course. CONCLUSION: The first etiology radiologists should look for in front of HPVG involving more than 3 hepatic segments and associated with PI is intestinal necrosis which announces a poor prognosis. This study shows that outside of shock situations, HPVG involving 2 or less hepatic segments without PI predicts a good outcome.

Computed Tomographic presentation of obstructive jejunal adenocarcinoma associated with celiac disease and incomplete intestinal malrotation.

INTRODUCTION: Small bowel adenocarcinoma is a rare entity most frequently observed with celiac disease. This is the first case report on the association of celiac disease, small bowel adenocarcinoma and intestinal malrotation. CASE REPORT: A 40 year-old male patient diagnosed with celiac disease since the age of 5 years complained of epigastric pain and vomiting for three days. Computed tomography (CT) showed a significant gastroduodenal dilatation with thickened intestinal wall proximal to the duodenojejunal flexure. The lumen contained a food bezoar in the center. The duodenojejunal angle was abnormally on the right side of the abdomen and the superior mesenteric vein was anterior to the superior mesenteric artery. Endoscopy after aspiration found a hemi-circumferential and irregular mass which bled at the contact of fibroscope. Biopsies showed an adenocarcinoma and small bowel resection was performed. DISCUSSION: Celiac disease is associated with a high risk of small bowel cancer. The association of incomplete intestinal malrotation, duodenojejunal flexure tumor and celiac disease made the surgery challenging. CONCLUSION: Patients with celiac disease should be carefully monitored and endoscopic or radiologic investigations should be carried out in patients with any doubtful symptoms.

Spontaneous uretero-sigmoid fistula secondary to calculus.

A 25-year-old man was referred to the urology department after a subacute history of left back pain, burning micturition associated with pneumaturia and fecaluria. Ultrasonography was performed showing hydronephrosis, and plain film radiography demonstrated a long vertical left pelvic calculi. Uro-computed tomography (CT) combined with a water enema CT showed a 10 cm long calculus with the cranial extremity fistulating the sigmoidal wall. Surgical treatment included left nephroureterectomy and sigmoidectomy with a colorectal anastomosis. Postoperative course was uneventful.

Association of the long QT syndrome With goiter and deafness.

We report on the long QT syndrome occurring in conjunction with nontoxic multinodular goiter and sensorineural deafness in several siblings of a large family. Autosomal and X-linked recessive and dominant modes of inheritance are possible for the different phenotypes. The affected family members had various phenotype combinations, suggesting variable expressivity and incomplete penetrance.

Conservative treatment of a cervical twin pregnancy with uterine artery embolization.

Cervical pregnancy is a rare form of ectopic pregnancy. Its treatment has been described by different authors. We report our successful experience of a cervical twin pregnancy that was diagnosed by transabdominal and transvaginal ultrasound and confirmed by magnetic resonance imaging. To preserve fertility, our patient was treated by a bilateral hyperselective uterine artery embolization followed by dilatation and curettage of the cervical canal without ancillary procedures for cervical hemostasis. Arterial embolization by a resorbable agent reduces arterial circulation by providing a temporary occlusion of the vessels in order to decrease the risk of massive hemorrhage.

[Percutaneous bile drainage: complications and efficiency at short and mean terms: about 50 cases]. / Drainage biliaire percutané: efficacité et complications a court et a moyen terme a travers une série de 50 cas.

PURPOSE: Our purpose is to evaluate the efficiency and mean term complications in percutaneous bile drainage through a retrospective study of 50 cases METHODS: Fifty patients aged from 24 to 87 years underwent perecutaneous bile drainage in the Radiology Department of Mongi Slim hospital. Patients had external drainage, internal drainage or endoprotheses. Clinical and biological features were used to evaluate efficiency and complications. RESULTS: Bile drainage was successful in 49 cases. Intra hepatic bile ducts were dilated in most of the cases (40 patients). A right lobe puncture was performed preferentially (41 patients). Fifteen patients had endoprotheses, 3 patients had internal drainage and 31 patients had external drainage. Clinical and biological improvement was noted in all patients. Complications were noted in 16 cases mainly in patients with neoplastic obstruction. CONCLUSION: Percutaneous bile drainage is an effective method for the treatment of biliary obstruction. Complications are rare, occurring most frequently in patients with neoplastic obstruction.

[A novel mutation in infant hypophophatasia: a case report]. / Une nouvelle mutation de l'hypophosphatasie de l'enfant: a propos d'un cas Tunisien.

BACKGROUND: Hypophosphatasia is a rare inherited disorder characterized by defective bone and teeth mineralization and deficiency of serum and bone alkaline phosphatase activity. Several mutations in the TNSALP gene are identified. AIM: The authors describe a Tunisian case having a mutation that has not been described up to now. CASE: It is about an infant, in the antecedents of recurring disease of the lungs in child since the age of seven months, which presents clinical and radiological signs of rickets. The diagnosis of hypophosphatasia is strongly suspected in front of Reduced serum alkaline phosphatase activity and confirmed by the genetic study. The child is homozygous for a new mutation L282P in the ninth exon of the gene. The parents and two brother and sister are heterozygous for the same mutation.

Pseudo-tumoral renal actinomycosis.

Actinomycosis is a rare granulomatous chronic infection that is localized most frequently in the cranio-cervical region. Renal localization is exceptional. The authors report a new case of pseudo-tumoral actinomycosis and discuss diagnostic and treatment issues of this disease.

Familial vertebral segmentation defects, Sprengel anomaly, and omovertebral bone with variable expressivity.

A 10-year-old boy was found to have an unusual presentation of the Sprengel anomaly, omovertebral bones, and segmentation defects of the vertebral column at the cervical, thoracic, and sacral level. In addition, he showed hypertelorism, downslanting palpebral fissures, ptosis, webbing, and hypoplasia of the thenar and hypothenar areas. He had moderate mental delay. In addition to the segmentation defects and omovertebral bones, radiological studies showed a small pelvis and 11 pairs of ribs. Some of the features were present in the mother, and minimal symptoms were present in the father. The parents were consanguineous. A paternal cousin had segmentation defects, omovertebral bones, and a Sprengel deformity as well, although with milder presentation than the proband. We were unable to find a similar combination of manifestations in literature. The familial occurrence is best compatible with autosomal dominant inheritance, showing wide variability of expression. It is possible that the more notable signs in the proband can be explained by homozygosity for the disorder.

[Acute leukoencephalitis in infant treated by high-dose corticosteroid. A case report]. / Leucoencephalite aigue du nourrisson traitee par fortes doses de corticoides.

The authors report a case of acute post infectious leukoencephalitis observed in a tow-years and a half children admitted to our hospital for fiver with suddent condition deterioration, obnibulation, coma and paralysis of the 6th and 7th cranial nerve. Cerebrospinal fluid study showed lymphocytosis with negative culture. Head magnetic resonance imaging demonstrated diffuse high signals over the white matter on T2 weighted images so the diagnosis was confirmed. High dose corticosteroid therapy was effective.

[Acrania ultrasonography diagnosis: a rare embryologic malformation]. / Diagnostic échographique de l'acranie: malformation embryologique rare.

The authors report a case of acrania diagnosed on antenatal period in a routine morphologic ultrasonography at 19 weeks of intra uterine gestation on a 20-year-old patient, gravida 1, para 0. Acrania associated with exencephaly is a rare and lethal malformation characterized by a defect of development of the flat bones of the scalp whereas cerebral tissue is present often abnormal. Ultrasonography had allowed to find besides the cerebral malformation, spinal and visceral fetal malformations as well as cord abnormality that included only two vessels.

[Acute pancreatitis due to a metallic foreign body]. / Pancréatite aiguë à corps étranger métallique.

We report a case of acute pancreatitis caused by a metallic foreign body located in the pancreatic head in a 38-Year-old woman. Only 15 cases of acute pancreatitis due to foreign bodies, metallic in five cases, have been published to date. In our case, diagnosis was established by the computed tomography scan. The patient declined surgery.