Association between early and current gastro-intestinal symptoms and co-morbidities in children and adolescents with Angelman syndrome.

BACKGROUND: Angelman syndrome (AS) is a neurogenetic disorder that causes severe intellectual disability, expressive language deficits, motor impairment, ataxia, sleep problems, epileptic seizures and a happy disposition. People with AS frequently experience gastrointestinal (GI) symptoms. METHOD: This study used data from the Global Angelman Syndrome Registry to explore the relationship between early and current GI symptoms and co-morbidity in children and adolescents with AS (n = 173). Two groups that experienced a high (n = 91) and a low (n = 82) frequency of GI symptoms were examined in relation to feeding and GI history in infancy, sleep and toileting problems, levels of language and communication and challenging behaviours. Predictors of GI symptoms were then investigated using a series of logistic regressions. RESULTS: This analysis found that constipation and gastroesophageal reflux affected 84% and 64%, of the sample, respectively. The high frequency of GI symptoms were significantly associated with: 'refusal to nurse', 'vomiting', 'arching', 'difficulty gaining weight', gastroesophageal reflux, 'solid food transition', frequency of night-time urinary continence and sleep hyperhidrosis during infancy. GI symptoms were not significantly associated with sleep, toileting, language or challenging behaviours. Significant predictors of high frequency GI symptoms were gastroesophageal reflux and sleep hyperhidrosis. CONCLUSIONS: Future research needs to investigate the association between AS and GI co-morbidity in adults with AS.

Relationships among gastrointestinal symptoms, sleep problems, challenging behaviour, comorbid psychopathology and autism spectrum disorder symptoms in children and adolescents with 15q duplication syndrome.

BACKGROUND: Comorbidity is the presence of at least two disorders in one person at one time. This study examined the frequency of gastrointestinal (GI) symptoms, sleep problems, comorbid psychopathology, challenging behaviour and autism spectrum disorder (ASD) symptoms in children and adolescents with duplication 15q syndrome (Dup15q), aged 3-17 years. This study also examined whether challenging behaviour in Dup15q is predicted by age, gender, presence of an intellectual disability, sleep problems, GI symptoms and comorbid psychopathology. METHOD: Parental measures were completed by 101 parents of children and adolescents with Dup15q. Questionnaires were composed of the Children's Sleep Habits Questionnaire, Behavior Problems Inventory - Short Form, GI Symptom Inventory, Social Communication Questionnaire and the Child Behavior Checklist. RESULTS: Sleep problems (94%), GI symptoms (87%) and challenging behaviour (100%) were common comorbidities represented in the sample in this study. Significant relationships were found between challenging behaviour and the presence of co-occurring sleep problems, GI symptoms, comorbid psychopathology and ASD symptoms. Further analysis revealed that these comorbidities also predicted challenging behaviour. CONCLUSION: This research demonstrated the importance of studying the relationships between GI symptoms, sleep problems, comorbid psychopathology, ASD symptoms and challenging behaviour in Dup15q and how these conditions can shape the Dup15q phenotype.

Effect of cardiogenic shock on pharmacokinetics and tissue distribution of ampicillin-sulbactam.

A reversible cardiogenic shock model in pigs investigated shock-induced changes in the pharmacokinetics and tissue distribution of ampicillin-sulbactam and the efficacy of this antibiotic regimen in eliminating enteric bacterial translocation. Sixteen pigs were randomly allocated to 3 groups: group I (shock, ampicillin-sulbactam, n = 6), group II (no shock, ampicillin-sulbactam, n = 6), and group III (shock, no ampicillin-sulbactam, n = 4). Nalidixic acid-resistant E. coli (60 x 10(6) CFU) were instilled into a jejunal loop created in each pig, and bacterial cultures were taken from thoracic duct lymph, periportal, and mesenteric lymph nodes. Ampicillin-sulbactam was administered intravenously at a standard dose of 3 g. Results showed that 1) ampicillin and sulbactam concentrations generally increase during cardiogenic shock; 2) cardiogenic shock does not increase ampicillin concentrations in jejunum and liver; 3) during resuscitation, thoracic duct lymph ampicillin concentrations decrease; and 4) during and immediately after cardiogenic shock, standard doses of ampicillin-sulbactam appear efficacious in eliminating translocated bacteria.