Bronchoalveolar lavage and transbronchial biopsy in children following heart-lung and lung transplantation.

Between July 1985 and March 1992, 20 children received either heart-lung (11), double lung (8), or single lung (1) transplants at our center. Since 1988, flexible fiberoptic bronchoscopy with bronchoalveolar lavage and transbronchial biopsy have been carried out to monitor for rejection or infection in these patients. As of March 31, 1992, we have performed a total of 112 transbronchial biopsies in our patients, who ranged from 6.8 to 18 years of age and 19.3 to 67.3 kg in weight. All but two of these procedures were carried out using conscious sedation and a transnasal approach. Four to seven biopsy samples were obtained at each procedure. One patient had hemorrhage (< 100 ml) and no patient had pneumothorax as a complication. Of the biopsy samples, 72.4 percent had a surface area of greater than 2 mm2, and 89.5 percent of the biopsy samples were deemed adequate for pathologic interpretation. We believe that for the majority of pediatric lung or heart-lung recipients, flexible bronchoscopy and transbronchial biopsy using conscious sedation and a transnasal approach is safe and permits the recovery of adequate tissue for pathologic evaluation. The avoidance of general anesthesia, endotracheal intubation, and mechanical ventilation at the time of bronchoscopy and transbronchial biopsy probably decreases the likelihood of pneumothorax as a complication of the procedure.

Rigid bronchoscopy balloon catheter dilation for bronchial stenosis in infants.

Stenosis of the tracheobronchial tree can be a life-threatening problem. Management options for symptomatic stenosis include serial dilation, cryotherapy, laser resection, and open surgical correction. Recently, balloon angioplasty catheters have been used to dilate stenotic airway segments. The experience in infants is limited and has for the most part utilized guide wires and fluoroscopy for balloon placement. We present two infants with symptomatic bronchial stenosis who underwent endoscopic angioplasty balloon catheter dilation. Operative technique involved catheter placement under direct vision with a rigid bronchoscope. Catheters (6F) with 8-mm-diameter balloons were used. Balloon expansion was controlled with a hand-held manometer. Both infants demonstrated significant lumen size improvement intraoperatively and marked clinical improvement postoperatively, substantiated by endoscopy and radiographs. One infant has required one repeat dilation and has subsequently been asymptomatic. The other infant has had no further respiratory problems. Our technique, using a rigid bronchoscope with direct visualization of catheter placement, obviates the need for guide wires and C-arm fluoroscopy as previously described. Endoscopic placement enables direct visualization of balloon position, and fine adjustments are possible if further dilation is necessary. Rigid bronchoscopic balloon catheter dilation can be a successful technique for bronchial stenosis and should be considered prior to attempting more invasive surgical correction.

Diabetic ketoacidosis in cystic fibrosis.

OBJECTIVE: To differentiate the insulin-dependent glucose intolerance associated with cystic fibrosis from type I diabetes mellitus in patients with cystic fibrosis. DESIGN: Patient report. SETTING: Tertiary care referral center. PARTICIPANT: An 11-year-old boy with cystic fibrosis who developed diabetic ketoacidosis. MEASUREMENT/MAIN RESULT: Biochemical, immunologic, and molecular techniques were used to support the sporadic association of type I diabetes mellitus in a patient with cystic fibrosis. Cystic fibrosis was confirmed by sweat test and further supported by the demonstration of a heterozygous deletion of the F508 locus. Evidence for the diagnosis of type I diabetes mellitus was developed from the clinical presentation of diabetic ketoacidosis with hyperglycemia, ketonemia, and ketonuria. Immunologic evidence included the demonstration of anti-insulin antibodies. The demonstration of homozygous absence of aspartic acid at position 57 of the HLA DQ-beta chain placed this child at high risk of type I diabetes mellitus. CONCLUSION: The clinical presentation and the presence of immunologic and genetic markers characteristic of type I diabetes mellitus supports the concordance of cystic fibrosis and type I diabetes mellitus in this patient.

Expression of pulmonary metallothionein genes in late gestational lambs.

Metallothioneins (MT) are low molecular weight proteins that are important in providing protection against heavy metals such as cadmium. Other precise physiologic roles for this family of proteins are less clear, but fetal hepatic cell proliferation and differentiation may be regulated through changes in MT levels and attendant MT-mediated regulation of zinc levels. The role of MT in other developing tissue, most notably lung, is far less clear. Although expression of MT has been reported to be extremely low in early postnatal and mature lung, we hypothesized that MT has a more ubiquitous role in organ development and that pulmonary MT levels may be elevated during periods of rapid lung growth. Thus, we studied expression of MT in late-gestation fetal lambs. Sheep are particularly useful because alveolarization of lung parenchyma occurs before birth (by d 120 of a normal 147-d gestation). Immunoreactive MT was localized to bronchial epithelium of fetal, newborn, and mature sheep. The intensity of staining was greatest in the 130-d gestational age (saccular) lung, where positive reaction product was noted in the cytoplasm and nucleus of alveolar epithelial and interstitial cells. We next evaluated MT expression in developing lung tissue using Northern blot analysis and 32P-cDNA probes against the 3'-untranslated regions of mRNA encoding each of four known functional sheep MT (sMT) isoforms. Expression of sMT-II, sMT-Ia, and sMT-Ib was restricted to the saccular stage (120-132 d gestational age), and sMT-Ic mRNA was not detected in pulmonary samples from any stage of development.(ABSTRACT TRUNCATED AT 250 WORDS)

Pancreatitis in young children with cystic fibrosis.

Five young children with cystic fibrosis and abdominal pain were found to have pancreatitis. Diagnosis was delayed in four patients because of the belief that pancreatitis occurs only in older patients with cystic fibrosis. In one patient pancreatitis was diagnosed before cystic fibrosis and diagnosis of cystic fibrosis was delayed. Pancreatitis should be considered as a possible cause of abdominal pain in pancreatic-sufficient children with cystic fibrosis and cystic fibrosis should be considered as a possible cause of pancreatitis, even in the young child.

Arachidonate 12-lipoxygenase and cyclooxygenase: PGE isomerase are predominant pathways for oxygenation in bovine tracheal epithelial cells.

The capacity of bovine tracheal epithelial cells to convert arachidonic acid to oxygenation products with potential biologic activity was studied in homogeneous preparations of isolated cells. Purified epithelial cell suspensions were incubated with radiolabeled arachidonic acid, and oxygenated metabolites were identified using high-pressure liquid chromatography and gas chromatography-mass spectrometry. The cells released predominantly two products during incubation with 0.3 to 150 microM arachidonic acid for 1 to 60 min at 37 degrees C: prostaglandin E2 (PGE2) and 12-hydroxyeicosatetraenoic acid (12-HETE). Concentration-response curves for the two products yielded half-maximal effects at 2 and 45 microM arachidonic acid, respectively. Stereochemical analysis by chiral-phase high-pressure liquid chromatography demonstrated that the epithelial 12-HETE consisted exclusively of the 12(S) isomer, providing supporting evidence that it was derived from an arachidonate 12-lipoxygenase. Epithelial cells prelabeled with arachidonic acid and incubated with 5 microM A23187 to stimulate endogenous arachidonic acid metabolism also released two predominant products with the chromatographic properties of PGE2 and 12-HETE. The findings demonstrate that bovine tracheal epithelial cells express both a cyclooxygenase:PGE isomerase and a 12-lipoxygenase pathway and therefore implicate this pathway as a new source of epithelial cell mediators.