MCID: HYP009
MIFTS: 42

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
Congenital Hypertrophic Pyloric Stenosis 12 32
Pyloric Stenosis, Hypertrophic 43 71
Congenital or Infantile Stricture of Pylorus 12

Classifications:



External Ids:

Disease Ontology 12 DOID:12638
ICD9CM 34 750.5
MeSH 43 D046248
NCIt 49 C98952
SNOMED-CT 67 48644003
ICD10 32 Q40.0
UMLS 71 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 congenital hypertrophic pyloric stenosis, is related to achalasia and intestinal obstruction. An important gene associated with Hypertrophic Pyloric Stenosis is NOS1 (Nitric Oxide Synthase 1), and among its related pathways/superpathways are Neuroscience and Netrin-1 signaling. The drugs Azithromycin and Fentanyl have been mentioned in the context of this disorder. Affiliated tissues include heart, thyroid and pancreas, 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 198)
# Related Disease Score Top Affiliating Genes
1 achalasia 30.9 RET NOS1 GAST
2 intestinal obstruction 30.6 SST RET MLN
3 hernia, hiatus 30.1 GAST FOXF1 BARX1
4 gastroparesis 30.0 SST NOS1 MLN GAST
5 hirschsprung disease 1 29.9 RET NTRK3 NTRK1 NOS1
6 constipation 29.7 SST RET NTRK3 NOS1 MLN GAST
7 pyloric stenosis 27.4 TRPC6 TRPC5 TRPC1 SST RET NTRK1
8 acquired gastric outlet stenosis 12.4
9 pyloric stenosis, infantile hypertrophic, 1 12.0
10 esophageal atresia 10.6
11 pylorospasm 10.6
12 muscle hypertrophy 10.6
13 gastroesophageal reflux 10.5
14 gastritis 10.5
15 tracheoesophageal fistula with or without esophageal atresia 10.4
16 gastric ulcer 10.4
17 pertussis 10.4
18 gastroenteritis 10.4
19 postcholecystectomy syndrome 10.4 MLN GAST
20 gilbert syndrome 10.3
21 inguinal hernia 10.3
22 bilirubin metabolic disorder 10.3
23 eosinophilic gastroenteritis 10.3
24 rectum neuroendocrine neoplasm 10.3 SST GAST
25 gastrointestinal neuroendocrine benign tumor 10.3 SST GAST
26 non-functioning pancreatic endocrine tumor 10.3 SST GAST
27 gastritis, familial giant hypertrophic 10.3 GAST BARX1
28 gastric neuroendocrine neoplasm 10.3 SST GAST
29 rare tumor 10.3 SST RET
30 duodenal gastrinoma 10.3 SST GAST
31 pancreatic cholera 10.3 SST GAST
32 functional colonic disease 10.3 SST MLN
33 glucagonoma 10.3 SST GAST
34 pancreatic gastrinoma 10.3 SST GAST
35 volvulus of midgut 10.3
36 multinucleated neurons, anhydramnios, renal dysplasia, cerebellar hypoplasia, and hydranencephaly 10.3
37 sudden infant death syndrome 10.3
38 ichthyosis, x-linked 10.3
39 esophagitis 10.3
40 ichthyosis 10.3
41 myopathy 10.3
42 peptic ulcer disease 10.3
43 47,xyy 10.3
44 gastrointestinal neuroendocrine tumor 10.3 SST GAST
45 ectopic cushing syndrome 10.3 SST RET
46 carcinoid tumors, intestinal 10.3 SST GAST
47 gastric antral vascular ectasia 10.2 SST GAST
48 colonic pseudo-obstruction 10.2 SST MLN
49 paroxysmal dystonia 10.2 SST NOS1
50 multiple mucosal neuroma 10.2 RET NTRK1

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:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 behavior/neurological MP:0005386 10.17 DMD MBNL1 MTA3 NOS1 NTRK1 NTRK3
2 cardiovascular system MP:0005385 10.1 DMD FOXF1 MBNL1 NKX2-5 NOS1 NTRK3
3 mortality/aging MP:0010768 10.03 BARX1 DMD FOXF1 GAST MBNL1 MTA3
4 digestive/alimentary MP:0005381 10.02 BARX1 DMD FOXF1 GAST NKX2-5 NOS1
5 growth/size/body region MP:0005378 10.02 BARX1 DMD MBNL1 NKX2-5 NOS1 NTRK1
6 muscle MP:0005369 9.76 DMD FOXF1 MBNL1 NKX2-5 NOS1 NTRK1
7 nervous system MP:0003631 9.7 DMD MBNL1 NKX2-5 NOS1 NTRK1 NTRK3
8 no phenotypic analysis MP:0003012 9.23 DMD NKX2-5 NOS1 NTRK1 NTRK3 RET

Drugs & Therapeutics for Hypertrophic Pyloric Stenosis

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

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

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Neonates and Azithromycin, an Innovation in the Treatment of Children in Burkina Faso Recruiting NCT03682653 Phase 4 Azithromycin;Placebo
2 Laparoscopic Versus Open Pyloromyotomy for Infants With Idiopathic Hypertrophic Pyloric Stenosis Completed NCT00195949 Phase 3
3 Pre-delivery Administration of Azithromycin to Prevent Neonatal Sepsis and Death: a Phase III Double-blind Randomized Clinical Trial Recruiting NCT03199547 Phase 3 Azithromycin;Placebo Oral Tablet
4 The Effect of Hypertrophic Pyloric Stenosis (HPS) on Sodium Intake in Childhood Unknown status NCT01159509
5 A Retrospective Comparative Study, Post Operative Outcomes of Pyloromyotomy Procedure Under Spinal Anesthesia Compared to General Anesthesia Unknown status NCT03095144
6 Post-operative Outcomes in the Pyloromyotomy Procedure Under Spinal Anesthesia: A Retrospective Study in 100 Cases Unknown status NCT02879292
7 Prospective Randomized Trial Evaluating the Feeding Regimen After Pyloromyotomy. Completed NCT01509417
8 A Prospective Randomized Trial to Assess Optimal Postoperative Feeding Regiments Following Pyloromyotomy Completed NCT02415049
9 Per-oral Pyloromyotomy for Treating Infantile Hypertrophic Pyloric Stenosis Recruiting NCT04148040

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:

40
Heart, Thyroid, Pancreas, Kidney, Colon, Smooth Muscle, Liver

Publications for Hypertrophic Pyloric Stenosis

Articles related to Hypertrophic Pyloric Stenosis:

(show top 50) (show all 1489)
# Title Authors PMID Year
1
General surgery of childhood in the UK: a general surgeon's perspective. 61
31757504 2020
2
Focal pyloric hypertrophy in adults: a diagnostic pitfall-a case report and review of the literature. 61
31346979 2020
3
Does Maternal Omega 3 Supplementation Protect Against Infantile Hypertrophic Pyloric Stenosis? 61
31990865 2020
4
A Delphi Analysis to Reach Consensus on Preoperative Care in Infants with Hypertrophic Pyloric Stenosis. 61
31958865 2020
5
Neonatal eosinophilic gastroenteritis mimicking hypertrophic pyloric stenosis. 61
31997530 2020
6
Differences in Outcomes Based on Sex for Pediatric Patients Undergoing Pyloromyotomy. 61
31421364 2020
7
Comparison of laparoscopic and open pyloromyotomy: Concerns for omental herniation at port sites after the laparoscopic approach. 61
31941898 2020
8
Low socioeconomic status and formula feeding directly correlate with increased incidence of hypertrophic pyloric stenosis. 61
31757506 2019
9
Pyloric Stenosis: National Trends in the Incidence Rate and Resource Use in the United States From 2012 to 2016. 61
31748239 2019
10
Risk factors for unsuccessful atropine therapy in hypertrophic pyloric stenosis. 61
31520503 2019
11
Are we performing ultrasound measurements of the wall thickness in hypertrophic pyloric stenosis studies the same way? 61
31758244 2019
12
Primary acquired gastric outlet obstruction in children: A retrospective single center study. 61
30922687 2019
13
Retrospective Cohort Study on the Optimal Timing of Orogastric Tube/Nasogastric Tube Insertion in Infants With Pyloric Stenosis. 61
30234537 2019
14
Gastric duplication cyst in an infant with Finnish-type congenital nephrotic syndrome: concurrence or coincidence? 61
31587616 2019
15
Laparoscopic hybrid pyloromyotomy for infantile hypertrophic pyloric stenosis: A simplified technique. 61
31670292 2019
16
Peroral pyloromyotomy for the treatment of infantile hypertrophic pyloric stenosis. 61
31652470 2019
17
Presentation and outcomes in hypertrophic pyloric stenosis: An 11-year review. 61
30677197 2019
18
Implementation of a Quality Control System for Laparoscopic Pyloromyotomy in Hypertrophic Pyloric Stenosis: Hurdles and Pitfalls. 61
30086575 2019
19
Spinal anesthesia compared with general anesthesia for neonates with hypertrophic pyloric stenosis. A retrospective study. 61
31322795 2019
20
Neonatal azithromycin administration to prevent infant mortality: study protocol for a randomised controlled trial. 61
31488494 2019
21
The Impact of Sociodemographic and Hospital Factors on Length of Stay Before and After Pyloromyotomy. 61
30782541 2019
22
The spectrum of self inflicted injuries managed at a major trauma centre in South Africa. 61
31342690 2019
23
Infantile hypertrophic pyloric stenosis: profile, management and outcome of patients admitted to a tertiary hospital in Bloemfontein, South Africa. 61
31342689 2019
24
Co-occurrence of infantile hypertrophic pyloric stenosis and congenital heart defects: a nationwide cohort study. 61
30862960 2019
25
Gastric Peroral Endoscopic Myotomy for Treatment of Congenital Pyloric Stenosis-First Clinical Experience. 61
31017541 2019
26
Venous Physiology Predicts Dehydration in the Pediatric Population. 61
30776742 2019
27
Neuromuscular Blocking Agents and Rapid Sequence Induction for Laparoscopic Pyloromyotomy: Impact on Time to Extubation and Perioperative Complications. 61
31242510 2019
28
Caterpillar Sign in an Infant with Hypertrophic Pyloric Stenosis. 61
30723016 2019
29
The incidence of infantile hypertrophic pyloric stenosis and its association with folic acid supplementation during pregnancy: A nested case-control study. 61
29884554 2019
30
Association between exposure to macrolides and the development of infantile hypertrophic pyloric stenosis: a systematic review and meta-analysis. 61
30470884 2019
31
Developmental outcome at 3 years of age of infants following surgery for infantile hypertrophic pyloric stenosis. 61
30402682 2019
32
Congenital absence of lingual frenum in a non-syndromic patient: a case report. 61
30851733 2019
33
Minimally Invasive Supraumbilical Approach for Pyloromyotomy. 61
31237854 2019
34
ACTG2-Associated Visceral Myopathy With Chronic Intestinal Pseudoobstruction, Intestinal Malrotation, Hypertrophic Pyloric Stenosis, Choledochal Cyst, and a Novel Missense Mutation. 61
30019982 2019
35
Prevalence and descriptive epidemiology of infantile hypertrophic pyloric stenosis in the United States: A multistate, population-based retrospective study, 1999-2010. 61
30549250 2019
36
The association of prenatal and postnatal macrolide exposure with subsequent development of infantile hypertrophic pyloric stenosis: a systematic review and meta-analysis. 61
30717812 2019
37
The Application of Pyloric chisel in the Treatment of Hypertrophic Pyloric Stenosis by Single-Site Umbilical Laparoscopic Pyloromyotomy. 61
30289351 2019
38
Our experience of laparoscopic pyloromyotomy with ultrasound-guided parameters. 61
29582798 2019
39
Genome-wide meta-analysis identifies BARX1 and EML4-MTA3 as new loci associated with infantile hypertrophic pyloric stenosis. 61
30281099 2019
40
Late-Onset Hypertrophic Pyloric Stenosis in a 14-Weeks-Old Full Term Male Infant. 61
31362816 2019
41
Idiopathic Hypertrophic Pyloric Stenosis with Complete Ladd's Band: A Rare Association. 61
31763128 2019
42
Effects of Hospital Volume on Patient Outcomes and Costs in Infants With Pyloric Stenosis. 61
30502289 2019
43
Impact of a multimedia teaching tool on parental anxiety and knowledge during the informed consent process. 61
30255353 2018
44
Optimizing peripheral venous pressure waveforms in an awake pediatric patient by decreasing signal interference. 61
29511972 2018
45
Safety of azithromycin in infants under six months of age in Niger: A community randomized trial. 61
30419040 2018
46
Review of bedside surgeon-performed ultrasound in pediatric patients. 61
29807830 2018
47
Epidemiological and clinical characteristics of 304 patients with infantile hypertrophic pyloric stenosis in Anhui Province of East China, 2012-2015. 61
28701060 2018
48
Atropine Treatment for Hypertrophic Pyloric Stenosis: A Systematic Review and Meta-Analysis. 61
28701002 2018
49
Hypertrophic pyloric stenosis following repair of oesophageal atresia and tracheo-oesophageal fistula in a neonate. 61
30244227 2018
50
Hypertrophic pyloric stenosis following persistent pulmonary hypertension of the newborn: a case report and literature review. 61
30176827 2018

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 11.89 TRPC6 RET NTRK3 NTRK1 NOS1
2
Show member pathways
11.31 TRPC6 TRPC5 TRPC1
3 11.17 RET NTRK3 NTRK1
4 10.6 NOS1 DMD
5 10.2 TRPC6 TRPC5 TRPC1

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 axon GO:0030424 9.62 RET NTRK3 NTRK1 DMD
2 membrane raft GO:0045121 9.56 TRPC5 RET NOS1 DMD
3 receptor complex GO:0043235 9.46 TRPC1 RET NTRK3 NTRK1
4 neuronal cell body GO:0043025 9.35 TRPC5 SST RET NTRK1 DMD
5 cation channel complex GO:0034703 8.8 TRPC6 TRPC5 TRPC1

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

(show all 20)
# Name GO ID Score Top Affiliating Genes
1 positive regulation of cell proliferation GO:0008284 9.89 TRPC5 NTRK3 NKX2-5 MTA3 DMD
2 nervous system development GO:0007399 9.85 TRPC5 RET NTRK3 NTRK1 MBNL1
3 calcium ion transport GO:0006816 9.77 TRPC6 TRPC5 TRPC1
4 peptidyl-tyrosine phosphorylation GO:0018108 9.75 RET NTRK3 NTRK1
5 calcium ion transmembrane transport GO:0070588 9.73 TRPC6 TRPC5 TRPC1
6 transmembrane receptor protein tyrosine kinase signaling pathway GO:0007169 9.71 RET NTRK3 NTRK1
7 positive regulation of MAPK cascade GO:0043410 9.7 RET NTRK3 NTRK1
8 neuron differentiation GO:0030182 9.67 TRPC6 TRPC5 RET DMD
9 regulation of cardiac conduction GO:1903779 9.61 TRPC1 NOS1 NKX2-5
10 innervation GO:0060384 9.58 RET NTRK1
11 regulation of cardiac muscle contraction GO:0055117 9.57 NOS1 NKX2-5
12 regulation of ryanodine-sensitive calcium-release channel activity GO:0060314 9.55 NOS1 DMD
13 regulation of protein kinase B signaling GO:0051896 9.51 NTRK3 NTRK1
14 regulation of cytosolic calcium ion concentration GO:0051480 9.5 TRPC6 TRPC5 TRPC1
15 negative regulation of dendrite morphogenesis GO:0050774 9.48 TRPC6 TRPC5
16 positive regulation of neuron projection development GO:0010976 9.46 RET NTRK3 NTRK1 DMD
17 mechanoreceptor differentiation GO:0042490 9.4 NTRK3 NTRK1
18 neurotrophin signaling pathway GO:0038179 9.26 NTRK3 NTRK1
19 positive regulation of neuron differentiation GO:0045666 9.26 TRPC6 TRPC5 NKX2-5 DMD
20 manganese ion transport GO:0006828 8.8 TRPC6 TRPC5 TRPC1

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

# Name GO ID Score Top Affiliating Genes
1 protein tyrosine kinase activity GO:0004713 9.65 RET NTRK3 NTRK1
2 hormone activity GO:0005179 9.63 SST MLN GAST
3 calcium channel activity GO:0005262 9.61 TRPC6 TRPC5 TRPC1
4 transmembrane receptor protein tyrosine kinase activity GO:0004714 9.5 RET NTRK3 NTRK1
5 actinin binding GO:0042805 9.4 TRPC6 TRPC5
6 GPI-linked ephrin receptor activity GO:0005004 9.37 NTRK3 NTRK1
7 neurotrophin binding GO:0043121 9.26 NTRK3 NTRK1
8 neurotrophin receptor activity GO:0005030 9.16 NTRK3 NTRK1
9 inositol 1,4,5 trisphosphate binding GO:0070679 9.13 TRPC6 TRPC5 TRPC1
10 store-operated calcium channel activity GO:0015279 8.8 TRPC6 TRPC5 TRPC1

Sources for Hypertrophic Pyloric Stenosis

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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