Objective: This study described the prevalence and duration of mothers' breastfeeding infants with phenylketonuria (PKU) and explored factors related to duration of breastfeeding as a surrogate for breastfeeding success. Subjects and Methods: Descriptive analysis as performed from an international Internet survey of mothers (n = 103) who met the inclusion criteria: (1) at least 21 years of age, (2) able to read and write in English, (3) child with PKU, and (4) living in the United States or Canada. Results: Of the 103 mothers, 89 (86%) initiated breastfeeding immediately following delivery, whereas 14 (14%) chose bottle feeding. In comparison to breastfeeding after delivery, significantly fewer mothers breastfed after diagnosis (McNemar's v 2 = 30.33, p < 0.001; n = 72 vs. n = 89). Breastfeeding duration ranged from less than 1 month to 24 months with one modal duration category (n = 20, 22%) at less than 1 month. The timing of the addition of commercial infant formula to supplement breastfeeding or expressed mothers' milk was associated with a shorter duration of breastfeeding among infants with PKU: v 2 (42, n = 73) = 88.13, p < 0.001. Conclusions: PKU is treated with phenylalanine (Phe) restriction. Breastfeeding infants with PKU is challenging in part because Phe intake is difficult to determine precisely. We studied breastfeeding duration in infants with PKU and factors associated with success. Further research should identify the unique needs of mothers' breastfeeding infants with PKU to guide the development of interventions specific to these mothers to support their efforts to continue breastfeeding after the diagnosis of PKU.
The skin serves many purposes, acting as a barrier to infection, protecting internal organs, contributing to temperature regulation, storing insulating fats, excreting electrolytes and water, and providing tactile sensory input. This article focuses on a review of normal skin structure and function and selected neonatal skin disorders. The disorders reviewed are Staphylococcal scalded skin syndrome, epidermolysis bullosa, and the ichthyoses. The basis for each skin disorder is presented. Nursing management and skin care are incorporated into the review of each selected disorder.
Findings demonstrate the importance mothers attribute to breastfeeding and their willingness to invest considerable effort to breastfeed. Health care providers working with these mothers should help them strategize for success.
With recent advances in laboratory technology with tandem mass spectrometry (MS/MS), the number of infants identified with a fatty acid oxidation disorder has increased dramatically. Disorders of fatty acid oxidation comprise one of the most rapidly growing groups within the field of errors of metabolism. This review will explore the recent developments in newborn screening related to the use of tandem mass spectrometry and disorders of fatty acid oxidation.
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