Unique Hemoglobinopathy Pattern Following Treatment with Voxelotor.

OBJECTIVE: Voxelotor, a FDA-approved drug for the treatment of patients with sickle cell disease (SCD), inhibits hemoglobin S (HbS) polymerization and increases total hemoglobin via hemolysis reduction. This drug has shown unique patterns in hemoglobin fractionation, affecting its interpretation. We aimed to evaluate whether these voxelotor-induced changes can be linked to improvement of hemolysis markers in pediatric patients on voxelotor. METHODS: A total of 15 patients (age 12 to 20 years; 40% females) on voxelotor were evaluated to compare changes in the hemoglobin fractionation by capillary electrophoresis, total hemoglobin, reticulocyte percentage (retic%), lactate dehydrogenase (LDH), and bilirubin measurements before and after the recorded date of voxelotor prescription. RESULTS: Hemoglobin fractionation showed changes in the profile of 60% (9/15) of the patients studied. Out of the 9 patients for which voxelotor showed changes in the hemoglobin fractionation, 44% (4/9) had an increase of >1 g/dL in their total hemoglobin after voxelotor treatment was started. Assessment of other hemolysis markers available showed decreased LDH (4/4), retic % (6/8), and bilirubin (3/4). CONCLUSIONS: Unique pattern of hemoglobin fractionation analysis following therapy with voxelotor has potential as a tool for the assessment of response and/or compliance to voxelotor for the treatment of SCD.

Managing Patient With Coexisting Emphysematous Pyelonephritis And Cystitis.

Emphysematous pyelonephritis (EPN) is a type of critical renal infection having dire consequences at times. It is said to be result of gases produced inside renal parenchymal tissue or pelvicalyceal system. Rarely coexistence of emphysematous pyelonephritis and emphysematous cystitis (EC) may lead to an intimidating condition in case it is not dealt with swiftly. The resent case report narrates the management of a 45-year-old female patient who suffered from EPN with concomitant EC. Right-sided emergency percutaneous nephrostomy was passed. Afterwards, Double J stent was passed under general anaesthesia. Although she had an initial improvement clinically but later on due to recurrent urinary tract infections and non-resolving right kidney abscess and fever right sided nephrectomy was done. This is a very rare presentation and has not previously reported much in literature.