CPFE
MCID: CMB039
MIFTS: 20

Combined Pulmonary Fibrosis-Emphysema Syndrome (CPFE)

Categories: Rare diseases, Respiratory diseases

Aliases & Classifications for Combined Pulmonary Fibrosis-Emphysema Syndrome

MalaCards integrated aliases for Combined Pulmonary Fibrosis-Emphysema Syndrome:

Name: Combined Pulmonary Fibrosis-Emphysema Syndrome 58
Cpfe 58

Characteristics:

Orphanet epidemiological data:

58
combined pulmonary fibrosis-emphysema syndrome
Inheritance: Not applicable; Age of onset: Adult; Age of death: adult;

Classifications:

Orphanet: 58  
Rare respiratory diseases


External Ids:

ICD10 via Orphanet 33 J84.1
Orphanet 58 ORPHA300564

Summaries for Combined Pulmonary Fibrosis-Emphysema Syndrome

MalaCards based summary : Combined Pulmonary Fibrosis-Emphysema Syndrome, also known as cpfe, is related to fibrosis of extraocular muscles, congenital, 1 and pulmonary hypertension, primary, 3. The drugs Treprostinil and Pharmaceutical Solutions have been mentioned in the context of this disorder. Affiliated tissues include lung, testes and cortex.

Wikipedia : 74 Combined pulmonary fibrosis and emphysema (CPFE), describes a medical syndrome involving both pulmonary... more...

Related Diseases for Combined Pulmonary Fibrosis-Emphysema Syndrome

Diseases related to Combined Pulmonary Fibrosis-Emphysema Syndrome via text searches within MalaCards or GeneCards Suite gene sharing:

(show all 13)
# Related Disease Score Top Affiliating Genes
1 fibrosis of extraocular muscles, congenital, 1 10.4
2 pulmonary hypertension, primary, 3 10.4
3 pulmonary hypertension 10.3
4 hair whorl 10.3
5 pulmonary fibrosis, idiopathic 10.3
6 idiopathic interstitial pneumonia 10.3
7 pulmonary emphysema 10.3
8 pulmonary disease, chronic obstructive 10.1
9 autoimmune pancreatitis 10.1
10 igg4-related disease 10.1
11 interstitial emphysema 10.1
12 47,xyy 10.1
13 pulmonary fibrosis 10.1

Graphical network of the top 20 diseases related to Combined Pulmonary Fibrosis-Emphysema Syndrome:



Diseases related to Combined Pulmonary Fibrosis-Emphysema Syndrome

Symptoms & Phenotypes for Combined Pulmonary Fibrosis-Emphysema Syndrome

Drugs & Therapeutics for Combined Pulmonary Fibrosis-Emphysema Syndrome

Drugs for Combined Pulmonary Fibrosis-Emphysema Syndrome (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):


# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Treprostinil Approved, Investigational Phase 2, Phase 3 81846-19-7 6918140 54786
2 Pharmaceutical Solutions Phase 2, Phase 3
3 Antihypertensive Agents Phase 2, Phase 3

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 A Multicenter, Randomized, Double-Blinded, Placebo-Controlled Trial to Evaluate the Safety and Efficacy of Inhaled Treprostinil in Subjects With Pulmonary Hypertension Due to Parenchymal Lung Disease Completed NCT02630316 Phase 2, Phase 3 Inhaled Treprostinil;Placebo
2 An Open-Label Extension Study of Inhaled Treprostinil in Subjects With Pulmonary Hypertension Due to Parenchymal Lung Disease Active, not recruiting NCT02633293 Phase 2, Phase 3 Inhaled Treprostinil
3 Genetics of the Combined Pulmonary Fibrosis and Emphysema Syndrome Unknown status NCT02439528
4 Study on Phenotypic Characterization of Combined Pulmonary Fibrosis and Emphysema Completed NCT01121367

Search NIH Clinical Center for Combined Pulmonary Fibrosis-Emphysema Syndrome

Genetic Tests for Combined Pulmonary Fibrosis-Emphysema Syndrome

Anatomical Context for Combined Pulmonary Fibrosis-Emphysema Syndrome

MalaCards organs/tissues related to Combined Pulmonary Fibrosis-Emphysema Syndrome:

40
Lung, Testes, Cortex, Prefrontal Cortex, Brain, Cerebellum, Amygdala

Publications for Combined Pulmonary Fibrosis-Emphysema Syndrome

Articles related to Combined Pulmonary Fibrosis-Emphysema Syndrome:

(show top 50) (show all 322)
# Title Authors PMID Year
1
Protective role of overexpressed MUC5AC against fibrosis in MHV-68-induced combined pulmonary fibrosis and emphysema mouse model. 61
32557739 2020
2
Prognosis of patients with acute exacerbation of combined pulmonary fibrosis and emphysema: a retrospective single-centre study. 61
32434532 2020
3
Infant rats can acquire, but not retain contextual associations in object-in-context and contextual fear conditioning paradigms. 61
32441808 2020
4
Secondary Pulmonary Hypertension Among Patients Qualified for Lung Transplantation: Single-Center Study. 61
32336653 2020
5
Combined Pulmonary Fibrosis and Emphysema. 61
32279289 2020
6
Differential expression of caveolin-1 during pathogenesis of combined pulmonary fibrosis and emphysema: Effect of phosphodiesterase-5 inhibitor. 61
32311453 2020
7
NMDA receptors and the ontogeny of post-shock and retention freezing during contextual fear conditioning. 61
31621064 2020
8
Photoactivated silicon-oxygen and silicon-nitrogen heterodehydrocoupling with a commercially available iron compound. 61
32073589 2020
9
Long-term maintenance of nutritional status with ninjinyoueito in terminal patients with chronic respiratory disease: Two case reports. 61
32042420 2020
10
Lobe-specific outcomes of surgery for lung cancer patients with idiopathic interstitial pneumonias. 61
32040817 2020
11
Synthesis and Post-Polymerization Functionalization of Halogen-Substituted Polyphosphinoboranes to Access Alkyne-Functionalized Derivatives. 61
31880037 2020
12
The Right Anterior Thoracotomy Approach to Resect a Cardiac Papillary Fibroelastoma of the Aortic Valve. 61
32257681 2020
13
Characteristics of tobacco-related lung diseases in Fukuoka Prefecture, Japan: A prospective, multi-institutional, observational study. 61
31727581 2020
14
Uncovering the mechanisms of exertional dyspnoea in combined pulmonary fibrosis and emphysema. 61
31649067 2020
15
The effect of emphysema on survival in patients with idiopathic pulmonary fibrosis: A retrospective study in Taiwan. 61
31800533 2019
16
Medial prefrontal and ventral hippocampal contributions to incidental context learning and memory in adolescent rats. 61
31542328 2019
17
Hardening and Strain Localisation in Helium-Ion-Implanted Tungsten. 61
31797894 2019
18
Combined pulmonary fibrosis and emphysema and idiopathic pulmonary fibrosis in non-small cell lung cancer: impact on survival and acute exacerbation. 61
31615505 2019
19
Combined Pulmonary Fibrosis and Emphysema: Pulmonary Function Testing and a Pathophysiology Perspective. 61
31509942 2019
20
Combined pulmonary fibrosis and emphysema: How does cohabitation affect respiratory functions? 61
30947142 2019
21
Coordination Chemistry of P4 S3 and P4 Se3 towards the Iron Fragments [Fe(Cp)(CO)2 ]+ and [Fe(Cp)(PPh3 )(CO)]. 61
31287589 2019
22
Citrus peel flavonoids improve lipid metabolism by inhibiting miR-33 and miR-122 expression in HepG2 cells. 61
31017523 2019
23
Cholinergic rescue of neurocognitive insult following third-trimester equivalent alcohol exposure in rats. 61
31185278 2019
24
From Phosphidic to Phosphonium? Umpolung of the P4 -Bonding Situation in [CpFe(CO)(L)(η1 -P4 )]+ Cations (L=CO or PPh3 ). 61
31066932 2019
25
Combined pulmonary fibrosis and emphysema in systemic sclerosis: A syndrome associated with heavy morbidity and mortality. 61
30409416 2019
26
Cardiac papillary fibroelastoma of a bicuspid aortic valve in an adolescent: A case report. 61
31579491 2019
27
Distribution of emphysema and fibrosis in idiopathic pulmonary fibrosis with coexisting emphysema. 61
30715748 2019
28
Adenosine and hyaluronan promote lung fibrosis and pulmonary hypertension in combined pulmonary fibrosis and emphysema. 61
31036697 2019
29
Inflammatory signature in lung tissues in patients with combined pulmonary fibrosis and emphysema. 61
30411980 2019
30
The Bonding Situation in the Dinuclear Tetra-Hydrido Complex [{5CpFe}2(μ-H)4] Revisited by Hard X-Ray Spectroscopy. 61
30596494 2019
31
Reduced area of the normal lung on high-resolution computed tomography predicts poor survival in patients with lung cancer and combined pulmonary fibrosis and emphysema. 61
30472091 2019
32
Combined pulmonary fibrosis and emphysema characteristics in a Greek cohort. 61
30895186 2019
33
Neonatal ethanol exposure impairs long-term context memory formation and prefrontal immediate early gene expression in adolescent rats. 61
30447241 2019
34
Synthetic Designs and Structural Investigations of Biomimetic Ni-Fe Thiolates. 61
30707014 2019
35
Imine hydrosilylation using an iron complex catalyst: A computational study. 61
30351480 2019
36
Differential involvement of amygdalar NMDA receptors across variants of contextual fear conditioning in adolescent rats. 61
30142395 2019
37
Prognosis of combined pulmonary fibrosis and emphysema: comparison with idiopathic pulmonary fibrosis alone. 61
31722622 2019
38
Overall mortality in combined pulmonary fibrosis and emphysema related to systemic sclerosis. 61
30886735 2019
39
Effects of emphysema on physiological and prognostic characteristics of lung function in idiopathic pulmonary fibrosis. 61
30136753 2019
40
Combined pulmonary fibrosis and emphysema as a clinicoradiologic entity: Characterization of presenting lung fibrosis and implications for survival. 61
30665507 2019
41
Impact Of Underlying Pulmonary Diseases On Treatment Outcomes In Early-Stage Non-Small Cell Lung Cancer Treated With Definitive Radiotherapy. 61
31631997 2019
42
Improvement of pulmonary arterial compliance by pulmonary vasodilator in pulmonary hypertension from combined pulmonary fibrosis and emphysema. 61
31667076 2019
43
Impairment of the context preexposure facilitation effect in juvenile rats by neonatal alcohol exposure is associated with decreased Egr-1 mRNA expression in the prefrontal cortex. 61
30346189 2018
44
{CpFeII(CO)2SnII(Macrocycle•3-)} Radicals with Intrinsic Charge Transfer from CpFe(CO)2 to Macrocycles (Cp: Cp or Cp*); Effective Magnetic Coupling between Radical Trianionic Macrocycles•3. 61
31458154 2018
45
Cationic Cyclopentadienyliron Complex as a Novel and Successful Nucleating Agent on the Crystallization Behavior of the Biodegradable PHB Polymer. 61
30347768 2018
46
Synthesis of N-Heterocycle Substituted Silyl Ligands within the Coordination Sphere of Iron. 61
30270963 2018
47
A computational study on a multimode spin conductance switching by coordination isomerization in organometallic single-molecule junctions. 61
30039822 2018
48
Presence of lung cancer and high gender, age, and physiology score as predictors of acute exacerbation in combined pulmonary fibrosis and emphysema: A retrospective study. 61
30075563 2018
49
Likelihood of pulmonary hypertension in patients with idiopathic pulmonary fibrosis and emphysema. 61
29237236 2018
50
Trend-Analysis of Solid-State Structures: Low-Energy Conformational 'Reactions' Involving Directed and Coupled Movements in Half-Sandwich Compounds [CpFe(CO){C(=O)R}PPh3]. 61
29744281 2018

Variations for Combined Pulmonary Fibrosis-Emphysema Syndrome

Expression for Combined Pulmonary Fibrosis-Emphysema Syndrome

Search GEO for disease gene expression data for Combined Pulmonary Fibrosis-Emphysema Syndrome.

Pathways for Combined Pulmonary Fibrosis-Emphysema Syndrome

GO Terms for Combined Pulmonary Fibrosis-Emphysema Syndrome

Sources for Combined Pulmonary Fibrosis-Emphysema Syndrome

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
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
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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