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pulmonary hypertension in infants

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https://www.readbyqxmd.com/read/28923474/beyond-the-inhaled-nitric-oxide-in-persistent-pulmonary-hypertension-of-the-newborn
#1
REVIEW
Mei-Yin Lai, Shih-Ming Chu, Satyan Lakshminrusimha, Hung-Chih Lin
Persistent pulmonary hypertension (PPHN) is a consequence of failed pulmonary vascular transition at birth and leads to pulmonary hypertension with shunting of deoxygenated blood across the ductus arteriosus (DA) and foramen ovale (FO) resulting in severe hypoxemia, and it may eventually lead to life-threatening circulatory failure. PPHN is a serious event affecting both term and preterm infants in the neonatal intensive care unit. It is often associated with diseases such as congenital diaphragmatic hernia, meconium aspiration, sepsis, congenital pneumonia, birth asphyxia and respiratory distress syndrome...
August 10, 2017: Pediatrics and Neonatology
https://www.readbyqxmd.com/read/28922348/stem-cell-biology-and-regenerative-medicine-for-neonatal-lung-diseases
#2
REVIEW
Martin Kang, Bernard Thébaud
Lung diseases remain one of the main causes of morbidity and mortality in neonates. Cell therapy and regenerative medicine have the potential to revolutionize the management of life-threatening and debilitating lung diseases that currently lack effective treatments. Over the past decade, the repair capabilities of stem/progenitor cells has been harnessed to prevent/rescue lung damage in experimental neonatal lung diseases. Mesenchymal stromal cells and amnion epithelial cells exert pleiotropic effects and represent ideal therapeutic cells for bronchopulmonary dysplasia, a multifactorial disease...
September 18, 2017: Pediatric Research
https://www.readbyqxmd.com/read/28917066/effectiveness-of-the-critical-congenital-heart-disease-screening-program-for-early-diagnosis-of-cardiac-abnormalities-in-newborn-infants
#3
Abdulmajid M Almawazini, Hamdi K Hanafi, Hasan A Madkhali, Noura B Majrashi
To evaluate the effectiveness of critical congenital heart disease (CCHD) screening program for early diagnosis of cardiac anomalies in newborn infants.  Methods: This is a hospital-based prospective cross-sectional study conducted in the Pediatric and Neonatology Department, King Fahad Hospital at  Albaha, Saudi Arabia, between February 2016 and February 2017. Results: We screened 2961 (95.4%) of 3103 patients in a nursery unit; 142 (4.6%) patients were not screened. The test was positive in 114 (3.9%) patients and negative in 2847 (96...
October 2017: Saudi Medical Journal
https://www.readbyqxmd.com/read/28880261/bronchopulmonary-dysplasia-associated-pulmonary-hypertension-clues-from-placental-pathology
#4
A M Kunjunju, K R Gopagondanahalli, Y Chan, A Sehgal
OBJECTIVES: Bronchopulmonary dysplasia (BPD) and the associated complication of pulmonary hypertension (PH) leads to increased mortality and a longer length of stay among survivors. Placental histopathology may give early clues of subsequent events. The objective was to evaluate the relationship of maternal vascular underperfusion (MVU) changes on placental histopathology with subsequent development of BPD-associated PH in a cohort of extremely premature infants. STUDY DESIGN: In a cohort of preterm infants '⩽28 weeks' gestational age (GA) and with 'severe' BPD, this retrospective study evaluated specific placental histopathological changes and assessed the relationship with subsequent development of PH...
September 7, 2017: Journal of Perinatology: Official Journal of the California Perinatal Association
https://www.readbyqxmd.com/read/28868233/intestinal-perforation-during-the-stabilization-period-in-a-preterm-infant-with-congenital-diaphragmatic-hernia
#5
Zbyněk Straňák, Karel Pýcha, Simona Feyereislova, Jaroslav Feyereisl, Michal Rygl
Background  Delayed surgery after stabilization of infants with congenital diaphragmatic hernia (CDH) is an accepted strategy. However, the evidence favoring delayed versus immediate surgical repair is limited. We present an extremely rare case of a very low-birth-weight infant with prenatally diagnosed left-sided CDH and unexpected transmural bowel perforations developing within the postnatal stabilization period. Case Report  A neonate born at 31st week of gestation with a birth weight of 1,470 g with antenatally diagnosed left-sided CDH presented with bowel dilation leading to transmural bowel perforations on the 2nd day of life...
January 2017: European Journal of Pediatric Surgery Reports
https://www.readbyqxmd.com/read/28838007/role-of-early-pulmonary-hypertension-as-a-risk-factor-for-late-pulmonary-hypertension-in-extremely-preterm-infants
#6
Dinushan C Kaluarachchi, Kaitlin M Woo, Tarah T Colaizy
No abstract text is available yet for this article.
August 24, 2017: American Journal of Perinatology
https://www.readbyqxmd.com/read/28837121/diagnostic-approach-to-pulmonary-hypertension-in-premature-neonates
#7
REVIEW
Vasantha H S Kumar
Bronchopulmonary dysplasia (BPD) is a form of chronic lung disease in premature infants following respiratory distress at birth. With increasing survival of extremely low birth weight infants, alveolar simplification is the defining lung characteristic of infants with BPD, and along with pulmonary hypertension, increasingly contributes to both respiratory morbidity and mortality in these infants. Growth restricted infants, infants born to mothers with oligohydramnios or following prolonged preterm rupture of membranes are at particular risk for early onset pulmonary hypertension...
August 24, 2017: Children
https://www.readbyqxmd.com/read/28828053/ultrasound-prenatal-diagnosis-and-emergency-interventional-radiologic-therapy-of-galen-aneurysmal-malformation-in-a-newborn
#8
Roxana Elena Bohiltea, Natalia Turcan, Cristian Mihalea, Bogdan Dorobat, Eliza Elena Cinteza, Adriana Dan, Magdalena Mihai, Adela Dimitriade, Cristian Boros, Mircea Dumitru, Ionut Gobej, Octavian Munteanu, Monica Mihaela Cirstoiu
Located under the cerebral hemispheres and draining the anterior and central regions of the brain into the sinus of the posterior cerebral fossa, the vein of Galen aneurysmal malformation is considered to be a rare cause of hydrocephaly. The presence of this condition in the neonatal period typically includes intractable heart failure and a poor prognosis. We report a case of aneurysm of the vein of Galen diagnosed prenatally at 28 weeks of gestation, with the delivery at term by caesarean section of a female infant...
December 2016: Mædica
https://www.readbyqxmd.com/read/28822439/-multisystemic-smooth-muscle-dysfunction-syndrome-in-children-a-case-report-and-literature-review
#9
Y L Zhou, Y Y Zhang, B L Cheng, D Xu, L F Tang, Z M Chen
Objective: To analyze the clinical characteristics and diagnosis of multisystemic smooth muscle dysfunction syndrome(MSMDS). Method: Clinical data of a case diagnosed as MSMDS and hospitalized in our hospital in July 2016 was retrospectively analyzed. Literature search was performed at databases of PubMed, Wanfang, China National Knowledge Infrastructure and VIP with the key words "multisystemic smooth muscle dysfunction syndrome" "ACTA2" . The literature retrieval was confined from January 1980 to November 2016...
August 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/28808495/the-most-frequent-abca3-nonsense-mutation-p-tyr1515-y1515x-causing-lethal-neonatal-respiratory-failure-in-a-term-neonate
#10
AlNashmi AlAnazi, Ralph Epaud, Humariya Heena, Alix de Becdelievre, Abeer Mohammad Miqdad, Pascale Fanen
Defects in the surfactant biosynthesis are associated with respiratory distress syndrome, commonly occurring in premature infants due to lung immaturity. However, interstitial lung diseases have also been observed in full-term infants with mutations in the SFTPC, SFTPB, NKX2-1, or ABCA3 genes, involved in the surfactant metabolism. Herein, we report a newborn baby with neonatal respiratory distress and diffuse lung disease caused by ABCA3 mutation. The baby died at 5 weeks of age after developing pulmonary hypertension...
July 2017: Annals of Thoracic Medicine
https://www.readbyqxmd.com/read/28792962/aberrant-cgmp-signaling-persists-during-recovery-in-mice-with-oxygen-induced-pulmonary-hypertension
#11
Marta Perez, Keng Jin Lee, Herminio J Cardona, Joann M Taylor, Mary E Robbins, Gregory B Waypa, Sara K Berkelhamer, Kathryn N Farrow
Bronchopulmonary dysplasia (BPD), a common complication of preterm birth, is associated with pulmonary hypertension (PH) in 25% of infants with moderate to severe BPD. Neonatal mice exposed to hyperoxia for 14d develop lung disease similar to BPD, with evidence of associated PH. The cyclic guanosine monophosphate (cGMP) signaling pathway has not been well studied in BPD-associated PH. In addition, there is little data about the natural history of hyperoxia-induced PH in mice or the utility of phosphodiesterase-5 (PDE5) inhibition in established disease...
2017: PloS One
https://www.readbyqxmd.com/read/28782495/rapidly-progressive-pulmonary-veno-occlusive-disease-in-an-infant-with-down-syndrome
#12
Jun Muneuchi, Shinichiro Oda, Daisuke Shimizu
A 4-month-old girl with Down syndrome showed unexpected deterioration of pulmonary hypertension. Despite aggressive pulmonary vasodilation therapy, the patient died at 5 months of age. Lung autopsy showed that the pulmonary veins were obliterated by intimal fibrous thickening, and the media of the veins was arterialised with an increase in elastic fibres. Pulmonary veno-occlusive disease should be considered in the management of individuals with Down syndrome.
September 2017: Cardiology in the Young
https://www.readbyqxmd.com/read/28777888/sildenafil-for-pulmonary-hypertension-in-neonates
#13
REVIEW
Lauren E Kelly, Arne Ohlsson, Prakeshkumar S Shah
BACKGROUND: Persistent pulmonary hypertension in the neonate (PPHN) is associated with high mortality. Currently, the therapeutic mainstay for PPHN consists of assisted ventilation and administration of inhaled nitric oxide (iNO). However, nitric oxide is costly, and its use may not be appropriate in resource-poor settings. Approximately 30% of patients fail to respond to iNO. High concentrations of phosphodiesterases in the pulmonary vasculature have led to the use of phosphodiesterase inhibitors such as sildenafil or milrinone...
August 4, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28777121/raf1-variants-causing-biventricular-hypertrophic-cardiomyopathy-in-two-preterm-infants-further-phenotypic-delineation-and-review-of-literature
#14
Danielle Thompson, Jessica Patrick-Esteve, Jeffrey W Surcouf, Dana Rivera, Bianca Castellanos, Pooja Desai, Christian Lilje, Yves Lacassie, Michael Marble, Regina Zambrano
Noonan syndrome (NS) is an autosomal dominant disorder characterized by distinctive facial features, short neck, short stature, congenital heart defects, pectus deformities, and variable developmental delays. NS is genetically heterogeneous as pathogenic variants in several genes involved in the Ras/mitogen-activated protein kinase pathway have been associated with a NS phenotype. Overall, 50% of patients harbor pathogenic variants in PTPN11, whereas 3-17% of patients have variants in RAF1. We present two premature neonates with progressive biventricular hypertrophy found to have RAF1 variants in the CR2 domain...
August 3, 2017: Clinical Dysmorphology
https://www.readbyqxmd.com/read/28771211/the-fetus-can-teach-us-oxygen-and-the-pulmonary-vasculature
#15
REVIEW
Payam Vali, Satyan Lakshminrusimha
Neonates suffering from pulmonary hypertension of the newborn (PPHN) continue to represent an important proportion of patients requiring intensive neonatal care, and have an increased risk of morbidity and mortality. The human fetus has evolved to maintain a high pulmonary vascular resistance (PVR) in utero to allow the majority of the fetal circulation to bypass the lungs, which do not participate in gas exchange, towards the low resistance placenta. At birth, oxygen plays a major role in decreasing PVR to enhance pulmonary blood flow and establish the lungs as the organ of gas exchange...
August 3, 2017: Children
https://www.readbyqxmd.com/read/28768234/human-mesenchymal-stem-cells-ameliorate-experimental-pulmonary-hypertension-induced-by-maternal-inflammation-and-neonatal-hyperoxia-in-rats
#16
Chung-Ming Chen, Willie Lin, Liang-Ti Huang, Hsiu-Chu Chou
Pulmonary hypertension is a critical problem in infants with bronchopulmonary dysplasia. This study determined the therapeutic effects of human mesenchymal stem cells (MSCs) on pulmonary hypertension in an animal model. Pregnant Sprague-Dawley rats were intraperitoneally injected with lipopolysaccharide (LPS, 0.5 mg/kg/day) on gestational days 20 and 21. The pups were randomly assigned to two treatment conditions: room air (RA) or an O2-enriched atmosphere. On postnatal day 5, they were intratracheally transplanted with human MSCs (3 × 105 and 1 × 106 cells) in 0...
July 19, 2017: Oncotarget
https://www.readbyqxmd.com/read/28764231/large-isolated-major-aortopulmonary-collateral-artery-causing-severe-pulmonary-hypertension-in-an-infant-a-rare-and-challenging-diagnosis
#17
Brajesh Kumar Kunwar, Snigdha Paddalwar, Mahesh Ghogare
Major Aortopulmonary Collateral Artery (MAPCA) as an isolated congenital anomaly, without evidence of any structural heart disease, is a very rare observation. Previously published reports indicate that symptomatic infants with large isolated MAPCA usually present with congestive heart failure or recurrent respiratory tract infections. To the best of our knowledge, the present case of an infant with large isolated MAPCA is a unique case with a diagnostic dilemma due to presentation with severe pulmonary hypertension as a predominant sign...
June 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28745124/dysfunction-of-the-foetal-arterial-duct-results-in-a-wide-spectrum-of-cardiovascular-pathology
#18
Marc Gewillig, Stephen C Brown, Mieke Roggen, Benedicte Eyskens, Ruth Heying, Patrice Givron, Bjorn Cools, Luc de Catte
OBJECTIVE: Foetal ductal problems may have various cardiopulmonary consequences. This study aimed to identify the spectrum of ductus arteriosus (DA) dysfunction (closure, constriction, kinking, aneurysm and thrombosis) and the resultant clinical and echocardiographic presentation in foetuses and neonates. METHODS AND RESULTS: This is a retrospective analysis of serial pre- and post-natal data of 27 cases of foetal ductal dysfunction diagnosed at a median gestational age of 33 weeks (range 20-39)...
July 26, 2017: Acta Cardiologica
https://www.readbyqxmd.com/read/28742524/early-postnatal-echocardiographic-assessment-of-pulmonary-blood-flow-in-newborns-with-congenital-diaphragmatic-hernia
#19
Florian Kipfmueller, Katrin Heindel, Lukas Schroeder, Christoph Berg, Oliver Dewald, Heiko Reutter, Peter Bartmann, Andreas Mueller
OBJECTIVE: Echocardiography is the most important tool to assess infants with congenital diaphragmatic hernia (CDH) for pulmonary hypertension (PH). The pattern of blood flow in the pulmonary artery [described as time to peak velocity (TPV)/right ventricular ejection time (RVET) ratio] provides distinct information about pulmonary arterial pressure. The aim of our study was to investigate the correlation of TPV/RVET measurements with the most commonly used classification system for PH in CDH newborns and the association of these measurements with outcome parameters...
July 25, 2017: Journal of Perinatal Medicine
https://www.readbyqxmd.com/read/28737730/sildenafil-in-infants-and-children
#20
REVIEW
Larisa Simonca, Robert Tulloh
Pulmonary arterial hypertension (PAH) management has been transformed in recent times with the advent of cheap and effective diagnostic tools and therapy. Sildenafil, a phosphodiesterase-V inhibitor, has been at the centre of this treatment, and its success in treating PAH has led to its widespread uptake in adult and paediatric pulmonary hypertension (PH), as a first line treatment choice. This might apply to persistent pulmonary hypertension of the newborn (PPHN) or bronchopulmonary dysplasia, as well as to more complex diseases, such as idiopathic pulmonary hypertension...
July 24, 2017: Children
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