Berkowitz’s – Section XIII: Cancer In Children

Cancer in Children

Eduard H. Panosyan, MD, and Joseph L. Lasky III, MD
CASE STUDY
A 10-year-old boy has a history of intermittent fevers of 102.2°F (39.0°C) for 1 month. For 2 days, he has experienced increasing shortness of breath with rapid respirations. His face is dusky and plethoric, and the veins in his neck are prominent. He has bilaterally enlarged cervical lymph nodes. The remainder of the examination is normal. His blood cell count is normal, but the erythrocyte sedimentation rate is 110. A chest radiograph reveals a large mediastinal mass.
Questions
1. What signs and symptoms are associated with malignant conditions in children?
2. What oncologic emergencies require immediate attention?
3. What factors correlate with the development of cancer in children?

Chronic Kidney Disease

Mark Hanudel, MD; Justine Bacchetta, MD, PhD; Walter Jimenez, MD; and Isidro B. Salusky, MD
CASE STUDY
During a consultation for diarrhea and dehydration, the pediatrician notes that a 7-year-old boy has growth retardation. His parents report a decreased appetite, a decreased level of physical activity, and bed-wetting, despite having been previously potty trained. His medical history is significant for multiple episodes of fever due to presumed ear infections during his first years of life and one episode of urinary tract infection, without further studies. After hydration, the physical examination reveals a pale and short patient (height 101 cm [,fifth percentile]) with blood pressure above the 99th percentile for age, gender, and height. Routine laboratory studies reveal anemia, a serum creatinine of 1.4 mg/dL, and 31 proteinuria.
Questions
1. How do you estimate renal function in children?
2. What are the relevant questions to ask about medical and family history in children who present with chronic kidney disease (CKD)?
3. What additional diagnostic tests should be performed to determine the etiology of the kidney disease?
4. What are the approaches to the management of children with CKD?

Diabetes Mellitus

Jennifer K. Yee, MD, and Catherine S. Mao, MD
CASE STUDY
A 10-year-old girl presents with a 3-week history of nocturnal polyuria. Her mother reports that her daughter, previously overweight, seems to have lost weight in the past 2 months after receiving nutrition education. Laboratory tests reveal that the girl’s serum sodium level is 130 mEq/L; potassium, 3.2 mEq/L; glucose, 324 mg/dL; and 11 ketones. Urinalysis reveals specific gravity of 1.025
and moderate glucose and ketones. Her height and weight are within normal ranges for her age, and the remainder of her physical examination is unremarkable.
Questions
1. What is the pathophysiology of type 1 and type 2 diabetes?
2. What are diagnostic criteria for differentiating type 1 and type 2 diabetes?
3. What are the objectives of therapeutic interventions in children with diabetes?
4. What diagnostic evaluations are used in ongoing management of diabetes?
5. What are the acute and chronic complications associated with diabetes?
6. What is the role of “tight glycemic control” in children and adolescents?

Juvenile Idiopathic Arthritis and Benign Joint Pains of Childhood

Miriam F. Parsa, MD, MPH, and Deborah McCurdy, MD
CASE STUDY
A 4-year-old white girl is evaluated for limp. Parents are unclear about the duration of her symptoms, although they believe knee complaints started after she was playing with her brother 3 months ago. Parents have observed the patient to walk “like her grandmother” every morning, with marked improvement in her gait approximately 1 hour after moving around. Her activity level has remained about the same, although the morning limp does limit her ability to keep up with her siblings. She has no history of rash, fever, weight loss, severe pain complaints, or other joint involvement. On physical examination, vital signs are normal, her left knee is swollen with a 20-degree flexion contracture, and the left leg is 1.5-cm longer than the right leg.
Questions
1. What findings are indicative of juvenile idiopathic arthritis (JIA)?
2. What is the differential diagnosis for monarthric arthritis?
3. Which laboratory tests are important in the diagnostic workup of a child with suspected JIA?
4. What are the most common complications of uncontrolled JIA?
5. What are the long-term outcomes for patients with JIA?
6. What types of agents are used in the treatment of JIA?

Autoimmune Connective Tissue Diseases

Deborah McCurdy, MD
CASE STUDY
A 14-year-old girl has a 1-month history of severe fatigue with difficulty sleeping and a non-restorative sleep, hand swelling, generalized aches, low-grade fever, weight loss, and facial and leg rashes. She has not felt well enough to go to school for several weeks and complains her fingers are stiff and she can’t type. On examination, she appears fatigued and does not look well. Vital signs show a temperature of 100.5oF (38.1oC), blood pressure of 138/84 mm Hg, pulse of 98 beats/min, and respiratory rate of 22 breaths/ min. Her eyes appear puffy and her ankles and feet are swollen. She has swelling over the joints of her fingers and is having difficulty with buttons.
Questions
1. What findings are indicative of systemic lupus erythematosus (SLE)?
2. How can the clinical history and laboratory evaluation assist in determining the diagnosis and treatment?
3. What are the long-term outcomes for patients with SLE?
4. What similar clinical findings are present in other autoimmune diseases?
5. What types of agents are used in the treatment of autoimmune diseases?

Nephrotic Syndrome

Elaine S. Kamil, MD
CASE STUDY
A 2-year-old boy is brought to the office because of abdominal distention. He has just recovered from a runny nose that lasted a week, with no fever or change in activity. His mother complains that his eyelids were very swollen that morning, and she says that his thighs look “fat.” She has noticed that he has fewer wet diapers. He has always been a healthy child, and his immunizations are up-to-date. The family has a history of asthma and allergic rhinitis. Physical examination shows an active 2-yearold boy. Head and neck examination is clear, except for a few shotty anterior cervical lymph nodes and some minimal periorbital edema. Chest examination reveals some decreased breath sounds at the bases. The abdomen is moderately distended; bowel sounds are active and a fluid wave is detectable. There is 21 pitting edema of the lower legs, extending up to the knees. The urine has a specific gravity of 1.030; pH 6; 41 protein; and trace, non-hemolyzed blood. Microscopic examination shows 4 to 6 red blood cells per high-power field and 10 to 20 hyaline and fine granular casts per low-power field.
Questions
1. What is the differential diagnosis of edema and ascites in previously healthy young children?
2. What criteria are used to determine if children with nephrotic syndrome require hospitalization or can be managed as outpatients?
3. What laboratory evaluation and therapy are instituted initially?
4. What are the important issues to address in parent education?
5. What is the prognosis of young children with nephrotic syndrome?

Seizures and Epilepsy

Kenneth R. Huff, MD
CASE STUDY
A 6-year-old boy is evaluated for unusual episodic behaviors. The previous week his mother was awakened by the boy’s brother and found her son lying in bed unresponsive and drooling, with his head and eyes averted to the right, his right arm slightly raised, and his body stiff. His face was jerking intermittently. When the paramedics arrived, the boy’s posturing and movements had stopped. After the event, he could speak but was somewhat incoherent. He was taken to the local emergency department, where his examination and mental status were normal. Screening blood and urine tests were normal, and he was discharged with instructions to see his pediatrician for further recommendations. His father remembers 2 or 3 other episodes of a somewhat different nature in the past month. These occurred as the boy was being put to bed. They involved some body stiffening and facial grimacing, with the mouth slightly open and the tongue twisted and deviated to one side. The child could not speak but appeared to be trying to talk. The episodes lasted 20 to 30 seconds. Afterward the boy was his usual self and could tell his father what had been said to him.
The child has had no intercurrent illnesses or abnormal behavior apart from these “spells,” and he has lost no abilities. A paternal cousin and grandfather had seizures during childhood but “grew out of them.” The examination is completely normal.
Questions
1. How likely is it that the episodic behavior represents a seizure?
2. What historical data about an event support the diagnosis of a seizure disorder?
3. How does an electroencephalogram help in classifying the type of seizure disorder?
4. What rationale should be used to formulate short-term and long-term treatment plans?

Pediatric Palliative Care: Principles and Practice

Kevin Madden, MD, and Rick Goldstein, MD
CASE STUDY
Jason is a 17-year-old boy with spastic quadriplegia, severe global developmental delays, a seizure disorder, cortical blindness, and dystonia. Since leaving the neonatal intensive care unit (NICU) at 3 months of age, he has lived in a skilled nursing facility and spends weekends at home with his family. He was born after 24 weeks’ gestation and had a turbulent NICU course. During that hospitalization, in accord with his neonatologists, he was treated for comfort and extubated due to his severe respiratory failure but managed to survive and breathe independently. Once beyond the newborn period, his medical course was fairly stable, notable for episodic respiratory decompensations requiring hospitalization and numerous orthopedic procedures. In the past 2 years, however, he has spent substantially more time in the intensive care unit due to increasing respiratory fragility. With so much time spent in the hospital and so little time when he is comfortable “at home,” his parents have begun questioning whether the intensive medical care he has been receiving translates into quality of life and whether they should begin to limit efforts at resuscitation. They do not know what it would mean to place limitations on his care and, whatever their choices, they do not want Jason to suffer.
Questions
1. What is palliative care?
2. What is involved in the practice of palliative care?
3. How do children understand death? How does their understanding vary with their age?
4. What role does a primary care pediatrician play in the palliative care of her patients?