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Weight loss, Night sweats & Diarrhea Imported from the Ukraine
Dr. Yael Weintraub
Pediatric Gastroenterology Unit
Dana-Dwek Children’s Hospital
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• A.N, ♂, 14 y.o., generally healthy, Ukraine citizen.
• Admitted d/t general deterioration, night sweats, weight loss, and watery diarrhea.
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l 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.
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l Past Medical Hx:
• Family Hx- ø
• Past illness- ø.
• Permanent medical Tx- ø
• Immunization- according to age.
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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.
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l 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
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l 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.
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l PICU Treatment:
• Empiric Abx: Tazocin + Garamycin .
• Packed Cells X2.
• Nutritional & electrolytes support.
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DA
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l Differential Diagnosis:
• Malignancy
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l Imaging :
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l Imaging- Bone marrow aspiration:
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l Differential Diagnosis:
• Malignancy
• Infectious disease
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l Infectious workup:
• Stool cultures- neg. • Stool parasites-neg. • Stool CDT –neg. • Stool Rota Ag- neg. • Criptosporidium- neg. • Stool strongiloides PCR-neg.
• HAV IgM -0.18 (neg.<0.8) • HBsAg- 0.13 (neg<1) • HCV Ab- 0.13 (neg<0.8)
• 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
• Toxocara canis, leishmania,
strongyloides- pending.
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l Differential Diagnosis:
• Malignancy
• Infectious disease
• Autoimmune disease- IBD
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Varices
Gastroscopy:
Chronic gastritis, HP neg.
Chronic & erosive duodenitis,
Esophageal Varices grade 1
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l Colonoscopy:
Normal small bowel - Pancolitis Chronic active colitis- IBD type,
m/p UC
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l Autoimmune/IBD workup:
• RF- <11.50 (0.0-15.0)
• C3- 1.23 (0.8-1.6)
• C4- 0.24 (0.15-0.4)
• TTG-1.1, EMA-neg.
• cANCA- neg.
• pANCA-neg.
• ASCA IgA- 21.0 (0.0-18.0)
• ASCA IgG- 20.0 (0.0-18.0)
• IgG 34.4(H)
IgM 1.62(N)
IgA 4.15(H)
• Anti LKM- neg.
• Anti SM (+) 1:40
• ANA- neg.
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Masson Dye
Liver Biopsy:
Cytokeratin 7 Dye
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.
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l Final Diagnosis:
1. Pancytopenia- non malignant, non-infectious
2. Ulcerative Colitis (pancolitis)
3. Primary Sclerosing Cholangitis
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DA
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DA
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l 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.
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l Albendazole Metabolism in the liver
Albendazole
Albendazole sulphoxide
albendazole sulfone +oxidative metabolites
extensive 1st pass metabolism
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l 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
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l 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.
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DA
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l UC + PSC
Susp Filariasis Albendazole Tx.
Pancytopenia
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l Labs on Dismissal:
• CBC: Hb-9.1 WBC- 13.7 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.