By H. Ben. University of Advancing Technology. 2018.
Legrand B generic 0.5mg dostinex mastercard, Filipe G buy dostinex 0.5mg otc, Blamoutier A buy 0.5mg dostinex visa, Khouri N purchase dostinex 0.5mg on line, Mary P (2003) Péné- on functional ability, ambulation and perceived competence. Vitale M, Guha A, Skaggs D (2002) Orthopaedic manifestations Mot 89:57–61 of neurofibromatosis in children: an update. Lipton GE, Guille JT, Kumar SJ (2002) Surgical treatment of sco- 107–18 liosis in Marfan syndrome: guidelines for a successful outcome. Widmann R, Bitan F, Laplaza F, Burke S, Di Maio M, Schneider R J Pediatr Orthop 22:302–7 (1999) Spinal deformity, pulmonary compromise, and quality of 22. McMaster MJ (1994) Spinal deformity in Ehlers-Danlos syn- life in osteogenesis imperfecta. Wynne-Davies R, Gormley J (1985) The prevalence of skeletal (Br) 76: 773–7 dysplasias. Occurrence A Canadian study investigated 3,200 patients, including 174 who were still in the growing phase (5. While children are less likely to suffer a spinal injury than adults, when a child does sustain such an in- jury, the risk of an associated neurological lesion is much higher than for adults. The incidence of spinal cord injury is around 30– 40/1,000,000 inhabitants [9, 22]. Another study on cervical spine injuries found that these occurred less frequently in children under 11 than in adults, but were associated with a high mortality. The incidence of cervical spine injuries in over-11-year olds matches that in adults and was cited as 74/1,000,000 of the popula- tion/year. Distribution of fracture levels in children and adolescents (after). Fractures at the thoracic level are commonest in this age Etiology group, but rarely occur at this site in adults Traffic accidents and falls from a great height are the predominant causes of injury in children under 10 years [7, 16, 21]. In adolescents, on the other hand, sporting accidents are the commonest cause. In our own investiga- to the fact that the thorax is much more elastic in children tion, the sporting activity that resulted in (severe) and adolescents than in adults. A second frequency peak spinal injuries was skiing in 33% of cases, swimming for the pediatric age group was observed for the thoraco- in 13%, horse riding and gymnastics both in 12% of cases, lumbar junction, where most of the adult fractures also mountaineering in 8%, paragliding in 4% and diving in occur. An increased frequency of accidents has Classification also been reported for trampolining. The risk of spinal A special feature of pediatric spinal trauma is traumatic injuries during skiing is higher in adolescence than either paraplegia without any detectable changes on the x-ray before or after this period. By contrast, the currently (known as SCIWORA syndrome, which stands for spinal popular youth-oriented sport of snowboarding does not cord injury without radiographic abnormality). Such appear to involve an increased risk of spinal injuries (in injuries are not included in the usual classifications since contrast with injuries to the upper extremities) as the they do not produce any radiographically visible lesion. The injuries with radiographically visible frac- Localization tures can be classified as for adult fractures. The principal sites of injury in adults are the lower cervi- cal spine and the thoracolumbar junction (T11–L3). In general, lesions of the lumbar spine are more common To this end we use the AO classification, in which the than cervical injuries. With the exception of vertebral fractures are subdivided according to the mechanism bodies T11 and T12, fractures of the thoracic section are of injury: extremely rare. By contrast, in our own study with A: Compression 51 children and adolescents with 113 fractures we found B: Distraction that the thoracic spine was actually the most frequently C: Torsion affected site of injury (⊡ Fig. AO classification of spinal trauma one week can prove helpful in uncertain cases. In particu- Type Features lar, the presence or absence of any instability can then be established with a (careful) functional x-ray in inclination A: Compression and reclination. A 1: Impaction Radiographs of the thoracic and lumbar spine are eas- 3 ier to evaluate than those of the cervical spine. Compres- A 2: Split fracture sion fractures can be differentiated from wedge vertebrae A 3: Burst fracture in Scheuermann disease since the endplate of compressed B: Distraction vertebral bodies tends to overlap the anterior edge slightly.
The increased curva- Maissoneuve fracture: Fracture of the proximal one- ture is most often associated with arthrosis of the third of the fibula associated with rupture of the acromion generic 0.25 mg dostinex with visa. Today it is used to classify of the ulna with anterior dislocation of the radial chondrosis thoughout the knee and other joints order 0.5 mg dostinex free shipping, as head cheap 0.25mg dostinex mastercard. This classification is often used incorrectly dostinex 0.5mg lowest price, and Nightstick fracture: Fracture of the ulnar shaft a new classification system is needed. Until then, the Piedmont fracture: Isolated, closed fracture of the author recommends the lesions be described on the radius at the junction of the middle or distal-thirds basis of anatomic location, dimension of lesions, and Rolando’s fracture: Y-shaped intra-articular fracture depth of penetration in order to avoid any misunder- at the base of the thumb metacarpal standings in communication. MODIFIED OUTERBRIDGE CLASSIFICATION Torus fracture (“buckle” fracture): Incomplete frac- ture with buckling of one cortex predominantly in Grade I: Cartilage softening and swelling skeletally immature patients. It usually occurs with a Grade II: Fragmentation and fissuring <1 cm in dia- fall on an outstretched hand and is a stable fracture. Sunderland S: A classification of peripheral nerve injuries pro- ducing loss of function. NERVE INJURIES (SEDDON CLASSIFICATION) Tria AJ, Klein KS: An Illustrated Guide to the Knee. The sarcomere, an area from Z-disk to Z-disk and the functional contraction unit 1st Degree: Minimal damage to the muscle, tendon, of a myofibril, contains two types of myofilaments: or musculotendinous unit thick (myosin) and thin (actin) myofilaments, which 2nd Degree: Partial tear to the muscle, tendon, or are repeated throughout the muscle myofibril. Transverse tubules (T-tubules: see Excitation-Contraction Coupling), found at the A–I junction of the sarcomere, and the sarcoplasmic reticulum (SR) are the primary regula- BIBLIOGRAPHY tors of calcium influx into muscle units. MUSCLE PROTEINS Bigliani LU, Morrison D, April EW: The morphology of the Key skeletal muscle proteins include actin, myosin, acromion and its relationship to rotator cuff tears. JBJS(B) found in the thin filaments of muscle fibers, where it 30(4):664–670, 1948. The electrical impulse travels Two specific motor neurons for initiating and regulat- quickly into the interior of the muscle cell, down the ing muscle contraction are the alpha and gamma neu- T-tubules with release of calcium from the SR to the rons. Muscle fibers EXCITATION-CONTRACTION COUPLING achieve gradation of contraction strength by recruiting (EC COUPLING) fewer or more motor neurons to initiate contraction M uscle contraction is triggered by an electrical and/or by changing the frequency of action potentials to impulse involving acetylcholine release, arrival at the sustain contractions. Former Type IIb human muscle fibers are currently referred to as Type IId(x) because of MHC types found in human muscle. Type IIb muscle fibers and Type IIb MHC are found in rodents and other species (Pette and Staron, 2001; Staron, 1997; Zhen-He et al, 2000). SENSORY RECEPTORS Recent advances in fiber typing have brought about a Muscle spindles: These specialized intrafusal muscle change in nomenclature for human muscle fiber types: fibers are located between and among extrafusal Type IIb is now referred to as Type IId(x) due to the fibers deep within the interior of the muscle. As pro- expression of the myosin heavy chain (MHC) form prioceptors, they sense and relay the length or veloc- IId(x). MHC IIb is expressed in rodents and other ity of a muscle’s movement (e. Golgi tendon organs (GTO) act to protect against injury due to overloading. ENERGY METABOLISM ENERGY SYSTEMS SKELETAL MUSCLE FIBER TYPES Energy for muscular activity can be derived from Skeletal muscle is extremely dynamic, with various three specific systems (Table 8-2, Fig. The fiber types characterized by differences in morphol- immediate, or phosphagen, system consists of adeno- ogy, histochemistry, enzyme activity, surface charac- sine triphosphate (ATP) and creatine phosphate (PC teristics, and functional capacity (Pette and Staron, or phosphocreatine); it allows for very short bursts of 2001; Staron, 1997). The energy formed allows for only nalling, training/functional demands, and aging (Pette 1–1. Muscle fiber type appears to power output as compared to the phosphagen system. Immediate-, Short-Term, and Long-Term Energy Systems Amino acids can also enter the TCA cycle after deami- MOLE OF TIME TO nation/transamination and conversion into forms such as ENERGY SYSTEMS ATP/MIN FATIGUE acetyl CoA and/or acetoacetate. The reduced coen- Immediate: Phosphagen 4 5 to 10 s zymes, NADH and FADH2, formed during glycolysis (Phosphocreatine and ATP) and the TCA cycle, are shuttled to the electron transport Short-term: Glycogen-lactic 2. There they are oxidized acid (Glycolytic) Long-term: Aerobic 1 Unlimited time to NAD and FAD, respectively, and ATP is regenerated from ADP with 2. Other organic compounds can also enter the One molecule of glucose going through the glycolytic TCA cycle. Free fatty acids (FFA) from triglycerides pathway to lactate yields 2 ATP, whereas one mole- are broken down into two-carbon acetyl fragments and cule of glucose going through the aerobic pathway joined with CoA to form acetyl CoA. SM: Sarcolemma, Glc: Glucose; FA: Free Fatty Acid; OMM: Outer mitochondrial membrane; IMM: Inner Mitochondrial Membrane.
The muscle biopsy fiber type disproportion discount 0.25mg dostinex with mastercard, shows a variable picture with focal fiber atrophy and other forms generic dostinex 0.25mg otc. Whereas the De Lange myopathy is a malignant condi- The course and prognosis differ discount dostinex 0.25mg with amex, varying from a very slow tion buy discount dostinex 0.25 mg line, with death often occurring even during infancy, progression and a normal life expectancy to a loss of walk- the Batten-Turner type is benign, often with slow or ab- 4 ing ability in early adulthood. The aim of treatment is to sent progression, even though the overall clinical picture preserve mobility in the upper extremities. As Under histological examination, the cell nuclei in central- the dystrophy progresses, the muscle power is no longer core myopathy are located centrally in the muscle fibers. Corresponding clinical skeletal deformities (flatfoot, clubfoot: 21%, hip disloca- and radiological follow-up is indicated. Emery-Dreifuss muscular dystrophy The manifestation of nemaline myopathy (recessive or This X-linked recessive form of muscular dystrophy pri- dominant) is highly variable: some patients die during the marily affects older children, adolescents and adults. The neonatal period, whereas others are able to walk normally signs and symptoms consist of walking difficulties and and only show slight muscle weakness. The spine stiffens and fixed defor- problems arise from the development of scoliosis and mities occur in the extremities. As laboratory pa- The myotubular myopathy is variable with differing rameters the muscle enzymes are slightly to moderately modes of inheritance. The rare X-linked recessive vari- elevated, and the EMG shows myopathic changes. The muscle biopsy shows a dystrophic histological pattern, more common autosomal recessive type tends to produce partially mixed with muscular atrophies [4, 19, 21]. The clubfeet, lordoscolioses and winged scapula in the second condition can become life-threatening if it affects the decade of life. The orthopaedic management aims to preserve logically characterized by a predominance of excessively walking ability and prevent and treat skeletal deformities. This myopathy is not associated with a consistent clinical If life-threatening arrhythmias are present a pacemaker picture, but involves histological and functional changes will need to be inserted [26, 28]. The severity of the signs and symptoms Limb-girdle dystrophy is highly variable. Spinal deformities may require surgical Limb-girdle dystrophy is an autosomal-recessive form of correction. The disease can appear between child- Other rare forms of congenital myopathy exist, in- hood and adulthood. Patients experience problems with cluding minicore myopathy, the mitochondrial myopathies walking and climbing stairs and suffer from muscle cramps. The gait pattern is abnormal and produces a compensatory lordotic posture. The muscle weakness is of varying sever- Curschmann-Steinert myotonic dystrophy ity, and the prognosis is not uniform. The laboratory tests The illness usually occurs during early adulthood with show elevated muscle enzymes of varying degree, and myo- the main symptoms of weakness and stiffness. Dystrophic changes ing clinical feature is spontaneous myotonia after sus- are observed on the muscle biopsy [4, 19]. The facial muscles are also resembles that of Duchenne or Becker muscular dystrophy weak and ptosis is present. Cataracts and delayed intel- but the patient has a normal dystrophin level. The progno- thopaedic management aims to preserve the ability to walk sis depends on any accompanying cardiomyopathy and and prevent musculoskeletal deformities. The EMG shows a myotonia with myopathy, while the ECG shows conduction disorders Congenital forms of myopathy and arrhythmias. The muscle biopsy reveals dystrophic These forms include: changes with central cell nuclei. The orthopaedic mea- ▬ congenital muscular dystrophy, De Lange type, sures are aimed at preventing deformities and preserving ▬ congenital muscular dystrophy, Batten-Turner type, motor skills. Fasciculations are apparent on the tongue, as is The hereditary sensorimotor neuropathies are a het- a tremor of the hands.
Recent The objective of our efforts is to preserve the physical 0.25mg dostinex with mastercard, investigations cheap dostinex 0.5 mg without a prescription, however proven dostinex 0.25 mg, have shown a high complication and thus also the mental discount dostinex 0.5mg visa, integrity of the patient. Many rate, but a loosening frequency comparable with adult specialists are involved in the decision concerning the prostheses [2, 11, 14]. A recent innovation is the MU- appropriate treatment and its implementation. Since ma- TARS prosthesis with an externally controlled extendable lignant bone tumors are rare the necessary experience 643 4 4. But in return team includes, in addition to the orthopaedic surgeon, an we can offer – at least in respect of bone tumors – a con- oncologist, radiologist and bone pathologist. All of these siderable improvement in life expectancy and quality of specialists should – if not exclusively at least primarily life, and this at an age when no-one can ask whether this – be working in the field of bone tumors. A particularly useful diagnostic resource at our related (particularly if it occurs at night), an x-ray disposal is the bone tumor register, which was set up in should always be arranged. If a bone tumor is sus- 1972 by the Basel Institute for Pathology and currently pected, the patient should be referred, if possible includes over 11,000 bone tumors and tumor-like lesions. This particu- larly applies to bone tumors, since they are not only rare, but also subject to substantial variability in terms of their References appearance and prognosis. Abudu A, Carter SR, Grimer RJ (1996) The outcome of functional lead to the death of the patient, while overestimating a results of diaphyseal endoprosthesis after tumour excision. J Bone Joint Surg (Br) 78: 652–7 tumor can result in the unnecessary sacrifice of major 2. Ayoub K, Fiorenza F, Grimer R, Tillman R, Carter S (1999) Exten- sections of an extremity. J Bone Joint Surg Br 81: 495–500 tumors represent a »challenge« to the surgeon in both di- 3. Bielack S, Kempf-Bielack B, Delling G, Exner G, Flege S, Helmke agnostic and therapeutic respects. There is a great tempta- K, Kotz R, Salzer-Kuntschik M, Werner M, Winkelmann W, Zoubek A, Jurgens H, Winkler K (2002) Prognostic factors in high-grade tion to want to treat bone tumors oneself, despite a lack of osteosarcoma of the extremities or trunk: an analysis of 1,702 pa- experience. The saying »good results come from experience tients treated on neoadjuvant cooperative osteosarcoma study – experience comes from bad results« is particularly appli- group protocols. Blackley H, Wunder J, Davis A, White L, Kandel R, Bell R (1999) that complications also commonly occur, and are often Treatment of giant-cell tumors of long bones with curettage and bone-grafting. Borggraeve (1930) Kniegelenksersatz durch das in der Beinläng- happens that a resection does not include a full margin of sachse um 180° gedrehte Fußgelenk. Arch Orthop Unfallchir 28: health tissue despite a meticulous procedure. Moreover, 175–8 the loosening rate for tumor prostheses and the infection 6. Cammisa FP Jr, Glasser DB, Otis JC, Kroll MA, Lane JM, Healey JH rate associated with large allogeneic bone grafts are very (1990) The Van Nes tibial rotationplasty. J Bone Joint Surg (Am) 72: 1541–7 Psychological support for the patients is an important 7. It is important to talk openly and Heidelberg Vienna New York honestly also with children and adolescents about their 8. Chew W, Low C, Tan S (1995) Long-term results of free vascular- illness and prognosis. Crist W, Anderson J, Meza J, Fryer C, Raney R, Ruymann F, Bren- you be able to gain their trust and give them the confi- eman J, Qualman S, Wiener E, Wharam M, Lobe T, Webber B, dence needed to cope with these kinds of extensive treat- Maurer H, Donaldson S (2001) Intergroup rhabdomyosarcoma ment (see the quote at the start of Chapter 4. J Clin experience is required on the part of all those involved in Oncol 19: 3091–102 order to manage the patients in the right way. Deijkers RLM, Bloem RM, Petit PLC, Brand R, Vehmeyer SBW, Veen therefore includes a psycho-oncologist. Dominkus M, Krepler P, Schwameis E, Windhager R, Kotz R (2001) in Greece. A current Clin Orthop 390: 212–20 treatment involving expensive diagnosis, chemotherapy 12.