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Discuss Iron deficiency anemia, thalassemia syndromes and sickle cell anemia

A four-year-old African American male diagnosed with sickle cell disease in the newborn period was admitted to the hospital with abdominal pain. Two days prior to admission, he was seen in the emergency room for abdominal pain and sent out on pain medicine.
Height 100 cm (25th percentile on growth chart)
Weight 15 kg (25th percentile on growth chart)
Temperature: 38.9ºC
Heart Rate: 135
Respiratory Rate: 40
Blood Pressure 100/60 mmHg
Oxygen Saturation Level: 87% (normal range: 92%-98%)

HEENT: Normocephalic, pupils reactive, tympanic membranes clear, oropharynx clear
Neck: No adenopathy
Chest: Mild subcostal retractions. Audible rales at lung bases.
Heart: Tachycardic with III/VI murmur
Abdomen: Mild distension, diffusely tender to palpation
Genitourinary: Circumcised male, no priapism
Extremities: Warm
Neurologic: Crying, alert boy. Face was symmetric. Moved all extremities.

Patient Value Normal Value
WBC 15,000 4,000-12,000/μL
HGB 6.3 11.5-13.5 g/dL
HCT 18 34%-40%
PLT 560,000 140,000-440,000/μL
MCV 89.0 75-87 fl
Retic % 14% 0.5%-1.5%
Rectic Absolute 0.2125 0.024-0.084 M/μL

1 What history, including symptoms, would be most helpful in evaluating this patient?
2 What does a prior history of abdominal pain reflect? What does Bone pain and swollen, painful fingers (dactylitis) reflect in this disease?
3 He had a temperature of 101 degrees Fahrenheit yesterday.what does that indicate?
4 He has been coughing 2-3 times a day and intermittently through the night.what does that indicate??
5 Does family history indicate sickle disease?
6 What additional physical findings might occur in patients with sickle cell disease? Discuss Jaundice and Splenomagaly??
7 What other labs would you request? Compare the lab findings with normal ranges
Discuss Peripheral smear, hemoglobin electrophoresis,
Blood culture; Blood Type and screen for antibodies
LDH; Haptoglobin levels
Amylase and Lipase
8 Discuss MCV, MCH, RDW, ESR, Hematocrit and red cell morphology in this disease
9 What was your differential diagnosis when you first saw the patient? Discuss the following in diagnosing the disease
Cholecystitis; Pneumonia; Upper respiratory tract infection; Vaso-occlusive pain crisis
10 what are the differences between sickle cell disease, HSBC, HbE and Tallahassee diseases?
11 Discuss Iron deficiency anemia, Tallahassee syndromes and sickle cell anemia
12 How would you treat this patient?


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