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 33
Pyloric Stenosis, Hypertrophic 44 72
Congenital or Infantile Stricture of Pylorus 12

Classifications:



External Ids:

Disease Ontology 12 DOID:12638
ICD9CM 35 750.5
MeSH 44 D046248
NCIt 50 C98952
SNOMED-CT 68 48644003
ICD10 33 Q40.0
UMLS 72 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 pyloric stenosis. 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 Neuroscience. The drugs Azithromycin and Fentanyl have been mentioned in the context of this disorder. Affiliated tissues include heart, pancreas and smooth muscle, 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 152)
# Related Disease Score Top Affiliating Genes
1 achalasia 30.6 RET NOS1
2 pyloric stenosis 29.2 TRPC6 TRPC5 RET NOS1 NKX2-5 MBNL1
3 acquired gastric outlet stenosis 12.4
4 pyloric stenosis, infantile hypertrophic, 1 12.0
5 esophageal atresia 10.6
6 pylorospasm 10.6
7 tracheoesophageal fistula with or without esophageal atresia 10.6
8 muscle hypertrophy 10.6
9 gastroesophageal reflux 10.5
10 gastritis 10.5
11 gastric ulcer 10.4
12 pertussis 10.4
13 tracheoesophageal fistula 10.4
14 gilbert syndrome 10.3
15 inguinal hernia 10.3
16 dyspepsia 10.3
17 gastroenteritis 10.3
18 bilirubin metabolic disorder 10.3
19 multinucleated neurons, anhydramnios, renal dysplasia, cerebellar hypoplasia, and hydranencephaly 10.3
20 sudden infant death syndrome 10.3
21 ichthyosis, x-linked 10.3
22 esophagitis 10.3
23 ichthyosis 10.3
24 eosinophilic gastroenteritis 10.3
25 myopathy 10.3
26 peptic ulcer disease 10.3
27 intestinal obstruction 10.3
28 intestinal volvulus 10.3
29 eosinophilic enteropathy 10.3
30 diaphragmatic hernia, congenital 10.2
31 intussusception 10.2
32 down syndrome 10.2
33 congenital heart defects, hamartomas of tongue, and polysyndactyly 10.2
34 phenylketonuria 10.2
35 insulin-like growth factor i 10.2
36 helicobacter pylori infection 10.2
37 pyloric stenosis, infantile hypertrophic, 2 10.2
38 pyloric stenosis, infantile hypertrophic, 5 10.2
39 cyanosis, transient neonatal 10.2
40 pulmonary hypertension 10.2
41 metabolic acidosis 10.2
42 polycystic kidney disease 10.2
43 meconium aspiration syndrome 10.2
44 hydronephrosis 10.2
45 megaesophagus 10.2
46 ventricular septal defect 10.2
47 heart septal defect 10.2
48 constipation 10.2
49 adenomyoma 10.2
50 gastric dilatation 10.2

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 10.03 DMD MBNL1 MTA3 NOS1 NTRK1 RET
2 cardiovascular system MP:0005385 9.95 DMD FOXF1 MBNL1 NKX2-5 NOS1 RET
3 mortality/aging MP:0010768 9.92 BARX1 DMD FOXF1 MBNL1 NKX2-5 NOS1
4 muscle MP:0005369 9.86 DMD FOXF1 MBNL1 NKX2-5 NOS1 NTRK1
5 digestive/alimentary MP:0005381 9.85 BARX1 DMD FOXF1 NKX2-5 NOS1 RET
6 nervous system MP:0003631 9.76 DMD MBNL1 NKX2-5 NOS1 NTRK1 RET
7 no phenotypic analysis MP:0003012 9.43 DMD NKX2-5 NOS1 NTRK1 RET TRPC5
8 respiratory system MP:0005388 9.02 DMD FOXF1 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 12)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Azithromycin Approved Phase 4 83905-01-5 447043 55185
2
Fentanyl Approved, Illicit, Investigational, Vet_approved 437-38-7 3345
3
Sevoflurane Approved, Vet_approved 28523-86-6 5206
4
Propofol Approved, Investigational, Vet_approved 2078-54-8 4943
5
Epinephrine Approved, Vet_approved 51-43-4 5816
6
Racepinephrine Approved 329-65-7 838
7
Rocuronium Approved 119302-91-9, 143558-00-3 441290
8
Bupivacaine Approved, Investigational 2180-92-9, 38396-39-3 2474
9 Central Nervous System Depressants
10 Anesthetics
11 Epinephryl borate
12 Bromides

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

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
Heart, Pancreas, Smooth Muscle, Liver, Kidney, Breast, Brain

Publications for Hypertrophic Pyloric Stenosis

Articles related to Hypertrophic Pyloric Stenosis:

(show top 50) (show all 1475)
# Title Authors PMID Year
1
Differences in Outcomes Based on Sex for Pediatric Patients Undergoing Pyloromyotomy. 38
31421364 2019
2
Spinal anesthesia compared with general anesthesia for neonates with hypertrophic pyloric stenosis. A retrospective study. 38
31322795 2019
3
Focal pyloric hypertrophy in adults: a diagnostic pitfall-a case report and review of the literature. 38
31346979 2019
4
The Impact of Sociodemographic and Hospital Factors on Length of Stay Before and After Pyloromyotomy. 38
30782541 2019
5
Gastric Peroral Endoscopic Myotomy for Treatment of Congenital Pyloric Stenosis-First Clinical Experience. 38
31017541 2019
6
Co-occurrence of infantile hypertrophic pyloric stenosis and congenital heart defects: a nationwide cohort study. 38
30862960 2019
7
Infantile hypertrophic pyloric stenosis: profile, management and outcome of patients admitted to a tertiary hospital in Bloemfontein, South Africa. 38
31342689 2019
8
The spectrum of self inflicted injuries managed at a major trauma centre in South Africa. 38
31342690 2019
9
Venous Physiology Predicts Dehydration in the Pediatric Population. 38
30776742 2019
10
Neuromuscular Blocking Agents and Rapid Sequence Induction for Laparoscopic Pyloromyotomy: Impact on Time to Extubation and Perioperative Complications. 38
31242510 2019
11
Caterpillar Sign in an Infant with Hypertrophic Pyloric Stenosis. 38
30723016 2019
12
The incidence of infantile hypertrophic pyloric stenosis and its association with folic acid supplementation during pregnancy: A nested case-control study. 38
29884554 2019
13
Association between exposure to macrolides and the development of infantile hypertrophic pyloric stenosis: a systematic review and meta-analysis. 38
30470884 2019
14
Developmental outcome at 3 years of age of infants following surgery for infantile hypertrophic pyloric stenosis. 38
30402682 2019
15
Congenital absence of lingual frenum in a non-syndromic patient: a case report. 38
30851733 2019
16
Primary acquired gastric outlet obstruction in children: A retrospective single center study. 38
30922687 2019
17
Minimally Invasive Supraumbilical Approach for Pyloromyotomy. 38
31237854 2019
18
ACTG2-Associated Visceral Myopathy With Chronic Intestinal Pseudoobstruction, Intestinal Malrotation, Hypertrophic Pyloric Stenosis, Choledochal Cyst, and a Novel Missense Mutation. 38
30019982 2019
19
Prevalence and descriptive epidemiology of infantile hypertrophic pyloric stenosis in the United States: A multistate, population-based retrospective study, 1999-2010. 38
30549250 2019
20
The association of prenatal and postnatal macrolide exposure with subsequent development of infantile hypertrophic pyloric stenosis: a systematic review and meta-analysis. 38
30717812 2019
21
The Application of Pyloric chisel in the Treatment of Hypertrophic Pyloric Stenosis by Single-Site Umbilical Laparoscopic Pyloromyotomy. 38
30289351 2019
22
Genome-wide meta-analysis identifies BARX1 and EML4-MTA3 as new loci associated with infantile hypertrophic pyloric stenosis. 38
30281099 2019
23
Our experience of laparoscopic pyloromyotomy with ultrasound-guided parameters. 38
29582798 2019
24
Late-Onset Hypertrophic Pyloric Stenosis in a 14-Weeks-Old Full Term Male Infant. 38
31362816 2019
25
Presentation and outcomes in hypertrophic pyloric stenosis: An 11-year review. 38
30677197 2019
26
Effects of Hospital Volume on Patient Outcomes and Costs in Infants With Pyloric Stenosis. 38
30502289 2019
27
Impact of a multimedia teaching tool on parental anxiety and knowledge during the informed consent process. 38
30255353 2018
28
Optimizing peripheral venous pressure waveforms in an awake pediatric patient by decreasing signal interference. 38
29511972 2018
29
Safety of azithromycin in infants under six months of age in Niger: A community randomized trial. 38
30419040 2018
30
Review of bedside surgeon-performed ultrasound in pediatric patients. 38
29807830 2018
31
Epidemiological and clinical characteristics of 304 patients with infantile hypertrophic pyloric stenosis in Anhui Province of East China, 2012-2015. 38
28701060 2018
32
Atropine Treatment for Hypertrophic Pyloric Stenosis: A Systematic Review and Meta-Analysis. 38
28701002 2018
33
Hypertrophic pyloric stenosis following repair of oesophageal atresia and tracheo-oesophageal fistula in a neonate. 38
30244227 2018
34
Hypertrophic pyloric stenosis following persistent pulmonary hypertension of the newborn: a case report and literature review. 38
30176827 2018
35
Retrospective Cohort Study on the Optimal Timing of Orogastric Tube/Nasogastric Tube Insertion in Infants With Pyloric Stenosis. 38
30234537 2018
36
Implementation of a Quality Control System for Laparoscopic Pyloromyotomy in Hypertrophic Pyloric Stenosis: Hurdles and Pitfalls. 38
30086575 2018
37
Case of hepatic portal venous gas in an infant with hypertrophic pyloric stenosis. 38
29980514 2018
38
Infantile Hypertrophic Pyloric Stenosis: An Epidemiological Review. 38
30567916 2018
39
Trends and surgical outcomes of laparoscopic versus open pyloromyotomy. 38
29340829 2018
40
A Simulation Model to Support Laparoscopic Pyloromyotomy Teaching. 38
29630440 2018
41
A simulated training model for laparoscopic pyloromyotomy: Is 3D printing the way of the future? 38
29506814 2018
42
Transient neonatal myasthenia gravis with infantile hypertrophic pyloric stenosis: coincidence or causation? 38
29099348 2018
43
Postoperative outcomes of open versus laparoscopic pyloromyotomy for hypertrophic pyloric stenosis. 38
29506847 2018
44
Laparoscopic pyloromyotomy for hypertrophic pyloric stenosis: a survey of 407 children. 38
29411105 2018
45
Perioperative Complications of Surgery for Hypertrophic Pyloric Stenosis. 38
29510427 2018
46
Population-level surgical outcomes for infantile hypertrophic pyloric stenosis. 38
28576429 2018
47
A Novel Nonelectrosurgical Technique for Incising the Pylorus in Laparoscopic Pyloromyotomy. 38
29236590 2018
48
Infantile hypertrophic pyloric stenosis - Our experience and challenges in a developing country. 38
30829305 2018
49
Infantile hypertrophic pyloric stenosis: a 4-year experience from two tertiary care centres in Cameroon. 38
29338765 2018
50
Endoscopic pyloroplasty for adult hypertrophic pyloric stenosis associated with esophageal achalasia. 38
29905182 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

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 cytoplasm GO:0005737 9.92 TRPC6 TRPC5 NTRK1 NOS1 NKX2-5 MTA3
2 protein-containing complex GO:0032991 9.56 NTRK1 NOS1 NKX2-5 DMD
3 neuronal cell body GO:0043025 9.46 TRPC5 RET NTRK1 DMD
4 cation channel complex GO:0034703 8.96 TRPC6 TRPC5
5 membrane raft GO:0045121 8.92 TRPC5 RET NOS1 DMD

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

(show all 17)
# Name GO ID Score Top Affiliating Genes
1 positive regulation of transcription, DNA-templated GO:0045893 9.84 RET NOS1 NKX2-5 FOXF1
2 positive regulation of cell proliferation GO:0008284 9.8 TRPC5 NKX2-5 MTA3 DMD
3 nervous system development GO:0007399 9.76 TRPC5 RET NTRK1 MBNL1
4 aging GO:0007568 9.65 TRPC6 NTRK1 DMD
5 cardiac muscle contraction GO:0060048 9.57 NKX2-5 DMD
6 regulation of cytosolic calcium ion concentration GO:0051480 9.56 TRPC6 TRPC5
7 regulation of cardiac conduction GO:1903779 9.55 NOS1 NKX2-5
8 spleen development GO:0048536 9.52 NKX2-5 BARX1
9 positive regulation of peptidyl-threonine phosphorylation GO:0010800 9.51 TRPC6 TRPC5
10 positive regulation of neuron projection development GO:0010976 9.5 RET NTRK1 DMD
11 innervation GO:0060384 9.43 RET NTRK1
12 regulation of cardiac muscle contraction GO:0055117 9.4 NOS1 NKX2-5
13 regulation of ryanodine-sensitive calcium-release channel activity GO:0060314 9.37 NOS1 DMD
14 manganese ion transport GO:0006828 9.26 TRPC6 TRPC5
15 neuron differentiation GO:0030182 9.26 TRPC6 TRPC5 RET DMD
16 negative regulation of dendrite morphogenesis GO:0050774 9.16 TRPC6 TRPC5
17 positive regulation of neuron differentiation GO:0045666 8.92 TRPC6 TRPC5 NKX2-5 DMD

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

# Name GO ID Score Top Affiliating Genes
1 sequence-specific DNA binding GO:0043565 9.62 NKX2-5 MTA3 FOXF1 BARX1
2 DNA-binding transcription activator activity, RNA polymerase II-specific GO:0001228 9.43 NKX2-5 FOXF1 BARX1
3 transmembrane receptor protein tyrosine kinase activity GO:0004714 9.4 RET NTRK1
4 clathrin binding GO:0030276 9.37 TRPC6 TRPC5
5 inositol 1,4,5 trisphosphate binding GO:0070679 9.16 TRPC6 TRPC5
6 store-operated calcium channel activity GO:0015279 8.96 TRPC6 TRPC5
7 actinin binding GO:0042805 8.62 TRPC6 TRPC5

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 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 TGDB
71 Tocris
72 UMLS
73 UMLS via Orphanet
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