By Y. Barrack. Crown College.
Individuals affected with childhood acid maltase deficiency generally die from respiratory failure between the ages of three and 24 viagra soft 50 mg discount. Individuals affected IAcrocallosal syndrome with adult acid maltase deficiency generally die from respiratory failure within 10 to 20 years of the onset of Definition symptoms viagra soft 100mg mastercard. Acrocallosal syndrome is a rare congenital disorder Human clinical trials involving enzyme replacement in which the individual has absence or only partial for- therapy quality 100mg viagra soft, in which a synthetic form of acid maltase is mation of the corpus callosum purchase 100mg viagra soft with visa. This is accompanied by administered to affected individuals, were begun in 1999 skull and facial malformations, and some degree of fin- at Duke University Medical Center in North Carolina and ger or toe malformations. The term acrocallosal refers to the procedure that produces a three-dimensional pic- involvement of the acra (fingers and toes) and the corpus ture of organs or structures inside the body, such as callosum, the thick band of fibers joining the hemispheres the brain. The major characteris- Consanguinity—A mating between two people tic of the syndrome is the incomplete formation (hypopla- who are related to one another by blood. Facial Corpus callosum—A thick bundle of nerve fibers appearance is typically similar among affected people. Individuals have a Hypertelorism—A wider-than-normal space degree of webbing or fusion (syndactyly), or duplication between the eyes. Occasionally, those affected may have a short upper lip, cleft palate, cysts that Hypotonia—Reduced or diminished muscle tone. Less frequently, affected chil- Syndactyly—Webbing or fusion between the fin- dren have congenital heart defects, internal organ (vis- gers or toes. Individuals usually display some form of poor muscle tone (hypotonia), and there It affects both males and females. There are some reports may be a delay or absence of motor activities, walking, of webbing of the fingers or toes (syndactyly) and relat- and talking. There is great variation of functioning and edness (consanguinity) of the parents of affected chil- symptoms with this disorder, ranging from normal devel- dren. However, affected children may also have opment to severe mental and motor retardation. At birth, those with acrocallosal syndrome present There are sporadic, or random, cases, and reports of mul- the characteristic pattern of facial and limb malforma- tiple cases within families. Limb appearance ranges from minor webbing families have suggested an autosomal recessive pattern of between the fingers or toes to near duplication of the inheritance. Forehead prominence, increased distance altered form of the gene and the affected child inherited between the eyes, and an enlarged head are the main fea- both copies. The To help determine which chromosome or gene loca- infant will usually display reduced muscle tone (hypoto- tion causes the syndrome, acrocallosal syndrome has nia). This may lead to a drooling condition or feeding dif- been compared with similar disorders. Hypotonia can also contribute to a delay in presents similar symptoms and has a known genetic growth and motor skills. To date, no specific genetic cause for acrocal- Progress and functioning during the first year of life losal syndrome is known, and the disorder can only be is dependent upon the severity of the symptoms. Some children develop normally and will walk and Demographics talk within normal age limits, while others may experi- Acrocallosal syndrome is extremely rare. Some GALE ENCYCLOPEDIA OF GENETIC DISORDERS 27 Acrocallosal Syndrome Polydactyly Severe mental delays Congenital heart defect Prominent forehead Muscle weakness Congenital heart defect Polydactyly (Gale Group) children may develop seizure disorders. The degree and Prognosis progression of mental retardation also varies by individ- At present, there are no preventative measures for ual. It has been found that the Diagnosis lifestyle of an individual with acrocallosal syndrome is The diagnosis of acrocallosal syndrome is based ini- dependent upon the degree of mental retardation and tially on the distinct pattern of facial and limb malforma- reduced muscle tone, rather than the extent of facial and tions. Hand and foot x rays can be taken Resources to confirm finger or toe abnormalities, and will determine PERIODICALS the extent of fusion, webbing, or duplication of the digits Bonatz, E. However, prenatal ultrasound Partial Agenesis of the Corpus Callosum: An Example of can detect duplication of the digits (polydactyly) and the Variable Clinical Spectrum of the Acrocallosal cerebral malformations. Surgery to separate or remove affected toes may assist in walking and the comfort of footwear. ORGANIZATIONS Anti-epileptic therapy should be considered if a seizure Agenesis of the Corpus Callosum (ACC) Network.
For simplic- eral presynaptic terminals innervate a single ganglion ity buy viagra soft 50 mg without prescription, the figure has been divided into a type A synapse buy viagra soft 50 mg on line, cell quality viagra soft 50mg, and several preganglionic axon terminals must which includes SIF cells cheap viagra soft 50 mg without prescription, and a type B synapse, which fire simultaneously for transmission to take place. The drugs the type of receptor mediating the response, and the pri- either interact with the nicotinic–cholinergic receptor mary transmitter or mediator that activates the receptor. Interaction of ACh with the postsynaptic ganglionic cell muscarinic receptor is responsible for slowly devel- GANGLIONIC STIMULANTS oping depolarization, the slow EPSP, which has a longer latency than the fast EPSP and a duration of 30 to 60 A variety of agents, including nicotine, lobeline, and di- seconds. The slow EPSP is due to inhibition of a voltage- methylphenyl piperazinium (DMPP), can stimulate gan- dependent K current called the M current, and inhibi- glionic nicotinic receptors. First, drugs such as nicotine that both M4 and M5) have been identified using functional stud- stimulate and block ganglionic receptors have proved ies and at least five subtypes (m1,m,m,m,2 3 4 and m5) valuable as an aid in identifying and localizing postgan- identified by molecular cloning techniques. In this case, acti- Mechanism of Ganglionic Stimulation vation of a muscarinic receptor on the SIF cells results in the release of a catecholamine; this in turn activates a Nicotine, lobeline, trimethylammonium, and DMPP receptor on the postganglionic cell, leading to the slow stimulate all autonomic ganglia by simple combination IPSP. The catecholamine most frequently released from with ganglionic nicotinic receptors on the postsynaptic SIF cells appears to be dopamine. This leads to membrane depolarization, an EPSP, lasting for 1 to 2 minutes, can be seen at some influx of sodium and calcium ions, and the generation of ganglionic synapses. These agents produce general stimulation now well established that there are a large number of of autonomic ganglia and a complex pattern of mixed peptides in the ganglia, including luteinizing hormone– sympathetic and parasympathetic responses. In addition to the cholinergic and adrenergic recep- Activation of nicotinic receptors on the plasma mem- tors on autonomic ganglion cells, there also appear to be brane of the cells of the adrenal medulla leads to the exo- receptors for a variety of excitatory and inhibitory sub- cytotic release of epinephrine and norepinephrine; stimu- stances, including angiotensin, bradykinin, histamine, lation of nicotinic receptors at the neuromuscular 5-hydroxytryptaimine (serotonin), and substance P. For simplicity, it has been divided into a type A synapse containing interneurons or small intensely fluorescent (SIF) cells and a type B synapse lacking interneurons. In the type A synapse, ACh is released from the preganglionic neuron and activates nicotinic and muscarinic receptors on the SIF cells (when present), leading to the release of a catecholamine, presumably dopamine. The insert depicts the temporal postganglionic action potential, consisting of a fast excitatory postsynaptic potential (EPSP) due to activation of nicotinic receptors by ACh, a slow inhibitory postsynaptic potential (IPSP) due to dopamine or another catecholamine activating the appropriate receptor, and a slow EPSP due to activation by ACh of an M1 muscarinic cholinergic receptor on the postganglionic nerve cell body. In this case, ACh activates both nicotinic receptors leading to the fast EPSP and muscarinic receptors leading to the slow IPSP and slow EPSP. Stimulation of nicotinic receptors in adren- ganglionic transmission produced by most ganglionic ergic nerve terminals leads to the release of norepineph- blocking agents, that is, a nondepolarizing competitive rine; and activation of nicotinic chemoreceptors in the antagonism, the blockade produced by nicotine consists aortic arch and carotid bodies causes nausea and vomit- of two phases. After a few seconds, however, this discharge stops and transmission is Mechanism of Ganglionic Blockade blocked. At this time, antidromic stimuli fail to induce an Large doses of nicotine produce a prolonged blockade action potential. Unlike the blockade of fail to respond to the administration of any ganglionic 144 II DRUGS AFFECTING THE AUTONOMIC NERVOUS SYSTEM stimulant, regardless of the type of receptor it activates. Central Nervous System The main reason for the loss of electrical or receptor- The actions of nicotine on the central nervous system mediated excitability during a period of maintained de- are the result of a composite of stimulatory and depres- polarization is that the voltage-sensitive sodium channel sant effects. These can include tremors, convulsions, res- is inactivated and no longer opens in response to a brief piratory stimulation or depression, and release of antid- depolarizing stimulus. Nausea and emesis all ganglionic stimulants that are not nicotinic, such as are frequently observed after the initial use of nicotine histamine, angiotensin, bradykinin, and serotonin, be- in the form of tobacco smoke. This is in contrast to the Phase 1 is followed by a postdepolarization phase effects of nicotine on the cardiovascular system, where (phase 2) during which only the actions of nicotinic re- tolerance develops much more slowly. The main factor responsible for phase 2 block Additional effects of nicotine include an increase in gas- appears to be desensitization of the receptor to ACh, tric acid secretion and an increase in the tone and motil- which causes transmission failure. These effects are pro- duced because of the predominance of cholinergic input Pharmacological Actions of Nicotine to these effector systems. Nicotine is present in varying amounts in all forms of Absorption, Distribution, and Excretion tobacco smoke. Following its absorption from the lungs, of Nicotine the blood nicotine levels are sufficient to cause stimula- tion but not blockade of nicotinic receptors. In addition Nicotine is well absorbed from the mucous membranes to stimulating receptors on autonomic ganglia, all other in the oral cavity, gastrointestinal tract, and respiratory nicotinic receptors mentioned earlier can be activated.
Maximum benefits clotting ability generic viagra soft 100 mg on line, buy viagra soft 100 mg low price, bone marrow damage 50 mg viagra soft fast delivery, insulin are reportedly attained after approximately three months shock buy viagra soft 100 mg with mastercard, thrombophlebitis with embolism, and even rare after a treatment series. However, some doctors feel that the latter groups able, but it is a fraction of the cost of an expensive medical of complications occurred before the safer method cur- procedure like cardiac bypass surgery. The A candidate for chelation therapy should initially have treatment is approved by the United States Food and Drug a thorough history and physical to define the type and ex- Administration (FDA) for lead poisoning and seriously high tent of clinical problems. However, for the treatment of atherosclerotic determine whether there are any conditions present that, EDTA chelation therapy is not endorsed by may prevent the use of chelation. Patients who have preex- the American Heart Association (AHA), the FDA, the Na- isting hypocalcemia, poor liver or kidney function, conges- tional Institutes of Health (NIH), or the American College of tive heart failure,,, clotting Cardiology. The AHA reports that there are no adequate, problems, or potentially allergic conditions are at higher controlled, published scientific studies using currently ap- risk for complications from chelation therapy. A Doppler proved scientific methods to support this therapy for the ultrasound may be performed to determine the adequacy of treatment of coronary artery disease. And in 2002, the American College of Advancement in Medicine pledged its full sup- port to a $30 million federal study aimed at determining the It is important for people who receive chelation safety and efficacy of chelation therapy in patients with heart therapy to work with medical personnel who are experi- disease. The five-year clinical trial involves more than 2,000 enced in the use of this treatment. The staff must be forthcoming about test results and should answer any questions the patient may have. The American Board of Chelation Therapy (ABCT) Evaluation and treatment should be individualized and provides minimum standards for members administering involve assessment of kidney function before each treat- chelation. Diplomates have passed written and oral tests ment with chelation, since the metals bound by the and received supervision of treatment in order to receive EDTA are excreted through the kidneys. Although EDTA binds harmful, toxic metals like One professional group that makes recommenda- mercury, lead, and cadmium, it also binds some essential tions for treatment methods is the American College for nutrients of the body, such as,, calcium, Advancement in Medicine (ACAM). If contacted, the or- Causes & symptoms COMMON ROUTES OF EXPOSURE TO TOXIC CHEMI- CALS. New “Occupational Exposure to Organic Solvents Places Fetus at York, NY: The Berkley Publishing Group, 1996. Stellaria media In addition to enhancing digestive processes, chico- Chicory is available over the counter in bulk as ry helps to keep the liver healthy. To prepare the herb as a tea, ry root supplements in the diet supports the proper me- also known as an infusion, for home use: steep 1 tsp (5 tabolism of. To treat jaundice, spleen problems, gallstones, or, drink 8-12 oz (225- 350 ml) of chicory tea per day. While the medicinal uses of chicory are numerous, the plant is also often used as a food additive, as a flavor- As a dietary supplement, 1 tsp (5 ml) of juice from ing agent, and in meals. Inulin can be used to improve chicory stems may be squeezed by hand and taken in the texture of processed foods as well as sweeten them. It can also be used to make biodegradable nonfood sub- stances with many industrial applications. This versatili- ty is important to environmentalists because chicory is a renewable natural resource. Chicory has shown to be safe for a variety of med- icinal uses and as a food source. There are no neces- Wild and cultivated chicory leaves may be added to sary precautions to observe when including the herb in salads or sautéed and served alone. Studies have shown that chicory complements coffee when it is used as a supplement due to its lactucin and There are no known health hazards or side effects lactucopicrin. The only possible the bitter taste of chicory, and may serve to counteract the minor side effect is skin irritation. Chicory by itself actually irritated after handling chicory, it is best to cover them has a sedative action on the central nervous system. Definition Anorexia Bulimia Causes & symptoms Gout hypoglycemia Malnutrition Megavitamin therapy Seborrheic dermatitis OTHER Diagnosis Cayenne 446 GALE ENCYCLOPEDIA OF ALTERNATIVE MEDICINE 2 vomiting nosebleeds cornus Chinese yam infertility impotence foxglove gas diar- fever rhea nausea pain constipation hot grains-of-paradise fruit flashes insomnia low back pain childbirth aging —A point or site on the body where qi tends to accumulate.