Case report: Pai syndrome with multiple ventricular septal defect and without cleft palate.

Pai syndrome is described as the association of a midline cleft lip, midline facial polyps, and lipoma of the central nervous system. However, only a few patients present the full triad, and most exhibit a wide spectrum of phenotypic variability. Its entire clinical spectrum is still poorly delineated and the etiology remains unknown. In this report, a newborn was presented with congenital nasal septal lipoma, lipoma of the corpus callosum, multiple ventricular septal defect, and additional minor facial dysmorphism. This entity, multiple ventricular septal defect, which has never been reported in PS. Cytogenetic analysis showed normal male 46, XY karyotype. Chromosomal microarray analysis (750 K array) was also unremarkable. This case draws attention with the presence of multiple ventricular septal defect in Pai syndrome and is important in terms of providing phenotypic diversity. To our knowledge, this is also the first genetically evaluated case of Pai syndrome from Turkey.

Association of cardiometabolic risks with body composition in hemodialysis patients.

OBJECTIVE: High body fat ratio is known as one of the main reasons that increase the risk of cardiovascular disease. This study examined the relationship between body composition and cardiometabolic risks in hemodialysis (HD) patients. PATIENTS AND METHODS: This study was conducted with chronic kidney disease (CKD) patients who received HD treatment between March 2020 and September 2021. Anthropometric measurements of the individuals and their body composition analyses were performed using the bioelectrical impedance analysis (BIA) method. Framingham risk scores were calculated in order to determine the cardiometabolic risk factors of individuals. RESULTS: According to the Framingham risk score, 15.96% of individuals were found to have a high cardiometabolic risk. The lean-fat tissue index (LTI)/(FTI), body shape index (BSI) and visceral adiposity index (VAI) (female-male) values of individuals with high risk according to the Framingham risk score were found to be 11.34±2.29, 13.52±2.88, 8.50±3.89, 9.60±3.07, 0.086±0.024, respectively. The effect of anthropometric measurements in estimating the Framingham risk score was examined using the linear regression analysis. The regression analysis performed with BMI, LTI, VAI values, it was determined that 1-unit increase in VAI increased the Framingham risk score by 1,468 units (OR: 0.951-1.952) (p=0.002). CONCLUSIONS: It has been discovered that indices indicating adipose tissue increase the Framingham risk score in HD patients, independent of BMI. It is recommended to evaluate the ratios showing body fat ratio in cardiovascular diseases.

Multiple factors influencing mortality in hemodialysis patients.

OBJECTIVE: Both decreased food intake and elevated inflammation contribute to malnutrition in hemodialysis (HD) patients. Malnutrition, inflammation, anthropometric measurements, and other comorbidity factors were investigated in this study as potential indicators of mortality in HD patients. PATIENTS AND METHODS: By measuring geriatric nutritional risk index (GNRI), malnutrition inflammation score (MIS), and prognostic nutritional index (PNI), 334 HD patients' nutritional status was assessed. Through the use of four different models and logistic regression analysis, the predictors of each individual's survival status were examined. The models were matched using the Hosmer-Lemeshow test. On the survival of patients, the effects of malnutrition indices in Model 1, anthropometric measurements in Model 2, blood parameters in Model 3, and sociodemographic characteristics in Model 4 were investigated. RESULTS: Five years later, 286 individuals were still on hemodialysis. Patients who had a high GNRI value had a lower mortality rate in Model 1. The body mass index (BMI) value of the patients was found to be the best predictor of mortality in Model 2, and it was found that patients with high muscle percentage had a lower mortality risk. The difference in urea level measured at the beginning and end of hemodialysis was found to be the most potent predictor of mortality in Model 3, although C-reactive protein (CRP) level was also discovered to be one of the best predictors for this model. The final model, Model 4, revealed that mortality was lower in women than in men and that income status was a reliable predictor of mortality estimation. CONCLUSIONS: The best indicator of mortality in hemodialysis patients is the malnutrition index.

Effects of infliximab against carbon tetrachloride-induced spleen toxicity in rats.

OBJECTIVE: Carbon tetrachloride (CCl4) is a non-polar molecule used in industry in grain curing, insect-killing and especially in the production of chlorofluorocarbons. It is estimated that an average of 70,000 industry workers in Europe are exposed to this toxic compound. MATERIALS AND METHODS: Twenty-four male Sprague-Dawley rats were randomly allocated into four groups: control group (saline only, Group I), infliximab (INF) group (Group II), CCl4 group (Group III) and CCl4+INF group (Group IV). RESULTS: While there was an increase in the numerical density of CD3, CD68, and CD200R positive T lymphocytes and macrophages in the CCl4 administration group (p=0.000), this was not the case in the CCl4+INF administration group (p=0.000). CONCLUSIONS: TNF-α inhibitors have a protective effect against CCl4-induced spleen toxicity/inflammation as seen by the reduction in CD3, CD68, CD200R positive T lymphocytes and macrophages.

Los principales patrones de la trombosis maligna relacionada con el carcinoma hepatocelular en la 18F-FDG PET/TC / Different patterns of hepatocellular carcinoma-related malign thrombosis in 18F-FDG PET/CT

El trombo tumoral es una extensión de tumor maligno intravascular que puede ocurrir en varios tipos de cáncer. Los carcinomas hepatocelulares (CHC) son causas comunes de trombo maligno. La presencia de un trombo maligno debido al CHC tiene un pronóstico sombrío, que afecta las opciones de tratamiento. Presentamos 3 casos de trombos tumorales debidos a CHC avanzado detectado por 18F-FDG PET/TC

Tumor thrombus is an intravascular malign tumor extension that may occur in various types of cancer. Hepatocellular carcinomas (HCC) are common causes of malign thrombus. The presence of a malign thrombus due to HCC has a dismal prognosis, which affects treatment choices. We present three cases of tumor thrombi due to advanced HCC detected by 18F-FDG PET/CT

Randomized controlled trial of 8 weeks' vs 12 weeks' interval between neoadjuvant chemoradiotherapy and surgery for locally advanced rectal cancer.

AIM: The aim was to compare the pathological complete response (pCR) rate at 8 compared to 12 weeks' interval between completion of neoadjuvant chemoradiotherapy (CRT) and surgery in patients with locally advanced rectal cancer. METHOD: This was a randomized trial which included a total of 330 patients from two institutions. Patients with locally advanced (T3-4N0M0, TxN+M0) rectal cancer were randomized into 8- and 12-week interval groups. All the patients received long-course CRT (45 Gy in 1.8 Gy fractions and concomitant oral capecitabine or 5-fluorouracil infusion). Surgery was performed at either 8 or 12 weeks after CRT. The primary end-point was pCR. Secondary end-points were sphincter preservation, postoperative morbidity and mortality. RESULTS: Two-hundred and fifty-two patients (n = 125 in the 8-week group, n = 127 in the 12-week group) were included. Demographic and clinical characteristics were similar between groups. The overall pCR rate was 17.9% (n = 45): 12% (n = 15) in the 8-week group and 23.6% (n = 30) in the 12-week group (P = 0.021). Sphincter-preserving surgery was performed in 107 (85.6%) patients which was significantly higher than the 94 (74%) patients in the 12-week group (P = 0.016). Postoperative mortality was seen in three (1.2%) patients overall and was not different between groups (1.6% in 8 weeks vs 0.8% in 12 weeks, P = 0.494). Groups were similar in anastomotic leak (10.8% in 8 weeks vs 4.5% in 12 weeks, P = 0.088) and morbidity (30.4% in 8 weeks and 20.1% in 12 weeks, P = 0.083). CONCLUSION: Extending the interval between CRT and surgery from 8 to 12 weeks resulted in a 2-fold increase in pCR rate without any difference in mortality and morbidity.

Different patterns of hepatocellular carcinoma-related malign thrombosis in 18F-FDG PET/CT. / Los principales patrones de la trombosis maligna relacionada con el carcinoma hepatocelular en la 18F-FDG PET/TC.

Tumor thrombus is an intravascular malign tumor extension that may occur in various types of cancer. Hepatocellular carcinomas (HCC) are common causes of malign thrombus. The presence of a malign thrombus due to HCC has a dismal prognosis, which affects treatment choices. We present three cases of tumor thrombi due to advanced HCC detected by 18F-FDG PET/CT.

Weaning practices in phenylketonuria vary between health professionals in Europe.

BACKGROUND: In phenylketonuria (PKU), weaning is considered more challenging when compared to feeding healthy infants. The primary aim of weaning is to gradually replace natural protein from breast milk or standard infant formula with solids containing equivalent phenylalanine (Phe). In addition, a Phe-free second stage L-amino acid supplement is usually recommended from around 6 months to replace Phe-free infant formula. Our aim was to assess different weaning approaches used by health professionals across Europe. METHODS: A cross sectional questionnaire (survey monkey®) composed of 31 multiple and single choice questions was sent to European colleagues caring for inherited metabolic disorders (IMD). Centres were grouped into geographical regions for analysis. RESULTS: Weaning started at 17-26 weeks in 85% (n = 81/95) of centres, >26 weeks in 12% (n = 11/95) and < 17 weeks in 3% (n = 3/95). Infant's showing an interest in solid foods, and their age, were important determinant factors influencing weaning commencement. 51% (n = 48/95) of centres introduced Phe containing foods at 17-26 weeks and 48% (n = 46/95) at >26 weeks. First solids were mainly low Phe vegetables (59%, n = 56/95) and fruit (34%, n = 32/95).A Phe exchange system to allocate dietary Phe was used by 52% (n = 49/95) of centres predominantly from Northern and Southern Europe and 48% (n = 46/95) calculated most Phe containing food sources (all centres in Eastern Europe and the majority from Germany and Austria). Some centres used a combination of both methods.A second stage Phe-free L-amino acid supplement containing a higher protein equivalent was introduced by 41% (n = 39/95) of centres at infant age 26-36 weeks (mainly from Germany, Austria, Northern and Eastern Europe) and 37% (n = 35/95) at infant age > 1y mainly from Southern Europe. 53% (n = 50/95) of centres recommended a second stage Phe-free L-amino acid supplement in a spoonable or semi-solid form. CONCLUSIONS: Weaning strategies vary throughout European PKU centres. There is evidence to suggest that different infant weaning strategies may influence longer term adherence to the PKU diet or acceptance of Phe-free L-amino acid supplements; rendering prospective long-term studies important. It is essential to identify an effective weaning strategy that reduces caregiver burden but is associated with acceptable dietary adherence and optimal infant feeding development.

Early feeding practices in infants with phenylketonuria across Europe.

BACKGROUND: In infants with phenylketonuria (PKU), dietary management is based on lowering and titrating phenylalanine (Phe) intake from breast milk or standard infant formula in combination with a Phe-free infant formula in order to maintain blood Phe levels within target range. Professionals use different methods to feed infants with PKU and our survey aimed to document practices across Europe. METHODS: We sent a cross sectional, survey monkey® questionnaire to European health professionals working in IMD. It contained 31 open and multiple-choice questions. The results were analysed according to different geographical regions. RESULTS: Ninety-five centres from 21 countries responded. Over 60% of centres commenced diet in infants by age 10 days, with 58% of centres implementing newborn screening by day 3 post birth. At diagnosis, infant hospital admission occurred in 61% of metabolic centres, mainly in Eastern, Western and Southern Europe. Breastfeeding fell sharply following diagnosis with only 30% of women still breast feeding at 6 months.53% of centres gave pre-measured Phe-free infant formula before each breast feed and 23% alternated breast feeds with Phe-free infant formula. With standard infant formula feeds, measured amounts were followed by Phe-free infant formula to satiety in 37% of centres (n = 35/95), whereas 44% (n = 42/95) advised mixing both formulas together. Weaning commenced between 17 and 26 weeks in 85% centres, ≥26 weeks in 12% and < 17 weeks in 3%. DISCUSSION: This is the largest European survey completed on PKU infant feeding practices. It is evident that practices varied widely across Europe, and the practicalities of infant feeding in PKU received little focus in the PKU European Guidelines (2017). There are few reports comparing different feeding techniques with blood Phe control, Phe fluctuations and growth. Controlled prospective studies are necessary to assess how different infant feeding practices may influence longer term feeding development.

¿Pueden contribuir los parámetros tumorales metabólicos, establecidos mediante 18F-FDG PET/TC para estadificación primaria, a la estratificación del riesgo de los linfomas del sistema nervioso central, para el tratamiento del paciente como modelo pronóstico? / Can metabolic tumor parameters on primary staging 18F-FDG PET/CT aid in risk stratification of primary central nervous system lymphomas for patient management as a prognostic model?

Objetivo. Los linfomas del sistema nervioso central (SNC) son linfomas no Hodgkin extranodales, agresivos y fatales, albergados en el SNC en el diagnóstico inicial. Su pronóstico es malo, teniendo la enfermedad un resultado fatal en comparación con el linfoma no Hodgkin sistémico. Se han indicado una serie de sistemas de estratificación del riesgo para estimar el pronóstico, basados principalmente en el nivel sérico de lactato deshidrogenasa, la edad, el índice de Karnofsky, el compromiso de las estructuras profundas del cerebro y la concentración proteica del líquido cefalorraquídeo. La PET/TC con 18F-FDG tiene un elevado valor pronóstico en cuanto a la supervivencia global y la supervivencia libre de enfermedad en muchos cánceres y linfomas. Nuestro objetivo fue investigar los índices tumorales metabólicos mediante 18F-FDG PET/TC para estadificación primaria, como marcadores pronósticos de los linfomas primarios del SNC. Material y métodos. Se incluyó en este estudio retrospectivo a una cohorte de 14 pacientes con linfoma primario de células B grandes del SNC (estadio i). Se realizó una PET/TC con 18F-FDG de estadificación primaria, calculándose en todos los pacientes, y con anterioridad al tratamiento, los parámetros cuantitativos tales como el valor estandarizado de captación máximo, el valor de captación medio, el volumen tumoral metabólico y la glucólisis tumoral total (TLG). Se realizaron modelos de regresión de Cox para determinar su relación con el tiempo de supervivencia. Resultados. En la evaluación de todos los factores de riesgo potencial con impacto sobre la recidiva/metástasis (edad, sexo, lactato deshidrogenasa sérico, compromiso de las estructuras profundas del cerebro, el valor estandarizado de captación máximo, el valor de captación medio, el volumen tumoral metabólico y la TLG), realizada mediante análisis univariante, el valor de TLG reflejó una significación estadística (p=0,02). Conclusión. Los parámetros tumorales metabólicos resultan de utilidad para la estimación pronóstica de los linfomas primarios del SNC. En especial, la TLG constituye el parámetro más importante, y puede desempeñar un papel en el tratamiento del paciente (AU)

Objective. Primary central nervous system (CNS) lymphoma is an aggressive and fatal extranodal non-Hodgkin lymphoma jailed in CNS at initial diagnosis. Its prognosis is poor and the disease has a fatal outcome when compared with systemic non-Hodgkin lymphoma. A few baseline risk stratification scoring systems have been suggested to estimate the prognosis mainly based on serum lactate dehydrogenase level,age, Karnofsky performance score, involvement of deep brain structures and cerebrospinal fluid protein concentration. 18F-FDG PET/CT has a high prognostic value with respect to overall survival and disease-free survival in many cancers and lymphomas. We aimed to investigate metabolic tumor indexes on primary staging 18F-FDG PET/CT as prognostic markers in primary CNS lymphoma. Material and methods. Fourteen patients with primary CNS diffuse large B-cell lymphoma (stage i) were enrolled in this retrospective cohort study. Primary staging 18F-FDG PET/CT was performed and quantitative parameters like maximum standardized uptake value, average standardized uptake value, metabolic tumor volume and total lesion glycolysis (TLG) were calculated for all patients before the treatment. Cox regression models were performed to determine their relation with survival time. Results. In the evaluation of all potential risk factors impacting recurrence/metastases (age, sex, serum lactate dehydrogenase, involvement of deep brain structures, maximum standardized uptake value, average standardized uptake value, metabolic tumor volume, and TLG) with univariate analysis, TLG remained statistically significant (P=.02). Conclusion. Metabolic tumor parameters are useful in prognosis estimation of primary CNS lymphomas, especially TLG, which is the most important one and may play a role in patient management (AU)

First report of carglumic acid in a patient with citrullinemia type 1 (argininosuccinate synthetase deficiency).

WHAT IS KNOWN AND OBJECTIVE: Carglumic acid is a structural analogue of human N-acetylglutamate, which has become an alternative therapeutic option for hyperammonaemia in organic acidaemias such as isovaleric acidaemia, methylmalonic acidaemia and propionic acidaemia, and it has been suggested in other urea cycle disorders such as ornithine transcarbamylase deficiency and carbamoyl phosphate synthetase 1 deficiency. CASE DESCRIPTION: A male newborn was diagnosed with citrullinemia after serum amino acid analyses revealed markedly elevated citrulline concentration together with homozygous p.Gly390Arg mutation in ASS1 gene. The ammonia concentration decreased and blood gas analysis normalized after peritoneal dialysis was performed for three days. Also, sodium benzoate, L-arginine and parenteral nutrition with glucose and lipid therapy were initiated. Until 1 year of age, low adherence to sodium benzoate therapy due to unpleasant taste caused hyperammonaemic episodes and obligated us to initiate carglumic acid (100 mg/kg/day) therapy. During treatment with carglumic acid, the median ammonia level was 45.6 µmol/L. The patient's treatment was switched from carglumic acid to sodium phenylbutyrate when he was 4.5 years old. Currently, the patient is 6.5 years old and remains under follow-up with sodium phenylbutyrate, L-arginine and protein-restricted diet. Plasma ornithine level was found to be significantly lower during the carglumic acid treatment compared to other treatments (P=.039). Also, glutamic acid was found to be higher during the sodium benzoate treatment period compared to other treatment periods (P=.024). WHAT IS NEW AND CONCLUSION: To the best of our knowledge, this is the first report describing the long-term use of carglumic acid in a patient with argininosuccinate synthetase deficiency.

Can metabolic tumor parameters on primary staging 18F-FDG PET/CT aid in risk stratification of primary central nervous system lymphomas for patient management as a prognostic model? / ¿Pueden contribuir los parámetros tumorales metabólicos, establecidos mediante 18F-FDG PET/TC para estadificación primaria, a la estratificación del riesgo de los linfomas del sistema nervioso central, para el tratamiento del paciente como modelo pronóstico?

OBJECTIVE: Primary central nervous system (CNS) lymphoma is an aggressive and fatal extranodal non-Hodgkin lymphoma jailed in CNS at initial diagnosis. Its prognosis is poor and the disease has a fatal outcome when compared with systemic non-Hodgkin lymphoma. A few baseline risk stratification scoring systems have been suggested to estimate the prognosis mainly based on serum lactate dehydrogenase level,age, Karnofsky performance score, involvement of deep brain structures and cerebrospinal fluid protein concentration. 18F-FDG PET/CT has a high prognostic value with respect to overall survival and disease-free survival in many cancers and lymphomas. We aimed to investigate metabolic tumor indexes on primary staging 18F-FDG PET/CT as prognostic markers in primary CNS lymphoma. MATERIAL AND METHODS: Fourteen patients with primary CNS diffuse large B-cell lymphoma (stage i) were enrolled in this retrospective cohort study. Primary staging 18F-FDG PET/CT was performed and quantitative parameters like maximum standardized uptake value, average standardized uptake value, metabolic tumor volume and total lesion glycolysis (TLG) were calculated for all patients before the treatment. Cox regression models were performed to determine their relation with survival time. RESULTS: In the evaluation of all potential risk factors impacting recurrence/metastases (age, sex, serum lactate dehydrogenase, involvement of deep brain structures, maximum standardized uptake value, average standardized uptake value, metabolic tumor volume, and TLG) with univariate analysis, TLG remained statistically significant (P=.02). CONCLUSION: Metabolic tumor parameters are useful in prognosis estimation of primary CNS lymphomas, especially TLG, which is the most important one and may play a role in patient management.

A different scintigraphic approach to evaluate the glomerular filtration rate / Una técnica gammagráfica diferente para evaluar la tasa de filtración glomerular

Objective: Multiple nuclear medicine techniques for measuring renal glomerular filtration rate (GFR) are available but some of them are not practical in daily routine use and others have some accuracy issues. Hence the aim of the study was to design a new camera-based approach to measure the GFR and to compare our results with other measured GFR (mGFR) and estimated GFRs (eGFRs) derived from available measurements and equations used in daily clinical practice. Material and methods: 34 patients were included in the study. ∼74MBq (2mCi) Technetium 99m diethylene-triamine-pentaacetic acid (99mTc-DTPA) was administered to the patients during 5min. A simple formula based on a dilution principle was used to measure GFR (ScinGFR). Results: Our formula provided similar mGFR results in narrower range as creatinine clearance did and our results correlated well with results derived from other equations. When ScinGFR values were compared to others, there was a significant difference among them (p=0.031) due to difference between the ScinGFR and Cockroft-Gault. When the results of the ScinGFR compared to others without Cockroft-Gault, the difference among them was not significant (p=0.164). Conclusion: A simple formula considering the extracellular fluid volume was used to predict the split and global kidney functions and despite some discrepancies, good correlation among our results and those derived from available formulas was detected (AU)

Objetivo: Existen múltiples técnicas de medicina nuclear disponibles para medir la tasa de filtración glomerular (GFR), aunque algunas de ellas no resultan muy útiles en la rutina diaria, y otras no son muy precisas. Por tanto, el objetivo de este estudio fue diseñar una nueva técnica basada en el uso de una cámara para medir la GFR y comparar nuestros resultados con otras mediciones de GFR (mGFR) y estimaciones de GFR (eGFRs), derivadas de las mediciones disponibles y las ecuaciones utilizadas en la práctica clínica diaria. Material y métodos: Se incluyó en el estudio a 34 pacientes. Se infundió alrededor de 74MBq (2mCi) de 99mTc-ácido dietilen-triamin-pentaacético (99mTc-DTPA) a los pacientes durante 5min. Se utilizó una fórmula simple, basada en un principio de disolución, para medir la GFR (ScinGFR). Resultados: Nuestra fórmula aportó resultados similares de mGFR en el rango más estrecho logrado por la depuración de creatinina, guardando una correlación muy positiva con los resultados derivados de otras ecuaciones. Al comparar los valores de ScinGFR con otros valores, se produjo una diferencia estadísticamente significativa entre ellos (p=0.031), debido a la diferencia entre ScinGFR y Cockroft-Gault. Al comparar los resultados de ScinGFR con otros valores, sin considerar Cockroft-Gault, la diferencia entre ellos fue estadísticamente significativa (p=0.164) Conclusión: Se utilizó una fórmula simple considerando el volumen de líquido extracelular, para predecir las funciones globales e individuales renales y, a pesar de ciertas discrepancias, se detectó una buena correlación entre nuestros resultados y aquellos derivados de las fórmulas disponibles (AU)

A different scintigraphic approach to evaluate the glomerular filtration rate.

OBJECTIVE: Multiple nuclear medicine techniques for measuring renal glomerular filtration rate (GFR) are available but some of them are not practical in daily routine use and others have some accuracy issues. Hence the aim of the study was to design a new camera-based approach to measure the GFR and to compare our results with other measured GFR (mGFR) and estimated GFRs (eGFRs) derived from available measurements and equations used in daily clinical practice. MATERIAL AND METHODS: 34 patients were included in the study. ∼74MBq (2mCi) Technetium 99m diethylene-triamine-pentaacetic acid (99mTc-DTPA) was administered to the patients during 5min. A simple formula based on a dilution principle was used to measure GFR (ScinGFR). RESULTS: Our formula provided similar mGFR results in narrower range as creatinine clearance did and our results correlated well with results derived from other equations. When ScinGFR values were compared to others, there was a significant difference among them (p=0.031) due to difference between the ScinGFR and Cockroft-Gault. When the results of the ScinGFR compared to others without Cockroft-Gault, the difference among them was not significant (p=0.164). CONCLUSION: A simple formula considering the extracellular fluid volume was used to predict the split and global kidney functions and despite some discrepancies, good correlation among our results and those derived from available formulas was detected.

Long-term Results of Living Donors in Simultaneous Kidney and Liver Transplantations.

INTRODUCTION: Because of the shortage of organs available for transplantation, living related sequential transplantation with the use of liver and a kidney from the same donor has emerged as a reasonable therapeutic alternative. However, there is insufficient literature about the complications that living donors experience after simultaneous kidney and liver transplantations. METHODS: From December 2001 to October 2009, 5 living donors provided simultaneous donation of livers and kidneys and 1 living donor donated first her kidney and then her liver. Demographic data of the donors and information concerning the surgery and postoperative observation were collected prospectively. RESULTS: All of the donors were female. The median age was 27.5 (range, 19-36) years. Indications requiring the simultaneous transplantation of livers and kidneys were primary hyperoxaluria type 1 (PH1) in 5 potential recipients and cirrhosis due to chronic hepatitis B infection and idiopathic chronic renal insufficiency in 1 potential recipient. Four recipients underwent right hepatectomy (segments 5-8) and right nephrectomy; 1 recipient underwent left hepatectomy (segments 2-4) and right nephrectomy; and 1 recipient underwent left lobectomy (segments 2-3) and right nephrectomy. There were no complications except in 1 donor (postoperative ileus). No donor developed hypertension or microalbuminuria. CONCLUSIONS: With the right indications, appropriate preoperative evaluation, meticulous surgical technique, proper postoperative care, and long-term close monitoring to minimize morbidity and mortality risks, liver and kidney donation from the same donor can be considered for simultaneous kidney and liver transplantation.

Functionalized imidazolium and benzimidazolium salts as paraoxonase 1 inhibitors: Synthesis, characterization and molecular docking studies.

Paraoxonase (PON) is a key enzyme in metabolism of living organisms and decreased activity of PON1 was acknowledged as a risk for atherosclerosis and organophosphate toxicity. The present study describes the synthesis, characterization, PON1 inhibitory properties and molecular docking studies of functionalized imidazolium and benzimidazolium salts (1a-5g). The structures of all compounds were elucidated by IR, NMR, elemental analysis and structures of compounds 2b and 2c were characterized by single-crystal X-ray diffraction. Compound 1c, a coumarin substituted imidazolium salt showed the best inhibitory effect on the activity of PON1 with good IC50 value (6.37 µM). Kinetic investigation was evaluated for this compound and results showed that this compound is competitive inhibitor of PON1 with Ki value of 2.39 µM. Molecular docking studies were also performed for most active compound 1c and one of least active compound 2c in order to determine the probable binding model into active site of PON1 and validation of the experimental results.

Cytoreductive surgery (SRC) and hyperthermic intraperitoneal chemotherapy (HIPEC) for treatment of peritoneal carcinomatosis: Our initial experience and technical details.

OBJECTIVE: The aim of this study is to present our initial experience in peritoneal carcinomatosis treatment and the technical details of cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) in the light of current literature. MATERIAL AND METHODS: Data of 27 consecutive patients who were treated with CRS and HIPEC for peritoneal carcinomatosis in Medical Park Samsun Hospital, between November 2012 and September 2014 were retrospectively reviewed. Treatment indication and management were evaluated at the multidisciplinary oncology council. All patients underwent CRS and HIPEC with the aim of complete cytoreduction. Patients with unresectable disease and/or palliative surgery were excluded from analysis. Perioperative complications were classified according to Clavien-Dindo classification, and HIPEC-related side effects were identified using National Cancer Institute Common Terminology Criteria for Adverse Events (CTCAE) criteria. Demographic, clinical and histopathological data of the patients were analyzed. RESULTS: The mean age was 54 (32-72). Nineteen patients were female. The origin of peritoneal carcinomatosis was colorectal cancer in 12 patients, ovarian cancer in 12 patients, gastric cancer in 2 patients and pseudomyxoma peritonei in 1 patient. The mean Peritoneal Carcinomatosis Index was 12 (3-32), with a mean operative time of 420 (300-660) minutes. Perioperative morbidity, HIPEC-related toxicity and perioperative mortality were observed in eight (30%), one (3.7%) and four patients (14.8%), respectively. During a mean follow up of 13 (1-22) months, overall and disease-free survival rates were 95.8% and 82.6%, respectively. Two patients with colorectal cancer (after 9 and 12 months) and one patient with ovarian cancer (after 11 months) had intra-abdominal recurrence. One patient with ovarian cancer had liver metastases 13 months after surgery, and underwent resection of segments 6-7. The remaining patients are being followed-up without any recurrence. CONCLUSION: Cytoreductive surgery and HIPEC have favorable results in the treatment of patients with peritoneal carcinomatosis. Compatible with the literature, surgical outcomes of the presented series are encouraging for this treatment modality that have been recently popularized in our country. Careful perioperative evaluation, proper patient selection and multidisciplinary approach are essential for success in curative treatment of peritoneal carcinomatosis.

Evaluation of the intraocular pressure in obese adolescents.

AIM: The aim of this study was to evaluate the intraocular pressure (IOP) levels of children with normal and high Body Mass Index (BMI) and to find out the potential correlation between glaucoma and obesity. METHODS: Thirty obese and thirty healty children were enrolled in this study. Physical examinations and anthropometric measurements of all patients and controls were performed. Obesity was defined as a BMI exceeding the 95th percentile for the patients according to age and sex. All participants were underwent a complete eye examination. The results of these measurements were considered for statistical analysis. RESULTS: Mean age was 13.5±2.1 years in obese group and 13.3±2.0 years in control group. Mean BMIs were 28.1±3.9 kg/m2 and 19.7±1.2 kg/m2 in obese and control groups, respectively. No significant difference was found in mean IOP levels of both right and left eyes between two groups (P=0.837 and P=0.755, respectively). There was no significant difference in cup/disc ratios of each eyes and mean central corneal thickness of both right and left eyes between obese patients and controls. In visual field analysis, no statistically significant difference in mean false negativeness and mean false positiveness were found between two groups. There were not also any significant correlations in both mean deviation of each eyes (P=0.78 and P=0.94, respectively) and pattern standart deviation of right and left eyes (P=0.89 and P=0.90, respectively) between obese cases and controls. CONCLUSION: In this study, there were no significant difference in IOP measurements, central corneal thicknesses, cup/disc ratios and visual field parameters between obese and normal children. No significant correlation was found between obesity and glaucoma or elevated IOP in children.

Direct and solvent-assisted keto-enol tautomerism and hydrogen-bonding interactions in 4-(m-chlorobenzylamino)-3-phenyl-4,5-dihydro-1H-1,2,4-triazol-5-one: a quantum-chemical study.

The tautomeric equilibrium of the title triazole compound was computationally analyzed at the B3LYP/6-311++G(d,p) and MP2/6-311++G(d,p) levels of theory. The solvent effect was considered for three solvents (chloroform, methanol, and water). Two distinct mechanisms were applied: a direct intramolecular transfer using the polarizable continuum model (PCM) and a solvent-assisted mechanism. The calculations indicated that the keto form is more stable in all cases. It was found that the barrier heights for the tautomerization reaction are very high, indicating a relatively disfavored process. Although the barrier heights for solvent-assisted reactions are significantly lower than those for the unassisted tautomerization reaction, implying the importance of the superior catalytic effect of the solvents, monosolvation was not found to be sufficient for the reaction to occur. Finally, the two intermolecular hydrogen-bonding interactions in the crystal structure were investigated in the gas phase; according to the calculated energies and structural parameters, the order of stability is N3-H3···O1 > N1-H1···O1.

Evaluation of serum xenin and ghrelin levels and their relationship with nonalcoholic fatty liver disease and insulin resistance in obese adolescents.

AIM: Xenin is a peptide of the neurotensin/xenopsin/xenin family secreted from gastric cells and other tissues. The first aim of this study was to investigate the serum xenin and ghrelin levels in obese children and compare the patients with healthy controls. The second aim was to compare the xenin levels in patients with nonalcoholic fatty liver disease (NAFLD) and also with insulin resistance with the patients without these complications. METHODS: 62 obese adolescents (27 with NAFLD) and 32 healthy controls were enrolled in the study. Obesity was defined as a body mass index exceeding the 95th percentile for the patients' age and sex. NAFLD was diagnosed via ultrasonographic examination. The insulin resistance was calculated by a homeostasis model assessment (HOMA-IR) index. Serum xenin and ghrelin levels were assessed by enzyme-linked immunosorbent assay. RESULTS: The mean serum xenin concentration was significantly higher in obese adolescents than the healthy peers (68.15 ± 0.63 vs 16.54 ± 0.07 pg/mL, p = 0.000). Serum xenin levels were not different between the patients with and without NAFLD and also between the patients with and without IR (p > 0.05). There was a positive correlation between xenin levels and relative weight (r = 0.663, p < 0.001) and HOMA-IR (r = 0.612, p < 0.001). Ghrelin was negatively correlated with relative weight (r = -0.283, p < 0.05). CONCLUSION: In this study, serum xenin levels of both groups of obese patients were found higher than controls. On the other hand, xenin levels were not different in patients with and without NAFLD. High levels of xenin may be in relation with obesity.