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

Mon Jun 20 17:53:02 EDT 2016

I still think its infection..lung, omentun/livre bipsy?!

Dr Leonardo Oliveira Mendonça

Médico do Serviço de Imunologia Clínica e Alergia Hospital Das Clinicas,
Faculdade de Medicina da Universidade de São Paulo

Unidade Convênios e Particulares

Leonardo Oliveira Mendonça,MD

Departament of Clinical Immunology and Allergy  at Hospital das Clínicas,
School of Medicine - University of São Paulo

Private Healthcare Unit

email: leonardo.mendonca at hc.fm.usp.br

telefones/phone number: +55-11-26619571/ +55-11-26617825/ FAX: 011-2661 8173

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
Code: CDRCP  I  Portland, OR 97239

(Office): 503-494-0829  I  (Fax): 503-494-0714

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