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]

• Recurrent Pleural Effusion

  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]

  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]

  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]

• 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]

  ribs, vertebrae, humerus, femur), liver, and skin Presentation Dyspnea Cough Hemoptysis Chest pain Milky sputum Pleural effusions Mediastinal enlargement Laboratory Tests Pulmonary functional tests demonstrate Mixed pattern obstructive/restrictive [basicmedicalkey.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]

  One month later after his discharge, he complained of mild cough and dyspnea again. Follow-up chest CT revealed more extended crazy paving appearance on both lungs with recurred pleural effusion. [lungkorea.org]

• Hemoptysis

  ribs, vertebrae, humerus, femur), liver, and skin Presentation Dyspnea Cough Hemoptysis Chest pain Milky sputum Pleural effusions Mediastinal enlargement Laboratory Tests Pulmonary functional tests demonstrate Mixed pattern obstructive/restrictive [basicmedicalkey.com]

  […] 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]

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

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

• Exertional Dyspnea

  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]

  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]

• 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]

• 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]

  ribs, vertebrae, humerus, femur), liver, and skin Presentation Dyspnea Cough Hemoptysis Chest pain Milky sputum Pleural effusions Mediastinal enlargement Laboratory Tests Pulmonary functional tests demonstrate Mixed pattern obstructive/restrictive [basicmedicalkey.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]

  The complications of Diffuse Pulmonary Lymphangiomatosis may include the following: In general, lymphangiomatosis can affect any part of the body Severe breathing difficulties leading to respiratory failure resulting in death Severe chest pain Frequent [dovemed.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]

• Facial Edema

  edema(1/18,5.56%) and bone or vertebral lesions(3/18,16.67%).Conclusion MSCT direct lymphangiography can clearly show the lymphatic abnormal return and the dilatation and proliferation of lymphatic channels by abnormal distribution of contrast agent, [en.cnki.com.cn]

[…] 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

  The converse may also be true, with the severity of symptoms and/or pulmonary function test results seemingly out of proportion to the degree of x-ray abnormality. [anyflip.com]

• Right Pleural Effusion

  Chest and abdominal radiograph showed massive right pleural effusion causing collapse of the right lung with contralateral mediastinal shift and ascites [Figure 2]. [ijamhrjournal.org]

• 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]

• Human Herpesvirus 8

  Kaposi’s sarcoma is a cancer caused by infection with human herpesvirus 8 (HHV8), also resulted in patches of abnormal tissue to grow under the skin, in the lining of the mouth, nose, and throat or in other organs. [atm.amegroups.com]

  Dow DE, Cunningham CK, Buchanan AM: A Review of Human Herpesvirus 8, the Kaposi's Sarcoma-Associated Herpesvirus, in the Pediatric Population. J Pediatric Infect Dis Soc 3 (1): 66-76, 2014. [northshore.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]

• 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]

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]

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

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

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]

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]

[…] 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]

Pulmonary lymphangioleiomyomatosis (LAM) is a benign, uncommon disorder of unknown etiology affecting women of child-bearing age ( 7 ). [atm.amegroups.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]

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]

[…] 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]

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]

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]

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]

Somatic activating mutations of GNAQ and GNA11 have been found to be associated with congenital hemangiomas.[ 81 ] Additional research is necessary to assess the significance of these findings, as this may aid in diagnosis and pathophysiology. [northshore.org]

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]

This procedure thereby obliterates the space between the pleura and prevents the reaccumulation of fluid. [lymphedemapeople.com]

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