Hepatic abscesses in a 14 yearold boy

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Hepatic abscesses in a 14 year-old boy

• Division of Infectious Diseases
• Seattle Childrens Hospital and Regional Medical
  Center
• Fred Hutchinson Cancer Research Center, Seattle
  WA
• YaeJean Kim, MD

Acknowledgement Dr. Steve Pergam
2
Presentation

• CC abdominal pain and fever

• HPI
• A 14 year old male with intermittent abdominal
  pain for 2 weeks in his mid-epigastric area
• Fevers developed and abdominal pain continued.
• No change of abdominal pain with eating, no
  associated nausea, vomiting, diarrhea or
  constipation. No rectal bleeding or other GI
  complaints.

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Presentation (cont)

• Seen by primary care physician
• RUQ pain on exam and elevated WBC count
• Abdominal ultrasound was performed.

4
Physical Exam and History

• Vitals Tm 40.0 P 90-100, BP 112/63
• Physical exam was positive for
• Abdomen Tender in RUQ, 1 cm liver edge, no
  splenomegaly
• Skin multiple areas of folliculitis on face,
  chest, back and arms, and post-auricular area.
• PMH and FH were significant for
• Multiple episodes of swimmers ear, URI,
  sinusitis, and otitis media as a child. Acne and
  skin infections.
• Sister (19 yo) with history of multiple skin
  infections.

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Presentation (continued)

• Lab
• Normal LFTs
• CRP 15.4 , ESR 98
• WBC 12 (84 polys / 12 lymphs / 4 bands)
• Started empirically on Pip/Tazo, Metronidazole,
  and Vancomycin
• CT scan of abdomen was performed

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CT Scan
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Disease course

• Oxidative burst test was consistent with CGD.
• Cultures grew Staphylococcus aureus (MSSA).
• Initial drainage -gt partial right hepatectomy,
  incision and debridement of multiple abscesses in
  both lobes
• Switched to Nafcillin and discharged home.
• Itraconazole prophylaxis was started
• Continued Nafcillin for 4 month, switched to
  Bactrim and Rifampin with clinical improvement.
  IFN-? treatment was also added.
• Sister was also diagnosed with CGD.
• Genetic test revealed c.75_75delGT mutation in
  exon 2 of the NCF1 gene (p47)

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Chronic Granulomatous Disease

• Defect in the oxygen metabolic burst machinery
• Mutations in genes that encode subunits of
  phagocyte NADPH oxidase gp91-, p22-, p47- and
  p67-phox
• Characterized by greatly increased susceptibility
  to severe bacterial and fungal infections
• X-linked (2/3) and Autosomal Recessive

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TX of Hepatic abscess in CGD

• CGD abscesses dense, septated masses with a
  fibrous pseudocapsule and thick inspissated fluid
• Surgery vs drainage vs antibiotics vs combination
  therapy
• Two of the largest studies recommend surgery
• 69/168 patients with hepatic abscesses from 1978
• 61 hepatic abscesses treated with surgery and
  antibiotics (1986-2000) with multiple
  interventions needed

Johnston BB, Newman SL. Chronic Granulomatous
Disease. Pediatr Clin North Am
197862365-376 Lublin, M, et al. Hepatic
Abscess in Patient with Chronic Granulomatous
Disease 2002235383-91
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Strategies to decrease infections

• Review in 1990 of TMP/SMX prophylaxis decrease
  in bacterial infections (7.1 to 2.4 per 100
  patient months) Margolis DM. J Infect Dis.
  1990162(2)723-6
• IFN-? decrease rates of infection in a
  randomized control trial of 128 patients (14 vs.
  30 had serious infections) International CGD
  Cooperative Study Group. NEJM. 1991324509-16
• Itraconazole prophylaxis decreased risk for
  invasive fungal disease (1 vs. 7)
• Gallin JI. NEJM. 20033482416-22