الفهرس 
CYSTIC FIBROSISOnly 14 pages are availabe for public view
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Abstract
This study aimed to assess growth of CF children before and after nutritional rehabilitation and to correlate this to levels of IGF-1 and growth hormone.
To achieve this aim, this case- control study was conducted on 41 cystic fibrosis patients group (A) who were compared to 41 apparently healthy control participants group (B). The study was conducted over 2 phases. In phase 1, nutritional rehabilitation was done for all participants (group A), using high caloric formulas, vitamins, and pancreatic enzyme replacement for six months. After period of nutritional rehabilitation group (A) classified into two groups: group (A1) patients who reached to optimal growth and group (A2) patients who did not reach optimal growth. Phase 2 included those with non- optimal growth (group A2), then they were undergone further laboratory and imaging assessment. Insulin- like growth factor 1 was assessed in both study and control groups.While GH was assessed in patients who did not reach optimal growth after nutritional rehabilitation (groupA2). Then, the association between the levels of insulin- like growth factor and growth hormone and different growth parameters, clinical and laboratory parameters were assessed.
After analysis of the data, the results could be summarized as follows:
- The study included 41 cystic fibrosis patients and 41 age and sex matched control group with no significant differences as regard residence, consanguinity and socioeconomic status.
- The main presenting symptoms of cystic fibrosis patients were productive cough with expectoration, steatorrhea, dysnea, exercise intolerance and failure to gain weight.
- In the last year, median frequency of exacerbations was 3 and ranged from 1 to 7 per year and median frequency of hospital admission was 4 and ranged from 1 to 6 per year.
- Pulmonary function tests revealed restrictive pattern in 60% of patients and the main radiological findings were bilateral bronchiectasis and mucus plugging.
- Sputum cultures were negative for organism growth in 85.4% of patients and the most isolated organism was pseudomonas.
- Median values for insulin- like growth factor were higher among cystic fibrosis than control group with significant differences. Insulin- like growth factor had the ability to differentiate between severe and non- severe cases with 64.7% sensitivity and 78.3% specificity at cut-off value equal to 58.5.
- Insulin- like growth factor correlated positively to weight and height after rehabilitation therapy and calories intake and correlated inversely incidence of moderate exacerbations, days of exacerbation and ICU admission.
- Insulin- like growth factor correlated inversely to total leucocytic count and aspartate transaminases.
- Stimulate growth hormone by clonidine had the ability to differentiate between severe and non severe cases with sensitivity 70% and specificity 100% at cut off value equal to 5.25.
- The median GH level was 5.19 (3.06 – 8.15) among 14 patients who did not reach optimal growth, while the low GH level was prevalent 8 (57.1%) among studied patients, borderline level of GH was presented in 5 (35.7%) studied patients and normal level of GH was detected in 1 (7.1%) studied patients.
- Comparison between group (A1) and group (A2) regarding IGF-1, anthropometric measures shows that there was statistically significant between weight, height and BMI in patients who did not reach optimal growth than in patients who reached optimal growth after nutritional rehabilitation. Also, shows that there was statistical significant found between patients with low insulin growth factor 1 and patients who did not reach optimal growth 13(92.9%) than studied patients who reached optimal growth 2(7.4%)
- Growth hormone levels correlated inversely to frequency, days of hospital admission and number of emergencies visits.
CONCLUSION
The current study, it was concluded that:
Nutritional rehabilitation improved growth parameters in 66% of CF cases. Also, Insulin- like growth factor -1 was significantly lower among CF patients compared to control and low growth hormone level was detected among 8(57.1%) from 14 studied CF patients who did not reach optimal growth after nutritional rehabilitation. Furthermore, IGF-1 was significantly correlated with weight in kg, height in cm, moderate pulmonary exacerbations, number days of admission during exacerbations in the last year, while growth hormone was significantly correlated with number of days of admission during exacerbations in the last year, frequency of hospital admissions in the last year.
RECOMMENDATIONS
- The mean serum level of IGF-1 in CF children was significantly lower in patients with growth failure, so, its measurement might be needed for detection and follow-up of growth failure in CF children.
- Increase awareness campaigns of nutritional rehabilitation among CF patients.
- Pediatric CF patients had a considerable impairment in their nutritional status. Therefore, proper caloric intake and vitamin supplementation may had a valuable impact on their growth and prognosis.
- Future cohort studies with large sample size is needed to evaluate whether this GH deficiency in CF patients is directly related to defective CFTR or secondary to malnutrition.
- GH deficiency is not uncommon among CF patients and its regular assessment may be of value specially in pediatric patients with presistant growth retardation.
- Further clinical trials are needed to assess effect of the GH therapy in pediatric CF patients and determine its relation to growth parameters and clinical severity.