Bmc Pediatr
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Review Case Reports
Exon sequencing of the alpha-2-globin gene for the differential diagnosis of central cyanosis in newborns: a case report.
Cyanosis is usually associated with serious conditions requiring urgent treatment in the neonatal intensive care unit (NICU). Hemoglobin M (Hb M) disease is one type of congenital methemoglobinemia characterized by cyanosis. Among these variants, α-globin chain mutations such as Hb M Boston present cyanosis from birth while other variants usually manifest later in life. ⋯ Hb M disease is a benign disease and does not require any treatment whereas acquired methemoglobinemia is a potentially fatal condition. Neonatologists should be aware that low oxygenation status on pulse oximetry in the face of normal arterial blood saturation values might indicate the possibility of Hb M disease in early neonatal cyanosis, irrespective of MetHb value.
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Review Case Reports
Is vitamin C enough? A case report of scurvy in a five-year-old girl and review of the literature.
Numerous cases of scurvy secondary to diet limitations have been reported in the literature with most being boys with special needs. To date, the focus of the literature describing vitamin C deficiency has been the medical sequelae of the deficiency. There has been little attention given underlying diet limitations causing the vitamin C deficiency. ⋯ This case is unusual as most reported studies describe males with special needs. The severity of her eating issues suggest providers may consider referral to allied health professionals to address diet limitations for both children identified with nutrient deficiencies as well as children whose selective eating places them at risk for nutritional deficiencies or problems with growth. The child we described was anemic, like 42% of children described in the case literature on scurvy and like 32% of the children in this literature, our patient was underweight. In the literature, comorbid nutrient deficiencies were reported in 22% of the scurvy case studies. We suggest vitamin C supplementation is a necessary component for addressing vitamin C deficiency, but insufficient for addressing the diet limitations causing the nutrient deficiency.
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Despite the high burden of pediatric mortality from preventable conditions in low and middle income countries and the existence of multiple tools to prioritize critically ill children in low-resource settings, no analysis exists of the reliability and validity of these tools in identifying critically ill children in these scenarios. ⋯ Studies evaluating triage tools for pediatric patients in low and middle income countries are scarce. Furthermore the methodology utilized in the conduct of these studies varies greatly and does not allow for the comparison of tools across study sites.
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Despite the high burden of pediatric mortality from preventable conditions in low and middle income countries and the existence of multiple tools to prioritize critically ill children in low-resource settings, no analysis exists of the reliability and validity of these tools in identifying critically ill children in these scenarios. ⋯ Studies evaluating triage tools for pediatric patients in low and middle income countries are scarce. Furthermore the methodology utilized in the conduct of these studies varies greatly and does not allow for the comparison of tools across study sites.
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Review Meta Analysis
Arginine supplementation in prevention of necrotizing enterocolitis in the premature infant: an updated systematic review.
Hypoxic-ischemic injury is thought to play a significant role in necrotizing enterocolitis (NEC). Nitric Oxide (NO) is the principal inhibitory neurotransmitter in the gut and is involved in regulation of mucosal blood flow and maintenance of mucosal integrity. NO is synthesized from L-arginine by NO synthases. Our primary objective was to determine the effectiveness of supplemental L-arginine versus placebo in prevention of NEC in preterm infants ≤ 34 weeks gestational age by systematic review of published randomized controlled trials (RCTs). ⋯ L-arginine supplementation appears to be protective in prevention of NEC in preterm infants and without any significant impact on neurodevelopmental outcomes at 36 months of corrected age. With the addition of the results of one more study to the literature, an intriguing role for L-arginine supplementation continues to gain support. However, large multi-centre RCTs are needed before this can become common practice.