Exocrine Pancreatic Insufficiency disease: Malacards - Research Articles, Drugs, Genes, Clinical Trials

MCID: EXC002 MIFTS: 41	Exocrine Pancreatic Insufficiency Categories: Endocrine diseases
Genes Tissues Related diseases Publications Pathways Drugs Expand all tables

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Aliases & Classifications for Exocrine Pancreatic Insufficiency

MalaCards integrated aliases for Exocrine Pancreatic Insufficiency:

Name: Exocrine Pancreatic Insufficiency ¹² ⁵⁴ ⁴³ ¹⁵ ⁷¹

Classifications:

MalaCards categories:
Anatomical: Endocrine diseases

See all MalaCards categories (disease lists)

External Ids:

Disease Ontology ¹² DOID:13316

MeSH ⁴³ D010188

NCIt ⁴⁹ C84316

SNOMED-CT ⁶⁷ 47367009

ICD10 ³² K86.81

UMLS ⁷¹ C0267963

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Summaries for Exocrine Pancreatic Insufficiency

MalaCards based summary : Exocrine Pancreatic Insufficiency is related to shwachman-diamond syndrome 1 and pancreatic agenesis 1. An important gene associated with Exocrine Pancreatic Insufficiency is COX4I2 (Cytochrome C Oxidase Subunit 4I2), and among its related pathways/superpathways are Pancreatic secretion and Gastric acid production. The drugs Pharmaceutical Solutions and Omeprazole have been mentioned in the context of this disorder. Affiliated tissues include Pancreas, testes and liver, and related phenotypes are digestive/alimentary and growth/size/body region

Wikipedia : ⁷⁴ Exocrine pancreatic insufficiency (EPI) is the inability to properly digest food due to a lack of... more...

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Related Diseases for Exocrine Pancreatic Insufficiency

Diseases related to Exocrine Pancreatic Insufficiency via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 158)

#	Related Disease	Score	Top Affiliating Genes
1	shwachman-diamond syndrome 1	32.6	SBDS ELANE EFL1
2	pancreatic agenesis 1	32.3	SPINK1 SCT PNLIP PDX1 CFTR
3	diarrhea	30.9	TNF SCT CFTR CCK
4	calvarial hyperostosis	30.7	COX4I2 COX4I1
5	pancreas, annular	30.4	SPINK1 PTF1A
6	maturity-onset diabetes of the young, type 8, with exocrine dysfunction	30.3	PDX1 CEL
7	alcoholic pancreatitis	30.2	SPINK1 SCT CFTR CCK
8	autoimmune pancreatitis	30.1	TNF CTLA4 CFTR
9	pancreatic steatorrhea	29.9	SCT PNLIP CELA3B CELA1 CCK
10	maturity-onset diabetes of the young	29.9	PTF1A PDX1 CELA3B CEL
11	celiac disease 1	29.8	TNF SCT CTLA4 CELA3B CELA1 CCK
12	bronchiectasis	29.8	TNF ELANE CFTR
13	diabetes mellitus	29.6	TNF SPINK1 SCT PTF1A PDX1 CTLA4
14	pancreatic adenocarcinoma	29.6	TNF SPINK1 SCT CFTR CCK
15	acute pancreatitis	29.3	TNF SPINK1 SCT PNLIP ELANE CFTR
16	pancreatitis	28.2	UBR1 TNF SPINK1 SCT PTF1A PNLIP
17	exocrine pancreatic insufficiency, dyserythropoietic anemia, and calvarial hyperostosis	13.0
18	johanson-blizzard syndrome	11.9
19	cystic fibrosis	11.8
20	poikiloderma, hereditary fibrosing, with tendon contractures, myopathy, and pulmonary fibrosis	11.6
21	pancreatic agenesis 2	11.4
22	neutropenia, severe congenital, 8, autosomal dominant	11.4
23	pancreatic cancer	10.5
24	pancreas, dorsal, agenesis of	10.5	PTF1A PDX1
25	overgrowth syndrome	10.5
26	neutropenia	10.4
27	functionless pituitary adenoma	10.4	CCKAR CCK
28	acalculous cholecystitis	10.4	CCKAR CCK
29	microscopic polyangiitis	10.4	TNF CTLA4
30	gastroduodenal crohn's disease	10.4	TNF CCK
31	spotted fever rickettsiosis	10.4	TNF CTLA4
32	skin sarcoidosis	10.3	TNF CTLA4
33	dysostosis	10.3
34	prss1-related hereditary pancreatitis	10.3	SPINK1 CFTR
35	opioid addiction	10.3	CCKAR CCK
36	rickets	10.3
37	alcohol use disorder	10.3
38	irritable bowel syndrome	10.3
39	duodenal ulcer	10.3	TNF SCT CCK
40	nontuberculous mycobacterial lung disease	10.2	TNF CFTR
41	mycobacterium abscessus	10.2	TNF CFTR
42	fibrosis of extraocular muscles, congenital, 1	10.2
43	pyle disease	10.2
44	vitamin b12 deficiency	10.2
45	pancreas disease	10.2
46	lipomatosis	10.2
47	liver cirrhosis	10.2
48	intestinal disease	10.2
49	hypoglycemia	10.2
50	pustular psoriasis	10.2	TNF ELANE

Graphical network of the top 20 diseases related to Exocrine Pancreatic Insufficiency:

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Symptoms & Phenotypes for Exocrine Pancreatic Insufficiency

MGI Mouse Phenotypes related to Exocrine Pancreatic Insufficiency:

⁴⁵

#	Description	MGI Source Accession	Score	Top Affiliating Genes
1	digestive/alimentary	MP:0005381	10.11	CCKAR CEL CFTR CTLA4 PDX1 PNLIP
2	growth/size/body region	MP:0005378	10.03	CCKAR CEL CFTR COX4I1 COX4I2 PDX1
3	endocrine/exocrine gland	MP:0005379	10.02	CCK CCKAR CFTR CTLA4 PDX1 PTF1A
4	homeostasis/metabolism	MP:0005376	10	CCK CCKAR CEL CFTR COX4I2 CTLA4
5	liver/biliary system	MP:0005370	9.61	CCKAR CEL CFTR CTLA4 PDX1 PNLIP
6	respiratory system	MP:0005388	9.17	CCKAR CELA1 CFTR COX4I2 CTLA4 PTF1A

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Drugs & Therapeutics for Exocrine Pancreatic Insufficiency

Drugs for Exocrine Pancreatic Insufficiency (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 43)

Name

Status

Phase

Clinical Trials

Cas Number

PubChem Id

Pharmaceutical Solutions

Phase 3

Omeprazole

Approved, Investigational, Vet_approved

Phase 2

73590-58-6

4594

Choline

Approved, Nutraceutical

Phase 2

62-49-7

305

Bile Acids and Salts

Phase 2

Omega 3 Fatty Acid

Phase 2

Nootropic Agents

Phase 2

Hypolipidemic Agents

Phase 2

Flax

Phase 2

Lecithin

Phase 2

Lipid Regulating Agents

Phase 2

Sunflower

Phase 2

Antimetabolites

Phase 2

Soy Bean

Phase 2

Proton Pump Inhibitors

Phase 2

Anti-Ulcer Agents

Phase 2

Antacids

Phase 2

Phylloquinone

Approved, Investigational

Phase 1

84-80-0

Menadione

Approved, Nutraceutical

Phase 1

58-27-5

4055

Menaquinone

Investigational

Phase 1

1182-68-9

Vitamins

Phase 1

Trace Elements

Phase 1

Micronutrients

Phase 1

Nutrients

Phase 1

Hemostatics

Phase 1

Vitamin K

Phase 1

naphthoquinone

Phase 1

Antifibrinolytic Agents

Phase 1

Coagulants

Phase 1

Saxagliptin

Approved

361442-04-8

11243969

Repaglinide

Approved, Investigational

135062-02-1

65981

Glucagon

Approved

16941-32-5

carbamide peroxide

Approved

124-43-6

gastric inhibitory polypeptide

Investigational

100040-31-1

HIV Protease Inhibitors

Hypoglycemic Agents

protease inhibitors

Incretins

Dipeptidyl-Peptidase IV Inhibitors

Sitagliptin Phosphate

insulin

Insulin, Globin Zinc

Glucagon-Like Peptide 1

Liver Extracts

Interventional clinical trials:

(show top 50) (show all 84)

#	Name	Status	NCT ID	Phase	Drugs
1	Evaluation of the Digestive and Metabolic Utilisation of Dietary Protein in Patients With Chronic Pancreatitis	Completed	NCT00957151	Phase 4	Creon
2	An Open-Label Study Evaluating the Efficacy and Safety of Liprotamase in Subjects With Exocrine Pancreatic Insufficiency Due to Cystic Fibrosis	Completed	NCT02823964	Phase 4	Liprotamase
3	A Multicenter, Open-Label, Safety Extension of Study PR-011 Titled: A Multicenter, Randomized, Open-Label, Crossover Study to Evaluate the Mode of Administration and Safety of EUR-1008 in Infants 1 to 12 Months of Age With Exocrine Pancreatic Insufficiency (EPI) Associated With Cystic Fibrosis (CF)	Completed	NCT01131507	Phase 4	EUR-1008 (APT-1008)
4	An Open-label, Multicenter, Randomized, Cross-over Study to Compare the Safety and Efficacy of PANZYTRAT® 25,000 to KREON® 25,000 in the Control of Steatorrhea in Subjects Aged 7 Years and Older With Cystic Fibrosis (CF) and Exocrine Pancreatic Insufficiency (EPI)	Completed	NCT01327703	Phase 4	Panzytrat® 25,000;Kreon® 25,000
5	A Multicenter, Randomized, Open-Label, Crossover Study to Evaluate the Mode of Administration and Safety of EUR-1008 in Infants 1 to 12 Months of Age With Exocrine Pancreatic Insufficiency (EPI) Associated With Cystic Fibrosis (CF)	Completed	NCT01100606	Phase 4	EUR-1008 (APT-1008);EUR-1008 (APT-1008)
6	One Week Double-Blind, Randomized, Placebo-Controlled, Parallel-Group, Multi-Center Study With Creon® 25000 Minimicrospheres™ in Subjects With Pancreatic Exocrine Insufficiency After Pancreatic Surgery, Followed by an Open-Label Long-Term Extension of 1 Year	Completed	NCT00535756	Phase 4	Creon;Placebo
7	Enzyme Substitution in Exocrine Pancreatic Insufficiency; Self Administration Against a Fixed Dose Regimen	Completed	NCT01430234	Phase 4	Panzytrat 25.000 FIP-E units of Lipase
8	MALABSORPTION BLOOD TEST: Toward a Novel Approach to Quantify Steatorrhea	Completed	NCT00572975	Phase 4
9	A Phase 4 Study to Compare US Marketed Creon Drug Product With Drug Product Manufactured With a Modernized Process at an Alternate Manufacturing Site, in Subjects With Exocrine Pancreatic Insufficiency (EPI) Due to Cystic Fibrosis	Recruiting	NCT03924947	Phase 4	Pancrelipase
10	H.O.P.E. Healing Optimization Through Preoperative Engagement: A Prospective Single Cohort Study to Evaluate the Effect of a Prehabilitation Program on Preoperative Outcomes in Upper GI Surgical Oncology Patients	Enrolling by invitation	NCT03642093	Phase 4
11	Creon (Pancrelipase) Therapy for Subjects With Exocrine Pancreatic Insufficiency (EPI) Due to Pancreatic Cancer: A Double-blind, Randomized, Parallel Design With 2 Dose Cohorts of Pancrelipase in Resected Pancreatic Cancer Subjects and an Open-label Single Dose Cohort in Non-resected Pancreatic Cancer Subjects	Not yet recruiting	NCT03859869	Phase 4	Pancrelipase;Placebo
12	Intraduodenal Aspiration Study to Assess the Bioavailability of Oral Pancrecarb® Compared to Placebo Control in Patients With Pancreatic Insufficiency	Terminated	NCT00744250	Phase 4	Pancrelipase
13	Randomized, Double Blind, Placebo-controlled Trial of Creon in Patients With Low Faecal Pancreatic Elastase	Terminated	NCT01865695	Phase 4	Creon
14	A Double-blind, Randomized, Multi-center, Placebo-controlled, Parallel-group Study to Assess the Effect of Creon® on Pancreatic Exocrine Insufficiency in Subjects With Diabetes Mellitus Type 2	Withdrawn	NCT02009410	Phase 4	Creon;Creon 25000 matching Placebo
15	Exocrine Pancreatic Insufficiency in Pancreatic Cancer: Evaluating the Need and Efficacy of Pancreatic Enzyme Replacement Therapy	Withdrawn	NCT01401387	Phase 4
16	Clinical Non-Inferiority of Pancreatic Enzymes (Norzyme ® - Bergamo) Compared To Creon ® (Solvay Pharmaceutical) in Patients With Exocrine Pancreatic Insufficiency	Unknown status	NCT01012908	Phase 2, Phase 3
17	An Open-Label Clinical Study Evaluating the Long-Term Safety of ALTU-135 for the Treatment of Patients With Cystic Fibrosis-Related Exocrine Pancreatic Insufficiency	Completed	NCT00449904	Phase 3	Liprotamase
18	A Phase 3, Open-Label Study Evaluating the Efficacy and Safety of Liprotamase in Subjects With Cystic Fibrosis-Related Exocrine Pancreatic Insufficiency	Completed	NCT02734810	Phase 3	Liprotamase Powder for Oral Solution
19	A Randomised, Double-Blind, Active-Controlled, Two-Treatment, Crossover, Multinational, Multicentre Study to Compare Two Pancreatic Enzyme Products in the Treatment of Exocrine Pancreatic Insufficiency in Subjects With Cystic Fibrosis	Completed	NCT01641393	Phase 3	EUR-1008 25,000 Units;Kreon 25,000 Units
20	A Phase III, Randomized, Double-Blind, Placebo-Controlled Clinical Study Evaluating the Efficacy and Safety of ALTU-135 Treatment in Patients With Cystic Fibrosis-Related Exocrine Pancreatic Insufficiency	Completed	NCT00449878	Phase 3	Liprotamase;Placebo
21	A Study to Investigate the Effect of Delayed Release Pancrelipase on Maldigestion in Patients With Exocrine Pancreatic Insufficiency Due to Chronic Pancreatitis and Pancreatectomy	Completed	NCT00414908	Phase 3	Pancrelipase delayed release capsule;Placebo Comparator
22	An Open-Label Study to Evaluate the Efficacy and Safety of Pancreatic Enzyme Product (PEP) Microtabs in Pediatric Patients With Cystic Fibrosis and Exocrine Pancreatic Insufficiency	Completed	NCT00981214	Phase 3	EUR-1008 (APT-1008)
23	A Double-blind, Placebo-controlled, Parallel-group, Comparative Study to Confirm the Safety and Efficacy of Oral 1.5 g/Day and 3.0 g/Day of SA-001 in Patients With Pancreatic Exocrine Insufficiency Caused by Chronic Pancreatitis or by Pancreatectomy	Completed	NCT00400842	Phase 3	SA-001;SA-001;Placebo
24	A Phase 3, Randomized, Open-Label, Assessor-Blind, Noninferiority, Active-Comparator Study Evaluating the Efficacy and Safety of Liprotamase in Subjects With Cystic Fibrosis-Related Exocrine Pancreatic Insufficiency	Completed	NCT02279498	Phase 3	Liprotamase;porcine (pig) PERT
25	A Multicenter, Randomized, Double-Blind, Crossover Study to Compare the Safety and Efficacy of Ultrase® MT20 to Placebo for the Correction of Steatorrhea in Patients With Cystic Fibrosis (CF)	Completed	NCT00408317	Phase 3	Ultrase® MT20;Placebo
26	A Multicenter, Randomized, Double-blind, Parallel, Placebo-controlled, Phase III Study to Assess the Safety and Efficacy of Viokase® 16 for the Correction of Steatorrhea in Patients With Exocrine Pancreatic Insufficiency	Completed	NCT00559364	Phase 3	Viokase® 16;Placebo;Proton pump inhibitor (PPI);Omeprazole
27	An Open Label, Multi-center, Study to Assess the Safety and Tolerability of Pancrelipase Delayed Release Capsules in Infants and Children Less Than 7 Years of Age With Pancreatic Exocrine Insufficiency Due to Cystic Fibrosis	Completed	NCT00775528	Phase 3	Pancrelipase Delayed Release
28	A Double-blind, Randomized, Multi-center, Placebo-controlled, Cross-over Study to Assess the Efficacy and Safety of Pancrelipase Delayed Release 12,000 Unit Capsules in Subjects Aged 7 - 11 With Pancreatic Exocrine Insufficiency Due to Cystic Fibrosis	Completed	NCT00690820	Phase 3	Pancrelipase Delayed Release;Placebo Comparator
29	A Double-blind, Randomized, Multicenter, Cross-over Study to Compare the Effect of Creon N and Creon® on Fat Digestion in Subjects ≥ 12 Years of Age With Pancreatic Exocrine Insufficiency Due to Cystic Fibrosis	Completed	NCT02137382	Phase 3	Creon®;Creon N
30	A Randomized Double-blind (Withdrawal) Phase 3 Study to Evaluate the Efficacy and Tolerability of Pancrelipase MT Capsules Compared With Placebo in the Treatment of Subjects With Cystic Fibrosis-dependent Exocrine Pancreatic Insufficiency	Completed	NCT00662675	Phase 3	Pancrease MT 10.5, or MT 21;Placebo for Pancrease MT 10.5 or MT 21
31	A Randomized, Double-Blind, Placebo-Controlled, Two-Treatment, Crossover Study to Evaluate the Safety and Efficacy of Eurand Pancreatic Enzyme Product (PEP) in Patients With Cystic Fibrosis and Exocrine Pancreatic Insufficiency	Completed	NCT00297167	Phase 3	EUR-1008 (APT-1008);Placebo;Placebo;EUR-1008 (APT-1008)
32	A One-year Open-label Study to Assess the Safety of Oral Long-term Use of SA-001 in Patients With Pancreatic Exocrine Insufficiency Caused by Chronic Pancreatitis or by Pancreatectomy	Completed	NCT00401076	Phase 3	SA-001
33	A One Week Double-Blind, Randomized, Placebo-Controlled, Parallel Group, Multi-Center Study With Creon 40,000 MMS in Subjects With Pancreatic Exocrine Insufficiency Due to Chronic Pancreatitis, Followed by an Open-Label Long-Term Extension	Completed	NCT00705978	Phase 3	Pancreatin;Placebo
34	A Randomized, Double-Blind, Dose Response-Control, Crossover Study to Evaluate the Safety and Efficacy of Two Doses of EUR-1008 in Chronic Pancreatitis (CP) Patients With Exocrine Pancreatic Insufficiency (EPI)	Completed	NCT00788593	Phase 3	Placebo;EUR-1008 (APT-1008) High Dose;EUR-1008 (APT-1008) Low Dose
35	A Randomized, Double-Blind, Placebo-Controlled, Multi-Center, Crossover Study to Evaluate the Effectiveness and Safety of PANCRECARB® MS-16 (Pancrelipase) in Reducing Steatorrhea in Children and Adults With Cystic Fibrosis	Completed	NCT00432861	Phase 3	PANCRECARB® (pancrelipase);Placebo
36	An Open Label, Multicenter Study Evaluating Safety and Tolerability of Creon Micro in Pediatric Subjects From at Least One Month to Less Than Four Years of Age With Pancreatic Exocrine Insufficiency Due to Cystic Fibrosis	Completed	NCT01747330	Phase 3	Pancreatin
37	Efficacy and Safety of Ultrase MT12 in the Control of Steatorrhea in Cystic Fibrosis (CF) and Pancreatic Insufficient (PI) Children Aged 2 to 6 Years Old	Completed	NCT00880100	Phase 3	Ultrase® MT12
38	Efficacy and Safety of Ultrase® MT20 in Improving the Coefficient of Fat Absorption (CFA%) in Children With Cystic Fibrosis (CF) and Pancreatic Insufficiency (PI)	Completed	NCT00513682	Phase 3	Ultrase® MT20
39	Open-label, Multicenter, Randomized, Cross-Over, Equivalence Study to Compare the Efficacy and Safety of Two Strengths of Pancreatin Enteric-Coated Capsules in Pancreatic Exocrine Insufficiency in Chinese Subjects	Recruiting	NCT03450772	Phase 3	Creon® 25000;Creon® 10000
40	A Phase 3, Randomized, Open-Label, Assessor-Blind, Non-Inferiority, Active-Comparator Study Evaluating the Efficacy and Safety of Liprotamase in Subjects With Cystic Fibrosis-Related Exocrine Pancreatic Insufficiency	Active, not recruiting	NCT03051490	Phase 3	Liprotamase;porcine PERT
41	An Open-Label Clinical Study Evaluating the Long Term Safety of ALTU-135 in the Treatment of Patients With Exocrine Pancreatic Insufficiency Due to Chronic Pancreatitis or Pancreatectomy	Terminated	NCT00500084	Phase 3	Liprotamase
42	A Phase 2, Randomized, Double Blind, Parallel Dose Ranging Study of Oral TheraCLEC™ - Total in Cystic Fibrosis Subjects With Exocrine Pancreatic Insufficiency	Completed	NCT00095732	Phase 2	Liprotamase;Placebo
43	A Randomized, Double-blind, Multicenter, Two-period Crossover Study to Assess the Efficacy and Tolerability of NM-BL (Burlulipase) in Patients With Exocrine Pancreatic Insufficiency Due to Cystic Fibrosis	Completed	NCT01710644	Phase 1, Phase 2	Burlulipase;Placebo (Caramel in sterile water)
44	A Multicentre Open-label Phase IIa Study With Escalating Dose of MS1819- SD, to Investigate the Efficacy and Safety of a Yarrowia Lipolytica Lipase Preparation for the Compensation of Exocrine Pancreatic Insufficiency Caused by Chronic Pancreatitis and/or Distal Pancreatectomy	Completed	NCT03481803	Phase 2	MS1819-SD
45	An Open-Label Cross-over Study to Evaluate the Intraduodenal Delivery of Lipase, Protease and Amylase From Administration of VIOKASE16 in Chronic Pancreatitis Subjects With Exocrine Pancreatic Insufficiency (EPI).	Completed	NCT00559052	Phase 2	VIOKASE 16
46	A Phase II, Multicenter, Parallel-Group, Active-Controlled, Randomized, Double-blind, Dose-Ranging Study to Evaluate the Efficacy and Safety of Different Doses of Creon IR in Subjects With Pancreatic Exocrine Insufficiency Due to Cystic Fibrosis	Completed	NCT02415959	Phase 2	Creon IR;Creon® (DR/GR)
47	An Open Label, Exploratory Study on the Effect of rhBSSL on the Fat Absorption in Patients With Cystic Fibrosis and Pancreatic Insufficiency	Completed	NCT00743483	Phase 2	rhBSSL
48	A Two-part, Open-label, Randomised, Crossover, Multicentre, Phase II Study to Investigate the Presence of Pancreatic Exocrine Insufficiency (PEI) in Patients With Type 2 Diabetes Mellitus, and to Investigate the Pharmacokinetics of EPANOVA® and OMACOR® Following a Single Oral Dose in Patients With Different Degrees of PEI	Completed	NCT02370537	Phase 2	Epanova® (omega-3 carboxylic acids);Omacor® (omega-3-acid ethyl esters)
49	Phase II Study: LYM-X-SORB™, an Organized Lipid Matrix: Fatty Acids and Choline in CF	Completed	NCT00406536	Phase 2
50	A Phase 2, Open-Label, Multicenter, 2x2 Crossover Trial to Assess the Safety and Efficacy of MS1819-SD in Patients With Exocrine Pancreatic Insufficiency Due to Cystic Fibrosis	Recruiting	NCT03746483	Phase 2	MS1819-SD;Porcine PERT

Search NIH Clinical Center for Exocrine Pancreatic Insufficiency

Cochrane evidence based reviews: exocrine pancreatic insufficiency

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Genetic Tests for Exocrine Pancreatic Insufficiency

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Anatomical Context for Exocrine Pancreatic Insufficiency

MalaCards organs/tissues related to Exocrine Pancreatic Insufficiency:

⁴⁰

Testes, Pancreas, Liver, Bone, Lung, Bone Marrow, Small Intestine

Data from LifeMap, the Embryonic Development and Stem Cells Database

Cells/anatomical compartments in embryo or adult related to Exocrine Pancreatic Insufficiency:

#	Tissue Anatomical CompartmentCell	Relevance
1	Pancreas Pancreatic Acini Acinar Cells	Affected by disease, potential therapeutic candidate

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Publications for Exocrine Pancreatic Insufficiency

Articles related to Exocrine Pancreatic Insufficiency:

(show top 50) (show all 1145)

#	Title	Authors	PMID	Year
1	13C-labeled mixed triglyceride breath test (13C MTG-BT) in healthy children and children with cystic fibrosis (CF) under pancreatic enzyme replacement therapy (PERT): a pilot study. ⁵⁴ ⁶¹	Herzog DC...Seidman EG	18817766	2008
2	Exocrine pancreatic insufficiency in the Eurasian dog breed--inheritance and exclusion of two candidate genes. ⁵⁴ ⁶¹	Proschowsky HF...Fredholm M	17302791	2007
3	Is exocrine pancreatic insufficiency in adult coeliac disease a cause of persisting symptoms? ⁵⁴ ⁶¹	Leeds JS...Sanders DS	17269988	2007
4	Does secretin-stimulated MRCP predict exocrine pancreatic insufficiency?: A comparison with noninvasive exocrine pancreatic function tests. ⁵⁴ ⁶¹	Schneider AR...Stein J	17016144	2006
5	Homozygous (TG)11 allele in intron 8 of the cystic fibrosis transmembrane conductance regulator (CFTR) gene has a protective role against bicarbonate decrease in pure pancreatic juice among Japanese male alcoholics. ⁵⁴ ⁶¹	Nakamura Y...Maruyama K	15645646	2004
6	[New fecal markers: recent developments and perspectives]. ⁵⁴ ⁶¹	Kapel N...Gobert JG	15550893	2004
7	Low fecal elastase: potentially related to transient small bowel damage resulting from enteric pathogens. ⁵⁴ ⁶¹	Salvatore S...Nespoli L	12604981	2003
8	Faecal elastase 1 concentration is a marker of duodenal enteropathy. ⁵⁴ ⁶¹	Schappi MG...Lindley KJ	11806885	2002
9	[Maldigestion during chronic pancreatitis]. ⁵⁴ ⁶¹	Laugier R...Ville E	11458612	2001
10	Comparison of fecal elastase-1 determination with the secretin-cholecystokinin test in patients with cystic fibrosis. ⁵⁴ ⁶¹	Walkowiak J...Herzig KH	10192202	1999
11	Comparative clinical evaluation of the 13C-mixed triglyceride breath test as an indirect pancreatic function test. ⁵⁴ ⁶¹	Loser C...Folsch UR	9548629	1998
12	13C-starch breath test--comparative clinical evaluation of an indirect pancreatic function test. ⁵⁴ ⁶¹	Loser C...Folsch UR	9106982	1997
13	Intraduodenal free fatty acids rather than triglycerides are responsible for the release of CCK in humans. ⁵⁴ ⁶¹	Guimbaud R...Frexinos J	8981511	1997
14	Immunoreactive elastase I: clinical evaluation of a new noninvasive test of pancreatic function. ⁵⁴ ⁶¹	Stein J...Lembcke B	8595714	1996
15	Maldigestion after total gastrectomy is associated with pancreatic insufficiency. ⁵⁴ ⁶¹	Friess H...Buchler MW	8607504	1996
16	[Measurement of fecal elastase 1 by immunoreactivity: a new indirect test of the pancreatic function]. ⁵⁴ ⁶¹	Stein J...Caspary WF	8761139	1996
17	Lack of accuracy of plasma alpha-amino nitrogen profiles as an indicator of exocrine pancreatic function both after continuous and bolus stimulation of the pancreas with secretin and cholecystokinin-pancreozymin. ⁵⁴ ⁶¹	Lembcke B...Caspary WF	7871858	1994
18	Adenovirus-mediated transfer of human lipase complementary DNA to the gallbladder. ⁵⁴ ⁶¹	Maeda H...Crystal RG	8194711	1994
19	[The diagnostic value of the amino acid absorption test in detection of a disorder of exocrine pancreatic function]. ⁵⁴ ⁶¹	Kemmer TP...Ditschuneit H	1636270	1992
20	Fecal isoamylase activity in patients with pancreatic diseases. ⁵⁴ ⁶¹	Moriyoshi Y...Watanabe S	1704633	1991
21	Inflammatory bowel disease in Shwachman-Diamond syndrome; is there an association? ⁶¹	Nissen LHC...de Jonge HJM	31196706	2020
22	Broadening the phenotypic spectrum of Pearson syndrome: Five new cases and a review of the literature. ⁶¹	Wild KT...Ganetzky RD	31825167	2020
23	Association of multiple patient and disease characteristics with the presence and type of pain in chronic pancreatitis. ⁶¹	Olesen SS...Scandinavian Baltic Pancreatic Club	31314128	2020
24	ACG Clinical Guideline: Chronic Pancreatitis. ⁶¹	Gardner TB...Whitcomb DC	32022720	2020
25	Characterization of all the lipolytic activities in pancreatin and comparison with porcine and human pancreatic juices. ⁶¹	Salhi A...Carriere F	31288050	2020
26	Surgical Management of Solid Pseudopapillary Neoplasms of Pancreas: A Single-Center Experience of 60 Patients. ⁶¹	Lin X...Huang H	31958791	2020
27	Exocrine Pancreatic Insufficiency after Gastrectomy for Cancer Is Not Severe. ⁶¹	DiMagno MJ	30650423	2020
28	Severe forms of Johanson-Blizzard syndrome caused by two novel compound heterozygous variants in UBR1: Clinical manifestations, imaging findings and molecular genetics. ⁶¹	Liu S...Zeng W	31980351	2020
29	Chronic Pancreatitis: Managing a Difficult Disease. ⁶¹	Hart PA...Conwell DL	31764092	2020
30	Histopathology and Feline Pancreatic Lipase Immunoreactivity in Inflammatory, Hyperplastic and Neoplastic Pancreatic Diseases in Cats. ⁶¹	Torner K...Aupperle-Lellbach H	31955805	2020
31	Diagnostic and Therapeutic Management of Post-Gastric Bypass Chronic Diarrhea: a Systematic Review. ⁶¹	Sollier C...Rives-Lange C	31902043	2020
32	Exocrine pancreatic and enterocyte function in patients with advanced pancreatic cancer. ⁶¹	Witvliet-van Nierop JE...Wierdsma NJ	30579666	2019
33	Growth and Nutrition in Cystic Fibrosis. ⁶¹	Brownell JN...Stallings VA	31659726	2019
34	The baseline nutritional status assessed by MUST score has a low accuracy in predicting the risk of hospitalization during follow-up in patients with chronic pancreatitis: A cohort study. ⁶¹	Stigliano S...Capurso G	31926768	2019
35	Acute oxalate nephropathy due to high vitamin C doses and exocrine pancreatic insufficiency. ⁶¹	Fijen L...Weijmer M	31748360	2019
36	In vitro digestion models to assess lipolysis: The impact of the simulated conditions of gastric and intestinal pH, bile salts and digestive fluids. ⁶¹	Calvo-Lerma J...Andres A	31554063	2019
37	The 2019 ESPEN Arvid Wretlind lecture perioperative nutritional and metabolic care: Patient-tailored or organ-specific approach? ⁶¹	Gianotti L...Sandini M	31732291	2019
38	Pancreatic Malnutrition in Children. ⁶¹	Normatov I...Sentongo T	31710363	2019
39	Management of Exocrine Pancreatic Insufficiency in Children. ⁶¹	Sankararaman S...Sferra TJ	31535732	2019
40	Alcohol-related chronic exocrine pancreatic insufficiency: diagnosis and therapeutic management. A proposal for treatment by the Italian Association for the Study of the Pancreas (AISP) and the Italian Society of Alcohology (SIA). ⁶¹	Pezzilli R...Italian Society of Alcohology (SIA)	30938130	2019
41	Secondary hyperoxaluria due to pancreatic insufficiency. ⁶¹	de Martines DGL...G van der Meijden WA	31814577	2019
42	Comparison of pancreas-sparing duodenectomy (PSD) and pancreatoduodenectomy (PD) for the management of duodenal polyposis syndromes. ⁶¹	Walsh RM...Morris-Stiff G	31474487	2019
43	Recurrent Leg Ulcer, Underweight, Short Stature, and Exocrine Pancreatic Insufficiency. ⁶¹	Sun Y...Zhang F	31634262	2019
44	Natural history of SPINK1 germline mutation related-pancreatitis. ⁶¹	Muller N...Rebours V	31628023	2019
45	Role of Fibre in Nutritional Management of Pancreatic Diseases. ⁶¹	Ribichini E...Severi C	31540004	2019
46	Chronic Pancreatitis and Bone Disease. ⁶¹	Barkin JA...Barkin JS	31558406	2019
47	Acute Recurrent and Chronic Pancreatitis as Initial Manifestations of Cystic Fibrosis and Cystic Fibrosis Transmembrane Conductance Regulator-Related Disorders. ⁶¹	Baldwin C...Perito ER	31268981	2019
48	Lower Gastrointestinal Conditions: Malabsorption Syndromes. ⁶¹	Hogue G...Adams R	31411845	2019
49	Prehabilitation in patients undergoing pancreaticoduodenectomy: a randomized controlled trial. ⁶¹	Ausania F...Casal-Nunez E	31232076	2019
50	Risk Factors for Rapid Progression From Acute Recurrent to Chronic Pancreatitis in Children: Report From INSPPIRE. ⁶¹	Liu QY...Uc A	31136562	2019

Sources

Genes for Exocrine Pancreatic Insufficiency

Genes related to Exocrine Pancreatic Insufficiency (0 elite genes):

(show all 22)

- Elite gene

- Cancer Census gene in COSMIC

#	Symbol	Description	Category	Score	Evidence	PubmedIds
1	COX4I2	Cytochrome C Oxidase Subunit 4I2	Protein Coding	34.17	DISEASES inferred ¹⁵ GeneCards inferred via : Disorders Publications Variants (show sections)
2	SCT	Secretin	Protein Coding	23.8	DISEASES inferred ¹⁵ Novoseek inferred ⁵⁴ GeneCards inferred via : Publications (show sections)	1636270 1704633 17016144
3	PDX1	Pancreatic And Duodenal Homeobox 1	Protein Coding	22.55	DISEASES inferred ¹⁵ GeneCards inferred via : Disorders Publications (show sections)
4	CCK	Cholecystokinin	Protein Coding	21.33	DISEASES inferred ¹⁵ Novoseek inferred ⁵⁴ GeneCards inferred via : Publications (show sections)	1636270 7871858 10192202 (more) 8981511 15017660 8607504 17302791 12604981
5	PNLIP	Pancreatic Lipase	Protein Coding	16.46	DISEASES inferred ¹⁵ Novoseek inferred ⁵⁴	11458612 8194711 18817766
6	CELA3B	Chymotrypsin Like Elastase 3B	Protein Coding	16.32	DISEASES inferred ¹⁵ GeneCards inferred via : Publications (show sections)
7	UBR1	Ubiquitin Protein Ligase E3 Component N-Recognin 1	Protein Coding	15.54	DISEASES inferred ¹⁵ GeneCards inferred via : Disorders (show sections)
8	CFTR	CF Transmembrane Conductance Regulator	Protein Coding	15.23	DISEASES inferred ¹⁵ Novoseek inferred ⁵⁴	18493878 12677168 15645646 (more) 19126627
9	PTF1A	Pancreas Associated Transcription Factor 1a	Protein Coding	14.55	DISEASES inferred ¹⁵ GeneCards inferred via : Disorders (show sections)
10	EFL1	Elongation Factor Like GTPase 1	Protein Coding	13.61	DISEASES inferred ¹⁵ GeneCards inferred via : Publications (show sections)
11	SPINK1	Serine Peptidase Inhibitor Kazal Type 1	Protein Coding	13.44	DISEASES inferred ¹⁵ GeneCards inferred via : Publications (show sections)
12	ELANE	Elastase, Neutrophil Expressed	Protein Coding	12.73	DISEASES inferred ¹⁵ Novoseek inferred ⁵⁴	9106982 11806885 9548629 (more) 10192202 15550893 8595714 8761139 17269988
13	CCKAR	Cholecystokinin A Receptor	Protein Coding	11.59	DISEASES inferred ¹⁵ GeneCards inferred via : Publications (show sections)
14	COX4I1	Cytochrome C Oxidase Subunit 4I1	Protein Coding	11.33	DISEASES inferred ¹⁵ GeneCards inferred via : Publications (show sections)
15	CEL	Carboxyl Ester Lipase	Protein Coding	11.28	DISEASES inferred ¹⁵ Novoseek inferred ⁵⁴	2253333
16	TNF	Tumor Necrosis Factor	Protein Coding	10.81	DISEASES inferred ¹⁵ GeneCards inferred via : Publications (show sections)
17	CELA1	Chymotrypsin Like Elastase 1	Protein Coding	9.18	DISEASES inferred ¹⁵
18	SBDS	SBDS Ribosome Maturation Factor	Protein Coding	8.06	DISEASES inferred ¹⁵
19	CTLA4	Cytotoxic T-Lymphocyte Associated Protein 4	Protein Coding	6.98	GeneCards inferred via : Publications (show sections)
20	IGES	Immunoglobulin E Concentration, Serum	Genetic Locus	6.98	GeneCards inferred via : Publications (show sections)
21	IGHE	Immunoglobulin Heavy Constant Epsilon	Protein Coding	6.98	GeneCards inferred via : Publications (show sections)
22	PPY	Pancreatic Polypeptide	Protein Coding	6.84	DISEASES inferred ¹⁵

Sources

Variations for Exocrine Pancreatic Insufficiency

Sources

Expression for Exocrine Pancreatic Insufficiency

Search GEO for disease gene expression data for Exocrine Pancreatic Insufficiency.

Sources

Pathways for Exocrine Pancreatic Insufficiency

Pathways related to Exocrine Pancreatic Insufficiency according to GeneCards Suite gene sharing:

#	Super pathways	Score	Top Affiliating Genes
1	Pancreatic secretion ³⁶	11.18	SCT PNLIP CFTR CELA3B CEL CCKAR
2	Gastric acid production ¹	9.8	SCT CCK

Sources

GO Terms for Exocrine Pancreatic Insufficiency

Cellular components related to Exocrine Pancreatic Insufficiency according to GeneCards Suite gene sharing:

#	Name	GO ID	Score	Top Affiliating Genes
1	extracellular space	GO:0005615	9.28	TNF SPINK1 SCT PNLIP ELANE CELA3B
2	mitochondrial respiratory chain complex IV	GO:0005751	8.96	COX4I2 COX4I1

Biological processes related to Exocrine Pancreatic Insufficiency according to GeneCards Suite gene sharing:

#	Name	GO ID	Score	Top Affiliating Genes
1	embryonic digestive tract development	GO:0048566	9.43	TNF SCT
2	positive regulation of interleukin-8 biosynthetic process	GO:0045416	9.4	TNF ELANE
3	intestinal cholesterol absorption	GO:0030299	9.37	PNLIP CEL
4	mature ribosome assembly	GO:0042256	9.32	SBDS EFL1
5	pancreatic juice secretion	GO:0030157	9.26	SCT CEL
6	lipid digestion	GO:0044241	9.16	PNLIP CEL
7	generation of precursor metabolites and energy	GO:0006091	9.13	PDX1 COX4I2 COX4I1
8	exocrine pancreas development	GO:0031017	8.8	PTF1A PDX1 CELA1

Sources

Sources for Exocrine Pancreatic Insufficiency

¹ BioSystems

² BitterDB

³ CDC

⁴ Cell Signaling Technology

⁵ ClinicalTrials

⁶ ClinVar

⁷ CNVD

⁸ Cochrane Library

⁹ Cosmic

¹⁰ dbSNP

¹¹ DGIdb

¹² Disease Ontology

¹³ diseasecard

¹⁴ DiseaseEnhancer

¹⁵ DISEASES

¹⁶ DrugBank

¹⁷ EFO

¹⁸ ExPASy

¹⁹ FMA

²⁰ GeneAnalytics

²¹ GeneCards

²² GeneGo (Thomson Reuters)

²³ GeneHancer via GeneCards

²⁴ GeneReviews

²⁵ Genetics Home Reference

²⁶ GenomeRNAi

²⁷ GEO DataSets

²⁸ GO

²⁹ GTR

³⁰ HMDB

³¹ HPO

³² ICD10

³³ ICD10 via Orphanet

³⁴ ICD9CM

³⁵ IUPHAR

³⁶ KEGG

³⁷ LifeMap

³⁸ LncRNADisease

³⁹ LOVD

⁴⁰ MalaCards

⁴¹ MedGen

⁴² MedlinePlus

⁴³ MeSH

⁴⁴ MESH via Orphanet

⁴⁵ MGI

⁴⁶ miR2Disease

⁴⁷ NCBI Bookshelf

⁴⁸ NCI

⁴⁹ NCIt

⁵⁰ NDF-RT

⁵¹ NIH Clinical Center

⁵² NIH Rare Diseases

⁵³ NINDS

⁵⁴ Novoseek

⁵⁵ Novus Biologicals

⁵⁶ OMIM

⁵⁷ OMIM via Orphanet

⁵⁸ Orphanet

⁵⁹ PathCards

⁶⁰ PharmGKB

⁶¹ PubMed

⁶² PubMed Health

⁶³ QIAGEN

⁶⁴ R&D Systems

⁶⁵ Reactome

⁶⁶ Sino Biological

⁶⁷ SNOMED-CT

⁶⁸ SNOMED-CT via HPO

⁶⁹ TGDB

⁷⁰ Tocris

⁷¹ UMLS

⁷² UMLS via Orphanet

⁷³ UniProtKB/Swiss-Prot

⁷⁴ Wikipedia