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. Author manuscript; available in PMC: 2013 Aug 1.
Published in final edited form as: Pediatr Blood Cancer. 2012 Apr 19;59(2):365–371. doi: 10.1002/pbc.24178

Table III.

Potential Indications and Strength of Recommendation* for Hydroxyurea Therapy in Children with HbSS

Category Indication Strength of Recommendation
Acute vaso-occlusive Frequent Painful Events
Dactylitis
Acute Chest Syndrome
Strong
Strong
Moderate
Laboratory markers of severity Low Hb Concentration
Low HbF
Elevated WBC
Elevated LDH
Weak
Weak
Weak
Weak
Organ Dysfunction
     Brain


     Lungs

     Renal

Elevated TCD velocities
Silent MRI, MRA changes
Stroke prophylaxis

Moderate
Weak
Moderate
Hypoxemia
Comorbid Asthma
Weak
Weak
Proteinuria Weak
Miscellaneous Sibling on hydroxyurea
Parental Request
Weak
Moderate

Hb indicates hemoglobin; HbF, hemoglobin F; WBC, white blood cell count; LDH, lactate dehydrogenase; TCD, transcranial Doppler ultrasound; MRI, magnetic resonance imaging; MRA, magnetic resonance angiography;

*

Strength of recommendation based on published data and expert opinion when data are unavailable (sibling on hydroxyurea, parental request)