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Pediatrics Blood 1ce52cb8

Painful "hand - foot syndrome" is a manifestation of:

A
Malaria
B
Sickle cell Anemia
C
Autoimmune haemolytic anemia
D
Idiopathic Thrombocytopenic purpura
High-Yield Explanation
Ans. B. Sickle cell AnemiaSickle cell disease (SCD) and its variants are genetic disorders resulting from the presence of a mutated form of hemoglobin, hemoglobin S (HbS) caused by a single amino acid substitution within the hemoglobin A (HbA) gene, creating the abnormal HbS. Patients with sickle cell anemia can present with sickle cell crisis which can be of three types: vaso occlusive, sequestration and aplastic. Vaso occlusive crisis are painful episodes due to intravascular sickling and tissue anoxia, predominantly affecting bones, lung liver, spleen, brain and penis. Patients develop throbbing bony pain, sometimes accompanied by local tenderness, erythema, warmth and swelling. These attacks often get precipitated following infections, climatic changes and psychological stress. In small children under 5 years, hands and feet bones are often affected. Painful "hand-foot syndrome" may be the first clinical manifestation of sickle cell disease.

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