MCID: PLM013
MIFTS: 31

Pulmonary Immaturity

Categories: Fetal diseases, Respiratory diseases

Aliases & Classifications for Pulmonary Immaturity

MalaCards integrated aliases for Pulmonary Immaturity:

Name: Pulmonary Immaturity 12 15
Primary Atelectasis, in Perinatal Period 12 70
Primary Atelectasis of Newborn 12 32

Classifications:



External Ids:

Disease Ontology 12 DOID:424
ICD9CM 34 770.4
SNOMED-CT 67 42908004
ICD10 32 P28.0
UMLS 70 C0270163

Summaries for Pulmonary Immaturity

Disease Ontology : 12 A lung disease that is characterized by pulmonary collapse, accompanied by hypoventilation which can affect a lobe, segment, or all of the lung, resulting in a decrease in the ventilation/perfusion ratio.

MalaCards based summary : Pulmonary Immaturity, also known as primary atelectasis, in perinatal period, is related to surfactant metabolism dysfunction, pulmonary, 1 and respiratory distress syndrome in premature infants. An important gene associated with Pulmonary Immaturity is SFTPB (Surfactant Protein B), and among its related pathways/superpathways are Phagosome and Diseases of metabolism. Affiliated tissues include lung, fetal lung and endothelial, and related phenotypes are Increased shRNA abundance (Z-score > 2) and Increased shRNA abundance (Z-score > 2)

Related Diseases for Pulmonary Immaturity

Diseases related to Pulmonary Immaturity via text searches within MalaCards or GeneCards Suite gene sharing:

(show all 38)
# Related Disease Score Top Affiliating Genes
1 surfactant metabolism dysfunction, pulmonary, 1 30.5 SFTPC SFTPB ABCA3
2 respiratory distress syndrome in premature infants 29.9 SFTPC SFTPB SFTPA1 ABCA3
3 respiratory failure 29.9 SFTPD SFTPC SFTPB ABCA3
4 newborn respiratory distress syndrome 29.1 SFTPD SFTPC SFTPB SFTPA2 SFTPA1 ABCA3
5 tracheal agenesis 10.2 SFTPD SFTPB
6 respiratory distress syndrome, infant 10.1 SFTPC ABCA3
7 pulmonary fibrosis predisposition 10.1 SFTPC SFTPA2
8 pulmonary hemosiderosis 10.1 SFTPB ABCA3
9 pneumonia 10.1 SFTPD SFTPC SFTPB
10 myringitis bullosa hemorrhagica 10.1 SFTPA2 SFTPA1
11 ventilation pneumonitis 10.1 SFTPD ABCA3
12 chronic congestive splenomegaly 10.1 SFTPC ABCA3
13 diaphragm disease 10.1 SFTPC SFTPB
14 meconium aspiration syndrome 10.1 SFTPB ABCA3
15 lipid pneumonia 10.0 SFTPC SFTPB ABCA3
16 diaphragmatic hernia, congenital 10.0
17 nonspecific interstitial pneumonia 10.0 SFTPD SFTPC ABCA3
18 cryptogenic organizing pneumonia 10.0 SFTPD SFTPC SFTPA2
19 allergic bronchopulmonary aspergillosis 9.9 SFTPD SFTPA2 SFTPA1
20 aspergillosis 9.9 SFTPD SFTPA2 SFTPA1
21 pulmonary alveolar microlithiasis 9.9 SFTPD SFTPA2 ABCA3
22 streptococcus pneumonia 9.9 SFTPA2 SFTPA1
23 surfactant dysfunction 9.9 SFTPC SFTPB SFTPA1 ABCA3
24 pulmonary alveolar proteinosis 9.9 SFTPD SFTPC SFTPB SFTPA1
25 preterm premature rupture of the membranes 9.9
26 adult respiratory distress syndrome 9.8 SFTPD SFTPC SFTPB ABCA3
27 middle ear disease 9.8 SFTPA2 SFTPA1
28 hermansky-pudlak syndrome 9.8 SFTPC SFTPB SFTPA2 ABCA3
29 pulmonary interstitial emphysema 9.7 SFTPD SFTPC SFTPB SFTPA1 ABCA3
30 idiopathic interstitial pneumonia 9.7 SFTPD SFTPC SFTPB SFTPA2 SFTPA1
31 neonatal respiratory failure 9.7 SFTPD SFTPC SFTPB SFTPA2 ABCA3
32 interstitial pneumonitis, desquamative, familial 9.7 SFTPD SFTPC SFTPB SFTPA2 ABCA3
33 acute interstitial pneumonia 9.7 SFTPD SFTPC SFTPB SFTPA2 ABCA3
34 interstitial lung disease 9.7 SFTPD SFTPC SFTPB SFTPA2 ABCA3
35 pulmonary fibrosis 9.6 SFTPD SFTPC SFTPA2 SFTPA1 ABCA3
36 ischiocoxopodopatellar syndrome with or without pulmonary arterial hypertension 9.5 SFTPD SFTPC SFTPB SFTPA2 SFTPA1 ABCA3
37 lung disease 9.5 SFTPD SFTPC SFTPB SFTPA2 SFTPA1 ABCA3
38 pulmonary fibrosis, idiopathic 9.5 SFTPD SFTPC SFTPB SFTPA2 SFTPA1 ABCA3

Graphical network of the top 20 diseases related to Pulmonary Immaturity:



Diseases related to Pulmonary Immaturity

Symptoms & Phenotypes for Pulmonary Immaturity

GenomeRNAi Phenotypes related to Pulmonary Immaturity according to GeneCards Suite gene sharing:

26 (show all 18)
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Increased shRNA abundance (Z-score > 2) GR00366-A-109 9.64 MAPK4 MAPKAPK5
2 Increased shRNA abundance (Z-score > 2) GR00366-A-123 9.64 MAPKAPK5
3 Increased shRNA abundance (Z-score > 2) GR00366-A-142 9.64 MAPK4
4 Increased shRNA abundance (Z-score > 2) GR00366-A-146 9.64 SFTPB
5 Increased shRNA abundance (Z-score > 2) GR00366-A-191 9.64 MAPK4
6 Increased shRNA abundance (Z-score > 2) GR00366-A-198 9.64 MAPKAPK5
7 Increased shRNA abundance (Z-score > 2) GR00366-A-20 9.64 SFTPB
8 Increased shRNA abundance (Z-score > 2) GR00366-A-213 9.64 MAPK4
9 Increased shRNA abundance (Z-score > 2) GR00366-A-32 9.64 SFTPB
10 Increased shRNA abundance (Z-score > 2) GR00366-A-38 9.64 MAPK4
11 Increased shRNA abundance (Z-score > 2) GR00366-A-48 9.64 CEBPA
12 Increased shRNA abundance (Z-score > 2) GR00366-A-55 9.64 SFTPB
13 Increased shRNA abundance (Z-score > 2) GR00366-A-60 9.64 MAPKAPK5
14 Increased shRNA abundance (Z-score > 2) GR00366-A-65 9.64 SFTPB
15 Increased shRNA abundance (Z-score > 2) GR00366-A-67 9.64 MAPK4
16 Increased shRNA abundance (Z-score > 2) GR00366-A-72 9.64 CEBPA
17 Increased shRNA abundance (Z-score > 2) GR00366-A-77 9.64 SFTPB
18 Increased shRNA abundance (Z-score > 2) GR00366-A-85 9.64 MAPK4 MAPKAPK5 SFTPB

MGI Mouse Phenotypes related to Pulmonary Immaturity:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 mortality/aging MP:0010768 9.7 ABCA3 CEBPA FOXJ1 MAPK4 MAPK6 MAPKAPK5
2 respiratory system MP:0005388 9.32 ABCA3 CEBPA FOXJ1 MAPK4 MAPK6 NDST1

Drugs & Therapeutics for Pulmonary Immaturity

Search Clinical Trials , NIH Clinical Center for Pulmonary Immaturity

Genetic Tests for Pulmonary Immaturity

Anatomical Context for Pulmonary Immaturity

MalaCards organs/tissues related to Pulmonary Immaturity:

40
Lung, Fetal Lung, Endothelial, Skin

Publications for Pulmonary Immaturity

Articles related to Pulmonary Immaturity:

(show top 50) (show all 63)
# Title Authors PMID Year
1
Efficacy and Safety of Corticosteroids' Administration for Pulmonary Immaturity in Anticipated Preterm Delivery. 61
33423644 2020
2
SP-D loaded PLGA nanoparticles as drug delivery system for prevention and treatment of premature infant's lung diseases. 61
32473376 2020
3
Persistent Respiratory Distress in the Term Neonate: Genetic Surfactant Deficiency Diseases. 61
31544695 2020
4
Concomitant deletion of HRAS and NRAS leads to pulmonary immaturity, respiratory failure and neonatal death in mice. 61
31685810 2019
5
Prematurity detection evaluating interaction between the skin of the newborn and light: protocol for the preemie-test multicentre clinical trial in Brazilian hospitals to validate a new medical device. 61
30842119 2019
6
Use of Insulin Glargine in the Management of Neonatal Hyperglycemia in an ELBW Infant. 61
29610159 2018
7
Columellar Reconstruction following Nasal Continuous Positive Airway Pressure Injury. 61
28938361 2018
8
Irx1 regulates dental outer enamel epithelial and lung alveolar type II epithelial differentiation. 61
28746823 2017
9
Structural and transcriptomic response to antenatal corticosteroids in an Erk3-null mouse model of respiratory distress. 61
27143398 2016
10
Administration of antenatal glucocorticoids and postnatal surfactant ameliorates respiratory distress syndrome-associated neonatal lethality in Erk3(-/-) mouse pups. 61
24732107 2014
11
Airway epithelial cell differentiation during lung organogenesis requires C/EBPα and C/EBPβ. 61
22411169 2012
12
ACOG Committee Opinion No. 475: antenatal corticosteroid therapy for fetal maturation. 61
21252775 2011
13
Targeted inactivation of Mapk4 in mice reveals specific nonredundant functions of Erk3/Erk4 subfamily mitogen-activated protein kinases. 61
20956558 2010
14
Techniques of early respiratory management of very low and extremely low birth weight infants. 61
20472532 2010
15
Loss of Erk3 function in mice leads to intrauterine growth restriction, pulmonary immaturity, and neonatal lethality. 61
19805361 2009
16
Lung development in the Holtzman rat is adversely affected by gestational exposure to 2,3,7,8-tetrachlorodibenzo-p-dioxin. 61
18990726 2009
17
Pretreatment with recombinant human vascular endothelial growth factor reduces virus replication and inflammation in a perinatal lamb model of respiratory syncytial virus infection. 61
17425433 2007
18
[Dilemmas about the antenatal use of corticosteroids for prevention of neonatal morbidity and mortality]. 61
15909887 2005
19
Toriello-Carey syndrome associated with respiratory failure and non-mechanical ileus. 61
12884435 2003
20
Increased expression of ICAM-1 and VCAM-1 in the lung of nitrofen-induced congenital diaphragmatic hernia in rats. 61
12759764 2003
21
Antenatal dexamethasone administration increases fetal lung DNA synthesis and RNA and protein content in nitrofen-induced congenital diaphragmatic hernia in rats. 61
11102548 2000
22
[Congenital hernia of the diaphragm. A retrospective study of 123 cases recorded in the Neonatal Medicine Department, URHC in Lille between 1985 and 1996]. 61
10701057 2000
23
Antenatal dexamethasone improves atrial natriuretic peptide receptors in hypoplastic lung in nitrofen-induced diaphragmatic hernia in rats. 61
10898224 2000
24
Exogenous surfactant therapy and mucus rheology in chronic obstructive airway diseases. 61
10656543 2000
25
Surfactant-deficient respiratory distress after elective delivery at 'term'. 61
10591427 1999
26
Time trends in twin perinatal mortality in northern England, 1982-94. Northern Region Perinatal Mortality Survey Steering Group. 61
10100810 1998
27
The value of classifying interstitial pneumonitis in childhood according to defined histological patterns. 61
9777385 1998
28
Prenatal glucocorticoids improve pulmonary morphometrics in fetal sheep with congenital diaphragmatic hernia. 61
9044125 1997
29
Prenatal glucocorticoid therapy reverses pulmonary immaturity in congenital diaphragmatic hernia in fetal sheep. 61
8857848 1996
30
[What does remain about bronchopulmonary dysplasia?]. 61
8685119 1996
31
Elevated cytokine levels in tracheobronchial aspirate fluids from ventilator treated neonates with bronchopulmonary dysplasia. 61
8775225 1996
32
Pulmonary surfactant dysfunction in congenital diaphragmatic hernia: experimental and clinical findings. 61
8790910 1996
33
[Pathogenesis of bronchopulmonary dysplasia]. 61
8528953 1995
34
Respiratory distress syndrome of the newborn infant. 61
7566832 1995
35
Prediction of 12-month neurodevelopmental outcome from a 6-month neurologic examination in premature infants. 61
7656508 1995
36
A clinico-pathological classification of perinatal deaths in the Faroe Islands. 61
7612533 1995
37
Antenatal glucocorticoid corrects pulmonary immaturity in experimentally induced congenital diaphragmatic hernia in rats. 61
7520565 1994
38
Combined antenatal thyrotropin-releasing hormone and low-dose glucocorticoid therapy improves the pulmonary biochemical immaturity in congenital diaphragmatic hernia. 61
8176620 1994
39
[Premature rupture of fetal membranes: problems and obstetric management]. 61
8119565 1993
40
Does maternal diabetes delay fetal pulmonary maturity? 61
8456880 1993
41
Surfactant: clinical applications. 61
1621634 1992
42
Cerebroelectrical depression following surfactant treatment in preterm neonates. 61
1557244 1992
43
[Ultrasound diagnosis of fetal lung maturity]. 61
1609529 1992
44
Hyaline membrane disease and early neonatal aldosterone metabolism in infants of less than 33 weeks gestation. 61
1867079 1991
45
Effects of maternal diabetes on fetal rat lung ion transport. Contribution of alveolar and bronchiolar epithelial cells to Na+,K(+)-ATPase expression. 61
1847938 1991
46
Pulmonary changes in congenital syphilis. 61
1749670 1991
47
Study on a new index of fetal respiratory function: a novel optical analysis of the amniotic fluid by the vacuum-dried infrared attenuated total reflection (ATR) method. 61
2131649 1990
48
Contribution to the assessment of steroid therapy in the prevention of respiratory distress syndrome in the neonate. 61
2292142 1990
49
[Continuous respiratory therapy of newborn and premature infants with respiratory disorders]. 61
2771389 1989
50
Immature alveolar/blood barrier and low disaturated phosphatidylcholine in fetal lung after intrauterine exposure to O,O,S-trimethylphosphorothioate. 61
2764722 1989

Variations for Pulmonary Immaturity

Expression for Pulmonary Immaturity

Search GEO for disease gene expression data for Pulmonary Immaturity.

Pathways for Pulmonary Immaturity

Pathways related to Pulmonary Immaturity according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1 11.73 SFTPD SFTPA2 SFTPA1
2
Show member pathways
11.52 SFTPD SFTPC SFTPB SFTPA2 SFTPA1
3 11.3 SFTPC SFTPA2 SFTPA1
4
Show member pathways
11.1 SFTPD SFTPA2 SFTPA1
5 11.05 MAPK6 MAPK4
6 10.82 MAPK6 MAPK4
7
Show member pathways
10.75 SFTPD SFTPC SFTPB SFTPA2 SFTPA1
8
Show member pathways
10.73 SFTPD SFTPC SFTPB SFTPA2 SFTPA1 ABCA3

GO Terms for Pulmonary Immaturity

Cellular components related to Pulmonary Immaturity according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 extracellular space GO:0005615 9.93 SFTPD SFTPC SFTPB SFTPA2 SFTPA1 ABCA3
2 endoplasmic reticulum membrane GO:0005789 9.83 SFTPD SFTPC SFTPB SFTPA2 SFTPA1
3 collagen trimer GO:0005581 9.58 SFTPD SFTPA2 SFTPA1
4 multivesicular body GO:0005771 9.56 SFTPD SFTPB SFTPA2 SFTPA1
5 rough endoplasmic reticulum GO:0005791 9.5 SFTPD SFTPA2 SFTPA1
6 multivesicular body lumen GO:0097486 9.37 SFTPC SFTPB
7 alveolar lamellar body GO:0097208 9.33 SFTPC SFTPB ABCA3
8 lamellar body GO:0042599 9.26 SFTPC SFTPB SFTPA2 SFTPA1
9 clathrin-coated endocytic vesicle GO:0045334 9.02 SFTPD SFTPC SFTPB SFTPA2 SFTPA1

Biological processes related to Pulmonary Immaturity according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 cellular protein metabolic process GO:0044267 9.43 SFTPD SFTPC SFTPB SFTPA2 SFTPA1 ABCA3
2 toll-like receptor signaling pathway GO:0002224 9.33 SFTPD SFTPA2 SFTPA1
3 negative regulation of T cell proliferation GO:0042130 9.32 SFTPD FOXJ1
4 surfactant homeostasis GO:0043129 9.26 SFTPD ABCA3
5 respiratory gaseous exchange GO:0007585 9.1 SFTPD SFTPC SFTPB SFTPA2 SFTPA1 NDST1

Molecular functions related to Pulmonary Immaturity according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 carbohydrate binding GO:0030246 9.33 SFTPD SFTPA2 SFTPA1
2 lipid transporter activity GO:0005319 8.96 SFTPA1 ABCA3
3 MAP kinase activity GO:0004707 8.62 MAPK6 MAPK4

Sources for Pulmonary Immaturity

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 20-May-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
Content
Loading form....