A 1-month-old infant with a strong family history of sickle-cell anemia is brought to the emergency room with an
incarcerated inguinal hernia. Which of the following should be carried out before surgery?
A. Sickle cell prep
B. Hemoglobin electrophoresis
C. Peripheral smear
D. Hematology consultation
E. None of the above
Answer
(E) At birth, the concentration of hemoglobin F (fetal hemoglobin) is about 80% and reaches its lowest level by 2 to 3 months of age. Sickle-cell anemia (hemoglobin SS) is an inherited disorder of the β-chain of the adult hemoglobin molecule caused by a single amino acid substitution. It has an incidence of about 0.2% in the African-American population, in contrast to the relatively benign heterozygous condition, sickle cell trait (hemoglobin AS), which affects 8% to 10% of the same group. Sickling can occur in homozygous patients who become hypoxic, acidotic, hypothermic, or dehydrated. The predominant hemoglobin in this 1-month-old infant is hemoglobin F, which would temporarily protect the infant from the manifestations of sickle-cell anemia were he or she homozygous for hemoglobin S. The patient should, however, be worked up for sickle-cell anemia at
some point in early life, but such a workup is not a prerequisite for surgery at 1 month of age (Miller: Anesthesia,ed 6, pp 1112-1113; Motoyama: Smith’s Anesthesia for Infants and Children, ed 7, pp 49, 397-398; Stoelting: Anesthesia and Co-Existing Disease, ed 5, pp 411-412).
incarcerated inguinal hernia. Which of the following should be carried out before surgery?
A. Sickle cell prep
B. Hemoglobin electrophoresis
C. Peripheral smear
D. Hematology consultation
E. None of the above
Answer
(E) At birth, the concentration of hemoglobin F (fetal hemoglobin) is about 80% and reaches its lowest level by 2 to 3 months of age. Sickle-cell anemia (hemoglobin SS) is an inherited disorder of the β-chain of the adult hemoglobin molecule caused by a single amino acid substitution. It has an incidence of about 0.2% in the African-American population, in contrast to the relatively benign heterozygous condition, sickle cell trait (hemoglobin AS), which affects 8% to 10% of the same group. Sickling can occur in homozygous patients who become hypoxic, acidotic, hypothermic, or dehydrated. The predominant hemoglobin in this 1-month-old infant is hemoglobin F, which would temporarily protect the infant from the manifestations of sickle-cell anemia were he or she homozygous for hemoglobin S. The patient should, however, be worked up for sickle-cell anemia at
some point in early life, but such a workup is not a prerequisite for surgery at 1 month of age (Miller: Anesthesia,ed 6, pp 1112-1113; Motoyama: Smith’s Anesthesia for Infants and Children, ed 7, pp 49, 397-398; Stoelting: Anesthesia and Co-Existing Disease, ed 5, pp 411-412).
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