Cow's milk allergy (CMA) is usually transient, but recent studies have shown a later acquisition of tolerance to CM. Our aims were to characterize a population of Portuguese children with CMA and to identify predictive factors for the persistence of this food allergy. Children with CMA observed in our Paediatric Allergy Clinic between 1997 and 2006 were selected. Demographic and clinical data were collected from medical records. The group of children who tolerated CM before the age of 2 was compared with the group of children who tolerated CM beyond that age or persisted with CMA until the end of the study. Multivariate logistic regression analysis was used to investigate independent predictive factors for the persistence of CMA beyond the age of 2. In the subgroup of children with IgE-mediated CMA, the acquisition of tolerance was analysed using Cox regression. In this population of 139 children, the majority presented more than one symptom (73%) affecting more than one organ (51%), with cutaneous (81%), gastrointestinal (55%), respiratory (16%) manifestations and/or anaphylaxis (3%). Thirty-two per cent developed asthma, 20% atopic eczema, 20% rhinoconjunctivitis and 19% other food allergies over time. The acquisition of tolerance was different in the whole population versus the subgroup with IgE-mediated CMA: 34%versus 0% at the age of 2, 55%versus 22% at the age of 5 and 68%versus 43% at the age of 10. Immediate allergic symptoms, asthma and other food allergies were independent factors for the persistence of CMA beyond the age of 2. Higher maximum weal diameter on skin prick test to CM and higher maximum level of specific IgE to CM were associated with reduced likelihood of acquiring tolerance in the subgroup of children with IgE-mediated CMA. In conclusion, children with IgE-mediated CMA acquire tolerance to CM at older age. Clinical parameters and allergy tests may be helpful in defining prognosis. CM-allergic children tend to develop other atopic conditions and need specialized follow-up in the long term.
The bacilli Calmette-Guérin (BCG) vaccine is administered to all newborns in countries where tuberculosis is endemic. Immunocompromised hosts, namely patients with human immunodeficiency virus infection or primary immunodeficiencies, are especially prone to serious complications from this vaccine. We report three cases of BCG disease in children with primary immunodeficiencies: one with a partial recessive interferon-γ receptor 1 deficiency, who developed BCG dissemination; and two relatives with ZAP70 deficiency, a severe combined immunodeficiency, both of whom presented with regional and distant BCG disease. All had severe axillary lymphadenitis. These clinical cases underline the importance of considering the diagnosis of immunodeficiency in a child with severe axillary lymphadenitis after BCG vaccination and of disseminated BCG disease in an immunodeficient child in the appropriate clinical setting. Moreover, BCG vaccination should be delayed in every newborn with a family history of primary immunodeficiency until the condition has been ruled out.
Our population of CM allergic children presented immediate symptoms with cutaneous expression in the majority. Severe reactions were common on accidental exposure. By the age of 10 years, 44% maintained CMA, highlighting the importance of a multidisciplinary follow-up.
Galactose epimerase deficiency is an inborn error of metabolism due to uridine diphosphate-galactose-4'-epimerase (GALE) deficiency. We report the clinical presentation, genetic and biochemical studies in two siblings with generalized GALE deficiency.Patient 1: The first child was born with a dysmorphic syndrome. Failure to thrive was noticed during the first year. Episodes of heart failure due to dilated cardiomyopathy, followed by liver failure, occurred between 12 and 42 months. The finding of a serum transferrin isoelectrofocusing (IEF) type 1 pattern led to the suspicion of a congenital disorder of glycosylation (CDG). Follow-up disclosed psychomotor disability, deafness, and nuclear cataracts.Patient 2: The sibling of patient 1 was born with short limbs and hip dysplasia. She is deceased in the neonatal period due to intraventricular hemorrhage in the context of liver failure. Investigation disclosed galactosuria and normal transferrin glycosylation.Next-generation sequence panel analysis for CDG syndrome revealed the previously reported c.280G>A (p.[V94M]) homozygous mutation in the GALE gene. Enzymatic studies in erythrocytes (patient 1) and fibroblasts (patients 1 and 2) revealed markedly reduced GALE activity confirming generalized GALE deficiency. This report describes the fourth family with generalized GALE deficiency, expanding the clinical spectrum of this disorder, since major cardiac involvement has not been reported before.
RESUMOIntrodução: Determinar a incidência de infeções da corrente sanguínea associadas ao uso de cateter venoso central, após reforço de medidas multidisciplinares de boa prática e a sua comparação com a taxa de incidência de infeções da corrente sanguínea associadas ao uso de cateter venoso central prévia. Results: Seventy five children were included, with a median age of 23 months: 22 (29.3%) newborns; 28 (37.3%) with recent surgery and 32 (43.8%) with underlying illness. A total of 105 central venous catheter were inserted, the majority a single central venous catheter (69.3%), with a mean duration of 6.8 ± 6.7 days. The most common type of central venous catheter was the short-term, nontunneled central venous catheter (45.7%), while the subclavian and brachial flexure veins were the most frequent insertion sites (both 25.7%). There were no cases of central-line associated bloodstream infection reported during this study. Comparing with historical controls (group 1), both groups were similar regarding age, gender, department of origin and place of central venous catheter insertion. In the current study (group 2), the median length of stay was higher, while the mean duration of central venous catheter (excluding peripherally inserted central line) was similar in both groups. There were no statistical differences regarding central venous catheter caliber and number of lumens. Fewer children admitted to Pediatric Intensive Care Unit had central venous catheter inserted in group 2, with no significant difference between single or multiple central venous catheter. Discussion: After multidimensional strategy implementation there was no reported central-line associated bloodstream infection Conclusions: Efforts must be made to preserve the same degree of multidimensional prevention, in order to confirm the effective reduction of the central-line associated bloodstream infection rate and to allow its maintenance.
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