CCAM
MCID: CYS021
MIFTS: 33

Cystic Adenomatoid Malformation of Lung (CCAM)

Categories: Fetal diseases, Rare diseases, Respiratory diseases

Aliases & Classifications for Cystic Adenomatoid Malformation of Lung

MalaCards integrated aliases for Cystic Adenomatoid Malformation of Lung:

Name: Cystic Adenomatoid Malformation of Lung 52
Congenital Pulmonary Airway Malformation 52 58
Congenital Cystic Adenomatoid Malformation of the Lung 58
Congenital Cystic Adenomatous Malformation of the Lung 58
Congenital Cystic Adenomatoid Malformation 52
Congenital Cystic Disease of the Lung 58
Ccam 58
Cpam 58

Characteristics:

Orphanet epidemiological data:

58
congenital pulmonary airway malformation
Prevalence: 1-9/100000 (Europe),1-9/100000 (Belgium),1-5/10000 (France),1-9/100000 (Germany),1-9/100000 (Hungary),1-9/100000 (Ireland),1-9/100000 (Italy),1-5/10000 (Netherlands),1-9/100000 (Norway),1-9/1000000 (Poland),1-9/100000 (Spain),1-5/10000 (Switzerland),1-5/10000 (United Kingdom),1-9/100000 (Ukraine); Age of onset: Antenatal,Neonatal;

Classifications:

Orphanet: 58  
Rare respiratory diseases
Developmental anomalies during embryogenesis


External Ids:

ICD10 32 Q33.0
ICD10 via Orphanet 33 Q33.0
UMLS via Orphanet 72 C0010668 C0158641
Orphanet 58 ORPHA2444

Summaries for Cystic Adenomatoid Malformation of Lung

MalaCards based summary : Cystic Adenomatoid Malformation of Lung, also known as congenital pulmonary airway malformation, is related to congenital pulmonary airway malformation type 3 and congenital pulmonary airway malformation type 2. The drugs Fentanyl and Bupivacaine have been mentioned in the context of this disorder. Affiliated tissues include lung, liver and fetal lung, and related phenotypes are respiratory insufficiency and polyhydramnios

Wikipedia : 74 Congenital pulmonary airway malformation (CPAM), formerly known as congenital cystic adenomatoid... more...

Related Diseases for Cystic Adenomatoid Malformation of Lung

Diseases related to Cystic Adenomatoid Malformation of Lung via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 142)
# Related Disease Score Top Affiliating Genes
1 congenital pulmonary airway malformation type 3 11.9
2 congenital pulmonary airway malformation type 2 11.5
3 congenital pulmonary airway malformation type 4 11.5
4 congenital pulmonary airway malformation type 0 11.5
5 congenital pulmonary airway malformation type 1 11.5
6 pulmonary sequestration 10.8
7 pneumothorax 10.7
8 hydrops fetalis, nonimmune 10.7
9 overgrowth syndrome 10.6
10 respiratory failure 10.6
11 embryoma 10.6
12 pleuropulmonary blastoma 10.6
13 lung disease 10.5
14 emphysema, congenital lobar 10.5
15 diaphragmatic hernia, congenital 10.5
16 rhabdomyosarcoma 10.5
17 bronchogenic cyst 10.5
18 prune belly syndrome 10.4
19 pneumothorax, primary spontaneous 10.4
20 lung abscess 10.4
21 mucinous adenocarcinoma 10.4
22 appendix adenocarcinoma 10.4
23 chromosomal triplication 10.4
24 pectus excavatum 10.3
25 pulmonary hypertension 10.3
26 patau syndrome 10.3
27 cerebrofacial arteriovenous metameric syndrome 10.3
28 virus-associated trichodysplasia spinulosa 10.3
29 esophageal duplication cyst 10.3
30 tracheoesophageal fistula with or without esophageal atresia 10.2
31 renal hypodysplasia/aplasia 1 10.2
32 congenital heart defects, hamartomas of tongue, and polysyndactyly 10.2
33 cystic disease of lung 10.2
34 patent ductus arteriosus 1 10.2
35 cyanosis, transient neonatal 10.2
36 esophageal atresia 10.2
37 arteriovenous malformation 10.2
38 potter's syndrome 10.2
39 persistent fetal circulation syndrome 10.2
40 heart septal defect 10.2
41 embryonal rhabdomyosarcoma 10.2
42 pulmonary blastoma 10.2
43 pneumonia 10.2
44 bronchiectasis 10.2
45 encephalocele 10.2
46 immune hydrops fetalis 10.2
47 hydrocephalus 10.1
48 myopathy 10.1
49 tracheopathia osteoplastica 10.1
50 aspergillosis 10.1

Graphical network of the top 20 diseases related to Cystic Adenomatoid Malformation of Lung:



Diseases related to Cystic Adenomatoid Malformation of Lung

Symptoms & Phenotypes for Cystic Adenomatoid Malformation of Lung

Human phenotypes related to Cystic Adenomatoid Malformation of Lung:

58 31 (show all 6)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 respiratory insufficiency 58 31 occasional (7.5%) Occasional (29-5%) HP:0002093
2 polyhydramnios 58 31 occasional (7.5%) Occasional (29-5%) HP:0001561
3 premature birth 58 31 occasional (7.5%) Occasional (29-5%) HP:0001622
4 abnormal pleura morphology 31 occasional (7.5%) HP:0002103
5 abnormality of the pleura 58 Occasional (29-5%)
6 abnormality of the respiratory system 58 Very frequent (99-80%)

Drugs & Therapeutics for Cystic Adenomatoid Malformation of Lung

Drugs for Cystic Adenomatoid Malformation of Lung (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 71)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Fentanyl Approved, Illicit, Investigational, Vet_approved Phase 3 437-38-7 3345
2
Bupivacaine Approved, Investigational Phase 3 2180-92-9, 38396-39-3 2474
3 Narcotics Phase 3
4 Anesthetics Phase 3
5 Anesthetics, General Phase 3
6 Anesthetics, Intravenous Phase 3
7
Vinorelbine Approved, Investigational Phase 2 71486-22-1 60780 44424639
8
Trastuzumab Approved, Investigational Phase 2 180288-69-1 9903
9
Epirubicin Approved Phase 2 56420-45-2 41867
10
Capecitabine Approved, Investigational Phase 2 154361-50-9 60953
11
Bevacizumab Approved, Investigational Phase 2 216974-75-3
12
Docetaxel Approved, Investigational Phase 2 114977-28-5 148124
13
Peginterferon alfa-2a Approved, Investigational Phase 1, Phase 2 198153-51-4 5360545
14
Entecavir Approved, Investigational Phase 1, Phase 2 142217-69-4 153941
15
Vincristine Approved, Investigational Phase 1, Phase 2 57-22-7, 2068-78-2 5978
16
Doxorubicin Approved, Investigational Phase 1, Phase 2 23214-92-8 31703
17
Prednisone Approved, Vet_approved Phase 1, Phase 2 53-03-2 5865
18
Lenograstim Approved, Investigational Phase 1, Phase 2 135968-09-1
19
Sargramostim Approved, Investigational Phase 1, Phase 2 83869-56-1, 123774-72-1
20
rituximab Approved Phase 1, Phase 2 174722-31-7 10201696
21
Cyclophosphamide Approved, Investigational Phase 1, Phase 2 50-18-0, 6055-19-2 2907
22
Betamethasone Approved, Vet_approved Phase 1, Phase 2 378-44-9 9782
23
Tenofovir Experimental, Investigational Phase 1, Phase 2 147127-20-6 464205
24 Molgramostim Investigational Phase 1, Phase 2 99283-10-0
25 Antineoplastic Agents, Immunological Phase 2
26 Angiogenesis Inhibitors Phase 2
27 Antimetabolites Phase 2
28 Anti-Infective Agents Phase 1, Phase 2
29 Reverse Transcriptase Inhibitors Phase 1, Phase 2
30 Anti-HIV Agents Phase 1, Phase 2
31 Antiviral Agents Phase 1, Phase 2
32 Anti-Retroviral Agents Phase 1, Phase 2
33 Liver Extracts Phase 1, Phase 2
34 Antineoplastic Agents, Hormonal Phase 1, Phase 2
35 Immunosuppressive Agents Phase 1, Phase 2
36 Alkylating Agents Phase 1, Phase 2
37 Antiemetics Phase 1, Phase 2
38
Liposomal doxorubicin Phase 1, Phase 2 31703
39 Tubulin Modulators Phase 1, Phase 2
40 Anti-Bacterial Agents Phase 1, Phase 2
41 Immunologic Factors Phase 1, Phase 2
42 Antibiotics, Antitubercular Phase 1, Phase 2
43 Antirheumatic Agents Phase 1, Phase 2
44 Antimitotic Agents Phase 1, Phase 2
45 Respiratory System Agents Phase 1, Phase 2
46 Hormones Phase 1, Phase 2
47 Anti-Asthmatic Agents Phase 1, Phase 2
48 glucocorticoids Phase 1, Phase 2
49 Hormone Antagonists Phase 1, Phase 2
50 Anti-Inflammatory Agents Phase 1, Phase 2

Interventional clinical trials:

(show all 20)
# Name Status NCT ID Phase Drugs
1 Evaluation of a Synthetic Bone Substitute Associated With Absorbable Collagen Membrane for Ridge Preservation After Tooth Extraction. Clinical and Tomographic Randomized Study in Humans. Completed NCT02894528 Phase 4 BioMend and ReproBone;BioMend without ReproBone
2 Evaluation of Effects of Additional Fentanyl to Epidural Bupivacaine for Post-Thoracotomy Pain in Neonates on Perioperative Outcome Completed NCT00286143 Phase 3 Fentanyl
3 "Phase II Study of PET Guided Neoadjuvant Chemotherapy (NAC) and Oncotype Guided Hormonal Therapy of Breast Cancer" Unknown status NCT01641406 Phase 2 Docetaxel, Epirubicin, Cyclophosphamide/Navelbine, Capecitabine, Trastuzumab, Bevacizumab;Docetaxel, Epirubicin, Cyclophosphamide/Navelbine, Capecitabine, Trastuzumab, Bevacizumab;Docetaxel, Epirubicin, Cyclophosphamide/Navelbine, Capecitabine, Trastuzumab, Bevacizumab
4 A Phase 1b/2a, Dose-Ranging Study of the Safety, Tolerability, Pharmacokinetics, and Initial Efficacy of ABI-H0731 in Patients With Chronic Hepatitis B Completed NCT03109730 Phase 1, Phase 2 ABI-H0731;Placebo for ABI-H0731;Entecavir;Tenofovir Disoproxil Fumarate;Pegasys
5 Protocol CCAM 05-02 " Phase I-II Study of Dose Dense of PEG Filgrastim and GM-CSF as Support for Dose Desnse CHOP-R Front Line Therapy for Aggressive Non Hodgkin's Lymphoma" Completed NCT01527422 Phase 1, Phase 2 Cyclophosphamide, Doxorubicin, Vincristine and Prednisone
6 A Phase II, Randomised, Adaptive, Open-Label Platform Trial To Evaluate Efficacy And Safety Of Multiple Combination Therapies In Participants With Chronic Hepatitis B Recruiting NCT04225715 Phase 2 Nucleos(t)ide (NUC);CpAM (RO7049389);TLR7 (RO7020531)
7 Investigation of Prenatal Steroids for Treatment of Prenatally Diagnosed CCAMs Terminated NCT00670956 Phase 1, Phase 2 Betamethasone;Placebo
8 A Phase 1, Open-Label, Multicenter, Multi-Dose Escalation Study of CM-24 (MK-6018) as Monotherapy and In Combination With Pembrolizumab (MK-3475) in Subjects With Selected Advanced or Recurrent Malignancies Terminated NCT02346955 Phase 1
9 Genetic and Molecular Abnormalities in Congenital Cystic Adenomatoid Malformations Unknown status NCT01732185
10 Review of the Paediatric Pleuropulmonary Blastoma French Series Unknown status NCT03044834
11 Nutrition Environnement et Santé Cardio Vasculaire NESCAV Unknown status NCT01414179
12 LIVER FIBROSIS PREVALENCE IN FRANCE Unknown status NCT01927133
13 Caffeine Citrate for the Treatment of Apnea Associated With Bronchiolitis in Young Infants: A Randomized, Double Blind, Controlled Trial (RCT) Completed NCT01435486 Caffeine citrate;Normal saline
14 Medical Economic Evaluation of the Care of the Severe Asthma : Cohort COBRA Completed NCT01669681
15 Current Incidence and Treatments Performed of Fractures of the Acetabulum and Pelvis in France in 2013 Completed NCT02848924
16 Rehabiliation-Aftercare for an Optimal Transfer Into Autonomous Daily Life (RENATA) - an eHealth Intervention Study Completed NCT01909349
17 Follow up of Congenital Lung Anomalies (CLA) With Antenatal Diagnosis - a Swiss Multicentric Database Recruiting NCT03044769
18 Project CHROMA (Cognitive Health Research on Musical Arts) Recruiting NCT04137913
19 CSP #2009 - Sequential and Comparative Evaluation of Pain Treatment Effectiveness Response: The SCEPTER Trial Not yet recruiting NCT04142177
20 Prediction of Recruitment Potential of Participating Centers in Clinical Trials by Standardized Translation of Selection Criteria and Queries From the French DRG Database (PMSI) Not yet recruiting NCT03019068

Search NIH Clinical Center for Cystic Adenomatoid Malformation of Lung

Genetic Tests for Cystic Adenomatoid Malformation of Lung

Anatomical Context for Cystic Adenomatoid Malformation of Lung

MalaCards organs/tissues related to Cystic Adenomatoid Malformation of Lung:

40
Lung, Liver, Fetal Lung, Bone, Breast, Testes

Publications for Cystic Adenomatoid Malformation of Lung

Articles related to Cystic Adenomatoid Malformation of Lung:

(show top 50) (show all 224)
# Title Authors PMID Year
1
Intrathoracic shunt displacement with massive pleural effusion after successful shunt placement in a hydropic fetus with multilocular macrocystic congenital pulmonary airway malformation. 61
32562426 2020
2
Atypical goblet cell hyperplasia occurs in CPAM 1, 2, and 3, and is a probable precursor lesion for childhood adenocarcinoma. 61
31858221 2020
3
Computed tomography features can distinguish type 4 congenital pulmonary airway malformation from other cystic congenital pulmonary airway malformations. 61
32224324 2020
4
Three-dimensional Ultrashort Echotime Magnetic Resonance Imaging for Combined Morphologic and Ventilation Imaging in Pediatric Patients With Pulmonary Disease. 61
32453280 2020
5
Applications of Pediatric Body CT Angiography: What Radiologists Need to Know. 61
32130044 2020
6
Sex differences in surgically correctable congenital anomalies: A systematic review. 61
32061363 2020
7
Comparison of the Prognostic Factors of Fetuses With Congenital Pulmonary Airway Malformations According to Type. 61
32420654 2020
8
Further delineation of the phenotypic spectrum of nevus comedonicus syndrome to include congenital pulmonary airway malformation of the lung and aneurysm. 61
31961058 2020
9
Pleuropulmonary blastoma type I and congenital pulmonary airway malformation type 4: distinct entities or sides of the same coin? 61
32300881 2020
10
Thoracoscopic Lobectomy for Congenital Pulmonary Airway Malformation: Where Are We in 2019? 61
32146716 2020
11
Pleuropulmonary blastoma type I might arise in congenital pulmonary airway malformation type 4 by acquiring a Dicer 1 mutation. 61
32193603 2020
12
Analysis of Wnt7B and BMP4 expression patterns in congenital pulmonary airway malformation. 61
31962011 2020
13
A clinical guideline for structured assessment of CT-imaging in congenital lung abnormalities. 61
32178987 2020
14
Congenital pulmonary airway malformation associated with mosaic Klinefelter syndrome. 61
31566736 2020
15
A case report of an unusual non-mucinous papillary variant of CPAM type 1 with KRAS mutations. 61
32093717 2020
16
Incidence of congenital thoracic malformations detected by prenatal ultrasound. 61
31705721 2020
17
Value of routine ultrasound examination at 35-37 weeks' gestation in diagnosis of fetal abnormalities. 61
31595569 2020
18
The Utility of the Congenital Pulmonary Airway Malformation-Volume Ratio in the Assessment of Fetal Echogenic Lung Lesions: A Systematic Review. 61
31593968 2020
19
Does fissure status affect the outcome of thoracoscopic pulmonary lobectomy? 61
31555863 2020
20
In fetuses with congenital lung masses, decreased ventricular and atrioventricular valve dimensions are associated with lesion size and clinical outcome. 61
31742724 2020
21
Congenital Pulmonary Airway Malformation - 19-Year Experience from a Tertiary Care Center in India. 61
31084392 2019
22
Congenital perianal lipoma: a case report and review of the literature. 61
31845000 2019
23
Introduction of thoracoscopic surgery for congenital pulmonary airway malformation in infants: review of 13 consecutive surgical cases. 61
32030224 2019
24
Establishment of a biobank for human lung tissues of congenital diaphragmatic hernia and congenital pulmonary airway malformation. 61
31130348 2019
25
Early prenatal ultrasound diagnosis of congenital thoracic malformations. 61
29720002 2019
26
Outcomes of Cases of Prenatally-Diagnosed Congenital Pulmonary Airway Malformation. 61
31745958 2019
27
Congenital pulmonary airway malformation: A case report of a rare cause of neonatal respiratory distress and review of the literature. 61
31719287 2019
28
New insights on congenital pulmonary airways malformations revealed by proteomic analyses. 61
31779656 2019
29
Obstetric and neonatal outcomes in pregnancies complicated by fetal lung masses: does final histology matter? 61
31722592 2019
30
Type I pleuropulmonary blastoma presenting as congenital pulmonary airway malformation: A report of two cases. 61
31611448 2019
31
Diagnosis of fetal non-chromosomal abnormalities on routine ultrasound examination at 11-13 weeks' gestation. 61
31408229 2019
32
The Pediatric Surgical Team: a Model for Increased Surgeon Index Case Exposure. 61
30765153 2019
33
Concurrence of Two Rare Conditions: Simultaneous Bilateral Spontaneous Pneumothorax and Congenital Pulmonary Airway Malformation. 61
31404456 2019
34
Neonate with persisting respiratory distress after resolution of pneumothorax. 61
31506322 2019
35
Adenocarcinoma in situ in type I congenital pulmonary airway malformation. 61
31226882 2019
36
Pneumomediastinum Mimicking Congenital Pulmonary Airway Malformation. 61
31533336 2019
37
Airway management of congenital pulmonary airway malformation resection in neonates and infants: A case cohort study. 61
31013391 2019
38
An adult patient with congenital pulmonary airway malformation and an esophageal cyst. 61
31555710 2019
39
Manifestation of congenital pulmonary airway malformation in a 26-year-old adult, requiring surgery. 61
30844060 2019
40
Esophageal duplication cyst presenting with stridor in a child with congenital pulmonary airway malformation: A case report and literature review. 61
31305433 2019
41
[Retrospective Analysis on the Safety and Feasibility of Thoracoscopic Anatomical Segmentectomy in the Treatment of Congenital Lung Malformations]. 61
31642241 2019
42
Lung Ultrasound: A New Tool in the Management of Congenital Lung Malformation. 61
31238368 2019
43
Invasive Mucinous Adenocarcinoma in Congenital Pulmonary Airway Malformation: A Case Report. 61
30595422 2019
44
Invasive mucinous adenocarcinoma of the lung arising in a type 1 congenital pulmonary airway malformation in a 68-year-old patient: a case report. 61
31262240 2019
45
Diagnostic accuracy of imaging studies in congenital lung malformations. 61
30049725 2019
46
Current Management of Pleuropulmonary Blastoma: A Surgical Perspective. 61
31349569 2019
47
Congenital pulmonary airway malformation mimicking lung cancer: A case report. 61
31192965 2019
48
Congenital Lobar Overinflation: A Rare Enigmatic Lung Lesion on Prenatal Ultrasound and Magnetic Resonance Imaging. 61
30208226 2019
49
In prenatally diagnosed CPAM, does the affected lobe influence the timing of symptom onset? 61
30778700 2019
50
Evolution of fetal lung stiffness during gestation in two different congenital malformations. 61
30854743 2019

Variations for Cystic Adenomatoid Malformation of Lung

Expression for Cystic Adenomatoid Malformation of Lung

Search GEO for disease gene expression data for Cystic Adenomatoid Malformation of Lung.

Pathways for Cystic Adenomatoid Malformation of Lung

GO Terms for Cystic Adenomatoid Malformation of Lung

Sources for Cystic Adenomatoid Malformation of Lung

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
Content
Loading form....