Fever and Renal Failure in a Child With DiGeorge Syndrome and Tetralogy of Fallot

The patient is a 9-year-old male with DiGeorge syndrome and tetralogy of Fallot; he underwent cardiac repair at 7 months. Seven years later, in early 2012, he underwent a pulmonary artery to right ventricle conduit replacement with a tricuspid valve repair. In November 2013, he developed increased fatigue with abdominal pain and intermittent emesis and was ultimately admitted in February 2014 with progressive cough and low-grade fevers. His initial vital signs were the following: temperature 101.7°F, blood pressure 119/66, heart rate 109, respiratory rate 13, and O2 saturation 97% on room air. Examination was notable for nontoxic appearance, III/VI holosystolic murmur at the left upper sternal border, crackles at lung bases, and 1+ edema in the bilateral lower extremities. His liver edge was 3–4 cm below the right costal margin with a palpable spleen tip. There was no lymphadenopathy or rashes or stigmata of infective endocarditis, such as Janeway lesions or Osler nodes. The patient’s white blood cell count was 7300 cells/mm (73% neutrophils, 16% lymphocytes, and 10% monocytes), his hemoglobin was 4.9 g/dL, and his platelet count was 90,000 cells/mm. Blood urea nitrogen and creatinine were 84 mg/mL and 5.3 mg/dL, respectively. Hemolysis laboratory results (haptoglobin, lactate dehydrogenase) were normal, and a reticulocyte count was elevated to 4.3% (0.5–2.3% normal). There was mild coagulopathy (international normalized ratio 1.4), hypocomplementemia with C3 47 mg/dL (93– 203 mg/dL normal), and the erythrocyte sedimentation rate was elevated to 100 mm/h (0–15 mm/h normal). Urinalysis showed 3+ blood, 1+ protein, negative nitrites and leukocyte esterase, positive casts, and crystals. A renal ultrasound scan showed enlarged echogenic kidneys and a chest radiograph revealed marked cardiomegaly with bibasilar atelectasis. A transthoracic echocardiogram showed a thickened tricuspid valve, concerning for vegetations, with moderately increased tricuspid regurgitation. Vegetations were also present in the right ventricle to pulmonary artery conduit with mild aortic insufficiency. The patient met Duke criteria for infective endocarditis (IE) with 1 major criterion, based on echocardiographic findings, and 3 minor criteria, including predisposition for endocarditis, fever 38.0°C, and evidence of immunologic phenomena as demonstrated by glomerulonephritis. Five high-volume blood cultures (including a fungal isolator) were obtained prior to initiation of antibiotics. After initiation of vancomycin, gentamicin, and cefepime, the patient quickly defervesced. No evidence of embolic events was seen on chest computed tomography scan, abdominal ultrasound, or ophthalmologic examination. On exposure history, the patient was born in the United States and lived in California before moving to Mexico at age 3. He returned briefly to the United States at age 7 for his second cardiac surgery, but otherwise was living on a farm near Guadalajara, Mexico where he had routine contact with farm animals. He denied participation in animal birthing or consuming raw meat, but frequently ate homemade queso fresco. He returned to live in California Central Valley in December 2013.