A 34-year-old G3P2 delivers a baby by spontaneous vaginal delivery. She had scant prenatal care and no ultrasound, so she is anxious to know the sex of the baby. At first glance you notice female genitalia, but on closer examination the genitalia are ambiguous.
Which of the following is the best next step in the evaluation of this neonate?
Ambiguous genitalia at birth is a medical emergency, not only for psychological reasons for the parents, but also because hirsute female infants with congenital adrenal hyperplasia (CAH) may die if undiagnosed. CAH is an autosomally inherited disease of adrenal failure that causes hyponatremia and hyperkalemia due to lack of mineralocorticoids. A thorough physical examination is the best initial evaluation. While it will not provide the definitive diagnosis of the gender, it can provide clues. Examination should include inspection and palpation of the genitalia, palpation for gonads in the inguinal canal or labioscrotal folds, evaluation for fused labia, evaluation for presence of a vagina or pouch, and assessment for other nongenital dysmorphic features. The newborn should also be quickly evaluated for presence of hyper- or hypotension, or signs of dehydration. Karyotype, electrolyte analysis, blood or urine assays for progesterone, 17a-hydroxyprogesterone, and serum androgens such as dehydroepiandrosterone sulfate are essential to the workup as well. Pelvic ultrasound or MRI can detect ovaries or undescended testes, but that is not the first step in management. Laparotomy or laparoscopy is sometimes necessary for ectopic gonadectomy after puberty has occurred.
A 24-year-old primigravid woman, who plans to breastfeed, decides to have a home delivery. Immediately after the birth of a 4.1-kg (9-lb) newborn, the patient experiences massive hemorrhage from extensive vaginal and cervical lacerations. She is brought to the nearest hospital in shock. Over the course of 2 hours, nine units of blood are transfused, and the patient’s blood pressure returns to a reasonable level. A hemoglobin value the next day is 7.5 g/dL, and three more units of packed red blood cells are given.
The most likely late sequela to consider in this woman is which of the following?
A disadvantage of home delivery is the lack of facilities to control postpartum hemorrhage. The woman described in the question delivered a large baby, suffered multiple soft tissue injuries, and went into shock, needing nine units of blood by the time she reached the hospital. Sheehan syndrome seems a likely possibility in this woman. This syndrome of anterior pituitary necrosis related to obstetric hemorrhage can be diagnosed by 1-week postpartum, as lactation fails to commence normally. Other symptoms of Sheehan syndrome include amenorrhea, atrophy of the breasts, and loss of thyroid and adrenal function. The other presented choices for late sequelae are less likely. Hemochromatosis would not be expected to occur in this healthy young woman, especially since she did not receive prolonged transfusions. Cushing, Simmonds, and Stein-Leventhal syndromes are not known to be related to postpartum hemorrhage. It is important to note that home delivery is not a predisposing factor to postpartum hemorrhage.
A 27-year-old G4P3 at 37 weeks presents to labor and delivery with heavy vaginal bleeding and painful uterine contractions. A bedside ultrasound demonstrates a fundal placenta. The patient’s vital signs are: blood pressure 140/92 mm Hg, pulse 118 beats per minute, respiratory rate 20 breaths per minute, and temperature 37°C (98.6°F). The fetal heart rate tracing reveals tachycardia with decreased variability and intermittent late decelerations. She is taken to the OR for an emergency cesarean, and delivers a male infant with Apgar scores of 4 and 9. When the placenta is delivered, a large retroplacental clot is noted. The patient becomes hypotensive, and bleeding is noted from the wound edges and her IV catheter sites.
Which of the following blood products will most quickly resolve her cause of hemorrhage?
This patient has a large placental abruption, which is the most common cause of consumptive coagulopathy in pregnancy. The bleeding described signifies that the patient has a significant coagulopathy with hypofibrinogenemia. Prompt and vigorous transfusion is needed. Packed red blood cells will restore blood volume and increase oxygen carrying capacity. FFP contains about 600 mg to 700 mg of fibrinogen and will promote clotting, and is the best choice to quickly resolve her cause of hemorrhage. Cryoprecipitate contains clotting factors and fibrinogen, but in a much lower amount (200 mg) than FFP, and has no advantage over the use of FFP in this bleeding patient. Recombinant factor VII can be used for the treatment of severe obstetrical hemorrhage but will not be effective if fibrinogen is low. Platelet transfusion is considered in bleeding patients with platelets less than 50,000.
A 30-year-old G5P3 has undergone a repeat cesarean delivery. She wants to breastfeed. Her past medical history is significant for hepatitis B infection, hypothyroidism, depression, and breast reduction. She is receiving intravenous antibiotics for endometritis. The baby latches on appropriately and begins to suckle.
In the mother, which of the following is a response to newborn suckling?
The normal sequence of events triggered by suckling is as follows: through a response of the central nervous system, dopamine is decreased in the hypothalamus. Dopamine suppression decreases production of PIF, which normally travels through a portal system to the pituitary gland; because PIF production is decreased, production of prolactin by the pituitary is increased. At this time, the pituitary also releases oxytocin, which causes milk to be expressed from the alveoli into the lactiferous ducts. Suckling suppresses the production of luteinizing hormone—releasing factor and, as a result, acts as a mild (but not reliable) contraceptive (because the midcycle surge of luteinizing hormone does not occur).
Which of the following aspects of her history might prevent this patient from breastfeeding?
There are very few contraindications to breastfeeding. Most medications taken by the mother enter into breast milk to some degree. Breastfeeding is inadvisable when the mother is being treated with antimitotic drugs, tetracyclines, diagnostic or therapeutic radioactive substances, or lithium carbonate. Acute puerperal mastitis may be managed quite successfully while the mother continues to breastfeed. Reduction mammoplasty with autotransplantation of the nipple makes breastfeeding impossible.
However, there are reduction mammoplasty techniques that do potentially allow for some amount of breastfeeding; this would be most likely in patients who underwent a surgery where the areola and nipple were not completely severed. Ampicillin or levothyroxine can be safely used by breastfeeding mothers. A past history of hepatitis B is not a contraindication to breastfeeding. With some acute viral infections such as hepatitis B, there is the possibility of transmitting the virus in milk.
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