The Effect of Nutritional Support on the Disease Progression and Survival in Pediatric Patients with Solid Tumors.

Cancer is one of the leading causes of death for children; however, appropriate nutritional status can positively affect survival. The aim of this study was to determine to what extent malnutrition risk screening and intensified nutrition support, provided by a professional team, promoted disease progression and survival in pediatric patients with solid tumors. 145 pediatric cancer patients (average age 6.3 ± 5.6 and 6.7 ± 5.4 years) with solid tumors undergoing chemotherapy participated in the study. Two 3-year periods were studied: 2009-2011 and 2012-2014. Patient characteristics and treatment protocols were identical, but in Period 2, with the foundation of our nutrition support team malnutrition risk screening was made mandatory upon every hospital admission. As a result of intensified nutrition support the time from diagnosis to completion of treatment (802 vs. 512 day, p < 0.001) and the need for antimycotic treatment reduced significantly (47.8% vs. 29.1%, p = 0.036). The total percentage of surviving children was 60.3% and 75.0% in Period 1 and 2 respectively. Decrease in weight-for-height percentile during treatment and central nervous system tumors are significant predictors of a less favorable survival. Malnutrition risk screening and intensified nutrition therapy have positive effects on nutritional status and therefore patient survival in pediatric cancer patients.

[Clinical nutrition therapy in patients with short bowel syndrome in line with principles of personalized medicine]. / Klinikai táplálásterápia rövidbél-szindrómában a személyre szabott orvoslás elvei szerint.

Home parenteral nutrition administered in selected care centres has been financed in Hungary since January, 2013. The authors discuss diagnostic issues, treatment and nutrition therapy of short bowel syndrome patients in line with the principles of personalised medicine. The most severe form of short bowel syndrome occurs in patients having jejunostomy, whose treatment is discussed separately. The authors give a detailed overview of home parenteral feeding, its possible complications, outcomes and adaptation of the remaining bowel. They describe how their own care centre operates where they administer home parenteral nutrition to 12 patients with short bowel syndrome (5 females and 7 males aged 51.25±14.4 years). The body mass index was 19.07±5.08 kg/m2 and 20.87±3.3 kg/m2, skeletal muscle mass was 25.7±6.3 kg and 26.45±5.38 kg, and body fat mass was 14.25±8.55 kg and 11.77±2.71 kg at the start of home parenteral nutrition and presently, respectively. The underlying conditions of short bowel syndrome were tumours in 4 patients, bowel ischaemia in four patients, surgical complications in three patients, Crohn's disease in one patient, and Crohn's disease plus tumour in one patient.

Different nutritional screening tools and recommended screening algorithm for pediatric oncology patients.

BACKGROUND & AIMS: Cancer is one of the leading causes of death for children; however, appropriate nutritional status can positively affect disease progression and outcome. The aim of this study was to present our self-developed nutritional risk screening method, relate it to another validated tool and to objective bio-impedance measures. We intended to recommend a screening algorithm which can be used in our pediatric oncology facilities. METHODS: We analysed data from 109 pediatric oncology patients (age 3-18) at the 2nd Department of Pediatrics, Semmelweis University between 2017 and 2018. The nutritional status was assessed by the Nutrition screening tool for childhood cancer (SCAN), Nutrition risk screening for pediatric cancer (NRS-PC) our own self-developed screening tool and Bio-impedance analysis (InBody 720 and S10). Classifier properties for low muscle mass measured by Bio-impedance analysis were compared for SCAN and NRS-PC in the overall sample and in the different phases of the disease. RESULTS: The AUC of 0.67 [95% CI:0.58,0.75] of the SCAN was significantly lower (Z = -2.46, p = 0.014) than in the case of the NRS-PC (AUC = 0.75 [95% CI:0.67,0.82]), indicating that NRS-PC has better classifier properties to identify children with lower muscle mass. No significant difference was found in the different phases of the disease. CONCLUSIONS: Based on our results, we suggest screening high BMI patients first with NRS-PC. However, in case of low BMI bio-impedance measures provide more precise information on muscle mass and nutritional risk. Further data are needed to decide whether the NRS-PC is sensitive enough in normal BMI patients.

Adherence to the Porto Criteria Based on the Hungarian Nationwide Pediatric Inflammatory Bowel Disease Registry (HUPIR).

Objectives: According to the Porto criteria, upper endoscopy and ileocolonoscopy with histology for patients with pediatric inflammatory bowel disease (pIBD) are recommended with small bowel imaging (SBI). We aimed to evaluate the adherence to the Porto criteria and biopsy sampling practice and to evaluate the diagnostic yield of magnetic resonance enterography (MRE) first time in a nationwide pIBD inception cohort. Methods: Newly diagnosed pIBD cases (ages 0-18 years) are registered in the prospective, nationwide Hungarian Paediatric IBD Registry (HUPIR). We analyzed the diagnostic workup of patients recorded between the 1st of January 2007 and the 31st of December 2016. Results: Data for diagnostic workup was available in 1,523 cases. Forty percent of the cases had complied with the Porto criteria. Adherence to the Porto criteria increased significantly from 20 to 57% (p < 0.0001) between 2007 and 2016. The most frequent reason for the incomplete diagnostic work-up was the lack of small bowel imaging (59%). In 2007, 8% of cases had a biopsy from all segments, and this rate reached 51% by 2016 (p < 0.0001). We analyzed the diagnostic yield of MRE in 113 patients (10.1%), who did not have any characteristic lesion for Crohn's disease. The MRE was positive for the small bowel in 44 cases (39%). Conclusions: Adherence to the Porto criteria increased significantly during the 10-year period. This is the first study that reports multiple biopsy sampling as the less accepted recommendation. The diagnostic yield of MRE in patients without characteristic lesion for Crohn's disease is 39%.

Safety and efficacy of a lipid emulsion containing a mixture of soybean oil, medium-chain triglycerides, olive oil, and fish oil: a randomised, double-blind clinical trial in premature infants requiring parenteral nutrition.

OBJECTIVES: Safety, tolerability, and efficacy of a novel lipid emulsion containing a mixture of soybean oil, medium-chain triglycerides, olive oil, and fish oil (SMOFlipid 20%) with reduced n-6 fatty acids (FA), increased monounsaturated and n-3 FA, and enriched in vitamin E were evaluated in premature infants compared with a soybean oil-based emulsion. PATIENTS AND METHODS: Sixty (30/30) premature neonates (age 3-7 days, gestational age ≤ 34 weeks, birth weights 1000-2500 g) received parenteral nutrition (PN) with either SMOFlipid 20% (study group) or a conventional lipid emulsion (Intralipid 20%, control group) for a minimum of 7 up to 14 days. Lipid supply started at 0.5 g · kg body weight(-1) · day(-1) on day 1 and increased stepwise (by 0.5 g) up to 2 g · kg body weight(-1) · day(-1) on days 4 to 14. Safety and efficacy parameters were assessed on days 0, 8, and 15 if PN was continued. RESULTS: Adverse events, serum triglycerides, vital signs, local tolerance, and clinical laboratory did not show noticeable group differences, confirming the safety of study treatment. At study end, γ-glutamyl transferase was lower in the study versus the control group (107.8 ± 81.7 vs 188.8 ± 176.7 IU/L, P < 0.05). The relative increase in body weight (day 8 vs baseline) was 5.0% ± 6.5% versus 5.1% ± 6.6% (study vs control, not significant). In the study group, an increase in n-3 FA in red blood cell phospholipids and n-3:n-6 FA ratio was observed. Plasma α-tocopherol (study vs control) was increased versus baseline on day 8 (26.35 ± 10.03 vs 3.67 ± 8.06 µmol/L, P < 0.05) and at study termination (26.97 ± 18.32 vs 8.73 ± 11.41 µmol/L, P < 0.05). CONCLUSIONS: Parenteral infusion of SMOFlipid was safe and well tolerated and showed a potential beneficial influence on cholestasis, n-3 FA, and vitamin E status in premature infants requiring PN.

[Analysis of infliximab treated pediatric patients with Crohn disease in Hungary]. / Crohn-beteg gyermekek infliximabkezelésének kezdeti tapasztalatai hazánkban.

Infliximab, the chimeric antibody to tumor necrosis factor-alpha, is indicated for medically refractory pediatric Crohn disease. Aim of our study was to examine the efficacy and side effects of infliximab therapy in Hungarian pediatric patients with Crohn disease since the authorisation of medicine for children to 31.12.2008. 23 children with refractory Crohn disease received infliximab during this period. Induction therapy with 5 mg/kg infliximab at weeks 0, 2, and 6 was introduced. 18 patients (81.8%) achieved clinical response, and 13 patients (59.1%) were in remission at the 6th week of the observation period. The evaluation was based on data of 22 children. Fistula closure rate was 70% at the at the 6th week. Two patients had acute infusion reaction, one had severe anaphilactic reaction after infliximab infusion. Chronic side effects were also observed in three cases. In our study infliximab induction therapy was effective in most pediatric patients with therapy refractory Crohn disease.

Effect of diet and physical exercise treatment on insulin resistance syndrome of schoolchildren.

BACKGROUND: Insulin resistance syndrome (IRS) of schoolchildren may contribute to cardiovascular diseases (CVD) of young adults. The investigation of different steps, baseline screening parameters and treatment of IRS may help the prevention. METHODS: Schoolchildren (53 boys and 61 girls age 5-17 years) because of adverse family history of CVD, hypertension, and obesity were investigated. Patients were divided into 3 groups according to baseline plasma glucose level (PGL) 120 and 180 min. after glucose consumption (GC): (1) PGL < or = 5.5 mmol/L 180 min. after GC, (2) PGL > or = 5.5 mmol/L 180 min. but < or = 7.8 mmol/L 120 min. after GC (3) PGL > or = 7.8 mmol/L 120 min. after GC. Body mass index (BMI), blood pressure (BP) and parameters of glucose and lipid metabolism were measured at baseline and after two year's lifestyle modification. RESULTS: No significant difference was found in the prevalence of cardiovascular risk factors (CRF) between groups 2 and 3. Fasting PGL > 5.5 mmol/L was found in 1, 2, and 6 cases; HOMA index > 4.4 in 7 (24%), 21 (37%), and 9 (35%) subjects; OGIS index < 400 in 3(10%), 29(51%) and 11 (42%) schoolchildren of groups 1, 2, 3, respectively. Lifestyle modification significantly improved BMI, systolic BP, serum triglyceride and HDL-cholesterol levels and insulin sensitivity. CONCLUSIONS: PGL measured 180 minutes after GC may define an important subgroup of pre-diabetic children. The similar prevalance of CRF in both praediabetic groups underlines the importance of this subgroup. Lifestyle modification for two years improves CRF in this population.

Tissue transglutaminase ELISA positivity in autoimmune disease independent of gluten-sensitive disease.

BACKGROUND: Our aim was to understand why some sera from patients with a broad spectrum of autoimmune diseases or non-autoimmune diseases involving enhanced apoptosis, cell lysis and/or putative secondary autoimmune processes show reactions in the tissue transglutaminase (TGc) ELISA used for diagnosis of gluten-sensitive disease. METHODS: Sera were compared from groups of patients with autoimmune diseases, diseases involving organ specific enhanced cell death, celiac disease or dermatitis herpetiformis, diseases of non-autoimmune origin, and a group without known disease. IgA antibodies against TGc were detected using human antigen (produced recombinantly in bacterial or human cells) in different systems (non-commercial ELISA with buffers of differing NaCl concentrations, and anti-TGc sandwich ELISA). Anti-gliadin and anti-endomysium antibodies were also determined. RESULTS: Many sera from patients with autoimmune disorders gave a positive signal in the human TGc ELISAs. The signal appeared related to minor impurities in the recombinant human TGc used and to raised serum IgA antibody levels rather than to the occurrence of TGc specific antibodies in these patients. CONCLUSIONS: No association of anti-TGc Abs and autoimmune conditions independent of gluten-sensitive disease could be shown. Care should be taken to exclude copurification of chaperones, like heat shock protein 70, where preparing antigens for TGc ELISAs.

Diagnostic yield of upper endoscopy in paediatric patients with Crohn's disease and ulcerative colitis. Subanalysis of the HUPIR registry.

BACKGROUND, AIMS: According to Porto Criteria upper gastrointestinal (UGI) endoscopy is recommended in patients with suspected inflammatory bowel disease (IBD). Nevertheless, previous studies revealed frequent involvement of UGI tract even in patients with ulcerative colitis (UC). The aim of the present study was to determine the diagnostic role of esophagogastroduodenoscopy (EGD) and assess the prevalence and different aspects of UGI involvement in children registered in the Hungarian Pediatric IBD Registry (HUPIR) from 1st of January 2007 to 31th of December 2009. METHODS: Twenty seven institutes provided prospective follow-up data about newly diagnosed IBD patients to HUPIR. The registry was based on detailed questionnaire (76 parameters) involving anamnestic data, laboratory findings, activity indexes, diagnostic procedures, endoscopic examinations (EGD and ileocolonoscopy), and histological data. Localization and phenotype of disease were based on the Montreal classification criteria. RESULTS: During the 3-year period 420 children were diagnosed with IBD, 265 (63%) of them had Crohn's disease (CD), 130 (31%) UC, and 25 (6%) IBD-unclassified (IBD-U). The mean age at diagnosis was 13.2 years (range: 1.2-18 years). EGD was performed in 237 patients (56%), in most cases in patients suffering from CD. Macroscopic lesions on EGD were noted in 64% of patients with CD and 40% of children with UC. Characteristic lesions for CD (ulcer, erosion, aphthous lesion, and granuloma) were noted in 31% of CD patients, however, EGD helped to establish the final diagnosis in 9% of CD patients (diagnostic yield, 9%). CONCLUSIONS: There was a high frequency of UGI involvement in children with CD and UC. One third of CD patients showed significant lesions at upper endoscopy and one patient out of ten had real diagnostic help from EGD.

Hyperhomocysteinemia, enzyme polymorphism and thiobarbituric Acid reactive system in children with high coronary risk family history.

OBJECTIVES: To investigate both the frequency and the genetic background of hyperhomocysteinemia and the frequency of increased plasma thiobarbituric acid reactive system (TBARS) levels in children and adolescents whose parents had premature coronary heart disease (CHD). METHODS: The study was performed on children and adolescents aged 4-18 years (105 offspring of parents with CHD before age 45 and 74 referents from families without any evidence of premature atherosclerosis). Fasting serum total cholesterol (TC), high density lipoprotein cholesterol (HDL-C), and total triglyceride (TG) levels were measured by enzymatic methods. Low density lipoprotein cholesterol (LDL-C) level was calculated by the Friedewald formula. Plasma total homocysteine (THCy) level was measured by fluorescence polarisation immunoassay. Plasma TBARS level was determined by fluorimetric method. 5,10-methylenetetrahydrofolate reductase (MTHFR) enzyme polymorphism was analyzed by polymerase chain reaction with restriction fragment length polymorphism (PCR-RFLP). RESULTS: Hyperhomocysteinemia was found in 32 cases and in 4 controls. Increased plasma THCy level was found in 10 children and adolescents from 12 cases homozygous for the C677T polymorphism of the MTHFR gene. No similar high frequency was observed in heterozygous subjects. Elevated fasting plasma TBARS levels were found in 38 cases and in 8 controls. The frequency differences were significant (p < 0.01). Allele frequency of the MTHFR polymorphism among cases and controls was similar. Significant correlation (r = 0.53, p < 0.02) was detected between plasma THCy and TBARS levels. One child had high serum TC level, 5 had low serum HDL-C level and all other children had normal serum TC, LDL-C, HDL-C and TG levels from children with hyperhomocysteinemia and/or high plasma TBARS levels. A significant correlation (r = 0.64, p < 0.01) was observed between plasma THCy levels of parents and children in the case group. CONCLUSION: The measurement of plasma THCy and TBARS levels may contribute to the detection of the risk of children and adolescents with high CHD risk family history.