Case presentation: a severe case of cobalamin c deficiency presenting with nephrotic syndrome, malignant hypertension and hemolytic anemia.

BACKGROUND: The etiology of nephrotic syndrome can vary, with underlying metabolic diseases being a potential factor. Cobalamin C (cblC) defect is an autosomal recessive inborn error of metabolism caused by mutations in the MMACHC gene, resulting in impaired vitamin B12 processing. While cblC defect typically manifests with hematological and neurological symptoms, renal involvement is increasingly recognized but remains rare. CASE PRESENTATION: We describe a 7-month-old male patient presenting with fatigue and edema. His first laboratory findings showed anemia, thrombocytopenia, hypoalbuminemia and proteinuria and further examinations reveals hemolysis in peripheric blood smear. During his follow up respiratory distress due to pleural effusion in the right hemithorax was noticed. And fluid leakage to the third spaces supported nephrotic syndrome diagnosis. The patient's condition deteriorated, leading to intensive care admission due to, hypertensive crisis, and respiratory distress. High total plasma homocysteine and low methionine levels raised suspicion of cobalamin metabolism disorders. Genetic testing confirmed biallelic MMACHC gene mutations, establishing the diagnosis of cblC defect. Treatment with hydroxycobalamin, folic acid, and betaine led to remarkable clinical improvement. DISCUSSION/CONCLUSION: This case underscores the significance of recognizing metabolic disorders like cblC defect in atypical presentations of nephrotic syndrome. Early diagnosis and comprehensive management are vital to prevent irreversible renal damage. While cblC defects are more commonly associated with atypical hemolytic uremic syndrome, this case highlights the importance of considering cobalamin defects in the differential diagnosis of nephrotic syndrome, especially when associated with accompanying findings such as hemolysis. Our case, which has one of the highest homocysteine levels reported in the literature, emphasizes this situation again.

Imaging of supratentorial intraventricular masses in children:a pictorial review- part 1.

PURPOSE: This article is the first in a two-part series designed to provide a comprehensive overview of the range of supratentorial intraventricular masses observed in children. Our primary objective is to discuss the diverse types of intraventricular masses that originate not only from cells within the choroid plexus but also from other sources. METHODS: In this article, we review relevant epidemiological data, the current genetics/molecular classification as outlined in the fifth edition of the World Health Organization's Classification of tumours of the Central Nervous System and noteworthy imaging findings. We conduct an exhaustive analysis of primary choroid plexus tumours as well as other conditions such as choroid plexus hyperplasia, choroid plexus cyst, choroid plexus xanthogranuloma, atypical teratoid rhabdoid tumour, meningioma, arteriovenous malformation and metastasis. RESULTS: We comprehensively evaluated each supratentorial intraventricular mass, providing an in-depth analysis of their unique clinical and histological characteristics. The fifth edition of the World Health Organization Classification of Tumours of the Central Nervous System introduces major modifications. These important changes could potentially have a profound impact on the management strategies and subsequent outcomes of these tumours. CONCLUSION: Intraventricular masses in children can arise from various sources. Surgical intervention is key for certain supratentorial intraventricular masses in paediatric patients, with preoperative neuroimaging essential to decide the best treatment approach, surgical or otherwise, as some cases may not require surgery.

Imaging of supratentorial intraventricular masses in children: a pictorial review-part 2.

PURPOSE: This article is the second in a two-part series aimed at exploring the spectrum of supratentorial intraventricular masses in children. In particular, this part delves into masses originating from cells of the ventricular lining, those within the septum pellucidum, and brain parenchyma cells extending into the ventricles. The aim of this series is to offer a comprehensive understanding of these supratentorial intraventricular masses, encompassing their primary clinical findings and histological definitions. METHODS: We conducted a review and analysis of relevant epidemiological data, the current genetics/molecular classifications as per the fifth edition of the World Health Organization (WHO) Classification of Tumors of the Central Nervous System (WHO CNS5), and imaging findings. Each supratentorial intraventricular mass was individually evaluated, with a detailed discussion on its clinical and histological features. RESULTS: This article covers a range of supratentorial intraventricular masses observed in children. These include colloid cysts, subependymal giant cell astrocytomas, ependymomas, gangliogliomas, myxoid glioneuronal tumors, central neurocytomas, high-grade gliomas, pilocytic astrocytomas, cavernous malformations, and other embryonal tumors. Each mass type is characterized both clinically and histologically, offering an in-depth review of their individual imaging characteristics. CONCLUSION: The WHO CNS5 introduces notable changes, emphasizing the vital importance of molecular diagnostics in classifying pediatric central nervous system tumors. These foundational shifts have significant potential to impact management strategies and, as a result, the outcomes of intraventricular masses in children.

Neuroimaging of Ocular Abnormalities in Children.

In this article, we will discuss the essential MR imaging protocol required for the assessment of ocular abnormalities including malignancies. Then we will describe relevant anatomy, ocular embryogenesis, and genetics to establish a profound understanding of pathophysiology of the congenital ocular malformations. Finally, we will discuss pediatric ocular malignancies, benign mimics, and the most common congenital ocular malformations with case examples and illustrations and give tips on how to distinguish these entities on neuroimaging.

A systematic review and meta-analytic synthesis of the relationship between compulsory citizenship behaviors and its theoretical correlates.

Background: Compulsory citizenship behaviors (CCBs) are increasingly endorsed and expected of workers in contexts where managerial worker protections are low and performance demands on workers are high. Although studies on compulsory citizenship behaviors have shown a significant increase in recent years, the literature still lacks a comprehensive meta-analysis. To fill this gap the purpose of this study is to synthesize the collective outcomes of prior quantitative research on CCBs with the objective of identifying the factors linked to the concept and offering a primary reference for future researchers. Methods: Forty-three different correlates with CCBs were synthesized. The dataset of this meta-analysis consists of 53 independent samples with a sample size of 17.491, contributing to 180 effect sizes. PRISMA flow diagram and PICOS framework were used for the study design. Result: Results showed only gender and age were significant among demographic characteristics related to CCBs. Correlates between CCBs and counterproductive workplace behaviors, felt obligation, work-family conflict, organizational-based self-esteem, organizational cynicism, burnout, anger toward the organization, and work alienation were found as large. We also found turnover intention, moral disengagement, careerism, abusive supervision, citizenship pressure, job stress, facades of conformity, and feeling trusted to be moderately related to CCBs. Next, there was a small relationship between CCBs and social loafing. On the other hand, LMX, psychological safety, organizational identification, organizational justice, organizational commitment, job satisfaction, and job autonomy were found as significant deterrents of CCBs. These results suggest that CCBs flourish in contexts with low levels of worker protection and low road practices to people management. Conclusion: In sum, we found solid cumulative evidence that CCBs are a harmful and undesirable phenomenon for employees and organizations. Also, positive correlations of felt obligation, feeling trusted, and organization-based self-esteem with CCBs, showed that, contrary to general acceptance, positive factors could also cause CCBs. Lastly, we found CCBs as a dominant phenomenon in eastern culture.

Metal concentrations, selenium-mercury balance, and potential health risk assessment for consumer of whiting (Merlangius merlangus euxinus L., 1758) from different regions of the southern Black Sea.

The significant increase in the human population and the associated industrial wastes have a widespread impact on coastal areas. It is important to monitor trace elements that affect food safety and pose a potential consumer health threat. All over the Black Sea coast, people enjoy consuming whiting as both meat and roe. In February 2021, whitings were caught by bottom trawling from four different locations on the coasts of Kastamonu, Sinop (Sarikum, Adabasi), and Samsun in the southern Black Sea region. The meat and roe extracts obtained from whiting samples were analyzed through an optical emission spectrophotometer (ICP-MS). Trace element concentrations of whiting meat and roe in this study were Zn > Fe > Sr > As > Al > Se > B > Mn > Cu > Hg > Li > Ni > Ba > Pb > Cr > Cd and Zn > Fe > Al > As > Cu > Sr > Mn > Se > B > Ba > Li > Ni > Hg > Cr > Pb > Cd, respectively. These amounts were lower than the acceptable values accepted by the EU Commission. In case of whiting and roe consumption of a maximum of three portions (860.33 g) for Adabasi, six portions for Kastamonu (1432.37 g), three portions for Samsun (828.55 g), and five portions (1253.04 g) for Sarikum per month do not pose a health risk.

How do compulsory citizenship behaviors affect moral disengagement in organizations? Significance of anger toward the organization during the COVID-19 pandemic.

Background: With the COVID-19 pandemic, healthcare professionals, especially nurses, are confronted with an intensified workload. The literature on compulsory citizenship behaviors and their consequences is still far from explaining the cognitive and emotional mechanisms that underlie this relationship. Methods: Drawing on the resource depletion theory, we unpack the mechanism by which compulsory citizenship behaviors influence moral disengagement with the mediation effects of anger toward the organization. We are reporting a cross-sectional survey of nurses (n = 294) in private and public hospitals in Istanbul, Turkey. The data analysis involved structural equation modeling and Bayesian mediation. Results: The study revealed that compulsory citizenship behaviors positively influenced anger toward the organization and moral disengagement. Further, anger toward the organization mediates the link between compulsory citizenship behaviors and moral disengagement. Likewise, the Bayesian mediation analysis indicated that the proportion mediated (PM), which ensures a prediction of the extent to which the pathway explains the total effect through the mediation effect, was 33.74%. Conclusion: The findings show that exposure to compulsory citizenship behaviors lead to negative emotional (anger toward to organization) and cognitive (moral disengagement) consequences in nurses. Practical implications: Hospital managers should not force nurses to display discretionary work tasks outside their job descriptions. Providing an organizational milieu where voluntarily extra-role behaviors are encouraged may help reduce nurses' moral disengagement and, in turn, ease their anger toward the organization.

Counterintuitive consequences of COVID-19 on healthcare workers: A meta-analysis of the relationship between work engagement and job satisfaction.

Background: Studies conducted in the health sector have determined a positive relationship between job satisfaction and work engagement. However, this paper reveals that this relationship turns into a negative or non-significant relationship during the COVID-19 pandemic. We explore the reasons for inconsistency in research findings in this critical period through a meta-analysis. Methods: This study was conducted according to the PRISMA guidelines and PICO framework. Online databases including Web of Science, Scopus, PubMed, ProQuest, Google Scholar, and additional records from other databases were searched without any time limitation, and all studies published in English that reported the correlation between work engagement and job satisfaction were included in the analysis. In total, 36 individual correlation coefficients were synthesized. R statistical language was used to analyze the data. Result: A total of 36 studies with a sample size of 16,087 were synthesized. The overall effect size was found as r = 0.57 (95% CI [0.50-0.64]). While the moderating effect of national culture was not statistically significant, presence of COVID-19 as the significant moderator explained 37.08% of effect size heterogeneity. Such that the presence of COVID-19 has transformed the positive relationship between work engagement and job satisfaction into a negative but statistically non-significant relationship. Conclusion: This study empirically challenges the existing assumptions about the positive link between work engagement and job satisfaction. The results of the research can be a guide for managers and policymakers. Specifically, based on these results, different mechanisms can be put in place to support work engagement and, in turn, job satisfaction in the COVID-19 process.

Testing the validity and reliability of a Turkish version of the social cyberloafing scale.

PURPOSE: This paper aims to evaluate the validity and reliability of the Turkish version of the social cyberloafing (SC) scale as an important predictor of the mental health of healthcare employees. METHODS: The data for this descriptive cross-sectional study was obtained from 202 Turkish healthcare employees. We followed Dima's 6-step protocol for validation. FINDINGS: The one-dimensional structure of the scale was found to be homogenous (H = 0.54) and reliable (α = 0.87). The CFA demonstrated that the 7-item and one-factor construct was a valid instrument to measure the SC (GFI = 0.98, CFI = 0.98, TLI = 0.95, SRMR = 0.03, RMSEA = 0.09). PRACTICAL IMPLICATIONS: These results demonstrated that the Turkish version of the SC scale is an accurate measurement tool.

COVID-19-Associated Cerebral White Matter Injury in a Newborn Infant With Afebrile Seizure.

Coronavirus disease 2019 (COVID-19) symptoms in newborn infants are incompletely described. We present the first case of neuroradiologic abnormality associated with COVID-19 in a newborn infant with afebrile seizure. This case underlines the possible neurologic involvement of severe acute respiratory syndrome coronavirus 2 in this age group.

Relationship between work-family conflict and turnover intention in nurses: A meta-analytic review.

AIM: This meta-analytic review aimed to synthesize and analyse studies that explored the relationship between nurses' work-family conflicts and turnover intentions. DESIGN: This meta-analytical review was conducted according to the Joanna Briggs Institute guidelines and PRISMA checklist. DATA SOURCES: A total of 191 (k = 14) publications published between 2005 and 2019 in English, including grey literature on turnover intention and work-family conflict, were retrieved from PubMed, PsycINFO, Web of Science, ProQuest and Scopus databases. REVIEW METHODS: Studies on the relationship between work-family conflict and turnover intention were summarized. RESULTS: An overall effect size of r = .28 (N = 5781, 95% CI [0.23-0.33]) was obtained, indicating a moderate, positive and significant relationship between work-family conflict and turnover intention. The moderator analysis showed that individualism and long-term orientation accounted for 90% of effect size heterogeneity of work-family conflict and turnover intention relationship. CONCLUSION: Exploring the correlation between work-family conflict and turnover intention can provide guidelines and recommendations for the development of strategies to promote nurse retention and alleviate the nursing shortage. National culture, particularly individualism and long-term orientation, were found to play a significant moderator role in this relationship. Cultures that are highly individualistic and have a long-term orientation have a diminishing effect on the relationship between work-family conflict and turnover intention. IMPACT: Work-family conflict and turnover intention are significantly correlated factors regardless of the studies' cultural characteristics examined in this study. Policymakers and managers should consider this finding and develop strategies that provide a balance-oriented work design to prevent nurse shortage.

A pin detected by ultrasonography within the normal appendix: prior to surgery, an impressive use of ultrasonography to localize an ingested foreign body exactly.

We present a 1-year-old boy who was asymptomatic and brought to the emergency room on suspicion of his having swallowed a pin. Confirmation of ingestion of the pin and its passage through the gut was achieved with abdominal radiography. The pin, which was followed with serial abdominal radiographs, was expected to leave the gastrointestinal tract, but was fixed to the right lower quadrant. When the pin had not passed after 10 days, and with increasing concern about the likelihood of perforation, ultrasonography was used to locate its exact position and allow surgical removal. Only a few cases involving the use of ultrasonography to reveal the exact location of an ingested foreign body prior to surgery have been reported in the literature. This case presents an impressive example of the use of ultrasonography to reveal the intra-appendiceal location of an ingested foreign body, and to facilitate its surgical removal.

Biliary fascioliasis: sonographic appearance patterns.

PURPOSE: To describe the sonographic findings of biliary fascioliasis. METHOD: The study included 27 patients with fascioliasis and abdominal sonographic findings in biliary system. All diagnoses were confirmed via serologic enzyme-linked immunosorbent assay, and 5 patients underwent cholecystectomy. Sonographic findings in the biliary system were defined as primary and secondary. RESULTS: Primary findings included spontaneously moving echogenic structures, linear echoes, curvilinear echoes, oval-shaped echogenic structure, matted echogenic particle, echogenic particle adherent to the gallbladder wall, motionless freely floating round echogenic foci, and leaf-like echogenic structures. Secondary findings were dilatation or wall thickening of the biliary system. In the liver, multiple confluent subcapsular nodules were also noted on sonography and CT in 14 of 27 patients. CONCLUSION: Sonography can detect biliary system lesions in fascioliasis and can aid diagnosis of the disease. However, the radiologist should be familiar with the different sonographic appearances of biliary fascioliasis.

Accessory cardiac bronchus and tracheal bronchus anomalies: CT-bronchoscopy and CT-bronchography findings.

Most common developmental anomalies of bronchial tree include accessory cardiac bronchus (ACB) and tracheal bronchus (TB). Minor bronchial anomalies include variants of TB, displaced segmental bronchi, and bronchial agenesis. We present CT-bronchoscopy and CT-bronchography findings of three cases with either ACB or TB. Recognition of these anomalies is important, as associated clinical complications, including recurrent episodes of infection, hemoptysis, and perhaps malignancies may be anticipated in a small percentage of patients.

Evaluation of CSF flow patterns of posterior fossa cystic malformations using CSF flow MR imaging.

INTRODUCTION: Differential radiologic diagnosis of cystic malformations of the posterior fossa is often difficult with conventional imaging techniques because of overlapping features of these entities. Posterior fossa cystic malformations occupy the cerebrospinal fluid (CSF) spaces. They may create secondary dynamic effects on the movements of CSF. The aim of this study was to investigate CSF flow alterations in posterior fossa cystic malformations with CSF flow MR imaging. METHODS: The study included 40 patients with cystic malformations of the posterior fossa. The patients underwent cardiac-gated phase-contrast cine MR imaging. CSF flow was qualitatively evaluated using an in-plane phase-contrast sequence in the midsagittal plane. The MR images were displayed in a closed-loop cine format. RESULTS: Twelve of the patients had communicating arachnoid cyst, seven had non-communicating arachnoid cyst, ten had mega cisterna magna, six had Dandy-Walker malformation, two had Dandy-Walker variant, and three had Blake's pouch cyst. CSF flow MR imaging indicated the regions of no, slow or higher flow, direction of flow, and abnormal cystic fluid motion. Each malformation displayed a distinct CSF flow pattern. CONCLUSION: Phase-contrast cine MR imaging for CSF flow evaluation may be a useful adjunct to routine MR imaging in the evaluation of the cystic malformations of the posterior fossa because it can improve the specificity in differentiating such malformations.

Complete resolution of an alveolar echinococcosis liver lesion following percutaneous treatment.

Herein we present a 63-year-old male patient with a solid hepatic alveolar echinococcosis diagnosed by surgical biopsy. His liver lesion, which was infected, was drained by percutaneous catheterization. The lesion surprisingly disappeared completely after the treatment. The patient was followed-up without any symptoms for 20 months after the drainage. As alveolar echinococcosis of the liver behaves like a slow-growing liver cancer, the disappearance of our patient's lesion was a very unusual and rare outcome, which, to the best of our knowledge, has never been published in the literature.

Intracranial lipomas: importance of localization.

Intracranial lipomas are rare congenital malformations. They are usually pericallosal asymptomatic midline lesions. Other brain malformations are often seen in association with intracranial lipomas. We describe the findings of imaging studies, including computed tomography (CT), magnetic resonance (MR) imaging, and MR angiography, along with a brief review of the literature. The frequency and the spectrum of the associated brain malformations are also discussed. We retrospectively reviewed CT and MR findings of 24 patients (14 female, 10 male, mean age 38.6 years) diagnosed with intracranial lipoma between December 2000 and June 2004 in two different radiology departments. Seventeen of the patients were diagnosed using cranial MR and seven with cranial CT. The CT density of all lesions was measured. Imaging characteristics of lipomas, morphological findings and associated malformations were described. The intracranial locations of the lipomas were left-sided quadrigeminal cistern (n=3), right-sided quadrigeminal cistern (n=4), interpeduncular cistern (n=1), sylvian fissure (n=3), interhemispheric fissure (n=3), choroid plexus (n=2), intercerebellar fissure (n=3), corpus fornicis (n=1) and the periphery of the corpus callosum (n=4). Eighteen of the intracranial lipomas were tubulonodular; six were curvilinear. Associated anomalies were observed in six patients. All of the patients with sylvian fissure lipoma had seizures. The two preferential sites of intracranial lipomas were pericallosal and dorsal mesencephalic. Most intracranial lipomas are found incidentally during neuroradiological investigations. CT and MR examination usually lead to the diagnosis, because of the very low attenuation values of lipomas on CT and the short T1 and T2 on MR. Midline anomalies and other malformations such as aneurysms are frequently associated with intracranial lipomas. Careful radiologic evaluation is therefore necessary to evaluate associated pathologies. Sylvian fissure lipomas should be considered in the differential diagnosis of patients with epilepsy.