Mesh : Biomarkers / blood Collagen Type IV / blood Disseminated Intravascular Coagulation Down Syndrome / blood complications diagnosis Fatal Outcome Female Fetal Diseases / blood diagnosis Humans Hyaluronic Acid / blood Infant, Newborn Liver Cirrhosis / blood diagnosis etiology Liver Failure / blood diagnosis etiology Male Myelopoiesis Peptide Fragments / blood Pregnancy Procollagen / blood Prognosis

来  源:   DOI:10.1080/08880010490277088   PDF(Sci-hub)

Abstract:
The authors recently encountered a lethal case of Down syndrome with transient abnormal myelopoiesis (TAM). Although the peripheral white blood cell count and blast cells had improved without specific treatment, the patient died of severe coagulopathy due to liver fibrosis when he was 5 years old. The prognosis of TAM with liver fibrosis was poor. The patient had high levels of N-terminal peptide of III procollagen, type IV collagen, and hyaluronic acid. These serum makers are noninvasive indicators of liver fibrosis and may be useful as prognostic indicators of TAM in Down syndrome.
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