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Chronic pancreatitis

MedGen UID:
101753
Concept ID:
C0149521
Disease or Syndrome
Synonyms: Chronic Pancreatitis; Pancreatitis, Chronic
SNOMED CT: Chronic pancreatitis (235494005); CP - Chronic pancreatitis (235494005)
 
HPO: HP:0006280
Monarch Initiative: MONDO:0005003
OMIM®: 167790

Definition

A chronic form of pancreatitis. [from HPO]

Term Hierarchy

Conditions with this feature

Inborn glycerol kinase deficiency
MedGen UID:
82803
Concept ID:
C0268418
Disease or Syndrome
NR0B1-related adrenal hypoplasia congenita includes both X-linked adrenal hypoplasia congenita (X-linked AHC) and Xp21 deletion (previously called complex glycerol kinase deficiency). X-linked AHC is characterized by primary adrenal insufficiency and/or hypogonadotropic hypogonadism (HH). Adrenal insufficiency is acute infantile onset (average age 3 weeks) in approximately 60% of affected males and childhood onset (ages 1-9 years) in approximately 40%. HH typically manifests in a male with adrenal insufficiency as delayed puberty (i.e., onset age >14 years) and less commonly as arrested puberty at about Tanner Stage 3. Rarely, X-linked AHC manifests initially in early adulthood as delayed-onset adrenal insufficiency, partial HH, and/or infertility. Heterozygous females very occasionally have manifestations of adrenal insufficiency or hypogonadotropic hypogonadism. Xp21 deletion includes deletion of NR0B1 (causing X-linked AHC) and GK (causing glycerol kinase deficiency), and in some cases deletion of DMD (causing Duchenne muscular dystrophy). Developmental delay has been reported in males with Xp21 deletion when the deletion extends proximally to include DMD or when larger deletions extend distally to include IL1RAPL1 and DMD.
Phosphate transport defect
MedGen UID:
87455
Concept ID:
C0342749
Disease or Syndrome
Glycogenosis due to glucose-6-phosphatase deficiency (G6P) type b, or glycogen storage disease (GSD) type 1b, is a type of glycogenosis due to G6P deficiency (see this term).
Tropical pancreatitis
MedGen UID:
334069
Concept ID:
C1842402
Disease or Syndrome
Tropical calcific pancreatitis (TCP) is a type of chronic pancreatitis unique to tropical regions. Large intraductal calculi are present, and evidence of pancreatic calcification is seen on imaging studies (summary by Mahurkar et al., 2006).
Neutral lipid storage myopathy
MedGen UID:
339913
Concept ID:
C1853136
Disease or Syndrome
Neutral lipid storage disease with myopathy (NLSDM) is an autosomal recessive muscle disorder characterized by adult onset of slowly progressive proximal muscle weakness affecting the upper and lower limbs and associated with increased serum creatine kinase; distal muscle weakness may also occur. About half of patients develop cardiomyopathy later in the disease course. Other variable features include diabetes mellitus, hepatic steatosis, hypertriglyceridemia, and possibly sensorineural hearing loss. Leukocytes and muscle cells show cytoplasmic accumulation of triglycerides (summary by Reilich et al., 2011). Neutral lipid storage disease with myopathy belongs to a group of disorders termed neutral lipid storage disorders (NLSDs). These disorders are characterized by the presence of triglyceride-containing cytoplasmic droplets in leukocytes and in other tissues, including bone marrow, skin, and muscle. Chanarin-Dorfman syndrome (CDS; 275630) is defined as NLSD with ichthyosis (NLSDI). Patients with NLSDM present with myopathy but without ichthyosis (summary by Fischer et al., 2007).
Nephronophthisis-like nephropathy 1
MedGen UID:
461769
Concept ID:
C3150419
Disease or Syndrome
Nephronophthisis-like nephropathy-1 (NPHPL1) is an autosomal recessive cystic kidney disease characterized by the onset of progressive renal insufficiency in childhood. End-stage renal disease occurs in the first 3 decades of life. The disorder may be associated with extrarenal manifestations, including hepatic and central nervous system involvement (summary by O'Toole et al., 2010). For a general phenotypic description and a discussion of genetic heterogeneity of nephronophthisis, see NPHP1 (256100).

Professional guidelines

PubMed

Szatmary P, Grammatikopoulos T, Cai W, Huang W, Mukherjee R, Halloran C, Beyer G, Sutton R
Drugs 2022 Aug;82(12):1251-1276. Epub 2022 Sep 8 doi: 10.1007/s40265-022-01766-4. PMID: 36074322Free PMC Article
Beyer G, Hoffmeister A, Lorenz P, Lynen P, Lerch MM, Mayerle J
Dtsch Arztebl Int 2022 Jul 25;119(29-30):495-501. doi: 10.3238/arztebl.m2022.0223. PMID: 35945698Free PMC Article
Zhao Z, Liu W
Technol Cancer Res Treat 2020 Jan-Dec;19:1533033820962117. doi: 10.1177/1533033820962117. PMID: 33357065Free PMC Article

Curated

UK NICE Guideline NG104, Pancreatitis, 2020

Recent clinical studies

Etiology

Hines OJ, Pandol SJ
BMJ 2024 Feb 26;384:e070920. doi: 10.1136/bmj-2023-070920. PMID: 38408777
Beyer G, Habtezion A, Werner J, Lerch MM, Mayerle J
Lancet 2020 Aug 15;396(10249):499-512. doi: 10.1016/S0140-6736(20)31318-0. PMID: 32798493
Arvanitakis M, Ockenga J, Bezmarevic M, Gianotti L, Krznarić Ž, Lobo DN, Löser C, Madl C, Meier R, Phillips M, Rasmussen HH, Van Hooft JE, Bischoff SC
Clin Nutr 2020 Mar;39(3):612-631. Epub 2020 Jan 22 doi: 10.1016/j.clnu.2020.01.004. PMID: 32008871
Hart PA, Conwell DL
Am J Gastroenterol 2020 Jan;115(1):49-55. doi: 10.14309/ajg.0000000000000421. PMID: 31764092Free PMC Article
Gupte A, Goede D, Tuite R, Forsmark CE
BMJ 2018 Jun 7;361:k2126. doi: 10.1136/bmj.k2126. PMID: 29880587

Diagnosis

Hines OJ, Pandol SJ
BMJ 2024 Feb 26;384:e070920. doi: 10.1136/bmj-2023-070920. PMID: 38408777
Maatman TK, Zyromski NJ
Curr Probl Surg 2021 Mar;58(3):100858. Epub 2020 Jul 10 doi: 10.1016/j.cpsurg.2020.100858. PMID: 33663691
Beyer G, Habtezion A, Werner J, Lerch MM, Mayerle J
Lancet 2020 Aug 15;396(10249):499-512. doi: 10.1016/S0140-6736(20)31318-0. PMID: 32798493
Hart PA, Conwell DL
Am J Gastroenterol 2020 Jan;115(1):49-55. doi: 10.14309/ajg.0000000000000421. PMID: 31764092Free PMC Article
Gupte A, Goede D, Tuite R, Forsmark CE
BMJ 2018 Jun 7;361:k2126. doi: 10.1136/bmj.k2126. PMID: 29880587

Therapy

Arvanitakis M, Ockenga J, Bezmarevic M, Gianotti L, Krznarić Ž, Lobo DN, Löser C, Madl C, Meier R, Phillips M, Rasmussen HH, Van Hooft JE, Bischoff SC
Clin Nutr 2024 Feb;43(2):395-412. Epub 2023 Dec 27 doi: 10.1016/j.clnu.2023.12.019. PMID: 38169174
Gupte A, Goede D, Tuite R, Forsmark CE
BMJ 2018 Jun 7;361:k2126. doi: 10.1136/bmj.k2126. PMID: 29880587
Yang D, Forsmark CE
Curr Opin Gastroenterol 2017 Sep;33(5):396-403. doi: 10.1097/MOG.0000000000000377. PMID: 28771447
Majumder S, Chari ST
Lancet 2016 May 7;387(10031):1957-66. Epub 2016 Mar 2 doi: 10.1016/S0140-6736(16)00097-0. PMID: 26948434
Kocher HM
Am Fam Physician 2008 Mar 1;77(5):661-2. PMID: 18350765

Prognosis

Petrov MS, Yadav D
Nat Rev Gastroenterol Hepatol 2019 Mar;16(3):175-184. doi: 10.1038/s41575-018-0087-5. PMID: 30482911Free PMC Article
Xiao AY, Tan ML, Wu LM, Asrani VM, Windsor JA, Yadav D, Petrov MS
Lancet Gastroenterol Hepatol 2016 Sep;1(1):45-55. Epub 2016 Jun 28 doi: 10.1016/S2468-1253(16)30004-8. PMID: 28404111
Kocher HM
Am Fam Physician 2008 Mar 1;77(5):661-2. PMID: 18350765
Schneider A, Singer MV
Dig Dis 2005;23(3-4):222-31. doi: 10.1159/000090169. PMID: 16508286
Fernández-Cruz L, Margarona E, Llovera J, López-Boado MA, Saenz H
Hepatogastroenterology 1993 Apr;40(2):150-4. PMID: 8509046

Clinical prediction guides

Beyer G, Habtezion A, Werner J, Lerch MM, Mayerle J
Lancet 2020 Aug 15;396(10249):499-512. doi: 10.1016/S0140-6736(20)31318-0. PMID: 32798493
Issa Y, Kempeneers MA, Bruno MJ, Fockens P, Poley JW, Ahmed Ali U, Bollen TL, Busch OR, Dejong CH, van Duijvendijk P, van Dullemen HM, van Eijck CH, van Goor H, Hadithi M, Haveman JW, Keulemans Y, Nieuwenhuijs VB, Poen AC, Rauws EA, Tan AC, Thijs W, Timmer R, Witteman BJ, Besselink MG, van Hooft JE, van Santvoort HC, Dijkgraaf MG, Boermeester MA; Dutch Pancreatitis Study Group
JAMA 2020 Jan 21;323(3):237-247. doi: 10.1001/jama.2019.20967. PMID: 31961419Free PMC Article
Gutta A, Fogel E, Sherman S
Expert Rev Gastroenterol Hepatol 2019 Nov;13(11):1089-1105. Epub 2019 Nov 8 doi: 10.1080/17474124.2019.1685871. PMID: 31663403Free PMC Article
Laterza L, Scaldaferri F, Bruno G, Agnes A, Boškoski I, Ianiro G, Gerardi V, Ojetti V, Alfieri S, Gasbarrini A
Eur Rev Med Pharmacol Sci 2013;17 Suppl 2:65-71. PMID: 24443071
Lindkvist B
World J Gastroenterol 2013 Nov 14;19(42):7258-66. doi: 10.3748/wjg.v19.i42.7258. PMID: 24259956Free PMC Article

Recent systematic reviews

Cohen SM, Kent TS
JAMA Surg 2023 Jun 1;158(6):652-661. doi: 10.1001/jamasurg.2023.0367. PMID: 37074693
Beyer G, Hoffmeister A, Lorenz P, Lynen P, Lerch MM, Mayerle J
Dtsch Arztebl Int 2022 Jul 25;119(29-30):495-501. doi: 10.3238/arztebl.m2022.0223. PMID: 35945698Free PMC Article
Pekgöz M
World J Gastroenterol 2019 Aug 7;25(29):4019-4042. doi: 10.3748/wjg.v25.i29.4019. PMID: 31413535Free PMC Article
de la Iglesia-García D, Huang W, Szatmary P, Baston-Rey I, Gonzalez-Lopez J, Prada-Ramallal G, Mukherjee R, Nunes QM, Domínguez-Muñoz JE, Sutton R; NIHR Pancreas Biomedical Research Unit Patient Advisory Group
Gut 2017 Aug;66(8):1354-1355. Epub 2016 Dec 9 doi: 10.1136/gutjnl-2016-312529. PMID: 27941156Free PMC Article
Xiao AY, Tan ML, Wu LM, Asrani VM, Windsor JA, Yadav D, Petrov MS
Lancet Gastroenterol Hepatol 2016 Sep;1(1):45-55. Epub 2016 Jun 28 doi: 10.1016/S2468-1253(16)30004-8. PMID: 28404111

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Curated

    • NICE, 2020
      UK NICE Guideline NG104, Pancreatitis, 2020

    Consumer resources

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