[CIS PIDD] [cis-pidd] Febrile 7 month old boy

[CIS-PIDD]

I thank you in advance as I am struggling with this case and could use your
expertise.  This is my first posting and I apologize for it being so very
long.







Pt is a 7 month old male who presents with nearly constant fevers
of unknown origin. He was born full term, uncomplicated pregnancy, only
child in the family, and from unconsanguinous parents.  Fevers started at
2-3 weeks of age and have been ongoing with at most a few days to a week
between febrile episodes.  There is no discernable pattern; fevers range
from 101-103F and do respond to antipyretics.  He has no consistent
symptoms with his fever other than being slightly fussy (no vomiting,
respiratory symptoms, rash, etc.).  He did have mild truncal hypotonia a
few times and, during 1 fever, an episode of upper extremity tremor.  He
has crossed percentiles for weight from birth to 4 months, but has been
tracking curve appropriately and current weight is 98% of IBW.  He was
exclusively breast fed until 2 weeks ago when mom began occasionally
supplementing with formula and adding oat to thicken.



Fevers have resulted in 3 hospitalizations and extensive workup.  No source
or cause has yet been found.



*Most Noteable Laboratory Workup (abnormal values in red):*

   -

   2 weeks of age (inpatient):  CBC/diff normal; CMP with ionized calcium
   1.34 (nl = 1.12-1.32); UA clear; RSV negative; CRP normal at 0.6;
   negative urine culture, blood culture, stool culture, and LP
   -

   4 weeks of age (inpatient): CBC/diff normal; negative urine culture,
   blood culture; bone scan normal
   -

   2.5 months:  CBC/diff with platelet count elevated at 548; normal IgG,
   IgA, IgM
   -

   4 months: CMV quantitative normal; lymphocyte subset panel normal; CMP
   with calcium 11.1 (nl = 9-11); CBC with platelets 631,000; ESR elevated
   at 24, CRP elevated at 6.71
   -

   5 months:  CRP elevated at 7.4; parvovirus PCR negative; EBV PCR
   negative; IgG, IgM, IgE, and IgA normal; IgD <1; nasopharyngeal swab/viral
   respiratory panel by PCR—negative for all viruses, Bordetella,
   Chlamydophilia and mycoplasma; Quantiferon TB Gold – negative; Fecal
   calprotectin elevated at 391; cocci, toxo, parvo, EBV – negative; CMV
   PCR- negative; ferritin normal; CH50 normal; ANA negative
   -

   6 months (inpatient):  CBC with low WBC of 3,600, low Hgb 10.1, low ANC
   of 1000, and low ALC of 2.0; lymphocyte subsets with low CD3 1348, CD4 931,
   CD8 366; CMP normal; CK 134 and 177 (H); LDH 488 (H); ferritin 132 (nl); TSH
   6.16 (H); FreeT4 1.24 (nl); C3 77; C4 18; CRP  <0.3; Sweat chloride -
   nl; peripheral smear with mild normochromic/normocytic anemia, reactive
   appearing lymphocytes, and borderline thrombocytosis with large platelets
   -

   6.25 months:  CBC with low Hb of 9.0 and elevated platelets of 833; normal
   eye exam
   -

   6.5 months:  Quantiferon TB Gold – negative; HIV Ab negative; Fecal
   Elastase – normal; lymphocyte enumeration normal with no activated T cells
   and normal switch memory B cells; elevated IL2R of 1808 (normal 406 to
   1100); Bone marrow biopsy normal though anemia of chronic disease
   (normal iron sat and ferritin, but low iron saturation) and a reactive
   thrombocytosis, without evidence of HLH
   -

   7 months (this week):  planning to send fever gene panel; GI planning to
   EGD and flex/sig



*Radiology:*

   -

   Ultrasound abdomen complete- Normal exam
   -

   Echocardiogram - normal
   -

   Brain MRI - normal
   -

   Bone scan - normal
   -

   CXR normal
   -

   PET normal except for slightly increased splenic uptake



As above, I am planning to send the fever gene panel this week.  I am not
sure on starting any treatment (IL-1 antagonist for example) as I don’t
know what I’m treating!



Sincerely,



*Cindy Salm Bauer, MD*

Co-Director, Eosinophilic Gastrointestinal Disease Clinic

Division of Allergy and Immunology, Department of Pediatric Pulmonology

Phoenix Children's Hospital

1919 East Thomas Road / Phoenix, AZ  85016 / Tel:  602-933-4063

cbauer at phoenixchildrens.com



Clinical Assistant Professor, Department of Child Health,

University of Arizona College of Medicine

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