Cystic fibrosis course

As discussed above, in the case of phenylketonuria, early intervention can make the difference between mental retardation and a near normal life course for a newborn. Congenital adrenal hyperplasia and maple syrup urine disease are two examples of neonatal hereditary disorders where early diagnosis and medical intervention can make the difference between life and death for the newborn. In addition, in a number of genetic diseases, early diagnosis and treatment can help ameliorate symptoms these include fragile X syndrome, homocystinuria, sickle cell anemia, cystic fibrosis, and many /1-thalassemias. 

Preparations are of animal origin and variable potency. Pancreatin, as Cotazym and Nutrizym, appears to be satisfactory. A reasonable course is to start the patient on the recommended dose of a reliable formulation and to vary this according to the individual s needs, and the size and composition of meals. Enteric-coated formulations (pancreatin granules, tablets) are available. High-potency pancreatic enzymes should not be used in patients with cystic fibrosis as they may cause ileocaecal and large bowel strictures. 

Hearing loss was attributed to repeated exposure to aminoglycosides in 12 of 70 patients with cystic fibrosis (one child) (25). There was a non-linear relation between the number of courses of therapy and the incidence of hearing loss. The severity of loss was not related to the number of courses. Assuming that the risk of hearing loss was independent of each course, the preliminary estimate of the risk was less than 2 per 100 courses. 

A retrospective analysis of allergic reactions (drug-induced fever and rash) in 90 adults with cystic fibrosis, of whom 26 developed probable allergic reactions to parenteral beta-lactams. There was drug-induced fever in 54 and skin reactions in 28 of 897 treatment courses (6 and 3.1% respectively). There was one case of non-fatal anaphylaxis. The numbers of allergic reactions per number of patients receiving specific antibiotics were carbenicillin 4/56, mezlocillin 7/42, piperacillin 11/31, ticarcUlin 1/20, cefazolin 0/24, ceftazidime 1/35, imipenem + cilastatin 4/16, and nafcillin 3/36 (17). 

Koch C, Hjelt K, Pedersen SS, Jensen ET, Jensen T, Lanng S, Valerius NH, Pedersen M, Hoiby N. Retrospective clinical study of hypersensitivity reactions to aztreonam and six other beta-lactam antibiotics in cystic fibrosis patients receiving multiple treatment courses. Rev Infect Dis 1991 13(Suppl 7) S608-11. 

Optic neuropathy has been seen after prolonged courses of chloramphenicol (13). Alterations in color perception and optic neuropathy, in some cases resulting in optic atrophy and blindness, have been observed, especially in children with cystic fibrosis receiving relatively high doses for many months (12,14). Most of these complications were reversible and were attributed to a deficiency of B vitamins. 

Adams JP, Conway SP, Wilson C. Hypomagnesaemic tetany associated with repeated courses of intravenous tobramycin in a patient with cystic fibrosis. Respir Med 1998 92(3) 602-4. 

Colonization with P. aeruginosa is a sentinel event in the course of cystic fibrosis. Chronic infection with this organism is associated with progressive loss of lung function attributed to chronic inflammation and recurrent pulmonary exacerbations. Thus, there is interest in strategies that may delay the acquisition of this organism. 

However, while these findings are of potential significance to the amplification and perpetuation of the airway inflammation in adult cystic fibrosis patients, other studies, especially studies in children with cystic fibrosis, have not consistently detected the marked reductions in IL-10 (73). The reason for these inconsistencies is not immediately apparent, though the discrepancies may in part reflect the time course of evolution of pulmonary inflammation. 

Abman SH, Ogle JW, Harbeck RJ, Butler-Simon N, Hammond KB, Accurso FJ. Early bacteriologic, immunologic, and clinical courses of young infants with cystic fibrosis identified by neonatal screening. J Pediatr 1991 119 211. 

Accurso FJ, Sokol RJ, Hammond KB, Abman SH. Early respiratory course in infants with cystic fibrosis relevance to newborn screening. Pediatr Pulmonol Suppl 1991 7 42. 

Urinary tract High rates of chronic kidney disease, ototoxicity, and vestibulotoxicity have been reported in patients with cystic fibrosis who require multiple and prolonged courses of aminoglycoside antibiotics [1 ]. The incidence of chronic kidney disease has been reported as up to 42% in patients with cystic fibrosis [2 ]. 

Chloramphenicol may cause retinal bleeding (as a symptom of aplastic anaemia), alterations in the perception of colours, and optic neuritis. The latter type of side effect has been observed especially in children with cystic fibrosis receiving chloramphenicol in relatively high doses for fairly long periods (38 ). Deficiency of B vitamins is believed to be the cause of the optic neuritis, and perhaps also of the polyneuritis, peripheral neuritis, and paraesthesiae of the legs sometimes seen after prolonged courses of treatment with chloramphenicol and thiam-phenicoL Administration of extra vitamins may protect patients from this type of reversible side effect (SED VIII, p. 610 38 ). 

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