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Pediatric disorders can present unique challenges to patients, families, and healthcare providers. Disorders in these areas are complicated by the fact that young patients can have difficulties communicating symptoms. Furthermore, the manner in which disease and disorders manifest in children may be unique.

APRNs working to support these patients and their loved ones must demonstrate not only support and compassion but the expertise to communicate and guide understanding of diagnoses and treatment plans. This includes an understanding of disease and disorders at the pediatric level.

This week, you examine pathophysiology in pediatrics. You apply key terms, concepts, and principles in this area to demonstrate an understanding of the impact they have on altered physiology in children.

QUESTION 1


Scenario 1: Acute Lymphoblastic Leukemia (ALL)

An 11-year-old boy is brought to the clinic by his parents who states that the boy has not been eating and listless. The mother 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.

PMH: Negative. Easily reached developmental milestones. 

PE: reveals a thin, very pale child who has bruises on his arms and legs in no particular pattern.

LABS: 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.

DIAGNOSIS: 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 parents.

CONFIRMED DX: acute lymphoblastic leukemia (ALL) was made after extensive testing. 

Question

     Explain what ALL is?  

QUESTION 2


Scenario 1: Acute Lymphoblastic Leukemia (ALL)

An 11-year-old boy is brought to the clinic by his parents who states that the boy has not been eating and listless. The mother 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.

PMH: Negative. Easily reached developmental milestones. 

PE: reveals a thin, very pale child who has bruises on his arms and legs in no particular pattern.

LABS: 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.

DIAGNOSIS: 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 parents.

CONFIRMED DX: acute lymphoblastic leukemia (ALL) was made after extensive testing. 

Question

 Why does ARF occur in some patients with ALL? 

QUESTION 3


Scenario 2: Sickle Cell Disease (SCD)

A 15-year-old male with known sickle cell disease (SCD) present to the ER in sickle cell crisis. The patient is crying with pain and states this is the third acute episode he has had in the last 10-months. Both parents are present and appear very anxious and teary eyed. A diagnosis of acute sickle cell crisis was made. 

Question

  Explain the pathophysiology of acute SCD crisis. Why is pain the predominate feature of acute crises?  

QUESTION 4


Scenario 2: Sickle Cell Disease (SCD)

A 15-year-old male with known sickle cell disease (SCD) present to the ER in sickle cell crisis. The patient is crying with pain and states this is the third acute episode he has had in the last 10-months. Both parents are present and appear very anxious and teary eyed. A diagnosis of acute sickle cell crisis was made. 

Question

 Discuss the genetic basis for SCD.

QUESTION 5


Scenario 3: Hemophilia

8-month infant is brought into the office due to a swollen right knee and excessive bruising. The parents have noticed bruising about a month ago but thought the bruising was due to the 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. 

FH: negative for any history of bleeding disorders or other major genetic diseases. 

PE: 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.

DIAGNOSIS: hemophilia A.  

Question

 What is the pathophysiology of Hemophilia 

READING RESOURCES

McCance, K. L. & Huether, S. E. (2019). Pathophysiology: The biologic basis for disease in adults and children (8th ed.). St. Louis, MO: Mosby/Elsevier.

· Chapter 14: Cancer in Children, including Summary Review

· Chapter 20: Alterations of Neurologic Function in Children (stop at Childhood tumors); Summary Review

· Chapter 34: Alterations of Cardiovascular Function in Children (stop at Defects decreasing pulmonary blood flow); Summary Review

· Chapter 37: Alterations of Pulmonary Function in Children (stop at Congenital malformations); Summary Review

· Chapter 40: Alterations of Renal and Urinary Tract Function in Children, including Summary Review

· Chapter 43: Alterations of Digestive Function in Children, including Summary Review

· Chapter 46: Alterations of Musculoskeletal Function in Children (stop at Avascular diseases); (start at Cerebral palsy) (musculoskeletal tumors in children); Summary Review

· Chapter 48: Alterations of the Integument in Children, including Summary Review

· Chapter 50: Shock, Multiple Organ Dysfunction Syndrome, and Burns in Children, including Summary Review

U.S. National Library of Medicine. (2019). Normal growth and development. Retrieved from https://medlineplus.gov/ency/article/002456.htm