Amyloidosis is a disease characterized by the extracellular accumulation of fibrils in different organs in the body. The precursor proteins may vary but all contain an antiparallel β-pleated sheet configuration thus resulting to their amyloidogenic properties and unique reaction (the production of a green birefringence under polarized light) when stained with Congo red. All types of amyloid are comprised of a serum amyloid P component, a 25-kD glycoprotein, and a member of the C-reactive-protein-containing pentraxin family. Amyloidosis may be classified into diverse groups of local and systemic diseases with primary amyloidosis as the most widely occurring.
In primary amyloidosis, the fibrils that build up in various organs are made up of the N-terminal acid residues of the variable section of an immunoglobin light chain; hence the other name primary amyloidosis is known for: immunoglobin light chain amyloidosis. Here, the clonal plasma cells predominantly express λ light chains over κ light chains and an increased incidence of monoclonal λ type VI can be observed. There are various symptoms that could hint people of primary amyloidosis but in order to be confirmed, a biopsy documentation of the amyloid is required.
The typical number of cases of primary amyloidosis reaches 8 per million yearly. It normally occurs in patients over the age of 50; only about 1% are below the age of 40. And it occurs in men in about twice as much as it does in women. Patients with primary amyloidosis are typically proven to have only a median survival of 2 years or less. One reason to this may be the fact that multisystem organ involvement is quite usual in this disease, with the most commonly affected ones being the kidney, the heart, and the pe...
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... is also of note that high-dose chemotherapy with peripheral blood stem cell transportation has led to the treatment of nephrotic syndrome and biopsy-improvement of amyloid organ involvement in some isolated cases, and that it has shown very promising results. However, strict care measures must still be observed regardless of whether chemotherapy or marrow transplant is used.
Works Cited
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Alzheimer’s can be diagnosed before age 65, although rare, and is caused by a mutation in 3 known genes. About 5 percent of those who are under 65 and possess the ailment have AD in their family history. Given that the symptoms of AD are caused by plaque in the brain, causing loss of nerve cells that help the body communicate with the brain, mutations to these genes; amyloid precursor protein, presenilin 1, and presenilin 2 cause a excessive production of certain proteins (primarily a B-42 form of amyloid protein), and therefore spark an excessive growth of plaque cells which are toxic to the neurons of the brain. For those cases of Alzheimer’s that occur after age 65, a genetic mutation has yet to be proven, although some may be linked, to the fact that a difference may cause an increased chance of developing the ailment. Whatever the case may be for patients over 65 years old, the disease and its symptoms are caused by neurofibrillary tangles of almyloid plaques. It is impossible for someone to test positive for Alzheimer’s Disease, because the only way to determine an affirmative case i...
Hereditary Spherocytosis is the most common form of inherited hemolytic anemia in the US, northern Europe, and notably in Japan, affecting one person in 2000. HS can be found in most racial groups, but it is less common in African American and south Asia...
Data found by Rabinovici et. al. in 2010 supported that early amyloid beta accumulation increased vulnerability to amyloid beta pathology and plays critical roles in susceptibility to Alzheimer’s in young patients. The quantity of beta amyloid in her brain shows that the build up has been happening for the last few years. The doctors also inform her that there is a 50/50 chance of passing the gene on to the carriers' children and the odds of developing the disease, as a gene carrier is 100%. Alice and John inform their three grown children, Anna, Tom and Lydia of these findings and inform them that there are options if they want to get tested for it. Anna her oldest daughter who is pregnant with twins tested positive, Tom is negative and Lydia chose not to get tested. Alice offers to stop everything and go visit Anna to see if she’s okay but Anna wishes to be left alone. Alice is hurt that her daughter does not want any of her
Bevel, Tom, and Gardner, Ross M. Blood Pattern Analysis, Second Edition. CRC Press, Boca Raton, FL (2002).
In 1906, Dr. Alois Alzheimer discovered a “peculiar disease”. Dr. Alzheimer was an expert in linking symptoms to microscopic brain changes. Dr. Alzheimer noticed changes in the brain tissue of Auguste D., a patient who had died of an unusual mental disease. Her symptoms ranged from memory loss to unpredictable behavior. Afte...
The study of structure, function and pathological disorders in blood is called “Haematology”. Blood consists of 55% plasma and 45% blood cells. Plasma is a mixture of 7% protein, 91% water and 2% other solutes. The types of proteins present in blood are albumin, globulin, and fibrinogen. Other remaining solutes consist of ions, nutrients, gases and respiratory substances and waste products. On th...
Alzheimer’s disease comes from the last name of a neuro-psychiatrist from Germany, Alois Alzheimer. The disease was first diagnosed when a woman in her early fifties began experience memory problems. “Alzheimer recounted the now famous case of ‘Auguste D.’ a 51-year-old housewife who had been failing mentally for several years. As a result she had been admitted to his care in the Asylum for the Insane and Epileptic…” (Maurer and Maurer 1). After her death, he continued to examine her brain to find causes and explanations for her behavior. He discovered “…classic neuro-pathological signs of plaques and tangles” (Maurer and Maurer 1). “Plaques are chains of amino acids that are pieces of the amyloid precursor protein…tangles are aggregates of the protein tau” (Secko 1). As plaques develop they produce tangles and “these two abnormalities ultimately lead to loss of cognitive function” (Secko 1) Alois Alzheimer’s research has allowed many specialist to conclude that the apolipoproetein E gene may contribute to the disease.
The first case of dementia was discovered in 1906 by a German physiatrist Alois Alzheimer. It was first observed in a female patient and she was forty-one years old her name was Auguste D. Dr. Alzheimer observed a decline in the patient’s cognitive abilities. She lost her memory, she exhibited behavioral issues, and she suffered from hallucinations, lost the ability to comprehend language, disorientation and lost her speech. After Augusta’s passing Dr. Alzheimer preformed an autopsy that showed the classic triangles and knots we associate today with Alzheimer. Those triangles and knots are a proteins and plaque. The brain is self looked smaller and had distinct characteristics. Still with modern medicine the only way to diagnose a person with Alzheimer is after their death with an autopsy. (THE ALZHEIMER'S PROJECT, 2014).
Alzheimer’s disease got its name from the German doctor, Dr. Alois Alzheimer. In 1906, he noticed that there were abnormal clumps and bundles of fibers i...
Scientists know that Alzheimer disease is characterized by a gradual spread of sticky plaques and clumps of tangled fibers that disrupt the organization of nerve cells in the brain. However , a definite cause, prevention, or cause has not been found.
Alzheimer’s Disease is named after a German doctor, who specializes in the brain and nervous system, named Alois Alzheimer. This Disease forms in the brain. Alzheimer’s is the most common form of Dementia, a general term for memory loss and other intellectual abilities serious enough to enter. The Tau protein ensures the tubes in your brain stay straight allowing molecules to pass through freely. In Alzheimer’s Disease the protein collapses into strands or tangles, making the tubes disintegrate. There is visible differences of brain tissue in the from misfolded proteins called plaques and tangles. Beta-Amyloid clumps block signals and communication between cells in the brain. Researchers agree that Alzheimer’s Disease is m...
In 1906, Dr. Alois Alzheimer, noticed some changes in the brain tissue of a woman who had died of an unusual mental illness. Her symptoms were comprised of memory loss, language problems, and unpredictable behavior. After her death, Dr. Alzheimer examined her brain and found many abnormal clumps (amyloid plaques) and tangled bundles of fibers (neurofibrillary tangles).
Red blood cell indices:(mean corpuscular volume [MCV], mean corpuscular hemoglobin (MCH), and mean corpuscular hemoglobin concentration [MCHC]
my check off sheet with the hematology supervisor. One of the first things I learned was