Which of the following is true about congenital heart block in neonatal lupus erythematosus?
Correct Answer C:
In severe cases, surgical implantation of a pacemaker, along with the correction of structural abnormalities in the heart, may be necessary. Congenital heart block, an important manifestation of neonatal lupus erythematosus (NLE) that carries a poor prognosis, may merit preventive therapy in pregnancy during fetal cardiac development. It should be considered in pregnant women with systemic lupus erythematosus who are anti-SSA/Ro antibody positive and have previous children with congenital heart block. Congenital complete heart block is the most serious manifestation of NLE. NLE is responsible for 80 to 95 % of all cases of congenital complete heart block diagnosed in utero or in the neonatal period. NLE is a much less common cause of heart block presenting after the neonatal period.
A general management plan of pregnancy in mothers with systemic lupus erythematosus includes treatment of disease flares using drugs that are effective but also safe for the fetus. Such an approach may diminish or reduce the prevalence of complete heart block associated with NLE. Corticosteroids and some immunosuppressive drugs are sometimes used, but long-term outcome data in children exposed to immunosuppressive drugs in utero is lacking.
An 18-month-old infant is brought to the clinic because of pallor and irritability. Her mother says the infant's diet consists almost exclusively of whole milk, approximately 40 oz per day. On physical examination, the infant has a pulse of 160/min, respirations of 50/min, and normal heart sounds with a grade 2/6 systolic ejection murmur. Liver is palpable 3 cm below the right costal margin. Laboratory studies show:
Following appropriate evaluation, which of the following is the most appropriate treatment?
Correct Answer D:
Starting transfusion with packed erythrocytes is the most appropriate management because this patient has severe iron deficiency anemia. Pallor is the most important clue to iron deficiency anemia. However, patients may also have other symptoms such as tachycardia, tachypnea, heart murmurs, and hepatomegaly. In patients with hemoglobin under 40 g/L, a blood transfusion with packed erythrocytes should be given slowly. The quantity of packed erythrocytes administered should be sufficient to raise the level of hemoglobin to a safe level while awaiting the response to iron therapy.
Which of the following problems is most likely to occur in a 12-month-old infant who began taking whole cow milk at 6 months of age?
Infants who drink cow's milk in the first year of life are at risk for iron deficiency anemia. Cow's milk is the most common dietary cause of iron deficiency in infants. Iron deficiency is the most common cause of anemia and usually results from blood loss. Symptoms are usually nonspecific. RBCs tend to be microcytic and hypochromic, and iron stores are low as shown by low serum ferritin and low serum iron with high serum transferrin. If the diagnosis is made, occult blood loss is suspected. Treatment involves iron replacement and treatment of blood loss.
A 1-year-old infant is brought to your office for a well child visit. A screening CBC reveals her hemoglobin to be 105 g/L with a mean corpuscular volume of 69 fL.
Which one of the following is the most likely cause of her anemia?
Correct Answer A: This child has evidence of mild microcytic anemia, which is most commonly caused by iron deficiency related to reduce dietary intake. Less common causes are thalassemia and lead poisoning. Hemolysis usually causes a normocytic anemia with an elevated reticulocyte anemias.
A 5-day-old neonate is brought to the office because of the development of yellow skin and a rash 1 day after hospital discharge. She weighed 3400 g (7 lb 8 oz) at birth and is the product of a normal pregnancy. The neonate was breast-feeding and was doing well at the time of discharge. Her skin color has become yellow. On physical examination the neonate weighs 3250 g (7 lb 2 oz). There is scleral and skin icterus. Studies on the neonate confirm your presumptive diagnosis of physiologic jaundice.
The most appropriate advice to give the mother regarding the icterus is to:
Correct Answer E:
Physiologic jaundice is very common in newborns. There should be no change in childcare or feeding of the neonate. Phenobarbital is not indicated because this patient has physiologic jaundice and no therapy is required.
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