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What is Hemophilia?

Deficiency of clotting factor 8 9 11

Clinical manifestations of hemophilia?

Excessive bleeding Spontaneous hematuria and epistaxis life long problem life threatening for intracranial hemorrhage and bleeding into the tissues of the neck or abdomen

Reflexes present at birth

Moro- startle reflex elicited by loud noise or sudden change in position Tonic neck- elicted when infant lies supine and head is turned to one side; the finant will assume a fencing position gag, cough, blink, pupillary: protective reflexes grasp: infant's hands and feet will grasp when hand or foot is stimulated rooting: elicited when side of mouth is touched, causing the child to turn to that side Babinski- fanning of toes when sole of foot is stroked upward All reflexes are gone by 4 months except babinski

The home care nurse visits a new parent and a 2-week-old infant. The client asks the nurse which solid foods to give the child first. Which response does the nurse give? 1. Rice cereal is usually the first solid food and is started around 4 to 5 months. 2. Strained fruits are well tolerated as the first solid food, and infants like them. 3. Introduction of solid foods is not important at this time. 4. Solid foods are usually not started until the infant is around 6 months old.

Strategy: All answers are implementation. Determine the outcome of each answer. Is it desired? 1) CORRECT - infants are less likely to be allergic to rice cereal than to any other solid food; usually started between 4 and 5 months of age; breast-fed infants may be started on solids even later 2) inaccurate 3) does not answer the parent's question 4) usually started between 4 and 5 months of age

The nurse cares for a 3-month-old infant scheduled for a barium swallow in the morning. Prior to the procedure, it is most appropriate for the nurse to take which action? 1. Offer the infant only clear liquids. 2. Make the infant NPO for 3 hours. 3. Feed the infant regular formula. 4. Maintain the infant NPO for 6 hours.

Strategy: All answers are implementation. Determine the outcome of each answer. Is it desired? 1) inappropriate 2) CORRECT — infant should be NPO 3 hours prior to the procedure 3) inappropriate 4) unnecessary for an infant to be NPO for 6 hours

The client develops a postoperative infection and receives ceftriaxone sodium IV every day. It is most important for the nurse to monitor for which changes? 1. The surface of the tongue. 2. Hemoglobin and hematocrit. 3. Skin surfaces in skin folds. 4. Changes in urine characteristics.

Strategy: Answer choices indicates a complication. 1) CORRECT — cephalosporin, long-term use of ceftriaxone sodium can cause overgrowth of organisms; monitoring of tongue and oral cavity is recommended 2) does not reflect a problem with this medication 3) does not reflect a problem with this medication 4) does not reflect a problem with this medication

Several days after the delivery of a stillborn, the parents say, "We wish we could talk with other couples who have gone through this trauma." Which response by the nurse is best? 1. "SIDS will provide you with this opportunity." 2. "SHARE will provide you with this opportunity." 3. "RESOLVE will provide you with this opportunity." 4. "CANDLELIGHTERS will provide you with this opportunity."

Strategy: Answers are implementation. Determine the outcome of each answer. Is it desired? 1) support group for parents who have had an infant die from sudden infant death syndrome 2) CORRECT — SHARE is a support group for parents who have lost a newborn or have experienced a miscarriage 3) support group for infertile clients 4) support group for families who have lost a child to cancer

Which observation suggests to the nurse the client has developed an Addisonian crisis? 1. Muscular weakness and fatigue. 2. Restlessness and rapid, weak pulse. 3. Dark pigmentation of the skin. 4. Gastrointestinal disturbances and anorexia.

Strategy: Determine how each answer relates to Addisonian crisis. 1) signs and symptoms of Addison's disease, but do not indicate a crisis 2) CORRECT — may be signs of shock related to an Addisonian crisis 3) signs and symptoms of Addison's disease, but do not indicate a crisis 4) signs and symptoms of Addison's disease, but do not indicate a crisis

The 7-year-old child is seen in the clinic with a diagnosis of pituitary dwarfism. Which clinical manifestation is the nurse most likely to observe? 1. Abnormal body proportions. 2. Early sexual maturation. 3. Delicate features. 4. Coarse, dry skin.

Strategy: Determine how each answer relates to dwarfism. 1) see small size but normal body proportions 2) usually have delayed sexual maturity 3) CORRECT — appear younger than chronological age 4) usually see fine, smooth skin

Which action is the best way for the nurse to assess the fluid balance of an elderly client? 1. Assess the client's blood pressure. 2. Check the client's tissue turgor. 3. Determine if the client is thirsty. 4. Maintain an accurate intake and output.

Strategy: Determine how each answer relates to hydration. 1) may be elevated because of age-related hypertension 2) not accurate because of changes in skin elasticity due to the aging process 3) not reliable indicator; may have diminished sensation of thirst 4) CORRECT—best indicator of fluid status

The toddler diagnosed with lead poisoning is admitted to the pediatric unit. The health care provider writes an order to encourage fluids. Which fluid is best for the nurse to offer to the toddler? 1. Milk. 2. Water. 3. Orange juice. 4. Fruit punch.

Strategy: Determine how each answer relates to lead poisoning. 1) CORRECT — milk contains calcium; calcium binds to lead and inhibits its absorption 2) good for fluid replacement; does not relate to the lead poisoning 3) good for fluid replacement; does not relate to the lead poisoning 4) good for fluid replacement; does not relate to the lead poisoning

The health care provider orders naproxen sodium for the elderly client. The nurse assesses the client for which symptoms? 1. Stomatitis and photosensitivity. 2. Bradycardia and dry mouth. 3. Fluid retention and dizziness. 4. Gynecomastia and impotence.

Strategy: Determine how each answer relates to naproxen sodium. 1) not adverse effects seen with this medication; may see headache, nausea 2) not adverse effects seen with this medication; may see epigastric distress and rash 3) CORRECT — NSAID (nonsteroidal anti-inflammatory drug) used as analgesic; adverse effects include headache, dizziness, gastrointestinal distress, pruritus, and rash 4) not adverse effects seen with this medication; may see nephrotoxicity and pruritus

The nurse cares for the client diagnosed with a pneumothorax resulting from a motor vehicle accident three days ago. The client has a chest tube connected to a three-chamber water-seal drainage system with 20 cm suction. The nurse determines the lung has re-expanded if which observation is made? 1. There is no drainage in the collection chamber for 3 hours. 2. The fluid in the water-seal chamber does not fluctuate with respirations. 3. There is continuous bubbling in the water-seal chamber. 4. There is gentle bubbling in the suction-control chamber.

Strategy: Determine how each observation relates to a chest tube. 1) doesn't indicate re-expansion 2) CORRECT — indicates no more air leaking into pleural space 3) indicates air leak; need to check for location of leak; clamp tubing close to chest and check for bubbling, and then clamp tubing close to container and check for bubbling 4) normal finding

Which is most important for the rehabilitation nurse to assess during a new client's admission? 1. The client's expectations of family members. 2. The client's understanding of available supportive services. 3. The client's personal goals for rehabilitation. 4. The client's past experiences in the hospital.

Strategy: Determine the outcome and how it relates to rehabilitation. 1) important to assess but is not as crucial for future success as the client's goals 2) important to assess but is not as crucial for future success as the client's goals 3) CORRECT — it is important for the nurse to understand what the client expects from the rehabilitation program for future success 4) important to assess but is not as crucial for future success as the client's goals

Which statement is documented by the nurse to reflect a client's emotional adjustment to being hospitalized in the intensive care unit? 1. "The client is unable to complete activities of daily living without assistance." 2. "The client appears to be depressed and anxious regarding impending surgery." 3. "The client constantly calls for nurses and cries uncontrollably." 4. "The family is unable to visit more often than once a week because they live far away."

Strategy: Good documentation is the objective. 1) does not describe emotional adjustment 2) draws conclusions without supporting data 3) CORRECT — gives an objective description of the client's behavior and affect 4) describes the client's family, not the client

The nursing team consists of an RN who has been practicing for 6 months, an LPN/LVN who has been practicing for 15 years, and a nursing assistive personnel who has been caring for clients for 3 years. The RN cares for which client? 1. The client 1 day postop after an internal fixation of a fractured left femur. 2. The client receiving diltiazem and phenytoin. 3. The client ordered to receive two units of packed cells. 4. The client admitted yesterday with exhaustion and a diagnosis of acute bipolar disorder.

Strategy: The RN cares for clients who require assessment, teaching, and nursing judgment. 1) care can be assigned to the nursing assistive personnel; standard, unchanging procedure 2) medication can be given by the LPN 3) CORRECT — requires the assessment and teaching skills of the RN 4) offer food and fluids; assign to the LPN

The middle-aged client is admitted to an inpatient psychiatric unit. The client reports a family member is trying to steal the client's property. The client is diagnosed with paranoid disorder. The nurse suspects the client is demonstrating which symptom? 1. Delusions of persecution. 2. Command hallucinations. 3. Delusions of reference. 4. Persecution hallucinations.

Strategy: Think about each answer. 1) CORRECT — client has delusions of persecution; delusion is a strongly held belief that is not validated by reality; the idea that a family member is trying to steal property is a belief not validated by reality 2) hallucinations are sensory perceptions that take place without external stimuli; most common are auditory, or hearing voices; other types of hallucinations are tactile, visual, gustatory, and olfactory; command hallucinations involve client experiencing auditory hallucinations that are telling him/her to do something; for example, to kill someone 3) delusions of reference are a false belief that public events or people are directly related to the individual 4) are not hallucinations

The client is evaluated for infertility, and the health care provider prescribes clomiphene citrate 50 mg daily for 5 days. The client asks the nurse how the medication works. Which response by the nurse is best? 1. Clomiphene citrate induces ovulation by changing hormonal effects on the ovary. 2. Clomiphene citrate changes the uterine lining to be more conducive to implantation. 3. Clomiphene citrate alters the vaginal pH to increase sperm motility. 4. Clomiphene citrate produces multiple pregnancy for those who desire twins.

Strategy: Think about each answer. 1) CORRECT — clomiphene citrate induces ovulation by altering estrogen and stimulating follicular growth to produce a mature ovum 2) infertility problem, but clomiphene citrate does not affect it 3) infertility problem, but clomiphene citrate does not affect it 4) not a desired effec

The child is in the early stages of nephrotic syndrome. The nurse discusses which dietary change with the parents? 1. Adequate protein, low sodium intake. 2. Low protein, low potassium intake. 3. Low potassium, low calorie intake. 4. Limited protein, high carbohydrate intake.

Strategy: Think about each answer. 1) CORRECT — if child can tolerate the protein intake, then this diet is encouraged to speed healing; sodium is usually restricted 2) low protein contraindicated in clients with kidney disease 3) does not address protein need at all 4) may be appropriate only if the child cannot tolerate protein intake

The health care provider inserts a temporary pacemaker in a client following a myocardial infarction. The nurse knows that which outcome is the primary purpose of the pacemaker? 1. Increases the force of myocardial contraction. 2. Increases the cardiac output. 3. Prevents premature ventricular contractions (PVCs). 4. Prevents systemic overload.

Strategy: Think about each answer. 1) action of cardiac glycosides such as digoxin 2) CORRECT — acts to regulate cardiac rhythm 3) action of antiarrhythmics such as quinidine 4) action of diuretics such as furosemide

The nurse knows that cortisol is responsible for which action? 1. Preparing the body for "flight or fight." 2. Regulating the calcium metabolism. 3. Converting proteins and fat into glucose. 4. Enhancing musculoskeletal activity.

Strategy: Think about each answer. 1) action of epinephrine 2) action of parathyroid hormone parathormone 3) CORRECT — action of cortisol; is also an anti-inflammatory agent 4) action of norepinephrine

The nurse cares for the postoperative client diagnosed with type 2 diabetes controlled with oral antihyperglycemic agents. The client asks why the health care provider ordered subcutaneous insulin injections after surgery. The nurse's response is based on knowing which physiological process? 1. Tissue injury after surgery decreases blood glucose. 2. Anesthesia acts to increase glycogen stores. 3. Being NPO inhibits normal blood glucose control. 4. Surgery often leads to insulin dependency.

Strategy: Think about each answer. 1) inaccurate 2) inaccurate 3) CORRECT - temporary control by insulin is needed due to inability to control diabetes mellitus by diet and oral agents, surgically induced metabolic changes, being NPO both before and after surgery, and the infusion of intravenous fluids 4) inaccurate

The nurse on a psychiatric unit of the hospital declines the client's request to organize a party on the unit for the client's friends. The client becomes angry and uses abusive language toward the nurse. Which statement indicates the nurse has an understanding of the client's behavior? 1. Allowing the client to use abusive language will undermine the authority of the nurse. 2. Responding in kind to a client who uses abusive language will perpetuate the behavior. 3. Abusive language is one of the behaviors symptomatic of the client's illness. 4. The nurse should model acceptable behavior and language for all clients.

Strategy: Think about each answer. 1) inaccurate; doesn't undermine authority of staff 2) shows lack of understanding of cause for client's behavior 3) CORRECT — symptoms will respond to treatment 4) suggests that using acceptable language will change client's behavior; shows lack of understanding of client's behavior

The health care provider orders mannitol for the client with a closed head injury. Which response does the nurse recognize as desired to this medication? 1. The blood pressure increases to 150/90. 2. Urinary output increases to 175 mL/hour. 3. There is a decrease in the level of activity. 4. There is an absence of fine tremors of the fingers.

Strategy: Think about each answer. 1) increase in blood pressure is not desired 2) CORRECT — mannitol is an osmotic diuretic; increases urinary output and decreases intracranial pressure 3) does not indicate desired effect of medication 4) does not indicate desired effect of medication

A postoperative cataract client is cautioned about not making sudden movements or bending over. The nurse understands that the rationale for this recommendation is to prevent which complication? 1. Impairment of cerebral blood flow and headaches. 2. Increased intracranial pressure. 3. Pressure on the ocular suture line. 4. Displacement of the lens implant.

Strategy: Think about each answer. 1) not relevant to this situation 2) not relevant to this situation 3) CORRECT—sudden changes in position, constipation, vomiting, stooping, or bending over increase the intraocular pressure and put pressure on the suture line 4) occurs because of pressure on suture area; not all clients have lens implants; answer choice 3 is a more comprehensive answer

The parent of a child with chickenpox asks the clinic nurse why the child will not come down with chickenpox again if exposed to the virus at school at a later date. Which explanation does the nurse give? 1. Natural passive immunity occurs because the child receives antibodies from outside the body. 2. Artificial active immunity occurs because the child receives specific antigens against the chickenpox virus. 3. Natural active immunity occurs because the child's body actively makes antibodies against the chickenpox virus. 4. Artificial passive immunity occurs because of the inflammatory process of chickenpox.

Strategy: Think about each answer. 1) occurs when antibodies are passed from mother to fetus via placenta, colostrum, and breast milk 2) small amounts of specific antigens are used for vaccination; body responds by actively making antibodies 3) CORRECT - antigen enters the body without human assistance; body responds by actively making antibodies 4) involves injection with antibodies that were produced in another person or animal; used to protect person exposed to serious disease

The nurse cares for the client receiving a blood transfusion for approximately 30 minutes. Which symptom indicates a severe allergic reaction is occurring? 1. Bounding peripheral pulses. 2. Chills. 3. Respiratory wheezing. 4. Lower back discomfort.

Strategy: Think about each answer. 1) seen with circulatory overload; severe anaphylactic reaction may cause hypotension 2) indicative of a hemolytic or febrile transfusion reaction 3) CORRECT — allergic reaction is characterized by wheezing, urticaria (hives), facial flushing, and epiglottal edema 4) indicative of a hemolytic transfusion reaction

The parents of a child diagnosed with hemophilia ask the nurse to explain the cause of the disease. Which response by the nurse is best? 1. "The father transmits the gene to the son." 2. "Both the mother and the father carry a recessive trait." 3. "The mother transmits the gene to her son." 4. "There is a 50% chance that the mother will pass the trait to each of the daughters."

Strategy: Think about each statement. Is it true? 1) affected male inherits gene from the mother and can transmit it only to the daughters 2) it is not an autosomal recessive trait 3) CORRECT — hemophilia is a sex-linked disorder 4) there is a 50% chance the mother will pass the trait to each of her children

Which symptoms might alert the nurse to consider an alcohol problem in a client hospitalized for a physical illness? Select all that apply. 1. Tremors. 2. Elevated temperature. 3. Depression. 4. Nocturnal leg cramps. 5. Night sweats. 6. Decreased concentration.

Strategy: Think about symptoms of withdrawal from alcohol. 1) CORRECT - Symptom of withdrawal. 2) CORRECT - Symptom of withdrawal. 3) Seen in a depressed client 4) CORRECT - Symptom of withdrawal. 5) Seen in clients with tuberculosis, leukemia, or other infections. 6) Seen in a depressed client.

The 6-month-old is brought to the clinic for a well-baby checkup. During the exam, the nurse expects to observe which assessment findings? 1. A pincer grasp. 2. Sitting with support. 3. Tripling of the birth weight. 4. Presence of the posterior fontanelle 5. Playing peek-a-boo. 6. Rolling from back to abdomen.

Strategy: Think of behaviors of a 6-month-old child. 1) Present at 9 months of age. 2) CORRECT - Should occur at this age. 3) Should happen at 1 year. 4) Posterior fontanelle closes at 2-3 months of age. 5) CORRECT - Should be present at this time. 6) CORRECT - Should be able to do this.

Labs of hemophilia?

Thrombin Time - measures fibrinogen PT (11-15 sec)- decrease indicates deficiency of 2,5,7,10 PTT or APTT (25-35)- Assesses for factors 12,11,9,8

The soft spot on the head of the 4 day old feels slightly elevated when asleep

bulging fontanelle may indicate increased intracranial pressure and is most serious

The circumcision site of the 3 day old is slightly swollen

circumcision should have yellowish exudate at this time, but swelling is not normal and may interfere with urination

When bed is bumped, a 2-day-old rapidly extends the extremities

describes the Moro Reflex and is normal

The umbilical cord of the 5 day old is soft and draining exudate

umbilical cord should be dry and hard; draining indicates a possible infection and needs to be assessed

During the mother's fourth stage of labor, the nurse palpates the client's fundus in which location?

uterus is normally contracted and palpable at the umbilicus


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