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            2008 by the author
      L.A. female: b.d. 13.09.1945

History
Previous mild smoker (5 pack-year).
Motor milestones development referred as normal
2 pregnancies to confinement
No significant pathologies until the age of 38 (1993)

1993: myialgia and limb muscular weakness and
progressive gait difficulty (unable to climb stairs without
banister)

For these reasons she referred to the neurologist which
scheduled the following diagnostic work up
     Diagnostic work up
   MRI: contrast enhancement with gadolinum in dorsal
    muscles of the thigh suggesting an acute
    inflammatory involvement
   Main EMG findings: spontaneous activity at rest;
    myopathic pattern during voluntary activation
   Muscular biopsy: dystrophic features with variation
    in fiber size. Immunoistochemical stains showed
    endomysial inflammatory infiltrate and rimmed
    vacuoles.
   Laboratory: Serum Creatine Kinase moderately
    elevated (2083 U/l; normal range < 269) ALAT and
    ASAT in the normal range
 Question n.1

   Based on these clinical and
laboratory findings what would you
    suggest to be the probable
             diagnosis?
 Answers to question n.1

1.   Amyotrophic lateral sclerosis (ALS)
2.   Muscular dystrophy
3.   Body inclusion Myositis
4.   Limb Girdle Muscular Dystrophy
Lancet Neurol 2007; 6: 620–31

Proposed diagnostic criteria for
inclusion body myositis
  Time goes by … 1998

The patient is wheel-chair bound and she
  further complains with:
 Morning headache

 Chest tightness

 Orthopnea



For these reasons she is finally referred to
              a pulmonologist
Question n.2

Which further diagnostic step
    would you suggest?
 Answers to question n.2

1. Chest X-ray and CT scan of encephalus
2. Chest X-ray and Magnetic Resonance
3. Magnetic Resonance and Pulmonary
     Function Testing
4.   Muscular biopsy and Pulmonary Function
     Testing
Muscular biopsy
Pulmonary function testing
   Question n. 3

In the light of these new findings

1. Would you reconsider diagnosis?
2. Would you confirm diagnosis ?
3. Would you reconsider diagnosis and ask
   for further diagnostic tools?
    Further diagnostic tool


Measurement of alpha-glucosidase (GAA)
 activity in blood leukocytes was low
Question n.4


Which diagnosis would you
        suggest ?
 Answers to question n. 4

1. Facioscapulohumeral dystrophy
     (FSHD)
2.   Limb girdle muscular dystrophy
3.   Pompe disease
4.   Danon disease
5.   Beckers disease
       GLYCOGENOSIS type II:
                        Pompe Disease

                        UDP    UTP glucose
1, 4                                         1, 4        Piruvate
Glycogen                                      Glycogen

                                                         Glucose

1, 4                                        Destrane     ACID
1, 6                                                    MALTASE
Glycogen                                                   (acid- -
           AMP                                           glucosidase
           c                                              lysosomial
                                                          Glycogen
  Glucose-1-phosphate         Glucose- 6 - phosphate
Molecular Genetics and Metabolism 93 (2008) 275–281
  Question n.5


 Which of the following steps
    would you suggest ?

Post scriptum: in 1998 enzyme replacement
was not available yet
     Answers to question n. 5
1. Follow up
2. Positioning of a diaphragmatic stimulator
     (pace-maker)
3.   Institution of non invasive mechanical
     ventilation
4.   Trachestomy and institution of invasive
     mechanical ventilation
   2005: New Hospital admission

•Worsening of dyspnoea during activities of
 daily living, which requires 16-20 hours/day
 of NIV.
•Worsening of patient’s quality of life
 Question n. 6

Which of the following strategies
 would you suggest to relieve
     patient’s symptoms?
     Answers to question n. 6
1. Nothing to do
2. Trachestomy and institution of invasive
     mechanical ventilation
3.   Change interface
4.   Change interface and shift to volume preset
     ventilation
5.   Associate positive pressure ventilation with
     negative mechanical ventilation
                                              200
                                                         400
                                                                600
                                                                       800
                                                                                  1000
                                                                                               1200




                                          0
                                 nov-98

                                 nov-99

                                 nov-00

                                 nov-01

                                 nov-02

                                 nov-03
                                                                                                  VC (ml)



                                 nov-04

                                 nov-05

                                 nov-06

                                 nov-07

                                 nov-08
                                          0
                                              5
                                                    10
                                                           15
                                                                20
                                                                      25
                                                                             30
                                                                                    35
                                                                                           40




                                 dic-98

                                 dic-99

                                 dic-00

                                 dic-01

                                 dic-02
Starting of enzyme replacement

                                 dic-03
                                                                                                  MIP
                                                                                         MEP




                                 dic-04

                                 dic-05

                                 dic-06

                                 dic-07
                                                                                                            Time-course of pulmonary function




                                 dic-08
   Issues of further discussion

1. Delay in diagnosis
2. Delay of pulmonary function assessment
3. Delay of institution of mechanical
  ventilation
4. Pay attention to patient’s comfort to
  assure best compliance to treatment
1. Delay in diagnosis
2. Delay in Pulmonary function testing
3. Delay in institution of MV
3. Delay in institution of MV
              measurement of HbA1c ≤ 7,5%

                        % of target population (patients with ischaemic heart disease and LDL-
Secondary prevention
                        cholesterol <130 mg/dl) treated with statins

                        % of patients with diagnosis of HT and DMII whose most recent systolic
Cardiovascular risk
                        BP<130 mm Hg and diastolic BP<85 mm Hg

                                                                                                    8
European ACG Conference, Karlskrona, Sweden 18-19 Sep, 2007


  RESULTS - STUDY DATA (general)


            General data
                           N       per patient
       Population        105,778
       Patients           81,085
       Visits            645,096      8.0
       Episodes          404,104      5.0
       Contacts          837,347      10.3
       Laboratory         43,794       0.5
       Referrals          50,271       0.6
       Transportation      1,630       0.0
       Radiology           8,189       0.1
       Source: BSA-AP



                                                              9
European ACG Conference, Karlskrona, Sweden 18-19 Sep, 2007



RESULTS - STUDY DATA (resources)

 Concept          Cost (euros)    %     % variable % total
 Fixed cost       9,044,245.92    25.9%
 Variable cost   25,881,371.63    74.1%
      laboratory    774,715.86               3.0%   2.2%
       radiology    288,823.00               1.1%   0.8%
        referrals 3,862,823.64              14.9% 11.1%
 transportation      32,600.00               0.1%   0.1%
     other tests    157,310.69               0.6%   0.5%
           drugs 20,765,098.44              80.2% 59.5%
 Total cost      34,925,617.55   100.0%    100.0% 100.0%
 Mean cost:             430.73
 Source: BSA-AP


                                                              10
  European ACG Conference, Karlskrona, Sweden 18-19 Sep, 2007


           RESULTS - EFFICIENCY
                     Prof. PCT – “A”
        Visits   Drugs     Lab      Ref      Rad     Total EI
FM-1     1.01     1.02     0.83     0.86     0.84     0.98
FM-2     0.92     0.83     0.95     1.08     1.65     0.88
FM-3     0.95     1.04     1.10     1.20     1.36     1.02
FM-4     0.96     0.87     0.84     0.97     1.05     0.89
FM-5     0.96     0.77     0.84     0.88     1.16     0.82
FM-6     1.06     0.84     0.83     0.97     1.60     0.91
FM-7     0.98     0.73     0.87     0.86     1.60     0.80
FM-8     1.08     0.94     0.68     0.82     0.92     0.94
FM-9     0.98     1.18     0.72     1.03     1.04     1.09

PED-1    1.02     0.90     0.35     0.85     0.71     0.92
PED-2    1.03     1.02     0.37     0.83     0.57     0.94
PED-3    1.01     0.72     0.43     0.81     0.62     0.87
                                                                11
        European ACG Conference, Karlskrona, Sweden 18-19 Sep, 2007


                       RESULTS E & E
                      Professionals PCT - “A”
          Visits   Drugs    Lab      Ref        Rad      Total EI   QSI
FM-1      1.01     1.02     0.83     0.86      0.84       0.98      61%
FM-2      0.92     0.83     0.95     1.08      1.65       0.88      65%
FM-3      0.95     1.04     1.10     1.20      1.36       1.02      67%
FM-4      0.96     0.87     0.84     0.97      1.05       0.89      69%
FM-5      0.96     0.77     0.84     0.88      1.16       0.82      61%
FM-6      1.06     0.84     0.83     0.97      1.60       0.91      67%
FM-7      0.98     0.73     0.87     0.86      1.60       0.80      70%
FM-8      1.08     0.94     0.68     0.82      0.92       0.94      68%
FM-9      0.98     1.18     0.72     1.03      1.04       1.09      61%

PED-1     1.02     0.90     0.35     0.85      0.71       0.92      72%
PED-2     1.03     1.02     0.37     0.83      0.57       0.94      67%
PED-3     1.01     0.72     0.43     0.81      0.62       0.87      76%
                                            Question EI & QSI!!           12
                      European ACG Conference, Karlskrona, Sweden 18-19 Sep, 2007


                             QUALITY OF CARE – EI & QSI
Important Slide                            Family Medicine

                               FM: Efficiency and effectiveness adjusted by ACG

                                                                                        R2 = 0.3597
                     1.5
                     1.4
                     1.3
  Efficiency index




                     1.2
                     1.1
                     1.0
                     0.9
                     0.8
                     0.7
                     0.6
                       20%     30%         40%          50%          60%          70%            80%
                                               Quality sinthetic index


                                                                                                       13
                    European ACG Conference, Karlskrona, Sweden 18-19 Sep, 2007


                           QUALITY OF CARE – E & E
                                               Pediatrics

                                  PED: Efficiency and quality adjusted by ACG


                   1.6

                   1.4
Efficiency index




                   1.2

                    1
                   0.8

                   0.6

                   0.4
                     20%    30%          40%         50%         60%       70%   80%

                                            Quality sinthetic index



                                                                                       14
      European ACG Conference, Karlskrona, Sweden 18-19 Sep, 2007


                        CONCLUSIONS
1.   Efficiency index is correlated with health results in usual practice
     settings

2.   Relationship within efficiency and effectiveness dimensions
     could be a good proxy of quality of care measurement for PCT
     and for individual physicians

3.   ACGs are a useful tool for risk adjustment and, further, they
     allow to identify chances of improvement in resource
     management by the primary care setting

4.   The absence of standard criteria in the quality of data and in the
     organizational models requires a careful interpretation of
     comparisons (external validity)

5.   Important - BSA (year 2007!) – EI to influence pay



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