[CIS PIDD] [cis-pidd] Mannose Binding Lectin Deficiency
CIS-PIDD
cis-pidd at lists.clinimmsoc.org
Thu Feb 9 10:58:52 EST 2017
Thank you everyone for all your help and input!
I will update you as soon as I have more information!
Thank you
pamella
On Thu, Feb 9, 2017 at 10:52 AM, CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
wrote:
>
> I meant Primary Ciliary Dyskinesia, sorry.
>
>
>
> Kathleen A. Haines, M.D.
>
> Section Chief, Pediatric Immunology
>
> Section of Pediatric Rheumatology & Immunology
>
> Joseph M. Sanzari Children’s Hospital
>
>
>
>
> 30 Prospect Ave., Hackensack, NJ 07601
>
> T: 551-996-5306 <(551)%20996-5306> | F: 201-996-9815 <(201)%20996-9815> |
>
> Kathleen. Haines at HackensackMeridian.org
>
> HackensackMeridianHealth.org <http://hackensackmeridianhealth.org/>
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> *From:* CIS-PIDD [mailto:cis-pidd at lists.clinimmsoc.org]
> *Sent:* Thursday, February 09, 2017 7:48 AM
> *To:* CIS-PIDD <cis-pidd at lyris.dundee.net>
> *Subject:* RE: [cis-pidd] Mannose Binding Lectin Deficiency
>
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> *From:* CIS-PIDD [cis-pidd at lists.clinimmsoc.org]
> *Sent:* Wednesday, February 08, 2017 11:16 PM
> *To:* CIS-PIDD
> *Subject:* Re: [cis-pidd] Mannose Binding Lectin Deficiency
>
> Good Evening, These levels are pre pneumococcal titers and I had counted
> 14/23 as greater than 1.3 which is around 60 percent so I didn't think a
> booster shot was indicated.
>
> Do you think I should boost her and recheck?
>
>
>
> Thank you
>
>
>
> Pam A
>
> Sent from my iPhone
>
>
> On Feb 8, 2017, at 11:05 PM, CIS-PIDD <cis-pidd at lists.clinimmsoc.org>
> wrote:
>
> Assuming that these are post vaccination pneumococcal titers, there are
> 9/23 of the pneumococcal serotypes which are less than 1.3, so it is less
> than 70%, which would classify as SAD moderate phenotype. There is an
> evidence based review regarding replacement Ig in the Working Group Report
> to be published next month in JACI which includes all patients with SAD,
> including the mild phenotype. Similar findings were in the Practice
> Parameter.
>
> Regards,
>
> Tony Tichenor
> ------------------------------
>
> *From:* CIS-PIDD [cis-pidd at lists.clinimmsoc.org]
> *Sent:* Wednesday, February 08, 2017 8:48 PM
> *To:* CIS-PIDD
> *Subject:* [cis-pidd] Mannose Binding Lectin Deficiency
>
> Good Afternoon,
>
>
>
> I have a patient who is 69 year old AAF with Pmhx of asthma, hymenoptera
> allergy, allergic rhinitis, several antibiotic allergies including
> penicillin and recurrent pneumonias (2 times ended up in ICU at an outside
> hospital), and sinusitis and an abscess
>
> She is on a beta blocker for her HTN and is not very excited about
> pursuing hymenoptera or PCN testing or shots.
>
> 9/23
>
> However, when I completed her immune workup
>
> HIV Negative
>
> IgG/A/M/E all normal
>
> Pneumococcal Protective
>
> Diptheria/Tetanus Protective
>
> CH50 normal
>
> Cell Markers: CD8 mildly low and CD 56/16 high and CD19 high
>
> And her Mannose Binding Lectin was practically 0
>
>
>
>
>
> Below I have included her detailed immune history and her immune lab
> results.
>
> What do we currently have to offer these patients besides Abx or
> prophylaxis? Is anyone’s lab providing those infusions of MBL and does it
> work? Is there any additional workup that needs to be done? (I thought
> about oxidative burst, but we couldn’t get it done that day because it was
> a send out)
>
> Any help would be appreciated!
>
>
>
> Thank you
>
> Pamella Abghari
>
> MetroHealth System
>
> Cleveland, Ohio
>
>
>
>
>
> Component 1/26/2017
> Pneumo Ab Type1 4.1
> Serotype 2 2.0
> Pneumo Ab Type3 <0.3
> Pneumo Ab Type4 2.0
> Pneumo Ab Type 5 2.9
> Pneumo Ab Type8 2.1
> Pneumo Ab Type9N 4.5
> Pneumo Ab Typ12 0.6
> Pneumo Ab Typ14 11.0
> Serotype 17F 0.4
> Pneumo Ab Type 19F 0.8
> Serotype 20 4.2
> Serotype 22F <0.3
> Pneumo Ab Typ23 0.3
> Pneumo Ab Type6B 5.9
> Serotype 10A 2.2
> Serotype 11A 2.0
> Pneumo Ab Type7F 1.2
> Serotype 15B 0.9
> Pneumo Ab Type 18C 1.7
> Serotype 19A 0.3
> Pneumo Ab Type 9V 3.6
> Serotype 33F 4.5
>
> WBC 5.7
> RBC 5.30 (H)
> Hemoglobin 12.8
> Hematocrit, spun 41.1
> MCV 78 (L)
> MCH 24.2 (L)
> MCHC 31.1 (L)
> Platelet 149 (L)
> RDW-CV% 14.5
> MPV 11.5
> Neutrophils 44.7
> Lymphocytes 44.3 (H)
> Monocytes 6.9
> Eosinophil 3.4
> Basophils 0.7
> Neutrophil # 2.54
> Lymph Absolute 2.51
> Monocyte Absolute 0.39
> Eosinophil Absolute 0.19
> Basophil # 0.04
>
> CD3 % Lymphs 67
> CD3 Absolute Count 1703
> CD3/CD4 % Lymphs 54
> CD3/CD4 Absolute Count 1361
> CD3/CD8 % Lymphs 13
> CD3/CD8 Absolute Count 323 (L)
> CD4/CD8 Ratio 4.2 (H)
> CD16/56 % Lymphs 13
> CD16/56 Absolute Count 334 (H)
> CD19 % Lymphs 18
> CD19 Absolute Count 456 (H)
>
> Tetanus Antibody 3.20
> Diphtheria Antibody 0.16
>
> CH50 Complement >60 (H)
>
> IgA, Quantitative 271
> IgE, Serum 6.4
> IgG, Quantitative 913
> IgM, Quantitative 118
>
> HIV-1/2 Eia Ab Nonreactive
>
> Mannose Binding Protein <0.5 (L)
>
>
>
>
>
> Birth History: C/S FT Complications Denies
> Mom have any miscarriages Maybe, before her
>
>
>
>
> Additional History:
> FTT Denies
> Chronic Diarrhea Denies
> Abnormal facies Denies
> Cardiac Defects Denies
> Developmental or functional delays Denies
> Fractures Hit her foot to the wall and broke her toe, fell 5 years ago and
> broke her ankle
> Teeth came in at age <1 year old Any abnormalities (weird shape, had to be
> pulled) Denies
> What age did umbilical cord fall off Denies
> Immunizations UTD
> Asthma Yes
>
>
>
>
>
>
>
> Infection History:
> Otitis Media: A lot as a child, Had tymp tubes as an adult.
> Pneumonia Yes Treated with Abxs Yes Ever admitted for antibiotic treatment
> Yes
> Any complications : 4in her lifetime. Admitted for 2 of them with one in
> the CCU
> Sinusitis Yes but doesn't get abx treatment
> Meningitis Denis
> Abscess Drained an abscess in her fallopian tube eventually had
> hysterectomy
> Cellulitis Denies
> Osteomyelitis Denies
> Thrush Denies
> Viral infections (per year) 1-2x a year
>
>
>
>
> ICU Yes refer to above
> Hospitalizations Yes refer to above
>
>
>
> Family Hx of PID or Autoimmune Dzs (HIV, CVID, SCID,CGD, LAD, Crohns, UC,
> RA): Denies
>
>
>
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