Nutritional status of pediatric patients with inflammatory bowel diseases is related to disease duration and clinical picture at diagnosis.

This cross-sectional study presents the nutritional status of newly diagnosed pediatric patients with Crohn's disease (CD) and ulcerative colitis (UC) and its association with the duration of the disease and selected clinical features. We analyzed the data of 41 pediatric patients with CD and 29 with UC (mean age: 13.1 y, range: 5.2-18.0 y) up to 3 mo. from diagnosis. Anthropometry included body weight, body height, body mass index (BMI), three skinfold thicknesses, mid-upper arm circumference and mid-upper arm muscle circumference adjusted for age and sex using national standards. Anthropometry was linked to the disease duration, location of the disease, symptoms, and blood test results. Both studied groups presented significantly lower BMI compared to the reference population, but only children with CD characterized with significantly worse nutritional status according to arm anthropometry. In CD, better nutritional status was associated mainly with longer disease duration and, to a lesser extent, with extraintestinal manifestations, perianal disease, and small intestinal lesions. In UC, anemia at diagnosis was associated with poor nutritional status. Our finding emphasizes the need for more attentive diagnostic care for pediatric patients who exhibit extraintestinal symptoms or perianal disease with no obvious signs of malnutrition, to avoid diagnostic delays.

Prevalence of haplotype DQ2/DQ8 and celiac disease in children with type 1 diabetes.

Type 1 diabetes (T1D) and celiac disease (CD) coexist very often. Identification of the human leukocyte antigen (HLA) DQ2/DQ8 can confirm the genetic predisposition to CD. Negative result of this test allows to exclude CD with a high probability. It was suggested that in individuals with higher risk of CD, including T1D patients, the implementation of genetic testing should reduce the number of patients requiring systematic immunological screening. The aim of this study was to analyze the prevalence of different haplotypes predisposing to CD in children and adolescents with previously diagnosed T1D. MATERIAL AND METHODS: A retrospective analysis was performed on 166 T1D children (91 girls) in whom HLA DQ2/DQ8 alleles were tested. In 9.6% CD was also diagnosed. RESULTS: In 12.7% both HLA DQ2/DQ8 were negative. In 87.3% patients HLA DQ2 and/or DQ8 was positive, including 27.7% patients with both haplotypes DQ2.5 and DQ8 positive. In all CD patients the disease predisposing alleles were positive, while none of the HLA DQ2/DQ8 negative children were diagnosed with CD. CONCLUSIONS: The prevalence of HLA DQ2.5 and the HLA DQ2.5 / HLA DQ8 configuration is higher in patients with T1D, and CD compared to children with T1D alone. The combination of HLA DQ2 and HLA DQ8 most significantly increases the risk of developing CD. The group of HLA DQ2/DQ8 negative patients with improbable CD diagnosis, is relatively small. Most of T1D patients HLA DQ2/DQ8 positive need further regular antibody assessment. In patients with T1D, who are at high risk of developing CD, genetic testing may be considered to select those who require further systematic serological evaluation. Due to its retrospective nature, the study was not registered in the database of clinical trials and the Clinical trial registration number is not available.

Selective Forms of Therapy in the Treatment of Inflammatory Bowel Diseases.

Selective interference with the functioning of the immune system consisting of the selective blockade of pro-inflammatory factors is a modern, promising, and developing strategy for the treatment of diseases resulting from dysregulation of the immune system, including inflammatory bowel disease. Inhibition of the TNF alpha pathway, group 12/23 cytokines, and lymphocyte migration is used in the treatment of severe or moderate ulcerative colitis and Crohn's disease. Intracellular signal transduction by influencing the phosphorylation of SAT (signal transducer and activator of transcription) proteins remains in clinical trials.

Endoscopic Ultrasonography in Children with Eosinophilic Esophagitis-A Review.

Endoscopic ultrasonography (EUS) is a diagnostic endoscopy of the upper gastrointestinal tract, during which ultrasound of nearby organs is also performed. It is also possible to perform a fine needle aspiration biopsy. Currently, EUS is performed more frequently in adults. Despite some limitations, this diagnostic method is also more and more often performed in pediatric patients. Eosinophilic esophagitis (EoE) is a chronic inflammatory disease of the esophagus, which also occurs in children, and leads to irreversible fibrosis of the esophagus wall, if left untreated. Traditional methods of diagnosing and monitoring EoE treatment have significant limitations, and the use of EUS and total esophageal wall thickness (TWT) assessment may bring measurable benefits. Several studies have shown an increased thickening of TWT in EoE in children compared to pediatric patients with gastroesophageal reflux disease, and a decrease in TWT in adults who responded to EoE treatment. These results suggest that EUS and TWT measurement may become an important test in diagnostics, monitoring the effectiveness of therapy, assessing disease progression, and in individualizing the method and duration of EoE treatment also in children.

The Incidence of Inflammatory Bowel Disease in the Paediatric Population in the District of Lower Silesia, Poland.

The incidence of IBD has been rising over the last decades. The trend applies not only to well-developed countries but also to the regions with limited number of cases so far, e.g., Asia or Middle East. AIM: The aim of the study was to determine the incidence of paediatric IBD in the district of Lower Silesia, Poland, between 2016 and 2018. METHODS: The number of newly diagnosed IBD per 100,000 children, living in the region, was calculated. The characteristics of the group were established. RESULTS: There were 81 cases of paediatric IBD diagnosed between the 1st of January 2016 and 31st of December 2018. The diagnosis of ulcerative colitis (UC) was made for 42 children. In the same period of time 39 cases of Crohn disease (CD) were recognised. The incidences were calculated as 2.57 for UC, and 2.38 for CD. The total incidence of IBD between 2016 and 2018 was 4.96/100,000/year which is rise in the last 20 years. CONCLUSION: An increase in incidence of IBD in the district of Lower Silesia has been observed.

Probiotics, Prebiotics and Synbiotics in Inflammatory Bowel Diseases.

Inflammatory bowel diseases (IBD), which include Crohn's disease (CD) and ulcerative colitis (UC), are chronic inflammatory diseases of the digestive tract with periods of remission and relapses. The etiopathogenesis of IBD is multifactorial and has not been fully understood. Hence, only symptomatic treatment of these diseases is possible. The current pharmacological treatment has variable efficacy and is associated with the risk of significant side effects. Therefore, there is a constant need to search for new types of therapies with a high safety profile. Considering that the qualitative and quantitative profile of the gastrointestinal microbiome is often different in patients with IBD than in healthy individuals, there is a need for looking for therapies aimed at restoring intestinal microbiome homeostasis. Thus, the use of strictly defined probiotics, prebiotics and synbiotics may become an alternative form of IBD therapy. There is evidence that treatment with certain probiotic strains, e.g., VSL#3 and Escherischia coli Nissle 1917, is an effective form of therapy to induce remission in patients with mild to moderate UC. So far, the effectiveness of the use of probiotics, prebiotics and synbiotics in inducing or maintaining remission in patients with CD has not been confirmed. There are also reports of possible beneficial effects of fecal microbiota transplantation (FMT) on the course of IBD, especially UC. Further, well-planned studies on a large group of patients are needed to determine the role of specific probiotic strains, prebiotics, synbiotics and FMT in the treatment of IBD in adults and in children.

Apolipoprotein E variants correlate with the clinical presentation of paediatric inflammatory bowel disease: A cross-sectional study.

BACKGROUND: It has been suggested that apolipoprotein E (APOE) polymorphisms are associated with the risk of developing inflammatory bowel disease (IBD) and the early age of disease onset. However, there are no reports regarding the relationship with clinical characteristics and disease severity. AIM: To summarise that APOE polymorphisms are associated with the risk of developing IBD and the early age of disease onset. METHODS: In total, 406 patients aged 3-18 with IBD (192 had ulcerative colitis and 214 had Crohn's disease) were genotyped using the TaqMan hydrolysis probe assay. Clinical expression was described at diagnosis and the worst flare by disease activity scales, albumin and C-reactive protein levels, localisation and behaviour (Paris classification). Systemic steroid intake with the total number of courses, immunosuppressive, biological, and surgical treatment with the time and age of the first intervention were determined. The total number of exacerbation-caused hospitalisations, the number of days spent in hospital due to exacerbation, the number of relapses, and severe relapses were also estimated. RESULTS: Ulcerative colitis patients with the APOEε4 allele had lower C-reactive protein values at diagnosis (P = 0.0435) and the worst flare (P = 0.0013) compared to patients with the APOEε2 allele and genotype APOEε3/ε3. Crohn's disease patients with the APOEε2 allele scored lower on the Pediatric Crohn's Disease Activity Index at diagnosis (P = 0.0204). IBD patients with APOEε2 allele spent fewer days in the hospital due to relapse (P = 0.0440). CONCLUSION: APOE polymorphisms are associated with the risk of developing IBD and the clinical expression of IBD. However, the clinical relevance of the differences identified is rather modest.

Laugier-Hunziker syndrome: a case report of the pediatric patient and review of the literature.

Laugier-Hunziker syndrome (LHS) is a rare, idiopathic pigmentary disorder especially affecting the lips and oral mucosa. At present, no more than 200 cases of patients diagnosed with LHS syndrome have been described worldwide. To date, three patients under the age of 20 have been described, including the youngest patient who is a 12-year-old child. The exact etiology of LHS still remains uncertain, as there is no evidence of systemic symptoms or increased cancer risk. The final diagnosis of LHS is possible after the exclusion of other, more serious diseases involving skin-mucosal hyperpigmentation, mainly Peutz-Jeghers syndrome (PJS) and Addison's disease (AD). Herein, we present a 16-year-old patient who has been diagnosed with oral hyperpigmentation since the age of 13. We reviewed the clinical and histological findings. In addition, we discussed the differential diagnosis of mucocutaneous hyperpigmentation.

Neuroendocrine tumors of the gastrointestinal tract and pancreas: Is it also a challenge for pediatricians?

Neuroendocrine tumors (NET) of the gastrointestinal tract and pancreas are extremely rare in the pediatric population and limited data is available. In most cases, NET of the gastrointestinal tract in children are located in the appendix. Pancreatic NET are a small but partially distinct group of the gastrointestinal neuroendocrine neoplasms. The most common in this group are insulinomas; however, in some research, the gastrinoma type neoplasms are perceived to be most common in children. This study reviews the typical clinical presentation, appropriate diagnostics, staging, and treatment of these uncommon neoplasms. It is important to know the epidemiology and symptomatology in this age group despite the fact that the majority of physicians treating the youngest patients will never have to deal with it. This will facilitate an early diagnosis in case of symptoms that may suggest neuroendocrine cancer. It appears necessary to create harmonized recommendations regarding the diagnosis, treatment and post-treatment follow-up for pediatric patients.

[Acute pancreatitis as a complication of diagnostic gastroduodenal endoscopy at 10 years old girl--case report]. / Ostre zapalenie trzustki jako powiklanie gastroduodenoskopii diagnostycznej u 10-letniej dziewczynki--opis przypadku.

A 10-years old girl with chronic pancreatitis, in whom the symptoms of acute pancreatitis appeared after gastroduodenal endoscopy is described. One hour after endoscopy the following symptoms appeared: unigastric and epigastric pain, intense vomiting, elevated amylase activity in the serum (2744 micro/l) and in urine (23738 micro/l) as well as serum lipase activity (4350 micro/l). Ultrasound of the abdomen revealed the enlargement of the pancreas with hypodense structure in comparison to the examination conducted couple hours earlier, and trace of fluid around the pancreas. In the treatment intravenous omeprazole, a strict diet and intravenous fluid and electrolytes were administered. After two days vomiting and abdominal pain subsided, biochemical results improved and the reduction of pancreas dimensions in ultrasound study was observed.

[Severe toxic diarrhoea in cyclosporin treated 11-years old girl with nephrotic syndrome]. / Ciezka biegunka toksyczna w przebiegu leczenia cyclosporyna A u jedenastoletniej dziewczynki z zespolem nerczycowym.

A 11-years old cyclosporin-treated girl with nephrotic syndrome, complicated by severe toxic diarrhoea with excessive metabolic alkalosis and dyselektrolitemia (hyponatremia, hypokaliemia, hypochloremia) and transient immunoglobulin deficiency is reported. Ultrasonography shows thickeness of the colonic wall as a symptom of colonic inflammation. After discontinuation of cyclosporin therapy, excluding infectious origin of diarrhoea (bacterial, viral and fungal infection), partial parenteral nutrition, enteral nutrition (Peptisorb), probiotics and antidiarrhoeal drugs were used in the treatment. After introducing steroids orally (Entocort) complete resolution of symptoms were observed.

[Management of fever without source in children]. / Trudnosci diagnostyczne w rozpoznawaniu goraczki niewiadomego pochodzenia u dzieci.

INTRODUCTION: Infectious diseases are the most common cause of fever in children. Viral and bacterial infections of respiratory, gastrointestinal, central nervous system and urinary tract dominates. In the group of children with chronic fever in some cases inflammatory bowel diseases, immunodeficiency syndromes and infections of rare pathogens are diagnosed. AIM: Authors analysed retrospectively 10 children with chronic fever treated in the II Department of Pediatric Gastroenterology & Nutrition, Medical University of Wroclaw. RESULTS: In those children we diagnosed Crohn's disease (1 case), cat scratch disease (1 case), systemic fungial infection in the child with short bowel syndrome (1 case), severe immunodeficiency syndrome (3 cases), intraperitoneal abscess in a child with Crohn's disease (1 case), severe toxic diarrhoea in the course of treatment with cyclosporin (1 case), Yersinia enterocolitica infection (2 cases). CONCLUSION: In the diagnostics of the prolonged fever in children various conditions and diseases ought to be considered.

[Diagnostic difficulties in pediatric abdominal pain with potential appendicitis]. / Trudnosci diagnostyczne przyczyn bólów brzucha okolicy wyrostka robaczkowego u dzieci.

BACKGROUND: Pediatric abdominal pain is one of the most common symptom of children brought to attention of primary care physicians and pediatric surgeons. In many children clinical symptoms may be uncharacteristic and may lead to diagnostic difficulties. AIM: Clinical analysis of children with right lower quadrant abdominal pain suspected of appendicitis or children with periappendicular mass. MATERIALS AND METHODS: The histories of 14 children aged from 18 months to 17 years treated in Pediatric Clinic were analysed. All children were operated because of right lower quadrant abdominal pain or abdominal mass before admission to the Pediatric Clinic or during hospitalization in Pediatric Clinic. Intraoperatively in all children pathologies other than appendicitis were the cause of symptoms. RESULTS: The most often Crohn's disease were recognized (9 children), in 2 cases with concomitant other pathologies (fecal tumor of appendix in one case and with peritoneal abscess after perforation of intestinal wall). Sporadically the inflammation of the mesenterial lymph nodes caused by Yersinia infection suggested appendicitis. In one boy with ulcerative colitis, during exacerbation of the disease appendicitis complicated by rupture and peritonitis was observed. In 18-month old child with right lower quadrant abdominal mass invagination complicated by perforation of the ileum was recognized. In the case of 14-years old boy 6 months after appendectomy we observed mechanical intestinal obstruction complicated by perforation and peritonitis. Carcinoid of the appendix was the cause of abdominal pain in one child. CONCLUSIONS: 1. Appendicitis is the most frequent surgical etiology of the right lower quadrant pediatric abdominal pain. 2. Despite new diagnostic imagines there are no definite criteria to recognize appendicitis, in most cases physical examination and very carefull evaluation of abdominal pain are the most important. 3. All children with periappendipected of Crohn's disease. 4. All children with equivocal presentations of appendicitis and with normal appendix during operation should undergo further diagnostic evaluation.

[Predisposing factors and results of pharmalogical treatment using ursodeoxycholic acid of gallbladder stones in children]. / Czynniki predysponujace i wyniki leczenia farmakologicznego kwasem ursodezoksycholowym kamicy p cherzyka zólciowego u dzieci.

UNLABELLED: Gallbladder stones (GBS) in adults is one of the most often disease of the gastrointestinal tract, it occurs in about 0.2% of children. Risk factors of the GBS in children are different that those in adults. Therapeutical standards in GBS depend on the age, clinical symptoms, complications and coexistent diseases. AIM: The estimation and comparison of risk factors of GBS in children in two periods (1989-1996 and 1997-2004), and the estimation of the results of the pharmacological treatment with UDCA. MATERIAL AND METHODS: In 112 children aged from 3 months to 18 yrs. with GBS recognized in the period of 1989-2004 the estimation and comparison of the frequency of risk factors in two periods: 1989-1996 (39 children aged from 4 months to 17 yrs.) and in years 1997-2004 (73 children aged from 3 months to 18 yrs.) was done. In the second step we analyzed results of pharmacological treatment with UDCA in 41 children after completion of the therapy. Only children with noncalcifying stones smaller than <15 mm in diameter were qualified to the treatment. Statistical analysis (chi2-test) was used to estimate results of the analysis. RESULTS: Gallbladder stones was recognized more often in girls (65%), obesity was present in 42% of children, family history of the GBS was positive in 28.6%, prior severe disease (e.g. sepsis) in 15.9%. In 16% of children GBS was idiopathic. In the period of 1997-2004 significantly more often than in the period of 1989-1996 in children with GBS were observed: serum lipid abnormalities (28.4 vs. 10.3%; p < 0.03), prior severe diseases requiring intensive care (21.6 vs. 5.1%; p = 0.02) and intensive reducing of the body weight (9.5 vs. 0%; p < 0.05). Any of the risk factors had influence on the positive result of pharmacological treatment. Dissolution of the small stones (<5 mm) in comparison with stones bigger than 10 mm (p < 0.05) was observed significantly more often. CONCLUSIONS: (1) Gallbladder stones in children was observed more often in girls, children with prior severe disease, and in obese children with the positive family history of GBS. (2) Good results of pharmacological treatment of GBS in children depends on the size of the stones. (3) Risk factors of the GBS have no significant influence to the positive result of the pharmacological treatment using UDCA in children.

[Intestinal complications and extraintestinal manifestations in children with inflammatory bowel disease]. / Powiklania jelitowe i objawy pozajelitowe w przebiegu nieswoistych zapalen jelit u dzieci.

UNLABELLED: In 15-20% of patients with inflammatory bowel disease (IBD) the disease starts during children period. The often observed consequences of the early start of the IBD are growth delay, undernutrition and pubertal delay. These problems are specific for pediatric population, in comparison with adults IBD patients. AIM: The assessment of the frequency and character of intestinal complications (IC) and extraintestinal manifestations (ElM) in children with IBD (ulcerative colitis - UC and Crohn disease - CD). MATERIAL AND METHODS: The frequency and character of IC and EIM in 184 children with IBD: 158 with UC (76 boys and 82 girls) aged from 2 to 18 years, and 26 with CD (14 boys and 12 girls) aged from 2 to 18 years were assessed. The frequency of IC and EIM in both groups of children Was compared statistically (chi-square test). RESULTS: Massive intestinal haemorrhage was observed in 11 children with UC. Perianal changes were present in 5 children with CD (significantly more often than in children with UC, p<0.05). External intestinal fistulae (p<0.0005), abdominal abscess, intestinal perforation and ileus (p<0.05) were characteristic complications of CD in comparison with UC. 50% of children with UC and 80% those with CD (statistically significant, p<0.005) had experienced, at least one ElM. Secondary anemia and growth delay were observed in 40,5% and 27,8% of children with UC and in 70% (p<0.05) and 80% (p<0.0001) of children with CD respectively. In children with IBD we observed also autoimmunologic hepatitis, primary sclerosing cholangitis, nephrolithiasis, pulmonary interstitial fibrosis, pancreatitis, neurological changes, skin manifestations and arthritis. Osteopenia was observed in 41% of children with CD and in 25% of children with UC. a CONCLUSIONS: A high prevalence of intestinal complications ane extraintestinal manifestations of IBD in pediatric population is observed. In children with IBD extraintestinal manifestations often occur before the onset of gastrointestinal symptoms. The presence of intestinal complications and extraintestinal manifestations in children with IBD should be considered during modification of therapeutic strategies.

[Genotype and phenotype of gastrointestinal symptoms analysis in children with cystic fibrosis]. / Ocena genotypu i objawów ze strony ukladu trawiennego u dzieci chorych na mukowiscydoze.

UNLABELLED: Cystic fibrosis is the most common autosomal recessive genetic defect of one gene CFTR, where a variety of mutations were revealed. Cystic fibrosis is a variable disease and to date the genotype-phenotype correlation is difficult to clarify. The aim of the study was to analyse retrospectively the genotype and phenotype of children with cystic fibrosis and to search the correlation between type of mutation in CFTR and clinical manifestation of the gastrointestinal tract. MATERIAL AND METHODS: The study group comprised 52 patients. Molecular DNA analyses were performed in 43 cases. Statistical analysis was done by using Fisher test. RESULTS: In 34 (79%) cases two mutations in the CFTR gene were identified. In this group 21 cases were identified as a homozygous for AF508 mutation, in single case other mutations were found. A mutation of one CFTR allel was revealed in 11 patients, cystic fibrosis was not confirmed by genetic test in 9 children. Mean age of diagnosis was 34 months. In 38 children (73%) pancreatic insufficiency in the course of disease was found. Exocrine insufficiency of pancreas was showed significant frequently in homozygous group. Liver dysfunction in 20 children (38.5%) was revealed. In this group 12 patients was identified as a homozygous for deltaF508 mutation. On the base of oral glucose tolerance test the diabetes mellitus and glucose intolerance was diagnosed in 4 cases with homozygous genotype. Seven patients died in the endstage of the illness, in two of them homozygous mutation deltaF508 was found, in next 5 patients genetic analysis was not performed. CONCLUSIONS: The frequency and severity of clinical manifestation of the gastrointestinal tract correlates with deltaF508 mutation. Early genetic test and demonstration of molecular defect in CFTR gene confirms the clinical diagnosis of cystic fibrosis and improves a quality of life and prolongs survival time of cystic fibrosis patients.

[Incidence of acute pancreatitis in children with inflammatory bowel disease]. / Ocena czestosci wystepowania ostrego zapalenia trzustki w przebiegu przewleklych nieswoistych zapalen jelit u dzieci.

The aim of our study was to estimate the frequency of acute pancreatitis and the frequency of increased activity of pancreatic enzymes in serum of children with inflammatory bowel disease (IBD). Analysis comprises 101 children aged from 3 to 18-years treated because of IBD in the period of 1998-2002: 79 children with ulcerative colitis (UC) and 22 children Crohn's disease (CD). The authors analyzed together 191 admissions because of UC and 51 because of CD. Acute pancreatitis was observed in 4.5% of children with CD and in 5.1% of children with UC. Significantly more often acute pancreatitis was recognized in children with moderate and severe stage of UC. Hyperamylasemia was observed in 27.3% of children with CD and in 12.7% of children with UC. Hyperlipasemia was observed only in children with UC (3.8%), elevated urinary amylase was observed in 4.5% of children with CD and in 8.86% children with UC. No correlations between the frequency of acute pancreatitis and medication were observed.