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 pyloric stenosis and achalasia. An important gene associated with Hypertrophic Pyloric Stenosis is NOS1 (Nitric Oxide Synthase 1), and among its related pathways/superpathways are DREAM Repression and Dynorphin Expression and TRP channels. The drugs Azithromycin and tannic acid have been mentioned in the context of this disorder. Affiliated tissues include kidney, liver 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 83)
# Related Disease Score Top Affiliating Genes
1 pyloric stenosis 30.5 DMD FOXF1 HMOX2 MBNL1 NKX2-5 NOS1
2 achalasia 30.5 NOS1 RET
3 hirschsprung disease 1 29.9 HMOX2 NOS1 RET
4 acquired gastric outlet stenosis 12.2
5 tracheoesophageal fistula with or without esophageal atresia 10.5
6 esophageal atresia 10.5
7 pylorospasm 10.4
8 tracheoesophageal fistula 10.4
9 gilbert syndrome 10.2
10 ichthyosis, x-linked 10.2
11 gastric ulcer 10.2
12 pertussis 10.2
13 ichthyosis 10.2
14 diaphragmatic hernia, congenital 10.2
15 pulmonary hypertension 10.2
16 heart disease 10.2
17 gastroenteritis 10.2
18 adenomyoma 10.2
19 eosinophilic gastroenteritis 10.2
20 intestinal obstruction 10.2
21 eosinophilic enteropathy 10.2
22 ectopic cushing syndrome 10.0 RET SST
23 interatrial communication 10.0 DMD NKX2-5
24 endocrine organ benign neoplasm 10.0 RET SST
25 muscular dystrophy, becker type 10.0 DMD NOS1
26 gastroesophageal reflux 10.0
27 hernia, hiatus 10.0
28 intussusception 10.0
29 visceral myopathy 10.0
30 osteoglophonic dysplasia 10.0
31 down syndrome 10.0
32 achalasia, familial esophageal 10.0
33 cystic fibrosis 10.0
34 glaucoma 3, primary congenital, a 10.0
35 jejunal atresia 10.0
36 myasthenia gravis 10.0
37 phenylketonuria 10.0
38 insulin-like growth factor i 10.0
39 sudden infant death syndrome 10.0
40 bile duct cysts 10.0
41 muscle hypertrophy 10.0
42 corpus callosum, agenesis of, with facial anomalies and cerebellar ataxia 10.0
43 crohn's disease 10.0
44 muscle disorders 10.0
45 congenital hypothyroidism 10.0
46 esophageal atresia/tracheoesophageal fistula 10.0
47 polycystic kidney disease 10.0
48 hydronephrosis 10.0
49 esophagitis 10.0
50 nephrocalcinosis 10.0

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 10.11 DMD HMOX2 MBNL1 NOS1 NTF4 RET
2 cardiovascular system MP:0005385 10.03 DMD FOXF1 HMOX2 MBNL1 NKX2-5 NOS1
3 homeostasis/metabolism MP:0005376 9.97 DMD HMOX2 NKX2-5 NOS1 NTF4 RET
4 mortality/aging MP:0010768 9.97 DMD FOXF1 HMOX2 MBNL1 NKX2-5 NOS1
5 digestive/alimentary MP:0005381 9.95 DMD FOXF1 HMOX2 NKX2-5 NOS1 RET
6 muscle MP:0005369 9.86 DMD FOXF1 HMOX2 MBNL1 NKX2-5 NOS1
7 nervous system MP:0003631 9.85 DMD HMOX2 MBNL1 NKX2-5 NOS1 NTF4
8 no phenotypic analysis MP:0003012 9.43 DMD NKX2-5 NOS1 RET SST TRPC5
9 respiratory system MP:0005388 9.1 DMD FOXF1 HMOX2 NKX2-5 NOS1 RET

Drugs & Therapeutics for Hypertrophic Pyloric Stenosis

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

(show all 14)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Azithromycin Approved Phase 4 83905-01-5 55185 447043
2 tannic acid Approved Phase 4
3
Benzocaine Approved, Investigational Phase 4 94-09-7, 1994-09-7 2337
4
Sevoflurane Approved, Vet_approved 28523-86-6 5206
5
Propofol Approved, Investigational, Vet_approved 2078-54-8 4943
6
Racepinephrine Approved 329-65-7 838
7
Fentanyl Approved, Illicit, Investigational, Vet_approved 437-38-7 3345
8
Bupivacaine Approved, Investigational 2180-92-9, 38396-39-3 2474
9
Epinephrine Approved, Vet_approved 51-43-4 5816
10
Rocuronium Approved 119302-91-9, 143558-00-3 441290
11 Anesthetics
12 Central Nervous System Depressants
13 Bromides
14 Epinephryl borate

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 MORDOR II Burkina Faso Neonates Not yet recruiting NCT03682653 Phase 4 Azithromycin;Placebo
2 MORDOR II Burkina Faso: Longitudinal Trial Not yet recruiting NCT03676751 Phase 4 Azithromycin;Placebo
3 Laparoscopic Versus Open Pyloromyotomy for Infants With Idiopathic Hypertrophic Pyloric Stenosis Completed NCT00195949 Phase 3
4 The Effect of Hypertrophic Pyloric Stenosis (HPS) on Sodium Intake in Childhood Unknown status NCT01159509
5 Post-operative Outcomes in the Pyloromyotomy Procedure Under Spinal Anesthesia Unknown status NCT02879292
6 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
Kidney, Liver, Heart, Testes, Pancreas, Smooth Muscle, Thyroid

Publications for Hypertrophic Pyloric Stenosis

Articles related to Hypertrophic Pyloric Stenosis:

(show top 50) (show all 865)
# 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
ACTG2-Associated Visceral Myopathy With Chronic Intestinal Pseudoobstruction, Intestinal Malrotation, Hypertrophic Pyloric Stenosis, Choledochal Cyst, and a Novel Missense Mutation. ( 30019982 )
2018
9
Implementation of a Quality Control System for Laparoscopic Pyloromyotomy in Hypertrophic Pyloric Stenosis: Hurdles and Pitfalls. ( 30086575 )
2018
10
Hypertrophic pyloric stenosis following persistent pulmonary hypertension of the newborn: a case report and literature review. ( 30176827 )
2018
11
Hypertrophic pyloric stenosis following repair of oesophageal atresia and tracheo-oesophageal fistula in a neonate. ( 30244227 )
2018
12
Genome-wide meta-analysis identifies BARX1 and EML4-MTA3 as new loci associated with infantile hypertrophic pyloric stenosis. ( 30281099 )
2018
13
The Application of Pyloric chisel in the Treatment of Hypertrophic Pyloric Stenosis by Single-Site Umbilical Laparoscopic Pyloromyotomy. ( 30289351 )
2018
14
Developmental outcome at 3 years of age of infants following surgery for infantile hypertrophic pyloric stenosis. ( 30402682 )
2018
15
Association between exposure to macrolides and the development of infantile hypertrophic pyloric stenosis: a systematic review and meta-analysis. ( 30470884 )
2018
16
Prevalence and descriptive epidemiology of infantile hypertrophic pyloric stenosis in the United States: A multistate, population-based retrospective study, 1999-2010. ( 30549250 )
2018
17
Transient neonatal myasthenia gravis with infantile hypertrophic pyloric stenosis: coincidence or causation? ( 29099348 )
2017
18
Epidemiological and clinical characteristics of 304 patients with infantile hypertrophic pyloric stenosis in Anhui Province of East China, 2012-2015. ( 28701060 )
2017
19
Atropine Treatment for Hypertrophic Pyloric Stenosis: A Systematic Review and Meta-analysis. ( 28701002 )
2017
20
50 Years Ago in The Journal of Pediatrics: The Radiographic Diagnosis of Congenital Hypertrophic Pyloric Stenosis. ( 28648281 )
2017
21
Caterpillar Sign in Infantile Hypertrophic Pyloric Stenosis. ( 29236645 )
2017
22
Excessive Bright Echoes Sign for Hypertrophic Pyloric Stenosis Suggest the Diagnosis: Gastric Pneumatosis and Portal Venous Gas in Infants Suggest HPS. ( 28295432 )
2017
23
Corrected to uncorrected? The metabolic conundrum of hypertrophic pyloric stenosis. ( 28189444 )
2017
24
Population-level surgical outcomes for infantile hypertrophic pyloric stenosis. ( 28576429 )
2017
25
Perinatal risk factors for infantile hypertrophic pyloric stenosis: A meta-analysis. ( 28318599 )
2017
26
Application of color Doppler ultrasound combined with Doppler imaging artifacts in the diagnosis and estimate of congenital hypertrophic pyloric stenosis. ( 28842652 )
2017
27
A Difficult Recurrent Hypertrophic Pyloric Stenosis. ( 28770145 )
2017
28
Does metabolic alkalosis influence cerebral oxygenation in infantile hypertrophic pyloric stenosis? ( 28550912 )
2017
29
Infantile hypertrophic pyloric stenosis with unusual presentations in Sagamu, Nigeria: a case report and review of the literature. ( 27642452 )
2016
30
Adult-Onset Idiopathic Hypertrophic Pyloric Stenosis Associated With Osteoglophonic Dysplasia and HIV: Case Report and Review of Literature. ( 27599310 )
2016
31
Efficacy of Medical Treatment for Infantile Hypertrophic Pyloric Stenosis: AA Meta-analysis. ( 27215474 )
2016
32
Hypertrophic pyloric stenosis in the Maritimes: examining the waves of change over time. ( 27669400 )
2016
33
Gastric adenomyoma clinically simulating hypertrophic pyloric stenosis. ( 26837724 )
2016
34
Operative outcomes of infantile hypertrophic pyloric stenosis in patients with congenital heart disease. ( 27528563 )
2016
35
Optimizing fluid resuscitation in hypertrophic pyloric stenosis. ( 26876090 )
2016
36
Late-onset hypertrophic pyloric stenosis with gastric outlet obstruction: case report and review of the literature. ( 27506212 )
2016
37
Adult idiopathic hypertrophic pyloric stenosis. ( 27362602 )
2016
38
Health disparities in infants with hypertrophic pyloric stenosis. ( 27586849 )
2016
39
Laparoscopic pyloromyotomy decreases postoperative length of stay in children with hypertrophic pyloric stenosis. ( 27292596 )
2016
40
Post-natal erythromycin exposure and risk of infantile hypertrophic pyloric stenosis: a systematic review and meta-analysis. ( 27655365 )
2016
41
Is there any correlation between radiologic findings and eradication of symptoms after pyloromyotomy in hypertrophic pyloric stenosis? ( 27251656 )
2016
42
A randomized trial to assess advancement of enteral feedings following surgery for hypertrophic pyloric stenosis. ( 27829521 )
2016
43
Formula-feeding and hypertrophic pyloric stenosis: is there an association? A case-control study. ( 26944185 )
2016
44
Asymmetric Hypertrophic Pyloric Stenosis with Concurrent Pancreatic Rest Presenting as Gastric Outlet Obstruction. ( 27179550 )
2016
45
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
46
Recent changes in the features of hypertrophic pyloric stenosis. ( 26615824 )
2016
47
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
48
The cervix sign and other sonographic signs of hypertrophic pyloric stenosis. ( 27315074 )
2016
49
Treating idiopathic hypertrophic pyloric stenosis with sequential therapy: A clinical study. ( 27439633 )
2016
50
Successful combination of endoscopic pyloromyotomy and balloon dilatation for hypertrophic pyloric stenosis in an older child: A novel procedure. ( 27915443 )
2016

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
11.79 DMD NKX2-5 NOS1
2 10.35 TRPC5 TRPC6
3 9.9 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 cation channel complex GO:0034703 8.96 TRPC5 TRPC6
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 13)
# Name GO ID Score Top Affiliating Genes
1 positive regulation of transcription, DNA-templated GO:0045893 9.76 FOXF1 NKX2-5 NOS1 RET
2 response to heat GO:0009408 9.52 NOS1 SST
3 cardiac muscle contraction GO:0060048 9.51 DMD NKX2-5
4 regulation of cytosolic calcium ion concentration GO:0051480 9.49 TRPC5 TRPC6
5 regulation of cardiac conduction GO:1903779 9.48 NKX2-5 NOS1
6 positive regulation of peptidyl-threonine phosphorylation GO:0010800 9.43 TRPC5 TRPC6
7 innervation GO:0060384 9.4 NTF4 RET
8 regulation of cardiac muscle contraction GO:0055117 9.37 NKX2-5 NOS1
9 regulation of ryanodine-sensitive calcium-release channel activity GO:0060314 9.32 DMD NOS1
10 manganese ion transport GO:0006828 9.26 TRPC5 TRPC6
11 neuron differentiation GO:0030182 9.26 DMD RET TRPC5 TRPC6
12 negative regulation of dendrite morphogenesis GO:0050774 9.16 TRPC5 TRPC6
13 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 clathrin binding GO:0030276 9.26 TRPC5 TRPC6
2 inositol 1,4,5 trisphosphate binding GO:0070679 9.16 TRPC5 TRPC6
3 actinin binding GO:0042805 8.96 TRPC5 TRPC6
4 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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