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Crohn's disease in childhood is a chronic relapsing condition with a high morbidity. Growth failure is common. The aim of therapy is to induce and then maintain disease remission and thereby promote well-being and normal growth and development. Enteral nutrition (either polymeric or elemental) is effective and used as initial therapy. This is employed as sole therapy over a 6- to 8-week period followed by a period of controlled food reintroduction. The relapse rate is high and further courses of enteral nutrition or alternative therapies are frequently required. Corticosteroids are also effective as initial therapy and are required in difficult cases but there are problems with their long term use, particularly their adverse effects on growth. Many patients develop either corticosteroid-dependent or corticosteroid-resistant disease. In this instance, additional immunosuppression, such as azathioprine, can be used. Surgery is required for those patients with disease resistant to medical therapy and this will result in remission; however, the relapse rate with surgery is high. There are many areas for future research. Very little is known about why enteral nutrition works, how long it should be given or its role as maintenance therapy. Newer immunosuppressive strategies based on cytokine modulation may be helpful in children once more experience is gained from their use in adults.

Pain is a common complaint in children with gastrointestinal tract pathology and it has significant consequences for patients’ quality of life. A thorough evaluation should be performed to determine the cause and severity. Understanding the pathophysiology of pain in various conditions is key to effective management. This chapter outlines the aetiology and general principles of the management of pain in gastrointestinal disease. The specific management of common gastrointestinal conditions associated with pain, e.g. inflammatory bowel disease, gastro-oesophageal reflux disease, pancreatitis, and gut dysmotility disorders are discussed.

Crohn’s disease is characterised by transmural inflammation located at any point within the gastro-intestinal tract from mouth to anus. Crohn’s disease is diagnosed by histological findings in individuals with compatible clinical history and examination. The hallmark feature of Crohn’s is discontinuous inflammation with associated non-caseating granulomata.

Recent data are scarce on the provision of home parenteral nutrition (HPN) in children from the UK but would help to commission intestinal failure services. Our aim was to describe 10 years of HPN experience in our centre, which serves a population of 650,000 children.Outcome and complication data were collected retrospectively from hospital records of children receiving HPN from April 2001. Data from other centres were used to compare complications and outcomes in the provision of HPN.Nineteen children (12 females) received 10,213 days (28 years) of HPN. In this group, incidence of blood culture positive sepsis was four episodes/1000 days PN. Two children had early intestinal failure-associated liver disease. Of the 19, seven still receive HPN at our centre, six survived PN, three were transferred to other services while still on HPN and three died.Outcome and complication data for HPN from a single UK regional paediatric centre are similar to larger centres. These data provide recent evidence of the disease burden of HPN, which are important for the commissioning of intestinal failure services.

Inflammatory bowel disease 288• 25 % of inflammatory bowel disease (IBD) presents in childhood, usually as Crohn's disease or ulcerative colitis. The UK incidence is 5.2/100 000 children

Bacterial overgrowth 250Bacterial overgrowth refers to the syndrome of stasis of the small-bowel contents leading to bacterial proliferation and excessive numbers of bacteria being present in the small bowel (normally most of the gut bacteria are in the colon). It is also known as ‘blind loop syndrome’ or ‘stagnant loop syndrome’....

• Vitamin deficiency 58• Mineral deficiency 61• Trace element deficiency 62• Vitamin supplementation for infants and young children 64The term ‘micronutrients’ includes two main classes of nutrient substances required in the diet in very small amounts: the essential organic micronutrients (vitamins) and the essential inorganic micronutrients (trace elements). Vitamin and mineral deficiencies may complicate malnutrition arising from underlying disease or inadequate diet. Key features are given below. However, micronutrients have wide-ranging effects, far beyond the simple prevention of deficiency states....

Carbohydrates in the diet 128Carbohydrate digestion 129Hypolactasia/lactose intolerance 130Congenital sucrase–isomaltase deficiency 130Glucose–galactose malabsorption 131Confirmation of diagnosis of carbohydrate malabsorption 131Carbohydrates make up at least half the energy intake in the diet. The principal carbohydrates are the storage polysaccharides (starch, glycogen and cellulose), the disaccharides lactose and sucrose, and the monosaccharides glucose and fructose....

Who to investigate 234How to investigate 236Diagnosis 238Treatment 240Follow-up and support 242Coeliac disease is an immune-mediated enteropathy caused by a permanent sensitivity to gluten which is present in wheat, barley, and rye. It occurs in genetically susceptible children and adults. The classical presentation is with chronic diarrhoea, abdominal distension, and failure to thrive. The widespread availability of antibody screening has considerably changed the clinical spectrum of cases seen. The testing of children with less classical symptoms and screening of children at high risk has brought increasing recognition of the varied presentation and increased prevalence of this now very common condition....

Definition 488Pathophysiology 488Clinical features 489Causes 490Investigations 491Management 492Portal hypertension is increased blood pressure within the portal venous system and defined as an increase in the pressure gradient between the portal veins and the hepatic veins (>5 mmHg).The portal vein carries nutrient-rich blood to the liver from the GI tract and spleen. At the hilum of the liver it divides into the major right and left portal veins. Within the liver these veins undergo further divisions to supply each segment, and terminate in small branches, which pierce the limiting plate of the portal tract and enter the hepatic sinusoids through small channels (...

Appetite 114Common feeding problems in 1–5 year olds 116How to increase energy intake 117Food refusal is common in early life. During the first year infants will try food because they are hungry, or because they are using their mouths to explore the environment. Later on, there has to be motivation to try new foods, and this usually comes from imitation of other people eating. In early childhood it is the presentation of safe and socially appropriate foods and their repeated ingestion that leads to them being liked....

Benefits of breast-feeding 25Breast-feeding basics 26Contraindications to breast-feeding 27Promotion of breast-feeding 28Breast milk is the ideal food for infants. The World Health Organization recommends exclusive breast-feeding for at least the first 6 months of life. Until March 2001, the WHO recommended exclusive breast-feeding only for the first 4–6 months of life. This change in policy was based on a systematic review of the published scientific literature which highlighted a protective effect of prolonged breast-feeding against gastrointestinal disease, and confirmed health benefits to mothers. The applicability of these findings to developed countries has been questioned....

Epidemiology 340Unconjugated hyperbilirubinaemia 340Specific conditions 342Conjugated hyperbilirubinaemia 343Idiopathic neonatal hepatitis 347• 30–50% of normal term newborns are jaundiced after birth.• Physiological and breast milk jaundice account for the majority of cases.• 1 in 2500 infants has conjugated hyperbilirubinaemia....

Introduction 252Appendicitis 253Intussusception 253Miscellaneous conditions 254The commonest surgical diagnosis in children who present to hospital with acute abdominal pain is appendicitis. The differential diagnosis is wide, however (see box below), and in >50% of admissions no specific cause is found....

Clinical features 200Diagnostic criteria 200Triggers 200Investigation 201Management 201Prophylaxis 202Cyclical vomiting was first described by Samuel Gee in 1882. It refers to intense periods of vomiting with symptom-free intervals. The incidence is unknown. It occurs principally in pre-school or early school age children. Epilepsy is a risk factor. Other risk factors include a history of recurrent headache, migraine (50%), travel sickness, and irritable bowel syndrome (50%) in children and their families....

Access routes for enteral tube feeding 73Complications of enteral nutritional support 76Liquid feed composition and choice of feed 78Assessment of a patient needing nutritional support will include a decision regarding the most appropriate method of feeding. The enteral route is preferred for children who have an adequately functioning gastrointestinal tract. Oral intake can simply be increased by use of food fortification, sip feeds, or energy supplements. If oral intake is poor or contraindicated, tube feeding may be used....

Hamartoms 220Adenomas 221Hyperplastic polyps 221Inflammatory polyps 222Polyps generally present with painless rectal bleeding or through genetic screening of affected families with polyposis syndromes. There are various types, as listed in Table 31.1. Juvenile polyps (hamartomas) are the most commonly seen and generally benign....

Guidelines for the prevention of refeeding syndrome 82Refeeding syndrome is a term used to describe the various metabolic complications that can arise as a result of instituting nutritional support (enteral or parenteral) in malnourished patients.• Such patients are catabolic and their major sources of energy are fat and muscle; total body stores of nitrogen, phosphate, magnesium, and potassium are depleted....

Definition 350Incidence 350Types 350Pathogenesis 350Clinical presentation 351Diagnosis 351Treatment 351Complications 352Prognosis 352Biliary atresia (BA) is a progressive cholangiopathy of unknown aetiology affecting extra- and intrahepatic biliary system presenting within the first several weeks of life.BA is a sporadic condition with an estimated worldwide incidence of around 1/12 000–15 000 live births. It has been described in isolation in twin and triplet pregnancies, with no seasonal pattern. Children are typically born at term, with no gender predominance....

• Indications and contraindications 496• Timing of transplantation 497• Pre-transplant assessment 498• Types of liver transplantation 500• Immediately after transplantation 501• Complications 502Liver transplantation is now a standard treatment for: • Acute liver failure• Chronic liver failure•...

Introduction 182Reflux oesophagitis 182Symptoms and signs of GORD 183Investigation 184Indications for investigation of presumed gastro-oesophageal reflux 186Management 188Specific treatment 190Gastro-oesophageal reflux is very common. It refers to the passage of gastric contents into the lower oesophagus. It is a normal physiological phenomenon, particularly common in infancy. It is also seen in older children and adults, particularly after meals. It is secondary to transient relaxation of the lower oesophageal sphincter not associated with swallowing....

Anorexia nervosa 110Bulimia nervosa 112Eating disorders are defined as persistent disturbance of eating (± behaviour) that impairs physical health or psychosocial functioning or both and that is not secondary to any other medical or psychiatric disorder.Anorexia nervosa is a complex disorder described in a number of different ways and recognized for >100 years. It involves voluntary self-starvation, with weight loss, or avoidance of weight gain during adolescence. Peak age of onset is in mid-teens, with a female to male ratio of 10:1, and a prevalence of around 1%. Genetic factors are important, with 55% of monozygotic twins being concordant for anorexia. Sociocultural factors are highly relevant, with the illness occurring predominantly in Western societies where thinness has become increasingly valued as an element of the feminine ideal. Reported mortality rates vary from 0 to 22%....

Necrotizing enterocolitis 46Necrotizing enterocolitis (NEC) is the most common gastroenterological emergency in the neonatal intensive care unit (NICU) and the major cause of death for all newborns undergoing surgery. The mortality is greater than that from all the congenital disorders of the gastrointestinal tract combined. Survivors may be left with short-bowel syndrome as well as other long-term gastrointestinal, growth and neurodevelopmental sequelae. NEC frequently presents as feed intolerance with bile-stained gastric residuals, abdominal distension, blood in the stools, apnoea, and acidosis. It may develop insidiously, or be a rapidly progressive illness culminating in shock followed by death. The characteristic finding on abdominal radiograph is intramural gas (pneumatosis), produced by bacteria that have invaded the bowel wall. Other radiographic findings include portal gas, persistently dilated loops of bowel and pneumoperitoneum. Immediate management involves stopping enteral feeding, and giving intravenous fluids with broad-spectrum antibiotics. Blood and platelet transfusion may be required. Hypotheses regarding aetiology include the possibility that enteric bacteria ferment maldigested carbohyhdrate creating an acidic intraluminal environment that adversely affects mucosal blood flow. Immaturity of gastrointestinal motor function, digestion, immunity, and circulation are all implicated in the pathogenesis....

Clinical presentations 310Investigation 311Clinical course 311Management 31225% of inflammatory bowel disease presents in childhood, 1/3 as ulcerative colitis. Presentation can occur at any age and ulcerative colitis is the commonest cause of inflammatory bowel disease in the younger child. Family history of Crohn's disease or ulcerative colitis is common in index cases....

Malnutrition 68Suggested core composition of the NST 69Roles of the NST 69There has been a considerable increase in the use of intensive nutritional support (both parenteral and enteral) in the management of children with chronic disorders. In addition, awareness of overt or potential malnutrition among hospital inpatients has increased. The identification of those with (or at risk from) malnutrition, and provision of effective nutritional intervention requires a multidisciplinary team approach since the skills required to deal with the details of assessment, prescription, administration, and monitoring of treatment frequently fall outside the remit of a single practitioner....

Pathophysiology 162Clinical features 162Diagnosis 163Management 164Cystic fibrosis (CF) is an autosomal recessive disease resulting from mutations in the gene coding for the cystic fibrosis transmembrane conductance regulator (CFTR) (see Chapter 21). CFTR functions as a transmembrane chloride channel in the apical membrane of most secretory epithelia and the disease thus affects lungs, pancreas, exocrine glands, gut, and liver. In CF-associated liver disease the biliary tract is most commonly involved in a spectrum from asymptomatic to biliary cirrhosis. The liver disease runs from mild and subclinical to severe cirrhosis and portal hypertension. Clinical disease is seen in 4–6% of cases, but there are biochemical abnormalities in 20–50%. At autopsy, fibrosis is present in 20% and steatosis in 50%....

Definitions 150Epidemiology 150Evaluation 151Treatment 152Complications 153Weight gain in an individual is the result of an energy intake from food in excess of energy expenditure. Unhealthy diets and lack of physical activity are considered to be the leading causes of avoidable illness and premature death in Europe, and the rising prevalence of obesity is a major public health concern. There is a strong tendency for excess weight to continue to accumulate from childhood through to middle age. While 40–70% of the variation in fat mass between individuals may be genetically determined, environmental factors remain crucial. Important lifestyle choices predetermining health risks in adulthood are made during childhood and adolescence. Schools must come to play a key role in promoting healthy diets and enjoyment of physical activity....

Definition 476Aetiology 476Therapy 480Complications 480Transplantation 484Liver support system 484Prognosis 485Acute liver failure (ALF) in children is defined as ‘a rare multisystem disorder in which severe impairment of liver function, with or without encephalopathy, occurs in association with hepatocellular necrosis in a patient with no recognized underlying chronic liver disease’. Liver function is considered severely impaired if prothrombin time (PT) is >15 s or international normalized ratio (INR) is >1.5 and not corrected by vitamin K, in the presence of hepatic encephalopathy (HE) or a PT >20 s or INR >2.0 in the absence of HE....

Infantile haemangiomata 440Mesenchymal hamartoma 441Focal nodular hyperplasia (FNH) 441Nodular regenerative hyperplasia (NRH) 441Hepatoblastoma 442Hepatocellular carcinoma (HCC) 442Inflammatory pseudotumour 443Fibropolycystic liver disease 443Liver tumours in children are rare, accounting for 0.5–2% of all neoplasms in the paediatric age group....

Pathogenesis 267Clinical assessment 269Investigation 270Practical management 271Outcome 276Indications to refer for specialist advice 276Chronic functional constipation is a common problem in childhood. Without early treatment the condition is likely to impact on all aspects of the child's life, including education and psychological well-being as well as physical growth and development....

Definitions (World Allergy Organization) 120Types of allergic reaction 121Diagnosing and managing food allergy 122Food allergy most commonly affects preschool children and is being increasingly recognized. Careful history taking is central to making the diagnosis.•Hypersensitivity: objectively reproducible symptoms or signs initiated by exposure to a defined stimulus at a dose tolerated by normal persons....

Short-bowel syndrome 98Excessive diarrhoea 100Motility disorders 101Mucosal disorders 102The term intestinal failure (IF) refers to a functionally impaired gastrointestinal tract unable to maintain biochemical homeostasis and support normal growth. Short-bowel syndrome (SBS) is a common cause of IF and usually defined as a severe reduction in functional gut mass below the minimal amount necessary for digestion and absorption adequate to satisfy the nutrient and fluid requirements for growth. Other causes of IF include mucosal abnormalities giving rise to protracted diarrhoea, and neuromuscular disorders resulting in chronic idiopathic intestinal pseudo-obstruction syndrome (CIIPS). See ...

Definitions 210Initial assessment 210Examination 212Investigation 212Differential diagnosis 216Treatment 217Overt gastrointestinal bleeding may occur as part of an acute illness or as a feature of chronic disease. Covert or obscure gastrointestinal bleeding may be uncovered in the investigation of anaemia....

Introduction 256Classification 257Personality type and family factors 259Common stresses in children with recurrent abdominal pain 259Therapeutic options 260Outcome 261Recommended clinical approach 261• Recurrent abdominal pain is common in school-aged children and is a frequent presenting complaint in general practice and general paediatric and paediatric gastroenterology clinics. Patients often have vague symptomatology and investigation usually results in a low yield of organic disease. Treatment strategies are varied and often subjective with very little evidence upon which to base them....

Genetics 358Clinical features 358Differential diagnosis 359Investigations 359Management: multidisciplinary approach 360Prognosis 360Alagille syndrome is a complex multisystem disorder with a prevalence of 1:40 000–1:100 000, characterized by hepatic, cardiac, renal, and ocular involvement. At least three of the following five major criteria are required to establish the diagnosis: ...

Assessment 166Differential diagnosis 167Management 167Vomiting is a common symptom of gut disease but can also reflect disease outside the gastrointestinal tract. Vomiting refers to the forceful expulsion of gastric contents. It generally occurs as a consequence of activation of the emetic reflex....

Pyloric stenosis 204Infantile hypertrophic pyloric stenosis is a common cause of vomiting in infancy with an incidence of 2 to 3 per 1000 live births. Males are more commonly affected than females and there is a genetic predisposition. First-born males are more commonly affected....

Indications for parenteral nutrition 87Parenteral nutrition regimens 87Parenteral nutrition products 89Monitoring of parenteral nutrition 93Complications and their management 94Parenteral nutrition (PN) is the supply of nutrients directly into a vein. The first case report of successful long-term PN (in an infant with small-bowel atresia) was published in 1968; since that time products for PN have been developed and refined with the result that metabolic complications are less common, and use in clinical practice has become widespread. For children with short-bowel syndrome, protracted diarrhoea, or pseudo-obstruction PN has become a life-saving intervention. Although it is also widely used in the premature infant with immaturity of gastrointestinal function, the benefit in these patients is less well defined. This is reflected by wide variation in the approach to PN support on different neonatal units. The main indication for PN is when nutritional status cannot be maintained or restored to normal using enteral feeding....

Epidemiology 424Risk of transmission 424Clinical features 424Specific viral tests 425Diagnosis of HCV infection in infants born to HCV +ve mother 425Management 425• Hepatitis C virus (HCV) is an RNA virus of the flaviviride family.• More than 150 million people are infected with HCV worldwide....

Anal fissure 284Perianal streptococcal infection, ‘soggy bottom’ 284Threadworm infestations 285Rectal prolapse 285Solitary rectal ulcer syndrome 285Inflammatory bowel disease 285The perianal examination is an important part of the examination of the gastrointestinal tract. This is best done by inspection with the patient lying in the left lateral position. The perianal region can be inspected by gently parting the buttocks....

General principles 38Parenteral nutrition 39Enteral feeding 40Developments in care for the premature newborn have lead to increasing survival (50% of infants born at 24 weeks gestation) and an increased awareness of the importance of nutrtional support. Many have difficulty tolerating enteral nutrition in the early weeks of life until gastrointestinal motility has matured. Some develop necrotizing enterocolitis (NEC) which carries a high risk of morbidity and mortality, and may be regarded as a failure of adaptation to postnatal life. Optimum nutrition should allow adequate growth in the short term, free of metabolic and other complications, with long-term fulfilment of both genetic growth and developmental potential....

Bottle-feeding 32Type of formula feed 33Complementary feeding 34Although the WHO recommends exclusive breast-feeding for 6 months and partial breast-feeding thereafter, it was recognized that some mothers would be unable, or would choose not to breast-feed, and that these mothers also deserved support to optimize their infant's nutrition. It is important that formula feeds are made up according to instructions and that both formula milk and expressed breast milk is handled in a way that minimizes the risk of bacterial proliferation in the feed. Some simple guidelines are given below: ...

Diagnosis 54Management 55Iron deficiency is the most common nutritional deficiency in the world, affecting around 5 billion people, most of them from developing countries. The prevalence of iron deficiency anaemia in UK preschool children is ~8%, increasing considerably in inner city children; ~9% of under 5s in the USA are thought to be iron deficient. Depletion of iron stores is followed by the development of anaemia, initially with a normal mean cell volume (MCV). Continuing deficiency leads to impairment of erythropoiesis, with hypochromia and microcytosis apparent on blood film. Iron is essential in haemoglobin for oxygen transport, and is also found in myoglobin, and some enzymes (peroxidase, catalase, and cytochromes). Iron from red blood cell breakdown is recycled and excess iron stored as ferritin and haemosiderin....

Factors influencing growth 51Pitfalls 51Management 51Growth faltering or failure to thrive (FTT) is a descriptive term implying failure not only of growth, but also impairment of other aspects of a child's well-being. It is a dynamic process involving a failure to meet expected potential, and there is no universally accepted definition. Weight crossing down two major centile lines is often taken as an indicator of need for referral to a paediatrician. In the absence both of symptoms suggesting specific organ dysfunction (e.g. vomiting, diarrhoea, breathlessness, etc.) and physical findings other than poor growth, an underlying illness is unlikely. It is important to bear in mind common factors influencing growth, such as parental size and ...

Demographics 406Pathophysiology 406Differential diagnoses 407Presenting features 407Investigation 408Management 409Fatty liver disease is now increasingly recognized in children, particularly in the setting of obesity.The term non-alcoholic steatohepatitis (NASH) was first coined in 1980 by Ludwig to describe a pattern of liver injury in adults in which the liver histology was consistent with alcoholic hepatitis, but in whom significant alcohol consumption was denied. NASH can be considered as part of a broader spectrum of non-alcoholic fatty liver disease that extends from simple steatosis through steatohepatitis that is characterized by the potential to progress to fibrosis, cirrhosis and subsequent end stage liver disease....

Subepithelial collagen deposition is a classical feature of collagenous colitis (CC), but is also seen in untreated coeliac disease. The end-stage mediator of excess cellular collagen production is connective tissue growth factor (CTGF). The aim of this study was to investigate CTGF expression by in situ hybridization (ISH) and polymerase chain reaction (PCR) in CC and coeliac disease as well as lymphocytic colitis (LC), Crohn's colitis and ulcerative colitis (UC).For coeliac disease we analysed fresh frozen material by quantitative reverse transcription-polymerase chain reaction (RT-PCR) and archival material for ISH. PCR transcripts in coeliac disease were moderately elevated and labelled cells were significantly increased in the subepithelial zone. For CC, LC and UC we investigated archival material because of the rarity of the first two conditions. There was a marked increase in CTGF expression in the subepithelial zone in CC, localizing to cells with the morphology of smooth muscle cells, which was not seen in LC.The colocalization of CTGF transcripts with areas of excessive collagen deposition in coeliac disease and CC suggest that it might be the end-stage mediator of local fibrosis in these conditions.

Summary Background & aims Little is known about the impact of disease and treatment on the pattern of growth in children with Inflammatory Bowel Disease (IBD). Significant deficits in height and weight in children with Crohn’s disease have been reported but changes in fat and fat free mass are less well defined. This study aims to describe the height, weight and body composition of a cohort of children with IBD. Methods Height, weight, skinfold thicknesses and bioelectrical impedance analysis was performed. Disease activity was assessed with clinical scoring systems. Results 55 children, median age 13.7 years (range 6.5–17.7) were studied. Median (25th, 75th percentile) Standard Deviation Score for BMI, Height and Weight were − 0.3 (− 0.97, 0.65), − 0.56 (− 1.42, 0.06), − 0.62 (− 1.43, 0.19). In Crohn’s disease, using multiple regression analysis disease activity measured by PCDAI was significantly inversely related to fat free mass (β − 0.2, 95% CI −0.17, −0.03, p 0.005). Conclusions Children with IBD were both under and overweight. Nutritional deficits were more common in Crohn’s disease. Fat free mass was related to disease activity in children with Crohn’s disease regardless of changes in weight. Weight or BMI may mask deficits in lean tissue in the presence of normal or increased proportions of body fat.

right © 2010 by From the Department Hospital for Children enterology, Chelsea zDepartment of Pae Hospital, Southampton St George’s Universit Edinburgh, and the D Cardiff, UK. The nutritional supplemen George’s House, Croy Ltd, 100 Wavertree B Supported, in part, by Hepatology, and Nutri and the National Asso The authors report no co Copyright # 2010 by E Hepatology, and Nut Gastroenterology, He DOI: 10.1097/MPG.0b01