Lymphangiectasia pulmonary congenital

Common Name(s)

Lymphangiectasia pulmonary congenital

Congenital pulmonary lymphangiectasia is a rare developmental disorder present from birth that affects the lungs. Infants with this condition have abnormally widened lymphatic vessels within the lungs. The lymphatic system, which helps the immune system protect the body against infection and disease, consists of a network of tubular channels that drain a thin watery fluid known as lymph from different areas of the body into the bloodstream. Lymph, which is made up of proteins, fats and certain white blood cells called lymphocytes, accumulates in the tiny spaces between tissue cells. Infants with congenital pulmonary lymphangiectasia often develop severe, potentially life-threatening, respiratory distress shortly after birth. They may also develop cyanosis, a condition caused by low levels of circulating oxygen in the blood which causes the skin to have a bluish tint. The exact cause of the condition is unknown.

Congenital pulmonary lymphangiectasia can occur as a primary or secondary disorder. Primary congenital pulmonary lymphangiectasia can occur as an isolated defect within the lungs or as part of a a generalized form of lymphatic vessel malformation that affects the entire body. Secondary congenital pulmonary lymphangiectasia occurs secondary to another condition, often involving the heart.
 

Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Lymphangiectasia pulmonary congenital" for support, advocacy or research.

There are currently no organizations listed in Disease InfoSearch that support this condition. Create a listing.

 

 

General Support Organizations

Not finding the support you need? Show General Support Organizations

 
 
Top

How do you compare to others with this condition?

Privately answer questions about your health. Let resources, you select, come to you.

Anonymously share and see how your answers compare with others with this condition while privately providing key pieces of information to medical researchers, disease advocacy groups, and others ONLY YOU select to help speed up cures and better alternatives.

 
 

Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Lymphangiectasia pulmonary congenital" for support, advocacy or research.

There are currently no organizations listed in Disease InfoSearch that support this condition. Create a listing.

 

 

General Support Organizations

Not finding the support you need? Show General Support Organizations

 
 
 
 
Top

Scientific Literature

Articles from the PubMed Database

Research articles describe the outcome of a single study. They are the published results of original research.
The terms "Lymphangiectasia pulmonary congenital" returned 4 free, full-text research articles on human participants. First 3 results:

Recurrent congenital pulmonary lymphangiectasia.
 

Author(s): A Delabaere, H Laurichesse-Delmas, M N Varlet, A Clemenson, P J Dechelotte, A M Beaufrere, B Jacquetin, D Lemery, D Gallot

Journal: Ultrasound Obstet Gynecol. 2008 Apr;31(4):479-81.

 

Last Updated: 3 Apr 2008

Go To URL
Familial congenital non-immune hydrops, chylothorax, and pulmonary lymphangiectasia.
 

Author(s): David A Stevenson, Theodore J Pysher, Robert M Ward, John C Carey

Journal: Am. J. Med. Genet. A. 2006 Feb;140(4):368-72.

 

Pulmonary lymphangiectasia is an uncommon congenital anomaly, and familial occurrence has rarely been reported. We report on two sibs with bilateral pleural effusion/chylothorax and hydrops who died neonatally. One sib required prenatal intrauterine hemithoracic drainage. Autopsy ...

Last Updated: 26 Jan 2006

Go To URL
Congenital pulmonary lymphangiectasia.
 

Author(s): Jonathan Finder, Jonathan Steinfeld

Journal: N. Engl. J. Med.. 2004 Feb;350(9):948; author reply 948.

 

Last Updated: 26 Feb 2004

Go To URL

Reviews from the PubMed Database

Review articles summarize what is currently known about a disease. They discuss research previously published by others.
The terms "Lymphangiectasia pulmonary congenital" returned 1 free, full-text review articles on human participants. First 3 results:

Congenital pulmonary lymphangiectasia.
 

Author(s): Carlo Bellini, Francesco Boccardo, Corradino Campisi, Eugenio Bonioli

Journal:

 

Congenital pulmonary lymphangiectasia (PL) is a rare developmental disorder involving the lung, and characterized by pulmonary subpleural, interlobar, perivascular and peribronchial lymphatic dilatation. The prevalence is unknown. PL presents at birth with severe respiratory distress, ...

Last Updated: 19 Nov 2006

Go To URL
 
 
Top

Symptoms, Diagnosis, and Treatment

There are currently no related results available in GeneReviews.

 
 
Top

Clinical Trial Information This information is provided by ClinicalTrials.gov

There are currently no open clinical trials for this condition.