An observational study examining the relationship between respiratory symptoms, airway inflammation and bacteriology in children with severe neurodisability.

BACKGROUND: Children with severe neurodisability (ND) commonly suffer from chronic respiratory symptoms that impact greatly on quality of life, and lead to recurrent hospital admissions. This morbidity (and its causes) is poorly described, despite being well recognised by paediatricians. In this study, we characterised respiratory symptoms in children with ND at times of stability and deterioration. We also assessed the relationship between respiratory symptoms, lower airway inflammatory markers and levels of infection/colonisation. METHODS: ND children were recruited upon admission for elective surgery (Elective-ND [n = 16]), or acutely upon admission to Intensive Care (PICU-ND [n = 19]), and compared to healthy control children [n = 12]. Parents completed a validated respiratory symptom questionnaire in which symptoms associated with activity were removed (total maximal score of 108). Bronchoalveolar lavage (BAL) was collected, and BAL neutrophil counts, IL-8 and TGFß-1 levels measured. BAL microbial analysis was performed using a 16S/18S rRNA gene based assay and Pseudomonas aeruginosa PCR. RESULTS: All ND children had high levels of respiratory symptoms (median [IQR] symptom score PICU-ND, 55[38-64]; Elective-ND, 26[7-45]; Control, 4[0-7]: p<0.01), which affected their families, particularly at nighttime. Elective-ND patients with a total respiratory symptom score >20 invariably had BAL neutrophilia. Elective patients with 16S/18S microbial rDNA positive BAL had higher neutrophil counts (positive, 33[18-70]%; negative, 8[4-38]%: p<0.05) and generally higher symptom scores (positive, 17[5-32]; negative, 5[0-9]: p = 0.097). Streptococcus mitis was commonly identified in BAL from ND children; Pseudomonas aeruginosa was not identified in any sample. CONCLUSIONS: Children with severe ND often have high levels of chronic respiratory symptoms, which may relate to lower airway inflammation. Bacterial airway colonisation, particularly with oral commensals, may play a role in both symptom generation and inflammation.

Hidden treasure in an endoscopically retrieved oesophageal trichobezoar.

A 12-year-old girl with Smith-Lemli-Opitz syndrome and gastrostomy dependency presented with multiple episodes of coffee ground vomits. An upper gastrointestinal endoscopy revealed a trichobezoar in the lower oesophagus, with a 'hidden treasure'-a retained end of a G tube at the core. Endoscopic retrieval led to resolution of symptoms. Literature is scant with only one previous report of an oesophageal trichobezoar. Techniques of removal of percutaneous endoscopic gastrostomy in children are reviewed. The pathogenesis, preventative measures and management for oesophageal trichobezoars are discussed.

Histiocytic sarcoma presenting with chylous ascites in a 7-month-old infant: a case report.

We describe in this report what we believe to be the first report of a rare presentation of a very rare tumor, especially in this age group. We highlight the importance of early consideration of malignancy as a cause of chylous ascites in infancy and we discuss different causes of chylous ascites.

Nutritional support via percutaneous endoscopic gastrostomy in children with cardiac disease experiencing difficulties with feeding.

Adequate nutrition is crucial to the management of children and infants with cardiac disease. Difficulties with feeding are extremely common, and maintaining an adequate caloric intake, in order to achieve sustained growth, is often not possible without nutritional support. We retrospectively reviewed our experience between 1995 and 1999 in treating 37 children with cardiac disease who underwent percutaneous endoscopic construction of a gastrostomy to augment nutritional needs. We stratified the patients into those with cyanotic heart disease, when saturations of oxygen were less than 95%; those with non-cyanotic heart disease with saturations greater than 95%, and those with minor cardiac disease associated with a systemic disorder. Each group was compared to control children matched for age, sex, and diagnosis. We evaluated, the variation in standard deviation score for body weight over a median period of follow-up of 295 days. Improvements in the standard deviation score for body weight occurred in each of the groups, whereas children in the control groups demonstrated a decrease in standard deviation score for body weight. The median change of the score for body weight was significantly higher in patients managed with gastrostomy compared to controls. We conclude that supplementation using a gastrostomy tube allows the safe delivery of the caloric intake needed to support malnourished children with cardiac disease.