GI Board Review: The important S**T April 13, 2010 Jillian Parekh, MD Question: A 12 y/o F presents to the ED with abdominal pain. Her parents report that she awakened with a temp of 101 this am, c/o abdominal pain, and has vomited twice. Denies diarrhea, and there are no sick contacts. She reports nausea and no interest in food or drink. She was previously healthy. UA shows SG 1015, otherwise normal. Of the following, the finding that MOST indicated the need for immediate surgical intervention is: A. Abdominal distention B. Hyperactive bowel sounds C. Pain in the right lower quadrant D. Rigidity of the abdominal wall E. Voluntary guarding Answer: A. Abdominal distention B. Hyperactive bowel sounds C. Pain in the right lower quadrant D. Rigidity of the abdominal wall E. Voluntary guarding Explanation: Abdominal tenderness can occur in children with nonsurgical abdomens and those with peritoneal inflammation Presence of bowel sounds is usually reassuring Simple abdominal distention may occur with non surgical causes of abodminal pain RLQ pain is suggestive when other signs of peritonitis are present, but can also occur in other processes (ovarian, AGE) Voluntary guarding is common on exam, esp with children Distention with tenderness, rigidity of the abdominal wall to palpation are more specific signs of peritoneal irritation surgical abdomen Appendicitis: Child > 2 usually Acute RLQ, periumbilical or midepigastric abdominal pain – classically McBurney’s point Associated with nausea, vomiting, anorexia, low grade fever Xray findings: sentinel loop, absence of air in the RLQ NSAID induced dyspepia NSAIDs inhibit cyclooxygenase (COX) COX needed for prostaglandin synthesis Prostaglandins protect the gastric lining If they describe a patient with rheumatoid condition p/w epigastric pain…think NSAIDs. Frequent causes of acute pain: Constipation Adenitis Mono Pancreatitis Hepatitis Infection (UTI) Trauma Recurrent or chronic pain: 3 or more episodes of abdominal pain in more than 3 months Pain must be severe enough to interrupt normal activity On the boards, think of psych factors Encorporesis LLQ pain with a palpable mass on exam School aged child, soils pants, denies diarrhea, no systemic complaints Treatment: Education (avoid blame) Emptying colon (enema, cathartics, stool softeners) Maintenance (daily stools) Giardia Lamblia Several weeks of intermittent watery diarrhea, abdominal distension, anorexia Afebrile Hx of drinking bad water on camping trip or daycare exposure Dx = string test “Entero-Test” via ELISA Diarrhea: Never give anti-diarrheal in children Watery diarrhea: derives from small intestine – high volume, non bloody Inflammatory diarrhea: small and frequently contains blood, mucous and WBCs. More toxic presentation Continue normal diet ORT if needed: 2% glucose, 90mEq NaCl Avoid fatty foods, high glycemic foods BRAT diet is too limited No bowel rest – decreasing gut motility can result in pooling of fluids and unnoticed dehydration Viral diarrhea Rotavirus is the leading cause worldwide Adenovirus is 2nd leading cause Bacterial Diarrhea Enteropathogenic E.Coli: Seen in areas with poor sanitation p/w fever, vomiting, non-bloody stools Traveller’s diarrhea p/w severe diarrhea, cramping Causes HUS Entertoxigenic E. Coli: Enterohemorrhagic E. Coli: Hemolytic anemia Thrombocytopenia Uremia: renal failure Enteroinvasive: Stools are blood and mucous tinged, tenesmus Similar to dysentery Salmonella Green, malodorous stools Can present in infancy, unlike shigella Typhoid fever (S. Typhi) is more invasive form of this disease p/w fever, HA, abdominal pain, myalgias and rose spots Treat with Ceftriaxone/Cefotaxime if: < 3 months Immunocompromised Severe colitis Shigella Present with watery diarrhea and fever, bloody diarrhea usually appears after fever subsides Increased bandemia on CBC, even if WBC not elevated Diarrhea + seizure = Shigella Treat with Bactrim (high resistance) Pseudomembranous Colitis Presents as diarrhea (often bloody), abdominal pain and vomiting. Caused by C. Difficile toxin Often will give hx of prior abx use (Clinda) Rx: Flagyl PO Vanco if resistant Infants can be asymptomatic carriers – rarely become symptomatic even when the toxin is present in stool Don’t treat < 6 months old (unless sx) Chronic Diarrhea: Diarrhea that lasts > 2 weeks and can’t be attributed to AGE. Nutrition and growth often affected Transient lactase deficiency Cyrptosporidium Toddler’s diarrhea Malnutrition Abetalipoproteinemia Intestinal lymphangiectasia Question: A 10 y/o African American boy presents to clinic c/o a 1 year history of stomach pain, nausea, bloating and diarrhea that occurs 45-60 mins after eating dairy foods. Sx occur only when he eats too much. He denies emesis, hematochezia, or pruritis associated with these episodes. On PE, the boy appears healthy and has normal VS. His abdomen is soft and has normal BS, stool guaiac is negative. Of the following, the MOST likely cause for the symptom is: A. allergic eosinophilic gastroenteritis B. lactose intolerance C. milk protein allergy D. milk protein enterocolitis E. oral allergy syndrome Answer: A. allergic eosinophilic gastroenteritis B. lactose intolerance C. milk protein allergy D. milk protein enterocolitis E. oral allergy syndrome Explanation: Lactose intolerance is consistent as has onset of only GI sx, can tolerate small amounts of dairy, sx occur 30 mins later. Results from decreased lactase activity. In children, lactase activity doesn’t decline to clinically significant levels until age 6. Milk protein allergy is IgE mediated – develops in first year of life (urticaria, angioedema, AD…). Milk protein enterocolitis, is non IgE mediated, but p/w hematochezia within first months of life. Usually cross react with soy milk- need elemental formula. Allergic eosinophilic gastro will always present with element of weight loss or FTT. Usu presents with reflux and dysphagia. Oral allergy syndrome is a localized reaction that occurs in 10-40% of individuals with allergic rhinitis. Usually occurs with raw fruits or vegetables – causes immediate pruritis and swelling. Milk Issues: Cow Milk Protein Allergy: only 1% population, usually resolves by age 2. Present with associated sx (AD, Asthma) Milk Protein Intolerance: Milk Protein Allergy – IgE mediated, can cause anaphylaxis, can trigger AD Food Sensitivity – FPIES (Food Protein Enterocolitis Syndrome). More common than IgE form. Causes vomiting and bloody diarrhea. Cow and soy milk are frequent triggers. Rx: elimination diet. -avoid milk products for 1-2 years Neonatal Vomiting Antral Web: Similar to pyloric stenosis, but outlet obstruction is before the pylorus. Presents with NON-bilious vomiting Presents in first 6 mos (later than PS) Tend to describe low BW and polyhydramnios Dx: US Radiolucent filling defect in prepylori region RX: surgical resection Pyloric Stenosis: Males > Females Maternal hx of PS increases the risk more than paternal hx Presents with progressive non-bilious vomiting Will mention: projectile vomiting and palpable olive Presents in first months (1-5 mos) HYPOCHLOREMIC HYPOKALEMIC METABOLIC ALKALOSIS vomit HCL (lose acid and chloride) Volume loss – kidney holds onto Na, dumps K Pyloric length > 14 mm Pyloric muscle thickness > 4mm Dx: US Rx: Surgical (after electrolyle repletion) Duodenal Atresia: Bilious vomiting Presents in 1st day of life Frequently icteric (decreased enterhepatic circulation) Plain XRay: Double Bubble sign No air distal to the atresia (if complete) Rx: NT decompression, surgery Question: A 5 d/o term infant presents to the ED with a h/o bile stained emesis. She is well nourished and hydrated and had an unremarkable course in the WBN. She was discharged at 48 hrs and was breastfeeding, but her mother states that the baby always has vomited. PE reveals an afebrile patient who has normal VS, but no audible bowel sounds. An abdominal XRay shows paucity of bowel gas. Of the following, the MOST likely diagnosis is: A. anorectal atresia B. cystic fibrosis C. malrotation of the bowel D. septic ileus E. tracheoesophageal fistula Answer: A. anorectal atresia B. cystic fibrosis C. malrotation of the bowel D. septic ileus E. tracheoesophageal fistula Explanation: Bilious emesis is always a surgical emergency in the newborn – signifies anatomic or functional obstruction. No bowel sounds with paucity of gas on XR is concerning for malrotation with midgut volvulus. Classic xray finding is double bubble sign If malrotation is not diagnosed quickly, most of small intestine can be lost. 50% of malro cases that occur in first year of life, will happen in first week, 25% in weeks 1-4, final 25% in 1month-1year. Anorectal atresia would present with absent or delayed passage of meconium. TEF usually presents with resp distress or inability to handle oral secretions. CF can be associated with meconium ileus and delayed passage of meconium Septic ileus: patient would appear systemically ill. Malrotation: Surgical emergency Cecum fails to descend or be fixed to the posterior right abdominal wall. Can rotate causing compression of duodenum and duodenal obstruction Presents as bilious vomiting, abdominal tenderness & abdominal distension Volvulus: Presents as bilious vomiting and R sided abdominal distension Associated with Ladd Bands which constrict the large and small bowel XRay Gastric and duodenal dilatation Decreased intestinal air Corkscrew appearance of duodenum Annular Pancreas: Pancreas literally forms a ring around the intestine causing significant obstruction. History of polyhdramnios (fluid not swallowed effectively in utero) Inborn Errors of Metabolism: Common cause of vomiting in infancy Will be afebrile Look for metabolic acidosis with and increased AG Question: A 5 month old infant p/w history of vomiting b/w 1020 times/day. She is growing and developing normally. There is no blood in the vomitus, no resp sx, and no history of apnea. The parents are frustrated and want something done. PE and upper GI results are normal. Of the following, the MOST accurate statement about this patient is that she: A. is at increased risk of SIDS B. is likely to develop an esophageal stricture later in life C. probably will outgrow the condition by 1 y/o D. should be referred for a head CT E. should undergo colonoscopy to r/o eosinophilic esophagitis Answer: A. is at increased risk of SIDS B. is likely to develop an esophageal stricture later in life C. probably will outgrow the condition by 1 y/o D. should be referred for a head CT E. should undergo colonoscopy to r/o eosinophilic esophagitis Explanation: GER described has no signs of pathologic reflux (recurrent pneumonia, hematemesis, or FTT), so likely that she will outgrow her sx by age 1. GER occurs in about 50% of term infants and peaks b/w 4-6 months. By 12 mos the GER resolves in 9095% of infants. GER rarely causes esophageal strictures No evidence that GER places you at greater risk for SIDS Head CT not warranted at this time Eosinophilic esophagitis should only be considered if strong atopic history GER: Regurgitation or spitting up, increases when infant lying down Can present with severe emesis, FTT, or apnea in newborn period Usually presents around 2 months of age Uncomplicated GER resolves by age 2 without intervention Vomiting is effortless, no other signs of illness Sandifer Syndrome: unusual dystonic movements of the head and neck along with GER RX: limited to those with symptomatic disease and those with neurologic impairment Antacids H2 blockers PPI Other causes of vomiting: DKA Cyclic vomiting- emotional overtones, also at risk for migraines and IBS. Schol aged patient, episodes separated by asx periods, dx of exclusion Munchausen Syndrome By Proxy Rumination: frequent regurgitation of ingested food into the mout that is rechewed and swallowed or spit out. Seen in infants of severely disturbed mothers. Induce vomiting to seek attention. Resolving emotional trigger is best Rx. Mouth: Cyst on the floor of mouth – ranula (treat with excision) Midline mass on floor of mouth – may be ectopic thyroid, shouldn’t be removed Parotitis: most cases are idiopathic and don’t require Rx. (think mumps, HIV) Mikulicz’ disease: parotid swelling, dry mouth and poor tear production Ectodermal hypoplasia: X linked, underdeveloped or absent teeth, absence of sweat glands. Dx: skin bx no sweat pores Hallerman Streiff Syndrome: underdeveloped small teeth, bird nose. Gardener’s syndrome: extra teeth and polyps in large and small intestines (premalignant). AD inheritance. Rx: surgical removal of polyps. Esophagus: Varices: liver disease causes portal HTN– bright red/bloody stools, hematemesis, tarry stools. TEF: with upper esophageal pouch is most common type. Presents with coughing during feeds in neonatal period. Film will show feeding tube coiled in blind ending esophagus. Keep NPO, drain blind ending puch, surgery. Will describe copious secretions, “can’t pass NG”, polyhdramnios. Question: 15 y/o M p/w melena and anemia. EGD shows nodular gastritis of the antrum and an ulcer. Biopsies demonstrate spiral-shaped organisms c/w H. Pylori. You prescribe Amox, Clarithromycin, and Lansoprazole x 2 weeks. At f/u visit, family asks if Rx was successful. Of the following, the PREFERRED NONIVASIVE test to evaluate whether the pathogen is eradicated is: A. Fecal campylobacter-like organisms (CLO) B. Fecal H. Pylori antigen C. Salivary H. Pylori antibody D. Serum H. Pylori immunoglobulin G serology E. Serum H. Pylori urease concentrations Answer: A. Fecal campylobacter-like organisms (CLO) B. Fecal H. Pylori antigen C. Salivary H. Pylori antibody D. Serum H. Pylori immunoglobulin G serology E. Serum H. Pylori urease concentrations Explanation: H. Pylori is known risk factor for gastritis and duodenal ulcers. Gold standard for dx is EGD with biopsy Best non invasive test: H. Pylori fecal antigen Urease breath test also good test H. Pylori IgG is a useful marker of epidemiologic studies of past or current infection, but sensitivity and PPV in children is suboptimal. + IgG screen should be confirmed with a second test. Testing for eradication of organisms after treatment should be done 1 month after therapy completed. Stomach: PUD: vomiting after eating, epigastric pain severe enough to wake kid out of sleep, guaiac positive stools. EGD is best study with bx for H. Pylori. Rx: H2 blockers, Sucralfate (coats damaged mucosa), misoprostol (PG analogues, enhance bicarb production and decrease gastric acid production), PPI (inhibit gastric acid pump) H. Pylori: Dx: H. pylori IgG only as screening tool, confirm with fecal antigen or Urea breath test, or bx. Triple therapy: PPI + 2 abx (Amox and Flagyl; Amox and Clarithro) x 14 days. NSAID dyspepsia: cuase GI sx by interfering with PG synthesis. Rx: antacids and food. Zollinger Ellison Syndrome: gastrin secreting tumor, present with sx of PUD. Dx: fasting gastrin levels. Question: A 16 y/o M comes to your office with a 6 month hx of abdominal cramping. States that the cramps immediately precede a BM and that passage of stool results in pain relief. No clear association with food. His BMs are variable, ranging from hard stools every other day to loose stools several times/day. Weight and height are normal, as is PE findings. Stool guaiac, CBC, ESR, LFTs, IgA, TTG are all normal. Stool studies negative for Giradia, C. Diff and enteric pathogens. Of the following, the MOST appropriate next step is: A. Colonoscopy and biopsy B. Fiber supplementation C. Observation D. Oral tegaserod E. Referral to psychiatrist. Answer: A. Colonoscopy and biopsy B. Fiber supplementation C. Observation D. Oral tegaserod E. Referral to psychiatrist. Explanation: IBS is a functional GI disorder that typically occurs in teens and young adults. Defined as abdominal discomfort that is relieved with defecation and associated with either a change in frequency or consistency of stool. Diarrhea is common, rectal bleeding is not. Need to exclude infectious causes of diarrhea. Thought to be due to altered colonic motility, alterations in colonic microflora and excess gas production. Psych factors can also worsen sx. Treatment often begins with trial of fiber supplementation – helps up to 50%. Tegaserod: effective for constipation predominant IBS (5HT4 agonist) – not first line and not indicated for pts with diarrhea. If no red flags, endoscopic evaluation should be postponed until after trial of therapy. Question: A 13 m/o infant p/w a 1 month hx of chronic diarrhea and weight loss. The baby tolerates cow milk formula well, but the diarrhea began around the time he was transitioned to whole milk. There is a family history of multiple food allergies. PE demonstrates a thin infant whose weight is in the 10% and height is at 50%. Stool cultures for bacteria and viruses are negative. Results of CBC, Chem, and serum IgA are normal. Celiac serologies demonstrate a positive antigliadin IgG, negative antiendomysial Ab, and negative tissue transglutaminase Ab. A small bowel bx demonstrates increased cellularity of the intestinal lamina propria and partial villous atrophy. Of the following, a TRUE statement regarding the patient’s small bowel bx is that the findings: A. are diagnostic for giardiasis B. are nonspecific C. are pathognomonic for rotavirus D. exclude celiac disease E. rule out milk protein allergy Answer: A. are diagnostic for giardiasis B. are nonspecific C. are pathognomonic for rotavirus D. exclude celiac disease E. rule out milk protein allergy Explanation: Altho the pt has a + antigliadin IgG, the antibodies are not the most sensitive and specific for Celiac disease. The biopsy findings described suggest intestinal injury, but are nonspecific. They can’t differentiate b/w celiac, allergy and infection. Biopsy findings with Celiac show varying degrees of villous atrophy and increased intraepithelial lymphocytes. Increased Eos suggests allergic enteritis and in infectious enteritis you usually see the pathogen. Immunostaining can be done on biopsy tissue, but the more samples taken the better as lesions are “patchy”. Intestines: Celiac disease: bulky, pale, frothy and foul smelling stools. Proximal muscle wasting and abdominal distension. Dx: biopsy (antigliadin or antiendomysial abs in serum – need bx confirmation). IBS: high emotional component. Constipation and diarrhea. Rx: high fiber diet and close attention to emotional factors. Cystic Fibrosis: Chronic diarrhea, steatorrhea, and hyponatremia (sweat losses). malabsorption secondary to poor exocrine function. Often associated with rectal prolapse or 10% present with meconium plug syndrome. Pernicious anemia: B12 absorbed in terminal ileum – if describe patient with small bowel resection and include a CBC, think B12. Question: The father of 3 children in your practice recently was diagnosed with Crohn’s disease. His wife doesn’t have the dz. He asks you if his children, ages 10,12,and 16, are at increased rik for developing Crohn’s. Of the following, you are MOST likely to advise the father that A. although his children are at increased risk of developing Crohn’s, their risk of developing UC is decreased B. Crohn disease in childhood usually presents in children younger than age 5 C. each of his children has at least a 20% chance of developing Crohn’s during their lifetime D. most patients who have Crohn dz can be diagnosed by genetic testing E. smoking is associated with an increased risk of developing Crohn disease Answer: A. although his children are at increased risk of developing Crohn’s, their risk of developing UC is decreased B. Crohn disease in childhood usually presents in children younger than age 5 C. each of his children has at least a 20% chance of developing Crohn’s during their lifetime D. most patients who have Crohn dz can be diagnosed by genetic testing E. smoking is associated with an increased risk of developing Crohn disease Explanation: Crohn disease is characterized by regional intestinal inflammation, most commonly affecting the terminal ileum or colon. Upper bowel and stomach may be involved. Presents with abdominal pain, diarrhea, rectal bleeding, growth failure or perianal inflammation. IBD is a complex polygenic condition that result from interactions b/w genetic predisposition and the environment. Northern Europeans and Jews are at increased risk. Family history is a known risk factor, and can increase your risk for Crohns or UC. It typically presents in teen years, rare in <5 y/o Currently there is no genetic test available (althouth there is a mutation to NOD2 identified in ~25% of patients with Crohn’s). Environmental triggers may play a role – and smoking is the best established environmental risk factor – studies suggest a two-fold relative risk in smokers. Question: You are following an 11 y/o F who has Crohn disease involving the stomach, ileum, and colon. Her maintenance meds are mesalamine and 6-mercaptopurine. Over the past year, she has received 4 courses of steroid treatment, but continues to have intermittent abdominal pain and diarrhea. Upon review of her growth curve, you note that her height has been the same over the past 12 months. You suspect that the combination of Crohn disease and steroid therapy has resulted in growth arrest. You discuss your concerns with GI. Of the following, the MOST appropriate medication to control this patient’s disease and reduce her dependence on steroids is: A. cyclophosphamide B. infliximab C. mycohpenalate mofetil D. tacrolimus E. thalidomide Answer: A. cyclophosphamide B. infliximab C. mycohpenalate mofetil D. tacrolimus E. thalidomide Explanation: CD and UC (IBD) are serious illnesses mediated by the immune system that cause intestinal inflammation. Inflammation in CD is transmural and can involve any region of GI tract (unlike UC – inflammation limited to mucosal layer of large bowel). Patient with CD present with diarrhea, rectal bleeding, anorexia, growth failure, anemia, abdominal disease and perianal disease. Most commonly corticosteroids are used to induce remission in moderateto-severe CD or UC. Salicylates (mesalamine) are useful in maintaining remission in UC, but less effective in CD. Immunomodulators (6-MP, azathioprine, MTX) are used to maintain remission in patients with UC who fail salicylate therapy or patients with CD. Patients (like the one in question) who fail salicylate and immunomodulators benefit from addition of INFLIXIMAB – antibody of tumor necrosis factor (TNF). Infliximab is highly effective, but does increase risk of opportunistic infections (esp TB) and lymphoma. Thalidomide and tacrolimus can be used for highly resistant CD patients. Cyclophosphamide and Mycophenolate are rarely used to treat IBD. Colon: Intussusception: sudden onset, severe paroxysmal colicky pain in previously healthy kid. 3 mos- 6 years. “Draw up legs and vomit”, pain relieved by passing stool. “currant jelly stool”, palpation of sausage like mass. Later stages – bilious vomiting. Rx: Air enema. R/O lymphosarcoma when child >6 gets intussusception Associated with HLA B27 antigen and AS. UC: usually in teen years, hx chronic crampy lower abdominal pain +/- hx of bloody stools. Can see hypoalbuminemia and anemia on labs. Lesions are continous. Rx: 5-ASA (first line), steroids or immunomodulators (6MP, MTX, cyclosporine). Use Flagyl if infection suspected. Colectomy will eliminate risk for cancer (cancer rate is 20% per decade after first 10 yrs of disease). Peutz-Jeghers syndrome: mucosal pigmentation of lips and gums, multpile polyps. Cancer is biggest complication. Rx: removing polyps. IBD: Extra-intestinal: arthritis, mucocutaneous lesions, liver disease. Extra-Intestinal: pydoderma gangrenosum of feet, erythema nodosum (red tender nodules over shin), arthritis, Uveities, Liver disease, renal stones. Crohn’s: Can initially p/w weight loss. See aphthus ulcers and perianal fistulas. Skip lesions on Xray, cobblestone on endoscopy (also non caseating granulomas). RX: don’t change long term course only decrease morbidity: Steroids, aminosalicylates, immunomodulators, abx, nutrition. Question: You are evaluating a 10 y/o M who just moved to the US from Africa. He reports fever and abdominal pain of 2 weeks duration. PE reveals an ill appearing boy who has a temp of 103, resp rate of 40, hepatomegaly, tenderness of RUQ. Abdominal sono shows a liver mass c/w an abscess. Of the following, the most likely etiologic agent is: A. Ascaris lumbricoides B. Entamoeba histolytica C. Strongyloides stercoralis D. Teania solium E. Wuchereria bancrofti Answer: A. Ascaris lumbricoides B. Entamoeba histolytica C. Strongyloides stercoralis D. Teania solium E. Wuchereria bancrofti Explanation: Intestinal amebiasis causes weight loss, fever and severe bloody diarrhea with abdominal pain – can progress to fulminant colitis or toxic megacolon. Noninvasive intestinal infection results in vague, nonspecific abdominal complaints. Extraintestinal disease occurs most commonly as liver abscess: causes fever, tachypnea and tender hepatomegaly. Transmission occurs via ingestion of amebic cysts. Hirschsprung’s Disease: Think of this for any significant defecation problem (intermittent loose stools or constipation) in a newborn, especially male. Usually diagnosed before age 2. Presents with failure to pass meconium in the hospital, bilious vomiting, poor PO and abdominal distension. DDx: anal stenosis – infant strains to pass small liquid stools, tight narrowing of anus. Self limited and remits by age 1. Functional constipation- will not mention delayed passage of meconium, no signs of obstruction, no FTT, + soiling, can present after age 2. Congenital hypothyroidism: also p/w poor growth, hoarse cry, umbilical hernia and delayed closure of ant fontanelle. Rectal biopsy is diagnostic. Rx: surgical excision of aganglionic segment (primary or secondary repair). Question: A 15 y/o F presents with an episode of “feeling faint” and melena. On PE, you note a gallop rhythm and mild, nonspecific abdominal tenderness. Stoll is guaiac positive. Labs demonstrate anemia (Hct of 18%). You give fluid resuscitation and PRBCs, and the patient’s hemodynamic status stabilizes. Of the following, the next MOST appropriate diagnostic test is: A. angiography B. barium contrast upper GI C. doppler ultrasonography of portal and esophageal veins D. upper gastrointestinal endoscopy E. video capsule study Answer: A. angiography B. barium contrast upper GI C. doppler ultrasonography of portal and esophageal veins D. upper gastrointestinal endoscopy E. video capsule study Explanation: Upper GI bleeding (proximal to ligament of Treitz) when acute p/w melena and hemodynamic instability. When chronic p/w anemia. UGI endoscopy remains the initial diagnostic test of choice b/c it is highly sensitive for mucosal lesions and also allows endoscopist to treat bleeding lesions. Common causes UGI bleeds: gastric or duodenal ulcers, chronic gastritis, esophageal or gastric varices, reflux esophagitis. Vascular lesions (AVM or telengectasias) can also occur. Question: A 3 y/o child p/w history of intermittent painless rectal bleeding. Approximately once or twice/week she passes a formed stool that contains up to a “teaspoon” of blood. PE demonstrates no fissures or hemorrhoids. Hematocrit and coag studies are normal. The bleeding persists despite stool softeners. Of the following, the test that is MOST likely to establish a diagnosis is: A. Colonoscopy B. computed tomogroaphy scan of abdomen C. Meckel scan D. magnetic resonance angiography E. stool culture Answer: A. Colonoscopy B. computed tomogroaphy scan of abdomen C. Meckel scan D. magnetic resonance angiography E. stool culture Explanation: Small volume painless rectal bleeding that persists despite stool softeners is most consistent with a colonic polyp. No signs of systemic illness to suggest an infection. Colonoscopy is most likely to identify a polyp. A radionuclide scan can reveal a meckel diverticulum, but usually these present with large-volume rectal bleeding. Absence of fever or cramping argues against stool infection. Abdominal CT and MRI sometimes are useful in bleeding lesions, but not indicated until polyp is ruled out. Occult blood may arise from anywhere in GI tract. In contrast, visible maroon or bright red blood usually arises from the distal small bowel or colon. Although constipation is probably the most common casue of rectal bleeding, patients who have constipation usually produce hard stools with small amount of blood on surface of stool. Hemoirrhoidal bleeding usually results in blood on the toilet paper but not on the stool. With colitis, patients generally have significant abdominal pain, esp with defecations. Painless rectal bleeding is generally caused by anatomic rather than inflammatory lesions. Meckel diverticulum is an extra piece of intestine, typically located in distal ileum, that can ulcerate and cause large-volume painless rectal bleeding. Colonic polyps can be single or multiple and can be removed at colonoscopy. GI bleeding: Nasogastric lavage is the first thing you should do to distinguish upper from lower GI bleeding. Lower GI bleed in Neonates: Apt test determines if the blood is the mother’s or infant’s. Consider HD, malrotation with volvolus and NEC Anal fissures are most common cause Intussusception typically presents in 9 m/o+. Juvenile polyp (painless rectal bleeding in otherwise healthy child). No increased risk of malignancy. Entamoeba histolytica can cause bloody diarrhea. 90% of patients with amebic colitis have + serology. Rx with Flagyl. Meckel Diverticulum: etopic gastric mucosa, 2-3% of newborns, manifests during first 2 years of life, 2 inches in length, 2 feet from ilecocecal valve. Diagnosed by technetium -99m pertechnetate scintigraphic study. Treat surgically. Lower GI bleed in 1-2 y/o Lower GI bleed in 2-5 y/o: Lower GI bleed in School aged: Same as preschoolers +: IBD Question: A 4 y/o M presents to clinic for a second opinion. He has a 3 week hx of diarrhea, abdominal pain, and tenesmus. Parents think he is getting worse and nobody has helped, despite testing his poop. His stool output has increased from 4-5/day to 8-10/day during the past week. He is now febrile to 102. They are starting to see blood in the toilet after he poops. The boy was in good health until 1 week ago when he returned from a fishing trip on the Amazon river. PE reveals a moderately ill-appearing boy who has diffuse abdominal pain. During exam, he passes a very foul smelling stool that appears to be mixture of stool and pus, which you send to lab. Of the following, the MOST appropriate next step is: A. abdominal ultrasound B. barium enema C. colonic biopsy D.gallium scan E. liver function test Answer: A. abdominal ultrasound B. barium enema C. colonic biopsy D.gallium scan E. liver function test Explanation: Boy described has Entamoeba histolytica– presents within 2 weeks of infection with colicky abdominal pain and diarrhea. Amebic colitis is esp common in children <5 y/o and can produce toxic megacolon, fulminant colitis, ulceration of colon, and more rarely bowel perforation. In severe cases amebiasis can become systemic or cause a liver abscess. In this case, since so ill appearing, need Abdominal sono to r/o liver abscess. Dx by seeing trophozoites or cysts in stool. Colonic bx can diagnose, but not at this stage of illness LFTS can still be normal in the presence of liver abscess Gallium scan may help find liver abscess, but sono is more cost effective and easier. Barium enema isn’t indicated secondary risk of perforation RX: Flagyl, followed by iodoquinol or paromomycin. Question: You are evaluating a 1 month old term infant who has persistent jaundice. The parents explain that his stools were green 2 weeks ago, but are now pale yellow. PE findings are unremarkable, except for a liver that is palpable 2 cm below the costal margin. The infant’s total bili is 6.1 and direct bili is 4.2. ALT is 240, AST is 160. A hepatobiliary iminodiacetic acid (HIDA) nuclear medicine scan demonstrates absence of excretion of tracer into the bowel. Of the following, the MOST definitive diagnostic test to establish the diagnosis is: A. intraoperative cholangiography B. magnetic resonance cholangiopancreatography C. measurement of serum alpha-1-antitrypsin D. sweat chloride test E. ultrsonography of biliary tree Answer: A. intraoperative cholangiography B. magnetic resonance cholangiopancreatography C. measurement of serum alpha-1antitrypsin D. sweat chloride test E. ultrsonography of biliary tree Explanation: The initial evaluations of an infant who has a history of persistent jaundice: review perinatal hx for blood group incompatibility, CBC, total and direct bili. In this case, there is a direct hyperbilirubinemia c/w neonatal cholestatic syndrome. Cholestasis is present if conjugated (direct) bili is >2 or if conjugated fraction exceeds 20% of total serum bili. In infants with neonatal cholestasis, must rule out biliary atresia. If HIDA doesn’t show excretion into the bowel – most reliable test to exclude or diagnose biliary atresia is intraoperative cholangiogram. At same time, surgeon will perform a wedge biopsy to evaluate hepatic histology more carefully. Differential often very dependent on history and presentation. Biliary atresia is very likely in a healthy term infant p/w with direct hyperbili and acholic stools. But would be less likely in a preterm infant who has been on parenteral nutrition x 6 weeks. US and MRI can be helpful, at this time they are not sensitive enough to rule out biliary atresia definitevely. Sweat test and alpha 1 AT are useful for the evaluation of cholestasis, but not to r/o biliary atresia. Alagille syndrome: intrahepatic cholestasis, posterior embryotoxon of the iris, vertebral anomalies, peripheral pulmonic stenosis and typical facies (prominent forehead, pointed chin, hypertelorism) Labs needed: liver chemistries and liver function (albumin, coags) Liver – Cholestatic Jaundice: Presents with elevated direct bilirubin, acolic stools and hepatomegaly. Is caused by liver/parenchymal disease or anatomical/obstructive disease Intervention is required to prevent severe liver disease Hepatbiliary scintigraphy is good first step. Isotope is taken up and makes its way to the biliary system. With obstruction, theere will be uptake in the liver but no excretion down the biliary tree. Biliary Atresia: Will describe an elevated direct bilirubin in a child over 1 m/o. First test should be an ultrasound, followed by HIDA scan and ultimately a biopsy. Rx: Kasai procedure – essentially joins the liver to the intestine. Remember that most common cause of cholestatic jaundice in newborn is TPN. Can use Alk Phos to help distinguish cholestatic jaundice (increase AP) from hepatocellular jaundice (high ALT/AST). Gilbert Syndrome: Due to glucuronyl transferase deficiency Causes intermittent elevated serum bili (especially at times of illness and stress). Often describe family history Reye’s Syndrome: Will mention: recent URI during which aspirin was given. Typically presents to the ICU, sometimes comatose, with elevated LFTs and ammonia levels. Wilson’s Disease: Secondary to excess copper – which deposits in different tissues. Eyes Kayser Fleisher rings Liver Brain Kidney RTA Rx: D-penicillamine (remember copper pennies). Penicillamine can cause aplastic anemia. Hepatitis A: Fecal oral transmission Best dx: IgM specific Ab (IgG levels persist for life, so not useful for recent dz). IgM Abs remain elevated x 6 months. Prevalent in areas of poor hygiene and sanitation P/w flu like sx, elevated LFTs, and usually travel to endemic area 90% of children < 5 y/o have asx infection and will not be jaundiced. Hepatitis B: Transmission via blood, sex or perinatally Hep B is a major cause of morbidity and mortality in the US (and rest of world) The earlier the age of infection, the higher the incidence of chronic HBV (90% for infants, 10% for adults). Fulminant hepatic failure and hepatocellular cancer are worst complications. Serologies: Acute: HBsAg + (x 6 months) is earliest indicator of acute infection, IgM anti HBc +, HBeAg indicates high viral load (most infective), HBV DNA marks viral replication. Recovery: Disappearance of HBV-DNA and HBsAg (around 6 months). Anti-HBS appears, anti-HBc and anti-HBe. Window period: anti-HBc Chronic infection: persistence of HBsAg beyond 6 months. Can be there for life in chronic carriers. Immunization: anti-HBsAg Hepatitis C: Transmission via blood and sex (perinatally too). Hep C associated with liver Cancer and Cirrhosis. Most common bloodborne infection in the US Most common cause of chronic viral hepatitis Hepatitis D: Virus can not replicate on its own, requires presence of HBsAg to provide its outer coat D stands for Dependent Can be infected with or after Hep B Can be associated with chronic hepatitis (rarely) and cirrhosis Hepatitis E: Fecal oral transmission Most common in parts of Asia, Africa and Mexico Associated with exposure to contaminated water Does not lead to chronic hepatitis Pancreas: Pancreatitis presents with mid epigastric pain radiating to the back – guarding, rebound and decreased BS. Abdominal U/S, not serum amylase, is the most specific test for diagnosis. ERCP is used to follow recurrent pancreatitis Normal amylase does not rule pancreatitis – lipase is more specific test for pancreatic disease ** Question: A 16 y/o F presents with 4 month history of RUQ abdominal pain. The pain occurs at different times, but seems to strike primarily after meals, more frequently after she eats fatty foods. In your office, she c/o intermittent pain to deep palpation of the RUQ. CBC, ALT, alk phos, bili, amylase and lipase are normal. Abdominal ultrasound shows no evidence of stones or gallbladder thickening. Upper endoscopy and biopsy results are normal – with no evidence of ulcers or gastritis. Of the following, the MOST appropriate step is: A. abdominal computed tomography scan B. endoscopic retrograde cholangiopancreatography (ERCP) C. nuclear medicine gallbladder emptying scan with fatty meal D. psychiatric consultation to rule out depression or anxiety E. referral to an acupuncturist for chronic pain management Answer: A. abdominal computed tomography scan B. endoscopic retrograde cholangiopancreatography (ERCP) C. nuclear medicine gallbladder emptying scan with fatty meal D. psychiatric consultation to rule out depression or anxiety E. referral to an acupuncturist for chronic pain management Explanation: Colicky abdominal pain in RUQ after fatty foods is very suggestive of gallbladder disease. With all tests being normal, chronic acalculous cholecystitis with gallbladder dysmotility should be considered. Need radionuclide gallbladder emptying scan. If patient has marked delay in GB emptying, consider cholecystectomy. Chronic acalculous cholecystitis = GB dysmotility. Patients p/w RUQ pain after meals, but labs and sono findings are normal. Up to 90% improve after cholecystectomy. Consider ERCP or abdominal CT if pancreatic disease is suspected. Classically cholecystitis occurs when GB is inflamed and irritated by gallstones. Stones made of cholesterol or pigment (bilirubin). RF for cholesterol stones: older age, female sex, pregnancy, overweight RF for pigment stones: parenteral nutrition and hemolysis Cholecystitis: Jaundice is a presenting sign in > ¼ of children with cholecystitis. Also p/w fatty food intolerance, fever, pain radiating to right scapula and a palpable mass in the RUQ. Dx: U/S Predisposing conditions: Hemolytic disease Prolonged use of TPN Small intestinal disease Obesity or pregnancy