congenital pulmonary Lymphangiectasia

A hereditary form of lymphangiectasis that affects the lungs and is frequently lethal in utero. It is characterized by chronic pleural effusions, pachypleuritis, pleural membrane thickenings, EDEMA; HYDROPS FETALIS, growth retardation, and recurrent RESPIRATORY INFECTIONS. OMIM: 265300

Also Known As:

Lymphangiectasia, pulmonary, congenital; Lymphangiectasia pulmonary congenital; Lymphangiomatosis, pulmonary; Pulmonary cystic lymphangiectasis

Networked: 5 relevant articles (0 outcomes, 0 trials/studies)

Disease Context: Research Results

Experts

1.	Malleske, D T: 1 article (05/2015)
2.	Yoder, B A: 1 article (05/2015)
3.	Enlow, E: 1 article (09/2014)
4.	French, H M: 1 article (09/2014)
5.	Gray, M: 1 article (09/2014)
6.	Itkin, M: 1 article (09/2014)
7.	Keller, M S: 1 article (09/2014)
8.	Kovatis, K Z: 1 article (09/2014)
9.	Stuart, T: 1 article (09/2014)
10.	Hwang, Joon Ho: 1 article (04/2014)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to congenital pulmonary Lymphangiectasia:

1.	Octreotide (Sandostatin)FDA LinkGeneric 05/01/2015 - "We report a case of CC in a late preterm infant with congenital pulmonary lymphangiectasia where octreotide was not effective, but management with oral sildenafil was successful. "
2.	Ethiodized Oil (Ethiodol)FDA Link 09/01/2014 - "Treatment of congenital pulmonary lymphangiectasia using ethiodized oil lymphangiography."
3.	Sildenafil Citrate (Viagra)FDA Link 05/01/2015 - "We report a case of CC in a late preterm infant with congenital pulmonary lymphangiectasia where octreotide was not effective, but management with oral sildenafil was successful. "

Therapies and Procedures

1.	Therapeutics 09/01/2014 - "Historically, congenital pulmonary lymphangiectasia (CPL) has had poor treatment outcomes despite aggressive therapy. " 01/01/2007 - "Congenital pulmonary lymphangiectasia in a newborn: a response to autologous blood therapy."
2.	Pneumonectomy (Lung Volume Reduction) 04/01/2014 - "Pneumonectomy case in a newborn with congenital pulmonary lymphangiectasia."
3.	Critical Care (Surgical Intensive Care) 01/01/2008 - "The recent advances in neonatal and pediatric intensive care have changed the outcome of patients with congenital pulmonary lymphangiectasia of different types, including those cases with early neonatal symptoms. "