Cancers, Vol. 16, Pages 1420: Growth Charts for Shwachman & ndash;Diamond Syndrome at Ages 0 to 18 Years

Cancers, Vol. 16, Pages 1420: Growth Charts for Shwachman–Diamond Syndrome at Ages 0 to 18 Years Cancers doi: 10.3390/cancers16071420 Authors: Anna Pegoraro Valentino Bezzerri Gloria Tridello Cecilia Brignole Francesca Lucca Emily Pintani Cesare Danesino Simone Cesaro Francesca Fioredda Marco Cipolli Shwachman–Diamond syndrome (SDS) is one of the most common inherited bone marrow failure syndromes. SDS is characterized by hypocellular bone marrow, with a severe impairment of the myeloid lineage, resulting in neutropenia, thrombocytopenia, and, more rarely, anemia. Almost 15% of patients with SDS develop myelodysplastic syndrome or acute myeloid leukemia as early as childhood or young adulthood. Exocrine pancreatic insufficiency is another common feature of SDS. Almost all patients with SDS show failure to thrive, which is associated with skeletal abnormalities due to defective ossification. Considering these observations, it remains unfeasible to use the common growth charts already available for the general population. To address this issue, we report how we drew up growth charts of patients with SDS aged 0 to 18 years. We analyzed height, weight, and body max index (BMI) in 121 Italian patients with SDS. Results indicated that the 50th and 3rd percentiles of weight and height of the pediatric general population correspond to the 97th and 50th percentiles of patients with SDS aged 0–18 years, respectively. In ad...
Source: Cancers - Category: Cancer & Oncology Authors: Tags: Communication Source Type: research