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Interventional Cardiology Clinics Jul 2024Transcatheter ductal closure has proven to be safe and effective to occlude the patent ductus arteriosus (PDA) in premature infants. We have developed a robust... (Review)
Review
Transcatheter ductal closure has proven to be safe and effective to occlude the patent ductus arteriosus (PDA) in premature infants. We have developed a robust multidisciplinary PDA program. After gaining experience performing transcatheter ductal closure in the cardiac catheterization laboratory, we transitioned to performing the procedure at the patient's bedside in the neonatal intensive care unit using echocardiographic guidance. The bedside approach has the potential to expand this therapy to patients who currently undergo expectant PDA management because of lack of availability of this therapy, and allow for earlier referral, which will reduce time of exposure to this pathologic shunt.
Topics: Humans; Ductus Arteriosus, Patent; Infant, Newborn; Infant, Premature; Cardiac Catheterization; Septal Occluder Device; Echocardiography
PubMed: 38839169
DOI: 10.1016/j.iccl.2024.02.001 -
ELife Jun 2024Ninein is a centrosome protein that has been implicated in microtubule anchorage and centrosome cohesion. Mutations in the human gene have been linked to Seckel...
Ninein is a centrosome protein that has been implicated in microtubule anchorage and centrosome cohesion. Mutations in the human gene have been linked to Seckel syndrome and to a rare form of skeletal dysplasia. However, the role of ninein in skeletal development remains unknown. Here, we describe a ninein knockout mouse with advanced endochondral ossification during embryonic development. Although the long bones maintain a regular size, the absence of ninein delays the formation of the bone marrow cavity in the prenatal tibia. Likewise, intramembranous ossification in the skull is more developed, leading to a premature closure of the interfrontal suture. We demonstrate that ninein is strongly expressed in osteoclasts of control mice, and that its absence reduces the fusion of precursor cells into syncytial osteoclasts, whereas the number of osteoblasts remains unaffected. As a consequence, ninein-deficient osteoclasts have a reduced capacity to resorb bone. At the cellular level, the absence of ninein interferes with centrosomal microtubule organization, reduces centrosome cohesion, and provokes the loss of centrosome clustering in multinucleated mature osteoclasts. We propose that centrosomal ninein is important for osteoclast fusion, to enable a functional balance between bone-forming osteoblasts and bone-resorbing osteoclasts during skeletal development.
Topics: Animals; Mice; Centrosome; Mice, Knockout; Nuclear Proteins; Osteoblasts; Osteoclasts; Osteogenesis
PubMed: 38836552
DOI: 10.7554/eLife.93457 -
Journal of Perinatology : Official... Jun 2024Review a cohort of preterm infants ≤29 weeks of gestation at birth and compare morbidities and neurodevelopmental outcomes based on PDA status and type of PDA closure.
OBJECTIVE
Review a cohort of preterm infants ≤29 weeks of gestation at birth and compare morbidities and neurodevelopmental outcomes based on PDA status and type of PDA closure.
STUDY DESIGN
Single center observational retrospective-prospective case control study of premature infants who had no hsPDA, underwent surgical ligation or percutaneous transcatheter closure of the PDA. Neurodevelopmental testing was done using the Bayley Scales of Infant Development 3rd ed.
RESULTS
The percutaneous transcatheter closure group had an older post menstrual age and greater weight at the time of procedure, and started enteral feeds and achieved room air status at an earlier post procedure day. Infants in the surgical ligation group were more likely to experience vocal cord paralysis. There was no difference in neurodevelopmental outcomes between groups.
CONCLUSION
Waiting for infants to achieve the appropriate size for percutaneous transcatheter closure of the PDA may lead to reduced short-term complications without increasing the risk of neurodevelopmental impairment.
PubMed: 38831120
DOI: 10.1038/s41372-024-02019-w -
BMJ Paediatrics Open May 2024To compare the neurodevelopmental outcomes of preterm infants before and during the COVID-19 pandemic.
OBJECTIVE
To compare the neurodevelopmental outcomes of preterm infants before and during the COVID-19 pandemic.
DESIGN
Premature infants born in 2018 were assigned to the pre-pandemic group, while those born in 2019 were assigned to the during-pandemic group.
SETTING
Nationwide cohort study.
PATIENTS
Very low birthweight premature infants registered in the Taiwan Premature Infant Follow-up Network database.
INTERVENTIONS
Anti-epidemic measures, including quarantine and isolation protocols, social distancing, the closure of public spaces and restrictions on travel and gatherings during COVID-19 pandemic.
MAIN OUTCOME MEASURES
Outcomes were measured by Bayley Scales of Infant and Toddler Development Third Edition at corrected ages of 6, 12 and 24 months old. Generalised estimating equation (GEE) was applied to incorporate all measurements into a single model.
RESULTS
Among the 1939 premature infants who were enrolled, 985 developed before the pandemic, while 954 developed during the pandemic. Premature infants whose development occurred during the pandemic exhibited better cognitive composite at the corrected age of 6 months (beta=2.358; 95% CI, 1.07 to 3.65; p<0.001), and motor composite at corrected ages of 12 months (beta=1.680; 95% CI, 0.34 to 3.02; p=0.014). GEE analysis showed that infants who had grown during the pandemic achieved higher scores in cognitive composite (beta=1.416; 95% CI, 0.36 to 2.48; p=0.009).
CONCLUSION
Premature infants in Taiwan who developed during the pandemic showed better neurodevelopment compared with those born before the pandemic.
Topics: Humans; COVID-19; Taiwan; Infant, Premature; Male; Female; Infant, Newborn; Infant; Retrospective Studies; Child Development; SARS-CoV-2; Neurodevelopmental Disorders; Infant, Very Low Birth Weight; Pandemics; Cohort Studies
PubMed: 38823798
DOI: 10.1136/bmjpo-2024-002493 -
Journal of Evaluation in Clinical... May 2024Contextual information which is implicitly available to physicians during clinical encounters has been shown to influence diagnostic reasoning. To better understand the...
AIMS AND OBJECTIVES
Contextual information which is implicitly available to physicians during clinical encounters has been shown to influence diagnostic reasoning. To better understand the psychological mechanisms underlying the influence of context on diagnostic accuracy, we conducted a review of experimental research on this topic.
METHOD
We searched Web of Science, PubMed, and Scopus for relevant articles and looked for additional records by reading the references and approaching experts. We limited the review to true experiments involving physicians in which the outcome variable was the accuracy of the diagnosis.
RESULTS
The 43 studies reviewed examined two categories of contextual variables: (a) case-intrinsic contextual information and (b) case-extrinsic contextual information. Case-intrinsic information includes implicit misleading diagnostic suggestions in the disease history of the patient, or emotional volatility of the patient. Case-extrinsic or situational information includes a similar (but different) case seen previously, perceived case difficulty, or external digital diagnostic support. Time pressure and interruptions are other extrinsic influences that may affect the accuracy of a diagnosis but have produced conflicting findings.
CONCLUSION
We propose two tentative hypotheses explaining the role of context in diagnostic accuracy. According to the negative-affect hypothesis, diagnostic errors emerge when the physician's attention shifts from the relevant clinical findings to the (irrelevant) source of negative affect (for instance patient aggression) raised in a clinical encounter. The early-diagnosis-primacy hypothesis attributes errors to the extraordinary influence of the initial hypothesis that comes to the physician's mind on the subsequent collecting and interpretation of case information. Future research should test these mechanisms explicitly. Possible alternative mechanisms such as premature closure or increased production of (irrelevant) rival diagnoses in response to context deserve further scrutiny. Implications for medical education and practice are discussed.
PubMed: 38818694
DOI: 10.1111/jep.14023 -
Treatment strategies of the thromboembolic risk in kidney failure patients with atrial fibrillation.Nephrology, Dialysis, Transplantation :... May 2024The incidence and prevalence of atrial fibrillation (AF) in patients affected by kidney failure, i.e. glomerular filtration rate <15 ml/min/1.73 m2, is high and...
The incidence and prevalence of atrial fibrillation (AF) in patients affected by kidney failure, i.e. glomerular filtration rate <15 ml/min/1.73 m2, is high and probably underestimated. Numerous uncertainties remain regarding how to prevent thromboembolic events in this population because both cardiology and nephrology guidelines do not provide clear recommendations. The efficacy and safety of oral anticoagulant therapy (OAC) in preventing thromboembolism in patients with kidney failure and AF has not been demonstrated for either vitamin K antagonists (VKA) or direct anticoagulants (DOAC). Moreover, it remains unclear which is more effective and safer between them, because estimated creatinine clearance < 25-30 ml/min was an exclusion criterion of the randomized control trials (RCTs). Three RCTs comparing DOACs and VKAs in kidney failure failed to reach the primary endpoint because they were underpowered. The left atrial appendage is the main source of thromboembolism in the presence of AF. Left atrial appendage closure (LAAC) has recently been proposed as an alternative to OAC. RCTs comparing the efficacy and safety of LAAC vs. OAC in kidney failure were terminated prematurely due to recruitment failure. A recent prospective study showed a reduction in thromboembolic events in hemodialysis patients with AF and undergoing LAAC compared to patients taking or not taking OAC. We review current treatment standards and discuss recent developments in managing the thromboembolic risk in kidney failure patients with AF. The importance of shared decision-making with the multidisciplinary team and the patient, to consider individual risks and benefits of each treatment option is underlined.
PubMed: 38816212
DOI: 10.1093/ndt/gfae121 -
Journal of Cardiovascular Development... Apr 2024(1) Background: To identify reasons for the persistence of surgical ligation of the patent ductus arteriosus (PDA) in premature infants after the 2019 Food and Drug...
Who Still Gets Ligated? Reasons for Persistence of Surgical Ligation of the Patent Ductus Arteriosus Following Availability of Transcatheter Device Occlusion for Premature Neonates.
(1) Background: To identify reasons for the persistence of surgical ligation of the patent ductus arteriosus (PDA) in premature infants after the 2019 Food and Drug Administration (FDA) approval of transcatheter device closure; (2) Methods: We performed a 10-year (2014-2023) single-institution retrospective study of premature infants (<37 weeks) and compared clinical characteristics and neonatal morbidities between neonates that underwent surgical ligation before (epoch 1) and after (epoch 2) FDA approval of transcatheter closure; (3) Results: We identified 120 premature infants that underwent surgical ligation ( = 94 before, = 26 after FDA approval). Unfavorable PDA morphology, active infection, and recent abdominal pathology were the most common reasons for surgical ligation over device occlusion in epoch 2. There were no differences in demographics, age at closure, or outcomes between infants who received surgical ligation in the two epochs; (4) Conclusions: Despite increasing trends for transcatheter PDA closure in premature infants, surgical ligation persists due to unfavorable ductal morphology, active infection, or abdominal pathology.
PubMed: 38786954
DOI: 10.3390/jcdd11050132 -
Orphanet Journal of Rare Diseases May 2024Trigonocephaly occurs due to the premature fusion of the metopic suture, leading to a triangular forehead and hypotelorism. This condition often requires surgical...
BACKGROUND
Trigonocephaly occurs due to the premature fusion of the metopic suture, leading to a triangular forehead and hypotelorism. This condition often requires surgical correction for morphological and functional indications. Metopic ridges also originate from premature metopic closure but are only associated with mid-frontal bulging; their surgical correction is rarely required. Differential diagnosis between these two conditions can be challenging, especially in minor trigonocephaly.
METHODS
Two hundred seven scans of patients with trigonocephaly (90), metopic rigdes (27), and controls (90) were collected. Geometric morphometrics were used to quantify skull and orbital morphology as well as the interfrontal angle and the cephalic index. An innovative method was developed to automatically compute the frontal curvature along the metopic suture. Different machine-learning algorithms were tested to assess the predictive power of morphological data in terms of classification.
RESULTS
We showed that control patients, trigonocephaly and metopic rigdes have distinctive skull and orbital shapes. The 3D frontal curvature enabled a clear discrimination between groups (sensitivity and specificity > 92%). Furthermore, we reached an accuracy of 100% in group discrimination when combining 6 univariate measures.
CONCLUSION
Two diagnostic tools were proposed and demonstrated to be successful in assisting differential diagnosis for patients with trigonocephaly or metopic ridges. Further clinical assessments are required to validate the practical clinical relevance of these tools.
Topics: Humans; Craniosynostoses; Female; Male; Infant; Imaging, Three-Dimensional; Skull
PubMed: 38762603
DOI: 10.1186/s13023-024-03197-8 -
Clinical Neurology and Neurosurgery Jul 2024Craniosynostosis, a developmental craniofacial anomaly, can impair brain development and cause abnormal skull shape due to premature closure of one or more cranial... (Meta-Analysis)
Meta-Analysis Review
BACKGROUND
Craniosynostosis, a developmental craniofacial anomaly, can impair brain development and cause abnormal skull shape due to premature closure of one or more cranial sutures. Traditional surgical treatments have evolved from open operations to minimally invasive endoscopic techniques. This systematic review and meta-analysis aim to evaluate the effectiveness and safety of the endoscopic approach in craniosynostosis correction.
METHODS
Adhering to Cochrane Group standards and the PRISMA framework, this review utilized databases like PubMed, Embase, and Web of Science, focusing on clinical and surgical outcomes of endoscopic craniosynostosis operations up to December 2023. Inclusion criteria emphasized studies with at least five patients undergoing endoscopic procedures, while exclusion criteria involved non-English papers, incomplete texts, and overlapping data. Statistical analysis used R software with various packages, and methodological bias was assessed using the ROBINS-I framework.
RESULTS
The review included 30 studies (4 prospective, 26 retrospective) with 2561 patients. The median age at operation was 3.20 months. Findings showed a mean operative time of 68.06 min, median hospital stay of 1.28 days, and mean blood loss of 29.89 ml. Blood transfusion was required in 9.97% of cases. Helmet therapy post-operation was common, with a median duration of 9 months. The rate of postoperative complications was 1.86%, and the reoperation rate was 3.07%. No procedure-related mortality was observed. The study noted substantial variations in the handling of craniosynostosis and a lack of consensus on the optimal timing and surgical approach.
CONCLUSION
Endoscopic techniques for craniosynostosis repair demonstrate safety and effectiveness, characterized by low complication risks and favorable surgical outcomes. However, due to the limitations of observational studies and inherent heterogeneity, further comprehensive and controlled trials are needed to validate these findings and understand the long-term outcomes of the endoscopic approach.
Topics: Craniosynostoses; Humans; Neuroendoscopy; Treatment Outcome; Postoperative Complications; Endoscopy; Infant; Operative Time; Length of Stay
PubMed: 38749357
DOI: 10.1016/j.clineuro.2024.108296 -
Pediatric Cardiology May 2024This study aimed to evaluate the current state of congenital interventional cardiology training worldwide, with a focus on case volumes, competency assessment, and the...
This study aimed to evaluate the current state of congenital interventional cardiology training worldwide, with a focus on case volumes, competency assessment, and the need for ongoing mentorship during early career stages. A survey was conducted among program directors (PDs) of congenital interventional training programs across the globe. The survey gathered data on training pathways, case volumes, types of procedures performed, trainee competency assessment, and the role of ongoing mentorship. Of the 79 PDs who completed the survey, it was observed that training pathways and case volumes varied significantly, particularly between the United States and other countries. Most PDs reported an annual laboratory case volume of >500 congenital cardiac cases, with most cases being interventional. While trainees demonstrated competency in simple procedures (diagnostic cases, simple ASD closure), complex interventions (such as patent ductus arteriosus closure in premature infants) require ongoing mentorship for graduates. PDs recommended a minimum case volume of 400 total cases for trainees, including 250 interventional cases. In addition to case volumes, assessing trainee competency was deemed important, with clinical reasoning, judgment, skillset, teamwork, and complication management being key areas of evaluation. The study highlights the variability in congenital interventional cardiology training and the need for ongoing mentorship during the early career years. External mentorship programs, facilitated by national and international societies, are proposed to provide critical support for early career interventionalists thus enhancing patient care for congenital heart disease. Ultimately, the findings of this survey may serve as a framework for future training standards and guidelines in this specialized field.
PubMed: 38727825
DOI: 10.1007/s00246-024-03501-0