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MCID: HYP009
MIFTS: 45

Hypertrophic Pyloric Stenosis

Categories: Gastrointestinal diseases
Genes Tissues Related diseases Publications Pathways Drugs
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Aliases & Classifications for Hypertrophic Pyloric Stenosis

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MalaCards integrated aliases for Hypertrophic Pyloric Stenosis:

Name: Hypertrophic Pyloric Stenosis 12 14
Pyloric Stenosis, Hypertrophic 41 69
Congenital or Infantile Stricture of Pylorus 12
Congenital Hypertrophic Pyloric Stenosis 12

Classifications:

MalaCards categories:
Anatomical: Gastrointestinal diseases
See all MalaCards categories (disease lists)
ICD10: 32


External Ids:

Disease Ontology 12 DOID:12638
ICD10 32 Q40.0
ICD9CM 34 750.5
MeSH 41 D046248
NCIt 46 C98952
SNOMED-CT 64 48644003
UMLS 69 C0700639
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Summaries for Hypertrophic Pyloric Stenosis

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Disease Ontology : 12 A pyloric stenosis characterized by the enlargement of the muscle surrounding the pylorus, causing severe projectile non-bilious vomiting.

MalaCards based summary : Hypertrophic Pyloric Stenosis, also known as pyloric stenosis, hypertrophic, is related to achalasia and hirschsprung disease 1. An important gene associated with Hypertrophic Pyloric Stenosis is NOS1 (Nitric Oxide Synthase 1), and among its related pathways/superpathways are Human Embryonic Stem Cell Pluripotency and Neuroscience. The drugs Bupivacaine and Epinephrine have been mentioned in the context of this disorder. Affiliated tissues include pancreas, breast and smooth muscle, and related phenotypes are behavior/neurological and cardiovascular system

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Related Diseases for Hypertrophic Pyloric Stenosis

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Diseases in the Hypertrophic Pyloric Stenosis family:

Pyloric Stenosis, Infantile Hypertrophic, 1 Pyloric Stenosis, Infantile Hypertrophic, 4
Pyloric Stenosis, Infantile Hypertrophic, 2 Pyloric Stenosis, Infantile Hypertrophic, 3
Pyloric Stenosis, Infantile Hypertrophic, 5

Diseases related to Hypertrophic Pyloric Stenosis via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 75)
# Related Disease Score Top Affiliating Genes
1 achalasia 30.1 NOS1 RET
2 hirschsprung disease 1 29.8 NOS1 NTRK3 RET
3 pyloric stenosis 28.0 DMD FOXF1 MBNL1 NKX2-5 NOS1 NTF4
4 acquired gastric outlet stenosis 12.0
5 esophagitis 10.3
6 pyloric stenosis, infantile hypertrophic, 1 10.3
7 tracheoesophageal fistula with or without esophageal atresia 10.3
8 aging 10.3
9 esophageal atresia 10.3
10 pylorospasm 10.2
11 neuronitis 10.2
12 streptococcal group a invasive disease 10.2
13 tracheoesophageal fistula 10.2
14 gilbert syndrome 10.1
15 ichthyosis, x-linked 10.1
16 mucositis 10.1
17 pertussis 10.1
18 ichthyosis 10.1
19 diaphragmatic hernia, congenital 10.0
20 heart disease 10.0
21 gastroenteritis 10.0
22 adenomyoma 10.0
23 eosinophilic gastroenteritis 10.0
24 pancreatitis 10.0
25 duodenitis 10.0
26 type c thymoma 10.0 NTRK3 SST
27 ectopic cushing syndrome 10.0 RET SST
28 phaeochromocytoma 9.9 RET SST
29 gastroesophageal reflux 9.9
30 fibrosis of extraocular muscles, congenital, 1 9.9
31 osteoglophonic dysplasia 9.9
32 down syndrome 9.9
33 citrullinemia, classic 9.9
34 cystic fibrosis 9.9
35 jejunal atresia 9.9
36 myasthenia gravis 9.9
37 phenylketonuria 9.9
38 insulin-like growth factor i 9.9
39 sudden infant death syndrome 9.9
40 pyloric stenosis, infantile hypertrophic, 5 9.9
41 muscle hypertrophy 9.9
42 corpus callosum, agenesis of, with facial anomalies and cerebellar ataxia 9.9
43 pulmonary hypertension 9.9
44 congenital hypothyroidism 9.9
45 esophageal atresia/tracheoesophageal fistula 9.9
46 hydronephrosis 9.9
47 nephrocalcinosis 9.9
48 megaesophagus 9.9
49 neonatal myasthenia gravis 9.9
50 hypothyroidism 9.9

Graphical network of the top 20 diseases related to Hypertrophic Pyloric Stenosis:



Diseases related to Hypertrophic Pyloric Stenosis
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Symptoms & Phenotypes for Hypertrophic Pyloric Stenosis

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MGI Mouse Phenotypes related to Hypertrophic Pyloric Stenosis:

43
# Description MGI Source Accession Score Top Affiliating Genes
1 behavior/neurological MP:0005386 10.13 DMD MBNL1 NOS1 NTF4 NTRK3 RET
2 cardiovascular system MP:0005385 10.06 DMD FOXF1 MBNL1 NKX2-5 NOS1 NTRK3
3 mortality/aging MP:0010768 10.02 DMD FOXF1 MBNL1 NKX2-5 NOS1 NTF4
4 growth/size/body region MP:0005378 10.01 RET SST DMD MBNL1 NKX2-5 NOS1
5 nervous system MP:0003631 9.96 DMD MBNL1 NKX2-5 NOS1 NTF4 NTRK3
6 digestive/alimentary MP:0005381 9.91 DMD FOXF1 NKX2-5 NOS1 RET SST
7 muscle MP:0005369 9.87 DMD FOXF1 MBNL1 NKX2-5 NOS1 RET
8 no phenotypic analysis MP:0003012 9.7 NOS1 NTRK3 RET SST TRPC5 DMD
9 normal MP:0002873 9.43 DMD NKX2-5 NOS1 NTRK3 RET SST
10 respiratory system MP:0005388 9.1 DMD FOXF1 NKX2-5 NOS1 NTRK3 RET
Sources

Drugs & Therapeutics for Hypertrophic Pyloric Stenosis

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Drugs for Hypertrophic Pyloric Stenosis (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 11)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Bupivacaine Approved, Investigational 38396-39-3, 2180-92-9 2474
2
Epinephrine Approved, Vet_approved 51-43-4 5816
3
Fentanyl Approved, Illicit, Investigational, Vet_approved 437-38-7 3345
4
Propofol Approved, Investigational, Vet_approved 2078-54-8 4943
5 Racepinephrine Approved 329-65-7
6
Rocuronium Approved 119302-91-9, 143558-00-3 441290
7
Sevoflurane Approved, Vet_approved 28523-86-6 5206
8 Anesthetics
9 Central Nervous System Depressants
10 Bromides
11 Epinephryl borate

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Laparoscopic Versus Open Pyloromyotomy for Infants With Idiopathic Hypertrophic Pyloric Stenosis Completed NCT00195949 Phase 3
2 The Effect of Hypertrophic Pyloric Stenosis (HPS) on Sodium Intake in Childhood Unknown status NCT01159509
3 Post-operative Outcomes in the Pyloromyotomy Procedure Under Spinal Anesthesia Not yet recruiting NCT02879292
4 Spinal Anesthesia and General Anesthesia for Pyloromyotomy - Surgical Outcomes a Comparison Retrospective Study Not yet recruiting NCT03095144

Search NIH Clinical Center for Hypertrophic Pyloric Stenosis

Cochrane evidence based reviews: pyloric stenosis, hypertrophic

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Genetic Tests for Hypertrophic Pyloric Stenosis

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Anatomical Context for Hypertrophic Pyloric Stenosis

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MalaCards organs/tissues related to Hypertrophic Pyloric Stenosis:

38
Pancreas, Breast, Smooth Muscle, Heart, Testes, Endothelial, Cervix
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Publications for Hypertrophic Pyloric Stenosis

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Articles related to Hypertrophic Pyloric Stenosis:

(show top 50) (show all 568)
# Title Authors Year
1
Laparoscopic pyloromyotomy for hypertrophic pyloric stenosis: a survey of 407 children. ( 29411105 )
2018
2
Infantile hypertrophic pyloric stenosis: a 4-year experience from two tertiary care centres in Cameroon. ( 29338765 )
2018
3
Excessive Bright Echoes Sign for Hypertrophic Pyloric Stenosis Suggest the Diagnosis: Gastric Pneumatosis and Portal Venous Gas in Infants Suggest HPS. ( 28295432 )
2017
4
Application of color Doppler ultrasound combined with Doppler imaging artifacts in the diagnosis and estimate of congenital hypertrophic pyloric stenosis. ( 28842652 )
2017
5
Transient neonatal myasthenia gravis with infantile hypertrophic pyloric stenosis: coincidence or causation? ( 29099348 )
2017
6
Perinatal risk factors for infantile hypertrophic pyloric stenosis: A meta-analysis. ( 28318599 )
2017
7
Population-level surgical outcomes for infantile hypertrophic pyloric stenosis. ( 28576429 )
2017
8
A Difficult Recurrent Hypertrophic Pyloric Stenosis. ( 28770145 )
2017
9
Atropine Treatment for Hypertrophic Pyloric Stenosis: A Systematic Review and Meta-analysis. ( 28701002 )
2017
10
Does metabolic alkalosis influence cerebral oxygenation in infantile hypertrophic pyloric stenosis? ( 28550912 )
2017
11
Corrected to uncorrected? The metabolic conundrum of hypertrophic pyloric stenosis. ( 28189444 )
2017
12
Epidemiological and clinical characteristics of 304 patients with infantile hypertrophic pyloric stenosis in Anhui Province of East China, 2012-2015. ( 28701060 )
2017
13
Caterpillar Sign in Infantile Hypertrophic Pyloric Stenosis. ( 29236645 )
2017
14
50 Years Ago in The Journal of Pediatrics: The Radiographic Diagnosis of Congenital Hypertrophic Pyloric Stenosis. ( 28648281 )
2017
15
Health disparities in infants with hypertrophic pyloric stenosis. ( 27586849 )
2016
16
Ultrasound assessment of the gastric contents for the guidance of the anaesthetic strategy in infants with hypertrophic pyloric stenosis: a prospective cohort study. ( 27106968 )
2016
17
Hypertrophic pyloric stenosis in the Maritimes: examining the waves of change over time. ( 27669400 )
2016
18
Operative outcomes of infantile hypertrophic pyloric stenosis in patients with congenital heart disease. ( 27528563 )
2016
19
Infantile hypertrophic pyloric stenosis with unusual presentations in Sagamu, Nigeria: a case report and review of the literature. ( 27642452 )
2016
20
Efficacy of Medical Treatment for Infantile Hypertrophic Pyloric Stenosis: AA Meta-analysis. ( 27215474 )
2016
21
Recent changes in the features of hypertrophic pyloric stenosis. ( 26615824 )
2016
22
Post-natal erythromycin exposure and risk of infantile hypertrophic pyloric stenosis: a systematic review and meta-analysis. ( 27655365 )
2016
23
The cervix sign and other sonographic signs of hypertrophic pyloric stenosis. ( 27315074 )
2016
24
Is there any correlation between radiologic findings and eradication of symptoms after pyloromyotomy in hypertrophic pyloric stenosis? ( 27251656 )
2016
25
Formula-feeding and hypertrophic pyloric stenosis: is there an association? A case-control study. ( 26944185 )
2016
26
Gastric adenomyoma clinically simulating hypertrophic pyloric stenosis. ( 26837724 )
2016
27
Laparoscopic pyloromyotomy decreases postoperative length of stay in children with hypertrophic pyloric stenosis. ( 27292596 )
2016
28
Successful combination of endoscopic pyloromyotomy and balloon dilatation for hypertrophic pyloric stenosis in an older child: A novel procedure. ( 27915443 )
2016
29
Adult-Onset Idiopathic Hypertrophic Pyloric Stenosis Associated With Osteoglophonic Dysplasia and HIV: Case Report and Review of Literature. ( 27599310 )
2016
30
A randomized trial to assess advancement of enteral feedings following surgery for hypertrophic pyloric stenosis. ( 27829521 )
2016
31
Asymmetric Hypertrophic Pyloric Stenosis with Concurrent Pancreatic Rest Presenting as Gastric Outlet Obstruction. ( 27179550 )
2016
32
Optimizing fluid resuscitation in hypertrophic pyloric stenosis. ( 26876090 )
2016
33
A novel missense mutation in the transcription factor FOXF1 co-segregating with infantile hypertrophic pyloric stenosis in the extended pedigree linked to IHPS5 on chromosome 16q24. ( 27855150 )
2016
34
Treating idiopathic hypertrophic pyloric stenosis with sequential therapy: A clinical study. ( 27439633 )
2016
35
Adult idiopathic hypertrophic pyloric stenosis. ( 27362602 )
2016
36
Late-onset hypertrophic pyloric stenosis with gastric outlet obstruction: case report and review of the literature. ( 27506212 )
2016
37
Infantile Hypertrophic Pyloric Stenosis in Postoperative Esophageal Atresia with Tracheoesophageal Fistula. ( 26290814 )
2015
38
The use of ultrasonography in infantile hypertrophic pyloric stenosis: does the patient's age and weight affect pyloric size and pyloric ratio? ( 25745654 )
2015
39
Spontaneous Intestinal Perforation and Infantile Hypertrophic Pyloric Stenosis in a Preterm Neonate: A Linked Etiopathogenesis? ( 26055488 )
2015
40
Association of prematurity with the development of infantile hypertrophic pyloric stenosis. ( 25950452 )
2015
41
Birth outcomes of male and female patients with infantile hypertrophic pyloric stenosis - a population-based case-control study. ( 26135791 )
2015
42
Ultrasound-guided paravertebral block for pyloromyotomy in 3 neonates with congenital hypertrophic pyloric stenosis. ( 26123148 )
2015
43
Adult idiopathic hypertrophic pyloric stenosis. ( 26154756 )
2015
44
Epidermolysis Bullosa with Hypertrophic Pyloric Stenosis in a Newborn. ( 26500857 )
2015
45
Brain oxygenation during laparoscopic correction of hypertrophic pyloric stenosis. ( 25768674 )
2015
46
Incidence and Concurrent Laparoscopic Repair of Hypertrophic Pyloric Stenosis and Patent Processus Vaginalis. ( 25836622 )
2015
47
Maternal Factors in the Origin of Infantile Hypertrophic Pyloric Stenosis - a Population-based Case-control Study. ( 26394719 )
2015
48
Infantile hypertrophic pyloric stenosis at a tertiary care hospital in Tanzania: a surgical experience with 102 patients over a 5-year period. ( 26581339 )
2015
49
Association between NKX2-5 rs29784 and infantile hypertrophic pyloric stenosis in Chinese Han population. ( 25932253 )
2015
50
Is There a Relation Between Pyloric Muscle Thickness and Clinical and Laboratory Data in Infants with Hypertrophic Pyloric Stenosis? ( 27011465 )
2015
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Variations for Hypertrophic Pyloric Stenosis

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Expression for Hypertrophic Pyloric Stenosis

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Search GEO for disease gene expression data for Hypertrophic Pyloric Stenosis.
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Pathways for Hypertrophic Pyloric Stenosis

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Pathways related to Hypertrophic Pyloric Stenosis according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1
Human Embryonic Stem Cell Pluripotency 60
Show member pathways
 11.88 NKX2-5 NTF4 NTRK3
2
Neuroscience 4
11.46 NOS1 NTRK3 RET TRPC6
3
TRP channels 62
10.55 TRPC5 TRPC6
4
Muscular Dystrophies and Dystrophin-Glycoprotein Complex 60
10.23 DMD NOS1
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GO Terms for Hypertrophic Pyloric Stenosis

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Cellular components related to Hypertrophic Pyloric Stenosis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 neuronal cell body GO:0043025 9.26 DMD RET SST TRPC5
2 membrane raft GO:0045121 8.92 DMD NOS1 RET TRPC5

Biological processes related to Hypertrophic Pyloric Stenosis according to GeneCards Suite gene sharing:

(show all 18)
# Name GO ID Score Top Affiliating Genes
1 positive regulation of transcription, DNA-templated GO:0045893 9.84 FOXF1 NKX2-5 NOS1 RET
2 nervous system development GO:0007399 9.81 MBNL1 NTRK3 RET TRPC5
3 positive regulation of cell proliferation GO:0008284 9.78 DMD NKX2-5 NTRK3 TRPC5
4 heart development GO:0007507 9.73 FOXF1 NKX2-5 NTRK3
5 positive regulation of cell migration GO:0030335 9.7 FOXF1 NTRK3 RET
6 response to heat GO:0009408 9.58 NOS1 SST
7 cardiac muscle contraction GO:0060048 9.57 DMD NKX2-5
8 regulation of cytosolic calcium ion concentration GO:0051480 9.55 TRPC5 TRPC6
9 transmembrane receptor protein tyrosine kinase signaling pathway GO:0007169 9.54 NTF4 NTRK3 RET
10 positive regulation of peptidyl-threonine phosphorylation GO:0010800 9.49 TRPC5 TRPC6
11 regulation of cardiac muscle contraction GO:0055117 9.46 NKX2-5 NOS1
12 innervation GO:0060384 9.43 NTF4 RET
13 regulation of ryanodine-sensitive calcium-release channel activity GO:0060314 9.4 DMD NOS1
14 manganese ion transport GO:0006828 9.32 TRPC5 TRPC6
15 negative regulation of dendrite morphogenesis GO:0050774 9.26 TRPC5 TRPC6
16 neuron differentiation GO:0030182 9.26 DMD RET TRPC5 TRPC6
17 mechanoreceptor differentiation GO:0042490 9.16 NTF4 NTRK3
18 positive regulation of neuron differentiation GO:0045666 8.92 DMD NKX2-5 TRPC5 TRPC6

Molecular functions related to Hypertrophic Pyloric Stenosis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 inositol 1,4,5 trisphosphate binding GO:0070679 9.16 TRPC5 TRPC6
2 actinin binding GO:0042805 8.96 TRPC5 TRPC6
3 store-operated calcium channel activity GO:0015279 8.62 TRPC5 TRPC6
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Sources for Hypertrophic Pyloric Stenosis

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3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 FMA
27 GO
28 GTR
29 HGMD
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 MedGen
41 MeSH
42 MESH via Orphanet
43 MGI
45 NCI
46 NCIt
47 NDF-RT
50 NINDS
51 Novoseek
53 OMIM
54 OMIM via Orphanet
58 PubMed
60 QIAGEN
65 SNOMED-CT via HPO
66 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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