[CIS PIDD] [cis-pidd] Rib abnormalities in CGD !!!

[CIS-PIDD]

Dear Sagar,

I'm no expert, but abnormal DHR is reported also in SAPHO/Majeed ( Ferguson
PJ, Lokuta MA, El-Shanti HI, Muhle L, Bing X, Huttenlocher A. Neutrophil
dysfunction in a family with a SAPHO syndrome-like phenotype. Arthritis
Rheum. 2008;58:3264–9 )
elevated IgE and eosinophilia, and hepatomegaly are described as well
The ribs abnormalities could be the hyperostosis
cutaneous rash...

Best wishes,

Boaz

---

Boaz Palterer, MD
University of Florence
Dept. of Clinical and Experimental Medicine
Allergology and Clinical Immunology
email - boaz.palterer at gmail.com



On Wed, May 17, 2017 at 4:36 PM, CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
wrote:

> Sorry for the typo error...
> CD3 is 58%
>
> DHR repeatef twice..... and is abnormal
> NBT is also abnormal.
>
> Will look for activation markers
>
> Thanks
>
> On 17 May 2017 8:01 pm, "CIS-PIDD" <cis-pidd at lists.clinimmsoc.org> wrote:
>
>> There are many features in this patient that seem unusual for CGD – might
>> be worth repeating the DHR test.  There also seems to be a significant
>> deficit of lymphocytes if you add CD3, CD19, and CD56 – perhaps some type
>> of Omenn syndrome?  Have you looked at activation markers on T cells – MHC
>> Class II etc?
>>
>>
>>
>> Prescott
>>
>>
>>
>> T. Prescott Atkinson, MD PhD, Professor and Director
>>
>> Division of Pediatric Allergy, Asthma & Immunology
>>
>> University of Alabama at Birmingham
>>
>> Tel: 205-638-6457 <(205)%20638-6457>
>>
>> Fax: 205-975-7080 <(205)%20975-7080>
>>
>>
>>
>>
>>
>> *From:* cis-pidd at lyris.dundee.net [mailto:cis-pidd at lyris.dundee.net] *On
>> Behalf Of *CIS-PIDD
>> *Sent:* Wednesday, May 17, 2017 7:56 AM
>> *To:* CIS-PIDD <cis-pidd at lyris.dundee.net>
>> *Subject:* [cis-pidd] Rib abnormalities in CGD !!!
>>
>>
>>
>> Dear all
>>
>>
>>
>> I am Dr Sagar Bhattad, a clinical Immunologist at Bangalore, India.
>>
>>
>>
>> I need your expert opinion in the following case
>>
>>
>>
>> 4 months old boy
>>
>> Born of consanguineous wedlock
>>
>> Failure to thrive (birth weight 2.7 kg and at 4 months - 2.8 kg)
>>
>> Oral thrush
>>
>> Erythematous rash over trunk, face and limbs
>>
>> Loss of hairs over eyelids
>>
>> Hepatosplenomegaly, lymphadenopathy
>>
>>
>>
>> Investigations
>>
>> Absolute lymphocyte count - 3450
>>
>> Absolute eosinophil count - 9200
>>
>>
>>
>> CD3  33%
>>
>> CD19 23%
>>
>> CD56 10%
>>
>> CD4  26%
>>
>> CD8 7%
>>
>>
>>
>> IgG 568
>>
>> IgM 1094
>>
>> IgA  < 40
>>
>>
>>
>> IgE > 2500
>>
>>
>>
>> NBT - no reduction
>>
>>
>>
>> DHR - oxidative index 4.2 and control 102
>>
>>
>>
>> Abnormal NBT and DHR strongly suggest Chronic granulomatous disease
>>
>>
>>
>>
>>
>> *Other issues*
>>
>> Ribcage abnormality ------ bilateral several ribs look abnormal and are
>> fused at places
>>
>>
>>
>> *Query*
>>
>> My clinical diagnosis in this child was *Omenn syndrome*. But abnormal
>> NBT and DHR make me think of *CGD. *
>>
>>
>>
>> Can experts please opine on the diagnosis and the link with rib
>> abnormalities
>>
>>
>>
>> Warm regards
>>
>> Sagar
>>
>>
>>
>>
>>
>>
>>
>>
>>
>>
>>
>>
>>
>>
>> *Dr.Sagar Bhattad*
>>
>> MD Pediatrics, DM Pediatric Clinical Immunology and Rheumatology (PGI,
>> CHD)
>>
>> *Giannina Gaslini Institute, Italy (Observership in Rheumatology)*
>>
>> Consultant, Pediatric Immunologist and Rheumatologist, ASTER CMI
>> Hospitals, Bengaluru
>>
>>
>>
>> *Aster CMI Hospital, No 43/2, International Airport Rd, Sahakara Nagar,
>> Bengaluru -560 092, Karnataka*
>>
>> * (**Book appointment - 08043420100**)*
>>
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