Pulmonary metastases after nephrectomy only for small, stage I/favorable-histology Wilms' tumor.

The prognosis for children with Wilms' tumor is reported to be excellent in those who are less than 2 years of age at diagnosis and who have a stage I/favorable-histology tumor with specimen weight less than 550 g. We report on a patient with Wilms' tumor who belonged to this group but who developed pulmonary metastases, and we discuss the diagnostic and therapeutic problems in such patients. The importance of careful evaluation of the renal sinus should be emphasized.

Investigation of methyl tert-butyl ether levels in river-, ground-, and sewage-waters analyzed using a purge-and-trap interfaced to a gas chromatograph-mass spectrometer.

River water collected from 27 sites in 21 rivers, groundwater from 66 sites in 34 cities, and 2 sewage plants in the Niigata Prefecture, Japan (total 301 samples) were analyzed for methyl tert-butyl ether (MTBE) using an improved purge-and-trap-GC-MS method. The amount of MTBE found in water samples from the 27 river sites ranged from 0.003 to 5.

Remission induced by interferon alfa in a patient with massive osteolysis and extension of lymph-hemangiomatosis: a severe case of Gorham-Stout syndrome.

The treatment of massive osteolysis with lymphangioma and/or hemangioma (Gorham-Stout syndrome) has been controversial. The authors report on a patient with multiple massive osteolyses and extensive lymph-hemangiomatosis whose lesions were reduced by interferon alfa therapy. A 2-year-old girl had complained of left chylothorax.

Usefulness of epiphyseal quotient measurement on magnetic resonance images for outcome prediction in patients with early-stage Legg-Calve-Perthes disease.

To investigate the usefulness of epiphyseal quotient (EQ) measurement for outcome prediction in patients with Legg-Calve-Perthes disease (LCPD), magnetic resonance (MR) imaging was performed in 26 patients with unilateral LCPD within 6 months of the disease onset and subsequently once every 3 months. All the patients were treated conservatively in our institute between 1990 and 1999. The mean follow-up period was 80 months.

Evaluation of radial optic neurotomy for central retinal vein occlusion by indocyanine green videoangiography and image analysis.

Purpose: To evaluate the effects of radial optic neurotomy (RON) on retinal circulation in patients with central retinal vein occlusion (CRVO) by indocyanine green (ICG) videoangiography and a computer-assisted image analysis.

Design: An interventional case series.

Methods: RON was performed in 15 eyes of 15 patients with CRVO.

Gastrointestinal manometric findings in a patient with total intestinal aganglionosis.

Background/purpose: The authors studied gastrointestinal motility in a patient with total intestinal aganglionosis (TIA) and the effect of octreotide (OCT) on ileal motility in this patient.

Methods: The 3200-g girl received ileostomy at 50 cm proximal to the ileocecal site and jejunostomy at 15 cm distal to the ligament of Treitz because of severe ileus owing to TIA. Histology of the intestines, including jejunostomy, showed no ganglion cells.

An optical telemetry system for underwater recording of electromyogram and neuronal activity from non-tethered crayfish.

We have developed an optical telemetry system for recording electrical signals associated with muscle and neuronal activities from freely walking crayfish under water. The device was made from conventional electronic parts which are commercially available, utilizing infrared light (880 nm) for signal transmission. Two or four channels of biological signals were multiplexed, the voltage of each data point modulated to the duration of subcarrier pulses and further to the interval of narrower carrier pulses that directly drove the infrared light emission diode (IRLED) under water.

Time-frequency analysis of electronystagmogram signals in patients with congenital nystagmus.

Purpose: To evaluate the usefulness of the short-time Fourier transform (STFT) in the analysis of temporal congenital nystagmus characteristics.

Methods: We carried out an observational case series in patients with congenital nystagmus. An electronystagmogram (ENG) recording was performed for 300 s in 13 consecutive subjects with congenital nystagmus (age: 7-68 years).

Dual signaling and effector pathways mediate human eosinophil activation by platelet-activating factor.

Platelet-activating factor (PAF) induces various cellular functions in eosinophils including chemotaxis, adhesion, superoxide anion (O2-) production, and degranulation. While PAF shares many biological effects with other chemotactic factors such as N-formyl-methionyl-leucyl-phenylalanine, complement fragments, and lipid mediators, PAF is unique in that its action is relatively resistant to pertussis toxin (PTX), and in activating eosinophils more strongly than neutrophils. In this review we consider how PAF might activate human eosinophils in preference to neutrophils, and discuss possible mechanisms of PAF-induced activation of human eosinophils via two distinct signaling and effector pathways.

NMR structure of the N-terminal domain of SUMO ligase PIAS1 and its interaction with tumor suppressor p53 and A/T-rich DNA oligomers.

A member of the PIAS (protein inhibitor of activated STAT) family of proteins, PIAS1, have been reported to serve as an E3-type SUMO ligase for tumor suppressor p53 and its own. It also was proposed that the N-terminal domain of PIAS1 interacts with DNA as well as p53. Extensive biochemical studies have been devoted recently to understand sumoylations and its biological implications, whereas the structural aspects of the PIAS family and the mechanism of its interactions with various factors are less well known to date.

Evaluation of arteriovenous crossing sheathotomy for branch retinal vein occlusion by fluorescein videoangiography and image analysis.

Purpose: We quantitatively evaluated the effects of arteriovenous (A/V) crossing sheathotomy on retinal circulation in patients with branch retinal vein occlusion (BRVO) accompanied by macular edema.

Design: Interventional case series.

Methods: In 18 consecutive patients (18 eyes) with BRVO accompanied by macular edema who underwent A/V crossing sheathotomy between August 1999 and April 2002, changes in retinal circulation after the surgery were evaluated by fluorescein videoangiography with a scanning laser ophthalmoscope and by image analysis using dye dilution technique.

A trial of intranasal ACTH(1-24) administration to a patient with isolated ACTH deficiency.

Background: Isolated adrenocorticotropic hormone (ACTH) deficiency is a rare cause of secondary adrenocortical insufficiency. Normally it could be used therapeutically as an alternative to glucocorticoid treatment in these patients. We investigated the possibility of therapeutic approach as intranasal ACTH replacement therapy in patients with ACTH deficiency.

Neonatal ovarian cysts: management with reference to magnetic resonance imaging.

Objective: Ultrasound (US) has been used as a tool to determine the indication for surgery for neonatal ovarian cysts. The purpose of this study was to investigate whether magnetic resonance imaging (MRI) contributes to optimal management.

Methods: Between 1993 and 2001, US and MRI studies were simultaneously performed on 13 consecutive infants younger than 2 months of age with ovarian cysts.

Evidence for segmental bile drainage by hepatic portoenterostomy for biliary atresia: Cholangiographic, hepatic venographic, and histologic evaluation of the liver taken at liver transplantation.

Background: The result of hepatic portoenterostomy for biliary atresia (BA) has improved, but there are some patients who experience worsened liver function in the long term after one decrease in jaundice owing to portoenterostomy. However, the cause of the liver dysfunction in the long term has not been clearly ascertained.

Methods: Five patients (5 to 28 years of age) with BA underwent liver transplantation (LT) because of liver dysfunction after successful portoenterostomy.

Intestinal aganglionosis associated with the Waardenburg syndrome: report of two cases and review of the literature.

The authors report two cases of the rare concurrence of intestinal aganglionosis and Waardenburg syndrome in Japanese infants. The patients were a 1-month-old girl and a 3-month-old boy at diagnosis, and both of them had either short segment or ultra-short segment aganglionosis. A review of 48 cases in the literature showed that the extent of the aganglionic segment is quite variable, from nearly total to ultra-short.

Current treatment and future directions in neuroblastoma.

Objective: The International Neuroblastoma Staging System (INSS) and Pathology Classification (INPC) were applied to analyze the results of treatment of 644 patients with neuroblastoma treated in Japan during the years from 1995 to 1999, and it was found that the pathology classification (INPC) showed the strongest relevance to prognosis compared to other factors such as stage, MYNC amplification, DNA ploidy and 1p-deletion. Current results of treatment for advanced neuroblastoma are still not satisfactory, so innovative therapeutic methods have been sought during the past 10 years.

Methods: Prospects for irinotecan and recombinant human endostatin (rhEndostatin) were studied expertimentally and clinically.

Evaluation of the dynamics of choroidal circulation in experimental acute hypertensionusing indocyanine green-stained leukocytes.

Purpose: To evaluate the dynamics of choroidal circulation in experimental acute hypertension, using the indocyanine green leukocyte angiography (ILA) method, which the authors have developed for the evaluation of leukocyte dynamics in choroidal circulation.

Methods: Japan White rabbits were used in the present study. Leukocytes were collected by centrifugal separation of the autologous blood, and were stained with indocyanine green (ICG) dye.

Continuous versus intermittent administration of human endostatin in xenografted human neuroblastoma.

Purpose: The authors examined whether recombinant human endostatin (rhEndostatin), an antiangiogenic agent, is effective against a human neuroblastoma cell line (designated TNB9) using a human neuroblastoma xenograft model and investigated whether continuous infusion is more effective than intermittent administration.

Methods: In the first experiment, when tumors on the back of nude mice reached a weight of 90 to 95 mg, rhEndostatin, 10 mg/kg/d mouse weight, was administered subcutaneously to the mice (n = 5) every day for 10 consecutive days. In the second experiment, the same daily dose of rhEndostatin was administered continuously to the TNB9-bearing mice (n = 6) via subcutaneous infusion pumps for 3 consecutive days with total dose being 30% of that in the first experiment.

Aberrations of p16INK4A, p14ARF and p15INK4B genes in pediatric solid tumors.

Expression of the p16INK4A (p16), p15INK4B (p15), and p14ARF genes, located at 9p21, was examined in pediatric neuroblastoma (NB), Ewing's sarcoma (ES), and rhabdomyosarcoma (RMS). p16 expression was absent in 4 of 5 ESs, and 2 of these 4 cases died. p16 expression was reduced or absent in 10 of 12 RMSs, and 4 of these 10 cases died.

Emergency endovascular stent-grafting for infected pseudoaneurysm of brachial artery.

The use of covered stents in an infected field is controversial. It is generally recommended that infected aneurysms be treated using autografts or allografts. We report a case of infected brachial pseudoaneurysms that developed after medical debridement of a methicillin-resistant Staphylococcus aureus (MRSA)-infected wound of the right arm and emergency brachial artery bypass-grafting using the saphenous vein, which was successfully treated by endovascular stent-grafting followed by antibiotic administration.

Estimated daily intake of plasticizers in 1-week duplicate diet samples following regulation of DEHP-containing PVC gloves in Japan.

Duplicate hospital diet samples obtained over 1 week in 2001 were analysed to estimate the daily intake of plasticizers and the results were compared with those obtained in 1999. The plasticizers quantified in this study were: dibutyl phthalate, butylbenzyl phthalate (BBP), di(2-ethylhexyl) phthalate (DEHP), diisononyl phthalate (DINP), di(2-ethylhexyl) adipate (DEHA), diisononyl adipate (DINA) and O-acetyl tributyl citrate (ATBC). Dipropyl, dipentyl, dihexyl and dicyclohexyl phthalate were also analysed but not detected.

Intrahepatic biliary cysts in biliary atresia in the era of liver transplantation.

Objectives: The development of intrahepatic biliary cysts (IBC) after Kasai operation in patients with biliary atresia (BA) is recognized as an important problem; however, management strategy for IBC has not been clarified, particularly in the light of the increased use of liver transplantation.

Methods: Forty consecutive BA patients underwent hepatic portoenterostomy during 18 years from 1983 to 2000. We compared the clinical course and prognosis of the patients who developed IBC with those who did not.