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Hyposplenism
Asplenia is the absence of normal spleen function and is associated with some serious infection risks. Hyposplenism is the condition of reduced ('hypo-'), but not absent, splenic functioning. ''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. * polysplenia Acquired Acquired asplenia occurs for several reasons: * Following splenectomy due to splenic rupture from trauma or because of tumor * After splenectomy with the ''goal'' ...
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Autosplenectomy
An autosplenectomy (from'' 'auto-' ''self,'' '-splen-' ''spleen,'' 'List of -ectomies, -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 fibrosis, 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 s ...
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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 individual ...
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Celiac Disease
Coeliac disease (British English) or celiac disease (American English) is a long-term autoimmune disorder, primarily affecting the small intestine. Patients develop intolerance to gluten, which is present in foods such as wheat, rye, spelt and barley. Classic symptoms include gastrointestinal problems such as chronic diarrhoea, abdominal distention, malabsorption, loss of appetite, and among children failure to grow normally. 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. Due to the frequency of these symptoms, coeliac disease is often considered a systemic disease, rather than a gastrointestinal condition. Coeliac disease was first described as a disease which initially presents during childhood; however, it may develop at any age. It is associated with other autoimmune diseases, such as Type 1 ...
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Sickle-cell Disease
Sickle cell disease (SCD), also simply called sickle cell, is a group of inherited haemoglobin-related blood disorders. The most common type is known as sickle cell anemia. Sickle cell anemia results in an abnormality in the oxygen-carrying protein haemoglobin found in red blood cells. This leads to the red blood cells adopting an abnormal sickle-like shape under certain circumstances; with this shape, they are unable to deform as they pass through capillaries, causing blockages. 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) in joints, anemia, swelling in the hands and feet, bacterial infections, dizziness and stroke. The probability of severe symptoms, including long-term pain, increases with age. Without treatment, people with SCD rarely reach adulthood but with good healthcare, median life expectancy is between 58 and 66 years. All of the majo ...
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Vaccination
Vaccination is the administration of a vaccine to help the immune system develop immunity from a disease. Vaccines contain a microorganism or virus in a weakened, live or killed state, or proteins or toxins from the organism. In stimulating the body's Adaptive immune system, adaptive immunity, they help prevent sickness from an infectious disease. When a sufficiently large percentage of a population has been vaccinated, herd immunity results. Herd immunity protects those who may be immunocompromised and cannot get a vaccine because even a weakened version would harm them. The effectiveness of vaccination has been widely studied and verified. Vaccination is the most effective method of preventing infectious diseases; widespread immunity due to vaccination is largely responsible for the Eradication of infectious diseases, worldwide eradication of smallpox and the elimination of diseases such as polio and tetanus from much of the world. According to the World Health Organization ...
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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 ones which cause the common cold or influenza. Drugs which inhibit growth of viruses are termed antiviral drugs or antivirals. Antibiotics are also not effective against fungi. Drugs which inhibit growth of fungi are called antifungal drugs. 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 ...
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Thrombocytosis
In hematology, thrombocythemia is a condition of high platelet (thrombocyte) count in the blood. Normal count is in the range of 150 to 450 platelets per liter of blood, but investigation is typically only considered if the upper limit exceeds 750/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 clini ...
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AAP Red Book
The '' AAP Red Book'', or ''Report of the Committee on Infectious Diseases of the American Academy of Pediatrics The American Academy of Pediatrics (AAP) is the largest professional association of pediatricians in the United States. It is headquartered in Itasca, Illinois, and maintains an office in Washington, D.C. The AAP has published hundreds of poli ...'', is a hardcover, softcover, and electronic reference to the "manifestations, etiology, epidemiology, diagnosis, and treatment of some 200 childhood infectious diseases". The ''Red Book'' first appeared as an eight-page booklet in 1938. The most-recent 33rd edition, published in 2024 and covering through 2027, has grown to 1261 pages. External links''AAP Red Book'' online Medical manuals {{med-book-stub ...
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Meningococcus
''Neisseria meningitidis'', often referred to as the 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 causes 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 Sexually transmitted infection, transmitted through oral sex and cause urethritis in men. It infects its hos ...
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Haemophilus Influenzae
''Haemophilus influenzae'' (formerly called Pfeiffer's bacillus or ''Bacillus influenzae'') is a Gram-negative, Motility, non-motile, Coccobacillus, coccobacillary, facultative anaerobic organism, facultatively anaerobic, Capnophile, capnophilic pathogenic bacterium of the family Pasteurellaceae. The bacteria are Mesophile, mesophilic and grow best at temperatures between 35 and 37 °C. ''H. influenzae'' was first described in 1893 by Richard Friedrich Johannes Pfeiffer, Richard Pfeiffer during an influenza pandemic when he incorrectly identified it as the causative microbe, which is why the bacteria was given the name "influenzae". ''H. influenzae'' is responsible for a wide range of localized and invasive infections, typically in infants and children, including pneumonia, meningitis, or bloodstream infections. Treatment consists of antibiotics; however, ''H. influenzae'' is often resistant to the penicillin family, but amoxicillin/clavulanic acid can be used in mild cases ...
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Streptococcus Pneumoniae
''Streptococcus pneumoniae'', or pneumococcus, is a Gram-positive, spherical bacteria, hemolysis (microbiology), alpha-hemolytic member of the genus ''Streptococcus''. ''S. pneumoniae'' cells are usually found in pairs (diplococci) and do not form Bacterial morphological plasticity, spores and are non motile. As a significant human pathogenic bacterium ''S. pneumoniae'' was recognized as a major cause of pneumonia in the late 19th century, and is the subject of many humoral immunity studies. ''Streptococcus pneumoniae'' resides asymptomatically in healthy carriers typically colonizing the respiratory tract, sinuses, and nasopharynx, nasal cavity. However, in susceptible individuals with immunocompromised, weaker immune systems, such as the elderly and young children, the bacterium may become pathogenic and spread to other locations to cause disease. It spreads by direct person-to-person contact via respiratory droplets and by auto inoculation in persons carrying the bacteria in ...
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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. Signs and symptoms OPSI may initially present with mild viral symptoms such as fever or coughing, however later in infection ...
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