Pediatric Investigation最新文献

Importance: Parent-infant closeness and active parent participation in neonatal care are important for parent and infant health.

Objective: To give an overview of current neonatal settings and gain an in-depth understanding of facilitators and barriers to parent-infant closeness, zero-separation, in 19 countries.

Methods: Neonatal intensive care unit (NICU) professionals, representing 45 NICUs from a range of geographic regions in Europe and Canada, were purposefully selected and interviewed June-December 2018. Thematic analysis was conducted to identify, analyze and report patterns (themes) for parent-infant closeness across the entire series of interviews.

Results: Parent-infant separation during infant and/or maternity care is very common (42/45 units, 93%), despite the implementation of family integrated care (FICare) practices, including parent participation in medical rounds (17/45, 38%), structured education sessions for parents (16/45, 36%) and structured training for healthcare professionals (22/45, 49%). NICU professionals encountered four main themes with facilitators and barriers for parent-infant closeness on and between the hospital, unit, staff, and family level: Culture (jointly held characteristics, values, thinking and behaviors about parental presence and participation in the unit), Collaboration (the act of working together between and within different levels), Capacities (resources and policies), and Coaching (education to acquire and transfer knowledge and skills).

Interpretation: Implementing parent-infant closeness in the NICU is still challenging for healthcare professionals. Further optimization in neonatal care towards zero-separation and parent-infant closeness can be achieved by enforcing the 'four Cs for Closeness': Culture, Collaboration, Capacities, and Coaching.

Kawasaki disease (KD) is a systemic vasculitis of unknown etiology, which tends to involve coronary arteries and can lead to acquired heart disease in children. The immuno-inflammatory response and vascular endothelial dysfunction are important causes of coronary artery disease in patients with KD. Multisystem inflammatory syndrome in children (MIS-C) is a rare inflammatory disease in children identified in recent years, which is caused by severe acute respiratory syndrome coronavirus 2 infection; this disease overlaps with KD. This review examines research progress concerning the immuno-inflammatory response and vascular endothelial dysfunction associated with KD, as well as differences between KD and MIS-C.

Importance: During the coronavirus disease 2019 (COVID-19) lockdown, changes in the visiting rules in neonatal units might have affected the initiation and continuation of breastfeeding.

Objective: To investigate the effects of the implementation of the COVID-19 lockdown in the UK on mother's own milk (MOM) feeding in hospital and at the time of discharge in two UK neonatal units.

Methods: Retrospective cohort study using routinely recorded data from electronic patient records. Data were retrieved from two neonatal services in the UK East Midlands region. Adjusted logistic regression was used to compare the odds of MOM feeding before, and after the implementation of the UK lockdown.

Results: Among 2073 infants, after adjusting for maternal and infant characteristics and underlying trends over time, there were no differences in the odds of infants receiving any MOM during admission; any MOM at discharge or exclusive MOM at discharge before and after the imposition of the lockdown. Infants with birthweight <1000 g were three times less likely to receive any MOM at discharge compared to those with birthweight >2500 g (adjusted odds ratio [OR] 0.33, 95% confidence interval [CI]: 0.22-0.50). Younger mothers were less likely, and Black British mothers more likely, to be feeding MOM to their infants at discharge, while women in the least deprived Index of Multiple Deprivation (IMD) quintiles were 2-4 times more likely to do so, compared to those in the most deprived IMD quintile (adjusted OR 2.78, 95% CI: 1.97-3.90).

Interpretation: Despite the difficulties faced during COVID-19 pandemic-induced restrictions, infants in the participating neonatal units continued to receive MOM in similar proportions as before the pandemic.

Since October 2021 in Alabama, the United States, and March 2022 in central Scotland, the United Kingdom, the number of cases of severe acute hepatitis of unknown etiology/causes in children was found to increase, and the total number of cases has reached 920 worldwide by June 22 this year, 45 cases (5%) required liver transplantation, and 18 cases (2%) died according to World Health Organization (WHO). To understand the basic characteristics of this disease/syndrome, a literature search was performed at PubMed, websites of WHO, UK Health Security Agency, and US and European Centers for Disease Control and Prevention, and more than 20 reports were enrolled as references for this review. The main clinical manifestations are anorexia, vomiting, fatigue, jaundice, and so forth. Most of the cases seemed to have a self-limited course of the disease, about 6% of cases may develop life-threatening acute liver failure. The disease seems to be transmissible from person to person. Human adenovirus was detected in up to 75% of cases, but this virus seems not to be the only and major etiologic agent, other cofactors probably are involved. Researchers proposed many hypotheses concerning the etiology and pathogenesis, and many important works and studies are ongoing. This mini-review is aimed at summarizing, reviewing, and further understanding the characteristics of the disease, raising some clinically relevant questions, and trying to discuss some questions that may be related to the treatment of the disease for consideration.

Importance: There are a variety of musculoskeletal malformations and injuries that can occur in newborns. These can be a significant cause of perinatal death or a reason for miscarriage and can lead to long-term functional issues if not managed appropriately. There is no systematic and well-established screening program for neonatal musculoskeletal malformations and injuries in China now.

Objective: To report the incidence and types of congenital musculoskeletal malformations in two hospitals in Shenzhen City, to explore and discuss the details of the screening procedure and improve future prevention and treatment.

Methods: From October 2013 to May 2014, 2564 one-day-old newborns were screened by a pediatric orthopedic physical examination, in combination with ultrasonography when required, and the incidence and variety of diseases were recorded statistically.

Results: Among 2564 screened newborns, the following musculoskeletal conditions were identified: congenital muscular torticollis (CMT) (seven cases, 0.27%), hip subluxation (four cases, 0.16%), hip dysplasia (47 cases, 1.83%), congenital talipes equinovarus (CTEV) (two cases, 0.08%), congenital talipes calcaneovalgus (15 cases, 0.58%), polydactyly (nine cases, 0.35%), syndactyly (one case, 0.04%), and spinal hemivertebra (one case, 0.04%). Additionally, there were five (0.19%) neonates with birth injuries.

Interpretation: It is feasible to carry out neonatal screening and identification of musculoskeletal malformations and birth injuries in China. This is helpful as timely detection and early intervention for many of these conditions can avoid permanent functional impairment in these children.

Food protein-induced enterocolitis syndrome (FPIES) is a non-Immunoglobulin (non-IgE)-mediated food allergy. The elimination diet is the only therapy, the culprit food will be reintroduced if tolerance is acquired. However, it is possible that patients do not follow the recommendations given by the healthcare professional. We investigated if our advice to avoid the trigger food in patients with active FPIES and to reintroduce it in the diet in patients who achieved tolerance had been implemented. We interviewed by telephone the parents of children who were diagnosed with acute FPIES. About 23.2% of our patients disregarded our dietary recommendations: 6/42 (14.3%) of patients who passed a tolerance oral food challenge (OFC) did not eat the trigger food, 4/22 (18.2%) of patients who failed OFC ate the trigger food, and 9/18 (50.0%) of patients who did not perform a tolerance OFC ate the trigger food. We have analyzed some possible influencing factors and no difference was found to be statistically significant. Our results are in line with those reported for IgE-mediated food allergies. As has already been proposed by others, we suggest reassessing food consumption in all patients after a food challenge.

Introduction: Pathogenic variants in PLOD3, encoding lysyl hydroxylase-3 (LH3), can cause a hereditary connective tissue disorder that has rarely been reported. It is a multi-system disease, presenting with craniofacial dysmorphisms, skeletal and eye manifestations, sensorineural hearing loss, and variable skin manifestations. Severe central nervous system involvement has not been reported.

Case presentation: A 10-month-old girl was admitted with development delay and clustered epileptic spasms. Hypertelorism, an upturned nose, and low-set ears were noted in physical examination. Cerebral magnetic resonance imaging showed multiple intracranial malacias and bleeding foci, extensive abnormal signals in the white matter, and obvious brain atrophy, which was consistent with cerebral small vessel disease (SVD). Electroencephalography suggested hypsarrhythmia. The vertebrae were flattened. The distal end of the metacarpal bone in the left hand was irregular. She was diagnosed with West syndrome. Whole-exome sequencing revealed a novel homozygous variant of c.1216_1218delCTC (p.L406del) in PLOD3, which was found to be inherited from her heterozygous parents.

Conclusion: We report a patient with pathogenic PLOD3 mutation who presented with cerebral SVD. This report expands the phenotypic spectrum of LH3 deficiency.