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Pericardial lymphangiectasia

MedGen UID:
341077
•Concept ID:
C1856140
•
Finding
HPO: HP:0005183

Definition

An abnormal dilatation of lymph vessels in the pericardium. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPericardial lymphangiectasia

Conditions with this feature

Hennekam lymphangiectasia-lymphedema syndrome 1
MedGen UID:
860487
•Concept ID:
C4012050
•
Disease or Syndrome
Hennekam lymphangiectasia-lymphedema syndrome (HKLLS1) is an autosomal recessive disorder characterized by generalized lymphatic dysplasia affecting various organs, including the intestinal tract, pericardium, and limbs. Additional features of the disorder include facial dysmorphism and cognitive impairment (summary by Alders et al., 2014). Genetic Heterogeneity of Hennekam Lymphangiectasia-Lymphedema Syndrome See also HKLLS2 (616006), caused by mutation in the FAT4 gene (612411) on chromosome 4q28, and HKLLS3 (618154), caused by mutation in the ADAMTS3 gene (605011) on chromosome 4q13.
See: Condition Record
Hennekam lymphangiectasia-lymphedema syndrome 2
MedGen UID:
863376
•Concept ID:
C4014939
•
Disease or Syndrome
Hennekam lymphangiectasia-lymphedema syndrome is an autosomal recessive disorder characterized by generalized lymphatic dysplasia affecting various organs, including the intestinal tract, pericardium, and limbs. Additional features of the disorder include facial dysmorphism and cognitive impairment (summary by Alders et al., 2014). For a discussion of genetic heterogeneity of Hennekam lymphangiectasia-lymphedema syndrome, see HKLLS1 (235510).
See: Condition Record
Hennekam lymphangiectasia-lymphedema syndrome 1
Hennekam lymphangiectasia-lymphedema syndrome 2

Professional guidelines

PubMed

Abnormal pulmonary lymphatic flow in patients with paediatric pulmonary lymphatic disorders: Diagnosis and treatment.
Itkin M, Chidekel A, Ryan KA, Rabinowitz D
Paediatr Respir Rev 2020 Nov;36:15-24. Epub 2020 Jul 10 doi: 10.1016/j.prrv.2020.07.001. PMID: 32792289
Interventional Treatment of Pulmonary Lymphatic Anomalies.
Itkin M
Tech Vasc Interv Radiol 2016 Dec;19(4):299-304. Epub 2016 Oct 8 doi: 10.1053/j.tvir.2016.10.005. PMID: 27993326

Recent clinical studies

Etiology

Mulibrey nanism and immunological complications: a comprehensive case report and literature review.
Gazzin A, Pala F, Bosticardo M, Niemela J, Stoddard J, Biasin E, Quarello P, Carli D, Ferroni F, Delmonte OM, Montin D, Rosenzweig SD, Licciardi F, Notarangelo LD
Front Immunol 2023;14:1303251. Epub 2023 Dec 5 doi: 10.3389/fimmu.2023.1303251. PMID: 38116000Free PMC Article
Correlation between capsule endoscopy classification and CT lymphangiography of primary intestinal lymphangiectasia.
Zhao Q, Sun X, Liu K, Peng Y, Jin D, Shen W, Wang R
Clin Radiol 2023 Mar;78(3):219-226. Epub 2022 Dec 9 doi: 10.1016/j.crad.2022.10.001. PMID: 36509551
Kaposiform lymphangiomatosis and kaposiform hemangioendothelioma: similarities and differences.
Ji Y, Chen S, Peng S, Xia C, Li L
Orphanet J Rare Dis 2019 Jul 5;14(1):165. doi: 10.1186/s13023-019-1147-9. PMID: 31277673Free PMC Article
Diffuse Pulmonary Lymphangiomatosis: MDCT Findings After Direct Lymphangiography.
Sun X, Shen W, Xia S, Wen T, Wang R
AJR Am J Roentgenol 2017 Feb;208(2):300-305. Epub 2016 Nov 15 doi: 10.2214/AJR.16.16589. PMID: 27845836
Interventional Treatment of Pulmonary Lymphatic Anomalies.
Itkin M
Tech Vasc Interv Radiol 2016 Dec;19(4):299-304. Epub 2016 Oct 8 doi: 10.1053/j.tvir.2016.10.005. PMID: 27993326

Diagnosis

Correlation between capsule endoscopy classification and CT lymphangiography of primary intestinal lymphangiectasia.
Zhao Q, Sun X, Liu K, Peng Y, Jin D, Shen W, Wang R
Clin Radiol 2023 Mar;78(3):219-226. Epub 2022 Dec 9 doi: 10.1016/j.crad.2022.10.001. PMID: 36509551
Abnormal pulmonary lymphatic flow in patients with paediatric pulmonary lymphatic disorders: Diagnosis and treatment.
Itkin M, Chidekel A, Ryan KA, Rabinowitz D
Paediatr Respir Rev 2020 Nov;36:15-24. Epub 2020 Jul 10 doi: 10.1016/j.prrv.2020.07.001. PMID: 32792289
Kaposiform lymphangiomatosis and kaposiform hemangioendothelioma: similarities and differences.
Ji Y, Chen S, Peng S, Xia C, Li L
Orphanet J Rare Dis 2019 Jul 5;14(1):165. doi: 10.1186/s13023-019-1147-9. PMID: 31277673Free PMC Article
Interventional Treatment of Pulmonary Lymphatic Anomalies.
Itkin M
Tech Vasc Interv Radiol 2016 Dec;19(4):299-304. Epub 2016 Oct 8 doi: 10.1053/j.tvir.2016.10.005. PMID: 27993326
Lupus protein-losing enteropathy (LUPLE): a systematic review.
Al-Mogairen SM
Rheumatol Int 2011 Aug;31(8):995-1001. Epub 2011 Feb 23 doi: 10.1007/s00296-011-1827-9. PMID: 21344315

Therapy

Mulibrey nanism and immunological complications: a comprehensive case report and literature review.
Gazzin A, Pala F, Bosticardo M, Niemela J, Stoddard J, Biasin E, Quarello P, Carli D, Ferroni F, Delmonte OM, Montin D, Rosenzweig SD, Licciardi F, Notarangelo LD
Front Immunol 2023;14:1303251. Epub 2023 Dec 5 doi: 10.3389/fimmu.2023.1303251. PMID: 38116000Free PMC Article
Redefining WILD syndrome: a primary lymphatic dysplasia with congenital multisegmental lymphoedema, cutaneous lymphovascular malformation, CD4 lymphopaenia and warts.
Mansour S, Josephs KS, Ostergaard P, Gordon K, Van Zanten M, Pearce J, Jeffery S, Keeley V, Riches K, Kreuter A, Wieland U, Hägerling R, Ratnam L, Sackey E, Grigoriadis D, Ho B, Smith F, Rauter E, Mortimer P, Macallan D
J Med Genet 2023 Jan;60(1):84-90. Epub 2021 Dec 16 doi: 10.1136/jmedgenet-2021-107820. PMID: 34916230Free PMC Article
Interventional Treatment of Pulmonary Lymphatic Anomalies.
Itkin M
Tech Vasc Interv Radiol 2016 Dec;19(4):299-304. Epub 2016 Oct 8 doi: 10.1053/j.tvir.2016.10.005. PMID: 27993326
Primary Intestinal Lymphangiectasia Manifested as Unusual Edemas and Effusions: A Case Report.
Wang X, Jin H, Wu W
Medicine (Baltimore) 2016 Mar;95(10):e2849. doi: 10.1097/MD.0000000000002849. PMID: 26962779Free PMC Article
Lupus protein-losing enteropathy (LUPLE): a systematic review.
Al-Mogairen SM
Rheumatol Int 2011 Aug;31(8):995-1001. Epub 2011 Feb 23 doi: 10.1007/s00296-011-1827-9. PMID: 21344315

Prognosis

Mulibrey nanism and immunological complications: a comprehensive case report and literature review.
Gazzin A, Pala F, Bosticardo M, Niemela J, Stoddard J, Biasin E, Quarello P, Carli D, Ferroni F, Delmonte OM, Montin D, Rosenzweig SD, Licciardi F, Notarangelo LD
Front Immunol 2023;14:1303251. Epub 2023 Dec 5 doi: 10.3389/fimmu.2023.1303251. PMID: 38116000Free PMC Article
Kaposiform lymphangiomatosis and kaposiform hemangioendothelioma: similarities and differences.
Ji Y, Chen S, Peng S, Xia C, Li L
Orphanet J Rare Dis 2019 Jul 5;14(1):165. doi: 10.1186/s13023-019-1147-9. PMID: 31277673Free PMC Article
Diffuse Pulmonary Lymphangiomatosis: MDCT Findings After Direct Lymphangiography.
Sun X, Shen W, Xia S, Wen T, Wang R
AJR Am J Roentgenol 2017 Feb;208(2):300-305. Epub 2016 Nov 15 doi: 10.2214/AJR.16.16589. PMID: 27845836
Interventional Treatment of Pulmonary Lymphatic Anomalies.
Itkin M
Tech Vasc Interv Radiol 2016 Dec;19(4):299-304. Epub 2016 Oct 8 doi: 10.1053/j.tvir.2016.10.005. PMID: 27993326
Lupus protein-losing enteropathy (LUPLE): a systematic review.
Al-Mogairen SM
Rheumatol Int 2011 Aug;31(8):995-1001. Epub 2011 Feb 23 doi: 10.1007/s00296-011-1827-9. PMID: 21344315

Clinical prediction guides

Mulibrey nanism and immunological complications: a comprehensive case report and literature review.
Gazzin A, Pala F, Bosticardo M, Niemela J, Stoddard J, Biasin E, Quarello P, Carli D, Ferroni F, Delmonte OM, Montin D, Rosenzweig SD, Licciardi F, Notarangelo LD
Front Immunol 2023;14:1303251. Epub 2023 Dec 5 doi: 10.3389/fimmu.2023.1303251. PMID: 38116000Free PMC Article
Correlation between capsule endoscopy classification and CT lymphangiography of primary intestinal lymphangiectasia.
Zhao Q, Sun X, Liu K, Peng Y, Jin D, Shen W, Wang R
Clin Radiol 2023 Mar;78(3):219-226. Epub 2022 Dec 9 doi: 10.1016/j.crad.2022.10.001. PMID: 36509551
Kaposiform lymphangiomatosis and kaposiform hemangioendothelioma: similarities and differences.
Ji Y, Chen S, Peng S, Xia C, Li L
Orphanet J Rare Dis 2019 Jul 5;14(1):165. doi: 10.1186/s13023-019-1147-9. PMID: 31277673Free PMC Article
A primary intestinal lymphangiectasia hiding the diagnosis of pleural and pericardial tuberculosis: a clinical observation.
Hammi S, Berrani H, Benouchen T, Lamlami N, Elkhiyat I, Bourkadi JE
Pan Afr Med J 2017;26:89. Epub 2017 Feb 23 doi: 10.11604/pamj.2017.26.89.11125. PMID: 28491220Free PMC Article
Interventional Treatment of Pulmonary Lymphatic Anomalies.
Itkin M
Tech Vasc Interv Radiol 2016 Dec;19(4):299-304. Epub 2016 Oct 8 doi: 10.1053/j.tvir.2016.10.005. PMID: 27993326

Recent systematic reviews

Lupus protein-losing enteropathy (LUPLE): a systematic review.
Al-Mogairen SM
Rheumatol Int 2011 Aug;31(8):995-1001. Epub 2011 Feb 23 doi: 10.1007/s00296-011-1827-9. PMID: 21344315

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