[CIS PIDD] [cis-pidd] Hyper Ig E is susspected
CIS-PIDD
cis-pidd at lists.clinimmsoc.org
Tue Jul 12 14:58:14 EDT 2016
I was wondering if you could help me with this patient, whom Hyper Ig E is
susspected. I wolud like to get the gene study in order to confirm this
patology at my patient.
Thanks!
Age: 1 year 11 months
*I. **CURRENT ILLNESS*
Time of disease: 1 year
Liver disease (fibrosis)
*II. **BACKGROUND:** Recurrent pneumonias*
· *Pathological: *
1. 20 days: Pneumonia + Infected dermatitis
2. 4 months: Pneumonia (7 days)
· 4 months and 20 days: Urinary tract infection, atopic dermatitis
3. 6 months : Pneumonia + acute diarrhea + CMV pneumonia (PCR:
negative) + hyper IgE syndrome, hepatic cirrhosis ( hepatic biopsy:
fibrosis grade II ) + failure to thrive
4. 1year 4 months : Hepatic cirrhosis, Hyper IgE syndrome, duodenal
lymphangiectasy.
· *Family*: No contributory
*III. **PHYSICAL EXAMINATION: *
*Weight:* 7,08 Kg Height*:* 68 cm
*Abdomen:* Globular, RHA (+), hepatic spam 12 cm., palpable spleen 13 cm.
*IV. **EVOLUTION:*
Cytomegalovirus (serological clinical diagnosis) treated with
valganciclovir. Immunoglobulin starts August 25, 2015.
*V. **TESTS AND PROCEDURES: *
*HEPATIC BIOPSY (07/22/15):*
· Micronodular Cirrhosis. Chronic inflamation process and
scarce eosinophils:
+++ does not pass the limiting.
· DUDENITIS NODULAR BIOPSY: Duodenum: 10 EOSINOPHILS / 40X
.Intestinal lymphangiectasy.
*LAB*
· Molecular study quantitative PCR for CMV : Negative (2 occasions).
· LKM -1 ANTIBODY : Negative
--
*Atte *
Wilmer Córdova
*Inmunología Alergología*
*Instituto Nacional de Salud del Niño - Perú*
http://www.isn.gob.pe
wcordova at insn.gob.pe
facebook.com/wilmer.cordova.71
twitter: @wilmer_cordova
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