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Harvey Aiges, MD Joel Rosh, MD. Compared to human milk, cow milk formula is more likely to contain which one of the following? 1. More essential fatty.

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Presentation on theme: "Harvey Aiges, MD Joel Rosh, MD. Compared to human milk, cow milk formula is more likely to contain which one of the following? 1. More essential fatty."— Presentation transcript:

1 Harvey Aiges, MD Joel Rosh, MD

2 Compared to human milk, cow milk formula is more likely to contain which one of the following? 1. More essential fatty acids 2. Higher protein concentration 3. Increased lactose content 4. Lower Calcium-phosphate ratio 5. Lower iron concentration

3 Human Milk has: Low protein (very bio-available) High lactose Low iron (very bio-available if taken alone) Low Calcium-Phosphate ratio Inadequate Vitamin K ? Adequate Vitamin D Immunoglobulins (including SIgA)

4 A 4 mo old boy with “short gut” from extensive small bowel resection at 2 wks of life is receiving amino acids, hypertonic glucose and trace mineral by PN and is growing well. Last week drying and thickening of skin with desquamation began. The most likely cause of a deficiency is: 1. Riboflavin 2. Protein 3. Essential fatty acids 4. Vitamin B12 5. Copper

5 A 4 mo old boy with “short gut” from extensive small bowel resection at 2 wks of life is receiving amino acids, hypertonic glucose and trace mineral by PN and is growing well. Last week drying and thickening of skin with desquamation began. The most likely cause is a deficiency of: A) Riboflavin B) Protein C) Essential fatty acids D) Vitamin B12 E) Copper

6 A 4 wk old boy has diarrhea and intermittent vomiting for 2 wks. He is getting cow milk formula, 175 to 200 ml q3h (8 feeds/24 hrs). Birth wt = 3.2Kg. PE = afebrile, wt 5.0Kg (90 th %ile). Abdomen is slightly protuberant. No tenderness and bowel sounds are hyperactive. Which is most appropriate at this time? 1. Change feeds to soy- based formula 2. Obtain stool cultures 3. Determine stool pH 4. Instruct parents to reduce volume of feeds 5. Schedule rectal manometry

7 A 7 yr old boy who has had school problems for the past 2 months received a megavitamin that supplies 50,000 u of Vitamin A, 100 mgs of thiamine, 100 mg of niacin, 1 g of ascorbic acid, 2000 u of Vit D, and 500 mg of Vit E. The most likely effect of this regimen will be: 1. Improved school performance 2. Flushing and sweating 3. Increased thiamine level in CSF 4. Increased intracranial pressure 5. Less URIs than in his peers

8 Hypervitaminosis Vit A (>20,000 IU/d) – Inc ICP (pseudotumor), irritability, headaches, dry skin, Hepatosplenomegaly, cortical thickening of bones of hands and feet Vit D (>40,000IU/d)-Hypercalcemia, constipation, vomiting, nephrocalcinosis Vit E (100mg/kg/d) – NEC/hepatotoxicity - ?due to polysorbate 80 (solubilizer)

9 An adolescent girl on a strict vegan diet is most likely to develop deficiency of which of the following water-soluble vitamins? 1. Folic acid 2. Niacin 3. Riboflavin 4. Cobalamin 5. Thiamine 6

10 Vitamin Sources Thiamine – grains, cereals, legumes Riboflavin – dairy, meat, poultry, leafy vegetables Pyridoxine – all foods Niacin – meats, poultry, fish, wheat Biotin – yeast, liver, kidneys, legumes, nuts Folic acid – leafy vegetables,fruits, grains B12 (Cobalamin) – eggs, dairy, meats (not in plants) Vit C – fresh fruits and vegetables

11 Vitamin Deficiencies ( fat soluble) A – night blindness, xerophthalmia, Bitot spots, keratomalacia D – rickets/osteomalacia, low Ca/Phosp E – neurologic deficit (ataxia, ocular palsy, decreased DTRs) K - coagulapathy

12 Vitamin Deficiencies (water-soluble) Thiamine (B1) –beriberi, cardiac failure Riboflavin (B2) – seborrheic dermatitis, cheilosis, glossitis Pyridoxine (B6) – dermatitis, cheilosis, glossitis, peripheral neuritis, irritability Vit B12 – megaloblastic anemia, post spinal column changes

13 Vitamin Deficiencies (water-soluble) Vit C – scurvy, poor wound healing, bleeds Folic acid – megaloblastic anemia, FTT Niacin – pellagra (diarrhea, dermatitis, dementia), glossitis, stomatitis Biotin – organic acidemia, alopecia, seizures

14 A previously healthy 15 mo appears pale. He has been fed goat milk exclusively since birth. Labs reveal: HgB=6.1, WBC=4800, plts=144K, MCV=109. Diff is 29%polys, 68%lymphs, 3%monos. Polys are hypersegmented. What is the most likely cause of these lab findings? 1. ALL 2. Fanconi anemia 3. Folate deficiency 4. Iron deficiency 5. Vitamin B12 deficiency 6

15 An 8 mo old white infant is noted to have yellow skin. The sclerae are normal in color. Of the following, which is the most useful diagnostic test? 1. Measure serum bilirubin level 2. Measure urine urobilinogen conc 3. Measure serum Vitamin A level 4. Evaluate dietary history 5. Measure serum T4 level 6

16 A previously well 10 yr old has fever and persistent vomiting. Initially the emesis was clear, then bile-stained and now it contains bright red blood. Brother has AGE 1 wk ago. PE and CBC/SMA-7 are normal. The most likely cause of hematemesis is: 1. Esophageal varices 2. Esophagitis 3. Gastric duplication 4. Mallory-Weiss tear 5. Peptic ulcer disease 6

17 Upper Presentation Hematemesis Rapid bleeding lesion Coffee ground emesis Slower bleed Hematochezia Melena

18 Upper GI Bleeding Esophagitis Gastritis Ulcer disease H. pylori Mallory-Weiss Tear Caustic Ingestion/Foreign Body Esophageal varices Esophageal and gastric tumors Vascular anomalies Coagulapathy Epistaxis Tonsillitis/ENT Varices Duplication of gut IBD HSP Munchausen’s syndrome by proxy

19 Upper GI Bleeding (Infants) Swallowed maternal blood Hemorrhagic disease of the newborn Coagulopathy Stress ulceration/gastritis Mallory-Weiss tear Allergy Esophagitis (GERD) Vascular anomaly Epistaxis Varices Duplication of gut Munchausen’s syndrome by proxy

20 Evaluation PE and VS Labs Stool guaiac Upper endoscopy with biopsy* AXRay Tagged rbc study

21 A 5 yr old girl was tx with amoxicillin for OM. One week later, she developed abd pain, and has been passing 6 stools daily that contain blood and mucus. PE has T of 101, abdominal distention and diffuse abd tenderness. Among the following, the most appropriate initial diagnostic study to perform is: 1. Barium enema 2. Colonoscopy 3. Clostridium difficile toxin evaluation 4. Stool for O & P 5. Stool for rotavirus 6

22 For the past 6 wks, a 4 yr old has had painless, bright red rectal bleeding assoc with bowel movements. PE of abdomen and anus are normal. The rectal vault is empty and no blood is noted on gross inspection. The most likely cause of hematochezia is: 1. Hemolytic-Uremic syndrome 2. Henoch-Schonlein purpura 3. Intussusception 4. Juvenile Polyps 5. Meckel’s diverticulum 6

23 Lower GI Bleed – 0 to 30 days Anorectal lesions Swallowed maternal blood (APT test) Milk allergy NEC Midgut volvulus Hirschsprung’s disease

24 A 4 week old is brought to you for streaks of bright red blood in the stool. Child is breast fed, thriving and content. Exam shows seborrhea, benign abdomen and perianal exam. Your next intervention: 1. Remove milk and soy from the maternal diet 2. GI referral for colonoscopy 3. Call child welfare for possible abuse

25 Lower GI bleed – 30 days to 1 yr Anorectal lesions Milk Allergy Intussusception Meckel’s diverticulum Infectious diarrhea Hirschsprung’s disease

26 Allergic Colitis Well appearing ± Irritable Occurs with formula and breast milk Remove milk and soy from diet Protein hydrolysate Flex sig Reintroduce dairy at 1 year

27 Lower GI Bleed – 1-12 years COMMON : Anal fissure Juvenile polyp Meckel’s diverticulum Infectious diarrhea IBD LESS COMMON : Henoch-Scholein purpura Hemolytic uremic syndrome Intestinal duplication Hemorrhoids

28 Meckel Diverticulum Painless rectal bleeding < 4 years of age Failure of omphalomesenteric duct to obliterate 2% of population Within 2 feet of ileocecal valve Meckel scan technetium 99m pertechnetate scan

29 amtColorStoolPainThink SmallRedHardYesfissure Sm- mod RedlooseVaries (abd) IBD,H US, inf “Rednl, coated NoPolyp ModRed-TnlYes- abd HSP Mod“nl“Intuss Mod“loose“HD?? ??? Large“nlNoMD

30 A 3,200 gm newborn is noted to be jaundiced on postnatal day #10. Total Bili is 9.0 with a direct Bili of 0.8 mg/dl. Hct is 48%. Baby and mom are blood type O, Rh+. Baby is breast fed exclusively. The most likely explanation of high Bili is: 1. Biliary atresia 2. “breast milk” jaundice 3. Choledochal cyst 4. Hypothyroidism 5. Neonatal hepatitis 6

31 Unconjugated Hyperbilirubinemia Physiologic – exaggerated by hemolysis or hematoma Breast feeding Breast Milk (late onset) Crigler-Najjar syndrome I & II Hypothyroid Intestinal obstruction

32 A 3 wk old girl has fever and vomiting. PE include bulging fontanelle and hepatomegaly. The pt had jaundice and vomiting during the 1 st wk after birth. She has been breast-fed. What is the most likely Dx? 1. Fructose aldolase deficiency 2. Fructose 1,6 diphosphatase deficiency 3. Glycogen Storage Disease type 1 4. Neonatal adrenoleukodystrophy 5. Galactosemia 6

33 Direct Hyperbilirubinemia Extrahepatic 1.*** Extrahepatic Biliary Atresia 2. ***Choledochal Cyst 3. Choledocholithiasis 4. Extrinsic bile duct compression

34 Direct Hyperbilirubinemia Intrahepatic 1. Metabolic 2. Familial intrahepatic cholestasis 3. Infectious 4. Anatomic – Paucity of intrahepatic bile ducts 5. Misc – TPN, Neonatal Lupus

35 EHBA Direct Hyperbili Acholic stool Elevated transaminases and GGT DISIDA scan (99mTc-disofenin) Liver biopsy Kasai portoenterostomy <8 weeks

36 Alagille syndrome Hereditary condition Mutations JAGGED-1 gene Bile duct paucity Peripheral pulmonary artery stenosis Vertebral anomalies Posterior embroyotoxin Characteristic facies

37 A 12 yr old girl has recurrent bouts of scleral icterus, often after viral illnesses. She is otherwise well and is taking no meds. Labs reveal: Total Bili of 3.4 mg/dl with direct Bili of 0.3 mg/dl. ALT/PT/APPT are all normal. The most likely cause of hyperbilirubinemia is: 1. Chronic active hepatitis 2. Dubin-Johnson syndrome 3. Gilbert syndrome 4. Hepatitis A 5. Infectious Mononucleosis 6

38 Hepatitis Most common infectious cause viral Mild-Asymptomatic transaminase elevation Hep A, EBV, CMV Clinical Hepatitis Fulminant Hepatic Failure Rare in HepA, Hep B

39 Hepatitis A Transmission oral/fecal RNA virus Incubation 2-6 weeks Milder in young children Does not cause chronic infection Some patients may experience a relapsing course Hepatitis A vaccine to all children at 1 year

40 Severe RUQ pain, intense jaundice and dark urine in a 9 yr old girl w chronic mild jaundice from spherocytosis. Which diagnostic test is most likely to give correct diagnosis of her current state: 1. Determine AST/ALT levels 2. Determine presence of Hepatitis B surface Ag 3. Radionuclide scan of liver 4. Ultrasound of abdomen 5. PAPIDA scan 6

41 A 6 year old boy is brought to your office with a history of 36 hours of increasing anorexia and periumbilical pain. Last night he had his first episode of nocturnal enuresis in 3 years. He is afebrile and has a benign abdominal exam. Your diagnosis: 1. Acute appendicitis 2. Strep Pharyngitis 3. School (first grade) avoidance 4. Constipation 6

42 Evaluation of Acute Abdominal Pain History- Other sxs (Vomit, BM changes, Systemic) Physical Exam Skin, Lungs, Abdomen, Rectal exam (guaiac) Imaging Plain Films, Sonogram Lab CBC, lytes, Liver/albumin, Pancreas, Urine

43 A 12 year girl comes to the office with 36 hours of abdominal pain, fever and anorexia. Pain is periumbilical and worse in the car than now. You think of appendicitis. Helpful lab tests could include all except 1. Stool for guaiac 2. CBC 3. Urinalysis 4. Abdominal sonogram 6

44 A 17 year old member of the track team comes in with epigastric discomfort and nausea. The big meet is tomorrow and he has been training hard for his last chance to win the medal in his event. He has no significant past medical history other than mild exercise induced asthma and uses an inhaler as he needs. He also uses ibuprofen for muscle pain when training. Your diagnosis:

45 Diagnosis 1. Atypical asthma 2. Performance anxiety 3. Intestinal parasite 4. NSAID complication 6

46 An 18 year old who saw you to start birth control pills prior to going to college now comes in with recurrent, crampy post- prandial epigastric pain that sometimes travels below her right ribs. On exam you find that she has slight scleral icterus, vague epigastric tenderness and a belly button ring. Her urine pregnancy test is negative. Your next step: 1. GI referral for upper endoscopy 2. Counseling for drug and alcohol abuse 3. Switch the form of birth control 4. Order an abdominal sonogram 6

47 A seven year old is seen for a bicycle accident. He is fine other than a few abrasions and an ecchymotic area on his abdomen where he hit the handlebars. 24 hours later, he has significant abdominal and back pain and recurrent non- bilious vomiting. You make the diagnosis with: 1. Further family and social history 2. Liver chemistries, amylase and lipase 3. An upper GI series 4. Stool for guaiac 6

48 Trauma Duodenal Hematoma Handlebar, seatbelts, abuse NGT relieves distention Pancreatic pseudocyst Pancreatitis

49 A 2 year old is brought to you for trouble stooling. Over the last 18 hours he has become “tired and miserable”. He now seems to vomit when straining to pass stool. On exam you notice that he appears lethargic and has a palpable mass in the mid-abdomen. Your next intervention is: 1. disimpaction dose of PEG (polyethelene glycol) 2. counseling on toilet training 3. stat abdominal CT scan for appendicitis 4. barium enema 6

50 Intussusception Most common cause of intestinal obstruction between 3 months – 6 years Sudden acute onset of severe, paroxysmal colicky pain that recurs at frequent intervals Well in between, can become weak and lethargic Current jelly stools 70-90% reduction

51 A 11 year girl comes to see you for recurrent periumbilical pain for the last 9 months. It is worse in the morning, especially on school days. There is no vomiting or weight loss but she does frequently have non-bloody diarrhea with resolution of the pain. Her exam is benign and stool is guaiac negative.

52 Your preferred working diagnosis: 1. school avoidance 2. Crohn Disease 3. irritable bowel syndrome 4. ulcerative colitis 6

53 Her symptoms persist so you plan an evaluation that should include all of the following EXCEPT: 1. celiac serology 2. lactose breath test 3. abdominal CT scan 4. stool for ova and parasites 6

54 Reasonable interventions for this patient would not include: 1. Cognitive behavioral therapy 2. Dietary manipulation 3. Trial of low dose Tri-cyclic antidepressants 4. Empiric therapy for Helicobacter pylori 5. Symptom-based therapy 6

55 RAP—Red Flag Symptoms Nocturnal awakening Persistent Vomiting Dysphagia Bleeding Systemic Signs (Fever, Rash, Arthritis) Affected Growth/Development

56 Organic Causes of RAP Crohn’s Disease Celiac Disease Acid-Peptic/GERD Carbohydrate malabsorption Infection (eg Giardia)

57 Symptom Based Diagnoses Irritable Bowel Syndrome: Diarrhea Predominant Constipation Predominant Alternating Stool Pattern Nonulcer Dyspepsia Functional Abdominal Pain Abdominal Migraine Aerophagia

58 IBS Functional disorder Rome criteria Abdominal discomfort or pain relieved with defecation an is associated with a change in frequency or consistency of stool No rectal bleeding Exclude other etiologies Infection, inflammatory, celiac

59 IBS--Treatment Education and reassurance Proper nutrition/food avoidance Some studies up to 50% improve with fiber Counseling/Cognitive-Behavior Medications: Antispasmodic Anti-diarrheal Probiotics Tricyclic antidepressants Serotonin receptor agents

60 A concerned 22 year old first time mom brings in her 6 week old “vomiter”. After every feed her son “vomits the whole thing”. You note the child is slightly above birth weight and the mother states he seems to be urinating less. You make the diagnosis with: 1. A metabolic evaluation 2. Stat head CT scan 3. Upper endoscopy by your local Pediatric GI 4. Abdominal sonography 6

61 Pyloric Stenosis Projectile, non-bilious emesis Most common cause of gastric outlet obstruction in neonates, M>F “olive” pyloric sonogram Hypochloremic metabolic alkalosis Surgery

62 Your previous patient is now 2 and accompanies his mother with his 6 week old brother who has “vomiting”. This has increased over the last 24 hours. The mother is tired, overwhelmed and complains of her increased dry cleaning expenses as she shows you her vomit stained white blouse that now has green and yellow stains. As your nurse provides her a sympathetic ear, you 1. Get samples of a low allergy formula 2. Order a pyloric sonogram 3. Call the ED to alert them of a neonatal bowel obstruction patient 4. Send in your junior partner “to deal with it” 6

63 Once in the emergency room, proper management of this infant would include: 1. Intravenous fluid resuscitation 2. Stat pediatric surgical consultation 3. Contrast imaging of the bowel 4. Nasogastric decompression 5. All of the above 6

64 Volvulus Abnormal fixation of bowel mesentery during fetal development Most occur in utero or early infancy Sudden onset of abdominal pain and bilious emesis Ischemia and necrosis UGI series

65 The previous mother is grateful and sends her own 45 year old post-partum mother to see you with her Trisomy 21 infant who was just sent home from the hospital “vomiting”. The child is just at birth weight. You send her to the ED and a series of radiographs do not show an obstructive pattern. Rather, there are only two pockets of air in the epigastric region. You are again the star as you diagnose: 1. Vulnerable child syndrome 2. Celiac disease 3. Milk protein allergy 4. Duodenal atresia 6

66 Duodenal Atresia Double-bubble sign 1:4,500 newborns 2-5% Trisomy 21 Assoc with obstructive processes i.e. annular pancreas Also found in fetal alcohol syndrome

67 Differentiating GER and GERD GER Gastroesophageal Reflux. Passage of gastric contents into the esophagus Regurgitation Passage of refluxed gastric contents into oral pharynx Vomiting Expulsion of refluxed gastric contents from mouth GERD Gastroesophageal Reflux Disease. Symptoms or complications that occur when gastric contents reflux into esophagus or oropharynx

68 Prevalence of Regurgitation in Healthy Infants Nelson et al. Arch Pediatr Adolesc Med. 1997;151:569 Infants (%) Age (months) ≥ 1 time a day ≥ 4 times a day n = 948

69 Delayed gastric emptying time Transient lower esophageal sphincter relaxation; decreased LES pressure Impaired esophageal clearance Pathophysiology of GERD Orlando et al, eds. Textbook of Gastroenterology: JB Lippincott Co;1995:1214. Fennerty et al. Arch Intern Med. 1996;156:477. Kawahara et al. Gastroenterology 1997;113:399.

70 Presenting Symptoms and Signs of GERD Infants Feeding refusal Recurrent vomiting Poor weight gain Irritability Sleep disturbance Apnea or Apparent Life- Threatening Event (ALTE) Rudolph et al. J Pediatr Gastroenterol Nutr. 2001;32:S1. Older child/adolescent  Recurrent vomiting  Heartburn  Dysphagia  Asthma  Recurrent pneumonia  Upper airway symptoms (chronic cough, hoarse voice)

71 Diagnosis of GERD Barium swallow/Upper gastrointestinal series (anatomy) Ambulatory single or dual-channel pH monitoring Impedance Endoscopy and biopsy Radionuclide scanning

72 Complications of GERD Erosive esophagitis Peptic stricture Barrett’s esophagus Adenocarcinoma Rudolph et al. J Pediatr Gastroenterol Nutr. 2001;32:S1.

73 Step-Up Therapy for GERD FOR INFANTS Normalize feeding volume and frequency Consider thickened formula Positioning Consider trial of hypoallergenic formula FOR OLDER CHILDREN Avoid large meals Do not lie down immediately after eating Lose weight, if obese Avoid caffeine, chocolate, and spicy foods that provoke symptoms Eliminate exposure to cigarette smoke Shalaby et al. J. Ped. 2003;142:57.

74 Rudolph et al. J Pediatr Gastroenterol Nutr. 2001;32:S1. Gold. Paediatric Drugs 2002;4:673. Gibbons et al. Paediatric Drugs 2003;5:25. Pharmacologic Management of Moderate-to-Severe GERD Prokinetics Metoclopramide Many possible side effects which may include tardive dyskinesis (may be irreversible) Other agents include domperidone, bethanechol* and erythromycin H 2 RAs Available in tablet, elixir, or rapid dissolve form (must be dissolved in water, not on tongue) Pediatric safety, dosing data for ranitidine and famotidine PPIs Available in capsule, liquid suspension, or rapid dissolve form Pediatric safety, dosing data for lansoprazole and omeprazole *Bethanechol not approved for pediatric GERD.

75 Ingestions Foreign bodies present with dysphagia and possibly poor handling of secretions Not all foreign bodies are seen on plain film—may need barium Endoscopic removal by 24 hours Alkali ingestions may burn esophagus and not the mouth

76 Diarrhea--Infectious Viral—less than one week Rotavirus: most common cause of viral diarrheal disease in infants and toddlers Bacterial—sick, blood Salmonella, Shigella, Yersinia, Campylobacter, E Coli Parasitic—persistent **C. Difficile: After antibiotics/hospitalization Check for toxin A and B Colonization not pathogen in neonates

77 Diarrhea Leading cause of morbidity and mortality in developing countries Daycare centers important reservior In US million episodes annually in kids <5 yrs 170,00 hospitalizations 300 deaths

78 Food Borne Fecal-oral Salmonella and Campylobacter Poultry, unpasteurized milk Yersinia enterocolitica Pork Norwalk virus Raw seafood Water Giardia lamblia, Campylobacter, Cryptosporidium, Norwalk virus

79 E. Coli 0157:H7: associated Hemolytic Uremic Syndrome Often presents with colitis (bloody diarrhea) Hemolysis, uremia develop Poly-- then oligouric renal failure Thrombocytopenia Severity varies Risk factors Uncooked meat, unpasteurized milk **associated with anti-diarrheal and antibiotic use***

80 Malabsorption Defect in intraluminal digestion Cholestasis Pancreatic insufficiency Damage to intestine Infection Viral, giardia Allergic enteropathy Celiac disease Short Bowel

81 Malabsorption Evaluation Carbohydrate Check stool pH Check stool reducing substances Fat Qualitative vs. Quantitative (72 hour) measures Think CF—treated with enzyme replacement Protein

82 Dietary Diarrhea Clinically: Well No blood, fever, etc. Contributors: Sorbitol, fruit juice, excessive fluids Lactose intolerance

83 Toddler’s Diarrhea Clinically well Good wt gain and growth Stool Frequent, undigested food Low fat - most commonly due to milk restriction High osmolarity fluids - juice, gatorade, powerade, ice tea, etc.

84 Lactose Intolerance Primary vs Secondary Primary rare < 5 years Management Restriction v. supplement If restriction  supplement calcium Diagnosis: Clinical Breath test Disaccharidase levels in tissue ??genetics

85 Celiac Disease Autoimmune Triggered by gluten Associated with hi risk populations Type 1 DM Down syndrome Chronic lymphocytic thyroiditis (Hashimoto) IgA deficiency Family history

86 Gastrointestinal Manifestations (“Classic”) Most common age of presentation: 6-24 months Chronic or recurrent diarrhea Abdominal distension Anorexia Failure to thrive or weight loss Rarely: Celiac crisis Abdominal pain Vomiting Constipation Irritability

87 Non-Gastrointestinal Manifestations Dermatitis Herpetiformis Dental enamel hypoplasia of permanent teeth Osteopenia/Osteoporosis Short Stature Delayed Puberty Iron-deficient anemia resistant to oral Fe Hepatitis Arthritis Epilepsy with occipital calcifications Most common age of presentation: older child to adult Listed in descending order of strength of evidence

88 Serological Tests Antigliadin antibodies (AGA) Antiendomysial antibodies (EMA) Anti tissue transglutaminase antibodies (TTG) – first generation (guinea pig protein) – second generation (human recombinant) HLA typing

89 Histological Features Normal 0Infiltrative 1Hyperplastic 2 Partial atrophy 3aSubtotal atrophy 3bTotal atrophy 3c Horvath K. Recent Advances in Pediatrics, 2002.

90 Treatment Only treatment for celiac disease is a gluten-free diet (GFD) Strict, lifelong diet Avoid: Wheat Rye Barley

91 Hirschsprung’s Disease History of delayed passage of meconium Failure to thrive Abdominal distension Vomiting/obstructive picture Potential complications: Perforation esp. cecal Enterocolitis/sepsis death

92 Hirschsprung’s Disease: Diagnosis CLINICAL SUSPICION Obstructive series radiographs Barium enema (older child) Suction rectal biopsy—gold standard

93 A six year old is brought to you for diarrhea. Child stools multiple times during the day—seems to be all day. Often there is stool in the underwear. Your exam is notable for a tympanitic abdomen and LLQ mass. Your diagnosis: A. Neuroblastoma B. Giardiasis C. Lactose intolerance D. Fecal overflow incontinence

94 Treatment of Constipation Stimulant laxatives— Senna, bisacodyl Stool softeners/osmotics PEG Lactulose ducosate Lubricants Mineral oil

95 Rectal Prolapse CF till proven otherwise Constipation more common cause

96 IBD INFECTION? SMOKING GENETIC PSYCHOGENIC ENVIRONMENTAL DIETARY DRUGS?

97 Crohn Disease Autoimmune Inflammatory process Mouth to the anus; TI Presentation Abdominal pain, diarrhea, rectal bleeding, growth failure Associated symptom—GI/systemic Extra-intestinal manifestations Peri-anal changes, Fever, Apthous stomatitis, Uveitis/iritis, Skin Manifestations, Joint, Ankylosing spondylitis, Clubbing

98 IBD Interaction between genetic predisposition and environment Increased in Northern European and Jewish population Family History increased risk Typically presents in adolescence and young adulthood

99 Crohn’s and Growth Failure Can be presenting symptom Multi-factorial Nutritional ie. Poor intake Malabsorption Direct cytokine/inflammatory effect on bone

100 Ulcerative Colitis Inflammatory “the bloody diarrhea” Limited to colon Continuous disease Extra-intestinal manifestations

101 Refeeding Syndrome Malnourished patients Electrolyte abnormalities Hypophosphatemia Fluid retention Careful monitoring and slow refeeding Edema, muscle weakness, arrhythmias


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