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]

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

In summary, we present 2 cases of bilateral orbital lymphangiomas in patients with generalized lymphangiomatosis. [ajnr.org]

More than 75% of the cases have bone lesions that present as osteolytic foci. [1] The present cases differ from majority since no lytic lesion, bone absorption, or atrophy were seen both at the local and systemic level. [cancerjournal.net]

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]

Clinical Testing and Workup Pulmonary function tests may be used to help diagnose diffuse pulmonary lymphangiomatosis. This group of tests can evaluate how well the lungs are functioning. [rarediseases.org]

[…] histological features of lung specimens from nine patients in whom no extrathoracic lesions were identified, which they termed “diffuse pulmonary lymphangiomatosis” (DPL). 3 Recognition of the disease requires a high index of suspicion and an extensive workup [lgdalliance.org]

MRI showed obvious tumor shrinkage at 6 ( f ), 12 ( g ), and 24 ( h ) months after the start of sirolimus treatment Pathological findings All patients with KLA underwent incisional biopsy during the diagnostic workup (Fig. 1 d). [ojrd.biomedcentral.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]

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

Treatment with sirolimus and prednisolone was then initiated. [ojrd.biomedcentral.com]

Talk with your doctor to find the best treatment if you or your child has been diagnosed with pulmonary lymphangiomatosis. [resourcerepository.org]

Standard Therapies Treatment The treatment of diffuse pulmonary lymphangiomatosis is directed toward the specific symptoms that are apparent in each individual. Treatment may require the coordinated efforts of a team of specialists. [rarediseases.org]

It has a favorable prognosis when it is localized, and a poor prognosis when vital organs, including vertebral medulla, are involved. 3 When the disease is diffuse and extensive in a particular organ, as in the lungs in our case, the prognosis is also [scielo.br]

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 is generally poor. [3] There is no evidence of visceral involvement in the present cases. [cancerjournal.net]

[…] often in thoracic cavity, also bone, extremities, soft tissue, viscera Clinical features Rare, idiopathic, occurs mostly in children Cases with visceral involvement are often fatal ( Am J Surg Pathol 1993;17:329 ), but cases involving limbs have good prognosis [pathologyoutlines.com]

The systemic form which was noted in this case above differs from the localised forms of the disease – which are isolated to a single anatomic location, classically bone, and have a good prognosis. [ghrnet.org]

Etiology 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. [orpha.net]

The disease can be considered a failure of normal lymphatic development, but the etiology remains poorly understood. Treatment has been limited to surgical resection or drainage procedures because there is no accepted effective systemic therapy. [genome.jp]

The proliferative lymphatic aspect suggests a neoplastic etiology, and the structural disorganization indicates a hamartomatous origin. [scielo.br]

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]

Summary Epidemiology The prevalence is unknown. [orpha.net]

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]

There is limited information available on the pathophysiology, treatment responses, and long-term outcomes of these disorders. [atsjournals.org]

Treatment Treatment Options: No effective preventative treatment exists but individual lesions can be surgically removed when indicated. [disorders.eyes.arizona.edu]

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

Transplantation Substance Use and Addiction Surgery Surgical Innovation Surgical Pearls Teachable Moment Technology and Finance The Rational Clinical Examination Tobacco and e-Cigarettes Toxicology Trauma and Injury Treatment Adherence United States Preventive [jamanetwork.com]

The biologic effect of imatinib, when prescribed at higher concentrations, has been shown to inhibit angiogenesis by blocking the activity of c-kit tyrosine kinase cell surface receptor and PDGFR kinase, 22 and through this mechanism, is perhaps preventing [jnccn.org]