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https://www.readbyqxmd.com/read/28737730/sildenafil-in-infants-and-children
#1
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
https://www.readbyqxmd.com/read/28735030/effect-of-bosentan-therapy-in-persistent-pulmonary-hypertension-of-the-newborn
#2
Gunlawadee Maneenil, Anucha Thatrimontrichai, Waricha Janjindamai, Supaporn Dissaneevate
BACKGROUND: Persistent pulmonary hypertension of the newborn (PPHN) contributes to neonatal hypoxemia and is associated with a high mortality. Some PPHN patients are unresponsive to inhaled nitric oxide (iNO). Bosentan, an oral endothelin-1 receptor antagonist, reduces pulmonary vascular resistance and hence may play a role in the treatment of PPHN. METHODS: A retrospective medical records review was performed in newborns who received oral bosentan as an adjunctive therapy for treatment of PPHN between January 2013 and February 2016 at the neonatal intensive care unit of Songklanagarind Hospital...
July 11, 2017: Pediatrics and Neonatology
https://www.readbyqxmd.com/read/28714905/effects-of-eicosapentaenoic-acid-epa-and-docosahexaenoic-acid-dha-on-fetal-pulmonary-circulation-an-experimental-study-in-fetal-lambs
#3
Dyuti Sharma, Estelle Aubry, Thavarak Ouk, Ali Houeijeh, Véronique Houfflin-Debarge, Rémi Besson, Philippe Deruelle, Laurent Storme
Background: Persistent pulmonary hypertension of the newborn (PPHN) causes significant morbidity and mortality in neonates. n-3 Poly-unsaturated fatty acids have vasodilatory properties in the perinatal lung. We studied the circulatory effects of eicosapentaenoic acid (EPA) and docosahexaenoic acid (DHA) in fetal sheep and in fetal pulmonary arterial rings. Methods: At 128 days of gestation, catheters were placed surgically in fetal systemic and pulmonary circulation, and a Doppler probe around the left pulmonary artery (LPA)...
July 16, 2017: Nutrients
https://www.readbyqxmd.com/read/28658134/treprostinil-for-persistent-pulmonary-hypertension-of-the-newborn-with-early-onset-sepsis-in-preterm-infant-2-case-reports
#4
Bo Young Park, Sung-Hoon Chung
RATIONALE: Persistent pulmonary hypertension of the newborn (PPHN) is a syndrome of failed circulatory adaptation at birth with persisting increased pulmonary vascular resistance that is associated with high mortality rates especially in preterm infants. PATIENT CONCERNS: We reported 2 cases of PPHN in preterm infants with respiratory distress syndrome and early onset sepsis refractory to therapy with vasopressors, inotropes, and inhaled nitric oxide (iNO), in whom treatment with treprostinil was successful...
June 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28627661/altered-expression-of-ppar%C3%A2-%C3%AE-and-trpc-in-neonatal-rats-with-persistent-pulmonary-hypertension
#5
Yanna Du, Jianhua Fu, Li Yao, Lin Qiao, Na Liu, Yujiao Xing, Xindong Xue
Persistent pulmonary hypertension of the newborn (PPHN) is a life‑threatening disease that is commonly observed in the neonatal intensive care unit. PPHN is pathologically characterized by pulmonary vascular remodeling and, in particular, pulmonary artery smooth muscle cell (PASMC) proliferation. Decreased expression levels of peroxisome proliferator‑activated receptor γ (PPAR‑γ), which is a member of the nuclear receptor hormone superfamily, in combination with elevated expressions of transient receptor potential cation channel, subfamily C, member 1 (TRPC1) and TRPC6 contributes to the PASMC proliferation and excessive pulmonary vascular remodeling in adult pulmonary hypertension (PH)...
August 2017: Molecular Medicine Reports
https://www.readbyqxmd.com/read/28609431/polymorphisms-in-urea-cycle-enzyme-genes-are-associated-with-persistent-pulmonary-hypertension-of-the-newborn
#6
Dinushan C Kaluarachchi, Caitlin J Smith, Jonathan M Klein, Jeffrey C Murray, John M Dagle, Kelli K Ryckman
BACKGROUND: Persistent pulmonary hypertension of the newborn (PPHN) is characterized by elevated pulmonary vascular resistance. Endogenous nitric oxide is critical for regulation of pulmonary vascular resistance. Nitric oxide is generated from L -arginine, supplied by the urea cycle (UC). We hypothesized that polymorphisms in UC enzyme genes and low concentrations of UC intermediates are associated with PPHN. METHODS: We performed a family based candidate gene analysis to study 48 single nucleotide polymorphisms (SNPs) in 6 UC enzyme genes...
June 13, 2017: Pediatric Research
https://www.readbyqxmd.com/read/28494828/treatment-of-persistent-pulmonary-hypertension-of-the-newborn-use-of-pulmonary-vasodilators-in-term-neonates
#7
Caitlyn Luecke, Christopher McPherson
Persistent pulmonary hypertension of the newborn (PPHN) represents a challenging condition associated with significant morbidity. A successful transition from intrauterine to extrauterine life is contingent on adequate pulmonary vasodilation. Several pathophysiologies contribute to the failure of this cascade and may result in life-threatening hypoxia and acidosis in the newborn. Management includes optimal respiratory support, adequate sedation and analgesia, and support of vascular tone and cardiac function...
May 1, 2017: Neonatal Network: NN
https://www.readbyqxmd.com/read/28489830/-clinical-and-laboratory-characteristics-of-patients-with-pulmonary-hypertension-and-pulmonary-vascular-complications-hospitalized-at-the-instituto-nacional-de-salud-del-ni%C3%A3-o
#8
Alexis Jose Ormeño Julca, Carlos Melchor Alvarez Murillo, Pedro Miguel Amoretti Alvino, Angel Aladino Florian Florian, Rosa Aurora Castro Johanson, Maria Danisa Celi Perez, Olga Rocío Huamán Prado
The hepatopulmonary syndrome (HPS) and portopulmonary hypertension (PPHN) are distinct pulmonary vascular complications of portal hypertension (PHT) and are associated with increased morbidity and mortality. OBJECTIVES: To describe the clinical and laboratory characteristics of patients with pulmonary hypertension and pulmonary vascular complications hospitalized at the Instituto Nacional de Salud del Niño. MATERIALS AND METHODS: We included patients with HTP hospitalized from January 2012 to June 2013 and that during its evolution progressed with SHP or HTPP...
January 2017: Revista de Gastroenterología del Perú: órgano Oficial de la Sociedad de Gastroenterología del Perú
https://www.readbyqxmd.com/read/28407823/-application-of-humidified-high-flow-nasal-cannula-in-neonates-with-meconium-aspiration-syndrome-and-pulmonary-hypertension-after-extubation
#9
Xiao-Yan Gao, Lin Feng, Yu-Fen Qiu, Xin-Nian Pan
OBJECTIVE: To investigate the clinical value of humidified high-flow nasal cannula (HHFNC) as a respiratory support after extubation by comparing it with nasal continuous positive airway pressure (NCPAP) in neonates with meconium aspiration syndrome (MAS) and persistent pulmonary hypertension of the newborn (PPHN). METHODS: A total of 78 neonates with MAS and PPHN were randomly administered with HHFNC or NCPAP immediately after extubation. The following indices were compared between the two groups: blood gas parameters, duration of noninvasive ventilation, rate of extubation failure, and incidence of complications, such as nasal damage, abdominal distension, and intraventricular hemorrhage...
April 2017: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
https://www.readbyqxmd.com/read/28382839/abnormal-muscularization-of-intra-acinar-pulmonary-arteries-in-2-cases-presenting-as-sudden-infant-death-sids
#10
Zainun Khairul, Hope Kirsten, Andrew G Nicholson, Marta C Cohen
Abnormal muscularization of acinar arteries is the hallmark of persistent pulmonary hypertension of newborn (PPHN), an uncommon disease with high rate of morbidity and mortality. PPHN presents with signs of respiratory distress immediately following birth. We herein report 2 cases presenting as a witnessed sudden unexpected death in the late neonatal period, preceded by respiratory deterioration and in whom the presence of abnormal muscularization of the acinar pulmonary arteries was reminiscent of PPHN. The significance of this report is twofold: to increase the awareness among pediatricians and pathologists of this feature that can present in some cases of sudden unexpected death in infancy/Sudden Infant Death Syndrome (SIDS), and to highlight the importance of performing a thorough autopsy in order to identify the abnormality...
January 1, 2017: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/28342684/persistent-pulmonary-hypertension-of-the-newborn
#11
REVIEW
Mamta Fuloria, Judy L Aschner
Failure of the normal circulatory adaptation to extrauterine life results in persistent pulmonary hypertension of the newborn (PPHN). Although this condition is most often secondary to parenchymal lung disease or lung hypoplasia, it may also be idiopathic. PPHN is characterized by elevated pulmonary vascular resistance with resultant right-to-left shunting of blood and hypoxemia. Although the preliminary diagnosis of PPHN is often based on differential cyanosis and labile hypoxemia, the diagnosis is confirmed by echocardiography...
March 23, 2017: Seminars in Fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/28331629/congenital-diaphragmatic-hernia-a-review
#12
REVIEW
Praveen Kumar Chandrasekharan, Munmun Rawat, Rajeshwari Madappa, David H Rothstein, Satyan Lakshminrusimha
Congenital Diaphragmatic hernia (CDH) is a condition characterized by a defect in the diaphragm leading to protrusion of abdominal contents into the thoracic cavity interfering with normal development of the lungs. The defect may range from a small aperture in the posterior muscle rim to complete absence of diaphragm. The pathophysiology of CDH is a combination of lung hypoplasia and immaturity associated with persistent pulmonary hypertension of newborn (PPHN) and cardiac dysfunction. Prenatal assessment of lung to head ratio (LHR) and position of the liver by ultrasound are used to diagnose and predict outcomes...
2017: Maternal Health, Neonatology and Perinatology
https://www.readbyqxmd.com/read/28326961/fatal-vernix-caseosa-aspiration-associated-with-persistent-pulmonary-hypertension-of-the-newborn
#13
Narongsak Nakwan, Wuttichart Kamolvisit, Charoen Napapongsuriya, Pornpreenun Chaiwiriyawong, Cheep Charoenlap
Vernix caseosa aspiration is an extremely rare condition resulting in high mortality if complicated by persistent hypertension of the newborn (PPHN). Herein we offer the first case report of PPHN due to massive vernix caseosa aspiration documented by histopathological examination. This case report is presented to provide a synopsis of the pathoetiology of PPHN related to vernix caseosa aspiration syndrome as likely to be encountered by neonatologists and general pediatricians involved with neonatal care.
March 2017: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/28276303/abnormal-muscularization-of-intra-acinar-pulmonary-arteries-in-two-cases-presenting-as-sudden-infant-death
#14
Khairul Zainun, Kirsten Hope, Andrew G Nicholson, Marta C Cohen
Abnormal muscularization of acinar arteries is the hallmark of persistent pulmonary hypertension of newborn (PPHN), an uncommon disease with high rate of morbidity and mortality. PPHN presents with signs of respiratory distress immediately following birth. We herein report 2 cases presenting as a witnessed sudden unexpected death in the late neonatal period, preceded by respiratory deterioration and in whom the presence of abnormal muscularization of the acinar pulmonary arteries was reminiscent of PPHN. The significance of this report is twofold: to increase the awareness among pediatricians and pathologists of this feature that can present in some cases of Sudden Unexpected Death in Infancy/Sudden Infant Death Syndrome, and to highlight the importance of performing a thorough autopsy in order to identify the abnormality...
January 2017: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/28202122/-role-of-calcium-sensing-receptor-in-neonatal-mice-with-persistent-pulmonary-hypertension
#15
Meng-Meng Wang, He Li, Fang-Fang Zhang, Ke-Tao Ma, Wei-Wei Cao, Qiang Gu
OBJECTIVE: To study the effect of calcium-sensing receptor (CaSR) agonists and antagonists on the expression of CaSR in neonatal mice with persistent pulmonary hypertension (PPHN), and to clarify the role of CaSR in neonatal mice with PPHN. METHODS: Forty-nine neonatal mice were randomly divided into four groups: control (n=10), hypoxia (PPHN; n=11), agonist (n=13), and antagonist (n=15). The mice in the PPHN, agonist, and antagonist groups were exposed to an oxygen concentration of 12%, and those in the control group were exposed to the air...
February 2017: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
https://www.readbyqxmd.com/read/28148930/mir-126a-5p-is-involved-in-the-hypoxia-induced-endothelial-to-mesenchymal-transition-of-neonatal-pulmonary-hypertension
#16
Yan-Ping Xu, Qi He, Zheng Shen, Xiao-Li Shu, Chen-Hong Wang, Jia-Jun Zhu, Li-Ping Shi, Li-Zhong Du
Persistent pulmonary hypertension of the newborn (PPHN) is a clinical syndrome characterized by increased medial and adventitial thickness of the lung vasculature. The underlying mechanisms that regulate the cell phenotype alteration during PPHN remodeling are largely unknown. We randomly selected newborn rats that were exposed to hypoxia (10-12%) or room air for 2 weeks and used a microarray to identify the lung tissue microRNAs (miRNAs) involved in PPHN progression. The role of a key miRNA that affects the endothelial-to-mesenchymal transition (EndMT) in primary cultured rat pulmonary microvascular endothelial cells (RPMECs) was investigated...
June 2017: Hypertension Research: Official Journal of the Japanese Society of Hypertension
https://www.readbyqxmd.com/read/28099979/milrinone-pharmacokinetics-and-pharmacodynamics-in-neonates-with-persistent-pulmonary-hypertension-of-the-newborn
#17
Annie Giaccone, Athena F Zuppa, Beena Sood, Meryl S Cohen, Michael L O'Byrne, Ganesh Moorthy, Amit Mathur, Haresh Kirpalani
Objective To describe the pharmacokinetics and pharmacodynamics of milrinone in infants with persistent pulmonary hypertension of the newborn (PPHN) and to explore the impact of age on milrinone disposition. Design Randomized, open label pilot study. Setting Multicenter; level 3 and level 4 neonatal intensive care units. Patients Six infants ≥34 weeks' gestational age and <10 days of life with persistent hypoxemia receiving inhaled nitric oxide. Intervention Intravenous milrinone lactate in one of two dosing regimens: (1) low dose, 20 mcg/kg bolus followed by 0...
July 2017: American Journal of Perinatology
https://www.readbyqxmd.com/read/28096983/pde3-inhibition-with-enoximone-as-first-line-therapy-for-severe-persistent-pulmonary-hypertension-of-the-newborn-during-neonatal-transport-a-case-report
#18
Robin van der Lee, Barbara Peels, Corine Koopman-Esseboom
Severe Persistent pulmonary hypertension of the newborn (PPHN) can be effectively treated with a PDE3 inhibitor as first-line treatment during neonatal transport when iNO is not readily available. Starting iNO as soon as possible is strongly advised because of the complementary actions of both therapeutics.
January 2017: Clinical Case Reports
https://www.readbyqxmd.com/read/28056166/nitric-oxide-for-respiratory-failure-in-infants-born-at-or-near-term
#19
REVIEW
Keith J Barrington, Neil Finer, Thomas Pennaforte, Gabriel Altit
BACKGROUND: Nitric oxide (NO) is a major endogenous regulator of vascular tone. Inhaled nitric oxide (iNO) gas has been investigated as treatment for persistent pulmonary hypertension of the newborn. OBJECTIVES: To determine whether treatment of hypoxaemic term and near-term newborn infants with iNO improves oxygenation and reduces rate of death and use of extracorporeal membrane oxygenation (ECMO), or affects long-term neurodevelopmental outcomes. SEARCH METHODS: We used the standard search strategy of the Cochrane Neonatal Review Group to search the Cochrane Central Register of Controlled Trials (CENTRAL; 2016, Issue 1), MEDLINE via PubMed (1966 to January 2016), Embase (1980 to January 2016) and the Cumulative Index to Nursing and Allied Health Literature (CINAHL; 1982 to January 2016)...
January 5, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28042638/black-lung-persistent-pulmonary-hypertension-of-the-newborn-saudi-experience-with-sildenafil-and-nitric-oxide
#20
AbdulRahman M Alnemri
To determine the clinical presentation, risk factors, diagnosis, and treatment outcome of Saudi infants with black lung persistent pulmonary hypertension of the newborn (PPHN). Methods: This is a retrospective review of all neonates with PPHN presented to the Armed Force Hospital Southern Region, Kingdom of Saudi Arabia from January 2012 to December 2014. Results: Ten term and near term infants presented with PPHN were included. Maternal diabetes and Down syndrome were the most common identified risk factors for PPHN in the study group...
January 2017: Saudi Medical Journal
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