Halina Wos

Usefulness of Faecal Calprotectin Measurement in Children with Various Types of Inflammatory Bowel Disease

Introduction. The aim of the study was to assess the usefulness of the FC measurement in children with various types of IBD and relation to the disease activity. Patients and Methods. 91 patients (49 boys: 53.85% and 42 girls: 46.15%, mean age: 13.38 years, range 6–18 years) were included in the analysis. Patients were divided into the groups: B1—24 children with CD, B2—16 patients with UC, and a group comprising 31 children with other types of colitis; the control group (K) comprised 20 healthy children. FC was assayed by ELISA method, using Phical test (Calpro). Results. The mean faecal calprotectin concentrations were higher in children with CD and UC as compared to healthy controls, patients with eosinophilic, lymphocytic, and nonspecific colitis. A positive correlation was observed between FC concentrations and the disease activity (the PCDAI scale, the Truelove-Witts Scale, and the endoscopic Rachmilewitz Index). Conclusion. It seems that the FC concentrations can be a useful, safe, and noninvasive test in children suspected for IBD, since FC concentration is higher in children with CD and UC than in patients with other inflammatory diseases.

Prognostic value of proangiogenic cytokines in children with lymphomas

Angiogenesis and proangiogenic cytokines are involved in neoplastic development. The role of these processes in lymphoma formation has not been established. The aim of the study was to assess angiogenesis on the basis of serum levels of vascular‐endothelial growth factor (VEGF) and basic fibroblast growth factor (bFGF) in childhood lymphomas. The prognostic value of these parameters was determined in the examined children.

Disaccharidase activity in children with inflammatory bowel disease.

BACKGROUND/AIMS The etiopathogenesis of inflammatory bowel disease (IBD) is multifactorial and not well explained. Environmental, genetic, and dietary factors play an important role. The aim of the study was the evaluation of lactase, saccharase, and maltase activity in patients with IBD. MATERIALS AND METHODS The study comprised 65 children, aged 3-18 years. During a routine endoscopy, we took biopsies from the descending part of the duodenum. In these biopsies, we determined disaccharidase activity using Dahlquists method. RESULTS Decreased lactase activity in the biopsies taken from the small intestine mucosa was most frequently observed in patients with Crohns disease (5/15-33%) and least frequently seen in children with lymphocytic colitis (in 1/10-10%). The lowest mean values of lactase activity were found in the children with Crohns disease and ulcerative colitis (1.7-2.5 U/1 g). Decreased saccharase activity in the biopsies obtained from the small intestine mucosa was most frequently observed in patients with lymphocytic colitis (in 5/10-50%) and ulcerative colitis (9/20-45%) and least frequently seen in children with non-specific undetermined colitis (in 7/20-35%). Decreased maltase activity in the small bowel mucosa was the most frequently observed in patients with Crohns disease (in 5/15-33%) and least frequently seen in children with ulcerative colitis (in 3/20-15%). The lowest mean values of maltase activity were found in the children with Crohns disease (5.4 U/1 g). CONCLUSION Therefore, it seems reasonable to perform diagnostic examinations aimed at lactose, saccharose, and maltose intolerance and to initiate a dietary regimen in children with IBD.

Cervical Ectopic Thymus in a 9-month-old Girl—diagnostic Difficulties

Ectopic cervical location of the thymus is a very rarely diagnosed developmental disorder in children. This anomaly is usually manifested clinically as a neck tumor suggesting lymphadenopathy, which is also differentiated from more frequently occurring cervical cysts, angiomas, or malignant neoplasms. A decisive examination is the histopathologic assessment of the tumor, supported by necessary immunoassays. A diagnostic process is sometimes very difficult and the results are ambiguous; what is more, this process has a decisive impact on the childs future life. Therefore, a histopathologic evaluation performed by 2 independent pathologists should become the standard procedure. We present the case of a 9-month-old girl, in whom, after a difficult diagnostic process, ectopic cervical location of the thymus was diagnosed.

Serum levels of vascular endothelial growth factor (VEGF) and basic fibroblast growth factor (bFGF) in children with brain tumours

BACKGROUND Angiogenesis is the process of new vessel formation originating from the existing vascular network. It plays an important role in the growth and spread of malignancies, including brain tumors. The process of angiogenesis is characterized by increased expression of vascular endothelial growth factor (VEGF) and basic fibroblast growth factor (bFGF), and by the release of their soluble forms into circulation. OBJECTIVES The aim of the study was to evaluate serum levels of VEGF and bFGF in children with malignant and benign brain tumors. MATERIAL AND METHODS The study group (group N) included 106 children diagnosed with brain tumors. The children in group N were classified according to tumor pathology into 3 subgroups: N1 (n = 63): patients with malignant tumors, excluding anaplastic astrocytoma (AA) and glioblastoma multiforme (GBM); N2 (n = 25): patients with benign tumors; and N3 (n = 18): patients with high grade gliomas (AA and GBM). VEGF and bFGF were determined by ELISA in blood samples before the initiation of chemotherapy. VEGF and bFGF levels were compared within the subgroups in relation to tumor grading and the extent of surgery. RESULTS The median VEGF in patients with brain tumors was significantly higher than in the control group. The median levels of VEGF and bFGF in subgroup N1 were significantly higher than in the control group. The differences in VEGF and bFGF concentrations between the subgroups in relation to the extent of tumor resection were not significant. CONCLUSIONS Significantly higher plasma VEGF levels in children with brain neoplasms may reflect enhanced angiogenesis in the tumors.

180 Evaluation of leptin and ghrelin levels in the blood serum of patients diagnosed with cystic fibrosis

Objectives Leptin and ghrelin are peptide hormones which, working antagonistically, regulate the energy balance of the body. The aim of the study was to evaluate the levels of leptin and ghrelin in the blood serum of patients diagnosed with cystic fibrosis (CF), in relation to their nutritional status and function of liver cells. Methods The study group consisted of 34 patients, aged from 3 months to 18 years (mean age 4.5 years) who were diagnosed with CF on the basis of screening and/or diagnostic tests. The analysis included the nutritional status, CFTR gene mutation, assessment of the exocrine pancreas function, abnormal lipid metabolism, as well as liver cell function parameters. In all children, serum leptin and ghrelin levels were measured by means of an immunoenzymatic test, using reagents from DRG Instruments. The results were statistically analyzed. Results Serum ghrelin levels were significantly lower in the youngest patients ( Conclusion Ghrelin and leptin levels in children with cystic fibrosis correlate with the nutritional status and can be an early marker of exocrine pancreatic insufficiency.

Concentrations of Ghrelin and Leptin in Children with Cystic Fibrosis

Ghrelin and leptin are peptide hormones which, working antagonistically, regulate the energy balance of the body. Abnormal concentrations of these hormones are observed in the course of gastrointestinal diseases, where the clinical picture is characterized by nutritional disorders. Approximately 70% of patients with cystic fibrosis have body weight deficiency due to exocrine pancreatic insufficiency, respiratory failure, and secondary circulatory disorders as well as eating disorders. The aim of the study was to evaluate the levels of ghrelin and leptin in the blood serum of patients diagnosed with cystic fibrosis, in relation to their nutritional status and function of liver cells. Patients and methods: The study group consisted of 34 patients, 17 girls (50%) and 17 boys (50%), aged from 3 months to 18 years (mean age 4.5 years) who were diagnosed with cystic fibrosis on the basis of screening and/ or diagnostic tests. The analysis included the nutritional status (body weight and height, BMI), CFTR gene mutation, assessment of the exocrine pancreas function (albumin and glucose concentration in the blood serum, acid steatocrit in stool), abnormal lipid metabolism (cholesterol and triglyceride levels in blood serum), as well as liver cell function parameters and cholestasis (serum activity amino-transpherase, gamma-glutamyl transpeptidase, concentration of bile acids, coagulation parameters). In all children, serum ghrelin and leptin levels were measured by means of an immunoenzymatic test, using reagents from DRG Instruments. The results were statistically analyzed. Results: Serum ghrelin levels were significantly lower in the youngest patients (<1 year of age) compared with older age groups. Ghrelin concentration was significantly lower in patients with salt wasting syndrome (p<0.05). The statistically lower serum leptin levels were observed in patients with growth deficiency and increased parameters of cholestasis, particularly in the youngest age group. Conclusion: Ghrelin and leptin levels in children with cystic fibrosis correlate with the nutritional status and can be an early marker of exocrine pancreatic insufficiency.

184 Evaluation of the impact of SERPINA gene mutation on the occurrence of liver damage and cholestasis in patients diagnosed with cystic fibrosis

SERPINA1 gene is present in about 2% of patients with cystic fibrosis, but more frequently, in approximately 5% of patients with cystic fibrosis (CF) and concomitant changes in the liver. Aim: The aim of this study was to assess the impact of SERPINA gene mutation on the occurrence of liver damage and cholestasis in patients diagnosed with cystic fibrosis. Patients and Methods: The analysis included 30 children, 13 girls (43.3%) and 17 boys (56.6%), aged from 6 months to 18 years, with diagnosed CF. All patients underwent genetic testing for SERPINA gene mutation. The analysis included age, sex, clinical symptoms, type of mutation of the CFTR protein, laboratory abnormalities (levels of transaminases, GGTP, FA, protein, acid steatocrit) and ultrasound examination of the abdomen. Results: Elevated transaminases were found in 9/30 (30%), whereas elevated levels of gamma-glutamyl transferase in 6/30 (20%) children. In 5/30 patients the ultrasound examination demonstrated liver enlargement with increased echogenicity. The mutation in SERPINA gene was found in 1/30 (3.3%) patient with cystic fibrosis. Currently, this patient has normal values of transaminases, GGTP and FA, whereas, a significant worsening of respiratory symptoms is observed. There was no correlation between the occurrence of SERPINA gene mutation and clinical symptoms, type of CFTR protein mutation, results of laboratory tests of liver function and hepatocyte damage, and ultrasound examination of the abdomen. Conclusions: There was no correlation between the occurrence of SERPINA gene mutation and the presence of features of liver damage and cholestasis in children diagnosed with cystic fibrosis.

193 Concentration of calprotectin in the stool of children with cystic fibrosis

Objectives: Investigation of the diagnostic process and treatment of Distal Intestinal Obstructive Syndrome (DIOS) in Norwegian Cystic Fibrosis patients in comparison with ECFS guidelines. Methods: Retrospective review of electronic patient files from 50 CF patients. 19 of these patients experienced at least one suspected episode of DIOS within the previous 5 years. The 19 patient records were systematically reviewed for information about symptoms of DIOS, clinical work-up and treatment. Results: The included patients had an equal gender distribution and were born in 1977–2007. 18 patients had a serious genetic mutation. 17 had pancreatic insufficiency. 18 patients reported two or more symptoms of DIOS. 7 patients were examined with an abdominal x-ray. 3 patients got a diatrizoinic acid lavage as a part of the diagnostic and treatment regimen. 8 patients were hospitalized and received 7 different treatment regimens. 18 patients got N-acetylcystein as prophylactic treatment after an episode of DIOS. None of the patients was treated with laxatives containing polyethylene glycol. Conclusion: Significant divergence from ECFS guidelines (2011) was identified on the hospitals performance on diagnostic work up and treatment for DIOS. Our opinion was that the attending physicians and the patients would benefit from specific guidelines in how they approach CF patients with possible DIOS. Clinical impact: Guidelines on diagnosis and management of DIOS, based mainly on the ECFS guidelines, were constructed and implemented in April 2013.

359 DIOS syndrome in 11-month-old infant with cystic fibrosis

Introduction: DIOS syndrome in patients with CF occurs with a frequency of 6.2/ 1000 patients/year, mainly in adolescents and young adults. It is extremely rare in younger children. Known risk factors for DIOS include: meconium obstructions in the neonatal period, abnormal enzyme supplementation, dietary errors, dehydration and others. Objective: Analysis of the clinical assessment of risk factors for DIOS syndrome in an 11-month-old infant suffering from cystic fibrosis. Material and Methods: We present a case of DIOS in an 11-month-old female infant diagnosed with CF by newborn screening test. The baby was referred to the district hospital because of febrile conditions, reduction in fluid consumption and eating, and ion disorders. On admission the child had features of dehydration and bloated belly with lazy motility. Additional examination revealed high rates of inflammation, electrolyte disturbances, hypoproteinaemia, and high rates of acidic steatocrit. Despite the persistence of observed treatment of infections parameters, growth of abdominal circumference, disposable vomiting. The diagnostic imaging studies, including the intestinal passage, showed retention of contrast around the ileocaecal bowel. Child consulted surgery. Oral feeding was stopped, starting rectal infusions of N-acetylcysteine, irrigation, included metronidazole, standard realimentation with enzyme supplementation. Improvement in clinical status was obtained. Conclusions: 1. Avoidance and effective treatment of states of dehydration and the proper enzyme supplementation may reduce the risk for DIOS syndrome in infants. 2. Coexistence of several risk factors in the same child greatly increases the risk of developing ovarian DIOS. 360 A case series of thrombotic and thromboembolic complications of central venous access in cystic fibrosis patients N. Kandamany1, M. Tariq1, B. Elnazir1, P. Greally1. 1Adelaide & Meath Hospital inc the National Children’s Hospital, Tallaght, Respiratory, Dublin, Ireland