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The Case:
A 36-week appropriate-for-gestational age male was born with hydrops fetalis that was not diagnosed prenatally. The birth hospital had a Level II nursery.
Pertinent Points in the History Include:
Born to a 24-year-old G2P0010 (previous first-trimester spontaneous abortionwoman by emergency cesarean section due to failure to progress and decreased fetal heart rate variability.
Prenatal Laboratory Results:
Blood type, B+
Antibody screen, negative
Serology, nonreactive
Rubella, immune
Group B Streptococcus, positive
3-hour glucose tolerance test, normal
Irregular fetal heart rate 2 months prior to delivery
Level II ultrasonography result, normal
Preterm labor occurred 1 week prior to delivery, but was stopped successfully with tocolysis, and the mother was sent home after 6 hours of observation. Rupture of membranes occurred 6 hours prior to delivery with clear fluid.
At delivery, the infant appeared grossly edematous, dusky, and floppy, with no spontaneous respiratory effort. Endotracheal intubation was difficult because of airway edema. Resuscitation consisted of positive pressure ventilation, chest compressions, and epinephrine administration via an umbilical venous catheter.
Initial Arterial Blood Gas Results:
pH, 6.96
PCO2, 89 mm Hg
PO2, 114 mm Hg
Bicarbonate, 19 mEq/L
Base excess, -16
A chest radiograph showed bilateral pleural effusions. A kidney-ureter-bladder (KUB) radiograph showed ascites. Pleurocentesis released 45 mL of straw-colored fluid from the left side of the chest, but none from the right side of the chest. A total of 15 mL of straw-colored fluid was removed from the abdomen via paracentesis.
Follow-Up Arterial Blood Gas Results:
pH 7.26
PCO2, 35 mm Hg
PO2,69 mm Hg
HCO3, 16 mEq/L
On physical examination, the infant exhibited gross body edema but no dysmorphic features. A II/VI systolic murmur was heard at the left sternal border. The liver was palpable 3 cm below the costal margin. There was mild glandular hypospadias with a minimal chordee, but testes were descended bilaterally.
The infant developed seizures and received a loading dose of 20 mg/kg of phenobarbital before being transferred …
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