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Evidence supports the need to develop strategies to increase patient acceptance for routine care, additional training for dentists to provide this care, and the development of more effective preventive strategies to minimize the need for this care. This chapter is based on the provision of dental service to people with intellectual disability in Israel during the past 30 years. In general, people with intellectual disability have poorer oral health and oral hygiene than those without this condition. Data indicate that people who have intel lectual disability have more untreated caries, higher prevalence of gingivitis, and I. Merrick other periodontal diseases than the general population affecting their ability to chew, speak, and look attractive [3]. With increasing age and life expectancy this popula tion will be in need of good dental care on a regular basis to prevent disease and increase their quality of life [4, 5]. Caries rates were the same as or higher than the general population, but the rates of untreated caries were consistently higher. Two subgroups at especially high risk for oral health problems are people with Down syndrome and people unable to cooperate with routine dental care [6]. Residential care is provided to about 7,000 persons in 63 residential centers all over the country (see Tables 21. The study showed that only 20% of the residents were able to fully cooperate with the dental procedure and treatment, while 84% of all residents were in need of treatment. About 50% of the residents needed pre-medication or general anesthesia in order to examine and treat them. Over 70% had suffered from caries in various degrees and 25% showed serious damage as indicated by seven and more decayed teeth per person. About 60% had lost one or more teeth and 17% more than seven teeth, whereas only 30% had fillings in one or more teeth. A total of 362 were able to collaborate with the exam, and in the 2 to 11-year-old group decayed teeth was found at decayed, D = 12. The sample was divided into four groups: (1) educable (n = 70), (2) train able (n = 92), (3) behavioral problems (n = 106), and (4) severe physical handicap (n = 119). Findings confirmed high dental morbidity and significant oral health differences by behavior group, age, and dental clinic status. Community studies have been conducted, but usually with smaller number or specialized populations like Down syndrome [12, 13]. The most frequent were visual (33%), hearing impairments (20%), and dental prob lems (30%). The issue and problems we have observed in Israel are also found in other countries like the United States [14], the United Kingdom [15], and India [16]. This has not been the situation in Israel, where large institutions of the size seen in the United States never existed, as the average residential care center today in Israel consists of about 122 residents. We have by now established 15 dental clinics spread around Israel to meet the national need for this population. The clinics are located on the premises of res idential care centers, usually in close connection with the general medical clinics with a waiting room area for groups of people from other centers or the community 21 Dental Aspects 345 arriving for dental care after appointments.

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Sensory tricks (eg, light touch of the face) may help to gressive disorder related to mutations in the ferritin light reduce the intensity of symptoms. It is characterized initially by chorea, are common, and a head tremor may be present. Anxiety, depression, a personality disorder, or some combination of these and other psychiatric disturbances may be present. However, anxiety and depression are com mon consequences of dystonia that has an organic basis. Features that help to support a diagnosis of psychogenic dystonia include variable and inconsistent findings, find ings that are incongruent with those of organic dystonia, a known psychologic precipitating factor, excessive pain, a prior history of somatoform disorder, other psychogenic signs (eg, nonanatomic sensory loss), multiple somatiza tions, abnormal posturing that disappears with distraction, lack of sensory tricks (to relieve the dystonia) and overflow dystonia, onset in the lower limbs in adults, and an impair ment of function that is out of proportion to the dystonia and is selective in a manner that is difficult to explain (eg, limits the ability to work but not the ability to dress and attend to other activities of daily living). Symptoms may be relieved by psychotherapy, suggestion, or treatment with a placebo. Treatment is of the underlying disinhibition and emotional lability are common even at an disorder. An action-specific facial dystonia (involving frontal lobe epilepsy characterized by intermittent dystonia symmetric frontalis and platysma contraction, giving a and choreoathetoid movements during sleep; it responds startled appearance) is characteristic. Dysexecutive syndromes, obsessive several hours characterize this disorder, which is inherited as compulsive disorder, depression, and psychosis may all an autosomal dominant trait with incomplete penetrance. The peripheral blood contains circulating acanthocytes Affected patients are likely to harbor mutations in the myofi (spiny red cells) but a normal lipid profile. Certain mitochondrionopathies may also be associated Onset may be in childhood or early adulthood. There may be a history of convulsions in enter the circulation and are subsequently deposited in infancy. Attacks begin in the first or second decade, last for tissues, including the brain, liver, kidney, and cornea. They often respond to anticonvulsant medication, suggest that free radical formation and oxidative damage, especially carbamazepine. Onset is usually before 30 years of age; attacks last for several Wilson disease usually presents in childhood or young adult minutes to hours and are poorly responsive to medication. The levels of serum copper and ceruloplasmin (an a2-globulin to which 90% of the circulating copper is bound) are low, and 24-hour urinary copper excretion is generally increased. Most physicians use penicilla mine, a copper-chelating agent that promotes extraction of copper from tissue deposition sites, even though instances (Figure 11-8): bilateral brown corneal rings that result of penicillamine-induced worsening have been described. The rings Treatment should be started as early as possible and cus are present in virtually all patients with neurologic involve tomarily employs approximately 1. Hepatic involvement leads to chronic cirrhosis, which may be complicated by splenomegaly, esophageal varices with hematemesis, or fulminant hepatic failure. Neurologic signs include resting or postural tremor, choreiform movements of the limbs, facial grimac ing, rigidity, hypokinesia, dysarthria, dysphagia, abnormal (flexed) postures, and ataxia.

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In these populations the carrier frequencies that have been isolated for centuries with high vary from 1:3 to 1:6, which are the highest ever rates of consanguinity indicates that most cases reported for autosomal recessive disorders. The ture and disease severity may vary among differ E148Q is the least penetrant mutation and is rec ent ethnic groups62. Another important ques prise fever and possibility of serositis or arthritis, tion is the possibility of genotype-phenotype cor lasting from 1 to 3 days, resolving spontaneous relations: in some surveys M694V homozygosis, ly. Attacks do not have a regular periodism in M680I homozygosis and heterozygosis for consequence of a frequency varying from one a M608I/M694V genotypes are associated with se week to one every three-four months or more; in Table I. Mutations Associations M694V Most frequent among Jews, Fever 96% Turks, Armenians and Syrians Peritonitis 91% Pleurisy 57% M680I More common in Armenians and Arthritis or arthralgias 45% Egyptians Erysipelas-like erythema at 13% M694I More common among Arabs the inferior limbs E148Q Mostly observed in Europeans Amyloidosis 2-12% and Turkish carriers Destructive chronic arthritis 2-5% V726A Mild phenotype Recurrent pericarditis 1-2. Stabile tervals between attacks are different both among splenomegaly and oral aphthae. Other organs involved mation with a clinical outline simulating an acute by amyloid deposition are gut, spleen, liver, heart abdominal pathology like appendicitis, cholecys and endocrine glands. Some au In addition amyloidosis appears to be less com thors recommend a laparoscopic appendectomy mon among Iraqis, Ashkenazi Jews and Arabs. Constipa mutation M694V is widely reported, but non tion may be present in some patients, but diar amyloid glomerulopathies such as IgM or IgA rhoea is more common in children. Recurrent nephropathy, focal and diffuse proliferative peritonitis can cause adherences compromising glomerulonephritis and rapidly progressive female fertility66. In in 13% patients and is often localized over legs patients with a typical clinical outline and appro or feet. Genetic testing might reveal tokines are greatly increased during acute at no known mutation in 30-40% patients or a sin tacks such as interleukin-1, interferon-gamma, gle known mutation: in this case if clinical mani interleukin-6 and interleukin-8. Metaraminol provocation test (with the seems to be useful, above all to control inflam intravenous infusion of 10 mg metaraminol in matory signs such as pleurisy pain. Continuative physiologic solution) could trigger typical at colchicine therapy, even if started during child tacks in a period ranging from 2 to 48 hours hood, has no effects both on the normal growth since the infusion80. Complete remission is achieved in tients who present typical acute attacks show a 65% patients and partial remission in 30% with generalized increase of the inflammatory para 5% patients remaining unresponsive. Protracted meters (erythrosedimentation rate, C-reactive arthritis, mostly affecting hip or knees, can be protein, serum amyloid-A, immunoglobulins) managed with intravenous infliximab or subcu taneous etanercept administration. Tel Hashomer criteria for the diagnosis of famil responders, but interferon-alpha or thalidomide ial Mediterranean fever. Periodic fever syndromes: a clinical rare condition only confined to the Eastern overview. Trans whilst the unrecognized disease has a negative Ass Am Phys 1908; 23: 504-518.

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Definition the definition of cerebral palsy has evolved over the years, refiecting changing understanding of the causes and consequences of this disorder. A recent definition, put forward by the American Academy for Cerebral Palsy and Developmental Medicine, is N. Dodge Cerebral palsy describes a group of disorders of the development of movement and posture, causing activity limitations that are attributed to non-progressive disturbances that occurred in the developing fetal or infant brain. The motor disorders of cerebral palsy are often accompanied by disturbances of sensation, cognition, communication perception and/or by a seizure disorder [1]. Explaining the definition of cerebral palsy to families can prevent unnecessary grief, as it is not unusual for families to confuse the condition with muscular dystrophy or to presume cognitive impairment. Epidemiology the incidence of cerebral palsy has remained virtually unchanged over the past 40 years at approximately 2. This represents a great disap pointment to those who anticipated that advances in perinatal care would eliminate many cases of cerebral palsy, but a relief to those that feared a drastic increase in numbers due to improved survival of critically ill newborns. This is best understood in the context of our current understanding that the etiology of most cases of cere bral palsy is prenatal in origin. These instead point to a role for infiammatory mediators either causing damage or affecting brain development at critical periods [4]. If we are someday going to be able to reduce the incidence of cere bral palsy, the areas most amenable to intervention are those related to preventing postnatal brain injury to infants and those for reducing preterm births [5]. Clinical Features the essential clinical findings of cerebral palsy include delayed motor milestones, abnormal muscle tone, hyperrefiexia, and absence of regression or evidence of a more specific diagnosis. Community factors, such as pressure to label so services can be obtained, may also arise.

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Auscultation: fi If the tube is in the stomach, you will be able to Inject a small amount of air(10 15 ml)into hear the air enter (a whooshing sound) If the tube the nasogastric tube while you listen with a is in the esophagus, injecting the air will be stethoscope approximately 3 inches (about 8 difficult or impossible. Obtain radiograph of placement of tube(as tube is in the larynx, the client usually is unable ordered by doctor. Clamp the end of nasal-gastric tube while you fi Bending tube prevents the inducing of secretion bend the tube by fingers not to open 14. Putt off and dispose the gloves, Perform hand fi To prevent the spread of infection hygiene 16. Record the date and time, the size of the fi Documentation provides coordination of care nasal-gastric tube, the amount and color of drainage aspirated and relevant client reactions. You should also wipe acetone out after removed tapes because acetone remained on the skin may irritate. And be alert for complains of discomfort, fi Giving signature maintains professional distension, or nausea after removal. To provide alternative manner to some specific clients who has potential or acquired swallowing difficulties Equipments required: 1. Stethoscope (1) 102 Fundamental of Nursing Procedure Manual Procedure: Care Action Rationale 1. Assemble all equipments and supplies after fi Organization facilitates accurate skill checking the Dr. Check expiration fi Outdated formula may be contaminated or have date lessened nutritional value. Allow formula to reach room fi Cold formula cause abdominal discomfort or temperature before using. Perform hand hygiene and put on disposable fi To prevent the spread of infection gloves if available 5. Position the client with the head of the bed fi this position helps avoiding aspiration of feeding elevated at least 30 degree angle to 45 degree solution into lungs angle 6. Aspiration of stomach secretions fi Aspiration of gastric fluid indicates that the tube fi Attach the syringe to the end of feeding tube is correctly placed in the stomach fi Gently pull back on plunger fi Measure amount of residual fluid fi the amount of residual reflects gastric emptying time and indicates whether the feeding should continue. Injecting 10 20 mL of air into tube: fi Inject 3-5 mL of air for children fi Attach syringe filled with air to tube fi A whooshing or gurgling sound usually indicates fi Inject air while listening with stethoscope that the tube is in the stomach over left upper quadrant 103 Fundamental of Nursing Procedure Manual Care Action Rationale c.

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