Case of the missing penny: thoracoscopic removal of a mediastinal coin

Complications of coin ingestion in children, although rare, include esophageal perforation, tracheoesophageal fistula, esophago-aortic fistula, and death. The authors describe thoracoscopic removal of a mediastinal coin that migrated extraluminally from the esophagus in a 23-month-old girl. Right-sided thoracoscopic exploration using a 3-trocar technique in a modified prone position was used. Coin location was assisted by manipulation of a transorally placed Foley catheter and intraoperative fluoroscopy. The coin was retrieved successfully with no intraoperative or postoperative complications and minimal postoperative pain. This is the first report of successful thoracoscopic removal of a mediastinal coin. Thoracoscopy may be a valuable approach for mediastinal foreign body removal in children.     

Intravenous cyclophosphamide-resistant systemic lupus erythematosus in Arizona

Systemic lupus erythematosus (SLE) tends to be severe and to have a variable response in childhood. We undertook this retrospective study to assess response rates and outcome in 14 children with SLE. Mean age at onset was 12.8±3.1 years. Ten patients were female and 4 were male, and 12 patients (86%) were Hispanic. Creatinine clearance prior to therapy was 104±36 ml/min. All had hematuria and proteinuria with a protein/creatinine ratio of 3.9±4.8. WHO classification of renal biopsies revealed class IV in 64%, class III in 21%, and class V in 14%. Patients were treated with 6-monthly pulses of intravenous cyclophosphamide (IVCY) followed by longer-duration pulses. The mean duration of follow-up was 3.7±3.3 years. Of the 14 patients, 3 (21%) achieved systemic remission but all relapsed subsequently; 7 of 14 achieved renal remission, although 6 relapsed. Six (42%) had adverse outcomes, defined by death, dialysis, or need for bone marrow transplant. All 6 had failed 6 months of IVCY, suggesting that patients who demonstrate resistance to initial IVCY therapy have an unfavorable outcome and a high likelihood of complications. In summary, we report a poor response to standard therapeutic protocols with higher relapse rates, as well as significant adverse outcomes.     

A comparative study of the effect of oxidative stress on the cytoskeleton in human cortical neurons

Cytoskeleton disruption is a process by which oxidative stress disrupts cellular function. This study compares and contrasts the effect of oxidative stress on the three major cytoskeleton filaments, microfilaments (MFs), microtubule (MT), and vimentin in human cortical neuronal cell line (HCN2). HCN2 cells were treated with 100 microM tertiary butylhydroperoxide (t-BuOOH), a free radical generating neurotoxin for 1, 3, or 6 h. Cell viability studies demonstrated significant cell death although the morphology studies showed that there was a substantial loss in neurites of neurons treated with t-BuOOH for 6 h. Because the cytoskeleton plays a role in neurite outgrowth, the effect of oxidative stress on the cytoskeletal was studied. In neurons subjected to oxidative stress for 30 min or 1 h, there were no major changes in microfilament distribution though there was altered distribution of microtubule and vimentin filaments as compared to controls. However, loss and disruption of all the three cytoskeletal filaments was observed at later times (3 and 6 h), which was confirmed by Western Blot analysis. Further studies were done to measure the gene expression levels of actin, tubulin, and vimentin. Results indicated that the overall loss of the cytoskeletal proteins in neurons treated with free radical generating toxin might not be a direct result of the downregulation of the cytoskeletal genes. This study shows that free radical generation in human neurons leads to the disruption of the cytoskeleton, though there may be a difference in the susceptibility to oxidative stress among the individual components of the cytoskeletal filaments.     

Hypophosphatemic rickets and osteomalacia in polyostotic fibrous dysplasia

A 17 year-old girl with polyostotic fibrous dysplasia and hypophosphatemia had inappropriately low tubular reabsorption of phosphate. She had radiological evidence of rickets and osteomalacia. The patient showed clinical improvement after treatment with phosphate supplementation, active vitamin D (calcitriol) and alendronate. It is postulated that either a phosphaturic substance elaborated from the dysplastic bone or target-organ (kidney) unresponsiveness may interfere with phosphate reabsorption in the renal tubule.     

Pheochromocytoma associated with pregnancy

Pheochromocytoma associated with pregnancy is rare with potentially lethal consequences. Antepartum diagnosis improves the maternal and perinatal outcome. The issue of mode of delivery is unresolved. Its definitive treatment is surgical resection preceded by medical management. Surgical resection may be done during caesarean section as is reported in the present case.     

Septo-optic dysplasia,subglottic stenosis and skeletal abnormalities: a case report

We report a girl with septo-optic dysplasia in association with subglottic stenosis, sagittal craniosynostosis, osteoporosis and dental anomalies. It is uncommon for patients with septo-optic dysplasia to have multiple, extra-cranial malformations. A number of differential diagnoses were considered in this case, including Cole-Carpenter syndrome, Pfeiffer syndrome and osteoglophonic dwarfism. However, none can account for all the abnormalities seen. We therefore believe that this is a previously unreported, but highly distinctive, phenotype.     

A rapid 'one-plate' in vitro test for pyrogens

A rapid, 'one-plate' monocyte-activation test is described for detecting endotoxin and non-endotoxin pyrogens in parenteral medicinal products. The one-plate test offers useful gains over conventional 'two-plate' (cell culture plate+ELISA plate) tests in terms of its limit of detection, robustness, speed and cost. The 'one-plate' test is likely to be applicable to a wide range of products because it allows less time for product interference in the test. The 'one-plate' test utilises pyrogen-free anti-cytokine (interleukin (IL)-6 or tumour necrosis factor alpha (TNFalpha)) antibodies (Ab), coated and stabilised onto (pyrogen-free) 96-well plates. Monocytes/monocytic cells, endotoxin (lipopolysaccharides, LPS) standard or sample and (pyrogen-free) second (labelled) Ab are cultured together (usually for 2-4 h) on the Ab-coated plate and then the plate is washed and the ELISA completed. There is no transfer from one plate to another and no (further) incubations of (released) cytokine with, first, coating Ab and, then, developing Ab since these steps have already taken place during the initial cell culture. The rapid, 'one-plate' test is readily automated. The preferred readout is IL-6, which gives a limit of detection of 0.015 endotoxin units (EU)/ml with peripheral blood mononuclear cell (PBMNC), 0.03 EU/ml with diluted whole blood and 0.05 EU/ml with a monocytic cell line (MONO MAC 6).     

Hydrogel tissue adhesive for sealing corneal incisions

The purpose of the study was to assess the efficacy of a newly developed hydrogel tissue adhesive for sealing corneal incisions in ex vivo rabbit eyes. Simple through-and-through central linear corneal incisions measuring 1-5 mm were created in enucleated rabbit eyes. As controls, the leaking pressures were measured immediately after the incisions had been made. Afterwards, the incisions were sealed with hydrogel adhesive in group 1 and with a single 10-0 nylon suture in group 2. The leaking pressure of each incision was measured after the wounds had been sealed in both groups. The leaking pressures of the controls and the sealed incisions were compared for statistical significance using the Wilcoxon signed rank test. The difference between the leaking pressures before and after sealing the incisions in groups 1 and 2 were compared using the Mann-Whitney rank sum test. The leaking pressures were significantly higher after sealing in both groups 1 and 2 compared to the controls for all incision sizes. The differences in the leaking pressures before and after adhesive application were higher than before and after suturing in all incision sizes. The differences were all statistically significant except for the 1-mm incisions. We conclude that the hydrogel glue has the potential to be used for sealing corneal incisions measuring up to 5 mm.