sickle cell disease Flashcards
Prophylactic cholecystectomy for asymptomatic gallstones is indicated for patients with which one of the following? (check one)
Sickle cell disease
A renal transplant
Diabetes mellitus
Cirrhosis
Sickle cell disease
Asymptomatic gallstones are not usually an indication for prophylactic cholecystectomy, as most patients remain asymptomatic throughout their lives, and only 1%–4% develop symptoms or complications from gallstones each year. Only 10% of patients found to have asymptomatic gallstones develop symptoms within the first 5 years after diagnosis, and only 20% within 20 years.
In the past, cholecystectomy was recommended for diabetic patients with asymptomatic gallstones, based on the assumption that autonomic neuropathy masked the pain and signs associated with acute cholecystitis, and that patients would therefore develop advanced disease and more complications. More recent evidence has shown that these patients have a lower risk of major complications than previously thought.
Prophylactic cholecystectomy is not recommended in renal transplant patients with asymptomatic gallstones. One study found that 87% of these patients remained asymptomatic after 4 years, with only 7% developing acute cholecystitis and requiring subsequent uncomplicated laparoscopic cholecystectomy. Other studies have shown that the presence of gallstone disease does not negatively affect graft survival.
Patients with hemoglobinopathies are at a significantly increased risk for developing pigmented stones. Gallstones have been reported in up to 70% of sickle cell patients, up to 85% of hereditary spherocytosis patients, and up to 24% of thalassemia patients. In sickle cell patients, complications from asymptomatic gallstones have been reported to be as high as 50% within 3–5 years of diagnosis. This has been attributed largely to the diagnostic challenge associated with symptomatic cholelithiasis versus abdominal sickling crisis. In the past these patients were managed expectantly because of the significant morbidity and mortality associated with open operations. The operative risk for these patients (especially sickle cell patients) has been lowered by laparoscopic cholecystectomy, along with improved understanding of preoperative hydration and transfusion, improved anesthetic technique, and better postoperative care. Prophylactic laparoscopic cholecystectomy in these patients prevents future diagnostic confusion, as well as the mortality and morbidity risk associated with emergency surgery.
Furthermore, cholecystectomy can and should be performed at the time of splenectomy, whether open or laparoscopic.
Studies have shown no significant differences in progression to symptoms from silent gallstones in cirrhotic patients compared with noncirrhotic patients. Expectant management is therefore recommended in patients with cirrhosis.
A 2-year-old African-American male with a history of sickle cell disease is brought to your office for a well child check. Which one of the following would be most appropriate for screening at this time? (check one)
A chest radiograph
A DXA scan
Abdominal ultrasonography
Renal Doppler ultrasonography
Transcranial Doppler ultrasonography
Transcranial Doppler ultrasonography
Individuals with sickle cell disease are at increased risk for vascular disease, especially stroke. All sickle cell patients 2–16 years of age should be screened with transcranial Doppler ultrasonography (SOR A). A chest radiograph, abdominal ultrasonography, a DXA scan, and renal Doppler ultrasonography are not recommended for screening patients with sickle cell disease.
