Prematurity presents a risk for higher order cognitive functions. Some of these deficits manifest later in development, when these functions are expected to mature. However, the causes and consequences of prematurity are still unclear. We conducted a longitudinal study to first identify clinical predictors of ultrasound brain abnormalities in 196 children born very preterm (VP; gestational age ≤32 weeks) and with very low birth weight (VLBW; birth weight ≤1500 g). At ages 8–16, the subset of VP-VLBW children without neurological findings (124) were invited for a neuropsychological assessment and an MRI scan (41 accepted). Of these, 29 met a rigorous criterion for MRI quality and an age, and gender-matched control group (n = 14) was included in this study. The key findings in the VP-VLBW neonates were: (a) 37% of the VP-VLBW neonates had ultrasound brain abnormalities; (b) gestational age and birth weight collectively with hospital course (i.e., days in hospital, neonatal intensive care, mechanical ventilation and with oxygen therapy, surgeries, and retinopathy of prematurity) predicted ultrasound brain abnormalities. At ages 8–16, VP-VLBW children showed: a) lower intelligent quotient (IQ) and executive function; b) decreased gray and white matter (WM) integrity; (c) IQ correlated negatively with cortical thickness in higher order processing cortical areas. In conclusion, our data indicate that facets of executive function and IQ are the most affected in VP-VLBW children likely due to altered higher order cortical areas and underlying WM.

Learning Objectives: Methemoglobinemia is uncommon in infancy. We report an unusual case of repeated episodes of methemoglobinemia secondary to foodprotein induced enterocolitis syndrome (FPIES). A full term 3.8 kg male neonate presented at 6 weeks of age with feeding intolerance, non-anion gap metabolic acidosis and incidental finding of methemoglobinemia of 17%. Prior to this presentation, he had symptoms of vomiting at 3 weeks of age that were treated with formula changes, ranitidine and pyloric stenosis was ruled out by ultrasound. At presentation, he was treated with 1 dose of methylene blue with response and was discharged home. He again presented with 2 similar episodes of vomiting, diarrhea, leukocytosis, metabolic acidosis, methemoglobin levels of 28% and 47% respectively in the next 2 weeks. Each episode responded to fluid resuscitation, sodium bicarbonate and methylene blue therapy. Investigations were negative for renal tubular acidosis and organic acidemia and his acylcarnitine profile was normal. His cytochrome b5 reductase levels were low and there was no history of drug ingestion or exposure to dyes. Each episode improved after cessation of his enteral formula and placement on oral pedialyte. Based on his clinical presentation, recurrent episodes of methemoglobinemia responsive to methylene blue, and other laboratory investigations, his enteral feeds were changed to elemental formula. On follow up after 1 and 2 months subsequently, he had no further episodes of vomiting or methemoglobinemia and he was gaining weight. FPIES is a non-IgE mediated severe systemic response to food protein, commonly to cow's milk or soybean formulas. Patients present with acute repetitive episodes of vomiting, diarrhea, dehydration that is associated with metabolic acidosis and methemoglobinemia in severe cases. Intestinal inflammation in FPIES causes reduction in catalase activity and increase in nitrites resulting in methemoglobinemia. There are no specific laboratory tests for FPIES and detailed history and investigations are required to establish a diagnosis of FPIES as in our patient.

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