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 14
Pyloric Stenosis, Hypertrophic 42 69
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 42 D046248
NCIt 47 C98952
SNOMED-CT 64 48644003
UMLS 69 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 acquired gastric outlet stenosis and pyloric stenosis. An important gene associated with Hypertrophic Pyloric Stenosis is NOS1 (Nitric Oxide Synthase 1), and among its related pathways/superpathways are Neuroscience and TRP channels. The drugs Bupivacaine and Epinephrine have been mentioned in the context of this disorder. Affiliated tissues include pancreas, heart 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, 3 Pyloric Stenosis, Infantile Hypertrophic, 2
Pyloric Stenosis, Infantile Hypertrophic, 5 Pyloric Stenosis, Infantile Hypertrophic, 4

Diseases related to Hypertrophic Pyloric Stenosis via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 67)
id Related Disease Score Top Affiliating Genes
1 acquired gastric outlet stenosis 11.6
2 pyloric stenosis 11.5
3 esophagitis 10.3
4 properdin deficiency 10.2 DMD NOS1
5 esophageal atresia 10.2
6 submandibular gland cancer 10.2 NOS1 RET
7 pylorospasm 10.1
8 tracheoesophageal fistula 10.1
9 neuronitis 10.1
10 oral leukoedema 10.1 NTRK3 SST
11 ichthyosis 10.0
12 mucositis 10.0
13 gilbert syndrome 10.0
14 pertussis 10.0
15 early-onset parkinson disease 10.0 RET SST
16 congenital diaphragmatic hernia 9.9
17 eosinophilic gastroenteritis 9.9
18 pancreatitis 9.9
19 gastroenteritis 9.9
20 adenomyoma 9.9
21 duodenitis 9.9
22 achalasia 9.9
23 heart disease 9.9
24 cardiomyopathy, dilated, 1d 9.9 DMD HMOX2
25 nevus cavernosus 9.9 DMD MBNL1
26 paraganglioma 9.9 RET SST
27 citrullinemia 9.8
28 phenylketonuria 9.8
29 gastritis 9.8
30 endotheliitis 9.8
31 gastroesophageal reflux 9.8
32 nephrocalcinosis 9.8
33 eosinophilic gastritis 9.8
34 patent urachus 9.8
35 insulin-like growth factor i 9.8
36 penile agenesis 9.8
37 down syndrome 9.8
38 hypothyroidism 9.8
39 congenital mesoblastic nephroma 9.8
40 androgen insensitivity 9.8
41 ventricular septal defect 9.8
42 jejunal atresia 9.8
43 kidney disease 9.8
44 pulmonary hypertension 9.8
45 intestinal perforation 9.8
46 conjunctivitis 9.8
47 congenital hypothyroidism 9.8
48 dyspepsia 9.8
49 esophageal atresia/tracheoesophageal fistula 9.8
50 polyhydramnios 9.8

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:

44 (show all 11)
id Description MGI Source Accession Score Top Affiliating Genes
1 behavior/neurological MP:0005386 10.19 DMD HMOX2 MBNL1 NOS1 NTF4 NTRK3
2 cardiovascular system MP:0005385 10.08 NOS1 NTRK3 RET TRPC6 DMD HMOX2
3 growth/size/body region MP:0005378 10.03 NOS1 NTF4 NTRK3 RET SST DMD
4 mortality/aging MP:0010768 10.02 DMD HMOX2 MBNL1 NKX2-5 NOS1 NTF4
5 homeostasis/metabolism MP:0005376 10.01 DMD HMOX2 NKX2-5 NOS1 NTF4 RET
6 nervous system MP:0003631 10 DMD HMOX2 MBNL1 NKX2-5 NOS1 NTF4
7 digestive/alimentary MP:0005381 9.93 DMD HMOX2 NKX2-5 NOS1 RET SST
8 muscle MP:0005369 9.87 DMD HMOX2 MBNL1 NKX2-5 NOS1 RET
9 no phenotypic analysis MP:0003012 9.7 DMD NKX2-5 NOS1 NTRK3 RET SST
10 normal MP:0002873 9.43 DMD NKX2-5 NOS1 NTRK3 RET SST
11 respiratory system MP:0005388 9.1 DMD HMOX2 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)
id 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
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:


id 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:

39
Pancreas, Heart, Smooth Muscle, Testes, Liver, Endothelial, Cervix

Publications for Hypertrophic Pyloric Stenosis

Articles related to Hypertrophic Pyloric Stenosis:

(show top 50) (show all 564)
id Title Authors Year
1
Population-level surgical outcomes for infantile hypertrophic pyloric stenosis. ( 28576429 )
2017
2
Atropine Treatment for Hypertrophic Pyloric Stenosis: A Systematic Review and Meta-analysis. ( 28701002 )
2017
3
Corrected to uncorrected? The metabolic conundrum of hypertrophic pyloric stenosis. ( 28189444 )
2017
4
A Difficult Recurrent Hypertrophic Pyloric Stenosis. ( 28770145 )
2017
5
50 Years Ago in The Journal of Pediatrics: The Radiographic Diagnosis of Congenital Hypertrophic Pyloric Stenosis. ( 28648281 )
2017
6
Does metabolic alkalosis influence cerebral oxygenation in infantile hypertrophic pyloric stenosis? ( 28550912 )
2017
7
Epidemiological and clinical characteristics of 304 patients with infantile hypertrophic pyloric stenosis in Anhui Province of East China, 2012-2015. ( 28701060 )
2017
8
Excessive Bright Echoes Sign for Hypertrophic Pyloric Stenosis Suggest the Diagnosis: Gastric Pneumatosis and Portal Venous Gas in Infants Suggest HPS. ( 28295432 )
2017
9
Application of color Doppler ultrasound combined with Doppler imaging artifacts in the diagnosis and estimate of congenital hypertrophic pyloric stenosis. ( 28842652 )
2017
10
Perinatal risk factors for infantile hypertrophic pyloric stenosis: A meta-analysis. ( 28318599 )
2017
11
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
12
Adult idiopathic hypertrophic pyloric stenosis. ( 27362602 )
2016
13
Adult-Onset Idiopathic Hypertrophic Pyloric Stenosis Associated With Osteoglophonic Dysplasia and HIV: Case Report and Review of Literature. ( 27599310 )
2016
14
Optimizing fluid resuscitation in hypertrophic pyloric stenosis. ( 26876090 )
2016
15
Laparoscopic pyloromyotomy decreases postoperative length of stay in children with hypertrophic pyloric stenosis. ( 27292596 )
2016
16
Efficacy of Medical Treatment for Infantile Hypertrophic Pyloric Stenosis: AA Meta-analysis. ( 27215474 )
2016
17
A randomized trial to assess advancement of enteral feedings following surgery for hypertrophic pyloric stenosis. ( 27829521 )
2016
18
Hypertrophic pyloric stenosis in the Maritimes: examining the waves of change over time. ( 27669400 )
2016
19
Is there any correlation between radiologic findings and eradication of symptoms after pyloromyotomy in hypertrophic pyloric stenosis? ( 27251656 )
2016
20
Formula-feeding and hypertrophic pyloric stenosis: is there an association? A case-control study. ( 26944185 )
2016
21
Infantile hypertrophic pyloric stenosis with unusual presentations in Sagamu, Nigeria: a case report and review of the literature. ( 27642452 )
2016
22
Asymmetric Hypertrophic Pyloric Stenosis with Concurrent Pancreatic Rest Presenting as Gastric Outlet Obstruction. ( 27179550 )
2016
23
Post-natal erythromycin exposure and risk of infantile hypertrophic pyloric stenosis: a systematic review and meta-analysis. ( 27655365 )
2016
24
Treating idiopathic hypertrophic pyloric stenosis with sequential therapy: A clinical study. ( 27439633 )
2016
25
The cervix sign and other sonographic signs of hypertrophic pyloric stenosis. ( 27315074 )
2016
26
Operative outcomes of infantile hypertrophic pyloric stenosis in patients with congenital heart disease. ( 27528563 )
2016
27
Gastric adenomyoma clinically simulating hypertrophic pyloric stenosis. ( 26837724 )
2016
28
Recent changes in the features of hypertrophic pyloric stenosis. ( 26615824 )
2016
29
Late-onset hypertrophic pyloric stenosis with gastric outlet obstruction: case report and review of the literature. ( 27506212 )
2016
30
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
31
Health disparities in infants with hypertrophic pyloric stenosis. ( 27586849 )
2016
32
Successful combination of endoscopic pyloromyotomy and balloon dilatation for hypertrophic pyloric stenosis in an older child: A novel procedure. ( 27915443 )
2016
33
Incidence and Concurrent Laparoscopic Repair of Hypertrophic Pyloric Stenosis and Patent Processus Vaginalis. ( 25836622 )
2015
34
Association of prematurity with the development of infantile hypertrophic pyloric stenosis. ( 25950452 )
2015
35
Maternal Factors in the Origin of Infantile Hypertrophic Pyloric Stenosis - a Population-based Case-control Study. ( 26394719 )
2015
36
Infantile Hypertrophic Pyloric Stenosis in Postoperative Esophageal Atresia with Tracheoesophageal Fistula. ( 26290814 )
2015
37
Ultrasound-guided paravertebral block for pyloromyotomy in 3 neonates with congenital hypertrophic pyloric stenosis. ( 26123148 )
2015
38
Brain oxygenation during laparoscopic correction of hypertrophic pyloric stenosis. ( 25768674 )
2015
39
Epidermolysis Bullosa with Hypertrophic Pyloric Stenosis in a Newborn. ( 26500857 )
2015
40
Association between NKX2-5 rs29784 and infantile hypertrophic pyloric stenosis in Chinese Han population. ( 25932253 )
2015
41
Infantile hypertrophic pyloric stenosis at a tertiary care hospital in Tanzania: a surgical experience with 102 patients over a 5-year period. ( 26581339 )
2015
42
Is There a Relation Between Pyloric Muscle Thickness and Clinical and Laboratory Data in Infants with Hypertrophic Pyloric Stenosis? ( 27011465 )
2015
43
Adult idiopathic hypertrophic pyloric stenosis. ( 26154756 )
2015
44
Spontaneous Intestinal Perforation and Infantile Hypertrophic Pyloric Stenosis in a Preterm Neonate: A Linked Etiopathogenesis? ( 26055488 )
2015
45
Birth outcomes of male and female patients with infantile hypertrophic pyloric stenosis - a population-based case-control study. ( 26135791 )
2015
46
The use of ultrasonography in infantile hypertrophic pyloric stenosis: does the patient's age and weight affect pyloric size and pyloric ratio? ( 25745654 )
2015
47
Images in emergency medicine. Infant with projectile vomiting. Peristaltic abdominal waves associated with infantile hypertrophic pyloric stenosis. ( 24528943 )
2014
48
Use of macrolides in mother and child and risk of infantile hypertrophic pyloric stenosis: nationwide cohort study. ( 24618148 )
2014
49
Infantile hypertrophic pyloric stenosis: closing in on the cause. ( 24787092 )
2014
50
The difficulty in evaluating all findings in study on use of macrolides in mother and child and risk of infantile hypertrophic pyloric stenosis. ( 25138170 )
2014

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:

id Super pathways Score Top Affiliating Genes
1 11.43 NOS1 NTRK3 RET TRPC6
2 10.55 TRPC5 TRPC6
3 10.23 DMD NOS1

GO Terms for Hypertrophic Pyloric Stenosis

Cellular components related to Hypertrophic Pyloric Stenosis according to GeneCards Suite gene sharing:

id Name GO ID Score Top Affiliating Genes
1 neuronal cell body GO:0043025 9.13 RET SST TRPC5
2 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 14)
id Name GO ID Score Top Affiliating Genes
1 nervous system development GO:0007399 9.8 MBNL1 NTRK3 RET TRPC5
2 positive regulation of cell proliferation GO:0008284 9.65 NKX2-5 NTRK3 TRPC5
3 cardiac muscle contraction GO:0060048 9.54 DMD NKX2-5
4 transmembrane receptor protein tyrosine kinase signaling pathway GO:0007169 9.54 NTF4 NTRK3 RET
5 regulation of cytosolic calcium ion concentration GO:0051480 9.52 TRPC5 TRPC6
6 positive regulation of peptidyl-threonine phosphorylation GO:0010800 9.51 TRPC5 TRPC6
7 regulation of cardiac muscle contraction GO:0055117 9.46 NKX2-5 NOS1
8 innervation GO:0060384 9.43 NTF4 RET
9 regulation of ryanodine-sensitive calcium-release channel activity GO:0060314 9.4 DMD NOS1
10 manganese ion transport GO:0006828 9.32 TRPC5 TRPC6
11 negative regulation of dendrite morphogenesis GO:0050774 9.26 TRPC5 TRPC6
12 mechanoreceptor differentiation GO:0042490 9.16 NTF4 NTRK3
13 neuron differentiation GO:0030182 9.13 RET TRPC5 TRPC6
14 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:

id Name GO ID Score Top Affiliating Genes
1 clathrin binding GO:0030276 9.26 TRPC5 TRPC6
2 actinin binding GO:0042805 9.16 TRPC5 TRPC6
3 store-operated calcium channel activity GO:0015279 8.96 TRPC5 TRPC6
4 inositol 1,4,5 trisphosphate binding GO:0070679 8.62 TRPC5 TRPC6

Sources for Hypertrophic Pyloric Stenosis

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 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 MedGen
42 MeSH
43 MESH via Orphanet
44 MGI
46 NCI
47 NCIt
48 NDF-RT
51 NINDS
52 Novoseek
54 OMIM
55 OMIM via Orphanet
59 PubMed
60 QIAGEN
65 SNOMED-CT via HPO
66 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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