Role of therapeutic drug monitoring of intravenous Busulfan for prevention of sinusoidal obstructive syndrome in children.

BACKGROUND: Therapeutic drug monitoring (TDM) of intravenous busulfan (Bu) has been recommended for safe engraftment and decreased toxicity in children undergoing hematopoietic stem cell transplantation (HSCT). This study aims to compare HSCT-related outcomes, such as acute or chronic graft-versus-host disease (GvHD), sinusoidal obstructive syndrome (SOS), event-free survival (EFS), and overall survival (OS) in children with and without TDM for busulfan. METHODS: This retrospective study conducted between February 2012 and February 2021 at our Bone Marrow Transplantation Unit included 172 patients (34% girls) with a median age of 4.70 years (IQR 2.41-10.01). Group A consisted of 46 patients whose Bu doses were adjusted according to actual body weight, and group B consisted of 126 patients whose Bu dose adjustments made according to TDM. RESULTS: Totally, 32 patients (19%) developed moderate or severe SOS. The incidence of SOS was significantly higher in the group without TDM (29% vs. 15%, p = .041). A multivariable analysis showed that the presence of acute GvHD and one alkylating drug-containing conditioning regimen compared with two or three were associated with SOS (p = .03 and p = .002, respectively). In patients with TDM, cumulative Bu dose and area under curve also were not associated with SOS. Other HSCT-related outcomes such as acute or chronic GvHD, relapse and graft rejection rates, OS and EFS rates did not differ between the groups. CONCLUSIONS: TDM and making dose adjustments with Bayesian forecasting over four days of Bu therapy optimizes exposure and reduces the risk of SOS in children undergoing HSCT.

Neonatal onset familial Mediterranean fever.

Objectives: Familial Mediterranean fever (FMF) is an autosomal recessive disease characterised by recurrent, self-limited attacks of fever with serositis. Recently, it was shown that FMF patients with early disease onset have more severe disease. The aim of this study was to describe the demographic, clinical and genetic features of FMF patients who had disease onset during the neonatal period. Methods: Medical records of all patients diagnosed as FMF and had been seen in the outpatient clinic of Paediatric Rheumatology department between January 2013 and January 2014 were retrospectively evaluated. Patients with disease onset during the first month of life were included to the study. Results: Among 317 patients; 19 (12 males) were included to the study. Approximately 60% of the patients had family history of FMF. Homozygous p.M694V mutation was detected in 42% of the cases. Thirteen patients present with attacks of fever and remaining had attacks in the form of restlessness, resembling infantile colic starting in the neonatal period. Majority of these patients developed classical abdominal attacks between the ages of 1 and 2.5 years. The diagnosis of FMF was significantly delayed; the median age at onset of therapy was 3.5 years (range 7 months-17 years). Conclusion: Patients with FMF could have complaints even in the neonatal period. Homozygous p.M694V mutation is a prominent mutation in this group of patients. In order to prevent diagnostic delay physicians dealing with these type of patients should be more vigilant.

Elevated lipocalin-2 level in aqueous humor of patients with central retinal vein occlusion.

PURPOSE: To assess the concentrations of lipocalin-2 (LCN2) in the serum and the aqueous humor of patients with central retinal vein occlusion (CRVO). METHODS: The concentrations of LCN2 in the serum and aqueous humor of 16 cataract patients and 16 patients with CRVO with macular edema were compared. Collection of aqueous samples was conducted in the operating theater under sterile conditions and just prior to intravitreal ranibizumab injection or cataract surgery. LCN2 levels in serum and aqueous humor samples were measured using a commercial kit (human lipocalin-2/NGAL PicoKine ELISA Kit, MyBioSource Inc., USA; Catalog No: MBS175829) based on standard sandwich enzyme-linked immunosorbent assay technology. RESULTS: The concentrations of LCN2 in the aqueous humors of the CRVO group were higher than those of the control group (p = 0.021). There was no significant difference in serum LCN2 level between the two groups (p = 0.463). CONCLUSIONS: Concentrations of LCN2 in aqueous humor are increased in CRVO. LCN2 may be part of a pro-catabolic phenotype, and it may play an important role in the dreaded complications of CRVO, such as macular edema, macular ischemia, and neovascularization, which lead to blindness.

Histopathological analysis of skin adnexal tumors: An experience at a tertiary center.

BACKGROUND: Skin appendage tumors (SATs) are benign and malignant neoplasms. Although there are many studies on their clinical features and epidemiology in the literature, most of these studies have a small number of patients and are not classified according to the WHO 2018 classification. OBJECTIVES: This study aimed to reveal the clinical and histopathological features of the SATs and compare the pre-diagnosis. MATERIALS AND METHODS: Cases diagnosed with SATs in the last 7 years in the Pathology Department were re-evaluated according to the WHO 2018 classification. Patients' ages and genders, as well as the location and pre-diagnosis of the lesion, were all recorded. RESULTS: A total of 437 patients, 198 (45.3%) male and 239 (54.7%) female, were included in the study. Of 437 patients, 399 (91.3%) were diagnosed with benign SATs and 38 (8.7%) were diagnosed with malignant SATs. Most of the cases were benign tumors with follicular differentiation. Malignant SATs were seen in patients significantly older than benign ones. The majority of SATs were in head and neck localization. Mammary Paget's disease was the most common malignant SAT (n = 14, 36.8% of patients diagnosed with malignant SATs). It was noted that clinicians were less likely to consider a specific diagnosis of SAT before excision. CONCLUSION: SAT, a diagnosis that can be seen at any age and is difficult to predict clinically, may be malignant, especially in elderly patients. Histopathology is the gold standard in diagnosing SATs, and immunohistochemical staining may be useful in diagnosing tumors with uncertain histopathological features.

Evaluating Postoperative Outcomes and Investigating the Usefulness of EU-TIRADS Scoring in Managing Pediatric Thyroid Nodules Bethesda 3 and 4

Objective: The aim was to assess postoperative outcomes in pediatric thyroid nodules with atypia of undetermined significance (AUS/FLUS) or suspicious for a follicular neoplasm (SFN) and their respective the European-Thyroid Imaging Reporting and Data System (EU-TIRADS) scores. Methods: Forty-four pediatric patients at a single center with thyroid nodules classified as AUS/FLUS or SFN from August 2019 to December 2022 were retrospectively reviewed. Data on demographics, thyroid function, nodule size, and ultrasonographic features were collected. Postoperative pathologies were categorized into benign, low-risk, and malignant neoplasms according to the World Health Organization 2022 criteria, and EU-TIRADS was used for retrospective radiological scoring. Results: Among 21 (47.7%) of patients who had surgical intervention, 72% had Bethesda 3 and 28% had Bethesda 4 thyroid nodules. Post-surgical histopathological classifications were 43% benign, 19% low-risk, and 38% malignant. Of note, EU-TIRADS 3 and 5 scores were present in 44% and 56% of the benign cases, respectively. Malignant cases tended to produce higher EU-TIRADS scores, with 64% rated as EU-TIRADS 5. Bethesda category 4 nodules had a 66% malignancy rate, significantly higher than the 27% in category 3. Conclusion: A substantial proportion of histologically benign cases were classified as EU-TIRADS 5, suggesting that EU-TIRADS may lead to unnecessary biopsies in benign cases. Malignant cases were more likely to have a higher EU-TIRADS score, indicating a positive correlation with malignancy risk, particularly in Bethesda 4 cases. However, the EU-TIRADS system's predictive value for malignancy in Bethesda 3 cases was poorer.

Comparison of machine learning algorithms for predicting diesel/biodiesel/iso-pentanol blend engine performance and emissions.

In this study, machine learning techniques, namely artificial neural network (ANN), support vector machine (SVM), and extreme gradient boosting (XGBoost), were used to comprehensively evaluate engine performance and exhaust emissions for different fuel blends. To obtain valuable insights on optimizing engine performance and emissions for alternative fuel blends and thus contribute to the advancement of knowledge in this field, we focused on iso-pentanol ratios while maintaining the biodiesel ratios constant. The maximum brake thermal efficiency (BTE) values for the diesel (30.13 %), D85B10P5 (29.92 %), D80B10P10 (29.89 %), and D70B10P20 (29.79 %) blends were achieved at 1600 rpm. At 1600 rpm, the brake-specific fuel consumption (BSFC) values for the diesel, D85B10P5, D80B10P10, and D70B10P20 blends were 189.93, 200.93, 202.93, and 203.95 g kWh-1, respectively. In engine performance prediction, the ANN model exhibited superior performance, yielding regression coefficient (R2), root mean square error, and mean absolute error values of 0.984, 0.411 %, and 0.112 %, respectively, in BTE prediction, and 0.958 %, 6.9 %, and 2.95 %, respectively, in BSFC prediction. In exhaust gas temperature prediction, the SVM model exhibited the best performance, yielding an R2 value of 0.981. Although all models successfully predicted NOx emissions, the ANN model exhibited the best performance, achieving an R2 value of 0.959. In CO2 and hydrocarbon estimation, the XGBoost model exhibited the best performance, yielding R2 values of 0.956 and 0.973, respectively. Therefore, the ANN model can be used to accurately predict engine performance, and the XGBoost model can be used to accurately predict emission parameters.

Three Cases of Catheter-related Bloodstream Infection Caused by Phialemonium curvata : A Case Report and Literature Review.

BACKGROUND: With recent developments in the field of microbiology, an increasing number of yeasts and molds with the potential to cause infections in humans are identified every year. In addition to the challenges in identifying clinical isolates, there is limited antifungal susceptibility data available for Phialemonium species, leading to uncertainty in optimal treatment recommendations. METHODS: In this article, catheter-related bloodstream infections caused by Phialemonium curvata (previously Phialemonium curvatum ) in 3 immunosuppressed patients are presented. Furthermore, the literature was reviewed to identify the clinical spectrum and treatment approaches for the reported infections. RESULTS: The cases presented here were analyzed along with 24 cases reported in the literature. Among all cases, 21 (77.7%) patients had an underlying condition. Nine (33.3%) patients had hematological/oncological malignancies and solid organ transplants. Twenty-two (81.4%) patients had a history of device or invasive interventions. Surgical procedures, removal of contaminated devices or tissue were found to reduce the risk of death by 86.7%. Correspondence analysis revealed a significant association between antifungal treatment and outcome ( P < 0.001). The correspondence analysis could explain 53.9% of this relationship. Monotherapy and combination therapy were associated with survival. While salvage treatment or no antifungal therapy was associated with mortality, intravitreal injection or topical application of voriconazole was associated with sequelae. CONCLUSIONS: Surgical intervention and removal of contaminated devices or tissue should be considered at an early stage.

A Rare Malignity of Childhood: Krukenberg Tumor-Case Report and Review of Literature.

Krukenberg tumor (KT) is a rare ovarian carcinoma containing mucin-filled signet ring cells. It accounts for 1%-2% of all ovarian tumors. It is seen at an average age of 40 years. Reported pediatric cases of KT in the literature are very limited. Herein, we present an adolescent with a KT that was compatible with metastatic ring cell colon carcinoma.

A novel computer-based method for measuring the acetabular angle on hip radiographs.

OBJECTIVE: The aim of this study was to propose a new computer based method for measuring acetabular angles on hip radiographs and to assess its practicality, sensitivity and reliability for acetabular angle measurement. METHODS: A total of 314 acetabulum were assessed on 157 pelvic X-ray images. Acetabular angles were measured with both the conventional method (Method 1) and our proposed method (Method 2). All the Acetabular Index (AI) angle, Acetabular Angle (AA) and Acetabular Center (ACM) angle were measured with both methods. RESULTS: The mean AI angle for Method 1 is 11.02° ± 2.7° and the mean AI angle for Method 2 is 10.08° ± 1.88°, the mean AA angle for Method 1 is 39.5° ± 5.3° and the mean AA angle for Method 2 is 39.36° ± 4.68°, the mean ACM angle for Method 1 is 50.5° ± 6.01° and the mean ACM angle for Method 2 is 55.42° ± 12.43°. CONCLUSION: Our novel automated method appear to be reliable and practical for acetabular angle measurement on hip radiographs. LEVEL OF EVIDENCE: Level III, Diagnostic study.

[Factorial structure, validity, and reliability of the Turkish temperament and character inventory]. / Mizaç ve Karakter Envanteri'nin Türkçe Formunun Faktör Yapisi, Geçerlik ve Güvenilirligi.

OBJECTIVE: To assess the factorial structure, reliability and validity of the Turkish version of the Temperament and Character Inventory (TCI), a 240-item, self-report, paper-and-pencil test, and true-false format inventory based on Cloninger's psychobiological model of personality. It measures the four higher-order temperament dimensions and three character dimensions. METHOD: Using samples consisting of 470 healthy volunteers and 544 psychiatric patients, psychometric features were explored. RESULTS: The internal consistency of the scales was good (Cronbach alpha coefficients between 0.68 and 0.84), but weak for Reward dependence (0.55) and Persistence (0.56). The factor structures of the temperament and character dimensions, explored separately, were in agreement with the hypothesized constructs, except for the scales NS1 (Novelty Seeking 1 = exploratory excitability) and SD4 (Self-directedness 4 -- self-acceptance). The present study also confirmed that the TCI scales were weakly related among themselves. On the whole, psychiatric patients had higher harm avoidance and lower self-directedness, persistence, cooperativeness, and self-transcendence scores than the normal subjects. Gender differences were also found for different dimensions. CONCLUSION: The findings of this study suggest that the TCI can be applied in the investigation of psychiatric and normal populations.

Perinatal risk factors and mode of delivery associated with mortality in very low birth weight infants.

OBJECTIVE: To investigate the association of perinatal risk factors including delivery mode with mortality in very low birthweight (VLBW) in a tertiary hospital setting. METHODS: Medical records of 241 live-born VLBW infants (≤1500 g) were retrospectively reviewed. Details of maternal, obstetrical, perinatal risk factors and their associations with infant mortality were evaluated. RESULTS: The overall infant mortality rate was 23.2%. Mortality was significantly higher for infants born at ≤27 gestational weeks and with a birthweight of ≤750 g (p = 0.000 and p = 0.000, respectively), showing a steep decrease thereafter. On ROC analysis, a cut off of 26.5 weeks was determined for mortality with a sensitivity of 57.1% and a specificity of 90.3% (area under the curve = 0.792, 95% CI: 0.719-0.866). On multivariate regression analysis, gestational week at birth, birthweight, antenatal steroid treatment and pathologic Doppler ultrasound findings were found as independent risk factors for mortality. CONCLUSIONS: Gestational week at birth, birthweight and antenatal steroid treatment remain the most important perinatal risk factors for infant mortality in VLBW infants. Mode of delivery does not seem to be associated with mortality when adjusted for other perinatal risk factors.

The history of childhood trauma among individuals with ultra high risk for psychosis is as common as among patients with first-episode schizophrenia.

AIM: Childhood trauma (CT) is more common in patients with psychosis than in general population and is found to be related to the severity of symptoms. The objective of this study was to investigate the severity of CT, and its relationship with clinical features in two different groups: first-episode schizophrenia (FES) and ultra high risk for psychosis (UHR) groups. METHODS: In this cross-sectional study, 83 patients with FES, 41 individuals with UHR and 69 healthy controls were included. Clinical features were evaluated with the Brief Psychiatric Rating Scale, the Scale for the Assessment of Negative Symptoms and the Scale for the Assessment of Positive Symptoms (SAPS). We evaluated CT with the Childhood Trauma Questionnaire (CTQ). UHR group was also assessed with the Calgary Depression Scale for Schizophrenia. RESULTS: The emotional and physical abuse, physical and emotionalneglect subscale scores and CTQ total score of both the UHR group and FES group were higher than the control group. However, the CTQ total score and subscale scores did not differ between FES and UHR groups. UHR group had more Schneiderian symptoms in terms of both number and severity, and severity of sexual abuse was found to be correlated with SAPS scores especially for the 'commenting voices' item. The CTQ emotional abuse and neglect scores were correlated with the severity of depression. FES patients with higher CTQ scores obtained higher total scores on SAPS and higher total scores on Schneiderian items. CONCLUSION: We found that CT is related to the severity of psychotic symptoms in both FES and UHR groups. Therefore, it is possible that interventions aimed at preventing CT in children would reduce the manifestation of psychosis among young people.

Compassion fatigue in marriage and family therapy: implications for therapists and clients.

Given that marriage and family therapists are exposed to a wide range of circumstances that leave them uniquely vulnerable to experiencing compassion fatigue, it is important to examine the stresses and hazards they face and what those consequences mean for both themselves and clients. It is essential that they identify how compassion fatigue negatively affects the therapeutic relationship and overall treatment outcome as well as that of the personal life of the family therapist. The marriage and family therapist is responsible and ethically obligated to identify and implement ways in which he or she can prevent and remedy compassion fatigue.