Frontiers in pediatrics
-
Frontiers in pediatrics · Jan 2017
ReviewPrimary Ciliary Dyskinesia: An Update on Clinical Aspects, Genetics, Diagnosis, and Future Treatment Strategies.
Primary ciliary dyskinesia (PCD) is an orphan disease (MIM 244400), autosomal recessive inherited, characterized by motile ciliary dysfunction. The estimated prevalence of PCD is 1:10,000 to 1:20,000 live-born children, but true prevalence could be even higher. PCD is characterized by chronic upper and lower respiratory tract disease, infertility/ectopic pregnancy, and situs anomalies, that occur in ≈50% of PCD patients (Kartagener syndrome), and these may be associated with congenital heart abnormalities. ⋯ Standardized care at specialized centers using a multidisciplinary approach that imposes surveillance of lung function and of airway biofilm composition likely improves patients' outcome. Pediatricians, neonatologists, pulmonologists, and ENT surgeons should maintain high awareness of PCD and refer patients to the specialized center before sustained irreversible lung damage develops. The recent creation of a network of PCD clinical centers, focusing on improving diagnosis and treatment, will hopefully help to improve care and knowledge of PCD patients.
-
Frontiers in pediatrics · Jan 2017
Challenges and Priorities for Pediatric Critical Care Clinician-Researchers in Low- and Middle-Income Countries.
There is need for more data on critical care outcomes and interventions from low- and middle-income countries (LMIC). Global research collaborations could help improve health-care delivery for critically ill children in LMIC where child mortality rates remain high. ⋯ LMIC clinician-researchers must be better empowered and resourced to lead and influence the local and global health research agenda for critically ill children. Increased funding options, access to training and mentorship in research methodology, and improved data collection systems for LMIC PCC researchers were recognized as key needs for success.
-
Frontiers in pediatrics · Jan 2017
Review3D Printing in Surgical Management of Double Outlet Right Ventricle.
Double outlet right ventricle (DORV) is a heterogeneous group of congenital heart diseases that require individualized surgical approach based on precise understanding of the complex cardiovascular anatomy. Physical 3-dimensional (3D) print models not only allow fast and unequivocal perception of the complex anatomy but also eliminate misunderstanding or miscommunication among imagers and surgeons. Except for those cases showing well-recognized classic surgical anatomy of DORV such as in cases with a typical subaortic or subpulmonary ventricular septal defect, 3D print models are of enormous value in surgical decision and planning. Furthermore, 3D print models can also be used for rehearsal of the intended procedure before the actual surgery on the patient so that the outcome of the procedure is precisely predicted and the procedure can be optimally tailored for the patient's specific anatomy. 3D print models are invaluable resource for hands-on surgical training of congenital heart surgeons.
-
Frontiers in pediatrics · Jan 2017
Use of Therapeutic Plasma Exchange during Extracorporeal Life Support in Critically Ill Cardiac Children with Thrombocytopenia-Associated Multi-Organ Failure.
Thrombocytopenia-associated multi-organ failure (TAMOF) in children is a well-described factor for increased hospital mortality. Low cardiac output syndrome (LCOS) and the effects of cardiopulmonary bypass may manifest with several adverse physiologic and immunologic effects, with varying degrees of thrombocytopenia and multi-organ dysfunction, sometimes very similar to TAMOF. LCOS is a common occurrence in children with critical heart disease, presenting in as much as 23.8% of infants postoperative of congenital heart surgery. Therapeutic plasma exchange (TPE) has been offered as a promising therapy for TAMOF; however, the therapeutic implications of this modality in children with critical heart disease and a clinical diagnosis of TAMOF are unknown. ⋯ In children with critical cardiac disease and clinical diagnosis of TAMOF necessitating ECMO for hemodynamic support, concurrent TPE may be associated with an improvement in organ failure and platelet count, particularly when started early. Further studies are warranted to establish the most effective use of TPE and its effect on survival in this population.
-
Frontiers in pediatrics · Jan 2017
ReviewEchocardiographic Evaluation of Hemodynamics in Neonates and Children.
Hemodynamic instability and inadequate cardiac performance are common in critically ill children. The clinical assessment of hemodynamic status is reliant upon physical examination supported by the clinical signs such as heart rate, blood pressure, capillary refill time, and measurement of the urine output and serum lactate. Unfortunately, all of these parameters are surrogate markers of cardiovascular well-being and they provide limited direct information regarding the adequacy of blood flow and tissue perfusion. ⋯ Like any other investigation, it has certain limitations and the most important limitation is its intermittent nature. Sometimes acquiring high quality images for precise functional assessment in a ventilated child can be challenging. Therefore, it should be used in conjunction with the existing tools (physical examination and clinical parameters) for hemodynamic assessment while making clinical decisions.