[CIS PIDD] [cis-pidd] Auto-inflammatory Patient?

[CIS-PIDD]

IgD was normal. EBV PCR was undetected.

On Mon, Jun 20, 2016 at 2:34 PM, CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
wrote:

> Worth considering mevalonate kinase deficiency? With all those EBV
> antibodies, does he have any blood EBV DNA?
>
>
>
> Anthony Hayward
>
>
>
> *From:* CIS-PIDD [mailto:cis-pidd at lists.clinimmsoc.org]
> *Sent:* Monday, June 20, 2016 10:31 PM
> *To:* CIS-PIDD <cis-pidd at lyris.dundee.net>
> *Subject:* [cis-pidd] Auto-inflammatory Patient?
>
>
>
> All,
>
>
>
> We are currently caring for a 5 year old boy with fevers x 2 months and
> worsening respiratory status.
>
>
>
> He was in good health until he had a dental appointment. The next day, he developed
> fevers to 103, a right ear rash, pain and swelling of his feet, diffuse
> muscle pain, decreased energy and PO intake. He was admitted to an OSH from
> 4/6/16 - 5/5/16. Had extensive workup during the hospitalization which was
> negative for infection and auto-immunity. The pt was empirically started on
> high dose steroids (on which he improved) and discharged to home with plan
> to taper steroids slowly over time and follow up with PCP and rheumatology.
>
>
>
> Mom reports that as soon as she started to taper the steroids, he worsened
> again- with fevers and jaundice. He was then admitted to our hospital and
> found to have transaminitis, ascites, peritonitis, pancreatitis, persistent
> fevers, tachycardia, and tachypnea .
>
>
>
> Yesterday, he was transferred to the PICU for continued fevers and
> respiratory failure requiring intubation. So far, no source has been found
> for fevers and respiratory distress.
>
>
>
> Her work-up has included:
>
>
>
> Ferritin- highest of 1133 on 4/20 now down to 333
>
> CBC- just slight normocytic anemia
>
> AST high of 1368 on 5/2 now down to 482
>
> ALT high of 743 on 5/2 now down to 104
>
> GGT high of 1431 on 5/20 now down to 394
>
> LDH high of 707 on 5/2,
>
> Triglycerides- high of 514 on 5/16, now down to 161
>
> EBV ab to nuclear Ag IGG 4/7: >600 (high)
>
> EBV ab to viral capsid ag IGG 4/7: 291 (high)
>
> EBV ab to viral capsid ag IGM 4/7: 12.4
>
> Viral respiratory panel- negative
>
> ANA- negative
>
> ESR- high of 78 on 4/28 now down to 68
>
> C3 : 124
>
> C4: 22.6
>
> IgA: 257 (high)
>
> IgG: 1160 (high)
>
> IgM: 114 (nml)
>
> Lymphocyte subsets: normal except low CD56 cells
>
> Soluble IL-2R: 1462
>
> Perforin/granzyme- normal
>
> Glycogen storage disease work-up- normal
>
> BAL- negative
>
> Chest CT- Bilateral extensive groundglass/consolidation which is most
> characteristic for diffuse alveolar damage/acute lung injury
>
>
>
> Abdomen CT- Enlarged, hypoattenuating liver. Interval decrease in now
> moderate volume ascites, partially surrounded by thick/enhancing walls
> compatible with organizing, loculated fluid. Superimposed infection is not
> excluded.
>
>
> Edematous appearing pancreas with normal enhancement relative to the liver
> and spleen.
>
>
>
> Blood/CSF/urine cxs- negative
>
>
>
> He is currently on solumedrol 1mg/kg Q12 and his condition is continuing
> to deteriorate requiring more oxygen and ventilator support.
>
>
>
> A cytokine panel has been sent out and is pending.
>
>
>
> Any other ideas or considerations would be greatly appreciated.
>
>
>
> Thanks.
>
>
>
> -Evan
>
>
>
>
>
> *Evan Shereck, MD*
>
> Associate Professor of Pediatrics
>
> Director, Pediatric Hematology/Oncology Fellowship
>
> Block Co-Director, Blood and Host Defense
>
> Oregon Health & Science University I  3181 SW Sam Jackson Park Rd, Mail
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>
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