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By Q. Gelford. New York Institute of Technology. 2018.

She developed scoliosis during her early teenage years buy 100mg zenegra with amex. She denies having knowledge of any previous complications from her condition order zenegra 100 mg online. She asks you to explain her condition and its possible complications discount zenegra 100mg free shipping. Which of the following statements regarding kyphoscoliosis is true? The two distinct forms of costovertebral skeletal abnormalities— scoliosis and kyphosis—do not typically occur together in a given patient B cheap 100mg zenegra otc. Approximately 80% of cases of kyphoscoliosis are idiopathic C. Idiopathic kyphoscoliosis is most commonly a congenital abnormal- ity or an abnormality that develops in the aged population D. The incidence of kyphoscoliosis is distributed equally between the sexes Key Concept/Objective: To know the features of idiopathic kyphoscoliosis Kyphoscoliosis is an illness that can be associated with mild to severe respiratory com- promise. The two basic types of costovertebral skeletal deformity—scoliosis, a lateral curvature with rotation of the vertebral column, and kyphosis, an anterior flexion of the spine—are usually found in combination. Approximately 80% of cases of kyphosco- liosis are idiopathic. Idiopathic kyphoscoliosis commonly begins in late childhood or early adolescence and may progress in severity during these years of rapid skeletal growth. Idiopathic kyphoscoliosis is not to be confused with kyphoscoliosis caused by a known underlying condition, such as osteoporosis or compression fractures in elder- ly patients. The incidence of kyphoscoliosis in females is four times higher than that in males. A 37-year-old man arrives at your emergency center by ambulance shortly after being involved in a motor vehicle accident. The emergency medical technician (EMT) reports that the patient is hemody- namically stable with minimal external blood loss and no loss of consciousness. The EMT reports that the patient appears to be in moderate to severe respiratory distress; the patient has a respiratory rate of 40 breaths/min and an O2 saturation of 78% while receiving supplemental oxygen at a rate of 3 L/min by nasal cannula. On physical examination, you note a remarkable 15 cm right anterolateral chest con- tusion. The contused segment appears to move paradoxically with respect to respiration. The patient has clear bilateral breath sounds in the upper and lower regions of both lungs. Which of the following statements regarding flail chest injury is most accurate for this patient? In young, otherwise healthy patients, a large flail chest segment is not a life-threatening injury B. The most appropriate step to take next in treatment of this patient is to provide supplemental oxygen by 100% nonrebreathing mask to attain O2 saturations greater than 90% C. The most appropriate step to take next in the treatment of this patient is to provide positive pressure ventilation D. The most appropriate step to take next in the treatment of this patient is to order and evaluate a stat portable chest x-ray to rule out a tension pneumothorax Key Concept/Objective: To understand emergent therapy of flail chest segment with respiratory failure Flail chest is an acute process that may lead to life-threatening abnormalities of gas exchange and mechanical function. This patient is in acute respiratory failure as a result of the massive chest-wall trauma and resultant flail segment. Stability of the thoracic cage is necessary for the muscles of inspiration to inflate the lung. In flail chest, a local- ly compliant portion of the chest wall moves inward as the remainder of the thoracic 24 BOARD REVIEW cage expands during inhalation; the same portion then moves outward during exhala- tion. Consequently, tidal volume is diminished because the region of lung associated with the chest wall abnormality paradoxically increases its volume during exhalation and deflates during inhalation. The result is progressive hypoxemia and hypercapnia. Multiple rib fractures, particularly when they occur in a parallel vertical orientation, can produce a flail chest. The degree of dysfunction is directly proportional to the volume of lung involved in paradoxical motion.

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Although vertical transmission occurs buy zenegra 100 mg cheap, sexual contact remains the primary mode of transmission of the disease B buy cheap zenegra 100 mg online. In the United States cheap zenegra 100 mg visa, transmissible syphilis is primarily concentrated in a few geographic regions in the Southeast C discount zenegra 100mg mastercard. The resurgence of new cases of syphilis in the late 1980s has been linked to the epidemic use of crack cocaine and the exchange of sex for drugs D. White and minority populations are affected with equal frequency E. Incidence rates are higher in inner-city populations than in rural ones Key Concept/Objective: To understand the epidemiology of syphilis Disease caused by the spirochete Treponema pallidum has been recognized for centuries. With advances in drug therapy and diagnostic methods, the control and, possibly, the eradication of syphilis in the United States have become realistic goals. Syphilis is transmitted through sexual contact primarily, with few cases associated with nonsexual exposure. The highest rates of infection occur in inner-city populations with lower socioeconomic status and are largely confined in the 22 BOARD REVIEW southeastern United States. Minorities are affected to a much higher degree than whites (25:1 in some studies). The high rates of new syphilis cases among inner-city popula- tions in the late 1980s and early 1990s has been linked strongly to the epidemic use of crack cocaine. Which of the following findings would NOT be consistent with the secondary stage of syphilis? Diffuse, painless lymphadenopathy and patchy alopecia B. A hyperpigmented maculopapular rash involving the trunk, extrem- ities, palms, and soles C. Signs and symptoms of meningitis (fever, stiff neck, photophobia) accompanied by abnormalities of the cerebrospinal fluid D. A single indurated and nontender ulcerative genital lesion accompa- nied by nonsuppurative regional lymphadenopathy E. Raised, moist, nontender plaques in intertriginous areas and on mucosal surfaces, the swabbing of which reveals spirochetes on darkfield microscopy Key Concept/Objective: To know the distinguishing features of secondary syphilis Once T. The characteristic lesion of primary syphilis is the chancre, an indurated, painless ulcer that can be up to 1 to 2 cm in size. Without treatment, the chancre typically resolves in 2 to 8 weeks; in a majority of cases, the chancre is not present by the time signs and symptoms of dissemination (secondary syphilis) develop. The clinical findings of secondary syphilis are varied but often include fever, malaise, diffuse lymphadenopathy, patchy alopecia, and a charac- teristic maculopapular rash, which involves the palms and soles. Condylomata lata, which are moist, indurated plaques (not truly ulcers) that occur primarily in intertrigi- nous areas, are typically seen in patients with secondary syphilis. They are teeming with organisms and are highly infectious. Although symptomatic parenchymal neu- rosyphilis is commonly associated with late-stage (tertiary) syphilis, up to 40% of patients with secondary disease have involvement of the CNS, manifested clinically as meningitis. A positive CSF–Venereal Disease Research Laboratory (CSF-VDRL) test result confirms neurosyphilis in this setting, but the sensitivity ranges only from 30% to 70%. A 32-year-old man presents to the health department to establish primary care. He has not seen a physi- cian since childhood and reports no chronic medical problems. On review of systems, he relates that approximately 1 year ago he developed an illness consisting of fever, "swollen glands," and a diffuse rash, which involved the palms. The illness resolved after a few weeks, and he did not seek medical care. Over the past 2 years, he has had several sexual partners, and he states he has not routinely used condoms. For this patient, which of the following findings would be most consistent with latent syphilis infection? Diffuse, painless lymphadenopathy and a faint, widespread macular rash B.

Electrophysiology: Nerve conduction studies are usually normal (except reduced CMAP in affected atrophic muscles) buy zenegra 100 mg with amex. EMG shows increased insertional activity only in affected muscles buy 100 mg zenegra fast delivery. Short duration polyphasic motor unit action potentials buy 100mg zenegra fast delivery, mixed with normal and long duration units are seen in the affected muscle/s buy zenegra 100mg with mastercard. Imaging: Focal enlargement, edema, and fatty infiltration especially observed on T2 weighted and T1 images with gadolinium. Muscle biopsy: Characterized by endomysial fibrosis (Fig. Genetic testing: Exonic or multiexonic deletions (60–65%), duplication (5–10%), or missense mutations that generate stop codons may be found. Spinal fusion may be required where there is respiratory compromise: according to Hart and McDonald, fusion should be used before the curvature is greater than 30 and vital capacity is less than° 35% of predicted. Carriers should also be checked for cardiac defects. Myoblast, DNA, and stem cell transfer are potential therapies. Prognosis Patients usually survive to their mid-twenties. References Cohn RD, Campell KP (2000) Molecular basis of muscular dystrophies. Muscle Nerve 23: 1456–1471 Fenichel GM, Griggs RC, Kissel J, et al (2001) A randomized efficacy and safety trial of oxandrolone in the treatment of Duchenne dystrophy. Neurology 56: 1075–1079 Grady RM, Zhou H, Cunningham JM, et al (2000) Maturation and maintenance of the neuromuscular synapse: genetic evidence of for the roles of the dystrophin-glycoprotein complex. Neuron 25: 279–293 Hart DA, McDonald CM (1998) Spinal deformity in progressive neuromuscular disease. Phys Med Rehab Clin N America 9: 213–232 Jacobsen C, Cote PD, Rossi SG, et al (2001) The dystrophoglycan complex is necessary for stabilization of acetylcholine receptor clusters at neuromuscular junctions and formation of the synaptic basement membrane. J Cell Biol 152: 435–450 Mirabella M, Servidei S, Manfredi G, et al (1993) Cardiomyopathy may be the only clinical manifestation in female carriers of Duchenne muscular dystrophy. Neurology 43: 2342– 2345 383 Becker muscular dystrophy (BMD) Genetic testing NCV/EMG Laboratory Imaging Biopsy +++ ++ – + +++ BMD affects proximal greater than distal muscles. Worse in the quadriceps and Distribution hamstrings. BMD is a progressive disorder with a slower rate of progression than DMD. Time course BMD is much milder than DMD with later clinical onset. Patients may have Onset/age difficulty walking by their late teens. Weakness is present in Clinical syndrome approximately 20% of affected patients. In general the severity and onset age correlate with muscle dystrophin levels. As with DMD, affected subjects may have calf muscle hypertrophy and contrac- tures in the lower extremities. Patients with BMD often have a severe cardio- myopathy as part of the muscle weakness syndrome, or may have an isolated dilated cardiomyopathy. In general the average IQ of affected children is re- duced compared to the general population and may be a major presenting symptom in BMD. Some patients may present with an atypical neuromuscular disorder mimicking SMA, a focal myopathy, or a limb girdle muscular dystrophy. Most are exonic or multiexonic (70–80%), although duplications can occur in Pathogenesis 10%, and missense mutations in < 10%. Although dystrophoglycan is reduced in BMD, the molecular abnormality is unknown although it is likely similar to DMD.

Courtesy Churchill-Livingstone (Saunders) Press Figure 5 buy 100 mg zenegra otc. The lower field shows primarily collagen (yellow) with a few muscle fibers (red) purchase 100mg zenegra amex. Courtesy Churchill-Livingstone (Saunders) Press ©2002 CRC Press LLC Figure 5 order zenegra 100mg on line. Courtesy Churchill-Livingstone (Saunders) Press Figure 5 generic zenegra 100 mg with amex. In the lower field, there are almost normal muscle fibers with visible mitochondria. Courtesy Churchill- Livingstone (Saunders) Press ©2002 CRC Press LLC Figure 5. There is degeneration of muscle with a few transverse Z-lines in a sea of debris. Courtesy Churchill-Livingstone (Saunders) Press Figure 5. The regeneration process can be seen in the development of new Z-lines. Courtesy Churchill-Livingstone (Saunders) Press ©2002 CRC Press LLC Figure 5. There are two new vertical Z-lines and a few transverse muscle filaments. Courtesy Churchill-Livingstone (Saunders) Press Figure 5. Courtesy Churchill-Livingstone (Saunders) Press ©2002 CRC Press LLC muscle fiber and the amount of actin and myocin BIBLIOGRAPHY increases. The Z-lines begin to re-form and the nuclei migrate to the periphery of the fiber. Alterations during immobilization and regen- On CT and MR imaging of the spine, it is possi- eration of skeletal muscle. J Bone Joint Surg 1972;54A:919 ble to visualize these changes within the posterior musculature. With immobilization, the posterior Kirkaldy-Willis WH, McIvor GWD. Philadelphia: which increases with prolonged periods of inactivity. JB Lippincott 1976;115:114 ©2002 CRC Press LLC 6 Spinal deformity Traumatic, congenital and degenerative changes can Figure 6. Many of these abnormalities are of no clinical consequence, but under certain circumstances can predispose a patient to increasing pain. Other deformities such as scoliosis can result in cosmetic and functional diffi- culties. SPONDYLOLYSIS The vertebral arch attaches to the vertebral body through the pedicles. The laminae originate from the pedicle at a comparatively weak area known as the pars interarticularis or isthmus. In childhood and adolescence, this area is subject to fatigue fracture, which may not heal properly and can lead to a fibrous union rather than a stable bony union. If it occurs a b Oblique radiographic view of the lumbar spine with a spondylolysis at L3 (arrow) (a). Axial CT demonstrates the neural arch defect in the pars interarticularis (b) ©2002 CRC Press LLC Figure 6. The L3–L4, L4–L5 spondylolisthesis at L5–S1 (upper arrow).

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