MCID: HYP009
MIFTS: 45

Hypertrophic Pyloric Stenosis

Categories: Gastrointestinal diseases

Aliases & Classifications for Hypertrophic Pyloric Stenosis

MalaCards integrated aliases for Hypertrophic Pyloric Stenosis:

Name: Hypertrophic Pyloric Stenosis 12 15
Pyloric Stenosis, Hypertrophic 44 73
Congenital or Infantile Stricture of Pylorus 12
Congenital Hypertrophic Pyloric Stenosis 12

Classifications:



External Ids:

Disease Ontology 12 DOID:12638
ICD10 33 Q40.0
ICD9CM 35 750.5
MeSH 44 D046248
NCIt 50 C98952
SNOMED-CT 68 48644003
UMLS 73 C0700639

Summaries for Hypertrophic Pyloric Stenosis

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 hirschsprung disease 1 and achalasia. 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, smooth muscle and heart, and related phenotypes are behavior/neurological and cardiovascular system

Related Diseases for Hypertrophic Pyloric Stenosis

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 79)
# Related Disease Score Top Affiliating Genes
1 hirschsprung disease 1 29.9 NOS1 NTRK3 RET
2 achalasia 29.8 NOS1 RET
3 pyloric stenosis 25.4 DMD FOXF1 MBNL1 NKX2-5 NOS1 NTF4
4 acquired gastric outlet stenosis 12.0
5 esophagitis 10.3
6 tracheoesophageal fistula with or without esophageal atresia 10.3
7 aging 10.3
8 esophageal atresia 10.3
9 pylorospasm 10.2
10 neuronitis 10.2
11 tracheoesophageal fistula 10.2
12 gilbert syndrome 10.1
13 ichthyosis, x-linked 10.1
14 mucositis 10.1
15 pertussis 10.1
16 ichthyosis 10.1
17 diaphragmatic hernia, congenital 10.0
18 heart disease 10.0
19 gastroenteritis 10.0
20 adenomyoma 10.0
21 eosinophilic gastroenteritis 10.0
22 pancreatitis 10.0
23 duodenitis 10.0
24 eosinophilic enteropathy 10.0
25 type c thymoma 10.0 NTRK3 SST
26 ectopic cushing syndrome 10.0 RET SST
27 phaeochromocytoma 9.9 RET SST
28 gastroesophageal reflux 9.9
29 hernia, hiatus 9.9
30 osteoglophonic dysplasia 9.9
31 down syndrome 9.9
32 cystic fibrosis 9.9
33 glaucoma 3, primary congenital, a 9.9
34 jejunal atresia 9.9
35 myasthenia gravis 9.9
36 phenylketonuria 9.9
37 insulin-like growth factor i 9.9
38 sudden infant death syndrome 9.9
39 muscle hypertrophy 9.9
40 corpus callosum, agenesis of, with facial anomalies and cerebellar ataxia 9.9
41 pulmonary hypertension 9.9
42 bartter disease 9.9
43 hepatitis 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

Symptoms & Phenotypes for Hypertrophic Pyloric Stenosis

MGI Mouse Phenotypes related to Hypertrophic Pyloric Stenosis:

46
# 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 DMD MBNL1 NKX2-5 NOS1 NTF4 NTRK3
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 MBNL1 NKX2-5 NOS1 RET TRPC6 DMD
8 no phenotypic analysis MP:0003012 9.7 DMD NKX2-5 NOS1 NTRK3 RET SST
9 normal MP:0002873 9.43 NKX2-5 NOS1 NTRK3 RET SST DMD
10 respiratory system MP:0005388 9.1 DMD FOXF1 NKX2-5 NOS1 NTRK3 RET

Drugs & Therapeutics for Hypertrophic Pyloric Stenosis

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 2180-92-9, 38396-39-3 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 329-65-7 838
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

Genetic Tests for Hypertrophic Pyloric Stenosis

Anatomical Context for Hypertrophic Pyloric Stenosis

MalaCards organs/tissues related to Hypertrophic Pyloric Stenosis:

41
Pancreas, Smooth Muscle, Heart, Testes, Endothelial, Cervix, Liver

Publications for Hypertrophic Pyloric Stenosis

Articles related to Hypertrophic Pyloric Stenosis:

(show top 50) (show all 573)
# Title Authors Year
1
Perioperative Complications of Surgery for Hypertrophic Pyloric Stenosis. ( 29510427 )
2018
2
The incidence of infantile hypertrophic pyloric stenosis and its association with folic acid supplementation during pregnancy: A nested case-control study. ( 29884554 )
2018
3
Infantile hypertrophic pyloric stenosis: a 4-year experience from two tertiary care centres in Cameroon. ( 29338765 )
2018
4
Adult idiopathic hypertrophic pyloric stenosis - a common presentation with an uncommon diagnosis. ( 29686790 )
2018
5
Laparoscopic pyloromyotomy for hypertrophic pyloric stenosis: a survey of 407 children. ( 29411105 )
2018
6
Case of hepatic portal venous gas in an infant with hypertrophic pyloric stenosis. ( 29980514 )
2018
7
Postoperative outcomes of open versus laparoscopic pyloromyotomy for hypertrophic pyloric stenosis. ( 29506847 )
2018
8
Transient neonatal myasthenia gravis with infantile hypertrophic pyloric stenosis: coincidence or causation? ( 29099348 )
2017
9
Epidemiological and clinical characteristics of 304 patients with infantile hypertrophic pyloric stenosis in Anhui Province of East China, 2012-2015. ( 28701060 )
2017
10
Atropine Treatment for Hypertrophic Pyloric Stenosis: A Systematic Review and Meta-analysis. ( 28701002 )
2017
11
50 Years Ago in The Journal of Pediatrics: The Radiographic Diagnosis of Congenital Hypertrophic Pyloric Stenosis. ( 28648281 )
2017
12
Caterpillar Sign in Infantile Hypertrophic Pyloric Stenosis. ( 29236645 )
2017
13
Excessive Bright Echoes Sign for Hypertrophic Pyloric Stenosis Suggest the Diagnosis: Gastric Pneumatosis and Portal Venous Gas in Infants Suggest HPS. ( 28295432 )
2017
14
Corrected to uncorrected? The metabolic conundrum of hypertrophic pyloric stenosis. ( 28189444 )
2017
15
Population-level surgical outcomes for infantile hypertrophic pyloric stenosis. ( 28576429 )
2017
16
Perinatal risk factors for infantile hypertrophic pyloric stenosis: A meta-analysis. ( 28318599 )
2017
17
Application of color Doppler ultrasound combined with Doppler imaging artifacts in the diagnosis and estimate of congenital hypertrophic pyloric stenosis. ( 28842652 )
2017
18
A Difficult Recurrent Hypertrophic Pyloric Stenosis. ( 28770145 )
2017
19
Does metabolic alkalosis influence cerebral oxygenation in infantile hypertrophic pyloric stenosis? ( 28550912 )
2017
20
Infantile hypertrophic pyloric stenosis with unusual presentations in Sagamu, Nigeria: a case report and review of the literature. ( 27642452 )
2016
21
Adult-Onset Idiopathic Hypertrophic Pyloric Stenosis Associated With Osteoglophonic Dysplasia and HIV: Case Report and Review of Literature. ( 27599310 )
2016
22
Efficacy of Medical Treatment for Infantile Hypertrophic Pyloric Stenosis: AA Meta-analysis. ( 27215474 )
2016
23
Hypertrophic pyloric stenosis in the Maritimes: examining the waves of change over time. ( 27669400 )
2016
24
Gastric adenomyoma clinically simulating hypertrophic pyloric stenosis. ( 26837724 )
2016
25
Operative outcomes of infantile hypertrophic pyloric stenosis in patients with congenital heart disease. ( 27528563 )
2016
26
Optimizing fluid resuscitation in hypertrophic pyloric stenosis. ( 26876090 )
2016
27
Late-onset hypertrophic pyloric stenosis with gastric outlet obstruction: case report and review of the literature. ( 27506212 )
2016
28
Adult idiopathic hypertrophic pyloric stenosis. ( 27362602 )
2016
29
Health disparities in infants with hypertrophic pyloric stenosis. ( 27586849 )
2016
30
Laparoscopic pyloromyotomy decreases postoperative length of stay in children with hypertrophic pyloric stenosis. ( 27292596 )
2016
31
Post-natal erythromycin exposure and risk of infantile hypertrophic pyloric stenosis: a systematic review and meta-analysis. ( 27655365 )
2016
32
Is there any correlation between radiologic findings and eradication of symptoms after pyloromyotomy in hypertrophic pyloric stenosis? ( 27251656 )
2016
33
A randomized trial to assess advancement of enteral feedings following surgery for hypertrophic pyloric stenosis. ( 27829521 )
2016
34
Formula-feeding and hypertrophic pyloric stenosis: is there an association? A case-control study. ( 26944185 )
2016
35
Asymmetric Hypertrophic Pyloric Stenosis with Concurrent Pancreatic Rest Presenting as Gastric Outlet Obstruction. ( 27179550 )
2016
36
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
37
Recent changes in the features of hypertrophic pyloric stenosis. ( 26615824 )
2016
38
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
39
The cervix sign and other sonographic signs of hypertrophic pyloric stenosis. ( 27315074 )
2016
40
Treating idiopathic hypertrophic pyloric stenosis with sequential therapy: A clinical study. ( 27439633 )
2016
41
Successful combination of endoscopic pyloromyotomy and balloon dilatation for hypertrophic pyloric stenosis in an older child: A novel procedure. ( 27915443 )
2016
42
Ultrasound-guided paravertebral block for pyloromyotomy in 3 neonates with congenital hypertrophic pyloric stenosis. ( 26123148 )
2015
43
Infantile Hypertrophic Pyloric Stenosis in Postoperative Esophageal Atresia with Tracheoesophageal Fistula. ( 26290814 )
2015
44
Spontaneous Intestinal Perforation and Infantile Hypertrophic Pyloric Stenosis in a Preterm Neonate: A Linked Etiopathogenesis? ( 26055488 )
2015
45
Association between NKX2-5 rs29784 and infantile hypertrophic pyloric stenosis in Chinese Han population. ( 25932253 )
2015
46
Is There a Relation Between Pyloric Muscle Thickness and Clinical and Laboratory Data in Infants with Hypertrophic Pyloric Stenosis? ( 27011465 )
2015
47
Association of prematurity with the development of infantile hypertrophic pyloric stenosis. ( 25950452 )
2015
48
Maternal Factors in the Origin of Infantile Hypertrophic Pyloric Stenosis - a Population-based Case-control Study. ( 26394719 )
2015
49
Infantile hypertrophic pyloric stenosis at a tertiary care hospital in Tanzania: a surgical experience with 102 patients over a 5-year period. ( 26581339 )
2015
50
Epidermolysis Bullosa with Hypertrophic Pyloric Stenosis in a Newborn. ( 26500857 )
2015

Variations for Hypertrophic Pyloric Stenosis

Expression for Hypertrophic Pyloric Stenosis

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

Pathways for Hypertrophic Pyloric Stenosis

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

# Super pathways Score Top Affiliating Genes
1
Show member pathways
11.88 NKX2-5 NTF4 NTRK3
2 11.46 NOS1 NTRK3 RET TRPC6
3 10.55 TRPC5 TRPC6
4 10.23 DMD NOS1

GO Terms for Hypertrophic Pyloric Stenosis

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 9.46 DMD RET SST TRPC5
2 axon GO:0030424 9.26 DMD NTF4 NTRK3 RET
3 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 19)
# Name GO ID Score Top Affiliating Genes
1 cell differentiation GO:0030154 9.88 DMD FOXF1 NKX2-5 NTRK3 RET
2 positive regulation of transcription, DNA-templated GO:0045893 9.85 FOXF1 NKX2-5 NOS1 RET
3 nervous system development GO:0007399 9.81 MBNL1 NTRK3 RET TRPC5
4 positive regulation of cell proliferation GO:0008284 9.8 DMD NKX2-5 NTRK3 TRPC5
5 heart development GO:0007507 9.74 FOXF1 NKX2-5 NTRK3
6 positive regulation of cell migration GO:0030335 9.71 FOXF1 NTRK3 RET
7 response to heat GO:0009408 9.58 NOS1 SST
8 cardiac muscle contraction GO:0060048 9.58 DMD NKX2-5
9 transmembrane receptor protein tyrosine kinase signaling pathway GO:0007169 9.58 NTF4 NTRK3 RET
10 regulation of cytosolic calcium ion concentration GO:0051480 9.56 TRPC5 TRPC6
11 positive regulation of peptidyl-threonine phosphorylation GO:0010800 9.52 TRPC5 TRPC6
12 regulation of cardiac muscle contraction GO:0055117 9.46 NKX2-5 NOS1
13 innervation GO:0060384 9.43 NTF4 RET
14 regulation of ryanodine-sensitive calcium-release channel activity GO:0060314 9.37 DMD NOS1
15 manganese ion transport GO:0006828 9.32 TRPC5 TRPC6
16 negative regulation of dendrite morphogenesis GO:0050774 9.26 TRPC5 TRPC6
17 neuron differentiation GO:0030182 9.26 DMD RET TRPC5 TRPC6
18 mechanoreceptor differentiation GO:0042490 9.16 NTF4 NTRK3
19 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

Sources for Hypertrophic Pyloric Stenosis

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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