A ten-year-old boy is brought to clinic by his mother who states that the boy has been listless and not eating. She also notes that he has been easily bruising without trauma as he says he is too tired to go out and play. He says his bones hurt sometimes. Mother states the child has had intermittent fevers that respond to acetaminophen. Maternal history negative for pre, intra, or post-partum problems. Child’s past medical history negative and he easily reached developmental milestones. Physical exam reveals a thin, very pale child who has bruises on his arms and legs in no particular pattern. The APRN orders complete blood count (CBC), and complete metabolic profile (CMP). The CBC revealed Hemoglobin of 6.9/dl, hematocrit of 19%, and platelet count of 80,000/mm3. The CMP demonstrated a blood urea nitrogen (BUN) of 34m g/dl and creatinine of 2.9 mg/dl. The APRN recognizes that the patient appears to have acute leukemia and renal failure and immediately refers the patient to the Emergency Room where a pediatric hematologist has been consulted and is waiting for the boy and his mother. The diagnosis of acute lymphoblastic leukemia (ALL) was made after extensive testing.
Question 1 of 2:
What is ALL? —
QUESTION 2
A ten-year-old boy is brought to clinic by his mother who states that the boy has been listless and not eating. She also notes that he has been easily bruising without trauma as he says he is too tired to go out and play. He says his bones hurt sometimes. Mother states the child has had intermittent fevers that respond to acetaminophen. Maternal history negative for pre, intra, or post-partum problems. Child’s past medical history negative and he easily reached developmental milestones. Physical exam reveals a thin, very pale child who has bruises on his arms and legs in no particular pattern. The APRN orders complete blood count (CBC), and complete metabolic profile (CMP). The CBC revealed Hemoglobin of 6.9/dl, hematocrit of 19%, and platelet count of 80,000/mm3. The CMP demonstrated a blood urea nitrogen (BUN) of 34m g/dl and creatinine of 2.9 mg/dl. The APRN recognizes that the patient appears to have acute leukemia and renal failure and immediately refers the patient to the Emergency Room where a pediatric hematologist has been consulted and is waiting for the boy and his mother. The diagnosis of acute lymphoblastic leukemia (ALL) was made after extensive testing.
Question 2 of 2:
How does renal failure occur in some patients with ALL?
QUESTION 3
A 12-year-old female with known sickle cell disease (SCD) present to the Emergency Room in sickle cell crisis. The patient is crying with pain and states this is the third acute episode she has had in the last nine months. Both parents are present and appear very anxious and teary eyed. A diagnosis of acute sickle cell crisis was made. Appropriate therapeutic interventions were initiated by the APRN and the patient’s pain level decreased, and she was transferred to the pediatric intensive care unit (PICU) for observation and further management.
Question 1 of 2:
What is the pathophysiology of acute SCD crisis and why is pain the predominate feature of acute crises?
QUESTION 4
A 12-year-old female with known sickle cell disease (SCD) present to the Emergency Room in sickle cell crisis. The patient is crying with pain and states this is the third acute episode she has had in the last nine months. Both parents are present and appear very anxious and teary eyed. A diagnosis of acute sickle cell crisis was made. Appropriate therapeutic interventions were initiated by the APRN and the patient’s pain level decreased, and she was transferred to the pediatric intensive care unit (PICU) for observation and further management.
Discuss the genetic basis for SCD.
QUESTION 5
The parents of a 9-month boy bring the infant to the pediatrician’s office for evaluation of a swollen right knee and excessive bruising. The parents have noticed that the baby began having bruising about a month ago but thought the bruising was due to the child’s attempts to crawl. They became concerned when the baby woke up with a swollen knee. Infant up to date on all immunizations, has not had any medical problems since birth and has met all developmental milestones. Pre-natal, intra-natal, and post-natal history of mother noncontributory. Family history negative for any history of bleeding disorders or other major genetic diseases. Physical exam within normal limits except for obvious bruising on the extremities and right knee. Knee is swollen but no warmth appreciated. Range of motion of knee limited due to the swelling. The pediatrician suspects the child has hemophilia and orders a full bleeding panel workup which confirms the diagnosis of hemophilia A.
Explain the genetics of hemophilia.
QUESTION 6
The parents of a 9-month boy bring the infant to the pediatrician’s office for evaluation of a swollen right knee and excessive bruising. The parents have noticed that the baby began having bruising about a month ago but thought the bruising was due to the child’s attempts to crawl. They became concerned when the baby woke up with a swollen knee. Infant up to date on all immunizations, has not had any medical problems since birth and has met all developmental milestones. Pre-natal, intra-natal, and post-natal history of mother noncontributory. Family history negative for any history of bleeding disorders or other major genetic diseases. Physical exam within normal limits except for obvious bruising on the extremities and right knee. Knee is swollen but no warmth appreciated. Range of motion of knee limited due to the swelling. The pediatrician suspects the child has hemophilia and orders a full bleeding panel workup which confirms the diagnosis of hemophilia A.
Question 2 of 2:
Briefly describe the pathophysiology of Hemophilia.
QUESTION 7
During a routine 16-week pre-natal ultrasound, spina bifida with myelomeningocele was detected in the fetus. The parents continued the pregnancy and labor was induced at 38 weeks with the birth of a female infant with an obvious defect at Lumbar Level 2. The Apgar Score was 7 and 9. The infant was otherwise healthy. The sac was leaking cerebral spinal fluid and the child was immediately taken to the operating room for coverage of the open sac. The infant remained in the neonatal intensive care unit (NICU) for several weeks then discharged home with the parents after a prescribed treatment plan was developed and the parents were educated on how to care for this infant.
What is the underlying pathophysiology of myelomeningocele?
QUESTION 8
During a routine 16-week pre-natal ultrasound, spina bifida with myelomeningocele was detected in the fetus. The parents continued the pregnancy and labor was induced at 38 weeks with the birth of a female infant with an obvious defect at Lumbar Level 2. The Apgar Score was 7 and 9. The infant was otherwise healthy. The sac was leaking cerebral spinal fluid and the child was immediately taken to the operating room for coverage of the open sac. The infant remained in the neonatal intensive care unit (NICU) for several weeks then discharged home with the parents after a prescribed treatment plan was developed and the parents were educated on how to care for this infant.
Question 2 of 2:
Describe the pathophysiology of hydrocephalus in infants with myelomeningocele.
QUESTION 9
A preterm infant was delivered at 32 weeks gestation and was taken to the NICU for critical care management. Physical assessment of the chest and heart remarkable for a continuous-machinery type murmur best heard at the left upper sternal border through systole and diastole. The infant had bounding pulses, an active precordium, and a palpable thrill. The infant was diagnosed with a patent ductus arteriosus (PDA).
Discuss the hemodynamic consequences of a PDA.
QUESTION 10
A 7-year-old male was referred to the school psychologist for disruptive behavior in the classroom. The parents told the psychologist that the boy has been difficult to manage at home as well. His scholastic work has gotten worse over the last 6 months and he is not meeting educational benchmarks. His parents are also worried that he isn’t growing like the other kids in the neighborhood. He has been bullied by other children which is contributing to his behaviors. The psychologist suggests that the parents have some blood work done to check for any abnormalities. The complete blood count (CBC) revealed a hypochromic microcytic anemia. Further testing revealed the child had a venous lead level of 21 mcg/dl (normal is
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