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Braces and corsets are often required not only for postural correction cheap 160 mg malegra fxt plus erectile dysfunction 37 years old, but also to replace the lost muscle function. The correction of abnormal spinal posture and spinal deformities is useful even for small angular deviations. Typical pseudohypertrophy of the calves in Duchenne cedure in a patient with a worse state of general health. The pronounced muscular atrophy, the lack of facial expression and the oversized tongue are additional visible a b signs of the muscular dystrophy of life and, in particular, the ability to sit and facilitates hood. Corset treatments are not very effective Muscle cramps are present during and after physical and useful only in exceptional cases when operations exertion. The muscle weakness tends to occur proximally are no longer possible or are declined. The pulmonary Since the Becker dystrophy progresses much more slowly, insufficiency manifests itself in the form of hypoxemic patients are still able to walk even after puberty. Life phases (particularly at night), which also affects mental expectancy is determined by the degree of respiratory performance during the day. A home ventilation program insufficiency and is between 40 and 60 years. The ortho- (assisted nocturnal ventilation) can significantly improve paedic and cardiac findings resemble those for Duchenne these symptoms. More difficult is the treatment of the muscular dystrophy, but are less pronounced. The inci- heart failure, which is likewise progressive and a life-limit- dence of scolioses is not known, but spinal deformities ing factor. In the context of surgery, patients with Duch- must be expected in severe cases. Duchenne muscular dystrophy, the muscle biopsy shows a picture of degeneration and regeneration. The abnormal dystrophin level can be confirmed by laboratory tests and 4. The orthopaedic measures are essentially no different symptoms are less pronounced and progress less rapidly. Occurrence, etiology Becker muscular dystrophy has an incidence of 3/100,000 and is thus 10 times rarer than the Duchenne type. The onset of signs and symptoms can Clinical features and diagnosis occur from early childhood right through to adulthood. The disease manifests itself between the age of five and The condition manifests itself as a weakness of the afore- adolescence. In some cases, it does not appear until adult- mentioned muscles, and the pelvic muscles can also be af- 752 4. Deafness and changes in the fundus central-core myopathy, oculi may also be present. The muscle biopsy fiber type disproportion, shows a variable picture with focal fiber atrophy and other forms. Whereas the De Lange myopathy is a malignant condi- The course and prognosis differ, varying from a very slow tion, 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.

The patient sits laterally at the examination table with the elbow flexed by 90° and the wrist extended and supi- Upper arm malegra fxt plus 160 mg overnight delivery erectile dysfunction age at onset, AP with shoulder and elbow nated. The patient stands or lies on his back with the elbow extended and the hand supinated. The wrist and fingers are extended, with the ulnar resting on the cassette. The central beam is aimed at the wrist, Upper arm, lateral radioulnar beam path (⊡ Fig. The patient sits laterally at the edge of the table with the upper arm abducted by 90°, the elbow flexed by 90° and Wrist, dorsovolar the hand supinated. The central beam is aimed vertically The hand is pronated on the cassette. Elbow, lateral with 90° flexion of the joint Scaphoid (navicular), dorsovolar The patient sits at the table with the arm abducted, the Position: The hand is pronated and abducted towards the ulnar side of the elbow resting on the cassette and the ulnar side, the wrist rests on the cassette with the fingers hand supinated. The central beam is aimed at the radial flexed and the thumb extended (⊡ Fig. Scaphoid (navicular), AP and lateral For the lateral view, the ulnar side rests on the cassette with the hand slightly dorsiflexed. The fist is loosely closed and the beam path is aimed at the scaphoid in a radioulnar direction (⊡ Fig. Thumb, volodorsal Position: The extensor side of the thumb rests on the cas- sette with the hand in maximum pronation. The central beam is aimed at the thumb metacarpophalangeal joint at right angles to the cassette. Thumb, lateral Position: The radial side of the thumb rests on the cas- sette. The central beam is aimed at the thumb metacarpopha- langeal joint (⊡ Fig. Whole hand, dorsovolar Position: The hand rests on the cassette with the fingers extended and slightly apart. The epiphyses of the injured shoulder at an angle of 40° to the cassette plane. This view is particularly radius and ulna must be included in the x-ray if the bone effective for showing any forward or backward displacement of the age needs to be established. With uncooperative toddlers, humeral head it is sometimes better to x-ray the hand in supination with 463 3 3. Recording technique for a b x-rays of the elbow: (a) AP, (b) lateral (see text) ⊡ Fig. Recording technique a b for x-rays of the wrist: (a) lateral and (b) AP (see text) a b ⊡ Fig. Specific view for the scaphoid bone: AP (a) and lateral (b, see text) ⊡ Fig. The central beam is a b aimed at the head of the 3rd metacarpal the aid of a 10 cm wide Plexiglas strip secured on both Occurrence sides with two sandbags. Figures on the occurrence of congenital deformities are difficult to obtain. In a study of 50,000 births in Edin- Whole hand, oblique burgh, the authors calculated that just 3. The ulnar side rests on the all malformations and hereditary disorders is estimated at cassette. The central beam is aimed at the head of the 3rd 2–3%, which roughly means that 1 anomaly of an upper metacarpal (⊡ Fig. Most cases result from dam- was attributable to the drug thalidomide, which caused age that occurs during early pregnancy, although certain serious damage when taken during pregnancy (between malformations are also inherited. After the connection was finally con- firmed in 1961, the incidence retuned to its previous level. Classification In the middle of the 19th century Saint-Hilaire in- troduced Greek terms to describe various malformations. Thus an »amelia« referred to the absence of an extremity (Greek: melos = limb).

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Chen W malegra fxt plus 160 mg line impotence under 30, Chen T, Huang C, Chiang C, Lo W (2002) Treatment of hager R, Kotz R (2002) Reconstruction of the pelvis after tumor malignant bone tumours by extracorporeally irradiated auto- resection in children and adolescents. Sucato D, Rougraff B, McGrath B, Sizinski J, Davis M, Papandonatos 1156–61 G, Green D, Szarzanowicz T, Mindell E (2000) Ewing’s sarcoma of 8. Davis R, Hamilton A, Biggart J (1998) Primary synovial chondro- the pelvis. Clin Or- matosis: a clinicopathologic review and assessment of malignant thop 373: 193–201 potential. Making use of the ankle of the shortened limb to control reconstruction by an allograft prosthesis composite. Dominkus M, Krepler P, Schwameis E, Windhager R, Kotz R (2001) of the proximal part of the femur. J Bone Joint Surg (Am) 68: Growth prediction in extendable tumor prostheses in children. Eckardt J, Kabo J, Kelley C, Ward W, Asavamongkolkul A, Wirgano- lignen Tumoren des Darmbeins. Z Orthop 126: 671–4 wicz P, Yang R, Eilber F (2000) Expandable endoprosthesis recon- struction in skeletally immature patients with tumors. Eggermont A, Schraffordt Koops H, Klausner J, Kroon B, Schlag P, Lienard D, van Geel A, Hoekstra H, Meller I, Nieweg O, Kettelhack C, Ben-Ari G, Pector J, Lejeune F (1996) Isolated limb perfusion with tumor necrosis factor and melphalan for limb salvage in 186 patients with locally advanced soft tissue extremity sarcomas. Ekkernkamp A, Muhr G, Lies A (1990) Die kontinuierliche Dekom- pression. Fahey M, Spanier SS, Vander Griend RA (1992) Osteosarcoma of the pelvis. Frassica FJ, Frassica DA, Pritchard DJ, Schomberg PJ, Wold LE, Sim FH (1993) Ewing sarcoma of the pelvis. Gonzalez Della Valle A, Piccaluga F, Potter H, Salvati E, Pusso R (2001) Pigmented villonodular synovitis of the hip: 2- to 23-year followup study. Grimer R, Carter S, Tillman R, Spooner D, Mangham D, Kabukcuo- glu Y (1999) Osteosarcoma of the pelvis. Ham S, Kroon H, Koops H, Hoekstra H (2000) Osteosarcoma of the pelvis–oncological results of 40 patients registered by The Netherlands Committee on Bone Tumours. Hansen M, Nellissery M, Bhatia P (1999) Common mechanisms of osteosarcoma and Paget’s disease. Differential diagnosis of hip pain 3 Age group Signs and symptoms Tentative diagnosis Additional measures Infant (0–2 years) Poss. MRI/CT Pain (movement-related) Juvenile rheumatoid arthritis Laboratory (infection parameters, of the hip rheumatoid factors), ultrasound Restricted movement, poss. Differential diagnosis of restricted hip movement Age group Restricted direction of movement Tentative diagnosis Additional measures Infant at birth Full extension (20°–30° flexion Normal findings None contracture) Infant Abduction only up to 70° Hip dysplasia/dislocation Ultrasound (from 2 months) Infant (0–2 years) Internal rotation, poss. MRI/CT Adolescent Internal rotation, abduction Slipped capital femoral AP and axial x-rays (from 10 years) epiphysis Poss. MRI/CT Internal rotation, extension, Juvenile rheumatoid Laboratory (infection parameters, abduction arthritis of the hip rheumatoid factors), ultrasound, AP x-ray Internal rotation, extension, Septic arthritis of the hip Laboratory (infection parameters), abduction AP x-ray, poss. Indications for imaging procedures for the hip Age Circumstances/Indication Tentative clinical Imaging procedures 3 diagnosis Infant Positive family history, positive clinical exam- Hip dysplasia Ultrasound, AP hip x-ray ination findings, additional malformations Infant, toddler, child Fever, restricted movement, pain, limping, Septic arthritis of the hip Ultrasound (effusion? Indications for physical therapy in hip disorders Disorder Indication Goal/type of treatment Duration Additional measures Septic arthritis of the Defective healing and Improve mobility, partic- As long as mobility is Poss. Frequent if movement is restricted, as swimming and cycling long as progress is possible Developmental In the older child with Improve gait As long as mobility is – dysplasia of the hip persistent dysplasia, poss. Improve mobility restricted and prog- (DDH) postoperatively ress is still possible Intoeing gait None Encouragement of sport- – Operation very rarely ing activity more useful indicated; watch for than physical therapy tibial torsion Slipped capital Postoperatively Strengthen the muscles Until the patient No strenuous sport femoral epiphysis (extensors/abductors), walks without a limp, until completion of improve mobility mobility is unre- growth stricted or no further progress is possible Femoral fractures If gait pattern is not normal Walking exercises As long as patient is – after 3 months symptomatic 279 3 3. The examination protocol for the knees is shown in – Did anything »give way« during the trauma, or was ⊡ Table 3. Is the pain load-related, movement-related, If so: or does it also occur at rest (e. If so, does the pain only occur when the – During what type of activity (sport, play, daily rou- patient changes position or does the patient awake at tine)? Lateral contours of the supine patient with 90° Lesion of the posterior cruciate ligament? Palpation Palpation of the patellar margins, shifting of Anterior knee pain?

Central venous catheter Central venous catheters are inserted into the veins of the antecu- bital fossa quality malegra fxt plus 160 mg impotence mayo clinic, the long saphenous vein or the superficial temporal Neonates 117 Fig. Note how the arterial catheter runs caudally initially before turning to run cranially with the aorta. When correctly positioned the tip should lie at the junction of the superior vena cava and the right atrium. Chest drain Achest drain is normally sited following identification of a pneumothorax (Fig. Common insertion sites are the anterior chest wall through the second or third intercostal spaces directly lateral to the mid-clavicular line, and the lateral chest wall through the fourth, fifth or sixth intercostal spaces directly anterior to the axillary line. The tube should be inserted approximately 3cm into the tho- racic cavity and directed towards the apex of the lung. A chest drain may also be used to drain collections of plural fluid, in which case the tube usually requires directing posteriorly and inferiorly15. In addition, they may also facilitate gastric or small bowel decompression in cases of excessive abdominal distension. When correctly positioned, the tip of the nasogastric/orogastric tube should be identified within the stomach or, where a nasojejunal feeding tube is used, within the jejunum. Radiographic technique for the chest Antero-posterior (supine) The antero-posterior supine chest radiograph is the most common neonatal chest projection. A clean sheet or a pillowcase should be used to cover the cassette in order to reduce the risk of cross-infection and prevent neonatal heat loss when in contact with a cold surface. If it is then care should be taken not to place the cassette under the mattress, as it may be visible on the resultant radiograph. If the neonate is placed directly onto a covered cassette then the apnoea alarm will need to be deactivated and then reset when the cassette is removed. The neonate’s arms should be flexed on either side of the head and the head held straight to prevent rotation. Care must be taken to avoid extending the arms as this may result in lordosis. If the neonate is quiet it may be possible to prop the head straight and to leave the arms abducted. This will avoid any irradia- tion to the health care worker who would otherwise be required to immobilise the patient. To avoid a lordotic projection a 15° pad should be used or, alterna- tively, the incubator tray could be angled with the head end raised 15°. Care must also be taken to ensure the chin does not obscure the upper chest; this can be achieved by placing a small pillow or roll of cotton wool under the neck. If the neonate is intubated then the head will probably be turned to the side. In this case the head should not be straightened but left in the presenting position as straightening the head may cause the distal end of the endotracheal tube to move slightly which may be significant if the radiograph is required to assess tube position. A rolled blanket placed under the legs may help to immobilise the neonate in the correct position. The neonate should be positioned in the incubator so as to avoid any curves/cut-outs in the incubator roof that may be visible on the resultant radiograph and detract from the quality of the film (Fig. Lead rubber should be placed on top of the incubator to provide protection to the neonate abdomen and head. Many intensive care units use small intensive care beds rather than incubators in order to facilitate easy access to the neonate Neonates 119 Fig. The curve must be excluded from the projection or it will be visible on the radiograph. In these instances protection for the head can be applied by use of a lead rubber glove and an additional lead shield held over the neonate (Fig. The x-ray beam should be perpendicular to the cassette and centred to the middle of the sternum with lower collimation at a level just above the lower costal margin. Neonates are abdominal breathers and therefore the rise and fall of the abdomen is a good indicator of the phase of respiration – inspiration being indicated when the abdomen is rising. It is essential to obtain an adequately inspired radiograph in order to opti- mise the visualisation of lung tissue and enable accurate assessment of the cardiac size and shape. If the neonate becomes distressed then the radiographer should wait to expose the film until the neonate has ceased crying. If the child is radiographed whilst crying then the lungs can appear overinflated and this hyperinflation can mimic pathology.

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