Page 1 of 1: Diagnosis????

sea_corel · Credits: 11961 My Scrapbook My Reading List 7 Books

A 9 month old child was referred for further management of her pneumonia. At 6 months of age, she was admitted with fever, cough and breathlessness and diagnosed to have bilateral bronchopneumonia and treated with IV antibiotics (amoxicillin –Sublactum), oxygen and nebulization for 5 days to which she clinically responded. However X-Ray Chest worsened and was given Cefaperazone & Linezolid for 14 days. Repeat X-Ray Chest after 14 days was the same and hence 3 drug Antituberculous therapy (AKT) started. However after 20 days, patients began to get fever and X-Ray Chest further worsened. At that time she was tested for HIV which was negative. Patient was started on Vancomycin, Fluconazole and Levofloxacin at that time and AKT was stopped. Autoimmune work up done was SSA +ve, SSB +ve, SCl – 70 +ve. Patient started on steroids. After 10 days, patient showed a clinical improvement. On presentation to us, she had bilateral crepitations, had failure to thrive (Weight = 5.75 kg, Height = 66 cm). Her investigations done are as follows:
Hemoglobin = 10.5 gm/dl
WBC = 18,100/cumm (P30 L66 M2)
Platelet count = 5,17,000/cumm
HRCT of chest = Bilateral diffuse consolidation
Serum immunoglobulins = Hypergammaglobulinemia
CD4/CD8/CD8/CD19/CD16/CD56 = normal
Sweat chlorides = normal
BAL awaited

She was treated with Erythromycin & 4 drug was AKT started.

Whats the Diagnosis?? and explanation for it.

sabkhan · Credits: 2782 My Scrapbook

Okay this is a tough one but let me try out!!

Hx and investigations suggestive of an autoimmune disease especially when its a female child but the question is which one it is.

As SSA and SSB are positive in sjogren syndrome while SCl-70 positive in scleroderma...... looks like a mixed connective disease to me which causes restrictive pulmonary disease.

But have never heard of connective tissue diseases presenting so early in life and aggressively.

Please give your comments.

sea_corel · Credits: 11961 My Scrapbook My Reading List 7 Books

i dont know the ans,as i too got it forwarded from my friend..so posted here seeking if anyone can tell me the right ans..
many DD are given i will list them.....

Atypical pneumonia
Sjorgon's synd
Connective tissue disorder
alpha-1-antitrypsin deficiency
....so me too cant ans this question.people here are requested to go throuh it and give their FEEDBACK ..

astrocles · Credits: 3953 My Scrapbook

as per the auto immune profile- MCTD seems likely;

but the clinical picture and the choice of antibiotics covering pseudomonas nd staph makes it seem like cystic fibrosis or alpha-1 antitrypsin deficiency; in which these org r likely to affect; esp keeping the age at presentation;

and kawasaki's is another dz, i'd like to rule out!
looking at thrombocytosis...and age of presentation, and fever with infections;and steroids being used as a treatment option.............

my take.........

shall think abt it.....

any more views.........???????///////

astrocles · Credits: 3953 My Scrapbook

any more takes?????????????????/////////////

zolt · Credits: 9181 My Scrapbook My Reading List 40 Books

A real tough one.

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