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Background/aim: Cytomegalovirus (CMV) is the most common congenital viral infection. Although most children with congenital CMV (approximately 85%-90%) are asymptomatic at birth, findings such as sensorineural hearing loss, microcephaly, and neurodevelopmental retardation can be observed during the follow-up. Among the brain magnetic resonance imaging (MRI) findings of CMV are white matter abnormalities, polymicrogyria, and periventricular calcification. Since a definitive diagnosis of congenital CMV cannot be made after the neonatal period, the identification of the associated phenotype is diagnostically important, but data are limited in patients who have been retrospectively diagnosed with congenital CMV infection. The aim of this study was to evaluate the short- and long-term neurological follow-up results of congenital CMV infections in a tertiary hospital. Materials and methods: The neurological results of fifteen patients under the age of 18 years, who had a definitive diagnosis of congenital CMV infection and were followed up in a tertiary care hospital between 2011 and 2020, were retrospectively evaluated. Results: Ten of the patients in our study group were male. The mean age at presentation for neurological evaluation was 2.02 ± 1.54 months, with a median follow-up time of 36.3 months (range: 9.3-129.4 months). Neurological disorders detected during the long-term follow-up included cerebral palsy (46.7%), cognitive impairment (46.7%), epilepsy (40%), and sensorineural hearing loss (26.7%). The most common abnormality observed on MRI scans was white matter involvement (53.3%). Conclusion: Early diagnosis and intervention are crucial in congenital CMV infection, as it commonly results in neurological involvement among the patients in our series. This preventable condition warrants further research regarding prenatal/neonatal screening.
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Infecciones por Citomegalovirus , Imagen por Resonancia Magnética , Humanos , Infecciones por Citomegalovirus/congénito , Infecciones por Citomegalovirus/complicaciones , Infecciones por Citomegalovirus/diagnóstico por imagen , Masculino , Femenino , Estudios Retrospectivos , Lactante , Recién Nacido , Preescolar , Niño , Pérdida Auditiva Sensorineural/virologíaRESUMEN
Background: This second harvest of the Congenital Heart Surgery Database intended to compare current results with international databases. Methods: This retrospective study examined a total of 4007 congenital heart surgery procedures from 15 centers in the Congenital Heart Surgery Database between January 2018 and January 2023. International diagnostic and procedural codes were used for data entry. STAT (Society of Thoracic Surgeons and European Association for Cardiothoracic Surgery) mortality scores and categories were used for comparison of the data. Surgical priority status was modified from American Society of Anesthesiologist guidelines. Centers that sent more than 5 cases to the database were included to the study. Results: Cardiopulmonary bypass and cardioplegic arrest were performed in 2,983 (74.4%) procedures. General risk factors were present in 22.6% of the patients, such as genetic anomaly, syndrome, or prematurity. Overall, 18.9% of the patients had preoperative risk factors (e.g., mechanical ventilation, renal failure, and sepsis). Of the procedures, 610 (15.2%) were performed on neonates, 1,450 (36.2%) on infants, 1,803 (45%) on children, and 144 (3.6%) on adults. The operative timing was elective in 56.5% of the patients, 34.4% were urgent, 8% were emergent, and 1.1% were rescue procedures. Extracorporeal membrane oxygenation support was used in 163 (4%) patients, with a 34.3% survival rate. Overall mortality in this series was 6.7% (n=271). Risk for mortality was higher in patients with general risk factors, such as prematurity, low birth weight neonates, and heterotaxy syndrome. Mortality for patients with preoperative mechanical ventilation was 17.5%. Pulmonary hypertension and preoperative circulatory shock had 11.6% and 10% mortality rates, respectively. Mortality for patients who had no preoperative risk factor was 3.9%. Neonates had the highest mortality rate (20.5%). Intensive care unit and hospital stay time for neonates (median of 17.8 days and 24.8 days, respectively) were also higher than the other age groups. Infants had 6.2% mortality. Hospital mortality was 2.8% for children and 3.5% for adults. Mortality rate was 2.8% for elective cases. Observed mortality rates were higher than expected in the fourth and fifth categories of the STAT system (observed, 14.8% and 51.9%; expected, 9.9% and 23.1%; respectively). Conclusion: For the first time, outcomes of congenital heart surgery in Türkiye could be compared to the current world experience with this multicenter database study. Increased mortality rate of neonatal and complex heart operations could be delineated as areas that need improvement. The Congenital Heart Surgery Database has great potential for quality improvement of congenital heart surgery in Türkiye. In the long term, participation of more centers in the database may allow more accurate risk adjustment.
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Background: Treatment of established portal vein narrowing after living donor hepatectomy is challenging. We aimed to present a new approach termed the "elbow patch reconstruction technique" to correct the narrowed remnant portal vein just or late after right lobe living donor hepatectomy. Methods: Demographic and clinical data of 12 living liver donors with narrowed remnant portal veins and treated with the "elbow patch reconstruction technique" were prospectively collected and retrospectively evaluated. Anatomic variation of the portal vein was defined in accordance with the Nakamura classification; six of the living liver donors had type A, three had type B, and the remaining three had type C. In eight of the living liver donors with a narrowed remnant portal vein, diagnosis was detected by intraoperative Doppler ultrasonography and visual inspection by experienced transplant surgeons in the living donor hepatectomy procedure. In the remaining four living liver donors, diagnosis was performed postoperatively when elevation of liver enzymes was noticed during the routine liver function test and Doppler US. The diagnosis was confirmed by multidetector computed tomography. Results: Data from nine males and three females aged 18 to 54 years were analyzed. All of the living liver donors were followed up for a median of 1710 days (min-max: 1178-4447 days; IQR: 1516 days), and none of the living liver donors had any structural or functional complications in the portal vein. Conclusions: Narrowing remnant portal veins are rare, but they are a life-threatening complication in living liver donors, and this condition requires urgent management. Image guided interventions and narrowed segment resection with end-to-end anastomosis using a vascular graft carried a potential risk for thrombosis and restenosis. To avoid these complications, we shared a technique named "elbow patch reconstruction technique". This technique can be very effective in relieving the narrowing of the remnant portal vein after right lobe living donor hepatectomy.
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A panel comprising of 84 Turkish winter wheat landraces (LR) and 73 modern varieties (MV) was analyzed with genome wide association study (GWAS) to identify genes/genomic regions associated with increased yield under favorable and drought conditions. In addition, selective sweep analysis was conducted to detect signatures of selection in the winter wheat genome driving the differentiation between LR and MV, to gather an understanding of genomic regions linked to adaptation and yield improvement. The panel was genotyped with 25 K wheat SNP array and phenotyped for agronomic traits for two growing seasons (2018 and 2019) in Konya, Turkey. Year 2018 was treated as drought environment due to very low precipitation prior to heading whereas year 2019 was considered as a favorable season. GWAS conducted with SNPs and haplotype blocks using mixed linear model identified 18 genomic regions in the vicinities of known genes i.e., TaERF3-3A, TaERF3-3B, DEP1-5A, FRIZZY PANICLE-2D, TaSnRK23-1A, TaAGL6-A, TaARF12-2A, TaARF12-2B, WAPO1, TaSPL16-7D, TaTGW6-A1, KAT-2B, TaOGT1, TaSPL21-6B, TaSBEIb, trs1/WFZP-A, TaCwi-A1-2A and TaPIN1-7A associated with grain yield (GY) and yield related traits. Haplotype-based GWAS identified five haplotype blocks (H1A-42, H2A-71, H4A-48, H7B-123 and H7B-124), with the favorable haplotypes showing a yield increase of > 700 kg/ha in the drought season. SNP-based GWAS, detected only one larger effect genomic region on chromosome 7B, in common with haplotype-based GWAS. On an average, the percentage variation (PV) explained by haplotypes was 8.0% higher than PV explained by SNPs for all the investigated traits. Selective sweep analysis detected 39 signatures of selection between LR and MV of which 15 were within proximity of known functional genes controlling flowering (PRR-A1, PPR-D1, TaHd1-6B), GY and GY components (TaSus2-2B, TaGS2-B1, AG1-1A/WAG1-1A, DUO-A1, DUO-B1, AG2-3A/WAG2-3A, TaLAX1, TaSnRK210-4A, FBP, TaLAX1, TaPIL1 and AP3-1-7A/WPA3-7A) and 10 regions underlying various transcription factors and regulatory genes. The study outcomes contribute to utilization of LR in breeding winter wheat.
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Estudio de Asociación del Genoma Completo , Triticum , Triticum/genética , Estaciones del Año , Sitios de Carácter Cuantitativo , Sequías , Turquía , Fitomejoramiento , Fenotipo , Grano Comestible/genética , GenómicaRESUMEN
BACKGROUND: The aim of this study was to investigate sleep habits, quality of life (QoL), and the relationship between them in children with epilepsy. METHODS: In this cross-sectional study, children aged two to 18 years being followed up for epilepsy were assessed using the Children's Sleep Habits Questionnaire (CSHQ) and the Pediatric Quality of Life Inventory (PedsQL). Pearson or Spearman correlation analysis was performed to examine the relationship between normally distributed and non-normally distributed variables, respectively. Linear regression analysis was used to examine independent variables associated with PedsQL total scale score. Level of significance was accepted as P < 0.05. RESULTS: The study included 112 children with a mean age of 10.5 ± 4.4 years (51.8% female). The frequency of poor sleep habits was 96.4%. There was a good level of agreement between children's and parents' PedsQL total, physical health, and psychosocial health scores (P < 0.001). Correlation analysis between QoL and sleep parameters revealed negative correlations between total sleep score and self-assessed PedsQL total scale, physical health, and psychosocial health scores (P < 0.05) and parent-assessed PedsQL total scale and psychosocial health scores (P < 0.05). The results of linear regression analysis indicated that the factors most significantly associated with lower QoL were high CSHQ total sleep score and exclusively daytime seizures (P < 0.001). CONCLUSIONS: It was found that children with epilepsy had poor sleep habits and low QoL and that poor sleep habits have a negative impact on QoL.
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Epilepsia , Calidad de Vida , Humanos , Femenino , Masculino , Niño , Estudios Transversales , Epilepsia/fisiopatología , Adolescente , Preescolar , Trastornos del Sueño-Vigilia/etiología , Trastornos del Sueño-Vigilia/fisiopatología , Sueño/fisiología , Encuestas y Cuestionarios , HábitosRESUMEN
Cardiac tumors are very rare in children, and echocardiography is very important in their detection. The clinical presentation can vary greatly depending on arrhythmia or obstruction. One of the most important factors determining the surgical approach is the clinical process. In this case report, we report the surgical treatment of a rhabdomyoma that caused refractory ventricular tachycardia.
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Neoplasias Cardíacas , Rabdomioma , Taquicardia Ventricular , Humanos , Niño , Rabdomioma/complicaciones , Rabdomioma/diagnóstico por imagen , Rabdomioma/cirugía , Ventrículos Cardíacos/diagnóstico por imagen , Ventrículos Cardíacos/cirugía , Ecocardiografía , Arritmias Cardíacas , Neoplasias Cardíacas/complicaciones , Neoplasias Cardíacas/diagnóstico por imagen , Neoplasias Cardíacas/cirugía , Taquicardia Ventricular/etiología , Taquicardia Ventricular/cirugíaRESUMEN
Background: In this study, we present our early results with the Ozaki procedure in the treatment of congenital aortic valve disease. Methods: Between July 2021 and July 2023, a total of 14 patients (10 males, 4 females; median age: 13.9 years; range, 8.5 to 15 years) who underwent neoaortic valve reconstruction of three leaflets using Ozaki procedure were retrospectively analyzed. Preoperative, postoperative, and follow-up echocardiogram images were evaluated. Results: Preoperative indications were aortic regurgitation (n=3) or combined aortic stenosis and regurgitation (n=11). The median aortic annular diameter was 23 (range, 19.5 to 25) mm on preoperative echocardiography. The median preoperative peak systolic aortic valve gradient for patients with aortic stenosis was 60 (range, 45 to 93) mmHg. The median preoperative aortic valve regurgitation grade was 4 (range, 3 to 4). Autologous pericardium and bovine pericardium were used in 12 and two patients, respectively. There was no conversion to valve replacement, myocardial infarction, or mortality in the early postoperative period. The median follow-up time was 8.5 (range, 6 to 19) months. One patient who performed the Ozaki procedure with bovine pericardium underwent valve replacement eight months later. Conclusion: The Ozaki procedure can be performed safely and effectively in congenital aortic valve stenosis and insufficiency with promising early results.
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Brugada syndrome (BrS) is a hereditary channelopathy caused by an autosomal dominant mutation in the cardiac sodium channel gene SCN5A alpha subunit. In individuals without structural heart disease, the risk of sudden cardiac death (SCD) increases in this channelopathy with ST-segment elevation in V1-3 precordials. Brugada phenocopy (BrP) is a condition in which transient ST-segment elevations are observed, mimicking BrS electrocardiographic changes, which can occur with electrolyte and metabolic disorder scenarios. In this study, we share a case of BrP that occurred due to hypovolemic hyponatremia and recovered spontaneously with the correction of electrolyte disturbance.
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Infants who undergo cardiac surgery frequently have complications that may advance to multiple organ failure and result in mortality. This study aims to compare three different multiple organ dysfunction scoring systems: the Neonatal Multiple Organ Dysfunction (NEOMOD) score, the modified NEOMOD score, and the Pediatric Logistic Organ Dysfunction-2 (PELOD-2) score in predicting postoperative 30-day mortality in neonates undergoing cardiac surgery. This retrospective cohort study was conducted between January 2019 and February 2021 in a single unit on neonates operated on due to congenital heart disease in the first 28 days of life. Patients who underwent off-pump surgeries were excluded from the study. The NEOMOD, modified NEOMOD, and PELOD-2 scores were calculated for each of the first 3 days following surgery. A total of 138 patients were included. All scores had satisfactory goodness-of-fit and at least good discriminative ability on each day. The modified NEOMOD score consistently demonstrated the best prediction among these three scores after the first day, reaching its peak performance on day 2 (area under curve: 0.824, CI: 0.75-0.89). Our findings suggest that NEOMOD and modified NEOMOD scores in the first 72 h could potentially serve as a predictor of mortality in this population.
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BACKGROUND: This study aimed to evaluate the etiology and prognosis of patients followed up for pediatric acute arterial ischemic stroke. METHODS: The clinical characteristics and etiology of patients aged 1 month-18 years who had acute arterial ischemic stroke between January 2010 and December 2020 were retrospectively evaluated. At last follow-up, the patients` functionality (Barthel Index, Functional Independence Measure), quality of life (SF-36 questionnaire), and motor outcomes (Gross Motor Function Classification System) were recorded prospectively/crosssectionally. RESULTS: Forty children (25 boys) with a median current age of 112.5 months (range: 3.6-294) were included in the study. The most frequent etiology was prothrombotic disorders, and the most important factor associated with long-term mortality was valvular heart disease. Of the 27 (67.5%) surviving patients, 29.6% had positive motor outcomes and 29.6% were independent according to the Barthel Index. In terms of quality of life, SF-36 scores were highest in the pain scale and lowest in emotional role difficulty. CONCLUSIONS: Determining the etiology and evaluating prognosis are important to plan effective treatment and rehabilitation for pediatric acute arterial ischemic stroke.
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Isquemia Encefálica , Accidente Cerebrovascular Isquémico , Accidente Cerebrovascular , Masculino , Niño , Humanos , Accidente Cerebrovascular/complicaciones , Estudios Retrospectivos , Accidente Cerebrovascular Isquémico/complicaciones , Calidad de Vida , Factores de RiesgoRESUMEN
OBJECTIVE: Anomalous aortic origin of the coronary artery is a rare congenital cardiac anomaly. The aim of this study was to present our experience with patients who underwent surgery for the anomalous aortic origin of the coronary artery. METHODS: This was a retrospective review of our experience with patients who had anomalous aortic origin of the coronary artery from 2019 to 2022. RESULTS: Seven patients were managed for anomalous aortic origin of the coronary artery, including five males and two females. The median age of the patients were 16 years (IQR, 14.5-26 years). Five patients had anomalous aortic origin of the right coronary artery and two patients had anomalous aortic origin of the left coronary artery. Five patients were treated surgically, one patient refused surgical treatment despite myocardial ischaemia symptoms, and the other one was not operated because she had no symptoms. Two patients underwent pulmonary root anterior translocation and left main coronary artery unroofing procedure, one patient underwent right coronary artery unroofing procedure, one patient underwent pulmonary artery lateral translocation procedure, and the last patient underwent right coronary artery osteal translocation procedure. The post-operative mortality or myocardial infarction was not observed in any patient. Patients were followed for a median of 10 months (IQR, 6.75-20.5 months) after repair. CONCLUSION: The data suggest that surgical repair of anomalous aortic origin of the coronary artery can be performed confidently and can be very effective for relieving myocardial ischaemia symptoms. Different surgical techniques can be used in anomalous aortic origin of the coronary artery according to the course and origin of the coronary arteries. To the our knowledge, pulmonary root anterior translocation and coronary artery unroofing procedure were performed for the first time in the literature.
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Procedimientos Quirúrgicos Cardíacos , Anomalías de los Vasos Coronarios , Infarto del Miocardio , Masculino , Femenino , Humanos , Adolescente , Adulto Joven , Adulto , Anomalías de los Vasos Coronarios/diagnóstico , Aorta/cirugía , Procedimientos Quirúrgicos Cardíacos/métodos , Infarto del Miocardio/etiología , Estudios RetrospectivosRESUMEN
Background: Electrical status epilepticus in sleep (ESES) is an epileptic syndrome specific to childhood and has a broad clinical spectrum that included seizures, behavioral/cognitive impairments, and motor neurological symptoms. Antioxidants are seen as promising neuroprotective strategies for the epileptic state by combating the harmful effects of excessive oxidant formation in mitochondria. Objective: This study aims to evaluate the thiol-disulfide balance and to determine whether it can be used in the clinical and electrophysiological follow-up of patients with ESES, especially in addition to the electroencephalography (EEG) examination. Methods: The study included 30 patients, aged 2-18 years and diagnosed with ESES in the Pediatric Neurology Clinic of the Training and Research Hospital and a control group of 30 healthy children. Total thiol, native thiol, disulfide, and ischemia-modified albumin (IMA) levels were measured, and disulfide-thiol ratios were calculated for both groups. Results: Native thiol and total thiol levels were significantly lower and IMA level and disulfide-native thiol percentage ratio were significantly higher in the ESES patient group than in the control group. Conclusion: Serum thiol-disulfide homeostasis is an accurate marker of oxidative stress in ESES, and standard and automated measures of thiol-disulfide balance as an indicator of oxidative stress showed a shift toward oxidation in ESES patients in this study. The negative correlation between spike-wave index (SWI) and thiol levels, and serum thiol-disulfide levels suggest that they can be used as biomarkers for follow-up of patients with ESES in addition to EEG. IMA can also be used for long-term response to monitoring purposes at ESES.
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Albúmina Sérica , Estado Epiléptico , Niño , Humanos , Biomarcadores , Homeostasis , Sueño , DisulfurosRESUMEN
OBJECTIVE: This study aimed to examine the timing and features of electroencephalography (EEG) as a predictor of seizure recurrence in children with a first unprovoked seizure. METHODS: We retrospectively evaluated the medical records and EEG recordings of pediatric patients who presented within 24 h of a first unprovoked seizure between January 2018-December 2019 and had at least 1 year of pediatric neurology clinical follow-up. RESULTS: The study included 108 patients (53.7% males) with a mean age of 98.75±57.75 months. Sixty-eight patients (63%) had an abnormal initial EEG, of which 55 (80.9%) were focal. The semiology of the first unprovoked seizure was focal in 50% of the patients and correlated with initial EEG findings (p<0.001). Forty-three patients had seizure recurrence during the follow-up period of mean 26.86±7.39 months. Recurrence was observed in the first 6 months in 30 patients and occurred twice in 4 patients. An abnormal EEG after the first unprovoked seizure was found to be an independent risk factor for recurrence, with a 2.42-fold higher recurrence risk in patients with focal EEG abnormalities compared to those with a normal EEG (p = 0.044). Analysis of 7 different timing patterns up to 96 h after the first unprovoked seizure showed that EEG timing was not associated with abnormality detection. DISCUSSION: Our study showed that EEG abnormalities, especially focal abnormalities, after a first unprovoked seizure are a predictor of seizure recurrence. But the rate of detection of EEG abnormalities was not related to the timing of EEG recording, relative to seizure occurrence.
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Electroencefalografía , Convulsiones , Masculino , Niño , Humanos , Femenino , Estudios Retrospectivos , Recurrencia , Convulsiones/diagnóstico , Factores de RiesgoRESUMEN
BACKGROUND: We aimed to evaluate patients with acute ataxia and to determine the warning clinical factors in the early prediction of neurological emergencies. METHOD: Patients with a history of balance and gait coordination disorder and clinically diagnosed as acute ataxia in pediatric emergency department were included in the study. As a result of final diagnosis, the characteristics of patients with and without clinically urgent neurological pathology (CUNP) were compared. CUNP was defined as any nervous system disorder requiring early diagnosis and prompt medical or surgical treatment and/or intensive care unit admission to prevent disabling or life-threatening evolution. RESULTS: Eighty-eight patients with a median age of 5 years were included in the study (37 [42%] patients with CUNP and 51 [58%] without CUNP). In the CUNP group, the median age of patients and symptom duration were significantly higher (P < 0.001 and P = 0.011, respectively). The most common etiologies were acute post/parainfectious cerebellar ataxias (n = 40 [45.4%]), acute cerebellitis (n = 9 [10.2%]), and Guillain-Barré syndrome (n = 8 [9%]). Hyporeflexia/areflexia and dysmetria were associated with a higher risk of CUNP. Headache, loss of consciousness, and visual dysfunction were the findings appearing exclusively in patients with CUNP. CONCLUSIONS: The most common etiologies in acute ataxia are benign and transient, whereas life-threatening conditions may occur rarely and may require urgent intervention. Older age; prolonged symptom duration; focal neurological deficits such as hemiparesis, hyporeflexia, and visual impairment; and nonspecific findings such as loss of consciousness and headache are the most striking "red flags" of a potential neurological emergency and should alert clinicians to CUNP.
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Ataxia Cerebelosa , Niño , Preescolar , Humanos , Enfermedad Aguda , Ataxia/diagnóstico , Ataxia/etiología , Ataxia Cerebelosa/diagnóstico , Servicio de Urgencia en Hospital , Cefalea/diagnóstico , Cefalea/etiología , Reflejo Anormal , InconscienciaRESUMEN
INTRODUCTION: The Actinomyces species is a fastidious, gram-positive, non-spore-forming bacteria that thrive in microareophilic and anaerobic conditions. Infection in the liver, an organ rarely affected by this pathogen, is presumed to be caused by hematogenous spread through the portal vein from a mucosal injury or other abdominal injury or a focus of infection. CASE DESCRIPTION: A 60-year-old male patient has a mass lesion of 15 × 10 cm in the left lobe on computed tomography. A tru-cut biopsy was performed with USG, and fragmented tissue pieces were obtained. In histopathological examination, these samples were reported as tumors with neuroendocrine differentiation. The biopsy sample contains a large amount of tumor neighborhood, and tumoral area is quite small. And, therefore, a clear diagnosis could not be found. A mass lesion with mildly increased Ga 68 DOTATATE uptake was observed in the left lobe of the liver (SUVmax value 3.8) and was interpreted in favor of the primary neuroendocrine tumor of the liver. DISCUSSION: Actinomyces cases are very rare and their diagnosis is usually delayed due to its slow and insidious course, and lack of specific clinical and radiological findings. It is difficult to make a correct diagnosis even in microbiological examinations and biopsy materials obtained in the presence of imaging methods. It can mimic tumors of abdominopelvic structures. CONCLUSION: Actinomyces should be kept in mind in cases with liver masses accompanied by previous abdominal surgery, abdominal trauma, high fever, and leukocytosis.
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Actinomicosis , Tumores Neuroendocrinos , Masculino , Humanos , Persona de Mediana Edad , Tumores Neuroendocrinos/diagnóstico , Tumores Neuroendocrinos/patología , Actinomicosis/diagnóstico , Actinomicosis/microbiología , Actinomicosis/patología , Actinomyces , Hígado/patología , Tomografía Computarizada por Rayos XRESUMEN
BACKGROUND: Abdominal aortic aneurysm (AAA) has risk factors similar to those of atherosclerosis. Salusin-ß and arterial stiffness are novel parameters that have been shown to predict atherosclerosis and related cardiovascular disorders. However, their predictive value for detecting AAA remains unclear. METHODS: Forty-eight patients with AAA and 47 age- and sex-matched participants without AAA were enrolled in the study. Arterial stiffness parameters were obtained via an oscillometric Mobil-O-Graph PWA Monitor device (IEM GmbH) with integrated ARCSolver software (Australian Institute of Technology). Plasma salusin-ß levels were analyzed using an enzyme-linked immunosorbent assay reagent kit (Abbkine, Inc). The measured salusin-ß levels and arterial stiffness parameters of the AAA and control groups were compared. RESULTS: Salusin-ß levels were significantly lower in patients with AAA (P = .014). There was a significant negative correlation between salusin-ß levels and abdominal aorta diameter. No significant difference was detected between AAA and control groups in terms of arterial stiffness parameters (P > .05). In backward multiple regression analysis, the presence of AAA, platelet count, and augmentation index were found to be independent predictors of salusin-ß levels (P = .006 and P = .023, respectively). CONCLUSION: Arterial stiffness parameters were not found to be associated with AAA. Contrary to previous results regarding atherosclerosis and related cardiovascular disorders, salusin-ß levels were found to be lower in patients with AAA. Although AAA is thought to have similar risk factors as atherosclerosis, the exact pathophysiologic mechanism remains unclear.
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Aneurisma de la Aorta Abdominal , Aterosclerosis , Rigidez Vascular , Humanos , Rigidez Vascular/fisiología , Australia , Aneurisma de la Aorta Abdominal/diagnóstico , Aorta Abdominal/diagnóstico por imagenRESUMEN
INTRODUCTION: Epilepsy is one of the leading chronic diseases of childhood, and an underlying IEM is an etiology that can easily be overlooked. The aim of this study was to determine the frequency of metabolic disease in patients diagnosed with epilepsy in the first two years of life, as well as to determine the clinical, radiological, and electroencephalographic (EEG) characteristics of the metabolic disease subtypes associated with epilepsy and evaluate treatment response in our study. MATERIALS AND METHODS: The records of patients diagnosed with epilepsy before the age of 2 years in our pediatric neurology clinic between 2014 and 2021 were reviewed retrospectively. Those diagnosed with an IEM and followed up in the pediatric neurology and pediatric metabolism departments of our hospital were included in the study. RESULTS: A total of 990 patients under the age of 2 years were diagnosed with epilepsy in the pediatric neurology clinic of our hospital and 74 (7.5%) of them had IEM. Thirty-nine (52.7%) of the 74 patients were female. The median age at admission was 144 days (min-max: 0-284). Of the 74 patients diagnosed with metabolic epilepsy, 38 patients were diagnosed with amino acid metabolism disorder, 17 with lysosomal storage disease, 9 with energy metabolism disorder, 5 with vitamin/cofactor/trace element metabolism disorders, 2 with fatty acid metabolism disorder, 2 with peroxisomal disease, and 1 with carbohydrate metabolism disorder. Epilepsy was refractory despite appropriate treatment in 39 patients (52.7%). CONCLUSION: Inborn errors of metabolism are a rare cause of epilepsy, in regions like our country with high rates of consanguineous marriage, IEM should be considered in patients presenting with seizures that do not respond to conventional antiepileptic treatments.
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Epilepsia , Enfermedades Metabólicas , Errores Innatos del Metabolismo , Niño , Humanos , Preescolar , Femenino , Lactante , Masculino , Estudios Retrospectivos , Epilepsia/epidemiología , Epilepsia/etiología , Epilepsia/diagnóstico , Electroencefalografía , Errores Innatos del Metabolismo/complicaciones , Errores Innatos del Metabolismo/diagnóstico , Errores Innatos del Metabolismo/epidemiologíaRESUMEN
OBJECTIVE: This study aims to compare both the pericardial roll technique with the patch augmentation technique of the unifocalization, and single-stage complete repair with the unifocalization and shunt for the repair of the ventricular septal defect, pulmonary atresia, and major aortopulmonary collateral arteries. METHODS: This was a retrospective review of the 48 patients undergoing unifocalization of the ventricular septal defect, pulmonary atresia, and major aorticopulmonary collateral arteries from a single center. Our cohort had two surgical pathways, including single-stage midline unifocalization (n = 40), unifocalization after pulmonary artery rehabilitation by creating an aortopulmonary window or central shunt (n = 8). There were two surgical techniques in single-stage midline unifocalizaton, including widening of the pulmonary arteries with a patch (n = 30), and connecting pulmonary arteries with a pericardial roll (n = 10). RESULTS: A total of 14 (29.2%) of 48 patients underwent single-stage complete repair, 26 patients underwent shunt palliation with unifocalization. Combined early and late mortality was seen in seven patients in those who underwent shunt palliation with unifocalization, while it was seen in one patient in those who underwent a single-stage complete repair (mortality ratio 26.8% vs. 7.1%, p = .22). There was no statistically significant difference between the pericardial roll and patch augmentation techniques in terms of pulmonary artery reintervention (p = .65). Although all pulmonary artery reinterventions were for unilateral pulmonary artery in the roll technique group, 41.7% of reinterventions were for bilateral pulmonary arteries in the pericardial augmentation group. CONCLUSION: Single-stage complete repair of the ventricular septal defect, pulmonary atresia, and major aorticopulmonary collateral arteries has better results than unifocalization with a shunt. In terms of nonvaluable raw material, the use of the pericardial roll technique is a considerable alternative for unifocalization.
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Defecto del Tabique Aortopulmonar , Defectos del Tabique Interventricular , Atresia Pulmonar , Aorta/anomalías , Aorta/cirugía , Defecto del Tabique Aortopulmonar/cirugía , Circulación Colateral , Defectos de los Tabiques Cardíacos , Defectos del Tabique Interventricular/cirugía , Humanos , Lactante , Arteria Pulmonar/anomalías , Arteria Pulmonar/cirugía , Atresia Pulmonar/cirugía , Estudios RetrospectivosRESUMEN
Kazakhstan is the fourteenth largest wheat producer in the world. Despite this fact, there has not been a comprehensive survey of wheat root and crown rot. A quantitative survey was conducted for the purpose of establishing the distribution of fungi associated with root and crown rot on wheat (Triticum spp.). During the 2019 growing season, samples were taken from the affected plants' roots and stem bases. A total of 1221 fungal isolates were acquired from 65 sites across the central (Karagandy region), eastern (East Kazakhstan region), and southeastern (Almaty region) parts of the country and identified using morphological and molecular tools. The internal transcribed spacer (ITS), translation elongation factor 1-alpha (EF1-α), and glyceraldehyde-3-phosphate dehydrogenase (GAPDH) sequences were successfully used to identify the species of fungal isolates. It was found that Bipolaris sorokiniana (44.80%) and Fusarium acuminatum (20.39%) were the most predominant fungal species isolated, which were present in 86.15 and 66.15% of the fields surveyed, respectively, followed by F. equiseti (10.16%), Curvularia spicifera (7.62%), F. culmorum (4.75%), F. oxysporum (4.10%), F. redolens (2.38%), Rhizoctonia solani AG2-1 (1.06%), Nigrospora oryzae (0.98%), C. inaequalis (0.90%), F. pseudograminearum (0.74%), F. flocciferum (0.74%), Macrophomina phaseolina (0.66%), F. cf. incarnatum (0.33%), Fusarium sp. (0.25%), and F. torulosum (0.16%). A total of 74 isolates representing 16 species were tested via inoculation tests on the susceptible Triticum aestivum cv. Seri 82 and the results revealed that F. culmorum and F. pseudograminearum, B. sorokiniana, Fusarium sp., R. solani, F. redolens, C. spicifera, C. inaequalis, and N. oryzae were virulent, whereas others were non-pathogenic. The findings of this investigation demonstrate the presence of a diverse spectrum of pathogenic fungal species relevant to wheat crown and root rot in Kazakhstan. To the best of our knowledge, this is the first report of F. pseudograminearum, Fusarium sp., C. spicifera, and C. inaequalis as pathogens on wheat in Kazakhstan.
RESUMEN
Background: In this study, we aimed to analyze the predictors and risk factors of mortality in patients who underwent Norwood I procedure with the diagnosis of hypoplastic left heart syndrome. Methods: Between January 2009 and December 2020, a total of 139 patients (95 males, 44 females) who underwent Norwood I procedure with the diagnosis of hypoplastic left heart syndrome in our center were retrospectively analyzed. Results: The median birth weight was 3,200 (range, 3,000 to 3,350) g and the median age at the time of operation was seven (range, 5 to 10) days. Pulmonary flow was achieved with a Sano shunt in the majority (72%) of patients. Survival rate was 41% after the first stage. Reoperation for bleeding (p=0.017), reoperation for residual lesion (p=0.011), and postoperative peak lactate level (p=0.029), were associated with in-hospital mortality. Nineteen (33%) of 57 patients died before the second stage. Thirty-three (58%) patients underwent second stage, and survival after the second stage was 94%. Thirteen patients underwent third stage, and survival after the third stage was 85%. Estimated probability of survival at six months, and one, two, three, and four years were 33%, 33%, 25%, 25%, and 22% respectively. Conclusion: Hospital and inter-stage mortality rates are still high and this seems to be the most challenging period in term of survival efforts of the patients with hypoplastic left heart syndrome. Early recognition and reintervention of anatomical residual defects, close follow-up in the inter-stage period, and the accumulation of multidisciplinary experience may help to improve the results to acceptable limits.