Acute pancreatitis in Turkey: Results of a nationwide multicenter study.

BACKGROUND: Acute pancreatitis (AP) is the most common gastrointestinal disease requiring hospitalization, with significant mortality and morbidity. We aimed to evaluate the clinical characteristics of AP and physicians' compliance with international guidelines during its management. METHODS: All patients with AP who were hospitalized at 17 tertiary centers in Turkey between April and October 2022 were evaluated in a prospective cohort study. Patients with insufficient data, COVID-19 and those aged below 18 years were excluded. The definitions were based on the 2012 revised Atlanta criteria. RESULTS: The study included 2144 patients (median age:58, 52 % female). The most common etiologies were biliary (n = 1438, 67.1 %), idiopathic (n = 259, 12 %), hypertriglyceridemia (n = 128, 6 %) and alcohol (n = 90, 4.2 %). Disease severity was mild in 1567 (73.1 %), moderate in 521 (24.3 %), and severe in 58 (2.6 %) patients. Morphology was necrotizing in 4.7 % of the patients. The overall mortality rate was 1.6 %. PASS and BISAP had the highest accuracy in predicting severe pancreatitis on admission (AUC:0.85 and 0.81, respectively). CT was performed in 61 % of the patients, with the majority (90 %) being within 72 h after admission. Prophylactic NSAIDs were not administered in 44 % of the patients with post-ERCP pancreatitis (n = 86). Antibiotics were administered to 53.7 % of the patients, and 38 % of those received them prophylactically. CONCLUSIONS: This prospective study provides an extensive report on clinical characteristics, management and outcomes of AP in real-world practice. Mortality remains high in severe cases and physicians' adherence to guidelines during management of the disease needs improvement in some aspects.

Liver injury after SARS-CoV-2 vaccination: Features of immune-mediated hepatitis, role of corticosteroid therapy and outcome.

BACKGROUND AND AIMS: A few case reports of autoimmune hepatitis-like liver injury have been reported after severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) vaccination. We evaluated clinical features, treatment response and outcomes of liver injury following SARS-CoV-2 vaccination in a large case series. APPROACH AND RESULTS: We collected data from cases in 18 countries. The type of liver injury was assessed with the R-value. The study population was categorized according to features of immune-mediated hepatitis (positive autoantibodies and elevated immunoglobulin G levels) and corticosteroid therapy for the liver injury. We identified 87 patients (63%, female), median age 48 (range: 18-79) years at presentation. Liver injury was diagnosed a median 15 (range: 3-65) days after vaccination. Fifty-one cases (59%) were attributed to the Pfizer-BioNTech (BNT162b2) vaccine, 20 (23%) cases to the Oxford-AstraZeneca (ChAdOX1 nCoV-19) vaccine and 16 (18%) cases to the Moderna (mRNA-1273) vaccine. The liver injury was predominantly hepatocellular (84%) and 57% of patients showed features of immune-mediated hepatitis. Corticosteroids were given to 46 (53%) patients, more often for grade 3-4 liver injury than for grade 1-2 liver injury (88.9% vs. 43.5%, p = 0.001) and more often for patients with than without immune-mediated hepatitis (71.1% vs. 38.2%, p = 0.003). All patients showed resolution of liver injury except for one man (1.1%) who developed liver failure and underwent liver transplantation. Steroid therapy was withdrawn during the observation period in 12 (26%) patients after complete biochemical resolution. None had a relapse during follow-up. CONCLUSIONS: SARS-CoV-2 vaccination can be associated with liver injury. Corticosteroid therapy may be beneficial in those with immune-mediated features or severe hepatitis. Outcome was generally favorable, but vaccine-associated liver injury led to fulminant liver failure in one patient.

Does apocynin increase liver regeneration in the partial hepatectomy model?

BACKGROUND: Hepayocyte loss may develop secondary to liver surgery and at this point liver regeneration plays a significant act in terms of liver reserve. The purpose of this research was to investigate the efficacy of apocynin on liver regeneration and preservation after partial hepatectomy in rats. METHODS: A total of 32 rats, have been divided into 4 groups (n: 8) for hepatectomy model. Inflammatory and antiinflammatory parameters were measured from blood and liver tissue samples. In addition, the effects of apocynin were examined immunohistochemically and histopathologically from liver tissue. RESULTS: In liver tissue samples, a significant difference has been found in glutathione peroxidase, total nitrite, catalase, oxidative stress index, total antioxidant and total oxidant status between sham and hepatectomy groups. A significant difference has been achieved between hepatectomy and posthepatectomy-Apocynin in terms of glutathione peroxidase and oxidative stress index. Total antioxidant status, oxidative stress index, and total oxidant status were significantly different only between the sham and the hepatectomy groups. Statistical differences were found between sham and hepatectomy groups and between hepatectomy and pre+post-hepatectomy-Apocynin groups in terms of serum glutathione, malondialdehyde, total nitrite, and L-Arginine. There were significant differences between the sham and hepatectomy groups, between hepatectomy and posthepatectomy-apocynin groups, between posthepatctomy-apocynin and pre+posthepatectomy-apocynin groups in terms of sinusoidal dilatation, intracytoplasmic vacuolization and glycogen loss (p < 0.001), in all histopathologic parameters except sinusoidal dilatation (p < 0.05). However, significant Ki-67 increases have been elaborated in hepatectomy, posthepatectomy-apocynin, and pre+posthepatectomy-apocynin groups compared to sham group (p < 0.001), in pre+posthepatectomy apocynin group compared to hepatectomy and posthepatectomy-apocynin groups (p < 0.001). DISCUSSION: Histopathology, immunohistochemistry, and biochemistry results of this study revealed that apocynin has a protective effect on enhancing liver regeneration in partial hepatectomy cases in rats.

Early therapeutic plasma exchange may improve treatment outcomes in severe acute toxic Hepatitis.

BACKGROUND AND OBJECTIVES: Acute toxic hepatitis can result in a different clinical course from a completely curable disease to subacute hepatitis, chronic hepatitis, and fulminant hepatitis failure, which is quite mortal. For this purpose, therapeutic plasma exchange (TPE) can be used for improving treatment outcomes by reducing the harmful substances caused with and/or without liver function in acute toxic hepatitis. We aimed to evaluate treatment outcomes in severe acute toxic hepatitis patients who applied early TPE procedure. MATERIALS AND METHODS: A total of 335 patients who received TPE between 2010-2021 were retrospectively screened and 59 (male/female, 30/29; min/max-age, 22-84) patients with acute toxic hepatitis who underwent TPE in the first 24 h were included in the study. TPE was performed in patients who had high total bilirubin level (>10 mg/dL). Laboratory parameters of the patients before and after the TPE procedure, number of patients developed complications of acute toxic hepatitis and mortality rates were evaluated for effectiveness of TPE. RESULTS: Acute toxic hepatitis was associated with hepatotoxic drugs in 44 (74.5 %), herbal medication 6 (10.2 %), mushroom poisoning 6 (10.2 %) and with substance abuse 3 (5.1 %) in patients. When the patients were compared based on INR, liver function tests, ammonia, lactate and Model For End-Stage Liver Disease (MELD) score at baseline, 48 h after TPE (independently of TPE number) and before final state a statistically significant decrease was observed in all parameters (p < 0.05). Fifty three (90 %) of patients improved without complications, the remaining 6 (10 %) patients were diagnosed with fulminant hepatitis. All these remaining patients died before liver transplantation (LTx) could be performed. CONCLUSION: TPE is a safe, tolerable therapy option and early TPE may improve treatment outcomes in severe acute toxic hepatitis.

Artificial vascular graft migration into hollow viscus organs in patients who underwent right lobe living donor liver transplantation.

BACKGROUND: To share our experience with hollow viscus migration of artificial vascular grafts (AVG) used for venous reconstruction of the right anterior sector in living donor liver transplantations (LDLT). METHODS: Clinical, radiological, and endoscopic data of 13 right lobe LDLT patients (range: 26-67 years) with a diagnosis of postoperative AVG migration into adjacent hollow viscus were analyzed. RESULTS: Biliary complications were detected in 12 patients. A median of four times endoscopic retrograde cholangiopancreatography (ERCP) procedures were performed in 11 patients prior to AVG migration diagnosis. A median of 2.5 times various percutaneous radiological interventional procedures were performed in eight patients prior to AVG migration diagnosis. The site of migration was the duodenum in eight patients, gastric antrum in four, and Roux limb in the remaining one patient. The migrated AVS were made of polytetrafluoroethylene (PTFE) in 10 patients and polyethylene terephthalate (Dacron) in three. The migrated AVGs were endoscopically removed in seven patients and surgically removed in six. Only one patient died due to sepsis unrelated to AVG migration. CONCLUSION: AVG migration into the adjacent hollow viscus following right lobe LDLT is a rare and serious complication. Repetitive ERCP, interventional radiological procedures, infection related to biliary leakage, and thrombosis of AVGs are among the possible risk factors.

Nucleotide-Binding Oligomerization Domain-Containing Protein 2 Variants in Patients with Spontaneous Bacterial Peritonitis.

BACKGROUND: The occurrence of spontaneous bacterial peritonitis (SBP) is significantly increased in carriers of nucleotide-binding oligomerization domain-containing protein 2 (NOD2) variants, suggesting that local immune alterations might be implicated in bacterial translocation (BT). AIMS: We aimed to assess the role of the NOD2 gene in conferring susceptibility to SBP. We also sought to determine whether levels of serum interleukin-6 (IL-6), lipopolysaccharide-binding protein, and soluble TNF-α receptor, along with the presence of bacterial DNA (bactDNA) in ascitic fluid, are appropriate markers for BT in patients with liver cirrhosis and SBP. METHODS: A cohort of 171 patients was divided into two groups: patients with SBP (n = 82) and those without SBP (n = 89). The presence of the most common NOD2 variants (p.R702W, p.G908R, and c.3020insC) was determined in these patients. RESULTS: We detected the p.G908R variant in four patients (4.9 %) of the SBP group. No significant difference was observed between the SBP and non-SBP groups for NOD2 risk variants. The frequency of bactDNA in ascitic fluid was higher for patients with NOD2 variants than for patients without variants (p = 0.021). Serum IL-6 levels in the SBP group were higher than those in the non-SBP group. CONCLUSIONS: The frequent detection of bactDNA in ascites of patients with the p.G908R variant suggests there is a strong association between NOD2 risk variants and BT in SBP patients. In addition, increased serum IL-6 levels and bactDNA in ascitic fluid could be considered surrogate markers for BT in patients with cirrhosis.

An obstruction not to forget: Pseudo-obstruction (Ogilvie syndrome): Single center experience.

PURPOSE: Colonic pseudo obstruction disease commonly seen in the elderly, immobile patient group can cause serious mortality and morbidity. Our objective in this retrospective study is to share our clinical experience by evaluating patients with Ogilvie syndrome who were followed and treated in our clinic. METHODS: Eleven cases with the diagnosis of Ogilvie syndrome followed up and treated between September 2010 and April 2013 were evaluated retrospectively. All the patients that had no symptoms of acute abdominal pain were initiated conservative treatment. Colonoscopic decompression was attempted for patients whose clinical pictures were not recovered. Patients underwent operation if they developed peritoneal irritation symptoms during these procedures and of their number of white blood cells seriously increased during the follow-up period. RESULTS: A total of 11 patients were treated for Ogilvie syndrome. 6 of the patients underwent surgical treatment, and 5 were treated medically. Mortality developed in two patients. The main cause of mortality was a delay in diagnosis and additional severe underlying diseases. Seven patients were given Neostigmine. Of these, 2 patients required surgery and 3 patients responded to Neostigmine. CONCLUSION: Ogilvie syndrome is a rare cause of ileus of the colon. It is more common particularly in old patients with additional problems. If the disease is suspected and diagnosed early, unnecessary surgical interventions can be prevented with medical treatment choices.

The Course of Endoscopic Treatment Success in Biliary Complications After Living Donor Liver Transplantation.

Objective: Our aim was to present the results of endoscopic retrograde cholangiopancreatography (ERCP) after living donor liver transplantation (LDLT) between February 2015 and June 2021. Methods: Clinical data included LDLT indications, time to perform ERCP after LDLT, number of ERCP procedures, indications for ERCP, and all treatment outcomes, including ERCP, percutaneous, and surgical interventions. We compared the obtained data with our previous study published by our team in 2018, which included 446 patients who underwent ERCP for biliary complications after LDLT between 2005 and 2015. Results: We performed ERCP in 283 of 1506 patients with LDLT who underwent duct-to-duct anastomosis during transplantation and then developed biliary complications. Our endoscopic success rates were 60.9% and 71.0% in the previous and present studies, respectively. Conclusions: Our findings suggest that the success rate of endoscopic treatment of biliary complications in patients with LDLT increases in correlation with the increasing experience of clinicians treating these patients.

Evaluation of Development of Nonalcoholic Fatty Pancreas Disease After Post-Endoscopic Retrograde Cholangiop ancreatography Pancreatitis in Liver Transplant Patients: Computerized Tomography Versus Ultrasound.

BACKGROUND/AIMS: Post-endoscopic retrograde cholangiopancreatography pancreatitis (PEP) is the most common serious adverse event in liver transplant patients The average incidence has been reported as 1.3%-15.1% in prospective series. In our study, we have prospectively evaluated the prevalence of nonalcoholic fatty pancreatic disease (NAFPD) after PEP via computerized tomography (CT) and determined the ratio of fatty pancreas by ultrasound imaging in liver transplant patients. MATERIALS AND METHODS: We have retrospectively analyzed 2922 patient files, and 146 patients were indicated for ERCP. PEP was observed in 32 patients. After presenting the significant association between the NAFPD and PEP, we prospectively reached 32 patients included in the study. Ten out of those patients have been performed ultrasound with regard to NAFPD. RESULTS: PEP was defined in 32 patients in whom CT was performed to investigate NAFPD. When the patients were contacted, it was observed that 12% were deceased, 71% were alive, but 15% of them were untraceable. Ultrasound has been performed on 10 of 32 patients to determine NAFPD. There was a significant reduction in post-PEP pancreas/spleen rate compared to pre-PEP pancreas/ spleen rate (P = .001). Both the pre-PEP and post-PEP pancreas-spleen difference dropped significantly (P = .002). CONCLUSION: Ultrasound imaging could be utilized as a scanning test and an alternative to evaluate and diagnose NAFPD, particularly in risky patients.

Management and Survival Analysis of Gastrointestinal Neuroendocrine Tumors by Different Tumor Characteristics: Tertiary Center Experience.

BACKGROUND: Gastrointestinal neuroendocrine tumor (GI-NET) is a group of neoplasia consisting of amine and acid producing cells with different hormonal profiles. Although the entire GIS accounts for about 2% of tumors, recent research reveals that the incidence rate has increased. Given the increasing incidence in GI-NETs, more and more extensive research is needed on this subject. In this regard, the demographic and clinicopathological features of the patients diagnosed with GI-NET and their relationship with survival were investigated in the present study. METHODS: Thirty-four patients diagnosed with GI-NETs between January 2009 and December 2019 at the Department of Gastroenterology, Inonu University Faculty of Medicine were analyzed retrospectively. Gender, age, tumor localization, metastasis status, tumor number, tumor diameter, tumor grade, Ki-67 index, and the relationships of these factors with overall survival were examined. RESULTS: Of the patients, 61.8% included in the study were male and 38.2% were female. The average age of the patients was 60.74 years. The most common tumor location was in the stomach (26.5%) and liver (26.5%). Apart from this, 17.6% of NETs were seen in the pancreas, 11.8% in the colon, 8.8% in the rectum, 5.9% in the small intestine, and 2.9% in the appendix. No significant relationship was observed between tumor location and gender (p = .326) and age (p = .641). The tumor diameter ranged from 0.2 cm to 13.91 cm, but the average tumor diameter was 3.84 cm. Solitary tumor was found in 51.5% of cases and multiple tumors in 48.5% of cases. The most common grade was Grade 1 with 41.2% of occurrence. The average of Ki-67 index was 18.36%. There was metastasis in 52.9% of cases. The rate of medical treatment was 48.5%. The median overall survival time of the patients was 23.1 months. In addition, 1-year overall survival was 74.9%, 2-year overall survival was 44.6%, and 5-year overall survival was 35.7%. A statistically significant difference was found between tumor grade and the presence of metastasis, and the overall survival (p = .003 and p = .005). CONCLUSIONS: The tumor grade and the presence of metastasis in gastrointestinal neuroendocrine tumors were found to be the most important prognostic factors affecting overall survival.

Effects of lavender oil intervention before endoscopic retrograde cholangiopancreatography on patients' vital signs, pain and anxiety: A randomized controlled study.

BACKGROUND: This study was carried out to determine the effects of lavender oil intervention before endoscopic retrograde cholangiopancreatography (ERCP) on patients' vital signs, pain and anxiety. MATERIALS AND METHODS: This study was designed as a randomized controlled study. The population of the study consisted of adult patients who were going to receive ERCP at the gastroenterology clinic of a university hospital. Ninety patients participated in the study. The data were collected using a Patient Information Form, a Pre-Post Test Record Form, VAS and the State Anxiety Scale. RESULTS: After lavender oil intervention, the pulse rate, systolic-diastolic blood pressure, pain and anxiety levels of the patients in the experiment group were reduced, their oxygen saturation levels were increased, and the difference between the groups was statistically significant (p < 0.05). CONCLUSION: Lavender oil applied on the patients before the ERCP procedure reduced their pulse rate, systolic-diastolic blood pressure, pain and anxiety levels, while it increased their oxygen saturation levels.

Therapeutic Effects of Plasmapheresis on Acute Exacerbations of Chronic Hepatitis B Infection.

Objective In this study, we aimed to demonstrate the effectiveness of plasmapheresis therapy in patients with acute exacerbation of chronic Hepatitis B (CHB) infection. Methods We selected 48 patients with acute exacerbation of CHB infection who were treated by plasmapheresis in our intensive care unit between 2009 and 2016. The patients' demographic characteristics and biochemical and hematological parameters, which were recorded before and after plasmapheresis, were assessed, and the effect of plasmapheresis on the course of patients' treatment was examined. The patients were also divided into three groups according to their clinical course (discharged: 24; transplanted: six; exitus: eight). The patients were further divided into four groups and compared based on the underlying causes that led to the exacerbation (spontaneous exacerbation: 25; caused by immunosuppressive drugs: nine; hepatotoxic drugs: six; other agents: eight). Results We observed significant improvements in terms of international normalized ratio (INR), aspartate aminotransferase (AST), alanine aminotransferase (ALT), gamma-glutamyl transferase (GGT), lactate dehydrogenase (LDH), total bilirubin, direct bilirubin, blood urea nitrogen (BUN), ammonia, and the Model for End-Stage Liver Disease (MELD) score after plasmapheresis therapy. However, there was no significant improvement in hemoglobin (Hb), white blood cell (WBC) count, platelets, albumin, and lactate values. Also, INR, ALP, and ALT values were found to be significantly correlated with transplants and exitus in patients. Conclusion Plasmapheresis therapy is a reliable treatment method that provides clinical recovery and improvement in laboratory parameters in patients with exacerbation of CHB infection.

Optimal Endoscopic Management of Anastomotic Strictures After Double- Biliary Reconstruction in Right Lobe Living-Donor Liver Transplantation.

BACKGROUND: There is no consensus on the optimal drainage technique in the management of biliary anastomotic strictures occurring after right-lobe living-donor liver transplantation (RL LDLT). AIMS: To investigate whether there is a superiority between unilateral and bilateral drainage groups in terms of efficacy and safety of biliary drainage in RL LDLT patients undergoing double-biliary reconstruction. STUDY DESIGN: Retrospective Cohort Methods: Between January 2009 and August 2019, 1693 patients underwent RL LDLT. Of these, 182 patients who developed biliary anastomotic strictures out of the 306 patients who had double-biliary reconstruction, were included in the study. One hundred fifty-five patients with technical success were divided into 2 groups as unilateral (n=116) and bilateral (n=39) drainage groups. The groups were compared in terms of variable parameters such as clinical success, additional procedure, post-ERCP complication, procedures after clinical failure, hospital stay, mortality, and survival. RESULTS: The clinical success was higher in the bilateral group (70% vs. 82%, P = .201). In the initial and the follow-up periods, a total of 44 (38%) patients in the unilateral group were switched to the bilateral drainage group due to the increased need for stenting. The placement of a stent successfully solved the problem only in 28% (32/117) of the patients in the unilateral group, while this rate was 44% (17/39) in the bilateral group. The median follow-up time of both groups was 42 months, and was equal. The number of stent-free follow-up patients in the unilateral drainage group was less than that in the bilateral drainage group (4 and 7, respectively). CONCLUSION: An active attempt should be made for bilateral drainage in patients with biliary anastomotic stricture following RL LDLT. However, for patients in whom bilateral drainage is not possible, unilateral drainage may be recommended, with the placement of a maximum number of stents following primary biliary balloon dilatation, depending on the degree of stricture.

Analysis of risk factors affecting the development of peptic ulcer perforation: case-control study.

AIM: The aim of the to determine the risk factors associated with increased risk of peptic ulcer perforation (PUP). MATERIAL AND METHODS: The demographic, clinic, and biochemical parameters of 65 patients (PUP group) who underwent PUP surgery at our clinic between June 2009 and September 2016 were compared with the data of 134 patients (control group) who underwent endoscopy at a gastroenterology clinic for dyspeptic complaints. The control group were matched at random in a 1 : 2 ratio with the PUP group. Univariate analyses were used to compare different variables and variables with clinical significance, and p ≤ 0.05 was used in the backward stepwise logistic regression model. RESULTS: This study included 65 patients with peptic ulcer perforation aged 17 to 92 years (PUP group) and 134 patients with dyspeptic complaints aged 18 to 87 years (control group). Univariate analysis showed that statistically significant differences were found between groups in terms of non-steroidal anti-inflammatory drugs usage (p = 0.042; OR = 1.868), smoking (p < 0.001; OR = 5.124), old age (p = 0.003), low body mass index (BMI) (p < 0.001), and low hemoglobin (Hb) (p = 0.002). However multivariate analysis showed that increasing age (p = 0.004; OR = 1.035), smoking (p = 0.007; OR = 3.591), decreasing Hb (p = 0.042; OR = 1.277), and decreasing BMI (p < 0.001; OR = 1.669) were independent clinically significant risk factors for development of PUP. CONCLUSIONS: This study showed that decreased BMI, decreased Hb, increased age, and smoking were independent risk factors for development of PUP. Thus, this group of patients needs particular attention paid to suggestive symptoms with early diagnosis and optimal management of peptic ulcer disease.

Endoscopic treatment of biliary complications in donors after living donor liver transplantation in a high volume transplant center.

BACKGROUND/AIMS: Although living donor liver transplantation (LDLT) has been accepted as a primary treatment for adults with end-stage liver disease, concerns about donor health have been emerged. As LDLT is technically complex, it creates perioperative morbidity and mortality risk in donors. Biliary complications such as stricture and leakage are seen most frequently in donors after liver transplantation. While some of these complications get treated with conservative approach, endoscopic, surgical, and percutaneous interventions may be required in some others. We aimed to present endoscopic retrograde cholangiography (ERC) results in donors who developed biliary complications after LDLT. MATERIALS AND METHODS: Between June 2010 and January 2018, a total of 1521 donors (1291 right lobe grafts, 230 left lobe grafts) of patients who underwent LDLT, were retrospectively reviewed. 63 donors who underwent ERC due to biliary complication, were included in the study. RESULTS: Biliary stricture was found in 1.6% (25/1521), biliary leakage in 2.1% (33/1521), and stricture and leakage together in 0.3% (5/1521) donors. Our endoscopic success rates in patients with biliary leakage, biliary stricture, and stricture and leakage were 85% (28/33), 92% (23/25), and 80% (4/5), respectively. Surgical treatment was performed on 12.6% (8/63) donors who failed ERC. CONCLUSION: We found that ERC is a successful treatment for post-LDLT donors who have biliary complications.

The risk factors of portal vein thrombosis in patients with liver cirrhosis.

This study was designed to identify and assess risk factors for portal vein thrombosis (PVT) in patients with cirrhosis. A total of 98 cirrhosis patients with PVT were identified and 101 cirrhosis patients without PVT were chosen as the control group in this retrospective study. Several variables were measured and the two groups PVT and non-PVT were compared statistically. PVT was identified in 98 patients (10%). Significant differences in hematocrit, international normalized ratio, albumin, bilirubin and glucose were determined between the groups (P<0.05). Out of the thrombophilic risk factors in the patients with PVT factor V Leiden was identified in 8.8%, prothrombin gene 6.6% and methylenetetrahydrofolate reductase 2.2%. There was no difference in survival time between groups (P>0.05).

A mitochondrial neurogastrointestinal encephalomyopathy with intestinal pseudo-obstruction resulted from a novel splice site mutation.

Mitochondrial neurogastrointestinal encephalopathy (MNGIE) is an autosomal recessive disorder characterized by gastrointestinal dysmotility, cachexia, ptosis, peripheral neuropathy and leukoencephalopathy. The diagnosis is often not made until 5-10 years after the onset of symptoms. MNGIE is caused by mutations in thymidine phosphorylase gene TYMP. Here, we present a 19-year-old boy with MNGIE who had a chronic intestinal pseudo-obstruction, and we describe his family history. Genetic analysis revealed a novel homozygous c.765+1G>C intronic mutation which is expected to disrupt splicing of TYMP in the patient. Family screening revealed that the brother was also affected and the mother was a carrier. MNGIE should be considered and genetic testing instigated if individuals with cachexia have neuromuscular complaints or symptoms of chronic intestinal pseudo-obstruction.

Thrombotic thrombocytopenic purpura associated with Brucella infection.

Thrombotic thrombocytopenic purpura (TTP) is characterized by disseminated thrombotic occlusions located in the microcirculation and a syndrome of microangiopathic hemolytic anemia (MAHA), thrombocytopenia, fever, and renal and neurologic abnormalities. Although several factors such as viral and bacterial pathogens, pancreatitis, drugs, collagen-vascular diseases, cancers, and pregnancy have been reported to be associated with TTP, brucellosis is an exceptional cause of this disorder. We represent a 19-year-old woman applying to our outpatient department with the complaints of headache, fever, sweat, malaise, and jaundice. Clinical signs and laboratory findings were consistent with TTP. Brucella agglutination was found to be 1/320 positive. After the administration of therapeutic plasma exchange, all symptoms and laboratory abnormalities improved dramatically. Antibiotic therapy directed to Brucella infection was initiated and no recurrence of TTP was seen.

Protective effect of dexpanthenol against cisplatin-induced hepatotoxicity.

The aim of the present study was to investigate the protective effect of dexpanthenol (Dexp) against cisplatin (Cis)-induced hepatotoxicity. Thirty-two Sprague Dawley rats were divided into four groups: Control group (n=8), Dexp group (n=8, 500 mg/kg/ip/daily single dose/3 days Dexp), Cis group (n=8, 7 mg/kg/ip/single dose Cis) and Cis+Dexp group (n=8, 500 mg/kg/ip/daily single dose/3 days Dexp +7 mg/kg/ip/single dose Cis). MDA, CAT, GSH, GSH-Px, TOS, TAS, OSI, Total Nitrit, IL-1ß, IL-6 and TNF-α levels were analyzed in liver tissue samples. After paraffinization of liver tissue samples, histopathological (congestion, loss of glycogen, number of Kupffer cells) and immunohistochemical (caspase-3 expression) parameters were assessed on the paraffinized liver sections. GSH, TAS, TOS, OSI, Tot Nit, L-Arginine, ADMA and SDMA levels were measured in the serum samples. Statistically significant differences were found between the groups in terms of all liver tissue biochemical parameters, with the exception of IL-1ß and TNF-α levels. GSH, CAT, GSH-Px, TAS and Tot Nit levels were significantly higher in the Cis+Dexp group compared to the Cis group, whereas MDA, TOS, OSI and IL-6 levels were higher in the Cis group. Similarly, serum GSH, TAS, Tot Nit levels were higher in the Cis+Dexp group whereas TOS, L-Arginine, ADMA and SDMA levels were higher in Cis group. There were statistically significant differences between Control and Cis groups in terms of congestion increase, increase of glycogen loss, increase of Kupffer cell number and increase of caspase-3 expression (P<0.001). There was a statistically significant difference between the Cis and the Cis+Dexp groups in terms of histopathologic parameters, with the exception of congestion (P<0.001). To conclude, histopathological, immunohistochemical, and biochemical results of this study demonstrated that Dexp has a protective effect against Cis-induced hepatotoxicity.

Is duodenal biopsy appropriate in areas endemic for Helicobacter pylori?

OBJECTIVE: The primary reason for obtaining duodenal biopsy sample is to diagnose celiac disease. Helicobacter pylori (H. pylori) and drug injury are common causes of duodenitis. The aim of this retrospective study was to explore effects of H. pylori and drugs on duodenal mucosa. METHODS: Duodenal biopsy samples of patients who underwent upper gastrointestinal endoscopy (UGIE) between February 2014 and December 2014 were retrospectively examined. Clinical symptoms, referral indications, endoscopic findings, H. pylori status, and drug history were recorded. Duodenal biopsy findings were compared based on presence of H. pylori and drug history. RESULTS: Of 2389 patients who underwent UGIE, 206 had duodenal biopsy. Eight patients (3.9%) were diagnosed with celiac disease. After excluding cases with celiac disease, 76 patients of remaining 198 patients (36.9%) had duodenal histopathological abnormality. H. pylori was found in 95 (47.9%) patients. Drug usage was less common (42%). Of patients who had histopathological duodenitis, 59% were H. pylori-infected. Rate of duodenitis was higher in H. pylori (+) group than in H. pylori (-) group (45% vs 27.1%; odds ratio, 2.4; 95% confidence interval, 1.3-4.4; p=0.005). There was no difference between groups regarding drug use in terms of histopathological duodenitis. CONCLUSION: H. pylori is the major contributor to duodenitis in high prevalence regions. Serological testing may be more appropriate before performing duodenal biopsy in patients with suspected celiac disease.