CCAM
MCID: CYS021
MIFTS: 34

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 54
Congenital Pulmonary Airway Malformation 54 60
Congenital Cystic Adenomatoid Malformation of the Lung 60
Congenital Cystic Adenomatous Malformation of the Lung 60
Congenital Cystic Adenomatoid Malformation 54
Congenital Cystic Disease of the Lung 60
Ccam 60
Cpam 60

Characteristics:

Orphanet epidemiological data:

60
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:

ICD10 34 Q33.0
ICD10 via Orphanet 35 Q33.0
UMLS via Orphanet 75 C0010668 C0158641
Orphanet 60 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, fetal lung and testes, and related phenotypes are respiratory insufficiency and abnormality of the pleura

Wikipedia : 77 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 66)
# 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 bronchiolo-alveolar adenocarcinoma 10.5
8 pleuropulmonary blastoma 10.5
9 emphysema, congenital lobar 10.4
10 lung abscess 10.4
11 patau syndrome 10.3
12 chromosomal triplication 10.3
13 mucinous adenocarcinoma 10.2
14 prune belly syndrome 10.2
15 pneumothorax, primary spontaneous 10.2
16 lymphatic malformation 7 10.2
17 pneumothorax 10.2
18 bronchogenic cyst 10.2
19 fetal edema 10.2
20 hydrops fetalis 10.2
21 tracheoesophageal fistula 10.2
22 esophageal duplication cyst 10.2
23 pleural empyema 10.1
24 exudative vitreoretinopathy 1 10.1
25 diaphragmatic hernia, congenital 10.1
26 tracheoesophageal fistula with or without esophageal atresia 10.1
27 renal hypodysplasia/aplasia 1 10.1
28 congenital heart defects, hamartomas of tongue, and polysyndactyly 10.1
29 hydrops fetalis, nonimmune 10.1
30 pulmonary arteriovenous fistulas 10.1
31 diabetes mellitus 10.1
32 hemangioma 10.1
33 esophageal atresia 10.1
34 meningocele 10.1
35 arteriovenous malformation 10.1
36 gestational diabetes 10.1
37 potter's syndrome 10.1
38 ehlers-danlos syndrome 10.1
39 epilepsy 10.1
40 capillary hemangioma 10.1
41 pulmonary tuberculosis 10.1
42 adenocarcinoma 10.1
43 rhabdomyosarcoma 10.1
44 pulmonary blastoma 10.1
45 lung disease 10.1
46 alveolar capillary dysplasia 10.1
47 pulmonary arteriovenous malformation 10.1
48 pulmonary artery agenesis 10.1
49 immune hydrops fetalis 10.1
50 fetal lung interstitial tumor 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:

60 33
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 respiratory insufficiency 60 33 occasional (7.5%) Occasional (29-5%) HP:0002093
2 abnormality of the pleura 60 33 occasional (7.5%) Occasional (29-5%) HP:0002103
3 polyhydramnios 60 33 occasional (7.5%) Occasional (29-5%) HP:0001561
4 premature birth 60 33 occasional (7.5%) Occasional (29-5%) HP:0001622
5 abnormality of the respiratory system 60 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 Central Nervous System Depressants Phase 3
4 Peripheral Nervous System Agents Phase 3
5 Analgesics, Opioid Phase 3
6 Anesthetics Phase 3
7 Anesthetics, Intravenous Phase 3
8 Anesthetics, Local Phase 3
9 Narcotics Phase 3
10 Adjuvants, Anesthesia Phase 3
11 Analgesics Phase 3
12 Anesthetics, General Phase 3
13
Betamethasone Approved, Vet_approved Phase 1, Phase 2 378-44-9 9782
14 glucocorticoids Phase 1, Phase 2
15 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 1, Phase 2
16 Respiratory System Agents Phase 1, Phase 2
17 Betamethasone Valerate Phase 1, Phase 2 2152-44-5
18 Hormone Antagonists Phase 1, Phase 2
19 Betamethasone benzoate Phase 1, Phase 2
20 Betamethasone sodium phosphate Phase 1, Phase 2
21 Hormones Phase 1, Phase 2
22 Anti-Asthmatic Agents Phase 1, Phase 2
23 Anti-Inflammatory Agents 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:

42
Lung, Fetal Lung, Testes

Publications for Cystic Adenomatoid Malformation of Lung

Articles related to Cystic Adenomatoid Malformation of Lung:

(show top 50) (show all 104)
# Title Authors Year
1
Thoracoscopic Pulmonary Lobectomy for Densely Fused Pulmonary Lobes in Children with Congenital Pulmonary Airway Malformation: Technical Tips. ( 30735092 )
2019
2
Cancer gene mutations in congenital pulmonary airway malformation patients. ( 30740464 )
2019
3
Manifestation of congenital pulmonary airway malformation in a 26-year-old adult, requiring surgery. ( 30844060 )
2019
4
Postnatally presented and spontaneously resolved congenital pulmonary airway malformation in a preterm baby. ( 30878962 )
2019
5
Case of mucinous adenocarcinoma of the lung associated with congenital pulmonary airway malformation in a neonate. ( 29441110 )
2018
6
Invasive Mucinous Adenocarcinoma in Congenital Pulmonary Airway Malformation: A Case Report. ( 30595422 )
2018
7
Dichorionic-diamniotic twins: One case each of esophageal atresia and congenital pulmonary airway malformation. ( 30187627 )
2018
8
Pleuropulmonary Blastoma Developing in a Case of Misinterpreted Congenital Pulmonary Airway Malformation: a Case Report. ( 30358469 )
2018
9
Congenital pulmonary airway malformation mimicking as pulmonary tuberculosis in five paediatric patients: a diagnostic dilemma. ( 29621946 )
2018
10
Accessory scrotum and congenital perineal lipoma in a child with type 2 congenital pulmonary airway malformation: A report of an unusual. ( 29623024 )
2018
11
Medium Term Pulmonary Function Test After Thoracoscopic Lobectomy for Congenital Pulmonary Airway Malformation: A Comparative Study with Normal Control. ( 29099644 )
2018
12
Three-dimensional computed tomography of congenital pulmonary airway malformation. ( 29356290 )
2018
13
Types II and III congenital pulmonary airway malformation with hydrops treated in utero with percutaneous sclerotherapy. ( 29665020 )
2018
14
Lung Ultrasound Findings in Congenital Pulmonary Airway Malformation. ( 29715700 )
2018
15
Congenital pulmonary airway malformation. ( 29780758 )
2018
16
Congenital pulmonary airway malformation: advances and controversies. ( 30169300 )
2018
17
Fetal Congenital Pulmonary Airway Malformation: The Role of an Objective Measurement of Cardiomediastinal Shift. ( 30199894 )
2018
18
An investigation on clinical differences between congenital pulmonary airway malformation and bronchial atresia. ( 30227995 )
2018
19
Predictors of early lobectomy after birth in prenatally diagnosed congenital pulmonary airway malformation. ( 30244937 )
2018
20
Long-term pulmonary function after lobectomy for congenital pulmonary airway malformation: is thoracoscopic approach really better than open? ( 30249357 )
2018
21
Congenital pulmonary airway malformation in the asymptomatic adult: A rare presentation. ( 30364709 )
2018
22
Mucinous adenocarcinoma with lepidic pattern and with K-RAS mutation in a newborn with antenatal diagnosis of congenital pulmonary airway malformation. ( 28889407 )
2017
23
It's Rare So Be Aware: Pleuropulmonary Blastoma Mimicking Congenital Pulmonary Airway Malformation. ( 28405541 )
2017
24
Is congenital pulmonary airway malformation really a rare disease? Result of a prospective registry with universal antenatal screening program. ( 27770196 )
2017
25
Optimal age for elective surgery of asymptomatic congenital pulmonary airway malformation: a meta-analysis. ( 28293700 )
2017
26
Congenital Bi-segmental Bronchial Atresia with Areas of Congenital Pulmonary Airway Malformation. ( 28408057 )
2017
27
An infected cyst due to a congenital pulmonary airway malformation. ( 28491811 )
2017
28
An Atypical Presentation of Congenital Pulmonary Airway Malformation (CPAM): A Rare Case with Antenatal Ultrasound Findings and Review of Literature. ( 28638495 )
2017
29
Images in Anesthesiology: Congenital Pulmonary Airway Malformation. ( 28719530 )
2017
30
Transthoracic catheter drainage for large symptomatic congenital pulmonary airway malformation. ( 29028157 )
2017
31
Perforated neuroenteric cyst masquerading as congenital pulmonary airway malformation. ( 29197847 )
2017
32
A Complication of Percutaneous Sclerotherapy for Congenital Pulmonary Airway Malformation: Intravascular Injection and Cardiac Necrosis. ( 29206544 )
2017
33
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
34
Surgical intervention for congenital pulmonary airway malformation (CPAM) patients with preoperative pneumonia and abscess formation: "open versus thoracoscopic lobectomy". ( 26661941 )
2016
35
Direct Tracheobronchopexy and Posterior Descending Aortopexy for Severe Left Mainstem Bronchomalacia Associated With Congenital Pulmonary Airway Malformation and Left Circumflex Aortic Arch. ( 27343517 )
2016
36
Congenital pulmonary airway malformation (CPAM) with initial presentation in an adult: a rare presentation of a rare disease. ( 27671988 )
2016
37
Congenital pulmonary airway malformation in a 36 year-old female. ( 27141433 )
2016
38
Surgical versus conservative management of congenital pulmonary airway malformation in children: A systematic review and meta-analysis. ( 26775193 )
2016
39
Congenital pulmonary airway malformation with atrial septal defect and pulmonary hypertension for lobectomy-anesthetic considerations. ( 27052089 )
2016
40
Refractory tension pneumothorax as a result of an internally displaced thoracoamniotic shunt in an infant with a congenital pulmonary airway malformation. ( 27469386 )
2016
41
Congenital pulmonary airway malformation - A study of 2 cases. ( 27772928 )
2016
42
Reply to the Letter to the Editor 'Type I Pleuropulmonary Blastoma versus Congenital Pulmonary Airway Malformation Type IV'. ( 27577236 )
2016
43
Type I Pleuropulmonary Blastoma versus Congenital Pulmonary Airway Malformation Type IV. ( 27562209 )
2016
44
Prenatal and Postnatal Management of Congenital Pulmonary Airway Malformation. ( 27070354 )
2016
45
Retrospective Evaluation of Children with Congenital Pulmonary Airway Malformation: A Single Center Experience of 20 Years. ( 26942995 )
2016
46
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
47
Type II congenital pulmonary airway malformation associated with intralobar pulmonary sequestration: report of a case and review of classification criteria. ( 28195247 )
2016
48
Surveillance of fetal lung lesions using the congenital pulmonary airway malformation volume ratio: natural history and outcomes. ( 26713859 )
2016
49
Prenatal Diagnosis and Evaluation of Sonographic Predictors for Intervention and Adverse Outcome in Congenital Pulmonary Airway Malformation. ( 26978067 )
2016
50
Pleuropulmonary Blastoma in a Neonate Diagnosed Prenatally as Congenital Pulmonary Airway Malformation. ( 26227654 )
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 EFO
18 ExPASy
20 FMA
29 GO
30 GTR
31 HGMD
32 HMDB
33 HPO
34 ICD10
35 ICD10 via Orphanet
36 ICD9CM
37 IUPHAR
38 KEGG
39 LifeMap
41 LOVD
43 MedGen
45 MeSH
46 MESH via Orphanet
47 MGI
50 NCI
51 NCIt
52 NDF-RT
55 NINDS
56 Novoseek
58 OMIM
59 OMIM via Orphanet
63 PubMed
65 QIAGEN
70 SNOMED-CT via HPO
71 SNOMED-CT via Orphanet
72 TGDB
73 Tocris
74 UMLS
75 UMLS via Orphanet
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