Examination of cardiac functions during acute attack and remission period in children with familial Mediterranean fever.

Familial Mediterranean fever (FMF) is an autosomal recessive autoinflammatory disease characterized by recurring serosal inflammation. Cardiac involvement in FMF commonly manifests as pericarditis and pericardial effusion; however, there is limited research on myocardial function. This study aimed to assess cardiac functions during active inflammation and remission periods of FMF patients and investigate the cardiac effects of inflammation during the attack period. Thirty-eight FMF patients without additional cardiac diseases were included in the study. Demographic characteristics, clinical symptoms, family history, and MEFV gene analysis results were obtained retrospectively. Blood tests, blood pressure measurements, electrocardiogram evaluations, conventional echocardiography, and speckle tracking echocardiography were performed during the attack and remission periods. Disease severity was assessed using the Pras scoring system. During the attack period, FMF patients exhibited significantly higher leukocyte count, neutrophil count, C-reactive protein, and erythrocyte sedimentation rate compared to the remission period (p < 0.005). Speckle tracking echocardiography revealed decreased function in the inferior segments of the left ventricle during the attack period (p < 0.005). Right ventricular function was more affected in the moderate disease group. FMF patients with lymphopenia during the attack demonstrated more impaired right ventricular function compared to those with normal lymphocyte count. Conclusions: FMF patients experience cardiac abnormalities during active inflammation, highlighting the importance of monitoring cardiac functions in these patients. Speckle tracking echocardiography can provide valuable insights into cardiac involvement in FMF. These findings emphasize the cardiac impact of FMF inflammation and the significance of long-term cardiac function monitoring in the management of FMF patients. What is Known: • The current literature lacks studies investigating myocardial function in the pediatric population during the attack period of this particular disease. • Our objective was to assess the alterations in cardiac function during the attack and remission periods, considering clinical manifestations, disease severity, acute phase reactant levels, and mutation type. We also evaluated the pattern of cardiac involvement and the affected cardiac areas by comparing remission and attack periods. What is New: • Several studies have demonstrated a rise in the prevalence of ischemic cardiac disease and mortality among individuals with FMF. • Investigating cardiac involvement during the attack period in FMF patients can provide valuable insights for the prevention of long-term complications.

The diagnostic and surgical challenges of small bowel disease not involving the duodenum.

BACKGROUND: Because of the lack of specific signs and symptoms, benign and malignant small bowel disease (SB) diagnoses and their treatments are very difficult. The aim of this study was to determine the challenges of diagnostic and surgical treatment of SB diseases. MATERIAL AND METHODS: Of 51 patients, 29 (56.9%) had undergone surgery for non-malignant small bowel (NMSB) diseases, whereas 22 (43.1%) had malignant small bowel (MSB) diseases. All data were collected and compared between the two groups. RESULTS: Patients with MSB had statistically higher levels of disease in the jejunum (50% versus 10.3%; p=0.004), while patients with NMSB had statistically higher disease levels in the ileum (89.7% versus 50%; p=0.002). Twelve (54.5 %) patients in MSB and 18 (62%) patients in NMSB had emergent laparotomy (P=0.76). There were not significant diferrences in postoperative complications (8 [36.4%] in MSB versus 4 [13.8%] in NMSB; p=0.10 and 5 [22.7%] versus 3 [10.3%]; p=0.374). Patients in the MSB group had a statistically signficant lower 5-year survival rate (p=0.038). CONCLUSION: Overall this study showed that preoperative evaluation may not always be capable of differentiating MSB from NMSB disease. Therefore, most patients present with advanced disease stages. KEY WORDS: Adenocarcinoma, Ileum, Small bowel, Jejenum.

Major complications of adult right lobe living liver donors.

BACKGROUND: The right lobe of the liver is generally preferred for living donor liver transplantation in adult patients with end-stage liver disease. It is important to know the preoperative factors relating to the major postoperative complications. We therefore evaluated the possible risk factors for predicting postoperative complications in right lobe liver donors. METHODS: Data from 378 donors who had undergone right lobe hepatectomy at our center were evaluated retrospectively. The factors we evaluated included donor age, gender, body mass index (BMI), remnant liver volume, operation time, history of previous abdominal surgery, inclusion of the middle hepatic vein and variations in the portal and bile systems. RESULTS: Of the 378 donors, 219 were male and 159 female. None of the donors died, but 124 (32.8%) donors experienced complications including major complications (Clavien scores III and IV) in 27 (7.1%). Univariate analysis showed that complications were significantly associated with male gender and higher BMI (P<0.05), but not with donor age, remnant liver volume, operation time, graft with middle hepatic vein, variations in the portal and bile systems and previous abdominal surgery (P<0.05). Multivariate logistic regression analysis showed that major complications were significantly associated with male gender (P=0.005) and higher BMI (P=0.029). Moreover, the Chi-square test showed that there were significant relationships between major complications and male gender (P=0.010, X2=6.614, df=1) and BMI >25 kg/m2 (P=0.031, X2=8.562, df=1). Of the 96 male donors with BMI >25 kg/m2, 14 (14.6%) with major complications had significantly smaller mean remnant liver volume than those (82, 85.4%) without major complications (32.50%+/-4.45% vs 34.63%+/-3.11%, P=0.029). CONCLUSION: Male donors with BMI >25 kg/m2 and a remnant liver volume ≤32.50% had a significantly increased risk for major complications.

Living Donor Liver Transplantation Outcomes for Hepatocellular Carcinoma Beyond Milan or UCSF Criteria.

Liver transplantation (LT) is the most effective treatment for hepatocellular carcinoma (HCC) that arises from cirrhosis. The Milan and the University of California, San Francisco (UCSF) selection criteria have resulted in major improvements in patient survival. We assessed our outcomes for patients with HCC that were beyond the Milan and UCSF criteria after living donor liver transplantation. We reviewed the data for 109 patients with cirrhosis and HCC who underwent living donor right lobe liver transplantation (living donor liver transplantation; LDLT) during the period from July 2004 to July 2012. Sixteen (14.7 %) patients had HCC recurrences during a mean follow-up of 35.4 ± 26.2 months (range 4-100 months). The mean time to recurrence was 11 ± 9.4 months (range 4-26 months). Survival rates were not significantly different between patients with HCC that met and were beyond the Milan and UCSF criteria (p = 0.761 and p = 0.861, respectively). The Milan and UCSF criteria were not independent risk factors for HCC recurrence or patient survival. Only poorly differentiated tumors were associated with a lower survival rate (OR = 8.656, 95 % confidence interval (CI) 2.01-37.16; p = 0.004). Survival rates for patients with HCC that were beyond conventional selection criteria should encourage reconsidering the acceptable thresholds of these criteria so that more HCC patients may undergo LT without affecting outcomes.

The Role of e-NOS in Chronic Cholestasis-Induced Liver and Renal Injury in Rats: The Effect of N-Acetyl Cysteine.

Introduction. The role of chronic cholestasis (CC) in liver injury and fibrosis remains unclear. The aims of this study were to define the role of endothelial nitric oxide synthase (e-NOS) in CC and the protective effect of N-acetyl-L-cysteine (NAC) in liver and kidney injury. Materials and Methods. Group A (sham group); Group B (CBDL); and Group C (CBDL + NAC). Group C received daily dosage of NAC (100 mg/kg) intraperitoneally for up to 4 weeks. Results. The rate of bridging fibrosis was higher (100% versus 20%, P = .025), but the intensity of e-NOS in liver was lower in rats that received NAC (1.3 versus 2.7, P = .046). The necrotic area in the kidneys among rats that received NAC was lower at week 4 (48% versus 57%; P < .001). The numbers of e-NOS stained cells in kidney were similar in sham group and the two groups with CBDL. Discussion. NAC reduced the stimulus for liver fibrosis in this rat model of CC and attenuated liver and kidney injury. Our study showed that e-NOS expression increased in liver tissue of rats with CC and that this was reversed by NAC. Treatment with NAC might restore e-NOS protein expression and prevent liver injury in CC.

Living donor liver transplantation for obese patients: challenges and outcomes.

Living donor liver transplantation (LDLT) is an accepted option for end-stage liver disease, particularly in countries in which there are organ shortages. However, little is known about LDLT for obese patients. We sought to determine the effects of obesity on pretransplant living donor selection for obese recipients and their outcomes. On the basis of body mass index (BMI) values, 148 patients were classified as normal weight (N), 148 were classified as overweight (OW), and 74 were classified as obese (O). O recipients had significantly greater BMI values (32.1 ± 1.6 versus 23.2 ± 1.9 kg/m(2), P < 0.001) and received larger actual grafts (918.9 ± 173 versus 839.4 ± 162 g, P = 0.002) than recipients with normal BMI values. Donors who donated to O recipients had a greater mean BMI (26.3 ± 3.8 kg/m(2)) than those who donated to N recipients (24.4 ± 3.2 kg/m(2), P = 0.001). Although O recipients were more likely to face some challenges in finding a suitable living donor, there were no differences in graft survival [hazard ratio (HR) = 0.955, 95% confidence interval (CI) = 0.474-1.924, P = 0.90] or recipient survival (HR = 0.90, 95% CI = 0.56-1.5, P = 0.67) between the 3 groups according to an adjusted Cox proportional hazards model. There were no significant differences in posttransplant complication rates between the 3 recipient groups or in the morbidity rates for the donors who donated to O recipients versus the donors who donated to OW and N recipients (P = 0.26). Therefore, we recommend that obese patients undergo pretransplant evaluations. If they are adequately evaluated and selected, they should be considered for LDLT.

Anatomical variations of donor portal vein in right lobe living donor liver transplantation: the safe use of variant portal veins.

In right lobe (RL) living donor liver transplantation (LDLT), portal vein (PV) variations are of immense clinical significance. In this study, we describe in detail our PV reconstruction techniques in RL grafts with variant PV anatomy and evaluate the impact of accompanying biliary variations on the recipient outcomes. In a total of 386 RL LDLTs performed between July 2004 and July 2012, the clinical data on 52 (13%) transplants using RL grafts with variant PV anatomy were retrospectively analyzed. Portal vein anatomy was classified as type 2 in 20 patients, type 3 in 24 patients, and type 4 in eight patients. The PV reconstruction techniques utilized included back-wall plasty (n = 21), back-wall plasty with saphenous vein graft interposition (n = 6), saphenous vein graft interposition (n = 5), cryopreserved iliac vein Y-graft interposition (n = 6), and quiltplasty (n = 3). There was no donor mortality. In a median follow-up of 29 months, none of the recipients had vascular complications. Anomalous PV anatomy was associated with a high (54%) incidence of biliary variations; however, these variations did not result in increased biliary complication rate. Overall, the 1- and 3-year patient survival rates of recipients were 91% and 81%, respectively. Vascular and biliary variations in RL grafts render LDLT technically more challenging. By employing appropriate reconstruction techniques, it is possible to successfully use RL grafts with PV variations without endangering recipient and donor safety.

Roux-en-Y gastric bypass achieves substantial resolution of migraine headache in the severely obese: 9-year experience in 81 patients.

BACKGROUND: Although migraine headache (MH) is more severe in the obese, the risk of developing MH in the obese population is controversial. The effect of surgical weight loss on morbidly obese patients with MH provides a unique opportunity to evaluate this potential association. METHODS: We analyzed the data from 702 morbidly obese patients who underwent Roux-en-Y gastric bypass (RYGB) from 2000 to 2009. We identified patients with physician-diagnosed MH taking antimigraine medication. RESULTS: The data are presented as the mean ± SEM, with the range in parentheses. Of the 102 patients with preoperative MH, 21 were excluded because they had <12-month follow-up data and 81 were followed up for 38.6 ± 3 months (range 12-123). Of the 81 patients, 90% were women. Their body mass index was 48 ± 1 kg/m(2) (range 37-85), and their age was 40 ± 1 years (range 18-62). After surgical weight loss, clinical improvement in MH was seen in 89% of patients within 5.6 ± .9 months (range 1-36; P < .01, chi-square test), with 57 reporting total resolution and 15 reporting partial resolution (9 experienced no change). Using logistic regression analysis, we showed that the improvement in MH after RYGB was independent of the improvement in migraine-associated co-morbidities, such as sleep apnea, menstrual dysfunction, depression, and anxiety. We also compared patients who developed MH after obesity onset with those who had MH before obesity. The MH after obesity onset group included 51 patients, of whom 48 showed clinical improvement (41 complete, 7 partial, and 3 no improvement). The MH before obesity group included 24 patients, of whom 18 showed clinical improvement (11 complete, 7 partial, and 6 no improvement). The MH after obesity group showed a greater rate of complete resolution of MH after RYGB than did the MH before obesity group (P < .01; chi-square test). CONCLUSIONS: Weight loss after RYGB substantially resolves MH, especially when obesity onset precedes MH onset. It remains to be determined whether RYGB-induced endocrine alterations or a reduction in adipokine burden contribute to migraine improvement.

The management of acute cholecystitis in chronic hemodialysis patients: percutaneous cholecystostomy versus cholecystectomy.

INTRODUCTION: Treatment of acute cholecystitis in chronic hemodialysis (HD) patients still remains controversial. Because of underlying disease that can influence surgical results, less invasive alternative managements have been tried over the last decades. The goal of this study was to analyze the results of cholecystectomy versus percutaneous cholecystostomy for acute cholecystitis (AC) in chronic HD patients. METHODS: All patients with end-stage renal disease who were treated for AC were identified retrospectively from our medical records. Between July 2007 and September 2011, 47 patients were treated for AC while they were on chronic HD. The records of these patients were reviewed for documented AC and its treatment. RESULTS: Of the 47 HD patients, 26 (55.3 %) underwent cholecystectomy (CC), while 21 (44. 7 %) had a percutaneous cholecystostomy (PC) for AC as an initial treatment. The mean length of follow-up was 20.4 ± 16 months in PC and 18 ± 15 months in CC patients. The success rate was higher in CC patients compared to PC patients (92. 3 versus 66.7 %, p = 0.0698). Eleven (52. 4 %) patients who had PC subsequently underwent CC; six open CC and five delayed laparoscopic CC were performed. Of the 26 patients who underwent CC, 18 were performed emergently due to the persistence of AC-related symptoms and gangrenous and perforated gallbladders. Eight were initially treated conservatively and then underwent elective cholecystectomy at an interval of 32 ± 24 (range = 14-59) days following initial treatment. In emergent CC, 10 (55.6 %) were completed laparoscopically, three were open, and five (33.3 %) had conversions. In elective CC patients, two were conversions, but the remainder (75 %) had laparoscopic CC. Readmission rates were higher in the PC group (33.3 versus 12.5 %, p = 0.1732). Although AC-related mortality was higher in PC patients, there was no statistically significant difference in the patient survival rate between the two groups (Kaplan-Meier analysis, Fig. 1, 19 versus 7.7 %; p = 0.4035), and the overall mortality rate was higher in the PC group (33.7 versus 15.7 %, p = 0.2737). CONCLUSION: This study confirms that the safety and effectiveness of CC has a higher success rate and lower morbidity and mortality rate compared with percutaneous cholecystostomy for acute cholecystitis in chronic HD patients.

Pancreatic metastasis of high-grade papillary serous ovarian carcinoma mimicking primary pancreas cancer: a case report.

Introduction. Reports of epithelial ovarian carcinomas metastatic to the pancreas are very rare. We herein present a metastasis of high grade papillary serous ovarian cancer to mid portion of pancreas. Case. A 42-year-old patient was admitted with a non-specified malignant cystic lesion in midportion of pancreas. She had a history of surgical treatment for papillary serous ovarian adenocarcinoma. A cystic lesion was revealed by an abdominal computerized tomography (CT) performed in her follow up . It was considered as primary mid portion of pancreatic cancer and a distal pancreatectomy was performed. The final pathology showed high-grade papillary serous adenocarcinoma morphologically similar to the previously diagnosed ovarian cancer. Discussion. Metastatic pancreatic cancers should be considered in patients who present with a solitary pancreatic mass and had a previous non-pancreatic malignancy. Differential diagnosis of primary pancreatic neoplasm from metastatic malignancy may be very difficult. A biopsy for tissue confirmation is required to differentiate primary and secondary pancreatic tumors. Although, the value of surgical resection is poorly documented, resection may be considered in selected patients. Conclusion. Pancreatic metastasis of ovarian papillary serous adenocarcinoma has to be kept in mind when a patient with pancreatic mass has a history of ovarian malignancy.

Roux-en-Y gastric bypass ameliorates polycystic ovary syndrome and dramatically improves conception rates: a 9-year analysis.

BACKGROUND: Polycystic ovary syndrome (PCOS) is a common cause of infertility, especially in the morbidly obese. We evaluated the long-term effects of Roux-en-Y gastric bypass on PCOS and infertility. METHODS: A total of 566 morbidly obese women underwent Roux-en-Y gastric bypass from 2000 to 2009. A total of 31 patients (5.5%) had a history of PCOS. Of the 31 patients, 6 were postmenopausal and 5 lost to follow-up and were excluded. Telephone interviews were conducted with the 20 eligible patients. RESULTS: The mean age and body mass index was 32 ± 5.8 years (range 22-42) and 52.8 ± 9.08 kg/m(2) (range 37-76) before surgery. All 20 patients had ≥ 2 of 3 diagnostic criteria for PCOS, including clinical or biochemical evidence of hyperandrogenism, anovulation, or polycystic ovaries. Of these, 85% had oligomenorrhea, 70% had hirsutism, and 45% had type 2 diabetes mellitus with medication. Before surgery, 8 patients conceived with or without hormonal treatment, 2 did not desire pregnancy, and 10 did not conceive. The mean follow-up was 46.7 months. After surgical weight loss, menstruation was corrected in 82%, hirsutism had resolved in 29%, and 77.8% of those with diabetes had complete remission. Of the 10 patients who did not conceive before surgery, 4 no longer desired pregnancy, and the remaining 6 patients had become pregnant within 3 years of surgery-5 without any hormonal treatment and 1 with in utero insemination. CONCLUSION: Surgical weight loss after Roux-en-Y gastric bypass achieves excellent amelioration of PCOS manifestations and the postoperative conception rate in infertile PCOS subjects desiring pregnancy was 100%.