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PedAM

Pediatric Disease Annotations & Medicines



   persistent fetal circulation syndrome
  

Disease ID 1080
Disease persistent fetal circulation syndrome
Definition
A syndrome of persistent PULMONARY HYPERTENSION in the newborn infant (INFANT, NEWBORN) without demonstrable HEART DISEASES. This neonatal condition can be caused by severe pulmonary vasoconstriction (reactive type), hypertrophy of pulmonary arterial muscle (hypertrophic type), or abnormally developed pulmonary arterioles (hypoplastic type). The newborn patient exhibits CYANOSIS and ACIDOSIS due to the persistence of fetal circulatory pattern of right-to-left shunting of blood through a patent ductus arteriosus (DUCTUS ARTERIOSUS, PATENT) and at times a patent foramen ovale (FORAMEN OVALE, PATENT).
Synonym
acd mpv
acd-mpv
acdmpv
alveolar capillary dysplasia with misalignment of pulmonary veins
alveolar capillary dysplasia with misalignment of pulmonary veins and other congenital anomalies
circ persistent fetal
circulation, persistent fetal
familial persistent pulmonary hypertension of the newborn
fetal circ persistent
fetal circulation
fetal circulation, persistent
hypertension pulm of newborn persistent
hypertension, pulmonary, of newborn, persistent
misalignment of the pulmonary vessels
persistent fetal circ
persistent fetal circulation
persistent fetal circulation (disorder)
persistent fetal circulation syndrome (disorder)
persistent fetal circulation syndrome [disease/finding]
persistent foetal circulation
persistent foetal circulation syndrome
persistent pulm hypertension of newborn
persistent pulmonary hypertension of newborn
persistent pulmonary hypertension of the newborn
persistent pulmonary hypertension of the newborn (disorder)
pfc - persistent fetal circulation
pfc - persistent foetal circulation
pfc syndrome
pphn
pphn - persistent pulmonary hypertension in newborn
pulmonary hypertension, familial persistent, of the newborn
OMIM
DOID
ICD10
UMLS
C0031190
MeSH
SNOMED-CT
Curated Gene
Entrez_id | Symbol | Resource(Total Genes:2)
CPS1  |  1373  |  CTD_human
FOXF1  |  2294  |  CLINVAR;ORPHANET;UNIPROT
Inferring Gene(Waiting for update.)
Text Mined Gene
Entrez_id | Symbol | Score | Resource(Total Genes:217)
126393  |  HSPB6  |  DISEASES
6515  |  SLC2A3  |  DISEASES
343641  |  TGM6  |  DISEASES
7023  |  TFAP4  |  DISEASES
3956  |  LGALS1  |  DISEASES
50  |  ACO2  |  DISEASES
5020  |  OXT  |  DISEASES
2158  |  F9  |  DISEASES
343  |  AQP8  |  DISEASES
1666  |  DECR1  |  DISEASES
11129  |  CLASRP  |  DISEASES
2217  |  FCGRT  |  DISEASES
6822  |  SULT2A1  |  DISEASES
8976  |  WASL  |  DISEASES
116449  |  CLNK  |  DISEASES
4012  |  LNPEP  |  DISEASES
2908  |  NR3C1  |  DISEASES
56920  |  SEMA3G  |  DISEASES
3485  |  IGFBP2  |  DISEASES
5624  |  PROC  |  DISEASES
335  |  APOA1  |  DISEASES
7276  |  TTR  |  DISEASES
9429  |  ABCG2  |  DISEASES
3764  |  KCNJ8  |  DISEASES
847  |  CAT  |  DISEASES
2693  |  GHSR  |  DISEASES
2806  |  GOT2  |  DISEASES
1948  |  EFNB2  |  DISEASES
51081  |  MRPS7  |  DISEASES
6945  |  MLX  |  DISEASES
3976  |  LIF  |  DISEASES
7166  |  TPH1  |  DISEASES
3630  |  INS  |  DISEASES
376497  |  SLC27A1  |  DISEASES
2056  |  EPO  |  DISEASES
6737  |  TRIM21  |  DISEASES
6599  |  SMARCC1  |  DISEASES
10267  |  RAMP1  |  DISEASES
23640  |  HSPBP1  |  DISEASES
10894  |  LYVE1  |  DISEASES
10468  |  FST  |  DISEASES
152831  |  KLB  |  DISEASES
3357  |  HTR2B  |  DISEASES
3569  |  IL6  |  DISEASES
8521  |  GCM1  |  DISEASES
1588  |  CYP19A1  |  DISEASES
6741  |  SSB  |  DISEASES
9360  |  PPIG  |  DISEASES
490  |  ATP2B1  |  DISEASES
10060  |  ABCC9  |  DISEASES
4659  |  PPP1R12A  |  DISEASES
9388  |  LIPG  |  DISEASES
5858  |  PZP  |  DISEASES
3290  |  HSD11B1  |  DISEASES
949  |  SCARB1  |  DISEASES
4756  |  NEO1  |  DISEASES
6521  |  SLC4A1  |  DISEASES
2294  |  FOXF1  |  DISEASES
28965  |  SLC27A6  |  DISEASES
25939  |  SAMHD1  |  DISEASES
5605  |  MAP2K2  |  DISEASES
25759  |  SHC2  |  DISEASES
5443  |  POMC  |  DISEASES
8614  |  STC2  |  DISEASES
5244  |  ABCB4  |  DISEASES
5243  |  ABCB1  |  DISEASES
6895  |  TARBP2  |  DISEASES
11001  |  SLC27A2  |  DISEASES
1583  |  CYP11A1  |  DISEASES
4225  |  MEP1B  |  DISEASES
3487  |  IGFBP4  |  DISEASES
55244  |  SLC47A1  |  DISEASES
5972  |  REN  |  DISEASES
1393  |  CRHBP  |  DISEASES
154091  |  SLC2A12  |  DISEASES
6581  |  SLC22A3  |  DISEASES
3484  |  IGFBP1  |  DISEASES
2041  |  EPHA1  |  DISEASES
1392  |  CRH  |  DISEASES
123  |  PLIN2  |  DISEASES
4851  |  NOTCH1  |  DISEASES
133  |  ADM  |  DISEASES
114571  |  SLC22A9  |  DISEASES
83706  |  FERMT3  |  DISEASES
5741  |  PTH  |  DISEASES
2321  |  FLT1  |  DISEASES
23576  |  DDAH1  |  DISEASES
8714  |  ABCC3  |  DISEASES
5468  |  PPARG  |  DISEASES
11309  |  SLCO2B1  |  DISEASES
1636  |  ACE  |  DISEASES
5739  |  PTGIR  |  DISEASES
6285  |  S100B  |  DISEASES
1234  |  CCR5  |  DISEASES
162417  |  NAGS  |  DISEASES
6006  |  RHCE  |  DISEASES
4880  |  NPPC  |  DISEASES
213  |  ALB  |  DISEASES
7349  |  UCN  |  DISEASES
4846  |  NOS3  |  DISEASES
360  |  AQP3  |  DISEASES
10999  |  SLC27A4  |  DISEASES
94274  |  PPP1R14A  |  DISEASES
124935  |  SLC43A2  |  DISEASES
55867  |  SLC22A11  |  DISEASES
5617  |  PRL  |  DISEASES
3479  |  IGF1  |  DISEASES
64137  |  ABCG4  |  DISEASES
7064  |  THOP1  |  DISEASES
203100  |  HTRA4  |  DISEASES
6579  |  SLCO1A2  |  DISEASES
6373  |  CXCL11  |  DISEASES
6383  |  SDC2  |  DISEASES
2147  |  F2  |  DISEASES
358  |  AQP1  |  DISEASES
54968  |  TMEM70  |  DISEASES
8557  |  TCAP  |  DISEASES
3952  |  LEP  |  DISEASES
23166  |  STAB1  |  DISEASES
92745  |  SLC38A5  |  DISEASES
9153  |  SLC28A2  |  DISEASES
1909  |  EDNRA  |  DISEASES
7094  |  TLN1  |  DISEASES
1442  |  CSH1  |  DISEASES
3291  |  HSD11B2  |  DISEASES
9370  |  ADIPOQ  |  DISEASES
55553  |  SOX6  |  DISEASES
9798  |  IST1  |  DISEASES
2520  |  GAST  |  DISEASES
71  |  ACTG1  |  DISEASES
796  |  CALCA  |  DISEASES
6007  |  RHD  |  DISEASES
6546  |  SLC8A1  |  DISEASES
2689  |  GH2  |  DISEASES
10057  |  ABCC5  |  DISEASES
2152  |  F3  |  DISEASES
8912  |  CACNA1H  |  DISEASES
57153  |  SLC44A2  |  DISEASES
1551  |  CYP3A7  |  DISEASES
10011  |  SRA1  |  DISEASES
51477  |  ISYNA1  |  DISEASES
66005  |  CHID1  |  DISEASES
1528  |  CYB5A  |  DISEASES
866  |  SERPINA6  |  DISEASES
7273  |  TTN  |  DISEASES
309  |  ANXA6  |  DISEASES
8654  |  PDE5A  |  DISEASES
1717  |  DHCR7  |  DISEASES
5879  |  RAC1  |  DISEASES
146059  |  CDAN1  |  DISEASES
493  |  ATP2B4  |  DISEASES
871  |  SERPINH1  |  DISEASES
3135  |  HLA-G  |  DISEASES
2993  |  GYPA  |  DISEASES
9619  |  ABCG1  |  DISEASES
5083  |  PAX9  |  DISEASES
23038  |  WDTC1  |  DISEASES
440730  |  TRIM67  |  DISEASES
7779  |  SLC30A1  |  DISEASES
25902  |  MTHFD1L  |  DISEASES
60676  |  PAPPA2  |  DISEASES
6675  |  UAP1  |  DISEASES
11000  |  SLC27A3  |  DISEASES
3284  |  HSD3B2  |  DISEASES
1244  |  ABCC2  |  DISEASES
6342  |  SCP2  |  DISEASES
5360  |  PLTP  |  DISEASES
7417  |  VDAC2  |  DISEASES
2268  |  FGR  |  DISEASES
23446  |  SLC44A1  |  DISEASES
19  |  ABCA1  |  DISEASES
23564  |  DDAH2  |  DISEASES
1460  |  CSNK2B  |  DISEASES
64078  |  SLC28A3  |  DISEASES
29979  |  UBQLN1  |  DISEASES
4878  |  NPPA  |  DISEASES
4524  |  MTHFR  |  DISEASES
1910  |  EDNRB  |  DISEASES
6518  |  SLC2A5  |  DISEASES
1906  |  EDN1  |  DISEASES
795  |  S100G  |  DISEASES
551  |  AVP  |  DISEASES
1107  |  CHD3  |  DISEASES
3486  |  IGFBP3  |  DISEASES
6547  |  SLC8A3  |  DISEASES
6649  |  SOD3  |  DISEASES
2706  |  GJB2  |  DISEASES
6736  |  SRY  |  DISEASES
94  |  ACVRL1  |  DISEASES
1443  |  CSH2  |  DISEASES
2643  |  GCH1  |  DISEASES
174  |  AFP  |  DISEASES
144195  |  SLC2A14  |  DISEASES
319  |  APOF  |  DISEASES
1394  |  CRHR1  |  DISEASES
4363  |  ABCC1  |  DISEASES
137970  |  UNC5D  |  DISEASES
7018  |  TF  |  DISEASES
501  |  ALDH7A1  |  DISEASES
4295  |  MLN  |  DISEASES
2086  |  ERV3-1  |  DISEASES
3481  |  IGF2  |  DISEASES
2880  |  GPX5  |  DISEASES
7124  |  TNF  |  DISEASES
1028  |  CDKN1C  |  DISEASES
6513  |  SLC2A1  |  DISEASES
30816  |  ERVW-1  |  DISEASES
5447  |  POR  |  DISEASES
2317  |  FLNB  |  DISEASES
10480  |  EIF3M  |  DISEASES
1961  |  EGR4  |  DISEASES
8972  |  MGAM  |  DISEASES
5053  |  PAH  |  DISEASES
5228  |  PGF  |  DISEASES
6625  |  SNRNP70  |  DISEASES
94161  |  SNORD46  |  DISEASES
100128252  |  ZNF667-AS1  |  DISEASES
Locus(Waiting for update.)
Disease ID 1080
Disease persistent fetal circulation syndrome
Integrated Phenotype(Waiting for update.)
Text Mined Phenotype(Waiting for update.)
Disease ID 1080
Disease persistent fetal circulation syndrome
Manually Symptom(Waiting for update.)
Text Mined Symptom(Waiting for update.)
Manually Genotype(Total Text Mining Genotypes:0)
(Waiting for update.)
All Snps(Total Genotypes:2)
snpId pubmedId geneId geneSymbol diseaseId sourceId sentence score Year geneSymbol_dbSNP CHROMOSOME POS REF ALT
rs121909336NA2294FOXF1umls:C0031190CLINVARNA0.363528744NAFOXF1;FENDRR1686510794CA
rs121909337NA2294FOXF1umls:C0031190CLINVARNA0.363528744NAFOXF11686513083TC
GWASdb Annotation(Total Genotypes:0)
(Waiting for update.)
GWASdb Snp Trait(Total Genotypes:0)
(Waiting for update.)
Mapped by lexical matching(Total Items:0)
(Waiting for update.)
Mapped by homologous gene(Total Items:0)
(Waiting for update.)
Chemical(Total Drugs:2)
CUI ChemicalName ChemicalID CasRN DiseaseName DiseaseID DirectEvidence PubMedIDs
C0031190indomethacinD00721353-86-1persistent fetal circulation syndromeMESH:D010547marker/mechanism15008397
C0031190nitric oxideD00956910102-43-9persistent fetal circulation syndromeMESH:D010547marker/mechanism11407344
FDA approved drug and dosage information(Total Drugs:1)
DiseaseID Drug_name active_ingredients strength Dosage Form/Route Marketing Status TE code RLD RS
MESH:D010547inomaxnitric oxide100PPM Federal Register determination that product was not discontinued or withdrawn for safety or efficacy reasonsGAS;INHALATIONDiscontinuedNoneYesNo
FDA labeling changes(Total Drugs:1)
DiseaseID Pediatric_Labeling_Date Trade_Name Generic_Name_or_Proper_Name Indications Studied Label Changes Summary Product Labeling BPCA(B) PREA(P) BPCA(B) and PREA(P) Pediatric Rule (R) Sponsor Pediatric Exclusivity Granted Date NNPS
MESH:D01054712/21/2010inomaxnitric oxidePrevention of bronchopulmonary dysplasiaINOmax is not indicated for prevention of BPD in preterm neonates d 34 weeks gestational age.Efficacy for the prevention of BPD in preterm infants was not established in three ldouble-blind, placebo-controlled clinical trials in a total of 2,149 preterm infants Information on clinical trials, adverse reactionLabelingB---INO Therapeutics2/11/2010FALSE'