The patients comprised seven males and two females with a mean age at presentation of 10 years (age range, 1 month to 33 years). Six patients were younger than 10 years of age. [ncbi.nlm.nih.gov]

The present article discusses clinical, radiographic and pathological features, and treatment options for DPL. [mayoclinic.pure.elsevier.com]

Despite the poor prognosis of the disease, 5 years after the treatment the patient has not presented new episodes of pleuropericardial effusions, nor has he required hospitalization for other symptoms, currently presenting only exertional dyspnea. [archbronconeumol.org]

In this paper, We would like to present an unusual case of Diffuse pulmonary lymphangiomatosis involving a 28-year-old young female, who presented with progressive chest pain and polypnea of one year duration, and CT showed much left hydrothorax. [atm.amegroups.com]

• Recurrent Pleural Effusion

  […] and aspiration with pleurodesis for patients with recurrent pleural effusion. [adc.bmj.com]

  Palliative procedures include medical therapy with interferon-alpha, draining the pleural effusions, or performing pleurodesis for recurrent pleural effusions. 5 Acknowledgement The authors thank Dr. G. Brink for review of the manuscript. [ispub.com]

  Clinical presentations varies from mild wheezing and moderate cough to marked respiratory insufficiency due to progressive in infiltrative disease and recurrent pleural effusions ( 14 ). [atm.amegroups.com]

  Recurrent pleural effusions may require pleural fluid drainage or pleurodesis. [ijri.org]

  Other surgical procedures have included parietal pleurectomy, pleurodesis and ligation of the thoracic duct to reduce recurrent pleural effusion [ 71 – 74 ]. [err.ersjournals.com]

• Pleural Effusion

  Seven of the eight had bilateral pleural effusions or smooth thickening of the pleura (or both), and two had regions of calcified pleural thickening. [ncbi.nlm.nih.gov]

  Definition / general Diffuse proliferation of abnormal, complex lymphatic channels (lymphangiomas) that may involve lung or other organs ( Lymphat Res Biol 2011;9:191 ) Pulmonary involvement often accompanied by chylous pleural effusions May be progressive [pathologyoutlines.com]

  effusion and a pericardial effusion. [unboundmedicine.com]

  Pleural effusions are often chylous, the same with our case, and patients may have associated asthma syndrome, chyloptysis, hemoptysis or chylopericardium. [atm.amegroups.com]

  Tube Pleurodesis Ligation of the thoracic duct Resolution of pleural effusion Stabilization of bony lesions 5 Bilateral pleural effusions Pericardial effusion Resolution of effusions 6 7 months Bilateral chylous pleural effusions Bone lesions T11-L4 [slideplayer.com]

• Dyspnea

  He came to the emergency unit due to dyspnea with minimal exertion that had been evolving over the period of a month. [archbronconeumol.org]

  Symptoms included "wheezing" or "asthma" (44%) and dyspnea (22%) present for 3 months to 20 years. Chest radiographs showed bilateral interstitial infiltrates, often greatest in the lower lobes. [ncbi.nlm.nih.gov]

  They present with chylous effusion and dyspnea. A restrictive pattern is noted in pulmonary function tests. [ispub.com]

  […] liver Pathophysiology / etiology Progressive disease with recurrent effusions and respiratory failure secondary to infections and chylous accumulation Congenital, possibly from abnormal lymphatic development Clinical features Generally presents with dyspnea [pathologyoutlines.com]

  Age Mean at presentation: 8.5 years May manifest at birth and rarely in adults Gender No predilection Site Localized to lung or chest Widespread distribution involving spleen, bones (e.g., ribs, vertebrae, humerus, femur), liver, and skin Presentation Dyspnea [basicmedicalkey.com]

• Hemoptysis

  This 50-year-old nonsmoking Hispanic woman presented with a 20-year history of cough, hemoptysis, chyloptysis, and pleuritic chest pain. [experts.umich.edu]

  […] respiratory failure secondary to infections and chylous accumulation Congenital, possibly from abnormal lymphatic development Clinical features Generally presents with dyspnea, wheezing, cough and may be confused with asthma Occasionally with chyloptysis or hemoptysis [pathologyoutlines.com]

  […] presentation: 8.5 years May manifest at birth and rarely in adults Gender No predilection Site Localized to lung or chest Widespread distribution involving spleen, bones (e.g., ribs, vertebrae, humerus, femur), liver, and skin Presentation Dyspnea Cough Hemoptysis [basicmedicalkey.com]

  In Kaposiform lymphangiomatosis, lymphatic spindle cells invade bronchoalveolar bundles and pulmonary septae, resulting in hemoptysis and hemothorax; thrombocytopenia and anemia may be associated with a positive disseminated intravascular coagulation [thoracic.org]

  A 19-year-old student presented with complaints of productive cough and progressive dyspnea for 18 months and hemoptysis for 6 months. He did not have palpitations, chest pain, or fever. [ijri.org]

• Pulmonary Disorder

  Diffuse pulmonary lymphangiomatosis is a rare pulmonary disorder affecting the lymphatic channels from the mediastinum to the pleura. The disease usually occurs in children and young adults and frequently ends with death due to progressive course. [ncbi.nlm.nih.gov]

  Diffuse pulmonary lymphangiomatosis is a rare pulmonary disorder affecting the lymphatic channels from the mediastinum to the pleura. [ispub.com]

• Exertional Dyspnea

  He came to the emergency unit due to dyspnea with minimal exertion that had been evolving over the period of a month. [archbronconeumol.org]

  Clinical presentation Pulmonary arterial hypertension manifests the following symptoms and signs: Symptoms Progressive onset of exertional dyspnea (60%) 14 Chest pain or discomfort (17%) Palpitations (5%) Dizziness and light-headedness. [mdedge.com]

• Chest Pain

  Case presentation The patient was a 28-year-old female who had complained of chest pain and polypnea for one year. [atm.amegroups.com]

  This 50-year-old nonsmoking Hispanic woman presented with a 20-year history of cough, hemoptysis, chyloptysis, and pleuritic chest pain. [experts.umich.edu]

  Widespread distribution involving spleen, bones (e.g., ribs, vertebrae, humerus, femur), liver, and skin Presentation Dyspnea Cough Hemoptysis Chest pain Milky sputum Pleural effusions Mediastinal enlargement Laboratory Tests Pulmonary functional tests [basicmedicalkey.com]

  Symptoms include chest pain and pressure, coughing up blood and shortness of breath. Talk with your doctor to find the best treatment if you or your child has been diagnosed with pulmonary lymphangiomatosis. [diseaseinfosearch.org]

  He tolerated treatment well and showed an excellent symptomatic response to therapy with a significant reduction in his dyspneic episodes, chest and body pain. [ijamhrjournal.org]

[…] histological features of lung specimens from nine patients in whom no extrathoracic lesions were identified, which they termed "diffuse pulmonary lymphangiomatosis" (DPL). [4] Recognition of the disease requires a high index of suspicion and an extensive workup [en.wikipedia.org]

In this series, four patients with pericardial effusions were at first thought to have nonneoplastic disease, the workups of whom included two negative bloody pericardial fluid cytology exams, and two patients were initially treated with prednisone for [nature.com]

Further workup for PHACE may be needed on the basis of central nervous system (CNS) findings. [northshore.org]

• Pulmonary Infiltrate

  The CT scans were analyzed for type of pulmonary infiltrative process and its distribution, presence or absence of pleural fluid or thickening, and presence or absence of thoracic lymphadenopathy. [ncbi.nlm.nih.gov]

  Página 54 - Ward HN: Pulmonary infiltrates associated with leukoagglutinin transfusion reactions. Ann Intern Med 73:689-694. 1970. 234. Thompson JSA. [books.google.es]

  Sometimes massive pleural effusion and progressive pulmonary infiltration cause respiratory failure [ 15 ]. [err.ersjournals.com]

• Kerley B Lines

  Key Facts Rare non-langerhans cell histiocytosis Pleural and perirenal soft tissue thickening Diffuse septal thickening Imaging Findings Chest radiograph Cardiomegaly, Kerley B lines, blunting costophrenic angles (mimic CHF) Sclerotic bone lesions CT/ [chestx-ray.com]

  His chest radiograph at presentation showed mediastinal widening, bilateral pleural effusions, and Kerley B lines. [ijri.org]

  Clin Radiol. 2002; 57:8–17. 4.14 Septal (Kerley B) lines Pulmonary lobules are the smallest lung units bounded by interlobular septa. Each lobule is supplied by a lobular airway and arterial branch. [clinicalgate.com]

• X-Ray Abnormal

  […] of tissue well before the abnormalities become detectable on the chest x-ray or CT scan. [anyflip.com]

• Dyslipidemia

  We present the clinical case of a 55-year-old male patient, ex-smoker with a history of hypertension, dyslipidemia and COPD with bronchodilator treatment. [archbronconeumol.org]

• Decreased Lung Compliance

  Decreased lung compliance increases the work of breathing and energy expenditure. Energy supplementation should be undertaken with consideration to the added difficulty in handling high carbohydrate loads with chronic lung disease. [emedicine.medscape.com]

• Pleural Effusion

  Seven of the eight had bilateral pleural effusions or smooth thickening of the pleura (or both), and two had regions of calcified pleural thickening. [ncbi.nlm.nih.gov]

  Definition / general Diffuse proliferation of abnormal, complex lymphatic channels (lymphangiomas) that may involve lung or other organs ( Lymphat Res Biol 2011;9:191 ) Pulmonary involvement often accompanied by chylous pleural effusions May be progressive [pathologyoutlines.com]

  effusion and a pericardial effusion. [unboundmedicine.com]

  Pleural effusions are often chylous, the same with our case, and patients may have associated asthma syndrome, chyloptysis, hemoptysis or chylopericardium. [atm.amegroups.com]

  Tube Pleurodesis Ligation of the thoracic duct Resolution of pleural effusion Stabilization of bony lesions 5 Bilateral pleural effusions Pericardial effusion Resolution of effusions 6 7 months Bilateral chylous pleural effusions Bone lesions T11-L4 [slideplayer.com]

• Pleural Adhesions

  Lungs were congested, edematous, of increased consistence, with bilateral pleural adhesions, pleural fibrinous reaction at the base of the lungs ( Figures 3C and 3D ) and a relevant bilateral pleural effusion. [shortnessofbreath.it]

The present article discusses clinical, radiographic and pathological features, and treatment options for DPL. [mayoclinic.pure.elsevier.com]

It may be erroneously diagnosed as asthma responding to bronchodilator treatment, which was the reason for the late diagnosis in our case, previously diagnosed with COPD with bronchodilator treatment. [archbronconeumol.org]

Related Articles High hopes, realistic expectations drive bevacizumab treatment. [connection.ebscohost.com]

Although both effusions initially recurred and required subsequent drainage and treatment by colchicine, evolution at 19 months of diagnosis is favorable. [unboundmedicine.com]

Oral propranolol, which had been proposed as an alternative treatment for lymphatic malformation, was attempted to administrate at 0.5 mg/kg/day initially, then it was increased to 1.5 mg/kg/day. 5 months after treatment with propranolol, the patient [lungkorea.org]

This case illustrates the fact that the prognosis of diffuse pulmonary lymphangiomatosis in adults may be characterized by a less aggressive evolution and by a more favorable prognosis than that observed in children. [unboundmedicine.com]

Although it is a benign disease of unknown etiology and pathogeny, it presents a high rate of relapse and a poor prognosis. [archbronconeumol.org]

The prognosis for the patients with this disease limited to the thorax is guarded and progressive although some patients have realtively indolent course. [lymphsystemdisorders.blogspot.com]

Milky sputum Pleural effusions Mediastinal enlargement Laboratory Tests Pulmonary functional tests demonstrate Mixed pattern obstructive/restrictive Restrictive pattern only Natural History Progressive condition leading to end-stage respiratory failure Prognosis [basicmedicalkey.com]

[…] if proliferation is focal Epidemiology Rare condition, typically presents in children and young adults No sex predilection Sites Localized to lung/chest or can also involve multiple organs, including bone, spleen, soft tissue, liver Pathophysiology / etiology [pathologyoutlines.com]

ETIOLOGY/PATHOGENESIS Developmental Anomaly Congenital disease due to abnormal lymphatic system development CLINICAL ISSUES Epidemiology Age Mean at presentation: 8.5 years May manifest at birth and rarely in adults Gender No predilection Site Localized [basicmedicalkey.com]

Imagining studies showed interstitial lung disease of unknown etiology. The lung tissue biopsy confirmed that the diagnosis was diffuse pulmonary lymphangiomatosis. [ispub.com]

Although the etiology of most cases of DAD cannot be determined histologically, the cause of the damage can usually be obtained from... [brainscape.com]

Sarcoidosis Sarcoidosis is a systemic noncaseating granulomatous disease of unknown etiology that affects the mediastinum, lungs, and other organs to varying degrees. [radiologykey.com]

On this page: Article: Epidemiology Radiographic features See also References There is no recognized gender predilection. It typically manifests in children and young adults. [radiopaedia.org]

[…] lymphangiomas) that may involve lung or other organs ( Lymphat Res Biol 2011;9:191 ) Pulmonary involvement often accompanied by chylous pleural effusions May be progressive and fatal from respiratory failure Terminology Lymphangioma if proliferation is focal Epidemiology [pathologyoutlines.com]

ETIOLOGY/PATHOGENESIS Developmental Anomaly Congenital disease due to abnormal lymphatic system development CLINICAL ISSUES Epidemiology Age Mean at presentation: 8.5 years May manifest at birth and rarely in adults Gender No predilection Site Localized [basicmedicalkey.com]

Epidemiology Lymphangiomatosis may present at any age but is predominantly a condition of children and young adults, with no gender predilection 8. Clinical features Patients present with "asthma", dyspnea or haemoptysis 8 ,12. [e-immunohistochemistry.info]

Screening also assists in epidemiological surveillance of diseases caused by exposure to asbestos. REFERENCES 1. [anyflip.com]

Terminology Lymphangioma if proliferation is focal Epidemiology Rare condition, typically presents in children and young adults No sex predilection Sites Localized to lung/chest or can also involve multiple organs, including bone, spleen, soft tissue, liver Pathophysiology [pathologyoutlines.com]

Large-vessel in situ thromboses Pulmonary emboli result in two main pathophysiologic consequences Occlusion of the large pulmonary arteries is almost always caused by... [brainscape.com]

[…] microcystic) 21 Evaluation CT MRI Lymphangiograms Pulmonary Function Tests 22 Treatment Treat the anomaly: medicine, intervention, surgery Pulmonary medications Respiratory therapy Physical therapy Lung transplant 23 Questions to be answered What is the pathophysiology [slideplayer.com]

Pathogenesis and pathophysiology Pulmonary hypertension can be caused by narrowing of the precapillary vessels (arteries and arterioles), loss of vascular surface area, or passive back pressure from the postcapillary vessels [see Table 2]. [what-when-how.com]

It highlights the need for further research into the pathophysiology and management of lymphangiomatosis as current treatment options are limited. http://www.liebertonline.com/doi/abs/10.1089/lrb.2011.0021 .................... [lymphedemapeople.com]

Lotery, Andrew; MacEwen, Carrie // BMJ: British Medical Journal;11/22/2014, Vol. 349 Issue 7894, pg6887 The authors stress the need for the British government to act to remove the hurdles from preventing the use of bevacizumab for the treatment of age [connection.ebscohost.com]

In the case of recurring chylothorax, drainage and pleurodesis are indicated, with the option of thoracic duct ligation 3 to prevent complications such as malnutrition, pulmonary fibrosis and deteriorated respiratory function. [archbronconeumol.org]

Radiation therapy to the brain may also help treat or prevent metastasis. [anyflip.com]

You agree to immediately notify us in the event you determine, or have reason to believe, that an unauthorized party has gained access to the Materials and to take all reasonable steps, both to ensure that such activity ceases and to prevent any recurrence [pediatriccare.solutions.aap.org]