Survey
* Your assessment is very important for improving the workof artificial intelligence, which forms the content of this project
* Your assessment is very important for improving the workof artificial intelligence, which forms the content of this project
Patient 1 – acute abdomen • 19 year old male – no prior medical history • Just back from a trip to Israel • Two days of “writhing in pain”, fever to 102.5 – Similar episode about a week ago in Israel resolved • Pediatrician – acute abdomen, wbc 30,000 • CT – pelvic abscess, inflamed small and large bowel, inflamed Ileum Questions • In an acute presentation, how does one differentiate between: – Perforated appendix – Newly presenting Crohn’s with perforation – Other causes of pelvic abscess • How do you determine which patients warrant emergency surgery, vs. which should be “cooled off” medically? • What is your protocol for medical management prior to surgery? Management 1. Abscess drained by IR 2. One week in ICU 3. IV antibiotics (Piperacillin/ Tazobactam) 3. NG suction to decompress 4. IV hydration 5. Pain medication 6. Parenteral nutrition 7. Re-imaging after 10 days Questions • What additional evaluation would you perform at this time to establish a Crohn’s diagnosis? • Would you consider treating this patient medically? – Short term vs. long term? – With what agent? • If you choose surgery, what is the appropriate time to intervene? – Laparoscopic vs. open? “Abdominal phlegmons in Crohn's disease: outcomes following antitumor necrosis factor therapy” • Thirteen patients with abdominal phlegmon treated with antibiotics and anti-TNF - 2004-2010 – 12 patients also had abscess • Anti-TNF with antibiotics – Two patients underwent surgery within a year • “Penetrating CD complicated by phlegmon formation may be safely and effectively managed with a combination of antibiotics and anti-TNF therapy” • Selection bias? Cullen et al, IBD Journal 2013 Management and outcome • Hospitalized with PN, NPO, decompression and medical management for approximately 3 weeks. • EGD/Colonoscopy – no gastric or colonic disease • Laparoscopic ileocecectomy – Fibrotic ileum and cecum – Residual abdominal abscesses – 30 cm of ileum plus cecum resected • Uneventful recovery Postoperative management • How would you monitor patient postoperatively? – Clinical follow-up and labs only? – Small bowel imaging? – Colonoscopy (timing) • What (if any) postoperative medical therapy would you utilize ? – Aminosalicylate – Thiopurine – Infliximab Postoperative management and outcome • Discussion with family and patient • Opted for mercaptopurine – Transaminitis developed – Changed to low dose 6MP and allopurinol • Surveillance 1 year after initial presentation – Normal MRI, normal colonoscopy • No clinical or laboratory recurrence 3 years after his initial surgery. – Follow up colonoscopy planned Presenting history • 7 year old female • Presented with Serratia osteomyelitis at 3 months of age • History of multiple GI issues in infancy – Poor weight gain requiring NG tube – Diarrhea – Rectal bleeding as infant while breast-fed, responsive to mother eliminating milk from diet. • Diagnosis of CGD by dihydrorhodamine test Chronic granulomatous disease Winkelstein et al 2000; Medicine 79:155 • Primary immunodeficiency characterized by inability of cells to kill bacteria and fungi. – Staphylococcus, Aspergillus cause serious infection – Catalase positive organisms – Pneumonia most common infection (80%) • 75% of patients present under age 5 years • Autosomal recessive or X-linked • GI manifestations – Colitis present in approximately 20% – Perianal abscess – Gastric outlet obstruction • Diagnosis – defective NADPH oxidase – Dihydrorhodamine test Dihydrorhodamine test Children’s Hospital of Wisconsin Neutrophils given DHR dye, activated Normal neutrophils produce superoxides that oxidize DHR, resulting in fluorescence. Chronic granulomatous disease • • • • • Mimics Crohn disease on endoscopy and histology Granulomas in only 34 % (Levine, Histopathology 2005) Paucity of neutrophils compared to UC (Shappi JPGN 2003) Decreased CD68+ macrophages (Liu et al, IBD Journal 2009) Therapy – gamma-IFN, steroids, thalidomide, SCT Clinical course • Continued with diarrhea, anemia for over a year despite therapy. – Labial abscess grew E. coli • By age 4: – – – – 3 BM daily, no bleeding Height at 25% ile, but falling off Colonoscopy with ileal and colonic granulomas Therapies included • Alpha - Interferon • Bactrim and fungal prophylaxis Medical options for this colitis • • • • • • • Aminosalicylates Antibiotics Probiotics Corticosteroids Azathioprine GM-CSF Anti-TNF agents Medical options for this colitis • • • • • Aminosalicylates - intolerant, got diarrhea Antibiotics – vancomycin for C. difficile Probiotics – VSL 3 Corticosteroids – recurrent courses Azathioprine – tried for over 6 months – unable to wean off steroids without worsening • GM-CSF • Anti-TNF agents? Caution with anti-TNF in CGD! • NIH published experience with 5 patients – All with active colitis despite thiopurines – Two deaths from opportunistic lung disease • Infliximab effective for GI disease (including fistulizing disease), but severe infectious complications developed – B. cepacia – CMV colitis – S. aureus liver abscess Potential options • GM-CSF – Wang et al, J . Allergy Clinical Immunology 2005 • Anakinra – Rationale – upregulation of IL-1 beta pathway – van de Veerdonk et al, Netherland Journal of Medicine 2011 • Stem cell transplant – Controversial in CGD – No matched siblings in this case. Current outcome • Anakinra 200 mg daily for 3 weeks – Azathioprine stopped • Diarrhea improving with 2 stools daily • Prednisone weaned to 6 mg daily • CRP improved from – 2.2 mg/dL to 0.9 mg/dL • Plan for 3-6 months of Anakinra, the reevaluate with colonoscopy Fever in CD Patient on 6-MP Presenting history • 18 year old male with CD • Maintained on 6-MP for four years with stable remission and mucosal healing • Headache, fever and malaise • Leukopenia and elevated transaminases • No lymphadenopathy or HSM • EBV IgM+ • EBV PCR 291 copies/mcg DNA Options • Additional diagnostic tests? • What to do with 6-MP? • Follow-up? Clinical course • Initially improved • Within a few weeks fevers & chills returned • Became jaundiced Options • Additional diagnostic tests? • Differential diagnosis? Diagnostic Tests • Hyponatremia, pancytopenia, elevated bilirubin and transaminases • Increased EBV PCR • Elevated sIL2R, ferritin • Abdominal ultrasound: massive HSM • Diagnosis? • Other tests? Diagnostic Tests • EGD/colon: multiple gastric polypoid lesions with path consistent with EBV+ lymphoma • Liver biopsy: EBV+ lymphoma infiltrate • Bone marrow: hemophagocytosis • PET-CT: widespread Discussion • What to do with 6MP with febrile illness/mono? • RISK of EBV associated HLH or lymphoma with 6-MP? • Different for primary EBV infection versus reactivation? • Monitoring for this?