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No treatment is available for most of these disorders. Mannose supplementation relieves the symptoms in PMI-CDG (CDG-Ib) for the most part, even though the hepatic fibrosis may persist. Fucose supplementation has had a partial effect on some SLC35C1-CDG (CDG-IIc or LAD-II) patients.
Management of Bleeding Canker of Chestnut is not definitive and treatments are currently being investigated. Because the pathogen can be spread by contaminated tools, cultural practices are important to management. Tools should be cleaned and used with caution after being used on infected trees. Recovery of trees is possible, so management strategies are focused on keeping trees healthy so they can recover. One recommendation is to add fertilizer that contains Potassium phosphate. Soil de-compaction, providing good drainage, and mulching to minimize fluctuation of soil temperature and moisture are all ways to improve or maintain tree health and to manage the pathogen.
Chemical methods can be used to help the tree maintain health and avoid progress of the disease. Management strategies are currently being developed. A study performed in 2015 examined the infection on trees and found that 41 F1 progeny parent tree source had the most promising lines of viability for resistance.
Research into globozoospermia is aimed at improving understanding of its cause and developing treatment options.
Since the essential pathology is due to the inability to absorb vitamin B from the bowels, the solution is therefore injection of IV vitamin B. Timing is essential, as some of the side effects of vitamin B deficiency are reversible (such as RBC indices, peripheral RBC smear findings such as hypersegmented neutrophils, or even high levels of methylmalonyl CoA), but some side effects are irreversible as they are of a neurological source (such as tabes dorsalis, and peripheral neuropathy). High suspicion should be exercised when a neonate, or a pediatric patient presents with anemia, proteinuria, sufficient vitamin B dietary intake, and no signs of pernicious anemia.
Bark-binding is a disease in trees, cured by slitting the bark, or cutting it along the grain of the tree.
Administration of GH has no effect on IGF-1 production, therefore treatment is mainly by biosynthetic IGF-1. IGF-1 must be taken before puberty to be effective.
The drug product Increlex (mecasermin), developed by the company Tercica, now Genentech, was approved by the US Food and Drug Administration in August 2005 for replacing IGF-1 in patients who are deficient.
IPLEX (Mecasermin rinfabate) is composed of recombinant human IGF-1 (rhIGF-1) and its binding protein IGFBP-3. It was approved by the U.S. Food and Drug Administration (FDA) in 2005 for treatment of primary IGF-1 deficiency or GH gene deletion. Side effects from IPLEX are hypoglycemia. IPLEX's manufacturing company, Insmed, after selling its protein production facility, can no longer develop proteins, thus can no longer manufacture IPLEX as of a statement released in July 2009.
The presence of round headed sperm in a semen analysis sample confirms the diagnosis of globozoospermia. The lack of acrosome can be ascertained by a technique known as immunofluorescence.
Until 1995, the only options for people with globozoospermia who wished to conceive were adoption or sperm donation. With the advancement of assisted reproductive techniques (ART) it is now possible for those with globozoospermia to conceive using their own sperm. The main technique used is intracytoplasmic sperm injection (ICSI) where fertilisation is achieved by a single sperm being injected into the egg. Some studies have shown it is possible for a viable embryo to be created with this technique alone, however others have found it necessary to also use calcium ionophore treatment for fertilisation to be successful. Calcium ionophore treatment is used to artificially activate the oocyte. This treatment may be necessary as globozoospermic sperm can be less likely to activate the oocyte, an important stage in fertilisation.
The treatment options currently available focus on overcoming the prognosis of infertility which is associated with globozoospermia. So far there are no treatment options to prevent or cure globozoospermia.
Foot binding was the custom of applying tight binding to the feet of young girls to modify the shape of their feet. The practice possibly originated among upper class court dancers during the Five Dynasties and Ten Kingdoms period in 10th century China, then became popular among the elite during the Song dynasty and eventually spread to all social classes by the Qing dynasty. Foot binding became popular as a means of displaying status (women from wealthy families, who did not need their feet to work, could afford to have them bound) and was correspondingly adopted as a symbol of beauty in Chinese culture. Foot binding limited the mobility of women, resulting in them walking in a swaying unsteady gait, although some women with bound feet working outdoor had also been reported. The prevalence and practice of foot binding varied in different parts of the country. Feet altered by binding were called lotus feet.
It has been estimated that by the 19th century, 40–50% of all Chinese women may have had bound feet, and up to almost 100% among upper class Han Chinese women. The Manchu Kangxi Emperor tried to ban foot binding in 1664 but failed. In the later part of the 19th century, Chinese reformers challenged the practice but it was not until the early 20th century that foot binding began to die out as a result of anti-foot-binding campaigns. Foot-binding resulted in lifelong disabilities for most of its subjects, and a few elderly Chinese women still survive today with disabilities related to their bound feet.
In all cases of the disease, thorough study of the distribution and intensity should be determined to help choose a management practice that’s cost effective. For example, if the disease is widespread in a pre-commercial stand, destroying the plantation may be the most effective measure. The area can then be replanted with immune or low-susceptible species. Aerial surveying is a viable tool available for use in areas where there are severely damaged systems. Other management tools include:
- Using up to date models to help predict the spread of the disease can help with management choice
- Susceptible species should not be planted within 100 feet of a disease center
- Remove as many infected roots and stumps as possible to avoid inoculation of healthy plants
- Cut all infected trees within the disease center and all uninfected trees within 50 ft of the disease center
- Stumping is an expensive, yet effective measure in gently sloping, high-quality sites with light soils. After an excavator removes the stump, pieces of the root are torn and fragmented so invading soil organisms deter long-term inocula.
- Push-falling is an alternative to post-harvest stumping. Whole trees are pushed over with machinery to expose diseased roots for removal. Push-falling is effective in areas with slopes less than 30 percent and soil textures that are sandy to sandy loam.
- Applications of chemical fumigation (such as chloropicrin) have been unsuccessful in dealing with Laminated root rot.
The term can also be seen in herpetoculture, as this condition can occur in female reptiles. It is inadvisable to attempt to break a reptile egg to remove it from an egg-bound female. This procedure may be done by a veterinarian, who will insert a needle into the egg, and withdraw the contents with a syringe, allowing the egg to collapse and be removed. Non-surgical interventions include administering oxytocin to improve contractions and allow the egg(s) to pass normally. In many cases, egg-bound reptiles must undergo surgery to have stuck eggs removed.
Egg binding in reptiles is quickly fatal if left untreated; therefore, gravid females who become very lethargic and cease feeding need immediate medical treatment in order to treat the potentially life-threatening condition. An episode of the Animal Planet reality show "E-Vet Interns" featured the treatment of an egg-bound turtle named Napoleon. Exotics specialist Dr. Kevin Fitzgerald of Alameda East Veterinary Hospital is shown treating her with oxytocin and then eventually having to resort to surgery with footage of the large number of eggs that were removed. Dr. Fitzgerald was shown explaining to the new interns how dangerous this condition can be for a pet turtle and the need for early veterinary intervention.
Egg binding can occur if an egg is malformed and/or too large, the animal is weakened by illness, improper husbandry, stress, or if hormonal balances are wrong (producing weak contractions). Factors that can contribute to the risk of egg binding include calcium deficiency, breeding animals that are too young or too small, not providing suitable laying areas (leading to deliberate retention of eggs), and overfeeding of species in which clutch size is dependent on food intake (such as Veiled Chameleons).
In the context of behavioral ecology, egg binding can be an important factor in limiting clutch size. Lizards that lay fewer, but larger eggs are at higher risk for egg binding, and so there is selection pressure towards a minimum clutch size. For example, in common side-blotched lizards, females that lay fewer than the average 4–5 eggs per clutch have significantly increased risk of egg binding.
Various methods are applied.
- The most effective method is to plant peach trees against a house wall under an overhanging roof, possibly covered by a mat during the winter, to keep winter rain from the buds before they burst (and incidentally to delay blossoming until spring frosts are over), until the temperature exceeds in the spring, deactivating the fungus.
- Commercially, spraying the leaves with fungicides is the most common control method. The toxicity of these fungicides means they are not legally available to noncommercial growers in some countries. Spraying should be done in the winter well before budding. If trees are not sprayed early enough, treatment is ineffective. Copper-based mixtures (such as Bordeaux mixture) and lime sulfurs are two fungicides commonly used.
- Peach cultivars can be planted which show some resistance to peach leaf curl, or at least regenerate rapidly, such as Peach 'Benedicte'. No similarly resistant nectarine cultivar is yet known.
If a plant appears to have signs of leaf curl in a particular year, the disease will take its course, but precautions can be taken to sustain the tree or maximize crop yield: for example, treating with nitrogen and excess water to minimize stress on the tree; applying greasebands around the trunk to protect from insect infestation; and thinning the fruit. It is unclear whether removal of infected leaves from the tree is beneficial. Removing the infected leaves and fruit after they fall to the ground is sometimes also suggested but superfluous if, in the following winter, fungicides or rain protection are applied.
There are many strategies to cultural management. Establishment of new trees that are disease free by trying to plant trees as soon as they are received from the nursery to reduce the amount of stress the tree undergoes to reduce the amount of dead tissue. Apply insecticides to prevent insects such as, peach tree borer to prevent disease causing conidia from entering wounded parts of the tree that the insects create. Prune trees appropriately and at the correct time when buds start to break to promote wide angled branching. Infection at pruning sites is less common when done during late spring because of the smaller amount of inoculum present at this time. Inspect trees occasionally and removed any dead branches to prevent infection at these sites. Training trees properly also helps foster decreased amount of disease. Training trees during the first season to have branches develop wide crotch angles to sustain long orchard life. Avoid excessive and late fertilization during cold season to avoid low temperature injury. Fertilize trees during the early spring to prevent cold-susceptible growth.
The therapy of an acute TTP episode has to be started as early as possible. The standard treatment is the daily replacement of the missing ADAMTS13 protease in form of plasma infusions or in more severe episodes by plasma exchange. In the latter the patients plasma is replaced by donated plasma. The most common sources of ADAMTS13 is platelet-poor fresh frozen plasma (FFP) or solvent-detergent plasma.
The benefit of plasma exchange compared to plasma infusions alone may result from the additional removal of ULVWF. In general both plasma therapies are well tolerated, several mostly minor complications may be observed. The number of infusion/exchange sessions needed to overcome a TTP episode are variable but usually take less than a week in USS. The intensive plasma-therapy is generally stopped when platelet count increases to normal levels and is stable over several days.
The process was started before the arch of the foot had a chance to develop fully, usually between the ages of 4 and 9. Binding usually started during the winter months since the feet were more likely to be numb, and therefore the pain would not be as extreme.
First, each foot would be soaked in a warm mixture of herbs and animal blood; this was intended to soften the foot and aid the binding. Then, the toenails were cut back as far as possible to prevent in-growth and subsequent infections, since the toes were to be pressed tightly into the sole of the foot. Cotton bandages, 3 m long and 5 cm wide (10 ft by 2 in), were prepared by soaking them in the blood and herb mixture. To enable the size of the feet to be reduced, the toes on each foot were curled under, then pressed with great force downwards and squeezed into the sole of the foot until the toes broke.
The broken toes were held tightly against the sole of the foot while the foot was then drawn down straight with the leg and the arch of the foot was forcibly broken. The bandages were repeatedly wound in a figure-eight movement, starting at the inside of the foot at the instep, then carried over the toes, under the foot, and around the heel, the freshly broken toes being pressed tightly into the sole of the foot. At each pass around the foot, the binding cloth was tightened, pulling the ball of the foot and the heel together, causing the broken foot to fold at the arch, and pressing the toes underneath the sole. The binding was pulled so tightly that the girl could not move her toes at all and the ends of the binding cloth were then sewn so that the girl could not loosen it.
The girl's broken feet required a great deal of care and attention, and they would be unbound regularly. Each time the feet were unbound, they were washed, the toes carefully checked for injury, and the nails carefully and meticulously trimmed. When unbound, the broken feet were also kneaded to soften them and the soles of the girl's feet were often beaten to make the joints and broken bones more flexible. The feet were also soaked in a concoction that caused any necrotic flesh to fall off.
Immediately after this agonizing procedure, the girl's broken toes were folded back under and the feet were rebound. The bindings were pulled even tighter each time the girl's feet were rebound. This unbinding and rebinding ritual was repeated as often as possible (for the rich at least once daily, for poor peasants two or three times a week), with fresh bindings. It was generally an elder female member of the girl's family or a professional foot binder who carried out the initial breaking and ongoing binding of the feet. It was considered preferable to have someone other than the mother do it, as she might have been sympathetic to her daughter's pain and less willing to keep the bindings tight.
For most the bound feet eventually became numb. However, once a foot had been crushed and bound, attempting to reverse the process by unbinding is painful, and the shape could not be reversed without a woman undergoing the same pain all over again.
Not all affected patients seem to need a regular preventive plasma infusion therapy, especially as some reach longterm remission without it. Regular plasma infusions are necessary in patients with frequent relapses and in general situations with increased risk to develop an acute episode (as seen above) such as pregnancy. Plasma infusions are given usually every two to three weeks to prevent acute episodes of USS but are often individually adapted.
Control of the beetle vector is the most effective management technique for disease prevention. Conventional methods of tree thinning and the use of insecticides have been used to combat the western bark beetles, but are only effective before the beetles have colonized and before the fungus has invaded the tree. Other cultural techniques of sanitation and overall health of the oak trees by keeping up with watering, fertilizer or mulch needs, and pruning may help. It is very important to diagnose foamy bark canker disease correctly and promptly in order to manage the disease properly because if a tree is already infected, the removal of the tree is the most effective way to prevent the disease from spreading.
Plant varieties that are resistant to "Armillaria" or species are resistant to other environmental or biological stressors. If the infected area has been cleared of trees, plants that are not vulnerable to the disease should be planted for five or so years until "Armillaria" is eradicated. Stump removal is also an effective management tool but can be expensive. Another way to reduce susceptibility is to maintain plant health by regular fertilization (if needed), watering during droughts, and trying not to create wounds on the plant. Fumigation can also be used to reduce the amount of inoculum.
Control of Leucostoma Canker is possible through a combination of pest and crop management techniques following life cycles of the trees. The strategy is implemented following techniques aimed at reducing number of pathogenic inoculum, minimizing dead or injured tissues to prevent infection, and improving tree health to improve rapid wound healing. Chemical controls have not been very effective at controlling this disease with no fungicides registered specifically for control of "Leucostoma" spp., and demethylation-inhibiting (DMI) fungicides having almost no effect on "L. persoonii".
Corticosteroids are administered through IV or orally. They cause lymphocytopenia, a condition where white blood cell levels are abnormally low. Corticosteroids cause white blood cell death, lowering their numbers throughout the body. They also cause white blood cells to recirculate away from the area of damage (the retina). This minimizes damage caused by the antibodies produced by the white blood cells. Often, this is treatment is combined with plasmapheresis. Instead of treating the plasma and blood cells, they are replaced with a healthy donor mixture. Patients who respond positively show improved visual fields and an almost complete disappearance of anti-retinal antibodies.
Thousand cankers disease can be spread by moving infected black walnut wood. Trees intended for shipment should be inspected for dieback and cankers and galleries after harvest. G. morbidia or the walnut twig beetle ("Pityophthorus juglandis") are not currently known to be moved with walnut seed . There is currently no chemical therapy or prevention available for the disease making it difficult to control the spread of the disease from the west to the eastern united states. Wood from infected trees can still be used for commercial value, but safety measures such as removing the bark, phloem, and cambium to reduce the risk of spreading the disease with shipment. Quarantines have been put in place in some states to reduce the potential movement of fungus or beetle from that region. On May 17th, 2010, the Director of the Michigan Department of Agriculture issued a quarantine from affected states to protect Michigan’s black walnut ecology and production. Contacting the appropriate entities about possible infections is important to stopping or slowing the spread of thousand cankers disease.
Cinchonism or quinism is a pathological condition caused by an overdose of quinine or quinidine, or their natural source, cinchona bark. Quinine and its derivatives are used medically to treat malaria. In much smaller amounts, quinine is an ingredient of tonic drinks, acting as a bittering agent. Cinchonism can occur from therapeutic doses of quinine, either from one or several large doses. Quinidine (Class 1A anti-arrhythmic) can also cause cinchonism symptoms to develop with as little as a single dose.
Due to the difficulty of diagnosis, managing this disease is a challenge. For this reason, there is no established treatment for AIR. Clinicians try to reduce and control the autoimmune system attack to prevent any irreversible retinal damage. Methods of treatment include intravenous immunoglobulin (IVIG), plasmapheresis, and corticosteroids.
Once a diagnosis is made, the treatment is based on an individual’s clinical condition. Based on the apparent activation of the mTOR pathway, Lucas and colleagues treated patients with rapamycin, an mTOR inhibitor. This effectively reduced hepatosplenomegaly and lymphadenopathy, most likely by restoring the normal balance of naïve, effector, and memory cells in the patients’ immune system. More research is needed to determine the most effective timing and dosage of this medication and to investigate other treatment options. Investigators at the National Institute of Allergy and Infectious Diseases at the US National Institutes of Health currently have clinical protocols to study new approaches to the diagnosis and treatment of this disorder.
CFTR has been a drug target in efforts to find treatments for related conditions. Ivacaftor (trade name Kalydeco, developed as VX-770) is a drug approved by the FDA in 2012 for people with cystic fibrosis who have specific CFTR mutations Ivacaftor was developed by Vertex Pharmaceuticals in conjunction with the Cystic Fibrosis Foundation and is the first drug that treats the underlying cause rather than the symptoms of the disease. Called "the most important new drug of 2012", and "a wonder drug" it is one of the most expensive drugs, costing over US$300,000 per year, which has led to criticism of Vertex for the high cost.
Symptoms of mild cinchonism (which may occur from standard therapeutic doses of quinine) include flushed and sweaty skin, ringing of the ears (tinnitus), blurred vision, impaired hearing, confusion, reversible high-frequency hearing loss, headache, abdominal pain, rashes, drug-induced lichenoid reaction (lichenoid photosensitivity), vertigo, dizziness, dysphoria, nausea, vomiting and diarrhea.
Large doses of quinine may lead to severe (but reversible) symptoms of cinchonism: skin rashes, deafness, somnolence, diminished visual acuity or blindness, anaphylactic shock, and disturbances in heart rhythm or conduction, and death from cardiotoxicity (damage to the heart). Quinine may also trigger a rare form of hypersensitivity reaction in malaria patients, termed blackwater fever, that results in massive hemolysis, hemoglobinemia, hemoglobinuria, and kidney failure. Most symptoms of cinchonism (except in severe cases) are reversible and disappear once quinine is withdrawn. Attempted suicide by intake of a large dose of quinine has caused irreversible tunnel vision and very severe visual impairment.
Patients treated with quinine may also suffer from low blood sugar, especially if it is administered intravenously, and hypotension (low blood pressure).
Quinine, like chloroquine, inactivates enzymes in the lysosomes of cells and has an anti-inflammatory effect, hence its use in the treatment of rheumatoid arthritis. However, inactivation of these enzymes can also cause abnormal accumulation of glycogen and phospholipids in lysosomes, causing toxic myopathy. It is possible this action is the root cause of cinchonism.