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 53
Congenital Pulmonary Airway Malformation 53 59
Congenital Cystic Adenomatoid Malformation of the Lung 59
Congenital Cystic Adenomatous Malformation of the Lung 59
Congenital Cystic Adenomatoid Malformation 53
Congenital Cystic Disease of the Lung 59
Ccam 59
Cpam 59

Characteristics:

Orphanet epidemiological data:

59
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:



External Ids:

Orphanet 59 ORPHA2444
ICD10 via Orphanet 34 Q33.0
UMLS via Orphanet 74 C0158641 C0010668
ICD10 33 Q33.0

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, fetal lung and testes, and related phenotypes are respiratory insufficiency and abnormality of the pleura

Wikipedia : 76 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 55)
# Related Disease Score Top Affiliating Genes
1 congenital pulmonary airway malformation type 3 11.7
2 congenital pulmonary airway malformation type 2 11.4
3 congenital pulmonary airway malformation type 4 11.4
4 congenital pulmonary airway malformation type 0 11.4
5 congenital pulmonary airway malformation type 1 11.4
6 pulmonary sequestration 10.6
7 pleuropulmonary blastoma 10.5
8 lung abscess 10.4
9 emphysema, congenital lobar 10.3
10 patau syndrome 10.3
11 prune belly syndrome 10.2
12 pneumothorax, primary spontaneous 10.2
13 hydrops fetalis, nonimmune, and/or atrial septal defect 10.2
14 pneumothorax 10.2
15 bronchogenic cyst 10.2
16 fetal edema 10.2
17 hydrops fetalis 10.2
18 tracheoesophageal fistula 10.2
19 esophageal duplication cyst 10.2
20 mucinous adenocarcinoma 10.2
21 exudative vitreoretinopathy 1 10.1
22 tracheoesophageal fistula with or without esophageal atresia 10.1
23 renal hypodysplasia/aplasia 1 10.1
24 hydrops fetalis, nonimmune 10.1
25 pulmonary arteriovenous fistulas 10.1
26 esophageal atresia 10.1
27 meningocele 10.1
28 arteriovenous malformation 10.1
29 potter's syndrome 10.1
30 ehlers-danlos syndrome 10.1
31 epilepsy 10.1
32 pulmonary tuberculosis 10.1
33 adenocarcinoma 10.1
34 pulmonary blastoma 10.1
35 lung disease 10.1
36 alveolar capillary dysplasia 10.1
37 chromosomal triplication 10.1
38 pulmonary arteriovenous malformation 10.1
39 pulmonary artery agenesis 10.1
40 immune hydrops fetalis 10.1
41 fetal lung interstitial tumor 10.1
42 pleural empyema 10.1
43 lipomatosis, multiple 9.9
44 bronchomalacia 9.9
45 lung cancer susceptibility 3 9.9
46 aspergillosis 9.9
47 pulmonary hypertension 9.9
48 lymphoma 9.9
49 pleomorphic lipoma 9.9
50 nephrotic syndrome 9.9

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:

59 32
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 respiratory insufficiency 59 32 occasional (7.5%) Occasional (29-5%) HP:0002093
2 abnormality of the pleura 59 32 occasional (7.5%) Occasional (29-5%) HP:0002103
3 polyhydramnios 59 32 occasional (7.5%) Occasional (29-5%) HP:0001561
4 premature birth 59 32 occasional (7.5%) Occasional (29-5%) HP:0001622
5 abnormality of the respiratory system 59 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 all 24)
# 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 Adjuvants, Anesthesia Phase 3
4 Peripheral Nervous System Agents Phase 3
5 Analgesics, Opioid Phase 3
6 Anesthetics Phase 3
7 Narcotics Phase 3
8 Central Nervous System Depressants Phase 3
9 Anesthetics, Intravenous Phase 3
10 Anesthetics, General Phase 3
11 Analgesics Phase 3
12 Anesthetics, Local Phase 3
13
Betamethasone Approved, Vet_approved Phase 1, Phase 2 378-44-9 9782
14 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 1, Phase 2
15 Betamethasone Valerate Phase 1, Phase 2 2152-44-5
16 Respiratory System Agents Phase 1, Phase 2
17 Betamethasone benzoate Phase 1, Phase 2
18 Anti-Inflammatory Agents Phase 1, Phase 2
19 Betamethasone sodium phosphate Phase 1, Phase 2
20 Hormone Antagonists Phase 1, Phase 2
21 Hormones Phase 1, Phase 2
22 Anti-Asthmatic Agents Phase 1, Phase 2
23 glucocorticoids Phase 1, Phase 2
24 Betamethasone-17,21-dipropionate Phase 1, Phase 2

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Effects of Additional Fentanyl to Epidural Bupivacaine for Post-Thoracotomy Pain in Neonates Completed NCT00286143 Phase 3 Fentanyl
2 Prenatal Steroids for Treatment of Congenital Cystic Adenomatoid Malformations (CCAM) Terminated NCT00670956 Phase 1, Phase 2 Betamethasone;Placebo
3 Genetic and Molecular Abnormalities in Congenital Cystic Adenomatoid Malformations Unknown status NCT01732185 Not Applicable
4 Congenital Lung Anomalies (CLA) Swiss Database Recruiting NCT03044769
5 Review of the Paediatric Pleuropulmonary Blastoma French Series Recruiting NCT03044834

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:

41
Lung, Fetal Lung, Testes, Heart

Publications for Cystic Adenomatoid Malformation of Lung

Articles related to Cystic Adenomatoid Malformation of Lung:

(show top 50) (show all 99)
# Title Authors Year
1
Case of mucinous adenocarcinoma of the lung associated with congenital pulmonary airway malformation in a neonate. ( 29441110 )
2018
2
Dichorionic-diamniotic twins: One case each of esophageal atresia and congenital pulmonary airway malformation. ( 30187627 )
2018
3
Pleuropulmonary Blastoma Developing in a Case of Misinterpreted Congenital Pulmonary Airway Malformation: a Case Report. ( 30358469 )
2018
4
Congenital pulmonary airway malformation mimicking as pulmonary tuberculosis in five paediatric patients: a diagnostic dilemma. ( 29621946 )
2018
5
Accessory scrotum and congenital perineal lipoma in a child with type 2 congenital pulmonary airway malformation: A report of an unusual. ( 29623024 )
2018
6
Medium Term Pulmonary Function Test After Thoracoscopic Lobectomy for Congenital Pulmonary Airway Malformation: A Comparative Study with Normal Control. ( 29099644 )
2018
7
Three-dimensional computed tomography of congenital pulmonary airway malformation. ( 29356290 )
2018
8
Types II and III congenital pulmonary airway malformation with hydrops treated in utero with percutaneous sclerotherapy. ( 29665020 )
2018
9
Lung Ultrasound Findings in Congenital Pulmonary Airway Malformation. ( 29715700 )
2018
10
Congenital pulmonary airway malformation. ( 29780758 )
2018
11
Congenital pulmonary airway malformation: advances and controversies. ( 30169300 )
2018
12
Fetal Congenital Pulmonary Airway Malformation: The Role of an Objective Measurement of Cardiomediastinal Shift. ( 30199894 )
2018
13
An investigation on clinical differences between congenital pulmonary airway malformation and bronchial atresia. ( 30227995 )
2018
14
Predictors of early lobectomy after birth in prenatally diagnosed congenital pulmonary airway malformation. ( 30244937 )
2018
15
Long-term pulmonary function after lobectomy for congenital pulmonary airway malformation: is thoracoscopic approach really better than open? ( 30249357 )
2018
16
Congenital pulmonary airway malformation in the asymptomatic adult: A rare presentation. ( 30364709 )
2018
17
Mucinous adenocarcinoma with lepidic pattern and with K-RAS mutation in a newborn with antenatal diagnosis of congenital pulmonary airway malformation. ( 28889407 )
2017
18
It's Rare So Be Aware: Pleuropulmonary Blastoma Mimicking Congenital Pulmonary Airway Malformation. ( 28405541 )
2017
19
Is congenital pulmonary airway malformation really a rare disease? Result of a prospective registry with universal antenatal screening program. ( 27770196 )
2017
20
Optimal age for elective surgery of asymptomatic congenital pulmonary airway malformation: a meta-analysis. ( 28293700 )
2017
21
Congenital Bi-segmental Bronchial Atresia with Areas of Congenital Pulmonary Airway Malformation. ( 28408057 )
2017
22
An infected cyst due to a congenital pulmonary airway malformation. ( 28491811 )
2017
23
An Atypical Presentation of Congenital Pulmonary Airway Malformation (CPAM): A Rare Case with Antenatal Ultrasound Findings and Review of Literature. ( 28638495 )
2017
24
Images in Anesthesiology: Congenital Pulmonary Airway Malformation. ( 28719530 )
2017
25
Transthoracic catheter drainage for large symptomatic congenital pulmonary airway malformation. ( 29028157 )
2017
26
Perforated neuroenteric cyst masquerading as congenital pulmonary airway malformation. ( 29197847 )
2017
27
A Complication of Percutaneous Sclerotherapy for Congenital Pulmonary Airway Malformation: Intravascular Injection and Cardiac Necrosis. ( 29206544 )
2017
28
Reply to Letter to the Editor: Surgical versus conservative management of congenital pulmonary airway malformation in children: A systematic review and meta-analysis" by Kapralik et al J Pediatr Surg 51 (2016) 508-512. ( 27577184 )
2016
29
Surgical intervention for congenital pulmonary airway malformation (CPAM) patients with preoperative pneumonia and abscess formation: "open versus thoracoscopic lobectomy". ( 26661941 )
2016
30
Direct Tracheobronchopexy and Posterior Descending Aortopexy for Severe Left Mainstem Bronchomalacia Associated With Congenital Pulmonary Airway Malformation and Left Circumflex Aortic Arch. ( 27343517 )
2016
31
Congenital pulmonary airway malformation (CPAM) with initial presentation in an adult: a rare presentation of a rare disease. ( 27671988 )
2016
32
Congenital pulmonary airway malformation in a 36 year-old female. ( 27141433 )
2016
33
Surgical versus conservative management of congenital pulmonary airway malformation in children: A systematic review and meta-analysis. ( 26775193 )
2016
34
Congenital pulmonary airway malformation with atrial septal defect and pulmonary hypertension for lobectomy-anesthetic considerations. ( 27052089 )
2016
35
Refractory tension pneumothorax as a result of an internally displaced thoracoamniotic shunt in an infant with a congenital pulmonary airway malformation. ( 27469386 )
2016
36
Congenital pulmonary airway malformation - A study of 2 cases. ( 27772928 )
2016
37
Reply to the Letter to the Editor 'Type I Pleuropulmonary Blastoma versus Congenital Pulmonary Airway Malformation Type IV'. ( 27577236 )
2016
38
Type I Pleuropulmonary Blastoma versus Congenital Pulmonary Airway Malformation Type IV. ( 27562209 )
2016
39
Prenatal and Postnatal Management of Congenital Pulmonary Airway Malformation. ( 27070354 )
2016
40
Retrospective Evaluation of Children with Congenital Pulmonary Airway Malformation: A Single Center Experience of 20 Years. ( 26942995 )
2016
41
Letter to the Editor: Surgical versus conservative management of congenital pulmonary airway malformation in children: A systematic review and meta-analysis" by Kapralik et al J Pediatr Surg 51 (2016) 508-512. ( 27497496 )
2016
42
Type II congenital pulmonary airway malformation associated with intralobar pulmonary sequestration: report of a case and review of classification criteria. ( 28195247 )
2016
43
Surveillance of fetal lung lesions using the congenital pulmonary airway malformation volume ratio: natural history and outcomes. ( 26713859 )
2016
44
Prenatal Diagnosis and Evaluation of Sonographic Predictors for Intervention and Adverse Outcome in Congenital Pulmonary Airway Malformation. ( 26978067 )
2016
45
Pleuropulmonary Blastoma in a Neonate Diagnosed Prenatally as Congenital Pulmonary Airway Malformation. ( 26227654 )
2015
46
Congenital pulmonary airway malformation and sequestration: Two standpoints for a single condition. ( 25883453 )
2015
47
Congenital Pulmonary Airway Malformation in an Adult Male: A Case Report with Literature Review. ( 26236529 )
2015
48
Congenital pulmonary airway malformation: A report of two cases. ( 25984523 )
2015
49
Unusual signal intensity of congenital pulmonary airway malformation on fetal magnetic resonance imaging. ( 25875635 )
2015
50
Comparison of congenital pulmonary airway malformation volume ratios calculated by ultrasound and magnetic resonance imaging. ( 26607147 )
2015

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 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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