Phakomatosis Pigmentovascularis

We report a patient with phakomatosis pigmentovascularis IIb and numerous iris hamartomas. Phakomatosis pigmentovascularis IIb is characterized by the simultaneous occurrence of a nevus flammeus, a mongolian spot, and sometimes a nevus anemicus in the same individual, with systemic involvement. To our knowledge, the association with multiple iris hamartomas has been reported only once. This second patient suggests that the association might be more common. Additional reports will indicate if such an association is more frequent than is now assumed

Value of urinary N-methylhistamine measurements m childhood mastocytosis

Background: Histamine is an indicator of mast cell activation. N- methylhistamine (NMH) is a metabolite of histamine that can be measured in urine. Objective: Our purpose was to assess the usefulness of determining urinary NMH levels for the diagnosis and follow-up of patients with mastocytosis. Methods: Urinary NMH levels were determined in 44 patients and were correlated with disease activity and extension. The control group consisted of 24 children without mastocytosis or any other skin disease. Results: A significant negative correlation was found between NMH and age in patients with active mastocytosis and in the control group. Adjusted for age, NMH values were significantly higher in patients with active mastocytosis. There was a significant difference in NMH values between patients with diffuse cutaneous mastocytosis, patients with active urticaria pigmentosa, and patients with active mastocytomas. However, there was a substantial overlap of NMH values in the different subgroups. Conclusion: Urinary NMH values tend to decrease with age. Urinary NMH values correlated with the extent and the activity of the disease. High NMH values suggest more extensive involvement

High body mass index is not associated with atopy in schoolchildren living in rural and urban areas of Ghana

<p>Abstract</p> <p>Background</p> <p>Factors which determine the development of atopy and the observed rural-urban gradient in its prevalence are not fully understood. High body mass index (BMI) has been associated with asthma and potentially atopy in industrialized countries. In developing countries, the transition from rural to urban areas has been associated with lifestyle changes and an increased prevalence of high BMI; however, the effect of high BMI on atopy remains unknown in this population. We therefore investigated the association between high BMI and atopy among schoolchildren living in rural and urban areas of Ghana.</p> <p>Methods</p> <p>Data on skin prick testing, anthropometric, parasitological, demographic and lifestyle information for 1,482 schoolchildren aged 6-15 years was collected. Atopy was defined as sensitization to at least one tested allergen whilst the Centres for Disease Control and Prevention (CDC, Atlanta) growth reference charts were used in defining high BMI as BMI ≥ the 85^thpercentile. Logistic regression was performed to investigate the association between high BMI and atopy whilst adjusting for potential confounding factors.</p> <p>Results</p> <p>The following prevalences were observed for high BMI [Rural: 16%, Urban: 10.8%, p < 0.001] and atopy [Rural: 25.1%, Urban: 17.8%, p < 0.001]. High BMI was not associated with atopy; but an inverse association was observed between underweight and atopy [OR: 0.57, 95% CI: 0.33-0.99]. Significant associations were also observed with male sex [Rural: OR: 1.49, 95% CI: 1.06-2.08; Urban: OR: 1.90, 95% CI: 1.30-2.79], and in the urban site with older age [OR: 1.76, 95% CI: 1.00-3.07], family history of asthma [OR: 1.58, 95% CI: 1.01-2.47] and occupational status of parent [OR: 0.33, 95% CI: 0.12-0.93]; whilst co-infection with intestinal parasites [OR: 2.47, 95% CI: 1.01-6.04] was associated with atopy in the rural site. After multivariate adjustment, male sex, older age and family history of asthma remained significant.</p> <p>Conclusions</p> <p>In Ghanaian schoolchildren, high BMI was not associated with atopy. Further studies are warranted to clarify the relationship between body weight and atopy in children subjected to rapid life-style changes associated with urbanization of their environments.</p

Antimicrobial protein and Peptide concentrations and activity in human breast milk consumed by preterm infants at risk of late-onset neonatal sepsis

Objective: We investigated the levels and antimicrobial activity of antimicrobial proteins and peptides (AMPs) in breast milk consumed by preterm infants, and whether deficiencies of these factors were associated with late-onset neonatal sepsis (LOS), a bacterial infection that frequently occurs in preterm infants in the neonatal period. Study design: Breast milk from mothers of preterm infants (≤32 weeks gestation) was collected on days 7 (n = 88) and 21 (n = 77) postpartum. Concentrations of lactoferrin, LL-37, beta-defensins 1 and 2, and alpha-defensin 5 were measured by enzyme-linked immunosorbent assay. The antimicrobial activity of breast milk samples against Staphylococcus epidermidis, Staphylococcus aureus, Escherichia coli, and Streptococcus agalactiae was compared to the activity of infant formula, alone or supplemented with physiological levels of AMPs. Samples of breast milk fed to infants with and without subsequent LOS were compared for levels of AMPs and inhibition of bacterial growth. Results: Levels of most AMPs and antibacterial activity in preterm breast milk were higher at day 7 than at day 21. Lactoferrin was the only AMP that limited pathogen growth >50% when added to formula at a concentration equivalent to that present in breast milk. Levels of AMPs were similar in the breast milk fed to infants with and without LOS, however, infants who developed LOS consumed significantly less breast milk and lower doses of milk AMPs than those who were free from LOS. Conclusions: The concentrations of lactoferrin and defensins in preterm breast milk have antimicrobial activity against common neonatal pathogens