EUROPEAN JOURNAL OF
PHARMACEUTICAL AND MEDICAL RESEARCH

( An ISO 9001:2015 Certified International Journal )

An International Peer Reviewed Journal for Pharmaceutical, Medical & Biological Sciences

An Official Publication of Society for Advance Healthcare Research (Reg. No. : 01/01/01/31674/16)

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 ISSN 2394-3211

Impact Factor: 6.222

 ICV - 79.57

Abstract

LYMPHANGIOLEIOMYOMATOSIS: A REVIEW

Jisna K. Philip*, Tasnim Nazeer, Dr. Subash Chandran M. P, Dr. Karthika Lal B., Dr. Prashobh G. R.

ABSTRACT

Lymphangioleiomyomatosis (LAM) is a cystic lung disease.[1] It is a multisystem disease of women. It is mainly reported in women of child bearing age, most commonly with dyspnea and pneumothorax. It is mainly characterized as proliferation of abnormal smooth muscle-like LAM cells, leading to the formation of lung cysts, fluid-filled cystic structures in the axial lymphatics (eg, lymphangioleiomyomas), and renal angiomyolipomas. The major cause of LAM mutations of the TSC1 or TSC2 genes, which encode for hamartin and tuberin, two proteins with a major role in control of the mammalian target of rapamycin (mTOR) signaling pathway. LAM may present with progressive dyspnea, recurrent pneumothorax, or chylothorax.[2] It is extremely difficult to treat and has poor prognosis. Pulmonary function tests indicates reduced flow rates (forced expiratory volume in the first second) and diffusion capacity. Exercise testing shows gas exchange abnormalities, ventilatory limitation, and hypoxemia.[3] Other test for finding out severity and progression of disease include lung histology scores, quantification of computed tomography, pulmonary function testing, 6-minute walk tests, cardiopulmonary exercise testing, and measurement of serum vascular endothelial growth factor D levels. Two mTOR inhibitors that are effective in stabilizing lung function and reducing the size of chylous effusions, lymphangioleiomyomas, and angiomyolipomas are Sirolimus and Everolimus. Combination of Sirolimus and hydroxychloroquine causes ihibition of autophagy and has been proposed as a possible treatment for LAM. Treatment with Simvastatin targets RhoA GTPases. It inhibits Rho GTPases and promotes apoptosis. This targets signaling pathways considered important in the pathogenesis of disease.

Keywords: lymphangioleiomyomatosis, dyspnoea, Sporadic LAM, TSC1 and TSC2 mutations, Sirolimus.


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Google Scholar Indian Science Publications InfoBase Index (In Process) SOCOLAR, China Research Bible, Fuchu, Tokyo. JAPAN International Society for Research activity (ISRA) Scientific Indexing Services (SIS) Polish Scholarly Bibliography Global Impact Factor (GIF) (Under Process) Universal Impact Factor International Scientific Indexing (ISI), UAE Index Copernicus CAS (A Division of American Chemical Society) USA (Under Process) Directory of Open Access Journal (DOAJ, Sweden, in process) UDLedge Science Citation Index CiteFactor Directory Of Research Journal Indexing (DRJI) Indian citation Index (ICI) Journal Index (JI, Under Process) Directory of abstract indexing for Journals (DAIJ) Open Access Journals (Under Process) Impact Factor Services For International Journals (IFSIJ) Cosmos Impact Factor Jour Informatics (Under Process) Eurasian Scientific Journal Index (ESJI) International Innovative Journal Impact Factor (IIJIF) Science Library Index, Dubai, United Arab Emirates Pubmed Database [NLM ID: 101669306] (Under Process) IP Indexing (IP Value 2.40) Web of Science Group (Under Process) Directory of Research Journals Indexing Scholar Article Journal Index (SAJI) International Scientific Indexing ( ISI )