PED - Chapter 28 - Alterations in Neuromuscular Function

The nurse is conducting a physical examination of a 10-year-old boy with a suspected neuromuscular disorder. Which finding is a sign of Duchenne muscular dystrophy? A. Gowers sign B. appearance of smaller than normal calf muscles C. indications of hydrocephalus D. lordosis

A. Gowers sign A sign of Duchenne muscular dystrophy (DMD) is Gowers sign, or the inability of the child to rise from the floor in the standard fashion because of weakness. Signs of hydrocephalus are not typically associated with DMD. Kyphosis and scoliosis occur more frequently than lordosis. A child with DMD has an enlarged appearance to their calf muscles due to pseudohypertrophy of the calves.

The nurse is reviewing the history of a 3-year-old child diagnosed with cerebral palsy as an infant. Which factor from the child's health history would the nurse identify as placing this child at risk for this condition? A. birth at 26 weeks' gestation B. birth weight 7 lb 2 oz (3,231 g) C. singleton birth D. paternal history of seizure disorder

A. birth at 26 weeks' gestation The child was premature at birth, which is a risk factor for cerebral palsy. Other factors that increase the risk for cerebral palsy include low birth weight, multiple births, and maternal history of seizure disorder.

The nurse caring for a client diagnosed with muscular dystrophy would expect which laboratory values to be most abnormal? A. bilirubin B. creatine kinase C. serum potassium D. sodium

B. creatine kinase Serum creatine kinase levels are elevated early in the disorder, when significant muscle wasting is actively occurring. Bilirubin is a by-product of liver function. Potassium and sodium levels can change due to various factors and aren't indicators of muscular dystrophy.

A 7-year-old child with cerebral palsy comes to the clinic for an evaluation. The child is prescribed medications to address muscle spasticity and seizures. When assessing the child's mouth, which condition would the nurse associate with the child's medication regimen? A. malocclusion B. enamel erosion C. gingival hyperplasia D. multiple dental caries

C. gingival hyperplasia Although enamel defects and malocclusion are common dental problems in children with cerebral palsy, the child is receiving anticonvulsant therapy, which can lead to gingival hyperplasia. Dental caries are unrelated to the child's diagnosis or medication therapy.

A parent brings an 18-month-old child to the pediatrician's office for a well-child visit. The child has mild cerebral palsy that affects the child's gait. The nurse wants to assess the child's neuromuscular system. What is the best way for the nurse to make that assessment? A. Quietly observe the child at play while interviewing the parent. B. Ask the parent to describe the child's development. C. Get down to the child's level and interact with the child. D. Review the child's health history to determine if the child is on track developmentally.

A. Quietly observe the child at play while interviewing the parent. The best way to assess a young child's neuromuscular system is to observe the child from a distance. Observing the child at play will allow the nurse to assess the child's gross and fine motor skills, as well as cognitive abilities. Asking the parent to describe the child's motor development may be appropriate in some cases such as the ability to feed. Getting down to the child's level may help assess the child's social development but observing the child play at a distance is best to assess a child's fine and gross motor development. Reviewing the child's history will give the nurse a sense of the child's past developmental level but will not give information about the child's current status.

The nurse caring for a client with suspected muscular dystrophy would prepare her client for which diagnostic test? A. X-ray B. muscle biopsy C. EEG D. assessment of ambulation

B. muscle biopsy Muscle biopsy provides definitive diagnosis of muscular dystrophy demonstrating the absence of dystrophin. X-ray is best for identifying an osseous deformity. Ambulation assessment alone wouldn't confirm diagnosis of this client's disorder. EEG wouldn't be appropriate in this case.

The nurse is caring for a school-age child diagnosed with Duchenne muscular dystrophy who is receiving glucocorticoid therapy. When teaching the parents about this therapy, the nurse would emphasize which aspect? A. importance of yearly cardiac exams B. safety measures for injury prevention C. steps for active and passive range-of-motion exercises D. techniques for assisted coughing

B. safety measures for injury prevention Although annual cardiac exams, active and passive range-of-motion exercises, and assisted coughing techniques are important components of care for a child with Duchenne muscular dystrophy (DMD), safety measures for injury prevention would be critical for this child because of the use of glucocorticoids. This therapy has been shown to improve neuromuscular outcomes but it is also associated with adverse effects on bone health. Long-term use predisposes the child to osteoporosis. Coupled with the fact that children with DMD have progressively weakening muscles, the risk of osteoporosis is compounded. Both vertebral fractures and fractures of the long bones are common even in low-level trauma. Therefore, safety would be the priority.

The nurse is conducting a physical examination of a 9-month-old infant with a suspected neuromuscular disorder. Which finding would warrant further evaluation? A. presence of symmetrical spontaneous movement B. absence of Moro reflex C. absence of tonic neck reflex D. presence of Moro reflex

D. presence of Moro reflex The persistence of a primitive reflex in a 9-month-old would warrant further evaluation. Symmetrical spontaneous movement and absence of the Moro and tonic neck reflexes are expected in a normally developing 9-month-old child.

The nurse is caring for a child with muscular dystrophy. Which prescription will the nurse question? A. calcium supplement B. vitamin D C. prednisone D. clozapine

D. clozapine Duchenne muscular dystrophy is the most common neuromuscular disorder of childhood, mostly affecting males. There is no cure, but treatments are available to slow progression and provide symptom management. Corticosteroids, such as prednisone, may be prescribed to protect muscle fibers from damage to the sarcolemma. Studies have shown males treated with prednisone have increased strength and function. Calcium supplements and vitamin D are prescribed to prevent osteoporosis. Antidepressants, not antipsychotics (such as clozapine), may be helpful when depression occurs related to the chronicity of the disease and/or as an effect of corticosteroid use

A young child with Duchenne muscular dystrophy is placed on both prednisone and calcium. Parents view these two medications as rather "common" and question their importance for the child. What explanation by the nurse will be most helpful to the parents? A. "Prednisone helps to keep muscle fibers strong and delays progression of the disease. Calcium protects against osteoporosis caused by both prednisone and lack of weight bearing." B. "Prednisone will help protect his vulnerable respiratory tract from developing reactive airway disease. Calcium is needed to guard against muscle cramping." C. "Prednisone will protect against nerve inflammation in his hips and legs. Calcium is necessary should dietary intake be insufficient to meet growth needs." D. "Prednisone will stimulate weight gain and appetite. Calcium is needed to ensure adequate supplies for the development of permanent teeth."

A. "Prednisone helps to keep muscle fibers strong and delays progression of the disease. Calcium protects against osteoporosis caused by both prednisone and lack of weight bearing." Studies have shown that boys treated with prednisone have improved muscle strength and function. This is thought to be due to the protection that prednisone provides to muscle fibers. Calcium is needed to prevent osteoporosis, which is a side effect of prednisone that also occurs when weight bearing is limited. Respiratory infection is a risk in that those muscles weaken with progression of the disease, but reactive airway disease is not a particular risk. No peripheral nerve involvement is observed in Duchenne muscular dystrophy. Side effects of prednisone include weight gain and appetite stimulation, but these are not the reasons for the prednisone therapy. Calcium does augment dietary intake of the mineral and is important for tooth development, and it may play a role in prevention of muscle cramps, but these are not the main reasons for taking the calcium supplement.

A 3-month-old infant is seen in the pediatric clinic. The infant's parent expresses concern that the child has developed cerebral palsy. The nurse assesses the infant. Which assessment finding indicates to the nurse that the parent's concern is valid? A. hypertonia in the upper extremities B. exhibits Gower sign C. unable to sit without support D. turns head toward sounds

A. hypertonia in the upper extremities Cerebral palsy manifests as hyper- or hypotonia, and cognitive and developmental delays. Gower sign is a manifestation of muscular dystrophy not cerebral palsy. A 3-month-old infant should be developmentally able to turn toward a voice or sound but is too young to sit without support.

The parents of a 4-year-old child bring the child to the provider's office for an evaluation. The parents are concerned about the child's weakness and problems with stair climbing. The nurse assesses the child and documents the findings. Based on the findings, the nurse suspects Duchenne muscular dystrophy (DMD). When discussing the findings with the primary care provider, the nurse anticipates which laboratory test being prescribed to provide additional information? A. white blood cell count B. creatinine kinase (CK) C. prothrombin time (PT) D. alkaline phosphatase

B. creatinine kinase (CK) In light of the child's assessment findings, the nurse would anticipate the need for a creatinine kinase (CK) level. An elevated creatinine kinase (CK) level further raises the suspicion for DMD and should prompt a referral to a genetic specialist. A normal CK level all but eliminates the possibility for DMD, and alternative diagnoses should be investigated. White blood cell count, prothrombin time, or alkaline phosphatase would be inappropriate and shed no further light on the child's possible diagnosis.

A nurse is providing care to a pediatric client hospitalized with a diagnosis of Duchenne muscular dystrophy (DMD). The nurse is reviewing the above laboratory results. Which laboratory result will have the greatest impact on the client's condition? A. creatinine B. calcium C. glucose D. potassium (2.4)

D. potassium The potassium level is low and will have the greatest impact on the client's condition. Children diagnosed with DMD often have issues with cardiac and respiratory function and are often on antihypertensive and diuretic medications. They will also be prescribed glucocorticoids to manage the neuromuscular effects of the disorder. The glucose level is elevated slightly and will need to be monitored (especially with the use of glucocorticoids) but the potassium level requires immediate intervention. The creatinine and calcium levels are within normal limits.