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Ileus

MedGen UID:
219874
Concept ID:
C1258215
Disease or Syndrome
Synonyms: Gastrointestinal atony
SNOMED CT: Ileus (81060008)
 
HPO: HP:0002595

Definition

Acute obstruction of the intestines preventing passage of the contents of the intestines. [from HPO]

Conditions with this feature

Insulin-dependent diabetes mellitus secretory diarrhea syndrome
MedGen UID:
83339
Concept ID:
C0342288
Congenital Abnormality
IPEX (Immune dysregulation, Polyendocrinopathy, Enteropathy, X-linked) syndrome is characterized by systemic autoimmunity, typically beginning in the first year of life. Presentation is most commonly the clinical triad of watery diarrhea, eczematous dermatitis, and endocrinopathy (most commonly insulin-dependent diabetes mellitus). Most children have other autoimmune phenomena including Coombs-positive anemia, autoimmune thrombocytopenia, autoimmune neutropenia, and tubular nephropathy. Without aggressive immunosuppression or bone marrow transplantation, the majority of affected males die within the first one to two years of life from metabolic derangements or sepsis; a few with a milder phenotype have survived into the second or third decade of life.
Creatine deficiency, X-linked
MedGen UID:
337451
Concept ID:
C1845862
Disease or Syndrome
The cerebral creatine deficiency syndromes (CCDS), inborn errors of creatine metabolism, include the two creatine biosynthesis disorders, guanidinoacetate methyltransferase (GAMT) deficiency and L-arginine:glycine amidinotransferase (AGAT) deficiency, and the creatine transporter (CRTR) deficiency. Intellectual disability and seizures are common to all three CCDS. The majority of individuals with GAMT deficiency have a behavior disorder that can include autistic behaviors and self-mutilation; about 40% have movement disorder. Onset is between ages three months and three years. Only 14 individuals with AGAT deficiency have been reported. The phenotype of CRTR deficiency in affected males ranges from mild intellectual disability and speech delay to severe intellectual disability, seizures, movement disorder and behavior disorder; age at diagnosis ranges from two to 66 years. Clinical phenotype of females heterozygous for CRTR deficiency ranges from asymptomatic to severe phenotype resembling male phenotype.
Lipodystrophy, congenital generalized, type 4
MedGen UID:
412871
Concept ID:
C2750069
Disease or Syndrome
Congenital generalized lipodystrophy type 4 combines the phenotype of classic Berardinelli-Seip lipodystrophy (608594) with muscular dystrophy and cardiac conduction anomalies (Hayashi et al., 2009). For a general description and a discussion of genetic heterogeneity of congenital generalized lipodystrophy, see CGL1 (608594).

Recent clinical studies

Etiology

Smart CJ, Malik KI
Expert Opin Investig Drugs 2017 Apr;26(4):489-493. Epub 2017 Mar 10 doi: 10.1080/13543784.2017.1301427. PMID: 28277883
Moran RA, Jalaly NY, Kamal A, Rao S, Klapheke R, James TW, Kambhampati S, Makary MA, Hirose K, Kumbhari V, Stein EM, Khashab MA, Lennon AM, Kalloo AN, Zaheer A, Hernaez R, Singh VK
Pancreatology 2016 Nov - Dec;16(6):966-972. Epub 2016 Oct 3 doi: 10.1016/j.pan.2016.10.002. PMID: 27727097
Moghadamyeghaneh Z, Hwang GS, Hanna MH, Phelan M, Carmichael JC, Mills S, Pigazzi A, Stamos MJ
Surg Endosc 2016 Feb;30(2):603-9. Epub 2015 May 28 doi: 10.1007/s00464-015-4247-1. PMID: 26017914
Badami A, Fehrenbach Prell EA, Murray MA, Johnson MR, Akhter SA, Lozonschi L, Osaki S, Lushaj E, Kohmoto T
Int J Surg 2015 Aug;20:135-9. Epub 2015 Jun 26 doi: 10.1016/j.ijsu.2015.06.054. PMID: 26118604
Gan TJ, Robinson SB, Oderda GM, Scranton R, Pepin J, Ramamoorthy S
Curr Med Res Opin 2015 Apr;31(4):677-86. Epub 2015 Feb 10 doi: 10.1185/03007995.2015.1005833. PMID: 25586296

Diagnosis

Moran RA, Jalaly NY, Kamal A, Rao S, Klapheke R, James TW, Kambhampati S, Makary MA, Hirose K, Kumbhari V, Stein EM, Khashab MA, Lennon AM, Kalloo AN, Zaheer A, Hernaez R, Singh VK
Pancreatology 2016 Nov - Dec;16(6):966-972. Epub 2016 Oct 3 doi: 10.1016/j.pan.2016.10.002. PMID: 27727097
Hashimoto K, Omori M, Yoshimura E, Asano M, Harada R, Toshimori J, Inoue M, Yokoyama M, Kobashi H
Nihon Shokakibyo Gakkai Zasshi 2016 May;113(5):798-803. doi: 10.11405/nisshoshi.113.798. PMID: 27151476
Kiyasu Y, Tsunoda A, Ohta T, Kusanagi H
Surg Today 2016 Aug;46(8):895-900. Epub 2015 Sep 25 doi: 10.1007/s00595-015-1255-7. PMID: 26407699
Bragg D, El-Sharkawy AM, Psaltis E, Maxwell-Armstrong CA, Lobo DN
Clin Nutr 2015 Jun;34(3):367-76. Epub 2015 Jan 31 doi: 10.1016/j.clnu.2015.01.016. PMID: 25819420
Gan TJ, Robinson SB, Oderda GM, Scranton R, Pepin J, Ramamoorthy S
Curr Med Res Opin 2015 Apr;31(4):677-86. Epub 2015 Feb 10 doi: 10.1185/03007995.2015.1005833. PMID: 25586296

Therapy

Smart CJ, Malik KI
Expert Opin Investig Drugs 2017 Apr;26(4):489-493. Epub 2017 Mar 10 doi: 10.1080/13543784.2017.1301427. PMID: 28277883
Kiyasu Y, Tsunoda A, Ohta T, Kusanagi H
Surg Today 2016 Aug;46(8):895-900. Epub 2015 Sep 25 doi: 10.1007/s00595-015-1255-7. PMID: 26407699
Moghadamyeghaneh Z, Hwang GS, Hanna MH, Phelan M, Carmichael JC, Mills S, Pigazzi A, Stamos MJ
Surg Endosc 2016 Feb;30(2):603-9. Epub 2015 May 28 doi: 10.1007/s00464-015-4247-1. PMID: 26017914
Gan TJ, Robinson SB, Oderda GM, Scranton R, Pepin J, Ramamoorthy S
Curr Med Res Opin 2015 Apr;31(4):677-86. Epub 2015 Feb 10 doi: 10.1185/03007995.2015.1005833. PMID: 25586296
Li RH, Li DC, Lv XP, Quan B
Cell Biochem Biophys 2015 Jun;72(2):509-14. doi: 10.1007/s12013-014-0496-1. PMID: 25567660

Prognosis

Moran RA, Jalaly NY, Kamal A, Rao S, Klapheke R, James TW, Kambhampati S, Makary MA, Hirose K, Kumbhari V, Stein EM, Khashab MA, Lennon AM, Kalloo AN, Zaheer A, Hernaez R, Singh VK
Pancreatology 2016 Nov - Dec;16(6):966-972. Epub 2016 Oct 3 doi: 10.1016/j.pan.2016.10.002. PMID: 27727097
Moghadamyeghaneh Z, Hwang GS, Hanna MH, Phelan M, Carmichael JC, Mills S, Pigazzi A, Stamos MJ
Surg Endosc 2016 Feb;30(2):603-9. Epub 2015 May 28 doi: 10.1007/s00464-015-4247-1. PMID: 26017914
You XM, Mo XS, Ma L, Zhong JH, Qin HG, Lu Z, Xiang BD, Wu FX, Zhao XH, Tang J, Pang YH, Chen J, Li LQ
Medicine (Baltimore) 2015 Nov;94(45):e1968. doi: 10.1097/MD.0000000000001968. PMID: 26559269Free PMC Article
Badami A, Fehrenbach Prell EA, Murray MA, Johnson MR, Akhter SA, Lozonschi L, Osaki S, Lushaj E, Kohmoto T
Int J Surg 2015 Aug;20:135-9. Epub 2015 Jun 26 doi: 10.1016/j.ijsu.2015.06.054. PMID: 26118604
Gan TJ, Robinson SB, Oderda GM, Scranton R, Pepin J, Ramamoorthy S
Curr Med Res Opin 2015 Apr;31(4):677-86. Epub 2015 Feb 10 doi: 10.1185/03007995.2015.1005833. PMID: 25586296

Clinical prediction guides

Moran RA, Jalaly NY, Kamal A, Rao S, Klapheke R, James TW, Kambhampati S, Makary MA, Hirose K, Kumbhari V, Stein EM, Khashab MA, Lennon AM, Kalloo AN, Zaheer A, Hernaez R, Singh VK
Pancreatology 2016 Nov - Dec;16(6):966-972. Epub 2016 Oct 3 doi: 10.1016/j.pan.2016.10.002. PMID: 27727097
Moghadamyeghaneh Z, Hwang GS, Hanna MH, Phelan M, Carmichael JC, Mills S, Pigazzi A, Stamos MJ
Surg Endosc 2016 Feb;30(2):603-9. Epub 2015 May 28 doi: 10.1007/s00464-015-4247-1. PMID: 26017914
Juárez-Parra MA, Carmona-Cantú J, González-Cano JR, Arana-Garza S, Trevino-Frutos RJ
Rev Gastroenterol Mex 2015 Oct-Dec;80(4):260-6. doi: 10.1016/j.rgmx.2015.08.002. PMID: 26601818
Tevis SE, Carchman EH, Foley EF, Harms BA, Heise CP, Kennedy GD
J Gastrointest Surg 2015 Sep;19(9):1684-90. Epub 2015 Jun 24 doi: 10.1007/s11605-015-2877-1. PMID: 26105552
Li RH, Li DC, Lv XP, Quan B
Cell Biochem Biophys 2015 Jun;72(2):509-14. doi: 10.1007/s12013-014-0496-1. PMID: 25567660

Recent systematic reviews

Wolthuis AM, Bislenghi G, Fieuws S, de Buck van Overstraeten A, Boeckxstaens G, D'Hoore A
Colorectal Dis 2016 Jan;18(1):O1-9. doi: 10.1111/codi.13210. PMID: 26558477
Earnshaw SR, Kauf TL, McDade C, Potashman MH, Pauyo C, Reese ES, Senagore A
J Am Coll Surg 2015 Nov;221(5):941-50. Epub 2015 Aug 15 doi: 10.1016/j.jamcollsurg.2015.08.004. PMID: 26353904
Wang M, Gao YH, Xu J, Chi Y, Wei XB, Lewith G, Liu JP
Complement Ther Med 2015 Jun;23(3):469-83. Epub 2015 Apr 13 doi: 10.1016/j.ctim.2015.03.013. PMID: 26051583Free PMC Article
Su'a BU, Pollock TT, Lemanu DP, MacCormick AD, Connolly AB, Hill AG
Int J Surg 2015 Feb;14:49-55. Epub 2015 Jan 7 doi: 10.1016/j.ijsu.2014.12.032. PMID: 25576763
Barletta JF, Senagore AJ
World J Surg 2014 Aug;38(8):1966-77. doi: 10.1007/s00268-014-2506-2. PMID: 24682277

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