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

Tue Jun 28 13:56:31 EDT 2016

As he hasn't emergency or any other sympton than fever i would not start
anti IL1.

If necessary, my suggestion is to start colchicine (in Brazil our best
brand is 'colchis'  but in other places the French - opocalcium - brand is
the best) at a 1,0 to 1,5 mg/day. We control response to colchcine with
fevers and acute reactants.

Wait for genetic test (NOD 2 mutation ?). We had an FMF patient just with
fever and positive persistent calprotectin with normal colonoscopy
(visceral omentum inflammation?).

leonardo

2016-06-27 21:40 GMT-03:00 CIS-PIDD <cis-pidd at lists.clinimmsoc.org>:

>
> 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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-- 
*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/ Ambulatório de Doenças Autoinflamatórias

*Leonardo Oliveira Mendonça,MD*
Departament of Clinical Immunology and Allergy  at Hospital das Clínicas,
School of Medicine - University of São Paulo
Private Healthcare Unit/ Autoinflammatory Disease Unit

email: *leonardo.mendonca at hc.fm.usp.br <leonardo.mendonca at hc.fm.usp.br>*
telefones/phone number: +55-11-26619571/ +55-11-26617825/ FAX: 011-2661 8173

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