|
Asplenia
Asplenia refers to the absence of normal spleen function and is associated with some serious infection risks. Hyposplenism is used to describe reduced ('hypo-') splenic functioning, but not as severely affected as with asplenism. ''Functional'' asplenia occurs when splenic tissue is present but does not work well (e.g. sickle-cell disease, polysplenia) -such patients are managed as if asplenic-, while in ''anatomic'' asplenia, the spleen itself is absent. Causes Congenital * Congenital asplenia is rare. There are two distinct types of genetic disorders: heterotaxy syndromeOnline Mendelian Inheritance in Man. OMIM entry 208530: Right atrial isomerism; RAI. Johns Hopkins University/ref> and isolated congenital asplenia.Online Mendelian Inheritance in Man. Johns Hopkins UniversityOMIM entry 271400: Asplenia, isolated congenital; ICAS./ref> * polysplenia Acquired Acquired asplenia occurs for several reasons: * Following splenectomy due to splenic rupture from trauma or because ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Isolated Congenital Asplenia
Isolated congenital asplenia is a rare disease in humans that can cause life-threatening bacterial infections in children due to primary immunodeficiency. The infections can include pneumococal sepsis and meningitis. ICAS is a ribosomopathy, due to autosomal dominant mutation of the ''RPSA'' gene on chromosome 3p21. Unlike heterotaxy syndrome, the absent spleen (asplenia) is not associated with other structural developmental defects. In some cases the spleen is present, but very small and nonfunctional (hyposplenism). Immunodeficiency The spleen is an organ within the lymphatic system and its primary function is to filter blood. However, the spleen also plays a key role in immune responses as it detects pathogens within the blood and secretes phagocytes to fight potential infection. Without these immune functions, individuals with isolated congenital asplenia are extremely susceptible to infection. ''Streptococcus pneumoniae'' is a common bacteria that affects individuals ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Heterotaxy Syndrome
Situs ambiguus is a rare congenital defect in which the major visceral organs are distributed abnormally within the chest and abdomen. Clinically heterotaxy spectrum generally refers to any defect of Left-right asymmetry and arrangement of the visceral organs; however, classical heterotaxy requires multiple organs to be affected. This does not include the congenital defect situs inversus, which results when arrangement of all the organs in the abdomen and chest are mirrored, so the positions are opposite the normal placement. Situs inversus is the mirror image of situs solitus, which is normal asymmetric distribution of the abdominothoracic visceral organs. Situs ambiguus can also be subdivided into left-isomerism and right isomerism based on the defects observed in the spleen, lungs and atria of the heart. Individuals with situs inversus or situs solitus do not experience fatal dysfunction of their organ systems, as general anatomy and morphology of the abdominothoracic organ-ve ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Overwhelming Post Splenectomy Infection
An overwhelming post-splenectomy infection (OPSI) is a rare but rapidly fatal infection occurring in individuals following removal (or permanent dysfunction) of the spleen. The infections are typically characterized by either meningitis or sepsis, and are caused by encapsulated organisms including ''Streptococcus pneumoniae''. It is a medical emergency and requires immediate treatment. Death has been reported to occur within 12 hours. The spleen is necessary for protection against encapsulated bacteria (see Mechanism) and as such when removed by splenectomy it can lead to rapid unchallenged infection by encapsulated bacteria. The rapid progression from mild viral symptoms to sepsis is one of the things that makes OPSI particularly dangerous. Another source of infection are species of ''Babesia'', which are tick-borne parasites that cause babesiosis. Infection with ''Capnocytophaga canimorsus'' after dog bites can also cause the condition. Signs and symptoms OPSI may initiall ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Splenectomy
A splenectomy is the surgical procedure that partially or completely removes the spleen. The spleen is an important organ in regard to immunological function due to its ability to efficiently destroy encapsulated bacteria. Therefore, removal of the spleen runs the risk of overwhelming post-splenectomy infection, a medical emergency and rapidly fatal disease caused by the inability of the body's immune system to properly fight infection following splenectomy or asplenia. Common indications for splenectomy include trauma, tumors, splenomegaly or for hematological disease such as sickle cell anemia or thalassemia. Indications The spleen is an organ located in the abdomen next to the stomach. It is composed of red pulp which filters the blood, removing foreign material, damaged and worn out red blood cells. It also functions as a storage site for iron, red blood cells and platelets. The rest (~25%) of the spleen is known as the white pulp and functions like a large lymph node ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Autosplenectomy
An autosplenectomy (from'' 'auto-' ''self,'' '-splen-' ''spleen,'' ' -ectomy' ''removal) is a negative outcome of disease and occurs when a disease damages the spleen to such an extent that it becomes shrunken and non-functional. The spleen is an important immunological organ that acts as a filter for red blood cells, triggers phagocytosis of invaders, and mounts an immunological response when necessary. Lack of a spleen, called asplenia, can occur by autosplenectomy or the surgical counterpart, splenectomy. Asplenia can increase susceptibility to infection. Autosplenectomy can occur in cases of sickle-cell disease where the misshapen cells block blood flow to the spleen, causing scarring and eventual atrophy of the organ. Autosplenectomy is a rare condition that is linked to certain diseases but is not a common occurrence. It is also seen in systemic lupus erythematosus (SLE). Consequences Absence of effective splenic function or absence of the whole spleen (asplenia) is associate ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Celiac Disease
Coeliac disease ( British English) or celiac disease (American English) is a long-term autoimmune disorder, primarily affecting the small intestine, where individuals develop intolerance to gluten, present in foods such as wheat, rye and barley. Classic symptoms include gastrointestinal problems such as chronic diarrhoea, abdominal distention, malabsorption, loss of appetite, and among children failure to grow normally. This often begins between six months and two years of age. Non-classic symptoms are more common, especially in people older than two years. There may be mild or absent gastrointestinal symptoms, a wide number of symptoms involving any part of the body, or no obvious symptoms. Coeliac disease was first described in childhood; however, it may develop at any age. It is associated with other autoimmune diseases, such as Type 1 diabetes mellitus and Hashimoto's thyroiditis, among others. Coeliac disease is caused by a reaction to gluten, a group of various ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Sepsis
Sepsis, formerly known as septicemia (septicaemia in British English) or blood poisoning, is a life-threatening condition that arises when the body's response to infection causes injury to its own tissues and organs. This initial stage is followed by suppression of the immune system. Common signs and symptoms include fever, increased heart rate, increased breathing rate, and confusion. There may also be symptoms related to a specific infection, such as a cough with pneumonia, or painful urination with a kidney infection. The very young, old, and people with a weakened immune system may have no symptoms of a specific infection, and the body temperature may be low or normal instead of having a fever. Severe sepsis causes poor organ function or blood flow. The presence of low blood pressure, high blood lactate, or low urine output may suggest poor blood flow. Septic shock is low blood pressure due to sepsis that does not improve after fluid replacement. Sepsis is caus ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Sickle-cell Disease
Sickle cell disease (SCD) is a group of blood disorders typically inherited from a person's parents. The most common type is known as sickle cell anaemia. It results in an abnormality in the oxygen-carrying protein haemoglobin found in red blood cells. This leads to a rigid, sickle-like shape under certain circumstances. Problems in sickle cell disease typically begin around 5 to 6 months of age. A number of health problems may develop, such as attacks of pain (known as a sickle cell crisis), anemia, swelling in the hands and feet, bacterial infections and stroke. Long-term pain may develop as people get older. The average life expectancy in the developed world is 40 to 60 years. Sickle cell disease occurs when a person inherits two abnormal copies of the β-globin gene (''HBB'') that makes haemoglobin, one from each parent. This gene occurs in chromosome 11. Several subtypes exist, depending on the exact mutation in each haemoglobin gene. An attack can be set off by ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Polysplenia
Polysplenia is a congenital disease manifested by multiple small accessory spleens, rather than a single, full-sized, normal spleen. Polysplenia sometimes occurs alone, but it is often accompanied by other developmental abnormalities. Conditions associated with polysplenia include gastrointestinal abnormalities, such as intestinal malrotation or biliary atresia, as well as cardiac abnormalities, such as dextrocardia. Associated conditions There are frequent associated congenital anomalies all related to deviations in the development of anatomical asymmetries in early embryonic stages. These conditions considered together are called "polysplenia syndrome". Associated conditions include heterotaxy syndrome, intestinal malrotation, situs inversus, biliary atresia, and several cardiac malformations. Associated cardiac conditions include dextrocardia Dextrocardia (from Latin ''dextro'', meaning "right hand side," and Greek ''kardia'', meaning "heart") is a rare congenital condi ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Thrombocytosis
Thrombocythemia is a condition of high platelet (thrombocyte) count in the blood. Normal count is in the range of 150x109 to 450x109 platelets per liter of blood, but investigation is typically only considered if the upper limit exceeds 750x109/L. When the cause is unknown, the term thrombocythemia is used, as either primary thrombocythemia or essential thrombocythemia. The condition arises from a fault in the bone marrow cells leading to over-production of platelets but the cause of the fault is unknown, and this type is not common. When the cause is known such as another disorder or disease, the term thrombocytosis is preferred, as either secondary or reactive thrombocytosis. Reactive thrombocytosis is the most common type and though it can often have no symptoms it can sometimes predispose to thrombosis. In contrast, thrombocytopenia refers to abnormally low blood platelet numbers in the blood. Signs and symptoms High platelet counts do not necessarily signal any clinical pr ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Antibiotic
An antibiotic is a type of antimicrobial substance active against bacteria. It is the most important type of antibacterial agent for fighting pathogenic bacteria, bacterial infections, and antibiotic medications are widely used in the therapy, treatment and antibiotic prophylaxis, prevention of such infections. They may either bactericide, kill or bacteriostatic agent, inhibit the growth of bacteria. A limited number of antibiotics also possess antiprotozoal activity. Antibiotics are not effective against viruses such as the common cold or influenza; drugs which inhibit viruses are termed antiviral drugs or antivirals rather than antibiotics. Sometimes, the term ''antibiotic''—literally "opposing life", from the Greek language, Greek roots ἀντι ''anti'', "against" and βίος ''bios'', "life"—is broadly used to refer to any substance used against microbes, but in the usual medical usage, antibiotics (such as penicillin) are those produced naturally (by one microorgani ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Meningococcus
''Neisseria meningitidis'', often referred to as meningococcus, is a Gram-negative bacterium that can cause meningitis and other forms of meningococcal disease such as meningococcemia, a life-threatening sepsis. The bacterium is referred to as a coccus because it is round, and more specifically a diplococcus because of its tendency to form pairs. About 10% of adults are carriers of the bacteria in their nasopharynx. As an exclusively human pathogen, it is the main cause of bacterial meningitis in children and young adults, causing developmental impairment and death in about 10% of cases. It causes the only form of bacterial meningitis known to occur epidemically, mainly in Africa and Asia. It occurs worldwide in both epidemic and endemic form. ''N. meningitidis'' is spread through saliva and respiratory secretions during coughing, sneezing, kissing, chewing on toys and through sharing a source of fresh water. It has also been reported to be transmitted through oral sex and cause ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
.jpg "Click for more on -> Thrombocytosis")
