TCP
MCID: TRP008
MIFTS: 46

Tropical Calcific Pancreatitis (TCP)

Categories: Endocrine diseases, Gastrointestinal diseases, Genetic diseases, Rare diseases

Aliases & Classifications for Tropical Calcific Pancreatitis

MalaCards integrated aliases for Tropical Calcific Pancreatitis:

Name: Tropical Calcific Pancreatitis 57 72 36 29 13 6 70
Tcp 57 58 72
Fibrocalculous Pancreatic Diabetes 57 70
Fibrocalculous Pancreatic Diabetes, Susceptibility to 57
Tropical Calcific Chronic Pancreatitis 58
Pancreatitis, Calcific, Tropical 39
Tropical Pancreatitis 58

Characteristics:

Orphanet epidemiological data:

58
tropical pancreatitis
Age of onset: Childhood;

OMIM®:

57 (Updated 20-May-2021)
Inheritance:
autosomal recessive
autosomal dominant

Miscellaneous:
median age at onset is 21 years
occurs most often in developing countries in tropical regions
no phenotypic difference between patients who are homozygous or heterozygous for mutations in the spink1 gene


HPO:

31
tropical calcific pancreatitis:
Inheritance autosomal dominant inheritance autosomal recessive inheritance


Classifications:

Orphanet: 58  
Rare gastroenterological diseases


External Ids:

OMIM® 57 608189
KEGG 36 H00932
ICD10 via Orphanet 33 K86.1
UMLS via Orphanet 71 C1842402
Orphanet 58 ORPHA103918
MedGen 41 C1842402
UMLS 70 C0271642 C1842402

Summaries for Tropical Calcific Pancreatitis

UniProtKB/Swiss-Prot : 72 Tropical calcific pancreatitis: Idiopathic, juvenile, nonalcoholic form of chronic pancreatitis widely prevalent in several tropical countries. It can be associated with fibrocalculous pancreatic diabetes (FCPD) depending on both environmental and genetic factors. TCP differs from alcoholic pancreatitis by a much younger age of onset, pancreatic calcification, a high incidence of insulin dependent but ketosis resistant diabetes mellitus, and an exceptionally high incidence of pancreatic cancer.

MalaCards based summary : Tropical Calcific Pancreatitis, also known as tcp, is related to alcoholic pancreatitis and acute pancreatitis. An important gene associated with Tropical Calcific Pancreatitis is SPINK1 (Serine Peptidase Inhibitor Kazal Type 1), and among its related pathways/superpathways are Lysosome and Antigen processing and presentation. The drugs Vitamin D and Vitamin D3 have been mentioned in the context of this disorder. Affiliated tissues include pancreas, bone and thyroid, and related phenotypes are abdominal pain and neoplasm of the pancreas

KEGG : 36 Tropical calcific pancreatitis (TCP) is a subtype of chronic pancreatitis unique to developing countries in tropical regions. Patients present with recurrent abdominal pain, nutritional deficiencies, and insulin-requiring diabetes resulting from chronic pancreatitis before age 30. Several environmental and genetic factors are implicated in TCP.

More information from OMIM: 608189

Related Diseases for Tropical Calcific Pancreatitis

Diseases related to Tropical Calcific Pancreatitis via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 141)
# Related Disease Score Top Affiliating Genes
1 alcoholic pancreatitis 30.3 SPINK1 PRSS1 CTRC
2 acute pancreatitis 29.9 SPINK1 PRSS1 CTSB
3 pancreatitis 29.7 SPINK1 REG1A PRSS2 PRSS1 CTSB CTRC
4 prss1-related hereditary pancreatitis 29.4 SPINK1 PRSS2 PRSS1 CTRC
5 primary hyperparathyroidism 29.3 SPINK1 ACE
6 pancreatitis, hereditary 28.8 SPINK1 REG1A PRSS2 PRSS1 CTRC
7 type 2 diabetes mellitus 10.4
8 type 1 diabetes mellitus 10.4
9 bone resorption disease 10.3
10 autonomic dysfunction 10.3
11 triiodothyronine receptor auxiliary protein 10.3
12 diabetes mellitus, ketosis-prone 10.2
13 autonomic neuropathy 10.2
14 exocrine pancreatic insufficiency 10.2
15 alcohol use disorder 10.2
16 rapidly involuting congenital hemangioma 10.2
17 gingival recession 10.2
18 glucose intolerance 10.2
19 rickets 10.2
20 periodontitis 10.2
21 osteogenic sarcoma 10.1
22 periodontitis, chronic 10.1
23 osteomyelitis 10.1
24 cholera 10.1
25 ascaris lumbricoides infection 10.1
26 body mass index quantitative trait locus 1 10.1
27 microvascular complications of diabetes 3 10.1
28 microvascular complications of diabetes 4 10.1
29 microvascular complications of diabetes 6 10.1
30 microvascular complications of diabetes 7 10.1
31 monocarboxylate transporter 1 deficiency 10.1
32 hemiplegia 10.1
33 hyperinsulinemic hypoglycemia 10.1
34 polyneuropathy 10.1
35 ascaridiasis 10.1
36 hyperglycemia 10.1
37 cataract 10.1
38 neuropathy 10.1
39 biliary tract disease 10.1
40 small cell cancer of the lung 10.1
41 helix syndrome 10.1
42 osteonecrosis 10.1
43 duodenal obstruction 10.1 SPINK1 PRSS1
44 celiac disease 1 10.1
45 cystic fibrosis 10.1
46 bone mineral density quantitative trait locus 3 10.1
47 protein-energy malnutrition 10.1
48 kwashiorkor 10.1
49 adenocarcinoma 10.1
50 pancreatic adenocarcinoma 10.1

Graphical network of the top 20 diseases related to Tropical Calcific Pancreatitis:



Diseases related to Tropical Calcific Pancreatitis

Symptoms & Phenotypes for Tropical Calcific Pancreatitis

Human phenotypes related to Tropical Calcific Pancreatitis:

31
# Description HPO Frequency HPO Source Accession
1 abdominal pain 31 HP:0002027
2 neoplasm of the pancreas 31 HP:0002894
3 pancreatic calcification 31 HP:0005213
4 chronic pancreatitis 31 HP:0006280
5 insulin-dependent but ketosis-resistant diabetes 31 HP:0008205

Symptoms via clinical synopsis from OMIM®:

57 (Updated 20-May-2021)
Abdomen Pancreas:
chronic pancreatitis
pancreatic calcifications
intraductal calculi, especially in the caput
increased risk of pancreatic cancer

Abdomen:
recurrent abdominal pain since childhood

Endocrine Features:
insulin-dependent but ketosis-resistant diabetes
increased incidence of fibrocalculus pancreatic diabetes (fcpd)

Neoplasia:
increased risk of pancreatic cancer

Clinical features from OMIM®:

608189 (Updated 20-May-2021)

MGI Mouse Phenotypes related to Tropical Calcific Pancreatitis:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 endocrine/exocrine gland MP:0005379 9.43 ACE CTRC CTSB REG1A SPINK1 TCF7L2
2 homeostasis/metabolism MP:0005376 9.17 ACE CTRC CTSB PRSS2 REG1A SPINK1

Drugs & Therapeutics for Tropical Calcific Pancreatitis

Drugs for Tropical Calcific Pancreatitis (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 13)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Vitamin D Approved, Nutraceutical, Vet_approved Phase 4 1406-16-2
2
Vitamin D3 Approved, Nutraceutical Phase 4 67-97-0 6221 5280795
3
Ergocalciferol Approved, Nutraceutical Phase 4 50-14-6 5280793
4 Trace Elements Phase 4
5 Nutrients Phase 4
6 Ergocalciferols Phase 4
7 Vitamins Phase 4
8 Hormones Phase 4
9 Vitamin D2 Phase 4
10 Calciferol Phase 4
11 Micronutrients Phase 4
12 Calcium, Dietary Phase 4
13
Calcium Nutraceutical Phase 4 7440-70-2 271

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Double-blind Randomized Study to Determine the Efficacy of Intramuscular Vitamin D3 Supplementation in Tropical Calcific Pancreatitis Completed NCT00956839 Phase 4 Vitamin D3 (Cholecalciferol)

Search NIH Clinical Center for Tropical Calcific Pancreatitis

Genetic Tests for Tropical Calcific Pancreatitis

Genetic tests related to Tropical Calcific Pancreatitis:

# Genetic test Affiliating Genes
1 Tropical Calcific Pancreatitis 29 SPINK1

Anatomical Context for Tropical Calcific Pancreatitis

MalaCards organs/tissues related to Tropical Calcific Pancreatitis:

40
Pancreas, Bone, Thyroid, Lung, Prostate, Pancreatic Islet, Thymus

Publications for Tropical Calcific Pancreatitis

Articles related to Tropical Calcific Pancreatitis:

(show top 50) (show all 154)
# Title Authors PMID Year
1
Mutations in the pancreatic secretory trypsin inhibitor gene (PSTI/SPINK1) rather than the cationic trypsinogen gene (PRSS1) are significantly associated with tropical calcific pancreatitis. 57 6 61
12011155 2002
2
Association of cathepsin B gene polymorphisms with tropical calcific pancreatitis. 57 61
16492714 2006
3
Pancreatitis in fibrocalculous pancreatic diabetes mellitus is not associated with common mutations in the trypsinogen gene. 57 61
11114105 2000
4
Familial aggregation in tropical fibrocalculous pancreatic diabetes. 57 61
2813331 1989
5
Lack of R117H mutation in the cationic trypsinogen gene in patients with tropical pancreatitis from Bangladesh. 57
9788542 1998
6
Incidence of diabetes in children and adolescents in Dhaka, Bangladesh. 61
33662193 2021
7
Fibrocalculous pancreatic diabetes. 61
32553576 2021
8
Comparison of CGM-Derived Measures of Glycemic Variability Between Pancreatogenic Diabetes and Type 2 Diabetes Mellitus. 61
31282179 2021
9
Incretins in fibrocalculous pancreatic diabetes: A unique subtype of pancreatogenic diabetes. 61
33247879 2020
10
Intra-abdominal fat estimation by bio-electrical impedance analysis in patients with fibrocalculous pancreatic diabetes compared with BMI matched type 2 diabetic subjects and healthy controls. 61
32531743 2020
11
Non-tropical fibrocalculous pancreatic diabetes: case reports and review of recent literature. 61
32691650 2020
12
Fibrocalculous Pancreatic Diabetes. 61
32277298 2020
13
The Clinical Spectrum of Fibrocalculous Pancreatic Diabetes in Kashmir Valley and Comparative Study of the Clinical Profile of Fibrocalculous Pancreatic Diabetes and Type 2 Diabetes Mellitus. 61
31803601 2019
14
Prevalence of End-Organ Damage, Beta Cell Reserve, and Exocrine Pancreas Defect in Fibrocalculous Pancreatic Diabetes: An Eastern India Perspective. 61
31741903 2019
15
Heterogeneity in the aetiology of diabetes mellitus in young adults: A prospective study from north India. 61
31411171 2019
16
A comparative study of the clinical profile of fibrocalculous pancreatic diabetes and type 2 diabetes mellitus. 61
31336514 2019
17
Assessment of insulin sensitivity and secretion in patients with fibrocalculous pancreatic diabetes. 61
31190936 2019
18
Structural, functional and molecular dynamics analysis of cathepsin B gene SNPs associated with tropical calcific pancreatitis, a rare disease of tropics. 61
31592339 2019
19
mutTCPdb: a comprehensive database for genomic variants of a tropical country neglected disease-tropical calcific pancreatitis. 61
30053238 2018
20
Risk Factors Associated with Microalbuminuria in Children and Adolescents with Diabetes in Bangladesh. 61
29535943 2018
21
High Prevalence of Serine Protease Inhibitor Kazal Type 1 Gene Variations Detected by Whole Gene Sequencing in Patients with Fibrocalculous Pancreatic Diabetes. 61
28670531 2017
22
Association Analysis of PRSS1-PRSS2 and CLDN2-MORC4 Variants in Nonalcoholic Chronic Pancreatitis Using Tropical Calcific Pancreatitis as Model. 61
26784911 2016
23
[A case of fibrocalculous pancreatic diabetes]. 61
27373294 2016
24
Fibrocalculous pancreatic diabetes in a Japanese girl with severe motor and intellectual disabilities. 61
26782097 2016
25
Atypical presentation of atypical mycobacteria in atypical diabetes. 61
27127641 2016
26
Fibrocalculous pancreatic diabetes. 61
26472503 2016
27
A Case of Chronic Calcific Nonalcoholic Pancreatitis. 61
27957355 2016
28
Emerging concepts in the pathogenesis of diabetes in fibrocalculous pancreatic diabetes. 61
25707547 2015
29
Fibrocalculous pancreatic diabetes: a case report. 61
25924662 2015
30
Fibrocalculous pancreatic diabetes (FCPD). 61
25395047 2015
31
Fibrocalculous pancreatic diabetes in adult. 61
26605223 2015
32
Polymorphism of Alcohol Metabolizing Gene ADH3 Predisposes to Development of Alcoholic Pancreatitis in North Indian Population. 61
26734614 2015
33
Genetic and phenotypic heterogeneity in tropical calcific pancreatitis. 61
25516642 2014
34
Resting energy expenditure in subjects with fibro-calculous pancreatic diabetes. 61
23773615 2014
35
Comprehensive screening of chymotrypsin C (CTRC) gene in tropical calcific pancreatitis identifies novel variants. 61
22580415 2013
36
Double blind randomized control study of intramuscular vitamin D3 supplementation in tropical calcific pancreatitis. 61
23564348 2013
37
Predictors of malignancy in chronic calcific pancreatitis with head mass. 61
23717745 2013
38
Cataract in children and adolescents with fibrocalculous pancreatic diabetes. 61
23715357 2013
39
Ketosis-resistant diabetes: a rare case in unlikely territory. 61
23544296 2013
40
Tropical calcific pancreatitis. 61
22446928 2012
41
Fibrocalculous pancreatic diabetes in a young Ugandan patient, a rare form of secondary diabetes. 61
23126518 2012
42
Prevalence of cardiac autonomic neuropathy in Asian Indian patients with fibrocalculous pancreatic diabetes. 61
23087858 2012
43
Stellate cell activation in tropical calcific pancreatitis compared to alcoholic pancreatitis, adenocarcinoma of pancreas and normal pancreas. 61
22797393 2012
44
Tropical calcific pancreatitis in HIV patient. 61
23188944 2012
45
Secular trends of fibrocalculous pancreatic diabetes and diabetes secondary to alcoholic chronic pancreatitis at a tertiary care diabetes centre in South India. 61
22406602 2012
46
What's there in a name: tropical calcific pancreatitis and idiopathic chronic pancreatitis in India. 61
20980338 2011
47
Images in clinical medicine. Tropical calcific pancreatitis. 61
21995390 2011
48
High prevalence of low bone mineral density in patients with tropical calcific pancreatitis. 61
21441842 2011
49
Genetic basis of chronic pancreatitis in Asia Pacific region. 61
21323990 2011
50
Idiopathic chronic pancreatitis in India: phenotypic characterisation and strong genetic susceptibility due to SPINK1 and CFTR gene mutations. 61
20551465 2010

Variations for Tropical Calcific Pancreatitis

ClinVar genetic disease variations for Tropical Calcific Pancreatitis:

6
# Gene Name Type Significance ClinVarId dbSNP ID Position
1 SPINK1 SPINK1, -215G-T, PROMOTER SNV Pathogenic 13763 GRCh37:
GRCh38:
2 SPINK1 NM_003122.4(SPINK1):c.194+2T>C SNV Pathogenic 132142 rs148954387 GRCh37: 5:147207583-147207583
GRCh38: 5:147828020-147828020
3 SPINK1 NM_003122.4(SPINK1):c.163C>T (p.Pro55Ser) SNV Likely benign 36778 rs111966833 GRCh37: 5:147207616-147207616
GRCh38: 5:147828053-147828053

UniProtKB/Swiss-Prot genetic disease variations for Tropical Calcific Pancreatitis:

72
# Symbol AA change Variation ID SNP ID
1 SPINK1 p.Asn34Ser VAR_011689 rs17107315

Expression for Tropical Calcific Pancreatitis

Search GEO for disease gene expression data for Tropical Calcific Pancreatitis.

Pathways for Tropical Calcific Pancreatitis

Pathways related to Tropical Calcific Pancreatitis according to KEGG:

36
# Name Kegg Source Accession
1 Lysosome hsa04142
2 Antigen processing and presentation hsa04612

Pathways related to Tropical Calcific Pancreatitis according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1
Show member pathways
12.28 PRSS2 PRSS1 CTSB
2
Show member pathways
12.09 PRSS2 PRSS1 CTSB
3
Show member pathways
11.32 PRSS2 PRSS1
4 10.99 CTSB ACE
5 10.32 CTSB ACE
6 9.95 PRSS1 CTRC

GO Terms for Tropical Calcific Pancreatitis

Cellular components related to Tropical Calcific Pancreatitis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 extracellular space GO:0005615 9.43 SPINK1 REG1A PRSS2 PRSS1 CTSB ACE
2 extracellular region GO:0005576 9.17 SPINK1 REG1A PRSS2 PRSS1 CTSB CTRC

Biological processes related to Tropical Calcific Pancreatitis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 positive regulation of protein binding GO:0032092 9.37 TCF7L2 ACE
2 extracellular matrix disassembly GO:0022617 9.32 PRSS2 PRSS1
3 collagen catabolic process GO:0030574 9.26 PRSS2 CTSB
4 digestion GO:0007586 9.16 PRSS2 PRSS1
5 proteolysis GO:0006508 9.02 PRSS2 PRSS1 CTSB CTRC ACE
6 cobalamin metabolic process GO:0009235 8.96 PRSS1 CTRC

Molecular functions related to Tropical Calcific Pancreatitis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 hydrolase activity GO:0016787 9.65 PRSS2 PRSS1 CTSB CTRC ACE
2 serine-type endopeptidase activity GO:0004252 9.33 PRSS2 PRSS1 CTRC
3 endopeptidase activity GO:0004175 9.32 CTSB ACE
4 serine-type peptidase activity GO:0008236 9.13 PRSS2 PRSS1 CTRC
5 peptidase activity GO:0008233 9.02 PRSS2 PRSS1 CTSB CTRC ACE

Sources for Tropical Calcific Pancreatitis

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
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
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 20-May-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
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