Acute Chest Syndrome malady

Disease Ontology: A lung disease that involves a vasoocclusive crisis of the pulmonary vasculature seen in patients with sickle cell anemia. the crisis is initiated by a lung infection, and the resulting inflammation and loss of oxygen tension leads to sickling of red cells and further vasoocclusion. the disease has symptom fever, has symptom pleuritic chest pain, has symptom tachypnea, has symptom cough, has symptom hypoxemia, has symptom marked leukocytosis, and has symptom diffuse infiltrates on chest radiograph.⁶

MalaCards: Acute Chest Syndrome, also known as acute chest syndrome in sickle cell disease, is related to sickle cell disease and sickle cell anemia, and has symptoms including fever, pleuritic chest pain and tachypnea. An important gene associated with Acute Chest Syndrome is PLA2G2A (phospholipase A2, group IIA (platelets, synovial fluid)), and among its related pathways are alpha-Linolenic acid metabolism and Anandamide biosynthesis and metabolism. The compounds apocynin and sodium nitroprusside have been mentioned in the context of this disorder. Affiliated tissues include lung and t cells, and related mouse phenotypes are reproductive system and hematopoietic system.

Wikipedia: The acute chest syndrome is a noninfectiousvaso-occlusive crisis of the pulmonary vasculature commonly...⁴⁴ more...

Aliases & Descriptions:

acute chest syndrome 6 8 32 43 acute chest syndrome in sickle cell disease 6

Diseases related to acute chest syndrome by text searches and GeneDecks gene sharing:

(show top 50)    (show all 248)

id	Related Disease	Score	Top Affiliating Genes
1	sickle cell disease	27.3	VCAM1, IL8, GP1BA, HLA-DQB1, HLA-DRB1, PLA2G10
2	sickle cell anemia	25.0	VCAM1, BLM, CFB, IL8, GP1BA, HLA-DQB1
3	asthma	24.3	VCAM1, IL8, HLA-DQB1, HLA-DRB1, PLA2G10, PLA2G1B
4	anemia	23.3	VCAM1, BLM, CFB, IL8, GP1BA, HLA-DQB1
5	spinal cord ischemia	13.5	PLA2G2A, NOS3
6	prinzmetal's variant angina	13.5	NOS3, EDN1
7	coronary artery vasospasm	13.4	NOS3, EDN1
8	pre-eclampsia	13.4	NOS3, EDN1
9	thromboangiitis obliterans	13.3	VCAM1, NOS3, EDN1
10	ischemic optic neuropathy	13.2	GP1BA, NOS3, G6PD
11	hereditary spherocytosis	13.1	G6PD, HBB
12	splenic infarction	13.1	BLM, HBB
13	meconium aspiration syndrome	13.1	PLA2G10, PLA2G1B, PLA2G2A, EDN1
14	hemoglobin s beta-thalassemia	13.1	G6PD, HBB
15	fetal erythroblastosis	13.1	G6PD, HBB
16	splenic sequestration	13.0	CFB, GP1BA, SLC12A2, GBA2
17	cluster headache	13.0	VCAM1, NOS1, NOS3
18	arteriosclerosis	13.0	VCAM1, GP1BA, NOS3, EDN1
19	pancreatic diseases	13.0	VCAM1, IL8, PLA2G2A
20	hypertriglyceridemia	13.0	VCAM1, GP1BA, NOS3, EDN1
21	alpha thalassemia	12.9	VCAM1, G6PD, HBB
22	adult respiratory distress syndrome	12.9	IL8, PLA2G2A, EDN1
23	uremia	12.9	VCAM1, CFB, PLA2G2A, NOS3, EDN1
24	septic shock	12.9	IL8, PLA2G1B, PLA2G2A, NOS3
25	priapism	12.9	BLM, CFB, NOS3, G6PD
26	impotence	12.9	NOS1, NOS3, EDN1
27	pulmonary emphysema	12.9	IL8, NOS3, EDN1
28	beta thalassemia	12.8	G6PD, HBB
29	cerebral artery occlusion	12.8	NOS1, NOS3, EDN1
30	pulmonary edema	12.8	IL8, NOS3, EDN1
31	cerebrovascular accident	12.8	VCAM1, GP1BA, PLA2G1B, NOS3, EDN1
32	cholelithiasis	12.8	GP1BA, G6PD, HBB
33	chronic granulomatous disease	12.8	KEL, IL8, G6PD
34	sleep disorder	12.7	VCAM1, IL8, HLA-DQB1
35	hyperlipidemia	12.7	VCAM1, GP1BA, NOS3, EDN1
36	sleep apnea	12.7	VCAM1, IL8, NOS3, EDN1
37	aortic valve stenosis	12.7	VCAM1, NOS1, NOS3, EDN1
38	multiple sclerosis	12.7	HLA-DQB1, HLA-DRB1
39	retinal vein occlusion	12.7	IL8, EDN1, G6PD
40	tubulointerstitial nephritis and uveitis	12.7	HLA-DQB1, HLA-DRB1
41	sepsis	12.6	CFB, IL8, GP1BA, PLA2G2A, NOS3
42	vascular disease	12.6	VCAM1, GP1BA, NOS3, EDN1, G6PD
43	congenital hemolytic anemia	12.6	G6PD, HBB
44	wegener's granulomatosis	12.6	VCAM1, IL8, HLA-DQB1, NOS3
45	takayasu's arteritis	12.6	VCAM1, HLA-DRB1, EDN1
46	gastric ulcer	12.5	IL8, NOS3, EDN1, G6PD
47	mayer-rokitansky-kuster-hauser syndrome	12.5	BLM, CFB, G6PD, HBB
48	congenital heart defect	12.5	VCAM1, IL8, PLA2G1B, NOS3, EDN1
49	ischemic heart disease	12.5	VCAM1, IL8, GP1BA, NOS3, EDN1
50	spontaneous abortion	12.5	CFB, IL8, HLA-DQB1, NOS3

Symptoms: fever, pleuritic chest pain, tachypnea, cough, hypoxemia, marked leukocytosis, diffuse infiltrates on chest radiograph.⁶

Approved drugs:

Drug clinical trials:

MalaCards organs/tissues related to acute chest syndrome:

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MGI Mouse Phenotypes related to acute chest syndrome:

²⁵

id	Description	MGI Source Accession	Score	Top Affiliating Genes
1	reproductive system phenotype	MP:0005389	9.5	HBB, SLC12A2, GBA2
2	hematopoietic system phenotype	MP:0005397	8.9	HLA-DQB1, GP1BA, BLM
3	cardiovascular system phenotype	MP:0005385	6.8	SLC12A2, HBB, G6PD, EDN1, NOS3, NOS1
4	homeostasis/metabolism phenotype	MP:0005376	6.4	VCAM1, GBA2, HBB, G6PD, EDN1, NOS3

Articles related to acute chest syndrome:

(show all 13)

id	Title	Authors	Year	Affiliating Genes
1	HLA DRB1*130101-DQB1*060101 haplotype is associated with acute chest syndrome in sickle cell anemia patients. (19254255)	Mahdi N.... Almawi W.Y.	2009	HLA-DQB1, HLA-DRB1
2	Physician-diagnosed asthma and acute chest syndrome: associations with NOS polymorphisms. (17351927)	Duckworth L.... Lima J.J.	2007	NOS3, NOS1
3	Sickle cell disease patients in eastern province of S audi Arabia suffer less severe acute chest syndrome than patients with African haplotypes. (19727367)	Alabdulaali M.K.	2007	G6PD
4	Transfusion prevents acute chest syndrome predicted by elevated secretory phospholipase A2. (17156397)	Styles L.A.... Kuypers F.A.	2007	PLA2G10
5	ET-1 and ecNOS gene polymorphisms andsusceptibility to acute chest syndrome and painful vaso-occlusive crises in children with sickle cell anemia. (16956834)	Chaar V.... Romana M.	2006	NOS3, EDN1
6	Serum biomarkers for identifying acute chest syndrome among patients who have sickle cell disease and present to the emergency department. (16099851)	Naprawa J.T.... Ranalli M.A.	2005	EDN1
7	The role of secretory phospholipase A2 in acute chest syndrome. (15040432)	Kuypers F.A.... Styles L.A.	2004	PLA2G10
8	Association of T-786C eNOS gene polymorphism with increased susceptibility to acute chest syndrome in females with sickle cell disease. (14687036)	Sharan K.... Keller M.	2004	NOS3
9	Low exhaled nitric oxide and a polymorphism in the NOS I gene is associated with acute chest syndrome. (11751185)	Sullivan K.J.... Lima J.J.	2001	NOS1
10	Elevated serum and bronchoalveolar lavage fluid levels of interleukin 8 and granulocyte colony-stimulating factor associated with the acute chest syndrome in patients with sickle cell disease. (11122088)	Abboud M.R.... Ballas S.K.	2000	IL8
11	Sickle cell acute chest syndrome: pathogenesis and rationale for treatment. (10477680)	Stuart M.J.... Setty B.N.	1999	VCAM1
12	Endothelin-1 production during the acute chest syndrome in sickle cell disease. (9230761)	Hammerman S.I.... Farber H.W.	1997	EDN1
13	Phospholipase A2 levels in acute chest syndrome of sickle cell disease. (8630425)	Styles L.A.... Kuypers F.A.	1996	PLA2G10

Genes related to acute chest syndrome according to GeneCards:

(show all 23)

id	Symbol	Description	Score	GeneCards Section Context
1	PLA2G2A	phospholipase A2, group IIA (platelets, synovial fluid)	11.0
2	VCAM1	vascular cell adhesion molecule 1	11.0	Publications
3	NOS3	nitric oxide synthase 3 (endothelial cell)	11.0	Publications
4	PLA2G10	phospholipase A2, group X	11.0	Publications
5	ART4	ADP-ribosyltransferase 4 (Dombrock blood group)	11.0
6	GBA2	glucosidase, beta (bile acid) 2	11.0
7	CYGB	cytoglobin	11.0
8	SUDS3	suppressor of defective silencing 3 homolog (S. cerevisiae)	11.0
9	KEL	Kell blood group, metallo-endopeptidase	11.0
10	PLA2G1B	phospholipase A2, group IB (pancreas)	11.0
11	PHC3	polyhomeotic homolog 3 (Drosophila)	11.0
12	CFB	complement factor B	11.0
13	GP1BA	glycoprotein Ib (platelet), alpha polypeptide	11.0
14	SLC12A2	solute carrier family 12 (sodium/potassium/chloride transporters), member 2	11.0
15	ST5	suppression of tumorigenicity 5	11.0
16	EDN1	endothelin 1	11.0	Publications
17	BLM	Bloom syndrome, RecQ helicase-like	11.0
18	G6PD	glucose-6-phosphate dehydrogenase	11.0	Publications
19	NOS1	nitric oxide synthase 1 (neuronal)	11.0	Publications
20	HBB	hemoglobin, beta	11.0
21	HLA-DQB1	major histocompatibility complex, class II, DQ beta 1	11.0	Publications
22	HLA-DRB1	major histocompatibility complex, class II, DR beta 1	11.0	Publications
23	IL8	interleukin 8	11.0	Publications

Pathways related to acute chest syndrome according to GeneDecks:

(show all 15)

id	Pathway	Score	Top Affiliating Genes
1	alpha-Linolenic acid metabolism20	10.1	PLA2G2A, PLA2G1B, PLA2G10
2	Anandamide biosynthesis and metabolism41	10.1	PLA2G2A, PLA2G1B, PLA2G10
3	Anandamide biosynthesis and metabolism10	10.1	PLA2G2A, PLA2G1B, PLA2G10
4	Linoleic acid metabolism20	10.0	PLA2G2A, PLA2G1B, PLA2G10
5	Ether lipid metabolism20	10.0	PLA2G10, PLA2G1B, PLA2G2A
6	VEGF signaling pathway20	10.0	PLA2G10, PLA2G1B, PLA2G2A, NOS3
7	Fat digestion and absorption20	9.9	PLA2G2A, PLA2G1B, PLA2G10
8	Arachidonic acid metabolism20	9.9	PLA2G10, PLA2G1B, PLA2G2A
9	Pancreatic secretion20	9.8	PLA2G10, PLA2G1B, PLA2G2A, SLC12A2
10	Long-term depression20	9.7	NOS1, PLA2G2A, PLA2G1B, PLA2G10
11	Malaria20	9.3	VCAM1, IL8, HBB
12	Chemokine Signaling36	9.3	PLA2G2A, PLA2G1B, PLA2G10, IL8
13	Endothelin-1 Signaling Pathway36	8.9	IL8, PLA2G1B, PLA2G2A, NOS1, NOS3, EDN1
14	Toxoplasmosis20	8.7	PLA2G2A, PLA2G1B, PLA2G10, HLA-DRB1, HLA-DQB1
15	Staphylococcus aureus infection20	8.5	CFB, HLA-DQB1, HLA-DRB1

Compounds related to acute chest syndrome according to GeneDecks:

(show top 50)    (show all 81)

id	Compound	Score	Top Affiliating Genes
1	apocynin32	10.7	VCAM1
2	sodium nitroprusside32	10.5	G6PD
3	adma32	10.4	NOS1
4	pacocf342	10.3	PLA2G10, PLA2G1B, PLA2G2A
5	N-Tridecanoic Acid9 9	11.3	PLA2G2A, PLA2G1B
6	rhc 8026742 32	11.3	PLA2G2A, PLA2G1B, PLA2G10
7	obaa42	10.3	PLA2G2A, PLA2G1B, PLA2G10
8	L-N(Omega)-Nitroarginine-(4r)-Amino-L-Proline Amide9 9	11.2	NOS3, NOS1
9	S-Ethyl-N-Phenyl-Isothiourea9 9	11.2	NOS3, NOS1
10	L-N(Omega)-Nitroarginine-2,4-L-Diaminobutyric Amide9 9	11.2	NOS3, NOS1
11	Nitroarginine9 9	11.2	NOS3, NOS1
12	N-{(4s)-4-Amino-5-[(2-Aminoethyl)Amino]Pentyl}-N'-Nitroguanidine9 9	11.2	NOS3, NOS1
13	l-nna42	10.2	NOS1, NOS3
14	N-{(3S,4S)-4-[(6-AMINO-4-METHYLPYRIDIN-2-YL)METHYL]PYRROLIDIN-3-YL}-N'-(4-CHLOROBENZYL)ETHANE-1,2-DIAMINE9 9	11.2	NOS1, NOS3
15	N-{(3R,4S)-4-[(6-amino-4-methylpyridin-2-yl)methyl]pyrrolidin-3-yl}-N'-(3-chlorobenzyl)ethane-1,2-diamine9 9	11.1	NOS1, NOS3
16	alpha-linolenic acid32 9 18 9	13.1	PLA2G2A, PLA2G1B, PLA2G10
17	n-(3-(aminomethyl)benzyl)acetamidine32 9 9	12.0	NOS3, NOS1
18	3-bromo-7-nitroindazole42 9 9	12.0	NOS3, NOS1
19	txb232	10.0	PLA2G10, PLA2G1B, PLA2G2A, EDN1
20	s-ethylisothiourea32 9 9	12.0	NOS3, NOS1
21	L-Arginine9 18 9	11.9	ART4, NOS1, NOS3
22	n5-(1-iminoethyl)-l-ornithine32	9.9	NOS3, NOS1
23	n(g)-nitro-l-arginine methyl ester32	9.9	EDN1, NOS3, NOS1
24	atorvastatin32 34 9 18 9	13.9	VCAM1, PLA2G2A, NOS3, EDN1
25	l-thiocitrulline32 9 9	11.8	NOS3, NOS1
26	ng-nitro-l-arginine32	9.8	NOS1, NOS3, EDN1
27	l-nmma32	9.7	EDN1, NOS3, NOS1, VCAM1
28	prostacyclin32	9.7	EDN1, NOS3, PLA2G2A, GP1BA, VCAM1
29	8-isoprostane32	9.7	EDN1, NOS3, IL8, VCAM1
30	phosphatidylcholine32	9.7	G6PD, PLA2G2A, PLA2G1B, PLA2G10, VCAM1
31	rottlerin32 42	10.7	EDN1, NOS3, IL8, VCAM1
32	fmlp32	9.6	PLA2G2A, PLA2G1B, PLA2G10, IL8, VCAM1
33	pdtc32	9.5	VCAM1, IL8, PLA2G2A, NOS1
34	carvedilol32 34 9 9	12.5	VCAM1, NOS3, EDN1
35	diphenyleneiodonium32	9.5	VCAM1, IL8, PLA2G2A, NOS3, G6PD
36	s-nitroso-n-acetylpenicillamine32	9.4	EDN1, NOS3, NOS1, IL8
37	glycerol32 9 18 9	12.3	G6PD, NOS3, NOS1, PLA2G2A, PLA2G1B, PLA2G10
38	hydroxyurea32 9 9	11.2	HBB, NOS1, BLM, VCAM1
39	indomethacin32 9 9	11.2	EDN1, NOS3, NOS1, PLA2G2A, PLA2G1B, GP1BA
40	genistein32 9 18 9	12.0	EDN1, NOS3, NOS1, PLA2G2A, IL8, VCAM1
41	pge232	8.8	EDN1, NOS1, PLA2G2A, PLA2G1B, PLA2G10, IL8
42	tamoxifen32 34 9 9	11.7	G6PD, NOS3, NOS1, VCAM1, HBB
43	sb 20358032 42	9.7	EDN1, NOS3, NOS1, IL8, VCAM1
44	superoxide32 18	9.6	G6PD, NOS3, NOS1, PLA2G2A, PLA2G1B, PLA2G10
45	lactate32	8.6	HBB, G6PD, PLA2G2A, PLA2G1B, GP1BA, IL8
46	valine32	8.4	HBB, G6PD, NOS1, HLA-DRB1, GP1BA
47	h2o232	8.2	HBB, G6PD, NOS3, NOS1, PLA2G2A, PLA2G1B
48	nitric oxide32 9 18 9	11.2	SLC12A2, HBB, EDN1, NOS3, NOS1, CYGB
49	arginine32	8.0	HBB, EDN1, NOS3, NOS1, PLA2G2A, HLA-DRB1
50	oxygen32 18	8.6	SLC12A2, HBB, G6PD, EDN1, NOS3, NOS1

Cellular components related to acute chest syndrome according to GeneDecks:

id	Name	GO ID	Score	Top Affiliating Genes
1	endocytic vesicle membrane	GO:030666	8.9	HLA-DQB1, HLA-DRB1, NOS3

Biological processes related to acute chest syndrome according to GeneDecks:

(show all 18)

id	Name	GO ID	Score	Top Affiliating Genes
1	phosphatidylinositol acyl-chain remodeling	GO:036149	10.2	PLA2G10, PLA2G1B, PLA2G2A
2	phosphatidylserine acyl-chain remodeling	GO:036150	10.2	PLA2G2A, PLA2G1B, PLA2G10
3	phosphatidylglycerol acyl-chain remodeling	GO:036148	10.2	PLA2G2A, PLA2G1B, PLA2G10
4	phosphatidylethanolamine acyl-chain remodeling	GO:036152	10.2	PLA2G10, PLA2G1B, PLA2G2A
5	phosphatidylcholine acyl-chain remodeling	GO:036151	10.2	PLA2G2A, PLA2G1B, PLA2G10
6	regulation of systemic arterial blood pressure by endothelin	GO:003100	10.2	EDN1, NOS3
7	phosphatidic acid biosynthetic process	GO:006654	10.1	PLA2G2A, PLA2G1B, PLA2G10
8	negative regulation of hydrolase activity	GO:051346	10.1	NOS3, NOS1
9	nitric oxide transport	GO:030185	9.8	EDN1, HBB
10	arginine catabolic process	GO:006527	9.8	NOS3, NOS1
11	oxygen transport	GO:015671	9.8	HBB, CYGB
12	regulation of blood vessel size	GO:050880	9.8	NOS3, HBB
13	neutrophil chemotaxis	GO:030593	9.6	EDN1, PLA2G1B, IL8
14	negative regulation of potassium ion transport	GO:043267	9.5	NOS1, NOS3
15	response to hypoxia	GO:001666	9.5	EDN1, NOS3, NOS1, VCAM1
16	immunoglobulin production involved in immunoglobulin mediated immune response	GO:002381	9.2	HLA-DRB1, HLA-DQB1
17	humoral immune response mediated by circulating immunoglobulin	GO:002455	9.1	HLA-DQB1, HLA-DRB1
18	interferon-gamma-mediated signaling pathway	GO:060333	8.8	VCAM1, HLA-DQB1, HLA-DRB1

Molecular functions related to acute chest syndrome according to GeneDecks:

(show all 9)

id	Name	GO ID	Score	Top Affiliating Genes
1	phospholipase A2 activity	GO:004623	10.1	PLA2G10, PLA2G1B, PLA2G2A
2	calcium-dependent phospholipase A2 activity	GO:047498	10.1	PLA2G1B, PLA2G2A
3	tetrahydrobiopterin binding	GO:034617	10.0	NOS3, NOS1
4	nitric-oxide synthase activity	GO:004517	10.0	NOS3, NOS1
5	arginine binding	GO:034618	9.8	NOS1, NOS3
6	NADP binding	GO:050661	9.5	NOS1, NOS3, G6PD
7	cadmium ion binding	GO:046870	9.5	NOS1, NOS3
8	heme binding	GO:020037	9.2	HBB, NOS3, NOS1, CYGB
9	iron ion binding	GO:005506	9.0	HBB, NOS3, NOS1, CYGB