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Lung transplantation for cystic fibrosis: immune system and autoimmunity

Sediva , Lischke, Jan Simonek, Jakub Tkaczyk, Vera Vavrova,

Jana Bartosova, Petr Pohunek, Jirina Bartunkova, Pavel Pafko,

Summary:

Background:In the current study we focused on changes in the immune

parameters of patients with CF after lung transplantation (Tx), with

particular emphasis on the interaction of the immune system, infection,

the autoimmune phenomenon observed in some CF patients, and

immunosuppression.

Material/Methods:Seven transplant patients with CF were investigated, 3

men and 4 women; the average age at Tx was 24.2 years (20.2-32.3). The

parameters of both humoral immunity (immunoglobulins, complement, CRP,

antinuclear and antineutrophil cytoplasmic antibodies) and cellular

immunity (T and B lymphocytes, NK cells) were traced.

Results:We observed marked initial hyperimmunoglobulinemia, with a sharp

drop in immunoglobulin levels within 1 month after Tx. Positivity for

antineutrophil cytoplasmic antibodies (ANCA) was found in 3 patients

before Tx. A strong ANCA positivity persisted 2 months after Tx despite

deep introductory immunosuppression. In one patient ANCA positivity,

after a transient negative result at months 2 and 12 after Tx, reappeared

one year after Tx. The Burkholderia cepacia infections found in 2

patients proved to be lethal.

Conclusions:In our series of CF lung transplant recipients, we found

Burkholderia cepacia infection to be a risk factor. The robust appearance

of autoantibodies and their persistent positivity for many months despite

deep immunosuppression is a remarkable feature observed in some CF

patients.

key words:lung transplantation • cystic fibrosis • autoantibodies

Full-text PDF:http://www.MedSciMonit.com/pub/vol_7/no_6/1881.pdf

Emergency double-lung-tetransplantation for late graft failure in a

13-year old child: a case report

Ireneusz Haponiuk, Wolfgang Harringer, Axel Haverich,

Summary:

This paper presents a case of a pediatric double-lung retransplantation

performed two years after a primary double-lung transplantation due to

cystic fibrosis. This pulmonary retransplantation was performed as an

emergency life-saving procedure for the child in the setting of pulmonary

insufficiency and preoperative mechanical ventilation. Retransplantation

for pediatric patients with end-stage respiratory disorder remains

controversial because of poor outcome and a current shortage of donor

lungs.

The effectiveness of coordinated care for people with chronic respiratory

disease

J , J McElroy, E Ruffin, A Frith,

R Heard, Malcolm W Battersby, J Esterman, Del Fante and

J Mc

MJA 2002 177 (9): 481-485

Abstract

—

Introduction

—

Methods

—

Design

—

Selection criteria

—

The coordinated care intervention

—

Outcome measures

—

Quality of life and functionality

—

Health service utilisation

—

Statistical methods

—

Health costs

—

Results

—

Baseline characteristics

—

Health services utilisation

—

Functionality and quality of life

—

Healthcare costs

—

Discussion

—

Competing interests

—

Acknowledgements

—

References

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Author details

Abstract

Objectives:

To evaluate the effectiveness of coordinated care for chronic respiratory

disease.

Design and setting:

Community-based geographical control study, in western (intervention) and

northern (comparison) metropolitan Adelaide (SA).

Participants:

377 adults (223 intervention; 154 comparison) with chronic obstructive

pulmonary disease, asthma or other chronic respiratory condition, July

1997 to December 1999.

Intervention:

Coordinated care (includes care coordinator, care guidelines, service

coordinator and care mentor).

Main outcome measures: Hospital admissions (any, unplanned and

respiratory), functionality (activities of daily living) and quality of

life (SF-36 and Dartmouth COOP).

Results:

At entry to the study, intervention and comparison subjects were

dissimilar. The intervention group was 10 years older (P < 0.001), less

likely to smoke (P = 0.014), had higher rates of hospitalisation in the

previous 12 months (P < 0.001) and had worse self-reported quality of

life (SF-36 physical component summary score [P < 0.001] and four of nine

COOP domains [P = 0.002–0.013]). After adjustment for relevant baseline

characteristics, coordinated care was not associated with any difference

in hospitalisation, but was associated with some improvements in quality

of life (SF-36 mental component summary score [P = 0.023] and three of

nine COOP domains [P = 0.008–0.031]) compared with the comparison group.

Conclusions:

Coordinated care given to patients with chronic respiratory disease did

not affect hospitalisation, but it was associated with an improvement in

some quality-of-life measures.

In the late 1990s, nine coordinated care trials took place across

Australia. Their overall aim was to test whether multidisciplinary care

planning and service coordination improved health and wellbeing for

people with chronic health conditions or complex care needs within

existing resources.1 A recent review of studies of outreach nursing,

which has features in common with coordinated care, found increased costs

and no reduction in hospitalisation, but slight improvements in quality

of life.2

In the South Australian trial, HealthPlus,3 one of eight components was

the Western Respiratory Project. Its aims included:

§    sharing of care between hospital-based specialists, general

practitioners, and other community-based healthcare practitioners

(including domiciliary care, Royal District Nursing Service) in a

community-based approach, with GPs central to the planning and monitoring

of prospective care;

§    pooling of federal and state health funds to provide efficient

healthcare service delivery and enhance patient care with similar or

reduced overall healthcare system costs; and

§    producing evidence on the change in health outcomes as a result of

the changes in healthcare delivery.

Methods

Design

Recruitment began in July 1997 and continued until September 1998.

Follow-up ended in December 1999. Patients were recruited from the

western (intervention group) and northern (comparison group) suburbs of

Adelaide, in collaboration with the Adelaide western and northern

Divisions of General Practice. The geographical comparison region was

selected pre-hoc on the basis of previous surveys4 as the region of

Adelaide that best matches the sociodemographic features of the western

region. The number of GPs per 1000 patients (northern, 1.21; western,

1.34) is also similar for the two regions. Participating GPs recruited

patients opportunistically, so details of the eligible population were

not collected.

This study should be considered as a form of " action research " , where a

cycle of action and critical review led to improved GP participation and

refinement of the methodology throughout the study.5,6

Ethics approval was obtained by the Ethics of Human Research Committee at

the North Western Adelaide Health Service and clearance to obtain data

was received from the Health Insurance Commission.

Selection criteria

Participants had to be at least 18 years old, have a chronic respiratory

illness, and have complex care needs. Initially, extra criteria were

used, including hospitalisation within the past 12 months. However, as

part of the action research methodology, these criteria were dropped to

ensure adequate recruitment. Selection criteria were applied by

participating GPs on a patient self-report basis, and were checked by the

service coordinators.

The coordinated care intervention

GPs took the role of " care coordinators " and supervised the

multidisciplinary management of each patient.7 To facilitate this

process, multidisciplinary care plan generators (CPGs) were constructed

with input from consumers (patients and their carers), GPs, respiratory

physicians, allied health professionals, the Royal District Nursing

Service, domiciliary care and an epidemiologist. Relevant published

medical evidence and the published guidelines of the thoracic societies

of Australia and New Zealand, Britain and America8-10 were incorporated.

The CPGs included a recommended annual number of GP visits, respiratory

function tests, other diagnostic tests and physician visits where

necessary.

GPs received and were reimbursed for two to four hours of orientation.

GPs were supported by " service coordinators " (nurses), who liaised with

the patient, GP, respiratory specialist and other healthcare

professionals, monitored the patient, and encouraged implementation of

the evolving care plan. Duties included booking investigations, arranging

case conferences, referrals, home visits and collecting data for

evaluation of the intervention.

Outcome measures

Quality of life and functionality

Quality of life was measured by the SF-3611 and the Dartmouth COOP

function charts (COOP).12,13 Using the SF-36 as an outcome measure was a

requirement of the coordinated care trials, and the physical and mental

component summary scores were selected pre-hoc as those most relevant to

our study.

Functionality was measured by part of the COOP function charts, the Older

Americans Resources and Services independent activities of daily living

questionnaire (OARS),14 and the Modified Barthel Index (MBI).15 The COOP

was chosen as a simple, graphic questionnaire suitable for frail, elderly

people with respiratory disease, including those with limited English.

All questionnaires were administered at baseline and on study termination

in December 1999, after at least 12 months' study participation. The

SF-36 was administered by telephone. Where telephone contact could not be

made because of lack of telephone or insufficient English, the interview

was conducted by the service coordinator with the assistance of a family

member where necessary. All the other questionnaires were administered by

service coordinators. Interviewers were not blinded to the study

hypotheses.

Becki

YOUR FAVORITE LilGooberGirl

YOUNGLUNG EMAIL SUPPORT LIST

www.topica.com/lists/younglung

Pediatric Interstitial Lung Disease Society

http://groups.yahoo.com/group/InterstitialLung_Kids/

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