Hi my name is diana torres and i’m going to talk about systemic wa amyloid which is caused by inflammatory cardiocellular animal first we have the definition definition is a complication of a chronic inflammatory disease that is caused by the deposition of insoluble aggregates of cleave and terminal fragment of serum amyloid a protein in tissues and organs
Throughout the body amyloidosis is the term used to describe a group of diseases in which deposits of an abnormal protein called amyloid form in tissues and organs throughout the body proteins are important molecules involved in almost every structure and function within our cells they are made up of chains of smaller units called amino acids which fold themselves
Into three-dimensional structures folding allows the protein to work properly and interact with other molecules in amyloidosis proteins are made in the normal way but when they are released from the cell they open up again these abnormally folded proteins collect together in long chains or fibrils the deposits they form are called amyloid applications the
Applications amazing systemic ameliorations usually use chemotherapy as the first line some people can be benefited from orion or stem cell transplant if it’s diagnosed by laboratory tests and aristological studies amyloidosis is a rare disease very often it is an underdiagnosed disease this is due to the fact that multiple organs can be affected and that the
Symptoms are sometimes very unspecific some of the patients with amyloidosis and only nerve involvement present this neuropathy and many many many years they have neuropathy without having an adequate diagnosis so that’s why for me it’s very important first of all to raise awareness that this disease exists because once the disease is treated very early we have
Excellent outcome and prognosis of these patients amyloidosis can affect multiple organs the heart the kidneys the gastrointestinal tract the nerves and that’s why we think it’s very important that multiple specialists treat amyloidosis patients that means in case the patient has a heart involvement that cardiology will see that patients the first time he or
She comes to us the patient can expect a comprehensive assessment so that means that we evaluate the patient carefully for symptoms to find out which organ is affected and the patient can also expect that we will work closely with appropriate specialists from cardiology from nephrology to provide a comprehensive care affecting or treating all his symptoms it
Is really important to find a tailored treatment for the patient for instance one of the standard treatment for younger patients is a high dose chemotherapy with autologous stem cell transplant in case the patient has heart involvement we really have to adjust the treatment and often we are not able to give that treatment so we for instance would treat those
Patients with a regular chemotherapy and not with a very aggressive form of chemotherapy with a hope to induce a remission that means a hematologic remission and later an organ response there is a huge progress in the treatment of already existing amyloid for instance we can use antibodies but also antisense that means pieces of dna against certain proteins
That can dissolve the amyloidosis unfortunately those treatments are only available in clinical trials but i’m very optimistic that in two to three years we have tools available to treat not only the source of the amyloid but also to resolve and treat the existing amyloid sometimes after we exhaust options of conventional treatment patients have a chance to get
Access to novel drugs and clinical trials so this is one of the advantages new york presbyterian offers we have multiple trials from different disciplines for instance i offer trials focusing on destroying the plasma cells producing the amyloidos the amyloid a cardiologist has trials focusing on treatment of heart amyloidosis and our neurologist offers clinical
Trials to treat patients with neuropathy and amyloidosis i think this together and our common discussion offers the patients a variety of treatment options and especially access to novel drugs and i think this complicated and really rare disease can only be treated in a team of specialists experienced with this disease pros and cons first we have the plus out
Of the 75 percent of the population that suffers from amyloidosis is only five percent shared manifestation of systemic amino acids most manifestation of growing patients older than 40 years old currently there are treatments available as well as cure using organ of stem cell transplant if it is diagnostic early the patient have a better prognostic and cause
It is difficult to obtain the official data of these diseases they feel that oxygen has been obtained of of doses even many cases are not reported and there are only hereditary associations the waiting period of the transplant came very greatly of the case by case basis issues are placed in nepal in apollo we don’t have investigation double system uwa amylose
Amyloidosis is caused by inflammatory part of cellular adenoma however there are cases of called danious amyloidosis especially in coastal areas in nepal conclusion in conclusion rare cases of inflammatory hepatocellular adenoma can induce massive hwa production by the tumor and non-thermos liver the doctor speculate that this subset of wa amyloidosis may be
Treated by surgical reception of the liver too my keynote plenary lecture will be on the advances in the understanding of the molecular basis of systemic amyloidosis and particularly the amino acids caused by the monoclonal light chain that is very pertinent to a mythologist of course and the progress that we had in diagnosis and in treatment which all come
From our advances in research in this disease yes one key points will be the importance of early diagnosis because that is the basis for effective treatment as in most diseases of course and that the fact that now we have very effective treatments that dermatologists should be aware of and this treatment can really give a hope to cure for a good proportion of
This patient now
Transcribed from video
Systemic AA Amyloidosis Caused by Inflammatory Hepatocellular Adenoma By – imastaygirl-
