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

MCID: EXC002 MIFTS: 43	Exocrine Pancreatic Insufficiency Categories: Endocrine diseases	Data Licensing For inquiries, contact: [email protected]
Genes Variations Tissues Related diseases Publications 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 ¹¹ ⁵³ ⁵ ⁴³ ¹⁴ ⁷¹ ⁷⁵

Classifications:

MalaCards categories:
Anatomical: Endocrine diseases

See all MalaCards categories (disease lists)

External Ids:

Disease Ontology ¹¹ DOID:13316

MeSH ⁴³ D010188

NCIt ⁴⁹ C84316

SNOMED-CT ⁶⁸ 47367009

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 johanson-blizzard syndrome. An important gene associated with Exocrine Pancreatic Insufficiency is SCT (Secretin), and among its related pathways/superpathways are Embryonic and Induced Pluripotent Stem Cells and Lineage-specific Markers and Type II diabetes mellitus. The drugs Glucagon and Dulaglutide have been mentioned in the context of this disorder. Affiliated tissues include Pancreas, liver and bone marrow, and related phenotypes are homeostasis/metabolism and liver/biliary system

Wikipedia: ⁷⁵ 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 216)

#	Related Disease	Score	Top Affiliating Genes
1	shwachman-diamond syndrome 1	32.1	SBDS ELANE EFL1
2	johanson-blizzard syndrome	32.0	UBR1 SBDS CEL
3	pancreatitis, hereditary	31.5	SPINK1 SCT CFTR CCK
4	cystic fibrosis	31.2	TNF SPINK1 SCT PNLIP ELANE CFTR
5	diarrhea	31.0	TNF SCT CFTR CCK
6	celiac disease 1	30.6	TNF SCT CTLA4 CELA3B CELA1 CCK
7	maturity-onset diabetes of the young, type 8, with exocrine dysfunction	30.4	PDX1 CEL
8	pancreatic steatorrhea	30.4	SCT PNLIP CELA3B CELA1
9	pancreas, annular	30.3	SPINK1 PTF1A
10	alcoholic pancreatitis	30.3	SPINK1 SCT CFTR CCK
11	bowel dysfunction	30.2	TNF CFTR CCK
12	type 2 diabetes mellitus	30.1	TNF SPINK1 SCT PNLIP PDX1 CCKAR
13	alpha-1-antitrypsin deficiency	30.0	TNF ELANE CFTR
14	acute pancreatitis	30.0	TNF SPINK1 SCT PNLIP ELANE CFTR
15	bronchiectasis	30.0	TNF ELANE CFTR
16	gastroesophageal reflux	30.0	TNF ELANE CCKAR CCK
17	gallbladder disease	30.0	CFTR CCKAR CCK
18	primary biliary cholangitis	30.0	TNF SCT CTLA4 CFTR
19	maturity-onset diabetes of the young	29.9	PTF1A PDX1 CFTR CEL
20	pancreatitis	29.9	UBR1 TNF SPINK1 SCT PNLIP ELANE
21	autoimmune pancreatitis	29.7	SPINK1 SCT CFTR CELA3B CELA1
22	diabetes mellitus	29.4	TNF SPINK1 SCT PTF1A PNLIP PDX1
23	exocrine pancreatic insufficiency, dyserythropoietic anemia, and calvarial hyperostosis	11.8
24	poikiloderma, hereditary fibrosing, with tendon contractures, myopathy, and pulmonary fibrosis	11.4
25	pancreatic agenesis 1	11.2
26	pancreatic agenesis 2	11.2
27	neurologic, endocrine, and pancreatic disease, multisystem, infantile-onset 1	11.2
28	neutropenia, severe congenital, 8, autosomal dominant	11.2
29	nutritional deficiency disease	10.6
30	overgrowth syndrome	10.6
31	vitamin b12 deficiency	10.5
32	type 1 diabetes mellitus	10.5
33	irritable bowel syndrome	10.4
34	alcohol dependence	10.4
35	pancreatic cancer	10.4
36	rickets	10.4
37	keratomalacia	10.4
38	neutropenia	10.4
39	gastrointestinal system disease	10.4
40	heart defects, congenital, and other congenital anomalies	10.3	PTF1A PDX1
41	alcohol use disorder	10.3
42	dysostosis	10.3
43	hypoglycemia	10.3
44	duodenal obstruction	10.3	SPINK1 CCK
45	functionless pituitary adenoma	10.3	CCKAR CCK
46	gastroduodenal crohn's disease	10.3	TNF CCK
47	chronic orbital inflammation	10.3	TNF CTLA4
48	common bile duct disease	10.2	SPINK1 CELA3B CCK
49	granulomatous hepatitis	10.2	TNF CTLA4
50	pyle disease	10.2

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

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Symptoms & Phenotypes for Exocrine Pancreatic Insufficiency

GenomeRNAi Phenotypes related to Exocrine Pancreatic Insufficiency according to GeneCards Suite gene sharing:

²⁵

#	Description	GenomeRNAi Source Accession	Score	Top Affiliating Genes
1	no effect	GR00402-S-1	10.19	CCK CCKAR CEL CELA1 CELA3B CFTR
2	no effect	GR00402-S-2	10.19	CCK CEL CELA1 CELA3B CFTR COX4I1

MGI Mouse Phenotypes related to Exocrine Pancreatic Insufficiency:

⁴⁵

#	Description	MGI Source Accession	Score	Top Affiliating Genes
1	homeostasis/metabolism	MP:0005376	10.16	CCK CCKAR CEL CFTR COX4I2 CTLA4
2	liver/biliary system	MP:0005370	9.97	CCKAR CEL CFTR CTLA4 PDX1 PNLIP
3	no phenotypic analysis	MP:0003012	9.91	CCK CFTR PDX1 PIEZO2 PTF1A SPINK1
4	endocrine/exocrine gland	MP:0005379	9.85	CCK CCKAR CFTR CTLA4 PDX1 PTF1A
5	digestive/alimentary	MP:0005381	9.7	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 48)

Name

Status

Phase

Clinical Trials

Cas Number

PubChem Id

Glucagon

Approved

Phase 2

16941-32-5

16133228 16186314

Dulaglutide

Approved, Investigational

Phase 2

923950-08-7

Esomeprazole

Approved, Investigational, Vet_approved

Phase 2

73590-58-6, 119141-88-7

9568614 4594

Lecithin

Approved, Experimental, Investigational, Nutraceutical

Phase 2

8002-43-5, 1446756-47-3, 18656-38-7

26197 9547096 6323481

Choline

Approved, Nutraceutical

Phase 2

62-49-7

305

Bile Acids and Salts

Phase 2

Omega 3 Fatty Acid

Phase 2

Flax

Phase 2

Soy Bean

Phase 2

Sunflower

Phase 2

Insulin, Globin Zinc

Phase 2

Insulin

Phase 2

Hypoglycemic Agents

Phase 2

Glucagon-Like Peptide 1

Phase 2

Anti-Ulcer Agents

Phase 2

Antacids

Phase 2

Proton Pump Inhibitors

Phase 2

Phylloquinone

Approved, Investigational

Phase 1

84-80-0

5284607

Menadione

Approved, Nutraceutical

Phase 1

58-27-5

4055

Menaquinone

Investigational

Phase 1

1182-68-9

Vitamins

Phase 1

Vitamin K

Phase 1

Trace Elements

Phase 1

Antifibrinolytic Agents

Phase 1

Hemostatics

Phase 1

Naphthoquinone

Phase 1

Micronutrients

Phase 1

Coagulants

Phase 1

Carbamide peroxide

Approved

124-43-6

Repaglinide

Approved, Investigational

135062-02-1

65981

Flurbiprofen

Approved, Investigational

5104-49-4

3394

Acarbose

Approved, Investigational

56180-94-0

441184 444254

Metformin

Approved

1115-70-4, 657-24-9

4091

Remifentanil

Approved

132875-61-7

60815

Diazepam

Approved, Illicit, Investigational, Vet_approved

439-14-5

3016

Benzocaine

Approved, Investigational

1994-09-7, 94-09-7

2337

Tannic acid

Approved

1401-55-4

16129878 16129778

Gastric inhibitory polypeptide

Investigational

100040-31-1

Sitagliptin Phosphate

654671-77-9

HIV Protease Inhibitors

Dipeptidyl-Peptidase IV Inhibitors

protease inhibitors

Pharmaceutical Solutions

Cardiac Glycosides

Glycoside Hydrolase Inhibitors

Incretins

bilirubin

635-65-4

5280352

Liver Extracts

Interventional clinical trials:

(show top 50) (show all 98)

#	Name	Status	NCT ID	Phase	Drugs
1	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
2	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)
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	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
5	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
6	A Phase 4 Study to Compare US Marketed Creon Drug Product With Drug Product Manufactured With a Modernized Process at an Alternate Manufacturing Site and With Drug Product Manufactured With the Approved Manufacturing Process at an Alternate Active Pharmaceutical Ingredient Site, in Subjects With EPI Due to Cystic Fibrosis	Completed	NCT03924947	Phase 4	Pancrelipase
7	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
8	MALABSORPTION BLOOD TEST: Toward a Novel Approach to Quantify Steatorrhea	Completed	NCT00572975	Phase 4
9	A Phase 4 Study to Assess Symptoms of Exocrine Pancreatic Insufficiency in Subjects With Cystic Fibrosis or Chronic Pancreatitis Treated With Creon® (Pancrelipase) With an Alternate Source of Active Pharmaceutical Ingredient	Recruiting	NCT05069597	Phase 4	CREON
10	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	Terminated	NCT03859869	Phase 4	Pancrelipase;Placebo
11	Intraduodenal Aspiration Study to Assess the Bioavailability of Oral Pancrecarb® Compared to Placebo Control in Patients With Pancreatic Insufficiency	Terminated	NCT00744250	Phase 4	Pancrelipase
12	Randomized, Double Blind, Placebo-controlled Trial of Creon in Patients With Low Faecal Pancreatic Elastase	Terminated	NCT01865695	Phase 4	Creon
13	Exocrine Pancreatic Insufficiency in Pancreatic Cancer: Evaluating the Need and Efficacy of Pancreatic Enzyme Replacement Therapy	Withdrawn	NCT01401387	Phase 4
14	A Phase 4 Open-Label Single-Arm Study To Evaluate The Use Of CREON In Subjects With EPI Due To Etiologies Other Than Cystic Fibrosis, Chronic Pancreatitis, Pancreatectomy, Or Pancreatic Cancer	Withdrawn	NCT04315311	Phase 4	CREON
15	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
16	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	Unknown status	NCT03051490	Phase 3	Liprotamase;porcine PERT
17	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
18	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
19	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
20	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
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	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
23	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
24	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
25	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
26	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)
27	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
28	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
29	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;Placebo
30	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
31	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
32	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
33	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
34	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
35	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
36	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
37	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
38	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
39	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
40	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
41	Recurrent Abdominal Pain and Exocrine Pancreatic Insufficiency	Not yet recruiting	NCT04231279	Phase 3	synthetic human secretin
42	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
43	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	Completed	NCT03746483	Phase 2	MS1819;Porcine PERT
44	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
45	OPTION 2: A Phase 2, Open-Label, Multicenter, 2x2 Crossover Trial to Assess the Safety and Efficacy of MS1819 in Enteric Capsules in Patients With Exocrine Pancreatic Insufficiency Due to Cystic Fibrosis; With an Extension Phase Evaluation of Immediate Release MS1819 Capsules	Completed	NCT04375878	Phase 2	MS1819;Porcine PERT
46	A Multicenter, Open-label Phase 2 Study With Escalating Doses of MS1819-SD on Top of a Stable Dose of PPEs, to Investigate the Efficacy and Safety of This Combination for the Compensation of Severe Exocrine Pancreatic Insufficiency in CF Patients Not Fully Compensated With Only PPEs	Completed	NCT04302662	Phase 2	MS1819-SD
47	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
48	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)
49	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
50	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

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

Organs/tissues related to Exocrine Pancreatic Insufficiency:

MalaCards : Pancreas, Liver, Bone Marrow, Small Intestine, Bone, Kidney, Brain

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 1348)

#	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. ⁵³ ⁶²	Herzog DC...Seidman EG	18817766	2008
2	Exocrine pancreatic insufficiency in the Eurasian dog breed--inheritance and exclusion of two candidate genes. ⁵³ ⁶²	Proschowsky HF...Fredholm M	17302791	2007
3	Is exocrine pancreatic insufficiency in adult coeliac disease a cause of persisting symptoms? ⁵³ ⁶²	Leeds JS...Sanders DS	17269988	2007
4	Does secretin-stimulated MRCP predict exocrine pancreatic insufficiency?: A comparison with noninvasive exocrine pancreatic function tests. ⁵³ ⁶²	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. ⁵³ ⁶²	Nakamura Y...Maruyama K	15645646	2004
6	[New fecal markers: recent developments and perspectives]. ⁵³ ⁶²	Kapel N...Gobert JG	15550893	2004
7	Low fecal elastase: potentially related to transient small bowel damage resulting from enteric pathogens. ⁵³ ⁶²	Salvatore S...Nespoli L	12604981	2003
8	Faecal elastase 1 concentration is a marker of duodenal enteropathy. ⁵³ ⁶²	Schappi MG...Lindley KJ	11806885	2002
9	[Maldigestion during chronic pancreatitis]. ⁵³ ⁶²	Laugier R...Ville E	11458612	2001
10	Comparison of fecal elastase-1 determination with the secretin-cholecystokinin test in patients with cystic fibrosis. ⁵³ ⁶²	Walkowiak J...Herzig KH	10192202	1999
11	Comparative clinical evaluation of the 13C-mixed triglyceride breath test as an indirect pancreatic function test. ⁵³ ⁶²	Loser C...Folsch UR	9548629	1998
12	13C-starch breath test--comparative clinical evaluation of an indirect pancreatic function test. ⁵³ ⁶²	Loser C...Folsch UR	9106982	1997
13	Intraduodenal free fatty acids rather than triglycerides are responsible for the release of CCK in humans. ⁵³ ⁶²	Guimbaud R...Frexinos J	8981511	1997
14	Immunoreactive elastase I: clinical evaluation of a new noninvasive test of pancreatic function. ⁵³ ⁶²	Stein J...Lembcke B	8595714	1996
15	Maldigestion after total gastrectomy is associated with pancreatic insufficiency. ⁵³ ⁶²	Friess H...Buchler MW	8607504	1996
16	[Measurement of fecal elastase 1 by immunoreactivity: a new indirect test of the pancreatic function]. ⁵³ ⁶²	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. ⁵³ ⁶²	Lembcke B...Caspary WF	7871858	1994
18	Adenovirus-mediated transfer of human lipase complementary DNA to the gallbladder. ⁵³ ⁶²	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]. ⁵³ ⁶²	Kemmer TP...Ditschuneit H	1636270	1992
20	Fecal isoamylase activity in patients with pancreatic diseases. ⁵³ ⁶²	Moriyoshi Y...Watanabe S	1704633	1991
21	Bone metabolism and incretin hormones following glucose ingestion in young adults with pancreatic insufficient cystic fibrosis. ⁶²	Lei WS...Kindler JM	36110921	2022
22	Impact of recurrent acute pancreatitis on the natural history and progression to chronic pancreatitis. ⁶²	Shah I...Sheth SG	36150985	2022
23	Adar1 deletion causes degeneration of the exocrine pancreas via Mavs-dependent interferon signaling. ⁶²	Rupani DN...McAllister F	36458554	2022
24	Overcoming the Pitfalls of Next-Generation Sequencing-Based Molecular Diagnosis of Shwachman-Diamond Syndrome. ⁶²	Peng X...Lu Y	36162759	2022
25	Exocrine Pancreatic Insufficiency is Undiagnosed in Some Patients with Diarrhea-Predominant Irritable Bowel Syndrome Using the Rome IV Criteria. ⁶²	Olmos JI...Olmos JA	35704255	2022
26	Undiagnosed Exocrine Pancreatic Insufficiency in Diarrhea-Predominant Irritable Bowel Syndrome. ⁶²	Lee AA...McNabb-Baltar J	35705731	2022
27	The clinical and laboratory impact of reporting faecal elastase-1 in watery stool samples. ⁶²	Jones AM	36424839	2022
28	In vitro evaluation of the gastrointestinal delivery of acid-sensitive pancrelipase in a next generation enteric capsule using an exocrine pancreatic insufficiency disease model. ⁶²	Jannin V...Madit N	36442722	2022
29	Community Perspective on Pancreatic Enzyme Replacement Therapy in Cystic Fibrosis. ⁶²	Borowitz D...Gallotto D	36070542	2022
30	Assessment of Exocrine Pancreatic Function Following Bariatric/Metabolic Surgery: a Prospective Cohort Study. ⁶²	Cicek Okuyan G...Akkus D	36437437	2022
31	Minimally invasive versus open central pancreatectomy: Systematic review and meta-analysis. ⁶²	Farrarons SS...Dokmak S	35987787	2022
32	Exocrine pancreatic insufficiency after bariatric surgery. ⁶²	Kwon JY...Bi Y	35931645	2022
33	Risk factors for exocrine pancreatic insufficiency after pancreatic surgery: a systematic review and meta-analysis. ⁶²	Budipramana VS...Putra AJ	36384688	2022
34	Analysis of INSPPIRE-2 Cohort: Risk Factors and Disease Burden in Children With Acute Recurrent or Chronic Pancreatitis. ⁶²	Uc A...Consortium for the Study of Chronic Pancreatitis, Diabetes and Pancreatic Cancer	35976273	2022
35	Regional gastrointestinal transit times in patients with chronic pancreatitis. ⁶²	Larsen IM...Drewes AM	36253998	2022
36	Meconium Ileus, Distal Intestinal Obstruction Syndrome, and Other Gastrointestinal Pathology in the Cystic Fibrosis Patient. ⁶²	Tobias J...Fialkowski E	36209752	2022
37	Responses of Ileal and Fecal Microbiota to Withdrawal of Pancreatic Enzyme Replacement Therapy in a Porcine Model of Exocrine Pancreatic Insufficiency. ⁶²	Hankel J...Vital M	36233002	2022
38	Pancreas-preserving total duodenectomy for advanced duodenal polyposis in patients with familial adenomatous polyposis: short and long-term outcomes. ⁶²	Aelvoet AS...Dekker E	35568653	2022
39	Experience in the resection of the uncinate process of the pancreas: indications and results. literature review. ⁶²	Bejarano N...Fabregat J	36283601	2022
40	Long-term changes of pancreatic function in patients with complicated walled-off necrosis. ⁶²	Nojgaard C...Novovic S	35546222	2022
41	Prescription patterns of pancreatic enzyme replacement therapy for patients with pancreatic cancer in the United States. ⁶²	Jain T...Dudeja V	35717430	2022
42	Fetal Description of the Pancreatic Agenesis and Holoprosencephaly Syndrome Associated to a Specific CNOT1 Variant. ⁶²	Cospain A...Dubourg C	35481434	2022
43	Effect of oral or injectable supplementation with cobalamin in dogs with hypocobalaminemia caused by chronic enteropathy or exocrine pancreatic insufficiency. ⁶²	Chang CH...Steiner JM	36054643	2022
44	[Clinical phenotypes and genotypic spectrum of cystic fibrosis with pancreatic insufficiency in children]. ⁶²	Guo ZY...Qian LL	36038301	2022
45	Pancreatic involvement due to immune checkpoint inhibitors: a proposed classification. ⁶²	Ashkar M...McWilliams RR	36161510	2022
46	CFTR regulates brown adipocyte thermogenesis via the cAMP/PKA signaling pathway. ⁶²	Choi KM...Yoon JC	36088207	2022
47	Ptf1a function and transcriptional cis-regulation, a cornerstone in vertebrate pancreas development. ⁶²	Duque M...Bessa J	34125483	2022
48	Exocrine pancreatic insufficiency and graft-versus-host disease. ⁶²	Lopez Cardona J...Albillos A	36148693	2022
49	Morphological and functional recovery following acute and acute recurrent pancreatitis in children: A prospective sequential 2-point evaluation. ⁶²	Anushree N...Verma S	35717306	2022
50	Emerging medicines to improve the basic defect in cystic fibrosis. ⁶²	Fajac I...Sermet-Gaudelus I	35731915	2022

Sources

Genes for Exocrine Pancreatic Insufficiency

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

(show all 24)

- Elite gene

- Cancer Census gene in COSMIC

#	Symbol	Description	Category	Score	Evidence	PubMed IDs
1	SCT	Secretin	Protein Coding	25.02	DISEASES inferred ¹⁴ Novoseek inferred ⁵³ GeneCards inferred via : Publications (show sections)	1636270 1704633 17016144
2	PIEZO2	Piezo Type Mechanosensitive Ion Channel Component 2	Protein Coding	25	Likely pathogenic ⁵
3	COX4I2	Cytochrome C Oxidase Subunit 4I2	Protein Coding	22.9	DISEASES inferred ¹⁴ GeneCards inferred via : Disorders Publications (show sections)
4	PDX1	Pancreatic And Duodenal Homeobox 1	Protein Coding	22.77	DISEASES inferred ¹⁴ GeneCards inferred via : Disorders Publications (show sections)
5	CCK	Cholecystokinin	Protein Coding	22.62	DISEASES inferred ¹⁴ Novoseek inferred ⁵³ GeneCards inferred via : Publications (show sections)	1636270 7871858 10192202 (more) 8981511 15017660 8607504 17302791 12604981
6	CELA3B	Chymotrypsin Like Elastase 3B	Protein Coding	17.45	DISEASES inferred ¹⁴ GeneCards inferred via : Publications (show sections)
7	PNLIP	Pancreatic Lipase	Protein Coding	16.45	DISEASES inferred ¹⁴ Novoseek inferred ⁵³	11458612 8194711 18817766
8	UBR1	Ubiquitin Protein Ligase E3 Component N-Recognin 1	Protein Coding	16.03	DISEASES inferred ¹⁴ GeneCards inferred via : Disorders (show sections)
9	EFL1	Elongation Factor Like GTPase 1	Protein Coding	15.44	DISEASES inferred ¹⁴ GeneCards inferred via : Publications (show sections)
10	SPINK1	Serine Peptidase Inhibitor Kazal Type 1	Protein Coding	15.31	DISEASES inferred ¹⁴ GeneCards inferred via : Publications (show sections)
11	CFTR	CF Transmembrane Conductance Regulator	Protein Coding	15.1	DISEASES inferred ¹⁴ Novoseek inferred ⁵³	18493878 12677168 15645646 (more) 19126627
12	PTF1A	Pancreas Associated Transcription Factor 1a	Protein Coding	14.77	DISEASES inferred ¹⁴ GeneCards inferred via : Disorders (show sections)
13	ELANE	Elastase, Neutrophil Expressed	Protein Coding	12.53	DISEASES inferred ¹⁴ Novoseek inferred ⁵³	9106982 11806885 9548629 (more) 10192202 15550893 8595714 8761139 17269988
14	TNF	Tumor Necrosis Factor	Protein Coding	12.15	DISEASES inferred ¹⁴ GeneCards inferred via : Publications (show sections)
15	COX4I1	Cytochrome C Oxidase Subunit 4I1	Protein Coding	11.49	DISEASES inferred ¹⁴ GeneCards inferred via : Publications (show sections)
16	CCKAR	Cholecystokinin A Receptor	Protein Coding	11.46	DISEASES inferred ¹⁴ GeneCards inferred via : Publications (show sections)
17	CEL	Carboxyl Ester Lipase	Protein Coding	11.26	DISEASES inferred ¹⁴ Novoseek inferred ⁵³	2253333
18	CTLA4	Cytotoxic T-Lymphocyte Associated Protein 4	Protein Coding	11.2	DISEASES inferred ¹⁴ GeneCards inferred via : Publications (show sections)
19	CELA1	Chymotrypsin Like Elastase 1	Protein Coding	9.1	DISEASES inferred ¹⁴
20	SBDS	SBDS Ribosome Maturation Factor	Protein Coding	8.02	DISEASES inferred ¹⁴
21	IGES	Immunoglobulin E Concentration, Serum	Genetic Locus	7.87	GeneCards inferred via : Publications (show sections)
22	IGHE	Immunoglobulin Heavy Constant Epsilon	Protein Coding	7.87	GeneCards inferred via : Publications (show sections)
23	PRSS1	Serine Protease 1	Protein Coding	6.98	DISEASES inferred ¹⁴
24	EIF6	Eukaryotic Translation Initiation Factor 6	Protein Coding	6.87	DISEASES inferred ¹⁴

Sources

Variations for Exocrine Pancreatic Insufficiency

ClinVar genetic disease variations for Exocrine Pancreatic Insufficiency:

⁵

#	Gene	Name	Type	Significance	ClinVarId	dbSNP ID	Position
1	PIEZO2	NM_001378183.1(PIEZO2):c.64+1G>A	SNV	Likely Pathogenic	1683711		GRCh37: 18:11148523-11148523 GRCh38: 18:11148524-11148524
2	CTR9	NM_014633.5(CTR9):c.85G>A (p.Glu29Lys)	SNV	Uncertain Significance	1707508		GRCh37: 11:10774258-10774258 GRCh38: 11:10752711-10752711

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	Embryonic and Induced Pluripotent Stem Cells and Lineage-specific Markers ⁶⁵	11.56	PTF1A PDX1 CFTR
2	Type II diabetes mellitus ⁷⁴	10.66	TNF PDX1
3	Genetic causes of porto-sinusoidal vascular disease ⁷⁴	10.37	SBDS EFL1 COX4I2
4	PTF1A related regulatory pathway ⁷⁴	10.2	PTF1A PDX1
5	Gastric acid production ⁷⁴	9.91	SCT CCK

Sources

GO Terms for Exocrine Pancreatic Insufficiency

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

#	Name	GO ID	Score	Top Affiliating Genes
1	generation of precursor metabolites and energy	GO:0006091	9.8	PDX1 COX4I2 COX4I1
2	intestinal cholesterol absorption	GO:0030299	9.71	PNLIP CEL
3	pancreatic juice secretion	GO:0030157	9.67	SCT CEL
4	cytosolic ribosome assembly	GO:0042256	9.56	SBDS EFL1
5	response to nutrient levels	GO:0031667	9.5	TNF SPINK1 SCT PDX1
6	positive regulation of pancreatic juice secretion	GO:0090187	9.46	SPINK1 SCT
7	exocrine pancreas development	GO:0031017	9.1	PTF1A PDX1 CELA1