Comparison of exhaled and nasal nitric oxide and exhaled carbon monoxide levels in bronchiectatic patients with and without primary ciliary dyskinesia.

BACKGROUND: Primary ciliary dyskinesia (PCD) is associated with chronic airway inflammation resulting in bronchiectasis. METHODS: The levels of exhaled nitric oxide (eNO), carbon monoxide (eCO) and nasal NO (nNO) from bronchiectatic patients with PCD (n=14) were compared with those from patients with non-PCD bronchiectasis without (n=31) and with cystic fibrosis (CF) (n=20) and from normal subjects (n=37) to assess the clinical usefulness of these measurements in discriminating between PCD and other causes of bronchiectasis. RESULTS: Exhaled NO levels were lower in patients with PCD than in patients with non-PCD non-CF bronchiectasis or healthy subjects (median (range) 2.1 (1.3-3.5) ppb v 8.7 (4.5-26.0) ppb, p<0.001; 6.7 (2.6-11.9) ppb, p<0.001, respectively) but not lower than bronchiectatic patients with CF (3.0 (1.5-7.5) ppb, p>0.05). Nasal levels of nNO were significantly lower in PCD patients than in any other subjects (PCD: 54.5 (5.0-269) ppb, non-PCD bronchiectasis without CF: 680 (310-1000) ppb, non-PCD bronchiectasis with CF: 343 (30-997) ppb, control: 663 (322-1343) ppb). In contrast, eCO levels were higher in all patient groups than in control subjects (PCD: 4.5 (3.0-24.0) ppm, p<0.01, other bronchiectasis without CF: 5.0 (3.0-15.0) ppm, p<0.001; CF: 5.3 (2.0-23.0) ppm, p<0.001 v 3.0 (0.5-5.0) ppm). Low values in both eNO and nNO readings (<2.4 ppb and <187 ppb, respectively) identified PCD patients from other bronchiectatic patients with a specificity of 98% and a positive predictive value of 92%. CONCLUSION: The simultaneous measurement of eNO and nNO is a useful screening tool for PCD.

[Respiratory insufficiency amd pregnancy in cystic fibrosis]. / Légzési elégtelenség és terhesség cisztás fibrosisban.

Cystic fibrosis is one of the most serious genetic disorders. The survival and the quality of life of our patients have also been improved in the last decade. The number of patients entering the reproductive age is increasing. The young women in good somatic condition may undertake pregnancy. The desire for a full life and for a child may arise at patients with respiratory insufficiency too. Authors list the maternal and fetal risks of the pregnancy in cystic fibrosis by a case report. As a result of multidisciplinary team work a boy was born. The mother died in progressive and uninfluencable respiratory insufficiency being on waiting-list for lung transplantation 15 months after the delivery.

[Lymphangiomyoma of the thoracic duct]. / A ductus thoracicus lymphangiomyomája.

From the right superior mediastinum of a 31-year old woman the authors extirpated a lymphangiomyoma originating from the thoracic duct. The clinical feature and pathology of this rare lesions are discussed. This case is the first successfully removed thoracic duct lymphangiomyoma in Hungary.