Adverse events associated with pediatric complementary and alternative medicine in the Netherlands:a national surveillance study

Pediatric use of complementary and alternative medicine (CAM) in the Netherlands is highly prevalent. The risks of pediatric CAM use are, however, largely unknown. Therefore, a 3-year survey was carried out at the Dutch Pediatric Surveillance Unit. Pediatricians were asked to register cases of adverse events associated with pediatric CAM usage. In 3 years, 32 unique adverse events were registered. Twenty-two of these adverse events were indirect and not related to the specific CAM therapy but due to delaying, changing, or stopping of regular treatment, a deficient or very restrictive diet or an incorrect diagnosis by a CAM therapist. These events were associated with many different CAM therapies. Nine events were deemed direct adverse events like bodily harm or toxicity and one-third of them occurred in infants. Only supplements, manual therapies, and (Chinese) herbs were involved in these nine events. In one case, there was a risk of a serious adverse event but harm had not yet occurred. Conclusion: Relatively few cases of adverse events associated with pediatric CAM usage were found, mostly due to delaying or stopping conventional treatment. Nevertheless, parents, pediatricians and CAM providers should be vigilant for both direct and indirect adverse events in children using CAM, especially in infants

The peroxisome and the eye

Several childhood multisystem disorders with prominent ophthalmological manifestations have been ascribed to the malfunction of the peroxisome, a subcellular organelle. The peroxisomal disorders have been divided into three groups: 1) those that result from defective biogenesis of the peroxisome (Zellweger syndrome, neonatal adrenoleukodystrophy, and infantile Refsum's disease); 2) those that result from multiple enzyme deficiencies (rhizomelic chondrodysplasia punctata); and 3) those that result from a single enzyme deficiency (X-linked adrenoleukodystrophy, primary hyperoxaluria type 1). Zellweger syndrome, the most lethal of the three peroxisomal biogenesis disorders, causes infantile hypotonia, seizures, and death within the first year. Ophthalmic manifestations include corneal opacification, cataract, glaucoma, pigmentary retinopathy and optic atrophy. Neonatal adrenoleukodystrophy and infantile Refsum's disease appear to be genetically distinct, but clinically, biochemically, and pathologically similar to Zellweger syndrome, although milder. Rhizomelic chondrodysplasia punctata, a peroxisomal disorder which results from at least two peroxisomal enzyme deficiencies, presents at birth with skeletal abnormalities and patients rarely survive past one year of age. The most prominent ocular manifestation consists of bilateral cataracts. X-linked (childhood) adrenoleukodystrophy, results from a deficiency of a single peroxisomal enzyme, presents in the latter part of the first decade with behavioral, cognitive and visual deterioration. The vision loss results from demyelination of the entire visual pathway, but the outer retina is spared. Primary hyperoxaluria type 1 manifests parafoveal subretinal pigment proliferation. Classical Refsum's disease may also be a peroxisomal disorder, but definitive evidence is lacking. Early identification of these disorders, which may depend on recognizing the ophthalmological findings, is critical for prenatal diagnosis, treatment, and genetic counselling.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/29438/1/0000520.pd

Glycogen storage disease type I : clinical, biochemical and genetic aspects, and implications for treatment and follow-up (management of glycogen storage disease type I)

In conclusion, the ESGSD I and its related studies have added to a better understanding of the clinical course, treatment, outcome and pathophysiology of GSD I and its complications. This increased insight has offered the possibility to develop extended recommendations for long-term treatment and follow-up. However, about some hallmarks and the practical interpretation of dietary treatment controversy still exists. Furthermore, a lot of questions about the pathophysiology and management of the (long-term) complications are not solved yet. Continuation of the ESGSD I as the ISGSD I offers the possibility to tackle these questions. In 2008, we hope to present some of the answers along with improved consensus guidelines for the management of GSD I, and we will come up, for certainty, with new unsolved questions.