A breastfednewborn has just been diagnosed with galactosemia. The therapeutic management for this newborn is to:

Choice A:

Blood pressure. The normal blood pressure range for a newborn is 60 to 80 mm Hg systolic and 40 to 50 mm Hg diastolic. The baby's blood pressure is low, which could indicate shock, dehydration, infection, or heart failure. This requires immediate follow-up to identify and treat the cause.

Choice B:

Gastrointestinal disturbances. Gastrointestinal disturbances such as vomiting and diarrhea are common symptoms of neonatal abstinence syndrome (NAS), which is a withdrawal syndrome of infants after birth caused by in-utero exposure to drugs of dependence, most commonly opioids. These symptoms are not life-threatening and can be managed with supportive care such as hydration, nutrition, and comfort measures.

Choice C:

Skin color. Skin color is not a reliable indicator of NAS, as it can vary depending on the baby's ethnicity, temperature, oxygenation, and circulation. Skin color alone does not require immediate follow-up unless it is accompanied by other signs of distress such as cyanosis, pallor, or jaundice.

Choice D:

NAS score. NAS score is a tool used to assess the severity of withdrawal symptoms in infants with NAS. It includes items such as tremors, irritability, sleep problems, muscle tone, reflexes, seizures, yawning, sneezing, feeding, vomiting, stooling and temperature. A high NAS score indicates that the baby needs more intensive treatment such as medication to ease the withdrawal process. A low NAS score indicates that the baby is coping well and may not need medication. The NAS score should be monitored frequently and adjusted according to the baby's response.

Choice E:

Temperature. The normal temperature range for a newborn is 36.5 to 37.5°C (97.7 to 99.5°F). The baby's temperature is high, which could indicate infection, dehydration or hyperthermia. This requires immediate follow-up to identify and treat the cause.

Choice F:

Oxygen saturation. The normal oxygen saturation range for a newborn is 95 to 100%. The baby's oxygen saturation is within the normal range and does not require immediate follow- up unless it drops below 90% or rises above 100%, which could indicate hypoxia or hyperoxia respectively.

Choice G:

Central nervous system disturbances. Central nervous system disturbances such as seizures, tremors, irritability, and overactive reflexes are common symptoms of NAS. These symptoms are not life-threatening and can be managed with supportive care such as swaddling, rocking, dimming lights, and reducing noise.

Choice H:

Respiratory rate. The normal respiratory rate range for a newborn is 40 to 60 breaths per minute. The baby's respiratory rate is high, which could indicate respiratory distress, infection, pain, or anxiety. This requires immediate follow-up to identify and treat the cause.

Choice A reason:

All milk- and lactose-containing formulas, including breast milk, must be stopped during infancy. Soy protein is the formula of choice for newborns and infants with galactosemia. Breast milk should not be used in newborns and infants with galactosemia because it contains galactose, which they cannot metabolize properly.

Choice B reason:

Adding amino acids to the breast milk will not help the newborn with galactosemia, because the problem is not a lack of amino acids, but a deficiency of the enzyme that breaks down galactose. Amino acids are the building blocks of proteins, not sugars.

Choice C reason:

Substituting a lactose-containing formula for breast milk will worsen the condition of the newborn with galactosemia because lactose is composed of glucose and galactose. The newborns will still be exposed to galactose, which will accumulate in the blood and tissues and cause damage.

Choice D reason:

Giving the appropriate enzyme along with breast milk is not a feasible option for the newborn with galactosemia, because there is no oral enzyme replacement therapy available for this condition. The only treatment is dietary restriction of galactose.