Lobar atelectasis is a common complication of cystic fibrosis. The majority of cases respond to intravenous antibiotics and chest physiotherapy. In a subgroup of patients, atelectasis is resistant to medical therapy, and its persistence in the pediatric population is associated with a poor prognosis. Bronchoscopic instillation of human recombinant DNase expanded atelectatic lobes in three children resistant to at least 2 weeks of medical therapy. This method of administration of DNase has been successful in resistant cases of lobar atelectasis in patients with chronic obstructive pulmonary disease, quadriplegia, and status asthmaticus. Purulent cystic fibrosis sputum has a very high DNA content, and DNA has been shown to become more pourable in vitro when treated with rhDNase. Bronchoscopic instillation of rhDNase should be considered in cases of persistent lobar atelectasis unresponsive to medical therapy.
Two cases of acute intussusception in older children with cystic fibrosis are reported. Both cases presented with symptoms and signs consistent with meconium ileus equivalent, which delayed the final diagnosis. Both cases required abdominal surgery but made full and uneventful recoveries.Intussusception occurring after the age of 2 years is relatively rare, but occurs in at least 1% of the population with cystic fibrosis.' We report two cases of intussusception occurring in patients with cystic fibrosis. A specific aetiology could not be found in either case. A boy was diagnosed as suffering from cystic fibrosis at the age of 6 months by three positive sweat tests (his sweat sodium concentrations were 100, 105, and 108 mmol/l). He had been on pancreatic supplements with daily physiotherapy since then and regular courses of oral antibiotics for intercurrent infections. He had a history of intermittent bouts of abdominal pain with constipation. He was admitted to hospital at age 4 years complaining of colicky abdominal pains and constipation. Abdominal examination was normal. A clinical diagnosis of meconium ileus equivalent was made. Acetylcysteine was commenced (5 ml three times a day). The patient developed loose stools of increasing frequency over the subsequent three days, but otherwise showed no symptomatic improvement. A vague mass was identified in the left upper quadrant which was thought to be faeces. This theory was supported by a plain radiograph of his abdomen. Daily bowel washouts were commenced and the patient's condition improved sufficiently over the subsequent five days for him to be discharged from hospital. The next day he was readmitted with an occurrence of the original symptoms. Examination showed a distended abdomen but nothing else. A plain radiograph of his abdomen showed a complete absence of gas in the splenic flexure and descending colon but no dilated loops of bowel or fluid levels. Abdominal ultrasound showed echogenic masses throughout the colon that were thought to be due to a faecally loaded colon. Subsequent plain radiographs of his abdomen showed dilated loops of bowel and fluid levels. A laparotomy was performed because of the acute obstruction. An ileocaecal intussusception was found with rupture of the apex of the intussusception through the wall of the sigmoid colon. A resection of bowel was performed with ileocolic anastamosis. As the sigmoid colon at the site of rupture was of doubtful viability a colostomy was fashioned with a view to later repair and closure. This was done one month later. His recovery was uneventful. He was discharged on normal diet and pancreatic enzyme supplements and the problem has not recurred. DiscussionAcute intussusception is a common surgical problem in the first two years of life but is rarely observed in the older child.2 It is a recognised complication in the older children who suffer from cystic fibrosis and its incidence has been put at 1% with an average age of onset between 9 and 12 years. ' Despite this fact, it is a complicatio...
Thrombocytosis in low birthweight infants 717 infancy and childhood, thrombocytosis is seen in connection with various rare diseases (Addiego et al., 1974) of which iron deficiency and vitamin E deficiency are likely to be the most common causes in preterm babies. These results show that there were relatively high values at 2 weeks in the presence of ample iron stores. No difference was found between iron-supplemented and nonsupplemented infants. Nor were high platelet values associated with low Hb levels, except at 2 weeks. Accordingly there is no reason to suspect that a lack of iron in these infants individually, or as a group, would have resulted in thrombocytosis. Vitamin E deficiency as a cause is also unlikely as the infants were supplemented with vitamin E, and Hb levels and reticulocyte counts were similar whether their platelet counts were high orlow.The data suggest that thrombocytosis is a physiological phenomenon in preterm infants during the first 6 months of life. The increase in number might be compensatory to a decreased functional capacity shown in term infants (Corby and Schulman, 1971) and further suggests that values <160 x 109/1 are evidence of thrombocytopenia.
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