OESOPHAGEAL DISORDERS 327 viscous surface layer to reduce re \202 ux); prokinetic drugs, e.g. domperidone; mucosal p rotectors, e.g. sucralfate; corticosteroids (allergic oesophagitis). Surgery: treatment has failed: \200 usually Nissen \200\231s fundoplication is performed when medical Indications for surgery are failed intense medical treatment; oesophageal stricture; Barret t \200\231s esophagus; severe oesophagitis; recurrent apnoea; lower respiratory disease; fa ltering growth (FTT). Complications of surgery include: \200\230gas bloating \200\231 synd rome; dysphagia; profuse retching; \200\230dumping \200\231 syndrome. Prognosis Vast majority of infants outgrow symptoms by 1yr. In older children, 50% develop a chronic, relapsing course. Oesophageal foreign body See also b pp.856 \200\223857. This usually occurs in toddlers or older children with neurological or psychiatric conditions. If the object reaches the sto- mach 90% will pass spontaneously. Con \201 rm position with AP and lateral CXR. Remove end oscopically if: \200 Dysphagia or drooling persists. \200 Object is still in the esopha gus for >12hr. \200 Object is sharp (risk of perforation). \200 Object is hazardous, e. g. mercuric oxide disc batteries. Upper oesophageal dysfunction This disorder is usually due to diffuse CNS dysfunction. \200 \200 Presentation: Diagnosis: manometry. Treatment: myotomy is helpful. choking, cough, drooling, dysphagia, nasal regurgitation. barium swallow with video- \202 uoroscopy or oesophageal treat primary underlying disorder. Rarely, cricopharyngeal Achalasia This rare, idiopathic, condition of obstruction is due to failure of lower oesoph ageal sphincter relaxation. \200 \200 Presentation: Diagnosis: oesophageal manometry. Treatment: Heller \200\231s cardiomyotomy. vomiting, dysphagia with solids or liquids; FTT; aspiration. barium swallow (dilated tapering lower esophagus) or nifedipine (short-term); endoscopic balloon dilatation; Benign oesophageal stricture Causes include severe GORD; caustic ingestion; and radiotherap y. Treatment Treat the underlying cause, e.g. reduce gastric acid production in GORD; perform balloon endoscopic dilatation. 10_McClure_10.indd 327 11/14/2012 11:28:49 PM CHAPTER 10 Gastroenterology and nutrition 328 Pancreatitis Acute pancreatitis This rare disorder consists of acute pancreatic in \202 am mation with variable involvement of local tissues and remote organ systems. Causes \200 \200 \200 e.g. road traf \201 c accident. Blunt abdominal trauma: Viral infection: Multisystem disease: syndrome, IBD, hyperlipidaemi a. Drugs and toxins: Pancreatic duct obstruction: tumors. e.g. mumps, hepatitis A, coxsackie B. e.g. SLE, Kawasaki disease, haemolytic \200\223uraemic \200 \200 e.g. thiopurines, metronidazole, cytotoxic drugs. e.g. cystic \201 brosis, choledochal cyst, Presentation Abdominal pain involving the upper central abdomen, radiating to back, chest, or lower abdomen, vomiting, fever, abdominal tenderness. Severe cases also exhibit: hypotension; abdominal distension; Cullen \200\231s or gray \200 \223Turner \200\231s sign (bruising of peri-umbilical area and \202 anks, respectively); ascites; pleural effusion; jaundice; multi-organ failure. \200 \200 \200 \200 \200 Investigations \200 Blood: \200 Radiology: cholangiopancreatography (ERCP) if structura l or obstructive cause. amylase (ii); lipase (i); Ca2+ (d); ESR/CRP (i); deranged LFT. abdominal US or CT; endoscopic retrograde Treatment Mild \200 \200 Supportive only, e.g. NGT, analgesia. Start short period of nil by mouth to \200\230rest \200\231 pancreas. Severe Treat as for the mild form, plus: \200 Admit to intensive care unit. \200 Correc t hypotension. \200 Treat multi-organ failure. \200 Surgery if signi \201 cant pancrea tic necrosis, major ductal rupture (trauma), gallstones (cholecystectomy), presence of pseu docyst. Endoscopy (ERCP) may be therapeutic if structural obstructive cause. 10_McClure_10.indd 328 11/14/2012 11:28:49 PM PANCREATITIS 329 Prognosis \200 \200 Complete recovery is likely if there is minimal organ dysfunction. 20% mortality if there i s severe disease or organ failure present, or if local complications develop (e.g. pancreat ic pseudocyst). Most children have only a single acute episode. Chronic pancreatitis This very rare condition follows acute pancreatitis with continuing in \202 am- mation, destruction of pancreatic tissue, and \201 brosis, leading to perma- n ent exocrine or endocrine pancreatic failure. It is usually caused by cystic \201 brosis, congenital ductal anomalies, sclerosing cholangitis (IBD), hyper- lipidaemia, or hypercalc aemia. Presentation \200 \200 Repeated episodes of acute pancreatitis are separated by good health. Eventually, features of pancreatic exocrine failure or diabetes mellitus become apparent. Investigations \200 \200 Abdominal US or CT scan con \201 rms chronic pancreatitis. Pancreatic function tests may b e useful, e.g. stool chymotrypsin ( pancreozymin-secretin test, 72hr fecal fat measurement (i). i), Treatment \200 \200 Treat acute exacerbations as for acute pancreatitis. Give pancreatic enzyme replacement and nutritional supplementation (well-balanced diet with moderated fat intake and fat soluble vitamins \200\224involve a pediatric dietician). Relieve any ductal obstruction by endoscop y or surgery. Prognosis Recovery or risk of developing long-term pancreatic exocrine and/or endocrine fai lure is dependent on cause and severity. 10_McClure_10.indd 329 11/14/2012 11:28:49 PM CHAPTER 10 Gastroenterology and nutrition 330 Intestinal disorders Gastritis and peptic ulcer disease This disease is rare in children. I t most commonly affects the duodenum. Causes \200 Helicobactor increased risk of adult gastric cancer). Stress ulcers, e.g. post-trauma, HIE. Drug-related, e.g. NSAIDs. Increased acid secretion (Zollinger \200\223Ellison syndrome, m ultiple endocrine neoplasia type I, hyperparathyroidism). Crohn \200\231s disease. Eosinoph ilic gastroenteritis. Hypertrophic gastritis. Autoimmune gastritis. pylori infection (strong familial link, associated with \200 \200 \200 \200 \200 Presentation \200 \200 \200 \200 \200 \200 \200 Often asymptomatic. Chronic abdominal and epigastric pain. Nausea +/ \200\223 vomiting. GI hemorrhage. FTT +/ \200\223 anorexia. Iron de \201 ciency anemia. Perforation (very rare). Investigation C \200 \200 14 urea breath test (H. pylori). Upper GI endoscopy and biopsy ( H. pylori histology and culture). Treatment \200 Treat underlying cause, e.g. eradicate amoxycillin (clarithromycin), bismuth, metronidazole +/ \200\223 omeprazole (quadruple therapy). H. pylori with 7 \200\22310 days oral d \200 gastric acid production, e.g. proton pump inhibitors (PPI), H2 antagonists; sucralfate (cytoprotective). Antacids, e.g. aluminum hydroxide. Protein-losing enteropathy This disorder is characterized by chronic intestinal protein los s. Causes \200 \200 \200 \200 \200 \200 GI infection, e.g. giardiasis. Severe food hypersensitivity. Coeliac disease or IBD. Severe cardiac failure. SLE; graft vs. host disease. Polyposis or lymphatic obstruction. Presentation There is hypoalbuminaemia +/ \200\223 diarrhea or abdominal pain. Increased fe cal A 1-antitrypsin con \201 rms the condition. 10_McClure_10.indd 330 11/14/2012 11:28:49 PM INTESTINAL DISORDERS 331 Treatment Treat the underlying disease; give nutritonal support and albu- min infusions as required. Short bowel syndrome Is due to severe intestinal disease or the surgical removal of a large por- tion of the small intestine. The condition manifests as malabsorption, \202 uid and electrolyte loss, and malnutrition. Presentation \200 \200 \200 \200 \200 \200 \200 Diarrhea, steatorrhoea. FTT. Dehydration, electrolyte loss (Na, K, Mg, Ca). Cholestasis (bi le salt loss). Peptic ulcer disease (due to increased gastrin). Speci \201 c (e.g. vitamin B Renal stones (oxalate). 12) +/ \200\223 generalized malnutritional disorders. Treatment \200 \200 \200 \200 \200 \200 \200 \200 \200 Correct \202 uid and electrolyte disturbance. Speci \201 c nutritional supplements; hydr olysed protein/elemental diets. PN. Gastric acid reducing drugs, e.g. PPI or H Anti-diarrho eal drugs, e.g. loperamide. Cholestyramine (chelates bile salts). Parenteral somatostatin. Oral antibiotics to reduce bacterial overgrowth. Surgery to reduce GI motility or small bow el transplant. 2 antagonists. Prognosis is improving, with 90% 5yr survival. Retention of ileo-caecal valve signi \201 c antly improves prognosis. Intestinal polyps Most juvenile polyps are hamartomas, single, and located in the distal co lon. Polyposis (multiple polyps) syndromes include: \200 \200 \200 Peutz \200\223Jegher \200\231s syndrome (mucocutaneous pigmentation). Familial polyposis co li. Gardner \200\231s syndrome (GI polyps, osteomas and soft tissue tumors). Presentation \200 \200 \200 \200 \200 \200 Often asymptomatic. Haematochezia. Rectal polyp prolapse. Protein-losing enteropathy. Intus susception. Mucoid diarrhea. Investigation Gastroscopy and ileo-colonoscopy, barium radiology. Treatment \200 \200 Endoscopic or surgical removal. Periodic colonoscopy surveillance is required in polyposis syndromes because of signi \201 cant risk of neoplasia. 10_McClure_10.indd 331 11/14/2012 11:28:49 PM CHAPTER 10 Gastroenterology and nutrition 332 In \202 ammatory bowel disease Includes Crohn \200\231s disease (CD) and ulcerative coliti s (UC). UK incidence is 75.2/100,000/yr. CD is twice as common as UC. The cause is unknown, although there is a recognized genetic disposition. Ulcerative colitis \200 \200 Involves colon only. Rectal (proctitis) is most common or may extend continuously up to inv olving the entire colon (pancolitis). Terminal ileum may be affected by \200\230backwash i leitis \200\231. Crohn \200\231s disease \200 May affect any part of GI tract, but terminal ileum and proximal colon are commonest sites of involvement. Unlike UC, bowel involvement is non-continuous ( \200\230skip \200\231 lesi ons). Presentation Symptoms \200 \200 \200 \200 Anorexia, weight loss, lethargy. Abdominal cramps. Diarrhea +/ \200\223 blood/mucus, urgenc y and tenesmus (proctitis). Fever. GI signs \200 \200 \200 \200 Aphthous oral ulcers. Abdominal tenderness. Abdominal distension (UC > CD), right iliac fos sa (RIF) mass (CD). Peri-anal disease (CD), i.e. abscess, sinus, \201 stula, skin tags, \201 ssure, stricture. Non-GI signs and associations \200 \200 \200 \200 \200 \200 \200 \200 \200 \200 \200 Fever. Finger clubbing. Anemia. Skin: Joints: Eyes: Poor growth. Delayed puberty. Sclerosin g cholangitis. Renal stones. Nutritional de \201 ciencies, e.g. vitamin B erythema nodosum; pyoderma gangrenosum. arthritis; ankylosing spondylitis. iritis; conjunct ivitis; episcleritis. I2. Complications \200 \200 \200 \200 \200 \200 \200 \200\230Toxic \200\231 colon dilatation (UC > CD). GI perforation or strictures. Pseudopol yps (apparent \200\234polyps \200\235 resulting from in \202 ammation). Massive GI hemorr hage. Colon carcinoma (UC: 50% risk after 10 \200\22320yrs disease). Fistula involving bowe l only or bowel and skin, vagina, or bladder (CD). Abscesses (CD). 10_McClure_10.indd 332 11/14/2012 11:28:49 PM INFLAMMATORY BOWEL DISEASE 333 Investigations \200 FBC; ESR/CRP (i); U&E; LFT; albumin (d); blood culture; serum Blood: iron (d); vitamin B12 and folate (d). Serum serological markers: ASCA (anti-Saccharo myces cerevisiae antibodies, better for CD); p-ANCA (perinuclear antineutrophil cytoplasmic antibody, better for UC). Stool colonoscopy to determine extent and pattern of abnormal En doscopy: mucosa and intestinal biopsy (UC histology: crypt abscesses, mucosal in \202 amma tion only, goblet cell depletion; CD: crypt abscesses granulomas, transmural in \202 ammat ion); upper GI endoscopy (CD). Radiology: barium radiology/ultrasound (CD: mucosal \200 \230cobblestone \200\231 appearance, ulceration, dilatation, narrowed segments, \201 stul a, \200\230skip \200\231 lesions; UC: mucosal ulceration, haustration loss, colonic narrow ing +/ \200\223 shortening). \200 M,C&S: (infectious colitis can mimic CD/UC). Treatment Supportive treatment If severe, e.g. bowel rest, IV hydration, PN. Drug treatment \200 oral 5-aminosalicylic acid (ASA) dimers, Mild to moderate disease: e.g. mesalazine, may be useful to induce and maintain colonic dis ease remission in UC. ASA or corticosteroid enemas are effective for treating rectal diseas e. Dietary treatment may be useful to induce remission (see Dietary treatment). Moderate to severe disease: or IV methylprednisolone, 1 \200\2232mg/kg/day until condition improved (< 2wks) then wean over 6 \200\2238wks. Antibiotics: Maintenance treatment, or to treat resist ant active disease: immunomodi \201 ers, e.g. azathioprine, ciclosporin, tacrolimus, metho trexate, or in \202 iximab (anti-TNF antibody). induce remission with oral prednisolone \200 e.g. cipro \202 oxacin or metronidazole, may also be useful. Dietary treatment Polymeric/elemental diets are useful to induce remission (CD>UC), but the relapse rate is high. Dietary supplementation often required to mini- mize poor growth and correct speci \201 c nutritional de \201 ciencies, e.g. vitamin and mineral supplements. Involve a pediatric dietitian. Surgery \200 UC: total colectomy and ileostomy, and later pouch creation and anal anastom osis, cures UC. There is 10 \200\22320% complication rate, e.g. pouchitis. CD: \201 stula , may be indicated, but there is a high re-operation rate as in \202 ammation recurrence i s universal. local surgical resection for severe localized disease, e.g. strictures, Prognosis UC and CD are marked by relapse and remission. Patients can have very good qualit y of life with current therapy. Poor prognostic factors include extensive disease, frequent remissions, and young age at diagnosis. 10_McClure_10.indd 333 11/14/2012 11:28:49 PM CHAPTER 10 Gastroenterology and nutrition 334 Malabsorption De \201 ned as subnormal intestinal absorption of dietary constituents with excessive fecal nutrient loss. The prognosis depends on the cause. Reduced adult height, te eth enamel defects, and osteoporosis may result from long-term malabsorption. Causes are li sted in Box 10.5. Presentation \200 Diarrhea. \200 Steatorrhoea. \200 Flatulence. \200 FTT/weight los s. \200 Muscle wasting. \200 Abdominal distension. \200 Peri-anal excoriation. \200 Delayed puberty. \200 Features of underlying illness, e.g. abdominal pain in Crohn \200 \231s disease. \200 Signs of nutritional de \201 ciency states, e.g. ascites due to hypo albuminaemia. Investigations Initial screening tests should include: FBC; U&E; creatinine; albumin; total 3 \200\223 ; LFT; iron status, coeliac antibody screen; coagulation protein; Ca2+; PO4 scr een, stool M,C&S. If diagnosis still unclear, consider: Upper GI endoscopy with biopsy to look for an enterop athy, ileo- colonoscopy if features suggest colitis (ensure clotting screen normal before p rocedure). Sweat test. Immune function tests. Fecal fat measurement. Fecal elastase. A Feca l Exocrine pancreatic function tests. \200 \200 \200 \200 \200 1-antitrypsin. Treatment \200 Treat underlying disease, e.g. metronidazole for giardiasis, gluten-free diet for coeliac d isease. Supplemental digestive enzymes, e.g. pancreatic enzymes in cystic \201 brosis. Nu tritional supplements to correct de \201 ciencies. PN if malabsorption severe or slow to r ecover. \200 10_McClure_10.indd 334 11/14/2012 11:28:49 PM MALABSORPTION 335 Box 10.5 Causes of malabsorption Intraluminal digestive defect \200 \200 \200 \200 \200 \200 \200 \200 Carbohydrate intolerance (most commonly lactose intolerance) Protein \200\223energy malnutr ition Cystic \201 brosis Shwachman \200\223Diamond syndrome ( Chronic pancreatitis Choles tasis Pernicious anemia Speci \201 c digestive enzyme de \201 ciency, e.g. lipase b p.627) Mucosal abnormality \200 Coeliac disease \200 Short bowel syndrome \200 Dietary prote in intolerance, e.g. milk protein allergy \200 Intestinal infection or parasites, e.g. gi ardiasis IBD \200 \200 Abetalipoproteinaemia (disorder of lipid metabolism \200\224FTT, steatorrhoea, progressive ataxia, retinitis pigmentosa, acanthocytes on FBC) Protein \200\223energy malnutrition; int estinal venous or lymphatic obstruction, e.g. congestive cardiac failure, intestinal lympha ngiectasia Miscellaneous \200 \200 \200 Immunode \201 ciency syndromes, e.g. HIV Drug reaction, e.g. cytotoxics, post-radiation Ba cterial overgrowth, e.g. pseudo-obstruction 10_McClure_10.indd 335 11/14/2012 11:28:49 PM CHAPTER 10 Gastroenterology and nutrition 336 Coeliac disease Coeliac disease is an enteropathy due to lifelong intolerance to gluten pro - tein (present in wheat, barley, rye, and oats by cross contamination). Prevalence is approximately 1% when populations are screened. It is asso- ciated with: \200 \200 \200 \200 A positive family history. Type 1 diabetes. Down syndrome. IgA de \201 ciency. Presentation The condition may present at any age after starting solids containing glu- ten . The \200\230classic \200\231 initial features include: Pallor. Diarrhea. Pale, bulky \202 oating stools. Anorexia. FTT. Irritability. \200 \200 \200 \200 \200 Later, there is: \200 Apathy. \200 Gross motor developmental delay. \200 Ascites. \200 Peripheral oedema. \200 Anemia. \200 Delayed puberty. \200 Arthralgia. \200 H ypotonia, muscle wasting. \200 Speci \201 c nutritional disorders. Increased recognition, and the widespread practice of antibody screening of children at hig h risk, has changed considerably the clinical spectrum of cases seen, with less classical a nd severe symptoms now more common at time of initial diagnosis. There are three settings in which the diagnosis of coeliac disease should be considered and screened for: \200 \200 \200 Children with frank gut symptoms. Children with the non-gastrointestinal manifestations des cribed here. Asymptomatic individuals with conditions that are associated with coeliac dise ase. Coeliac crisis Life-threatening dehydration due to diarrhea accompanying malabsorp- tion. T his condition is now very rare except in the less-developed world. 10_McClure_10.indd 336 11/14/2012 11:28:50 PM COELIAC DISEASE 337 Investigations \200 Measurement of serum tissue transglutaminase IgA antibody (TTG) is recommended for initial testing for coeliac disease. IgA sensitivity and speci \201 city approach 100%, although f alse positives are occasionally seen. Anti-endomysium IgA antibody is observer-dependent an d expensive. Anti-gliadin antibody tests are less accurate and are now not advised. It is i mportant to exclude IgA de \201 ciency as a cause of falsely negative serology. Endoscopic small bowel biopsy of the third part of the duodenum shows diffuse, subtotal villus atroph y, increased intraepithelial lymphocytes, and crypt hyperplasia. The villi return to normal on a gluten-free diet. Most clinicians consider positive mucosal histology and full clinical recov- ery on gluten- free diet +/ \200\223 positive IgA antibodies suf \201 cient to make a diagnosis. Antibody levels should return to normal on treatment and nega- tive serology is a marker of complia nce. Avoid gluten challenge (>10g oral gluten per day for 3 \200\2234mths and re-biopsy) un less diagnosis is in doubt, e.g. initial biopsy is inadequate or not typical, or alternativ e diagnosis is possible, e.g. transient gluten intolerance may occur after gastroenteritis, giardiasis, or cow \200\231s milk protein intolerance. Treatment \200 \200 \200 \200 Gluten-free diet under the supervision of a pediatric dietitian. Gluten-free foods are pres cribable in UK. Gluten avoidance should be life-long if coeliac disease is con \201 rmed. Nutritional supplements may be required. Prognosis Excellent if patient is compliant with strict, life-long gluten-free diet. There is a possible increased risk of intestinal lymphoma if gluten is ingested, even in asymptom atic coeliac disease. 10_McClure_10.indd 337 11/14/2012 11:28:50 PM CHAPTER 10 Gastroenterology and nutrition 338 Gastrointestinal infections GI infections are the second commonest cause of primary care co nsultation after the common cold. These infections also cause over 3 million children death s per year (mostly in developing world) (see also b pp.1022 \200\2231023). Viral gastroenteritis Transmission is by the fecal \200\223oral route, including contaminat ed water. Epidemics are frequent and usually occur during winter. Breastfeeding is protecti ve. Severity is increased in malnourished children. Causes \200 \200 Rotavirus (most common). Small round structural virus, e.g. winter vomiting disease caused by \200\230Norwalk agent \200\231. Enteric adenovirus. Astrovirus. CMV (in immune-comprise d patients). \200 \200 Presentation \200 \200 \200 \200 \200 \200 \200 \200 Investigation Is rarel y necessary (see b p.302). Stool electron micros- copy or immunoassay can sometimes be usef ul. Watery diarrhea (rarely bloody). Vomiting. Cramping abdominal pain. Fever. Dehydration. Ele ctrolyte disturbance. Upper respiratory tract signs common with rotavirus. Vomiting predomi nates with Norwalk virus. Treatment Give supportive rehydration orally or with a nasogastric tube, or IV glu- cose an d electrolyte solution. Hospitalization is rarely needed (e.g. (cid:2)10% dehydration, or u nable to tolerate oral \202 uids). Prognosis Symptoms generally last <7 days, except in enteric adenovirus, when diar- rhoea f requently goes on beyond 14 days. The child may develop tempo- rary secondary lactose intol erance. Prevention Rotavirus immunization is now available and effective. 10_McClure_10.indd 338 11/14/2012 11:28:50 PM GASTROINTESTINAL INFECTIONS 339 Bacterial gastroenteritis Causes secretory and in \202 ammatory diarrhea. It is most commo n under 2yrs of age. Commonest causative organisms include: \200 \200 \200 \200 \200 \200 \200 \200 Salmonella Campylobacter jejuni ; Shigella spp.; Yersinia enterocolitica ; ; Escherichia co li Clostridium dif \201 cile ; Bacillus cereus ; . Vibrio cholerae spp.; Sources of infection include contaminated water, poor food hygiene (meat, fresh produce, ch icken, eggs, previously cooked rice), fecal \200\223oral route. Presentation As for viral gastroenteritis plus: \200 \200 \200 \200 malaise; dysentery (bloody and mucous diarrhea); abdominal pain may mimic appendicitis or I BD; tenesmus. Complications \200 Bacteraemia. \200 Secondary infections (particularly pneumonia, oste omyelitis, meningitis. Reiter \200\231s syndrome ( Haemolytic \200\223uraemic syndrome ( Gu illain \200\223Barr syndrome ( Reactive arthropathy ( Haemorrhagic colitis. Salmonella, Campylobacter), e.g. \200 \200 \200 \200 Shigella, Campylobacter). E. coli 0157, Shigella). Campylobacter). Yersinia). Investigation \200 Stool +/ \200\223 blood culture (some organisms need speci \201 c culture medium). Stool S igmoidoscopy if in \202 ammatory bowel disease or colitis. \200 Clostridium dif \201 cile toxin. Treatment \200 \200 Rehydration as for viral gastroenteritis. Antibiotics are not indicated, as the duration of symptoms is not altered and may increase chronic carrier status, unless there is high risk of disseminated disease, presence of arti \201 cial implants (e.g. V-P shunt), severe col itis, severe systemic illness, age <6mths, enteric fever, cholera or E. coli 0157. Most org anisms are sensitive to ampicillin, co-trimoxazole, or third generation cephalosporins. Con sider: \200 \200 Campylobacter; erythromycin if oral vancomycin or metronidazole if pseudomembranous colitis). Clostridium dif \201 cile (causes 10_McClure_10.indd 339 11/14/2012 11:28:50 PM CHAPTER 10 Gastroenterology and nutrition 340 Intestinal parasites Infection is usually via the fecal \200\223oral route. Pets and livest ock can be hosts. Parasitic infection can mimic IBD (b p.332), hepatitis (b p.342), sclero- sing cholangitis, peptic ulcer disease, and coeliac disease (b p.336). Presentation \200 \200 \200 \200 \200 \200 \200 \200 Abdominal pain. Diarrhea; dysentery; \202 atulence. Malabsorption and FTT. Abdominal dist ension. Intestinal obstruction. Biliary obstruction; liver disease. Pancreatitis. Fever. Investigations \200 \200 \200 \200 \200 \200 Stool M,C&S for ova, cysts, parasites, and leukocytes. Speci \201 c stool staining for cry ptosporidiosis. Stool ELISA for giardiasis and cryptosporidiosis. Blood speci \201 c serol ogy, e.g. Duodenal \202 uid aspiration for M,C&S. Duodenal villus biopsy, e.g. giardiasis . Entamoeba histolytica. Protozoa Giardia lamblia \200 \200 Very common. Swallowed cysts develop into trophozoites that attach to the small intestinal villi, causing mucosal damage. Presentation \200 \200 Diarrhea, \202 atulence, abdominal discomfort. Sometimes FTT. Treatment Metronidazole. Entamoeba histolytica Symptoms are usually mild, but may cause: \200 \200 \200 \200 Fulminating colitis (amoebic dystentery can mimic ulcerative colitis). Intestinal obstructi on due to chronic localized lesion (an \200\230amoeboma \200\231). Amoebic hepatitis. Live r abscess (right upper quadrant pain, fever, hepatomegaly). Treatment Metronidazole. Cryptosporidium This organism causes a mild self-limiting illness except in immune-compro- mised patients, where it can cause: \200 \200 \200 \200 Severe chronic watery diarrhea, \202 atulence. Malaise. Abdominal pain. Weight loss. 10_McClure_10.indd 340 11/14/2012 11:28:50 PM INTESTINAL PARASITES 341 Treatment Erythromycin, metronidazole, or spiramycin. Nematodes Ascaris lumbricoides The most common parasitic worm infection in humans, with up to 25% of the world \200\231s population infected (rare in industrialized countries). They look like earthworms and can cause Loef \202 er \200\231s syndrome (an eosino- philic pneu monia, that can mimic asthma, also caused by the parasites Strongyloides stercoralis, and t he hookworms Ancylostoma duodenale and Necator americanus. Heavy infestation can cause spec i \201 c nutritional de \201 ciencies or bowel obstruction. Infection occurs by fecal-ora l trans- mission of eggs. Treatment Mebendazole, albendazole, pyrantel pamoate. Trichuris trichiura (whip worm) Lives in the colon and causes diarrhea, abdominal pain, and weight loss. Treatment Mebendazole or albendazole. Hookworms (Necator americanus, Ancylostoma duodenale) Infection is by larvae penetrating th e skin, e.g. bare feet. The adult worms live in the intestine voraciously sucking blood lea ding to anemia and hy- poproteinaemia. Treatment Mebendazole. Strongyloides stercoralis \200 Penetrates the skin and migrates to the lungs. Then coughed up and ingested into the gut. C auses bloating, heartburn, and malabsorption. Treatment Mebendazole, albendazole, or thiabendazole. Enterobius vermicularis (thread or pinworm) \200 Very common and causes anal pruritis as females emerge and lay eggs in peri-anal region. In fection: Diagnosis: area or in stool, or microscopy of sellotape previously applied to the anus. \200 occurs by fecal-oral transmission of eggs. is con \201 rmed by direct visualization of wor ms on peri-anal Treatment Mebendazole. Cestodes (tapeworms) \200 Infection: results from ingesting undercooked contaminated pork (Taenia solium), beef (Taen ia saginata), or \201 sh (Diphyllobothrium latum). is by microscopy of eggs or proglottid es in stool. Diagnosis: Treatment Praziquantel. 10_McClure_10.indd 341 11/14/2012 11:28:50 PM CHAPTER 10 Gastroenterology and nutrition 342 Acute hepatitis Viral causes Hepatitis A (HAV) incubation 2 \200\2236wks, fecal \200\223ora l transmission. Hepatitis B (HBV) incubation 6wks to 6mths. Endemic in the Far East and Africa. Infection m ay be transmitted from: \200 \200 \200 \200 \200 Blood products. IV drug abuse, contaminated needles, or syringes. Sexual intercourse. Close direct contact (e.g. intrafamilial, health workers). Vertical (may cause fulminant hepatit is). Hepatitis C (HCV) incubation 2wks to 6mths. Transmission is as for HBV. Usually causes a mi ld severity acute illness or is asymptomatic. HCV rarely causes acute hepatitis. Hepatitis E fecal \200\223oral transmission, endemic in India. Hepatitis D requires previous HBV infection. Hepatitis G parenteral transmission. Other organisms can cause hepatitis as part of systemic infection: Epstein \200\223 Barr vi rus (EBV, common in adolescents, only 40% have hepatitis); TORCH organisms (neonatal hepati tis); HIV; CMV (immune-compromised); Listeria. Other causes \200 \200 \200 \200 Poisons Metabolic Autoimmune Reye \200\231s aspirin therapy and microvesicular fatty in \201 ltration of the liver. \200 and drugs: e.g. paracetamol, isoniazid, halothane. disease: e.g. Wilson \200\231s disease, tyrosinaemia type I. hepatitis: May present with ac ute hepatitis. syndrome: a rare, acute encephalopathic illness associated with Prodrome: 2 \200\2233 days before onset of jaundice or abnormal LFT (see also b pp.343, 960 , 970). (nausea, vomiting, hypoglycaemia, abdominal pain) occurs Presentation Acute fulminant hepatic failure (encephalopathy and coagulopathy) may rarely o ccur. Many infections are asymptomatic, particularly HAV and HCV. There are many presentati ons which include: \200 \200 \200 \200 \200 \200 \200 \200 \200 \200 \200 fever; fatigue; malaise; anorexia; nausea; arthralgia; right upper quadrant abdominal pain; jaundice +/ \200\223 hepatomegaly; splenomegaly; adenopathy; urticaria. 10_McClure_10.indd 342 11/14/2012 11:28:50 PM ACUTE HEPATITIS 343 Investigations \200 d \200 \200 i bilirubin >20mg/L; i AST/ALT ( \227 2 \200\223100). Blood glucose (especially in Reye \200\231s syndrome). LFT: Viral serology (IgM antibodies), viral PCR (HCV), EBV heterophil antibodies (Monospot or Pa ul \200\223Bunnell). Blood culture if appropriate. Paracetamol level or halothane antibodie s, if relevant. Serum immunoglobulin, complement (C3, C4), positive autoimmune antibodies ( anti-smooth muscle, anti-mitochondrial, and/or anti-liver and kidney microsomal) in autoimm une hepatitis. Serum copper/caeruloplasmin, 24hr urinary copper (Wilson \200\231s disease). Urinary succinylacetone (tyrosinaemia type I). \200 \200 Management Usually none is required, except support and rest. \200 \200 Alcohol avoidance in teenagers. There is no place for antivirals unless the child is immune - compromised. Fulminant hepatitis requires referral to a specialist unit for intensive car e management and possible liver transplantation. Reye \200\231s syndrome: provide intensive care support. maintain blood glucose >4mmol/L; prevent sepsis; Prognosis \200 \200 Acute hepatitis is usually self-limiting. 730% if both cerebral oedema Mortality after fulm inant hepatitis is and renal failure are absent, 770% if both are present without liver tra nsplant. There is a long-term risk of: \200 \200 \200 \200 785%); chronic hepatitis (HAV 0%; HBV 5 \200\22310%; HCV cirrhosis; hepatocellular carcinoma (HBV and HCV); glomerulonephritis (circulating immune-complexes). Prevention Active immunization exists for both HAV and HBV. Within 24hr after an infectious contact, infection may be prevented by giving pooled serum immunoglobulin for HAV and CMV, or speci \201 c HBV serum immunoglob- ulin for HBV. 10_McClure_10.indd 343 11/14/2012 11:28:50 PM CHAPTER 10 Gastroenterology and nutrition 344 Chronic liver failure Causes \200 \200 \200 A \200 \200 \200 \200 \200 \200 \200 \200 \200 Chronic hepatitis (after viral hepatitis B or C). Biliary tree disease, e.g. biliary atresi a. Toxin-induced, e.g. paracetamol, alcohol. 1-antitrypsin de \201 ciency. Autoimmune hepatitis. Wilson \200\231s disease (age >3yrs). Cystic \201 brosis. Alagille syndrome or non-syndromic paucity of bile ducts. Tyrosinaemia. Primary sclerosing cholangit is. PN-induced. Budd \200\223Chiari syndrome. Presentation \200 \200 \200 \200 \200 \200 \200 \200 \200 \200 \200 \200 Jaundice (not always). GI hemorrhage (portal hypertension and variceal bleeding). Pruritis. FTT. Anemia. Enlarged hard liver (though liver often small in cirrhosis). Non-tender splen omegaly. Hepatic stigmata, e.g. spider naevi. Peripheral oedema and/or ascites. Nutritional disorders, e.g. rickets. Developmental delay or deterioration in school performance. Chron ic encephalopathy. Investigations Blood tests \200 LFT ( \200 FBC ( \200 Coagulation (prothrombin time d \200 or n blood glucose. \200 U&E ( rickets). Viral serology or PCR for hepatitis B and C. IgG, d complement (C3, C4), autoimmune antibodies (see b p.343). i or n bilirubin, i AST/ALT (x 2 \200\22310), albumin <35g/L). d Hb if GI bleeding); d WCC and platelets (hypersplenism). i if vitamin K de \201 ciency). d Na+, d Ca2+, i PO4 3 \200\223, i alkaline phosphatase if biochemical i \200 Metabolic studies \200 d \200 i \200 A Serum copper and caeruloplasmin (Wilson \200\231s disease). 24hr urinary copper (Wilson \200\231s disease). 1-antitrypsin level and phenotype. Sweat test (cystic \201 brosis); Abdominal US \200 \200 \200 \200 Hepatomegaly. Echogenic liver. Splenomegaly. Ascites. 10_McClure_10.indd 344 11/14/2012 11:28:50 PM CHRONIC LIVER FAILURE 345 Upper GI endoscopy \200 \200 Oesophageal or gastric varices. Portal hypertension related gastritis. EEG To con \201 rm chronic encephalopathy if suspected. Liver biopsy Histology; enzymes; electron microscopy. Management \200 \200 \200 Treat the underlying cause and give nutritional support. Lower protein, increased energy, h igher carbohydrate diet. Vitamin supplementation, particularly fat soluble vitamins A, D, E , K. Involve a pediatric dietitian. Drug therapy \200 \200 \200 \200 \200 Prednisolone +/ \200\223 azathioprine for autoimmune hepatitis. Interferon- Penicillamine f or Wilson \200\231s disease. Colestyramine may be useful to control severe pruritis. Vitami n K A +/ \200\223 ribavirin for chronic viral hepatitis. 1 and FFP (10mL/kg) if signi \201 cant coagulopathy or bleeding. Oesophageal varices Endoscopy, i.e. sclerotherapy or surgery. Ascites \200 + restriction (2/3 maintenance and 1mmol/kg/day, Fluid and Na respectively). Spironolactone (1 \200\2232mg/kg 12-hourly). Consider IV 20% al bumin if ascites is resistant to above treatment. \200 Encephalopathy Reduce GI ammonia absorption using oral or rectal lactu- lose, neomycin, or soluble \201 bre pectin. Liver transplantation See b p.347. Prognosis There is up to 50% 5yr mortality without liver transplant. Poor prognostic factor s are: \200 \200 \200 \200 \200 \200 bilirubin >50 mol/L; albumin <30g/L; PT >6s; ascites; encephalopathy; malnutrition. 10_McClure_10.indd 345 11/14/2012 11:28:50 PM CHAPTER 10 Gastroenterology and nutrition 346 Alpha1-antitrypsin de \201 ciency Alpha1-antitrypsin is a serum protease inhibitor respons ible for control- ling in \202 ammatory cascades. \200 It is the commonest genetic cause of liver disease in children, with autosomal dominant inh eritance. Prevalence is 1:2000 to 1:7000. Genetic variants are identi \201 ed by enzyme el ectrophoretic mobility as medium (M), slow (S), or very slow (Z). S is associated with 760% Alpha1-antitrypsin level of normal; Z 715%. Normal genotype is designated PiMM. Only PiZZ individuals are at risk of liver disease. Presentation \200 \200 Cholestasis in infancy, may progress to liver failure. Cirrhosis can occur in late childhoo d to adult. Chronic liver disease affects 25% of patients in late adulthood. Pulmonary emph ysema is the commonest presentation in adulthood. Diagnosis \200 1-antitrypsin level d. A Serum \200 Phenotyping by enzyme isoelectric fo cusing (see Alpha de \201 ciency). 1-antitrypsin Treatment \200 \200 \200 Supportive treatment of liver complications. Strongly advise against smoking. Liver transpl ant for end-stage liver failure. Wilson \200\231s disease A rare autosomal recessive disorder leading to toxic accumulation of cop- per in the liver and, subsequently, other tissues especially the brain and eye. See also b p.976. Presentation \200 Kayser \200\223Fleischer rings (copper deposition in Descemet \200\231s membrane of the eye ) often present (45% with hepatic presentation and 90% with neurological) and are pathognom onic. May require slit-lamp examination to visualize. Hepatic problems usually present in c hildhood (hepatitis, cirrhosis, fulminant hepatic failure). Adolescents/young adults usuall y present with neurological disease. Investigations \200 \200 d. Serum copper and caeruloplasmin 24hr urinary copper excretion >100microgram (normal <40micr ogram). Molecular genetic testing \200\224Wilson \200\231s disease gene ( ATP7B) mutation. Treatment \200 Lifelong chelation therapy with penicillamine (reverses pre-cirrhotic liver disease, but no t neurological damage). Liver transplantation if end-stage hepatic failure. 10_McClure_10.indd 346 11/14/2012 11:28:50 PM LIVER TRANSPLANTATION 347 Liver transplantation Indications for liver transplantation The commonest underlying condit ions leading to irreversible liver failure and transplant are: \200 \200 \200 \200 Fulminant hepatic failure: Biliary atresia. Chronic end-stage liver disease: Liver based me tabolic conditions: de \201 ciency, Crigler \200\223Najjar syndrome, tyrosinaemia. Acute l iver failure following a liver transplant: of transplant or hepatic artery thrombosis. Neon atal hepatitis. Autoimmune hepatitis. Unresectable tumor con \201 ned to the liver: e.g. viral, toxic, Wilson \200\231s disease. e.g. post-viral hepatitis with cirrhosis. e.g. Wilson \200\231s disease, 1-antitrypsin e.g. primary non-function \200 \200 e.g. hepatoblastoma. Clinical features requiring consideration for transplantation \200 \200 \200 \200 \200 \200 \200 Bleeding varices due to portal hypertension. Failure of growth or development. Resistant as cites. Hepatic encephalopathy. Poor quality of life: Coagulopathy (PTT >2 Multi-organ failu re: syndrome. e.g. pruritis, lethargy. x normal). e.g. hepatorenal syndrome, hepatopulmonary Preparation for transplant Requires multidisciplinary evaluation to include the following: \200 \200 \200 \200 \200 \200 Nutritional support. Development and psychological assessment of child and family. Educatio n and counseling. Ensure vaccinations are current: e.g. MMR, varicella, hepatitis A and B. Cardiac evaluation (ECG, echocardiogram). Abdominal US (patency of major hepatic blood vess els). Post-transplant complications \200 \200 \200 \200 \200 \200 Primary non-function of the liver (<5%). Hepatic artery thrombosis (10 \200\22315%). Biliar y leaks and strictures (20%). Acute rejection (50%). Chronic rejection (5 \200\22310%). Sep sis (main cause of death).