MCID: HYP009
MIFTS: 41

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 hernia, hiatus. 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 Anti-Bacterial Agents have been mentioned in the context of this disorder. Affiliated tissues include heart, smooth muscle and pancreas, and related phenotypes are behavior/neurological and mortality/aging

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 199)
# Related Disease Score Top Affiliating Genes
1 achalasia 30.9 RET NOS1 GAST
2 hernia, hiatus 30.4 GAST BARX1
3 paralytic ileus 30.2 TYMP MLN
4 intestinal obstruction 30.1 TYMP SST RET MLN
5 gastroparesis 30.1 SST NOS1 MLN GAST
6 constipation 30.0 SST RET NOS1 MLN GAST
7 pyloric stenosis 27.7 TRPC6 TRPC5 TRPC1 SST RET NTRK1
8 acquired gastric outlet stenosis 12.4
9 pyloric stenosis, infantile hypertrophic, 1 12.1
10 esophageal atresia 10.6
11 pylorospasm 10.6
12 muscle hypertrophy 10.6
13 gastroesophageal reflux 10.5
14 gastritis 10.5
15 postcholecystectomy syndrome 10.4 MLN GAST
16 tracheoesophageal fistula with or without esophageal atresia 10.4
17 gastric ulcer 10.4
18 pertussis 10.4
19 gastroenteritis 10.4
20 gastrointestinal neuroendocrine benign tumor 10.4 SST GAST
21 non-functioning pancreatic endocrine tumor 10.3 SST GAST
22 rectum neuroendocrine neoplasm 10.3 SST GAST
23 gilbert syndrome 10.3
24 inguinal hernia 10.3
25 bilirubin metabolic disorder 10.3
26 eosinophilic gastroenteritis 10.3
27 gastric neuroendocrine neoplasm 10.3 SST GAST
28 duodenal gastrinoma 10.3 SST GAST
29 functional colonic disease 10.3 SST MLN
30 appendix lymphoma 10.3 SST GAST
31 glucagonoma 10.3 SST GAST
32 pancreatic cholera 10.3 SST GAST
33 rare tumor 10.3 SST RET
34 gastritis, familial giant hypertrophic 10.3 GAST BARX1
35 pancreatic gastrinoma 10.3 SST GAST
36 endocrine pancreas disease 10.3 SST GAST
37 gastrointestinal neuroendocrine tumor 10.3 SST GAST
38 carcinoid tumors, intestinal 10.3 SST GAST
39 ectopic cushing syndrome 10.3 SST RET
40 peptic esophagitis 10.3 MLN GAST
41 volvulus of midgut 10.3
42 multinucleated neurons, anhydramnios, renal dysplasia, cerebellar hypoplasia, and hydranencephaly 10.3
43 sudden infant death syndrome 10.3
44 ichthyosis, x-linked 10.3
45 gastrointestinal ulceration, recurrent, with dysfunctional platelets 10.3
46 esophagitis 10.3
47 ichthyosis 10.3
48 myopathy 10.3
49 peptic ulcer disease 10.3
50 47,xyy 10.3

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 10.07 DMD MBNL1 MTA3 NOS1 NTRK1 RET
2 mortality/aging MP:0010768 10 BARX1 BNIP1 DMD GAST MBNL1 MTA3
3 digestive/alimentary MP:0005381 9.92 BARX1 DMD GAST NKX2-5 NOS1 RET
4 muscle MP:0005369 9.7 DMD MBNL1 NKX2-5 NOS1 NTRK1 RET
5 nervous system MP:0003631 9.7 DMD MBNL1 NKX2-5 NOS1 NTRK1 RET
6 no phenotypic analysis MP:0003012 9.17 DMD NKX2-5 NOS1 NTRK1 RET SST

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 447043 55185
2 Anti-Bacterial Agents Phase 4
3 Anti-Infective Agents Phase 4
4
Fentanyl Approved, Illicit, Investigational, Vet_approved 437-38-7 3345
5
Epinephrine Approved, Vet_approved 51-43-4 5816
6
Rocuronium Approved 119302-91-9, 143558-00-3 441290
7
Propofol Approved, Investigational, Vet_approved 2078-54-8 4943
8
Racepinephrine Approved 329-65-7 838
9
Sevoflurane Approved, Vet_approved 28523-86-6 5206
10
Bupivacaine Approved, Investigational 2180-92-9, 38396-39-3 2474
11 Epinephryl borate
12 Anesthetics
13 Bromides
14 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, Smooth Muscle, Pancreas, Kidney, Liver, Breast, Brain

Publications for Hypertrophic Pyloric Stenosis

Articles related to Hypertrophic Pyloric Stenosis:

(show top 50) (show all 1496)
# Title Authors PMID Year
1
Pediatric emergency gastrointestinal ultrasonography: pearls & pitfalls. 61
32438254 2020
2
Infantile hypertrophic pyloric stenosis in patients with esophageal atresia. 61
32298054 2020
3
Does Maternal Omega 3 Supplementation Protect Against Infantile Hypertrophic Pyloric Stenosis? 61
31990865 2020
4
Temporal caterpillar sign in a case of infantile hypertrophic pyloric stenosis. 61
32463578 2020
5
Incidence and importance of portal venous gas in children with hypertrophic pyloric stenosis. 61
32462306 2020
6
The Safety and Effectiveness of Laparoscopic versus Open Surgery for Congenital Hypertrophic Pyloric Stenosis in Infants. 61
32407297 2020
7
Peroral pyloromyotomy for the treatment of infantile hypertrophic pyloric stenosis. 61
31652470 2020
8
Perioperative apnea in infants with hypertrophic pyloric stenosis: A systematic review. 61
32298502 2020
9
Long-term follow-up in surgical newborns: A single-institution experience. 61
32201108 2020
10
Are we performing ultrasound measurements of the wall thickness in hypertrophic pyloric stenosis studies the same way? 61
31758244 2020
11
General surgery of childhood in the UK: a general surgeon's perspective. 61
31757504 2020
12
Focal pyloric hypertrophy in adults: a diagnostic pitfall-a case report and review of the literature. 61
31346979 2020
13
A Delphi Analysis to Reach Consensus on Preoperative Care in Infants with Hypertrophic Pyloric Stenosis. 61
31958865 2020
14
Neonatal eosinophilic gastroenteritis mimicking hypertrophic pyloric stenosis. 61
31997530 2020
15
Comparison of laparoscopic and open pyloromyotomy: Concerns for omental herniation at port sites after the laparoscopic approach. 61
31941898 2020
16
Differences in Outcomes Based on Sex for Pediatric Patients Undergoing Pyloromyotomy. 61
31421364 2020
17
Low socioeconomic status and formula feeding directly correlate with increased incidence of hypertrophic pyloric stenosis. 61
31757506 2019
18
Pyloric Stenosis: National Trends in the Incidence Rate and Resource Use in the United States From 2012 to 2016. 61
31748239 2019
19
Risk factors for unsuccessful atropine therapy in hypertrophic pyloric stenosis. 61
31520503 2019
20
Primary acquired gastric outlet obstruction in children: A retrospective single center study. 61
30922687 2019
21
Retrospective Cohort Study on the Optimal Timing of Orogastric Tube/Nasogastric Tube Insertion in Infants With Pyloric Stenosis. 61
30234537 2019
22
Laparoscopic hybrid pyloromyotomy for infantile hypertrophic pyloric stenosis: A simplified technique. 61
31670292 2019
23
Implementation of a Quality Control System for Laparoscopic Pyloromyotomy in Hypertrophic Pyloric Stenosis: Hurdles and Pitfalls. 61
30086575 2019
24
Presentation and outcomes in hypertrophic pyloric stenosis: An 11-year review. 61
30677197 2019
25
Gastric duplication cyst in an infant with Finnish-type congenital nephrotic syndrome: concurrence or coincidence? 61
31587616 2019
26
Spinal anesthesia compared with general anesthesia for neonates with hypertrophic pyloric stenosis. A retrospective study. 61
31322795 2019
27
Neonatal azithromycin administration to prevent infant mortality: study protocol for a randomised controlled trial. 61
31488494 2019
28
The Impact of Sociodemographic and Hospital Factors on Length of Stay Before and After Pyloromyotomy. 61
30782541 2019
29
The spectrum of self inflicted injuries managed at a major trauma centre in South Africa. 61
31342690 2019
30
Infantile hypertrophic pyloric stenosis: profile, management and outcome of patients admitted to a tertiary hospital in Bloemfontein, South Africa. 61
31342689 2019
31
Co-occurrence of infantile hypertrophic pyloric stenosis and congenital heart defects: a nationwide cohort study. 61
30862960 2019
32
Gastric Peroral Endoscopic Myotomy for Treatment of Congenital Pyloric Stenosis-First Clinical Experience. 61
31017541 2019
33
Venous Physiology Predicts Dehydration in the Pediatric Population. 61
30776742 2019
34
Neuromuscular Blocking Agents and Rapid Sequence Induction for Laparoscopic Pyloromyotomy: Impact on Time to Extubation and Perioperative Complications. 61
31242510 2019
35
Caterpillar Sign in an Infant with Hypertrophic Pyloric Stenosis. 61
30723016 2019
36
The incidence of infantile hypertrophic pyloric stenosis and its association with folic acid supplementation during pregnancy: A nested case-control study. 61
29884554 2019
37
Association between exposure to macrolides and the development of infantile hypertrophic pyloric stenosis: a systematic review and meta-analysis. 61
30470884 2019
38
Developmental outcome at 3 years of age of infants following surgery for infantile hypertrophic pyloric stenosis. 61
30402682 2019
39
Congenital absence of lingual frenum in a non-syndromic patient: a case report. 61
30851733 2019
40
Minimally Invasive Supraumbilical Approach for Pyloromyotomy. 61
31237854 2019
41
ACTG2-Associated Visceral Myopathy With Chronic Intestinal Pseudoobstruction, Intestinal Malrotation, Hypertrophic Pyloric Stenosis, Choledochal Cyst, and a Novel Missense Mutation. 61
30019982 2019
42
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
43
Prevalence and descriptive epidemiology of infantile hypertrophic pyloric stenosis in the United States: A multistate, population-based retrospective study, 1999-2010. 61
30549250 2019
44
The Application of Pyloric chisel in the Treatment of Hypertrophic Pyloric Stenosis by Single-Site Umbilical Laparoscopic Pyloromyotomy. 61
30289351 2019
45
Genome-wide meta-analysis identifies BARX1 and EML4-MTA3 as new loci associated with infantile hypertrophic pyloric stenosis. 61
30281099 2019
46
Our experience of laparoscopic pyloromyotomy with ultrasound-guided parameters. 61
29582798 2019
47
Idiopathic Hypertrophic Pyloric Stenosis with Complete Ladd's Band: A Rare Association. 61
31763128 2019
48
Late-Onset Hypertrophic Pyloric Stenosis in a 14-Weeks-Old Full Term Male Infant. 61
31362816 2019
49
Effects of Hospital Volume on Patient Outcomes and Costs in Infants With Pyloric Stenosis. 61
30502289 2019
50
Impact of a multimedia teaching tool on parental anxiety and knowledge during the informed consent process. 61
30255353 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 11.99 TRPC6 RET NTRK1 NOS1
2
Show member pathways
11.01 TRPC6 TRPC5 TRPC1
3 10.54 NOS1 DMD
4 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 membrane raft GO:0045121 9.46 TRPC5 RET NOS1 DMD
2 neuronal cell body GO:0043025 9.35 TRPC5 SST RET NTRK1 DMD
3 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 12)
# Name GO ID Score Top Affiliating Genes
1 calcium ion transport GO:0006816 9.69 TRPC6 TRPC5 TRPC1
2 calcium ion transmembrane transport GO:0070588 9.63 TRPC6 TRPC5 TRPC1
3 positive regulation of neuron projection development GO:0010976 9.61 RET NTRK1 DMD
4 neuron differentiation GO:0030182 9.56 TRPC6 TRPC5 RET DMD
5 regulation of cardiac conduction GO:1903779 9.5 TRPC1 NOS1 NKX2-5
6 regulation of cardiac muscle contraction GO:0055117 9.49 NOS1 NKX2-5
7 innervation GO:0060384 9.48 RET NTRK1
8 regulation of ryanodine-sensitive calcium-release channel activity GO:0060314 9.4 NOS1 DMD
9 negative regulation of dendrite morphogenesis GO:0050774 9.37 TRPC6 TRPC5
10 regulation of cytosolic calcium ion concentration GO:0051480 9.33 TRPC6 TRPC5 TRPC1
11 positive regulation of neuron differentiation GO:0045666 9.26 TRPC6 TRPC5 NKX2-5 DMD
12 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 hormone activity GO:0005179 9.54 SST MLN GAST
2 calcium channel activity GO:0005262 9.43 TRPC6 TRPC5 TRPC1
3 ATPase binding GO:0051117 9.33 TRPC6 TRPC5 TRPC1
4 actinin binding GO:0042805 9.32 TRPC6 TRPC5
5 inositol 1,4,5 trisphosphate binding GO:0070679 9.13 TRPC6 TRPC5 TRPC1
6 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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