PedAM

Pediatric Disease Annotations & Medicines


	wolff-parkinson-white syndrome

Disease ID	109
Disease	wolff-parkinson-white syndrome
Definition	A form of ventricular pre-excitation characterized by a short PR interval and a long QRS interval with a delta wave. In this syndrome, atrial impulses are abnormally conducted to the HEART VENTRICLES via an ACCESSORY CONDUCTING PATHWAY that is located between the wall of the right or left atria and the ventricles, also known as a BUNDLE OF KENT. The inherited form can be caused by mutation of PRKAG2 gene encoding a gamma-2 regulatory subunit of AMP-activated protein kinase.
Synonym	anomalous ventricular excitation syndrome auriculoventricular accessory pathway syndrome false bundle-branch block syndrome parkinson syndrome white wolff parkinson syndrome whites wolf parkinson syndrome whites wolfs parkinson wolf white syndrome parkinsons syndrome white wolf parkinsons syndrome white wolff parkinsons syndrome white wolfs syndrome wolf parkinsons white syndrome wolff parkinsons white syndrome wolff-parkinson-white syndrome wpw syndrome, wolf-parkinson-white syndrome, wolff-parkinson-white syndrome, wpw ventricular pre-excitation with arrhythmia ventricular pre-excitation with arrhythmia, nos wolf parkinson white syndrome wolf white parkinson syndrome wolf-parkinson-white syndrome wolff - parkinson - white syndrome wolff parkinson syndrome white wolff parkinson white syndrome wolff-park-white syndr. wolff-parkinson-white arrhythmia wolff-parkinson-white pattern wolff-parkinson-white pattern (disorder) wolff-parkinson-white pattern (finding) wolff-parkinson-white syndrome [disease/finding] wolff-parkinson-white ventricular preexcitation wpw wpw - wolff-parkinson-white pattern wpw - wolff-parkinson-white syndrome wpw syndrome wpws
OMIM	OMIM:194200
DOID	DOID:384
UMLS	C0043202
MeSH	D014927
SNOMED-CT	SNOMEDCT_US_2016_09_01:74390002
Comorbidity	UMLS \| Disease \| Sentences' Count(Total Sentences:9) C0018801 \| heart failure \| 2 C0007194 \| hypertrophic cardiomyopathy \| 1 C0027127 \| myotonia congenita \| 1 C0026269 \| mitral stenosis \| 1 C0175704 \| leopard syndrome \| 1 C0001125 \| lactic acidosis \| 1 C0023976 \| long qt syndrome \| 1 C0027059 \| myocarditis \| 1 C0878544 \| cardiomyopathy \| 1
Curated Gene	Entrez_id \| Symbol \| Resource(Total Genes:1) PRKAG2 \| 51422 \| CLINVAR;CTD_human;UNIPROT;GHR
Inferring Gene	(Waiting for update.)
Text Mined Gene	Entrez_id \| Symbol \| Score \| Resource(Total Genes:73) 5837 \| PYGM \| DISEASES 2218 \| FKTN \| DISEASES 79176 \| FBXL15 \| DISEASES 29098 \| RANGRF \| DISEASES 55856 \| ACOT13 \| DISEASES 53632 \| PRKAG3 \| DISEASES 471 \| ATIC \| DISEASES 6909 \| TBX2 \| DISEASES 7291 \| TWIST1 \| DISEASES 5908 \| RAP1B \| DISEASES 2521 \| FUS \| DISEASES 182 \| JAG1 \| DISEASES 6926 \| TBX3 \| DISEASES 3757 \| KCNH2 \| DISEASES 2702 \| GJA5 \| DISEASES 4851 \| NOTCH1 \| DISEASES 60496 \| AASDHPPT \| DISEASES 2180 \| ACSL1 \| DISEASES 2697 \| GJA1 \| DISEASES 126731 \| CCSAP \| DISEASES 4838 \| NODAL \| DISEASES 7547 \| ZIC3 \| DISEASES 51422 \| PRKAG2 \| DISEASES 5224 \| PGAM2 \| DISEASES 339302 \| CPLX4 \| DISEASES 171558 \| PTCRA \| DISEASES 2548 \| GAA \| DISEASES 171389 \| NLRP6 \| DISEASES 54968 \| TMEM70 \| DISEASES 9377 \| COX5A \| DISEASES 2997 \| GYS1 \| DISEASES 64388 \| GREM2 \| DISEASES 3615 \| IMPDH2 \| DISEASES 78987 \| CRELD1 \| DISEASES 2837 \| UTS2R \| DISEASES 6794 \| STK11 \| DISEASES 10498 \| CARM1 \| DISEASES 349149 \| GJC3 \| DISEASES 6331 \| SCN5A \| DISEASES 3266 \| ERAS \| DISEASES 259307 \| IL4I1 \| DISEASES 875 \| CBS \| DISEASES 3614 \| IMPDH1 \| DISEASES 4625 \| MYH7 \| DISEASES 781 \| CACNA2D1 \| DISEASES 4519 \| MT-CYB \| DISEASES 4540 \| MT-ND5 \| DISEASES 4538 \| MT-ND4 \| DISEASES 3664 \| IRF6 \| DISEASES 7139 \| TNNT2 \| DISEASES 9748 \| SLK \| DISEASES 55170 \| PRMT6 \| DISEASES 5563 \| PRKAA2 \| DISEASES 8565 \| YARS \| DISEASES 2170 \| FABP3 \| DISEASES 4814 \| NINJ1 \| DISEASES 3055 \| HCK \| DISEASES 9882 \| TBC1D4 \| DISEASES 10631 \| POSTN \| DISEASES 1854 \| DUT \| DISEASES 5799 \| PTPRN2 \| DISEASES 64065 \| PERP \| DISEASES 5817 \| PVR \| DISEASES 3276 \| PRMT1 \| DISEASES 3920 \| LAMP2 \| DISEASES 161 \| AP2A2 \| DISEASES 4700 \| NDUFA6 \| DISEASES 149466 \| C1orf210 \| DISEASES 8914 \| TIMELESS \| DISEASES 27229 \| TUBGCP4 \| DISEASES 102723508 \| KANTR \| DISEASES 4567 \| MT-TL1 \| DISEASES 103752588 \| PACERR \| DISEASES
Locus	(Waiting for update.)

Disease ID	109
Disease	wolff-parkinson-white syndrome
Integrated Phenotype	HPO \| Name(Total Integrated Phenotypes:10) HP:0004757 \| Paroxysmal atrial fibrillation HP:0001716 \| Wolff-Parkinson-White syndrome HP:0001645 \| Sudden cardiac death HP:0001962 \| Palpitations HP:0001297 \| Cerebral vascular events HP:0001638 \| Cardiomyopathy HP:0006684 \| Ventricular preexcitation with multiple accessory pathways HP:0005165 \| Shortened PR interval on EKG HP:0006677 \| Prolonged QRS complex on EKG HP:0004763 \| Episodic supraventricular tachycardia
Text Mined Phenotype	HPO \| Name \| Sentences' Count(Total Phenotypes:21) HP:0005110 \| Atrial fibrillation \| 8 HP:0001649 \| Tachycardia \| 5 HP:0011675 \| Arrhythmias \| 3 HP:0010316 \| Ebstein's anomaly of the tricuspid valve \| 2 HP:0001663 \| Ventricular fibrillation \| 2 HP:0004756 \| Ventricular tachycardia \| 2 HP:0004755 \| Supraventricular tachycardia \| 2 HP:0001635 \| Congestive heart failure \| 2 HP:0011644 \| Coronary sinus diverticulum \| 1 HP:0004309 \| Pre-excitation syndrome \| 1 HP:0002486 \| Myotonia \| 1 HP:0001718 \| Mitral stenosis \| 1 HP:0001638 \| Cardiomyopathy \| 1 HP:0012272 \| Osborne waves \| 1 HP:0006789 \| Mitochondrial encephalopathy \| 1 HP:0001298 \| Encephalopathy \| 1 HP:0001639 \| Hypertrophic cardiomyopathy \| 1 HP:0003128 \| Lactic acidosis \| 1 HP:0001279 \| Syncope \| 1 HP:0001657 \| Prolonged QT interval \| 1 HP:0012819 \| Myocarditis \| 1

Disease ID	109
Disease	wolff-parkinson-white syndrome
Manually Symptom	UMLS \| Name(Total Manually Symptoms:36) C2712322 \| tachycardia C2697375 \| brugada syndrome C2364072 \| depression C2108112 \| ventricular fibrillation C2108093 \| paroxysmal supraventricular tachycardia C2041143 \| right bundle branch block C1963244 \| supraventricular tachycardia C1660219 \| analgesia C1546602 \| diverticulum C1442837 \| myocardial necrosis C1290138 \| systemic sclerosis sine scleroderma C0949658 \| familial hypertrophic cardiomyopathy C0694539 \| chronic atrial fibrillation C0344432 \| multiform ventricular tachycardia C0340493 \| idiopathic ventricular fibrillation C0340477 \| atrioventricular reentrant tachycardia C0340471 \| atrioventricular tachycardia C0340425 \| hypertrophic cardiomyopathy C0242514 \| reciprocating tachycardia C0242514 \| reciprocal tachycardia C0235480 \| paroxysmal atrial fibrillation C0232197 \| fibrillation C0039240 \| supraventricular tachycardias C0039236 \| paroxysmal tachycardia C0039070 \| syncope C0038454 \| cerebral infarction C0034067 \| emphysema C0032915 \| pre-excitation C0030591 \| paroxysmal ventricular tachycardia C0027051 \| myocardial infarction C0027051 \| myocardial infarct C0024796 \| marfan's syndrome C0021308 \| infarction C0013384 \| dyskinesia C0011981 \| diaphragmatic eventration C0002963 \| prinzmetal's angina
Text Mined Symptom	UMLS \| Name \| Sentences' Count(Total Symptoms:9) C0232197 \| fibrillation \| 10 C0039231 \| tachycardia \| 5 C0042510 \| ventricular fibrillation \| 2 C0039240 \| supraventricular tachycardia \| 2 C0012817 \| diverticulum \| 1 C0007194 \| hypertrophic cardiomyopathy \| 1 C0242514 \| reciprocating tachycardia \| 1 C0039070 \| syncope \| 1 C0340493 \| idiopathic ventricular fibrillation \| 1

Manually Genotype(Total Text Mining Genotypes:0)
(Waiting for update.)

All Snps(Total Genotypes:5)
snpId	pubmedId	geneId	geneSymbol	diseaseId	sourceId	sentence	score	Year	geneSymbol_dbSNP	CHROMOSOME	POS	REF	ALT
rs121908987	20031621	51422	PRKAG2	umls:C0043202	BeFree	Humans with an R302Q mutation in AMPKgamma(2) (the PRKAG2 gene) develop a glycogen storage cardiomyopathy characterized by a familial form of Wolff-Parkinson-White syndrome and cardiac hypertrophy.	0.366253095	2009	PRKAG2	7	151576412	C	T,A
rs121908987	15611370	7453	WARS	umls:C0043202	BeFree	We identified a gene (PRKAG2) that encodes the gamma-2 regulatory subunit of AMP-activated protein kinase (AMPK) with a mutation (Arg302Gln) responsible for familial Wolff-Parkinson-White (WPW) syndrome.	0.001085767	2005	PRKAG2	7	151576412	C	T,A
rs121908987	15611370	51422	PRKAG2	umls:C0043202	BeFree	We identified a gene (PRKAG2) that encodes the gamma-2 regulatory subunit of AMP-activated protein kinase (AMPK) with a mutation (Arg302Gln) responsible for familial Wolff-Parkinson-White (WPW) syndrome.	0.366253095	2005	PRKAG2	7	151576412	C	T,A
rs121908987	NA	51422	PRKAG2	umls:C0043202	CLINVAR	NA	0.366253095	NA	PRKAG2	7	151576412	C	T,A
rs121908991	15877279	51422	PRKAG2	umls:C0043202	BeFree	Thus, recurrent heterozygous R531Q missense mutations in PRKAG2 give rise to a massive nonlysosomal cardiac glycogenosis of fetal symptomatic onset and rapidly fatal course, constituting a genotypically and clinically distinct variant of hypertrophic cardiomyopathy with Wolff-Parkinson-White syndrome.	0.366253095	2005	PRKAG2	7	151560610	C	T,A

GWASdb Annotation(Total Genotypes:0)
(Waiting for update.)

GWASdb Snp Trait(Total Genotypes:0)
(Waiting for update.)

Mapped by lexical matching(Total Items:1)
HP ID	HP Name	MP ID	MP Name	Annotation
HP:0001645	Sudden cardiac death	MP:0003393	decreased cardiac output;HP:0005165	Shortened PR interval

Mapped by homologous gene(Total Items:1)
HP ID	HP Name	MP ID	MP Name	Annotation
HP:0001645	Sudden cardiac death	MP:0005402	abnormal action potential;HP:0001297	Stroke

Chemical(Total Drugs:2)
CUI	ChemicalName	ChemicalID	CasRN	DiseaseName	DiseaseID	DirectEvidence	PubMedIDs
C0043202	diltiazem	D004110	42399-41-7	wolff-parkinson-white syndrome	MESH:D014927	therapeutic	2918157
C0043202	propranolol	D011433	525-66-6	wolff-parkinson-white syndrome	MESH:D014927	therapeutic	4067758

FDA approved drug and dosage information(Total Drugs:0)
(Waiting for update.)

FDA labeling changes(Total Drugs:0)
(Waiting for update.)

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