Sent for urgent mass spectroscopy and oral administration of a cocktail of vitamins (biotin, B 12, riboflavin and carnitine) was commenced, as well as IV bolus doses and then continuing infusions of sodium benzoate and arginine. Accordingly, extra intravenous (IV) dextrose was administered (increased to 9.3 mg/kg/min) a urine specimen was < 45 U/L]) and hyperammonaemia (plasma ammonia, 183 µmol/L Metabolic investigations showed a mild transaminitis (serum aspartate aminotransferase, 568 U/L and alanine aminotransferase, 946 U/L [RR for both, The unexplained anion-gap metabolic acidosis persisted. Apart from persistent tachypnoea, hyperpnoea and periods of appearing lethargic and less interactive, the infant’s vital signs and results of a physical examination were unremarkable. Results of a repeat abdominal ultrasound were normal.
Initial urinalysis showed a specific gravity of 1.025 (RR, 1.015–1.025), pH of 6.0 (RR, 5.0–8.0) and elevated ketones (80 mg/L RR, 5–30 mg/L), but was otherwise normal. Test results of arterial blood gas levels showed a well compensated anion-gap metabolic acidosis, with a lactate level elevated to 7.8 mmol/L (RR, < 2.0 mmol/L) pH, 7.38 (RR, 7.35–7.43) partial pressure of carbon dioxide (PaCO 2), 14 mmHg (RR, 32–45 mmHg) partial pressure of oxygen (PaO 2), 129 mmHg (RR, 69–116 mmHg) plasma bicarbonate (HCO 3), 8 mmol/L (RR, 22–32 mmol/L) base equivalent (BE), − 7 (RR, − 2 to + 2) sodium, 142 mmol/L (RR, 135–145 mmol/L) potassium, 4.9 mmol/L (RR,ģ.6–5.1 mmol/L) and chloride (Cl), 113 mmol/L (RR, 95–105 mmol/L). He appeared lethargic, with minimal motor and verbal response, although this improved when a low blood sugar level (2.0 mmol/L reference range, 3.0–5.5 mmol/L) was corrected. There was no family history of note, and the family denied giving him medications (including complementary or alternative treatment) apart from paracetamol. The infant’s medical history, including the perinatal period, was unremarkable. An abdominal ultrasound was thought to show possible intussusception.
0 kommentar(er)
