[CIS-PAGID] deficiency in terminal complement components

[Ochs, Hans]

We use a similar protocol for late C-component deficiencies
A caveat is the tendency of these patients, after several years of being asymptomatic, to become complacent and either loose the "emergency letter" or run out of meds.
hans

Hans D. Ochs, MD, Dr. med
Professor of Pediatrics | Jeffrey Modell Chair of Pediatric Immunology Research
Center for Immunity and Immunotherapies
Seattle Children's Research Institute | University of Washington 

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-----Original Message-----
From: pagid-bounces at list.clinimmsoc.org [mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of Nicholas Rider
Sent: Thursday, December 01, 2011 3:30 AM
To: pagid at list.clinimmsoc.org
Subject: Re: [CIS-PAGID] deficiency in terminal complement components

Dear Philipp,

Greetings!  We follow a group of patients with Properdin Deficiency.  They are Mennonite missionaries and as such far from medical care on a routine basis.  We do testing on infants (genetic and if needed AH50).  We recommend that all affected individuals carry disease identification (wallet card, bracelet, necklace, etc.) and recommend vaccination every 3-5  years against meningococcus (MCV4/Menactra), Hib and Pneumococcus.  Patients have azithromycin and levofloxacin on hand for illnesses.  They don't take prophylactic antibiotics, but begin azithromycin for any upper respiratory tract illnesses/low grade fevers and levofloxacin immediately for more severe illnesses.  They are encouraged to seek medical attention for ALL illnesses requiring antimicrobial therapy.  They all carry an emergency letter for ER encounters/acute illness presentation.  

We are in the process of completing a longitudinal study of outcome in these patients and expect data completion early in 2012.  It appears that there have been no deaths over a 6 year period because of early detection and preemptive antibiotic therapy/proactive vaccine strategies.  I will keep the community updated.

Sincerely,

Nick

Nicholas L. Rider, D.O.
Clinic for Special Children
535 Bunker Hill Road
Strasburg, PA 17579
Ph:  717-687-9407
Fx:   717-687-9237
nrider at clinicforspecialchildren.org

Medical Arts Allergy
220 Wilson Street
Carlisle, PA 

On Dec 1, 2011, at 6:08 AM, Philipp Henneke wrote:


> We have recently diagnosed a patient with recurrent meningococcal meningitis to be deficient in terminal complement components.

> My question: What is the experience with antibiotic prophylaxis versus preemptive treatment when non specific symptoms of infection occur?

> Philipp Henneke

> 

> Prof. Dr. med. Philipp Henneke

> 

> UNIVERSITÄTSKLINIKUM FREIBURG

> Centrum für Chronische Immundefizienz (CCI)

> Zentrum für Kinder- und Jugendmedizin

> 

> Tel Sekretariat 0761 270-77640

> Tel mobil 0162 285 2481

> Fax 0761 270 77600

> Breisacherstr. 117-2.OG, 79106 Freiburg

> philipp.henneke at uniklinik-freiburg.de

> http://www.cci.uniklinik-freiburg.de

> 

> 

> 




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