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not all cases of porphyria are referred to this institute and not allfamily members have yet been studied.Most patients with acute porphyria live in Warsaw and the

surrounding provinces, where, because complete biochemical

diagnosis is possible only at the Institute of Haematology inWarsaw, there is a good chance of porphyria being detected. If thedisease is evenly distributed throughout Poland the prevalence forthe Province of Warsaw (3 - 93 per 100 000) may more accuratelyreflect the national position than the figure of 0 . 66 per 100 000 thatwe have calculated.

We thank Prof. Y. Nordmann and his colleagues at the department ofbiochemistry, Faculty of Medicine, Colombes, France, for the copro-oxidaseassays.

Porphyria Unit,Institute of Haematology,00 957 Warsaw, Poland

EWA KOSTRZEWSKAANITA GREGOR

CEFTAZIDIME IN CYSTIC FIBROSIS

SIR,-Dr Gozzard and colleagues in their article on ceftazidime(May 22, p. 1152) describe the drug as a potent antibiotic againstPseudomonas and as being very active against a broad spectrum ofaerobic gram-negative bacteria. Because of these data, it seems

important to record experience with this drug in the treatment ofsevere pulmonary infections in cystic fibrosis (CF). Lungdestruction, an important life-shortening factor in this illness,seems to be correlated with chronic Pseudomonas infection.1-3Nevertheless, other colonising organisms, such as Staphylococcusaureus and Haemophilus influenzae, may also cause life-threateninginfections in the CF patient. For these reasons we started a trial ofceftazidime in these severe pulmonary infections.

1. Better J. Clinical management of the adolescent and adult patient. In: Perspectives incystic fibrosis: Proceedings of the 8th International Congress on Cystic Fibrosis(Toronto, 1980): 183-89.

2. Marks MI. Pathogenesis and treatment of pulmonary infections in patients with CF. JPediat 1981; 98: 173-79.

3. Mearus MB. Natural history in pulmonary infections in CF. In: Perspectives in cysticfibrosis: Proceedings ofthe 8th International Congress on Cystic Fibrosis (Toronto,1980): 325-34.

So far fifteen CF patients, varying between 6 months and 28 yearsof age, have been studied. All were in a severely deterioratedpulmonary condition; in nine of them high fever (38-402°C),cyanosis, and respiratory failure were the main symptoms. Wetreated all of them with intravenous ceftazidime monotherapy(doses varying between 40 and 115 mg/kg daily) for 3-14 days,without knowledge of the pathogen isolated from the sputum.Mixed cultures were found in eleven patients; Ps. aeruginosa wasisolated in thirteen, Staph. aureus in six, H. influenzae in four,Streptococcus pneumoniae in three, Escherichia coli in two,Enterobacter cloacae and Proteus vulgaris in one each (table).Minimal inhibitory concentrations of the organisms ranged from0 - 097 mg/1 for E. coli to 12-5 5 mg/l for one of the Staph. aureusisolates.

Clinical outcome was good in twelve patients, where no antibiotichad to be given during the follow-up period. The patients’symptoms subsided and organisms, if not eradicated, were oftenmarkedly diminished in bacterial count, as measured bysemiquantitative culture of sputum. Two patients improvedmarkedly, but relapsed within 2 weeks of treatment. There was onefailure: specific anti-staphylococcal antibiotics had to be adminis-tered after 3 days of ceftazidime treatment -because the patient’sclinical condition and fever got worse. No major side-effects werenoted in any of the fifteen patients.Ceftazidime levels varied between 5 and 100 mg/1 of serum (eleven

patients, mean value 45 mg/1) and between 4 and 15 mg/1 of sputum(eight patients, mean value 9 mg/1) 1 h after injection of the drug.These preliminary results suggest that ceftazidime may have an

important role in conditions where Pseudomonas usually plays amajor role but where other organisms have to be covered as well. Inchronic conditions like CF, where antibiotics have to beadministered frequently, monotherapy with ceftazidime may be agood alternative to combinations of antibiotics, includingaminoglycosides.

Infectious Disease Unit,Saint-Pierre University Hospital,B-1000 Brussels, Belgium

B. GORDTSI. DAB

J. P. BUTZLER

DETAILS OF FIFTEEN PATIENTS WITH PULMONARY INFECTIONS TREATED WITH CEFTAZIDIME