bluuebunny/crossref_metadata_2025_split huggingface dataset:
Density of CD163+CD11c+ Dendritic Cells Increases and CD103+ Dendritic Cells Decreases in the Coeliac Lesion
Authors: Beitnes A.‐C. R. , Ráki M. , Lundin K. E. A. , Jahnsen J. , Sollid L. M. , Jahnsen F. L. 
Published: March 2011

AbstractCoeliac disease is a chronic inflammation of the intestinal mucosa controlled by gluten‐specific T cells restricted by disease‐associated HLA‐DQ molecules. We have previously reported that mucosal CD11c+ dendritic cells (DCs) are responsible for activation of gluten‐reactive T cells within the coeliac lesion. In mice, intestinal CD11c+ DCs comprise several functionally distinct subsets. Here, we report that HLA‐DQ+ antigen‐presenting cells (APCs) in normal human duodenal mucosa can be divided into four subsets with striking similarities to those described in mice: CD163+CD11c− macrophages (74%), and CD11c+ cells expressing either CD163 (7%), CD103 (11%) or CD1c (13%). CD103+ and CD1c+ DCs belonged to partly overlapping populations, whereas CD163+CD11c+ APCs appeared to be a distinct population. In the coeliac lesion, we found increased density of CD163+CD11c+ APCs, whereas the density of CD103+ and CD1c+ DCs was decreased, suggesting that distinct subpopulations of APCs in coeliac disease may exert different functions in the pathogenesis.
Polyphase seismic faulting in the Ivrea zone (Italian Alps) revealed by 40Ar/39Ar dating of pseudotachylytes
Authors: Souquière François , Monié Patrick , Fabbri Olivier , Chauvet Alain 
Published: March 2011

Terra Nova, 23, 162–170, 2011AbstractMost fault‐related pseudotachylytes in the Val Sesia area, Ivrea zone, NW Italian Alps, occur in two areas: near the Canavese Line in the Kinzigite Formation paragneisses and in an elongate belt in the Mafic Complex gabbros away from the Canavese Line. 40Ar/39Ar dating indicates that pseudotachylytes from the Kinzigite Formation were formed in the late Eocene, likely during the early stages of the collision between the NW corner of the Adriatic indenter and the European margin. Pseudotachylytes from the Mafic Complex were formed under a transpressive strain regime, as suggested by kinematic indicators, and yield Early Cretaceous (Albian) 40Ar/39Ar ages. Their formation is possibly related to shortening during the inception of the tectonic inversion of the Adriatic passive margin.
Stroke: a realistic approach to a growing problem in sub‐Saharan Africa is urgently needed
Authors: Kolapo Kehinde O. , Vento Sandro 
Published: March 2011

SummaryStroke is an increasing problem in sub‐Saharan Africa, even in children. High rates of hypertension, diabetes, alcohol abuse, smoking, insufficient fruit and vegetable consumption, sickle cell disease, HIV infection, antiretroviral use and race are likely contributing factors. Although often considered as related to increasing wealth, stroke is more strongly related to poverty, and in turn increases it. Case‐fatality rates are high and premature death and years of life lost are a major problem. We propose an approach to stroke prevention and treatment that takes into account the real situation on the ground and can be applied in sub‐Saharan Africa, an area where stroke units are largely not feasible and many patients do not reach hospitals. Involvement of community and faith‐based organisations, use of simple diagnostic tests, emphasis on clinical examination to differentiate between haemorrhagic and ischaemic stroke, prompt initiation of aspirin therapy and training of community nurses on essential management of stroke should be urgently implemented.
Hypertrophic scar in a dog: histological and clinical features
Authors: Avallone Giancarlo , Bonaldi Martina , Caniatti Mario , Lombardo Rocco 
Published: March 2011

AbstractAn adult male bullmastiff dog was treated for paraparesis and ataxia due to discospondylitis and disc herniation. At this time, the dog had a nonhealing ulcer between the pads of the left hindfoot. At re‐evaluation, the dog had developed a large exophitic mass in the previously ulcerated area. Cytological examination revealed occasional spindle cells with mild atypia, and a soft tissue tumour was suspected. The mass was excised and submitted for histology. The lesion was characterized by superficial ulceration, an intermediate layer of granulation tissue and a deep portion containing vertically orientated capillaries and perpendicularly arranged fibroblasts and collagen. The histological features led to a diagnosis of hypertrophic scar. Eight weeks after surgery, the lesion recurred and was treated with an intralesional injection of methylprednisolone acetate. The lesion regressed in 10 days, but recurred after 3 months following severe self‐trauma. Hypertrophic scars and keloids are two types of exuberant scarring reported in human beings, the pathogenesis of which is still unclear but seems to involve several cytokines, growth factors and inflammatory cells. The histological features identified in this case paralleled those reported in hypertrophic scars in humans. In this case, intralesional corticosteroid therapy was useful in the management of the lesion, but the severe self‐trauma could have influenced the recurrence. Even if uncommon, hypertrophic scar should be included among the differential diagnoses of spindle cell tumours in dogs.
Novel mutation in GLRB in a large family with hereditary hyperekplexia
Authors: Al‐Owain M , Colak D , Al‐Bakheet A , Al‐Hashmi N , Shuaib T , Al‐Hemidan A , Aldhalaan H , Rahbeeni Z , Al‐Sayed M , Al‐Younes B , Ozand PT , Kaya N 
Published: March 2011

Al‐Owain M, Colak D, Al‐Bakheet A, Al‐Hashmi N, Shuaib T, l‐Hemidan A, Aldhalaan H, Rahbeeni Z, Al‐Sayed M, Al‐Younes B, Ozand PT, Kaya N. Novel mutation in GLRB in a large family with hereditary hyperekplexia.Hereditary hyperekplexia (HH) is a disorder of the inhibitory glycinergic neurotransmitter system. Mutations in five genes have been reported to cause the disease. However, only single mutation in GLRB, the gene encoding beta‐subunit of the glycine receptor, in a singleton patient with HH has been found to date. In this study, 13 patients with HH were identified through neurology and genetic clinics. Formal clinical examinations, linkage analysis, homozygosity mapping, in‐mutation screening of GLRB and in silico functional analyses were carried out. A novel mutation in GLRB among nine patients was identified. This c.596 T>G perturbation results in the change of the highly conserved methionine at position 177 to arginine. Besides the classical HH phenotype, seven patients had esotropia and few of them had behavioral problems. This study presents a large family with HH as a result of homozygous mutation in GLRB and expands the clinical spectrum of HH to include eye misalignment disorder. Moreover, the report of these familial cases supports the previous evidence in a single patient of an autosomal recessive inheritance of HH because of defects in GLRB.
Pre‐transplant course and risk of kidney transplant failure in IgA nephropathy patients
Authors: Bjørneklett Rune , Vikse Bjørn Egil , Smerud Hilde Kloster , Bostad Leif , Leivestad Torbjørn , Hartmann Anders , Iversen Bjarne M. 
Published: March 2011

Bjørneklett R, Vikse BE, Smerud HK, Bostad L, Leivestad T, Hartmann A, Iversen BM. Pre‐transplant course and risk of kidney transplant failure in IgA nephropathy patients. Clin Transplant 2011: 25: E356–E365. © 2011 John Wiley & Sons A/S.Abstract:  Background:  There is lack of knowledge to what degree clinical/morphological presentation and course of IgA nephropathy (IgAN) prior to end‐stage renal disease are risk factors for graft loss after kidney transplantation.Material and Methods:  Patients with IgAN between 1988 and 2006 (registered in the Norwegian Kidney Biopsy Registry) who later received a kidney transplant (registered in the Norwegian Renal Registry) were included. The cohort was followed up regarding death‐censored graft loss throughout 2008. Graft survival with a rapid progressive (RP) vs. a slow progressive (SP) course of pre‐Tx IgAN (annual GFR > or <30 mL/min/1.73 m2) was studied.Results:  Among 106 included patients, there were 14 graft losses giving a graft loss rate of 1.9/100 patient years. Follow‐up until the first kidney transplant was 6.9 ± 4.4 (range 0.1–19) yr. Patients with pre‐Tx RP had a higher graft loss rate compared with SP patients (6.3 vs.1.3/100 patient years, p < 0.001). Graft loss rate with living‐related donor (LRD) was similar to unrelated donor (UD) grafts. Most RP patients had received LRD grafts, and in SP patients, graft survival with LRD grafts was better than UD grafts (0.3 vs.2.1/100 patient years, p = 0.055).Conclusions:  A rapid pre‐transplant course is a strong risk factor for transplant failure in patients with IgAN.
Living donor liver transplantation for ornithine transcarbamylase deficiency
Authors: Wakiya T. , Sanada Y. , Mizuta K. , Umehara M. , Urahasi T. , Egami S. , Hishikawa S. , Fujiwara T. , Sakuma Y. , Hyodo M. , Murayama K. , Hakamada K. , Yasuda Y. , Kawarasaki H. 
Published: March 2011

Wakiya T, Sanada Y, Mizuta K, Umehara M, Urahasi T, Egami S, Hishikawa S, Fujiwara T, Sakuma Y, Hyodo M, Murayama K, Hakamada K, Yasuda Y, Kawarasaki H. Living donor liver transplantation for ornithine transcarbamylase deficiency. Pediatr Transplantation 2011: 15: 390–395. © 2011 John Wiley & Sons A/S.Abstract:  Ornithine transcarbamylase deficiency, the most common urea cycle disorder, causes hyperammonemic encephalopathy and has a poor prognosis. Recently, LT was introduced as a radical OTCD treatment, yielding favorable outcomes. We retrospectively analyzed LT results for OTCD at our facility. Twelve children with OTCD (six boys and six girls) accounted for 7.1% of the 170 children who underwent LDLT at our department between May 2001 and April 2010. Ages at LT ranged from nine months to 11 yr seven months. Post‐operative follow‐up period was 3–97 months. The post‐operative survival rate was 91.7%. One patient died. Two patients who had neurological impairment preoperatively showed no alleviation after LT. All patients other than those who died or failed to show recovery from impairment achieved satisfactory quality‐of‐life improvement after LT. The outcomes of LDLT as a radical OTCD treatment have been satisfactory. However, neurological impairment associated with hyperammonemia is unlikely to subside even after LT. It is desirable henceforth that more objective and concrete guidelines for OTCD management be established to facilitate LDLT with optimal timing while avoiding the risk of hyperammonemic episodes.
Birt–Hogg–Dubé syndrome with a renal angiomyolipoma: Further evidence of a relationship between Birt–Hogg–Dubé syndrome and tuberous sclerosis complex
Authors: Byrne Marguerite , Mallipeddi Raj , Pichert Gabriella , Whittaker Sean 
Published: March 2011

ABSTRACTBirt‐Hogg‐Dubé syndrome (BHDS) is an autosomal dominant condition caused by mutations in the gene which codes for folliculin (FLCN). It is characterised clinically by fibrofolliculomas, trichodiscomas, pulmonary cysts, spontaneous pneumothoraces and renal cancers. This case illustrates a patient with BHDS and a renal angiomyolipoma. Angiomyolipomas are not described as a feature of BHDS, but rather they can occur sporadically or in tuberous sclerosis complex (TSC). Recent studies suggest that clinical similarities between BHDS and TSC may be explained by FLCN and TSC proteins functioning on a common pathway, mammalian target of rapamycin. This case adds to the literature of cases with clinical similarities.
Primary care practice and health professional determinants of immunisation coverage
Authors: Grant Cameron C , Petousis‐Harris Helen , Turner Nikki , Goodyear‐Smith Felicity , Kerse Ngaire , Jones Rhys , York Deon , Desmond Natalie , Stewart Joanna 
Published: March 2011

Aim:  To identify primary care factors associated with immunisation coverage.Methods:  A survey during 2005–2006 of a random sample of New Zealand primary care practices, with over‐sampling of practices serving indigenous children. An immunisation audit was conducted for children registered at each practice. Practice characteristics and the knowledge and attitudes of doctors, nurses and caregivers were measured. Practice immunisation coverage was defined as the percentage of registered children from 6 weeks to 23 months old at each practice who were fully immunised for age. Associations of practice, doctor, nurse and caregiver factors with practice immunisation coverage were determined using multiple regression analyses.Results:  One hundred and twenty‐four (61%) of 205 eligible practices were recruited. A median (25th–75th centile) of 71% (57–77%) of registered children at each practice was fully immunised. In multivariate analyses, immunisation coverage was higher at practices with no staff shortages (median practice coverage 76% vs 67%, P= 0.004) and where doctors were confident in their immunisation knowledge (72% vs 67%, P= 0.005). Coverage was lower if the children's parents had received information antenatally, which discouraged immunisation (67% vs 73%, P= 0.008). Coverage decreased as socio‐economic deprivation of the registered population increased (P < 0.001) and as the children's age (P= 0.001) and registration age (P= 0.02) increased.Conclusions:  Higher immunisation coverage is achieved by practices that establish an early relationship with the family and that are adequately resourced with stable and confident staff. Immunisation promotion should begin antenatally.
Metabolic bone disease of prematurity and secondary hyperparathyroidism
Authors: Lothe Ashish , Sinn John , Stone Monique 
Published: March 2011

Aim:  To illustrate, via case histories, the importance of laboratory investigations for the early diagnosis and management of metabolic bone disease (MBD).Methods:  We report three cases of extreme premature infants with MBD.Results:  These three infants had several risk factors for MBD of prematurity: very low birthweight, delayed enteral feeds, cholestatic liver disease, intolerance of fortification, the use of glucocorticoids and diuretics. Serum alkaline phosphatase and parathyroid hormone (PTH) were elevated despite relatively normal calcium and phosphate levels. These parameters were corrected with additional supplementation of calcium, phosphate and vitamin D.Conclusions:  Infants born extremely prematurely have significant calcium and phosphate depletion by the time they reach full term compared with the normal fetal accretion rate. This is exacerbated if there is poor tolerability to feeds where extra calcium and phosphate could not be added either by additives or via human milk fortifier. Serum calcium and phosphate levels may be normal despite inadequate intake or stores due to the counter‐regulatory effect of PTH. In infants at risk of MBD, testing serum alkaline phosphatase, vitamin D and PTH with calcium and phosphate may assist in the monitoring and management of MBD.
Testicular microlithiasis: The importance of self‐examination
Authors: Price Neil R , Charlton Amanda , Simango Itayi , Smith Grahame HH 
Published: March 2011

Aim:  To explore the issue of appropriate management of testicular microlithiasis. We report the third ever case of tumour arising from a testis previously known to have microlithiasis in childhood and review the literature to provide an evidence‐based approach to management of testicular microlithiasis.Methods:  Case report and review of previous literature.Results and Conclusions:  Although there is a strong association between testicular microlithiasis and testicular malignancy at diagnosis, there are only three reported cases of subsequent tumour development in childhood. Testicular microlithiasis is an increasingly recognised entity. There is insufficient evidence in the current literature to support any regime of clinical surveillance. Self‐examination is the most important factor in the early detection of testicular malignancy.
Triple flap vaginoplasty for agenesis of the vagina
Authors: Prakash Vishwa 
Published: March 2011

AbstractAim:  Various procedures have been described for the creation of neovagina. McIndoe operation is gold standard for vaginoplasty in which split thick skin graft is used for lining the neovagina. However the rate of contraction of the graft is very high. We have used labia minora flaps along with the third flap created from suburethral tissue for lining the neovaginal space in 17 patients with good results.Methods:  The method was used in 17 patients. After creation of neovaginal space three flaps were raised – two labia minora flaps and the third flap from suburethral tissue. The flaps were inturned and lined the neovaginal space. If whole space was not lined with flaps, supplementary grafting was used to cover the unlined space.Results:  Thirteen patients came for follow up out of whom in one patient the labia minora flap necrosed and patient developed contracture of the vagina. In all other patients the result was satisfactory. There was no need of added lubrication during sex in any of the patients.Conclusion:  A three flap method of vaginoplasty in agenesis of the vagina has been described with good result.
Sleep deprivation, physical activity and low income are risk factors for inadequate weight gain during pregnancy: A cohort study
Authors: Abeysena Chrishantha , Jayawardana Pushpa 
Published: March 2011

AbstractAim:  To determine the possible risk factors for inadequate gestational weight gain.Methods:  A population‐based cohort study was carried out in Sri Lanka from May 2001 to April 2002. Pregnant women were recruited on or before 16 weeks' gestation and followed up until delivery; the sample size was 710. Trimester‐specific exposure status and potential confounding factors were gathered on average at the 12th, 28th and 36th weeks of gestation. Maternal weight was measured at the first antenatal clinic visit and at delivery. Inadequate weight gain was defined as weight gain below the Institute of Medicine recommendations in 2009. Multiple logistic regression was applied and the results were expressed as odds ratios (OR) and 95% confidence intervals (95% CI).Results:  The risk factors for inadequate weight gain were low per‐capita monthly income (OR 1.63, 95% CI 1.03, 2.58), multiparity (OR 1.96, 95% CI 1.34, 2.87), sleeping <8 h/day during the second, third, or both second and third trimesters (OR 1.60, 95% CI 1.05, 2.46), standing and walking ≥5 h/day during the second trimester (OR 1.50, 95% CI 1.04, 2.15), and the newborn being of the male sex (OR 1.50, 95% CI 1.04, 2.16), controlling for the effect of body mass index and gestational age.Conclusions:  Risk factors for inadequate gestational weight gain were low income, being multiparous, sleep deprivation, physical activity in terms of standing and walking, and the male sex of baby.
Pheochromocytoma‐related cardiomyopathy during the antepartum period in a preterm pregnant woman
Authors: Nakajima Yoshiyuki , Masaoka Naoki , Sodeyama Masako , Tsuduki Yoko , Sakai Masato 
Published: March 2011

AbstractWe report a rare case of pheochromocytoma‐related cardiomyopathy during the preterm period. The patient was a 33‐year‐old woman who was referred to our hospital at 34 weeks' gestation. Her pulse was 130 beats/min, blood pressure of 186/90 mmHg and oxygen saturation was 30–40%. Fetal bradycardia prompted an emergency cesarean section and, nine minutes after admission, a boy weighing 2774 g was delivered with Apgar scores of 2 at 1 min and 5 at 5 min. A preoperative echocardiogram showed reduced fractional shortening of 19%. At 8 h after the operation, cardiac arrest developed. An intra‐aortic balloon pump and percutaneous cardiopulmonary support were inserted, and continuous hemodiafiltration was also commenced, after which her cardiac function and respiratory function gradually improved. Urinary catecholamine levels were elevated and abdominal computerized tomography confirmed the presence of a right suprarenal mass. Four months after delivery, she underwent a laparoscopic adrenalectomy and the mass was found to be a pheochromocytoma.
Cell–cell and cell–surface interactions mediated by cellulose and a novel exopolysaccharide contribute to Pseudomonas putida biofilm formation and fitness under water‐limiting conditions
Authors: Nielsen Lindsey , Li Xiaohong , Halverson Larry J. 
Published: March 2011

SummaryThe composition of the exopolysaccharide matrix of Pseudomonas putida mt2 biofilms is relatively undefined as well as the contributions of each polymer to ecological fitness. Here, we describe the role of two putative exopolysaccharide gene clusters, putida exopolysaccharide A (pea) and bacterial cellulose (bcs) in biofilm formation and stability, rhizosphere colonization and matrix hydration under water‐limiting conditions. Our findings suggest that pea is involved in the production of a novel glucose, galactose, and mannose‐rich polymer that contributes to cell–cell interactions necessary for pellicle and biofilm formation and stability. In contrast, Bcs plays a minor role in biofilm formation and stability, although it does contribute to rhizosphere colonization based on a competition assay. We show that pea expression is highly induced transiently under water‐limiting conditions but only slightly by high osmolarity, as determined by qRT‐PCR. In contrast, both forms of water stress highly induced bcs expression. Cells deficient in making one or more exopolysaccharide experienced greater dehydration‐mediated cell‐envelope stress, leading to increased alginate promoter activity. However, this did not lead to increased exopolysaccharide production, except in bcs or pea mutants unable to produce alginate, indicating that P. putida compensates by producing, presumably more Pea or Bcs exopolysaccharides, to facilitate biofilm hydration. Collectively, the data suggest that Pea and Bcs contribute to biofilm formation and in turn their presence contributes to fitness under water‐limiting conditions, but not to the extent of alginate.
Essential metals for nitrogen fixation in a free‐living N2‐fixing bacterium: chelation, homeostasis and high use efficiency
Authors: Bellenger J.‐P. , Wichard T. , Xu Y. , Kraepiel A. M. L. 
Published: March 2011

SummaryBiological nitrogen fixation, the main source of new nitrogen to the Earth's ecosystems, is catalysed by the enzyme nitrogenase. There are three nitrogenase isoenzymes: the Mo‐nitrogenase, the V‐nitrogenase and the Fe‐only nitrogenase. All three types require iron, and two of them also require Mo or V. Metal bioavailability has been shown to limit nitrogen fixation in natural and managed ecosystems. Here, we report the results of a study on the metal (Mo, V, Fe) requirements of Azotobacter vinelandii, a common model soil diazotroph. In the growth medium of A. vinelandii, metals are bound to strong complexing agents (metallophores) excreted by the bacterium. The uptake rates of the metallophore complexes are regulated to meet the bacterial metal requirement for diazotrophy. Under metal‐replete conditions Mo, but not V or Fe, is stored intracellularly. Under conditions of metal limitation, intracellular metals are used with remarkable efficiency, with essentially all the cellular Mo and V allocated to the nitrogenase enzymes. While the Mo‐nitrogenase, which is the most efficient, is used preferentially, all three nitrogenases contribute to N2 fixation in the same culture under metal limitation. We conclude that A. vinelandii is well adapted to fix nitrogen in metal‐limited soil environments.