Hydroxyurea treatment effect on children with sickle cell disease and obstructive sleep apnea.

Background: While hydroxyurea is the mainstay of treatment for many of the comorbidities associated with sickle cell disease, its effect on obstructive sleep apnea has not been fully investigated. The purpose of this project is to help characterize the effects of hydroxyurea on obstructive sleep apnea in children with sickle cell disease and determine its therapeutic role in the condition.

Methods: Chart review was conducted on two pediatric patients with sickle cell disease who experienced resolution of obstructive sleep apnea following hydroxyurea administration.

Effects of adenotonsillectomy on polysomnographic parameters in children with sickle cell disease.

Obstructive sleep apnea (OSA) in the pediatric sickle cell disease (SCD) population can promote nightly hemoglobin oxygen desaturation, which increases the risk of central nervous system insult and may impair cognitive function. Adenotonsillectomy can ameliorate OSA symptoms, but its effect in children with SCD has not been fully investigated. We reviewed the effects of adenotonsillectomy in thirteen children with SCD by comparison of pre and post-adenotonsillectomy polysomnography (PSG) parameters.

Excessive daytime sleepiness and sleep-disordered breathing disturbances in survivors of childhood central nervous system tumors.

Background: Improvements in treatment and management for pediatric central nervous system (CNS) tumors have increased survival rates, allowing clinicians to focus on long-term sequelae, including sleep disorders. The objective of this study was to describe a series of CNS tumor survivors who had sleep evaluations that included polysomnography (PSG) with attention to sleep disorder in relation to the tumor site.

Procedure: We report on 31 patients who had retrievable reports including an overnight PSG; 17 also underwent multiple sleep latency tests (MSLT) to characterize their sleepiness.

Body mass index as an indicator of obstructive sleep apnea in pediatric Down syndrome.

Objective: Our objective was to determine if higher body mass index (BMI) increases the likelihood of, obstructive sleep apnea (OSA) in pediatric Down syndrome (DS) patients.

Methods: We performed a, retrospective chart review of 63 DS patients evaluated by overnight polysomnography from December 1995 to February 2005. Patients aged less than 2 years were excluded.

Recurrent stridor in a 9-year-old child after a choking and aspiration event.

This is a case report of a 9-year-old boy with new onset stridor 5 days after a choking event. Symptoms would last 5-45 minutes. His stridor was unresponsive to nebulized epinephrine but improved when he relaxed.

Psychometric and clinical assessment of the 10-item reduced version of the Fatigue Scale-Child instrument.

Fatigue is one of the most debilitating conditions associated with cancer and anticancer therapy. The lack of reliable and valid self-report instruments has prevented accurate assessment of fatigue in pediatric oncology patients. The purpose of this study was to identify the most sensitive and specific score, that is, the "cut score," on the Fatigue Scale-Child (FS-C) to identify those children with high cancer-related fatigue in need of clinical intervention.

Effects of adeno-tonsillectomy on polysomnography patterns in Down syndrome children with obstructive sleep apnea: a comparative study with children without Down syndrome.

Objective: To determine if adeno-tonsillectomy (T&A) in children with Down syndrome (DS) improves breathing, measured by apnea hypopnea index (AHI), rapid eye movement AHI (REM-AHI) and the lowest oxygen desaturation (SaO2), and sleep disruption, measured by arousal index (ArI) and time spent in stages 1-4 and rapid eye movement (REM) sleep and compare these results with a group of non-DS children with obstructive sleep apnea (OSA).

Study Design: Retrospective chart review at pediatric sleep center.

Patients: Eleven DS and nine non-DS children underwent pre- and post-T&A polysomnography between 1997 and 2005.

Airway evaluation and management in 7 children with malignant infantile osteopetrosis before hematopoietic stem cell transplantation.

Malignant infantile osteopetrosis (MIOP) is a rare disorder caused by dysfunctional osteoclasts. The classic MIOP features, such as frontal bossing, micrognathia, and small thorax, may place these children at risk for developing obstructive sleep apnea (OSA) and chronic hypoxemia. To objectively document OSA, airway evaluations were performed; results impacted management.

A clinical inflammatory syndrome attributable to aerosolized lipid-DNA administration in cystic fibrosis.

Immunologic reactivity to lipid-DNA conjugates has traditionally been viewed as less of an issue than with viral vectors. We performed a dose escalation safety trial of aerosolized cystic fibrosis transmembrane conductance regulator (CFTR) cDNA to the lower airways of eight adult cystic fibrosis patients, and monitored expression by RT-PCR. The cDNA was complexed to a cationic lipid amphiphile (GL-67) consisting of a cholesterol anchor linked to a spermine head group.

Plastic bronchitis occurring late after the Fontan procedure: treatment with aerosolized urokinase.

Objective: To describe the use of aerosolized urokinase in a patient with plastic bronchitis after a Fontan procedure.

Design: Case report.

Setting: Pediatric intensive care unit in a university-affiliated children's hospital.

Evaluation and management of hemoptysis in infants and children. A report of nine cases.

Hemoptysis is an occasional complication of adult pulmonary disease and is rare in children. The most common causes in adults are infection, bronchiectasis, pulmonary neoplasm, cystic fibrosis, pulmonary infarct, and trauma; in children the most common causes are infection and congenital abnormalities of the cardiopulmonary vasculature. Nine cases of hemoptysis in seven infants and two children will be presented.

Comparison of manual and mechanical chest percussion in hospitalized patients with cystic fibrosis.

We compared the efficacy of manual and mechanical chest percussion during hospitalization for acute exacerbations of cystic fibrosis by evaluating changes in spirometry values. Fifty-one participants were randomly assigned to receive manual or mechanical chest percussion three times a day. Twenty-two participated during one subsequent admission and were assigned to the opposite form of chest percussion.

Cystic fibrosis mutations in white and black Americans: an approach to identification of unknown mutations with implications for cystic fibrosis screening.

Objective: We sought to define the frequency of common cystic fibrosis mutations in white and black American probands from our geographic region.

Study Design: Mutation analysis was performed for delta F508, G551D, G542X, R553X, S549N, an d N1303K. For probands with undetermined mutations single-stranded conformational polymorphism analysis was performed.

cAMP-activated Cl channels in CFTR-transfected cystic fibrosis pancreatic epithelial cells.

Retrovirus-mediated transfection of cDNA for the cystic fibrosis (CF) gene into the CF pancreatic cell line, CFPAC-1, confers adenosine 3',5'-cyclic monophosphate (cAMP)-dependent regulation of Cl conductance. We used patch-clamp techniques to identify the single-channel basis of this conductance pathway and to study its properties. Forskolin or cAMP activated Cl channels with a conductance of 9 +/- 1 pS in 26 of 62 cell-attached patches of cystic fibrosis transmembrane conductance regulator (CFTR)-transfected CFPAC-1 cells.

Characterization of trypsinogens 1 and 2 in two human pancreatic adenocarcinoma cell lines; CFPAC-1 and CAPAN-1.

Proteins with trypsin-like immunoreactivity (first detected by a specific immunoenzymatic assay) were isolated from CAPAN-1 and CFPAC-1 cell culture-conditioned media by chromatography on an immunoadsorbent prepared with a polyclonal antibody directed against trypsin 1. The adsorbed proteins were devoid of free trypsin activity but trypsin activity was present after enterokinase activation demonstrating that the immunoreactive trypsin present in cell supernatants corresponds to trypsinogens. When characterised by Western blotting using a monoclonal antibody directed against human trypsin 1 two protein bands corresponding to trypsinogen 1 (23 kDa) and trypsinogen 2 (25 kDa) gave a positive reaction.

A cystic fibrosis pancreatic adenocarcinoma cell line.

We established a pancreatic adenocarcinoma cell line (CFPAC-1) from a patient with cystic fibrosis (CF) and assessed some of its properties. The cells show epithelial morphology and express cytokeratin and oncofetal antigens characteristic of pancreatic duct cells. Basal and stimulated levels of cAMP and cAMP-dependent protein kinase and the biophysical properties of single Cl- channels in CFPAC-1 are similar to those of airway and sweat gland primary cultures and Cl(-)-secreting epithelial cell lines.

Enhanced clearance of vancomycin by hemodialysis in a child.

Clearance of vancomycin by hemodialysis has previously been reported to be negligible. We describe a child undergoing chronic hemodialysis using a hollow fiber dialyzer with cellulose acetate membrane. When the patient was treated with intravenous vancomycin for staphylococcal bacteremia, the serum vancomycin half-life was found to decrease by more than 90% during each course of hemodialysis.

Apical membrane chloride channels in a colonic cell line activated by secretory agonists.

We characterized the anion channel responsible for the increase in apical membrane Cl secretion using a model salt-secreting epithelium, the T84 colonic cell line. The adenosine 3',5'-cyclic monophosphate (cAMP)-mediated secretagogues, prostaglandin E2, forskolin, and 8-bromo-cAMP, evoked activity of an outwardly rectifying Cl channel in previously quiet cell-attached membrane patches. The channel remained active in excised, inside-out membranes, where its single-channel conductance was 40-45 pS at 0 mV with 160 mM NaCl in pipette and bath.

Biophysical properties of a chloride channel in the apical membrane of a secretory epithelial cell.

1. Patch clamp studies on colonic tumor cell line T84 show the presence of chloride channels. 2.

Phosphorylation fails to activate chloride channels from cystic fibrosis airway cells.

Chloride impermeability of epithelial cells can account for many of the experimental and clinical manifestations of cystic fibrosis (CF). Activation of apical-membrane Cl- channels by cyclic AMP-mediated stimuli is defective in CF airway epithelial cells, despite normal agonist-induced increases in cellular cAMP levels. This defect in Cl- channel regulation has been localized to the apical membrane by exposing the cytoplasmic surface of excised membrane patches to the catalytic subunit (C subunit) of cAMP-dependent protein kinase and ATP.