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Deep Learning Technology: Sebastian Arnold, Betty van Aken, Paul Grundmann, Felix A. Gers and Alexander Löser. Learning Contextualized Document Representations for Healthcare Answer Retrieval. The Web Conference 2020 (WWW'20)
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If a child finds it difficult to blow, pinching the nose can help regulate airflow. The child should then practice speech sounds without pinching the nose.
These exercises only work as treatments if hypernasality is small. Severe deviations should be treated surgically.
Prostheses are used for nonsurgical closure in a situation of velopharyngeal dysfunction. There are two types of prosthesis: the speech bulb and the palatal lift prosthesis. The speech bulb is an acrylic body that can be placed in the velopharyngeal port and can achieve obstruction. The palatal lift prosthesis is comparable with the speech bulb, but with a metal skeleton attached to the acrylic body. This will also obstruct the velopharyngeal port. It is a good option for patients that have enough tissue but a poor control of the coordination and timing of velopharyngeal movement. It is also used in patients with contraindications for surgery. It has also been used as a reversible test to confirm whether a surgical intervention would help.
There is insufficient evidence to support the use of traditional non-speech oral motor exercises can reduce hypernasality. Velopharyngeal closure patterns and their underlying neuromotor control may differ for speech and nonspeech activities. Therefore, the increase in velar movement through blowing, sucking, and swallowing may not transfer to speech tasks. Thus, hypernasality remains while individual speak. Kuehn proposed a new way of treatment by using a CPAP machine during speech tasks. The positive pressure provided by a CPAP machine provides resistance to stregthen velopharyngeal muscles. With nasal mask in place, an individual is asked to produce VNCV syllables and short sentences. It is believed that CPAP therapy can increase both muscle endurance as well as strength because it overloads the levator veli palatini muscle and involves a regimen with a large number of repetitions of velar elevation. Research findings proved that patients with hypernasality due to flaccid dysarthria, TBI or cleft palate do eliminate hypernasality after receiving this training program.
The main objective of physical treatment is to achieve adequate velopharyngeal (VP) function and normal oral-nasal resonance.
Cleft lip and palate is very treatable; however, the kind of treatment depends on the type and severity of the cleft.
Most children with a form of clefting are monitored by a "cleft palate team" or "craniofacial team" through young adulthood. Care can be lifelong. Treatment procedures can vary between craniofacial teams. For example, some teams wait on jaw correction until the child is aged 10 to 12 (argument: growth is less influential as deciduous teeth are replaced by permanent teeth, thus saving the child from repeated corrective surgeries), while other teams correct the jaw earlier (argument: less speech therapy is needed than at a later age when speech therapy becomes harder). Within teams, treatment can differ between individual cases depending on the type and severity of the cleft.
A craniofacial team is routinely used to treat this condition. The majority of hospitals still use craniofacial teams; yet others are making a shift towards dedicated cleft lip and palate programs. While craniofacial teams are widely knowledgeable about all aspects of craniofacial conditions, dedicated cleft lip and palate teams are able to dedicate many of their efforts to being on the cutting edge of new advances in cleft lip and palate care.
Many of the top pediatric hospitals are developing their own CLP clinics in order to provide patients with comprehensive multi-disciplinary care from birth through adolescence. Allowing an entire team to care for a child throughout their cleft lip and palate treatment (which is ongoing) allows for the best outcomes in every aspect of a child's care. While the individual approach can yield significant results, current trends indicate that team based care leads to better outcomes for CLP patients. .
A common method to treat Velopharyngeal insufficiency is pharyngeal flap surgery, where tissue from the back of the mouth is used to close part of the gap. Other ways of treating velopharyngeal insufficiency is by placing a posterior nasopharyngeal wall implant (commonly cartilage or collagen) or type of soft palate lengthening procedure (i.e. VY palatoplasty).
Velopharyngeal inadequacy (VPI) is a malfunction of a velopharyngeal mechanism.
The velopharyngeal mechanism is responsible for directing the transmission of sound energy and air pressure in both the oral cavity and the nasal cavity. When this mechanism is impaired in some way, the valve does not fully close, and a condition known as 'velopharyngeal inadequacy' can develop. VPI can either be congenital or acquired later in life.
Endoscopic injection involves applying a gel around the ureteral opening to create a valve function and stop urine from flowing back up the ureter. The gel consists of two types of sugar-based molecules called dextranomer and hyaluronic acid. Trade names for this combination include Deflux and Zuidex. Both constituents are well-known from previous uses in medicine. They are also biocompatible, which means that they do not cause significant reactions within the body. In fact, hyaluronic acid is produced and found naturally within the body.
Medical treatment entails low dose antibiotic prophylaxis until resolution of VUR occurs. Antibiotics are administered nightly at half the normal therapeutic dose. The specific antibiotics used differ with the age of the patient and include:
- Amoxicillin or ampicillin – infants younger than 6 weeks
- Trimethoprim-sulfamethoxazole (co-trimoxazole) – 6 weeks to 2 months
After 2 months the following antibiotics are suitable:
- Nitrofurantoin {5–7 mg/kg/24hrs}
- Nalidixic acid
- Bactrim
- Trimethoprim
- Cephalosporins
Urine cultures are performed 3 monthly to exclude breakthrough infection. Annual radiological investigations are likewise indicated. Good perineal hygiene, and timed and double voiding are also important aspects of medical treatment. Bladder dysfunction is treated with the administration of anticholinergics.
Evidence is insufficient to support the use of medications to treat obstructive sleep apnea. This includes the use of fluoxetine, paroxetine, acetazolamide and tryptophan among others.
While there is no specific treatment for the underlying genetic cause of LFS; corrective procedures, preventive intervention measures and therapies may be considered in the treatment and management of the many craniofacial, orthopedic and psychiatric problems associated with the disorder. More pressing issues such as cardiac involvement or epileptic seizures should be routinely examined and monitored. Close attention and specialized follow-up care, including neuropshycological evaluation methods and therapies, and special education, should be given to diagnose and prevent psychiatric disorders and related behavioral problems such as psychosis and outbursts of aggression.
Surgical treatments to modify airway anatomy, known as sleep surgery, are varied and must be tailored to the specific airway obstruction needs of a patient. Surgery is not considered a frontline treatment for obstructive sleep apnea, as prospective, randomized, comparative clinical evidence against current front line treatments is lacking. For those obstructive sleep apnea sufferers unable or unwilling to comply with front line treatment, a properly selected surgical intervention will be the result of considering an individual's specific anatomy and physiology, personal preference and disease severity. There is little randomized clinical trial evidence for all types of sleep surgery.
There are a number of different operations that may be performed including:
- Nasal surgery, including turbinectomy (removal or reduction of a nasal turbinate), or straightening of the nasal septum, in patients with nasal obstruction or congestion which reduces airway pressure and complicates OSA.
- Tonsillectomy and/or adenoidectomy in an attempt to increase the size of the airway.
- Removal or reduction of parts of the soft palate and some or all of the uvula, such as uvulopalatopharyngoplasty (UPPP) or laser-assisted uvulopalatoplasty ("LAUP"). Modern variants of this procedure sometimes use radiofrequency waves to heat and remove tissue.
- Reduction of the tongue base, either with laser excision or radiofrequency ablation.
- Genioglossus advancement, in which a small portion of the lower jaw that attaches to the tongue is moved forward, to pull the tongue away from the back of the airway.
- Hyoid suspension, in which the hyoid bone in the neck, another attachment point for tongue muscles, is pulled forward in front of the larynx.
- Maxillomandibular advancement
In the morbidly obese, a major loss of weight (such as what occurs after bariatric surgery) can sometimes cure the condition.
OSA in children is sometimes due to chronically enlarged tonsils and adenoids. Tonsillectomy and adenoidectomy are curative. The operation may be far from trivial, especially in the worst apnea cases, in which growth is retarded and abnormalities of the right heart may have developed. Even in these extreme cases, the surgery tends to cure not only the apnea and upper airway obstruction but allows normal subsequent growth and development. Once the high end-expiratory pressures are relieved, the cardiovascular complications reverse themselves. The postoperative period in these children requires special precautions (see "Surgery and obstructive sleep apnea syndrome" below).
Intervention services will be guided by the strengths and needs determined by the speech and language evaluation. The areas of need may be addressed individually until each one is functional; alternatively, multiple needs may be addressed simultaneously through the intervention techniques. If possible, all interventions will be geared towards the goal of developing typical communicative interaction. To this end, interventions typically follow either a preventive, remedial, or compensatory model. The preventive service model is common as an early intervention technique, especially for children whose other disorders place them at a higher risk for developing later communication problems. This model works to lessen the probability or severity of the issues that could later emerge. The remedial model is used when an individual already has a speech or language impairment that he/she wishes to have corrected. Compensatory models would be used if a professional determines that it is best for the child to bypass the communication limitation; often, this relies on AAC.
Language intervention activities are used in some therapy sessions. In these exercises, an SLP or other trained professional will interact with a child by working with the child through play and other forms of interaction to talk to the child and model language use. The professional will make use of various stimuli, such as books, objects, or simple pictures to stimulate the emerging language. In these activities, the professional will model correct pronunciation, and will encourage the child to practice these skills. Articulation therapy may be used during play therapy as well, but involves modeling specific aspects of language—the production of sound. The specific sounds will be modeled for the child by the professional (often the SLP), and the specific processes involved in creating those sounds will be taught as well. For example, the professional might instruct the child in the placement of the tongue or lips in order to produce certain consonant sounds.
Technology is another avenue of intervention, and can help children whose physical conditions make communication difficult. The use of electronic communication systems allow nonspeaking people and people with severe physical disabilities to engage in the give and take of shared thought.
Students identified with a speech and language disability often qualify for an Individualized Education Plan as well as particular services. These include one-on-one services with a speech and language pathologist. Examples used in a session include reading vocabulary words, identifying particular vowel sounds and then changing the context, noting the difference. School districts in the United States often have speech and language pathologists within a special education staff to work with students. Additionally, school districts can place students with speech and language disabilities in a resource room for individualized instruction. A combination of early intervention and individualized support has shown promise increasing long-term academic achievement with students with this disability.
Students might work individually with a specialist, or with a specialist in a group setting. In some cases, the services provided to these individuals may even be provided in the regular education classroom. Regardless of where these services are provided, most of these students spend small amounts of time in therapy and the large majority of their time in the regular education classroom with their typically developing peers.
Therapy often occurs in small groups of three or four students with similar needs. Meeting either in the office of the speech-language pathologist or in the classroom, sessions may take from 30 minutes to one hour. They may occur several times per week. After introductory conversations, the session is focused on a particular therapeutic activity, such as coordination and strengthening exercises of speech muscles or improving fluency through breathing techniques. These activities may take the form of games, songs, skits, and other activities that deliver the needed therapy. Aids, such as mirrors, tape recorders, and tongue depressors may be utilized to help the children to become aware of their speech sounds and to work toward more natural speech production.
Hearing loss with craniofacial syndromes is a common occurrence. Many of these multianomaly disorders involve structural malformations of the outer or middle ear, making a significant hearing loss highly likely.
No cure is known for 22q11.2 deletion syndrome. Certain individual features are treatable using standard treatments. The key is to identify each of the associated features and manage each using the best available treatments.
For example, in children, it is important that the immune problems are identified early, as special precautions are required regarding blood transfusion and immunization with live vaccines. Thymus transplantation can be used to address absence of the thymus in the rare, so-called "complete" 22q11.2 deletion syndrome. Bacterial infections are treated with antibiotics. Cardiac surgery is often required for congenital heart abnormalities. Hypoparathyroidism causing hypocalcaemia often requires lifelong vitamin D and calcium supplements. Specialty clinics that provide multi-system care allow for individuals with 22q11.2 deletion syndrome to be evaluated for all of their health needs and allow for careful monitoring of the patients. An example of this type of system is the 22q Deletion Clinic at SickKids Hospital in Toronto, Canada, which provides children with 22q11 deletion syndrome ongoing support, medical care and information from a team of health care workers.
Individuals with Nager syndrome typically have the malformations of the auricle, external auditory canal, and middle ear, including the ossicles. These malformations were found in 80% of individuals with Nager syndrome. Inner ear malformations, however, are not typically seen in this population. Middle ear disease is common among individuals with Nager syndrome. Chronic otitis media and Eustachian tube deformity can result in conductive hearing loss. For this reason, early detection and treatment for middle ear disease is crucial in this population. Sensorineural hearing loss is not a typical characteristic of Nager syndrome; however, a subset of individuals present with a mixed hearing loss, due to a progressive sensorineural component combined with the typical conductive hearing loss (Herrman "et al.", 2005).
Botulinum toxin A (Botox) has been considered as a treatment option, under the idea of temporarily reducing the hypertonicity of the pelvic floor muscles. Although no random controlled trials have been done with this treatment, experimental studies with small samples have shown it to be effective, with sustained positive results through 10 months. Similar in its mechanism of treatment, lidocaine has also been tried as an experimental option.
Anxiolytics and antidepressants are other pharmacotherapies that have been offered to patients in conjunction with other psychotherapy modalities, or if these patients experience high levels of anxiety from their condition. Results from these types of pharmacologic therapies have not been consistent.
Often, when faced with a person experiencing painful intercourse, a gynecologist will recommend Kegel exercises and provide some additional lubricants. Strengthening the muscles that unconsciously tighten during vaginismus may be extremely counter-intuitive for some people. Although vaginismus has not been shown to affect a person's ability to produce lubrication, providing additional lubricant can be helpful in achieving successful penetration. This is due to the fact that women may not produce natural lubrication if anxious or in pain. Treatment of vaginismus may involve the use Hegar dilators, (sometimes called vaginal trainers) progressively increasing the size of the dilator inserted into the vagina.
Ectrodactyly–ectodermal dysplasia–cleft syndrome, or EEC, and also referred to as EEC syndrome (also known as "Split hand–split foot–ectodermal dysplasia–cleft syndrome") is a rare form of ectodermal dysplasia, an autosomal dominant disorder inherited as an genetic trait. EEC is characterized by the triad of ectrodactyly, ectodermal dysplasia, and facial clefts. Other features noted in association with EEC include vesicoureteral reflux, recurrent urinary tract infections, obstruction of the nasolacrimal duct, decreased pigmentation of the hair and skin, missing or abnormal teeth, enamel hypoplasia, absent punctae in the lower eyelids, photophobia, occasional cognitive impairment and kidney anomalies, and conductive hearing loss.
Acetylcholinesterase inhibitors can provide symptomatic benefit and may not fully remove a person's weakness from MG. While they might not fully remove all symptoms of MG, they still may allow a person the ability to perform normal daily activities. Usually, acetylcholinesterase inhibitors are started at a low dose and increased until the desired result is achieved. If taken 30 minutes before a meal, symptoms will be mild during eating, which is helpful for those who have difficulty swallowing due to their illness. Another medication used for MG, atropine, can reduce the muscarinic side effects of acetylcholinesterase inhibitors. Pyridostigmine is a relatively long-acting drug (when compared to other cholinergic agonists), with a half-life around four hours with relatively few side effects. Generally, it is discontinued in those who are being mechanically ventilated as it is known to increase the amount of salivary secretions. A few high-quality studies have directly compared cholinesterase inhibitors with other treatments (or placebo); their practical benefit may be such that it would be difficult to conduct studies in which they would be withheld from some people. The steroid prednisone might also be used to achieve a better result, but it can lead to the worsening of symptoms for 14 days and takes 6–8 weeks to achieve its maximal effectiveness. Due to the myriad symptoms that steroid treatments can cause, it is not the preferred method of treatment. Other immune suppressing medications may also be used including rituximab.
If the myasthenia is serious (myasthenic crisis), plasmapheresis can be used to remove the putative antibodies from the circulation. Also, intravenous immunoglobulins (IVIGs) can be used to bind the circulating antibodies. Both of these treatments have relatively short-lived benefits, typically measured in weeks, and often are associated with high costs which make them prohibitive; they are generally reserved for when MG requires hospitalization.
Ectrodactyly involves the deficiency or absence of one or more central digits of the hand or foot and is also known as split hand–split foot malformation (SHFM). The hands and feet of people with ectrodactyly are often described as "claw-like" and may include only the thumb and one finger (usually either the little finger, ring finger, or a syndactyly of the two) with similar abnormalities of the feet.
Ectodermal dysplasia describes abnormalities of structures derived from the embryonic ectoderm. These abnormalities affect both the superficial ectodermal layer, as well as the mesectodermal layer constituted by the neural crest.
Over time, the approach to CP management has shifted away from narrow attempts to fix individual physical problems such as spasticity in a particular limb to making such treatments part of a larger goal of maximizing the person's independence and community engagement. Much of childhood therapy is aimed at improving gait and walking. Approximately 60% of people with CP are able to walk independently or with aids at adulthood. However, the evidence base for the effectiveness of intervention programs reflecting the philosophy of independence has not yet caught up: effective interventions for body structures and functions have a strong evidence base, but evidence is lacking for effective interventions targeted toward participation, environment, or personal factors. There is also no good evidence to show that an intervention that is effective at the body-specific level will result in an improvement at the activity level, or vice versa. Although such cross-over benefit might happen, not enough high-quality studies have been done to demonstrate it.
Because cerebral palsy has "varying severity and complexity" across the lifespan, it can be considered a collection of conditions for management purposes. A multidisciplinary approach for cerebral palsy management is recommended, focusing on "maximising individual function, choice and independence" in line with the International Classification of Functioning, Disability and Health's goals. The team may include a paediatrician, a health visitor, a social worker, a physiotherapist, an orthotist, a speech and language therapist, an occupational therapist, a teacher specialising in helping children with visual impairment, an educational psychologist, an orthopaedic surgeon, a neurologist and a neurosurgeon.
Various forms of therapy are available to people living with cerebral palsy as well as caregivers and parents. Treatment may include one or more of the following: physical therapy; occupational therapy; speech therapy; water therapy; drugs to control seizures, alleviate pain, or relax muscle spasms (e.g. benzodiazepines); surgery to correct anatomical abnormalities or release tight muscles; braces and other orthotic devices; rolling walkers; and communication aids such as computers with attached voice synthesisers. A Cochrane review published in 2004 found a trend toward benefit of speech and language therapy for children with cerebral palsy, but noted the need for high quality research. A 2013 systematic review found that many of the therapies used to treat CP have no good evidence base; the treatments with the best evidence are medications (anticonvulsants, botulinum toxin, bisphosphonates, diazepam), therapy (bimanual training, casting, constraint-induced movement therapy, context-focused therapy, fitness training, goal-directed training, hip surveillance, home programmes, occupational therapy after botulinum toxin, pressure care) and surgery (selective dorsal rhizotomy).