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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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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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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.