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Children pulmonary hypertension

Mostafa Behjati-Ardakani, Mohammad Golshan, Sedigheh Akhavan-Karbasi, Seyed-Masood Hosseini, Mohammad-Amin Behjati-Ardakani, Mohammadtaghi Sarebanhassanabadi
BACKGROUND: Atrial septal defect (ASD) is a common congenital heart disease. OBJECTIVES: The aim of this study was to assess the clinical course of ASD, as well as its frequency of spontaneous closure and regression of diameter. METHODS: In a study conducted from 2000 to 2011, 192 consecutive patients with an isolated ASD were followed up regularly with periodic echocardiographic evaluations according to a standardized protocol. The study was conducted in two series of patients: infants and children and adults...
August 2016: Iranian Journal of Pediatrics
Toshiharu Matsuura, Yoshiaki Takahashi, Yusuke Yanagi, Koichiro Yoshimaru, Kenichiro Yamamura, Eiji Morihana, Hazumu Nagata, Kiyoshi Uike, Hidetoshi Takada, Tomoaki Taguchi
BACKGROUND: Congenital portosystemic shunts (CPSS) with intrahepatic portal vein (IHPV) hypoplasia or absence cause encephalopathy or pulmonary hypertension (PH). Acute shunt closure may result in postoperative portal hypertension. The aim of this study was to propose a surgical strategy according to the anatomical types of CPSS and IHPV. METHODS: Twenty-three CPSS patients were diagnosed from1990 to 2015. All patients were evaluated by computed tomography, angiography, and PV pressure monitoring under a shunt occlusion test...
September 21, 2016: Journal of Pediatric Surgery
Sae Yun Kim, Seung Han Shin, Han-Suk Kim, Young Hwa Jung, Ee-Kyung Kim, Jung-Hwan Choi
OBJECTIVE: To describe the clinical course and risk factors for pulmonary arterial hypertension (PAH) after ibuprofen treatment to close patent ductus arteriosus. STUDY DESIGN: All neonates weighing < 1500 g at birth who received ibuprofen to close patent ductus arteriosus and were admitted to Seoul National University Children's Hospital's neonatal intensive care unit in 2010-2014 were eligible for this study. The study population was divided into the PAH and non-PAH groups, and medical records were retrospectively reviewed...
September 28, 2016: Journal of Pediatrics
Steven H Abman, D Dunbar Ivy, Stephen L Archer, Kevin Wilson
Although pulmonary hypertension (PH) contributes significantly to poor outcomes in diverse pediatric diseases, approaches toward the care of children with PH have been limited by the lack of consensus guidelines from experts in the field. In a joint effort from the American Heart Association and American Thoracic Society, a committee of experienced clinicians was formed to systematically identify, synthesize, and appraise relevant evidence and then to formulate evidence-based recommendations regarding the diagnosis and management of pediatric PH...
October 1, 2016: American Journal of Respiratory and Critical Care Medicine
Yan-Ping Xu
OBJECTIVES: Bronchopulmonary dysplasia (BPD) is a chronic pulmonary disorder affecting preterm infants. We studied the factors and echocardiographic evidence of early pulmonary hypertension (PH) associated with moderate or severe BPD. METHODS: We retrospectively reviewed preterm infants who were born at <32 weeks gestation and admitted to the neonatal intensive care unit at the Children's Hospital of Zhejiang University School of Medicine between July 2013 and July 2015...
2016: Global Pediatric Health
Enas A Hamed, Mostafa M El-Saied, Khaled Saad, Hazem Abu-Zeid Yousef, Amany O Mohamed, Dina Sabry
OBJECTIVE: This study aimed to evaluate fibrosis and elastin destruction in childhood interstitial lung disease (chILD) patients. METHODS: Sixty patients and twenty healthy children were recruited. On admission, evaluation of chILD severity was made using Fan chILD score. Participants provided urine and blood samples. Plasma levels of transforming growth factor (TGF)-β1, connective tissue growth factor (CCN2), soluble factor related apoptosis (sFas) and long non-coding RNAs and urinary levels of desmosine/urinary creatinine (UDes/UCr) were measured...
September 21, 2016: Pathophysiology: the Official Journal of the International Society for Pathophysiology
Willemijn M H Zijlstra, Johannes M Douwes, Mark-Jan Ploegstra, Usha Krishnan, Marcus T R Roofthooft, Hans L Hillege, D Dunbar Ivy, Erika B Rosenzweig, Rolf M F Berger
Congenital heart disease (CHD) is a frequent cause of pediatric pulmonary arterial hypertension (PAH), with diverse etiology and outcome. We aimed to describe phenotypic heterogeneity in pediatric PAH associated with CHD (PAH-CHD), assess the applicability of the Nice CHD classification, and explore whether this classification accurately reflects patient/disease characteristics and survival. All children with CHD from a contemporary cohort of consecutive pediatric PAH patients followed in three major referral centers (Denver, New York, the Netherlands) were characterized and classified on the basis of the latest proposed clinical classification for PAH-CHD (World Symposium on Pulmonary Hypertension, Nice, 2013)...
September 2016: Pulmonary Circulation
Douglas Bush, Steven H Abman, Csaba Galambos
OBJECTIVES: To determine the frequency of histologic features of impaired lung vascular and alveolar development and to identify the presence of intrapulmonary bronchopulmonary anastomoses (IBA) in infants and children who died with Down syndrome. STUDY DESIGN: A retrospective review of autopsy reports and lung histology from 13 children with Down syndrome (ages: 0-8 years) was performed. Histologic features of abnormal lung development were identified and semiquantified, including the presence of IBA...
September 22, 2016: Journal of Pediatrics
T Pincez, L Calamy, Z Germont, A Lemoine, A-A Lopes, A Massiot, J Tencer, C Thivent, A Hadchouel
Acute and chronic pulmonary complications are frequent in sickle cell disease (SCD), with different spectrum and characteristics in children and adults. Chronic hypoxia is frequent and plays a role in several respiratory complications in SCD. Furthermore, hypoxia has been associated with a higher risk of cerebral ischemia. Despite differing oxygen affinity between hemoglobin A and S, standard pulse oximetry was shown to be accurate in diagnosing hypoxia in SCD patients. Whereas acute hypoxia management is similar to non-SCD patients, chronic hypoxia treatment is mainly based on a transfusion program rather than long-term oxygen therapy...
October 2016: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
Philip T Levy, Meghna D Patel, Georgeann Groh, Swati Choudhry, Joshua Murphy, Mark R Holland, Aaron Hamvas, Mark R Grady, Gautam K Singh
BACKGROUND: Pulmonary artery acceleration time (PAAT) is a noninvasive method to assess pulmonary hemodynamics, but it lacks validity in children. The aim of this study was to evaluate the accuracy of Doppler echocardiography-derived PAAT in predicting right heart catheterization (RHC)-derived pulmonary artery pressure (PAP), pulmonary vascular resistance (PVR), and compliance in children. METHODS: Prospectively acquired and retrospectively measured Doppler echocardiography-derived PAAT and RHC-derived systolic PAP, mean PAP (mPAP), indexed PVR (PVRi), and compliance were compared using regression analysis in a derivation cohort of 75 children (median age, 5...
September 15, 2016: Journal of the American Society of Echocardiography
Rogatus Kabyemera, Neema Chami, Neema Kayange, Respicius Bakalemwa, Antke Zuechner, Tumaini Mhada, Gustave Buname, Adolfine Hokororo, Johannes Kataraihya
Upper airway obstruction (UAO) due to adenotonsillar hypertrophy represents one of the rare causes of pulmonary hypertension in children. We report a case of adenotonsillar hypertrophy, managed at pediatric and otorhinolaryngology departments in Bugando Medical Centre (BMC), northwestern Tanzania, with complete remission of symptoms of pulmonary hypertension following adenotonsillectomy. A 17-month-old boy presented with difficulty breathing, dry cough, and noisy breathing since 1 year. He had facial and lower limb oedema with a pan systolic murmur at the tricuspid area, fine crepitations, and tender hepatomegaly...
2016: Case Reports in Pediatrics
Kathryn Browning Carmo, Tracey Lutz, Andrew Berry, Martin Kluckow, Nick Evans
AIM: To determine the role of clinician performed ultrasound (CPU) during the retrieval and transport of critically ill term and near term newborns. METHODS: A neonatologist with portable ultrasound accompanied a sample of newborn retrievals to perform cardiac and cerebral ultrasound before and after transportation. RESULTS: A total of fifty-five babies were studied. Median birthweight: 3350 g (2220-5030 g). CPU led to a change in the planned receiving hospital in ten babies...
September 15, 2016: Acta Paediatrica
Yves Wyss, Daniel Quandt, Roland Weber, Brian Stiasny, Benedikt Weber, Walter Knirsch, Oliver Kretschmar
AIMS: This study set out to assess indications, feasibility, complications, and clinical outcome of percutaneous transcatheter device closure of atrial septal defects (ASDs) in infants with a bodyweight below 10 kg. METHODS AND RESULTS: Retrospective single center chart and echocardiography review study from 8/2005-12/2013. Twenty-eight children with ASD (13 female) with a median age of 1.15 years (0.2-2.8) and a median weight of 7.2 kg (4.5-9.9) were analyzed...
September 13, 2016: Journal of Interventional Cardiology
Enrico Danzer, Casey Hoffman, Jo Ann D'Agostino, Marsha Gerdes, Judy Bernbaum, Ryan M Antiel, Natalie E Rintoul, Lisa M Herkert, Alan W Flake, N Scott Adzick, Holly L Hedrick
OBJECTIVE: To evaluate neurodevelopmental sequelae in congenital diaphragmatic hernia (CDH) children at 5years of age. MATERIALS AND METHODS: The study cohort of 35 CDH patients was enrolled in our follow-up program between 06/2004 and 09/2014. The neurodevelopmental outcomes assessed at a median of 5years (range, 4-6) included cognition (Wechsler Preschool and Primary Scale of Intelligence [WPPSI], n=35), Visual-Motor-Integration (n=35), academic achievement (Woodcock-Johnson Tests of Achievement, n=25), and behavior problems (Child Behavior Check List [CBCL], n=26)...
August 30, 2016: Journal of Pediatric Surgery
Hala Mahmoud Koura, Neamat M Abdalla, Mona Hamed Ibrahim, Maha M A Abo Hashish, Sherif Mohamed Zaki
BACKGROUND: B-type natriuretic peptide (BNP) levels are elevated in children with congenital heart disease involving a left-to-right shunt (LRS) and are also raised in dilated cardiomyopathy (DCM). As far as we know, there are few reports in the literature comparing the change of the NT-proBNP in LRS and DCM especially in the pediatric age group. OBJECTIVES: The aim of the study was to compare the changes of the NT-proBNP in pediatric patients with LRS and DCM. Correlation between the levels of NT-proBNP and the echocardiographic parameters in both groups was determined...
June 2016: Iranian Journal of Pediatrics
Christoph Kampmann, Tariq Abu-Tair, Seyfullah Gökce, Christina Lampe, Jörg Reinke, Eugen Mengel, Julia B Hennermann, Christiane M Wiethoff
BACKGROUND: Mucopolysaccharidosis (MPS) IVA is a rare lysosomal storage disorder with multiple skeletal and non-skeletal abnormalities requiring multiple surgical interventions. It is well known that patients with MPS IVA suffer from tachycardia, but cardiac and hemodynamic alterations have not been reported to date. We investigated the cardiovascular and hemodynamic alterations in patients with MPS IVA and developed a possible patho-mechanism for cardiovascular deterioration during anesthesia...
2016: PloS One
Hiroyuki Matsuura
Cardiac catheterization of childhood pulmonary arterial hypertension (PAH) plays a pivotal role in assessing the severity of the disease as well as its prognosis, selection of the most adequate pulmonary vasodilators, and evaluation of effectiveness. However, sudden deterioration of cardiovascular hemodynamics can be easily induced by pain, a patient's agitation, catheter manipulation, and by vasodilator provocation tests; these could trigger a pulmonary hypertension crisis, vagotony, respiratory distress, and hemoptysis resulting in critical complications, including death...
September 7, 2016: Pediatrics International: Official Journal of the Japan Pediatric Society
Jason Boehme, Xutong Sun, Kathryn V Tormos, Wenhui Gong, Manuela Kellner, Sanjeev A Datar, Rebecca Johnson Kameny, Jason X-J Yuan, Gary W Raff, Jeffrey R Fineman, Stephen M Black, Emin Maltepe
Vascular cell hyperproliferation and metabolic reprogramming contribute to the pathophysiology of pulmonary arterial hypertension (PAH). An important cause of PAH in children with congenital heart disease (CHD) is increased pulmonary blood flow (PBF). To better characterize this disease course we studied early changes in pulmonary artery smooth muscle cell (PASMC) proliferation and metabolism using a unique ovine model of pulmonary overcirculation. Consistent with PAH in adults, PASMCs derived from 4-wk-old lambs exposed to increased PBF (shunt) exhibited increased rates of proliferation...
October 1, 2016: American Journal of Physiology. Heart and Circulatory Physiology
Marilyne Levy, Mélanie Eyries, Isabelle Szezepanski, Magalie Ladouceur, Sophie Nadaud, Damien Bonnet, Florent Soubrier
The prevalence of germline mutations in paediatric pulmonary hypertension (PH) is poorly documented. The objective of this study was to determine the mutation frequency in PH genes in a paediatric cohort and describe the clinical characteristics of mutation carriers.The study involved 66 index cases with PH: 35 children with idiopathic pulmonary arterial hypertension (IPAH); five children with familial PAH (FPAH); three children with pulmonary veno-occlusive disease (PVOD); and 23 children with PAH associated with congenital heart disease (APAH-CHD)...
October 2016: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
Steven H Abman
PURPOSE OF REVIEW: Pediatric pulmonary vascular disease contributes to morbidities and death in diverse clinical settings, ranging from idiopathic or heritable forms of pediatric arterial hypertension, congenital heart disease, developmental lung disorders, chronic lung disease, left heart disease, sickle cell disease, oncologic disease, and systemic disorders. Despite its impact on the clinical courses in so many diseases, information is limited on how to best approach the diagnosis and evaluation of pediatric pulmonary hypertension...
October 2016: Current Opinion in Pediatrics
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