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Article: Prevalence of malnutrition in pediatric pulmonary hypertension cohort and role for registered dietitian involvement.

Crowell, Presley R / Frederick, Mackenzie R / Fombin, Rozmeen A / Varghese, Nidhy P / Ruiz, Fadel E

2023 Volume 11, Page(s) 995470

Abstract: Introduction: Pediatric pulmonary hypertension (PH) is a serious condition with increased risk for malnutrition due to increased caloric needs and reduced energy intake. This combination of disease and dynamic elements make it particularly challenging ... ...

Abstract	Introduction: Pediatric pulmonary hypertension (PH) is a serious condition with increased risk for malnutrition due to increased caloric needs and reduced energy intake. This combination of disease and dynamic elements make it particularly challenging to meet expected growth patterns. Pediatric PH patients require close monitoring and individualized nutrition interventions to best meet nutrient needs. The prevalence of malnutrition and effective nutrition interventions in pediatric PH has not been studied. Methods: Using our electronic medical record (EMR) patient care dashboard, malnutrition prevalence was assessed by reviewing the active problem list of all active PH patients at our center. A chart review compared patients with diagnosed malnutrition in the EMR to those with malnutrition identified by a registered dietitian (RD) using a standardized tool. Chart reviews also assessed outcomes of RD interventions. Results: 195 patients were identified as active PH patients followed by our PH center during the study period (November 2021 to January 2023). Of these, 5% (10/195) had an ICD-10 code for malnutrition listed in their chart. However, upon further chart review of the remaining 185 patients, 22% (41/185) had malnutrition identified by a RD using Texas Children's Malnutrition Tool, totaling 51/195 (26%) malnourished patients. The PH RD saw 25/51 (49%) patients during PH clinic visits in the study period. At follow up visits (3-4 months after initial assessment), 56% (14/25) patients seen by the PH RD either improved or resolved their malnutrition status by Conclusion: Malnutrition is present in pediatric PH, although underappreciated and underdiagnosed. Managing malnutrition in pediatric PH requires close monitoring, multidisciplinary involvement, and individualized nutrition recommendations. This is best achieved by a dedicated PH RD who is familiar with the unique needs of this population and available to provide consistent nutritional assessments and interventions to reduce malnutrition in this population.
Language	English
Publishing date	2023-04-04
Publishing country	Switzerland
Document type	Journal Article
ZDB-ID	2711999-3
ISSN	2296-2360
ISSN	2296-2360
DOI	10.3389/fped.2023.995470
Database	MEDical Literature Analysis and Retrieval System OnLINE

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Article: Tracheostomy in Pulmonary Hypertension: Not for the Faint of Heart.

Villafranco, Natalie / Chartan, Corey / Varghese, Nidhy P / Coleman, Ryan D

Pediatric critical care medicine : a journal of the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies

2022 Volume 23, Issue 9, Page(s) 754–756

MeSH term(s)	Heart ; Humans ; Hypertension, Pulmonary/surgery ; Tracheostomy
Language	English
Publishing date	2022-08-29
Publishing country	United States
Document type	Editorial ; Comment
ZDB-ID	2052349-X
ISSN	1947-3893 ; 1529-7535
ISSN (online)	1947-3893
ISSN	1529-7535
DOI	10.1097/PCC.0000000000003021
Database	MEDical Literature Analysis and Retrieval System OnLINE

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Article: The cascade screening in heritable forms of pulmonary arterial hypertension.

Varghese, Nidhy P / Padhye, Akhilesh A / Magoulas, Pilar L / Mallory, George B / Ruiz, Fadel E / Sahay, Sandeep

Pulmonary circulation

2023 Volume 13, Issue 3, Page(s) e12259

Abstract: Heritable pulmonary artery hypertension (HPAH) is an increasingly recognized type of pulmonary arterial hypertension, in both pediatric and adult population. Intrinsic to hereditary disease, screening for genetic mutations within families is an important ...

Abstract	Heritable pulmonary artery hypertension (HPAH) is an increasingly recognized type of pulmonary arterial hypertension, in both pediatric and adult population. Intrinsic to hereditary disease, screening for genetic mutations within families is an important component of diagnosis and understanding burden of disease. Recently, consensus guidelines are published for genetic screening in PAH. These guidelines include recommendations for screening at diagnosis, noting individuals with presumed PAH due to familial, or idiopathic etiologies. Cascade genetic testing is specifically recommended as a testing paradigm to screen relatives for detection of mutation carriers, who may be asymptomatic. Without targeted genetic testing, familial mutation carriers may only come to attention when pulmonary vascular disease burden is high enough to cause symptoms, suggesting more advanced disease. Here, we present our collective experience with HPAH in five distinct families, specifically to report on the clinical courses of patients who were diagnosed with genetic mutation at diagnosis versus those who were offered genetic screening. In three families, asymptomatic mutation carriers were identified and monitored for clinical worsening. In two families, screening was not done and affected family members presented with advanced disease.
Language	English
Publishing date	2023-06-28
Publishing country	United States
Document type	Case Reports
ZDB-ID	2638089-4
ISSN	2045-8940 ; 2045-8932
ISSN (online)	2045-8940
ISSN	2045-8932
DOI	10.1002/pul2.12259
Database	MEDical Literature Analysis and Retrieval System OnLINE

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Article ; Online: Bronchopulmonary dysplasia - associated pulmonary hypertension: An updated review.

El-Saie, Ahmed / Varghese, Nidhy P / Webb, Melissa K / Villafranco, Natalie / Gandhi, Bheru / Guaman, Milenka Cuevas / Shivanna, Binoy

Seminars in perinatology

2023 Volume 47, Issue 6, Page(s) 151817

Abstract: Bronchopulmonary dysplasia (BPD) is the leading cause of chronic lung disease in infants and the commonest complication of prematurity. Advances in respiratory and overall neonatal care have increased the survival of extremely low gestational age ... ...

Abstract	Bronchopulmonary dysplasia (BPD) is the leading cause of chronic lung disease in infants and the commonest complication of prematurity. Advances in respiratory and overall neonatal care have increased the survival of extremely low gestational age newborns, leading to the continued high incidence of BPD. Pulmonary hypertension (PH) represents the severe form of the pulmonary vascular disease associated with BPD, and affects almost one-third of infants with moderate to severe BPD. PH responds suboptimally to pulmonary vasodilators and increases morbidity and mortality in BPD infants. An up-to-date knowledge of the pathogenesis, pathophysiology, diagnosis, treatment, and outcomes of BPD-PH can be helpful to develop meaningful and novel strategies to improve the outcomes of infants with this disorder. Therefore, our multidisciplinary team has attempted to thoroughly review and summarize the latest advances in BPD-PH in preventing and managing this morbid lung disorder of preterm infants.
MeSH term(s)	Infant ; Infant, Newborn ; Humans ; Bronchopulmonary Dysplasia/complications ; Bronchopulmonary Dysplasia/therapy ; Bronchopulmonary Dysplasia/epidemiology ; Infant, Premature ; Hypertension, Pulmonary/diagnosis ; Hypertension, Pulmonary/etiology ; Hypertension, Pulmonary/therapy ; Lung ; Gestational Age
Language	English
Publishing date	2023-09-09
Publishing country	United States
Document type	Review ; Journal Article
ZDB-ID	752403-1
ISSN	1558-075X ; 0146-0005
ISSN (online)	1558-075X
ISSN	0146-0005
DOI	10.1016/j.semperi.2023.151817
Database	MEDical Literature Analysis and Retrieval System OnLINE

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Je nach Verfügbarkeit (siehe Angabe bei Bestand)
bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular
Jg. 1995 - 2021: Lesesall (1.OG)
ab Jg. 2022: Lesesaal (EG)

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Article: Prematurity and Pulmonary Vein Stenosis: The Role of Parenchymal Lung Disease and Pulmonary Vascular Disease.

Vyas-Read, Shilpa / Varghese, Nidhy P / Suthar, Divya / Backes, Carl / Lakshminrusimha, Satyan / Petit, Christopher J / Levy, Philip T

Children (Basel, Switzerland)

2022 Volume 9, Issue 5

Abstract: Pulmonary vein stenosis (PVS) has emerged as a critical problem in premature infants with persistent respiratory diseases, particularly bronchopulmonary dysplasia (BPD). As a parenchymal lung disease, BPD also influences vascular development with ... ...

Abstract	Pulmonary vein stenosis (PVS) has emerged as a critical problem in premature infants with persistent respiratory diseases, particularly bronchopulmonary dysplasia (BPD). As a parenchymal lung disease, BPD also influences vascular development with associated pulmonary hypertension recognized as an important comorbidity of both BPD and PVS. PVS is commonly detected later in infancy, suggesting additional postnatal factors that contribute to disease development, progression, and severity. The same processes that result in BPD, some of which are inflammatory-mediated, may also contribute to the postnatal development of PVS. Although both PVS and BPD are recognized as diseases of inflammation, the link between them is less well-described. In this review, we explore the relationship between parenchymal lung diseases, BPD, and PVS, with a specific focus on the epidemiology, clinical presentation, risk factors, and plausible biological mechanisms in premature infants. We offer an algorithm for early detection and prevention and provide suggestions for research priorities.
Language	English
Publishing date	2022-05-12
Publishing country	Switzerland
Document type	Journal Article ; Review
ZDB-ID	2732685-8
ISSN	2227-9067
ISSN	2227-9067
DOI	10.3390/children9050713
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Article: Case Report: Selexipag in pediatric pulmonary hypertension: Initiation, transition, and titration.

Faircloth, Jenna M / Bhatt, Neelam D / Chartan, Corey A / Coleman, Ryan D / Villafranco, Natalie / Ruiz, Fadel E / Morales-Demori, Raysa / Whalen, Elise / Ely, Erin / Fombin, Rozmeen / Varghese, Nidhy P

Frontiers in pediatrics

2023 Volume 11, Page(s) 1050508

Abstract: Selexipag, a selective prostacyclin receptor agonist, is approved for treating pulmonary arterial hypertension in WHO Group 1 adult patients. Compared to parenteral prostacyclin formulations, selexipag offers a significant improvement in patient's and ... ...

Abstract	Selexipag, a selective prostacyclin receptor agonist, is approved for treating pulmonary arterial hypertension in WHO Group 1 adult patients. Compared to parenteral prostacyclin formulations, selexipag offers a significant improvement in patient's and caregiver's quality of life because of its oral formulation, frequency of administration, and mechanism of action. Although experience in the pediatric population is limited to case reports in older adolescent patients and selexipag is not approved for use in the pediatric pulmonary hypertension population, many pediatric centers are expanding the use of this therapy to this population. We report our institution's experience in the use of selexipag to treat pulmonary hypertension in children under 10 years of age, between 10 and 30 kg. Seven patients were initiated on selexipag therapy including
Language	English
Publishing date	2023-03-08
Publishing country	Switzerland
Document type	Case Reports
ZDB-ID	2711999-3
ISSN	2296-2360
ISSN	2296-2360
DOI	10.3389/fped.2023.1050508
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Article: Hereditary pulmonary arterial hypertension burden in pediatrics: A single referral center experience.

Ishizuka, Maki / Zou, Wenxin / Whalen, Elise / Ely, Erin / Coleman, Ryan D / Lopez-Terrada, Dolores H / Penny, Daniel J / Fan, Yuxin / Varghese, Nidhy P

Frontiers in pediatrics

2023 Volume 11, Page(s) 1050706

Abstract: Introduction: Hereditary pulmonary arterial hypertension (HPAH) is a rare yet serious type of pulmonary arterial hypertension (PAH). The burden in the pediatric population remains high yet underreported. The objective of this study is to describe the ... ...

Abstract	Introduction: Hereditary pulmonary arterial hypertension (HPAH) is a rare yet serious type of pulmonary arterial hypertension (PAH). The burden in the pediatric population remains high yet underreported. The objective of this study is to describe the distribution of mutations found on targeted PAH panel testing at a large pediatric referral center. Methods: Children with PAH panel administered by the John Welsh Cardiovascular Diagnostic Laboratory at Texas Children's Hospital and Baylor College of Medicine in Houston, Texas between October 2012 to August 2021 were included into this study. Medical records were retrospectively reviewed for clinical correlation. Results: Sixty-six children with PAH underwent PAH genetic testing. Among those, 9 (14%) children were found to have pathogenic mutations, 16 (24%) children with variant of unknown significance and 41 (62%) children with polymorphism (classified as likely benign and benign). BMPR2 mutation was the most common pathogenic mutation, seen in 6 of the 9 children with detected mutations. Hemodynamic studies showed higher pulmonary vascular resistance among those with pathogenic mutations than those without (17.4 vs. 4.6 Wood units). All children with pathogenic mutations had severe PAH requiring triple therapy. There were tendencies for higher lung transplantation rate but lower mortality among those with pathogenic mutations. Conclusions: Abnormalities on genetic testing are not uncommon among children with PAH, although majority are of unclear significance. However, children with pathogenic mutations tended to present with more severe PAH requiring aggressive medical and surgical therapies. Genetic testing should be routinely considered due to consequences for treatment and prognostic implications. Larger scale population studies and registries are warranted to characterize the burden of HPAH in the pediatric population specifically.
Language	English
Publishing date	2023-03-29
Publishing country	Switzerland
Document type	Journal Article
ZDB-ID	2711999-3
ISSN	2296-2360
ISSN	2296-2360
DOI	10.3389/fped.2023.1050706
Database	MEDical Literature Analysis and Retrieval System OnLINE

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Article: Corrigendum: Case report: Selexipag in pediatric pulmonary hypertension: initiation, transition, and titration.

Frontiers in pediatrics

2023 Volume 11, Page(s) 1275389

Abstract: This corrects the article DOI: 10.3389/fped.2023.1050508.]. ...

Abstract	[This corrects the article DOI: 10.3389/fped.2023.1050508.].
Language	English
Publishing date	2023-08-21
Publishing country	Switzerland
Document type	Published Erratum
ZDB-ID	2711999-3
ISSN	2296-2360
ISSN	2296-2360
DOI	10.3389/fped.2023.1275389
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Article: Atrial septal defect closure is associated with improved clinical status in patients ≤ 10 kg with bronchopulmonary dysplasia.

Webb, Melissa K / Cuevas Guaman, Milenka / Sexson Tejtel, S Kristen / Cambronero, Neil / Coleman, Ryan D / Chartan, Corey A / Yilmaz Furtun, Betul / Morris, Shaine A / Varghese, Nidhy P / Villafranco, Natalie M

Pulmonary circulation

2023 Volume 13, Issue 4, Page(s) e12299

Abstract: Patients with bronchopulmonary dysplasia (BPD) have shown clinical improvement after secundum atrial septal defect (ASD) closure. We sought to determine if this post-ASD closure improvement is secondary to the expected course in BPD patients or related ... ...

Abstract	Patients with bronchopulmonary dysplasia (BPD) have shown clinical improvement after secundum atrial septal defect (ASD) closure. We sought to determine if this post-ASD closure improvement is secondary to the expected course in BPD patients or related to the closure itself. A novel BPD-ASD score was created to assess patients' clinical status (higher score = worse disease) and applied to 10 BPD-ASD inpatients weighing ≤ 10 kg who underwent ASD closure. The score and its subcomponents were retrospectively calculated serially ranging from 8 weeks pre- to 8 weeks post-intervention, and pre- and post-intervention score slopes were created. These slopes were compared using mixed regression modeling with an interaction term. There was a significant difference in pre- versus post-intervention slope with the most score drop the first week post-intervention (-2.1 + /- 0.8,
Language	English
Publishing date	2023-10-20
Publishing country	United States
Document type	Journal Article
ZDB-ID	2638089-4
ISSN	2045-8940 ; 2045-8932
ISSN (online)	2045-8940
ISSN	2045-8932
DOI	10.1002/pul2.12299
Database	MEDical Literature Analysis and Retrieval System OnLINE

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Article: Endothelial Adenosine Monophosphate-Activated Protein Kinase-Alpha1 Deficiency Potentiates Hyperoxia-Induced Experimental Bronchopulmonary Dysplasia and Pulmonary Hypertension

Elsaie, Ahmed / Menon, Renuka T. / Shrestha, Amrit K. / Gowda, Sharada H. / Varghese, Nidhy P. / Barrios, Roberto J. / Blanco, Cynthia L. / Konduri, Girija G. / Shivanna, Binoy

Antioxidants. 2021 Nov. 29, v. 10, no. 12

2021

Abstract: Bronchopulmonary dysplasia and pulmonary hypertension, or BPD-PH, are serious chronic lung disorders of prematurity, without curative therapies. Hyperoxia, a known causative factor of BPD-PH, activates adenosine monophosphate-activated protein kinase ( ... ...

Abstract	Bronchopulmonary dysplasia and pulmonary hypertension, or BPD-PH, are serious chronic lung disorders of prematurity, without curative therapies. Hyperoxia, a known causative factor of BPD-PH, activates adenosine monophosphate-activated protein kinase (AMPK) α1 in neonatal murine lungs; however, whether this phenomenon potentiates or mitigates lung injury is unclear. Thus, we hypothesized that (1) endothelial AMPKα1 is necessary to protect neonatal mice against hyperoxia-induced BPD-PH, and (2) AMPKα1 knockdown decreases angiogenesis in hyperoxia-exposed neonatal human pulmonary microvascular endothelial cells (HPMECs). We performed lung morphometric and echocardiographic studies on postnatal day (P) 28 on endothelial AMPKα1-sufficient and -deficient mice exposed to 21% O₂ (normoxia) or 70% O₂ (hyperoxia) from P1–P14. We also performed tubule formation assays on control- or AMPKα1-siRNA transfected HPMECs, exposed to 21% O₂ or 70% O₂ for 48 h. Hyperoxia-mediated alveolar and pulmonary vascular simplification, pulmonary vascular remodeling, and PH were significantly amplified in endothelial AMPKα1-deficient mice. AMPKα1 siRNA knocked down AMPKα1 expression in HPMECs, and decreased their ability to form tubules in normoxia and hyperoxia. Furthermore, AMPKα1 knockdown decreased proliferating cell nuclear antigen expression in hyperoxic conditions. Our results indicate that AMPKα1 is required to reduce hyperoxia-induced BPD-PH burden in neonatal mice, and promotes angiogenesis in HPMECs to limit lung injury.
Keywords	AMP-activated protein kinase ; adenosine ; angiogenesis ; bronchopulmonary dysplasia ; echocardiography ; humans ; hyperoxia ; hypertension ; lungs ; mice ; morphometry ; normoxia ; premature birth
Language	English
Dates of publication	2021-1129
Publishing place	Multidisciplinary Digital Publishing Institute
Document type	Article
ZDB-ID	2704216-9
ISSN	2076-3921
ISSN	2076-3921
DOI	10.3390/antiox10121913
Database	NAL-Catalogue (AGRICOLA)

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