Presentation on theme: "Harvey Aiges, MD Joel Rosh, MD"— Presentation transcript:
1 Harvey Aiges, MD Joel Rosh, MD GI and NutritionHarvey Aiges, MDJoel Rosh, MD
2 Compared to human milk, cow milk formula is more likely to contain which one of the following? More essential fatty acidsHigher protein concentrationIncreased lactose contentLower Calcium-phosphate ratioLower iron concentration5
3 Human Milk has: Low protein (very bio-available) High lactose Low iron (very bio-available if taken alone)Low Calcium-Phosphate ratioInadequate Vitamin K? Adequate Vitamin DImmunoglobulins (including SIgA)
4 Riboflavin Protein Essential fatty acids Vitamin B12 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 of a deficiency is:RiboflavinProteinEssential fatty acidsVitamin B12Copper5
5 The most likely cause is a deficiency of: Riboflavin Protein 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:RiboflavinProteinEssential fatty acidsVitamin B12Copper
6 Change feeds to soy-based formula Obtain stool cultures 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 (90th %ile). Abdomen is slightly protuberant. No tenderness and bowel sounds are hyperactive. Which is most appropriate at this time?Change feeds to soy-based formulaObtain stool culturesDetermine stool pHInstruct parents to reduce volume of feedsSchedule rectal manometry6
7 Improved school performance Flushing and sweating 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:Improved school performanceFlushing and sweatingIncreased thiamine level in CSFIncreased intracranial pressureLess URIs than in his peers4
8 HypervitaminosisVit A (>20,000 IU/d) – Inc ICP (pseudotumor), irritability, headaches, dry skin, Hepatosplenomegaly, cortical thickening of bones of hands and feetVit D (>40,000IU/d)-Hypercalcemia, constipation, vomiting, nephrocalcinosisVit E (100mg/kg/d) – NEC/hepatotoxicity - ?due to polysorbate 80 (solubilizer)
9 Folic acid Niacin Riboflavin Cobalamin Thiamine An adolescent girl on a strict vegan diet is most likely to develop deficiency of which of the following water-soluble vitamins?Folic acidNiacinRiboflavinCobalaminThiamine6
14 ALL Fanconi anemia Folate deficiency Iron deficiency 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?ALLFanconi anemiaFolate deficiencyIron deficiencyVitamin B12 deficiency6
15 An 8 mo old white infant is noted to have yellow skin 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?Measure serum bilirubin levelMeasure urine urobilinogen concMeasure serum Vitamin A levelEvaluate dietary historyMeasure serum T4 level6
16 Esophageal varices Esophagitis Gastric duplication Mallory-Weiss tear 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:Esophageal varicesEsophagitisGastric duplicationMallory-Weiss tearPeptic ulcer disease6
18 Upper GI Bleeding Esophagitis Gastritis Ulcer disease H. pyloriMallory-Weiss TearCaustic Ingestion/Foreign BodyEsophageal varicesEsophageal and gastric tumorsVascular anomaliesCoagulapathyEpistaxisTonsillitis/ENTVaricesDuplication of gutIBDHSPMunchausen’s syndrome by proxy
19 Upper GI Bleeding (Infants) Swallowed maternal bloodHemorrhagic disease of the newbornCoagulopathyStress ulceration/gastritisMallory-Weiss tearAllergyEsophagitis (GERD)Vascular anomalyEpistaxisVaricesDuplication of gutMunchausen’s syndrome by proxy
20 Evaluation PE and VS Labs Stool guaiac Upper endoscopy with biopsy* AXRayTagged rbc study
21 Clostridium difficile toxin evaluation Stool for O & P 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:Barium enemaColonoscopyClostridium difficile toxin evaluationStool for O & PStool for rotavirus6
22 Hemolytic-Uremic syndrome Henoch-Schonlein purpura Intussusception 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:Hemolytic-Uremic syndromeHenoch-Schonlein purpuraIntussusceptionJuvenile PolypsMeckel’s diverticulum6
23 Lower GI Bleed – 0 to 30 days Anorectal lesions Swallowed maternal blood (APT test)Milk allergyNECMidgut volvulusHirschsprung’s disease
24 Remove milk and soy from the maternal diet GI referral for colonoscopy 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:Remove milk and soy from the maternal dietGI referral for colonoscopyCall child welfare for possible abuse
25 Lower GI bleed – 30 days to 1 yr Anorectal lesionsMilk AllergyIntussusceptionMeckel’s diverticulumInfectious diarrheaHirschsprung’s disease
26 Allergic Colitis Well appearing ± Irritable Occurs with formula and breast milkRemove milk and soy from dietProtein hydrolysateFlex sigReintroduce dairy at 1 year
27 Lower GI Bleed – 1-12 years COMMON: LESS COMMON: Anal fissure Juvenile polypMeckel’s diverticulumInfectious diarrheaIBDLESS COMMON:Henoch-Scholein purpuraHemolytic uremic syndromeIntestinal duplicationHemorrhoids
28 Meckel Diverticulum Painless rectal bleeding < 4 years of age Failure of omphalomesenteric duct to obliterate2% of populationWithin 2 feet of ileocecal valveMeckel scantechnetium 99m pertechnetate scan
30 “breast milk” jaundice Choledochal cyst Hypothyroidism 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:Biliary atresia“breast milk” jaundiceCholedochal cystHypothyroidismNeonatal hepatitis6
31 Unconjugated Hyperbilirubinemia Physiologic – exaggerated by hemolysis or hematomaBreast feedingBreast Milk (late onset)Crigler-Najjar syndrome I & IIHypothyroidIntestinal obstruction
32 Fructose aldolase deficiency Fructose 1,6 diphosphatase deficiency A 3 wk old girl has fever and vomiting. PE include bulging fontanelle and hepatomegaly. The pt had jaundice and vomiting during the 1st wk after birth. She has been breast-fed. What is the most likely Dx?Fructose aldolase deficiencyFructose 1,6 diphosphatase deficiencyGlycogen Storage Disease type 1Neonatal adrenoleukodystrophyGalactosemia6
33 Direct Hyperbilirubinemia Extrahepatic1.*** Extrahepatic Biliary Atresia2. ***Choledochal Cyst3. Choledocholithiasis4. Extrinsic bile duct compression
34 Direct Hyperbilirubinemia IntrahepaticMetabolicFamilial intrahepatic cholestasisInfectiousAnatomic – Paucity of intrahepatic bile ductsMisc – TPN, Neonatal Lupus
35 EHBA Direct Hyperbili Acholic stool Elevated transaminases and GGT DISIDA scan (99mTc-disofenin)Liver biopsyKasai portoenterostomy <8 weeks
37 Chronic active hepatitis Dubin-Johnson syndrome Gilbert syndrome 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:Chronic active hepatitisDubin-Johnson syndromeGilbert syndromeHepatitis AInfectious Mononucleosis6
38 Hepatitis Most common infectious cause viral Mild-Asymptomatic transaminase elevationHep A, EBV, CMVClinical HepatitisFulminant Hepatic FailureRare in HepA, Hep B
39 Hepatitis A Transmission oral/fecal RNA virus Incubation 2-6 weeks Milder in young childrenDoes not cause chronic infectionSome patients may experience a relapsing courseHepatitis 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:Determine AST/ALT levelsDetermine presence of Hepatitis B surface AgRadionuclide scan of liverUltrasound of abdomenPAPIDA scan6
41 School (first grade) avoidance Constipation 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:Acute appendicitisStrep PharyngitisSchool (first grade) avoidanceConstipation6
43 Stool for guaiac CBC Urinalysis Abdominal sonogram 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 exceptStool for guaiacCBCUrinalysisAbdominal sonogram6
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:
46 GI referral for upper endoscopy Counseling for drug and alcohol abuse 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:GI referral for upper endoscopyCounseling for drug and alcohol abuseSwitch the form of birth controlOrder an abdominal sonogram6
47 Further family and social history 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:Further family and social historyLiver chemistries, amylase and lipaseAn upper GI seriesStool for guaiac6
49 disimpaction dose of PEG (polyethelene glycol) 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:disimpaction dose of PEG (polyethelene glycol)counseling on toilet trainingstat abdominal CT scan for appendicitisbarium enema6
50 IntussusceptionMost common cause of intestinal obstruction between 3 months – 6 yearsSudden acute onset of severe, paroxysmal colicky pain that recurs at frequent intervalsWell in between, can become weak and lethargicCurrent jelly stools70-90% 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: school avoidanceCrohn Diseaseirritable bowel syndromeulcerative colitis6
53 Her symptoms persist so you plan an evaluation that should include all of the following EXCEPT: celiac serologylactose breath testabdominal CT scanstool for ova and parasites6
54 Reasonable interventions for this patient would not include: Cognitive behavioral therapyDietary manipulationTrial of low dose Tri-cyclic antidepressantsEmpiric therapy for Helicobacter pyloriSymptom-based therapy6
58 IBS Functional disorder Rome criteria Abdominal discomfort or pain relieved with defecation an is associated with a change in frequency or consistency of stoolNo rectal bleedingExclude other etiologiesInfection, inflammatory, celiac
59 IBS--Treatment Education and reassurance Proper nutrition/food avoidanceSome studies up to 50% improve with fiberCounseling/Cognitive-BehaviorMedications:AntispasmodicAnti-diarrhealProbioticsTricyclic antidepressantsSerotonin receptor agents
60 A metabolic evaluation Stat head CT scan 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:A metabolic evaluationStat head CT scanUpper endoscopy by your local Pediatric GIAbdominal sonography6
61 Pyloric Stenosis Projectile, non-bilious emesis Most common cause of gastric outlet obstruction in neonates, M>F“olive”pyloric sonogramHypochloremic metabolic alkalosisSurgery
62 Get samples of a low allergy formula Order a pyloric sonogram 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, youGet samples of a low allergy formulaOrder a pyloric sonogramCall the ED to alert them of a neonatal bowel obstruction patientSend in your junior partner “to deal with it”6
63 Once in the emergency room, proper management of this infant would include: Intravenous fluid resuscitationStat pediatric surgical consultationContrast imaging of the bowelNasogastric decompressionAll of the above6
64 Volvulus Abnormal fixation of bowel mesentery during fetal development Most occur in utero or early infancySudden onset of abdominal pain and bilious emesisIschemia and necrosisUGI series
65 Vulnerable child syndrome Celiac disease Milk protein allergy 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:Vulnerable child syndromeCeliac diseaseMilk protein allergyDuodenal atresia6
66 Duodenal Atresia Double-bubble sign 1:4,500 newborns 2-5% Trisomy 21 Assoc with obstructive processes i.e. annular pancreasAlso found in fetal alcohol syndrome
67 Differentiating GER and GERD Gastroesophageal Reflux. Passage of gastric contents into the esophagusRegurgitationPassage of refluxed gastric contents into oral pharynxVomitingExpulsion of refluxed gastric contents from mouthGERDGastroesophageal Reflux Disease. Symptoms or complications that occur when gastric contents reflux into esophagus or oropharynxIn order to understand the pathophysiology and epidemiology of gastroesophageal reflux disease, or GERD, some basic definitions are needed. The literature has numerous studies which define GERD in a number of ways; in many, what is being described may in fact be normal physiological reflux. This slide highlights how to differentiate gastroesophageal reflux (GER) from GERD.Gastroesophageal reflux is actually normal and it is physiologic--simple passage of gastric contents into the esophagus.Regurgitation, which is a normal physiologic action and can also be pathologic depending upon its frequency, is passage of reflux gastric contents into the oral pharynx.Vomiting, expulsion of reflux gastric contents from the mouth, can be a component of GERD or other diseases, and is also physiologic.GERD are the symptoms and/or complications that occur with gastric content reflux into the esophagus or into the oral pharynx. GERD is the pathophysiologic or pathologic state.
68 Prevalence of Regurgitation in Healthy Infants 100n = 948≥ 1 time a day≥ 4 times a dayInfants (%)50Regurgitation is the most common manifestation of gastroesophageal reflux (GER) in childhood. A cross-sectional survey completed by 948 parents showed that the prevalence rate of regurgitation (at least 1 episode daily) was 50% in 0 to 3-month-old infants, reached a peak of 67% at 4 months, and dropped dramatically to 5% in 10- to 12-month-old infants. A similar pattern was reported for regurgitation of at least 4 episodes daily. Many subjects in this survey “outgrew” GER by 7 months and most by 1 year. At 1-year follow-up, infants with previously reported daily regurgitation no longer were symptomatic – not one of their parents described spitting up as a current problem. These findings support the concept that GER in most infants and children is a physiologic and self-limiting condition.0-34-67-910-12Age (months)Nelson et al. Arch Pediatr Adolesc Med. 1997;151:569
69 Pathophysiology of GERD Delayed gastric emptying timeTransient lower esophageal sphincter relaxation; decreased LES pressureImpaired esophageal clearanceOn the schematic in this slide, the pathophysiology of GERD is shown. There have been a number of well designed case-control studies which compared series of children with GERD to normal controls.Using a technique called esophageal manometry showed that transient lower esophageal sphincter relaxation was consistently more common in children with GERD. This was also associated with inhibition of lower esophageal body peristalsis.Other mechanisms contributing to GERD are overall decreased tone or pressure of the LES, as well as impaired esophageal clearance. These tend to occur more frequently in older children and adults than in infants.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 InfantsFeeding refusalRecurrent vomitingPoor weight gainIrritabilitySleep disturbanceApnea or Apparent Life-Threatening Event (ALTE)Older child/adolescentRecurrent vomitingHeartburnDysphagiaAsthmaRecurrent pneumoniaUpper airway symptoms (chronic cough, hoarse voice)The specific symptoms and signs of GERD in infants (on the left) are compared in this slide to older children and adolescents (on the right). First, whether the infant group or the older child group, GERD-related symptoms are different than you see in an adult. It’s not until they reach pre-adolescence and adolescence that you start to see the typical symptoms, such as heartburn or dysphasia.In the infant GERD symptoms such as feeding refusal, recurrent vomiting, poor weight gain are described. A syndrome called Sandifer’s syndrome can be seen in infants (i.e., arching or head tilting) and is more commonly described in children 18 months to three years of age. In this GERD-related syndrome, the child will turn their head to the side in order to increase LES pressure and lengthen the esophagus and decrease exposure of the esophagus to acids.In the older child and adolescent recurrent vomiting can occur, as well as asthma, recurrent pneumonia. These latter two symptoms are what are called extra- or supra-esophageal manifestations of GERD.Rudolph et al. J Pediatr Gastroenterol Nutr. 2001;32:S1.
71 Diagnosis of GERDBarium swallow/Upper gastrointestinal series (anatomy)Ambulatory single or dual-channel pH monitoringImpedanceEndoscopy and biopsyRadionuclide scanningSupra-esophageal GERD is diagnosed in similar ways as esophageal GERD, and at present, there is not one gold standard for diagnosis. Comprehensive history and physical exam and initiation of empiric therapy are common practice and appear to be effective. Diagnostic tests like barium swallow, endoscopy and biopsy and ambulatory dual channel pH probe have all been used or considered and may be indicated based on the presenting symptoms of the patient, but none are the definitively accurate test.
72 Rudolph et al. J Pediatr Gastroenterol Nutr. 2001;32:S1. Complications of GERDErosive esophagitisPeptic strictureBarrett’s esophagusAdenocarcinomaWhat are some of the complications of GERD?Four of the most serious GERD-related complications are shown in this slide: erosive esophagitis, peptic stricture, Barrett’s esophagus and adenocarcinoma.Erosive esophagitis used to be an entity that was felt to be relatively rare in the pediatric population. However a recent study performed by the Pediatric Endoscopy Database System Clinical Outcomes Research Initiative Registry (PEDS-CORI) led by Mark Gilger at Texas Children’s, demonstrated a high percentage of children with erosive esophagitis who underwent diagnostic upper endoscopy. In a cohort of over 8,000 pediatric patients, erosive esophagitis was observed in 13%; a much higher prevalence rate than expected or previously believed.Peptic stricture, also occurs in childhood, and now Barrett’s esophagus and adenocarcinoma, which previously had been felt to be adult diseases, have been described in the pediatric population.Rudolph et al. J Pediatr Gastroenterol Nutr. 2001;32:S1.
73 Step-Up Therapy for GERD FOR INFANTSNormalize feeding volume and frequencyConsider thickened formulaPositioningConsider trial of hypoallergenic formulaFOR OLDER CHILDRENAvoid large mealsDo not lie down immediately after eatingLose weight, if obeseAvoid caffeine, chocolate, and spicy foods that provoke symptomsEliminate exposure to cigarette smokeStep-up (i.e., start with the least invasive, most conservative approach first) or conservative therapy for GERD is shown in this slide. The left column depicts the approach for infant GERD; the right column the approach for older children with GERD.Shalaby et al. J. Ped. 2003;142:57.
74 Pharmacologic Management of Moderate-to-Severe GERD ProkineticsMetoclopramideMany possible side effects which may include tardive dyskinesis (may be irreversible)Other agents include domperidone, bethanechol* and erythromycinH2RAsAvailable in tablet, elixir, or rapid dissolve form (must be dissolved in water, not on tongue)Pediatric safety, dosing data for ranitidine and famotidinePPIsAvailable in capsule, liquid suspension, or rapid dissolve formPediatric safety, dosing data for lansoprazole and omeprazoleThere are three major classes of agents that are utilized to manage mild-to-moderate GERD; prokinetics, H2-receptor antagonists (H2RAs) and proton pump inhibitors (PPIs). Both the H2RAs and the PPIs are available in liquid or suspension forms and are indicated for managing mild-to-moderate symptoms of GERD. There are pediatric safety dosing data available for ranitidine and famotidine, as well as for lansoprazole and omeprazole. Metoclopramide is a prokinetic that is utilized in a number of children, yet has shown efficacy in a moderate number. However, metoclopramide should be used with caution since it has many side effects and it crosses the blood-brain barrier. Bethanachol and domperidone (unavailable in the U.S.) are two additional prokinetics that have been used in children. Both have significant side effects and have only been shown to be marginally effective. With the removal of cisapride from the U.S. market, there is insufficient evidence that the other prokinetic agents are effective in the treatment of GERD in infants and children (Gibbons TE and Gold BD. The use of proton pump inhibitors in children: a comprehensive review. Paediatric Drugs, 2003; 5(1):25-40).Rudolph et al. J Pediatr Gastroenterol Nutr. 2001;32:S1.Gold. Paediatric Drugs 2002;4:673.Gibbons et al. Paediatric Drugs 2003;5:25.*Bethanechol not approved for pediatric GERD.
75 IngestionsForeign bodies present with dysphagia and possibly poor handling of secretionsNot all foreign bodies are seen on plain film—may need bariumEndoscopic removal by 24 hoursAlkali ingestions may burn esophagus and not the mouth
76 Diarrhea--Infectious Viral—less than one weekRotavirus: most common cause of viral diarrheal disease in infants and toddlersBacterial—sick, bloodSalmonella, Shigella, Yersinia, Campylobacter, E ColiParasitic—persistent**C. Difficile:After antibiotics/hospitalizationCheck for toxin A and BColonization not pathogen in neonates
77 DiarrheaLeading cause of morbidity and mortality in developing countriesDaycare centers important reserviorIn US35-40 million episodes annually in kids <5 yrs170,00 hospitalizations300 deaths
79 E. Coli 0157:H7: associated Hemolytic Uremic Syndrome Often presents with colitis (bloody diarrhea)Hemolysis, uremia developPoly-- then oligouric renal failureThrombocytopeniaSeverity variesRisk factorsUncooked meat, unpasteurized milk**associated with anti-diarrheal and antibiotic use***
80 Malabsorption Defect in intraluminal digestion Damage to intestine CholestasisPancreatic insufficiencyDamage to intestineInfectionViral, giardiaAllergic enteropathyCeliac diseaseShort Bowel
81 Malabsorption Evaluation CarbohydrateCheck stool pHCheck stool reducing substancesFatQualitative vs. Quantitative (72 hour) measuresThink CF—treated with enzyme replacementProtein
82 Dietary Diarrhea Clinically: Contributors: Well No blood, fever, etc. Sorbitol, fruit juice, excessive fluidsLactose intolerance
83 Toddler’s Diarrhea Clinically well Stool Low fat Good wt gain and growthStoolFrequent, undigested foodLow fat- most commonly due to milk restrictionHigh osmolarity fluids- juice, gatorade, powerade, ice tea, etc.
84 Lactose Intolerance Primary vs Secondary Management Diagnosis: Primary rare < 5 yearsManagementRestriction v. supplementIf restriction supplement calciumDiagnosis:ClinicalBreath testDisaccharidase levels in tissue??genetics
85 Celiac Disease Autoimmune Triggered by gluten Associated with hi risk populationsType 1 DMDown syndromeChronic lymphocytic thyroiditis (Hashimoto)IgA deficiencyFamily history
86 Gastrointestinal Manifestations (“Classic”) Most common age of presentation: 6-24 monthsChronic or recurrent diarrheaAbdominal distensionAnorexiaFailure to thrive or weight lossRarely: Celiac crisisAbdominal painVomitingConstipationIrritability
87 Non-Gastrointestinal Manifestations Most common age of presentation: older child to adultDermatitis HerpetiformisDental enamel hypoplasiaof permanent teethOsteopenia/OsteoporosisShort StatureDelayed PubertyIron-deficient anemiaresistant to oral FeHepatitisArthritisEpilepsy with occipitalcalcificationsListed in descending order of strength of evidence
88 Serological Tests first generation (guinea pig protein) Antigliadin antibodies (AGA)Antiendomysial antibodies (EMA)Anti tissue transglutaminase antibodies (TTG)first generation (guinea pig protein)second generation (human recombinant)HLA typingCommercially available tests for celiac disease include the antigliadin, anti endomysial and anti tissue-transglutaminase tests. Tissue transglutaminase has been identified as the auto-antigen in celiac disease against which endomysial antibodies are directed. Initial transglutaminase tests used guinea pig protein as the antigen. Cloning of the gene for human transglutaminase has allowed for tests using human recombinant protein.In addition to antibody tests, some commercial laboratories are offering tests to identify the HLA DQ2 and DQ8 genotypes that are known to be strongly associated with celiac disease.
89 Histological Features Normal 0Infiltrative 1Hyperplastic 2This slide illustrates the various histolopathological findings that occur in celiac disease.Partial atrophy 3aSubtotal atrophy 3bTotal atrophy 3cHorvath K. Recent Advances in Pediatrics, 2002.
90 TreatmentOnly treatment for celiac disease is a gluten-free diet (GFD)Strict, lifelong dietAvoid:WheatRyeBarley
91 Hirschsprung’s Disease History of delayed passage of meconiumFailure to thriveAbdominal distensionVomiting/obstructive picturePotential complications:Perforation esp. cecalEnterocolitis/sepsisdeath
92 Hirschsprung’s Disease: Diagnosis CLINICAL SUSPICIONObstructive series radiographsBarium enema (older child)Suction rectal biopsy—gold standard
93 Fecal overflow incontinence 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:NeuroblastomaGiardiasisLactose intoleranceFecal overflow incontinence
94 Treatment of Constipation Stimulant laxatives—Senna, bisacodylStool softeners/osmoticsPEGLactuloseducosateLubricantsMineral oil
95 Rectal Prolapse CF till proven otherwise Constipation more common cause