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Digestive Disorders in

the Pediatric Age
Maria Victoria Matias-Villarica RN, MD, FPSECP
OLFU College of Medicine

Session Outline:
 Hepatitis

 Liver Abscess

 Biliary Atresia

 Cirrhosis

 Cystic Fibrosis

 Acute Pancreatits

 Celiac Disease

Viral Hepatitis
 Caused by 5 hepatotropic viruses (A,B,C,D,E)

 Other causes: systemic infections, non-viral liver infections,
autoimmune, metabolic, drug-induced, anatomic,
hemodynamic and non-alcoholic fatty liver disease

 Manifestations: CLINICAL ICTERUS – mixed or conjugated
hyperbilirubinemia; enlarged, tender liver, splenomegaly,
lymphadenopathy; change in sensorium and hypereflexia –
indicate encephalopathy and ALF

 Direct cytopathic and immune-mediated injury

 Necrosis – centrilobular areas; acute mixed inflammatory
infiltrates; balloon degeneration and necrosis (parenchymal
cells; fatty change (HCV); diffuse Kupffer cell hyperplasia
(siunusoids); formation of GIANT CELLS (neonates)

 Parenchymal collapse (fulminant hepatitis)

 Liver morphology returns to normal – 3 mos. of the acute

Common Biochemical Profiles in the
Acute Infection
 3 main functional liver biochemical profile:
A.  ALT (alanine aminotransferase)and AST (aspartate
transaminase); rapidly falling +  bilirubin levels and prolonged PT–
poor outcome
- reflection of the cytopathic injury to the hepatocytes
B. Cholestasis -  serum conj. bili. levels due to abnormal bile flow
at the canalicular and cellular level due to hepatocyte damage
and inflammatory mediators
C. Altered synthetic function – most impt. marker of liver injury:
prolonged PT, high INR, low serum albumin, hypoglycemia, lactic
acidosis, poor clearance of drugs, altered sensorium (deep
tendon reflexes (hepatic encephalopathy)

jaundice)  Contagious 2 week before and 7 days after the onset of jaundice .Hepatitis A  Most prevalent  RNA virus (picornavirus)  Fecal. highly contagious.oral route. duration of symptoms: 7-14 days  Fecal excretion of the virus resolves 2 weeks after onset of jaundice  Clinical manifestations: anicteric illness (asymptomatic). common source food-borne and waterbourne outbreaks  IP: 3 weeks. malaise. older children: symptomatic (fever. vomiting. anorexia.

Hepatitis A  Diagnosis: antibodies to HAV (anti-HAV IgM): detectable with symptoms and lasts 4-6 mos. viral PCR . fat malabsorption)  Treatment: supportive treatment (Iv hydration. cholestatic syndrome (pruritus. antipruritic meds and fat-soluble vitamins)  If with ALF – transplantation . stool – viral particles.  Complication: No long term sequelae ALF (rare). neutralizing anti-HAV (IgG) – seen 8 weeks from onset and CONFERS long-term protection.

– 0. contact and standard precaution  Ig Pre-exposure: <3 mos.06ml/kg. – 0. . child care. chronic liver disease should receive both Ig and HAV vaccine.none  Vaccine: > 1 yr. chronic liver disease should receive both Ig and HAV vaccine. thereafter. careful handwashing.06ml/kg at departure then every 5 mos. > 2weeks .02ml/kg . – 2 doses (6-12months apart). older child. immunocompromised.Hepatitis A  Prevention: exclusion from school.02ml/kg. immunocompromised.<1yr: 0.  Post-exposure: <2 weeks . older child.5mos. >5mos. protective Ab titer persists for 10 yrs. Ig is optional to healthy 12mos-40 yrs since HAV vaccine is preferred . 3. – 0.

nonparticulate soluble Ag that SERVES as a marker of active viral replication and correlates with HAV DNA levels  Highest prevalence in subSaharan Africa.) HBeAg – nonstructural. Alsaka  Asymptomatic in many children . C (core). ME. Pacific Islands.) HBcAg – encodes the viral DNA b.Hepatitis B  Hepadnaviridae  4 genes: S (surface). Amazon basin. X and P (polymer)  Surface of the virus: HBsAg  Inner core of the virion: a. China.

IV drug use. children: perinatal exposure to HBsAg/HBeAg (+) mother at the time of delivery – serologic markers appear 1-3 mos after birth  Not spread by breastfeeding. semen  Transmitted: blood exposure. serum. hugging or sharing water or utensils. vaginal fluid. related to chronic liver disease and hepatocellular carcinoma .Hepatitis B  High concentration: blood. acupuncture. intimate contact with carriers. serous exudates  Moderate concentrations: saliva. should not be excluded from school unless prone to biting  Breastfeeding – not C/I  Chronic HBV infection – HBsAg (+) > 6 mos is inversely related to age of acquisition. sexual contact. tattoos. kissing.

cirrhosis and hepatocellular Ca is seen. Chronic hepatitis: tolerance phenomenon: perinatal transmission – no activation of cytotoxic lymphocytes. D – Southern Europe. H – Central America  Pathogenesis: Acute hepatitis: non-cytopathogenic virus that causes injury by immune-mediated response. G – US and France. F – US. E – Africa. B and C – Asia. immune-active and inactive . 3 phases: immune-tolerant.Hepatitis B  8 genotypes: A – pandemic. infection of hepatocytes  leads to expression of viral Ags on the cell surface (HBeAg and HBcAg) + class I MHC proteins  target for cytotoxic cell lysis.

antiHBe – marks improvement. if persists > 6mos.  ALT just before development of fatigue. anorexia and malaise – seen 6-7 weeks after exposure. jaundice – 8 weeks after exposure and lasts for 4 weeks  Diagnosis: HBsAg – 1st to appear. HBeAg – active acute or chronic hepatitis. infection.Hepatitis B  Clinical manifestations: asymptomatic. anti-HBc IgM – marker of acute infection before but is replaced by anti-HBc IgG – valuable serologic marker of acute infection. HBV DNA – acute infection and chronic carriers and in HBeAg and typically falls once anti- Hbe develops . anti-HBs – marks serologic recovery and protection and present in persons immunized with hep B vaccine. chronic infection.

autoimmune disorders) b.transplantation  Treatment: supportive. immunoactive individuals . Peginterferon-α2 and several new nucleotide/nucleoside analogs (Telbivudine. esp. HbeAg (+). Interferon – α- 2b – immunomodulator and antiviral effects. AE: bone marrow suppression. Tenofevir.Hepatitis B  Complications: ALF . YMDD). d. reduce viral replication a. with co-infection with HDV. c. Entecavir) – used in adults  Response is seen with patients with low HBV DNA titers. SC X 24 weeks. mortality to ALF is >30% . Adefovir – purine analog that inhibits viral replication in > 12 yrs. retinal changes. Lamivudine – oral nucleoside analog that prevents viral enzyme reverse transcriptase X 52 weeks (to >6mos after viral clearance – emergence of mutant viral strain. > 2x ALT (active inflammation). old.

4th dose – immunocompromised and infants < 2kg. within 14 days after sexual exposure.6 mos. 3rd dose – booster. immunocompromised 40/40ug  HBIG – given within 12 hrs. within 24 hrs. after blood exposure.5/10ug and > 19 yrs. . 1st dose – birth but can be delayed if mother is HBsAg (-).Hepatitis B  Prevention: HB Vaccine (Recombivax HB and Engerix-B) and HBIG (temporary protection 3-6 mos)  AE: pain at injection site  HB vaccine: < 19 yrs. after birth and post vaccination testing is done 9-18 mo later (importance!) .1. middle school entry . long term immunity..>95% sero(+) after 2nd dose.5 uL NB and 0. – 10/20ug at 0. preterm and HBsAg (-) mother – delayed 1 month of age or until hospital discharge. dose: 0.06uL/kg for all  Universal vaccination .

Hepatitis B  Prognosis: outcome after acute HBV infection is favorable despite risk of ALF  HBV infections and complications are effectively controlled and prevented with vaccination .

sexual contact. genotype 2 and 3 – responsive to treatment  Most common cause of chronic liver disease in adults  Risk for transmission: blood transfusion.jaundice .Hepatitis C  Single stranded RNA virus (Flaviviridae family)  6 genotypes – 1b – most common genotype but LEAST responsive to medications. perinatal transmission  Pathogenesis: cytotoxic and immune-mediated immunity  Clinical manifestations: 1-2mos.

HCC . commonly used: PCR assay (HCV RNA) – qualitative while quantitative identify patients who are likely to respond to therapy and monitors response to therapy  Screening for HCV – hx of illegal drug use. receiving clooting factors (before 1987) or blood products (before 1992). hemodialysis. cirrhosis. antibodies to HCV (2 mos after exposure) or viral RNA (PCR) (1-2 mos after exposure) – NEITHER can predict the severity of liver disease because anti-HCV is not a protective Ab and does not confer immunity. idiopathic liver disease and children born to HCV- infected women  Complication: Chronic hepatitis. it occurs simultaneously with the virus.Hepatitis C  Diagnosis: widely used: EIA (enzyme immunoassay) to detect anti-HCV.

weekly) combined with oral Ribavirin. should be stopped if still detectable on viral PCR at 24th week of therapy  Goal: SVR (sustained viral response or absence of viremia 6 months after stopping the medication)  Newer therapy: Peginterferon and teleprevir (NS3 viral protease inhibitor)  Yearly screening with a liver UTZ and serum αfetoprotein for HCC. referral to pediatric gastroenterologist . IFN-α2b (48weeks) .Hepatitis C  Treatment: Peginterferon (SC.

Hepatitis D  HDV. complication: ALF  Diagnosis: IgM antibody to HDV seen 2-4 weeks in co-infection and 10th week in super-infection. defective because it cannot produce infection without concurrent HBV infection (co-infection and super-infection)  Transmission: intrafamilial or intimate contact  IP: 2-8 week  Pathogenesis: cytopathic. PCR assay for viral RNA – research  Treatment: supportive  Prevention: no vaccine . smallest known animal virus.

Hepatitis E  HEV – not isolated but is cloned. causes ALF  Fecal-oral transmission. IgM and IgG assays  Prevention: HBE recombinant vaccine . non-B hepatitis. RNA virus (nonenveloped sphere shape with spikes)  Non-A.  Cytopathic virus  Diagnosis: Ab HEV. peak age: 15yrs and 34 yrs.

Hepatitis G  Blood-borne disease  Transmitted by organ transplantation  Associated with HCV  Unknown IP .

Approach to Acute and Chronic Hepatitis  Prevention  Referral to pediatric gastroenterologist  Avoid alcohol consumption and obesity. care when taking new medications and herbal medications .

cultures . E. abdominal pain (RUQ). canis  Manifestations: nonspecific: fever. Salmonella and anaerobic organisms. aureus. night sweats. portal vein pylephlebitis. E. Species. malaise. K. nausea. coli. pneumoniae. Strep. jaundice is uncommon  Diagnosis: percutaneous UTZ or CT-guided needle aspiration.Liver Abscess  Pyogenic liver abscess  Bacteria entering the liver via the portal circulation (omphalitis. fatigue. histolytica or T. intra-abdominal infection or abscess secondary to appendicitis or IBD or primary bacteremia  Organisms: S. chills. hepatomegaly.

3rd generation cephalosporin + metronidazole  Amoebic abscess: metronidazole or tinidazole + paramomycin  IV X 2-3 weeks followed by oral therapy to complete 4- 6week course . ticarcillin/clavulanic acid. or piperacillin/tazobactam.Liver Abscess  Treatment: open-surgical drainage (less often)  Antibiotics: ampi/sulbactam.

edema.Cirrhosis  End stage of chronic liver diseases. muscle weakness and lethargy. anemia and abdominal pain. infectious. autoimmune or toxic factors  Jaundice. GI bleeding. biliary atresia and chronic hepatitis. anorexia. spider angiomas and prominent blood vessels in the upper torso  Liver transplantation . ascites. poor growth.

resulting to obstructed bile flow. liver failure and death in 1st 2 yrs of life  cholestasis – accumulation of compounds that cannot be excreted resulting to injury to the liver . unknown cause – fetal/embryonic and postnatal (immune-mediated mechanism)  F>M.Biliary Atresia  Extrahepatic biliary atresia (EHBA)  Progressive inflammatory process that causes both intra.and extrahepatic bile duct fibrosis. prematures  Untreated – leads to cirrhosis.

Urine – dark and stains diaper 3. Irritability. poor weight gain 7. Pruritus 8.Biliary Atresia  Clinical manifestation: 1. most striking feature (sclerae) 2. Splenomegaly – later 6. Jaundice – earliest. Failure to thrive. Hepatomegaly and abdominal distention 5. difficult to comfort infant . Stools –lighter or white 4.

electrolyte.Biliary Atresia  Surgical laparotomy and intraoperative cholangiogram – definitive diagnosis  Endoscopic retrograde cholangiopancreatography (ERCP) – 80% diagnostic accuracy  Percutaneous liver biopsy  CBC. liver enzymes  Abdominal UTZ . bilirubin.

medical management – supportive (nutritional support.Biliary Atresia  Therapeutic management: 1. low in sodium. Ursodeoxycholic acid for pruritus abd hypercholesterolemia . not a cure 2. hepatic portoenterostomy (Kasai procedure) – a segment of the intestine is anastomosed to the resected porta hepatis to attempt bile drainage. liver transplantation 3. fat-soluble vitamin supplementation).

cirrhosis and other forms of hepatic dysfunction . cholelithiasis. rectal prolapse. severe chronic lung disease. nasal polyposis.Cystic fibrosis  Inherited multisystem disorder in children and adults  Most common life-limiting recessive genetic trait among white persons  dysfunction of the cystic fibrosis transmembrane conductance regulator protein (CFTR)  Characterized by obstruction and infection of airways and by maldigestion  Pancreatic insufficiency. responsible for many cases of salt depletion. pancreatitis. insulin-dependent hyperglycemia  Manifest as failure to thrive. pansinusitis.

GIT (pancreas and biliary system).Cystic Fibrosis  Inherited as autosomal recessive trait  CF gene codes for CFTR that is expressed in epithelial cells of the airway. sweat glands and GUT  CFTR (member of ATP-binding casette superfamily) that functions as cyclic AMP-stimulated protein kinase A (PKA) regulation of Cl-conductance  CFTR polymorphisms – 5 classes of mutations  Genotyping .

Cystic Fibrosis  Pathogenesis: a. chronic respiratory infection  Membranes of CF epithelial cells are unable to secrete Cl ions in response to cyclic AMP-mediated signals and excessive Na are absorbed through membranes . paucity of water in mucous secretions c. failure to clear mucous secretions b. elevated salt content of sweat and other serous secretions d.

Cystic Fibrosis  Hypothesis:1. inability to secrete salt and secondarily to secrete water in the presence of excessive reabsorption of salt and water  insufficient water on airway surface to hydrate secretions  dessicated secretions become more viscous and elastic (rubbery)  harder to clear by mucociliary mechanisms 2. altering mucus rheology and aggrevates poor mucociliary clearance . altered microenvironment with low HCO3 and a more acidic pH.

Cystic Fibrosis  Pathology: 1. bronchiolar obliteration.inspissated meconium 5. intestinal tract shows minimal change. paranasal sinuses 3. acini and ducts are filled with eosinophilic material to disruption of acini and replacement with fibrous tissue (2nd decade of life) 4. pancreas – cystic. focal biliary cirrhosis . bronchiolitis. bronchiectasis 2. bronchiolectasis.

primary defect is a result of abnormal Cl movement  Primary factor: mechanical obstruction caused by increase viscosity of mucous gland secretion  Instead of a thin.Cystic Fibrosis  Although both Na and Cl are affected. free-flowing secretion. it produce a thick. mucoprotein that acculmulates and dilates them  obstruction of small passages in all organs (ducts and glands) .

intestinal tract – meconium ileus. pancreas – pancreatic insufficiency. biliary tract – evidence of liver dusfunction 4. maldigestion from exocrine pancreatic insufficiency. sweat glands – hypochloremic alkalosis .dry and hacking. ground glass appearance on lower central abdomen. prolapse of the rectum 3.Cystic Fibrosis  Clinical manifestations: 1. hyperglycemia and glycosuria 5. respiratory tract – cough. GUT – sexual development is often delayed but sexual function is unimpaired 6. repeated pneumonia 2.

aeruginosa. aureus. obstructive emphysema. pancreatic function – quantification of elastase-1 activity in the fresh stool 4. Burkholderia cepacia . > 60mEq/L of Cl in sweat 2. sputum cultures – S. chest xray – patchy atelectasis. abnormal PFTs 5. sweat testing – difficult in 1st 2 week of life because of low sweat rates but recommended any time after 1st 48 hr of life. DNA testing – tests for CFTR mutations 3. P.Cystic Fibrosis  Diagnosis and assessment: 1.

initiation of treatment. social worker and psychologist)  Ff-up evaluations : every 1-3 mos.Cystic Fibrosis  Baseline assessment. respiratory therapist. dietician. . clearing of pulmonary involvement and education of patient and parents  Multidisciplinary approach (MD. depending on age at diagnosis  IV antibiotics may be required every 2-3mos. nurse.

Cystic Fibrosis  Pulmonary therapy: 1. aeruginosa ( 1 month on. spacer. aerosolized antibiotic (tobramycin. Inhalational therapy – aerosol – deliver medication and hydrate the lower respiratory tract (MDIs. TOBI) – suppressive therapy for P.5mg) > 12mos – single daily aerosol dose. exacerbations. 1 month off) . compressor use) Human Recombinant Dnase (2. promote a sense of well-being. improves pulmonary function. N- acetylcysteine – should be avoided since it is toxic to the epithelium. 7% hypertonic saline – 2-4X daily improves mucociliary clearance.

Linezolid. Antibiotic therapy – mainstay of therapy to control progression of lung infection oral (> 2 weeks) – Dicloxacillin. Cephalexin. Amikacin. routine aerobic exercises. Clindamycin.Cystic Fibrosis 2. Amoxicillin. Tobramycin. Colistin (2- 4x/day). Airway clearance technique (ACT) – Chest Physical Therapy (CPT) 1- 4x/day. Co0amixiclav. Gentamicin . huffing. Aztreonam. Meropenem. “forced expiration”. Ceftazidime. Vancomycin. Ciprofloxacin. weight training –slows the rate of pulmonary function decline 3. Chloramphenicol Aerosol (6 mos on alternate months) – Tobramycin (BID). Piperacillin. Azzithromycin IV (14 days) – Nafcillin. coughing.

ibuprofen – shown to slow down disease progression 6. alternative agents: Cromolyn sodium and ipratropium – no evidence to support 5. Anti-inflammatory agents – corticosteroids – useful for tx of allergic bronchopulmonary aspergillosis and severe reactive airway disease (modest efficacy and prohibitive side effects in a 4-yr double blind study).Cystic Fibrosis 4. mutation-specific therapies – denufosol and Moli1901 – bypasses the CFTR defect by regulating alternative ion channels . endoscopy and lavage – instillation of saline or mucolytic agent through fibroptic bronchoscope 7. expectorants – iodides and guaifenesin – not effective 8. Bronchodilator Therapy – βadrenergic agonist aerosol (improvement of >15% in airflow rates.

Cystic Fibrosis  Nutritional Therapy – high caloric diet 130 kcal /kgBW 1. stool softeners (polyethylene glycol. docusate sodium)  Proton pump.E.K and zinc  Diatrizoate (Gastrografin) enemas for meconium ileus. vitamin and mineral supplement – A. cholinergic agonist is C/I . recombinant GH therapy (3X/week) 2. pancreatic enzyme replacement – lipase capsules – 20.D. fundoplication for GERD.000IU capsules (<2.500 lipase units/kg/meal to prevent colonic strictures) 3.

life-limiting disorder although survival has improved dramatically .Cystic Fibrosis  Prognosis: .median cumulative survival exceeding 35 yrs. .achievement of an independent and productive adulthood is a realistic goal .

6-mercaptopurine. further enzyme activation. release of active proteases  phospholipase A2 activates lecithin to toxic lysolecithin  release of cytokine and proinflammatory mediators .Acute Pancreatitis  Common pancreatic disorder in children  Causes: blunt abdominal injury. biliary stones or microlithiasis (sludging) and drug toxicity (valproic acid. azathioprine)  Ductal disruption or obstruction  premature activation of trypsinogen to trypsin within the acinar cell  activation of other pancreatic enzymes  autodigestion. L-asparaginase. multisystem disease.

fever  Severe: acutely ill. sonolucent. jaundice. Cullen sign (bluish discoloration around umbilicus). pleural effusion. severe nausea. abdominal pain. edematous pancreas. vomiting. epigastric. persistent vomiting. hypoechoic. pancreatic masses and abscesses (80%).Acute Pancreatitis  Mild: severe abdominal pain (RUQ. shock. hypocalcemia. serum amylase. steady pain). MRI cholangiopancreatography (MRCP) and ERCP – for recurrent/nonresolving pancreatitis . fever. CT scan –pancreatic enlargement. Grey Turner sign (bluish discoloration in the flanks  Diagnosis: serum lipase – test of choice. more specific. ascites.

NGT or nasojejunal tube within 2-3 days of onset  Prognosis: uncomplicated – resolves within 4-5 days . gastric acid suppression.Acute Pancreatitis  Treatment: medical management to relieve pain and restore metabolic homeostasis  Fluid and electrolyte and mineral balance  NPO if vomiting  Recovery in 4-5 days  Severe pancreatitis: antibiotic prophylaxis. elemental alimentation by mouth.

biliary or liver disease. lactase deficiency 2. Absorptive defects – celiac disease or UC 3. Digestive defects – CF. Anatomic defects – short bowel syndrome .Malabsorption Syndrome  Chronic diarrhea and malabsorption of nutrients  Failure to thrive – complication 1.

barley. gluten-sensitive enteropathy (GSE) and celiac sprue  Disease of the proximal small intestine characterized by abnormal mucosa and permanent intolerance to gluten  CD is 2nd to CF in malabsorption cause in children  Pathophysiology: intolerance to gluten (wheat.Celiac Disease (CD)  Gluten-induced enteropathy. resulting to accumulation of toxic substance that is damaging to the mucosal cells  Gluten – “hydrolized vegetable protein” on canned labels . unable to digest gliadin component of gluten. rye and oats).

crypt hyperplasia. vitamin. substitute: corn and rice. iron and caloric supplementation  Prognosis: chronic disease  Lymphoma – most serious complication . enzyme tissue transglutaminase (tTG) – autoenzyme by antiendomysial Ab  Therapeutic management: “gluten-free” diet. villous atrophy. serologic testing – detect Abs to connective tissue (endomysium and reticulin – more specific markers)) and to gliadin (IgG and IgA antibodies).Celiac Disease  Failure to thrive and diarrhea  Diagnosis: biopsy of the SI – mucosal inflammation.

End of Session .