[CIS PIDD] autoimmune disorder

[Wilmer Cordova Calderon]

Dear collegues more data about de disorder autoinmune probably. We dont
have cytometry and cant do linfos subpoblation


comorbidities:

   1. hypertension
   2. hypokalemia
   3. hemolytic anemia


treatment:

   1. hydrocortisone 160mg/m2
   2. fresh plasma


labs:
Coombs 1/2: positive
Hb: 9.2 then 6.2
Reticulocytes: 2.9
Ex Urine: 100/campo erythrocytes, glucose 2 +, Leucos: 35/campo
Rheumatoid factor: 198 (very high)
ESR: 50 (high)
Uric acid: 6.4 (high)
PCR: 3.7 (high)
DHL: 1126 (very high)
C3 41 (declined)
C4 3.6 (greatly diminished)
Triglycerides: 333 (very high)
Albumin: 2 (decreased)
Skin biopsy: compatible with photodermatitis??
CK: 116 (normal range)
CK MB: 33 (high) (under 25)
TSH: 10.4 (high)
GGTP: 1408 (very high)
Renal ultrasound: Ascites and bilateral nephropathy
IgA: 128 (high), IgG: 842 (normal), IgM: 254 (high)



2012/7/22 Wilmer Cordova Calderon <wilmer.cordova at gmail.com>


> Writing and attached information for a case that has no definite diagnosis

> and appeal to their experience in patients with autoimmune background and

> perhaps a primary immunodeficiency disorder.

>

> In our medical board concluded that it biopsiará kidney, skin and pulse

> methylprednisolone is used in addition use of gamma globulin

> (immunomodulatory)

>

> If you have suggestions I am ready to discuss them well with my colleagues

>

> Thank you for your response and time

>

> Atte

>

> Wilmer Cordova

>

> Allergy immunology

>

> Lima Peru

>

>

> Case Report

>

> *I. PATIENT INFORMATION*

>

> AGE: 1 year 2 months

>

> SEX: Female

>

> SERVICE: Infectious Diseases

>

> * *

>

> *II. REASON FOR ADMISSION*

>

> 2 weeks episodes of rising thermal unquantified PAPULOERITEMATOSAS INJURY

> IN FACE AND IN TRUNK, productive cough and weakness. Increase

> papuloeritematosas lesions predominantly on face and trunk, liquid stools s

> / ms / s (submitted), lesions erythematous in oral cavity and gums.

> Hospitalization for persistent febrile episodes.

>

> *III. BACKGROUND:*

>

> *- PERSONAL:*

>

> Hospitalized

>

> Dx 06/03/2012: Sd febrile Sd  edematous, Sd peural, Diffuse Liver Disease

>  Acute mucocutaneous candidiasis, Erythema Multiforme, Hemolytic Anemia.DC

> child LES, DC congenital immunodeficiency.

>

> *LAB EXAMS:*

>

> PCR 4,

>

> Clumps Tifico O 1/320 Tifico H 1/40

>

> Ex Urine: 2-3 Leuc xc, Protein +, RBC 4-6xc

>

> TORCH IgM Toxoplasma 12.87,

>

> C3 40, C4 8

>

> FR 23.8

>

> ANA 1/320, Anti native DNA +, T Coombs + Ret 4%

>

> IMAGES:

>

> Eco Abd: Hepatomegaly, nephromegaly mild bilateral renal inflammatory

> process.

>

> Rx thorax mild left pleural effusion.

>

> TREATMENT: Methylprednisolone, Prednisone 08/04/12 to 06/07/12 2mg/K/d of.

>

>

>

> *IV. PHYSICAL EXAMINATION OF INCOME:*

>

> Pale, with TCSC increased Cushinoide facies, erythematous papulopustular

> lesions on the face and trunk

>

> DRCD hepatomegaly to 4 cm.

>

>

>

> *V. EVOLUTION*

>

> Get as treatment: Dexamethasone 2 mg c/8h, Azithromycin 100 mg C/24 h,

> Sucralfate 150 mg w / 6 h, Ranitidine 20 mg every 12 h

>

>

>

> *VI. DIAGNOSIS:*

>

> 1. Persistent Fever Syndrome

>

> 2. DC Acute Toxoplasmosis

>

> 3. DC Autoimmune Disease: Systemic Lupus Erythematosus Child

>

> 4. Congenital immunodeficiency vs. Primary

>

> 5. Exogenous Cushing syndrome.

>

>

>

> *VII. AUXILIARY TESTS:*

>

> CBC                       (07/07/2012)                     (13/07/12):

>

> Leukocytes        11000                                   8400

>

> A                            1%                                         5%

>

> S                             57%                                       65%

>

> L                             57%,                                     30%

>

> Hct                         32%                                       29%

>

> Hb                          10.4                                       9.4

>

> PLAQ.                   200000                 200000

>

> Ret 0.8%

>

> Glucose: 85

>

> Urea: 64

>

> Creatinine 0.63

>

> Biochemistry (7/14/12):

>

> DHL: 1528

>

> TGO: 555

>

> TGP: 126

>

> FA: 864

>

> Uric Ac: 8.7

>

> Ca 7

>

> P 4.9

>

> Mg 1.5

>

> Total Protein: 4.2

>

> Albumin: 1.9

>

> Globulins: 2.3.

>

> CPK, CK 116,

>

> CK-MB 33.4.

>

> C3 24

>

> C4: 3.6

>

> ASO: 15

>

> FR: 198.3

>

> Hematology: TC 5min TP TS 2 min 14.4 "APTT 46" TT 24.8 "135 Fibrinogen

>

> ESR 58

>

> Serology (7/10/12):

>

> Toxoplasma IgM 2041

>

> Coprocultivo (7/10/12): Campylobacter

>

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