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

The content of serotonin cells in duodenal biopsies of autistic patients

AbstractIntroductionAutistic spectrum disorders (ASD) don’t have the same etiology. Platelet hyperserotonemia remain the most common neurochemical abnormality in these patients. The main producer and storage of peripheral serotonin are enteric enterochromaffin cells – serotonin cells. Platelet hyperserotonemia may result from disorders in the synthesis and/or release of enteric serotonin. An increased number of people with ASD have gastrointestinal disorders. Some of them have a serotonergic background.AimThe aim was to assess the serotonin cells in the duodenal mucosa of patients with ASD.Material and methodsStudy group: 30 children with ASD, including 73% with duodenitis chronica. Control group (patients without ASD): 45 patients, 56% with duodenitis chronica. Immunohistochemical assessment of the number of serotonin cells was performed.’ResultsChildren with ASD and duodenitis have fewer serotonin cells than autistic children with a normal picture of the duodenum. Children with ASD and chronic duodenitis have fewer serotonin cells than patients from the control group. Patients from the control group, suffering from chronic duodenitis have an increased number of serotonin cells in relation to children without inflammatory lesions in the duodenum.ConclusionsThe serotonergic profiles of the GI tract of autistic patients and their peers without autistic symptoms are different. In the course of chronic duodenitis in patients with ASD the number of serotonin cells falls while in persons without autistic features it increases significantly. Chronic duodenitis contributes to an increase in the number of serotonin cells in persons without autistic features while decreasing it in patients with ASD

Clinical and neuropathological picture of familial encephalopathy with bifunctional protein deficiency

Peroxisomal diseases are a heterogeneous group of genetic metabolic disorders which are caused by incorrect biogenesis of peroxisomes or a defect in activity of particular enzymes located in those organelles. D-bifunctional protein (D-BP) deficiency belongs to the second group of peroxisomal diseases characterised by dysfunction of a single peroxisomal enzyme. Bifunctional protein is a catalyst in the second and third stage of the \beta-oxidation of fatty acids. Gene locus of bifunctional protein deficiency comprises chromosomes 5q2 and 3p23-p22. The authors present two siblings with progressing family encephalopathy. In the younger brother the diagnosis of a bifunctional protein deficiency was made. The girl died before a diagnosis was made; however, due to the presence of a very similar clinical condition a suspicion arises that the girl had a peroxisomal disease. In the siblings were ascertained characteristic dysmorphic features, delayed psychomotor development, polymorphic epileptic seizures and generalized muscular hypotonia with areflexia. The neuropathological findings were consistent in general with MRI findings showing features of hypomyelination. Also neuron heterotopias that were found in autopsy are a form of pathology typical for D-BP

Hipertransaminazemia w przebiegu ostrej biegunki u dzieci

Najczęstszą przyczyną ostrych biegunek u dzieci w Polsce są wirusy, zwłaszcza rotawirusy. Zarówno wirusy, jak i bakterie wywołujące ostrą biegunkę mogą być czynnikami wtórnie hepatotropowymi. Celem pracy była ocena stężeń aminotransferaz u dzieci hospitalizowanych z powodu ostrej biegunki w Klinice Pediatrii SUM w Katowicach w latach 2008–2009. Badaniami objęto 579 dzieci, 307 chłopców (53%) oraz 272 dziewczynki (47%), w wieku od 1. miesiąca życia do 18. roku życia (śr. wiek 2,5 roku). U 226 dzieci (41%) rozpoznano zakażenie rotawirusem, u 158 zakażenie bakteryjne (27,3%), w tym zakażenie Salmonellą 48/579 (8,3%), patogenną Escherichia coli 79/579 (13,4%), Campylobacter jejuni 38/579 (6,6%). U wszystkich pacjentów oznaczono stężenia aminotransferazy asparaginianowej (AspAT) i alaninowej (AlAT) z uwzględnieniem wieku, płci, etiologii ostrej biegunki, parametrów stanu zapalnego, zaburzeń gospodarki wodno- elektrolitowej i kwasowo-zasadowej oraz współwystępowania innych schorzeń. Otrzymane wyniki poddano analizie statystycznej. Podwyższone stężenie AspAT obserwowano u 268 dzieci (46,3%), jedynie u 13 dzieci (2,3%) wartości te przekraczały dwukrotną normę. Podwyższone stężenie AlAT obserwowano u 58 dzieci (10,1%). Jedynie u 6 dzieci (1%) stężenie AlAT było powyżej dwukrotnej wartości normy. Spośród wszystkich pacjentów z ostrą biegunką rotawirusową podwyższone stężenie aminotransferaz (głównie AspAT) obserwowano u 189/226 (83,6%). U wszystkich dzieci stężenia aminotransferaz znormalizowały się w ciągu miesięcznej obserwacji. Znamiennie statystycznie częściej podwyższone stężenia aminotransferaz obserwowano u dzieci w wieku poniżej 3. roku życia oraz ze znacznymi zaburzeniami gospodarki kwasowo-zasadowej. Wykonane badanie USG jamy brzusznej jedynie w pojedynczych przypadkach wykazało nieprawidłowości, głównie pod postacią powiększenia wątroby. Ze względu na obserwowaną podwyższoną przejściową hipertransaminazemię i szybką samoistną jej normalizację należy zastanowić się na celowością rutynowego oznaczania tych enzymów w przebiegu ostrej biegunki u dzieci. Wskazane wydaje się prowadzenie badań dążących do wyjaśnienia przyczyn hipertransaminazemii zwłaszcza u dzieci poniżej 3. roku życia. Forum Medycyny Rodzinnej 2011, tom 5, nr 6, 491–49

Serologic Investigations in Children with Inflammatory Bowel Disease and Food Allergy

The aim of the study was the evaluation of frequency and titre of IgA ASCA and IgG ASCA and p-ANCA, c-ANCA in children with IBD and occurrence of ASCA antibodies in relation to coexistence of FA. Patients and methods. The study comprised 95 children at the ages of 2 to 18 years. The diagnosis of IBD was established on the basis of Porto criteria. Tests of blood serum were performed in all children: IgA and IgG ASCA, p-ANCA, c-ANCA using ELISA method. Results. IgE-dependent FA was found in 32.5% children with UC and in 21% with CD. We did not observe any relation between the occurrence of FA and the frequency and ASCA titre. p-ANCA were significantly more frequent in the group of children with UC. The occurrence of ASCA antibodies was observed in 73.7% of children with CD, 17.5% with UC and almost 30% with allergic colitis. Conclusions. Patients with CD and the presence of ASCA revealed a significantly more frequent localization of lesions within the small bowel and a tendency towards older age. We observed a connection between the occurrence of antibodies and the examined mutations of gene NOD2/CARD15

Phase III Randomized Non-Inferiority Study of OSS Versus PEG + Electrolyte Colonoscopy Preparation in Adolescents

Objectives: Many protocols and preparations are used for bowel cleansing before pediatric colonoscopy but few are based on scientific evidence. We evaluated efficacy, safety, tolerability, and patient preference of oral sulfate solution (OSS) at 75% of the adult dose versus polyethylene glycol (PEG)-electrolyte solution in adolescents presenting for diagnostic colonoscopy. Methods: Phase III, randomized, evaluator-blinded, non-inferiority study of OSS and PEG in adolescents aged 12-17 years. OSS and PEG were administered in 2 doses on the day before colonoscopy. Primary endpoint included proportion of patients with successful overall preparation (4-point scale). Secondary endpoints included overall and segmental bowel cleansing (Boston Bowel Preparation Scale; BBPS), completed colonoscopies, duration of examination, time to cecal intubation, proportion of nasogastric tubes (NGTs), adverse events (AEs) and acceptability. Results: Successful cleansing was achieved in 71.4% and 79.0% of patients receiving OSS and PEG, respectively [adjusted difference -7.61 (95% confidence interval, CI, -18.45 to 3.24); P = 0.0907]. Segmental BBPS score for the left and transverse colon were similar between treatment groups, but better for the right colon with PEG than OSS [2.2 (95% CI, 2.0-2.4) and 1.9 (95% CI, 1.7-2.1), respectively; P = 0.0015]. Significantly fewer OSS patients needed NGT placement to ingest the whole solution [9/125 (7.2%)] than PEG patients [36/116 (31.0%); P < 0.0001]. Treatment acceptability was significantly higher with OSS than PEG ( P < 0.0001). Duration of examination, completed colonoscopies, and time to cecal intubation were similar between preparations. Gastrointestinal AEs including nausea, vomiting, abdominal pain, and distension were similar in both groups but more patients receiving PEG had AEs assessed as incapacitating. Conclusions: Non-inferiority of OSS to PEG was not demonstrated, but OSS was associated with a lower requirement for NGT, better acceptability, and less frequent severe AEs than with PEG

Clinical characteristics of 320 pediatric Crohn's disease patients registered in the nationwide Crohn's disease registry in Poland

Wstęp: Nieswoiste choroby zapalne jelit (inflammatory bowel diseases - IBD), zwłaszcza choroba Leśniowskiego-Crohna (Crohn’s disease - CD), są narastającym problemem w gastroenterologii pediatrycznej. Dostępne dane dotyczące klinicznych i demograficznych aspektów choroby w Polsce są ograniczone. Cel: Zebranie rzetelnych danych o klinicznych i demograficznych aspektach choroby Leśniowskiego-Crohna u dzieci w Polsce na podstawie utworzonego internetowo prospektywnego rejestru choroby mających pomóc w opracowaniu najbardziej optymalnych strategii terapeutycznych dla tej grupy pacjentów. Materiał i metody: We wrześniu 2005 roku został utworzony w Internecie ogólnopolski rejestr pacjentów z chorobą Leśniowskiego-Crohna. Do projektu włączono 10 jednostek szpitalnych (9 szpitali akademickich, 1 rejonowy szpital referencyjny). W celu zebrania danych demograficznych i klinicznych zastosowano dostępny internetowo kwestionariusz, który następnie przesyłano do centralnego rejestru do prospektywnej analizy. Ocenie poddano następujące dane: demografia, historia rodzinna, lokalizacja i postać choroby, objawy pozajelitowe, choroby współistniejące, diagnostyka oraz leczenie (włączając w to interwencje chirurgiczne). Wyniki: Przez 4 lata 320 pacjentów (płeć męska : płeć żeńska - 191 : 129) w wieku poniżej 16 lat ze zdiagnozowaną CD (średni wiek w momencie postawienia diagnozy: 9,2 ±6,8 roku) zostało zarejestrowanych w bazie danych. Tak zwany wczesny początek choroby (wiek przy rozpoznaniu poniżej 5 lat) stwierdzono u 68 dzieci (21,25%). Rodzinne występowanie (obciążony wywiad rodzinny) odnotowano u 16 pacjentów (5%). Główne miejsce zmian chorobowych (według Klasyfikacji montrealskiej: L1 - jelito cienkie, L2 - jelito grube, L3 - ileocolon, L4 - górny odcinek przewodu pokarmowego) stanowiła lokalizacja krętniczo-kątnicza (L3) - 217 (67,8,%). Postać niepenetrująca bez zwężeń była przeważającą postacią choroby - 225 (70,32%) pacjentów. Objawy pozajelitowe zaobserwowano u 20 chorych (6,25%). Wnioski: Badanie dostarcza pełnych informacji dotyczących aspektów demograficznych i klinicznych choroby Leśniowskiego-Crohna w Polsce. Uzyskane dane są zgodne z doniesieniami z innych krajów. Wnioski z badania są następujące: zbierane informacje muszą być dobrze zdefiniowane i określone już na samym początku badania, weryfikowane oraz aktualizowane systematycznie w trakcie jego trwania, aby usprawnić pracę i uzyskać jak najbardziej wiarygodne wyniki.Introduction: Inflammatory bowel disease, particularly Crohn’s disease (CD), is a rising problem in pediatric gastroenterology. Limited information is available on demographic and clinical aspects of pediatric CD in Poland. Aim: Preliminary data on demographic and clinical characteristic of pediatric CD in Poland based on the web-based prospective registry in order to gather reliable information to identify appropriate treatment strategies. Material and methods: In September 2005 a web-based prospective registry of CD patients was initiated in Poland. Ten institutes (9 academic centers, 1 referred regional hospital) took part in the project with the object of obtaining the demographic and clinical data of pediatric CD patients across the country. With this end in view, a computerized questionnaire was used and the collected data were sent prospectively to a central registry for analysis. The following data were analyzed: demographics, family history, location and behavior of disease, extraintestinal manifestation, coexisting diseases, diagnostic work-up, and medical treatment including surgical intervention. Results: Through the period of 4 years, 320 patients (male : female - 191 : 129) aged below 16 years with CD diagnosed at the mean age of 9.2 ±6.8 years were incorporated in the registry. Early onset of disease (age at diagnosis below 5 years) was recorded in 68 children (21.25%). Positive family history was reported for 16 patients (5%). The predominant localization of lesions described using the Montreal classification (L1 for small intestine, L2 for colon, L3 for ileocolon, and L4 for the upper gastrointestinal tract) was ileocolon (L3) - 217 patients (67.8%). The predominant behavior of disease was non-stricturing and non-penetrating - 225 patients (70.32%). Extraintestinal manifestation was reported in 20 patients (6.25%). Coexisting diseases occurred in 35 patients (10.93%). The predominant initial therapy was mesalazine (227 patients - 70.1%). Seventeen patients (5.31%) required a surgical intervention. Conclusions: This study provides comprehensive information on demographic and clinical aspects of pediatric CD in Poland. Our results are consistent with the previously published reports from other countries in terms of age of onset and male predominance in pediatric CD patients. Our conclusions are as follows: information needs to be well defined, validated at entry, and updated at every visit, which facilitates our work and makes the data more reliable

Serotype-Specific Pneumococcal Status prior to PCV 13 Administration in Children and Adolescents with Inflammatory Bowel Disease

The aim of this study was to evaluate the serotype-specific pneumococcal status of children and adolescents with inflammatory bowel disease (IBD) who were na&iuml;ve to pneumococcal vaccination before administering the 13-valent pneumococcal conjugate vaccine (PCV 13). This was an open, prospective study on children and adolescents aged 5&ndash;18 years who had IBD and were na&iuml;ve to pneumococcal vac&shy;cination. A single dose of PCV 13 was administered to each patient. The geometric mean concentrations (GMCs) were measured for all 13 serotypes. A total of 122 subjects completed the study. Prevaccination GMCs ranged from 0.55 &mu;g/ml (serotype 4) to 4.26 &mu;g/ml (sero&shy;type 19A). Prior to the administration of PCV 13, high GMCs were detected in older children and adolescents who had IBD and were na&iuml;ve to pneumococcal vaccination

Polish statement on food allergy in children and adolescents

An adverse food reaction is defined as clinical symptoms occurring in children, adolescents or adults after ingestion of a food or chemical food additives. This reaction does not occur in healthy subjects. In certain individuals is a manifestation of the body hypersensitivity, i.e. qualitatively altered response to the consumed food. The disease symptoms observed after ingestion of the food can be triggered by two pathogenetic mechanisms; this allows adverse food reactions to be divided into allergic and non-allergic food hypersensitivity (food intolerance). Food allergy is defined as an abnormal immune response to ingested food (humoral, cellular or mixed). Non-immunological mechanisms (metabolic, pharmacological, microbiological or other) are responsible for clinical symptoms after food ingestion which occur in non-allergic hypersensitivity (food intolerance). Food allergy is considered a serious health problem in modern society. The prevalence of this disorder is varied and depends, among other factors, on the study population, its age, dietary habits, ethnic differences, and the degree of economic development of a given country. It is estimated that food allergy occurs most often among the youngest children (about 6-8% in infancy); the prevalence is lower among adolescents (approximately 3-4%) and adults (about 1-3%). The most common, age-dependent cause of hypersensitivity, expressed as sensitization or allergic disease (food allergy), are food allergens (trophoallergens). These are glycoproteins of animal or plant origine contained in: cow's milk, chicken egg, soybean, cereals, meat and fish, nuts, fruits, vegetables, molluscs, shellfish and other food products. Some of these allergens can cause cross-reactions, occurring as a result of concurrent hypersensitivity to food, inhaled or contact allergens. The development of an allergic process is a consequence of adverse health effects on the human body of different factors: genetic, environmental and supportive. In people predisposed (genetically) to atopy or allergy, the development of food allergy is determined by four allergic-immunological mechanisms, which were classified and described by Gell-Coombs. It is estimated that in approximately 48-50% of patients, allergic symptoms are caused only by type I reaction, the IgEmediated (immediate) mechanism. In the remaining patients, symptoms of food hypersensitivity are the result of other pathogenetic mechanisms, non-IgE mediated (delayed, late) or mixed (IgE mediated, non-IgE mediated). Clinical symptomatology of food allergy varies individually and depends on the type of food induced pathogenetic mechanism responsible for their occurrence. They relate to the organ or system in which the allergic reaction has occurred (the effector organ). Most commonly the symptoms involve many systems (gastrointestinal tract, skin, respiratory system, other organs), and approximately 10% of patients have isolated symptoms. The time of symptoms onset after eating the causative food is varied and determined by the pathogenetic mechanism of the allergic immune reaction (immediate, delayed or late symptoms). In the youngest patients, the main cause of food reactions is allergy to cow’s milk. In developmental age, the clinical picture of food allergy can change, as reflected in the so-called allergic march, which is the result of anatomical and functional maturation of the effector organs, affected by various harmful allergens (ingested, inhaled, contact allergens and allergic cross-reactions). The diagnosis of food allergy is a complex, long-term and time-consuming process, involving analysis of the allergic history (personal and in the family), a thorough evaluation of clinical signs, as well as correctly planned allergic and immune tests. The underlying cause of diagnostic difficulties in food allergy is the lack of a single universal laboratory test to identify both IgE-mediated and non-IgE mediated as well as mixed pathogenetic mechanisms of allergic reactions triggered by harmful food allergens. In food allergy diagnostics is only possible to identify an IgE-mediated allergic process (skin prick tests with food allergens, levels of specific IgE antibodies to food allergens). This allows one to confirm the diagnosis in patients whose symptoms are triggered in this pathogenetic mechanism (about 50% of patients). The method allowing one to conclude on the presence or absence of food hypersensitivity and its cause is a food challenge test (open, blinded, placebo-controlled). The occurrence of clinical symptoms after the administration of food allergen confirms the cause of food allergy (positive test) whereas the time elapsing between the triggering dose ingestion and the occurrence of clinical symptoms indicate the pathogenetic mechanisms of food allergy (immediate, delayed, late). The mainstay of causal treatment is temporary removal of harmful food from the patient’s diet, with the introduction of substitute ingredients with the nutritional value equivalent to the eliminated food. The duration of dietary treatment should be determined individually, and the measures of the effectiveness of the therapeutic elimination diet should include the absence or relief of allergic symptoms as well as normal physical and psychomotor development of the treated child. A variant alternative for dietary treatment of food allergy is specific induction of food tolerance by intended contact of the patient with the native or thermally processed harmful allergen (oral immunotherapy). This method has been used in the treatment of IgE-mediated allergy (to cow's milk protein, egg protein, peanut allergens). The obtained effect of tolerance is usually temporary. In order to avoid unnecessary prolongation of treatment in a child treated with an elimination diet, it is recommended to perform a food challenge test at least once a year. This test allows one to assess the body's current ability to acquire immune or clinical tolerance. A negative result of the test makes it possible to return to a normal diet, whereas a positive test is an indication for continued dietary treatment (persistent food allergy). Approximately 80% of children diagnosed with food allergy in infancy "grow out" of the disease before the age of 4-5 years. In children with non-IgE mediated food allergy the acquisition of food tolerance is faster and occurs in a higher percentage of treated patients compared to children with IgE-mediated food allergy. Pharmacological treatment is a necessary adjunct to dietary treatment in food allergy. It is used to control the rapidly increasing allergic symptoms (temporarily) or to achieve remission and to prevent relapses (long-term treatment). Preventive measures (primary prevention of allergies) are recommended for children born in a "high risk" group for the disease. These are comprehensive measures aimed at preventing sensitization of the body (an appropriate way of feeding the child, avoiding exposure to some allergens and adverse environmental factors). First of all, the infants should be breast-fed during the first 4-6 months of life, and solid foods (non milk products, including those containing gluten) should be introduced no earlier than 4 months of age, but no later than 6 months of age. An elimination diet is not recommended for pregnant women (prevention of intrauterine sensitization of the fetus and unborn child). The merits of introducing an elimination diet in mothers of exclusively breast-fed infants, when the child responds with allergic symptoms to the specific diet of the mother, are disputable. Secondary prevention focuses on preventing the recurrence of already diagnosed allergic disease; tertiary prevention is the fight against organ disability resulting from the chronicity and recurrences of an allergic disease process. Food allergy can adversely affect the physical development and the psycho-emotional condition of a sick child, and significantly interfere with his social contacts with peers. A long-term disease process, recurrence of clinical symptoms, and difficult course of elimination diet therapy are factors that impair the quality of life of a sick child and his family. The economic costs generated by food allergies affect both the patient's family budget (in the household), and the overall financial resources allocated to health care (at the state level). The adverse socio-economic effects of food allergy can be reduced by educational activities in the patient’s environment and dissemination of knowledge about the disease in the society