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Trials Mar 2024Infants born with congenital diaphragmatic hernia (CDH) are at high risk of respiratory insufficiency and pulmonary hypertension. Routine practice includes immediate... (Randomized Controlled Trial)
Randomized Controlled Trial
Multicentre, randomised controlled trial of physiological-based cord clamping versus immediate cord clamping in infants with a congenital diaphragmatic hernia (PinC): statistical analysis plan.
BACKGROUND
Infants born with congenital diaphragmatic hernia (CDH) are at high risk of respiratory insufficiency and pulmonary hypertension. Routine practice includes immediate clamping of the umbilical cord and endotracheal intubation. Experimental animal studies suggest that clamping the umbilical cord guided by physiological changes and after the lungs have been aerated, named physiological-based cord clamping (PBCC), could enhance the fetal-to-neonatal transition in CDH. We describe the statistical analysis plan for the clinical trial evaluating the effects of PBCC versus immediate cord clamping on pulmonary hypertension in infants with CDH (PinC trial).
DESIGN
The PinC trial is a multicentre, randomised controlled trial in infants with isolated left-sided CDH, born ≥ 35.0 weeks of gestation. The primary outcome is the incidence of pulmonary hypertension in the first 24 h after birth. Maternal outcomes include estimated maternal blood loss. Neonatal secondary outcomes include mortality before discharge, extracorporeal membrane oxygenation therapy, and number of days of mechanical ventilation. Infants are 1:1 randomised to either PBCC or immediate cord clamping using variable random permutated block sizes (4-8), stratified by treatment centre and estimated severity of pulmonary hypoplasia (i.e. mild/moderate/severe). At least 140 infants are needed to detect a relative reduction in pulmonary hypertension by one third, with 80% power and 0.05 significance level. A chi-square test will be used to evaluate the hypothesis that PBCC decreases the occurrence of pulmonary hypertension. This plan is written and submitted without knowledge of the collected data. The trial has been ethically approved.
TRIAL REGISTRATION
ClinicalTrials.gov NCT04373902 (registered April 2020).
Topics: Infant, Newborn; Pregnancy; Animals; Female; Humans; Hernias, Diaphragmatic, Congenital; Umbilical Cord Clamping; Hypertension, Pulmonary; Constriction; Respiration, Artificial; Umbilical Cord
PubMed: 38509614
DOI: 10.1186/s13063-024-08027-7 -
Cureus Feb 2024Background Congenital diaphragmatic hernia (CDH) is a condition where abdominal contents protrude into the chest due to defects in the diaphragm muscle. It is considered...
Background Congenital diaphragmatic hernia (CDH) is a condition where abdominal contents protrude into the chest due to defects in the diaphragm muscle. It is considered an emergency that needs urgent intervention to prevent further complications or death. Our study aimed to estimate survival and evaluate predictors of mortality in newborns with CDH using available prediction tools in the literature. Methods This retrospective cohort study included neonates with CDH in King Abdulaziz Medical City (KAMC), Jeddah, from 2000 to 2021. Prevalence, demographics, and clinical characteristics were compared between surviving and deceased infants. C-statistics were used to measure the area under the curve for the prenatal and postnatal predictor tools, and a p-value of <0.05 was considered significant. Results Between 2000 and 2021, 45 neonates with CDH were included (six per 10,000 inborn live births). The mortality rate was 51.1%. The differences in demographics were not significant among surviving and deceased patients. One prenatal predictor tool, the lung-to-head ratio, was found to be significant; in addition, three postnatal predictor tools of mortality, SNAP-II, CDHSG-probability survival, and Brindle Score, had the highest concordance (C) statistics of 0.8, 0.79, and 0.8, respectively. Conclusion Although the incidence of CDH was found to be higher in our study compared to global statistics, our mortality rates correspond with international figures. The most significant differences between predictors and prediction models of mortality were lung-to-head ratio prenatally, SNAP-II, CDHSG-probability survival, and Brindle Score postnatally. Further multicentered studies are recommended with a larger sample size.
PubMed: 38500943
DOI: 10.7759/cureus.54364 -
Journal of Surgical Case Reports Mar 2024The aim of this report is to describe the management of an iatrogenic diaphragmatic eventration following surgery to relieve neurogenic symptoms of thoracic outlet...
The aim of this report is to describe the management of an iatrogenic diaphragmatic eventration following surgery to relieve neurogenic symptoms of thoracic outlet syndrome in a patient with a prior history of sleeve gastrectomy. We discuss the case of a 46-year-old woman with a 6-month history of gastro-oesophageal reflux and dyspnoea. Imaging demonstrated a left hemidiaphragm eventration and hiatus hernia. The patient underwent laparoscopic plication of the left hemidiaphragm, repair of the hiatus hernia, and an omega loop gastric bypass, with satisfactory resolution of her symptoms. This demonstrates that surgical diaphragmatic plication has good outcomes in cases where the abdominal anatomy is already altered as a result of previous bariatric surgery, and that concurrent hiatus hernia repair, plication of iatrogenic diaphragm eventration, and sleeve to gastric bypass conversion satisfactorily relieve reflux and dyspnoea in a morbidly obese patient.
PubMed: 38487395
DOI: 10.1093/jscr/rjae117 -
Taiwanese Journal of Obstetrics &... Mar 2024
Topics: Humans; Female; Pregnancy; Hernias, Diaphragmatic, Congenital; Trisomy 18 Syndrome; Choroid Plexus; Chromosomes, Human, Pair 18; Trisomy; Cysts; Ultrasonography, Prenatal
PubMed: 38485333
DOI: 10.1016/j.tjog.2024.01.032 -
Respirology (Carlton, Vic.) May 2024
Topics: Humans; Hernia, Hiatal; Alveolitis, Extrinsic Allergic; Manometry
PubMed: 38479405
DOI: 10.1111/resp.14701 -
Archives of Gynecology and Obstetrics Jun 2024A 29-year-old primigravida at 31 weeks of gestation was referred for intrathoracic kidney (ITK). Ultrasound revealed left kidney intrathoracic placement with an...
A 29-year-old primigravida at 31 weeks of gestation was referred for intrathoracic kidney (ITK). Ultrasound revealed left kidney intrathoracic placement with an anteriorly positioned ectopic adrenal gland. Magnetic resonance imaging confirmed diaphragmatic interruption and colon herniation. A female neonate, delivered at 37 weeks, underwent successful thoracoscopic repair for a left Bochdalek hernia. Despite compression of the left lung, notably optimistic lung-to-head ratio (LHR) values were observed, correlating with favorable outcomes. This case underscores the rare occurrence of ITK, its association with Bochdalek hernia, and the importance of comprehensive prenatal evaluations.
Topics: Humans; Female; Hernias, Diaphragmatic, Congenital; Adult; Pregnancy; Kidney; Adrenal Glands; Ultrasonography, Prenatal; Infant, Newborn; Magnetic Resonance Imaging; Choristoma
PubMed: 38459998
DOI: 10.1007/s00404-024-07449-7 -
Journal of Perinatology : Official... Apr 2024
Topics: Infant; Humans; Hernias, Diaphragmatic, Congenital; Epoprostenol; Extracorporeal Membrane Oxygenation; Retrospective Studies; Prostaglandins I
PubMed: 38443465
DOI: 10.1038/s41372-024-01920-8 -
Nature Medicine Mar 2024Isolation of tissue-specific fetal stem cells and derivation of primary organoids is limited to samples obtained from termination of pregnancies, hampering prenatal...
Isolation of tissue-specific fetal stem cells and derivation of primary organoids is limited to samples obtained from termination of pregnancies, hampering prenatal investigation of fetal development and congenital diseases. Therefore, new patient-specific in vitro models are needed. To this aim, isolation and expansion of fetal stem cells during pregnancy, without the need for tissue samples or reprogramming, would be advantageous. Amniotic fluid (AF) is a source of cells from multiple developing organs. Using single-cell analysis, we characterized the cellular identities present in human AF. We identified and isolated viable epithelial stem/progenitor cells of fetal gastrointestinal, renal and pulmonary origin. Upon culture, these cells formed clonal epithelial organoids, manifesting small intestine, kidney tubule and lung identity. AF organoids exhibit transcriptomic, protein expression and functional features of their tissue of origin. With relevance for prenatal disease modeling, we derived lung organoids from AF and tracheal fluid cells of congenital diaphragmatic hernia fetuses, recapitulating some features of the disease. AF organoids are derived in a timeline compatible with prenatal intervention, potentially allowing investigation of therapeutic tools and regenerative medicine strategies personalized to the fetus at clinically relevant developmental stages.
Topics: Pregnancy; Female; Humans; Hernias, Diaphragmatic, Congenital; Amniotic Fluid; Prenatal Care; Lung; Organoids
PubMed: 38438734
DOI: 10.1038/s41591-024-02807-z -
Maternal and Child Health Journal Jun 2024To compare 5-year survival rate and morbidity in children with spina bifida, transposition of great arteries (TGA), congenital diaphragmatic hernia (CDH) or...
The Association of Prenatal Diagnoses with Mortality and Long-Term Morbidity in Children with Specific Isolated Congenital Anomalies: A European Register-Based Cohort Study.
OBJECTIVES
To compare 5-year survival rate and morbidity in children with spina bifida, transposition of great arteries (TGA), congenital diaphragmatic hernia (CDH) or gastroschisis diagnosed prenatally with those diagnosed postnatally.
METHODS
Population-based registers' data were linked to hospital and mortality databases.
RESULTS
Children whose anomaly was diagnosed prenatally (n = 1088) had a lower mean gestational age than those diagnosed postnatally (n = 1698) ranging from 8 days for CDH to 4 days for TGA. Children with CDH had the highest infant mortality rate with a significant difference (p < 0.001) between those prenatally (359/1,000 births) and postnatally (116/1,000) diagnosed. For all four anomalies, the median length of hospital stay was significantly greater in children with a prenatal diagnosis than those postnatally diagnosed. Children with prenatally diagnosed spina bifida (79% vs 60%; p = 0.002) were more likely to have surgery in the first week of life, with an indication that this also occurred in children with CDH (79% vs 69%; p = 0.06).
CONCLUSIONS
Our findings do not show improved outcomes for prenatally diagnosed infants. For conditions where prenatal diagnoses were associated with greater mortality and morbidity, the findings might be attributed to increased detection of more severe anomalies. The increased mortality and morbidity in those diagnosed prenatally may be related to the lower mean gestational age (GA) at birth, leading to insufficient surfactant for respiratory effort. This is especially important for these four groups of children as they have to undergo anaesthesia and surgery shortly after birth. Appropriate prenatal counselling about the time and mode of delivery is needed.
Topics: Humans; Female; Registries; Prenatal Diagnosis; Infant, Newborn; Pregnancy; Male; Infant; Cohort Studies; Morbidity; Gestational Age; Congenital Abnormalities; Europe; Infant Mortality; Child, Preschool; Hernias, Diaphragmatic, Congenital; Length of Stay; Gastroschisis; Survival Rate
PubMed: 38438690
DOI: 10.1007/s10995-024-03911-9 -
Clinical Case Reports Mar 2024Acute gastric volvulus is a life-threatening condition. Especially, a history of adjacent organ deformities in children with intermittent vomiting and abdominal pain...
Acute gastric volvulus is a life-threatening condition. Especially, a history of adjacent organ deformities in children with intermittent vomiting and abdominal pain should suggest gastric volvulus, even if the symptoms resemble gastroenteritis.
PubMed: 38435501
DOI: 10.1002/ccr3.8549