Inflammatory bowel disease

Inflammatory bowel disease (IBD) describes a heterogeneous group of chronic inflammatory diseases of the gastrointestinal tract, of which Crohn’s disease and ulcerative colitis are the most common diagnoses. About a quarter of IBD presents in childhood, and the phenotype seen involves a wider disease distribution in both Crohn’s and ulcerative colitis than seen in adult-onset disease. The incidence of IBD appears to be rising and although we understand more about the genetic, immunological and environmental contributors to aetiology, we do not yet fully understand this rise. In paediatric practice, IBD and in particular Crohn’s disease, often results in impaired weight gain, poor linear growth and delayed puberty. Multiple treatment modalities exist for IBD from longstanding treatments such as steroids and immunosuppressants to modern, targeted therapies such as infliximab. This review discusses the current state of the art of clinical practice in relation to paediatric IBD.     

Paediatric inflammatory bowel disease- brief update on current practice

Paediatric inflammatory bowel disease (PIBD), consisting of Crohn's disease, ulcerative colitis and inflammatory bowel disease unclassified, is a chronic inflammatory condition associated with significant morbidity. The incidence of PIBD is increasing and disease phenotype remains more severe than adult onset disease. Diagnosis of PIBD is often slow and requires referral to specialist services; however, the emergence of faecal calprotectin as a tool for prioritizing further investigation, alongside improved use of treatments (including anti-TNF monoclonal antibody therapy) is changing diagnosis and management. Whilst significant challenges remain in the longer-term treatment of PIBD, including growth, nutrition and management of refractory disease there remains a strong research focus on understanding underlying disease pathogenesis and a move towards personalized medicine. This review describes investigations, diagnosis and management of PIBD and presents the most up to date evidence on nutritional and medical management.     

Inflammatory bowel disease

Till about 3 decades ago, inflammatory bowel disease (IBD) was considered as non-existent in our country. However, since that time several reports of IBD, mainly ulcerative colitis have been published. More recently, Crohn’s disease is also being reported from the country. This trend of UC appearing first in a population followed by CD also appears to be true in other developing nations. A substantial increase in the rates of CD over UC in the last few decades is reported from developed nations as well. Of the other epidemiological factors, an increased risk of CD and lower risk of UC in smokers is established in adults. However, it appears that smoking increases the risk of IBD in children. The etiology of IBD remains elusive. Within the triad of genetics, immunity and antigen responsible for the development of IBD, maximum advances have been made in the field of immune aberrations and this is being exploited to treat the disease. It is well established that IBD results from a disordered immune system in the gut, in response to an unidentified antigen in a predisposed individual. The immune response is enhanced and revolves around antigen-presenting cells, CD 4 T-lymphocytes and tumor necrosis factor alpha. CD results from an enhanced Th1 activity. The pathogenesis of UC is less clear but appears to be humoral. Advances in diagnostics include the availability of serology, ultrasound and nuclear scans, none of which have been tried in our setting where infectious diseases and tuberculosis is rampant. Growth failure and the importance of nutrition in IBD, especially CD, cannot be underemphasized. In many situations nutritional interventions have been used solely as a form of therapy for CD. Newer steroid molecules with minimal systemic effects are also being considered. Other treatment options highlighted are the use of immunosuppressive agents, biologic agents and role of surgery.     

Current diagnosis, management and morbidity in paediatric inflammatory bowel disease

In the 1970s several reports highlighted the long delay in diagnosis often experienced by children with Crohn's disease. In recent years this disorder has attracted much publicity, and many believe that the incidence has increased substantially. The aim of this investigation was to determine whether heightened awareness had shortened the interval to diagnosis, improved clinical management and reduced morbidity. A retrospective study was therefore carried out on 112 children with inflammatory bowel disease (64 Crohn's disease, 41 ulcerative colitis, 7 indeterminate colitis) referred to a paediatric gastroenterology department in the UK between 1994 and 1998. In Crohn's disease the median interval to diagnosis was 47 wk (maximum 7 y). In those without diarrhoea this was longer (66 vs 28 wk; p = 0.005). In ulcerative colitis the median interval was 20 wk (maximum 3 y). Even in severe colitis the median interval was 5.5 wk (range 3-9 wk) and 4 required urgent colectomy soon after referral. Many with unrecognized Crohn's disease had undergone inappropriate treatments, such as growth hormone or psychiatric therapy. Nineteen (17%) had undergone endoscopic investigations in adult units prior to referral. Malnutrition was equally common in Crohn's disease and ulcerative colitis (11%). Short stature was present in 19% with Crohn's disease, and 5% with ulcerative colitis, and was severe in 8% with Crohn's disease. There was a significant correlation between symptom duration and the degree of growth impairment present (r(s) = -0.4; p = 0.004). Conclusion: This study suggests that late diagnosis and inappropriate investigation and management are still significant problems.     

Incidence and prevalence of inflammatory bowel disease in children in South-Western Sweden

Hildebrand H, Brydolf M, Holmquist L, Krantz I, Kristiansson B. Incidence and prevalence of inflammatory bowel disease in children in South-Western Sweden Acta PEdiatr 1994;83:640–5. Stockholm. ISSN 0803–5253
The incidence and prevalence of inflammatory bowel disease were estimated in all children less than 16 years of age living in the city of Göteborg and in three counties in South-Western Sweden, from 1983 to 1987. One hundred and thirty-two patients were classified according to set criteria into one of four diagnostic categories: ulcerative colitis, Crohn's disease, probable Crohn's disease and indeterminate colitis. The crude incidence of inflammatory bowel disease was 5.3 per 100 000 children per year and the prevalence 21.5 per 100000 children. This study lends support to the hypothesis that Crohn's disease has increased among Swedish children. Crohn's disease now appears to be at least as common as ulcerative colitis. Thirty-five of 55 patients first classified as indeterminate colitis or probable Crohn's disease later fulfilled the criteria of ulcerative colitis or Crohn's disease during a mean follow-up period of 4.6 years. This study emphasizes the importance, in epidemiological studies of inflammatory bowel disease, of inciuding those cases where a definite diagnosis of ulcerative colitis or Crohn's disease cannot be established initially and of re-evaluating the initial diagnosis regularly.     

青少年炎症性肠病伴生长激素缺乏4例并文献复习

炎症性肠病（IBD）为慢性复发性非特异性肠道炎症性病变,10%~56%的克罗恩病患儿和10%左右的溃疡性结肠炎患儿存在生长迟缓。该研究报道4例伴生长激素缺乏的青少年IBD（均为克罗恩病）,其中男3例、女1例,确诊年龄11.0~13.9岁,确诊时病程11~85个月。4例患儿的病灶分别为：单纯小肠、单纯结肠、小肠及上消化道、小肠及结肠均累及,克罗恩病活动指数27.5~45分。4例患儿的年龄别身高Z评分（HAZ）均 < -2,生长激素刺激试验均提示生长激素缺乏症。2例患儿接受了重组人生长激素联合英夫利西单抗治疗,1例仅接受英夫利西单抗治疗,另1例接受重组人生长激素联合巯嘌呤的治疗。所有患儿经治疗后HAZ评分均有改善。     

Simultaneous presentation of coeliac disease and ulcerative colitis in a child

Coeliac disease and inflammatory bowel disease (IBD) individually are not uncommon in children, but the occurrence of both conditions together is rare. The combined presentation of coeliac disease and IBD in a girl of 7 years is presented with a review of the related literature. The occurrence of coeliac disease with IBD should be considered at the time of diagnosis and at relapse, or where there is difficulty maintaining remission in established IBD. Screening with serum antibody tests may be helpful.     

儿童炎症性肠病11例

目的探讨儿童炎症性肠病(IBD)的临床特点,以提高对儿童IBD的认识。方法对1995年1月-2005年12月本院收治的11例IBD患儿的临床资料进行回顾性分析。结果11例IBD患儿中克罗恩病(CD)8例,溃疡性结肠炎(UC)3例;男9例,女2例;发病年龄1个月～12岁9个月(中位年龄6岁)。11例IBD患儿均无阳性家族史。临床表现为贫血9例(81.8%),消瘦、腹痛各8例(72.7%),发热7例(63.6%),腹泻6例(54.5%),便血5例(45.5%)。实验室检查11例(100%)患儿均有CRP增高,ESR升高10例(90.9%),白细胞升高8例(72.7%)。病理结果表现为浅表小溃疡7例(63.6%)、黏膜慢性炎症6例(54.5%),患儿均未见非干酪样肉芽。其中10例予5-氨基水杨酸或柳氮磺胺吡啶、糖皮质激素治疗后缓解。结论IBD可在婴幼儿期发病,其诊断应根据临床表现、血液学指标、消化内镜及组织病理学等资料进行综合评价。如临床高度怀疑IBD,实验室检查虽无典型病理改变,在除外感染性疾病和其他自身免疫性疾病的基础上,可行诊断性治疗。     

MICI-MINOTS: Linguistic and metric validation of a pediatric questionnaire on knowledge about inflammatory bowel disease

BackgroundTherapeutic education is an essential part of the treatment of chronic diseases, such as inflammatory bowel disease (IBD). The IBD-KID, developed in Canada in English, assesses children's and adolescents’ acquired knowledge about their condition and has been validated in Canadian and Australian populations. However, there is no pediatric questionnaire in French to assess patients’ knowledge about IBD.ObjectiveTo report the linguistic validation process and metric validity of the MICI-MINOTS, the French version of the IBD-KID.MethodThe translation process consisted of three consecutive steps: forward–backward translation, acceptability testing, and cognitive interviews. The IBD-KID consists of 23 questions, but a 24th question about immunomodulatory therapy was added in the MICI-MINOTS. Psychometric testing was conducted with five groups: children with IBD, their parents, children in a control group, their parents, and health workers recruited from the Timone Pediatric Hospital and the Saint-Sébastien Maternal and Child Protection Center, Marseille, France. A total of 15 individuals completed the tool twice, with a 15-day interval. Internal consistency, reliability, external validity, reproducibility, and sensitivity to change were tested.ResultsA total of 38 children with IBD (sex: 20 boys, 18 girls; age: 13.90 [± 2.88] years; 25 with Crohn's disease), 20 children in the control group, 58 parents (every child was included with one parent), and 62 health workers were included in the analysis. Intraclass correlation was 0.94 (95% confidence interval 0.83–0.98) for test–retest assessment. Readability using the Scolarius score corresponded to elementary school level. Among the children with IBD, 89.5% answered all 24 questions. For 23 questions, the mean score of children with IBD was higher than among children in the control group: 9.58 (± 3.01) versus 5.47 (± 3.56), respectively (P < 0.01). Parents of children with IBD scored higher than parents of children in the control group: 10.63 (± 3.16) versus 8.4 (± 3.07), respectively (P = 0.012). In the health workers’ group, pediatric residents (17.82 ± 3.46) scored higher than nurses 11.75 (± 3.4) and ward clerks (8.67 ± 2.40; P < 0.01). Patients’ knowledge score was significantly related to their parents’ knowledge score (r = 0.402, P = 0.012) for 23 questions.ConclusionThe French version of the IBD-KID showed satisfactory psychometric properties to assess knowledge about the disease in French-speaking children.     

Health-related quality of life in paediatric patients with inflammatory bowel disease related to disease activity

Aim: Impaired health‐related quality of life (HRQoL) and an increased risk of psychosocial problems may encounter children and adolescents with inflammatory bowel disease (IBD). Generic HRQoL questionnaires, 15D designed for subjects over 16 years of age, 16D for adolescents aged 12–15 and 17D for younger children, allow comparison to healthy peers and have not been used in children with IBD before. Further, in paediatric IBD patients, HRQoL has not been related to disease activity. We evaluated the applicability of 15D, 16D and 17D questionnaires in the paediatric IBD population and examined how HRQoL is influenced by changes in clinical activity of IBD. Methods: The study subjects recruited at their scheduled, routine appointment in the outpatient clinic of the children's hospital completed the HRQoL questionnaire at baseline and again after 3–5 months. Disease activity was estimated by a three‐level scale. The HRQoL of the study population was compared with that of the age‐standardised general population. Results: Fifty‐five children, aged 7–19 years, were recruited. The HRQoL scores strongly correlated with the activity of the disease (P < 0.001). The two oldest age groups with IBD had lower HRQoL scores than age‐standardised peers (P= 0.001/0.04). There was no gender difference in HRQoL scores. Conclusions: IBD has a considerable impact on the HRQoL of children and adolescents. The generic HRQoL instruments used appeared to be promising tools for examining HRQoL in paediatric IBD patients in different age groups, but larger studies to establish their usefulness in the follow‐up of young patients are still warranted.