Good Morning! Thursday, February 5, 2015

Transcription

1 Good Morning! Thursday, February 5, 2015

2 Prep! A 12-year-old boy is brought to the emergency department by emergency medical services after sustaining a lower leg injury sliding into home plate during a baseball game. He tells you that he thinks his leg twisted when he slid. He reports that he had immediate pain in his right ankle and has been unable to walk since the injury occurred. Prior to transport, paramedics splinted his right lower leg. On physical examination, he has significant swelling and ecchymosis around his distal tibia and fibula. Following the administration of analgesia, radiographs are obtained. Of the following, the MOST likely complication of this injury is A. Avascular necrosis of the distal tibia B. Osteochondritis desiccans C. Osteomyelitis D. Tibial growth arrest E. Unicameral bone cyst

3 HPI:

4 What is diarrhea? When does a patient have diarrhea? Stool volume >10g/kg/day in infants and toddlers >200g/day in older children Is it chronic? 14 days of symptoms Is it persistent? >14 days but with more abrupt onset

5 Physical Exam: Vitals: Gen: HEENT: CV/Resp: Abd/GU: Ext: Neuro:

6 What s going on? Without Failure to Thrive With Failure to Thrive

7 What s going on?* Without Failure to Thrive Chronic Nonspecific diarrhea of Childhood or infancy Infectious colitis Lactose malabsorption Small bowel bacterial overgrowth IBS With Failure to Thrive Intractable diarrhea of infancy Allergic enteropathy Celiac disease IBD Immunodeficiency state* Congenital secretory diarrhea Tufting enteropathy Microvillous inclusion disease Autoimmune enteropathy Neuroendocrine tumors CF Hirschsprung disease Factitious Diarrhea*

8 What would you like to do?

9 CHRONIC DIARRHEA Let s start with some ridiculous humor: What s brown and sticky? A Stick!

10 Epidemiology 2-4 million childhood deaths worldwide/year 25% of cases present for medical care in US <1% present for diarrheal illness Rotavirus vaccine decreased hospitalization by 66% Osmotic Secretory Dysmotility-associated Inflammatory Pathophysiology

11 History & Exam Stool frequency, volume, appearance +/- blood or mucous Relationship to feeding or dietary intake Abdominal pain, Weight loss Rash, fatigue, emesis, joint aches, oral ulcers Recent travel, exposure to new water sources, family history, sick contacts Growth charts! Signs of nutrient deficiencies Abdominal exam/rectal exam

12 Workup* Stool studies! Electrolyte content of stool Leukocytes in stool CBC with RBC characteristics ESR/CRP TTG IgA ADEK Imaging Endoscopy

13 What s the cause? Chronic Nonspecific Diarrhea of Childhood Small Bowel Bacterial Overgrowth Infectious Colitis Irritable Bowel Syndrome Disaccharide Intolerance Intractable Diarrhea of Infancy Allergic Enteropathy Celiac Disease Inflammatory Bowel Disease Immunodeficiency Microvillous Inclusion Disease Congenital Secretory Diarrhea Autoimmune Enteropathy Tufting Enteropathy Neuroendocrine Tumors Hisrchsprung Disease Cystic Fibrosis Factitious

14 Disaccharide Intolerance aka lactose malabsorption 70% of world s adult population has 1 acquired deficiency* Hispanic, Asian, African American children symptomatic prior to 5 yoa White children after age 5 yoa, (adolescence) Secondary deficiency SI mucosal injury after lactase enzyme is lost from tips villi Incompletely digested lactose reaches colonic microbes fermented to hydrogen and other gases gassy discomfort and flatulence with bloating* Nonabsorbed lactose acts as osmotic agent diarrhea Non-bloody stools Diagnostics- hydrogen breath concentration after ingestion*

15 What s the cause? Chronic Nonspecific Diarrhea of Childhood Small Bowel Bacterial Overgrowth Infectious Colitis Irritable Bowel Syndrome Disaccharide Intolerance Intractable Diarrhea of Infancy Allergic Enteropathy Celiac Disease Inflammatory Bowel Disease Immunodeficiency Microvillous Inclusion Disease Congenital Secretory Diarrhea Autoimmune Enteropathy Tufting Enteropathy Neuroendocrine Tumors Hisrchsprung Disease Cystic Fibrosis Factitious

16 Celiac Immune-mediated enteropathy occurring in setting of gluten ingestion in those sensitive Prevalence in adults and children approaching 1% worldwide Classic pediatric triad: FTT, diarrhea, abdominal distension* Seen less frequently now Dx- Anti-endomysial IgA antibodies,* near 100% specificity, or enzyme-linked immunosorbent assay-based anti-ttg IgA antibodies Confirmed with histo* of duodenum of villous blunting and prominent intraepithelial lymphocytes Treatment: Avoid wheat, rye, barley*

17 What s the cause? Chronic Nonspecific Diarrhea of Childhood Small Bowel Bacterial Overgrowth Infectious Colitis Irritable Bowel Syndrome Disaccharide Intolerance Intractable Diarrhea of Infancy Allergic Enteropathy Celiac Disease Inflammatory Bowel Disease Immunodeficiency Microvillous Inclusion Disease Congenital Secretory Diarrhea Autoimmune Enteropathy Tufting Enteropathy Neuroendocrine Tumors Hisrchsprung Disease Cystic Fibrosis Factitious

18 Irritable Bowel Syndrome Recurrent abdominal pain/altered bowel habits, considered functional History often gives you the diagnosis! Alternating constipation with diarrhea Abdominal pain relieved with defecation No rectal bleeding, weight loss, fever, anemia ROME III criteria: Abdominal pain or discomfort at least 3 days per month in the last 3 months associated with 2 or more of the following: a) improvement with defecation, b) onset associated with a change in frequency of stooling, and c) onset associated with change in the form of the stool. Must rule out celiac disease! Treatment?

19 What s the cause? Chronic Nonspecific Diarrhea of Childhood Small Bowel Bacterial Overgrowth Infectious Colitis Irritable Bowel Syndrome Disaccharide Intolerance Intractable Diarrhea of Infancy Allergic Enteropathy Celiac Disease Inflammatory Bowel Disease Immunodeficiency Microvillous Inclusion Disease Congenital Secretory Diarrhea Autoimmune Enteropathy Tufting Enteropathy Neuroendocrine Tumors Hisrchsprung Disease Cystic Fibrosis Factitious

20 Infectious Colitis Affects all ages & usually an acute presentation with short course, but Rotavirus may cause diarrhea up to 20 days Salmonella- detected for up to 5 weeks, and may be excreted up to 1 year E coli, campylobacter, aeromonas Enterpathogenic E coli leading cause of chronic diarrhea in developing countries Yersinia- may mimic appendicitis or ileal Crohn s disease because it can affect terminal ileum; must seek out specifically Giardia*/Crypto* Contaminated water/exposure to undercooked meat +/- blood and/or mucous, +/- fever and/or abdominal pain Positive stool culture, ova and parasite exam, stool antigen test

21 What s the cause? Chronic Nonspecific Diarrhea of Childhood Small Bowel Bacterial Overgrowth Infectious Colitis Irritable Bowel Syndrome Disaccharide Intolerance Intractable Diarrhea of Infancy Allergic Enteropathy Celiac Disease Inflammatory Bowel Disease Immunodeficiency Microvillous Inclusion Disease Congenital Secretory Diarrhea Autoimmune Enteropathy Tufting Enteropathy Neuroendocrine Tumors Hisrchsprung Disease Cystic Fibrosis Factitious

22 Inflammatory Bowel Disease Crohn s and Ulcerative Colitis Mean age of diagnosis 12.5 years Industrialized nations Family History* 25% of children who develop IBD have a reported family history 1 st degree relative with CD/UC results in 10x higher risk of developing IBD Crohn s: crampy, diffuse of focal RLQ abdominal pain; nonbloody, melanotic or frank blood; weight loss*; fevers; recurrent ulcers* UC: diarrhea, rectal bleeding, abdominal pain +/- fecal urgency; malaise; +/- low-grade fevers or weight loss* Both: Left-sided colonic inflammation nocturnal diarrhea & urgency

23 What s the cause? Chronic Nonspecific Diarrhea of Childhood Small Bowel Bacterial Overgrowth Infectious Colitis Irritable Bowel Syndrome Disaccharide Intolerance Intractable Diarrhea of Infancy Allergic Enteropathy Celiac Disease Inflammatory Bowel Disease Immunodeficiency Microvillous Inclusion Disease Congenital Secretory Diarrhea Autoimmune Enteropathy Tufting Enteropathy Neuroendocrine Tumors Hisrchsprung Disease Cystic Fibrosis Factitious

24 Chronic Nonspecific Diarrhea* (of Childhood or Infancy) The most common form of persistent diarrhea in the first 3 yrs May last until age 5 years 4-10 loose bowel movements per day No blood or mucus Stool pattern? Stools passed only during waking hours Begins with large formed stool and become watery and smaller in volume as day progresses Undigested food remnants seen in stool Weight and height maintained Typically have normal appetite and activity level Due to increased motility and osmotic effects of intraluminal solutes Treatment?*

25 Allergic enteropathy (eosinophilic enteropathy) Vomiting and diarrhea + Failure to Thrive*** Must distinguish from allergic colitis Healthy and thriving infant Induced by food proteins Cow s milk and soy SI mucosal damage malabsorption of protein, carbs, and fat May lead to hypoalbuminemia and diffuse swelling Lethargy and dehydration may mimic sepsis Serum IgE Remove the inciting dietary protein

26 Our Patient s Results

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28 Ulcerative Colitis vs. Crohn s Rectal bleeding Usual Sometimes Abdominal pain Common Common Malaise, fever, weight loss Common Common Perianal disease Rare Common* Ileum involved None Common Strictures Rare Common Fistulas Rare Common Skip lesions - + Transmural - + Granulomas Rare Common Crypt Abscesses Usual Variable Risk of cancer * Cobblestoning - + Ulceration of IC valve - + Rectal sparing +/- +

29 Extra-intestinal Findings 1/3 develop extra-intestinal manifestations May occur before intestinal sxs Arthralgias/arthritis Pauciarticular arthritis disease correlates with intestinal activity Ankylosing spondylitis Typically UC Associated with HLA-B27 Bone findings Osteopenia/porosis BMD seen in 25% of patients before steroids started Aseptic necrosis Eyeballs Uveitis, corneal ulceration, retinal vascular damage

30 Extra-intestinal Findings Erythema nodosum More common in Crohn s Tender, warm, red nodules or plaques localized to the extensor surfaces Pyoderma gangrenosum More common in UC Associated w/ extensive colonic involvement Lesions: discrete pustules with surrounding erythema deep ulceration with well-defined border and deep color

31 Extra-intestinal Findings Primary sclerosing cholangitis (PSC)* More common in UC patients Increased GGT and Alkaline Phosphatase Cholangiography and liver biopsy help confirm diagnosis Increases risk of cancer Nutritional Deficiencies* Anemia (Folic acid and B12 deficiency) Vitamin D deficiency Hypocalcemia (related to low Vit, low albumin) Zinc deficiency

32 Treatment Proper nutrition Low residue diets or special formulas* TPN if severe disease and malnourishment Medications* Corticosteroids Budesonide 5-ASA (UC) Immunomodulators (AZA, 6-MP, MTX) Biologic therapy, monoclonal Ab (Infliximab - Remicade) Antibiotics (metronidazole, cipro for fistulas) Surgery* Crohn s-disease complications UC- may be curative

33 When to Admit Severe Colitis Fever >5 bloody stools/day Hypoalbuminemia Anemia Toxic megacolon Occurs in up to 5% of adults with UC At risk for perforation Treatment* Bowel rest TPN IV steroids Careful monitoring

34 Have a great day! Noon Conference: Class meetings- 3 rd yrs in 2-center, 2 nd yrs in 2031, 1 st yrs in 2034 Students off