Tuncer Temel

Amyloidosis and its related factors in Turkish patients with familial Mediterranean fever: a multicentre study

OBJECTIVE The primary aim of this study was to investigate the prevalence of amyloidosis and its related factors in a large number of FMF patients. METHODS Fifteen centres from the different geographical regions of Turkey were included in the study. Detailed demographic and medical data based on a structured questionnaire and medical records were collected. The diagnosis of amyloidosis was based on histological proof of congophilic fibrillar deposits in tissue biopsy specimens. RESULTS There were 2246 FMF patients. The male/female ratio was 0.87 (1049/1197). The mean age of the patients was 34.5 years (S.D. 11.9). Peritonitis was the most frequent clinical finding and it was present in 94.6% of patients. Genetic testing was available in 1719 patients (76.5%). The most frequently observed genotype was homozygous M694V mutation, which was present in 413 (24%) patients. Amyloidosis was present in 193 patients (8.6%). Male sex, arthritis, delay in diagnosis, M694V genotype, patients with end-stage renal disease (ESRD) and family history of amyloidosis and ESRD were significantly more prevalent in patients with amyloidosis compared with the amyloidosis-negative subjects. Patients with homozygous M694V mutations had a 6-fold higher risk of amyloidosis compared with the other genotypes (95% CI 4.29, 8.7, P < 0.001). CONCLUSION In this nationwide study we found that 8.6% of our FMF patients had amyloidosis and homozygosity for M694V was the most common mutation in these patients. The latter finding confirms the association of homozygous M694V mutation with amyloidosis in Turkish FMF patients.

Serum Thrombopoietin Levels and Its Relationship With Thrombocytopenia in Patients With Cirrhosis

Background: Patients with cirrhosis usually have thrombocytopenia in discrete levels. The mechanism of thrombocytopenia is thought as splenic sequestration and destruction of platelets, impaired bone marrow generation and diminished hepatic thrombopoietin synthesis. Objectives: The aim of this study was to evaluate serum thrombopoietin levels and its relationship with thrombocytopenia at patients with cirrhosis. Patients and Methods: Ninety–two cirrhotic patients and 45 healthy controls without history or findings of pathologies that can effect thrombopoietin levels were enrolled by simple random sampling to patient and control groups of this case control study performed at Eskisehir-Turkey. Thrombopoietin was measured in serum samples with a solid phase enzyme-linked immune absorbent assay. Additionally, spleen size and volume index were determined. Results: Platelet counts were lower in patients with cirrhosis (97000 ± 8000/mm3) than in healthy subjects (240000 ± 51000/mm3, P < 0.001). Significant difference was determined for platelet counts among child A, B and C stages (Child A vs. Child B P < 0.05 Child A vs. Child C P < 0.001–Child B vs. Child C P < 0.05). Serum TPO concentration was higher (69 ± 12 pg/mL) in cirrhotic group than healthy controls (49 ± 9 pg/ml) (P < 0.05). No significant difference in TPO levels were found among the Child A, B and C stages (64 ± 11 pg/mL, 75 ± 13 pg/mL and 68 ± 10 pg/mL, respectively). Spleen size and SVI was significantly higher in the cirrhotic patients than healthy controls (148 ± 14 mm vs. 98 ± 11 mm, P < 0.001-9167 ± 287 cm2 vs. 4118 ± 123 cm2). Significant difference was determined for spleen size and spleen index among child A, B and C stages (Child A vs. Child B P < 0.05 Child A vs. Child C P < 0.001–Child B vs. Child C P < 0.05). TPO levels were significantly different between cirrhotic patients with platelet levels below 50.000/mm3 (n = 16, plt-count: 41000 ± 8300/mm3, TPO levels: 73 ± 7 pg/mL) and above 50.000/mm3 (n = 76, plt-count: 105000 ± 9500/mm3, TPO levels: 65 ± 10 pg/mL) (P < 0.01). In correlation analysis, there was a strong negative correlation between platelet count-spleen size (P < 0.001, r = -0.74) and platelet count–serum TPO levels (P < 0.001, r = -0.71). Conclusions: Our results suggest that liver cirrhosis does not cause impaired thrombopoietin production even in the late stage of disease. Thrombopoietin has no contribution for the occurrence of thrombocytopenia in cirrhosis; splenic sequestration seems to be the main factor.

The long-term effects of anti-TNF-α agents on patients with chronic viral hepatitis C and B infections

To evaluate the long‐term effects of anti‐tumor necrosis factor‐alpha (TNF‐α) therapy on patients with chronic hepatitis B and C infections.

Comparison of 64-Detector CT Colonography and Conventional Colonoscopy in the Detection of Colorectal Lesions

Background: Colon cancer is a leading cause of morbidity and mortality in developed countries. The early detection of colorectal cancer using screening programs is important for managing early-stage colorectal cancers and polyps. Modalities that allow examination of the entire colon are conventional colonoscopy, double contrast barium enema examination and multi-detector computed tomography (MDCT) colonography. Objectives: To compare CT colonography and conventional colonoscopy results and to evaluate the accuracy of CT colonography for detecting colorectal lesions. Patients and Methods: In a prospective study performed at Gastroenterology and Radiology Departments of Medical Faculty of Eskisehir Osmangazi University, CT colonography and colonoscopy results of 31 patients with family history of colorectal carcinoma, personal or family history of colorectal polyps, lower gastrointestinal tract bleeding, change in bowel habits, iron deficiency anemia and abdominal pain were compared. Regardless of the size, CT colonography and conventional colonoscopy findings for all the lesions were cross - tabulated and the sensitivity, specificity, and positive and negative predictive values were calculated. To assess the agreement between CT colonography and conventional colonoscopy examinations, the Kappa coefficient of agreementt was used. Statistical analysis was performed by SPSS ver 15.0. Results: Regardless of the size, MDCT colonography showed 83% sensitivity and 95% specificity, with a positive predictive value of 95% and a negative predictive value of 83% for the detection of colorectal polyps and masses. MDCT colonography displayed 92% sensitivity and 95% specificity, with a positive predictive value of 92% and a negative predictive value of 95% for polyps ≥ 10 mm. For polyps between 6mm and 9 mm, MDCT colonography displayed 75% sensitivity and 100% specificity, with a positive predictive value of 100% and a negative predictive value of 90%. For polyps ≤ 5 mm MDCT colonography displayed 88% sensitivity and 100% specificity with a positive predictive value of 100% and a negative predictive value of 95%. Conclusions: CT colonography is a safe and minimally invasive technique, a valuable diagnostic tool for examining the entire colon and a good alternative compared to other colorectal cancer screening tests because of its high sensitivity values in colorectal lesions over 1 cm.

C-Reactive Protein and Platelet-Lymphocyte Ratio as Potential Tumor Markers in Low-Alpha-Fetoprotein Hepatocellular Carcinoma

The hepatocellular carcinoma (HCC) tumor marker alpha-fetoprotein (AFP) is only elevated in about half of the HCC patients, limiting its usefulness in following the effects of therapy or screening. New markers are needed. It has been previously noted that the inflammation markers C-reactive protein (CRP) and platelet-lymphocyte ratio (PLR) are prognostically important and may reflect HCC aggressiveness. We therefore examined these 2 markers in a low-AFP HCC cohort and found that for HCCs > 2 cm, both markers significantly rise with an increasing maximum tumor diameter (MTD). We calculated the sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), and Youden index value for each marker, and their area-under-the-curve values for each MTD group. Patients were dichotomized into 2 groups based on the CRP and PLR from the receiver-operating characteristic curve analysis. In the logistic regression models of the 4 different MTD patient groups, CRP and PLR levels were statistically significant to estimate MTD in univariate logistic regression models of MTD groups > 2 cm. CRP and PLR were then combined, and the combination was statistically significant to estimate MTD groups of 3-, 4-, and 5-cm cutoffs. CRP and PLR thus have potential as tumor markers for low-AFP HCC patients, and possibly for screening.

Ülseratif kolitle ilişkili dirençli pyoderma gangrenosum tedavisinde certolizumab pegol deneyimi

Oz: Pyoderma gangrenosum tekrarlayan kutanoz ulserlerle karakterize notrofilik bir dermatozdur. Hastalarin yarisinda inflamatuvar barsak hastaligi vardir. Tedavisinde temel ilac kortikosteroidlerdir. Ancak steroidden kurtarici tedavi olarak immunsupresif ilaclarin kullanilmasi da gerekmektedir. Direncli pyoderma gangrenosum tedavisi hastayi ve hekimi zorlayabilmektedir. Direncli vakalarda biyolojik ilaclardan infliximab basta olmak uzere anti-TNF ajanlarinn etkili oldugunu gosteren olgu bildirileri vardir. Pegile monoklonal anti-TNF ajan olan certolizumab pegolun etkisini gosteren literaturde sadece 2 olgu vardir. Bu yazida ulseratif kolit ile iliskili direncli pyoderma gangrenosumda certolizumab pegolun etkinligini gosteren literaturdeki ilk vaka sunularak direncli pyoderma gnagrenosumda alternatif tedavi secenegi olarak certolizumab pegole dikkat cekilmeye calisilmistir.

The Long-Term Outcomes for Patients with Budd-Chiari Syndrome Caused by Behcet's Disease: A Case Series on the Results, from Cirrhosis to Death.

Background Budd-Chiari syndrome, which is a rare complication of Behcet’s disease, carries a high mortality rate. Objectives The aim of the study was to present our long-term follow up experience with patients suffering from Budd-Chiari syndrome due to Behcet’s disease. Methods The records of 402 patients with Behcet’s disease were evaluated retrospectively. To facilitate detection of the long-term complications caused by Budd-Chiari syndrome, the patients were evaluated via physical examinations, laboratory tests, imaging modalities, and endoscopy results. Results The data for 402 patients diagnosed with Behcet’s disease, who were followed up at our hospital over 16 years, were analyzed retrospectively. Five of these 402 patients (1.2%) were diagnosed as having Budd-Chiari syndrome. The patients with Budd-Chiari syndrome were aged between 23 and 54, and all five were male. The interval between the onset of Behcet’s disease and the development of Budd-Chiari syndrome ranged from 1 to 8 years. All the patients had combined venous occlusion (affecting the hepatic vein and inferior vena cava). Portal venous thrombosis was detected in only one patient (Case 1), who died 1 month after the diagnosis of Budd-Chiari syndrome. The survival time for the other four patients after the diagnosis of Budd-Chiari syndrome ranged from 4 to 16 years. During the long-term follow-up, hepatic masses were detected via radiological surveillance in Case 3 (in the form of large regenerative nodules) and Case 4 (nodular regenerative hyperplasia and cirrhosis). Conclusions In our study, portal venous thrombosis was detected in the patient who died during the acute period only. A study including large numbers of Budd-Chiari-syndrome patients with Behcet’s disease and portal venous thrombosis would be helpful to determine the prognostic significance of portal venous thrombosis in Budd-Chiari-syndrome patients with Behcet’s disease. In addition, patients should be monitored regularly for the development of hepatic masses via a long-term surveillance program.

Complete Eradication of Bleeding Duodenal Varices with Endoscopic Polydocanol Sclerotherapy

ABSTRACT Bleeding from duodenal varices is a rare complication of portal hypertension, occurring in only 0.4% of these patients and is often life-threatening because of the difficulty in diagnosis and treatment. Treatment options include surgical procedures and endoscopic and endovascular treatments. A 48-year-old female cirrhotic patient was admitted to our clinic with upper gastrointestinal (GI) tract bleeding. Endoscopic examination revealed nonbleeding Lm, Cb, RC (+), F3-F3-F2 esophageal and nodular-bleeding-oozing duodenal varices. Esophageal varices were eradicated with band ligation at two sessions. After one session of 2% polydocanol sclerotheraphy, no signs of bleeding were determined. Complete eradication was achieved after five sessions and 1 year apart from the initial treatment duodenal varices were eradicated. Although duodenal varices are rare, they are frequently fatal. There are limited data regarding optimal treatment. Successful treatment depends both on patient factors (hepatic synthetic function, comorbidities, size/location of the varices) and center expertise. Long-term eradication is variable and may depend on the cause and extensiveness of the ectopic varices. How to cite this article Temel T, Aktas A, Ozgenel SM, Özakyol A. Complete Eradication of Bleeding Duodenal Varices with Endoscopic Polydocanol Sclerotherapy. Euroasian J Hepato-Gastroenterol 2016;6(2):176-178.

Abdominal Inflammatory Myofibroblastic Tumor: A Rare Case

ABSTRACT An inflammatory myofibroblastic tumor, also known as inflammatory pseudotumor, is a rare neoplasm characterized by myofibroblastic spindle and inflammatory cells that cause masses in many sites of body. It is often benign, but in some cases neoplastic transformation has been reported as a result of aggressive growing. In our case, an inflammatory myofibroblastic tumor was reported by biopsy of a 25 × 15 cm abdominal mass. How to cite this article Tastekin F, Ersoy M, Temel T, Ozgenel SM, Canaz F, Özakyol A. Abdominal Inflammatory Myofibroblastic Tumor: A Rare Case. Euroasian J Hepato-Gastroenterol 2016;6(2):183-185.