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Her father reported that he had been working on up 5 years later showed a complete nonunion of the right stretching Emily’s hips by having her do sit-up motions femoral neck and a healed fracture on the left (Figure on the floor cheap 100 mg penegra with amex, with her legs straight out in front of her as C3 cheap 50mg penegra visa. Both hips were painfree and she had excellent he pushed up on her trunk to flex her hips penegra 50mg discount. Range of motion in both hips was reduced purchase penegra 50mg with visa, to stop this in the past week because of the apparent hip but was adequate for sitting and personal hygiene order penegra 50 mg amex. On physical examination both hips were pain- case demonstrates the importance of reminding parents ful to motion; however, the right hip had a very free range to use limited force on doing stretching, especially after of motion, but the left was still limited to 80° of flexion. Most fractures of the lower ex- tremities are easy to immobilize with a bulky dressing made of rolled cotton. Although the soft cast may be large, it is light and has little risk of causing pressure areas on the skin (A). These bulky dressings also allow the child to get up into the wheelchair, although an ele- vating legrest usually needs to be obtained or constructed (B). It is also difficult to keep a child with a quadriplegic pattern CP in a stander for more than 1 to 2 hours a day. Our recommendation to parents is to try to get at least 1 hour per day of weight bearing. It makes good rational sense that even this short period of weight bearing in a stander would be better than nothing. Another prob- lem that occasionally arises is caretakers who become exceedingly cautious with the child because the fracture clearly occurred during a specific activity such as physical therapy stretching or dressing. This greatly increased cau- tion will only decrease the stress on the bones and further aggravate bone mineral loss. It must be explained to the therapist and caretakers that they should be careful to avoid fractures, but that it is very important to keep stretching and stressing these children’s bones. Treating the Osteoporosis and Osteomalacia Pathologically low bone mass should be investigated in nonambulatory chil- dren with spastic quadriplegia after the fracture has healed. All children over age 5 years who are nonambulatory with quadriplegic pattern CP should be considered at risk for low bone density, with those children on antiepileptic medications having the highest risk. The treatment should first focus on nu- tritional assessments,16, 21 especially dietary intake of calcium and vitamin D. The workup for all children should start with a complete 3-day dietary in- take history, which is then evaluated by a dietitian. The diet should then be augmented with calcium and vitamin D to the Recommended Daily Allowance (RDA) for the child’s age. Children who have had fractures and those who are nonambulatory with quadriplegia and are on seizure medications should have a bone mineral den- sity evaluation with dual-energy X-ray absorptiometry (DXA). This pattern of recurrent fracturing is relatively fracture in the same leg that had a tibial metaphyseal frac- common, and can usually be interrupted by limiting the ture 6 weeks earlier (Figure C3. These fractures immobilization as much as is comfortable, and starting were followed with five additional fractures over the next weight bearing on the uninvolved side. The fractures healed well with intravenous pamidronate for five cycles, and over with exuberant callus, which seemed to further deplete the next 4 years, has not had another fracture. We currently believe the full-body scan is best if it can be obtained and the child has no metal implants. Many children need seda- tion to obtain a good scan. A lumbar spine scan may also be obtained as a good correlation of the bone density, and we have been using the isolated distal femur scan because this is the area where most of the fractures occur. The validity of the distal femur is still not proven. This density evaluation 68 Cerebral Palsy Management should be normalized to age-matched normals to be of diagnostic use. This same group of children should also have a metabolic workup including serum and urinary calcium levels, and 25-hydroxyvitamin D, parathyroid hormone level, alkaline phosphatase, and phosphorus levels should be ob- tained. It is important to monitor calcium output in the urine because renal and bladder calculi can develop when chil- dren are given large doses of calcium and vitamin D, especially when the metabolic workup is normal. When children with severe dietary deficiency in calcium or vitamin D are treated with dietary supplementation, they have dramatic improvement in bone mineral density and a decrease in fracture rate. Currently, pami- dronate disodium is the best choice in these children.

When obstruction oc- ◗ Blood Disorders curs purchase 100 mg penegra fast delivery, there may be severe joint swelling and pain generic penegra 100 mg on-line, espe- cially in the fingers and toes cheap 50mg penegra amex, as well as abdominal pain generic penegra 100 mg with visa. Abnormalities involving the blood may be divided into This aspect of sickle cell anemia is referred to as sickle cell three groups: crisis cheap penegra 50 mg without a prescription. About 8% of African Americans have one of the blood and thus impaired delivery of oxygen to the tis- genes for the abnormal hemoglobin and are said to have sues. These disorders are characterized by an abnormal tendency to bleed due to a breakdown in the body’s clotting mechanism. Anemia Anemia may result from loss of red cells, as through ex- Sickle- cessive bleeding (hemorrhage), or from conditions that shaped cause the cells to hemolyze (rupture). In other cases, cell bone marrow failure or nutritional deficiences impede the production of red cells or hemoglobin. Abnor- mal cells take on a crescent (sickle) shape when they give up Excessive Loss or Destruction of Red Cells He- oxygen. Philadelphia: Lippincott Williams morrhagic loss of red cells may be sudden and acute or & Wilkins, 2003. The average adult has about 5 liters upper left corner of this picture? If a person loses as much as 2 liters suddenly, between the cells? It is only when the involved gene is that may injure the marrow include x-rays, atomic radia- transmitted from both parents that the clinical disease tion, radium, and radioactive phosphorus. About 1% of African Americans have two of The damaged bone marrow fails to produce either red these genes and thus have sickle cell disease. One drug or white cells, so that the anemia is accompanied by has been found to reduce the frequency of painful crisis leukopenia (lu-ko-PE-ne-ah), a drop in the number of in certain adults. Removal of the toxic agent, followed by blood some hemoglobin of an alternate form (fetal hemoglo- transfusions until the marrow is able to resume its activ- bin) so that the red cells are not as susceptible to sick- ity, may result in recovery. People taking hydroxyurea require blood tests every have also been successful. Some Impaired Production of Red Cells or Hemoglo- medications are now available to stimulate bone marrow bin Many factors can interfere with normal red cell pro- production of specific types of blood cells. Anemia that results from a deficiency of some nu- EPO made by recombinant methods (genetic engineer- trient is referred to as nutritional anemia. These conditions ing) can be given in cases of severe anemia to stimulate may arise from a deficiency of the specific nutrient in the red cell production. Deficiency Anemia The most common nutritional ane- Leukemia mia is iron-deficiency anemia. Iron is an essential con- Leukemia is a neoplastic disease of blood-forming tissue. The average diet usually provides It is characterized by an enormous increase in the num- enough iron to meet the needs of the adult male, but this ber of white cells. Although the cells are high in number, diet often is inadequate to meet the needs of growing they are incompetent and cannot perform their normal children and women of childbearing age. A diet deficient in proteins or vitamins can also result As noted earlier, the white cells have two main in anemia. Folic acid, one of the B complex vitamins, is sources: red marrow, also called myeloid tissue, and lym- necessary for the production of blood cells. If this wild proliferation of white cells stems ficiency anemia occurs in people with alcoholism, in eld- from cancer of the bone marrow, the condition is called erly people on poor diets, and in infants or others suffer- myelogenous (mi-eh-LOJ-en-us) leukemia. When the ing from intestinal disorders that interfere with the cancer arises in the lymphoid tissue, so that most of the absorption of this water-soluble vitamin. Both types of by a deficiency of vitamin B12, a substance essential for leukemia appear in acute and chronic forms. The cause is a permanent de- The cause of leukemia is unknown. Both inborn fac- ficiency of intrinsic factor, a gastric juice secretion that tors and various environmental agents have been impli- is responsible for vitamin B12 absorption from the in- cated.

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Muscle Length–Tension Relationship (Blix Curve) Another important aspect of a muscle’s ability to generate force is the length position in which the muscle fiber is stimulated relative to its resting length purchase 100 mg penegra otc. The arrangement of the muscle A fibers in the muscle is another variable in de- termining the excursion length of the muscle and the amount of force the muscle can gen- erate at the joint level (A) purchase penegra 50 mg with visa. The angle at which the muscle fiber inserts into the tendon is called the pennation angle discount penegra 50mg with visa, which can be very high for a muscle such as the deltoid buy penegra 50 mg without prescription. For most muscles of ambulation that have long muscle fibers order 100mg penegra otc, the pennation angle is so small that it has little impact on the force generated (B). B Thus, when a muscle is at resting length, the actin and myosin are in the relaxed position with slight overlap, and in this position, the muscle can generate its maximum force. If the muscle is distracted so that the sarco- meric subunits have less overlap, the muscle strength will decrease. Also, if the muscle is at an increased shortened position, it will use maximum force- generating ability because of too much overlap at the sarcomere level. This phenomenon has been defined by the Blix curve, or the muscle length–tension curve, and has been presented in many textbooks as a key mechanism to understand a muscle’s response in generating force (Figure 7. An under- standing of a muscle’s length relative to the Blix curve is especially impor- tant when planning muscle-lengthening procedures. The muscle fiber length–tension curve (Blix curve) is crucial to understanding the muscle force-generating ability. At rest length the muscle has the ability to generate the highest amount of active force. As the muscle shortens, this ability to generate force decreases to zero at approximately 60% of rest length. As the muscle lengthens, the ac- tive force-generating ability also decreases and reaches zero at approximately 170% of rest length. However, as the muscle lengthens, the passive collagen elements provide a pas- sive restraint to further lengthening, thereby increasing tension as the muscle is lengthened. This increases until approximately 200% of rest length, when the muscle starts to physi- cally fail. This force-generating ability is altered by changes in muscle fiber size, fiber pennation angles, length of the fiber relative to its resting length, and the cross-sectional size of the whole muscle. Endurance or fatigability of a muscle depends on the muscle fiber type, especially its primary metabolic function, which is either oxidative or anaerobic, and the muscle fiber’s velocity of contraction, meaning specifically whether it is concentric, eccentric, or iso- metric. A muscle’s selective control is altered mainly at the muscle level by the size of motor units. This means that an individual muscle has less selec- tive control when its motor units increase in size, such as expanding from 500 to 800 fibers per motor unit. The amount of angular joint force pro- duced by a given muscle is further defined by the mechanical anatomy, such as the course of the tendon, the moment arm length from the center of motion to the tendon insertion, and the angular velocity of the motion. Alteration of Muscle Mechanics Normal mechanics of a muscle unit change over time under the influence of many factors. Areas that are of specific concern in the treatment of children with CP are the influence of growth and development, the impact of muscle tone change, and the impact of stretching and strengthening stimuli. Muscle Control Each group of motor units is controlled by one motor neuron that can only contract or not be active. Variable control of muscle contraction is gained by how many motor units are contracting in concert. In normal individuals, each gastrocnemius has approximately 1800 motor units; therefore, the brain, via the central program generator, has a choice of how many motor units to fire at a specific time. The number of motor units can 260 Cerebral Palsy Management be decreased, but the muscle stays the same size if the muscle fibers are en- larged and the number of fibers per motor neuron is increased. The central program generator also has to consider any change in fiber types, from fast twitch to slow twitch, as to the muscle’s impact on activation of a specific motor unit. These fiber types are determined through motor neuron inter- action. Therefore, the muscles in children with spasticity organize toward slower-twitch, fatigue-resistant fibers, which are organized into larger motor units having fewer mechanoreceptors.

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