Data Availability StatementNot applicable. research. XL184 free base pontent inhibitor In relation to the first point we think that the number of hospitalizations for vaso-occlusive crisis (VOC), days of hospitalization per year and number of Acute Chest Syndrome (ACS) during life are the best modality to identify MSH6 patients with worst outcomes. On the other hand, VOC and ACS represent the most frequent complications in sickle cell disease [6C8]. The number of severe episodes of VOC (requiring hospitalization) and of ACS drives the clinicians XL184 free base pontent inhibitor in the decision to start the therapy with hydroxyurea (HU) or continue the close follow-up of these patients [9]. We reported an inverse correlation between number and day of hospitalizations per year and fetal hemoglobin levels with intake of carbohydrates, lipids, iron, phosphorus, vitamins B1, and B2, but with these data we dont want to suggest a beneficial effect of low intake of these nutritional elements for these patients. We would XL184 free base pontent inhibitor like to highlight that these data were unexpected even for us and further studies with more patients are needed to clarify the relationship between nutritional intake and fetal hemoglobin irrespectively from HU therapy. We are aware that HU is the only medication approved by the U.S. Food and Drug Administration and EMA for SCD and that there is a strong evidence to support the efficacy and the cost effectiveness of using HU in patients with SCD even from the first years of life [10C12]. Accordingly, many patients in follow-up in our center XL184 free base pontent inhibitor are treated with HU for its beneficial effects on SCD. Abbreviation SCD, sickle cell disease Acknowledgements None. Funding No funding sources to disclose. Availability of data and materials Not applicable. Authors contribution SB wrote the manuscript. Authors information SB primary area of interest clinically is usually sickle cell disease and his major research focus relates to health services and outcomes research. Competing interests The author declares that she has no competing interests. Consent for XL184 free base pontent inhibitor publication Not applicable. Ethics approval and consent to participate Not applicable..