Article
Pediatrics
Sung Hyeon Park, Mi Jin Kim, Ha Na Lee, Jeong Min Lee, Soo Hyun Kim, Jiyoon Jeong, Byong Sop Lee, Euiseok Jung
Summary: The LPA:MPA ratio measured by echocardiography can be used as an independent postnatal predictor of death or need for ECMO in patients with left CDH.
Review
Biochemistry & Molecular Biology
Flaminia Pugnaloni, Irma Capolupo, Neil Patel, Paola Giliberti, Andrea Dotta, Pietro Bagolan, Florian Kipfmueller
Summary: This review highlights the importance of miRNAs in CDH-related PH and summarizes the findings from animal and human CDH studies. The focus on epigenetic modulators of CDH-PH offers potential for developing innovative diagnostic tools and treatment approaches, and enhances researchers' understanding of CDH pathophysiology.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
Article
Surgery
Duy T. Dao, Carmen M. Burgos, Matthew T. Harting, Kevin P. Lally, Pamela A. Lally, Hong-An T. Nguyen, Jay M. Wilson, Terry L. Buchmiller
Summary: The study indicates that early repair of CDH after ECMO cannulation is associated with improved survival and lower incidence of non-repair compared to delayed surgical correction.
Review
Pediatrics
Rameshwar Prasad, Bijan Saha, Amit Kumar
Summary: The study found that early ventricular dysfunction is a potential prognostic factor in congenital diaphragmatic hernia. While evidence supports the predictive value of echocardiographic measurements in predicting survival and ECMO requirement in newborns with CDH, the overall certainty of the evidence was graded as very low for survival and low for ECMO, highlighting the need for standardized echocardiographic measurement reporting and high-quality studies to further elucidate its prognostic significance.
EUROPEAN JOURNAL OF PEDIATRICS
(2022)
Article
Pediatrics
Judith Leyens, Lukas Schroeder, Annegret Geipel, Christoph Berg, Bartolomeo Bo, Lotte Lemloh, Neil Patel, Andreas Mueller, Florian Kipfmueller
Summary: This study aims to describe the early course of pulmonary hypertension (PH) in infants with congenital diaphragmatic hernia (CDH) and its relation to prognostic markers and outcome measures. The severity of PH varied significantly in the first 48 hours of life. Patients with mild/no PH had a better prognosis, while patients with severe PH had a higher risk of mortality and ECMO. Assessing PH within 2-6 hours is crucial in the care for CDH neonates.
FRONTIERS IN PEDIATRICS
(2023)
Article
Surgery
Tim Jancelewicz, Max R. Langham, Mary E. Brindle, Zachary E. Stiles, Pamela A. Lally, Lei Dong, Jim Y. Wan, Yigit S. Guner, Matthew T. Harting
Summary: The use of ECMO is associated with excess mortality for low- and intermediate-risk neonates with CDH. However, it provides a significant survival advantage for high-risk infants, and this advantage is strongly influenced by center CDH volume and ECMO experience.
Article
Biotechnology & Applied Microbiology
Shiho Yoshida, Alexander M. Kreger, George K. Gittes
Summary: We propose that intra-amniotic sildenafil administration is an effective prenatal therapy for CDH-induced pulmonary hypertension. Intra-amniotic sildenafil treatment attenuated peripheral vascular muscularization, enhanced pulmonary blood flow, and improved lung architecture in CDH fetuses. Early intervention with intra-amniotic sildenafil treatment may be preferable to accelerate lung development and improve prognosis in CDH cases.
FRONTIERS IN BIOENGINEERING AND BIOTECHNOLOGY
(2023)
Article
Pediatrics
Dalya Munves Ferguson, Vikas S. Gupta, Pamela A. Lally, Matias Luco, KuoJen Tsao, Kevin P. Lally, Neil Patel, Matthew T. Harting
Summary: The study found that early CDH-PH is associated with increased risk for inpatient mortality or oxygen support at 30 days. Infants with moderate CDH-PH and severe CDH-PH had a higher incidence of the primary outcome, while severe CDH-PH was also associated with a higher likelihood of requiring ECLS support.
Article
Pediatrics
Song-Ming Hong, Xiu-Hua Chen, Si-Jia Zhou, Jun-Jie Hong, Yi-Rong Zheng, Qiang Chen, Jin-Xi Huang
Summary: Congenital diaphragmatic hernia (CDH) is a structural defect in which visceral organs protrude into the pleural cavity. Up to 30% of newborns with CDH require ECMO support. Cardiopulmonary function worsens after surgical CDH repair, and timely and effective treatments are needed for sudden cardiopulmonary failure.
FRONTIERS IN PEDIATRICS
(2023)
Article
Pediatrics
Arthur Gavotto, Pascal Amedro, Gilles Cambonie
Summary: We report a case of a neonate with a severe pulmonary hypertension associated with an abnormal origin of the right pulmonary artery from the right brachiocephalic artery, in a context of left congenital diaphragmatic hernia (CDH). To the best of our knowledge, this combination of malformations has not been reported before.
Article
Critical Care Medicine
Enrico Danzer, Shavonne L. Massey, Sabrina J. Flohr, Leny Mathew, Casey Hoffman, Abigail Abramson, Paige Selenski, Caroline E. Canning, Elizabeth Eppley, James T. Connelly, Lisa Herkert, Natalie E. Rintoul, N. Scott Adzick, Nicholas S. Abend, Holly L. Hedrick
Summary: The study aimed to determine the prevalence of electrographic seizures and their association with adverse outcomes in neonates with congenital diaphragmatic hernia (CDH) receiving extracorporeal membrane oxygenation (ECMO). The study found that nearly one in five neonates developed electrographic seizures during ECMO and these seizures were associated with greater odds of adverse outcomes.
PEDIATRIC CRITICAL CARE MEDICINE
(2023)
Review
Critical Care Medicine
K. Taylor Wild, Holly L. Hedrick, Anne M. Ades, Maria V. Fraga, Catherine M. Avitabile, Juliana S. Gebb, Edward R. Oliver, Kristen Coletti, Erin M. Kesler, K. Taylor Van Hoose, Howard B. Panitch, Sandy Johng, Renee P. Ebbert, Lisa M. Herkert, Casey Hoffman, Deanna Ruble, Sabrina Flohr, Tom Reynolds, Melissa Duran, Audrey Foster, Rebecca S. Isserman, Emily A. Partridge, Natalie E. Rintoul
Summary: Infants with congenital diaphragmatic hernia require comprehensive multidisciplinary care. Despite some advances, further improvement is needed in treatment outcomes.
JOURNAL OF INTENSIVE CARE MEDICINE
(2023)
Article
Pediatrics
Christian Wegele, Yannick Schreiner, Alba Perez Ortiz, Svetlana Hetjens, Christiane Otto, Michael Boettcher, Thomas Schaible, Neysan Rafat
Summary: Early initiation of ECMO is associated with a beneficial outcome in patients with congenital diaphragmatic hernia (CDH), while ECMO initiation >120 hours after birth is associated with increased mortality.
Article
Pediatrics
Alba Perez Ortiz, Carl E. Dempfle, Toni Jung, Thalia Doniga, Christel Weiss, Svetlana Hetjens, Thomas Schaible, Neysan Rafat
Summary: The study found that anti-factor Xa levels correlate better with heparin dose than activated clotting time in newborns with congenital diaphragmatic hernia on ECMO support. Using anti-factor Xa assay instead of activated clotting time for dosing of unfractionated heparin could reduce thrombotic complications in this population. Thromboelastometry showed no additional benefit for this purpose.
FRONTIERS IN PEDIATRICS
(2021)
Article
Pharmacology & Pharmacy
Alecsander F. Bressan, Victoria Oliveira Maia, Beatriz de Souza Rodrigues, Giuliana Bertozi, Sabrina S. Batah, Alexandre T. Fabro, Gloria Pelizzo, Ugo Maria Pierucci, Rita C. Tostes, Lourenco Sbragia, Fernando S. Carneiro
Summary: Congenital diaphragmatic hernia (CDH) can cause abnormalities in the pulmonary arteries of both the contralateral and ipsilateral sides of the diaphragm. Nitric oxide (NO) is commonly used to treat CDH but may not always be effective. This study investigated the different responses of the left and right pulmonary arteries to a NO donor in a rabbit model of left CDH. The results showed that newborns with CDH had increased vasorelaxant responses to the NO donor compared to the control group, possibly due to increased cGMP mobilization.
PHARMACOLOGICAL RESEARCH
(2023)
Article
Pediatrics
Lukas Schroeder, Flaminia Pugnaloni, Ramona Dolscheid-Pommerich, Annegret Geipel, Christoph Berg, Stefan Holdenrieder, Andreas Mueller, Florian Kipfmueller
Summary: This study analyzed the use of CA125 as a cardiac biomarker in infants with congenital diaphragmatic hernia (CDH). The results showed that CA125 correlated significantly with echocardiographic markers, disease severity, and the risk of ECMO and in-hospital mortality in CDH infants.
PEDIATRIC RESEARCH
(2023)
Article
Pediatrics
Lukas Schroeder, Manuel Kuelshammer, Ramona Dolscheid-Pommerich, Stefan Holdenrieder, Andreas Mueller, Florian Kipfmueller
Summary: The present study aimed to investigate the suitability of NT-proBNP as a biomarker for predicting extubation failure in critically ill neonates with pulmonary hypertension or ventricular dysfunction. The results showed that there were differences in the absolute values of NT-proBNP before and after extubation, and the Zlog transformation of NT-proBNP could better predict the occurrence of extubation failure.
PEDIATRIC PULMONOLOGY
(2023)
Article
Cardiac & Cardiovascular Systems
Lukas Schroeder, Paulina Monno, Martin Unger, Jakob Ackerl, Olga Shatilova, Joachim Schmitt, Till Dresbach, Andreas Mueller, Florian Kipfmueller
Summary: Landiolol hydrochloride is an effective treatment option for sinus tachycardia and heart failure in critically ill infants. It successfully reduces heart rate without severe adverse effects and leads to improvements in ventricular dysfunction and pulmonary hypertension.
Review
Genetics & Heredity
Flaminia Pugnaloni, Roberta Onesimo, Rita Blandino, Carolina Putotto, Paolo Versacci, Angelica Bibiana Delogu, Chiara Leoni, Valentina Trevisan, Ileana Croci, Federica Cali, Maria Cristina Digilio, Giuseppe Zampino, Bruno Marino, Giulio Calcagni
Summary: Chromosome 9p deletion syndrome is a rare autosomal dominant disorder associated with a wide range of clinical features, including congenital heart defects. A study was conducted on a cohort of 10 patients with confirmed 9p deletion syndrome, and it was found that even patients without major heart defects may exhibit subclinical cardiac structural changes and dysfunction. These findings emphasize the importance of comprehensive cardiac assessment and long-term follow-up in patients with 9p deletion syndrome.
Letter
Critical Care Medicine
Flaminia Pugnaloni, Bartolomeo Bo, Lennart Hale, Irma Capolupo, Andrea Dotta, Pietro Bagolan, Lukas Schroeder, Christoph Berg, Annegret Geipel, Andreas Mueller, Neil Patel, Florian Kipfmueller
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE
(2023)
Review
Biochemistry & Molecular Biology
Flaminia Pugnaloni, Irma Capolupo, Neil Patel, Paola Giliberti, Andrea Dotta, Pietro Bagolan, Florian Kipfmueller
Summary: This review highlights the importance of miRNAs in CDH-related PH and summarizes the findings from animal and human CDH studies. The focus on epigenetic modulators of CDH-PH offers potential for developing innovative diagnostic tools and treatment approaches, and enhances researchers' understanding of CDH pathophysiology.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
Article
Pediatrics
Florian Kipfmueller, Bartolomeo Bo, Joachim Schmitt, Hemmen Sabir, Lukas Schroeder, Andreas Mueller, Till Dresbach
Summary: This study retrospectively analyzed the use of ultrasound-guided percutaneous, Seldinger technique for VV ECMO cannulation in neonates. The results showed that a majority of the neonates (72%) were cannulated with a 13 French bicaval dual-lumen cannula using the multisite approach. The cannula placement was successful and the complications associated with cannulation and decannulation were minimal.
PEDIATRIC PULMONOLOGY
(2023)
Review
Infectious Diseases
Domenico Umberto De Rose, Flaminia Pugnaloni, Ludovica Martini, Iliana Bersani, Maria Paola Ronchetti, Andrea Diociaiuti, May El Hachem, Andrea Dotta, Cinzia Auriti
Summary: Staphylococcal infections in neonates can cause skin blistering, and early antibiotic therapy is beneficial for limiting infection spread and improving outcomes. This review examines the management of Staphylococcal infections in neonatal skin, discussing the appropriate clinical approach for four cases of neonatal blistering diseases with Staphylococcal infections.
Article
Medicine, General & Internal
Philipp Kosian, Karl-Philipp Gloning, Ute Germer, Brigitte Strizek, Christoph Berg, Ulrich Gembruch, Annegret Geipel
Summary: This study evaluated the risk for procedure-related complications and fetal loss in correlation to maternal risk factors in the context of cordocentesis. The results demonstrate that cordocentesis is a safe way to further fetal diagnostics when performed by experienced operators, with a low risk of complications.
JOURNAL OF CLINICAL MEDICINE
(2023)
Article
Obstetrics & Gynecology
Pauline Siebers, Ulrich Gembruch, Waltraut Maria Merz, Florian Recker, Andreas Mueller, Brigitte Strizek, Annegret Geipel, Christoph Berg, Eva Christin Weber
Summary: The purpose of this study was to investigate the effect of anemia and intrauterine transfusion (IUT) on NT-proBNP concentrations in fetuses with anemia and establish gestational age-dependent reference values of a control group. NT-proBNP levels in non-anemic fetuses are higher than in postnatal life, decreasing with ongoing pregnancy. Anemia severity correlates with circulating NT-proBNP levels. Highest concentrations occur in fetuses with hydrops and with PVB19 infection, respectively. Treatment by IUT leads to a normalization of NT-proBNP concentrations, so the measurement of its levels may be useful in therapy monitoring.
ARCHIVES OF GYNECOLOGY AND OBSTETRICS
(2023)
Article
Obstetrics & Gynecology
I. Gottschalk, A. Walter, T. Menzel, E. C. Weber, S. Wendt, N. Sreeram, U. Gembruch, C. Berg, J. S. Abel
Summary: This study retrospectively analyzed prenatal ultrasound and imaging data of fetuses with dextro-transposition of the great arteries (d-TGA) to identify reliable predictive markers for balloon atrial septostomy (BAS) in newborns. They found that a FO diameter < 7 mm, a fixed FO flap, and a hypermobile FO flap were highly significant markers for a restrictive FO. These markers could accurately predict the need for urgent BAS in fetuses with restrictive FO.
ARCHIVES OF GYNECOLOGY AND OBSTETRICS
(2023)
Review
Obstetrics & Gynecology
Carolin Schroeder, Elina Calite, Paul Boeckenhoff, Thomas Buettner, Johannes Stein, Ulrich Gembruch, Brigitte Strizek
Summary: Less than a dozen cases of psoas abscesses in pregnancy have been reported in the literature. We reviewed the literature and found ten case reports of psoas abscesses during pregnancy. The main treatment options for psoas abscesses include antibiotics and abscess drainage.
ARCHIVES OF GYNECOLOGY AND OBSTETRICS
(2023)
Article
Pediatrics
Florian Kipfmueller, Judith Leyens, Flaminia Pugnaloni, Bartolomeo Bo, Tamara Grass, Lotte Lemloh, Lukas Schroeder, Patrizia Nitsch-Felsecker, Christoph Berg, Andreas Heydweiller, Brigitte Strizek, Andreas Mueller
Summary: This study explored the feasibility and outcomes of a spontaneous breathing approach (SBA) versus immediate intubation in neonates with prenatally diagnosed very mild congenital diaphragmatic hernia (CDH). The results showed that SBA was successful in most cases, and it was associated with shorter hospital stay, less mechanical ventilation and oxygen supplementation, as well as improved early feeding tolerance compared to standard treatment.
PEDIATRIC PULMONOLOGY
(2023)