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Jeffrey A Brinker, M.D.

Jeffrey A Brinker, M.D.

  • Professor of Medicine
  • Joint Appointment in Radiology and Radiological Science


The other objective of this study was to effective 100mg lasix hypertension uncontrolled icd 9 examine the accuracy of respiratory flow estimation algorithms during sleep: these researchers investigated 2 approaches for calibrating the model parameters using the known data recorded during either wakefulness or sleep buy discount lasix 100 mg on line blood pressure guidelines. The results showed that the acoustical respiratory flow estimation parameters change from wakefulness cheap 40 mg lasix otc blood pressure kits for sale. Therefore, if the model was calibrated using wakefulness data, although the estimated respiratory flow follows the relative variations of the real flow, the quantitative flow estimation error would be high during sleep. On the other hand, when the calibration parameters were extracted from tracheal sound and respiratory flow recordings during sleep, the respiratory flow estimation error is less than 10 %. A total of 18 articles with 522 patients treated with 3 glossectomy techniques (midline glossectomy, lingualplasty, and submucosal minimally invasive lingual excision) met inclusion criteria. They stated that currently, there is insufficient evidence to analyze the role of glossectomy as a stand-alone procedure for the treatment of sleep apnea, although the evidence suggests positive outcomes in select patients. Furthermore, a review on “Alternative devices for obstructive sleep apnea” (Barone, 2013) states that “The future - Next-generation respiratory-triggered implantable devices have recently been designed and have been engineered to provide intermittent electrical impulses to the hypoglossal nerve via an implanted cuff electrode. These devices monitor respiration, via implanted thoracic leads, by sensing changes in motion of the chest wall. Electrical stimulation to the hypoglossal nerve is then provided cyclically during inspiration (which represents the most vulnerable period with regard to upper airway narrowing and collapse). The hypoglossal branches that innervate the genioglossus contain mostly efferent fibers, with minimal afferent input; this allows for activation of the genioglossus with less possibility of arousal. The rate of surgical success was 45 %, and the rate of surgical response was 65 %. One patient had post-operative bleeding that required cauterization, resulting in a major complication rate of 4. The findings of this small, non-randomized study need to be validated by well-designed studies. A systematic review reported that most of the evidence related to such surgical treatments is from case series. These meta-analyses were limited by a serious risk for bias and inconsistency among the series . Only a small number of trials have directly compared surgery to either conservative management or a nonsurgical therapy. Overall, the trials have failed to consistently demonstrate a benefit from surgical therapy. While this could be a true effect, it may also reflect the small sample sizes, the heterogeneous patient populations, or the use of short-term outcome measures”. Eighty-five patients underwent surgery to reduce velopharyngeal incompetence with either a pharyngeal flap (n = 75) or a dynamic sphincteroplasty (n = 10) performed between April 1958 and August 1989, and were evaluated pre-operatively and post-operatively by a plastic surgeon, speech pathologist, and otolaryngologist. Improvement in speech was noted in 75 % (n = 56) of the patients with pharyngeal flaps and 70 % (n = 7) of the patients with dynamic sphincteroplasties post-operatively; 30 % of the patients in both groups showed no improvement post-operatively in speech; 3 patients (4 %) who underwent pharyngeal flap procedures developed sleep apnea post-operatively. The authors concluded that persistent velopharyngeal incompetence may be treated effectively with either a pharyngeal flap or a dynamic sphincteroplasty. It is difficult to create enough tension in the lateral pharyngeal walls to prevent its collapse. To the authors’ knowledge, there has not been any surgery that specifically addresses this issue. The technique basically consists of a tonsillectomy, expansion pharyngoplasty, rotation of the palatopharyngeus muscle, a partial uvulectomy, and closure of the anterior and posterior tonsillar pillars. The procedure has promising results, is anatomically sound, and has minimal complications”. Mann et al (2011) noted that velopharyngeal dysfunction has been treated with either a pharyngeal flap or sphincteroplasty with varying degrees of success. Both of these entities have their own series of problems, with sleep apnea and nasal mucous flow disruptions at the forefront. All patients who were treated with double-opposing buccal flaps between October of 1994 and July of 2007 were reviewed. These patients presented with varying degrees of velopharyngeal dysfunction showing some degree of velar movement at the time of surgery. A total of 27 patients underwent palatal lengthening, with an average length of follow-up of 58 months. Only 1 patient required the addition of a pharyngeal flap for persistent velopharyngeal dysfunction, and there were no post operative issues with sleep apnea. The authors concluded that the double opposing buccal flap is an effective technique for lengthening the palate, improving speech, and decreasing the risks of post-operative sleep apnea. All patients experienced a dramatic improvement in their resonance and intelligibility. They stated that this technique appeared most effective in patients with intact velar movement who demonstrate a small-to-moderate posterior velar gap. The double opposing buccal flap is a useful means of treating velopharyngeal dysfunction, thus serving as an adjunct when improving pharyngeal closure. Also, and UpToDate review on “Overview of obstructive sleep apnea in adults” (Strohl, 2014) does not mention sphincteroplasty as a management option. The lowest oxygen saturation and stage 1 and stage 2 were also improved significantly. The arousal threshold for stimulation exceeded the motor recruitment threshold by 0. The findings of this small study need to be validated by well-designed studies with larger sample size and follow-ups. Moreover, they stated that further studies and stimulation-system refinements are presently underway, with hopes of establishing upper airway stimulation as a therapeutic option for this challenging disorder. Continuous refinement of electrodes design is likely to improve stimulation efficacy in coming years. In part 2, patients were enrolled using selection criteria derived from the experience in part 1.


  • Fontaine Farriaux Blanckaert syndrome
  • Spellacy gibbs watts syndrome
  • Chronic fatigue syndrome
  • Diaphragmatic agenesis radial aplasia omphalocele
  • Hemangioma
  • Prosopamnesia
  • Coloboma uveal with cleft lip palate and mental retardation
  • Ghosal syndrome
  • Erdheim disease

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No previous research on social norms and cervical cancer screening behavior in female Soldiers was found in a systematic review of the literature order lasix 100mg line arteria obstruida 50. The first buy 40mg lasix hypertension of the heart, likelihood order 100mg lasix fast delivery blood pressure juicing recipes, measured a respondent‟s evaluation of the consequences of a given behavior. The second, acceptability, measured a respondent‟s evaluation of that consequence for the same given behavior. The first, recommendations, was a measurement of a respondent‟s perception of the extent to which a salient other. The second, adherence, was a measurement of the respondent‟s likelihood to complete the behavior that was recommended by the salient other. The following research hypotheses were proposed in the study: H1 [P] Adherence for cervical cancer screening in for female Soldiers is greater than U. H3 [P] Female Soldiers will report generally positive attitudes (likelihood and acceptability) towards cervical cancer screening. H5 [P] Subjective norms (in particular the healthcare provider) predict more adherent behavior with cervical cancer screening, attitude predicts non adherent cervical cancer screening behavior. A correlation matrix was completed to describe the relationship between study variables. Findings Logistic regression analysis identified no significant relationships between attitude and social norms for cervical cancer screening, including when the subscales for attitude and social norms, race/ethnicity, months deployed, rank, supervisor‟s gender, and future cervical cancer screening intention were entered into the analysis. Additionally, the paired constructs of the Attitude and Social Norms subscales did not demonstrate a significant relationship with cervical cancer screening. Discussion of Primary Hypotheses Interpretation of findings from hypothesis testing are discussed below. The first hypothesis stated that female Soldiers would have an adherence rate for cervical cancer screening greater than the Healthy People 2010 goals. Limitations to access included no appointment availability for preventive examinations such as Pap smears and/or with a provider that the respondent was uncomfortable seeing. These respondents also reported they recognized there was incongruence between the recommendations by the healthcare provider and the most current Army regulations. The finding that nearly one in five female Soldiers had not had a cervical cancer screening exam in the previous 12 months is nearly identical to the previous research by Thomson and Nielsen (2006) and Herberger (2000). The conflict between unit leadership and healthcare providers is also highlighted by Jennings, Loan, Heiner, Hemman, and Swanson (2005) in terms of military healthcare providers not fully understanding the healthcare needs for Soldiers and the role of unit leadership to determine healthcare outcomes as well. The Army regulation clearly states a requirement for annual cervical cancer screening. Yet, when instructed during a clinic visit that screening may be extended for 24 to 36 months, the Soldier is placed in a position of conflict with the chain of command. Of the 139 women who answered the question, 84% reported that they planned to complete a screening exam in the next year, 3% were neutral, and 14% reported they were not planning on a cervical cancer screening exam in the next year. During the development of this research study, the recommendation by several agencies evolved and annual requirement for cervical cancer screening was changed to every two to three years for some women. Therefore, this discrepancy in future planned behavior may be a reflection of those women who had been told by their healthcare providers that they no longer require an annual exam. However, in Herberger‟s (2000) study of a sample of mostly active duty personnel assigned to a large healthcare facility, 63% of the respondents reported future intent for a Pap in the following year in spite of an annual requirement mandated by the Army regulations. Importantly, a significant increase in future intent for cervical cancer screening among female Soldiers was demonstrated by this current study. The third hypothesis stated that female Soldiers would report generally positive attitudes (likelihood and acceptability) towards cervical cancer screening. The sample of military women reported generally positive attitudes towards the likelihood and acceptability of cervical cancer associated with a screening exam. With a range of 15 105, the mean score for the Attitude subscale, likelihood was 58; the mean score for the acceptability subscale was 72, with a range of 14-98. The scores for the paired attitude constructs with lowest summed values, indicating a perception of low likelihood and low acceptability, included that the Pap smear could lead to surgery that would change how someone would look, requires one to think about infertility, and that the Pap is inconvenient. Additionally, this study is in direct contrast to the more recent qualitative study by Ackerman et al. Among the quantitative research studies that were reviewed, Jennings-Dozier (1999) reported that generally positive attitudes towards Pap smears were significantly correlated with a stronger intention for future cervical cancer screening. The respondent comments in this study may also highlight the propensity for respondent comments to focus on comfort when describing the Pap smear. In the content analysis, the term “discomfort” or “pain” were the most frequently appearing words, recorded 13 and 7 times, respectively. However, when respondents were asked about the acceptability of Pap smear testing in terms of acceptability, unacceptable (scored as a 1), neutral (scored as a 4), and acceptable (scored as a 7), the mean score for discomfort was 5. Therefore, although discomfort is common, most respondents found the discomfort acceptable which may not have been as 172 readily identifiable in the previous qualitative work or may reflect a striking difference in acceptability for Pap smears in military women. The fourth hypothesis stated that female Soldiers would report the healthcare provider and chain of command as providing the greatest encouragement and motivation to adhere to annual cervical cancer screening exams, while the media will provide the least motivation to comply with cervical cancer screening. Most of the participants reported the healthcare provider (85%), followed by the media (60%), and other family members (55%) as the strongest salient others to encourage annual cervical cancer screening; while the least likely salient others to recommend an exam were the chain of command (47. In terms of adherence to the recommendations by the salient others, the sample reported the strongest adherence when the recommendation made by the healthcare provider (90%), other family members (65%), and the chain of command (60%); while adherence to recommendations by the media (13. When the subscales for recommendations and adherence to the recommendations were paired, the sum of the scores indicated the strongest social norms to promote annual cervical cancer screening were healthcare providers, other family members, and chain of command. Therefore, although the perception by a respondent for the salient other to recommend an annual Pap smear may be lower. The hypothesis regarding the healthcare provider and chain of command as the strongest proponent for screening was not supported as the healthcare provider and other family members were demonstrated the highest paired scores for screening recommendations and adherence.

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Therefore order cheap lasix on line arrhythmia 4279, increasing access to purchase lasix american express pulse pressure stroke and improving quality Been Screened for Cervical Cancer buy line lasix hypertension symptoms high blood pressure, Selected Developing Countries of screening programs in the high-risk age group of 35-50 years has been identified as a key component of effective programs for Year of Survey Age Total the early detection of cervical cancer in low-resource settings. The most efficient and cost-effective screening techniques in Central America low-resource countries include visual inspection using either Ecuador 2004 15-49 49. Eastern Europe/ Two vaccines that protect against about 70% of viruses that EuroAsia cause cervical cancer are the new promise for preventing cervical Albania 2002 15-44 3. Central Asian republics to North-Central China, often referred to as the ”esophageal cancer belt”, 90% of cases are squamous Treatment: Pre-invasive lesions may be treated by electrocoag cell carcinomas. Invasive cervical cancers generally 2008, with more than 80% of these deaths occurring in developing are treated by surgery, radiation, or both, as well as chemotherapy countries. Similarly, cer is one of the most successfully treated cancers: in the United temporal trends in esophageal cancer rates also vary greatly States, the five-year relative survival rate is 91%. For example, while the incidence of esophageal at late stage, the rate drops to 17%. In contrast, the incidence of adenocarcinoma of the esophagus has been increasing rapidly in Western countries, such as the United States and England,106-108 Esophagus in recent decades most likely as a result of increases in overweight/ New cases: An estimated 482,300 new cases occured in 2008 obesity, chronic gastric reflux, and the premalignant condition worldwide. The highest rates are found in Asia, including may also be related to the declining prevalence of H. Esophageal cancer is three to four Signs and symptoms: It is unusual to have signs and symptoms times more common among men than women. When esophagus usually occurs as either squamous cell carcinoma in cancer is more advanced, the most common signs are painful or the middle or upper third of the esophagus, or as adenocarcinoma difficult swallowing and weight loss. International Variation in Age-standardized Esophageal Cancer Incidence Rates among Males, 2008 Rate per 100,000 ≥ 7. International Variation in Age-standardized Esophageal Cancer Incidence Rates among Females, 2008 Rate per 100,000 ≥ 7. A diet lacking Deaths: Bladder cancer is the 13th leading cause of cancer death fresh fruits and vegetables also increases risk. An estimated 150,200 deaths low fruit and vegetable consumption are also risk factors for from bladder cancer occured in 2008. The highest mortality adenocarcinoma of the esophagus; however, the main risk among men was in Egypt, where the estimated death rate (16. In high-risk areas such as Golestan bladder cancer trends, mortality patterns are easier to interpret (Iran) and Linxan (China), contributing risk factors are not well than incidence patterns because they are not affected by understood, but are thought to include poor nutritional status, differences in reporting of low-grade tumors. In the United low intake of fruits and vegetables, and drinking beverages at States, mortality rates have stabilized in males and decreased in high temperatures. The stable and/or decreasing physically active are the best ways to reduce the incidence of bladder cancer mortality trends among males are due in part to esophageal cancer. In addition, a healthy diet, especially one rich reductions in smoking prevalence in Western countries along in fruits and vegetables may lower one’s risk. Treating gastric with reductions in occupational exposures known to cause reflux with proton pump inhibitor drugs (Prilosec, Prevacid, bladder cancer. Bladder cancer continues to be the most common Nexium) or through surgery may be able to prevent Barrett’s cancer among males in Egypt despite recent declines in bladder esophagus and esophageal cancer. Further risk factor studies are cancer incidence as the result of the reductions in schistosomal necessary to elucidate primary prevention measures in high-risk infections, the primary cause of bladder cancer in Egypt. Schistosoma (parasite) control is being offset by tobacco-related Treatment: Options for treatment include surgery, chemotherapy, bladder cancer. Palliative treatment may also be used to Signs and symptoms: the most common symptom is blood in relieve symptoms, such as pain and trouble swallowing, but is the urine. Other symptoms may include increased frequency or not expected to cure the cancer. Survival: Because esophageal cancer is usually diagnosed at a Risk factors: Smoking is the most important risk factor for late stage, most people with esophageal cancer eventually die bladder cancer. The risk increases with increasing 11% of African American patients survive at least five years after 45 duration of smoking. In Europe, the average five-year relative survival 53 of bladder cancer deaths among men and 13% among women rate is 11%. Workers in the dye, rubber, or leather industries and people who live in communities with high levels of arsenic Urinary Bladder in the drinking water also have increased risk for bladder cancer. New cases: An estimated 386,300 new cases of bladder cancer Eating more fruits and vegetables and possibly drinking more occured in 2008, making it the ninth most common cause of fluids may lower the risk of bladder cancer. The majority of bladder cancer occurs in men, world, particularly Africa and the Middle East, chronic infection and there is a 15-fold variation in incidence rates internationally. Schistosomiasis, which is transmitted through are found in Southeast Asia and Middle Africa (Figure 16a and contaminated water, is responsible for an estimated 50% of 16b). Some of the differences in incidence among countries are bladder cancer cases in some parts of Africa and about 3% of cases 30 Global Cancer Facts & Figures 2nd Edition Figure 16a. International Variation in Age-standardized Urinary Bladder Cancer Incidence Rates among Males, 2008 Rate per 100,000 ≥ 10. International Variation in Age-standardized Urinary Bladder Cancer Incidence Rates among Females, 2008 Rate per 100,000 ≥ 10. Signs and symptoms: Symptoms may include swollen lymph Treatment: Surgery, alone or in combination with other treat nodes, itching, night sweats, fatigue, unexplained weight loss, ments, is used in more than 90% of cases in the United States. Superficial, localized cancers may also be treated by administering immunotherapy or chemotherapy directly into the bladder. Risk factors: In most cases, the cause is unknown, although Chemotherapy alone or with radiation before cystectomy (bladder various risk factors associated with altered immune function removal) has improved treatment results.

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To facilitate a single bolus swallow proven lasix 40mg hypertension jnc8, the entire bowl of a dry spoon can be placed on the tongue applying downward pressure to cheap lasix generic heart attack causes encourage the tongue to discount lasix amex blood pressure 6020 make a bowl then move back to initiate a swallow. If the child does not close his lips around the spoon, he is asked to and/or his lips are closed around the spoon to learn the correct sequence (Chigira, Omoto, Mukai, & Kaneko, 1994). All presentations of the spoon should be done with the child’s head in a neutral position and with him facing forward. Since there is no food on the spoon and no threat of aspiration, he is learning the correct sequence of movement for a single bolus swallow in a safe manner using the utensil that is used for feeding. As he becomes more proficient with this pattern, a small amount of food is placed on the spoon. The amount of food on the spoon is increased gradually until he can accept a level spoonful of food while always ensuring that he is continuing to exhibit the appropriate oral-motor pattern. Some children have difficulty grading the amount of mouth opening and closing needed for a variety of foods. When a child demonstrates wide jaw excursions, he will have difficulty using his tongue to manage the food. Providing jaw stability by placing your hand under the jaw will help the child to take bites and to keep the mouth closed while the tongue does the work of manipulating the food. As the child practices, thinner foods can be placed between the molars to chew using minimal jaw excursions. Empirical Support for Oral Stimulation and Oral-Motor Therapy Oral-Motor Therapy While the majority of published studies report on the efficacy of oral-motor therapy for high-risk infants born prematurely or children with neuromotor difficulties, a few case studies suggest the promising use of oral-motor treatment techniques for children with low muscle tone (Kumin, Von Hagel, & Bahr, 2001) and retardation/autism (Larrington, 1987). Using a two-group design, Gaebler and Hanzlik (1996) compared 5 minutes of pre-feeding stroking to 5 minutes of pre-feeding stroking and introral prefeeding stimulation in a sample of 18 premature infants. Assessments occurred on days 1, 3 and 5; the group that received oral stimulation fed significantly better than the control group on day 3 only. In addition, infants in the experimental group demonstrated greater overall intake, rate of milk transfer, and amplitude of the expression component of sucking. In contrast, there were no differences between experimental and control infants on sucking stage maturation, sucking frequency, amplitude of the suction component of sucking or endurance of sucking. An elegant series of studies investigating sensorimotor intervention on eating skills of children with cerebral palsy (average age ~ 5 years) has been conducted by Gisel and colleagues (Gisel, 1994; Gisel, Applegate-Ferrante, Benson, & Bosma, 1995, 1996). Oral-motor therapy, which was designed to increase tongue lateralization, lip control, and vigor of chewing, was delivered before the lunch meal for 5-7 minutes/day, 5 days/week for 10-20 weeks. Using a between subject experimental design, children’s spoon feeding, biting, and chewing improved with oral-motor therapy (Gisel, 1994). Using a within-subject design, spoon feeding, chewing, and swallowing improved after oral-motor therapy (Gisel et al. In addition, more children advanced food texture with oral motor therapy (Gisel et al. These functional changes in feeding skills appear to be a function of jaw stabilization. Behavioral Interventions Targeting Oral-Motor Problems Within the field of applied behavior analysis, several researchers have designed interventions using behavioral principles to improve specific oral-motor problems, such as swallowing dysfunction (Hagopian, Farrell, & Amari, 1996; Hoch, Babbitt, Coe, Duncan, & Trusty, 1995; Lamm & Greer, 1988), cup drinking (Patel, Piazza, Kelly, Ochsner, & Santana, 2001), and failure to advance texture (Luiselli & Gleason, 1987; Shore, Babbitt, Williams, Coe, & Snyder, 1998). The hallmark of all these interventions is 1) analyzing the “task” into small, discrete steps, 2) manipulating the antecedent condition by presenting food or liquid in a way that allows the child to complete the behavior expected successfully, and 3) clear consequences for completing or not completing the task. For example, in the swallowing induction procedures, swallowing was broken down into its component steps from food entering the mouth to the initiation of the swallowing reflex at the faucal arches. The researchers used forward or backward chaining with prompts (verbal and physical prompts on the tongue) to initiate swallowing. Although the data suggest that the intervention is promising (Kerwin, 1999), the technique assumes competent oral-motor tongue and jaw movements. Although the children had increased swallow frequency, increased food acceptance and increased quantity of food consumed orally, it is unclear from the study results if the oral-motor pattern acquired indirectly would allow the child to continue advancing oral-motor skills with other food textures. The current research literature suggests that a more expedient intervention might be direct targeting of the oral-motor movements. The behavior analyst can be instrumental in assisting members of other disciplines in teaching children these skills. Because behavior analysts are skilled in task analyses, they can also help members of the other disciplines task analyze their interventions and learn to look for and document specific responses that they expect from each procedure (see Kumin et al. Perhaps an ideal avenue for future investigation is combining the best of both approaches together to maximize the child’s benefit from systematic, gradual steps with clear instruction and consequences in the context of meaningful oral motor facilitation and therapy (Bailey & Angell, 2005). Peterson and Ottenbacher (1986) demonstrated this combined approach in teaching lip closure to three children with mental retardation. Conclusion Children requiring early, intensive intervention often have complex feeding and swallowing issues that requires a team approach in order to fully evaluate and treat the underlying issues. Low tone, abnormal sensory processing, altered postural alignment and movement patterns are often present in these populations. These issues place them at risk for having abnormal postural alignment of the pelvis, trunk, shoulder girdle, which leads to a forward head posture. This alters the biomechanics of breathing and swallowing and the corresponding sensory input. With altered sensory perception the child may have difficulty motor planning postural alignment and control for acquisition and mastery of feeding and swallowing behaviors. Once medical stability and postural alignment have been obtained, oral stimulation can be an effective adjunct to feeding treatment.

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It should be noted that a “second wind” is unique to buy lasix 100 mg on line arrhythmia headaches McArdle disease (Lucia et al buy discount lasix 100 mg online sinus arrhythmia. However cheap lasix online pulse pressure points body, many McArdle people do not know how to get into a 18 second wind or do not realise that this is occurring unless guided through it by a family doctor or specialist (Quinlivan and Vissing, 2007). Less common symptoms of McArdle disease (seen only in some McArdle people): Some McArdle people have “fixed proximal weakness”. Some of the more severe symptoms which can lead to diagnosis of McArdle disease: Kidney (renal) failure due to rhabdomyolysis and myoglobinuria can lead to hospital investigations which result in a diagnosis of McArdle’s (Biller, 2007). Muscle pain (myalgia), inflammation (myositis) and damage caused by statins (drugs taken to lower cholesterol) can sometimes lead to hospital investigations which result in a diagnosis of McArdle disease (Biller, 2007). A brief description of each, along with the pros and cons, and limitations is given in Table 2. An indication of how commonly I believe each method is used to diagnose McArdle’s is also given. No Use of this test was first described by Dr Brian McArdle and forearm exercise test has been in use for about 50 years. An overview of my opinion of how commonly each method is used, whether it produces a definitive diagnosis, and relevant notes. All three are intended to test whether the body is able to break down glycogen to produce glucose in order to provide the muscles with energy during exercise. What is tested: When a muscle of an unaffected person is exercised vigorously (anaerobic exercise), the free glucose is rapidly used up. Stored glycogen is then broken down by the process of glycogenolysis to produce energy. In people unaffected by McArdle’s, the amount of lactate and pyruvate should increase 5-6 fold (Dubowitz et al. In McArdle people, the absence of functional muscle glycogen phosphorylase enzyme blocks glycogenolysis. McArdle people therefore do not have the expected increase in lactate and pyruvate levels. However, recent studies have shown that similar results with less risk of muscle damage can be achieved with a non-ischaemic forearm test (Niepel, 2004). Cons of all exercise tests: the level of effort must be below the maximum so that severe complications like rhabdomyolysis and myoglobinuria do not occur (Fernandes, 2006). Following exercise, increased ammonia levels, increased uric acid levels (see section 13. An absence of increase in lactate and pyruvate levels indicates a metabolic disease caused by a block in glycogenolysis. Many other glycogen storage diseases prevent lactate production after anaerobic exercise (Lane, 1996). The exercise test does not distinguish whether the person has McArdle disease or another other metabolic disease, for example, another glycogen storage disease such as Tauri disease (phosphofructokinase deficiency) (Abramsky, 2001). Cori disease and Tauri disease can produce flat (not increasing) lactate levels after the forearm test (Biller, 2007). For this reason, the level of 21 ammonia in the blood (plasma ammonia) is usually measured before and after an ischaemic forearm test (Lane, 1996). The forearm is contracted by squeezing a ball or balloon, or the thigh is contracted at maximum force/strength for one minute or until extreme pain. The blood is analysed to determine whether the expected increase in lactate and pyruvate occurs. After exercise, the amount of lactate in the blood will not increase (Cush, 2005) in McArdle people, but McArdle people will have an increase in ammonia levels in the blood, which can go up to 360-560µg/dl (Lane, 1996). It is important that ammonia levels in the blood rise, as this shows that the person has exercised enough, as an incorrect result could be obtained if the person who is being tested does not exercise with enough effort (Lane, 1996). Cramping, muscle pain and contracture of the muscle may occur following the test (Cush, 2005) There is a small risk of the severe problem of compartment syndrome (discussed further in section 12. The risk of compartment syndrome is much lower if the non-ischaemic forearm test is performed. There is also a risk of the test causing severe muscle damage which could lead to kidney failure (see section 5 for further information on rhabdomyolysis and kidney failure). The muscle of the tested forearm was damaged, which resulted in myoglobinuria and raised creatine kinase levels in the blood. The person was placed under medical observation and instructed to drink plenty of fluids. How the non-ischaemic forearm test is carried out: A non-ischaemic forearm test (similar to that described above but without use of a cuff) is now recommended. The non-ischaemic forearm test is much less likely to cause damage (Niepel, 2004). The ischaemic forearm test can cause a lot of pain and discomfort for McArdle people, whereas the non-ischaemic test produces “almost no discomfort” (Abramsky, 2001). Cons of the non-ischaemic forearm test: I think that it seems possible that muscle damage could also be a side effect of the non-ischaemic forearm test if the person exercises too vigorously (as described by Meinck et al. Pros of both the ischaemic and non-ischaemic forearm exercise tests: It is not very invasive (the only invasive part is taking blood samples). It may produce a positive result in people with other similar diseases which affect glycogenolysis or glycolysis (like some of the other glycogen storage disease). It was suggested by Lane (1996) that false negative results could be seen in the rare cases of McArdle people with low levels of phosphorylase activity, but no experimental data was provided to support this theory. If both lactate and ammonia increase only a small amount, it suggests that either the person being tested did not put enough effort into the exercise or that the wrong vein was used to sample the blood the correct vein to use is called the “median cubital vein”, and one example of an incorrect vein to use is the basilica vein (Abramsky, 2001).


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