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Secondary intestinal lymphangiectasia

MedGen UID:
908114
•Concept ID:
C4273969
•
Disease or Syndrome
Synonyms: secondary intestinal lymphangiectasia; Secondary intestinal lymphangiectasis
SNOMED CT: Secondary intestinal lymphangiectasia (717255008); Secondary intestinal lymphangiectasis (717255008)
 
Monarch Initiative: MONDO:0019574
Orphanet: ORPHA90363

Definition

An acquired form of intestinal lymphangiectasia manifesting as a protein-losing enteropathy due to another disorder such as Crohn disease, congestive heart failure, sarcoidosis, Turner syndrome and often in patients who have undergone a Fontan operation. It is characterized by malabsorption, diarrhea, edema due to hypoproteinemia, steatorrhea and serosal effusions. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  

Professional guidelines

PubMed

In-silico assessment of high-risk non-synonymous SNPs in ADAMTS3 gene associated with Hennekam syndrome and their impact on protein stability and function.
Shinwari K, Wu Y, Rehman HM, Xiao N, Bolkov M, Tuzankina I, Chereshnev V
BMC Bioinformatics 2023 Jun 15;24(1):251. doi: 10.1186/s12859-023-05361-6. PMID: 37322437Free PMC Article
Implications of lymphatic alterations in the pathogenesis and treatment of inflammatory bowel disease.
Zhang L, Ocansey DKW, Liu L, Olovo CV, Zhang X, Qian H, Xu W, Mao F
Biomed Pharmacother 2021 Aug;140:111752. Epub 2021 May 24 doi: 10.1016/j.biopha.2021.111752. PMID: 34044275

Recent clinical studies

Etiology

Severe hypoalbuminaemia in a systemic lupus erythematosus-like patient.
Gattorno M, Buoncompagni A, Barabino A, Barbano G, Loy A, Tomà P, Picco P, Marmont AM
Eur J Pediatr 2002 Feb;161(2):84-6. doi: 10.1007/s00431-001-0868-6. PMID: 11954757

Diagnosis

Secondary Intestinal Lymphangiectasia.
Hoskins B, Song W, Guerrerio AL
J Pediatr Gastroenterol Nutr 2024 Jan;78(1):166. Epub 2023 Sep 6 doi: 10.1097/MPG.0000000000003936. PMID: 38291682
Secondary intestinal lymphangiectasia due to multiple myeloma.
Bhat M, Laneuville P, Marliss EB, Costea F, Marcus V, Seidman EG, Bitton A
Gastrointest Endosc 2011 Sep;74(3):718-20. Epub 2010 Nov 18 doi: 10.1016/j.gie.2010.09.019. PMID: 21092952
Severe hypoalbuminaemia in a systemic lupus erythematosus-like patient.
Gattorno M, Buoncompagni A, Barabino A, Barbano G, Loy A, Tomà P, Picco P, Marmont AM
Eur J Pediatr 2002 Feb;161(2):84-6. doi: 10.1007/s00431-001-0868-6. PMID: 11954757
Endoscopic and histopathological study on primary and secondary intestinal lymphangiectasia.
Asakura H, Miura S, Morishita T, Aiso S, Tanaka T, Kitahora T, Tsuchiya M, Enomoto Y, Watanabe Y
Dig Dis Sci 1981 Apr;26(4):312-20. doi: 10.1007/BF01308371. PMID: 7238258

Therapy

Severe hypoalbuminaemia in a systemic lupus erythematosus-like patient.
Gattorno M, Buoncompagni A, Barabino A, Barbano G, Loy A, Tomà P, Picco P, Marmont AM
Eur J Pediatr 2002 Feb;161(2):84-6. doi: 10.1007/s00431-001-0868-6. PMID: 11954757

Prognosis

Severe hypoalbuminaemia in a systemic lupus erythematosus-like patient.
Gattorno M, Buoncompagni A, Barabino A, Barbano G, Loy A, Tomà P, Picco P, Marmont AM
Eur J Pediatr 2002 Feb;161(2):84-6. doi: 10.1007/s00431-001-0868-6. PMID: 11954757

Supplemental Content

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