TY - JOUR
T1 - Hereditary spherocytosis
AU - Mahajan, Vasudha
AU - Jain, Sunil
PY - 2016
Y1 - 2016
N2 - Neonatal jaundice is commonly encountered in the neonatal period. Although it is mostly asymptomatic, severe cases may present as encephalopathy or kernicterus. Hereditary spherocytosis (HS) is the most common cause of nonimmune hemolytic anemia and the third most common cause of kernicterus after glucose-6-phosphate-dehydrogenase deficiency and ABO isoimmunization. Awareness of the clinical presentation and the laboratory tests necessary to diagnose the disease can lead to early detection and effective interventions, thereby preventing adverse outcomes. This article focuses on HS: its pathophysiology, clinical presentation in the neonatal period, natural history, and the relevant diagnostic features of evaluation.
AB - Neonatal jaundice is commonly encountered in the neonatal period. Although it is mostly asymptomatic, severe cases may present as encephalopathy or kernicterus. Hereditary spherocytosis (HS) is the most common cause of nonimmune hemolytic anemia and the third most common cause of kernicterus after glucose-6-phosphate-dehydrogenase deficiency and ABO isoimmunization. Awareness of the clinical presentation and the laboratory tests necessary to diagnose the disease can lead to early detection and effective interventions, thereby preventing adverse outcomes. This article focuses on HS: its pathophysiology, clinical presentation in the neonatal period, natural history, and the relevant diagnostic features of evaluation.
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U2 - 10.1542/neo.17-12-e697
DO - 10.1542/neo.17-12-e697
M3 - Article
AN - SCOPUS:85006008561
VL - 17
SP - e697-e704
JO - Pediatrics in Review
JF - Pediatrics in Review
SN - 0191-9601
IS - 12
ER -