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Pulmonary capillary hemangiomatosis

MedGen UID:
87404
Concept ID:
C0340548
Disease or Syndrome
Synonym: Pulmonary hemangiomas
SNOMED CT: Pulmonary capillary hemangiomatosis (233949008)
 
HPO: HP:0005954

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPulmonary capillary hemangiomatosis
Follow this link to review classifications for Pulmonary capillary hemangiomatosis in Orphanet.

Conditions with this feature

Von Hippel-Lindau syndrome
MedGen UID:
42458
Concept ID:
C0019562
Disease or Syndrome
Von Hippel-Lindau (VHL) syndrome is characterized by hemangioblastomas of the brain, spinal cord, and retina; renal cysts and clear cell renal cell carcinoma; pheochromocytoma, pancreatic cysts, and neuroendocrine tumors; endolymphatic sac tumors; and epididymal and broad ligament cysts. Cerebellar hemangioblastomas may be associated with headache, vomiting, gait disturbances, or ataxia. Spinal hemangioblastomas and related syrinx usually present with pain. Sensory and motor loss may develop with cord compression. Retinal hemangioblastomas may be the initial manifestation of VHL syndrome and can cause vision loss. Renal cell carcinoma occurs in about 70% of individuals with VHL and is the leading cause of mortality. Pheochromocytomas can be asymptomatic but may cause sustained or episodic hypertension. Pancreatic lesions often remain asymptomatic and rarely cause endocrine or exocrine insufficiency. Endolymphatic sac tumors can cause hearing loss of varying severity, which can be a presenting symptom. Cystadenomas of the epididymis are relatively common. They rarely cause problems, unless bilateral, in which case they may result in infertility.
Familial pulmonary capillary hemangiomatosis
MedGen UID:
90956
Concept ID:
C0340848
Disease or Syndrome
Pulmonary venoocclusive disease-2 is an autosomal recessive subtype of primary pulmonary hypertension (PPH; see 178600). It is characterized histologically by widespread fibrous intimal proliferation of septal veins and preseptal venules, and is frequently associated with pulmonary capillary dilatation and proliferation. The disorder can cause occult alveolar hemorrhage. High-resolution CT imaging of the chest shows patchy centrilobular ground-glass opacities, septal lines, and lymph node enlargement (summary by Eyries et al., 2014). For a discussion of genetic heterogeneity of pulmonary venoocclusive disease, see PVOD1 (265450).

Professional guidelines

PubMed

Ogawa A, Sakao S, Tanabe N, Matsubara H, Tatsumi K
Respir Investig 2019 Mar;57(2):183-190. Epub 2018 Nov 23 doi: 10.1016/j.resinv.2018.10.004. PMID: 30473253

Recent clinical studies

Etiology

Nakamura J, Tsujino I, Shima H, Nakaya T, Sugimoto A, Sato T, Watanabe T, Ohira H, Suzuki M, Tsuneta S, Hisada R, Kato M, Konno S
Am J Cardiovasc Drugs 2023 May;23(3):329-338. Epub 2023 Mar 30 doi: 10.1007/s40256-023-00577-6. PMID: 36995544
Neubert L, Borchert P, Shin HO, Linz F, Wagner WL, Warnecke G, Laenger F, Haverich A, Stark H, Hoeper MM, Kuehnel M, Ackermann M, Jonigk D
J Pathol Clin Res 2019 Apr;5(2):108-114. Epub 2019 Feb 27 doi: 10.1002/cjp2.125. PMID: 30697960Free PMC Article
Girerd B, Lau E, Montani D, Humbert M
Curr Opin Pulm Med 2017 Sep;23(5):386-391. doi: 10.1097/MCP.0000000000000414. PMID: 28661905
Ortiz-Bautista C, Hernández-González I, Escribano-Subías P
Med Clin (Barc) 2017 Mar 22;148(6):265-270. Epub 2017 Jan 22 doi: 10.1016/j.medcli.2016.11.031. PMID: 28118962
Best DH, Austin ED, Chung WK, Elliott CG
Curr Opin Cardiol 2014 Nov;29(6):520-7. doi: 10.1097/HCO.0000000000000105. PMID: 25159282

Diagnosis

Girerd B, Lau E, Montani D, Humbert M
Curr Opin Pulm Med 2017 Sep;23(5):386-391. doi: 10.1097/MCP.0000000000000414. PMID: 28661905
Ortiz-Bautista C, Hernández-González I, Escribano-Subías P
Med Clin (Barc) 2017 Mar 22;148(6):265-270. Epub 2017 Jan 22 doi: 10.1016/j.medcli.2016.11.031. PMID: 28118962
Chaisson NF, Dodson MW, Elliott CG
Clin Chest Med 2016 Sep;37(3):523-34. Epub 2016 Jun 30 doi: 10.1016/j.ccm.2016.04.014. PMID: 27514598
O'Keefe MC, Post MD
Arch Pathol Lab Med 2015 Feb;139(2):274-7. doi: 10.5858/arpa.2013-0500-RS. PMID: 25611112
Best DH, Austin ED, Chung WK, Elliott CG
Curr Opin Cardiol 2014 Nov;29(6):520-7. doi: 10.1097/HCO.0000000000000105. PMID: 25159282

Therapy

Nakamura J, Tsujino I, Shima H, Nakaya T, Sugimoto A, Sato T, Watanabe T, Ohira H, Suzuki M, Tsuneta S, Hisada R, Kato M, Konno S
Am J Cardiovasc Drugs 2023 May;23(3):329-338. Epub 2023 Mar 30 doi: 10.1007/s40256-023-00577-6. PMID: 36995544
Mussa A, Carli D, Giorgio E, Villar AM, Cardaropoli S, Carbonara C, Campagnoli MF, Galletto P, Palumbo M, Olivieri S, Isella C, Andelfinger G, Tartaglia M, Botta G, Brusco A, Medico E, Ferrero GB
Genes (Basel) 2021 Dec 21;13(1) doi: 10.3390/genes13010006. PMID: 35052347Free PMC Article
Ogawa A, Sakao S, Tanabe N, Matsubara H, Tatsumi K
Respir Investig 2019 Mar;57(2):183-190. Epub 2018 Nov 23 doi: 10.1016/j.resinv.2018.10.004. PMID: 30473253
Ogawa A, Takahashi Y, Matsubara H
J Cardiol 2018 Sep;72(3):255-260. Epub 2018 Mar 13 doi: 10.1016/j.jjcc.2018.02.009. PMID: 29548663
Akagi S, Nakamura K, Matsubara H, Ogawa A, Sarashina T, Ejiri K, Ito H
Acta Med Okayama 2015;69(3):129-36. doi: 10.18926/AMO/53519. PMID: 26101188

Prognosis

Nakamura J, Tsujino I, Shima H, Nakaya T, Sugimoto A, Sato T, Watanabe T, Ohira H, Suzuki M, Tsuneta S, Hisada R, Kato M, Konno S
Am J Cardiovasc Drugs 2023 May;23(3):329-338. Epub 2023 Mar 30 doi: 10.1007/s40256-023-00577-6. PMID: 36995544
Li Y, Gu Y, Hong C, Deng Y, Chen Z, Jiang J
Pathol Res Pract 2020 Sep;216(9):153100. Epub 2020 Jul 4 doi: 10.1016/j.prp.2020.153100. PMID: 32825965
Shi W, Jiao Y
QJM 2020 May 1;113(5):371-372. doi: 10.1093/qjmed/hcz233. PMID: 31501875
Girerd B, Lau E, Montani D, Humbert M
Curr Opin Pulm Med 2017 Sep;23(5):386-391. doi: 10.1097/MCP.0000000000000414. PMID: 28661905
O'Keefe MC, Post MD
Arch Pathol Lab Med 2015 Feb;139(2):274-7. doi: 10.5858/arpa.2013-0500-RS. PMID: 25611112

Clinical prediction guides

Delhaye C, Walsdorff M, Hackx M
Radiology 2020 Apr;295(1):240-244. doi: 10.1148/radiol.2020180473. PMID: 32176598
Ogawa A, Takahashi Y, Matsubara H
J Cardiol 2018 Sep;72(3):255-260. Epub 2018 Mar 13 doi: 10.1016/j.jjcc.2018.02.009. PMID: 29548663
Ogawa A, Miyaji K, Matsubara H
Respir Med 2017 Oct;131:215-219. Epub 2017 Sep 12 doi: 10.1016/j.rmed.2017.08.032. PMID: 28947033
Liu C, Chen J
Cochrane Database Syst Rev 2006 Jul 19;(3):CD004434. doi: 10.1002/14651858.CD004434.pub3. PMID: 16856046
Kradin R, Matsubara O, Mark EJ
Exp Mol Pathol 2005 Dec;79(3):194-7. Epub 2005 Oct 21 doi: 10.1016/j.yexmp.2005.08.010. PMID: 16246326

Recent systematic reviews

Ogawa A, Sakao S, Tanabe N, Matsubara H, Tatsumi K
Respir Investig 2019 Mar;57(2):183-190. Epub 2018 Nov 23 doi: 10.1016/j.resinv.2018.10.004. PMID: 30473253
Liu C, Chen J
Cochrane Database Syst Rev 2006 Jul 19;(3):CD004434. doi: 10.1002/14651858.CD004434.pub3. PMID: 16856046

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