A Young Man with Chronic Abdominal pain

1. A Young Man with Chronic Abdominal pain Aaron DeVries Interhospital Case Conference 3/29/05

2. 20 yo M Chronic Abdominal Pain Presenting to an ER 24 months of chronic intermittent abdominal pain Currently R sided extending to R Flank In past had been LUQ and RUQ Waxes and wanes but severe for the last 2 days Occurred from weekly to daily lasting for several hours each time No alteration in pain with eating, urination, defecation No Fevers/chills, cough, dysuria, hematuria, diarrhea, constipation, melena, hematochezia

3. PMHx – none SHx – refugee from Sudan, arrived in Twin Cities about 1 year ago 20 yo M Chronic Abdominal Pain

4. BP 110/78 P 76 RR 12 Afeb Thin uncomfortable, lying or sitting HEENT, PULM, CV – nml Abd tender in RLQ, extending to R flank U/A 0 WBC, 5 RBC CBC diff, lytes normal KUB - No calcifications observed 20 yo M Chronic Abdominal Pain

5. CT Abd w/o contrast Moderate dilation of the R collecting system with moderate to severe dilation of the ureter to the level of the distal ureter. Smooth linear high attenuation lesions of the mid and distal portions of the ureter which could represent calcification and dilated to 1.3 cm R distal ureter obstruction – small stone in distal ureter vs. congenital obstruction

6. What would be your next test? Any Thoughts?

7. Cystoscopy Yellowish, sandy appearing patch on the trigone as well as within the R ureter

8. Bilharzia a.k.a Schistosomiasis Theodore Bilharz was German anatomist teaching in Cairo. In 1850 connected death following hematuria with the parasite. Died 1852 of typhus

9. Schistosomiasis Life Cycle Vertebrates are the principle final hosts Reproduce exclusively in humans except S. japonicom Fresh water snails are required

13. Species and Location

16. Swimmers itch from invasion of cercaria into skin Acute Schistosomiasis a.k.a Katayama fever Onset of the female parasite laying eggs ~ 5 weeks after infection Fever, Nausea, Headache Hepatosplenomegaly Leukocytosis with eosinophilia Few weeks – months S. japonicum > S. mansoni and S. haematobium

17. Occurs with Granuloma formation surrounding eggs Hepatosplenic Schistosomiasis - S. mansoni and S. japonicum Several years after initial infection Granulomas form within the liver and biliary tree chronic hepato-obstructive disease portal hypertension – liver failure Intestinal Schistosomiasis - S. mansoni and S. japonicum Several years after initial infection Small and large intestine may be involved Adults migrate to intestinal wall Lay eggs which migrate into intestinal lumen and out into stool Severe anemia from chronic GI blood loss

18. Vesico-ureteral - S. haematobium Several years after infection Adults migrate to small venules around the bladder and ureter Eggs are deposited into surrounding tissue and penetrate out into bladder Causes calcifications where eggs are trapped Characterized by hematuria Leads to squamous-cell cancer of bladder

19. 200 million infected worldwide 80-85% of persons in Sub-Saharan Africa are infected In Egypt, squamous-cell carcinoma of the bladder accounts for 18-28% of all cancer Incidence of 10.8 per 100,000 persons Smoking increases risk even further Infected children have substantial growth delay even after treatment Co-infection with Hep B and C results in a much faster progression to liver failure

20. Pt referred to ID clinic for treatment PMHx – fell from a tree as a child with possible fractured ribs on R side SHx – Native of Darfur region of Sudan. At approximately the age of 15 his village was overrun by Sudanese government forces. His family was killed in front of him. He was captured and was enslaved by the Janjaweed. He was given no food or water. After 4 years of enslavement he escaped by train into Kartun where he contacted relatives in Cairo. They assisted him to Egypt and eventually was accepted as a refugee to the US facilitated by MN relatives

21. ID Clinic PE: nml vitals ENT, Pulm, CV – exam nml ABD mildly tender in RUQ Urine and stool w/o parasitic elements Treatment Praziquantal 20mg/kg x 2 doses separated by 8 hours

22. Treatment Praziquantal Kills all forms except schistosomula - migrating form 3-21 days Cure rate 60-90% Emerging resistance in Egypt and Kenya reported Oxamniquine and Metrifonate are alternatives Re-treatment – same drug and dose If shedding eggs at 4-6 weeks If symptoms do not resolve If anti-schistosomal antibody remains elevated.

23. He had significant decrease in abdominal pain at 4 weeks No schistosomal eggs seen in urine or stool At six weeks had near complete resolution of symptoms Anti-schistosomal Ab – 5.8 (nml <1) Follow Up

24. Serologic prevalence at a Sudanese refugee reunion in Arizona in 2004 464 participated in the laboratory testing. 204 (44%) seropositive for S. mansoni or S. haematobium 227 (49%) seropositive for strongyloides 103 (22%) seropositive for both 315 (69%) were seropositive for either 88% of + were treated (unclear whether remainder were previously treated or refused treatment) Recommendations – presumptive treatment of all “Lost Boys and Girls of Sudan” - All Sudanese refugees? Praziquantel 20 mg/kg x 2 doses 6-8 hours apart Albendazole 400 mg BID for 3 days.