Acid glycosaminoglycan (aGAG) excretion is increased in children with autism spectrum disorder, and it can be controlled by diet.

Autism research continues to receive considerable attention as the options for successful management are limited. The understanding of the autism spectrum disorder (ASD) etiology has now progressed to encompass genetic, epigenetic, neurological, hormonal, and environmental factors that affect outcomes for patients with ASD. Glycosaminoglycans (GAGs) are a family of linear, sulfated polysaccharides that are associated with central nervous system (CNS) development, maintenance, and disorders. Proteoglycans (PG) regulate diverse functions in the central nervous system. Heparan sulfate (HS) and chondroitin sulfate (CS) are two major GAGs present in the PGs of the CNS. As neuroscience advances, biochemical treatments to correct brain chemistry become better defined. Nutrient therapy can be very potent and has minimal to no side effects, since no molecules foreign to the body are needed. Given GAGs are involved in several neurological functions, and that its level can be somewhat modulated by the diet, the present study aimed to evaluate the role of GAGs levels in ASD symptoms. Both tGAG and its different fractions were evaluated in the urine of ASD and healthy control childrens. As levels differed between groups, a second trial was conduted evaluating if diet could reduce tGAG levels and if this in turn decrease ASD symptoms. The present study found that tGAG concentration was significantly higher in the urine of children with ASD compared to healthy control children and this was also evident in all GAG fractions. Within groups (controls and ASD), no gender differences in GAG excretion were found. The use of a 90 days elimination diet (casein-free, special carbohydrates, multivitamin/mineral supplement), had major effects in reducing urinary tGAG excretion in children with ASD.

[New technique for intestinal lengthening -- from the idea to the clinical application]. / Új bélhosszabbító mutéti technika -- az ötlettol a klinikumig.

INTRODUCTION: In severe short bowel syndrome, as a result of the natural adaptation, the bowel becomes overdilated, this interferes with the persitalsis and may lead to stasis, bacterial translocation and sepsis. At present two techniques are used to improve peristalsis. The Bianchi procedure is technically challenging, the Serial Transverse Enteroplasty (STEP) is easy however it results in an aphysiological ultrastructure altering the orientation of the muscle fibres. Our aim was to develop an easy technique, which does not alter intestinal muscular ultrastructure dramatically. MATERIAL AND METHODS: The idea, Spiral Intestinal Lengthening and Tailoring (SILT), is based on a spiral shape incision of the intestine and retubularisation in a longer but narrower fashion. The feasibility and the effect on the muscular ultrastructure were tested on bowelsimulator and porcine intestine. The intramural microcirculation was checked with intravital microscopy. The outcome was assessed on minipigs (n = 6) than clinical application was commenced. RESULTS: SILT was feasible, did not change the orientation of muscle fibres significantly, did not compromised microcirculation, no surgical complication was noted when tailoring did not exceed 75%. The first clinical application was successful. CONCLUSION: SILT is a safe and easy technique and not altering the intestinal musculature significantly.

High-throughput live-cell imaging reveals differential inhibition of tumor cell proliferation by human fibroblasts.

Increasing evidence indicates that cancer development requires changes both in the precancerous cells and in their microenvironment. To study one aspect of the microenvironmental control, we departed from Michael Stoker's observation (Stroker et al, J Cell Sci 1966;1:297-310) that normal fibroblasts can inhibit the growth of admixed cancer cells (neighbour suppression). We have developed a high-throughput microscopy and image analysis system permitting the examination of live mixed cell cultures growing on 384-well plates, at the single cell level and over time. We have tested the effect of 107 samples of low passage number (<5) primary human fibroblasts from pediatric and adult donors, on the growth of six human tumor cell lines. Three of the lines were derived from prostate carcinomas, two from lung carcinomas and one was an EBV transformed lymphoblastoid line. Labeled tumor cells were grown in the presence of unlabeled fibroblasts. The majority of the tested fibroblasts inhibited the proliferation of the tumor cells, compared to the control cultures where labeled tumor cells were co-cultured with unlabeled tumor cells. The proliferation inhibiting effect of the fibroblasts differed depending on their site of origin and the age of the donor. Inhibition required direct cell contact. Mouse 3T3 fibroblasts inhibited the growth of SV40-transformed 3T3 cells and human tumor cells, showing that the inhibitory effect could prevail across the species barrier. Our high-throughput system allows the quantitative analysis of the inhibitory effect of fibroblasts on the population level and the exploration of differences depending on the source of the normal cells.

High Levels of Glycosaminoglycans in the Urines of Children with Attention-Deficit/Hyperactivity Disorder (ADHD).

Attention-deficit/hyperactivity disorder (ADHD) is a common neurobehavioral/neurodevelopmental disorder. Some early studies indicated that increased intake of added sugars might have a role in ADHD. In the present study, we tested this possibility by evaluating the urinary excretion of oligosaccharides and glycosaminoglycans (GAGs) in ADHD and control subjects. Forty ADHD subjects matched with 34 controls were enrolled in the study. The subjects underwent a standardized dietary regimen. The urine levels of oligosaccharides and GAGs were quantified biochemically, and their covariance and association were evaluated statistically. Fructose (21/40, 52.5%), maltose (26/40, 65%), galactose (30/40, 75%), and lactose (38/40, 95%) excretions were frequently found in the urine of ADHD subjects (p < 0.05), an excretion which does not occur normally. Furthermore, these subjects showed a pathologic tGAG (glycosaminoglycan) excretion (40/40, 100%). The present study supports the thesis that carbohydrate metabolism differs in ADHD subjects compared with control subjects.

[Disease course, frequency of relapses and survival of patients with juvenile or adult dermatomyositis]. / Juvenilis és felnott kori dermatomyositisben szenvedo betegek kórlefolyásának jellegzetességei, illetve a kezelés és a késobbi kórlefolyás kapcsolatának vizsgálata.

INTRODUCTION: The idiopathic inflammatory myopathies are systemic autoimmune diseases characterized by chronic muscle inflammation resulting progressive weakness and frequent involvement of internal organs, mainly the pulmonary, gastrointestinal and cardiac systems. OBJECTIVE: To present clinical characteristics, disease course, frequency of relapses and survival of 79 patients with juvenile or adult dermatomyositis. METHODS: A national registry of patients with juvenile dermatomyositis was elaborated by the authors in Hungary. The authors summarize data of the register such as signs and symptoms, disease course, frequency of relapses and survival of patients with juvenile dermatomyositis. Analysis was performed using data of 44 patients diagnosed between 1976 and 2004 according to Bohan and Peter's criteria. Survival probability was calculated by Kaplan-Meier method. Data of patients with juvenile dermatomyositis were compared with data of 35 patients with adult dermatomyositis. RESULTS: In view of the disease course, the authors found that more than the half of patients have monophasic disease, while one third of them suffered from polycyclic disease. The risk of the relapse was found to be higher during the first year after the remission. None of the juvenile patients died. Among adult patients, 4 disease-specific deaths occurred. DISCUSSION: There was no correlation between relapse free survival and initial therapeutic regimen. Many of the patients had polycyclic or chronic disease. As relapses can occur after a prolonged disease-free interval, patients should be followed up for at least 2 years. Despite favourable survival probability, further investigations are needed to assess functional outcome.