Neonatal epidermolysis bullosa: a clinical practice guideline.

DEBRA International is undertaking a long-term initiative to develop clinical practice guidelines (CPGs) for epidermolysis bullosa (EB), to -improve the clinical care of people living with EB. Current neonatal care is based on evidence, clinical expertise and trial and error, with collaboration between the EB specialist team, parent or carer and patient, and is dependent on the neonate's individual presentation and type of EB. Early intervention based on research and clinical practice is needed to establish a foundation of knowledge to guide international practitioners to create and improve standards of care and to be able to work effectively with those newly diagnosed with EB. This CPG was created by an international panel with expertise working with persons with EB. The CPG focuses on neonatal care using a systematic review methodology covering four key areas: (i) diagnosis and parental psychosocial support; (ii) hospital management: medical monitoring, wound care and pain; (iii) feeding and nutrition; and (iv) discharge planning and EB education. These four areas highlight the importance of a multidisciplinary team approach, to provide a patient-specific holistic care model that incorporates the needs and wishes of the parents and carers. The Hospital Implementation Tool included promotes transfer of theory to clinical practice.

Gross motor development in children with epidermolysis bullosa.

BACKGROUND: Epidermolysis bullosa (EB) is a group of rare, congenital skin disorders, characterized by skin fragility and formation of blisters. The gross motor outcomes of children with EB are not known. OBJECTIVES: The primary objective of the study was to measure the proportion of gross motor delay in children with EB. The secondary objectives were to measure the difference in gross motor outcomes between EB sub-types and change in gross motor outcomes over time. METHODS: Children with EB, aged between one month and five and a half years of age, attending the Sydney Children's Hospital, Epidermolysis Bullosa Clinic, were eligible. Carers completed Ages and Stages Questionnaires, Third Edition, on behalf of their children. Questionnaires were scored, and outcomes were compared to age-expected norms. RESULTS: There were 24 participants to complete a questionnaire. Eleven participants completed additional questionnaires over the 24 month study duration. The proportion of children with EB with gross motor delay was greater than age-expected norms (29.17% vs. 2.5%). The delay occurred in children with recessive dystrophic (80%) and epidermolysis bullosa simplex (33.33%) sub-types, but not dominant dystrophic (0%). No children with Junctional EB or Kindler EB joined this study. CONCLUSIONS: This study demonstrates a difference in gross motor outcomes in children with EB. Children with recessive dystrophic and epidermolysis bullosa simplex should be prioritized for monitoring of, and intervention for, gross motor outcomes through multidisciplinary care. Further research investigating long-term outcomes for children with EB and the effectiveness of interventions would be beneficial.

Added Diagnostic Value of Peanut Component Testing: A Cross-Sectional Study in Australian Children.

BACKGROUND: Peanut components are widely used in clinical practice; however, their utility to predict challenge outcome in the Australian children, outside of infants, is not well studied. OBJECTIVE: Can peanut component testing predict outcome of challenge in peanut-allergic children. METHODS: All children attending peanut challenges, regardless of previous allergic reactions to peanut or sensitization (skin prick test or peanut IgE) alone, were recruited. Serum collected before the challenge was analyzed for peanut IgE and Ara h 1, 2, 3, 6, 8, and 9 (ImmunoCap). RESULTS: Of the 222 children recruited, 89 (40%) were allergic on oral food challenge. Ara h 2 and 6 performed similarly to peanut IgE and skin prick test in predicting challenge outcome (area under the curve, 0.84-0.87). No baseline clinical characteristics, including past history, predicted challenge outcome. By logistic regression, degree of polysensitization to Ara h 1, 2, or 3 increased the odds of allergic reaction at oral food challenge at 0.35 and 1.0 kUA/L cutoff levels (P < .001 for both). All 11 children sensitized (>0.35 kUA/L) to Ara h 1, 2, and 3 reacted to peanut challenge. Degree of polysensitization at more than 1.0 kUA/L was associated with a lower cumulative eliciting dose (P = .016) and with severity of allergic reaction on challenge (P = .007). CONCLUSIONS: In our cohort, sensitization to the combination of Ara h 1, 2, and 3 was highly predictive of peanut allergy. Overall, only Ara h 2 as individual component most correlated with severity of reaction at challenge and adrenaline use. Ara h 8 and 9 were not useful in predicting challenge outcome.

Skin testing with ultra-heat-treated (UHT) cow's milk in children with cow's milk allergy.

BACKGROUND: A large proportion of cow's milk (CM)-allergic children are able to tolerate extensively heated forms of CM such as baked goods. Little is known about whether ultra-heat-treated (UHT) forms of cow's milk are immunologically similar to extensively heated cow's milk and therefore may be tolerated by these children. OBJECTIVE: To determine whether skin test wheal size using UHT CM was significantly different from other forms of CM and CM extracts. METHODS: Children presenting for oral food challenges with either extensively heated or unheated cow's milk underwent skin prick test (SPT) to commercial CM, UHT CM, evaporated CM, and fresh whole CM. The results were compared between groups of children. RESULTS: At study exit, only 14% of children were avoiding all forms of CM, compared with 70% at study entry. No difference was seen in the mean SPT results for UHT CM between those children that could tolerate heated CM compared with those that could not. The mean SPT result for casein was significantly lower in those that could tolerate heated CM. However, within the group of heated milk-tolerant children, the mean SPT for UHT CM was significantly lower than the SPT for fresh whole CM. CONCLUSION: Ultra-heat-treated CM does not behave significantly differently from other forms of CM when evaluated by SPT in heated milk-allergic vs heated milk-tolerant children. This suggests that UHT CM is not sufficiently immunologically different from unheated CM to be tolerated by heated CM-tolerant children.

Repeat oral food challenges in peanut and tree nut allergic children with a history of mild/moderate reactions.

BACKGROUND: In peanut and tree nut allergic children a history of anaphylaxis is associated with subsequent severe reactions. OBJECTIVE: We aimed to prospectively rechallenge peanut and tree nut allergic children with a history of mild/moderate reactions to assess their allergy over time. METHODS: In this cohort study peanut and tree nut allergic children with a history of mild/moderate reactions during a controlled oral challenge were invited to have a follow-up oral challenge to the same food at least 1 year later. RESULTS: Twenty-six children participated in the study. The mean time interval between the first and second challenge for all participants was 35.5 months. Peanut or tree nut allergy resolved in 38.5% of participants. Those with persistent peanut or tree nut allergy showed a decrease in their reaction threshold and/or increased severity in 81% of cases. There were no demographic features or skin test results that were predictive of changes in severity over time. CONCLUSION: Peanut and tree nut allergic children with a history of mild/moderate reactions who remained allergic demonstrated a high rate of more severe reactions and/or reduced thresholds upon rechallenge over a year later, however, the rate of resolution of allergy in this group may be higher than previously reported.