PedAM

Pediatric Disease Annotations & Medicines


	hepatic veno-occlusive disease

Disease ID	252
Disease	hepatic veno-occlusive disease
Definition	Liver disease that is caused by injuries to the ENDOTHELIAL CELLS of the vessels and subendothelial EDEMA, but not by THROMBOSIS. Extracellular matrix, rich in FIBRONECTINS, is usually deposited around the HEPATIC VEINS leading to venous outflow occlusion and sinusoidal obstruction.
Synonym	disease liver veno-occlusive disease, hepatic veno-occlusive hepatic veno occlusive dis hepatic veno occlusive disease hepatic veno-occlusive disease (disorder) hepatic veno-occlusive disease [disease/finding] hepatic veno-occlusive diseases hvod liver veno-occlusive disease sinusoidal obstruction syndrome syndrome, sinusoidal obstruction veno occlusive dis hepatic veno occlusive disease, hepatic veno-occlusive disease hepatic veno-occlusive disease of the liver veno-occlusive disease of the liver (disorder) veno-occlusive disease, hepatic venoocclusive liver disease
Orphanet	ORPHANET:890
ICD10	ICD10CM:K76.5
UMLS	C0019156
MeSH	D006504
SNOMED-CT	SNOMEDCT_US_2016_09_01:65617004
Curated Gene	Entrez_id \| Symbol \| Resource(Total Genes:2) CSF3 \| 1440 \| CTD_human OCLN \| 100506658 \| CTD_human
Inferring Gene	Entrez_id \| Symbol \| Resource(Total Genes:4) 2938 \| GSTA1 \| infer 2944 \| GSTM1 \| infer 2950 \| GSTP1 \| infer 2952 \| GSTT1 \| infer
Text Mined Gene	Entrez_id \| Symbol \| Score \| Resource(Total Genes:95) 4706 \| NDUFAB1 \| DISEASES 64132 \| XYLT2 \| DISEASES 933 \| CD22 \| DISEASES 7066 \| THPO \| DISEASES 113278 \| SLC52A3 \| DISEASES 5327 \| PLAT \| DISEASES 5054 \| SERPINE1 \| DISEASES 1440 \| CSF3 \| DISEASES 55907 \| CMAS \| DISEASES 7035 \| TFPI \| DISEASES 51299 \| NRN1 \| DISEASES 2952 \| GSTT1 \| DISEASES 9084 \| VCY \| DISEASES 353513 \| VCY1B \| DISEASES 3431 \| SP110 \| DISEASES 3569 \| IL6 \| DISEASES 23205 \| ACSBG1 \| DISEASES 55729 \| ATF7IP \| DISEASES 7450 \| VWF \| DISEASES 945 \| CD33 \| DISEASES 6403 \| SELP \| DISEASES 440275 \| EIF2AK4 \| DISEASES 84162 \| KIAA1109 \| DISEASES 2028 \| ENPEP \| DISEASES 26277 \| TINF2 \| DISEASES 167410 \| LIX1 \| DISEASES 4613 \| MYCN \| DISEASES 4900 \| NRGN \| DISEASES 22808 \| MRAS \| DISEASES 4681 \| NBL1 \| DISEASES 6781 \| STC1 \| DISEASES 2220 \| FCN2 \| DISEASES 27306 \| HPGDS \| DISEASES 213 \| ALB \| DISEASES 1437 \| CSF2 \| DISEASES 168667 \| BMPER \| DISEASES 90678 \| LRSAM1 \| DISEASES 143501 \| C11orf40 \| DISEASES 2147 \| F2 \| DISEASES 5340 \| PLG \| DISEASES 947 \| CD34 \| DISEASES 2944 \| GSTM1 \| DISEASES 51773 \| RSF1 \| DISEASES 55620 \| STAP2 \| DISEASES 10938 \| EHD1 \| DISEASES 5345 \| SERPINF2 \| DISEASES 55 \| ACPP \| DISEASES 1555 \| CYP2B6 \| DISEASES 7490 \| WT1 \| DISEASES 23545 \| ATP6V0A2 \| DISEASES 3043 \| HBB \| DISEASES 55742 \| PARVA \| DISEASES 2152 \| F3 \| DISEASES 2938 \| GSTA1 \| DISEASES 445329 \| SULT1A4 \| DISEASES 63826 \| SRR \| DISEASES 6818 \| SULT1A3 \| DISEASES 545 \| ATR \| DISEASES 53827 \| FXYD5 \| DISEASES 8654 \| PDE5A \| DISEASES 2224 \| FDPS \| DISEASES 8794 \| TNFRSF10C \| DISEASES 11169 \| WDHD1 \| DISEASES 10724 \| MGEA5 \| DISEASES 4283 \| CXCL9 \| DISEASES 5455 \| POU3F3 \| DISEASES 1378 \| CR1 \| DISEASES 1380 \| CR2 \| DISEASES 462 \| SERPINC1 \| DISEASES 117145 \| THEM4 \| DISEASES 537 \| ATP6AP1 \| DISEASES 11146 \| GLMN \| DISEASES 11093 \| ADAMTS13 \| DISEASES 9682 \| KDM4A \| DISEASES 2155 \| F7 \| DISEASES 7056 \| THBD \| DISEASES 25820 \| ARIH1 \| DISEASES 54790 \| TET2 \| DISEASES 3704 \| ITPA \| DISEASES 3030 \| HADHA \| DISEASES 83650 \| SLC35G5 \| DISEASES 348801 \| LNP1 \| DISEASES 196410 \| METTL7B \| DISEASES 2950 \| GSTP1 \| DISEASES 8091 \| HMGA2 \| DISEASES 55120 \| FANCL \| DISEASES 644150 \| WIPF3 \| DISEASES 279 \| AMY2A \| DISEASES 7124 \| TNF \| DISEASES 3109 \| HLA-DMB \| DISEASES 3106 \| HLA-B \| DISEASES 196527 \| ANO6 \| DISEASES 133396 \| IL31RA \| DISEASES 2801 \| GOLGA2 \| DISEASES 567 \| B2M \| DISEASES
Locus	(Waiting for update.)

Disease ID	252
Disease	hepatic veno-occlusive disease
Integrated Phenotype	HPO \| Name(Total Integrated Phenotypes:12) HP:0002240 \| Hepatomegaly HP:0003573 \| Increased total bilirubin HP:0002910 \| Elevated hepatic transaminases HP:0002480 \| Hepatic encephalopathy HP:0003645 \| Prolonged partial thromboplastin time HP:0002878 \| Respiratory failure HP:0000952 \| Jaundice HP:0001928 \| Abnormality of coagulation HP:0001541 \| Ascites HP:0000083 \| Renal insufficiency HP:0002027 \| Abdominal pain HP:0004324 \| Increased body weight
Text Mined Phenotype	HPO \| Name \| Sentences' Count(Total Phenotypes:1) HP:0002721 \| Immunodeficiency \| 2

Disease ID	252
Disease	hepatic veno-occlusive disease
Manually Symptom	UMLS \| Name(Total Manually Symptoms:1) C0580174 \| portal hypertensive gastropathy
Text Mined Symptom	(Waiting for update.)

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

All Snps(Total Genotypes:1)
snpId	pubmedId	geneId	geneSymbol	diseaseId	sourceId	sentence	score	Year	geneSymbol_dbSNP	CHROMOSOME	POS	REF	ALT
rs1800562	15834437	3077	HFE	umls:C0019156	BeFree	We conclude that HFE C282Y is a risk factor for HVOD and that CPS polymorphisms may counteract its adverse effects.	0.002638474	2005	HFE	6	26092913	G	A

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

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

Mapped by lexical matching(Total Items:0)
(Waiting for update.)

Mapped by homologous gene(Total Items:0)
(Waiting for update.)

Chemical(Total Drugs:20)
CUI	ChemicalName	ChemicalID	CasRN	DiseaseName	DiseaseID	DirectEvidence	PubMedIDs
C0019156	amifostine	D004999	20537-88-6	hepatic veno-occlusive disease	MESH:D006504	therapeutic	11208352
C0019156	busulfan	D002066	55-98-1	hepatic veno-occlusive disease	MESH:D006504	marker/mechanism	10523796
C0019156	carmustine	D002330	154-93-8	hepatic veno-occlusive disease	MESH:D006504	marker/mechanism	2189592
C0019156	ciprofloxacin	D002939	85721-33-1	hepatic veno-occlusive disease	MESH:D006504	marker/mechanism	8832011
C0019156	cyclophosphamide	D003520	50-18-0	hepatic veno-occlusive disease	MESH:D006504	marker/mechanism	10083875
C0019156	cyclosporine	D016572	59865-13-3	hepatic veno-occlusive disease	MESH:D006504	marker/mechanism	3538535
C0019156	defibrotide	C036901	83712-60-1	hepatic veno-occlusive disease	MESH:D006504	therapeutic	18696182
C0019156	everolimus	D000068338	-	hepatic veno-occlusive disease	MESH:D006504	marker/mechanism	19135948
C0019156	floxuridine	D005467	50-91-9	hepatic veno-occlusive disease	MESH:D006504	marker/mechanism	2531719
C0019156	melphalan	D008558	148-82-3	hepatic veno-occlusive disease	MESH:D006504	marker/mechanism	10534061
C0019156	methotrexate	D008727	1959/5/2	hepatic veno-occlusive disease	MESH:D006504	marker/mechanism	1586725
C0019156	mitomycin	D016685	1950/7/7	hepatic veno-occlusive disease	MESH:D006504	marker/mechanism	2829807
C0019156	oxaliplatin	C030110	-	hepatic veno-occlusive disease	MESH:D006504	marker/mechanism	19387321
C0019156	sirolimus	D020123	53123-88-9	hepatic veno-occlusive disease	MESH:D006504	marker/mechanism	18776081
C0019156	tacrolimus	D016559	109581-93-3	hepatic veno-occlusive disease	MESH:D006504	marker/mechanism	17697264
C0019156	thiotepa	D013852	52-24-4	hepatic veno-occlusive disease	MESH:D006504	marker/mechanism	10534061
C0019156	vancomycin	D014640	1404-90-6	hepatic veno-occlusive disease	MESH:D006504	marker/mechanism	8832011
C0019156	vinblastine	D014747	865-21-4	hepatic veno-occlusive disease	MESH:D006504	marker/mechanism	15591910
C0019156	vincristine	D014750	-	hepatic veno-occlusive disease	MESH:D006504	marker/mechanism	11194540
C0019156	vincristine	D014750	-	hepatic veno-occlusive disease	MESH:D006504	therapeutic	15981233

FDA approved drug and dosage information(Total Drugs:9)
DiseaseID	Drug_name	active_ingredients	strength	Dosage Form/Route	Marketing Status	TE code	RLD	RS
MESH:D006504	busulfex	busulfan	6MG/ML	INJECTABLE;INJECTION	Prescription	AP	Yes	Yes
MESH:D006504	cipro	ciprofloxacin	400MG/40ML (10MG/ML)	INJECTABLE;INJECTION	Discontinued	None	Yes	No
MESH:D006504	cipro	ciprofloxacin	250MG/5ML	FOR SUSPENSION;ORAL	Prescription	AB	Yes	No
MESH:D006504	rapamune	sirolimus	1MG/ML	SOLUTION;ORAL	Prescription	None	Yes	Yes
MESH:D006504	rapamune	sirolimus	1MG	TABLET;ORAL	Prescription	AB	Yes	No
MESH:D006504	eloxatin	oxaliplatin	50MG/VIAL Federal Register determination that product was not discontinued or withdrawn for safety or efficacy reasons	INJECTABLE;IV (INFUSION)	Discontinued	None	Yes	No
MESH:D006504	eloxatin	oxaliplatin	50MG/10ML (5MG/ML)	INJECTABLE;IV (INFUSION)	Prescription	AP	Yes	Yes
MESH:D006504	oxaliplatin	oxaliplatin	50MG/10ML (5MG/ML)	INJECTABLE;IV (INFUSION)	Prescription	AP	Yes	Yes
MESH:D006504	afinitor	everolimus	5MG	TABLET;ORAL	Prescription	None	Yes	No

FDA labeling changes(Total Drugs:9)
DiseaseID	Pediatric_Labeling_Date	Trade_Name	Generic_Name_or_Proper_Name	Indications Studied	Label Changes Summary	Product Labeling	BPCA(B)	PREA(P)	BPCA(B) and PREA(P)	Pediatric Rule (R)	Sponsor	Pediatric Exclusivity Granted Date	NNPS
MESH:D006504	01/13/2003	busulfex	busulfan	Part of a conditioning regimen administered prior to hematopoietic progenitor cell transplantation for a variety of malignant hematologic or non-malignant diseases	The population pharmacokinetic estimates of busulfan for clearance and volume of distribution were determined in an open-label, uncontrolled PK study in 24 pediatric patients 5 months to 16 years who received busulfan as part of a conditioning regimen administered prior to hematopoietic progenitor cell transplantation for a variety of malignant hematologic or non-malignant diseases Suggested dosing regimen	Labeling	B	-	-	-	Orphan Medical	12/3/2002	FALSE'
MESH:D006504	03/25/2004	cipro	ciprofloxacin	Complicated UTI and pyelonephritis	Indicated for the treatment of complicated urinary tract infections (cUTIs) and pyelonephritis in pediatric patients 1 17 years of age Not drug of first choice due to increased adverse events compared to controls including events related to joints and/or surrounding tissues Information on PK and dose in pediatric patients 1 17 years of age The most frequent adverse events observed within 6 weeks of treatment initiation during the cUTI clinical trial were gastrointestinal 15% compared to 9% and musculoskeletal 9.3% compared to 6% in ciprofloxacin-treated compared to control-treated patients, respectively	Labeling	B	-	-	-	Bayer	12/18/2003	FALSE'
MESH:D006504	03/25/2004	cipro	ciprofloxacin	Complicated UTI and pyelonephritis	Indicated for the treatment of complicated urinary tract infections (cUTIs) and pyelonephritis in pediatric patients 1 17 years of age Not drug of first choice due to increased adverse events compared to controls including events related to joints and/or surrounding tissues Information on PK and dose in pediatric patients 1 17 years of age The most frequent adverse events observed within 6 weeks of treatment initiation during the cUTI clinical trial were gastrointestinal 15% compared to 9% and musculoskeletal 9.3% compared to 6% in ciprofloxacin-treated compared to control-treated patients, respectively	Labeling	B	-	-	-	Bayer	12/18/2003	FALSE'
MESH:D006504	11/3/2005	rapamune	sirolimus	Prophylaxis of organ rejection in patients undergoing renal transplants	Safety and efficacy established in children 13 years or older judged to be at low to moderate immunologic risk Safety was assessed in a controlled clinical trial in pediatric (	Labeling	B	-	-	-	Wyeth	11/17/2004	FALSE'
MESH:D006504	11/3/2005	rapamune	sirolimus	Prophylaxis of organ rejection in patients undergoing renal transplants	Safety and efficacy established in children 13 years or older judged to be at low to moderate immunologic risk Safety was assessed in a controlled clinical trial in pediatric (	Labeling	B	-	-	-	Wyeth	11/17/2004	FALSE'
MESH:D006504	10/1/2007	eloxatin	oxaliplatin	Solid tumors	The effectiveness of oxaliplatin in children has not been established No significant activity observed in 2 Phase I and 2 Phase II trials in 159 patients ages 7 months to 22 years with solid tumors Information on clinical studies and AEs	Labeling	B	-	-	-	Sanofi-Aventis	09/27/2006	FALSE'
MESH:D006504	10/1/2007	eloxatin	oxaliplatin	Solid tumors	The effectiveness of oxaliplatin in children has not been established No significant activity observed in 2 Phase I and 2 Phase II trials in 159 patients ages 7 months to 22 years with solid tumors Information on clinical studies and AEs	Labeling	B	-	-	-	Sanofi-Aventis	09/27/2006	FALSE'
MESH:D006504	10/1/2007	eloxatin	oxaliplatin	Solid tumors	The effectiveness of oxaliplatin in children has not been established No significant activity observed in 2 Phase I and 2 Phase II trials in 159 patients ages 7 months to 22 years with solid tumors Information on clinical studies and AEs	Labeling	B	-	-	-	Sanofi-Aventis	09/27/2006	FALSE'
MESH:D006504	10/29/2010	afinitor	everolimus	Treatment of patients with subependymal giant cell astrocytoma (SEGA) associated with tuberous sclerosis	Approved for treatment of patients with SEGA associated with TS An open-label, single-arm safety and efficacy trial was conducted in 28 patients 3-34 years with SEGA associated with TS Afinitor has not been studied in patients with SEGA < 3 years of ageMost common adverse reactions (incidence e30%) were stomatitis, upper respiratory tract infection, sinusitis, otitis media, and pyrexiaDose reduction and/or treatment interruption may be needed to manage adverse drug reactions Information on starting dose, therapeutic drug monitoring, clinical trial, and adverse reactions New indication	Labeling	B	-	-	-	Novartis	-	FALSE'