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Materials coated with aqueous fish protein extracts can reduce bacterial adhesion, but the mechanism behind the observed effect is not fully understood. In this study we explore the physicochemical properties of fish muscle protein adlayers on four substrates: gold, stainless steel, polystyrene and silicon dioxide. The aims were (i) to determine if the anti-adhesive effect is independent of the underlying substrate chemistry, (ii) to link the physicochemical properties of the adlayer to its ability to repel bacteria, and (iii) to elucidate the mechanism behind this effect. The main proteins on all surfaces were the muscle proteins troponin, tropomyosin, and myosin, and the lipid binding protein apolipoprotein. The quantity, viscoelasticity, and hydration of the protein adlayers varied greatly on the different substrates, but this variation did not affect the bacterial repelling properties. Our results imply that these proteins adsorb to all substrates and provide a steric barrier towards bacterial adhesion, potentially providing a universal antifouling solution., (Copyright © 2012 Elsevier B.V. All rights reserved.)

Mesenchymal stem cells (MSCs) have been shown to improve the outcome of acute renal injury models; but whether MSCs can delay renal failure in chronic kidney disease (CKD) remains unclear. In the present study, the were cultured in media containing various concentrations of basic fibroblast growth factor, epidermal growth factor and ascorbic acid 2-phosphate to investigate whether hepatocyte growth factor (HGF) secretion could be increased by the stimulation of these growth factors. Then, TGF-β1-treated renal interstitial fibroblast (NRK-49F), renal proximal tubular cells (NRK-52E) and podocytes were co-cultured with conditioned MSCs in the absence or presence of ascorbic acid 2-phosphate to quantify the protective effects of conditioned MSCs on renal cells. Moreover, male Sprague-Dawley rats were treated with 1 × 10(6) conditioned MSCs immediately after 5/6 nephrectomy and every other week through the tail vein for 14 weeks. It was found that basic fibroblast growth factor, epidermal growth factor and ascorbic acid 2-phosphate promoted HGF secretion in MSCs. Besides, conditioned MSCs were found to be protective against TGF-β1 induced epithelial-to-mesenchymal transition of NRK-52E and activation of NRK-49F cells. Furthermore, conditioned MSCs protected podocytes from TGF-β1-induced loss of synaptopodin, fibronectin induction, cell death and apoptosis. Rats transplanted with conditioned human MSCs had a significantly increase in creatinine clearance rate, decrease in glomerulosclerosis, interstitial fibrosis and increase in CD4(+)CD25(+)Foxp3(+) regulatory T cells counts in splenocytes. Together, our studies indicated that conditioned MSCs preserve renal function by their anti-fibrotic and anti-inflammatory effects. Transplantation of conditioned MSCs may be useful in treating CKD., (© 2012 The Authors Journal of Cellular and Molecular Medicine © 2012 Foundation for Cellular and Molecular Medicine/Blackwell Publishing Ltd.)

Background & Aims: Essential trace elements are involved in many biological processes for normal cell function including immunological defense against oxidation and infection. Deficiency of these elements generally leads to illness or even death in the general population. Therefore, we investigated the predictive values of trace element status on clinical outcomes in dialysis patients, who are more prone to trace element deficiency., Methods: We enrolled 111 prevalent patients on maintenance dialysis from a Taipei tertiary-care referral hospital and measured serum levels of selenium, copper, and zinc. Patients were followed for 2 years or until death or withdrawal., Results: Multivariate Cox regression analysis indicated that patients with diabetes mellitus (HR, 2.162 [95% CI, 1.105-4.232], p=0.024), prior stroke (HR, 3.876 [95% CI, 1.136-13.221], p=0.030), and zinc deficiency (HR, 0.979 [95% CI, 0.966-0.992], p=0.002) were more likely to be hospitalized for infectious diseases. Furthermore, beyond traditional risk factors, such as old age and hypoalbuminemia, multivariate Cox regression also indicated that lower serum level of zinc independently predicts overall mortality (HR, 0.973 [95% CI, 0.948-0.999], p=0.046)., Conclusions: In long-term dialysis patients, the serum level of zinc was an independent predictor of future hospitalization due to infectious diseases and of overall mortality., (Crown Copyright © 2012. Published by Elsevier Ltd. All rights reserved.)

Introduction: Spinal cord decompression sickness (DCS) is a rare condition that can lead to spinal cord infarction. Despite the low incidence of diving-related DCS, we have managed to collect the data and MRI findings of seven patients who have been diagnosed with and treated for DCS in our local hyperbaric facility. This study describes the clinical presentation, MRI spinal cord findings, treatment administered and outcome of these patients., Methods: The patient medical records, from 1997 to 2007, were retrospectively reviewed. All patients with a final diagnosis of DCS and who underwent examination were included. The images were independently reviewed by two radiologists who recorded the location and number of lesions within the spinal cord. The Frankel grading was used to assess the initial and clinical outcome response., Results: Patchy-increased T2W changes affecting several levels at the same time were found. Contrary to the popular notion that venous infarction is the leading cause of DCS, most of our patients also demonstrated affliction of grey matter, which is typically seen in an arterial pattern of infarction. Initial involvement of multiple (>6) spinal cord levels was associated with a poor outcome. Patients who continued to have multiple neurological sequelae with less than 50% resolution of symptoms despite recompression treatment were also those who had onset of symptoms within 30 min of resurfacing., Conclusions: DCS is probably a combination of both arterial and venous infarction. Short latency to the onset of neurological symptoms and multilevel cord involvement may be associated with a poorer outcome., (© 2012 The Authors. Journal of Medical Imaging and Radiation Oncology © 2012 The Royal Australian and New Zealand College of Radiologists.)

Mutations in the MID1 gene result in X-linked Opitz G/BBB syndrome (OS), a disorder that affects development of midline structures and comprises hypertelorism, cleft lip/palate, hypospadias, and laryngo-tracheo-esophageal abnormalities, and, at times, neurological, anal, and cardiac defects. MID1 gene abnormalities include missense, nonsense, and splicing mutations, small insertions, small deletions, and complex rearrangements. Here, we present a patient with Opitz G/BBB syndrome and a unique MID1 gene point mutation c.1703T

Objective: There has been very limited research on the clinical features of newborns exposed to combined use of heroin, methadone, and amphetamine in the uterus. We describe a technique for the quantification of drug metabolites in neonatal hair samples., Methods: In a tertiary neonatal care center in Taiwan, three neonates whose mothers self-reported heroin abuse with methadone treatment during pregnancy were studied. Involuntary exposure to amphetamine was not suspected before the births. To assess long-term illicit drug exposure during pregnancy, a quantifying technique of gas chromatography/mass spectrometry (GC/MS) for hair samples from neonates was developed to replace current methods for urine and blood specimens., Results: All three mothers were addicted to heroin and prescribed oral methadone treatment during pregnancy. Two males and one female were born and then admitted to the neonatal intensive care unit because of apparent neonatal abstinence syndrome (NAS) after birth. Additional hypertonicity and cerebral dysfunction were also diagnosed by electroencephalography in one case. Supportive care was given to the neonates, unless special treatments were needed in responding to tachypnea, fetal distress, or withdrawal symptoms. During follow-up periods from 10 months to 15 months, the signs of NAS remained and delays in milestones of development were observed. Further follow-up on the infants' neurobehavioral development is necessary. Measurement results of neonates' hair samples revealed high levels of metabolites of heroin, methadone, and amphetamine, reflecting the amount of illicit drug exposure 2-3 months before delivery., Conclusion: The current study suggested the possibility of polydrug exposure, which was previously unknown in pregnant women in Taiwan. Measurement of neonatal hair samples could provide a basis for clinical evaluation and potential corresponding treatment., (Copyright © 2012. Published by Elsevier B.V.)

We report on a newborn girl with facial anomalies, a congenital heart defect, severe pre- and postnatal growth retardation, feeding problems, and persistent hyperplastic primary vitreous. Cytogenetic analysis by high resolution GTG banding showed extra chromosomal material on the short arm of one chromosome 1 of the patient, but neither parent. SKY and CGH analysis demonstrated that the patient had a de novo 46,XX, der(1)t(1;6)(p36.3; p22). Compared with previously reported cases of partial trisomy 6p22 syndrome, this patient exhibited a unique condition for this syndrome: persistent hyperplastic primary vitreous (PHPV) with retinal detachment. The human genome database was searched for candidate genes and we propose the following nine genes located in the 6p22→6pter region for their potential contribution to the phenotype of partial trisomy 6p22→pter and persistent hyperplastic primary vitreous (PHPV) with retinal detachment: Forkhead box Q1 (FOXQ1), FOXF2, FOXC1, NRN1, EDN1, ATXN1, DEK oncogene, E2F3, and NRNS1., (Copyright © 2012 Wiley Periodicals, Inc.)

Background: Erythromycin is generally used as a prokinetic agent for the treatment of feeding intolerance in preterm infants; however, results from previous studies significantly vary due to different medication dosages, routes of administration, and therapy durations. The effectiveness and safety of intermediate-dose oral erythromycin in very low birth weight (VLBW) infants with feeding intolerance was examined in this study., Methods: Between November 2007 and August 2009, 45 VLBW infants with feeding intolerance, who were all at least 14 days old, were randomly allocated to a treatment group and administered 5mg/kg oral erythromycin every 6hours for 14 days (n=19). Another set of randomly selected infants was allocated to the control group, which was not administered erythromycin (n=26)., Results: The number of days required to achieve full enteral feeding (36.5±7.4 vs. 54.7±23.3 days, respectively; p=0.01), the duration of parenteral nutrition (p<0.05), and the time required to achieve a body weight ≥2500g (p<0.05) were significantly shorter in the erythromycin group compared with the control group. The incidence of parenteral nutrition-associated cholestasis (PNAC) and necrotizing enterocolitis (NEC) ≥ stage II after 14 days of treatment were significantly lower (p<0.05) in the erythromycin group. No significant differences were observed in terms of the incidences of sepsis, bronchopulmonary dysplasia, or retinopathy of prematurity. No adverse effects were associated with erythromycin treatment., Conclusions: Intermediate-dose oral erythromycin is effective and safe for the treatment of feeding intolerance in VLBW infants. The incidences of PNAC and ≥ stage II NEC were significant lower in the erythromycin group., (Copyright © 2012. Published by Elsevier B.V.)

Herlyn-Werner-Wunderlich (HWW) syndrome is a rare variant of Müllerian duct anomalies consisting of uterine didelphys, obstructed hemivagina, and ipsilateral renal agenesis. Patients with HWW syndrome are usually asymptomatic until menarche, when they present with acute lower abdominal pain. Here we report a case of a female newborn with right renal agenesis diagnosed during the pregnancy. The patient presented with a protruding mass over the vaginal introitus that was associated with an obstructed hemivagina and uterine didelphys., (Copyright © 2012. Published by Elsevier B.V.)

Aims: We investigated dialysis duration, dose of erythropoietin (EPO), and clinical manifestations in peritoneal dialysis (PD) patients with subclinical hypothyroidism., Methods: This cross-sectional study, performed in 3 centers, assessed 122 adult patients on PD for more than 6 months with regard to demographic data, dialysis duration, thyroid function, biochemical data, EPO dose, and clinical manifestations. Thyroid dysfunction was determined by serum thyroid-stimulating hormone, free thyroxine, total thyroxine, total triiodothyronine, antithyroid peroxidase antibodies, and auto-antibodies against thyroglobulin., Results: Of the 122 study patients, 98 (80.3%) were assessed as having euthyroidism; 19 (15.6%), subclinical hypothyroidism; and 5 (4.1%), subclinical hyperthyroidism. The proportion of women (74.2% vs. 57.1%, p = 0.038), the mean duration of PD (58.1 months vs. 37.9 months, p = 0.032), and the weighted mean monthly EPO dose (1.22 μg/kg vs. 1.64 μg/kg, p = 0.009) were significantly higher in the subclinical hypothyroidism group than in the euthyroidism group, but the prevalences of coronary artery disease and cerebrovascular disease were not. From the multivariate model, PD duration was more significant than sex as a risk factor for subclinical hypothyroidism (p = 0.0132)., Conclusions: Subclinical hypothyroidism is frequent in PD patients, especially female patients and patients with a longer PD duration. Compared with euthyroid patients, patients with subclinical hyperthyroidism need a higher dose of EPO to maintain a stable hemoglobin level.

Ankle-brachial index (ABI) is an accurate tool to diagnose peripheral arterial disease. The aim of this study was to evaluate whether ABI is also a good predictor of renal outcome and cardiovascular events in patients with chronic kidney disease (CKD). We enrolled 436 patients with stage 3-5 CKD who had not been undergoing dialysis. Patients were stratified into two groups according to the ABI value with a cut point of 0.9. The composite renal outcome, including doubling of serum creatinine level and commencement of dialysis, and the incidence of cardiovascular events were compared between the two groups. After a median follow-up period of 13 months, the lower ABI group had a poorer composite renal outcome (OR = 2.719, P = 0.015) and a higher incidence of cardiovascular events (OR = 3.260, P = 0.001). Our findings illustrated that ABI is a powerful predictor of cardiovascular events and renal outcome in patients with CKD.

Myotubular myopathy is a rare congenital disease characterized by hypotonia and respiratory compromise at birth in affected males. It causes high neonatal mortality. Most surviving newborns need prolonged ventilation and have significantly delayed motor development. Although all patients with congenital myotubular myopathy have respiratory problems such as atelectasis and recurrent lung infections, concurrent neonatal intrahepatic cholestasis is rare. We report a newborn with a myotubular myopathy, ventilator dependency, recurrent lung infections and pleural effusion, facial diplegia, ophthalmoplegia, and progressive intrahepatic cholestasis. A genetic study showed a novel mutation of the MTM1gene: c.1142 G>A (R381Q). We suggest that physicians consider probable concurrent disorders of other organs in neonates with congenital myotubular myopathy.

Background/aims: Fluid overload is an important factor causing cardiovascular complications in dialysis patients. We compared fluid status, blood pressure (BP) and heart function between peritoneal dialysis (PD) patients and hemodialysis (HD) patients., Methods: We recruited 94 PD and 75 HD patients in our hospital. Fluid status was assessed by bioimpedance spectroscopy. Home BP was recorded. Use of antihypertensives was retrieved by chart review. In each group, 39 patients received echocardiographic examinations., Results: PD patients' fluid status was similar to that of predialysis HD patients. PD patients had lower systolic BP. E/E' and left ventricular mass index (LVMI) showed no significant intergroup difference. In multiple linear regression analyses, overhydration (OH)/extracellular water ratio >0.15 was associated with higher systolic BP, E/E' and LVMI., Conclusions: While PD was associated with higher OH but non-inferior BP control and heart function, OH was indeed related to poor BP control, diastolic dysfunction and left ventricular hypertrophy., (Copyright © 2012 S. Karger AG, Basel.)

The aims of this study were to determine if marine bacteria from Danish coastal waters produce antifouling compounds and if antifouling bacteria could be ascribed to specific niches or seasons. We further assess if antibacterial effect is a good proxy for antifouling activity. We isolated 110 bacteria with anti-Vibrio activity from different sample types and locations during a 1-year sampling from Danish coastal waters. The strains were identified as Pseudoalteromonas, Phaeobacter, and Vibrionaceae based on phenotypic tests and partial 16S rRNA gene sequence similarity. The numbers of bioactive bacteria were significantly higher in warmer than in colder months. While some species were isolated at all sampling locations, others were niche specific. We repeatedly isolated Phaeobacter gallaeciensis at surfaces from one site and Pseudoalteromonas tunicata at two others. Twenty-two strains, representing the major taxonomic groups, different seasons, and isolation strategies, were tested for antiadhesive effect against the marine biofilm-forming bacterium Pseudoalteromonas sp. strain S91 and zoospores of the green alga Ulva australis. The antiadhesive effects were assessed by quantifying the number of strain S91 or Ulva spores attaching to a preformed biofilm of each of the 22 strains. The strongest antifouling activity was found in Pseudoalteromonas strains. Biofilms of Pseudoalteromonas piscicida, Pseudoalteromonas tunicata, and Pseudoalteromonas ulvae prevented Pseudoalteromonas S91 from attaching to steel surfaces. P. piscicida killed S91 bacteria in the suspension cultures, whereas P. tunicata and P. ulvae did not; however, they did prevent adhesion by nonbactericidal mechanism(s). Seven Pseudoalteromonas species, including P. piscicida and P. tunicata, reduced the number of settling Ulva zoospores to less than 10% of the number settling on control surfaces. The antifouling alpP gene was detected only in P. tunicata strains (with purple and yellow pigmentation), so other compounds/mechanisms must be present in the other Pseudoalteromonas strains with antifouling activity.

Severe combined immunodeficiency (SCID) patients with an inactivating mutation in recombination activation gene 1 (RAG1) lack B and T cells due to the inability to rearrange immunoglobulin (Ig) and T-cell receptor (TCR) genes. Gene therapy is a valid treatment option for RAG-SCID patients, especially for patients lacking a suitable bone marrow donor, but developing such therapy has proven challenging. As a preclinical model for RAG-SCID, we used Rag1-/- mice and lentiviral self-inactivating (SIN) vectors harboring different internal elements to deliver native or codon-optimized human RAG1 sequences. Treatment resulted in the appearance of B and T cells in peripheral blood and developing B and T cells were detected in central lymphoid organs. Serum Ig levels and Ig and TCR Vβ gene segment usage was comparable to wild-type (WT) controls, indicating that RAG-mediated rearrangement took place. Remarkably, relatively low frequencies of B cells produced WT levels of serum immunoglobulins. Upon stimulation of the TCR, corrected spleen cells proliferated and produced cytokines. In vivo challenge resulted in production of antigen-specific antibodies. No leukemia development as consequence of insertional mutagenesis was observed. The functional reconstitution of the B- as well as the T-cell compartment provides proof-of-principle for therapeutic RAG1 gene transfer in Rag1-/- mice using lentiviral SIN vectors.

Background: The use of internet search data has been demonstrated to be effective at predicting influenza incidence. This approach may be more successful for dengue which has large variation in annual incidence and a more distinctive clinical presentation and mode of transmission., Methods: We gathered freely-available dengue incidence data from Singapore (weekly incidence, 2004-2011) and Bangkok (monthly incidence, 2004-2011). Internet search data for the same period were downloaded from Google Insights for Search. Search terms were chosen to reflect three categories of dengue-related search: nomenclature, signs/symptoms, and treatment. We compared three models to predict incidence: a step-down linear regression, generalized boosted regression, and negative binomial regression. Logistic regression and Support Vector Machine (SVM) models were used to predict a binary outcome defined by whether dengue incidence exceeded a chosen threshold. Incidence prediction models were assessed using r² and Pearson correlation between predicted and observed dengue incidence. Logistic and SVM model performance were assessed by the area under the receiver operating characteristic curve. Models were validated using multiple cross-validation techniques., Results: The linear model selected by AIC step-down was found to be superior to other models considered. In Bangkok, the model has an r² = 0.943, and a correlation of 0.869 between fitted and observed. In Singapore, the model has an r² = 0.948, and a correlation of 0.931. In both Singapore and Bangkok, SVM models outperformed logistic regression in predicting periods of high incidence. The AUC for the SVM models using the 75th percentile cutoff is 0.906 in Singapore and 0.960 in Bangkok., Conclusions: Internet search terms predict incidence and periods of large incidence of dengue with high accuracy and may prove useful in areas with underdeveloped surveillance systems. The methods presented here use freely available data and analysis tools and can be readily adapted to other settings.

Elevated levels of serum pancreatic enzymes are frequently observed in hemodialysis (HD) patients. The complex hemodynamic, biochemical, and physiological alterations in uremia were speculated to cause excessive release of pancreatic enzymes beyond decreased renal clearance. However, hemodynamic factors are seldom explored in this aspect. We performed the study to evaluate the association between intradialytic hemodynamic change and elevated serum pancreatic amylase (SPA). Eighty-three prevalent HD patients without any clinical evidence of acute pancreatitis underwent pre-HD and post-HD blood sampling for serum pancreatic enzyme levels. Demographic, biochemical, and hematological data were collected from patient record review. Hemodialysis information including intradialytic blood pressure changes and ultrafiltration (UF) amount were collected and averaged for 1 month before the blood sampling day. Patients with elevated SPA during the HD session had greater mean systolic blood pressure and mean arterial pressure reduction, greater UF volume, greater pre-HD blood urea nitrogen and serum creatinine, higher serum phosphorus, lower pre-HD serum total CO2, and lower left ventricle ejection fraction (LVEF). Using multivariate linear and logistic regression analysis, the independent predictors of elevated SPA were determined to be mean arterial pressure reduction during HD, mean UF amount, pre-HD serum total CO2, and LVEF. Greater blood pressure reduction during HD, greater UF volume, lower pre-HD serum total CO2, and lower LVEF were significantly associated with elevated SPA during HD. This suggests that hemodynamic factors contribute to elevated serum pancreatic enzymes in HD patients., (© 2011 The Authors. Hemodialysis International © 2011 International Society for Hemodialysis.)

Trisomy 18 is the second most common chromosomal syndrome and has multiple dysmorphic features. However, ocular findings in trisomy 18 are rarely reported. Retinal folds are the most common ocular finding described to date, although retinal hypopigmentation, dysplasia, and areas of hemorrhage and gliosis are also found in trisomy 18. Dandy-Walker syndrome is a brain malformation that has been reported in association with numerous chromosomal abnormalities, although it has rarely been reported in association with trisomy 18. Here, we present a case of trisomy 18 with ocular pathology and variant of Dandy-Walker syndrome, a combination that has not previously been reported., (Copyright © 2010 Taiwan Pediatric Association. Published by Elsevier B.V. All rights reserved.)

Background: There is growing evidence of positive correlations between asthma (AS) and obesity in adults and children. Leptin is an obesity gene product secreted by white adipose tissue; elevated serum levels are found in obese adults and children. Recently, leptin has also been found to be associated with allergic rhinitis (AR). However, the links between serum leptin, atopic AS, and AR remained undetermined. Because AS and AR share common allergic inflammatory mechanisms, our aim was to determine if there were any differences in serum leptin levels between asthmatic children and nonasthmatic children with AR., Methods: We studied 114 children (67 boys and 47 girls): 68 with mild intermittent-to-moderate persistent atopic AS (AS children) and 46 with mild-to-moderate persistent AR without AS (AR children; overall mean age, 8.51 years; range, 5-18 years). Body mass index (BMI), serum leptin, pulmonary function, and atopy parameters (serum IgE and eosinophil levels) were measured., Results: Compared with AR children, AS children had higher body weights (kg), body mass indices (kg/cm²), and serum leptin levels (ng/mL). Multiple linear regression analyses showed that serum leptin concentrations differed significantly for girls, being overweight and between disease groups (AS and AR children)., Conclusion: Our results indicate that a higher serum leptin level has stronger association with mild-to-moderate persistent AS compared with AR. Hence, serum leptin may be a stronger predictor for childhood AS compared with AR. Among the asthmatic children, higher serum leptin levels also showed stronger associations with female gender and being overweight.

X-linked agammaglobulinemia (XLA) is the most common primary immunodeficiency (PID) in man and caused by mutations in the Bruton's tyrosine kinase (BTK) gene. XLA is characterized by a B-cell differentiation arrest in bone marrow, absence of mature B cells and immunoglobulins (Igs), and recurrent bacterial infections. We used self-inactivating lentiviral vectors expressing codon-optimized human BTK under the control of three different ubiquitous or B cell-specific promoters. Btk-/- mice engrafted with transduced cells showed correction of both precursor B-cell and peripheral B-cell development. Lentiviral vectors containing the wildtype BTK sequence did not correct the phenotype. All treated mice with codon-optimized BTK exhibited the recovery of B1 cells in the peritoneal cavity, and of serum IgM and IgG3 levels. Calcium mobilization responses upon B-cell receptor stimulation as well as in vivo responses to T cell-independent antigens were restored. Viral promoters overexpressing BTK >100-fold above normal resulted in erythro-myeloid proliferations independent of insertional mutagenesis. However, transplantation into secondary Btk-/- recipients using cellular promoters resulted in functional restoration of peripheral B cells and IgM levels, without any adverse effects. In conclusion, transduction of human BTK corrects B-cell development and antigen-specific antibody responses in Btk-/- mice, thus indicating the feasibility of lentiviral gene therapy for XLA, provided that BTK expression does not vastly exceed normal levels.

Background and Objectives: Because thrombosis and stenosis are the most frequent causes of arteriovenous graft (AVG) failure, several externally supported grafts were designed to enhance incompressibility and kinking resistance to improve the patency. However, consensus on advantage of these modifications having still not been reached might relate to the previous limited patient numbers and small studies. This study analyzed the longevity of externally supported and nonsupported AVG on the basis of the nationwide database to understand whether the externally supported design could improve the duration of patency of AVG., Design, Setting, Participants, & Measurements: Adult incident patients (n = 4402) were retrospectively enrolled who had began hemodialysis between January 1, 2002 and December 31, 2005. All incident patients with AVG, before or after beginning regular hemodialysis, between January 1, 2000 and December 31, 2006 were identified. The Cox proportional hazard model was used to compare the longevity of externally supported and nonsupported AVG by controlling other covariates., Results: There were 990 patients with externally supported AVG and 3412 patients with nonsupported AVG. The patency rates of externally supported AVG were 49.4%, 31.6%, and 20.2% at 1, 2 and 3 years, respectively; those of nonsupported AVG were 31.9%, 17.4%, and 10.8%. The survival of nonsupported AVG was significantly inferior to that of externally supported AVG., Conclusions: Externally supported AVG are superior to nonsupported AVG for long-term patency. The incompressibility and kinking resistance of ringed grafts may shed light on a direction for the future development of dialysis graft design.

Background: Atherosclerotic renal artery stenosis (ARAS) is a progressive but potentially reversible chronic kidney disease. Although the high sensitivity and specificity of renal Doppler scanning (RDS) for ARAS has been reported in western countries, ARAS has not been detected by RDS. This study used magnetic resonance angiography (MRA) to evaluate the sensitivity and specificity of RDS for detecting ARAS among outpatients at a nephrology clinic, and to calculate the degree of underestimation of ARAS by RDS., Methods: A total of 257 outpatients, aged > 50 years were examined for ARAS by RDS and MRA., Results: Thirty-seven (14.4%) and 139 (54.1%) of 257 patients had stenosis detected by RDS and MRA, respectively. Among the 220 patients whose RDS results were negative, MRA detected stenosis in 111 (50.45%). Multivariate logistic regression analysis showed that age > 65 years, duration of smoking, coronary artery disease, and serum creatinine levels > 354 mmol/L (4 mg/dL) were significant and independent factors that influenced ARAS in patients with negative results by RDS., Conclusion: RDS might still be the diagnostic procedure of choice for screening outpatients for ARAS because it is inexpensive, convenient, able to detect severity, and avoids the use of contrast media. When RDS is negative in aged people who have smoked longer than 20 years, with coronary artery disease or serum creatinine > 4 mg/dL, MRA is recommended for further evaluation of ARAS., (Copyright (c) 2010 Elsevier. Published by Elsevier B.V. All rights reserved.)

Most newborns with congenital unilateral facial palsy are expected to recover; however, when a patient has hearing loss, underlying developmental ear structure abnormalities should be investigated, particularly when the patient has internal auditory canal stenosis, which is rarely reported in newborns. All cases of internal auditory canal stenosis are accompanied by concomitant unilateral or bilateral hearing loss, but none with progressive hearing loss has been reported. We present the case of a newborn with rapidly declining hearing in the left ear within 1 month after birth. The hearing decline was associated with unilateral facial palsy. Using high-resolution computed tomography (CT) of the temporal bone, we diagnosed the patient with congenital internal auditory canal stenosis. This is the first case detected with progressive hearing loss after birth, which implies that prompt diagnosis and early habilitation are warranted, even when the hearing loss is initially mild.

Cerebro-costo-mandibular syndrome (CCMS) is an uncommon multiple congenital anomaly syndrome characterized by severe micrognathia, posterior rib-gap defects, and developmental delay. The cause of CCMS is unknown. Genes hypothesized to have a causal role in CCMS, include myogenic factor 5 (MYF5), goosecoid homeobox (GSC) and runt-related transcription factor 2 (RUNX2) [formerly known as core-binding factor (CBFA1)]. We report an infant with typical features of CCMS who, on prenatal ultrasound, was found to have severe micrognathia. We present the first image by three-dimensional computed tomography of posterior rib-defect, and we exclude mutations of the MYF5, GSC, RUNX2, and TCOF1 genes in our patient. Further molecular studies are needed to evaluate the cause of CCMS.

Vacuum-assisted deliveries are fairly commonly used in obstetrical practice. Most newborns who have a vacuum-assisted delivery undergo extracranial birth traumas that have no residual consequences. Vacuum-assisted deliveries that complicate intracranial vascular infarction are rarely reported. We present 2 cases of intracranial vessel infarction after vacuum-assisted deliveries. One newborn, with scalp erosion, showed an unusual left middle cerebral artery infarct, and the other, with a severe subgaleal hematoma, had a venous thrombosis. Before the diagnosis, made using brain ultrasonography, neither had specific observable neurological symptoms. In conclusion, vacuum-assisted deliveries should be given special attention, especially when they are combined with a severe extracranial birth trauma.

Cerebro-costo-mandibular syndrome (CCMS) consists of severe micrognathia, glossoptosis, posterior rib-gap defects and developmental delay. It may cause upper airway obstruction andflail chest, resulting in neonatal hypoxia, and possibly death. Early airway management or surgical intervention to maintain a patent airway is critical to avoid hypoxia in CCMS patients. We report a newborn with CCMS who was successfully weaned from a laryngeal mask after undergoing a tongue-lip adhesion operation at 164 days of age.

Background: Compared to the general population, patients with end-stage renal disease (ESRD) have increased peptic ulcer and upper GI bleeding complication rates. However, the risk factors for peptic ulcer among ESRD patients are unknown., Methods: In this retrospective study, we enrolled 827 incident dialysis patients and diagnosed peptic ulcer on the basis of endoscopic findings; information on the morbidities and medical prescription were obtained directly from medical records. A Cox regression hazard model was used to identify risk factors for peptic ulcer., Results: During the 10-year study period, 481 patients underwent an endoscopic exam. Peptic ulcers were detected in 153 patients. Age (p = 0.025), peritoneal dialysis (p = 0.022), diabetes mellitus (p = 0.020), congestive heart failure (p = 0.015), low serum albumin (p = 0.008) and high gamma-glutamyl transpeptidase (γ-GT) levels (p = 0.002) are risk factors for peptic ulcers among ESRD patients. Ulcer severity (p = 0.004) and aspirin prescription (p = 0.043), but not Helicobacter pylori infection, influenced the ulcer recurrence rate., Conclusion: The risk factors for peptic ulcer have some differences between ESRD patients and general population. In patients with high risk of upper GI bleeding, peritoneal dialysis and aspirin should be prescribed with caution., (Copyright © 2010 S. Karger AG, Basel.)

Background: The pathological recognition of secondary membranous nephropathy (MN) is sometimes difficult, especially in those showing primary idiopathic MN-like histomorphology. The ultrastructural finding of tubuloreticular inclusions (TRIs) in MN always evokes suspicion of their association with underlying diseases such as viral infections and autoimmune diseases. However, it is not clear whether some other underlying diseases are associated with TRI expression in MN. Since treatment of the underlying diseases is the primary consideration for the management of secondary MN, it is important to make out the clinical significance of TRI expression in MN., Methods: Excluding the patients fully qualified for systemic lupus erythematosus (SLE) diagnostic criteria, we recruited 36 cases having a renal biopsy featured with histopathology of primary idiopathic MN but ultrastructural appearance of TRIs in glomerular endothelial cells (GECs). We investigated their clinical and pathological profiles and focused on the potential connections with the underlying diseases and treatment outcomes., Results: One-third of our cases showed no identifiable underlying aetiology. Other underlying disease groups included autoimmune disease (25%), hepatitis (14.7%), potential Helicobacter pylori infection (13%), diabetes (5.6%) and lymphoma (5.6%). Pathologically, patients in the autoimmune group tended to have more heterogeneous membranous deposits with frequent mesangial and subendothelial deposits. While all patients of the autoimmune group presented complement C1q in glomeruli, more than two-thirds of the patients in others groups were negative for C1q. Clinically, the patients in autoimmune and hepatitis groups were younger in age and had less remission of proteinuria following treatment, while the other groups of patients achieved partial or complete remission more frequently., Conclusion: The underlying diseases of our patients were consistent with the major disease categories that have been frequently linked to secondary MN. The HP group was more akin to undefined groups regarding their pathological and clinical profiles. Since the MN in the undefined group might be the only renal manifestation antedating other clinical presentations of the corresponding underlying disease, a long-term follow-up and meticulous search for aetiological factors are required to validate this assumption.

Background: Functional, long-lasting vascular access is essential for maintaining effective long-term haemodialysis. Previous studies have documented demographic factors and comorbid conditions associated with long-term vascular access. However, no studies have examined the effect of demography and timing of vascular access maturation on primary patency in Chinese incident haemodialysis patients., Methods: We retrospectively enrolled 7028 adult incident patients who began haemodialysis between 1 January and 31 December 2002. A total of 5890 patients with mature arteriovenous fistula or arteriovenous graft, before or after beginning regular haemodialysis, were identified between 1 January 2000 and 31 December 2003. The Cox regression hazard model was used to assess the impact of sex, age, diabetes, type of access and timing of vascular access maturation on the duration of primary vascular access patency., Results: Of the study population, 2920 patients (50%) had diabetes; 4929 patients (84%) received fistulas and 961 (16%) grafts. Grafts, female sex and advanced age were significantly associated with shorter primary vascular access patency duration (P < 0.05). Diabetes was a risk factor for shorter primary vascular access patency duration for incident patients with mature fistulas before or after initiation, but not for patients with mature graft. Arteriovenous graft placement and maturation were better when completed >6 months prior to haemodialysis initiation for the duration of primary access patency., Conclusion: Demographic characteristics and timing of vascular access maturation affect access type and duration of primary access patency among incident patients. Individual programmes for vascular access may be necessary to establish functional long-term access.

We report the case of a female infant with Pfeiffer-like syndrome and holoprosencephaly. She had a cloverleaf skull, ocular proptosis, broad thumbs and halluces, and variable accompanying anomalies compatible with Pfeiffer syndrome. She also displayed microcephaly, short palpebral fissures, and a smooth philtrum, which are clinical signs consistent with fetal alcohol syndrome. She suffered from multiple congenital anomalies and died at 41 days of age. Cardio-pulmonary failure, brain abnormalities, prematurity, and multiple complications contributed to her death. The patient displayed normal chromosomal numbers and type. DNA analysis did not reveal fibrobtast growth factor receptor (FGFR) genes FGFR1, FGFR2, FGFR3 or TWIST gene mutations. We review the previous reports of Pfeiffer syndrome and holoprosencephaly and describe our infant patient with Pfeiffer-like syndrome, holoprosencephaly, and heavy in utero maternal alcohol and smoking exposures.

Fermentation of raw fish is a common process in Asia for improvement of shelf life and safety, however, little is known about the survival of pathogenic bacteria in these products. Raw fish may be contaminated with Salmonella and Vibrio species. The purpose of this study was to determine survival and potential growth of Salmonella enterica serovar Weltevreden, S. enterica serovar Enteritidis, Vibrio cholerae and V. parahaemolyticus as influenced by the preservation parameters (sodium chloride, garlic and lactic acid) present in the Thai fermented fish product som-fak. The inhibitory effects of sodium chloride (0-4%), garlic (0-10%) and lactic acid (pH levels as in som-fak) were measured in modified brain heart infusion (BHI) broth at 30 degrees C. All bacteria were inhibited by 8-10% sodium chloride. Salmonella grew in all concentrations of garlic whereas Vibrio spp. were inhibited by 1.0-1.5%. Lactic acid was inhibitory at levels above 1.5%. The combinations of sodium chloride, lactic acid and garlic showed a distinct hurdle effect in the broth system. Neither S. Enteritidis, V. cholerae nor V. parahaemolyticus grew in garlic (0.5-1%), regardless of the level of sodium chloride (0.5-4% (w/v)), when lactic acid (0.5-2%) was present. S. Weltevreden was the least inhibited of the four bacteria and grew in the combination of 0.5% garlic and 0.5% lactic acid regardless of the NaCl level (0.5-4% (w/v)). Som-fak with 0 to 10% garlic or 2% glucose was inoculated with either (i) 10(3) CFU/g Salmonella Weltevreden, (ii) 10(6) CFU/g garlic fermenting Lactobacillus plantarum strain 509 or (iii) a combination of the two strains and stored at 30 degrees C. The Salmonella count increased to >10(8) CFU/g (>10(6) CFU/g for 10% garlic) in all types of som-fak inoculated with S. Weltevreden within the first day. Only a combination of at least 6% garlic and L. plantarum 509 was enough to prevent growth of the inoculated Salmonella whereas adding the Lactobacillus strain alone or in combination with glucose was insufficient to prevent growth. Our results show that Salmonella Weltevreden can grow in som-fak independently of the inhibitory substances normally present in this type of product, emphasising the importance of preventing contamination. However, our results also suggest that the use of garlic fermenting starter cultures in combination with garlic could improve safety of fermented fish products.

Aims: Whether glucocorticoids induce gastric mucosal injury remains uncertain. We investigated whether very high-dose steroids caused gastric mucosal injury in systemic lupus erythematous (SLE) patients and evaluated the possible risk factors for mucosal injury., Methods: In this prospective paired study, 67 SLE patients who had received pulse methylprednisolone therapy were enrolled. Each patient underwent endoscopic examination and tissue and blood sampling before and after pulse steroid therapy. Mucosal injury was diagnosed if the follow-up injury scale was higher than the initial scale. Examined parameters included Helicobacter pylori infection, cyclooxygenase (COX)-1 and COX-2 activity, and current nonsteroidal anti-inflammatory drug (NSAID) usage including aspirin., Results: Eleven (16.4%) of 67 cases who developed gastric mucosal injury after pulse therapy had significantly higher rates of peptic ulcer history, NSAID/aspirin use, lower gastric thromboxane B(2) and prostaglandin E(2) levels when compared with cases without gastric mucosal injury (P < 0.05). Infection by H. pylori was not a risk factor for gastric mucosal injury. Multivariate logistic regression analysis showed that NSAID/aspirin use was the only risk factor for gastric mucosal injury in these patients (odds ratio 26.99, 95% confidence interval 4.91, 148.57, P < 0.0001). Pulse steroid therapy alone did not induce gastric mucosal injury in fifty SLE patients without taking any NSAID/aspirin., Conclusions: Use of NSAIDs/aspirin, but not H. pylori infection, increases gastric mucosal injury in SLE patients receiving pulse methylprednisolone therapy. Very high-dose steroids de novo seem not to induce gastric mucosal injury in these patients. A larger case-controlled study enrolling a heterogeneous population is needed to clarify the role of glucocorticoids in gastric mucosal injury.

BCR-ABL fusion proteins show increased signaling through their ABL tyrosine kinase domain, which can be blocked by specific inhibitors, thereby providing effective treatment. This makes detection of BCR-ABL aberrations of utmost importance for diagnosis, classification and treatment of leukemia patients. BCR-ABL aberrations are currently detected by karyotyping, fluorescence in situ hybridization (FISH) or PCR techniques, which are time consuming and require specialized facilities. We developed a simple flow cytometric immunobead assay for detection of BCR-ABL fusion proteins in cell lysates, using a bead-bound anti-BCR catching antibody and a fluorochrome-conjugated anti-ABL detection antibody. We noticed protein stability problems in lysates caused by proteases from mature myeloid cells. This problem could largely be solved by adding protease inhibitors in several steps of the immunobead assay. Testing of 145 patient samples showed fully concordant results between the BCR-ABL immunobead assay and reverse transcriptase PCR of fusion gene transcripts. Dilution experiments with BCR-ABL positive cell lines revealed sensitivities of at least 1%. We conclude that the BCR-ABL immunobead assay detects all types of BCR-ABL proteins in leukemic cells with high specificity and sensitivity. The assay does not need specialized laboratory facilities other than a flow cytometer, provides results within approximately 4 h, and can be run in parallel to routine immunophenotyping.

Aims: Preconditioning of stainless steel with aqueous cod muscle extract significantly impedes subsequent bacterial adhesion most likely due to repelling effects of fish tropomyosin. The purpose of this study was to determine if other food conditioning films decrease or enhance bacterial adhesion to stainless steel., Methods and Results: Attachment of Pseudomonas fluorescens AH2 to stainless steel coated with water-soluble coatings of animal origin was significantly reduced as compared with noncoated stainless steel or stainless steel coated with laboratory substrate or extracts of plant origin. Coating with animal extracts also decreases adhesion of other food-relevant bacteria. The manipulation of adhesion was not attributable to growth inhibitory effects. Chemical analysis revealed that the stainless steels were covered by homogenous layers of adsorbed proteins. The presence of tropomyocin was indicated by appearance of proteins with similar molecular weight based in sodium dodecyl sulfate-polyacrylamide gel electrophoresis, in several extracts that reduced adhesion but also extracts not containing this protein reduced bacterial adhesion, indicating that several molecular species may be involved in the phenomenon., Conclusions: It is a common perception that food materials facilitate bacterial adhesion to surfaces; however, this study demonstrates that aqueous coatings of food origin may actually reduce bacterial adhesion., Significance and Impact of the Study: Compounds from food extracts may potentially be used as nontoxic coatings to reduce bacterial attachment to inert surfaces.

Patients with autosomal dominant polycystic kidney disease (ADPKD) usually have extrarenal manifestations. We report the case of a 53-year-old man with ADPKD who presented with a retroperitoneal abscess of the left side after undergoing bilateral nephrectomy for a cyst that had repeatedly bled for 1 year. The abscess recurred despite drainage with a pig-tail catheter and antibiotic treatment. Fistulography with injection of diluted contrast medium via the pig-tail catheter showed an accumulation of contrast medium in the descending colon, which indicated a fistula between the abscess and the descending colon. A portion of the descending colon was resected, and multiple diverticulitis with 1 perforation in the resected specimen was observed. The findings support a diagnosis of retroperitoneal abscess caused by a perforated diverticulum---an extrarenal manifestation of ADPKD.

Background/aims: Pentoxifylline (PTX) has been shown to inhibit renal inflammation in a rat model of crescentic glomerulonephritis. The present study investigated the role of PTX in renal fibrosis in rats with crescentic glomerulonephritis., Methods: A rat model of accelerated anti-glomerular basement membrane glomerulonephritis was induced and treated with PTX or vehicle control for 3, 7, 14 and 28 days. The therapeutic effect and mechanism of PTX on renal fibrosis were examined by Northern blot and immunohistochemistry., Results: Diseased rats treated with vehicle control developed a severe crescentic glomerulonephritis with progressive renal fibrosis identified by a marked accumulation of alpha-SMA+ myofibroblasts and collagen matrix. This was associated with tubular epithelial-myofibroblast transition as evident by de novo expression of alpha-SMA and a loss of E-cadherin on damaged tubular epithelial cells. Further studies revealed that severe renal fibrosis was associated with upregulation of renal TGF-beta1 and activation of TGF-beta/Smad signaling, which was blocked by treatment with PTX., Conclusions: PTX may be an anti-fibrosis agent capable of inhibiting renal fibrosis in a rat model of crescentic glomerulonephritis. Blockade of TGF-beta1 expression and Smad2/3 activation may be a mechanism by which PTX inhibits renal fibrosis., (Copyright 2008 S. Karger AG, Basel.)

Hematopoietic stem cells (HSC) are rare with estimated frequencies of 1 in 10,000 bone marrow cells and 1 in every 100,000 blood cells. The most important characteristic of HSC is their capacity to provide complete restoration of all blood cell lineages after bone marrow ablation. Therefore they are considered as the ideal targets for various clinical applications including stem cell transplantation and gene therapy. In adult mice and men, the main stem cell source is the bone marrow. For clinical applications HSC derived from umbilical cord blood (UCB) and G-CSF mobilized peripheral blood (PB) have been demonstrated to have several advantages compared to bone marrow; therefore, they are slowly replacing BM as alternative source of stem cells. The mouse is the model organism of choice for immunological and hematological research; therefore, studies of murine HSC are an important research topic. Here we described the most often used protocols and methods to isolate human and mouse HSC to high purity.

The overall incidence of amiodarone-induced thyroid dysfunction ranges from 2% to 24%. One third to half of patients with hypothyroidism have anemia due to some decrease in normal red blood cell mass and erythropoietin (EPO) resistance. Therefore, for patients with chronic renal disease under medication with amiodarone, early regular thyroid function test should be checked in order to avoid amiodarone-induced hypothyroidism and EPO-resistant anemia. If amiodarone-induced hypothyroidism and EPO-resistant anemia occur in patients with chronic renal failure, early thyroxine should be given instead of waiting for spontaneous recovery by amiodarone discontinuation only. Here, we report a patient with chronic renal failure who developed EPO-resistant anemia after amiodarone treatment for arrhythmia. The hemoglobin level responded to EPO therapy rapidly after thyroxine administration and amiodarone discontinuation.

In clinical gene therapy trials for X-linked severe combined immunodeficiency, the development of leukemia has come up as a severe adverse effect. In all five cases, T-cell acute lymphoblastic leukemia (T-ALL) occurred as a direct consequence of insertional mutagenesis by the retrovirus used to deliver the therapeutic gene. Here, we review the mechanisms of insertional mutagenesis, the function of the Il2RG gene and the future developments in the field. New lentiviral and gamma retroviral vectors can significantly improve the safety profile of the tools used but still carry the risk of insertional mutagenesis, as shown in this issue of Leukemia. Finally, the unfortunate side effects of gene therapy have given more insight into the development of human T-ALL.

A gastroenteritis outbreak occurred in a military camp where a laboratory and epidemiological investigation was carried out. The early onset of symptoms indicated probable food contamination with Clostridium perfringens. Stool samples collected from affected patients were tested within 4 h via real-time polymerase chain reaction (PCR) for the presence of the C. perfringens plc gene. Ten out of the 12 stool samples were positive. Confirmation of the molecular test results was carried out by enumeration of C. perfringens in stool by culture and shown to be in excess of 106 spores/g stool. The isolates obtained from culture were further analysed by PCR for the presence of the chromosomal enterotoxin (cpe) gene. Based on the clinical symptoms, epidemiological and laboratory investigations, C. perfringens was implicated as the aetiological agent. The ability to conduct real-time PCR analysis greatly shortens the time to diagnosis and allows for preventive and control measures to be effected quickly.

Percutaneous renal biopsy is frequently performed, and postbiopsy arteriovenous fistula (AVF) formation is not uncommon. In most cases, it is asymptomatic and remains undiagnosed, because Doppler examination is not performed routinely. We describe 2 cases of postbiopsy AVF of the kidney that were detected 61 and 17 months after renal biopsy, respectively. The subsequent superselective transcatheter arterial embolization was successful in both cases. An undetected AVF of the kidney can progress and lead to serious consequences. A follow-up Doppler examination of the kidney soon after renal biopsy and 6 months later is recommended for early detection of nonresolving AVFs to prevent further complications., ((c) 2008 Wiley Periodicals, Inc.)

Background: Few patients are able to resume peritoneal dialysis (PD) therapy after an episode of peritonitis that requires catheter removal. PD catheter loss is therefore regarded as an important index of patient morbidity. The aim of the present study was to evaluate factors influencing catheter loss in patients suffering from continuous ambulatory PD (CAPD) peritonitis., Patients and Methods: We retrospectively reviewed 579 episodes of CAPD peritonitis from 1999 to 2006 in a tertiary-care referral hospital. Demographic, biochemical, and microbiological characteristics were recorded. Episodes resulting in PD catheter removal (n = 68; 12%) were compared by both univariate and multivariate analyses with those in which PD catheters were preserved., Results: The incidence of PD catheter loss increased as the number of organisms cultured increased (p = 0.001). Also, PD catheter removal was more likely to occur after peritonitis episodes with low serum albumin level (p = 0.004), those with long duration of PD effluent leukocyte count remaining above 100/microL (p < 0.001), those with concomitant tunnel infection (p < 0.001), those with concomitant exit-site infection (p = 0.005), and those with presence of catastrophic intra-abdominal visceral events (p < 0.001). Duration on PD preceding the peritonitis episode was of borderline significance (p = 0.080). On the contrary, initial PD effluent leukocyte count and serum level of C-reactive protein were not predictive of PD catheter loss. Micro-organisms of the Enterobacteriaceae family were the major pathogens responsible for PD catheter loss following polymicrobial peritonitis. Furthermore, we found that there was no association between polymicrobial peritonitis and the catastrophic intra-abdominal visceral event, although both resulted in a greater incidence of PD catheter loss. Among the single-organism group in our population, the microbiological determinants of PD catheter loss included fungi (p < 0.001), anaerobes (p = 0.018), and Pseudomonas sp (borderline significance: p = 0.095)., Conclusion: PD catheter loss as a consequence of peritonitis is related primarily to hypoalbuminemia, longer duration of PD effluent leukocyte count remaining above 100/muL, the etiologic source of the infection, and the organism causing the infection. Peritonitis associated with concomitant tunnel or exit-site infections and abdominal catastrophes were more likely to proceed to PD catheter loss. The microbiological determinants of PD catheter loss in the present study included polymicrobial infections caused by Enterobacteriaceae as well as monomicrobial pseudomonal, anaerobic, and fungal infections.

Xanthogranulomatous pyelonephritis is an uncommon form of chronic bacterial pyelonephritis characterized by the destruction of renal parenchyma and the presence of granulomas, abscesses, and collections of lipid-laden macrophages (foam cells) replacing the renal parenchyma. This case report illustrates the clinical course of bilateral diffuse xanthogranulomatous pyelonephritis with a subtle manifestation in contrast to those typically presenting with fever, flank pain or urinary tract infection. The patient therefore received supportive treatment for 18 months without hemodialysis, instead of the curative treatment, bilateral nephrectomy, which would have caused immediate loss of residual renal function and dependence on hemodialysis.

Background: The patient with difficult airways is a common challenge for emergency physicians., Aims: Our goal was to study the reasons for difficult airways in the emergency department., Methods: We performed a prospective observational study of patients requiring advanced airway management from 1 January 2000 to 31 December 2006., Results: There were 2,343 patients who received advanced airway management of which 93 (4.0%) were deemed difficult. The main diagnoses were cardiac arrest (28), trauma (27) and congestive heart failure (10). The main reasons for the difficult airways were attributed to an anterior larynx (38, 40.9%), neck immobility (22, 23.7%) as well as the presence of secretions and blood (14, 15.1%). The mean number of attempts at intubation was 3.6 versus 1.2 for all cases. The mortality rate of 40.5% among patients with difficult airways was not different from that of all patients who had airway management (41%). There were seven (0.3%) failed airways. Anaesthetists performed 21 (22.6%) of the rescue airways while surgeons performed 5 (5.4%). Of the rescue strategies performed, 24 were through the use of the bougie, 3 by cricothyroidotomy, 4 by tracheostomy, 6 with the GlideScope and 3 with the laryngeal mask airway. The rest the airways were secured by tracheal intubation using the laryngoscope., Conclusions: Emergency physicians manage most of the difficult airways successfully (68.8%). However, the success rate can be further improved through the more frequent use of the bougie or other rescue device. A possible suggestion would be for the emergency physician to use the bougie after the second or third attempt at direct orotracheal intubation.

The pentalogy of Cantrell was first described in 1958 by Cantrell and coworkers, who reported five cases in which they described a pentad of findings including a midline supraumbilical thoracoabdominal wall defect, a defect of the Lower sternum, abnormalities of the diaphragmatic pericardium and the anterior diaphragm, and congenital cardiac anomalies. Trisomy 18 has an incidence of about 0.3 per 1000 newborns. We present a case of trisomy 18 with incomplete Cantrell syndrome. The patient presented with hypogenesis of the corpus callosum, vermian-cerebellar hypoplasia (Dandy-Walker variant), ventricular septal defect, dextrocardia, patent ductus arteriosus, a defect of the lower sternum, a midline supraumbilical abdominal wall defect with omphalocele, congenital left posterior diaphragmatic hernia (Bochdalek hernia), micrognathia, low-set and malformed ears, rocker-bottom feet, dorsiflexed hallux, hypoplastic nails, short neck, and wrist deformity. Trisomy 18 syndrome was unusually combined with the pentalogy of Cantrell. We present this case because of its rarity and high risk of mortality.