• and rare forms of ILD such as lymphangioleiomyomatosis (LAM) or Langerhans cell histiocytosis (LCH) account for the remaining subgroups. (springer.com)
  • citation needed] Cystic lung diseases include: Langerhans cell histiocytosis (LCH) Lymphangioleiomyomatosis (LAM) Lymphocytic interstitial pneumonia (LIP) Birt-Hogg-Dubé syndrome Pneumocystis pneumonia Pulmonary amyloidosis Light chain deposition disease Lung metastases rarely cause multiple cystic lung lesions. (wikipedia.org)
  • Finally, a number of very rare forms of DPLDs exist, including pulmonary Langerhans cell histiocytosis (PLCH) (see Eosinophilic Granuloma (Histiocytosis X) ), tuberous sclerosis, lymphangioleiomyomatosis (LAM) (see Lymphangioleiomyomatosis ), and Hermansky-Pudlak syndrome. (medscape.com)
  • However, some diseases, such as sarcoidosis and lymphangioleiomyomatosis (LAM), may have only decreased breath sounds without adventitious sounds despite a markedly abnormal chest radiograph. (thoracickey.com)
  • The various etiologies identified were lymphangioleiomyomatosis (LAM), lymphocytic interstitial pneumonia, Birt-Hogg-Dube syndrome, hypersensitivity pneumonitis, Pneumocystis jirovecii pneumonia, and cystic metastasis, and in one patient, no definite cause was found despite detailed evaluation. (ijcdas.com)
  • Lymphangioleiomyomatosis (LAM) is a rare lung disease, resulting from proliferation in the lung, kidney, and axial lymphatics of abnormal smooth muscle-like cells (LAM cells) that exhibit features of neoplasia and neural crest origin. (medscape.com)
  • Earlier reports indicated a grim prognosis for lymphangioleiomyomatosis (LAM), with progressive respiratory failure and death within 10 years of diagnosis. (medscape.com)
  • Lymphangioleiomyomatosis: When both lymphangioleiomyomatosis (LAM) and renal AMLs are present, other features of tuberous sclerosis should be present before a definite diagnosis is assigned. (medscape.com)
  • Lymphangioleiomyomatosis (LAM ) is a rare disease characterized by morphofunctional alterations and caused by the proliferation of immature smooth muscle cells within different organs. (birdfoundation.org)
  • This contains not only ailments with well-defined clinical pathologic features, such as sarcoidosis, pneumoconiosis, lymphangioleiomyomatosis (LAM), or pulmonary Langerhans cell histiocytosis (PLCH), but also a cluster of common disorders as "idiopathic interstitial pneumonia" (IIP). (lungswiki.com)
  • Lymphangioleiomyomatosis (LAM) is a rare idiopathic disease affecting women that was first described by von Stossel in 1937. (medscape.com)
  • With clinical suspicion, lymphangioleiomyomatosis (LAM) has been diagnosed on the basis of compatible chest radiograph, pulmonary function tests (PFTs), and computed tomography (CT) scan findings. (medscape.com)
  • Chest radiograph and pulmonary function test (PFT) findings, while suggestive of lymphangioleiomyomatosis (LAM), can be nonspecific and may be normal despite the presence of symptoms. (medscape.com)
  • A high index of clinical suspicion for lymphangioleiomyomatosis (LAM) should be maintained in women of childbearing age who present with recurrent pneumothoraces, when chylous effusion or an interstitial pattern on chest radiograph is not identified. (medscape.com)
  • Idiopathic pulmonary fibrosis (31%) and connective-tissue disease related ILD (21.7%) were the two most common subtypes. (springer.com)
  • Interstitial lung disease (ILD) encompasses a heterogeneous group of respiratory disorders characterised by inflammation and/or fibrosis of the lung interstitium. (springer.com)
  • Firstly, there are the Idiopathic Interstitial Pneumonias (IIPs) including Idiopathic Pulmonary Fibrosis (IPF), the most common IIP, along with idiopathic non-specific idiopathic pneumonia (iNSIP), acute interstitial pneumonia (AIP) and respiratory bronchiolitis-associated ILD (RB-ILD), to name a few. (springer.com)
  • DPLD may be idiopathic, a classic illustration of which is idiopathic interstitial fibrosis (IPF), which is discussed in another article (see Idiopathic Pulmonary Fibrosis ). (medscape.com)
  • This article presents a broad overview, with an emphasis on those etiologies that result in pulmonary fibrosis not discussed elsewhere in this series. (medscape.com)
  • It is thought to begin with acute injury to the pulmonary parenchyma, leading to chronic interstitial inflammation, then to fibroblast activation and proliferation, and finally progressing to the common endpoint of pulmonary fibrosis and tissue destruction. (medscape.com)
  • When responding to any injury-whether from a specific exposure (e.g., asbestos, nitrofurantoin, or moldy hay), an autoimmune-mediated inflammation from a systemic connective tissue disease (e.g., rheumatoid arthritis), or unknown injury (e.g., idiopathic pulmonary fibrosis [IPF])-the lung must respond to the damage and repair itself. (thoracickey.com)
  • cIdiopathic pulmonary fibrosis. (medscape.com)
  • I have suffered from NSIP for four years, with IPF (idiopathic pulmonary fibrosis). (biotherapy.asia)
  • When the alveoli are damaged, thick-walled cysts are abandoned, causing the lungs to simulate a honeycomb and eventually leading to a treatment of pulmonary fibrosis. (lungswiki.com)
  • Cystic fibrosis and Langerhans cell histiocytosis (eosinophilic granuloma) share this feature. (medscape.com)
  • Lymphangiomyomatosis must be differentiated from other diseases that cause similar clinical features, such as asthma , spontaneous pneumothorax , emphysema , interstitial pulmonary fibrosis , eosinophilic granuloma (EG), Birt-Hogg-Dube syndrome, lymphangiomas , pulmonary lymphangiectasis, and leiomyosarcoma . (wikidoc.org)
  • Pulmonary fibrosis share the pathogenesis process of interstitial lung disease which involve the pulmonary parenchyma . (wikidoc.org)
  • The deposition of nanoparticles in the lung can lead to chronic inflammation, epithelial injury, and further to pulmonary fibrosis. (mcgill.ca)
  • Cases of particle-induced pulmonary fibrosis, namely pneumoconiosis, are mostly occupationally influenced, and continue to be documented around the world. (mcgill.ca)
  • The tremendous growth of nanotechnology, however, has spurred fears of increased rates of pulmonary diseases, especially fibrosis. (mcgill.ca)
  • Many acute and chronic lung disorders with variable degrees of pulmonary inflammation and fibrosis are collectively referred to as interstitial lung diseases (ILDs) or diffuse parenchymal lung diseases. (atsjournals.org)
  • Idiopathic pulmonary fibrosis (or cryptogenic fibrosing alveolitis) (IPF or CFA) is one of several idiopathic interstitial pneumonias. (atsjournals.org)
  • The purpose of this consensus statement is to provide assistance to clinicians in the diagnosis and management of idiopathic pulmonary fibrosis (IPF). (atsjournals.org)
  • At the outset it should be stressed that these mechanisms are very much speculative in nature and include a ball-valve effect causing bronchial dilatation, focal pulmonary necrosis and retractile fibrosis. (springeropen.com)
  • Transforming growth factor-β (TGFβ) is a major mediator of normal wound healing and of pathological conditions involving fibrosis, such as idiopathic pulmonary fibrosis. (aspetjournals.org)
  • Microarray analysis of human lung fibroblasts identified molecular fingerprints of these processes and showed that SD-208 had global effects on reversing TGFβ-induced genes involved in fibrosis, inflammation, cell proliferation, cytoskeletal organization, and apoptosis. (aspetjournals.org)
  • Our findings suggest that inhibitors such as SD-208 may be therapeutically useful in human interstitial lung diseases and pulmonary fibrosis. (aspetjournals.org)
  • The cyst for example in pneumocystis pneumonia is not the same as the pulmonary cyst. (wikipedia.org)
  • Other major histopathologic forms of idiopathic interstitial pneumonias include the following: desquamative interstitial pneumonia (DIP), respiratory bronchiolitis interstitial lung disease (RBILD), acute interstitial pneumonitis (AIP), also known as Hamman-Rich syndrome, nonspecific interstitial pneumonia (NSIP), cryptogenic organizing pneumonia (COP) (see Imaging in Bronchiolitis Obliterans Organizing Pneumonia ), and lymphocytic interstitial pneumonia (LIP) (see Lymphocytic Interstitial Pneumonia ). (medscape.com)
  • Large lung volumes and interstitial disease on plain film also can be seen with Langerhans cell histiocytosis, sarcoidosis, and extrinsic allergic alveolitis. (medscape.com)
  • The underlying histopathology of IPF is usual interstitial pneumonitis (UIP). (medscape.com)
  • The diseases cause inflammation or scarring of the lung tissue (interstitial lung disease) or result in filling of the air spaces with exudate and debris (pneumonitis). (medscape.com)
  • dNonspecific interstitial pneumonitis. (medscape.com)
  • Some of the other names for this condition are cryptogenic fibrosing alveolitis (CFA), diffuse interstitial lung disease, idiopathic pulmonary pneumonitis (IPP), and alveolitis. (lungswiki.com)
  • Dr. Jonathan Singer is an Associate Professor of Medicine in the Division of Pulmonary, Critical Care, Allergy and Sleep Medicine. (ucsf.edu)
  • Pulmonary hypertension is a condition in which blood pressure in the arteries of the lungs (the pulmonary arteries) is abnormally high. (msdmanuals.com)
  • Many disorders can cause pulmonary hypertension. (msdmanuals.com)
  • If the pressure of the blood in the pulmonary arteries increases to a sufficiently high level, the condition is called pulmonary hypertension. (msdmanuals.com)
  • In pulmonary hypertension, the right side of the heart must work harder to push the blood through the pulmonary arteries. (msdmanuals.com)
  • Cor Pulmonale Cor pulmonale is enlargement and thickening of the ventricle on the right side of the heart resulting from an underlying lung disorder that causes pulmonary hypertension (high pressures in the. (msdmanuals.com)
  • Pulmonary arterial hypertension can be caused by numerous different disorders. (msdmanuals.com)
  • Women are affected by idiopathic pulmonary hypertension twice as often as men, and the average age at which the diagnosis is made is about 35 years. (msdmanuals.com)
  • The actual mechanism by which these inherited genetic mutations cause pulmonary hypertension is not yet known. (msdmanuals.com)
  • A number of drugs and toxins have been identified as risk factors for pulmonary arterial hypertension such as fenfluramine (and other related weight-loss drugs), amphetamines, protein kinase inhibitors (such as dasatinib ), cocaine , and selective serotonin reuptake inhibitors (SSRIs). (msdmanuals.com)
  • Persistent Pulmonary Hypertension of the Newborn Persistent pulmonary hypertension of the newborn is a serious disorder in which the arteries to the lungs remain narrowed (constricted) after delivery, thus limiting the amount of blood flow. (msdmanuals.com)
  • Signs of pulmonary arterial hypertension with right ventricular dysfunction, such as lower extremity edema or jugular venous distention, may occur late in the course of any ILD and are not helpful in the diagnosis of a specific ILD. (thoracickey.com)
  • As the name ILD implies, the histologic abnormalities that characterize ILD involve the pulmonary interstitium to a greater extent than the alveolar spaces or airways, although exceptions exist. (thoracickey.com)
  • A, Diagram of the pulmonary parenchyma shows the respiratory bronchiole, alveolar duct, and alveolar sacs. (thoracickey.com)
  • B, The constituents of the interstitial space, including type I and type II alveolar epithelial cells, a capillary with vascular endothelial cells and erythrocytes in transit, resident macrophages, interstitial fibroblasts, and matrix substance. (thoracickey.com)
  • The mnemonic "PAINT" has been used to divide the causes of restrictive lung disease into pleural, alveolar, interstitial, neuromuscular, and thoracic cage abnormalities. (medscape.com)
  • This proliferation of immature muscle cells starts covering alveolar walls, bronchioles , pleura and vessels, including lymphatic routes. (wikidoc.org)
  • Pulmonary macrophages, such as tissue-resident alveolar and interstitial macrophages and recruited monocyte-derived macrophages, are the major macrophages present in the lungs during homeostasis and diseased conditions. (biomedcentral.com)
  • 2022 ). Alveolar macrophages (AM) and interstitial macrophages (IM) are two pulmonary resident macrophage populations that are present during homeostatic conditions. (biomedcentral.com)
  • Lung IM resides specifically in the interstitial space but can also be found in the alveolar space in a low percentage (Duan et al. (biomedcentral.com)
  • Previously, LAM was considered an interstitial lung disease but it is now considered to be a low-grade destructive metastasizing neoplasm. (medscape.com)
  • Figure 24-2 illustrates the components of the normal pulmonary parenchyma. (thoracickey.com)
  • Arterial hypoxemia in disorders of pulmonary parenchyma is primarily caused by ventilation-perfusion mismatching, with further contribution from an intrapulmonary shunt. (medscape.com)
  • DPLD (Diffused Parenchymal Lung Disease), commonly known as interstitial lung disease, refers to a collection of non-neoplastic diseases that affect the lung parenchyma, mostly the interstitium, but also peripheral airways, airspaces, and arteries to some extent. (lungswiki.com)
  • Interstitial lung disease is a group of disorders that involve pulmonary parenchyma . (wikidoc.org)
  • These arteries (except the thyroid artery) form a peribronchial plexus that follows the bronchial tree deep into the lung parenchyma to supply blood also to the visceral pleura and the walls of the pulmonary arteries and veins (vasa vasorum). (medscape.com)
  • A focal lung pneumatosis, is an enclosed pocket of air or gas in the lung and includes blebs, bullae, pulmonary cysts, and lung cavities. (wikipedia.org)
  • The presence of multiple pulmonary cysts may indicate a need to evaluate the possibility of bullous or cystic lung diseases. (wikipedia.org)
  • Pulmonary cysts identified on chest CT: are they part of aging change or of clinical significance? (wikipedia.org)
  • The typical appearance of LAM on HRCT is of thin-walled, air-containing cysts ranging from 2-50 mm in a diffuse symmetric pattern. (medscape.com)
  • The loss of these proteins along with the influence of estrogen allows the cell to grow and divide in an uncontrolled way, resulting in the tumors and cysts associated with lymphangiomyomatosis. (wikidoc.org)
  • Excessive proteolytic activity from the proliferation of the smooth muscle cells result in lung destruction and formation of cysts. (wikidoc.org)
  • Pulmonary cysts should first of all be differentiated from pulmonary cavities because these two entities have very different aetiologies. (springeropen.com)
  • In the early stages, nodules (which correspond with Langerhans cell granulomas) are the predominant features, while cysts tend to develop later [ 4 ]. (springeropen.com)
  • Video-assisted thoracoscopic surgery (VATS) provides better option concerning pathological diagnosis and curative intention of small pulmonary nodules (SPNs) that are sometimes challenging to localize. (bvsalud.org)
  • Additionally, the depth of pulmonary nodules was significantly associated with the occurrence of pneumothorax (P = 0.036) and parenchymal hemorrhage (P = 0.000). (bvsalud.org)
  • 2 Krochmal R, Arias S, Yarmus L, Feller-Kopman D, Lee H. Diagnosis and management of pulmonary nodules. (thieme-connect.com)
  • Specific questioning is often necessary, as TSC lesions often are ascribed to other causes, eg, pulmonary involvement as emphysema, renal lesions as "atypical Wilms tumors," etc. (medscape.com)
  • With the exception of centrilobular emphysema, pulmonary diseases characterised by cystic air spaces are uncommon or rare conditions. (springeropen.com)
  • Pneumothorax is a complication of LAM occurring in 40% of patients at presentation and 66% of patients during the course of the disease. (medscape.com)
  • Pulmonary involvement typically occurs in the second or third decade, with dyspnea, pneumothorax, or chylothorax. (medscape.com)
  • Pulmonary lymphangiomyomatosis (PLAM) is a rare interstitial lung disease characterized by diffuse cystic changes caused by the destructive proliferation of smooth muscle-like cells or LAM cells. (nih.gov)
  • LAM is characterized by nonneoplastic peribronchial, perivascular, and perilymphatic proliferation of atypical smooth muscle resulting in vascular and airway obstruction, cyst formation, and a progressive decline in lung function. (medscape.com)
  • Lymphangiomyomatosis is a disorder resulting from proliferation of abnormal smooth muscle like cells, mostly in the lungs but can also occur in other body parts such as kidney , mediastinum or axial lymphatics . (wikidoc.org)
  • TSC is characterized by autosomal-dominant mutations in the TSC1 or TSC2 genes (encoding for the protein Hamartin on chromosome 9q34 and Tuberin on chromosome 16q13 respectively) leading to overactivation of the mTOR (mechanistic target of rapamycin) pathway with increased cell proliferation and a range of other consequences. (uspharmacist.com)
  • Blood travels from the right side of the heart through the pulmonary arteries into the small blood vessels of the lungs (the capillaries) where carbon dioxide is removed from the blood and oxygen is added. (msdmanuals.com)
  • Normally, the pressure in the pulmonary arteries is low, allowing the right side of the heart to be less muscular than the left side (because relatively little muscle and effort are needed to push the blood through the lungs via the pulmonary arteries). (msdmanuals.com)
  • [ 7 ] Animal models suggest that estrogen may promote the metastasis of TSC2-deficient cells to the lungs. (medscape.com)
  • The interstitium (the pace tissue around the air sacs) of the lungs is affected by interstitial lung disease. (lungswiki.com)
  • Chest x-rays, electrocardiography (ECG), and echocardiography give clues to the diagnosis, but measurement of blood pressure in the right ventricle and the pulmonary artery is needed for confirmation. (msdmanuals.com)
  • The 2017 American Thoracic Society/Japanese Respiratory Society guidelines support a clinical diagnosis of LAM based on high-resolution computed tomography (HRCT) findings typical for LAM (eg, diffuse, thin-walled, round) and accompanied by any of the following clinical features: TSC, renal angiomyolipoma, cystic lymphangioleiomyoma, or chylous pleural effusions in the chest and/or abdomen. (medscape.com)
  • The guidelines give a strong recommendation for the use of vascular endothelial growth factor D testing to establish the diagnosis of LAM before considering lung biopsy in patients with cystic abnormalities on HRCT characteristic of LAM, but no other confirmatory clinical features. (medscape.com)
  • As many as 50% of people with TSC have normal intelligence, and increasingly the diagnosis is being newly made in adults with renal, cutaneous, or pulmonary manifestations. (medscape.com)
  • Renal AML: When both LAM and renal AMLs are present, other features of tuberous sclerosis should be present before a definite diagnosis is assigned (see previous remarks). (medscape.com)
  • When pulmonary features of LAM are identified in males, consider a diagnosis of TSC. (medscape.com)
  • Macrophages, white blood cells, and protein-rich fluid collect in the interstitial spaces in the early stages of various interstitial lung disorders, causing inflammation. (lungswiki.com)
  • In many cases of asthma, type 2 cytokines (IL-4, IL-5, IL-13) produced by type 2 helper T cells (Th2) and type 2 innate lymphocytes (ILC2) induce so-called "type 2 inflammation", resulting in eosinophil-dominated airway inflammation. (apsr2021.jp)
  • A cell block from a pleural effusion may be substituted for a core biopsy. (dana-farber.org)
  • 2013). "Comparing the differential effects of LPA on the barrier function of human pulmonary endothelial cells" . (wikidoc.org)
  • Endothelial cells are sentinels lining the innermost layer of blood vessel that gatekeep micro- and macro-vascular health by sensing pathogen/danger signals and secreting vasoactive molecules. (nature.com)
  • A number of viral species, such as dengue, ebola and cytomegalovirus can infect endothelial cells (ECs) and cause endothelial dysfunction [ 5 ]. (nature.com)
  • This interstitial lung disease (ILD) is also known as diffuse parenchymal lung disease (DPLD). (lungswiki.com)
  • With regard to pulmonary disease, e-cigarettes appear to be strongly implicated in the recent outbreak of acute e-cigarette, or vaping, product use-associated lung injury. (bcmj.org)
  • Figure 24-2 shows, in the normal state, this space allows close apposition of gas and capillaries with minimal connective tissue matrix, fibroblasts, and inflammatory cells such as macrophages. (thoracickey.com)
  • If the exposure or injury persists or if the injury repair process is imperfect, the lung may be permanently damaged with increased interstitial tissue replacing the normal capillaries, alveoli, and healthy interstitium. (thoracickey.com)
  • Typical radiographic findings of reticular interstitial lung disease, recurrent pneumothoraces, and recurrent chylous effusions have been described. (medscape.com)
  • 9 ] The presence of diacetyl in e-liquids is a concern because it is a known pulmonary toxin and has a propensity for causing bronchiolitis obliterans, also known as "popcorn lung. (bcmj.org)
  • This section is written to provide guidance in interpreting pulmonary function tests (PFTs) to medical directors of hospital-based laboratories that perform PFTs, and physicians who are responsible for interpreting the results of PFTs most commonly ordered for clinical purposes. (ersjournals.com)
  • SARS-CoV-2 infection primarily affects the pulmonary system, but accumulating evidence suggests that it also affects the pan-vasculature in the extrapulmonary systems by directly (via virus infection) or indirectly (via cytokine storm), causing endothelial dysfunction (endotheliitis, endothelialitis and endotheliopathy) and multi-organ injury. (nature.com)
  • 2021 ). Not only are macrophages among the first immune cells to encounter viral particles in the lung, but they also maintain lung function by engulfing small debris and regulating surfactant turnover (Aegerter et al. (biomedcentral.com)
  • Pulmonary Macrophages in health and disease: During homeostasis, AM helps in surfactant and cellular debris removal. (biomedcentral.com)
  • There are multiple initiating factors that cause pulmonary injury. (wikidoc.org)
  • Exposure to airborne nanoparticles contributes to many chronic pulmonary diseases. (mcgill.ca)
  • The relationship between e-cigarettes and chronic pulmonary disease is less clear, though possible associations with obstructive spirometric changes, chronic obstructive pulmonary disease, asthma, and chronic cough have been demonstrated. (bcmj.org)
  • Unlike obstructive lung diseases, such as asthma and chronic obstructive pulmonary disease (COPD), which show a normal or increased total lung capacity (TLC), restrictive disease are associated with a decreased TLC. (medscape.com)
  • This is performed in a specialized pulmonary function testing center that is capable of providing emergency asthma care if needed. (uth.edu)
  • Baulig A, Garlatti M, Bonvallot V. Involvement of Reactive Oxygen Species in the Metabolic Pathways triggered by Diesel Exhaust Particles in Human Airway Epithelial Cells. (mcgill.ca)
  • Pulmonary involvement develops most commonly in isolation and occurs in young patients, between the ages of 20 and 40 years, almost all of whom are cigarette smokers. (springeropen.com)
  • Frontal chest radiograph demonstrating bilateral reticular and nodular interstitial infiltrates with upper zone predominance. (medscape.com)
  • Subtle interstitial reticular pattern in a female patient with slightly increased lung volumes. (medscape.com)
  • A symmetrical, diffuse, reticular interstitial pattern (shown in the image below), caused by summation of multiple cyst walls, is typical. (medscape.com)
  • IRE uses HVEPs to cause cell death by inducing membrane disruption, and it is primarily used as a radical ablative therapy in the treatment of soft-tissue tumors in the liver, kidney, prostate, and pancreas. (rsna.org)
  • Interstitial Lung Disease (ILD) is a group of respiratory conditions affecting the lung interstitium often associated with progressive respiratory failure. (springer.com)
  • Some forms of DPLD are related to occupational, environmental, drug, and/or radiation exposure, as well as systemic illness such as collagen-vascular disease (see Interstitial Lung Disease Associated With Collagen-Vascular Disease ). (medscape.com)
  • Pathologically, all DPLDs manifest histologically with disease largely within the interstitial compartment of the lung. (medscape.com)
  • Pulmonary LCH is a rare disease found almost exclusively in cigarette smokers. (radiogyan.com)
  • [ 1 ] . Rates of interstitial lung disease are somewhat higher in men than in women, and the epidemiology is markedly affected by age and occupational exposures. (medscape.com)
  • Of patients referred to a pulmonary disease specialist, an estimated 10-15% have a DPLD. (medscape.com)
  • [ 2 ] However, less than 5% of patients with TSC have pulmonary disease. (medscape.com)
  • Diagnostic yield of transbronchial cryobiopsy in interstitial lung disease: a randomized trial. (thieme-connect.com)
  • Panel members were selected because of an interest and expertise in the interstitial lung disease and to provide a range of opinions, expertise, and geography. (atsjournals.org)
  • LAM is almost exclusively seen in adult women and occurs both sporadically and in patients with tuberous sclerosis complex (TSC), an inherited an autosomal dominant neoplastic syndrome due, in part, to mutations in the TSC1 or TSC2 gene. (medscape.com)
  • Tuberous sclerosis complex (TSC) and Sturge-Weber syndrome (SWS) are prototypical neurocutaneous disorders in which genetic mutations in pathways regulating cell growth cause developmental dysfunction of the brain, skin, and other organs. (uspharmacist.com)
  • He remained at UCSF, where he completed a residency in Internal Medicine, and fellowships in Pulmonary, Critical Care Medicine, and Lung/Heart-Lung Transplantation. (ucsf.edu)
  • He is board certified in Pulmonary Medicine and Critical Care Medicine. (ucsf.edu)
  • See the complete Pulmonary Pathology study guide by clicking on the link below. (iheartpathology.net)
  • The TSC mutations that occur in LAM result in abnormal signaling through the mammalian target of rapamycin (mTOR) pathway. (medscape.com)
  • Initial film is abnormal in more than 95% of patients with LAM. (medscape.com)