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Acute pancreatitis

MedGen UID:
7872
Concept ID:
C0001339
Disease or Syndrome
Synonyms: Acute Pancreatitides; Acute Pancreatitis; Pancreatitides, Acute; Pancreatitis, Acute
SNOMED CT: Acute pancreatitis (197456007); AP - Acute pancreatitis (197456007)
 
HPO: HP:0001735
Monarch Initiative: MONDO:0006515

Definition

A acute form of pancreatitis. [from HPO]

Term Hierarchy

Conditions with this feature

Hyperlipoproteinemia, type I
MedGen UID:
7352
Concept ID:
C0023817
Disease or Syndrome
Familial lipoprotein lipase (LPL) deficiency usually presents in childhood and is characterized by very severe hypertriglyceridemia with episodes of abdominal pain, recurrent acute pancreatitis, eruptive cutaneous xanthomata, and hepatosplenomegaly. Clearance of chylomicrons from the plasma is impaired, causing triglycerides to accumulate in plasma and the plasma to have a milky (lactescent or lipemic) appearance. Symptoms usually resolve with restriction of total dietary fat to =20 g/day.
Familial partial lipodystrophy, Kobberling type
MedGen UID:
318591
Concept ID:
C1720859
Disease or Syndrome
Familial partial lipodystrophy type 1 (FPLD1), or Kobberling-type lipodystrophy, is characterized by loss of adipose tissue confined to the extremities, with normal or increased distribution of fat on the face, neck, and trunk (Kobberling and Dunnigan, 1986). For a general description and a discussion of genetic heterogeneity of familial partial lipodystrophy (FPLD), see 151660.
Familial partial lipodystrophy, Dunnigan type
MedGen UID:
354526
Concept ID:
C1720860
Disease or Syndrome
Familial partial lipodystrophy is a metabolic disorder characterized by abnormal subcutaneous adipose tissue distribution beginning in late childhood or early adult life. Affected individuals gradually lose fat from the upper and lower extremities and the gluteal and truncal regions, resulting in a muscular appearance with prominent superficial veins. In some patients, adipose tissue accumulates on the face and neck, causing a double chin, fat neck, or cushingoid appearance. Metabolic abnormalities include insulin-resistant diabetes mellitus with acanthosis nigricans and hypertriglyceridemia; hirsutism and menstrual abnormalities occur infrequently. Familial partial lipodystrophy may also be referred to as lipoatrophic diabetes mellitus, but the essential feature is loss of subcutaneous fat (review by Garg, 2004). The disorder may be misdiagnosed as Cushing disease (see 219080) (Kobberling and Dunnigan, 1986; Garg, 2004). Genetic Heterogeneity of Familial Partial Lipodystrophy Familial partial lipodystrophy is a clinically and genetically heterogeneous disorder. Types 1 and 2 were originally described as clinical subtypes: type 1 (FPLD1; 608600), characterized by loss of subcutaneous fat confined to the limbs (Kobberling et al., 1975), and FPLD2, characterized by loss of subcutaneous fat from the limbs and trunk (Dunnigan et al., 1974; Kobberling and Dunnigan, 1986). No genetic basis for FPLD1 has yet been delineated. FPLD3 (604367) is caused by mutation in the PPARG gene (601487) on chromosome 3p25; FPLD4 (613877) is caused by mutation in the PLIN1 gene (170290) on chromosome 15q26; FPLD5 (615238) is caused by mutation in the CIDEC gene (612120) on chromosome 3p25; FPLD6 (615980) is caused by mutation in the LIPE gene (151750) on chromosome 19q13; FPLD7 (606721) is caused by mutation in the CAV1 gene (601047) on chromosome 7q31; FPLD8 (620679), caused by mutation in the ADRA2A gene (104210) on chromosome 10q25; and FPLD9 (620683), caused by mutation in the PLAAT3 gene (613867) on chromosome 11q12.
Congenital generalized lipodystrophy type 1
MedGen UID:
318592
Concept ID:
C1720862
Disease or Syndrome
Berardinelli-Seip congenital lipodystrophy (BSCL) is usually diagnosed at birth or soon thereafter. Because of the absence of functional adipocytes, lipid is stored in other tissues, including muscle and liver. Affected individuals develop insulin resistance and approximately 25%-35% develop diabetes mellitus between ages 15 and 20 years. Hepatomegaly secondary to hepatic steatosis and skeletal muscle hypertrophy occur in all affected individuals. Hypertrophic cardiomyopathy is reported in 20%-25% of affected individuals and is a significant cause of morbidity from cardiac failure and early mortality.
Congenital generalized lipodystrophy type 2
MedGen UID:
318593
Concept ID:
C1720863
Congenital Abnormality
Berardinelli-Seip congenital lipodystrophy (BSCL) is usually diagnosed at birth or soon thereafter. Because of the absence of functional adipocytes, lipid is stored in other tissues, including muscle and liver. Affected individuals develop insulin resistance and approximately 25%-35% develop diabetes mellitus between ages 15 and 20 years. Hepatomegaly secondary to hepatic steatosis and skeletal muscle hypertrophy occur in all affected individuals. Hypertrophic cardiomyopathy is reported in 20%-25% of affected individuals and is a significant cause of morbidity from cardiac failure and early mortality.
Seckel syndrome 10
MedGen UID:
934614
Concept ID:
C4310647
Disease or Syndrome
Any Seckel syndrome in which the cause of the disease is a mutation in the NSMCE2 gene.
Granulomatous disease, chronic, autosomal recessive, 5
MedGen UID:
1710326
Concept ID:
C5394542
Disease or Syndrome
Chronic granulomatous disease (CGD) is a primary immunodeficiency disorder of phagocytes (neutrophils, monocytes, macrophages, and eosinophils) resulting from impaired killing of bacteria and fungi. CGD is characterized by severe recurrent bacterial and fungal infections and dysregulated inflammatory responses resulting in granuloma formation and other inflammatory disorders such as colitis. Infections typically involve the lung (pneumonia), lymph nodes (lymphadenitis), liver (abscess), bone (osteomyelitis), and skin (abscesses or cellulitis). Granulomas typically involve the genitourinary system (bladder) and gastrointestinal tract (often the pylorus initially, and later the esophagus, jejunum, ileum, cecum, rectum, and perirectal area). Some males with X-linked CGD have McLeod neuroacanthocytosis syndrome as the result of a contiguous gene deletion. While CGD may present anytime from infancy to late adulthood, the vast majority of affected individuals are diagnosed before age five years. Use of antimicrobial prophylaxis and therapy has greatly improved overall survival.
Aicardi-Goutieres syndrome 9
MedGen UID:
1794176
Concept ID:
C5561966
Disease or Syndrome
Aicardi-Goutieres syndrome-9 (AGS9) is a type I interferonopathy characterized by severe developmental delay and progressive neurologic deterioration. Patients present in infancy with irritability and spasticity. Brain imaging shows diffusely abnormal white matter, cerebral atrophy, and intracranial calcification. Premature death has been associated with renal and/or hepatic failure (Uggenti et al., 2020). For a general phenotypic description and discussion of genetic heterogeneity of Aicardi-Goutieres syndrome, see AGS1 (225750).

Professional guidelines

PubMed

van den Berg FF, Boermeester MA
Curr Opin Crit Care 2023 Apr 1;29(2):145-151. Epub 2023 Jan 23 doi: 10.1097/MCC.0000000000001017. PMID: 36727757Free PMC Article
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
Greenberg JA, Hsu J, Bawazeer M, Marshall J, Friedrich JO, Nathens A, Coburn N, May GR, Pearsall E, McLeod RS
Can J Surg 2016 Apr;59(2):128-40. doi: 10.1503/cjs.015015. PMID: 27007094Free PMC Article

Curated

UK NICE Guideline NG104, Pancreatitis, 2020

Recent clinical studies

Etiology

Valverde-López F, Martínez-Cara JG, Redondo-Cerezo E
Med Clin (Barc) 2022 Jun 10;158(11):556-563. Epub 2022 Mar 8 doi: 10.1016/j.medcli.2021.12.012. PMID: 35277268
Hart PA, Bradley D, Conwell DL, Dungan K, Krishna SG, Wyne K, Bellin MD, Yadav D, Andersen DK, Serrano J, Papachristou GI
Lancet Gastroenterol Hepatol 2021 Aug;6(8):668-675. Epub 2021 Jun 3 doi: 10.1016/S2468-1253(21)00019-4. PMID: 34089654Free PMC Article
Mederos MA, Reber HA, Girgis MD
JAMA 2021 Jan 26;325(4):382-390. doi: 10.1001/jama.2020.20317. PMID: 33496779
Garg PK, Singh VP
Gastroenterology 2019 May;156(7):2008-2023. Epub 2019 Feb 12 doi: 10.1053/j.gastro.2018.12.041. PMID: 30768987Free PMC Article
Garg R, Rustagi T
Biomed Res Int 2018;2018:4721357. Epub 2018 Jul 26 doi: 10.1155/2018/4721357. PMID: 30148167Free PMC Article

Diagnosis

Valverde-López F, Martínez-Cara JG, Redondo-Cerezo E
Med Clin (Barc) 2022 Jun 10;158(11):556-563. Epub 2022 Mar 8 doi: 10.1016/j.medcli.2021.12.012. PMID: 35277268
Afzal S, Kleinhenz J
Pediatr Ann 2021 Aug;50(8):e330-e335. Epub 2021 Aug 1 doi: 10.3928/19382359-20210713-01. PMID: 34398718
Mederos MA, Reber HA, Girgis MD
JAMA 2021 Jan 26;325(4):382-390. doi: 10.1001/jama.2020.20317. PMID: 33496779
Ismail OZ, Bhayana V
Clin Biochem 2017 Dec;50(18):1275-1280. Epub 2017 Jul 16 doi: 10.1016/j.clinbiochem.2017.07.003. PMID: 28720341
Greenberg JA, Hsu J, Bawazeer M, Marshall J, Friedrich JO, Nathens A, Coburn N, May GR, Pearsall E, McLeod RS
Can J Surg 2016 Apr;59(2):128-40. doi: 10.1503/cjs.015015. PMID: 27007094Free PMC Article

Therapy

Huang Y, Badurdeen DS
Turk J Gastroenterol 2023 Aug;34(8):795-801. doi: 10.5152/tjg.2023.23175. PMID: 37404118Free PMC Article
Tan HC, Dampil OA, Marquez MM
J ASEAN Fed Endocr Soc 2022;37(2):65-72. Epub 2022 Aug 23 doi: 10.15605/jafes.037.02.14. PMID: 36578889Free PMC Article
de-Madaria E, Buxbaum JL, Maisonneuve P, García García de Paredes A, Zapater P, Guilabert L, Vaillo-Rocamora A, Rodríguez-Gandía MÁ, Donate-Ortega J, Lozada-Hernández EE, Collazo Moreno AJR, Lira-Aguilar A, Llovet LP, Mehta R, Tandel R, Navarro P, Sánchez-Pardo AM, Sánchez-Marin C, Cobreros M, Fernández-Cabrera I, Casals-Seoane F, Casas Deza D, Lauret-Braña E, Martí-Marqués E, Camacho-Montaño LM, Ubieto V, Ganuza M, Bolado F; ERICA Consortium
N Engl J Med 2022 Sep 15;387(11):989-1000. doi: 10.1056/NEJMoa2202884. PMID: 36103415
Parhofer KG, Laufs U
Dtsch Arztebl Int 2019 Dec 6;116(49):825-832. doi: 10.3238/arztebl.2019.0825. PMID: 31888796Free PMC Article
van Dijk SM, Hallensleben NDL, van Santvoort HC, Fockens P, van Goor H, Bruno MJ, Besselink MG; Dutch Pancreatitis Study Group
Gut 2017 Nov;66(11):2024-2032. Epub 2017 Aug 24 doi: 10.1136/gutjnl-2016-313595. PMID: 28838972

Prognosis

Iannuzzi JP, King JA, Leong JH, Quan J, Windsor JW, Tanyingoh D, Coward S, Forbes N, Heitman SJ, Shaheen AA, Swain M, Buie M, Underwood FE, Kaplan GG
Gastroenterology 2022 Jan;162(1):122-134. Epub 2021 Sep 25 doi: 10.1053/j.gastro.2021.09.043. PMID: 34571026
Hart PA, Bradley D, Conwell DL, Dungan K, Krishna SG, Wyne K, Bellin MD, Yadav D, Andersen DK, Serrano J, Papachristou GI
Lancet Gastroenterol Hepatol 2021 Aug;6(8):668-675. Epub 2021 Jun 3 doi: 10.1016/S2468-1253(21)00019-4. PMID: 34089654Free PMC Article
Trikudanathan G, Wolbrink DRJ, van Santvoort HC, Mallery S, Freeman M, Besselink MG
Gastroenterology 2019 May;156(7):1994-2007.e3. Epub 2019 Feb 15 doi: 10.1053/j.gastro.2019.01.269. PMID: 30776347
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
Roberts SE, Morrison-Rees S, John A, Williams JG, Brown TH, Samuel DG
Pancreatology 2017 Mar-Apr;17(2):155-165. Epub 2017 Jan 19 doi: 10.1016/j.pan.2017.01.005. PMID: 28159463

Clinical prediction guides

Lee DW, Cho CM
Medicina (Kaunas) 2022 Jun 11;58(6) doi: 10.3390/medicina58060787. PMID: 35744050Free PMC Article
Shah J, Rana SS
Indian J Gastroenterol 2020 Apr;39(2):123-132. Epub 2020 Apr 13 doi: 10.1007/s12664-020-01016-z. PMID: 32285399
Silva-Vaz P, Abrantes AM, Castelo-Branco M, Gouveia A, Botelho MF, Tralhão JG
Int J Mol Sci 2020 Jan 4;21(1) doi: 10.3390/ijms21010338. PMID: 31947993Free 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
Trikudanathan G, Wolbrink DRJ, van Santvoort HC, Mallery S, Freeman M, Besselink MG
Gastroenterology 2019 May;156(7):1994-2007.e3. Epub 2019 Feb 15 doi: 10.1053/j.gastro.2019.01.269. PMID: 30776347

Recent systematic reviews

Tan HC, Dampil OA, Marquez MM
J ASEAN Fed Endocr Soc 2022;37(2):65-72. Epub 2022 Aug 23 doi: 10.15605/jafes.037.02.14. PMID: 36578889Free 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
Larsson SC, Burgess S
EBioMedicine 2022 Aug;82:104154. Epub 2022 Jul 8 doi: 10.1016/j.ebiom.2022.104154. PMID: 35816897Free PMC Article
Gupta M, Liti B, Barrett C, Thompson PD, Fernandez AB
Am J Med 2022 Jun;135(6):709-714. Epub 2022 Jan 23 doi: 10.1016/j.amjmed.2021.12.006. PMID: 35081380
Iannuzzi JP, King JA, Leong JH, Quan J, Windsor JW, Tanyingoh D, Coward S, Forbes N, Heitman SJ, Shaheen AA, Swain M, Buie M, Underwood FE, Kaplan GG
Gastroenterology 2022 Jan;162(1):122-134. Epub 2021 Sep 25 doi: 10.1053/j.gastro.2021.09.043. PMID: 34571026

Supplemental Content

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      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

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