[18F]RO948 tau positron emission tomography in genetic and sporadic frontotemporal dementia syndromes.

PURPOSE: To examine [18F]RO948 retention in FTD, sampling the underlying protein pathology heterogeneity. METHODS: A total of 61 individuals with FTD (n = 35), matched cases of AD (n = 13) and Aß-negative cognitively unimpaired individuals (n = 13) underwent [18F]RO948PET and MRI. FTD included 21 behavioral variant FTD (bvFTD) cases, 11 symptomatic C9orf72 mutation carriers, one patient with non-genetic bvFTD-ALS, one individual with bvFTD due to a GRN mutation, and one due to a MAPT mutation (R406W). Tracer retention was examined using a region-of-interest and voxel-wise approaches. Two individuals (bvFTD due to C9orf72) underwent postmortem neuropathological examination. Tracer binding was additionally assessed in vitro using [3H]RO948 autoradiography in six separate cases. RESULTS: [18F]RO948 retention across ROIs was clearly lower than in AD and comparable to that in Aß-negative cognitively unimpaired individuals. Only minor loci of tracer retention were seen in bvFTD; these did not overlap with the observed cortical atrophy in the cases, the expected pattern of atrophy, nor the expected or verified protein pathology distribution. Autoradiography analyses showed no specific [3H]RO948 binding. The R406W MAPT mutation carriers were clear exceptions with AD-like retention levels and specific in-vitro binding. CONCLUSION: [18F]RO948 uptake is not significantly increased in the majority of FTD patients, with a clear exception being specific MAPT mutations.

Prenatal gyrification pattern affects age at onset in frontotemporal dementia.

The paracingulate sulcus is a tertiary sulcus formed during the third trimester. In healthy individuals paracingulate sulcation is more prevalent in the left hemisphere. The anterior cingulate and paracingulate gyri are focal points of neurodegeneration in behavioral variant frontotemporal dementia (bvFTD). This study aims to determine the prevalence and impact of paracingulate sulcation in bvFTD. Structural magnetic resonance images of individuals with bvFTD (n = 105, mean age 66.9 years), Alzheimer's disease (n = 92, 73.3), and healthy controls (n = 110, 62.4) were evaluated using standard protocol for hemispheric paracingulate sulcal presence. No difference in left hemisphere paracingulate sulcal frequency was observed between groups; 0.72, 0.79, and 0.70, respectively, in the bvFTD, Alzheimer's disease, and healthy control groups, (P = 0.3). A significant impact of right (but not left) hemispheric paracingulate sulcation on age at disease onset was identified in bvFTD (mean 60.4 years where absent vs. 63.8 where present [P = 0.04, Cohen's d = 0.42]). This relationship was not observed in Alzheimer's disease. These findings demonstrate a relationship between prenatal neuronal development and the expression of a neurodegenerative disease providing a gross morphological example of brain reserve.

Intraoperative neurophysiological monitoring in scoliosis surgery: literature review of the last 10 years.

INTRODUCTION: Patients with spinal deformities undergoing corrective surgery are at risk for iatrogenic spinal cord injury (SCI) and subsequent neurological deficit. Intraoperative neurophysiological monitoring (IONM) allows early detection of SCI which enables early intervention resulting in a better prognosis. The primary aim of this literature review was to search if there are threshold values of TcMEP and SSEP in the literature that are widely accepted as alert during IONM. The secondary aim was to update knowledge concerning IONM during scoliosis surgery. METHOD: PubMed/MEDLINE and Cochrane library electronic databases were used to search publication from 2012 to 2022. The following keywords were used: evoked potential, scoliosis, surgery, intraoperative monitoring and neurophysiological. We included all studies dealing with SSEP and TcMEP monitoring during scoliosis surgery. Two authors reviewed all titles and abstracts to identify studies that met the inclusion criteria. RESULTS: We included 43 papers. Rates of IONM alert and neurological deficit varied from 0.56 to 64% and from 0.15 to 8.3%, respectively. Threshold values varied from a loss of 50 to 90% for TcMEP amplitude, whereas it seems that a loss of 50% in amplitude and/or an increase of 10% of latency is widely accepted for SSEP. Causes of IONM changes most frequently reported were surgical maneuver. CONCLUSION: Concerning SSEP, a loss of 50% in amplitude and/or an increase of 10% of latency is widely accepted as an alert. For TcMEP, it seems that the use of highest threshold values can avoid unnecessary surgical procedure for the patient without increasing risk of neurological deficit.

Successful closed reduction of a trans-scaphoid perilunate dislocation in a 11-year-old boy: a case report.

CASE: Perilunate dislocation is an uncommon injury in children. We report the case of an 11-year-old boy who presented a trans-scapho-perilunate fracture-dislocation of the carpus after falling from his bike. Treatment consisted of a closed reduction under general anesthesia followed by cast immobilization for 12 weeks. The injury healed with good wrist function on follow-up. CONCLUSION: Closed reduction should be attempted because it can be successful and allows for closed treatment with the cast. It provides good radiological healing and satisfying functional results.

Who needs prenatal counselling with a pediatric surgeon? Experience from a large tertiary care university hospital.

OBJECTIVES: To describe our experience with prenatal counselling for surgical anomalies in a large volume center. The secondary aim is to suggest a list of prenatal abnormalities warranting counselling by a pediatric surgeon. METHODS: We reviewed all prenatal counselling consultations performed by the pediatric surgery team between January 1st, 2015 and December 31st, 2016. RESULTS: A total of 169 patients or couples had a prenatal consultation with a pediatric surgeon. Prenatal work-up included a fetal MRI in 26% of cases, mainly for digestive and thoracic pathologies (56.1% of cases). Consultation with the pediatric surgeon led mainly to recommendations concerning the place of delivery. Induction for reasons related to the fetal anomaly occurred in 22.2% of cases. Most children were surgically treated within the first year of life (63.5%). Correlation between predicted prognosis and actual status at four years of life was 96.9%. Correlation between prenatal and postnatal diagnosis was 87.4%. CONCLUSIONS: Prenatal counselling by a pediatric surgeon allows couples to obtain clear information on the pathology of their unborn child, giving them greater autonomy in their decision to continue the pregnancy.

Functional and radiological outcome after forearm plating in children and adolescent fracture.

The literature on forearm overgrowth after plating in traumatic conditions is relatively poor though this technique can be useful when intra-medullary nailing is not sufficient in pediatric cases. The goal of this study was to assess a potential overgrowth after plating and identify impact on function. We conducted a retrospective study of all pediatric patients who underwent open surgery of the radius and/or ulna diaphysis with internal fixation by plating, in our institution, between October 2013 and July 2019. At last follow-up, functional and radiological outcomes were compared between the operated and uninjured forearm. Range of motion (ROM) of the wrist and elbow, clinical scores, radial and ulnar length were measured. A positive bone length discrepancy of more than 2mm was considered as an overgrowth. Were also studied the radio-ulnar index, radial inclination and radiocarpal angle. Thirteen patients were included. The mean age was 12.1 years old (±3.0 years), they were plated on the radius (10 cases) or on the ulna (3 cases). Mean follow- up was 4.4 years (± 1.8). In two cases, the plated bone (radius) was significantly longer than the uninjured one. There was no significant difference regarding radio-ulnar index, radial inclination and radiocarpal angle. The only statistically significant difference between the operated and uninjured forearm was the pronation/supination range, which was greater in the uninjured forearm (mean 160 ±48° versus 175 ±49°, p=0.01). This study confirms the good functional and radiological outcomes after plating even in a skeletally immature forearm. Level of evidence : IV.

Divergent Intramedullary Nailing (DIN): A Modified Intramedullary Nailing Technique to Treat Paediatric Distal Tibial Fractures.

INTRODUCTION: Elastic stable intramedullary nailing (ESIN) allows for efficient reduction and stabilization of fractures of the tibial shaft in children and adolescents. However, for fractures of the distal third of the tibia, traditional ESIN could be inappropriate, thus compromising the stability and the healing of the fracture. The aim of this study was to present and to assess a new technique of modified ESIN to treat fracture of the distal third of the tibia, called divergent intramedullary nailing (DIN). METHODS: We performed a retrospective monocentric study. All patients less than 16 years old, managed in our pediatric orthopaedics department, and operated upon according to the DIN technique for a displaced and/or unstable fracture of the distal third of the tibia were included. Demographic and surgical data were collected. X-rays were performed preoperatively, postoperatively, at 6 weeks, and every 6 months. The surgical technique starts as does the classic ESIN. However, nails are not curved, so that they cross only once at the proximal part of the tibia; they are divergent and supported by the medial and lateral distal part of the tibial shaft. This allows for reduction and stabilization of the fracture. RESULTS: A total of 13 patients were included, with a mean age of 10 years and a mean follow-up of 32 months. The size of the nail varied between 2.5 and 4 mm. The mean surgical time was 54 minutes. The DIN technique provided a satisfying reduction (coronal and sagittal angulation <3 degrees) for the 13 fractures. In addition, there was no secondary displacement at 6 weeks. All patients were healed at 6 months, with no clinical torsion or axis malalignment. CONCLUSIONS: The current study confirms the feasibility and the efficiency of the DIN method to treat fracture of the distal third of the tibia.

Bladder Dysfunction in Children with Neurofibromatosis Type I: Report of Four Cases and Review of the Literature.

AIM: Neurofibromatosis type 1 (NF1) is a rare autosomal dominant disorder. Malignant transformation into malignant peripheral nerve sheath tumors (MPNST) can occur. However, urinary tract involvement is rare. We report 4 cases of NF1 with bladder dysfunction. METHODS: A retrospective single center analysis of 4 patients was conducted over a 17-year period, focusing on urinary tract involvement. RESULTS: NF1 was diagnosed at a median of 16.5 months (4-36) and urinary involvement occurred at a median of 5.25 years (4-9) after diagnosis. Bladder dysfunction was due to spinal cord compression in 2 cases, bladder invasion in 1 case, and cerebral lesions in 1 case. Malignant transformation of neurofibromas into MPNST occurred in 2 patients. Mechanisms of urinary involvement in NF1 are diverse and no pre-established protocol of management and follow-up exists. CONCLUSION: Although rare, dysfunction of the bladder can arise in NF1 and innovative strategies then need to be considered. This is best achieved with the help of a multidisciplinary team and a national reference center when available.

Preliminary experience using a tunica vaginalis flap as the dorsal component of Bracka's urethroplasty.

OBJECTIVE: To evaluate clinical use of a tunica vaginalis flap as the dorsal component of a two-stage urethroplasty in boys with cripple hypospadias. PATIENTS AND METHODS: We performed the first stage of a Bracka two-stage urethroplasty, using a tunica vaginalis flap as the dorsal component in six boys with cripple hypospadias. We analysed their clinical characteristics and the results of this technique. RESULTS: The mean (range) age of the boys was 57 (34-120) months. The mean (range) number of previous procedures the boys had undergone was 4 (3-5). At the 6-month follow-up, all the boys presented significant fibrosis of the dorsal graft rendering it unusable for tubularisation. CONCLUSIONS: Exposure to the external environment seems to induce retraction and fibrosis of the tunica vaginalis. We believe one should be very cautious about using tunica vaginalis as the dorsal component of a two-stage urethroplasty, as significant fibrosis might well render the flap unusable.

Can quantity of amniotic fluid reliably predict postnatal renal function in boys with posterior urethral valves: a decision curve analysis.

OBJECTIVE: Prenatal management of male fetuses with suspected posterior urethral valves depends on reliable markers for postnatal long-term renal function. Whether ultrasound parameters, including the presence or absence of oligohydramnios, are reliable remains the subject of debate. We decided to evaluate the reliability of quantity of amniotic fluid to predict postnatal renal function using decision curve analysis (DCA), a method for evaluating the clinical utility of a diagnostic test. METHODS: We analyzed retrospectively 51 male fetuses born with prenatally suspected posterior urethral valves between 2009 and 2012. We studied the relationship between quantity of amniotic fluid on prenatal ultrasound and the nadir creatinine during the first year of life as a proxy of postnatal renal function using DCA. RESULTS: Twelve fetuses presented with prenatal oligohydramnios. Thirty-one children had a normal nadir creatinine, of which one had prenatal oligohydramnios (3.2%). Thirteen had a nadir creatinine between 35 and 75 µmol/L, of which four had prenatal oligohydramnios (30.8%). Seven had a nadir creatinine >75 µmol/L, all of them had prenatal oligohydramnios. CONCLUSION: In this retrospective study, DCA confirms the relationship between prenatal quantity of amniotic fluid volume and postnatal renal function. © 2017 John Wiley & Sons, Ltd.

Critical Analysis of a Series of Early Inguinal Ureterostomies: Are They Useful and How Well Are They Tolerated?

INTRODUCTION: The study aimed to evaluate the advantages of temporary inguinal ureterostomy in the management of neonates with uropathies and early or recurrent pyelonephritis. PATIENTS AND METHODS: We performed a retrospective analysis of all patients who underwent ureterostomies between 1989 and 2012, with specific regards to indications and outcomes. We also performed a survey of parents to evaluate their acceptance of diversion. RESULTS: We included 18 patients (12 primary high-grade vesicoureteral reflux [VUR] and 6 primary obstructive megaureters [MUs]). Indications were recurrent febrile urinary tract infections (UTIs) despite antibiotic prophylaxis, doubtful function of the overlying kidney for the oldest cases, when renal function was only assessed by intravenous urography, or both. Cutaneous diversion was performed between the ages of 2 weeks to 5 months (median: 1.8 months). Renal function was assessed prior to undiversion to choose between reimplantation and nephrectomy. The incidence of febrile UTIs significantly decreased during the period of diversion. Urinary diversion was judged socially acceptable by parents. Ureterostomy did not modify the overlying kidney function. CONCLUSION: Temporary inguinal ureterostomy does not enable better evaluation of renal function by suppressing the pressure of an obstacle or refluxing urines. Its remaining indication seems to be the prevention of recurrent UTIs in neonates and infants with VUR or MU, pending reimplantation.

A novel approach to evaluating the benefit of post-urinary tract infection renal ultrasonography, using decision curve analysis.

BACKGROUND: The benefit of post-urinary tract infection (UTI) sonography to detect clinically significant renal abnormalities remains a subject open to debate. Decision curve analysis (DCA) is a novel method for evaluating the clinical usefulness of diagnostic tests. Our objective was to determine, using DCA, the benefit of post-UTI sonography and of post-UTI sonography with biological markers of inflammation to predict the risk of recurrence of febrile UTI in children aged 2 to 24 months without known uropathy. METHODS: We retrospectively analyzed all children aged 2 to 24 months, without known uropathy, who presented with a first episode of febrile UTI between 2009 and 2012 and followed them for 30 months. We then used DCA to estimate the benefit of post-UTI sonography or post-UTI sonography + biological markers of inflammation for detecting the risk of recurrence. RESULTS: A total of 318 children [144 boys (45.3 %) and 174 girls (54.7 %)], with a mean age of 6.9 ± 5.6 months, were identified. Of these, 210 children presented with a significant inflammation [66.2 %; 95 % confidence interval (CI) 61.0-71.4], and 30 (9.4 %; 95 % CI 6.2-12.6) presented with abnormal post-UTI sonographic findings. Eighteen (5.7 %; 95 % CI 3.1-8.2) children presented with recurrent UTI at 30 months. CONCLUSIONS: There were significantly more recurrences in those children who presented with abnormal sonographic findings than in those who did not (relative risk 7.68; 95 % CI 3.03-19.46). However, taking into account the effect of false-positives and false negatives, the DCA revealed that for threshold probabilities of >30 %, at which patients/doctors are concerned about unnecessary interventions (whether tests or treatments), neither post-UTI sonography nor post-UTI sonography + biological markers of inflammation have sufficient value to improve care.

Prescription errors in the National Health Services, time to change practice.

INTRODUCTION: Medication error is a major source of iatrogenic illness. Error in prescription is the most common form of avoidable medication error. We present our study, performed at two, UK, National Health Services Hospitals. MATERIAL AND METHODS: The prescription practice of junior doctor's working on general medical and surgical wards in National Health Service District General and University Teaching Hospitals in the UK was reviewed. Practice was assessed against standard hospital prescription charts, developed in accordance with local pharmacy guidance. RESULTS: A total of 407 prescription charts were reviewed in both initial audit and re-audit one year later. In the District General Hospital, documentation of allergy, weight and capital-letter prescription was achieved in 31, 5 and 40% of charts, respectively. Forty-nine per cent of discontinued prescriptions were properly deleted and signed for. In re-audit significant improvement was noted in documentation of the patient's name 100%, gender 54%, allergy status 51% and use of generic drug name 71%. Similarly, in the University Teaching Hospital, 82, 63 and 65% compliance was achieved in documentation of age, generic drug name prescription and capital-letter prescription, respectively. Prescription practice was reassessed one year later after recommendations and changes in the prescription practice, leading to significant improvement in documentation of unit number, generic drug name prescription, insulin prescription and documentation of the patient's ward. CONCLUSION: Prescription error remains an important, modifiable form of medical error, which may be rectified by introducing multidisciplinary assessment of practice, nationwide standardised prescription charts and revision of current prescribing clinical training.

Predicting clinically significant events in children with ureteropelvic junction obstruction.

Introduction: Ureteropelvic junction obstruction (UPJO) syndrome is one of the most common causes of neonatal hydronephrosis. Management varies from simple monitoring to surgical intervention, with indications differing between institutions. A consensus of 8 societies recently described a new Urinary Tract Dilation (UTD) classification which aims to standardize ultrasound description of hydronephrosis, but which is also supposed to have predictive value in children with hydronephrosis. Our aim was to compare, in a monocentric prospective cohort of children with UPJO, the ability of UTD to predict the occurrence of a clinically significant event within the first year of life, as compared to anteroposterior diameter of the renal pelvis (APD). Study design: We used a preexisting cohort of children followed in a prospective study on UPJO. A pediatric radiologist, blinded to the children's outcome, classified the last antenatal ultrasound and postnatal ultrasound according to the UTD-A and UTD-P classification. He also confirmed the APD-A and APD-P measures. We defined a clinically significant event as being: increased pelvic dilation (>5 mm) and/or the presence of a febrile urinary tract infection (fUTI) and/or impaired renal function on initial nuclear scan (<40%). We performed a ROC-AUC curve and Random Forest (RF) analysis to compare the ability of the APD-A, APD-P, UTD-A and UTD-P scores to predict a clinically significant event. Results: The cohort included 28 children. Clinically significant events were noted in 20 out of 28 patients: 13 children presented an increase >5 mm in dilation, 6 presented an episode of fUTI and 9 had impaired function of the affected kidney. APD-A was the most effective individual criterion for predicting the occurrence of a significant clinical event (AUC = 0.867). Conclusion: In our series, for children with UPJO, the most significant marker was prenatal APD >15 mm to predict an increase in dilation >5 mm.

Structural and functional connectivity associations with anterior cingulate sulcal variability.

Sulcation of the anterior cingulate may be defined by presence of a paracingulate sulcus, a tertiary sulcus developing during the third gestational trimester with implications on cognitive function and disease. In this cross-sectional study we examine task-free resting state functional connectivity and diffusion-weighted tract segmentation data from a cohort of healthy adults (< 60-year-old, n = 129), exploring the impact of ipsilateral paracingulate sulcal presence on structural and functional connectivity. Presence of a left paracingulate sulcus was associated with reduced fractional anisotropy in the left cingulum bundle and the left peri-genual and dorsal bundle segments, suggesting reduced structural organisational coherence in these tracts. This association was not observed in the offsite temporal cingulum bundle segment. Left paracingulate sulcal presence was associated with increased left peri-genual radial diffusivity and tract volume possibly suggesting increased U-fibre density in this region. Greater network dispersity was identified in individuals with an absent left paracingulate sulcus by presence of a significant, predominantly intraregional, frontal component of resting state functional connectivity which was not present in individuals with a present left paracingulate sulcus. Seed-based functional connectivity in pre-defined networks was not associated with paracingulate sulcal presence. These results identify a novel association between sulcation and structural connectivity in a healthy adult population with implications for conditions where this variation is of interest. Presence of a left paracingulate sulcus appears to alter local structural and functional connectivity, possibly as a result of the presence of a local network reliant on short association fibres.

Structural and functional connectivity associations with anterior cingulate sulcal variability.

Background: Sulcation of the anterior cingulate may be defined by presence of a paracingulate sulcus, a tertiary sulcus developing during the third gestational trimester with implications on cognitive function and disease. Methods: In this retrospective analysis we examine task-free resting state functional connectivity and diffusion-weighted tract segmentation data from a cohort of healthy adults (< 60-year-old, n = 129), exploring the impact of ipsilateral paracingulate sulcal presence on structural and functional connectivity. Results: Presence of a left paracingulate sulcus was associated with reduced fractional anisotropy in the left cingulum (P = 0.02) bundle and the peri-genual (P = 0.002) and dorsal (P = 0.03) but not the temporal cingulum bundle segments. Left paracingulate sulcal presence was associated with increased left peri-genual radial diffusivity (P = 0.003) and tract volume (P = 0.012). A significant, predominantly intraregional frontal component of altered resting state functional connectivity was identified in individuals possessing a left PCS (P = 0.01). Seed-based functional connectivity in pre-defined networks was not associated with paracingulate sulcal presence. Conclusion: These results identify a novel association between neurodevelopmentally derived sulcation and altered structural connectivity in a healthy adult population with implications for conditions where this variation is of interest. Furthermore, they provide evidence of a link between the structural and functional connectivity of the brain in the presence of a paracingulate sulcus which may be mediated by a highly connected local functional network reliant on short association fibres.

Elevating pediatric urology care: The crucial role of nursing research in quality improvement.

This educational article highlights the critical role of pediatric urology nursing research in improving the care of children with urological conditions. It discusses the multifaceted nature of pediatric urology nursing, addresses challenges such as limited nurse scientists and resource constraints, and highlights the need to overcome barriers to increase research involvement. The authors emphasize the importance of prioritizing research areas, the promotion of collaboration, and the provision of adequate funding and academic time for pediatric nurses to contribute to evidence-based practice, to improve patient outcomes. Furthermore, it highlights the importance of research in advancing nursing practice, shaping protocols, and advocating for the rights and needs of children with urological conditions and their families.