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Rumen Acidosis
Lactic acidosis refers to the process leading to the production of lactate by anaerobic metabolism. It increases hydrogen ion concentration tending to the state of acidemia or low pH. The result can be detected with high levels of lactate and low levels of bicarbonate. This is usually considered the result of illness but also results from strenuous exercise. The effect on pH is moderated by the presence of respiratory compensation. Lactic acidosis is usually the result of tissue hypoxia which is not the same as arterial hypoxia. Adequate circulation of blood and perfusion of metabolizing tissue to meet demand is necessary to prevent tissue hypoxia. Lactic acidosis can also be the result of illnesses, medications, poisonings or inborn errors of metabolism that interfere directly with oxygen utilization by cells. The symptoms are generally attributable to the underlying cause, but may include nausea, vomiting, shortness of breath, and generalised weakness. The diagnosis is made on ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Lactic Acid
Lactic acid is an organic acid. It has the molecular formula C3H6O3. It is white in the solid state and it is miscible with water. When in the dissolved state, it forms a colorless solution. Production includes both artificial synthesis as well as natural sources. Lactic acid is an alpha-hydroxy acid (AHA) due to the presence of a hydroxyl group adjacent to the carboxyl group. It is used as a synthetic intermediate in many organic synthesis industries and in various biochemical industries. The conjugate base of lactic acid is called lactate (or the lactate anion). The name of the derived acyl group is lactoyl. In solution, it can ionize by a loss of a proton to produce the lactate ion . Compared to acetic acid, its p''K'' is 1 unit less, meaning lactic acid is ten times more acidic than acetic acid. This higher acidity is the consequence of the intramolecular hydrogen bonding between the α-hydroxyl and the carboxylate group. Lactic acid is chiral, consisting of two en ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Systemic Inflammatory Response Syndrome
In immunology, systemic inflammatory response syndrome (SIRS) is an inflammation, inflammatory state affecting the whole body. It is the body's Immune response, response to an infectious or noninfectious Insult (medical), insult. Although the definition of SIRS refers to it as an "inflammatory" response, it actually has pro- and anti-inflammatory components. Presentation Complications SIRS is frequently complicated by failure of one or more organs or organ systems. The complications of SIRS include * Acute kidney injury * Shock (circulatory), Shock * Multiple organ dysfunction syndrome Causes The causes of SIRS are broadly classified as infectious or noninfectious. Causes of SIRS include: * bacterial infections * severe malaria * Physical trauma, trauma * burn (injury), burns * pancreatitis * ischemia * hemorrhage Other causes include: * Complications of surgery * Adrenal insufficiency * Pulmonary embolism * Complicated aortic aneurysm * Cardiac tamponade * Anaphylaxis * Dru ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Pyruvate Carboxylase Deficiency
Pyruvate carboxylase deficiency is an inherited disorder that causes lactic acid to accumulate in the blood. High levels of these substances can damage the body's organs and tissues, particularly in the nervous system. Pyruvate carboxylase deficiency is a rare condition, with an estimated incidence of 1 in 250,000 births worldwide. Type A of the disease appears to be much more common in some Algonkian Indian tribes in eastern Canada, while the type B disease is more present in European populations. Signs and symptoms Pyruvate carboxylase deficiency causes lactic acidosis and hyperammonaemia. Lactic acidosis may then lead to liver failure, hepatomegaly, reduced ketone body synthesis, and demyelination of neurons. Genetics Pyruvate carboxylase deficiency is caused by mutations in the ''PC'' gene. The ''PC'' gene provides instructions for making an enzyme called pyruvate carboxylase. This condition is inherited in an autosomal recessive pattern, which means two copies of t ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Pyruvate Dehydrogenase Deficiency
Pyruvic acid (CH3COCOOH) is the simplest of the alpha-keto acids, with a carboxylic acid and a ketone functional group. Pyruvate, the conjugate base, CH3COCOO−, is an intermediate in several metabolic pathways throughout the cell. Pyruvic acid can be made from glucose through glycolysis, converted back to carbohydrates (such as glucose) via gluconeogenesis, or converted to fatty acids through a reaction with acetyl-CoA. It can also be used to construct the amino acid alanine and can be converted into ethanol or lactic acid via fermentation. Pyruvic acid supplies energy to cells through the citric acid cycle (also known as the Krebs cycle) when oxygen is present (aerobic respiration), and alternatively ferments to produce lactate when oxygen is lacking. Chemistry In 1834, Théophile-Jules Pelouze distilled tartaric acid and isolated glutaric acid and another unknown organic acid. Jöns Jacob Berzelius characterized this other acid the following year and named pyruvic acid ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Mitochondrial Encephalomyopathy, Lactic Acidosis, And Stroke-like Episodes
MELAS (Mitochondrial Encephalopathy, Lactic Acidosis, and Stroke-like episodes) is one of the family of mitochondrial diseases, which also include MIDD (maternally inherited diabetes and deafness), MERRF syndrome, and Leber's hereditary optic neuropathy. It was first characterized under this name in 1984. A feature of these diseases is that they are caused by defects in the mitochondrial genome which is inherited purely from the female parent. The most common MELAS mutation is one in mitochondrial DNA (mtDNA) referred to as m.3243A>G. Signs and symptoms MELAS is a condition that affects many of the body's systems, particularly the brain and nervous system (encephalo-) and muscles (myopathy). As such, it is commonly referred to as a mitochondrial encephalomyopathy, due to the co-occurrence of these pathologies. In most cases, the signs and symptoms of this disorder appear in childhood following a period of normal development. Children with MELAS often have normal early psy ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
GRACILE Syndrome
GRACILE syndrome is a very rare lethal autosomal recessive genetic disorder, one of the Finnish heritage diseases. GRACILE syndrome has also been found in the UK and Sweden, but not nearly as much as in Finland. It is caused by a mutation in the '' BCS1L'' gene and it occurs in approximately 1 out of 50,000 live births in Finnish people. To date, there have only been 32 cases of GRACILE syndrome reported. GRACILE is an acronym for growth retardation, aminoaciduria (amino acids in the urine), cholestasis, iron overload, lactic acidosis and early death. Prior to birth, the growth of the fetus is abnormally slow. This slow growth leads to a smaller than average newborn that has difficulty growing at a normal rate. Signs and symptoms People with GRACILE syndrome can have a wide range of symptoms, but that does not mean every person affected will have the same symptoms as one another. It has been determined that 80% - 99% of people with GRACILE syndrome have at least one of these: ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Glucose-6-phosphatase Deficiency
The enzyme glucose 6-phosphatase (EC 3.1.3.9, G6Pase; systematic name D-glucose-6-phosphate phosphohydrolase) catalyzes the hydrolysis of glucose 6-phosphate, resulting in the creation of a phosphate group and free glucose: : D-glucose 6-phosphate + H2O = D-glucose + phosphate During fasting, adequate levels of blood glucose are assured by glucose liberated from liver glycogen stores by glycogenolysis, as well as BY glucose generated by gluconeogenesis in both the liver, and, to a lesser extent, the kidneys. D-glucose 6-phosphate is the product of both these pathways and must be converted to glucose before it can be exported from the cell into blood by membrane-bound glucose transporters. Glucose 6-phosphatase is therefore principally expressed in the liver and kidney - while skeletal muscle collectively contain the most substantial glycogen reserve in the body, glucose cannot be mobilised from it as muscle cells do not produce glucose 6-phosphatase. Insulin inhibits hepat ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Fructose Bisphosphatase Deficiency
In fructose bisphosphatase deficiency, there is not enough fructose bisphosphatase for gluconeogenesis to occur correctly. Glycolysis (the breakdown of glucose) will still work, as it does not use this enzyme. History The first known description of a patient with this condition was published in 1970 in ''The Lancet'' journal. Early research into the disorder was conducted by a team led by Anthony S. Pagliara and Barbara Illingworth Brown at Washington University Medical Center, based on the case of an infant girl from Oak Ridge, Missouri. Presentation Without effective gluconeogenesis (GNG), hypoglycaemia will set in after about 12 hours of fasting. This is the time when liver glycogen stores have been exhausted, and the body has to rely on GNG. When given a dose of glucagon (which would normally increase blood glucose) nothing will happen, as stores are depleted and GNG doesn't work. (In fact, the patient would already have high glucagon levels.) There is no problem with th ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Diabetes Mellitus And Deafness
Diabetes and deafness (DAD) or maternally inherited diabetes and deafness (MIDD) or mitochondrial diabetes is a subtype of diabetes which is caused from a mutation in mitochondrial DNA, which consists of a circular genome. It is associated with the genes MT-TL1, MT-TE, and MT-TK. The point mutation at position 3243A>G, in gene MT-TL1 encoding tRNA leucine 1, is most common. Because mitochondrial DNA is contributed to the embryo by the oocyte and not by spermatozoa, this disease is inherited from maternal family members only. As indicated by the name, MIDD is characterized by diabetes and sensorineural hearing loss. Some individuals also experience more systemic symptoms including eye, muscle, brain, kidney, heart, and gastrointestinal abnormalities, similar to other mitochondrial diseases. Signs and symptoms As suggested by the name, patients with MIDD are subject to sensorineural hearing loss. This begins with a reduction in the perception of frequencies above approximately ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Biotin
Biotin (also known as vitamin B7 or vitamin H) is one of the B vitamins. It is involved in a wide range of metabolic processes, both in humans and in other organisms, primarily related to the utilization of fats, carbohydrates, and amino acids. The name ''biotin'', borrowed from the German , derives from the Ancient Greek word (; 'life') and the suffix "-in" (a suffix used in chemistry usually to indicate 'forming'). Biotin appears as a white, needle-like crystalline solid. Chemical description Biotin is classified as a heterocyclic compound, with a sulfur-containing tetrahydrothiophene ring fused to a ureido group. A C5-carboxylic acid side chain is appended to the former ring. The ureido ring, containing the −N−CO−N− group, serves as the carbon dioxide carrier in carboxylation reactions. Biotin is a coenzyme for five carboxylase enzymes, which are involved in the catabolism of amino acids and fatty acids, synthesis of fatty acids, and gluconeogenesis. Biotinylat ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Multiple Carboxylase Deficiency
Multiple carboxylase deficiency is a form of metabolic disorder involving failures of carboxylation enzymes. The deficiency can be in biotinidase or holocarboxylase synthetase. These conditions respond to biotin. __TOC__ Types Forms include: * Holocarboxylase synthetase deficiency - neonatal; * Biotinidase deficiency - late onset; If left untreated, the symptoms can include feeding problems, decreased body tone, generalized red rash with skin exfoliation and baldness, failure to thrive, seizure, coma, developmental delay, foul smelling urine, lactic acidosis, and high levels of ketones and ammonia Ammonia is an inorganic chemical compound of nitrogen and hydrogen with the chemical formula, formula . A Binary compounds of hydrogen, stable binary hydride and the simplest pnictogen hydride, ammonia is a colourless gas with a distinctive pu ... in the blood. References External links * http://www.pmh.health.wa.gov.au/services/newborn/health_professionals/disorders/mc ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Biotinidase Deficiency
Biotinidase deficiency is an autosomal recessive metabolic disorder in which biotin is not released from proteins in the diet during digestion or from normal protein turnover in the cell. This situation results in biotin deficiency. Biotin is an important water-soluble nutrient that aids in the metabolism of fats, carbohydrates, and proteins. Biotin deficiency can result in behavioral disorders, lack of coordination, learning disabilities, and seizures. Biotin supplementation can alleviate and sometimes totally stop such symptoms. Signs and symptoms Signs and symptoms of a biotinidase deficiency (BTD) can appear several days after birth. These include seizures, hypotonia and muscle/limb weakness, ataxia, paresis, hearing loss, optic atrophy, skin rashes (including seborrheic dermatitis and psoriasis), and alopecia. If left untreated, the disorder can rapidly lead to coma and death. Neonates with BTD may not exhibit any signs, and symptoms typically appear after the f ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
