Rola terapii biologicznej w podtrzymaniu remisji u dzieci z chorobą Leśniowskiego i Crohna

AbstractIn recent years there has been an increase in the incidence of inflammatory bowel disease including Crohn's disease in children. The aim of treatment of Crohn's disease is the induction and maintenance of remission. Currently, it is believed that to achieve healing of the intestinal mucosa may be synonymous with getting complete remission and inhibition of disease progression. Biologics and immunomodulators have a definite place in the treatment of Crohn disease. However, there are no clear guidelines on the efficacy and safety of these two classes of drugs for the children treatment. In addition, questions about the safety of long-term use of biological drugs as well as the best moment of their inclusion needs further reaserch

Nowości pediatryczne na 9. Kongresie ECCO 2014 w Kopenhadze

W dniach 22–24 lutego bieżącego roku w Kopenhadze odbyła się coroczna konferencja Europejskiej Organizacji ds. Nieswoistych Chorób Zapalnych Jelit (ECCO). W tym roku zaprezentowane zostały nowy konsensus pediatryczny, który jest efektem dwuletniej pracy grupy roboczej, a także wiele streszczeń na sesji plakatowej. Celem artykułu jest przedstawienie i krótkie omówienie najciekawszych zagadnień pediatrycznych z kongresu

Postępy w terapii nieswoistych zapaleń jelit u dzieci

Niedożywienie u pacjentów z nieswoistymi chorobami zapalnymijelit jest najczęstszym powikłaniem, co szczególnieodbija się na rozwoju fizycznym pacjentów pediatrycznychprzed zakończeniem okresu wzrastania. Dotyczy to zwłaszczadzieci z chorobą Leśniowskiego-Crohna, u których w 65%stwierdzano niedobór wysokości ciała, a u 87% niedobórmasy ciała. W pracy przedstawiono korzystne wyniki leczeniażywieniowego, które u dzieci nie ustępuje steroidoterapii.Podsumowano korzystne wyniki leczenia biologicznegow chorobie Leśniowskiego-Crohna zarówno po zastosowaniuinfliksimabu, jak i adalimubabu. Pozwala ono nie tylko na uzyskanieremisji, ale także na jej dłuższe utrzymanie, uniknięciepowikłań, a co ważniejsze — na wygojenie zmian zapalnychbłony śluzowej jelita. Własne obserwacje wskazują na korzystnyefekt leczenia operacyjnego polegającego na usunięciuzwężonego lub znacznie zmienionego zapalnie odcinka jelitacienkiego i/ lub grubego, co skutkuje poprawą przyrostówwysokości i masy ciała, znaczną poprawą samopoczucia,a u zdecydowanej większości pacjentów wieloletnimi remisjamiw przebiegu choroby.Monitorowanie stężenia 6-tioguaniny (6-TGN) zapewnia bezpieczeństwoleczenia tiopurynami przy jednoczesnym stosowaniuinnych leków wpływających na metabolizm azatioprynyi merkaptopuryny, na przykład allopurinolu. Wydaje się zatemcelowe unowocześnienie terapii tiopurynami przez oznaczenie6-TGN w erytrocytach.Podawana doustnie lub dożylnie cyklosporyna może przynieśćbardzo szybką poprawę zarówno u pacjentów dorosłych, jaki dzieci, ale jej rola w podtrzymaniu remisji jest znikoma,o czym świadczy wysoki wskaźnik kolektomii w trakciepierwszego roku od rozpoczęcia leczenia

Czy mikroskopowe zapalenie jelita grubego należy do nieswoistych chorób zapalnych jelit?

Najnowszy konsensus histopatologiczny Europejskiej Organizacji ds. nieswoistych chorób zapalnych jelit (ECCO), wydany w 2013 roku, obejmuje opis i wytyczne diagnozowania mikroskopowego zapalenia jelita grubego (MZJG). W związku z tym powstaje pytanie, czy MZJG należy zaliczyć do nieswoistych chorób zapalnych jelit, wraz z chorobą Leśniowskiego-Crohna oraz wrzodziejącym zapaleniem jelita grubego. Zagadnienie to było również dyskutowane podczas 9. Kongresu ECCO, który odbył się w Kopenhadze w lutym 2014 roku

Research gaps in diet and nutrition in inflammatory bowel disease. A topical review by D-ECCO Working Group (Dietitians of ECCO)

Although the current doctrine of IBD pathogenesis proposes an interaction between environmental factors with gut microbiota in genetically-susceptible individuals, dietary exposures have attracted recent interest and are, at least in part, likely to explain the rapid rise in disease incidence and prevalence. The D-ECCO working group along with other ECCO experts with expertise in nutrition, microbiology, physiology and medicine reviewed the evidence investigating the role of diet and nutritional therapy in the onset, perpetuation and management of IBD. A narrative topical review is presented where evidence pertinent to the topic is summarized collectively under three main thematic domains: i) the role of diet as an environmental factor in IBD aetiology; ii) the role of diet as induction and maintenance therapy in IBD; and iii) assessment of nutritional status and supportive nutritional therapy in IBD. A summary of research gaps for each of these thematic domains is proposed which is anticipated to be agenda setting for future research in the area of diet and nutrition in IBD

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

Demographic characteristics of children with early clinical manifestation of inflammatory bowel disease

Abstract Introduction: Inflammatory bowel disease (IBD), which includes Crohn's disease (CD) and ulcerative colitis (UC), is a chronic condition of the colon and small intestine. The disease is common in young people (children and young adults), but it is rare in children younger than five years of age. Therefore, IBD developing during the first years of life (under the age of 5) is known as an early-onset IBD (EO-IBD), and it is considered to be a specific entity with a distinct phenotype. However, the available data on that issue are still insufficient. Aim: To determine the characteristics and clinical course of children with early-onset IBD. Material and methods: We performed a retrospective database analysis of 47 infants younger than 5 years old diagnosed with IBD. Patient's demographic data, including age, sex, and age at disease onset, were collected in 6 paediatric hospitals in Poland. Disease location was established on the basis of the review of all endoscopic, colonoscopic, histopathological, and radiological records. All possible complications were reported, as well as any treatment and its efficacy. Since the diagnosis was established all patients have been on follow up. Results: Among 47 children registered in the database, 23 (49%) had a diagnosis of CD, 16 (34%) had UC, and 8 (17%) had IC (indeterminate colitis). The mean age at diagnosis was 28.5 ±27.5 months; 57.4% were male. The most common location/type of disease was ileocolonic disease (L3). The most common complication of IBD was anaemia, found in 30 (63.8%) children. The observed course of the disease was either severe or moderate. In 4 children younger than 2 years old, surgery was performed. Conclusions: Inflammatory bowel disease in children younger than 5 years old includes UC, CD, and a relatively high proportion of IC. In early-onset IBD severe and moderate course of the disease is usually observed. Disease manifestation in these patients is predominantly ileocolonic

Home enteral nutrition in children—2010 nationwide survey of the polish society for clinical nutrition of children

Published epidemiologic data on the administration rates of enteral/parenteral home nutrition is very limited. The aim of this first nationwide study was to assess the availability of pediatric home enteral nutrition (HEN) services in Poland. The questionnaire was sent to all regional centers providing pediatric HEN services in Poland (n = 14). The analysis included the number of pediatric patients who received HEN in 2010, their demographic characteristics and geographical distribution. Furthermore, the distributions of indications and methods of enteral nutrition administration were analyzed, along with the reasons of withdrawal from the HEN program. The number and fraction of children receiving HEN increased in 2010, from 433 (11.34 per 1 million inhabitants) on January 1st to 525 (13.75) on December 31st. Marked differences were observed in geographical distribution of this parameter, from zero to up to 30 pediatric patients per 1 million inhabitants. Median age of patients was 6 years (range: 9 months–18 years). In most cases, HEN was prescribed due to neurological disorders (n = 337, 64.2%), and administered by means of gastrostomy (n = 450, 85.71%). This study revealed the dynamic development of pediatric HEN services in Poland but also documented their potential regional shortages

Dual Biologic Therapy in Moderate to Severe Pediatric Inflammatory Bowel Disease: A Retrospective Study

Background: Inflammatory bowel diseases in children are characterized by a wide variety of symptoms and often a severe clinical course. In the treatment, we aimed to induce and maintain remission. We focused on assessing the efficacy and safety of the concomitant use of two biologic therapies including: anti-TNF (infliximab, adalimumab) vedolizumab and ustekinumab in a refractory pediatric IBD cohort. Methods: Fourteen children (nine ulcerative colitis, one ulcerative colitis/IBD-unspecified, four Crohn’s disease) with a disease duration of 5.2 (8 months–14 years) years, initiated dual therapy at an age of 11.7 (3–17) years after failure of monotherapy with a biological drug. Five patients (36%) were treated with vedolizumab/adalimumab (VDZ + ADA), five (36%) with ustekinumab/adalimumab (UST + ADA), and three (21%) with infliximab/vedolizumab (IFX + VDZ). One patient (7%) was switched from a combination of vedolizumab and adalimumab to ustekinumab and adalimumab during follow-up. Results: A clinical improvement was obtained in ten children (73%; 5 UC, 1 UC/IBD-unspecified, 4 CD) on the PCDAI/PUCAI scale after 4 months of a second biological drug being added. The median fecal calprotectin decreased from 1610 µg/g (140–10,100) to 586 µg/g (5–3410; p = 0.028) between baseline and 4 months. Conclusions: Our clinical experience suggests that dual therapy may be an option for pediatric patients with moderate and severe courses of IBD with limited therapeutic option

Methylation of RUNX3 Promoter 2 in the Whole Blood of Children with Ulcerative Colitis

Ulcerative colitis (UC) results from a complex interplay between the environment, gut microbiota, host genetics, and immunity. Runt-related transcription factor 3 (RUNX3) regulates Th1/Th2 balance and, thus, the synthesis of cytokines and inflammation. We aimed to analyze the dependence of RUNX3 promoter 2 (P2) methylation level on: age, sex, body mass index (BMI), C-reactive protein (CRP), serum albumin, disease duration, Pediatric Ulcerative Colitis Activity Index (PUCAI), the Paris classification, and exposure to medications. This multicenter, cross-sectional study recruited hospitalized children with UC. Methylation of RUNX3 P2 was measured with methylation-sensitive restriction enzymes in the whole blood DNA. Sixty-four children were enrolled, with a mean age of 14.5 ± 2.8 years. Half of them were female (51.6%), and the average BMI Z-score was −0.44 ± 1.14. The mean methylation of RUNX3 P2 was 54.1 ± 13.3%. The methylation level of RUNX3 P2 did not correlate with age, sex, nutritional status, CRP, albumin, PUCAI, or the extent of colitis (Paris E1–E4). RUNX3 P2 methylation did not differ between patients recruited within two and a half months of diagnosis and children who had UC for at least a year. Current or past exposure to biologics, immunosuppressants, or steroids was not associated with RUNX3 P2 methylation. Methylation of RUNX3 promoter 2 in whole blood DNA does not seem to be associated with the characteristics of UC in children