Influence of percutaneous endoscopic gastrostomy on gastro-oesophageal reflux evaluated by multiple intraluminal impedance in children with neurological impairment.

AIM: The aim of the study was to estimate the influence of percutaneous endoscopic gastrostomy (PEG) placement on gastro-oesophageal reflux (GOR) by using multiple intraluminal impedance (MII/pH) measurements in children with neurological impairments. METHOD: Fifteen children with neurological impairments (cerebral palsy, n=10; cerebroidolipofuscinosis, n=2; Aicardi syndrome, n=1; and secondary encephalopathy, n=2) were investigated (interquartile range [IQR] 6y 4mo-14y 8mo; median age 10y 2mo; eight male, seven female). Individuals with nutritional disorders that could not be corrected by physiological means or with swallowing disorders that either caused chronic respiratory symptoms or prevented food intake were included in the study. The exclusion criteria included previous major abdominal surgery and a lack of consent for PEG. Participants underwent MII/pH for a 24-hour period and had an oesophagogastroduodenoscopy before PEG placement, which was repeated 6 to 8 months later. RESULTS: At baseline, GOR was detected in 6 of the 15 participants, and the second MII/pH session revealed GOR in 2 of the 15 children. Differences between quantitative GOR indices obtained before and after PEG were not statistically significant, except for the proportion of the acidic/weakly acidic reflux events - among all participants in the first examination, 159 reflux episodes were acidic and 244 were weakly acidic, while in the follow-up recordings the proportion was inverted (244 acidic, 136 weakly acidic; χ(2) =47.0; p<0.001). Baseline endoscopy did not reveal any macroscopic changes in any of the examined individuals, but the follow-up examination revealed oesophagitis in two participants. The median body weight gain after 6 months as 22.0% (IQR 14.4-29.2%). All participants tolerated PEG feeding well, regardless of MII/pH results. INTERPRETATION: Identification of GOR based on MII/pH in children with neurological impairments does not exclude a good clinical response to PEG feeding.

[Rare case of congenital cystic adenomatoid malformation associated with polycystic kidney disease]. / Rzadki przypadek wspolwystepowania wrodzonej torbielowatosci gruczolakowatej pluca z wielotorbielowatoscia nerek.

Congenital cystic adenomatoid malformation (CCAM) is a rare pulmonary abnormality that results from aberrant fetal lung development. It about 4-26% of cases it can be associated with other congenital abnormalities. We describe a case of congenital cystic adenomatoid malformation 2 associated with polycystic kidney disease. The association of these two congenital malformations is exceptional. Only four similar cases have been reported in the literature. A 2-year-old girl was referred to the Department of Paediatric Surgery and Oncology Medical University of Lodz with pneumonia and left pneumothorax. For three weeks prior to referral the patient was treated with antibiotics. Chest x-ray revealed hyperinflation of left upper lobe with mediastinal shift to right. Computer tomographic scan of the lung revealed multiple cyst in the left upper lobe, left-site pneumothorax and mediastinal shift to the right. The patient underwent thoracotomy. Intraoperatively, multiple cysts in the left upper lobe were found and left upper lobectomy was performed. Histologic study was compatible with type 2 congenital cystic adenomatoid malformation. Ultrasound examination showed multilocular cysts in both kidneys. The dimensions of the cysts were: MWR4. 54x45x45 mm and 25x21x24 mm on the left and right sides, respectively. Significant increase in cyst size on the left side was observed. Ten months after first hospitalization resection of the cystic lower pole of the left kidney was performed. The presence of even a single renal cyst in a child with CCAM is an indication for further follow up examinations.

Study of insulin-like growth factor-1 (IGF-1) and platelet-derived endothelial cell growth factor (PDEGF) expression in children with infantile hypertrophic pyloric stenosis.

BACKGROUND: The pathogenesis of infantile hypertrophic pyloric stenosis (IHPS) is not fully understood. Hypertrophy of the pyloric muscle is probably regulated by growth factors. Recent studies reported an increase in the local synthesis of insulin-like growth factor-1 (IGF-1). There are no reports concerning platelet-derived endothelial cell growth factor (PDEGF) playing an important role in the pathological angiogenesis. The aim of this study was to analyze the expressions of IGF-1 and PDEGF by immunohistochemistry (IHC) in the muscularis propria of the pyloric muscle in children with IHPS. MATERIAL/METHODS: Twenty-two muscle biopsies were obtained at the time of pyloromyotomy. The control group consisted of seven children. Specimens were evaluated by routine histopathological methods and by immunohistochemistry using monoclonal mouse anti-PDEGF or -IGF-1 antibodies. Cells showing positive reaction were counted in five random 200x high-power fields. Values were expressed as the mean +/-SD of the real expression area of the analyzed marker to the total analyzed area. RESULTS: In children with IHPS the average area of PDEGF expression was 62+/-52.5, whereas in the control group it was 15+/-12.1. The average area of IGF-1 expression was 1037+/-491.9) in study group and 259+/-221.44 in the controls. Statistically significant differences were found. CONCLUSIONS: These results show a local increase in the expressions of IGF-1 and PDEGF in the muscularis propria of the pyloric muscle in children with IHPS, which may have implications to the pathogenesis of the disease.