Randomised controlled trial comparing preoperative carbohydrate loading with standard fasting in paediatric anaesthesia.

BACKGROUND: Preoperative fasting is a major cause of perioperative discomfort in paediatric anaesthesia and leads to postoperative insulin resistance, thus potentially enhancing the inflammatory response to surgery. Addressing these problems by preoperative carbohydrate intake has not been a well-defined approach in children. METHODS: We randomised 120 children scheduled for gastroscopy under general anaesthesia to either a control group of standard preoperative fasting or a study group receiving a carbohydrate beverage (PreOp™; Nutricia, Erlangen, Germany). Their stomach contents were aspirated endoscopically, and the volume and pH measured. Perioperative discomfort was evaluated using, among other parameters, an observational pain scale in ≤4-yr-olds and a VAS in >4-yr-olds. The investigators doing the endoscopies and outcome evaluations were blinded to the study group allocation. RESULTS: Compared with fasting, carbohydrate loading was associated with significantly less gastric content (P=0.01), fewer patients experiencing postoperative nausea (P=0.028), with no significant difference in postoperative vomiting. High preoperative VAS scores (>5) were recorded for only one child in the carbohydrate group vs five children in the fasting group. Bowel cleansing for simultaneous colonoscopies (n=61) made no difference to any of the intergroup findings. CONCLUSIONS: Preoperative carbohydrates can reduce nausea and gastric content, the latter being a surrogate parameter for the risk and severity of gastric aspiration into the lungs during anaesthesia. Our study adds knowledge for preoperative fasting guidelines in paediatric anaesthesia. CLINICAL TRIAL REGISTRATION: DRKS00005020.

Persistence and reactivation of human adenoviruses in the gastrointestinal tract.

Reactivation of persistent human adenoviruses (HAdVs) is associated with high morbidity and mortality in paediatric haematopoietic stem cell transplant (HSCT) recipients. Although invasive HAdV infections mainly arise from the gastrointestinal (GI) tract, the specific sites of HAdV persistence are not well characterised. We prospectively screened biopsies from 143 non-HSCT paediatric patients undergoing GI endoscopy and monitored serial stool specimens from 148 paediatric HSCT recipients for the presence of HAdV by real-time PCR. Persistence of HAdV in the GI tract was identified in 31% of children, with the highest prevalence in the terminal ileum. In situ hybridisation and immunohistochemistry identified HAdV persistence in lymphoid cells of the lamina propria, whereas biopsies from five transplant recipients revealed high numbers of replicating HAdV in intestinal epithelial cells. The prevalence of HAdV species, the frequencies of persistence in the GI tract and reactivations post transplant indicated a correlation of intestinal HAdV shedding pre-transplant with high risk of invasive infection. HAdV persistence in the GI tract is a likely origin of infectious complications in immunocompromised children. Intestinal lymphocytes represent a reservoir for HAdV persistence and reactivation, whereas the intestinal epithelium is the main site of viral proliferation preceding dissemination. The findings have important implications for assessing the risk of life-threatening invasive HAdV infections.

Concurrent FOXP3- and CTLA4-associated genetic predisposition and skewed X chromosome inactivation in an autoimmune disease-prone family.

CLTA4 is relevant for FOXP3(+)Treg cells, and the link between skewed X chromosome inactivation (XCI) and autoimmunity is recognized. The observation of immune dysregulation polyendocrinopathy enteropathy X-linked syndrome and multiorgan endocrine autoimmune phenomena in various members of one family, associated with a CTLA4 polymorphism and skewed XCI, provides an in vivo model of how mechanisms of immune dysregulation may cooperate.

Reversibility of cirrhotic regenerative liver nodules upon NTBC treatment in a child with tyrosinaemia type I.

UNLABELLED: In a male patient with hereditary tyrosinaemia type I (HTI), NTBC [2-(2-nitro-4-trifluoro-methylbenzoyl)-1,3-cyclohexandion] treatment and a diet low in phenylalanine and tyrosine were started at the age of 4 wk. At the recommended average dosage (1 mg kg(-1)), liver failure improved transiently. After 4 mo of treatment, with increased body weight, the dose had decreased to 0.7 mg kg(-1), and diffuse cirrhotic changes in liver parenchyma and multiple nodules were visualized by ultrasonography. Multiple nodules in the liver parenchyma were differentiated from hepatocellular carcinoma by magnetic resonance imaging (MRI) using mangafodipir trisodium as a paramagnetic liver-specific contrast agent. Augmentation of NTBC dosage resulted in a decrease in serum alpha-fetoprotein levels and in significant regression of liver nodules on MRI. CONCLUSION: In HTI patients with a poor response to NTBC treatment and/or development of cirrhotic changes of liver parenchyma, augmentation of the recommended NTBC dosage may result in significant improvement of symptoms.

Prevalence of celiac disease and follow-up of EMA in children and adolescents with type 1 diabetes mellitus.

BACKGROUND: The prevalence of celiac disease (CD) in children with diabetes mellitus type 1 (DM) is significantly higher than in the nondiabetic population. Most patients with DM and associated CD do not experience typical gastrointestinal symptoms of CD. There is no agreement on the necessity of screening and management of silent CD for patients with DM or on the time scale for screening. Only few data on follow-up evaluation of children with DM and CD-related antibodies are available. METHODS: One hundred fifty-seven patients with DM (mean age, 14.8 years; range, 4-21 years; male, 83) were screened with endomysial antibodies (EMA) between 1993 and 2001. A follow-up period of at least 3 years, with at least 2 EMA measurements, was possible. Group 1 comprised 37 patients whose first measurement was at the onset of DM. Group 2 comprised 120 patients whose first measurement was during the course of the disease. In patients with positive EMA, small bowel biopsy was performed. Thyroid peroxidase (TPO), thyroglobulin (TgA), glutamate decarboxylase (GAD), antiinsulin (IAA), and islet cell antibodies (IA2) were measured in all patients. RESULTS: EMA was positive in 16 patients, in 5 at onset of DM and in 11 during the course of DM (mean duration, 33.6 months; range, 11-105 months). Biopsy results showed normal mucosa in seven patients, increased intraepithelial lymphocyte counts in one, and flat mucosa in eight. There was no significant difference between EMA-positive and EMA-negative patients regarding height, weight, HbA1c level, frequency of hypoglycemia or hyperglycemia, TPO, TgA, GAD, IAA, or IA2. CONCLUSION: This study emphasizes the high prevalence of CD in patients with DM. Although several patients already had positive EMA titers at the onset of DM, seroconversion may also occur during the course of the disease. Screening for CD with EMA or tissue transglutaminase should be included in the initial investigation of DM, but should also be repeated over time to detect late seroconversion.

Helicobacter pylori in children and adolescents: increase of primary clarithromycin resistance, 1997-2000.

BACKGROUND: The authors evaluate the prevalence of Helicobacter pylori resistance in 117 children and demonstrate the changes over a 4-year period. METHODS: In 117 children and adolescents, H. pylori-positive gastritis was revealed by diagnostic upper endoscopy. Biopsies from the antrum and body of the stomach were tested by histology, urease test, and culture. H. pylori was isolated using standard culture techniques, and susceptibility to amoxicillin, clarithromycin, and metronidazole was tested using the E-test (AB-Biodisk, Sweden). RESULTS: Endoscopy revealed gastric ulcers in 2 of 117 subjects, duodenal ulcers in 6 of 117, and erosive gastritis or duodenitis in 23 of 117. Almost all patients showed antral nodularity. Histology always showed chronic gastritis with different degrees of activity. During the 4-year study period, the authors noticed an increase of primary clarithromycin-resistant H. pylori strains, from 14.3% to 27.6% (mean, 20.3%). Metronidazole resistance varied between 5% and 25%. No resistance to amoxicillin was found. CONCLUSION: Eradication of H. pylori should take place only after testing of susceptibility. The general use of clarithromycin in children should be restricted to better-defined indications. Resistance to clarithromycin of H. pylori may also become a future problem for the treatment of adults.

Interventional stent implantation in a child with patent ductus venosus and pulmonary hypertension.

UNLABELLED: We report on the rare case of a 4-year-old boy with patent ductus venosus and pulmonary hypertension presenting with progressive fatigue, tachypnoea at rest and tachycardia. Cardiac catheterisation revealed suprasystemic pressure in the pulmonary arteries with severely elevated pulmonary vascular resistance. In order to reduce the diameter of the ductus venosus, a stent was implanted interventionally, which closed, as expected, spontaneously 2 years later. Pulmonary arterial pressure and pulmonary vascular resistance decreased significantly and the general condition of the boy improved dramatically. CONCLUSION: To the best of our knowledge, this represents the first report of successful interventional stent occlusion of a patent ductus venosus associated with severe pulmonary hypertension. The future will tell whether this intervention is curative or represents a bridging procedure for subsequent liver transplantation.

[Progressive familial intrahepatic cholestasis (Byler disease): current genetics and therapy]. / Die Progressive Familiäre Intrahepatische Cholestase (Morbus Byler): Aktuelles zu Genetik und Therapie.

Progressive familial intrahepatic cholestasis (PFIC) is a congenital liver disease. First symptoms can frequently be seen shortly after birth. Quality and expectation of life are substantially reduced due to severe pruritus and the complications of progressive liver cirrhosis. PFIC is diagnosed on the basis of characteristic clinical and laboratory parameters and genetic analysis after exclusion of other liver diseases leading to intrahepatic cholestasis. Medical therapy is only effective in a proportion of children with PFIC. Partial biliary diversion (PBD) is nowadays considered the therapy of choice in patients with therapy-refractive pruritus. If performed in time, damage to the liver can be delayed or arrested, thus orthotopic liver transplantation (OLT) can be postponed or even avoided in at least some patients with PFIC. Besides providing a current overview of PFIC, we report on three patients who were successfully treated surgically. One patient was subjected to a new technique of PBD (cholecysto-appendicostomy), the other two had OLT.

Budd-Chiari syndrome associated with coagulation abnormalities in a child with carbohydrate deficient glycoprotein syndrome type Ix.

A 6-year-old male patient presented with Budd-Chiari syndrome and glycoprotein abnormalities associated with carbohydrate deficient glycoprotein syndrome type I with yet unidentified molecular defect (type Ix). Budd-Chiari syndrome most likely developed after hepatic venous thrombosis caused by coagulation abnormalities resulting from hypoglycosylation and functional impairment of anticoagulant proteins.

Screening by anti-endomysium antibody for celiac disease in diabetic children and adolescents in Austria.

BACKGROUND: Unrecognized celiac disease (CD) may be found in a substantial proportion of patients with type I diabetes mellitus. METHODS: A cohort of 403 Austrian children and adolescents with type I diabetes mellitus (210 males and 193 females; age range, 1-22 years) was screened for celiac disease using the IgA anti-endomysium antibody test (EMA) and the immunoglobulin (Ig)G anti-gliadin (AGA-IgG) and IgA anti-gliadin (AGA-IgA) antibody test. RESULTS: Twelve patients' sera (2.98%) yielded positive EMA results and two patients' sera (0.49%) with IgA deficiency had high AGA-IgG values. All but one of these patients underwent intestinal biopsy. Six (1.49%) had clear histologic evidence of CD (flat mucosa), whereas three (0.74%) showed minor histologic changes (increase in intraepithelial lymphocytes) and four (0.99%), including the EMA-negative patients with IgA deficiency, had a normal mucosa. When the cases with silent and potential CD were combined, the overall prevalence in the current cohort was 2.98%. There was no difference in the hemoglobin (Hb)A1c level between antibody-positive and -negative patients, and subsequent gluten-free diet did not change this metabolic parameter. CONCLUSION: The prevalence of clinically unrecognized CD, found by EMA screening, is much higher in Austrian children with diabetes than in a comparable population without diabetes. The prevalence of CD in diabetic children in Austria is distinctly lower, however, than in several other countries.

Early sensitization to airborne allergens.

We report the case of a 7-month-old child with failure to thrive. Celiac disease was suspected because of highly raised antigliadin IgA and IgG antibodies and subtotal villous atrophy. In peripheral blood mononuclear-cells cellular proliferation was found in response to birch pollen, rye pollen and hazelnut extract. Born in June 1992 the infant had not yet experienced a birch pollen season. He had been fed with birch pollen allergy-associated carrot, apple and potato beginning at 6 weeks of life. In the serum, specific IgG, IgM and IgA to birch pollen and profilin, rye pollen and hazelnut antigens were detectable, indicating possible in utero sensitization or T cell cross-reactivity due to early sensitization with related food antigens.

[Adverse health effects in children caused by passive smoking]. / Gesundheitsschäden durch Passivrauchen bei Kindern.

The harmful effects of passive smoking (PSE) start early in intrauterine life and comprise direct toxic effects of components of tobaccos smoke on the fetus as well as indirect effects by impeding the normal nutrition of the growing child over effects on the placenta. Consequences are diminished birthweight, and increased perinatal mortality. The sudden infant death syndrome is associated with PSE as are increased incidence of respiratory illnesses in early childhood. Increased bronchial responsiveness, increased asthma prevalence, delayed lung growth and increased incidence of chronic respiratory symptoms later in childhood may well put these children at increased risk for developing chronic obstructive pulmonary disease in their later life.