FE(NO) but not 8-isoprostane levels in exhaled breath condensate are higher in children with AE without asthma. Our finding may indicate a predictive role for FE(NO) for the development of asthma.
We measured small intestinal permeability to lactulose and rhamnose in 18 healthy children and in 15 children with atopic eczema, before and after a 14-day elimination diet. The children with atopic eczema had higher initial urinary lactulose/rhamnose ratios than the controls. After dietary restriction, there was no overall change in permeability. Dietary therapy did however result in a large reduction in permeability in three of the nine children whose skin disease was improved, but a statistically significant decrease in permeability was not observed in the diet-responsive group as a whole. Larger studies would be required to confirm that dietary restrictions can reduce intestinal permeability in some children with atopic eczema.
A small but important proportion of patients with epidermolysis bullosa (EB) may develop significant renal and urological complications which can have a major impact on their morbidity and mortality. During the last 10 years, five of a large group of children with EB under our care, with either dystrophic or junctional types of disease, experienced major nephro-urological complications. Two patients with recessive dystrophic EB (REDB) developed macroscopic haematuria - one had renal failure and underwent a renal biopsy showing IgA nephropathy. A third patient with RDEB also developed renal failure and his biopsy demonstrated postinfectious glomerulonephritis/type III membranoproliferative (mesangiocapillary) glomerulonephritis. Both patients with renal failure underwent peritoneal dialysis. Two patients with junctional EB developed obstructive uropathies, which required bladder reconstruction and the fashioning of a Mitrofanoff channel in one.
Two infants experienced subcutaneous fat necrosis (SCFN) at a relatively late age after cardiac surgery with induced hypothermia. The condition resolved in both patients over four weeks without treatment. The appearance of SCFN after the newborn period is very unusual, and in these patients was probably related to the hypothermia.
A 9-year-old boy of Greek-Cypriot origin had been diagnosed at the age of 3 years as suffering from non-bullous ichthyosiform erythroderma. However, he also had hepatomegaly and abnormal liver function tests, biochemical evidence of myopathy, early cataracts, and lipid vacuoles in white blood cells and basal keratinocytes. A diagnosis of neutral lipid (triglyceride) storage disease was confirmed by lipid studies on cultured fibroblasts.
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