Weight loss, Night sweats & Diarrhea Imported from the Ukraine Dr. Yael Weintraub Pediatric Gastroenterology Unit Dana-Dwek Children s Hospital
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1 Weight loss, Night sweats & Diarrhea Imported from the Ukraine Dr. Yael Weintraub Pediatric Gastroenterology Unit Dana-Dwek Children s Hospital
2 A.N,, 14 y.o., generally healthy, Ukraine citizen. Admitted d/t general deterioration, night sweats, weight loss, and watery diarrhea.
3 Chief Complaint: Last month - general deterioration, extreme weight loss, night sweats. At onset- short episode of fever, diarrhea & vomiting. Last year- watery diarrhea X 6/day, few bloody episodes. Appetite intact, no vomiting. Recent Diagnosis Filariasis, Tx- Albendazole. R/O- oral ulcers, arthralgia / arthritis, skin rash.
4 Past Medical Hx: Family Hx- ø Past illness- ø. Permanent medical Tx- ø Immunization- according to age.
5 Physical Examination: HR 109, BP 97/27, Temp 37.8 Alert and oriented, extremely pale, no jaundice, cachectic, diffuse hair loss. HEART- normal heart sounds, systolic murmur 2/6. LUNGS- decreased breath sounds RLL, clear to auscultation. ABDOMEN - Splenomegaly, ascites LYMPH NODES- None detected.
6 Labs on Admission: CBC- Hb:5.3, WBC:700, ANC:100, PLT:85000 Na 128, K 3.1, Mg 1.34, P 2.98, Ca 7 AST 21, ALT 31, ALK 397 GGT 185 Total Bili 0.9, Dir. Bili 0.7 Albumin 25 ESR 140, CRP 128 Clotting functions- PT 14.9, INR 1.42, PTT 39.2
7 Problem List: 1. Weight loss >10%. 2. Night sweats. 3. Watery diarrhea. 4. Spleenomegaly & ascites. 5. Pancytopenia 6. Cholestatic abnormal liver functions 7. Prolonged INR. 8. Hypoalbuminemia 9. Elevated ESR/CRP.
8 PICU Treatment: Empiric Abx: Tazocin + Garamycin. Packed Cells X2. Nutritional & electrolytes support.
9
10 Differential Diagnosis: Malignancy
11 Imaging :
12 Imaging- Bone marrow aspiration:
13 Differential Diagnosis: Malignancy Infectious disease
14 Infectious workup: Blood culture- neg. HIV- neg. TB workup*-neg. CMV IgM-neg. IgG-pos. EBNA- pos. Measels IgG- pos. IgM-neg. VZV IgG-pos. IgM-neg. Parvovirus PCR neg. ASLO- 400 Stool cultures- neg. Stool parasites-neg. Stool CDT neg. Stool Rota Ag- neg. Criptosporidium- neg. Stool strongiloides PCR-neg. HAV IgM (neg.<0.8) HBsAg (neg<1) HCV Ab (neg<0.8) Toxocara canis, leishmania, strongyloides- pending.
15 Differential Diagnosis: Malignancy Infectious disease Autoimmune disease- IBD
16 Gastroscopy: Chronic gastritis, HP neg. Chronic & erosive duodenitis, Esophageal Varices grade 1 Varices
17 Colonoscopy: Normal small bowel - Pancolitis Chronic active colitis- IBD type, m/p UC
18 Autoimmune/IBD workup: RF- <11.50 ( ) C ( ) C ( ) TTG-1.1, EMA-neg. canca- neg. panca-neg. ASCA IgA ( ) ASCA IgG ( ) IgG 34.4(H) IgM 1.62(N) IgA 4.15(H) Anti LKM- neg. Anti SM (+) 1:40 ANA- neg.
19 Liver Biopsy: Fibrosis of the Portal tracts. Bile ducts show atrophy and degeneration. Mild ductal lymphocytic infiltrate. No interface hepatitis. Finding are compatible with PSC stage 3-4. Masson Dye Cytokeratin 7 Dye
20 Final Diagnosis: 1. Pancytopenia- non malignant, noninfectious 2. Ulcerative Colitis (pancolitis) 3. Primary Sclerosing Cholangitis
21
22
23 Albendazole-Mechanism of Action: A broad-spectrum antihelmintic, highly effective against a wide range of intestinal helminths. Mechanism of action: exhibits larvicidal, ovicidal and vermicidal activity, via inhibition of tubulin polymerization. This causes a cascade of metabolic disruption, including energy depletion, which immobilizes and then kills the susceptible helminth.
24 Albendazole Metabolism in the liver Albendazole extensive 1 st pass metabolism Albendazole sulphoxide albendazole sulfone +oxidative metabolites
25 Albendazole-Adverse Reactions: Dermatologic : alopecia, skin rashes, erythema multiforme Stevens-Johnson syndrome Gastrointestinal : Abdominal pain, diarrhoea, nausea, vomiting Hematologic : Low red cell count, leucopenia, pancytopenia, aplastic anaemia and agranulocytosis Hepatic : elevated liver enzymes, hepatitis, acute liver failure. Immunologic : Hypersensitvity. Neurologic : Dizziness, headache, symptoms associated with treatment for neurocysticercosis. Ophthalmic. Renal : Proteinuria Others: Bone pain and fever
26 Warnings and Precautions: Mild to moderate elevations of liver enzymes have been reported with albendazole. Elevations of liver enzymes increase risk of hepatotoxicity and bone marrow suppression. Can cause bone marrow suppression, aplastic anemia, and agranulocytosis in patients with or without underlying hepatic dysfunction.
27
28 UC + PSC Susp Filariasis Albendazole Tx. Pancytopenia
29 Labs on Dismissal: CBC: Hb-9.1 WBC PLT-309 SMAC: AST- 43, ALT- 36, ALP-587, GGT- 181 Albumin-34 Nutrients: low iron, vit D, vit A, vit B12. Further Work up: MRCP+ MRE. Tx: Rafasal, Ursolit, Multivitamins.
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