TITLE

Congenital pulmonary lymphangiectasia

AUTHOR(S)
Bellini, Carlo; Boccardo, Francesco; Campisi, Corradino; Bonioli, Eugenio
PUB. DATE
January 2006
SOURCE
Orphanet Journal of Rare Diseases;2006, Vol. 1, p43
SOURCE TYPE
Academic Journal
DOC. TYPE
Article
ABSTRACT
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, tachypnea and cyanosis, with a very high mortality rate at or within a few hours of birth. Most reported cases are sporadic and the etiology is not completely understood. It has been suggested that PL lymphatic channels of the fetal lung do not undergo the normal regression process at 20 weeks of gestation. Secondary PL may be caused by a cardiac lesion. The diagnostic approach includes complete family and obstetric history, conventional radiologic studies, ultrasound and magnetic resonance studies, lymphoscintigraphy, lung functionality tests, lung biopsy, bronchoscopy, and pleural effusion examination. During the prenatal period, all causes leading to hydrops fetalis should be considered in the diagnosis of PL. Fetal ultrasound evaluation plays a key role in the antenatal diagnosis of PL. At birth, mechanical ventilation and pleural drainage are nearly always necessary to obtain a favorable outcome of respiratory distress. Home supplemental oxygen therapy and symptomatic treatment of recurrent cough and wheeze are often necessary during childhood, sometimes associated with prolonged pleural drainage. Recent advances in intensive neonatal care have changed the previously nearly fatal outcome of PL at birth. Patients affected by PL who survive infancy, present medical problems which are characteristic of chronic lung disease.
ACCESSION #
30094010

 

Related Articles

  • Multiple Lung Hydatid Cysts and Cardiac Hydatid Cyst: A Rare Presentation. Garg, S. M.; Umesh; Singh, V. P.; Gupta, N.; Aggarwal, J.; Kumar, R. // Bangladesh Journal of Medical Science;Oct2015, Vol. 14 Issue 4, p393 

    Hydatid cysts are commonly present in liver. Cardiac hydatid cysts are very rare and represent less than 2% of all cases of hydatidosis. They can occur as widespread infection or as an isolated event. We present here a case with multiple lung hydatid cysts and a single cardiac hydatid cyst...

  • Proposed Definitions for Epidemiologic and Clinical Studies of Mycobacterium avium Complex Pulmonary Disease. Plotinsky, Rachel N.; Talbot, Elizabeth A.; von Reyn, C. Fordham // PLoS ONE;Nov2013, Vol. 8 Issue 11, p1 

    Background: Epidemiologic and clinical studies of Mycobacterium avium complex (MAC) pulmonary disease typically use strict ATS/IDSA definitions designed for decisions about treatment. Studies based on these criteria may exclude a substantial number of patients with true disease. We reviewed...

  • Rare Disorders and Diseases.  // Exceptional Parent;Aug2008, Vol. 38 Issue 8, p26 

    The article discusses various reports published within the issue, including 22q11 deletion syndrome by Mary Umlauf, and another about isovaleri acidemia by Jana Monaco.

  • Pulmonary Botryomycosis Mimicking Bronchogenic Carcinoma of the Lung. Alavi, Ali; Aghajanzadeh, Manoucher; Asgari, Korosh; Massahnia, Sara // Tanaffos;2013, Vol. 12 Issue 3, p62 

    Botryomycosis is a relatively rare disease found only in case reports. Most observed cases have been of cutaneous or visceral type. Given the prolonged duration and nature of symptoms, pulmonary botryomycosis may be mistaken for malignancy. We report the first case of pulmonary botryomycosis in...

  • Primary pulmonary Hodgkin lymphoma. Tanveer, Shumaila; El Damati, Ahmed; El Baz, Ayman; Alsayyah, Ahmed; ElSharkawy, Tarek; Regal, Mohamed // Rare Tumors;2015, Vol. 7 Issue 4, p145 

    Primary pulmonary Hodgkin lymphoma (PPHL) is a rare disease. Herein, we report a case of PPHL with diagnostic concerns encountered during initial evaluation which is of paramount importance to keep the differential diagnosis in cases with high index of suspicion for this rare entity.

  • Primary sclerosing cholangitis. Worthington, Joy; Chapman, Roger // Orphanet Journal of Rare Diseases;2006, Vol. 1, p41 

    Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease of unknown aetiology characterised by inflammation and fibrosis of the biliary tree. The mean age at diagnosis is 40 years and men are affected twice as often as women. There is a reported annual incidence of PSC of 0.9-...

  • Foetal and neonatal alloimmune thrombocytopaenia. Kaplan, Cecile // Orphanet Journal of Rare Diseases;2006, Vol. 1, p39 

    Foetal/neonatal alloimmune thrombocytopaenia (NAIT) results from maternal alloimmunisation against foetal platelet antigens inherited from the father and different from those present in the mother, and usually presents as a severe isolated thrombocytopaenia in otherwise healthy newborns. The...

  • Variability of the chronic obstructive pulmonary disease key epidemiological data in Europe: systematic review. Atsou, Kokuvi; Chouaid, Christos; Hejblum, Gilles // BMC Medicine;2011, Vol. 9 Issue 1, p7 

    Background: Chronic obstructive pulmonary disease (COPD) is predicted to become a major cause of death worldwide. Studies on the variability in the estimates of key epidemiological parameters of COPD may contribute to better assessment of the burden of this disease and to helpful guidance for...

  • Should We Be Using Oral Decontamination with Ventilated Patients? Luks, Andrew M. // Critical Care Alert;Jan2008, Vol. 15 Issue 10, p4 

    The article presents a study which demonstrates that oral decontamination with antiseptic preparations decreases the risk of ventilator-associated pneumonia.

Share

Read the Article

Courtesy of VIRGINIA BEACH PUBLIC LIBRARY AND SYSTEM

Sorry, but this item is not currently available from your library.

Try another library?
Sign out of this library

Other Topics