AHA Coding Clinic® for ICD-10-CM and ICD-10-PCS - 2023 Issue 4; New/Revised ICD-10-CM Codes

Shwachman-Diamond Syndrome

A new code was created to identify Shwachman-Diamond syndrome (D61.02). Shwachman-Diamond syndrome (SDS) is a rare, genetic condition with bone marrow failure, characterized by poor growth due to difficulty absorbing food, a low number of white blood cells, and in some cases, skeletal abnormalities. Symptoms in infants born with SDS typically appear by four to six months of age. Children with SDS have a higher chance of developing blood disorders such as leukemia or myelodysplastic syndrome. Nearly five percent of children with SDS will develop leukemia, with the risk growing to 25 percent by adulthood. In addition...

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