SUMMARY OBJECTIVE: It has been determined that adropin has a role in tissue healing. This study aimed to determine the effects of head trauma on the tissues and blood levels of patients admitted to the emergency department. METHODS: The study group was divided into two to compare the adropin level in healthy individuals and patients with head trauma. Blood tests from patients and healthy volunteers were compared using the adropin kit. Adropin levels, Glasgow Coma Scale, and revised scores of trauma patients were recorded and analyzed. RESULTS: All patients in the trauma group had significantly higher adropin levels than the control group. Among these patients, the adropin level of the discharged patients was higher than the others. In addition, patients with high Glasgow Coma Scale and normal blood pressure were found to have higher adropin levels than the others. CONCLUSION: Although adropin cannot make a sharp distinction in determining the prognosis, the increase in its level in trauma patients shows that it triggers a protective mechanism. OBJECTIVE healing department METHODS kit analyzed RESULTS others addition CONCLUSION prognosis mechanism

SUMMARY OBJECTIVE: This study aimed to compare the effectiveness of resection and extended end-to-end anastomosis between neonate and infant patients with coarctation. METHODS: This study was designed retrospectively and included 41 neonate (<30 days) and infant (30 days to 1 year) patients who were operated on using the resection and extended end-to-end anastomosis technique for aortic coarctation. Preoperative aortic annulus diameters and Z scores, all aortic arch diameters and Z scores, the presence of hypoplastic aortic segment, and the presence of prematurity were reviewed in both groups. Subsequently, we investigated whether these parameters were statistically related to the residual gradient in the operation area, whether there was a need for early re-intervention, and what was the incidence of mortality in the early postoperative period. In addition, the aortic arch Z scores of the patients at 6 months postoperatively were examined. RESULTS: While the mean age (p<0.001), body weight (p<0.001), and proximal arch Z score (p=0.029) were found to be significantly lower in the neonate group than in the infant group, the total length of the intensive care unit stay (p=0.013) and the total length of hospital stay (p=0.017) were found to be significantly higher. In addition, significant enlargement was detected in the proximal arch, distal arch, and isthmus segments in both patient groups. CONCLUSION: The resection and extended end-to-end anastomosis is an equally effective technique that can provide a marked decrease in gradient in the coarctation area and a significant enlargement of the aortic arch segments in the early period after coarctation repair in both neonate and infant patients. OBJECTIVE endtoend end METHODS 4 <30 30 (<3 (3 year segment groups Subsequently reintervention, reintervention re intervention, intervention re-intervention addition examined RESULTS p<0.001, p0001 p p<0.001 , 0 001 (p<0.001) p=0.029 p0029 029 (p=0.029 p=0.013 p0013 013 (p=0.013 p=0.017 p0017 017 (p=0.017 higher CONCLUSION <3 3 (< ( p000 p<0.00 00 (p<0.001 p=0.02 p002 02 (p=0.02 p=0.01 p001 01 (p=0.01 < p00 p<0.0 (p<0.00 p=0.0 (p=0.0 p0 p<0. (p<0.0 p=0. (p=0. p<0 (p<0. p=0 (p=0 p< (p<0 p= (p= (p< (p

ABSTRACT Introduction: Cyanotic congenital heart diseases constitute 40-45% of all congenital heart diseases. In patients who are not suitable for primary repair, modified BT (MBT) shunt and central shunt (CS) procedures are still frequently used. Methods: This study included 62 pediatric patients who underwent MBT shunt or CS via median sternotomy. Patients’ demographic, echocardiographic, operative, and postoperative data were collected retrospectively. The patients were classified as single ventricle and bi-ventricle according to their cardiac anatomy, and the presence of prematurity and heterotaxy was noted. Procedure details of the patients who underwent endovascular intervention prior to the surgery were investigated, and operation data were accessed from the surgery notes. Data regarding postoperative follow-ups were obtained and comparatively analyzed. Results: Of the total 62 patients, 32 (51.6%) were newborns and 16 (25.8%) had a body weight < 3 kg. MBT shunt was applied to 48 patients (77.4%), while CS was applied to 14 patients (22.6%). There was no significant difference between the two surgical procedures in terms of requirement for urgent shunt or cardiopulmonary bypass, additional simultaneous surgical intervention, need for high postoperative inotropes, and in-hospital mortality (P>0.05). The rate of congestive heart failure in patients with in-hospital mortality was determined as 66.7% and it was significantly higher than in patients without heart failure (P<0.001). Conclusion: MBT shunt and CS are still frequently used in cyanotic patients. The use of small-diameter shunts, particularly when centrally located, can prevent the onset of congestive heart failure and lower mortality. Introduction 4045% 4045 40 45% 45 40-45 repair (MBT (CS Methods 6 sternotomy Patients demographic echocardiographic operative retrospectively biventricle bi anatomy noted investigated notes followups follow ups analyzed Results 51.6% 516 51 (51.6% 1 25.8% 258 25 8 (25.8% kg 4 77.4%, 774 77.4% , 77 (77.4%) 22.6%. 226 22.6% . 22 (22.6%) bypass inotropes inhospital hospital P>0.05. P005 P P>0.05 0 05 (P>0.05) 667 66 7 66.7 P<0.001. P0001 P<0.001 001 (P<0.001) Conclusion smalldiameter small diameter shunts located 404 40-4 51.6 5 (51.6 25.8 2 (25.8 77.4 (77.4% 22.6 (22.6% P00 P>0.0 (P>0.05 66. P000 P<0.00 00 (P<0.001 40- 51. (51. 25. (25. 77. (77.4 22. (22.6 P0 P>0. (P>0.0 P<0.0 (P<0.00 (51 (25 (77. (22. P>0 (P>0. P<0. (P<0.0 (5 (2 (77 (22 P> (P>0 P<0 (P<0. ( (7 (P> P< (P<0 (P (P<

Resumen Evaluar la resistencia de unión al corte (SBS) del cemento de resina autoadhesivo cuando se utiliza con dos materiales diferentes de diseño asistido por computadora (CAD) y fabricación asistida por computadora (CAM) después de varios tratamientos superficiales. Se prepararon muestras de bloques CAD-CAM de resina Lava Ultimate (LU) y cerámica feldespática Vita Mark II (VM) con un espesor de 1,5mm, y se obtuvieron un total de 90 muestras (N=90), con cinco muestras de cada bloque. Las muestras se dividieron en los siguientes cinco grupos según los tratamientos superficiales (n=9): grupo 1, sin tratar (control); grupo 2, grabado con ácido fluorhídrico al 5%; grupo 3, irradiación con láser Er: YAG; grupo 4, recubrimiento triboquímico de sílice (Cojet); y grupo 5, aire-abrasión con Al2O3. Después de la aplicación de silano, se aplicó cemento de resina sobre una matriz transparente (diámetro, 3mm; altura, 2mm) sobre los bloques. La SBS se determinó usando un dispositivo de prueba universal a una velocidad de cruceta de 1mm/min. Se utilizaron análisis de varianza bidireccional (ANOVA) y pruebas post hoc de Tukey para analizar los valores de SBS. LU mostró el valor más alto de SBS en el grupo 4. Los valores promedio de SBS en los grupos 3 y fueron más bajos que en el grupo de control (p<0,05). Cuando se examinó VM, mientras que todos los tratamientos superficiales aumentaron significativamente los valores de SBS, el valor más alto de SBS se observó en el grupo 4 (p<0,05). Este estudio reveló que todos los tratamientos de superficie utilizados afectaron negativamente los valores de resistencia de la unión del cemento de resina autoadhesivo a LU, a excepción de la aplicación Cojet. Los valores de SBS del cemento de resina con VM aumentaron en todos los grupos de aplicación de tratamiento de superficie.

Abstract To evaluate the shear bond strength (SBS) of self-adhesive resin cement when used with two different computer-aided design (CAD)-computer-aided manufacturing (CAM) materials after various surface treatments. Nanoceramic resin Lava Ultimate (LU) and feldspathic ceramic Vita Mark II (VM) CAD-CAM block samples were prepared with 1.5-mm thickness, and a total of 90 samples were obtained (N=90), with five samples of each block. The samples were divided into the following five groups according to the surface treatments (n=9): group 1, untreated (control); group 2,5% hydrofluoric acid etching; group 3, Er: YAG laser irradiation; group 4, tribochemical silica coating (Cojet); and group 5, air-abrasion with Al2O3. After silane application, resin cement was applied on a transparent matrix (diameter, 3mm; height, 2mm) on the blocks. SBS was determined using a universal testing device at a crosshead speed of 1mm/min. Two-way analysis of variance (ANOVA) and Tukey's post hoc tests were used to analyze the SBS values. LU showed the highest SBS value in group 4. The average SBS values in groups 3 and were found to be lower than that in the control group (p<0.05). When VM was examined, while all surface treatments increased the SBS values significantly, the highest SBS value was observed in group 4 (p<0.05). This study revealed that all surface treatments used negatively affected the bond strength values of self-adhesive resin cement to LU, except for Cojet application. The SBS values of resin cement with VM increased in all surface treatment application groups.

SUMMARY OBJECTIVE: Autosomal dominant polycystic kidney disease is an inherited kidney disorder with mutations in polycystin-1 or polycystin-2. Autosomal recessive polycystic kidney disease is a severe form of polycystic kidney disease that is characterized by enlarged kidneys and congenital hepatic fibrosis. Mutations at PKHD1 are responsible for all typical forms of autosomal recessive polycystic kidney disease. METHODS: We evaluated the children diagnosed with polycystic kidney disease between October 2020 and May 2022. The diagnosis was established by family history, ultrasound findings, and/or genetic analysis. The demographic, clinical, and laboratory findings were evaluated retrospectively. RESULTS: There were 28 children (male/female: 11:17) evaluated in this study. Genetic analysis was performed in all patients (polycystin-1 variants in 13, polycystin-2 variants in 7, and no variants in 8 patients). A total of 18 variants in polycystin-1 and polycystin-2 were identified and 9 (50%) of them were not reported before. A total of eight novel variants were identified as definite pathogenic or likely pathogenic mutations. There was no variant detected in the PKDH1 gene. CONCLUSION: Our results highlighted molecular features of Turkish children with polycystic kidney disease and demonstrated novel variations that can be utilized in clinical diagnosis and prognosis.

SUMMARY OBJECTIVE: Chronic kidney disease (CKD) remains one of the major common health problems, and the number of people affected by the disease is progressively increasing in Turkey and worldwide. This study aimed to investigate molecular defects in Alport syndrome (AS) and other genes in patients with clinically suspected CKD using whole-exome sequencing (WES). METHODS: Patients with clinical suspicion of CKD were included in the study. Molecular genetic analyses were performed on genomic DNA by using WES. RESULTS: A total of 15 with 5 different pathogenic or likely pathogenic variants were identified in CKD patients, with a diagnostic rate of 30%. Eight variants of uncertain significance were also detected. In this study, 10 variants were described for the first time. As a result, we detected variants associated with CKD in our study population and found AS as the most common CKD after other related kidney diseases. CONCLUSIONS: Our results suggest that in heterogeneous diseases such as CKD, WES analysis enables accurate identification of underlying molecular defects promptly. Although CKD accounts for 10–14% of all renal dysfunction, molecular genetic diagnosis is necessary for optimal long-term treatment, prognosis, and effective genetic counseling.

Resumo Fundamento As arritmias ventriculares (AVs) são a principal causa de mortalidade e morbidade hospitalar em pacientes com síndrome coronariana aguda (SCA) e sua relação com o tiol é desconhecida. Objetivo Investigar a relação entre os níveis plasmáticos de tióis e os níveis de troponina em pacientes com SCA e estimar o desenvolvimento de AV intra-hospitalar durante a internação. Método O estudo incluiu 231 pacientes consecutivos com SCA com supradesnivelamento do segmento ST (SCA-SDST) e pacientes com SCA sem supradesnivelamento do segmento ST (SCA-SSDST). Após a aplicação dos critérios de exclusão, 191 pacientes foram incluídos na análise estatística. Os pacientes foram classificados em dois grupos: grupo SCA-SDST (n=94) e grupo SCA-SSDST (n=97). Os níveis plasmáticos de tiol, dissulfeto e troponina foram medidos e a razão de troponina para tiol nativo (RTTN) foi calculada. Considerou-se estatisticamente significativo um valor de p bilateral inferior a 0,05. Resultados Tiol nativo plasmático, tiol total, dissulfeto e suas razões foram semelhantes entre os grupos. A RTTN se mostrou significativamente maior no grupo SCA-SDST em comparação com o grupo SCA-SSDST. Houve correlação negativa significativa entre os níveis de troponina e tiol. Verificou-se que o tiol nativo é preditor independente do desenvolvimento de AV em pacientes com SCA-SDST e em todos os pacientes com SCA. Verificou-se que o RTTN é preditor independente do desenvolvimento de AV em pacientes com SCA-SSDST e em todos os pacientes com SCA. Conclusão Os níveis plasmáticos de tiol podem ser usados para identificar pacientes com alto risco de desenvolvimento de AV intra-hospitalar em pacientes com SCA. A correlação entre os níveis de troponina e tiol pode sugerir que os tióis possam ser marcadores importantes para o diagnóstico e prognóstico da SCA com a ajuda de estudos futuros.

Abstract Background Ventricular arrhythmias (VAs) are the main cause of in-hospital mortality and morbidity in acute coronary syndrome (ACS) patients and its relationship with thiol is not known. Objective To investigate the relationship between plasma thiol levels and troponin levels in patients with ACS and to estimate in-hospital VA development during hospital stay. Method The study included 231 consecutive ST-segment elevation ACS (STE-ACS) and non-ST-segment elevation ACS (NSTE-ACS) patients. After application of exclusion criteria, 191 patients were included in the statistical analysis. Patients were classified into two groups: STE-ACS group (n=94) and NSTE-ACS group (n=97). Plasma thiol, disulphide and troponin levels were measured and troponin-to-native thiol ratio (TNTR) was calculated. A two-sided p value of less than 0.05 was considered to be statistically significant. Results Plasma native thiol, total thiol, disulphide and their ratios were similar between the groups. TNTR was significantly higher in the STE-ACS group compared to the NSTE-ACS group. Troponin and thiol levels correlated negatively and significantly. Native thiol was found to be an independent predictor of VA development in STE-ACS patients and in all ACS patients. TNTR was found to be an independent predictor of VA development in NSTE-ACS patients and in all ACS patients. Conclusion Plasma thiol levels can be used to identify ACS patients at high risk for in-hospital VA development. Correlation between troponin and thiol levels may suggest that thiols may be an important marker for diagnosis and prognosis of ACS with the help of future studies.

Abstract Objective: The aim of this study is to compare the continuous and combined suturing techniques in regards to the needing epicardial pacing at the time of weaning from cardiopulmonary bypass (EP-CPB) and to evaluate permanent epicardial pacemaker (PEP) implantation in patients who had undergone surgical ventricular septal defect (VSD) closure. Methods: This single-centre retrospective survey includes 365 patients who had consecutively undergone VSD closure between January 2006 and October 2015. Results: The median age and weight of the patients were 15 months (range 27 days - 56.9 years) and 10 kg (range 3.5 - 100 kg), respectively. Continuous and combined suturing techniques were utilised in 302 (82.7%) and 63 (17.3%) patients, respectively. While 25 (6.8%) patients required EP-CPB, PEP was implanted in eight (2.2%) patients. Comparison of the continuous and combined suturing techniques regarding the need for EP-CPB (72% vs. 28%, P=0.231) and PEP implantation (87.5% vs. 12.5%, P=1.0) were not statistically significant. The rate of PEP implantation in patients with perimembraneous VSD without extension and perimembraneous VSD with inlet extension did not reveal significant difference between the suture techniques (P=1.0 and P=0.16, respectively). In both univariate and multivariate analyses, large VSD (P=0.001; OR 8.63; P=0.011) and perimembraneous VSD with inlet extension (P<0.001; OR 9.02; P=0.005) had a significant influence on PEP implantation. Conclusion: Both suturing techniques were comparable regarding the need for EP-CPB or PEP implantation. Caution should be exercised when closing a large perimembraneous VSD with inlet extension.

RESUMOUma criança feminina com seis meses de idade se apresentou à nossa clínica com edema palpebral bilateral, membranas brancas amareladas sob as pálpebras de ambos os olhos e descarga mucosa. Sua tia já havia apresentado problemas oculares semelhantes que não foram diagnosticados. As membranas conjuntivais foram excisadas e a investigação histopatológica das membranas demonstraram conjuntivite lenhosa. O diagnóstico de deficiência de plasminogênio foi obtido a partir de um exame laboratorial. Tratamento tópico com plasma fresco congelado (FFP) sem qualquer terapia sistêmica mostrou boa resposta. Não foram observadas recorrências das membranas. O tratamento tópico com FFP pode ajudar a reabilitação rápida e prevenir a recorrência em pacientes com conjuntivite lenhosa.

ABSTRACTA 6-month-old female infant presented to our clinic with bilateral eyelid swelling, yellowish-white membranes under both lids, and mucoid ocular discharge. Her aunt had similar ocular problems that were undiagnosed. The conjunctival membranes were excised and histopathological investigation of these membranes showed ligneous conjunctivitis. Further, laboratory examination revealed plasminogen deficiency. A good response was observed to topical fresh frozen plasma (FFP) treatment without systemic therapy, and the membranes did not recur during the treatment. Topical FFP treatment may facilitate rapid rehabilitation and prevent recurrence in patients with ligneous conjunctivitis.