As proposed by Diederich et al., the extent of bronchiectasis was classified into five categories on a lobar basis [ 11], and a progressive score was used to quantify bronchiectasis in each lobe: no involvement (0), less than 25%% involvement (1), 26 50 % involvement (2), 51 75 % involvement (3) and greater than 75%% involvement (4) resulting in a maximum score of 24 per patient.
Mutations in AHI1 encoding Jouberin are responsible for JS, with retinal involvement in 75% and renal involvement in less than 10% of all AHI1-associated patients (Kroes et al., 2008).
First, there was an association with marrow involvement (P less than 0.05) which usually accompanies involvement of the blood with morphologically abnormal cells.
Lesions were graded on a scale from 0 to 4: 0 = normal; 1 = mild, involvement of less than 25% of the cortex; 2 = moderate, involvement of 25% to 50% of the cortex; 3 = severe, involvement of 50 to 75% of the cortex; 4 = extensive damage involving more than 75% of the cortex.
The scoring levels 0−1−2 are used for all parameters, where 1 implies involvement of less than 50% of the lobe and 2 implies involvement of more than 50% of the lobe.
Lesions were graded on a scale from 0 to 5: 0 = normal; 1 = mild, involvement of less than 10% of the cortex; 2 = moderate, involvement of 10 to 25% of the cortex; 3 = severe, involvement of 25 to 50% of the cortex; 4 = very severe, involvement of 50 75% of cortex; 5 = extensive damage, involvement of more than 75% of the cortex.
33 In this registry, patients with SSc/myositis, who suffered from muscle weakness together with elevated CK serum levels, 59.1% had oesophageal involvement, 38.6% of patients had lung fibrosis, 34.1% suffered from cardiac involvement, while less than 15.0% had kidney failure or signs for PH.
In a subgroup of patients classified as overlap syndrome with myositis and CK elevation (44/325), we determined the frequency of gender distribution (70.5% women, 29.5% men), as well as clinical features; 59.1% had oesophageal involvement, 38.6% of patients had lung fibrosis, and 34.1% suffered from cardiac involvement, while less than 15.0% had kidney failure or signs for PH.
Both outcomes were comparable, with an increase of midwifery involvement from less than 10% in 1998 to approximately 25% in 2007, although there was a slightly (2-3%) larger involvement of midwives in the group of referrals during labour (Table 2).
Patients with bilateral involvement, weighing less than 50 kg, were corrected simultaneously.
