Archives of disease in childhood
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Mucociliary tracheal transport rates were measured in 20 patients with cystic fibrosis, in whom these rates ranged from 0 to 12.8 mm/min. The patients were divided into 3 roughly equal groups on the basis of their transport rates. (1) Those in whom no abnormality in mucociliary transport was detected in the trachea; (2) those in whom normal transport rates were measured but in whom abnormalities such as cessation, or reversal of bolus movement were observed; (3) those in whom no normal transport rates were observed. In the first group the rates were similar to those observed in a population of healthy adults. ⋯ The mean mucociliary tracheal transport rate increased with increasing maximum midexpiratory flow. Those patients with a low Shwachman score and poor arterial oxygen tension tended to fall into groups 2 and 3. In the ciliary dyskinesia assay in rabbit trachea the serum from the patients with the higher transport rates tended to initiate more rapid discharge of material from the epithelium and ciliary dyskinesia.
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This study describes the radiological and pathological findings in a necropsy series of 70 skulls of Nigerian patients with sickle cell gene, and 70 controls matched for sex and age with no sickle cell gene. 6 (35%) of the 17 patients with sickle anaemia, or 17% of 35 patients with sickle cell gene excluding the trait, all under age 10 years, were shown to have bone trabeculae within the diploe arranged in stripes parallel to the curvature of the cranial tables on radiography. Histological examination of the skulls with curvilinear stripes showed long trabeculae of bone within the diploe similarly arranged in parallel rows but joint at variable intervals by short bridges of bone. The radiolucent areas between trabeculae corresponded to areas of marrow hyperplasia. A skull radiograph in an African child presenting with this radiological sign should raise the suspicion of sickel cell disease.
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Four cases of septicaemia in children were traced to contaminated intravenous infusions and volume control sets. In each case Pseudomonas cepacia was isolated from multiple blood cultures and from intravenous fluid within the volume control set. The first patient died of septicaemia after a long and complicated postoperative period. The other three patients received appropriate antibiotics after removal of the contaminated intravenous sets and they recovered within 2 weeks.