Liver international : official journal of the International Association for the Study of the LiverSamer Gawrieh, Jingyi Tan, Xiuqing Guo, Linus Schwantes-An, Marco Abreu, Callie J Zaborenko, Eduardo Vilar-Gomez, Jeffrey B Schwimmer, Jerome I Rotter, Naga Ch…
BACKGROUND AND AIMS: The genetic susceptibility to MASLD histological severity remains incompletely defined. We assessed the associations between recently developed genome-wide association studies-derived polygenic risk scores (PRSs) and advanced fibrosis in MASLD. We also evaluated PRSs' associations with histological activity and selected PRSs' predictive ability for advanced fibrosis. METHODS: We analysed 2149 adults and 900 children with biopsy-proven MASLD from the NASH Clinical Research Network studies. Genotyping was performed by the Regeneron Genetics Center using whole-exome sequencing with targeted genotyping. Associations between seven published PRSs (Chen, Emdin, Whitfield, Schwantes-An, Vujkovic, and Ghouse) and MASLD histological features were examined using linear and logistic regression models. Penalized regression models were used to select top PRSs associated with MASLD traits. RESULTS: The Schwantes-An PRS in adults (OR 1.29, 95% CI 1.17-1.42, p < 0.01) and the Chen PRS in children (OR 1.46, 95% CI 1.17-1.82, p < 0.01) were associated with higher risk of advanced fibrosis versus other PRSs. The Chen PRS in adults (OR 1.29, 95% CI 1.18-1.41, p < 0.01) and Schwantes-An PRS in children (OR 1.39, 95% CI 1.20-1.61, p < 0.05) were associated with higher risk with MASLD activity. Although highest PRSs quartiles were associated with advanced fibrosis risk versus bottom quartiles, neither PRS had good diagnostic discrimination for advanced fibrosis (AUROC < 60%). CONCLUSIONS: In this large biopsy-proven MASLD cohort, two distinct PRSs were associated with disease histological severity and activity in adults versus children. Findings suggest current PRSs may improve personalized risk stratification for advanced fibrosis but not its diagnosis.
BACKGROUND: Unplanned births outside hospitals involve higher risks of complications for mothers and newborns and require special obstetric or pediatric skills. However, ambulance calls for childbirth are rare, making it difficult for ambulance staff to maintain their skills. In Europe, unplanned out-of-hospital deliveries constitute 0.10% to 0.61% of all births. The exact number of prehospital births in the Region of Southern Denmark remains unknown. The study aimed to determine the extent of unplanned prehospital births attended by the ambulance service outside hospitals in the Region of Southern Denmark from January 2016 through June 2024. We further aimed to identify where the unplanned pre-hospital births took place and to report any birth-related complications in the mothers or newborns encountered by ambulance staff. METHODS: The study was a cross-sectional study utilizing the pre-hospital electronic Patient Medical Record system over 8 years in a mixed urban-rural area with a population of 1.2 million people. All prehospital medical records for ambulance missions dispatched for childbirths were manually examined. The study period spanned from January 2016 through June 2024. RESULTS: Out of 1,137,222 ambulance dispatches, 3543 cases (0.31%) involved a dispatch code related to childbirth. Many labor incidents were considered uneventful by the prehospital personnel. Of all ambulance runs related to childbirth, we identified 310 births in the Region of Southern Denmark in which ambulance staff assisted with complicated out-of-hospital deliveries. An additional 10 labor cases were regarded as sufficiently complex that obstetric manoeuvres aimed at delaying the delivery of the baby were performed prehospitally, while the mother was rushed to hospital for delivery. CONCLUSION: Births outside the hospital while the mother is in the care of the EMS were rare, occurring in 0.03% of all ambulance missions. Most of the births took place without complications for both mother and neonate. In four cases, however, the neonate was in cardiac arrest and required resuscitation. Given the limited exposure to prehospital births, we suggest incorporating training in handling these rare events into routine training for prehospital caregivers. EDITORIAL COMMENT: This cohort study presents obstetrical cases that have been managed first in the prehospital setting by ambulance personnel. Obstetrical outcomes related to this are presented, and there are considerations for what can be desirable as preparation and competencies for ambulance responses for these types of cases.
European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic SurgeryRuggero De Paulis, Giulio Folino, Alain Berrebi, Filip P A Casselman, Rui J Cerqueira, Alessandro Della Corte, Laurent De Kerchove, Yves d'Udekem, Alberto Fort…
Aortic valve repair is a complex and evolving field that requires multidisciplinary teamwork among heart specialists to diagnose and treat various causes and mechanisms of aortic regurgitation and proximal thoracic aortic aneurysms across all age groups. From a surgical standpoint, aortic valve repair includes all procedures aimed at restoring or maintaining native valve function. This European Association for Cardio-Thoracic Surgery (EACTS) Expert Consensus Document critically evaluates the current evidence on the common causes and mechanisms of aortic regurgitation, the use of echocardiographic parameters in patient selection, intraoperative assessment, and prognostic evaluation as well as the surgical techniques in practice. It outlines fundamental surgical principles shared across different repair methods and provides consensus statements to guide clinical practice. These statements are intended for a broad audience, including paediatric and adult cardiologists, echocardiographers, cardiovascular imaging specialists, and cardiac surgeons. Where high-quality evidence is unavailable, the guidance relies on the collective experience and expert judgement of the multidisciplinary author team. This document presents the official stance of the EACTS and aims to promote standardized, evidence-based decision-making in the management of patients undergoing aortic valve repair.
Interdisciplinary cardiovascular and thoracic surgeryThomas Syburra, Edward D Nicol, Carlos A Mestres, Milos Matkovic, Joanna d'Arcy, Denis Bron, Filip Casselman, Martin Czerny, Stephen Fremes, Norbert Guettler, …
Civil aviation medicine and cardiothoracic surgery intersect at a point where individual clinical outcomes and public safety are tightly coupled. Professional aircrew and other safety-critical aviation personnel must meet legally defined medical standards that are designed around an engineering approach to risk, including a low annual tolerance for sudden incapacitation, whereas passengers and cabin crew are exposed to the physiological constraints of the flight environment (hypobaric hypoxia, gas expansion, limited access to medical care, immobility, and circadian rhythm disruption). Yet, the evidence base guiding fitness-to-fly decisions after cardiothoracic procedures, treatment of aortic disease, and contemporary transcatheter interventions remains sparse and unevenly distributed across procedures, licensing classes, and operational contexts. This European Association of Cardio-Thoracic Surgery Expert Consensus Document appraises the available literature and synthesizes multidisciplinary expert knowledge to provide pragmatic, safety-oriented guidance for aeromedical examiners, treating clinicians, regulators, employers, and affected individuals. Focusing on civil aviation, it addresses return-to-duty considerations for professional aircrew and postoperative air-travel decisions for passengers. Key clinical and occupational determinants include procedure choice and durability, residual ischaemia or haemodynamic burden, arrhythmia risk, anticoagulation and device-related considerations, rehabilitation and surveillance testing, and, where appropriate, the role of operational restrictions such as multicrew limitations. Finally, it identifies key knowledge gaps and research priorities to support more consistent, evidence-based decision-making in this high-stakes setting.
BMJ case reportsNisha Rajan, Kiruthika Asokan, Venkatesh Karthik, Pediredla Karunakar
Moyamoya disease (MMD) is a progressive cerebrovascular disorder characterised by distal internal carotid artery (ICA) stenosis and compensatory collateral vessel formation. Psychiatric manifestations are rare, particularly in children. We report the case of an early adolescent with previously diagnosed MMD who developed acute-onset psychosis following a febrile illness associated with headache and vomiting. Symptoms included auditory hallucinations, persecutory delusions, emotional lability and Fregoli syndrome, a rare condition in which different strangers are believed to be a familiar person in disguise. Neuroimaging revealed bilateral ICA narrowing with extensive collateral networks. Infectious, autoimmune and metabolic evaluations were unremarkable. The patient underwent superior temporal artery-middle cerebral artery bypass with encephalo-duro-arterio-myo-synangiosis. Psychotic symptoms resolved after surgery, and he returned to baseline functioning within 1 month. This case highlights the importance of considering cerebrovascular aetiologies in abrupt-onset childhood psychosis, even in the absence of major neurological deficits.
Immunologic researchJosé Roberto Mendes Pegler, Débora Linhares Rodrigues, Renata de Almeida Bordim, Patricia Palmeira, Andréia Rangel-Santos, Isabela de Jesus Silva Avelar, Ana C…
Early thymectomy during congenital heart disease repair may impair thymic output, but long-term effects across age groups remain incompletely defined. We evaluated T-cell receptor excision circles (TRECs) and a comprehensive panel of lymphocyte subsets in 20 adults (mean age, 29.6 years) and 16 children (mean age, 10.7 years) who underwent thymectomy within the first year of life, and in age- and sex-matched controls. TRECs were lower in patients than controls in adults (34 ± 25 vs. 79 ± 45 copies/μL; FDR-adjusted p = 0.001) and children (9 [7-15] vs. 95 [65-160] copies/μL; FDR-adjusted p < 0.0001). Total lymphocyte and T-cell counts were reduced in both age groups, mainly due to lower naïve CD4⁺ and CD8⁺ T cells, including recent thymic emigrants. Most non-naïve subsets did not differ significantly from controls. Regulatory T-cell (Treg) alterations differed by age: children showed broad reductions, whereas adults showed selective naïve Treg reduction. Early thymectomy is associated with persistent thymic output impairment, including in adults approximately three decades after surgery, and with age-specific Treg alterations.
The American journal of case reportsFaisal J Alqahtani
BACKGROUND Ovarian torsion is an uncommon but time-sensitive gynecologic emergency in children. Diagnosis may be challenging because symptoms can be nonspecific and Doppler ultrasonography may demonstrate residual ovarian blood flow. Although oophoropexy may be performed to reduce recurrence, torsion can recur after fixation. CASE REPORT A 9-year-old prepubertal girl with a history of left ovarian torsion treated by laparoscopic detorsion and oophoropexy presented with 2 days of severe intermittent left-sided abdominal pain and 1 day of vomiting. She was afebrile and hemodynamically stable, without peritoneal signs. Transabdominal ultrasonography demonstrated an enlarged left ovary measuring 41×37×32 mm (approximately 27 mL), with diminished vascularity and faint internal flow on color and power Doppler imaging. Urgent laparoscopy confirmed recurrent left ovarian torsion of approximately 360°. The ovary was viable, and no ovarian cyst or adnexal mass was identified. Detorsion and utero-ovarian ligament plication were performed, preserving the ovary. At 8-week follow-up, ultrasonography demonstrated normalization of the left ovarian size to 16×14×24 mm (approximately 2.7 mL), with no adnexal lesion or pelvic free fluid. CONCLUSIONS Recurrent ovarian torsion can occur despite previous oophoropexy. Residual Doppler flow should not exclude torsion when clinical suspicion remains high. Prompt surgical evaluation can permit detorsion and ovarian preservation.
Child's nervous system : ChNS : official journal of the International Society for Pediatric NeurosurgeryRebecca White, April Gormon, Duc T Nguyen, Travis Herzog, Daniel Curry
PURPOSE: This study's objective was to investigate barometric pressure changes that are most strongly associated with symptom exacerbations in shunted hydrocephalus patients. To achieve this, we conducted in-depth analysis of hospital encounter data and examined their correlation with variations in sea level pressure (SLP). Ultimately, our findings may inform hydrocephalus management strategies by identifying potential environmental triggers, reducing unnecessary hospital visits, and guiding the future design of CSF diversion technologies that are more responsive to external conditions. METHODS: We conducted a retrospective analysis of the daily hydrocephalus encounters census of a tertiary children's hospital and compared it with weather parameters, including fluctuations in SLP as measured in 8-h intervals, maximum daily rate of change, daily minimum SLP, and daily mean SLP. Data was analyzed in a univariate and multivariable Poisson generalized linear models, and census was compared to weather fluctuations on the day of encounter, the following day, and 2 days afterward. RESULTS: In univariable analysis, hydrocephalus encounters were associated with the daily maximum rate of change, daily maximum SLP, daily minimum SLP, and daily mean SLP. These associations maintained 1 day and 2 days after the hospital encounters. In multivariate analysis, the daily maximum SLP and daily minimum SLP were associated with patient encounters both 1 and 2 days after the analyzed census date. Associations were also observed between daily minimal SLP, daily maximum SLP, and maximum daily 8-h SLP. CONCLUSION: This study documents an association between fluctuations in atmospheric pressure and daily hydrocephalus census at a single tertiary medical center. Weather-related influences should be considered when evaluating shunt malfunction in patients with chronic shunted hydrocephalus.
European journal of orthopaedic surgery & traumatology : orthopedie traumatologieAlexa Zimmermann, Johanna Thiele, Christian Prangenberg, Jonas Roos, Davide Cucchi, Sebastian Scheidt, Kristian Welle, Lisa Fiona Roder
PURPOSE: Kirschner wires (K-wires) are widely used for fracture fixation in paediatric trauma surgery. Although infectious complications are uncommon, they remain clinically relevant. Subcutaneous burial of K-wires has been proposed to reduce the risk of infection, but robust data from large paediatric cohorts remain limited. This study aimed to evaluate infectious and non-infectious complications following routine use of buried K-wires in children. METHODS: In this retrospective cohort study, paediatric patients (≤ 18 years) who underwent fracture fixation with routinely buried K-wires at a Level I trauma centre between January 2005 and November 2025 were included. Only patients with complete follow-up, including radiographic assessment and wire removal at the study institution, were analysed. The primary outcome was postoperative infection related to K-wire fixation. Secondary outcomes included non-infectious wound complications, reoperations, and fracture healing. RESULTS: A total of 634 patients with a mean age of 10.2 ± 4.2 years were included; 96.8% sustained upper-extremity fractures. One superficial infection occurred (0.16%; 95% CI 0.00-0.88%), with no cases of deep infection or osteomyelitis. Secondary skin perforation without infection was observed in 8.36%, and minor wound-healing disturbances in 0.32%. No infection-related reoperations were required, and all fractures healed radiographically. CONCLUSIONS: Routine subcutaneous burial of K-wires in paediatric fracture fixation was associated with an exceptionally low rate of infectious complications and no cases of osteomyelitis in this large retrospective cohort. However, secondary skin perforation occurred in 8.36% of patients, and planned wire removal under anaesthesia represents an additional procedural burden. Given the predominance of upper-extremity fractures and the absence of an exposed-wire control group, these findings should be interpreted within the studied population and do not establish superiority over exposed K-wires.
BMJ openSruthi Pandipati Thomas, Sharon Ramey, Mark Conaway, Stephen Kralik, Rachel Arp, Jeffrey S Raskin, Nisha Gadgil, Neena Marupudi, Darcy Fehlings
INTRODUCTION: Dystonia, a form of elevated muscle tone in CP, significantly impairs function and quality of life. Multiple neurosurgical interventions are used to reduce dystonia, including intrathecal baclofen (ITB) and ventral dorsal rhizotomy (VDR). ITB is used most often for moderate to severe generalised dystonia in CP; however, the evidence supporting its effectiveness is weak due to patient variability and study limitations. Further, VDR is becoming more prevalent but there is minimal data available on its outcomes. This proposal aims to evaluate ITB and VDR's ability to reduce quantitative and functionally impactful dystonia as well as reduce spasticity, improve motor function, improve mood and improve quality of life. Secondary analysis will focus on identifying child characteristics of a positive response to ITB and VDR to allow for better patient selection. METHODS AND ANALYSIS: We will conduct a prospective observational study of 52 children with CP and moderate-severe dystonia (Barry Albright Dystonia Scale (BADS) greater than 14) who receive ITB (n=35) and VDR (n=17). Children will be enrolled at three sites in the USA: Texas Children's Hospital (Houston), Lurie Children's Hospital (Chicago) and C.S. Mott Children's Hospital (Ann Arbor). Changes will be measured in dystonia, spasticity, gross and fine motor function and multiple patient-reported outcomes related to quality of life, participation, depression, anxiety and pain. Children enrolled in the study will undergo detailed characterisation of brain malformation and injury patterns. The primary analysis will use repeated measures models to estimate changes from baseline in BADS scores at 3, 6 and 12 months. Secondary analyses will apply the same strategies to the other outcome measures. Subgroup analyses will investigate unique characteristics of children with a positive response to either intervention. A multidimensional or composite measure will also be created. ETHICS AND DISSEMINATION: Primary ethics approval was provided by the Baylor College of Medicine Institutional Review Board (H-54449). Primary caregivers of all participants were consented for the study. Participants with capacity were asked to assent. Results of the study will be disseminated via peer-reviewed presentations at scientific conferences and open access publication. TRIAL REGISTRATION NUMBER: NCT06606574.
BACKGROUND: The ESPGHAN biopsy-free diagnostic algorithm for pediatric celiac disease (CeD) requires serum IgA anti-tissue transglutaminase antibody (anti-tTG IgA) concentrations ≥10 times the upper limit of normal (ULN) in the first blood sample, together with positive IgA endomysial antibodies (EMA) in a second blood sample. However, the transferability of the 10×ULN anti-tTG IgA threshold across different analytical platforms remains uncertain owing to the lack of assay harmonization. METHODS: Eighty-four children (6-10 years) who underwent duodenal biopsy during the diagnostic work-up for suspected CeD were retrospectively evaluated. Histology was classified according to the Marsh-Oberhuber criteria (65 Marsh-Oberhuber 3, 12 Marsh-Oberhuber 2, and 7 Marsh-Oberhuber 1). Anti-tTG IgA antibodies were measured using one FEIA assay (EliA Celikey IgA) and two CLIA platforms (QUANTA Flash® and Maglumi®). EMA were assessed by indirect immunofluorescence on distal monkey esophagus. ROC analysis evaluated discrimination of mucosal atrophy (Marsh-Oberhuber 3) from non-atrophic lesions (Marsh-Oberhuber 1-2). RESULTS: Anti-tTG IgA concentrations correlated significantly with histological severity for all assays (Spearman's ρ=0.670-0.736; p<0.0001). AUCs ranged from 0.961 to 0.992. At 10×ULN, QUANTA Flash® showed 100% sensitivity and 47.4% specificity, whereas Maglumi® and EliA Celikey IgA showed 100% specificity with sensitivities of 70.8% and 86.2%, respectively. For QUANTA Flash®, 42×ULN was required to achieve 100% specificity. In analyses targeting PPV ≥95%, the corresponding thresholds were 7.54×ULN for Maglumi®, 28.55×ULN for QUANTA Flash®, and 6.10×ULN for EliA Celikey IgA. CONCLUSIONS: In this selected anti-tTG IgA-positive cohort, the quantitative relationship between anti-tTG IgA and mucosal atrophy differed across automated assays. Expression of results as multiples of the ULN did not fully compensate for inter-assay analytical variability. These findings support assay-specific verification of decision limits, but the retrospective design and selection based on QUANTA Flash positivity limit generalization of the diagnostic performance estimates to unselected children undergoing evaluation for CeD.
Current osteoporosis reportsAki S Puryear, Laura E Bess, Linda A DiMeglio
PURPOSE OF REVIEW: Pediatric osteoporosis is a heterogeneous group of primary and secondary disorders that creates challenges for orthopaedic surgeons. This review summarizes evidence on conditions commonly encountered in pediatric orthopedic practice, medical management strategies, and perioperative care for children with bone fragility. RECENT FINDINGS: Diagnostic approaches have been evolving toward integrating fracture characteristics, skeletal risk factors, and bone mineral density (BMD). A single low-trauma vertebral compression fracture is now sufficient for diagnosis of osteoporosis in children regardless of BMD. Intravenous bisphosphonates remain first-line therapy, with recent meta-analyses confirming consistent BMD gains and, observationally, a reduction in fracture risk. Bisphosphonates are generally given to children who have sustained low-trauma vertebral fractures and considered for other children with recurrent long bone fractures with low BMD. Early intervention with bisphosphonates may also be warranted for children with high-risk diseases, such as Duchenne's Muscular Dystrophy (DMD). Biologic agents are emerging in clinical trials and select specialized use, but further research is needed to understand their role in pediatric osteoporosis. Denosumab carries a substantial risk for rebound hypercalcemia in children with osteogenesis imperfecta (OI), limiting its use to selected indications. Preliminary reporting from a large trial of anti-sclerostin therapy in children with osteogenesis imperfecta suggests significant BMD increases while fracture-reduction endpoints were not met, underscoring the limitations of BMD as a surrogate in pediatric bone fragility trials. Providing optimal care for this unique group of patients requires multidisciplinary coordination amongst orthopaedic surgeons, endocrinologists, geneticists, and rehabilitation specialists. Evidence-based management includes preoperative bone health evaluation, procedure-specific modifications in fragile bone, nutritional optimization, and coordinated bisphosphonate timing.
INTRODUCTION AND AIMS: Traditional bone setters (TBS)-lay practitioners who manage fractures without formal medical qualification-treat an estimated 10%-85% of fracture patients in low- and middle-income countries (LMICs). Children are disproportionately vulnerable due to physeal immaturity and lower diastolic blood pressure thresholds that increase susceptibility to compartment syndrome at lower absolute intracompartmental pressures than in adults. No prior systematic review has restricted analysis exclusively to the paediatric population or generated pooled meta-analytic estimates of complication rates. This protocol describes a systematic review and meta-analysis to determine the pooled incidence of paediatric TBS-related complications across LMICs. METHODS AND ANALYSIS: Nine databases will be searched (PubMed/MEDLINE, Embase, Cochrane CENTRAL, African Index Medicus, Global Health/CABI, Scopus, LILACS, WHO ICTRP and the COSECSA Archive) supplemented by grey literature sources, using three complementary PubMed search strings and 15 verified vernacular synonyms for TBS practitioners (including Wegesha, Onisegun, Rebouteux, Huesero and Vaidya). Database searching commenced in April 2026 and is currently in progress. No screening, data extraction or analysis has been initiated. Eligibility requirements: children aged <18 years; confirmed TBS exposure; fracture of any long bone or periarticular region; LMIC setting (World Bank 2024 classification); primary data collected from January 2010 to February 2026; and quantitative reporting of at least one primary outcome. Studies reporting mixed-age populations are eligible only if a paediatric subgroup (0-17 years) is extractable in absolute numbers. Title and abstract screening will be performed independently by YeMK and YoMK; discrepancies will be resolved by HB.Primary outcomes are: major limb amputation, bonesetter's gangrene, Volkmann's Ischaemic Contracture (VIC) and sepsis-related mortality. Risk of bias will be assessed using the Newcastle-Ottawa Scale (NOS), MinCir scale and Mixed Methods Appraisal Tool (MMAT). Pooled complication proportions will be calculated using the Freeman-Tukey double arcsine transformation under a random-effects model (DerSimonian-Laird and REML). Lag time from injury to hospital presentation will be examined as a continuous moderator in meta-regression, and the certainty of evidence will be assessed using Grading of Recommendations Assessment, Development and Evaluation. ETHICS AND DISSEMINATION: No primary data are collected; formal ethics approval is not required. Findings will be disseminated through peer-reviewed publication and presentation at relevant orthopaedic and global surgery conferences. If pooled paediatric amputation rates exceed 30% in late-presenting patients, findings will additionally be submitted as a formal evidence brief to the WHO Global Surgery Programme, the College of Surgeons of East, Central and Southern Africa Global Surgery Commission, and national orthopaedic associations in Nigeria, Ghana, Ethiopia and Pakistan. This protocol is registered with PROSPERO (CRD420261349595). PROSPERO REGISTRATION NUMBER: CRD420261349595.
Child's nervous system : ChNS : official journal of the International Society for Pediatric NeurosurgeryAnaïs Chivet, Julie Bonheur, Diane Doummar, Sarah Ferrand-Sorbets, Laurent Goetz, Georg Dorfmuller
Hamartoma of the floor of fourth ventricle (HFFV) is an extremely rare entity known to be causing focal movement disorders in children. According to the few reported cases in literature, the only effective treatment is disconnection or resection of the lesion which can be very challenging in this anatomic area. We report a 15-year-old child with a fourth ventricle floor's limited nonexophytic hamartoma revealed by brief paroxysmal movement disorder episodes since the age of 5 months, without EEG correlate. He was treated for the first time by safe surgical electrophysiology-guided resection, with promising results on epileptic disease. This positive result opens interesting prospects for improving the surgical management of other lesions affecting this anatomical region, while limiting postoperative neurological deficits.
European journal of pediatricsShintaro Terashita, Akiko Tsuchida, Hidekuni Inadera, Chihaya Imai, Eiji Yoshioka, Japan Environment and Children’s Study Group
Cesarean delivery (CD) has been suggested as a potential risk factor for childhood overweight and obesity; however, evidence beyond early childhood remains inconsistent. We examined the association between CD and childhood overweight at age 6 years and explored early life body mass index patterns including adiposity rebound (AR), in a large Japanese birth cohort. We analyzed 64,281 singleton mother-child pairs from the Japan Environment and Children's Study. Overweight at age 6 years was defined according to the International Obesity Task Force criteria. Logistic regression models were used to estimate adjusted odds ratios (aORs). Changes in overweight status between ages 3 and 6 years were examined to distinguish incident overweight from persistent overweight, and AR timing was evaluated. CD accounted for 18.5% of the births. Overweight at age 6 years was more frequent among children born by CD than among those born by vaginal delivery (10.7% vs. 8.8%). After adjusting for the prespecified covariates, CD was associated with higher odds of overweight at age 6 years (aOR 1.09, 95% confidence interval 1.02-1.16). In sex-stratified analyses, CD was associated with incident overweight between ages 3 and 6 years among females but not among males; however, the interaction between delivery mode and sex was not statistically significant. CD was not significantly associated with earlier AR.Conclusion: In this large Japanese cohort, CD was modestly associated with childhood overweight at age 6 years.
BMJ case reportsJanvi Dave, Bhavna H Dave, Seema Dinesh Bargale, Vaishnavi Patil
Children with intellectual disability often experience difficulties in accessing routine dental care because behavioural limitations and associated medical conditions make treatment challenging. Untreated dental infections in such patients may pose significant health risks, particularly when systemic conditions are present. A child in middle childhood with moderate intellectual disability presented with pain and progressive swelling in the right posterior mandibular region. The patient was non-verbal and had underlying cardiac dysfunction and encephalopathy, which further complicated clinical management. Clinical and radiographic examination revealed a grossly carious mandibular first permanent molar associated with a periapical cyst, along with severe ankyloglossia. Due to limited cooperation and medical complexity, comprehensive dental management was performed under general anaesthesia, including extraction, cyst enucleation, diode laser-assisted frenectomy and preventive care. Recovery was uneventful, and the patient was referred for speech therapy. This case highlights the importance of coordinated multidisciplinary management for medically complex children requiring comprehensive dental rehabilitation.
Journal of robotic surgeryCiro Esposito, Anna Gargiulo, Claudia Di Mento, Marco Castagnetti, Vincenzo Coppola, Francesco Tedesco, Roberta Guglielmini, Maria Luisa Pirone, Chiara Caporal…
Robotic-assisted surgery offers significant technical advantages in pediatric procedures but presents complex, unresolved medico-legal challenges regarding liability. We retrospectively reviewed our ten-year experience comprising 205 pediatric robotic procedures. Technical issues related to the robot were encountered in 7 cases (3.4%). These included joystick latency (3/7), coagulation/cutting pedal malfunctions (2/7), and accidental power cable disconnections causing system shutdowns (2/7). A literature review was conducted to assess the current international state of the art regarding robotic medical liability. Although the technical events at our center did not result in clinical complications, they highlighted significant potential risks. The literature shows that specific guidelines governing human, product, and algorithm-related liability are still lacking. Robotic surgery shifts the focus from an exclusively fault-based liability of the surgeon to a network-based system involving multiple stakeholders: the surgeon, the healthcare institution, and the manufacturer. Determining whether an adverse event stems from human error, algorithmic malfunction, or a combination of both remains a critical challenge. Unlike open or laparoscopic surgery where liability is primarily attributed to the operator, robotic surgery involves a complex interaction between the surgeon's console inputs and the manufacturer's technology. Consequently, from a medico-legal standpoint, responsibility for complications should be shared among the surgeon, the hospital, and the device manufacturer.
European journal of endocrinologyPilar Pérez Reyes, Ángel Rebollo Román, María Rosa Alhambra Expósito, José Carlos Padillo Cuenca, María Del Carmen Serrano Laguna, Paloma Moreno Moreno, Juan M…
Hypoparathyroidism is the most common complication after total thyroidectomy and may be particularly severe in pediatric Graves' disease. We report the case of a 14-year-old boy with Graves' disease who developed profound postoperative hypocalcemia after total thyroidectomy, with serum calcium 5.9 mg/dL, ionized calcium 0.83 mmol/L, and undetectable parathyroid hormone. Despite intravenous and high-dose oral calcium plus calcitriol, biochemical control remained poor, with persistent hypocalcemia, hyperphosphatemia, and ongoing treatment requirements after 16 days. Off-label subcutaneous palopegteriparatide, a long-acting PTH(1-34) prodrug, was started after multidisciplinary assessment and informed consent from the family. Serum calcium improved within 48 h, intravenous calcium was stopped after 5 days, and calcitriol, sevelamer, and calcium acetate were discontinued within 11 days. Treatment was well tolerated. This case suggests that early PTH replacement may be a useful rescue strategy in selected children with severe refractory post-thyroidectomy hypocalcemia.
Orphanet journal of rare diseasesLucia Laugwitz, Pascal Martin, Nils Janzen, Sebastian Hegert, Thomas Neiße, Madeleine Wacker, Jan Kern, Nadja Kaiser, Christiane Kehrer, Annina-Clarissa Jaeger…
BACKGROUND: Metachromatic leukodystrophy (MLD) is a rapidly progressive leukodystrophy that leads to severe disability and early death if untreated. Autologous hematopoietic stem and progenitor cell gene therapy (HSPC-GT, atidarsagene autotemcel, arsa-cel) for early onset subtypes and allogenic hematopoietic stem cell transplantation (HSCT) for late onset disease substantially alters disease progression for early onset disease when administered before symptom onset, creating a strong rationale for newborn screening (NBS). At the same time, NBS technique for MLD in dried blood spots has recently been demonstrated to be robust and highly accurate. The aim was to give real-world results from the world's first NBS pilots for clinical management and treatment of identified children. METHODS: Between September 2021 and July 2025, 359,282 newborns underwent NBS for MLD in two different laboratories in Germany and Austria using a three-tier algorithm integrating sulfatide quantification, arylsulfatase A (ARSA) activity measurement, and ARSA sequencing. Screen-positive infants underwent a predefined care pathway including standardized confirmatory diagnostics, genotype-based and biochemical prediction of disease onset, clinical assessment and management guiding early treatment and surveillance at the qualified treatment center (QTC) in Tübingen. RESULTS: Nine newborns screened positive and all were confirmed to have MLD (detection rate approximately 1 per 40,000). Based on genotype and leukocyte ARSA enzyme activity, disease onset prediction was possible in all of them. Seven infants were classified as having pre-symptomatic early-onset MLD and were referred for HSPC-GT. All treated infants showed preserved neurological function up to 30 months after treatment (median 18.5 months). Two infants predicted to develop late-onset MLD entered structured surveillance for treatment with HSCT and have remained clinically asymptomatic for CNS signs of disease. No false-positive or known false-negative results were observed. CONCLUSION: These results from our pilot programs demonstrate that NBS enables reliable early identification of MLD and support streamlined care pathways leading to timely intervention. Importantly, this study provides real-world evidence illustrating that NBS for MLD can enable timely, pre-symptomatic treatment and structured surveillance within standard national healthcare systems. These findings further substantiate the value of NBS for MLD at a critical moment as several countries consider national implementation of MLD screening.
Child's nervous system : ChNS : official journal of the International Society for Pediatric NeurosurgeryLeonardo Di Cosmo, Christopher Peter Imbrogno, Jad El Choueiri, Arthur Nantes, Joshua Vishal Sahgal, Francesca Totis, Ismail Zaed, Noor Ul Owase Jeelani
BACKGROUND: Ventriculoperitoneal (VP) shunting is the most common long-term treatment for pediatric hydrocephalus; however, early shunt failure remains a significant cause of morbidity and economic burden. Although individual studies have investigated risk factors for early failure, findings are fragmented and inconsistent. This meta-analysis evaluates the prevalence, predictors, and causes of 30-day VP shunt failure in pediatric patients. METHODS: Following PRISMA guidelines, databases were searched through November 2025 for studies reporting 30-day VP shunt failure in pediatric cohorts. Randomized controlled trials, prospective, and retrospective studies were included. Random-effects models were used, with risk ratios (RR) calculated for dichotomous outcomes and pooled prevalence estimated using generalized linear mixed models. RESULTS: Our analysis included 11,596 pediatric patients, with one randomized trial and 8 observational studies. The pooled prevalence of 30-day shunt failure was 13.8% (95% CI 11.2-16.8), with significantly lower rates following primary compared with secondary shunt insertion (12.6% vs 18.0%). A separate comparative analysis confirmed this reduced risk of early failure after primary insertion (RR 0.58, 95% CI 0.49-0.69; p < 0.001). When pooled female sex was associated with decreased risk of early failure, while no hydrocephalus etiology demonstrated a significant effect. Poor ventricular catheter placement was the only modifiable predictor associated with increased risk of early failure from the pooled analyses (RR 1.64, 95% CI 1.08-2.50; p = 0.02); however, this association was based on only two studies and lost significance in leave-one-out analyses. Cause-specific failure mechanisms were poorly reported across studies. Among studies reporting these outcomes, proximal catheter obstruction and malposition were the most frequently reported mechanisms of early shunt failure, followed by shunt infection. Several additional predictors were significant in individual studies and were qualitatively reported, but could not be pooled due to heterogeneous reporting. CONCLUSION: VP shunt failure affects approximately one in eight pediatric patients in the first 30 days following placement, and occurs significantly more frequently following secondary shunt insertion. Among the pooled predictors, ventricular catheter placement quality represents the most significant modifiable factor associated with early shunt survival, although this finding was based on limited data. Several predictors showed estimates suggestive of increased risk; however, prospective studies with standardized reporting are necessary to define the size of these associations. SYSTEMATIC REVIEW REGISTRATION: PROSPERO CRD420261305777.
Journal of robotic surgeryNicolas Berte, Clémence Havret, Julie Lienard, Adrien Montazeaud, Samy Benbetka, Jean-Pierre Henry, Jacques Hubert, Charles Mazeaud, Nguyen Tran
Structured robotic surgery curricula increasingly integrate simulation, dry lab, microsurgery and high-fidelity wet lab training, yet few studies have comprehensively evaluate how performances across these educational components relate to final proficiency in a multimodal curriculum. We aimed to evaluate the WETPROF inter-university robotic surgery diploma curriculum by analyzing relationships between theoretical knowledge, simulator performance, practical training modules and final wet lab performance, while assessing the reliability and convergent validity of the assessment instruments used throughout the program. We retrospectively analyzed prospectively collected data from 131 surgeons enrolled in the WETPROF curriculum between 2023 and 2025. Training included theoretical assessment, DvTrainer virtual reality simulation, microsurgery, GEARS-rated FRS Dome exercises and a final porcine wet lab evaluated using GEARS, ARCS and NOTSS. Internal consistency was assessed using Cronbach's alpha, convergent validity with Spearman correlations, group comparisons using non-parametric tests, and multivariable regression models were used to identify predictors of simulator progression and final wet lab performance. The wet lab assessment instruments demonstrated excellent internal consistency (Cronbach's α=0.975) and very strong convergent validity (ρ=0.85-0.89; all p<0.001). Baseline theoretical knowledge was not associated with either simulator or wet lab performance. Prior robotic experience significantly improved simulator progression, particularly DvTrainer performance (effect size r=0.525), whereas its influence on final wet lab performance was limited. DvTrainer curriculum score was independently predicted by GEARS FRS Dome, microsurgery performance and GEARS Wet Lab (R²=0.480). In contrast, wet lab performance was only weakly explained by demographic characteristics (R²=0.156), with DvTrainer score emerging as the only independent external predictor (β=+0.220, p=0.016). These findings indicate that simulator progression and wet lab performance represent complementary dimensions of surgical competence. The WETPROF curriculum provides a coherent multimodal framework for robotic surgery training. The excellent reliability and convergent validity of the assessment instruments support their use within competency-based education. Although theoretical knowledge and prior experience contribute little to final wet lab performance, progression through the simulation curriculum is associated with better technical performance in a high-fidelity environment, supporting the educational value of a progressive simulation-based pathway before clinical practice.
MedicineYoung-Keun Lee, Ji Woong Ho, Jin Young Kim
Pediatric scaphoid nonunion is uncommon. Immobilization is initially attempted; however, it causes delays in both intervention and healing. Therefore, surgical intervention has gained broader acceptance and has been implemented in recent years. Among these, arthroscopic management has emerged as a contemporary technique. This study evaluated the clinical outcomes of pediatric scaphoid nonunion treated with arthroscopic-assisted bone grafting and Kirschner (K) wire fixation. Six pediatric patients with scaphoid nonunion treated with arthroscopic bone grafting and K-wire fixation between November 2009 and August 2021 were retrospectively reviewed. All included patients were male. Fracture locations included three distal thirds, two mid-thirds, and one proximal third. The mean time from injury to intervention was 7.83 months. Three patients underwent autogenous iliac bone grafting, and 3 received bone substitute grafts. The mean follow-up duration was 40.67 months. Bone union was evaluated using serial plain radiographs and computed tomography. Functional outcomes were assessed by comparing range of motion (ROM), grip strength, modified Mayo wrist score, quick disabilities of the arm, shoulder, and hand (DASH) score, and visual analog scale (VAS) before surgery and at the latest follow-up. Radiological assessment was performed by comparing the scapholunate (SL) and radiolunate (RL) angles preoperatively and at the last follow-up. Complete union was achieved in all patients. The mean time to radiological union was 9.83 weeks. Functional measures, including grip strength (% of normal side, 67→92), ROM (% of normal side, 77→94), average VAS score (4.17→0.83), quick DASH score (40.15→7.58), and modified Mayo wrist score (53.33→80.00), demonstrated significant improvement. Radiological analysis also demonstrated a significant improvement in the SL angle (59.83→50.92). Arthroscopic-assisted bone grafting with percutaneous K-wire fixation is an effective treatment modality for pediatric scaphoid nonunions. In addition, bone substitute grafts may serve as viable alternatives to autogenous bone grafts in pediatrics.
Indian journal of ophthalmologyAnjali Khadia, Swati Upadhyaya, G Bouvanesvary, P J Sruthi, Deivasenathipathi Gokulvasanth, Bhavya Annadevula, Sivagami Nachiappan, Fredrick Mouttapa, Kannusam…
Here, we review and summarize the evolution of surgical techniques and outcomes of minimally invasive, knotless (flanged) approaches for the repair of iridodialysis. A thorough literature search was performed using PubMed, Medline, Cochrane Library, and Embase databases to identify studies related to iridodialysis repair. Iridodialysis repair, flanged polypropylene suture, knotless iridodialysis repair, and ab-interno and ab-externo procedures were the keywords used. Their bibliographies provided further references. Articles on surgical repair of iridodialysis that were published before April 2025 were included. Five relevant studies were found and reviewed in depth. The conventional methods, though effective, were technically demanding, time-consuming, and associated with complications such as suture slippage, iris prolapse, conjunctival erosion, and postoperative astigmatism. Recent advancements introduced minimally invasive and knotless suture techniques, notably those using flanged polypropylene sutures. Both ab-interno and ab-externo approaches have been described by various authors including Kausaka et al ., Rosenberg et al ., Peng et al ., Ramakrishnan et al ., and Khadia et al . These techniques employ simple ophthalmic instruments, minimize intraocular manipulation, and eliminate conjunctival dissection and suture knots. These methods ensure iris reattachment, shorter operative time, improved cosmetic outcomes, and a shorter learning curve for surgeons. Minimal invasive knotless iridodialysis techniques represent a significant advancement in anterior segment reconstruction surgeries. They offer a minimally invasive, safe, and efficient alternative to traditional suturing methods, with reduced complication rates and enhanced patient comfort. Regular follow-up focusing on visual recovery, intraocular pressure, and gonioscopic evaluation remains essential to ensure long-term success.
Pediatric pulmonologyFabrizio Racca, Elena Conti, Giulia Racca, Luigi Barberis, David J Birnkrant
Duchenne muscular dystrophy (DMD) is an inherited neuromuscular disorder with multisystem involvement that causes progressive muscular weakness and cardiorespiratory dysfunction. The first comprehensive recommendations regarding anesthetic management are now almost 20 years old and, in the interval, DMD care has evolved. Patients with DMD are experiencing prolongation of survival and, with widespread use of assisted ventilation, cardiac function has become the main determinant of their survival. Newer procedures, such as advanced cardiac interventions, are now performed, in a population of older, medically fragile individuals. Regional anesthetic techniques have advanced, allowing more patients to avoid the risks of inhalational anesthesia, and newer techniques for reversing neuromuscular blockade and minimizing cardiorespiratory depression have become available. Additionally, new medications are being used that have anesthetic implications, including newer guideline-directed heart failure medications. Overall, it has become increasingly important for perioperative management to be addressed in a multidisciplinary way, with coordination between medical, surgical, and anesthetic clinicians. In this narrative review, we provide information on how DMD's multi-system involvement impacts perioperative assessment and management, and we advocate for a multidisciplinary approach to perioperative care.
Acta anaesthesiologica ScandinavicaSara Marija Abu-Bakr Christensen, Mathilde Fredensborg, Birgitte Brandstrup, Ann Merete Møller
BACKGROUND: Effective pain control is critical in pediatric gastrointestinal surgery to support recovery and improve postoperative outcomes. Regional anesthetic techniques have gained increasing interest, with the quadratus lumborum block (QLB) emerging as a promising option. As its use expands, this scoping review aimed to map the available evidence on QLB in pediatric gastrointestinal surgery. Specifically, it examined its application, gastrointestinal procedures involved, reported outcomes, and potential advantages and disadvantages. METHODS: This scoping review followed PRISMA-ScR guidelines and a published protocol with four predefined research questions. The PCC framework was used to define the review scope: Population, pediatric patients; Concept, QLB; Context, gastrointestinal surgery. A systematic search was performed in PubMed, Embase, and Cochrane CENTRAL. Studies investigating QLB in pediatric gastrointestinal surgery were included. Two reviewers independently conducted screening and data extraction. RESULTS: Twenty-nine studies were included. QLB was predominantly performed using the posterior approach (55%). The administered anesthetic dose varied from 0.3 to 1 mL/kg. No studies measured the plasma concentrations of local anesthetics or assessed dermatomal coverage. The block was applied across various gastrointestinal procedures, most frequently inguinal hernia repair (76%). Outcomes from different abdominal surgeries were often reported in aggregate (59%). Postoperative pain was the primary outcome, evaluated using six different measures, with variability in pain scales, analgesic protocols, and rescue analgesia thresholds. Pain scores were the most common measure, assessed most often using the Face, Legs, Activity, Cry, Consolability scale (76%). Few studies compared different QLB approaches (10%). QLB offered superior or comparable analgesia compared to other techniques. CONCLUSION: QLB appears effective for pediatric gastrointestinal surgery. However, the literature reveals considerable heterogeneity in outcome measures and study designs. Knowledge gaps remain regarding dose-related safety, dermatomal coverage, optimal dosing, and comparative efficacy of QLB approaches and gastrointestinal procedure-specific effectiveness. These findings may guide future research to optimize QLB use.
BACKGROUND: Transplantation of adult-sized kidneys into very small pediatric recipients can be limited by pronounced donor-recipient size mismatch. Although intraperitoneal placement is often considered, extraperitoneal implantation may reduce abdominal morbidity, provided that the graft can be accommodated safely. CASE PRESENTATION: We report transplantation of a left kidney from a 46-year-old deceased adult donor into a 15-month-old boy weighing 8.5 kg with congenital nephrotic syndrome and previous bilateral nephrectomy. The 14-cm graft had three arteries of comparable caliber. Because the right retroperitoneal space was insufficient to accommodate the graft, the upper pole, supplied by an independent polar artery, was resected ex vivo on the back table, reducing the graft volume by approximately one-third. The remnant kidney was implanted extraperitoneally with venous anastomosis to the inferior vena cava and two arterial anastomoses to the aorta. OUTCOME: Immediate graft function was excellent. Serum creatinine returned to normal within 24 h and surveillance biopsies at 1 month, 1, 3 , and 5 years showed preserved renal architecture. At 6-year follow-up, the child measured 116 cm, weighed 16 kg, had blood pressure of 90/60 mmHg, serum creatinine of 0.66 mg/dL, and a urine protein-creatinine ratio of 0.26 g/g while receiving mycophenolate monotherapy. CONCLUSION: In carefully selected circumstances, back-table reduction of a deceased adult donor kidney may be a technically feasible strategy to overcome extreme donor-recipient size mismatch in pediatric kidney transplantation without compromising medium-term graft structure or function.
INTRODUCTION: Screening and treatments for pediatric obstructive sleep apnea (pOSA) often use adult symptoms and conventional surgical approaches. This study tested the hypothesis that symptoms and treatment efficacy for OSA differ across age groups. METHODS: Single-center cohort study of 0 to 18-year-olds who were managed and underwent polysomnography (PSG) in a pediatric-dedicated sleep medicine center from 2020 to 2024. RESULTS: Of 491 children, 489 had pOSA, 57.7% were male, the median age was 6 years old (IQR1-3, 4-9), and 41% already had an adeno- or adenotonsillectomy prior to the PSG. Only 15.6% were obese, and 13.2% had an Epworth Sleepiness Score over 10. Snoring was not the most prevalent nocturnal symptom: restlessness (91.6%), chronic insomnia (86.1%), kicking legs (82.0%), and frequent awakening (78.4%) were all more common. Neurocognitive issues, present in 93.7%, were most commonly mood swings (86.5%) and ADHD (51.9%). Of the 270 without surgery before the PSG, 72.2% (n = 195) were referred, and 173 had surgery. Positive airway pressure (PAP) was prescribed for 240 (49.1%) patients, 176 (73%) of whom had enough CPAP usage to provide machine-estimated apnea-hypopnea index (AHI). After adenotonsillectomy, 27.0% had a post-operative AHI under 5 events per hour, and 73.1% had improvement in neurocognitive issues while that was 94.9% and 87.2% in those using PAP, respectively. After adjusting for age and AHI, PAP had threefold (aOR 3.85, 95%CI 1.39-10.67) higher odds of improving neurocognitive issues than surgery, p = 0.010. CONCLUSIONS: The majority of children with pOSA are not tired or obese. Insomnia and restless sleep are more common than snoring. Daytime neurocognitive issues affect almost all children with pOSA and improve with treatment, especially PAP.
PediatricsVasilis Grammeniatis, Andreas Eliades, Vasiliki Gketsi, Konstantinos Douros
Black pleural effusion (BPE) is exceptionally rare in children, with only 1 previously reported pediatric case linked to a pancreaticopleural fistula, while adult cases are typically associated with rupture of pancreatic pseudocysts, malignancy, fungal infection, or hemorrhage. A previously healthy, fully vaccinated 6-year-old boy presented with severe pneumonia, and thoracentesis/chest drainage revealed dark sanguineous, black fluid that tested positive by multiplex polymerase chain reaction for Streptococcus pneumoniae serotype 3, confirming empyema. He responded well to chest tube drainage, intrapleural fibrinolysis, and antibiotic therapy, and no underlying malignancy or pancreatic disease was identified. This case represents a rare instance of BPE caused by bacterial empyema in a child, expanding the pediatric differential diagnosis and underscoring that severe bacterial pneumonia-despite vaccination-can present with this unusual pleural fluid appearance.
Chronic stress (CS) acts as both an initiating and aggravating factor for depression and osteoporosis. Cumulative evidence indicates that Cathepsin K (Cat K), a cysteine protease, is functionally linked to chronic stress and exerts critical regulatory roles in bone metabolism. Nevertheless, the molecular mechanisms whereby CS promotes osteoporosis in the background of mood-related disturbances remain poorly defined. In the present work, we established a combined mouse model of osteoporosis and chronic stress. We evaluated anxiety- and depression-like behaviors, the expression profiles of Cat K, autophagy-, apoptosis-, and inflammation-associated proteins in bone tissue, as well as vascular senescence in experimental animals. Furthermore, we investigated the biological effects of serum collected from chronically-stressed mice on MC3T3-E1 osteoblast-like cells. Following 8-week chronic stress exposure, ovariectomized (OVX) mice developed prominent anxiety- and depression-like behaviors, accompanied by deteriorated osteoporosis and aortic senescence. Meanwhile, bone tissues exhibited elevated levels of Cat K, p62, pro-inflammatory mediators, and apoptosis-related proteins, whereas the autophagy marker LC3B/A was down-regulated. Administration of E64d, a cysteine protease inhibitor, markedly reversed these pathological alterations. In vitro culture revealed that serum from chronically-stressed mice suppressed the viability of MC3T3-E1 osteoblast-like cells. Pharmacological inhibition of Cat K restored autophagic activity, mitigated the overexpression of pro-inflammatory and pro-apoptotic mediators, and rescued MC3T3-E1 cells from stress-triggered injury. Collectively, our findings demonstrate that chronic stress induces anxiety- and depression-like phenotypes in OVX mice and accelerates bone loss, at least partially via Cat K activation and autophagy suppression.
Pediatric transplantationDavid Thomas, Erica Merlino, Chris Lamprecht, James Davis Cury, Kiran Upadhyay
BACKGROUND: Post transplant lymphoproliferative disease (PTLD) and infection remain the two most common complications of solid organ transplant and carry a risk of significant morbidity and mortality. PTLD is a highly variable disease with clinical signs and symptoms that are non-specific and require a high degree of suspicion to detect. The most common presenting symptoms of PTLD are malaise, fatigue, fever, and a mononucleosis-like picture; night sweats, weight loss, and lymphadenopathy are also frequent manifestations. An infection with Bartonella Henselae is another known cause of local lymphadenopathy. However, in immunocompromised individuals, Bartonella Henselae is far more likely to cause a systemic response that may mimic PTLD. The specifics regarding differentiating systemic bartonella and PTLD in pediatric solid organ transplant patients are not well described. There have been more detailed descriptions of disseminated bartonella mimicking PTLD in solid organ transplants in adult patients, but this has not been thoroughly reported or described in the pediatric population. Upon literature review, only five cases of disseminated bartonella in pediatric kidney transplant recipients have been described, with three of them possibly associated with PTLD. The past cases described antimicrobial diagnostics for bartonella and other infections, but none of these reported full histopathological findings or associated imaging studies for work-up of PTLD. In addition, past cases have only been described in Epstein-Barr virus (EBV) and Cytomegalovirus (CMV) negative children. There is no consensus for treatment duration and antibiotic choice for confirmed systemic bartonella infection in pediatric kidney transplant patients due to concerns of nephrotoxicity and drug interactions. METHODS: This patient who presented with fever, lymphadenopathy, nausea, vomiting, and abdominal pain was worked up and treated for both Bartonella Henslae and PTLD. PET Scan and biopsies were taken for the diagnosis of PTLD and the patient had Bartonella serologies and PCR testing done as part of a broader infectious workup. Clinical decisions regarding his treatment plan and the cause of his symptoms were made based on response to treatment and the likelihood of underlying etiology based on the timeline which his symptoms improved. RESULTS: After an extensive workup for PTLD and altering immunosuppressive regimens temporarily for possible PTLD we ultimately determined the cause of the patients' symptoms to be secondary to B. henselae infection. The patient was treated appropriately for disseminated Bartonella and improved significantly. He has followed up as an outpatient with continued improvement in symptoms and resumed his previous immunosuppression regimen as PTLD has been ruled out. CONCLUSION: Bartonella and PTLD are diagnoses that need to be carefully considered and treated in vulnerable populations. Pediatric patients should be managed cautiously with both etiologies under consideration, and more research is needed for specific recommendations regarding differentiating and treating the two simultaneously when clinical presentation is questionable. Given the overlapping symptoms, a thorough workup to rule out and treat PTLD if necessary is recommended given its significant mortality if missed. With biopsy being the gold standard, we recommend careful consideration of performing a biopsy while treating underlying infectious concerns in cases where PTLD is a possibility in pediatric populations.
Liver international : official journal of the International Association for the Study of the LiverPetriina Luokkala, Maria Hukkinen, Iiris Nyholm, Nelli Sjöblom, Mikko P Pakarinen
BACKGROUND & AIMS: As better understanding of pathophysiology and reliable predictors for portal hypertension (PH) are needed, we investigated how histological ductular reaction (DR) and liver fibrosis predict PH in biliary atresia (BA). METHODS: 60 BA patients treated between 1987 and 2024 at Helsinki University Hospital were enrolled. Patients underwent liver biopsies at Kasai portoenterostomy and 1 year postoperatively. PH was defined as endoscopic detection of oesophageal varices or thrombocytopenia (< 150*109) and splenomegaly. A neural network model was used to quantify proportional DR (DR%) relative to entire liver tissue, composed of cytokeratin-7-positive portal biliary epithelium (BE%) and parenchymal intermediate hepatocytes (PIH%). Fibrosis was measured by Metavir staging and computerized quantification of Sirius-Red-positive area (SRP%). Predictors of PH were studied with AUROC and survival analyses, and Cox regression models. RESULTS: After portoenterostomy, PH was detected in 70% (n = 42) of patients at median age 1.4 years (IQR 0.7-2.6). At portoenterostomy, DR% ≥ 6.9% (p = 0.016) and PIH% ≥ 1.6% (p < 0.001), but not liver fibrosis apart from cirrhosis (n = 5) predicted PH (p = 0.032). At 1 year, DR% ≥ 3.5% (p < 0.001), PIH% ≥ 0.7% (p < 0.001), BE% ≥ 3.2% (p = 0.006) as well as Metavir 3-4 vs. 1-2 (p = 0.037) and SRP% ≥ 28.4% (p = 0.006) predicted PH. Patients with PH showed progression of PIH% (p < 0.001) from portoenterostomy to follow-up biopsy, accompanied by more rapid progression of fibrosis. In univariable regression models, different DR components, but not fibrosis, were associated with PH both at portoenterostomy and follow-up also after successful portoenterostomy. CONCLUSION: DR predicted development of PH at and one year after portoenterostomy, associating with PH more consistently than fibrosis.
Journal of clinical apheresisGiovanni Ceschia, Ana C Navarro, Kelli A Krallman, Stuart L Goldstein
To characterize pediatric TPE utilization, safety, treatment response, and mortality across American Society for Apheresis (ASFA) categories and over time. Retrospective single-center cohort study of patients aged 0-25 years who underwent therapeutic plasma exchange (TPE) between 2011 and 2024. Primary outcomes included TPE utilization, temporal trends, and procedure-related complications. Secondary outcomes included treatment response according to indication-specific criteria and 28-day mortality overall and by ASFA category. A total of 416 TPE courses were performed in 384 patients (median age 10 years, IQR 2-16). TPE utilization increased over time (Spearman ρ = 0.88; p < 0.001), with ASFA Category II and III indications accounting for approximately 80% of courses throughout the study period. Procedure-related complications occurred in 76 of 3640 procedures (2%) and were more frequent in Category III than Categories I and II (3% vs. 1% and 2%; p < 0.001). Rates of treatment goal achievement were similar across ASFA categories (84.0% in Category I, 84.8% in Category II, and 75.4% in Category III; p = 0.11). Overall, 28-day mortality was 13%, including 4% in Category I, 6% in Category II, and 24% in Category III (p < 0.001). In Category III indications, treatment during 2018-2024 was associated with lower adjusted odds of 28-day mortality compared with 2011-2017 (aOR 0.33; 95% CI 0.13-0.83; p = 0.02). TPE use in our single center has expanded substantially, particularly for lower-evidence indications. Despite this shift, TPE remains safe and is associated with similar response rates across categories. Improved adjusted survival over time was observed in Category III.
European journal of pediatricsSalmas Watad, Adi Avniel Aran, Asaf Mandel, Yshia Langer, Ezra Weinblatt, Isaac Manaster, Natalie Barsoum, Sergei Elber Dorozko, Dina Averbuch, Oren Gordon, Ur…
UNLABELLED: Measles is resurgent worldwide, and although vaccine-preventable, severe disease can progress to cardiorespiratory failure refractory to conventional support. This study aimed to determine whether extracorporeal membrane oxygenation (ECMO) is feasible in children with measles. A retrospective case series at a tertiary pediatric intensive care unit (PICU) with a regional ECMO program, of all children younger than 18 years with laboratory-confirmed measles who received ECMO during the August 2025-May 2026 outbreak. Of 49 children with measles admitted to the PICU, 10 received ECMO. Five were male; median age was 11 months (IQR, 10-24), and none had completed age-appropriate vaccination. Indications were septic shock or cardiorespiratory failure (n = 5), acute respiratory distress syndrome with septic shock (n = 2), hypoxemic respiratory failure (n = 2), and suspected myocarditis (n = 1). Seven received venoarterial, 1 veno-venoarterial, and 2 venovenous ECMO. Bacteremia preceded ECMO in 5 children and positive sputum culture in 8. Candida tropicalis candidemia developed in 3 of the first 5 patients, with none among the subsequent 5 after antifungal prophylaxis at cannulation. Left ventricular dysfunction was present in 7 of 9 assessed, 4 required decompression, and neuroimaging was abnormal in 4 of 8 imaged. Six children (60%) survived to discharge. CONCLUSIONS: ECMO was feasible and potentially lifesaving in measles-associated cardiorespiratory failure, with survival comparable to other high-acuity pediatric indications, but the course was complicated by bacterial and fungal coinfection, cardiac dysfunction, and neurologic injury. These resource-intensive presentations occurred exclusively in unvaccinated children and were preventable, underscoring measles vaccination coverage as a public health priority. WHAT IS KNOWN: • Measles is resurgent worldwide and can cause severe respiratory, cardiac, and immune complications in children. • Measles-induced immune suppression predisposes to secondary bacterial and fungal infection. WHAT IS NEW: • First reported series of ECMO for measles: feasible, with 60% survival, but marked by coinfection, cardiac dysfunction, and neurologic injury. • Antifungal prophylaxis at ECMO cannulation was associated with elimination of candidemia in this cohort.
Journal of orthopaedic research : official publication of the Orthopaedic Research SocietyBernhard Guggenberger, Hans Kainz, Tanja Kraus, Willi Koller, Martin Svehlik
Increased femoral version is linked to patellofemoral instability, increased lateral loading of the patella and might be treated with femoral derotation osteotomy (FDO) in excessive cases. However, the effects of FDO on patellofemoral stability and loading while walking are not fully understood and not measurable in vivo. This study used personalized in-silico musculoskeletal modeling to investigate the impact of FDO on medio-lateral patellofemoral joint loading in individuals with patellofemoral instability. We analyzed retrospective data from 16 participants with recurrent patellofemoral instability and femoral version ≥ 30° using musculoskeletal simulations. We prepared two models per participant based on gait analysis and magnetic resonance data: (i) a model with personalized femoral version and tibial torsion and (ii) a post-surgery model with femoral version adjusted to 12° to simulate the derotation osteotomy. We assessed joint angles, joint moments, muscle moments, muscle forces, and patellofemoral joint contact forces. Simulated FDO significantly altered superior-inferior and anterior-posterior patellofemoral joint contact forces but did not significantly change medio-lateral patellofemoral joint loading at the group level. Six participants (37.5%) demonstrated a reduction in lateralizing patellofemoral joint contact forces, three (18.8%) showed no relevant change, and seven (43.7%) exhibited increased lateralizing forces. Changes in rectus femoris muscle force correlated with changes in medio-lateral patellofemoral joint loading. Participants with worsening patellofemoral loading had greater tibial torsion and exhibited distinct gait characteristics. These findings suggest that patient-specific functional and anatomical characteristics should be considered when selecting candidates for FDO and planning surgical treatment.
Pediatric transplantationAkosua Dansoa Odei, Haoran Jiang, Natasha Anders, Seth Erasmus Murray Wolf, Chelsea Harris, Rebekah Boyd, Matthew Galen Hartwig, Douglas Marvin Overbey, Joseph…
BACKGROUND: Heart transplantation is definitive therapy for children with end-stage heart failure. Because donor availability is unpredictable, transplantation occurs overnight or on weekends. Although studies report mixed findings on the association between operative timing and outcomes, pediatric data remain limited. We evaluated the impact of operative timing on pediatric heart transplant outcomes. METHODS: We studied pediatric heart transplant recipients (< 18 years) in the United Network for Organ Sharing registry from 2000 to 2018. Transplants were classified by time of day (day: 9 AM-8:59 PM; night: 9 PM-8:59 AM) and day of week (weekday vs. weekend). Primary outcome was survival. Secondary outcomes included graft survival, rejection, retransplantation, and postoperative complications. Survival was analyzed using Kaplan-Meier and log-rank testing. Multivariable Cox regression adjusted for recipient and donor characteristics and pre-transplant support. RESULTS: Among 6125 recipients, 2930 (47.8%) transplants occurred during the day and 3195 (52.2%) at night; 4408 (72.0%) occurred on weekdays and 1717 (28.0%) on weekends. Kaplan-Meier analysis demonstrated no significant survival difference between daytime and nighttime transplants (p = 0.266) or weekday and weekend transplants (p = 0.210). In multivariable Cox regression, operative timing was not associated with mortality (night vs. day: hazard ratio 0.97, 95% confidence interval 0.89-1.06, p = 0.554; weekend versus weekday: hazard ratio 1.04, 95% confidence interval 0.94-1.15, p = 0.445). Acute rejection prior to discharge and treatment for rejection within 1 year was more frequent in daytime transplants without affecting graft survival. CONCLUSIONS: Operative timing was not associated with patient survival, graft survival, or postoperative outcomes after pediatric heart transplantation.
CancerNicholas F Evageliou, Lindsay A Renfro, Kelly L Vallance, Carly R Varela, Daniel J Benedetti, Nicholas G Cost, Peter F Ehrlich, John A Kalapurakal, Geetika Kha…
BACKGROUND: Improvements in therapy for patients with favorable histology Wilms tumor (FHWT) have relied on refinement of risk stratification for therapeutic assignment through identification of favorable and unfavorable prognostic factors. Although well established in International Society of Pediatric Oncology-Renal Tumor Study Group protocols, the Children's Oncology Group (COG) has not historically used post-chemotherapy histology (PCH) to guide therapy. This study examines whether outcomes are associated with PCH classification in the COG treatment context. METHODS: The authors identified 2386 patients with stage III or IV FHWT enrolled on AREN0532, AREN0533, or AREN03B2-only from 2006 to 2019. Inclusion criteria included delayed nephrectomy more than 5 and fewer than 16 weeks after initial biopsy, treatment on or as per protocol with DD4A or Regimen M, and known PCH classification and outcome. RESULTS: Of 352 included patients, 45 (12.7%) were classified as low risk (LR), 287 (81.5%) as intermediate risk (IR), and 20 (5.6%) as high risk (HR). PCH classification was significantly prognostic for event-free survival (EFS) (p < .0001) and overall survival (OS) (p < .0001). Four-year EFS for LR, IR, and HR groups was 93% (95% CI, 86%-100%), 85% (95% CI, 81%-89%), and 53% (95% CI, 35%-81%); 4-year OS was 96% (95% CI, 90%-100%), 93% (95% CI, 89%-96%), and 62% (95% CI, 44%-89%). Compared with LR or IR, EFS and OS were significantly lower for HR, regardless of stage or treatment. CONCLUSION: PCH is a significant prognostic factor in unilateral FHWT treated on COG regimens, strongly supporting its incorporation into therapeutic stratification in prospective COG trials.
BACKGROUND: Pre-emptive kidney transplantation is the preferred treatment in patients with end-stage kidney disease (ESKD); however, many patients must unavoidably initiate dialysis prior to transplantation. Prolonged dialysis exposure is associated with increased morbidity and mortality, mandating prompt transition to transplant when possible. Delays in transplant readiness are unfortunately prevalent and are often driven by fragmented communication, limited education, and system-level barriers. OBJECTIVE: To reduce time from dialysis initiation to deceased donor kidney transplant (DDKT) activation among pediatric patients through a single-center quality improvement initiative. METHODS: This quality improvement (QI) project was conducted at a tertiary children's hospital affiliated with a pediatric outpatient dialysis unit. Baseline data from 2023 demonstrated a median dialysis-to-DDKT activation time of 211.5 days. Interventions were implemented in 2024 through three Plan-Do-Study-Act cycles (PDSA): (1) establishment of a multidisciplinary Guidance and Planning clinic for early transplant education; (2) implementation of biweekly transplant-dialysis coordination meetings supported by expanded transplant coordinator staffing; (3) distribution of a bilingual patient education booklet at dialysis initiation. The primary outcome measure was time to DDKT activation. Trends were evaluated using descriptive statistics and a run chart. RESULTS: In 2024, 10 patients initiated dialysis during the intervention phase. Excluding 2 patients who were activated for transplantation prior to outpatient dialysis initiation, the median time from dialysis initiation to DDKT activation decreased by 38%, from 211.5 days in 2023 to 130.5 days in 2024. Sixty percent of patients initiating dialysis in 2024 underwent transplantation within the same calendar year. Process measures demonstrated successful implementation of interventions. CONCLUSIONS: Implementation of a structured quality improvement approach was associated with reduced time to transplant activation in pediatric dialysis patients. Improved communication, education, and transplant coordination may represent modifiable factors that may enhance transplant readiness and outcomes.
Pediatric transplantationChun-Sing Huang, Naseem Ravanbakhsh, Shreya Tamma, Emma Hills, Sarah Van Winkle, Eric Lee, Annika Agnihotri, Rayhan Jhanji, Anna M Banc-Husu, Daniel H Leung, M…
BACKGROUND: Gestational alloimmune liver disease is the most common cause of acute liver failure in neonates. Treatment includes exchange transfusion and IVIG, though a subset of neonates requires liver transplantation for survival. METHODS: We conducted a retrospective study using the Organ Procurement and Transplantation Network database and an institutional case series to characterize waitlist outcomes and patient survival among transplanted patients with gestational alloimmune liver disease. RESULTS: 81 candidates were registered on the liver transplant waitlist with a neonatal hemochromatosis diagnosis code from October 1987 through March 2025. Of these, 36 (44.4%) received a liver transplant. Re-transplantation was required in 6 (16.7%) patients. Four patients developed graft failure within the first year of transplant, 3 (75%) of which were due to hepatic artery thrombosis. Patient survival was 88.8% at 30 days, 82.7% at 1 year, and 79.5% at 5 years. Of the 45 candidates (55.6%) who did not receive a transplant, 20 (24.7% of all listed) died on the waitlist, 15 (18.5%) experienced improvement and ultimately did not need transplant, 8 (9.9%) were too sick to transplant, 1 (1.2%) was removed for other reasons, and 1 (1.2%) was transferred to another center. Among the institutional case series, three neonates presented with acute liver failure with workup consistent with GALD. All three were listed as status 1A, underwent successful liver transplant, and are alive present day. CONCLUSIONS: Liver transplant should be offered without hesitation for treatment-refractory GALD. Early referral to a transplant center should occur simultaneously with initiation of medical therapy.
BACKGROUND: Guillain-Barré syndrome (GBS) is an acute, immune-mediated polyradiculoneuropathy most often triggered by infections such as Campylobacter jejuni, Epstein-Barr virus (EBV), or cytomegalovirus (CMV). Although transplant-associated GBS is recognized in adults, it remains rare in pediatric recipients, and distinguishing immune-mediated disease from medication toxicity or critical illness can be challenging. METHODS: We describe two pediatric cases of GBS following organ transplantation, one after liver transplant and one after small bowel transplant. Clinical features, diagnostic evaluation, immunosuppression exposure, electrodiagnostic findings, imaging, and treatment outcomes were reviewed to characterize presentation patterns and diagnostic challenges. RESULTS: The first patient developed rapidly progressive weakness shortly after liver transplantation in the setting of organ failure, adenovirus infection, and transient supratherapeutic tacrolimus levels. She demonstrated severe motor axonal involvement on nerve conduction studies and elevated neurofilament light chains, improving after intravenous immunoglobulin (IVIG). The second patient, years after small bowel transplantation complicated by chronic immune dysregulation and EBV viremia, presented with ascending weakness, areflexia, elevated CSF protein, and MRI enhancement of cauda equina and cranial nerves. Electrodiagnostics confirmed demyelinating polyneuropathy. Symptoms improved after IVIG despite concurrent tacrolimus level elevation. Across both cases, infectious triggers, immunosuppression, and critical illness obscured early recognition. CONCLUSION: GBS is an uncommon but important cause of acute weakness in pediatric transplant recipients. These cases show that GBS can occur both early and late after transplantation and may mimic medication toxicity or other complications. Early consideration of GBS, timely electrodiagnostic testing, and prompt immunotherapy are essential for optimizing outcomes in these patients.
Pediatric pulmonologyMyungyeon Lee, Wonik Lee, Chang Ho Shin, Dong In Suh, Tae-Joon Cho, Ji Soo Park, Mi Hyun Song
BACKGROUND: Children with neurological complex chronic conditions (NCCCs) frequently undergo hip reconstruction for hip displacement. Impaired airway clearance and respiratory muscle weakness may increase the risk of postoperative respiratory events. This study evaluated the incidence and predictors of postoperative respiratory events in this population. METHODS: We retrospectively reviewed children with NCCCs who underwent hip reconstruction between 2015 and 2024. Baseline characteristics included age, sex, diagnosis, comorbidities, feeding method, ambulatory status, and respiratory support. Operative and anesthesia-related variables included the number of osteotomies, operative duration and scheduling, estimated blood loss, airway device, and arterial blood gas measurements. The primary outcome was postoperative desaturation (SpO2 < 95%) requiring supplemental oxygen. Secondary outcomes included pulmonary complications and intensive care unit (ICU) utilization. Predictors were initially evaluated using an expanded multivariable logistic regression model, followed by a reduced model using Firth's penalized logistic regression. RESULTS: Among 126 children (mean age, 7.7 ± 4.1 years), postoperative desaturation occurred in 30 (23.8%). Pulmonary complications included respiratory failure (n = 6), atelectasis (n = 5), pulmonary edema (n = 3), and pneumonia (n = 1). ICU care was required for 22 children (17.5%). Predictors of postoperative desaturation included congenital brain malformation (adjusted odds ratio [aOR], 21.76; 95% confidence interval [CI], 3.33-249.48), surgery performed on Friday (aOR, 3.35; 95% CI, 1.11-10.24), ≥ 3 osteotomies (aOR, 7.82; 95% CI, 1.83-36.36), and elevated intraoperative bicarbonate (HCO3 - > 26 mEq/L; aOR, 6.49; 95% CI, 2.36-19.14). CONCLUSION: Postoperative desaturation was common after hip reconstruction in children with NCCCs, with a subset developing pulmonary complications or requiring ICU care. Identification of perioperative risk factors may improve risk stratification and support targeted respiratory monitoring and preventive strategies in this high-risk population. LEVEL OF EVIDENCE: Level III, retrospective cohort study.