Clementina Calabrese

P114 Also adipose tissue pays consequencies of perinatal asphyxia

D.R. is born at term of physiological pregnancy by caesarean section. Birth weight was 3520 g, APGAR 7/9. At birth he presented axial hypotonia, hyporeactivity, groan, mild respiratory distress. Started Non Invasive Ventilation for 2 days with good improvement of outcome. At 4 day life, we started antimicrobial therapy and immunoglobulins ev because of elevated inflammatory markers and low platelet counts (PLT 47000/mcl) confirmed at the blood smear. After 2 weeks, on the neck and on the right cheek, finding of erythematous nodules of a few millimetres of diameter. In the days after, the lesions are also extended to the left cheek and became larger up to about 3 cm. At the ultrasound they appeared as ‘delimited echogenic areas of 19 × 14 mm at left, 17 × 12 mm at right whit intrinsic vascularisation and of irregular structure. Laboratory pannels (hepatic, renal, coltural exams, procalcitonin) were negative, ESR 24 mm/h, CRP 23.6 mg/L. About 30 days after, we see a reduction of the lesions in spite of a persistent increase of inflammatory markers. Therefore, a bacterial aetiology appeared ulikely saw that antimicrobial therapy so soon established, had no effects on them. Subcutaneous fat necrosis of the newborn is a relatively rare and transient condition that appeared in the first weeks of life in term infants with a perinatal suffering. This condition is caractherized by single or multiple subcutaneous nodules, isolated or confluent in plaques, erythematous/purplish, sometimes with little depressions, calcifications or necrosis inside; symmetrically distributed on the back, shoulders, buttocks, cheeks and at the root of the limbs; often painful at palpation. These nodules grow for some weeks and completely resolve within few months. Etiopathogenesis is probably linked to ischaemic injury, hypoxia, hypothermia and/or stress damage on the immature adipose tissue in infants with perinatal asphyxia. Maternal hypertension, gestational diabetes, family history of thrombophilia and dyslipidemia are considered risk factors. The diagnosis is clinical; the cutaneous biopsy is of support. Important for diagnosis are also the Ultrasonography and RMN. Hypercalcemia is the most dangerous complication; less frequent consequencies are thrombocytopenia, metabolic disorders (hypertriglyceridemia, hypoglycemia) and atrophic development of the nodular lesions.

P295 Optical pathway glioma and neurofibromatosis type 1

Background Neurofibromatosis Type 1 (NF1), a autosomal dominant disorder, which mainly involves Skin and nervous system: 15% of children with NF1 develops optic pathway gliomas (OPG), typically Astrocytomas pilocytic low grade, usually in the first decade of life. In one third of cases OPG occur with proptosis, visual impairment, early puberty or other disorders of hypothalamus-pituitary axis. According to current guidelines, children with NF1 should be screened frequently for visual examination and in case of visual impairment, brain MRI with the orbits analysis should performed. The current SIOP-LGG 2004 recommendations, in all cases of no-symptomatic, no-evolving glioma, provide clinical observation with close monitoring; chemotherapy is indicated only in presence of clinical signs and symptoms, or neuroimaging of progression. In the optic nerve tumours, surgery is reserved for patients with blindness, severe proptosis; chiasmatic tumours need surgery in case of exophytic tumours, with cystic component causing severe hydrocephalus or brain compression. patients and methods This is an observational study of 14 cases of OPG (5 males and 9 females) in patients with NF1, diagnosed from 1999 to 2015 c/o Paediatric Oncology Unit of our Department. We evaluated the clinical and radiological course and response to treatment of OPG. Results OPG were located exclusively to the optic nerves level in 43% of patients; 38% presented hypothalamus-optical-chiasmatic localization and 19% had chiasm or optic tracts involvment. The 57% of patients needed of chemotherapy according to the Protocol for Low Grade Glioma, cause of worsening of visual acuity, exophthalmoses or rapid increase of mass-size. The average age of chemotherapy start was 4.6 years (range 1.9 to 7 years). In only two cases there was marked reduction in the size of glioma, while in other cases the radiological picture was confirmed stationary at the stop-Therapy. Chemotherapy has allowed to stabilise the visual impairment, improving in 50% of cases the visual field. Conclusions Our data, accordingly with literature, confirmed that chemotherapeutic treatment for OPG is still unsatisfactory in most cases.

P104 A case of allergic enterocolitis to milk protein in a down baby

Anna, a term baby born by Caesarean section, firstborn, Apgar 8/9, born SGA 2.630 g weight; performed in karyotype of Down syndrome suspected ’free trisomy of chromosome 21’, echocardiography and abdominal ultrasound normal, ABR REFER, discharged in 8th day of life with diet of mother’s milk integrated with zero infant formula. Hospitalisation at one month of life due to serious clinical conditions (dehydration, hypovolemic shock, metabolic acidosis, PCR 67,4 mg/L, PCT 4,45 ng/ml, leukocytosis 63870/microL, weight 2447 g); negative blood, liquor, urine and stool cultures; Chest and Abdomen X-ray showed meteoric distension of the intestinal loops. She began antibiotic (Ampicillin+Sulbactam, Netilmicin, Ceftazidime) and immunoglobulin therapy with improvement and reduction of inflammatory markers; feeding with zero infant formula. After the fourth day of hospitalisation spontaneous emission of greenish bloody stools and then liquid stools, hypotonus, metabolic acidosis, bulbous abdomen, fever, increased inflammatory markers and leukocytosis occurred despite antibiotic therapy. New negatives cultures tested, negative parasitological faecal examination, positive faecal calprotectin (702.59 mg/kg); lymphocyte subpopulations and normal immunoglobulins. Anaemia with need of blood transfusion. Therapy replaced with Meropenem and Vancomycin and diet with hydrolyzed infant formula with slight improvements. The appearance of two similar to previous episodes in the following days suggested to treat with antibiotics and immunoglobulins. After gastroenterological consulting, in allergic enterocolitis to milk protein suspect, it replaced diet with hydrolyzed infant formula with amino acids. The following days progressive clinical improvement noted, weight increase, normal inflammatory markers, good conditions and subsequently discharge. Three weeks later, during medical check-up, she appeared in good clinical condition, increase in weight of 650 g

EVALUATION OF VITAMIN D LEVELS IN A PAEDIATRIC POPULATION OF SOUTHERN ITALY

OBJECTIVES To evaluate the pharmacokinetics and acid-suppressive effects of esomeprazole in infants with gastroesophageal reflux disease (GERD). PATIENTS AND METHODS In this single-blind, randomized, parallel-group study, 50 infants 1 to 24 months old with symptoms of GERD, and ≥ 5% of time with intraesophageal pH <4 during 24-hour dual pH monitoring, received oral esomeprazole 0.25 mg/kg (n = 26) or 1 mg/kg (n = 24) once daily for 1 week. Intraesophageal and intragastric pH were recorded at 1 week, and blood samples were taken for pharmacokinetic analysis. RESULTS At baseline, mean percentages of time with intragastric pH > 4 and intraesophageal pH < 4 were 30.5% and 11.6%, respectively, in the esomeprazole 0.25 mg/kg group and 28.6% and 12.5% in the esomeprazole 1 mg/kg group. After 1 week of treatment, times with intragastric pH >4 were 47.9% and 69.3% in the esomeprazole 0.25 mg/kg and 1 mg/kg groups, respectively (P < 0.001 vs baseline), and times with intraesophageal pH < 4 were 8.4% (P < 0.05 vs baseline) and 5.5% (P < 0.001 vs. baseline), respectively. The mean number of acid reflux episodes of > 5 minutes duration decreased from 6 at baseline to 3 and 2 with esomeprazole 0.25 mg/kg and 1 mg/kg, respectively. The geometric mean AUC0-t of esomeprazole were 0.24 and 1.79 μmol · h/L for the 0.25 mg/kg and 1 mg/kg dosages of esomeprazole, respectively. Both esomeprazole dosages were well tolerated. CONCLUSIONS Oral treatment with esomeprazole 0.25 mg/kg and 1 mg/kg was well tolerated and provided dose-related acid suppression, dose-related exposure to esomeprazole, and decreased esophageal acid exposure in infants 1-24 months old with GERD.Objectives: To evaluate the pharmacokinetics and acid-suppressive effects of esomeprazole in infants with gastroesophageal reflux disease (GERD). Patients and Methods: In this single-blind, randomized, parallel-group study, 50 infants 1 to 24 months old with symptoms of GERD, and ≥5% of time with intraesophageal pH <4 during 24-hour dual pH monitoring, received oral esomeprazole 0.25 mg/kg (n = 26) or 1 mg/kg (n = 24) once daily for 1 week. Intraesophageal and intragastric pH were recorded at 1 week, and blood samples were taken for pharmacokinetic analysis. Results: At baseline, mean percentages of time with intragastric pH >4 and intraesophageal pH <4 were 30.5% and 11.6%, respectively, in the esomeprazole 0.25 mg/kg group and 28.6% and 12.5% in the esomeprazole 1 mg/kg group. After 1 week of treatment, times with intragastric pH >4 were 47.9% and 69.3% in the esomeprazole 0.25 mg/kg and 1 mg/kg groups, respectively (P < 0.001 vs baseline), and times with intraesophageal pH <4 were 8.4% (P < 0.05 vs baseline) and 5.5% (P < 0.001 vs. baseline), respectively. The mean number of acid reflux episodes of >5 minutes duration decreased from 6 at baseline to 3 and 2 with esomeprazole 0.25 mg/kg and 1 mg/kg, respectively. The geometric mean AUC0–t of esomeprazole were 0.24 and 1.79 &mgr;mol·h/L for the 0.25 mg/kg and 1 mg/kg dosages of esomeprazole, respectively. Both esomeprazole dosages were well tolerated. Conclusions: Oral treatment with esomeprazole 0.25 mg/kg and 1 mg/kg was well tolerated and provided dose-related acid suppression, dose-related exposure to esomeprazole, and decreased esophageal acid exposure in infants 1–24 months old with GERD.

PP30 LOW LEVELS OF 6-KETO PGF 1α AND INCREASED CAROTID INTIMA–MEDIA THICKNESS IN OBESE CHILDREN WITH INSULIN RESISTANCE

PP30 – Table 1 Glucose Insulin HOMA Cholesterol Triglyceride TNF-α 6-keto PG (mg/dl) (μUI/ml) (mg/dl) (mg/dl) (pg/ml) (pg/ml) Group A (76 pts) 89.3±6.6 14.6±7.7 3.2±1.7 161±29 105.4±60 10.1±5.4 185.1±24.1 (44 pts) Group B (80 pts) 88.0±8.5 11.0±5.8 2.4±1.3 162±32 85.4±50 14±7.5 492.6±91.8 (26 pts) p NS 0.001 0.002 NS 0.025 NS 0.002 and cirrhosis) usually occur in middle and late age, atherosclerotic process in the vascular wall and liver damage might begin very early in obese children. Aim of the study: was to investigate, in children attending our outpatient clinic for obesity, 1) any early vascular structural change and the presence of fatty liver; 2) any possible metabolic risk factor. Patients and methods: the study population consisted of 156 children (71 girls), whose BMI z-score was 4.5±1.4 and their mean age was 8.2±2.7 years. They all underwent ultrasonography to measure the intima-media thickness (IMT) of carotids and to study the morphology of the liver. Their serum glucose, insulin, HOMA index, lipids, TNF-α were measured as well. For those who gave their consent (70 pts), 6-keto PGF 1α was also evaluated. Results: according to IMT measurement, patients were divided in Group A (0.6-0.7 mm) with a BMI z-score of 4.6±1.4* and Group B (0.4-0.5 mm) with a BMI z-score of 4.1±0.9* (*p= 0.2). Their data are reported in Table 1. Liver steatosis was present in 23/76 pts of Group A (30.2%) and in 11/80 pts of Group B (13.7%) Conclusions: Increased carotid IMT was documented in 76/156 children (48.7%) and fatty liver in 34/156 (21.8%). Fasting insulin levels, HOMA index, triglyceride levels and 6-Keto-PGF1α were significantly different between Group A and Group B. These data might suggest that higher insulin resistance and triglyceride levels, together with low levels of 6-keto PGF 1α could be considered risk factors for early vascular damage and hepatic steatosis in obese children.