Shwachman-Kulczycki Score Research Articles

Osteoporosis and osteopenia in adults and adolescents with cystic fibrosis: prevalence and associated factors

BACKGROUNDPatients with cystic fibrosis (CF) have many risk factors for reduced bone mineral density (BMD). The aim of this study was to determine the prevalence of osteoporosis and osteopenia in...

Lipid intake and serum cholesterol level in cystic fibrosis patients

The authors evaluated the data of 30 patients with cystic fibrosis (18 male, 12 female), between ages 2–18 (average age: 9.57 years). In 60% of the patients serum cholesterol was below the 5th percentile value according to the data of theNational Cholesterol Education Program(1991). They examined the connection between the serum cholesterol levels and the total fat, fatty acids and cholesterol intake. No significant differences were found between total fat, fatty acids and cholesterol intake of patients with serum cholesterol levels below the 5th percentile value and patients with levels above the 5th percentile value. The analysis of the nutritional data showed that lipid intake was in accordance with the proportion prescribed for patients with CF (35.56% of energy). The intakes of saturated fatty acids (SFA), monounsaturated fatty acids (MUFA) and polyunsaturated fatty acids (PUFA) were 11%, 11% and 8,3% of total energy, respectively, the PUFA/SFA ratio was 0.78. The ratio of linoleic to alfa-linolenic acid was especially high: 29. The clinical condition of the patients in the low cholesterol level group, as shown by the Shwachman-Kulczycki score was significantly lower (P<0.05) than that of the group with serum cholesterol levels above the 5th percentile value. The authors therefore suggest the importance of the regular monitoring of serum cholesterol levels in patients with cystic fibrosis. Observing the changes in serum cholesterol levels is a fast, informative and inexpensive method of evaluating the clinical course of the disease.

Effets de la rhdnase sur la fonction respiratoire et le statut nutritionnel de l'enfant et de l'adolescent atteints de mucoviscidose

In 1994 we started recombinant human deoxyribonuclease (rhDNase) in every cystic fibrosis (CF) patient whatever his (her) clinical condition, provided they were aged more than 5 years and forced vital capacity (FVC) was > or = 40%. We reviewed retrospectively the effects of rhDNase in 69 CF children and adolescents during a 2-year follow-up. Patients (35 boys, 34 girls) received 2.5 mg of rhDNase once daily from a mean age of 8.5 years (range 5-16.4). Baseline spirometric values (% predicted) and nutritional status were as followed: FVC = 84.8 +/- 21.7; forced expiratory volume in 1 second (FEV1) = 80.8 +/- 22.2; peak flow = 89.7 +/- 34.2, forced expiratory fraction 25-75% (FEF 25-75) = 71.8 +/- 32.8; Z score weight/height = -0.41 +/- 1.14; Z score weight/age = -0.48 +/- 1.25, body mass index = 15.4 +/- 1.8; caloric intake = 107 +/- 25% of recommended dietary allowances (RDA). Patients had a Shwachman-Kulczycki's score of 87 +/- 9. Spirometric and nutritional data were analysed after 1, 3, 6, 12, 18 and 24 months of treatment and compared to baseline values (changes evaluated as percent change from mean baseline for spirometric data). Shwachman-Kulczycki's score was calculated after 24 months of rhDNase. An improvement of FVC (+10.7%, P < 0.001) and FEV1 (+12%, P < 0.01) was noted after one month of treatment and was maintained throughout the following 2 years around 8.7% (6.4-11.4) for FVC and 8.2% (7.3-9.1) for FEV1, P < or = 0.01. This was particularly observed in children aged 5 to 10 years, in boys and in patients with a baseline FVC under 70% predicted. There was no significant change in FEF 25-75. We observed an improvement of daily caloric intake from the third month (P < 0.05) and of body mass index from the sixth month (P = 0.02). This was particularly noted in girls. Z score weight/age was improved only during the first 3 months of treatment while Z score weight/height increased only after a 2 year follow-up. There was no significant change in Shwachman-Kulczycki's score after 24 months of rhDNase. rhDNase in CF children in effective on lung function as well as on nutritional status and the response to this treatment can be evaluated after the first 3 months.

Non-tuberculous mycobacteria in cystic fibrosis

BACKGROUNDThe clinical significance of the presence of non-tuberculous mycobacteria in the sputum of patients with cystic fibrosis is unclear. A retrospective case-control study was performed to assess possible risk factors...

Relationship between disease severity and inflammatory markers in cystic fibrosis.

To evaluate the clinical use of measuring neutrophil, lymphocyte, and eosinophil activities, serum myeloperoxidase (MPO), soluble interleukin-2 receptors (sIL-2R), and eosinophil cationic protein (ECP) were measured in 98 patients with cystic fibrosis and in 85 healthy children. Serum concentrations of MPO, sIL-2R, and ECP were increased in patients with cystic fibrosis (median 807 micrograms/l, 4452 pg/ml, 48.8 micrograms/l, respectively) compared with the controls (median 319 micrograms/l, 2743 pg/ml, 9.4 micrograms/l). ECP concentrations, but not serum MPO or sIL-2R, were significantly related to disease severity assessed by the Shwachman-Kulczycki score and by pulmonary function (forced expiratory volume in one second % predicted). Neither ECP nor sIL-2R was influenced by Pseudomonas aeruginosa infection, acute pulmonary exacerbation, or atopy. Serum MPO, however, was strongly correlated with acute pulmonary exacerbation. In the light of these findings the measurement of serum ECP might thus be used for clinical monitoring and for assessing disease severity in cystic fibrosis. The measurement of serum MPO and sIL-2R did not correlate with the disease severity.

Functional iron deficiency in adults with cystic fibrosis

Functional iron deficiency (transferrin saturation <16%) was found in 44 (62%) of 71 adult cystic fibrosis (CF) patients. Haemoglobin concentration and mean cell volume were lower in iron-deficient patients, in whom there was a non-significant trend for lower serum ferritin. Ten iron-deficient patients and two patients with transferrin saturation >=16% (normal iron) were anaemic. There were no significant differences between iron-deficient and normal-iron patients in intake of calories, protein; iron and vitamin C as determined by 4-day records of dietary intake. Dietary iron deficiency is not an important factor in functional iron deficiency in adult CF patients. Impairment of absorption by exogenous pancreatic enzyme supplements is unlikely to be significant as enzyme intake was the same in the two groups. Iron-deficient patients had lower Shwachman—Kulczycki scores and lower percent predicted forced expiratory volume in 1 s (FEV 1 % predicted) and forced vital capacity (FVC % predicted). There was a non-significant trend for higher values of white cell count and plasma viscosity in the iron-deficient group. Chronic inflammation is likely to be the primary cause of functional iron deficiency in adult CF patients. Fifteen patients completed 3-month courses of oral iron replacement with no deterioration in pulmonary function, but with no effect on haemoglobin concentration.

Ursodeoxycholic acid for liver disease associated with cystic fibrosis: A double-blind multicenter trial

Liver disease is increasingly recognized as a major cause of morbidity in cystic fibrosis (CF). Preliminary data suggest that ursodeoxycholic acid (UDCA) may be beneficial for treatment of this manifestation. We performed a double-blind, multicenter trial in these patients to establish efficacy and safety of UDCA in terms of the improvement of clinical and nutritional indicators besides standard liver function tests. We also intended to establish whether taurine supplementation has a beneficial effect in patients receiving UDCA. From June to December 1990, we enrolled in 12 centers 55 CF patients with liver disease (39 male subjects; median age, 13.8 years). They were randomly assigned to receive for 1 year one of the following treatments: UDCA (15 mg/kg body weight daily) plus taurine (30 mg/kg body weight daily), UDCA plus placebo, placebo plus taurine, or double placebo. Clinical and laboratory evaluations were performed every 3 months. After 1 year, deterioration of overall clinical conditions, as indicated by the Shwachman-Kulczycki score (SKS), occurred in patients who received placebo but not in those who received UDCA (P = .025). Patients treated with UDCA also showed an improvement in gamma-glutamyl transpeptidase (GGT) (P = .004) and 5'-nucleotidase (P = .006) levels. Treatment with taurine was followed by a significant increase in serum prealbumin levels (P = .053), a trend toward a reduction in fat malabsorption, and no effect on the biochemical profile. No severe side effects occurred with any treatment. Thus, we concluded that UDCA administration improves clinical and biochemical parameters in CF patients with liver disease. Taurine supplementation may be indicated in patients with severe pancreatic insufficiency and poor nutritional status. (Hepatology 1996 Jun;23(6):1484-90)

Myeloperoxidase and eosinophil cationic protein in serum and sputum during antibiotic treatment in cystic fibrosis patients with Pseudomonas aeruginosa infection

I order to study the time-course of myeloperoxidase (MPO) and eosinophil cationic protein (ECP) as parameters for monitoring inflammation in cystic fibrosis (CF), we investigated ten patients during both a 14-day intravenous antibiotic treatment and a corresponding self control. Modified Shwachman-Kulczycki score improved significantly (p < 0.008), C-reactive protein (CRP) levels decreased significantly (p < 0.05) during antibiotic treatment, while in the control phase there were no significant differences. Lung function parameters did not change significantly during antibiotic treatment or control phase. Serum MPO concentration (p < 0.006) and peripheral blood neutrophil granulocyte counts (p < 0.04) decreased significantly during antibiotic treatment, but not during the control phase. Sentm ECP concentration showed a tendency to decrease during antibiotic treatment, but this failed to reach significance. In general, sputum concentrations of MPO and ECP Were 500- to 1000-fold higher than in serum. However, neither MPO nor ECP in sputum showed a significan variation over time during antibiotic treatment or control phase. From our data we conclude that: (1) measurements of MPO, neutrophils and CRP in peripheral blood do correlate with clinical parameters such as the modified Shwachman-Kulczycki score; (2) neutrophils and MPO seem to reflect inflammatory changes induced by antibiotic treatment; (3) eosinophils may play a role in CF by an enhanced ‘releasability’ and (4) Sputum measurements of mediators of inflammation cannot be recommended.

Allergic Bronchopulmonary Aspergillosis in Patients With Cystic Fibrosis

In order to determine the incidence of allergic bronchopulmonary aspergillosis (ABPA) in patients with cystic fibrosis (CF), we reviewed the records of 236 patients followed up at the Duke CF Center. Sixty patients (25 percent) had colonies of Aspergillus fumigatus. These patients were older and had more severe disease as assessed by lower Shwachman-Kulczycki (S-K) scores than the patients who did not have evidence of A fumigatus. In 15 of the patients with A fumigatus (6.5 percent of the total population), the diagnosis was ABPA. Age and S-K scores were not significantly different from those of the patients with A fumigatus without ABPA. Diagnostic features of the affected patients included wheezing refractory to bronchodilator therapy, persistent pulmonary infiltrates, peripheral eosinophilia, positive skin reactivity to an A fumigatus antigen and elevated total serum IgE levels. Steroid therapy was started for all patients, and clinical improvement was noted within 1 month as evidenced by decreased symptoms and weight gain. Chest x-ray films usually showed improvement. Vital capacity improved in all but two patients. Total IgE did not consistently decrease in response to therapy. Although the diagnosis of ABPA may be difficult to establish, ABPA commonly is associated with CF. Most patients respond to steroid therapy; however, the effect of therapy on the course of the disease is difficult to assess.

Increased physiological dead space during exercise after bronchodilation in cystic fibrosis.

Bicycle ergospirometry was performed on 14 patients with cystic fibrosis (CF), for evaluating the effects of salbutamol and theophylline on the ventilatory response to exercise. After 1 week without bronchodilator therapy the patients cycled at 1/3 and 2/3 of their individual maximal working capacity (Wmax). The test was repeated three times after treatment with salbutamol, theophylline, or both drugs, respectively. After the combined therapy, physiological deadspace, ventilation, ventilatory equivalent of oxygen, and end-expiratory oxygen pressure increased significantly during steady state exercise at 1/3 Wmax. Similar, although not statistically significant changes, were observed after monotherapy with salbutamol or theophylline and during exercise at 2/3 Wmax. These effects could not be predicted by any lung function tests at rest or by the Shwachman-Kulczycki score. The results indicate that in some patients with CF bronchodilators can impair lung function during exercise. In conclusion, the effects of medication on exercise performance of patients with CF have to be considered. Especially, the use of bronchodilators requires a careful evaluation of their real benefit in each individual patient.

Radioaerosol Assessment of Lung Improvement in Cystic Fibrosis Patients Treated for Acute Pulmonary Exacerbations

We compared bronchopulmonary distribution homogeneity of a radioaerosol before and after hospitalization in 20 patients with cystic fibrosis (CF) with pulmonary exacerbations in order to assess lung improvement. Deposition homogeneity was quantified in terms of skew (an index of distribution symmetry), derived from frequency distribution histograms generated from gamma camera images of the lungs following radioaerosol inhalation. Lower skew values indicate enhanced distribution homogeneity. Right lung skew (RLS) was significantly reduced following therapy (1.00 +/- 0.49 to 0.84 +/- 0.47), whereas skew in the left lung was unchanged (0.95 +/- 0.38 to 0.87 +/- 0.40). The reduction in RLS was significant in patients with Shwachman-Kulczycki (SK) clinical scores less than 50 (1.27 +/- 0.53 to 0.90 +/- 0.42), but not in patients with scores greater than 50 (0.81 +/- 0.38 to 0.80 +/- 0.52). These results indicate that treatment affected the right lung more than the left lung, particularly in patients with SK scores less than 50, and suggests that radioaerosol lung imaging may be valuable in identifying sites of impairment to be targeted during treatment. Statistically, skew was less sensitive an indicator of acute change than several other clinical indices that improved following hospital treatment.

Oxygen saturation in cystic fibrosis.

The availability of non-invasive oxygen saturation (SaO2) measurement could prove to be a useful tool for following up the progress of patients with cystic fibrosis. The present study was undertaken to compare its use with other routine measurements in the clinic. A total of 100 patients with cystic fibrosis were compared with 50 patients with stable asthma. The children were aged between 5 and 16 years and were studied as outpatients. A positive correlation was found between forced expiratory volume in one second and SaO2 in the group with cystic fibrosis and in the comparison group. Median SaO2 in those with cystic fibrosis (94.0%) was significantly lower than in controls (97.0%). Significant correlations were also found in the patients with cystic fibrosis for the following: Shwachman-Kulczycki score, higher weight centiles, and chronic pseudomonas lung infection with or without staphylococci. An inverse correlation was found with the Chrispin-Norman chest x ray score. SaO2 measurements are useful in the outpatient assessment of patients with cystic fibrosis and compare well with other standard tests of disease severity.

Heart-lung transplantation for cystic fibrosis. 1: Assessment.

Forty five patients with cystic fibrosis (age 5-15 years) were assessed for heart-lung transplantation between September 1987 and March 1990. The two main conditions for acceptance were (i) life expectancy less than 2 years and (ii) a severely impaired quality of life. Patients were accepted onto an active waiting list (n = 26), a provisional waiting list (n = 15), or not accepted (n = 4). Selection was made on clinical grounds with objective measurements used as an aid in assessment. As expected, the mean values for objective measures were significantly different between patients on the active waiting list compared with the provisional waiting list/not accepted group for resting heart rate (118/minute v 101/minute), percentage of ideal weight (83.2% v 93.1%), forced expiratory volume at one second as percent of predicted normal (27.3% v 47.6%), Shwachman-Kulczycki score (33.6 v 52.5), Chrispin-Norman x ray score (25.8 v 22.1), 12 minute walk (540 m v 854 m), and minimal oxygen saturation (81.5% v 92%). Psychological evaluation demonstrated a 55% incidence of individual and a 50% incidence of family morbidity. Of the 26 patients accepted onto the active waiting list, 11 have been transplanted (mean waiting time 3.3 months), 10 have died within a mean of 3.7 months of acceptance, and five remain on the active list (mean 5.6 months).

Intravenous immune globulin treatment of pulmonary exacerbations in cystic fibrosis

The effect of intravenously administered immune globulin (IVIG) on patients with cystic fibrosis with an acute exacerbation of pulmonary infection was evaluated in a double-blind study. Patients at least 12 years of age, with chronic respiratory tract colonization with Pseudomonas aeruginosa and hospitalized with a reduction in pulmonary function, were randomly assigned to receive 20% dextrose (control subjects: n = 8) or 100 mg/kg IVIG (Gamimune) (experimental subjects: n = 8) on days 1, 2, and 3; all patients received intravenous antibiotics and chest physiotherapy. There were no differences between groups on admission; patients had moderate to severe disease as measured by Shwachman-Kulczycki scores and pulmonary function tests. Both groups improved clinically. The IVIG treatment was associated with significant increases in forced vital capacity and forced expiratory volume in 1 second (p less than 0.01) and with greater percent improvement in forced expiratory volume and forced expiratory flow (25% to 75%) (p less than 0.05). There was no effect on length of hospitalization (18.3 +/- 11.9 days control vs 17.6 +/- 6.5 experimental). The C3 level was decreased at discharge in IVIG-treated patients; circulating immune complex levels were unchanged. One patient in each group experienced side effects. There were no differences on follow-up at 6 weeks. We conclude that IVIG infusion early in treatment for pulmonary exacerbations in cystic fibrosis patients with moderate to severe disease may be associated with greater improvement in pulmonary function than standard treatment alone.

Interobserver Variance in Clinical Scoring for Cystic Fibrosis

Multicenter clinical research would benefit from a simple, reliable scoring system for comparison of the clinical status of patients at different centers. In this study, five physicians performed simultaneous, independent scoring of 41 individuals with cystic fibrosis using the Doershuk modification of the Shwachman-Kulczycki scoring system for history, physical examination, and nutrition, and the Birmingham scoring system for chest roentgenograms. These were added together to obtain a clinical score. Interobserver variance of the scores was calculated. Mean individual observer variance from the consensus mean was 1.6-2.9 score points of a possible 25 for each category, 4.5-6.0 of a possible 100 for the total score. Coefficient of variance about the mean was approximately 10 percent for the individual categories, 6.7 percent for the total score. We concluded that the interobserver variance of this scoring system is within acceptable limit for most clinical studies. The total consensus score correlated with the NIH clinical score, chest roentgenogram score alone, and predicted values for forced vital capacity and FEV1 with a high degree of confidence.

Intrauterine insemination for infertility due to cystic fibrosis

Mild CF is not associated with increased obstetric risk to patients or offspring. These patients can be identified on the basis of Shwachman-Kulczycki scores, nutritional status, normal chest x-ray and pulmonary function tests, normal lung volumes, and mild to moderate airway obstruction. Spontaneous pregnancy has been achieved in women with CF, but they appear to have a decreased fertility. One possible cause in mild disease is a cervical factor due to abnormal viscous mucus. This mucus has been shown to fail to hydrate, and at midcycle contains less than 80% water, whereas 93% to 96% hydration appears to be necessary for sperm migration. Cyclic electrolyte changes in cervical mucus are not noted in CF patients, and sodium concentration in dry residue at midcycle is 10% of normal. Intrauterine insemination with a washed sperm fraction offers a theoretically safe method of bypassing this barrier. Preliminary data have revealed that this may be a useful therapy in other cases of infertility in which the abnormality is an unexplained, negative postcoital test. CF patients should be carefully evaluated and advised of risks before becoming pregnant. In cases of mild CF in which no factor for infertility exists other than consistently abnormal cervical mucus, intrauterine insemination with washed motile sperm may be beneficial. We believe this to be the first case of a CF patient successfully treated in this manner.

Noninvasive evaluation of ventricular function in cystic fibrosis

The cardiac function of 21 patients with cystic fibrosis was studied using radionuclides and M-mode echocardiography. The patients (mean age 13.2 years, range 4 to 27) had a wide range of clinical and pulmonary function abnormalities (mean Shwachman-Kulczycki score 62.1). Decreased right ventricular ejection fraction was found in 13 of 18 patients (72%); an additional four patients had abnormal septal motion on ECHO. Left ventricular ejection fraction was abnormal at rest in four patients (19%); an additional four patients had borderline low LVEF. The LV pre-ejection period to ejection time ratio increased significantly (i.e., worsening LV performance) with deterioration of S-K score, chest radiograph score, and forced expiratory volume in 1 second. Three of eight patients with normal LVEF at rest had an abnormal response to supine bicycle exercise: LVEF fell in two patients and was unchanged in one. Thus evidence of LV dysfunction was observed in seven of 21 (33%) of the patients; four at rest and in three only during exercise.

The Chest Roentgenogram in Cystic Fibrosis: A New Scoring System

A roentgenogram scoring system is presented that is useful in the evaluation and follow-up of patients with cystic fibrosis. The system has been shown to be reproducible by and between observers and to correlate significantly with results of pulmonary function tests, Shwachman-Kulczycki scores, and, in a short-term evaluation, morbidity.

Incidence of serum antibodies to several Aspergillus species and to Candida albicans in cystic fibrosis.

The incidence of serum IgE antibodies to several species of aspergillus and the incidence of serum precipitins to these species and to Candida albicans were determined in 22 children with cystic fibrosis, 17 children with bronchial asthma, and 18 control subjects. IgE antibodies were found in 50 per cent of the patients with cystic fibrosis and in 29 per cent of the patients with bronchial asthma; none was found in the control subjects. Precipitins to aspergillus species were present in 59 per cent of the patients with cystic fibrosis, 35 per cent of the patients with bronchial asthma, and 11 per cent of the control subjects. Greater differences were noted when the total number of precipitin bands in each group was compared. There was a trend for the more severe cases of cystic fibrosis (based on the Shwachman-Kulczycki score) to have a greater incidence of precipitins and a lower frequency of IgE antibodies to aspergillus than the milder cases. Candida precipitins were found more frequently in patients with cystic fibrosis (45 per cent) than in patients with bronchial asthma (18 per cent); none was found in the control group. The potential role of fungal hypersensitivity in cystic fibrosis and the therapeutic implications are discussed in light of these findings.