[CIS PIDD] autoimmune disorder

[Yeşim Yılmaz Demirdağ]

Hi Wilmer,

has HIV been ruled out?
any lung involvement?

Y

On Tue, Jul 24, 2012 at 8:53 PM, Wilmer Cordova Calderon <
wilmer.cordova at gmail.com> wrote:


> 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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