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

[CIS-PIDD]

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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