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Showing posts with label Science. Show all posts
Showing posts with label Science. Show all posts

Long distance relationship? Physics it is!



It is evident that we humans prefer to go with someone who is nearly similar to us. This is also one of the basis of non-physical attraction. And if you spent more time with someone you'll not only get to know that person well but you might also be able to absorb some of that person's trait and the same goes with that person, this is what we all know as influence. Let me share you something related to physics.
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What is a year?

You gotta be kidding me..



You might be thinking that this post is hilariously stupid, of course a year is the revolution of the earth in the sun or exactly 365 and 1/4 days. Well somehow that's true, but somehow it's also incorrect in some sense. There are different types of years with different length for each years, the most common is the Tropical Year (which is the basis for most calendars) but there are more kinds of years not just two or three, but more than five excluding the other planet's year (Martian/Uranian Year).


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Rare Diseases : Dercum's Disease

Dercum's Disease



Dercum’s disease is an extremely rare disorder characterized by multiple, painful growths consisting of fatty tissue (lipomas). These growths mainly occur on the trunk, the upper arms and upper legs and are found just below the skin (subcutaneously). Pain associated with Dercum’s disease can often be severe. Pain may be caused by these growths pressing on nearby nerves. Dercum’s disease mainly occurs in adults and more women are affected than men. In some cases, affected individuals may also experience weight gain, depression, lethargy, and/or confusion. The exact cause of Dercum’s disease is unknown.



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Rare Diseases : Paraneoplastic Neurologic Syndromes (PNS)

 Paraneoplastic Neurologic Syndromes (PNS)

Paraneoplastic neurologic syndromes (PNS) are a group of conditions that affect the nervous system (brain, spinal cord, nerves and/or muscles) in patients with cancer. The term "paraneoplastic" means that the neurological syndrome is not caused by the tumor itself, but by the immunological reactions that the tumor produces. It is believed that the body’s normal immunological system interprets the tumor as an invasion. When this occurs, the immunological system mounts an immune response, utilizing antibodies and lymphocytes to fight the tumor. The end result is that the patient’s own immune system can cause collateral damage to the nervous system, which can sometimes be severe. In many patients, the immune response can cause nervous system damage that far exceeds the damage done to the tumor. The effects of PNS can remit entirely, although there can also be permanent effects.

Epidemiology

PNS are rare diseases occurring in less than about 0.01% of patients with cancer. Only the Lambert-Eaton myasthenic syndrome is relatively frequent, occurring in about 1% of patients with small-cell lung cancer  
Classes : 

* Endocrine - can be related to : 
                          > Cushing`s Syndrome
                          > Syndrome of Inappropriate Anti-Diuretic Hormone
                          > Hypercalcemia
                          > Hypoglycemia
                          > Carcinoid Hormone
                          > Polycythemia 
                          > Hyperaldosteronism 

* Neurological - can be related to :
                    > Lambert-Eaton myasthenic syndrome (LEMS)
                    > Paraneoplastic cerebellar degeneration
                    > Encephalomyelitis
                    > Limbic encephalitis
                    > Brainstem encephalitis
                    >Paraneoplastic Opsoclonus (involving eye movement)-Myoclonus
                    > Anti-NMDA receptor encephalitis
                    > Polymyositis


Diagnosis and management

PNS may affect any part of the central and peripheral nervous system, the neuromuscular junction and muscle. They can be isolated or occur in association. An important point to keep in mind is that none of these disorders are specifically paraneoplastic, as each disorder can also occur in patients without cancer. The incidence of malignancy in patients with potential PNS varies depending on the disorder and ranging from 5 to 60%. In almost 80% of patients, the PNS antedates the diagnosis of cancer by several months to several years. Most tumors are diagnosed within 4–6 months. It is accepted that the risk of cancer development decreases significantly two years after diagnosing PNS and becomes very low after four years. All these data explain why it is often difficult to recognize a given neurological disorder as paraneoplastic.

Treatment

The different subtypes of PNS are defined by the presence or the absence of paraneoplastic antibodies and the type of antibodies. Management and treatment should be tailored to each subtype. For example, patients with SCLC and PCA have different course of the neurological disorder, response to treatment, tumor prognosis and cause of death, according to the presence or the absence of Hu-Ab. Patients with Ma2-Ab generally develop limbic and brainstem encephalitis with testis tumors, but patients with Ma2-Ab and additional antibodies directed against other Ma proteins develop additional cerebellar symptoms and have tumors other than testis neoplasms. The mean survival time for patients with SCLC and CV2-Ab is 2.5 times longer than for patients with the same cancer, similar neurological symptoms and Hu-Ab. All these data suggest that the type of the immunological stimulation against the tumor can determine a specific course of the neurological disorder and give information of whether the immunological response against the tumor is more or less effective.
However, PNS rarely improves with immunomodulatory treatment as in the majority of cases the neurological symptoms are due to neuronal damage. The early and non-reversible destruction of neural structures by the inflammatory process accounts for both the severity of most PNS and for the usual ineffectiveness of immunomodulatory treatments. The best chance of at least stabilizing the syndrome is to induce a complete response of the tumor. Although immunotherapy is rarely effective, the use of intravenous immunoglobulins, steroids or plasmapheresis is indicated because a few patients have been reported to improve with these treatments. Patients with Tr-Ab and Ma2-Ab and testicular cancer are more likely to improve than those with other antibodies.

Sources : 

















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Rare Diseases : Progressive Multifocal Leukoencephalopathy (PML)

Progressive Multifocal Leukoencephalopathy (PML)

Progressive multifocal leukoencephalopathy (PML) is a neurological disorder characterized by destruction of the myelin, an oily substance that helps protect nerve cells in the brain and spinal cord, also known as central nervous system (CNS) white matter. It is caused by a virus called JC virus (JCV), named after the initials of the patient in whom it was first discovered. The virus is widespread, found in at least 85% of the general adult population. It remains inactive in healthy individuals and causes disease only when the immune system has been severely weakened, such as in people with HIV/AIDS, or hematological malignancies, and in organ transplant recipients who receive immuno- suppressant medications to avoid rejection of the transplanted organ. Altogether, PML occurs in approximately one in 200,000 people.
The term "progressive" in PML means that the disease continues to get worse and often leads to serious brain damage. The term "multifocal" means that JCV causes disease in multiple parts of the brain. However, it is possible for an individual with PML to have only one brain lesion instead of several lesions. The term "leukoencephalopathy" means that the disease affects mainly the white matter of the brain or myelin, although there are some rare cases in which the gray matter neurons is also involved.


Is there any treatment?

Currently, the best available therapy is reversal of the immune-deficient state, since there are no effective drugs that block virus infection without toxicity.  Reversal may be achieved by using plasma exchange to accelerate the removal of the therapeutic agents that put patients at risk for PML.  In the case of HIV-associated PML, immediately beginning anti-retroviral therapy will benefit most individuals.  Several new drugs that laboratory tests found effective against infection are being used in PML patients with special permission of the U.S. Food and Drug Administration.  Hexadecyloxypropyl-Cidofovir (CMX001) is currently being studied as a treatment option for JVC because of its ability to suppress JVC by inhibiting viral DNA replication.  

What is the prognosis?

In general, PML has a mortality rate of 30-50 percent in the first few months following diagnosis but depends on the severity of the underlying disease and treatment received.  Those who survive PML can be left with severe neurological disabilities.

What research is being done?

The National Institute of Neurological Disorders and Stroke (NINDS) and other institutes of the National Institutes of Health (NIH) conduct research related to PML in laboratories at the NIH, and support additional research through grants to majorresearch institutions across the country.  Much of this research focuses on finding better ways to prevent, treat, and ultimately cure disorders such as PML.


Sources : 
Annals of Neurology (Author[s]: Mateen, F. J.; Muralidharan, R.; Carone, M.; Van De Beek, D.; Harrison, D. M.; Aksamit, A. J.; Gould, M. S.; Clifford, D. B. et al)
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