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Review Question - QID 109379

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QID 109379 (Type "109379" in App Search)
A seven-day-old female presents to the emergency department for bloody stools. The patient was born at 39 weeks gestation to a 30-year-old primigravid via vaginal delivery. The patient’s mother declined all prenatal testing and delivered at home due to personal preference. She reports that the patient appeared well after delivery. The patient is being exclusively breastfed, and her mother reports that feeding is going well. The patient feeds every two hours and usually passes 3-4 loose, dark yellow stools per day. The patient’s mother reports that one day ago the patient began passing blood-streaked stools that have since become grossly bloody. She has also noticed that the patient has become more lethargic and has less interest in feeding. The patient’s mother denies any family history of bleeding disorders. The patient’s temperature is 97.7°F (36.5°C), blood pressure is 56/42 mmHg, pulse is 152/min, and respirations are 28/min. On physical exam, the patient appears somnolent. Her anterior fontanelle is tense and bulging, and she has scattered ecchymoses on her extremities. Laboratory testing is performed that reveals the following:

Prothrombin time (PT): 32 seconds
Partial thromboplastin time (PTT): 54 seconds

A head CT is obtained and can be seen in Figure A. Which of the following is most likely to be found in this patient?
  • A

Deficiency of factor VII

62%

28/45

Deficiency of factor VIII

4%

2/45

Deficiency of von Willebrand factor

7%

3/45

Schistocytes

18%

8/45

Thrombocytopenia

9%

4/45

  • A

Select Answer to see Preferred Response

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This neonatal patient presents after a home birth with bloody stools, scattered ecchymoses, evidence of an intracranial bleed, and prolonged PT and PTT, which suggests a diagnosis of vitamin K deficiency. Vitamin K deficiency causes a deficiency of factor X, IX, VII, and II.

Newborns are at risk of vitamin K deficiency in the first month of life due to poor placental transfer of vitamin K, low levels of vitamin K in breastmilk, gut sterility, and immature liver function. Deficiency of vitamin K will result in low levels of all vitamin K-dependent clotting factors, which include factors II, VII, IX, and X. Standard perinatal care of newborns includes parenteral injection of vitamin K - patients who do not receive this injection are at risk of developing easy bruising, bloody stools, and intracranial hemorrhage. Laboratory studies will reveal prolonged PT and PTT, and the plasma concentrations of vitamin K-dependent clotting factors will be decreased.

Figure A demonstrates a non-contrast head CT showing a left parietal hematoma and subarachnoid hemorrhage, which is consistent with bleeding secondary to vitamin K deficiency.

Incorrect Answers:
Answer 2: Deficiency of factor VIII characterizes hemophilia A, which most commonly presents with hemarthrosis, intramuscular hematoma, and gastrointestinal or genitourinary bleeding. Deficiency of factor VIII causes prolonged PTT with a normal PT.

Answer 3: Deficiency of von Willebrand factor is a bleeding disorder that presents with cutaneous and mucosal bleeding and menorrhagia in older patients. Von Willebrand disease also presents with a normal PT.

Answer 4: Schistocytes would be consistent with a microangiopathic hemolytic anemia such as hemolytic uremic syndrome (HUS). However, PT and PTT are both normal in HUS.

Answer 5: Thrombocytopenia has many possible etiologies in the newborn, including causes of increased platelet destruction (e.g. neonatal alloimmune thrombocytopenia), decreased platelet production (e.g. preeclampsia), and other causes. However, in most of these cases of thrombocytopenia the PT and PTT would be normal.

Bullet Summary:
Newborns are at high risk of vitamin K deficiency which presents with bruising, gastrointestinal bleeding, and intracranial hemorrhage, and should receive a vitamin K injection as part of their immediate postnatal care.

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