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MCID: PRS030
MIFTS: 42

Persistent Fetal Circulation Syndrome

Categories: Fetal diseases, Cardiovascular diseases
Genes Tissues Related diseases Publications Pathways Drugs
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Aliases & Classifications for Persistent Fetal Circulation Syndrome

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MalaCards integrated aliases for Persistent Fetal Circulation Syndrome:

Name: Persistent Fetal Circulation Syndrome 12 41 14 69
Congenital Alveolar Capillary Dysplasia with Misalignment of Pulmonary Veins 12
Persistent Pulmonary Hypertension of the Newborn 12
Persistent Fetal Circulation 12
Fetal Circulation 12

Classifications:

MalaCards categories:
Global: Fetal diseases
Anatomical: Cardiovascular diseases
See all MalaCards categories (disease lists)
ICD10: 32


External Ids:

Disease Ontology 12 DOID:13042
ICD10 32 P29.3
ICD9CM 34 747.83
MeSH 41 D010547
NCIt 46 C85006
UMLS 69 C0031190
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Summaries for Persistent Fetal Circulation Syndrome

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MalaCards based summary : Persistent Fetal Circulation Syndrome, also known as congenital alveolar capillary dysplasia with misalignment of pulmonary veins, is related to pulmonary hypertension and alveolar capillary dysplasia with misalignment of pulmonary veins. An important gene associated with Persistent Fetal Circulation Syndrome is CSH1 (Chorionic Somatomammotropin Hormone 1), and among its related pathways/superpathways are HIF-1 signaling pathway and Corticotropin-releasing hormone signaling pathway. The drugs Benzocaine and Nitric Oxide have been mentioned in the context of this disorder. Affiliated tissues include lung, brain and endothelial, and related phenotypes are homeostasis/metabolism and adipose tissue

Wikipedia : 72 Persistent fetal circulation (also called Persistent Pulmonary Hypertension of the Newborn, PPHN) is a... more...

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Related Diseases for Persistent Fetal Circulation Syndrome

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Diseases related to Persistent Fetal Circulation Syndrome via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 62)
# Related Disease Score Top Affiliating Genes
1 pulmonary hypertension 29.3 EDN1 EDNRA NOS3 PDE5A
2 alveolar capillary dysplasia with misalignment of pulmonary veins 11.3
3 prinzmetal's variant angina 10.5 EDN1 NOS3
4 hypothalamic disease 10.5 CRH POMC
5 acth deficiency, isolated 10.5 CRH POMC
6 coronary artery vasospasm 10.4 EDN1 NOS3
7 pituitary carcinoma 10.4 CRH POMC
8 non-gestational choriocarcinoma 10.4 CSH1 IGFBP1
9 nelson syndrome 10.4 CRH POMC
10 hypoadrenalism 10.4 CRH POMC
11 chronic mountain sickness 10.4 EDN1 NOS3
12 vasculogenic impotence 10.4 NOS3 PDE5A
13 orthostatic intolerance 10.3 EDN1 NOS3 POMC
14 urinary system disease 10.3 CRH EDN1 POMC
15 gestational choriocarcinoma 10.3 ABCG2 CSH1
16 acth-secreting pituitary adenoma 10.3 CRH POMC
17 sexual disorder 10.3 EDN1 NOS3 PDE5A
18 ischemic optic neuropathy 10.3 EDN1 NOS3 PDE5A
19 nonarteritic anterior ischemic optic neuropathy 10.3 EDN1 NOS3 PDE5A
20 impotence 10.3 EDN1 NOS3 PDE5A
21 limb ischemia 10.3 EDN1 NOS3 PGF
22 adrenal cortex disease 10.2 CRH HSD11B2 POMC
23 adrenal gland disease 10.2 CRH HSD11B2 POMC
24 disease of mental health 10.2 CRH PDE5A POMC
25 arteries, anomalies of 10.2 EDN1 NOS3 PGF
26 mild pre-eclampsia 10.2 IGFBP1 PGF
27 perinephritis 10.2 EDN1 EDNRA
28 adrenal cortical hypofunction 10.2 CRH POMC
29 mandibulofacial dysostosis with alopecia 10.2 EDN1 EDNRA
30 conn's syndrome 10.2 CRH HSD11B2 POMC
31 steroid inherited metabolic disorder 10.2 HSD11B2 POMC
32 acute chest syndrome 10.1 EDN1 NOS3
33 acidophil adenoma 10.1 IGF1 POMC
34 choriocarcinoma 10.1 ABCG2 CSH1 HSD11B2
35 pituitary infarct 10.1 IGF1 POMC
36 hepatopulmonary syndrome 10.1 EDN1 EDNRA NOS3
37 portal hypertension 10.1 EDN1 EDNRA NOS3
38 aortic coarctation 10.0 EDN1 NOS3
39 hyperpituitarism 10.0 IGF1 POMC
40 sheehan syndrome 9.9 CRH IGF1 POMC
41 hypothyroidism, congenital, nongoitrous, 4 9.9 IGF1 POMC
42 fasting hypoglycemia 9.9 CRH IGF1 POMC
43 endocrine organ benign neoplasm 9.9 CRH IGF1 POMC
44 pituitary gland disease 9.9 CRH IGF1 POMC
45 gonadal disease 9.9 CRH IGF1 POMC
46 hypopituitarism 9.9 CRH IGF1 POMC
47 bulimia nervosa 2 9.9 CRH IGF1 POMC
48 sleep apnea 9.9 EDN1 IGF1 NOS3
49 chronic fatigue syndrome 9.9 CRH IGF1 POMC
50 hyperandrogenism 9.9 IGF1 IGFBP1 POMC

Graphical network of the top 20 diseases related to Persistent Fetal Circulation Syndrome:



Diseases related to Persistent Fetal Circulation Syndrome
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Symptoms & Phenotypes for Persistent Fetal Circulation Syndrome

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MGI Mouse Phenotypes related to Persistent Fetal Circulation Syndrome:

43
# Description MGI Source Accession Score Top Affiliating Genes
1 homeostasis/metabolism MP:0005376 9.9 HSD11B2 IGF1 NOS3 PGF ABCG2 POMC
2 adipose tissue MP:0005375 9.73 IGF1 NOS3 PGF ABCG2 POMC CRH
3 immune system MP:0005387 9.61 FCGRT IGF1 IGFBP1 NOS3 PGF ABCG2
4 renal/urinary system MP:0005367 9.23 HSD11B2 IGF1 NOS3 ABCG2 POMC CRH
Sources

Drugs & Therapeutics for Persistent Fetal Circulation Syndrome

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Drugs for Persistent Fetal Circulation Syndrome (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 53)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Benzocaine Approved, Investigational Phase 4,Phase 3 1994-09-7, 94-09-7 2337
2
Nitric Oxide Approved Phase 4,Phase 3,Phase 1,Phase 2 10102-43-9 145068
3
Adenosine Approved, Investigational Phase 4 58-61-7 60961
4
Iloprost Approved, Investigational Phase 4,Phase 1,Phase 2 78919-13-8 6443959
5
Citric Acid Approved, Nutraceutical, Vet_approved Phase 4,Phase 3,Phase 2,Phase 1 77-92-9 311
6 tannic acid Approved, Nutraceutical Phase 4,Phase 3
7 Pharmaceutical Solutions Phase 4,Phase 3
8 Phosphodiesterase 5 Inhibitors Phase 4,Phase 3,Phase 2,Phase 1
9 Phosphodiesterase Inhibitors Phase 4,Phase 3,Phase 2,Phase 1
10 Sildenafil Citrate Phase 4,Phase 3,Phase 2,Phase 1 171599-83-0
11 Vasodilator Agents Phase 4,Phase 3,Phase 2,Phase 1
12 Anti-Asthmatic Agents Phase 4,Phase 3,Phase 2,Phase 1
13 Respiratory System Agents Phase 4,Phase 3,Phase 2,Phase 1
14 Antioxidants Phase 4,Phase 3,Phase 1,Phase 2
15 Autonomic Agents Phase 4,Phase 3,Phase 1,Phase 2
16 Bronchodilator Agents Phase 4,Phase 3,Phase 1,Phase 2
17 Endothelium-Dependent Relaxing Factors Phase 4,Phase 3,Phase 1,Phase 2
18 Neurotransmitter Agents Phase 4,Phase 3,Phase 1,Phase 2
19 Peripheral Nervous System Agents Phase 4,Phase 3,Phase 1,Phase 2
20 Protective Agents Phase 4,Phase 3,Phase 2,Phase 1
21 Endothelial Growth Factors Phase 4
22 Mitogens Phase 4
23 Platelet Aggregation Inhibitors Phase 4,Phase 2,Phase 1
24 Citrate Nutraceutical Phase 4,Phase 3,Phase 2,Phase 1
25
Progesterone Approved, Vet_approved Phase 3 57-83-0 5994
26
Bosentan Approved, Investigational Phase 3 147536-97-8 104865
27 Hormone Antagonists Phase 3,Phase 2
28 Hormones Phase 3,Phase 2
29 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 3,Phase 2
30 11-hydroxyprogesterone Phase 3
31 17-alpha-hydroxy-progesterone caproate Phase 3
32 Estrogen Antagonists Phase 3
33 Estrogens Phase 3
34 Progestins Phase 3
35 Antihypertensive Agents Phase 3,Phase 2
36 Endothelin Receptor Antagonists Phase 3
37 Caproate Nutraceutical Phase 3
38
Betamethasone Approved, Vet_approved Phase 2 378-44-9 9782
39
Treprostinil Approved, Investigational Phase 2 81846-19-7 6918140 54786
40
Milrinone Approved Phase 2 78415-72-2 4197
41 Anti-Inflammatory Agents Phase 2
42 Betamethasone benzoate Phase 2
43 Betamethasone sodium phosphate Phase 2
44 Betamethasone Valerate Phase 2 2152-44-5
45 Betamethasone-17,21-dipropionate Phase 2
46 glucocorticoids Phase 2
47 Antidepressive Agents, Tricyclic Phase 2
48 Tezosentan Phase 2
49 Phosphodiesterase 3 Inhibitors Phase 2
50 Antibodies

Interventional clinical trials:

(show all 31)

# Name Status NCT ID Phase Drugs
1 Oral Sildenafil in Persistent Pulmonary Hypertension Secondary to Meconium Aspiration Syndrome in Newborns Completed NCT01757782 Phase 4 Oral Sildenafil;Placebo (distilled water)
2 Inhaled Nitric Oxide by Oxygen Hood in Neonates Completed NCT00732537 Phase 4 inhaled Nitric Oxide;Oxygen (>90% by hood) - standard therapy
3 Early iNO for Oxidative Stress, Vascular Tone and Inflammation in Babies With Hypoxic Respiratory Failure Recruiting NCT01891500 Phase 4 Inhaled nitric oxide;Nitrogen Gas;Crossover iNO
4 Inhaled Iloprost for the Treatment of Persistent Pulmonary Hypertension in the Term and Near Term Infants. Terminated NCT00409526 Phase 4 Inhaled Iloprost
5 Sildenofil in Persistent Pulmonary Hypertension in Newborns Unknown status NCT01558466 Phase 3 Sildenafil;diluent
6 Trial of Progesterone in Twins and Triplets to Prevent Preterm Birth (STTARS) Completed NCT00099164 Phase 3 17 alpha-hydroxyprogesterone caproate (17P)
7 A Study To Evaluate Safety And Efficacy Of IV Sildenafil In The Treatment Of Neonates With Persistent Pulmonary Hypertension Of The Newborn Recruiting NCT01720524 Phase 3 placebo;iv sildenafil
8 Persistent Pulmonary Hypertension of the Newborn Terminated NCT01389856 Phase 3 Bosentan;Matching placebo
9 Inhaled Nitric Oxide Study for Respiratory Failure in Newborns Terminated NCT00005776 Phase 3 Inhaled nitric oxide;Placebo
10 Early Inhaled Nitric Oxide for Respiratory Failure in Newborns Terminated NCT00005773 Phase 3 Inhaled Nitric Oxide;Standard iNO therapy
11 Efficacy of Antenatal Steroids in Reducing Respiratory Morbidities in Late Preterm Infants Unknown status NCT01206946 Phase 2 Betamethasone
12 A Non-Interventional Follow Up Study For Subjects Who Received Sildenafil for Persistent Pulmonary Hypertension of the Newborn (PPHN) Completed NCT01801982 Phase 2
13 Remodulin as Add-on Therapy for the Treatment of Persistent Pulmonary Hypertension of the Newborn Recruiting NCT02261883 Phase 2 IV Remodulin;Placebo
14 Milrinone in Congenital Diaphragmatic Hernia Recruiting NCT02951130 Phase 2 Milrinone;Placebo (5% Dextrose)
15 Study To Investigate Safety And Efficacy Of Sildenafil In The Newborns With Persistent Pulmonary Hypertension (PPHN) Terminated NCT01069861 Phase 2 sildanefil
16 IV Sildenafil Persistent Pulmonary Hypertension Of The Newborn Withdrawn NCT01360671 Phase 2 Sildenafil
17 Inhaled Iloprost as an Adjunct to Inhaled Nitric Oxide in Pediatric Critical Care Patients Withdrawn NCT00981591 Phase 1, Phase 2 Iloprost;Placebo
18 Inhaled PGE1 in Neonatal Hypoxemic Respiratory Failure Withdrawn NCT00598429 Phase 2 Inhaled Prostaglandin E1
19 Pharmacokinetics of Sildenafil in Premature Infants Completed NCT01670136 Phase 1 1 dose of sildenafil
20 Nitro Oxide Inhalation Continued With Sildenafil on Neonatal Persistent Pulmonary Hypertension Unknown status NCT01373749 NO inhalation;NO inhalation continued with sildenafil
21 Circulatory Changes During Venovenous (VV)- and Venoarterial (VA) Extracorporeal Membrane Oxygenation (ECMO) Unknown status NCT00622492
22 Persistent Pulmonary Hypertension of the Newborn (PPHN) Observational Study Completed NCT01203423
23 NO Need to Ventilate: A Trial of Non-invasive Inhaled Nitric Oxide in Persistent Pulmonary Hypertension of the Newborn Completed NCT00139217 iNO
24 Risk Factors for Pulmonary Hypertension of the Newborn Completed NCT00005497
25 Epidemiology of Persistent Pulmonary Hypertension of the Newborn - SCOR in Lung Biology and Diseases in Infants and Children Completed NCT00005323
26 Antibody Secreting Cell and Cyotokine Profiles in Neonates on ECMO Completed NCT00371241
27 PTGS1 Genetic Variation and Increased Risk for Persistent Pulmonary Hypertension of the Newborn Recruiting NCT00710177
28 Markers of Inflammation and Lung Recovery in ECMO Patients for PPHN Recruiting NCT02940327
29 Plethismographic Perfusion Index in Neonates Recruiting NCT02380040
30 Pharmacokinetic Study of Milrinone in Babies With Persistent Pulmonary Hypertension of the Newborn Terminated NCT01088997 Milrinone Lactate
31 N-Terminal Pro-brain Natriuretic Peptide Hormone and Persistent Pulmonary Hypertension Terminated NCT00443859

Search NIH Clinical Center for Persistent Fetal Circulation Syndrome

Inferred drug relations via UMLS 69 / NDF-RT 47 :


Cochrane evidence based reviews: persistent fetal circulation syndrome

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Genetic Tests for Persistent Fetal Circulation Syndrome

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Anatomical Context for Persistent Fetal Circulation Syndrome

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MalaCards organs/tissues related to Persistent Fetal Circulation Syndrome:

38
Lung, Brain, Endothelial
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Publications for Persistent Fetal Circulation Syndrome

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Articles related to Persistent Fetal Circulation Syndrome:

# Title Authors Year
1
Treatment of persistent fetal circulation syndrome of the newborn. Comparison of different doses of tolazoline. ( 3104058 )
1987
2
Persistent pulmonary hypertension of the neonate (persistent fetal circulation syndrome). ( 6424420 )
1983
3
Altered lung mechanics in neonates with persistent fetal circulation syndrome. ( 7460588 )
1981
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Variations for Persistent Fetal Circulation Syndrome

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Expression for Persistent Fetal Circulation Syndrome

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Search GEO for disease gene expression data for Persistent Fetal Circulation Syndrome.
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Pathways for Persistent Fetal Circulation Syndrome

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Pathways related to Persistent Fetal Circulation Syndrome according to GeneCards Suite gene sharing:

(show all 13)
# Super pathways Score Top Affiliating Genes
1
HIF-1 signaling pathway 36
11.67 EDN1 IGF1 NOS3
2
Corticotropin-releasing hormone signaling pathway 1
11.6 CRH NOS3 POMC
3
HIF-1-alpha transcription factor network 1
11.39 ABCG2 EDN1 IGFBP1
4
HIF1Alpha Pathway 60
11.23 EDN1 NOS3 SLC2A3
5
Photodynamic therapy-induced HIF-1 survival signaling 1
11 EDN1 IGFBP1 SLC2A3
6
Endothelin Pathways 1
10.86 EDN1 EDNRA NOS3
7
Regulation of Insulin-like Growth Factor (IGF) transport and uptake by Insulin-like Growth Factor Binding Proteins (IGFBPs) 62
10.85 IGF1 IGFBP1
8
Zidovudine Pathway, Pharmacokinetics/Pharmacodynamics 57
10.81 ABCG2 SLC22A11
9
Prostaglandin Synthesis and Regulation 1
10.61 EDN1 EDNRA HSD11B2
10
NO-dependent CFTR activation (normal and CF) 21
10.59 NOS3 PDE5A
11
Uricosurics Pathway, Pharmacodynamics 57
10.51 ABCG2 SLC22A11
12
Glucocorticoid Pathway (HPA Axis), Pharmacodynamics 57
10.21 CRH POMC
13
EGFR Transactivation by Gastrin 62
Show member pathways
 10.21 EDN1 EDNRA IGF1
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GO Terms for Persistent Fetal Circulation Syndrome

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Biological processes related to Persistent Fetal Circulation Syndrome according to GeneCards Suite gene sharing:

(show all 18)
# Name GO ID Score Top Affiliating Genes
1 regulation of receptor activity GO:0010469 9.8 CSH1 EDN1 IGF1 PGF POMC
2 female pregnancy GO:0007565 9.69 CRH HSD11B2 PGF
3 response to drug GO:0042493 9.65 ABCG2 CRH EDN1 HSD11B2 PGF
4 response to hypoxia GO:0001666 9.62 EDN1 EDNRA HSD11B2 PGF
5 positive regulation of mitotic nuclear division GO:0045840 9.58 EDN1 IGF1
6 neural crest cell development GO:0014032 9.57 EDN1 EDNRA
7 vasoconstriction GO:0042310 9.56 EDN1 EDNRA
8 regulation of blood vessel size GO:0050880 9.55 EDN1 NOS3
9 negative regulation of cAMP biosynthetic process GO:0030818 9.54 EDN1 EDNRA
10 positive regulation of cell growth involved in cardiac muscle cell development GO:0061051 9.52 EDN1 IGF1
11 urate metabolic process GO:0046415 9.49 ABCG2 SLC22A11
12 artery smooth muscle contraction GO:0014824 9.48 EDN1 EDNRA
13 negative regulation of smooth muscle cell apoptotic process GO:0034392 9.46 EDN1 IGF1
14 glucocorticoid biosynthetic process GO:0006704 9.43 CRH HSD11B2
15 regulation of systemic arterial blood pressure by endothelin GO:0003100 9.37 EDN1 NOS3
16 glucose transport GO:0015758 9.33 EDN1 EDNRA SLC2A3
17 positive regulation of cardiac muscle hypertrophy GO:0010613 9.13 EDN1 IGF1 PDE5A
18 regulation of blood pressure GO:0008217 8.92 EDN1 EDNRA NOS3 POMC

Molecular functions related to Persistent Fetal Circulation Syndrome according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 receptor binding GO:0005102 9.46 CRH EDN1 IGFBP1 POMC
2 sodium-independent organic anion transmembrane transporter activity GO:0015347 9.26 SLC22A11 SLCO2B1
3 hormone activity GO:0005179 9.02 CRH CSH1 EDN1 IGF1 POMC
4 organic anion transmembrane transporter activity GO:0008514 8.96 SLC22A11 SLCO2B1
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Sources for Persistent Fetal Circulation Syndrome

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3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 FMA
27 GO
28 GTR
29 HGMD
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 MedGen
41 MeSH
42 MESH via Orphanet
43 MGI
45 NCI
46 NCIt
47 NDF-RT
50 NINDS
51 Novoseek
53 OMIM
54 OMIM via Orphanet
58 PubMed
60 QIAGEN
65 SNOMED-CT via HPO
66 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
The MalaCards human disease database index: 1-9 A B C D E F G H I J K L M N O P Q R S T U V W X Y Z
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