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A Rare Case of Isolated Congenital Asplenia Presenting in Septic Shock: Howell-Jolly Bodies a Clue to Early Diagnosis

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An 8-month-old female infant, previously healthy with up to date immunizations, presented to an outside emergency department with fever of 103°F. She had signs/symptoms consistent with upper respiratory tract infection… Click to show full abstract

An 8-month-old female infant, previously healthy with up to date immunizations, presented to an outside emergency department with fever of 103°F. She had signs/symptoms consistent with upper respiratory tract infection since past 1 week. She was managed symptomatically in the emergency department and discharged home. However, she returned with vomiting, lethargy, and signs of dehydration. She was fluid resuscitated and initially admitted to the pediatric floor. She received broad spectrum antibiotics after the cultures were obtained. Over the next several hours, she progressively developed hemodynamic instability and respiratory compromise requiring intubation, mechanical ventilation and initiation of vasoactive/inotropic support. She was transferred to our pediatric intensive care unit for further evaluation and advanced care. Within an hour of presentation, she needed higher doses of vasopressors, including epinephrine (0.2 μg/ kg/min), norepinephrine (0.15 μg/kg/min), and vasopressin (0.4 mU/kg/min) infusions. Echocardiography showed normal anatomy and biventricular function. Initial laboratory workup demonstrated leukopenia 4400/mm3 (normal range, 600017 500/mm3), thrombocytopenia 48 000/mm3 (normal range, 150 000-400 000/mm3), and hemoglobin 13.1 g/dL with HowellJolly bodies on the peripheral blood smear (Figure 1). She also had elevated blood urea nitrogen (BUN) 28 mg/dL (normal range, 10-20 mg/dL), creatinine 1.2 mg/dL (normal range, 0.2-0.4 mg/dL) consistent with renal dysfunction, elevated aspartate transferase (AST) 2446 U/L (normal range, 10-50 U/L) and alanine transferase (ALT) 661 U/L (normal range, 3-30 U/L), and plasma ammonia 85 μmol/L (normal range, 21-50 μmol/L) consistent with hepatic dysfunction. Elevated prothrombin time (PT) 30 seconds (normal range, 9-12 seconds), partial thromboplastin time (PTT) 87 seconds (normal range, 22-31 seconds), and decreased fibrinogen 142 mg/dL (normal range, 180-400 mg/dL) were consistent with disseminated intravascular coagulation (DIC). She had severe lactic acidosis 12.1 mEq/L (normal range, 0.5-2.2 mEq/L).

Keywords: rare case; range; normal range; case isolated; isolated congenital; mm3

Journal Title: Clinical Pediatrics
Year Published: 2018

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