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elicitors of host plant defenses partially suppress cacopsylla pyricola (hemiptera: psyllidae) populations under field conditions.defense elicitors are products that activate acquired defense responses in plants, thus rendering the plants less susceptible to attack by a broad range of pests. we demonstrated previously under laboratory conditions that foliar applications of the defense elicitors actigard (acibenzolar-s-methyl), employ (harpin protein), or odc (chitosan) to potted pear trees (pyrus communis l.) each caused an increase in mortality of cacopsylla pyricola (förster) (hemiptera: psyllidae) nymphs and altered the ...201728365771
sirolimus on gorham-stout disease. case report.gorham-stout disease (gsd) is a rare disease of unknown etiology characterized by vascular proliferation that produces destruction of bone matrix.201628348444
optimization of edible coating formulations for improving postharvest quality and shelf life of pear fruit using response surface methodology.the effect of composite edible films containing soy protein isolate (spi) in combination with additives like hydroxypropyl methylcellulose (hpmc) and olive oil on 'babughosha' pear (pyrus communis l.) stored at ambient temperature (28 ± 5 °c and 60 ± 10% rh) was evaluated using response surface methodology (rsm). a total of 30 edible coating formulations comprising of spi (2-6%, w/v), olive oil (0.7-1.1%, v/v), hpmc (0.1-0.5%, w/v) and potassium sorbate (0-0.4% w/v) were evaluated for optimizing ...201728242897
vanishing bone disease of chest wall and spine with kyphoscoliosis and neurological deficit: a case report and review of literature.vanishing bone disease is an extremely rare disorder of unknown etiology characterized by idiopathic osteolysis of bone. we describe a case of vanishing bone disease of chest wall and spine with kyphoscoliosis and neurological deficit. a 17-year-old male presented with gradually progressive deformity of back and dorsal compressive myelopathy with nonambulatory power in lower limbs. radiographs revealed absent 4th-7th ribs on the right side with dorsal kyphoscoliosis and severe canal narrowing at ...201728216760
changes of pectin nanostructure and cell wall stiffness induced in vitro by pectinase.structural modifications of fruit cell-wall pectins are controlled by various enzymes. in this in vitro study, the cell wall material (cwm) from pear fruit (pyrus communis l.) was treated using pectinases in two concentrations. water soluble (wsp), chelator soluble (csp) and sodium carbonate soluble (dasp) pectin fractions were extracted from cwm. by visualization of enzymatic-induced changes of structure and cwm stiffness using an atomic force microscopy (afm), the role of pectins in the mechan ...201728189229
cellulose nanocrystal reinforced chitosan coatings for improving the storability of postharvest pears under both ambient and cold storages.cellulose nanocrystal (cnc, 0%, 5%, and 10% w/w, in chitosan, dry basis) reinforced 2% chitosan aqueous coatings were evaluated for delaying the ripening and quality deterioration of postharvest green d'anjou (pyrus communis l.) and bartlett (pyrus communis l.) pears during 3 wk of ambient storage (20 ± 2 °c and 30 ± 2% rh) or 5 mo of cold storage (-1.1 °c and 90% rh), respectively. ethylene and co2 production, color, firmness, and internal fruit quality were monitored during both storage condit ...201728146285
fourteen years follow-up of massive pelvic girdle osteolysis caused by lymphatic malformation (gorham-stout disease). 201728062382
lymphatic endothelial cells produce m-csf, causing massive bone loss in mice.gorham-stout disease (gsd) is a rare bone disorder characterized by aggressive osteolysis associated with lymphatic vessel invasion within bone marrow cavities. the etiology of gsd is not known, and there is no effective therapy or animal model for the disease. here, we investigated if lymphatic endothelial cells (lecs) affect osteoclasts (ocs) to cause a gsd osteolytic phenotype in mice. we examined the effect of a mouse lec line on osteoclastogenesis in co-cultures. lecs significantly increase ...201728052488
gorham-stout disease: a clinical case report and immunological mechanisms in bone erosion.gorham-stout disease (gsd) is a rare condition of osteolysis with excessive lymphangiogenesis within bone tissue. the etiology of this condition remains unknown but seems to affect mainly children and young adults of both genders all over the world. unfortunately, there is no standardized method for diagnosis; however, histopathology remains as the gold standard. this condition is often misdiagnosed due to its varying clinical presentations from case-to-case. here, we report the case of an 8-yea ...201728004375
a review of the <i>tillicera</i> genus group with a revision of <i>plathanocera</i> schenkling (coleoptera, cleridae, clerinae).the tillicera genus group is revised and defined to contain 40 species in the following six genera: apopempsis schenkling, 1903; cardiostichus quedenfeldt, 1885; hemitrachys gorham, 1876; placocerus klug, 1837; plathanocera schenkling, 1902 and tillicera spinola, 1941. the definition of the tillicera genus group is mainly based on the presence of the dorsoventrally compressed and expanded antennae (from antennomere 3 onwards). the antennomeres are dilated at least from antennomeres 5 to 9 onward ...201627988691
characterization of tissue specific differences in cell wall polysaccharides of ripe and overripe pear fruit.cell walls from flesh, parenchyma cells, stone cells and skin were isolated from ripe and overripe pyrus communis l. cv "de cloche" using the phenol-buffer method. pear polysaccharides were solubilized from cell walls by sequential extractions with aqueous solutions of ammonium oxalate, na2co3, and increasing concentration of naoh, to explore overripening impact. cell walls were also differentiated using mir spectral data. stone cells contained high levels of xylose and lignin while parenchyma c ...201727842809
gorham disease: lymphangiomatosis with massive osteolysis.gorham disease (also referred to as vanishing bone disease or idiopathic massive osteolysis) is a rare skeletal condition that results in the localized replacement of bone with angiomas and fibrosis. the etiology and most effective treatment for this nonhereditary condition remain controversial in the medical literature. to our knowledge, we present the first case of post-traumatic gorham disease that includes mr imaging (before and after radiation therapy), post-radiation ct with evidence of tr ...201727829197
erratum: pregnancy complicated by gorham-stout disease and refractory chylothorax.[this corrects the article doi: 10.1055/s-0036-1593443.].201627822433
painful sporadic osteolysis of the parietal bone 'gorham's disease'.we report a 62-year old man, with painful osteolysis 'gorham disease' of skull. to ameliorate pain, large craniotomy and cranioplasty was performed. there was no sign of recurrence after one year. a brief review of the literature is presented.201627331246
cystic angiomatosis, a heterogeneous condition: four new cases and a literature review.cystic angiomatosis (ca) is a rare disorder causing bony cysts. it displays some similarity to gorham-stout disease (gsd), but has a much better local prognosis, despite the larger number of cysts. these 2 conditions also differ in terms of their location, visceral involvement, and response to treatment.201627787381
18f-naf and 18f-fdg pet/ct in gorham-stout disease.gorham-stout disease (gsd) is an extremely rare skeletal disorder of unknown etiology characterized by benign proliferation of vascular or lymphatic channels, leading to progressive bone resorption. we report on a patient diagnosed with gsd affecting the right scapula and the right ribs, who underwent pet/ct scans using f-fdg and f-naf. the remnant upper portion of the affected scapula did not show f-fdg uptake but demonstrated markedly increased f-naf activity. furthermore, intense f-naf activi ...201627648707
sirolimus in the treatment of vascular anomalies.aim of the study mtor inhibitors are showing promising results in the management of vascular anomalies. although current controlled trials remain to be completed, many individual experiences are being published. we present our series of children with complex vascular anomalies treated with sirolimus. patients and methods a retrospective review of 41 patients treated with sirolimus between january 2011 and december 2015 was performed: 15% (n = 6) had vascular tumors (4 kaposiform hemangioendothel ...201727723921
pregnancy complicated by gorham-stout disease and refractory chylothorax.introduction gorham-stout disease (gsd) is a rare disorder of bony destruction due to lymphangiomatosis, and is often triggered by hormones. one complication of gsd is the development of chylothorax, which carries a high mortality rate. very little experience has been published to guide management in gsd during pregnancy to optimize both fetal and maternal health. case study a 20-year-old woman with known gsd presented with shortness of breath at 18 weeks of pregnancy, due to bilateral chylothor ...201627708981
epilachnini (coleoptera: coccinellidae)-a revision of the world genera.based on the recent revised generic classification of the tribe epilachnini (szawaryn et al. 2015), all 27 genera are re-described, diagnosed, illustrated, and included in an identification key. the following nomenclatural changes are made: epilachna (hypsa) mulsant 1850, epilachna (cleta) mulsant 1850, solanophila weise 1898, epilachna (aparodentata) wang and cao 1993, and epilachna (uniparodentata) wang and cao 1993 are removed from synonymy of epilachna chervolat 1837. the subgenus cleta of e ...201627651424
nonmalignant adult thoracic lymphatic disorders.the thoracic lymphatic disorders are a heterogeneous group of uncommon conditions that are associated with thoracic masses, interstitial pulmonary infiltrates, and chylous complications. accurate diagnosis of the thoracic lymphatic disorders has important implications for the newest approaches to management, including embolization and treatment with antilymphangiogenic drugs. new imaging techniques to characterize lymphatic flow, such as dynamic contrast-enhanced magnetic resonance lymphangiogra ...201627514588
proverb comprehension in individuals with agenesis of the corpus callosum.comprehension of non-literal language involves multiple neural systems likely involving callosal connections. we describe proverb comprehension impairments in individuals with isolated agenesis of the corpus callosum (agcc) and normal-range general intelligence. experiment 1 compared gorham proverb test (gorham, 1956) performance in 19 adults with agcc and 33 neurotypical control participants of similar age, sex, and intelligence. experiment 2 used the proverbs subtest of the delis-kaplan execut ...201627448531
case report: gorham-stoute syndrome with involvement of majority of mandible, and partial maxillary, temporal and zygomatic bones.the gorham-stout syndrome is a rare condition in which spontaneous, progressive resorption of bone occurs. the diagnosis is based on clinical, radiological and histopathological findings after excluding other etiologies and as a result it is often delayed. we present the case of a 21-year-old male diagnosed with gorham-stout syndrome with involvement of the majority of the mandible, partial involvement of right maxillary, temporal and zygomatic bones and discuss its diagnostic and therapeutic fe ...201627408465
effect of sunlight-exposure on antioxidants and antioxidant enzyme activities in 'd'anjou' pear in relation to superficial scald development.influence of preharvest sunlight exposure on superficial scald development in 'd'anjou' pears during cold storage was investigated. the biochemical changes related to scald including α-farnesene, conjugated trienols (ctols), antioxidants, antioxidant enzyme activities were monitored among separated blushed and shaded peels of unbagged fruit as well as the whole peel of bagged fruit. in unbagged fruit, scald symptom was restricted to shaded peel; while there was no difference in α-farnesene betwe ...201627211615
increased intracranial pressure in a boy with gorham-stout disease.gorham-stout disease (gsd), also known as vanishing bone disease, is a rare disorder, which most commonly presents in children and young adults and is characterized by an excessive proliferation of lymphangiomatous tissue within the bones. this lymphangiomatous proliferation often affects the cranium and, due to the proximate location to the dura surrounding cerebrospinal fluid (csf) spaces, can result in csf leaks manifesting as intracranial hypotension with clinical symptoms to include orthost ...201727194986
pulmonary and pleural lymphatic endothelial cells from pediatric, but not adult, patients with gorham-stout disease and generalized lymphatic anomaly, show a high proliferation rate.gorham-stout disease (omim 123880) and generalized lymphatic anomaly are two rare disorders of lymphendothelial growth in which thoracic involvement with chylothorax is a feared complication. currently it is believed that both disorders are prenatal malformations that progress slowly after birth. several pharmaceuticals with antiproliferative properties, including interferon-α-2b, rapamycin and propranolol, have however been shown to affect the disease course in some patients. deeper knowledge o ...201627194137
gorham-stout syndrome of the shoulder.gorham-stout syndrome (gss) is a rare but severe subtype of idiopathic osteolysis. there are no guidelines for the treatment of gss. we analysed different diagnostic and therapeutic regimes and we describe the sucessful treatment of gss considering individual patient factors.201627194110
craniofacial ct findings of gorham-stout disease and generalized lymphatic anomaly.the present study aimed to assess the craniofacial ct imaging features for differentiating between gorham-stout disease (gsd) and generalized lymphatic anomaly (gla).201627126342
biochemical basis of co2-related internal browning disorders in pears (pyrus communis l. cv. rocha) during long-term storage.this study aimed at understanding the biochemical basis of internal browning disorders (ibds) in 'rocha' pear. for this purpose, the effects of storage under normal controlled atmosphere (ca) (3 kpa of o2 + 0.5 kpa of co2) and ibd-inducing ca (1 kpa of o2 + 10 kpa of co2) on the antioxidant and fermentative metabolisms and polyphenol oxidase (ppo) activity and phenolics concentration were studied. the higher ibd incidence in high co2-stored fruits was positively correlated with fermentative meta ...201627118401
massive craniofacial gorham disease treated successfully by cisplatin and 5-fluorouracil with ten years of follow-up: a case report and literature review.gorham disease (gd) is a rare osteolytic condition of unknown etiology that causes spontaneous, progressive bone resorption. the maxillofacial area is one of the regions most frequently involved in this disease. gd is characterized by its aggressiveness and rarity; therefore, the treatment modalities remain controversial, and no specific treatment method has been proved effective. the present report describes a case of gd with massive craniofacial bone involvement that was treated effectively us ...201627102925
anatomic and functional evaluation of central lymphatics with noninvasive magnetic resonance lymphangiography.accurate assessment of the lymphatic system has been limited due to the lack of optimal diagnostic methods. recently, we adopted noncontrast magnetic resonance (mr) lymphangiography to evaluate the central lymphatic channel. we aimed to investigate the feasibility and the clinical usefulness of noninvasive mr lymphangiography for determining lymphatic disease.ten patients (age range 42-72 years) with suspected chylothorax (n = 7) or lymphangioma (n = 3) who underwent mr lymphangiography were inc ...201627015184
more for less: improving the biomass yield of a pear cell suspension culture by design of experiments.plant cell suspension cultures are widely used for the production of recombinant proteins and secondary metabolites. one of the most important steps during process development is the optimization of yields by testing different cultivation parameters, including the components of the growth medium. however, we have shown that the biomass yield of a cell suspension culture derived from the pear cultivar pyrus communis cv. champagner bratbirne can be significantly improved solely by varying the temp ...201626988402
surgical management of gorham disease involving the upper cervical spine with occipito-cervical-thoracic fusion: a case report.gorham disease is a rare osteolytic condition, with only 17 reported cases primarily affecting the cervical spine.201626975457
chemical composition and antioxidant capacity of different anatomical parts of pear (pyrus communis l.).sugar, organic acid, triterpenoid and phenolic composition as well as antioxidant capacity of different anatomical parts of pear were studied. fruits and leaves of 'radana' pear (pyrus communis l.) were used. the results showed great quantitative differences in the composition of the pear pulp, peel, leaves and seeds. fructose was the major sugar in pulp, seeds and peel (227.46, 45.36 and 67.49 g/kg dry mass [dm], respectively), while sorbitol was predominant in leaves (40.66 g/kg dm). malic aci ...201626948642
gorham-stout disease of the pelvis: seven years follow up with complete radiological evaluation. 201626944827
novel approach of treating gorham-stout disease in the humerus--case report and review of literature.gorham-stout disease or the so-called vanishing bone syndrome is a rare disorder characterized by intra-osseous proliferation of vascular channels resulting in destruction and resorption of the osseous matrix. the exact pathology of this disease showed no evidence of malignant, neuropathic, or infectious components involved in the causation of this disorder except for the culprit of lympho-vascular malformations in the bone. the mechanism of bone resorption is yet to be clarified. the clinical p ...201626914115
gorham-stout disease successfully treated with sirolimus and zoledronic acid therapy.gorham-stout disease is a life-threatening disorder often manifested by lymphatic malformation and osteolysis. unfortunately, available therapies are not uniformly effective and often carry substantial morbidity. we report an 18-year-old male with gorham-stout disease manifested by lytic rib lesions and an intractable pleural effusion that responded dramatically to the combination of the mammalian target of rapamycin (mtor) inhibitor sirolimus and the aminobisphosphonate zoledronic acid after fa ...201626886375
rare incidence of gorham disease with limited involvement of the maxilla: case report. 201626818112
clinical features and prognosis of generalized lymphatic anomaly, kaposiform lymphangiomatosis, and gorham-stout disease.complex lymphatic anomalies are intractable lymphatic disorders, including generalized lymphatic anomaly (gla), gorham-stout disease (gsd), and kaposiform lymphangiomatosis (kla). the etiology of these diseases remains unknown and diagnosis is confused by their similar clinical findings. this study aimed to clarify the differences in clinical features and prognosis among gla, kla, and gsd, in japanese patients.201626806875
fast detection of phenolic compounds in extracts of easter pears (pyrus communis) from the atacama desert by ultrahigh-performance liquid chromatography and mass spectrometry (uhplc-q/orbitrap/ms/ms).a small chilean variety of pears growing in the town of toconao, an oasis located at the northeastern edge of the salar de atacama, northern chile, was studied by means of modern pda and high resolution mass spectral data (uhplc-pda-hesi-orbitrap-ms/ms). in addition, the antioxidant features of the fruits were compared with the varieties packhman's triumph and abate fetel and correlated with the presence of phenolic compounds. the non-pigmented phenolics were fingerprinted and related to the ant ...201626784158
quantitative proteomics-based reconstruction and identification of metabolic pathways and membrane transport proteins related to sugar accumulation in developing fruits of pear (pyrus communis).during their 6 month development, pear (pyrus communis) fruits undergo drastic changes in their morphology and their chemical composition. to gain a better understanding of the metabolic pathways and transport processes active during fruit development, we performed a time-course analysis using mass spectrometry (ms)-based protein identification and quantification of fruit flesh tissues. after pre-fractionation of the samples, 2,841 proteins were identified. a principal component analysis (pca) s ...201626755692
gorham-stout disease of the skull base with hearing loss: dramatic recovery and antiangiogenic therapy.gorham-stout disease (gsd) is a rare disorder of unknown etiology. we present a 6-year-old male with gsd involving the skull base who presented with recurrent cerebrospinal fluid (csf) rhinorrhea, severe hearing loss, and facial palsy secondary to cerebellar herniation into the internal auditory canal. after 2 months of treatment with pegylated interferon (ifn) α-2b (50 μg/week), his hearing recovered dramatically. two years later, new bone formation appeared radiologically and ifn was switched ...201626713883
expression of flowering locus t2 transgene from pyrus communis l. delays dormancy and leaf senescence in malus×domestica borkh, and causes early flowering in tobacco.annual and perennial plants represent two different evolutionary strategies based on differential synchronization of their reproductive development. the mobile signal protein flowering locus t (ft) plays a central role in mediating the onset of reproduction in both plant types. two novel ft-like genes from pear (pyrus communis)-pcft1 and pcft2-were isolated, and their expression profiles were determined for one annual cycle. the effects of pcft2 on flowering were investigated in annual (tobacco) ...201526706068
new synonymies for australian cleridae (coleoptera).the following synonymies are proposed based on examination of primary types (lectotypes are designated for all taxa except those marked with a '*'): lemidia spinnipennis lea, 1907 syn. n. and lemidia bicolor schenkling, 1906 syn. n. = lemidia biaculeata (westwood); lemidia mastersi lea, 1907 syn. n. = lemidia circumcincta schenkling, 1906; lemidia albonotata pic, 1941 syn. n. = lemidia laticeps lea, 1907; lemidia australiae lea, 1907 syn. n. = lemidia maculata schenkling, 1902; lemidia bilineatr ...201526701481
vanishing mandible: a rare case report with accent to recent concepts on aetio-pathogenesis.massive osteolysis is a rare idiopathic bone disease resulting in progressive destruction of bone. considering the rarity of encountering this fascinating bone disease, it is often misinterpreted as some other osteolytic disease. hence, we present a well-documented case of massive osteolysis in a 29-year-old female patient with complete clinical, radiographic, macroscopic and microscopic features along with surgical photographs, which has been effectively managed at our hospital.201526674137
gorham-stout disease of the proximal fibula treated with radiotherapy and zoledronic acid.gorham-stout disease is a rare disease characterized by anarchic lymphovascular proliferation causing resorption of bone sometimes leading to disastrous complications. bone tissue is progressively replaced by angiomatic and lymphangiomatic tissue and finally by fibrous tissue. this disease is known to be ubiquitous and of complex etiology. we present a case of gorham-stout disease of the proximal fibula invading the proximal tibia and soft tissues of the popliteal space that was successfully tre ...201526579487
surgical management of gorham-stout disease of the pelvis refractory to medical and radiation therapy.gorham-stout disease (gsd) is a rare condition characterized by spontaneous idiopathic bone resorption that can affect any part of the skeleton. treatment is aimed at halting osteolysis and alleviating complications associated with bone loss. often this can be achieved via observation and supportive management, medical treatment, and/or radiation therapy. we report a case of gsd of the pelvis that was refractory to medical and radiation therapy and was managed successfully with surgery. a 30-yea ...201526566566
erratum, vol. 12, october 15 release.[this corrects the article doi: 10.5888/pcd12.140583.]. in the article “effectiveness of fresh to you, a discount fresh fruit and vegetable market in low-income neighborhoods, on children’s fruit and vegetable consumption, rhode island, 2010–2011,” we inadvertently listed an author affiliation for sara gorham incorrectly. ms. gorham is affiliated with the institute for community health promotion, brown university school of public health, providence, rhode island. the changes were made to our web ...201526542140
[lymphangiomatosis and gorham-stout disease].lymphangiomatosis (recently renamed "generalized lymphatic anomaly") is a rare disease of unknown etiology that features an increase in the number of lymphatic vessels in many different tissues. gorham-stout disease(gsd) is a related disease characterized by lymphatic vessels involving the bones and resulting in progressive bone destruction. respective definitions remain unclear because these conditions largely overlap in the clinical setting and are both associated with pleural effusion and oth ...201526529945
the stiffening of the cell walls observed during physiological softening of pears.the young's modulus of the primary cell walls of pears decreases linearly during the pre-harvest on-tree maturation and increases during postharvest storage, and does not correlate with firmness of fruit. the determination of mechanical properties of cell walls is indispensable for understanding the mechanism of physiological softening and deterioration of quality of fruits during postharvest storage. the young's modulus of the primary cell walls from pear fruit (pyrus communis l., cultivars 'co ...201626498014
transcriptome profiling reveals differential gene expression in proanthocyanidin biosynthesis associated with red/green skin color mutant of pear (pyrus communis l.).anthocyanin concentration is the key determinant for red skin color in pear fruit. however, the molecular basis for development of red skin is complicated and has not been well-understood thus far. "starkrimson" (pyrus communis l.), an introduced red pear cultivated in the north of china and its green mutant provides a desirable red/green pair for identification of candidate genes involved in color variation. here, we sequenced and annotated the transcriptome for the red/green color mutant at th ...201526483812
testing spirotetramat as an alternative solution to abamectin for cacopsylla pyri (hemiptera: psyllidae) control: laboratory and field tests.aim of the study was to investigate the performance of the new insecticide "spirotetramat" as an alternative solution of "abamectin" for the control of cacopsylla pyri l. (hemiptera: psyllidae) in the context of an ipm program in european pear, pyrus communis l.. laboratory bioassays for the estimation of lc50 and lc90 of both insecticides were performed using four populations collected in emilia-romagna (italy) orchards where different pest management strategies were used (organic, integrated, ...201526470374
personalized therapy for generalized lymphatic anomaly/gorham-stout disease with a combination of sunitinib and taxol.the recently revised issva classification approved in melbourne in april 2014 recognizes generalized lymphatic anomaly and lymphatic malformation in gorham-stout disease. the 2 entities can overlap in presentation, as both are characterized by destructive lymphatic vessel invasion of the axial skeleton and surrounding soft tissues. at least at present, no standard therapeutic options exist, and due to the rarity of the disease, no clinical trials are available. we present 2 patients, 1 with gene ...201526458155
a transcriptome approach towards understanding the development of ripening capacity in 'bartlett' pears (pyrus communis l.).the capacity of european pear fruit (pyrus communis l.) to ripen after harvest develops during the final stages of growth on the tree. the objective of this study was to characterize changes in 'bartlett' pear fruit physico-chemical properties and transcription profiles during fruit maturation leading to attainment of ripening capacity.201526452470
efficacy of 1-methylcyclopropene on the mitigation of storage disorders of "rocha" pear under normal refrigerated and controlled atmospheres.alternatives are needed for long-term preservation of european pears (pyrus communis l.) after the ban on diphenylamine. "rocha" pear fruit harvested at commercial maturity were treated with 1-methylcyclopropene (1-methylcyclopropene, smartfresh™) and diphenylamine and stored at 0 ℃, 90-95% relative humidity, under normal atmosphere for up to six months or under controlled atmosphere (controlled atmosphere, 3 kpa o2 + 0.7 kpa co2) for up to 9.4 months. at 312 nl l(-1), 1-methylcyclopropene reduc ...201626437671
disappearing bone disease of the humerus and the cervico-thoracic spine: a case report with 42-year follow-up.disappearing bone disease (dbd) is a rare idiopathic musculoskeletal disorder that is distinguished by bone resorption without bone formation, vascular or lymphatic vessel proliferation, and soft-tissue swelling. long-term follow-up of a patient with dbd has rarely been reported in the literature.201626436955
natural polyamines and synthetic analogs modify the growth and the morphology of pyrus communis pollen tubes affecting ros levels and causing cell death.polyamines (pas) are small molecules necessary for pollen maturation and tube growth. their role is often controversial, since they may act as pro-survival factors as well as factors promoting programmed cell death (pcd). the aim of the present work was to evaluate the effect of exogenous pas on the apical growth of pear (pyrus communis) pollen tube and to understand if pas and reactive oxygen species (ros) are interconnected in the process of tip-growth. in the present study besides natural pas ...201526398794
genetic control of pear rootstock-induced dwarfing and precocity is linked to a chromosomal region syntenic to the apple dw1 loci.the vigour and precocity of trees highly influences their efficiency in commercial production. in apple, dwarfing rootstocks allow high-density plantings while their precocious flowering enables earlier fruit production. currently, there is a lack of pear (pyrus communis l.) rootstocks that are equivalent to the high yielding apple rootstock 'm9'. for the efficient breeding of new pyrus rootstocks it is crucial to understand the genetic determinants of vigour control and precocity. in this study ...201526394845
evaluating the influence of the microsatellite marker set on the genetic structure inferred in pyrus communis l.fingerprinting information can be used to elucidate in a robust manner the genetic structure of germplasm collections, allowing a more rational and fine assessment of genetic resources. bayesian model-based approaches are nowadays majorly preferred to infer genetic structure, but it is still largely unresolved how marker sets should be built in order to obtain a robust inference. the objective was to evaluate, in pyrus germplasm collections, the influence of the ssr marker set size on the geneti ...201526382618
99mtc(v)-dmsa spect-ct findings in a case of gorham-stout disease.the gorham-stout disease is a very rare condition, characterized by lymphovascular proliferation and massive bone resorption. we present a 48-year-old male patient with osteolysis involving the left femoral head and neck, as well as to the ipsilateral acetabulum. besides the morphological imaging, he underwent bone scintigraphy, technetium-99m-v-dimercaptosuccinic acid [99mtc(v)-dmsa] single photon emission computed tomography/computed tomography (spect/ct) and histological examination. together ...201526315871
polyphenolic profile of pear leaves with different resistance to pear psylla (cacopsylla pyri).the european pear psylla, cacopsylla pyri l. (hemiptera: psyllidae), is one of the most serious arthropod pests of pear. since proper control of this pest is essential, better understanding of the complex plant-pest relationship is mandatory. this research deals with constitutive polyphenolic profiles in leaves of 22 pear cultivars of diverse origin (p. communis, p. pyrifolia, and p. pyrifolia × p. communis) and different resistance to psylla. the study was designed to show which differences in ...201526278376
cycadophila, a new genus (coleoptera: erotylidae: pharaxonothinae) inhabiting cycas debaoensis (cycadaceae) in asia.asian species assigned to pharaxonotha reitter formed a separate clade from new world species of the genus and are distinguished on the basis of morphology and analysis of 16s rrna gene sequences. cycadophila, new genus, is described based on a consideration of four asian species in comparison with new world pharaxonotha. this new genus includes two new species (cycadophila debaonica new species and cycadophila fupingensis new species) and two previously described species (cycadophila nigra (gor ...201526250187
influence of maturity and storage period on physical and biochemical characteristics of pear during post cold storage at ambient conditions.the fruits of semi-soft pear (pyrus communis) cv. punjab beauty harvested at three different harvest dates were stored at 0-1(0) c temperature with 90-95 % relative humidity for 30, 45, 60 and 75 days to assess the physical and chemical changes during storage. after every storage interval, the fruits were removed and kept at room temperature for 3 and 6 days to study the shelf life of fruits. immature fruits always had the highest values of flesh firmness; optimum-mature fruits had the next and ...201526243965
the therapeutic response in gorham's syndrome to the beta-blocking agent propranolol is correlated to vegf-a, but not to vegf-c or flt1 expression.gorham's syndrome is a rare illness of unknown etiology. it is characterized by a local proliferation of blood or lymphatic vessels that in bones leads to progressive resorption and destruction. the cause of the disease is not elucidated, and therapeutic options remain limited.201526238450
gorham's disease: clinical case.gorham's disease, also known as idiopathic massive osteolysis, is a rare pathological condition characterized by vascular proliferation that results in destruction and reabsorption of the bone matrix, of unknown etiology. it was first described by jackson in 1838, but it was gorham and stout, in 1955, who defined this disease as a specific entity. it has variable clinical presentation and generally has progressive behavior. controversy continues regarding the treatment and there is no standard t ...201726229923
fatal progression of gorham disease: a case report and review of the literature.gorham disease is an idiopathic massive osteolysis, and maxillofacial involvement is rare. this report describes a case of a 12-year-old boy with severe progressive osteolysis in the mandible, hyoid bone, mastoid process, and cervical spine. radiation therapy and interferon-α therapy were followed by bisphosphonate therapy. the patient died of respiratory failure. to describe the clinicopathologic features of gorham disease of the jaws with an emphasis on the fatal types, 64 cases in the literat ...201526169484
metabolic profiling of developing pear fruits reveals dynamic variation in primary and secondary metabolites, including plant hormones.metabolites in the fruits of edible plants include sweet sugars, visually appealing pigments, various products with human nutritional value, and biologically active plant hormones. although quantities of these metabolites vary during fruit development and ripening because of cell division and enlargement, there are few reports describing the actual dynamics of these changes. therefore, we applied multiple metabolomic techniques to identify the changes in metabolite levels during the development ...201526168247
richard gorham. 201526114723
total joint replacement for severe bilateral condylar resorption--gorham-stout syndrome.gorham-stout syndrome is an extremely rare bone disorder characterized by osteolytic bony resorption. it is of unknown etiology and thought to result from localized endothelial proliferation of lymphatic vessels that cause the bony resorption. diagnosis of gorham-stout can only be made after excluding other common causes of bony resorption such as those caused by infection, inflammation, malignancy, and endocrine. the clinical symptoms vary from minor pain and swelling to non-healing fractures f ...201526080240
[atypical presentation of gorham-stout syndrome: case report].gorham-stout syndrome is an extremely rare pathology, of unknown etiology. it is characterized by proliferation of vascular channels that causes destruction and reabsorption of the bone matrix. we present a nine year-old male patient with an acute episode characterized by fever, chest pain, respiratory distress and dyspnea. the patient was submitted to computed tomography scan and a biopsy. the findings in the biopsy were multiple lytic lesions, osteolysis, and a mediastinal lymphangioma (lympha ...201525996335
notes on the genus mada mulsant with description of a new andean species (coleoptera: coccinellidae: epilachnini).mada andeana, a new species from the andes is here described. diagnostic characters are illustrated for both male and female. mada durantae gonzález et gómez is here synonymized with mada inepta (gorham), new synonymy.201525947436
gorham's disease with chest wall involvement: a case report and a review of the literature.gorham's disease is a rare disorder characterized by osteolysis and abnormal vascular growth within bones. diagnosis of gorham's disease is often delayed and for accurate and early diagnosis high clinical suspicion is crucial. no specific treatment is available. management options include surgery, radiation therapy and medical therapy. we aimed to present the first case of gorham's disease with chest wall involvement in iran. by review of the literature we discussed important issues of this rare ...201425763205
gorham disease of the mandible.gorham disease, or massive osteolysis, is a rare condition of unknown etiology. the disease is characterized by spontaneous progressive osteolysis of 1 or more skeletal bones. the mandible is the most commonly involved bone in the maxillofacial region. this article reports a case of gorham disease with mandibular involvement in a 46-year-old male patient with a 7-year follow-up. in this case, we performed lower right mandibular osteotomy and reconstruction with a phased titanium plate. postopera ...201525759934
degro guidelines for the radiotherapy of non-malignant disorders : part iii: hyperproliferative disorders.radiation therapy (rt) is an established and effective treatment modality in the management of a large variety of hyperproliferative disorders and benign neoplasms. objective of this article is to summarize the updated degro consensus s2e guideline recommendations.201525753848
a successfully treated case of gorham-stout syndrome with sternal involvement.gorham-stout syndrome (gss) is a rare disease characterized by localized bone resorption. any part of the skeleton may be affected; therefore, symptoms can vary depending on the site involved. pathological analysis reveals lymphovascular proliferation and osteolysis in the affected lesion, but the etiology of the disease is poorly understood. when gss occurs in the chest, chylothorax or respiratory failure may occur. thus far, a standard treatment for gss has not been established, and the progno ...201525705608
vertebroplasty in a 10-year-old boy with gorham-stout syndrome.to describe our experience using balloon vertebroplasty with polymethylmethacrylate filler in a 10-year-old boy with gorham-stout syndrome. gorham-stout syndrome is a rare disorder of unknown etiology characterized by intraosseous proliferation of fibrovascular or lymphatic tissue that results in progressive osteolysis and bone destruction. it can affect any part of the skeleton and lead to pathological fractures and muscular weakness or atrophy. the literature contains few reports on the surgic ...201525586760
gorham-stout disease presenting with dyspnea and bone pain in a 9-year-old girl.gorham-stout (gs) disease is a rare bone disorder of unknown etiology that is characterized by local proliferation of small vascular or lymphatic channels, resulting in progressive osteolysis and bone resorption. the diagnosis of gs disease is one of exclusion, with radiography and histopathology playing key roles. we describe a 9-year-old girl who presented to us with dyspnea and bone pain. she was found to have a cystic mass of the upper extremity, multiple cystic bone lesions, multiple fractu ...201527398124
minireview: nuclear receptor regulation of osteoclast and bone remodeling.osteoclasts are bone-resorbing cells essential for skeletal remodeling and regeneration. however, excessive osteoclasts often contribute to prevalent bone degenerative diseases such as osteoporosis, arthritis, and cancer bone metastasis. osteoclast dysregulation is also associated with rare disorders such as osteopetrosis, pycnodysostosis, paget's disease, and gorham-stout syndrome. the nuclear receptor (nr) family of transcription factors functions as metabolic sensors that control a variety of ...201525549044
molecular, phenotypic aspects and therapeutic horizons of rare genetic bone disorders.a rare disease afflicts less than 200,000 individuals, according to the national organization for rare diseases (nord) of the united states. over 6,000 rare disorders affect approximately 1 in 10 americans. rare genetic bone disorders remain the major causes of disability in us patients. these rare bone disorders also represent a therapeutic challenge for clinicians, due to lack of understanding of underlying mechanisms. this systematic review explored current literature on therapeutic direction ...201425530967
breast reduction in a patient with gorham-stout vanishing bone syndrome.the authors describe a 23-year-old woman with gorham's syndrome who underwent an uneventful bilateral reduction mammoplasty which has not been reported in the medical literature today. the patient had undergone multiple surgical and medical interventions before presentation in the senior author's clinic including a vascularized free fibular graft which ultimately disappeared due to disease progression. preoperatively, the patient complained of debilitating neck and back pain secondary to her mac ...201425426364
vanishing bone disease (gorham-stout syndrome): a review of a rare entity.vanishing bone disease (gorham-stout syndrome) is a rare entity of unknown etiology, characterized by destruction of osseous matrix and proliferation of vascular structures, resulting in destruction and absorption of bone. despite the extensive investigation of the pathogenetic mechanisms of the disease, its etiology hasn't been clarified and several theories exist. the syndrome can affect one or multiple bones of the patient, including the skull, the upper and lower extremities, the spine and p ...201425405099
thoracic osteotomy for gorham-stout disease of the spine: a case report and literature review.gorham-stout syndrome is an aggressive, non-heritable skeletal disease characterized by osteolysis following minor trauma. the primary involvement of the spine is less common (10 %) and has been described in only about 20 cases; there is no consensus about the best way to treat this condition.201425331037
changes in pectin methyl esterase activity with different packaging materials and stages of fruit harvesting during cold storage of pear cv. punjab beauty.pear cv. punjab beauty has become quite popular in punjab. excessive softening during cold storage leading to low shelf life is the major factor limiting its wider adoption. studies were, therefore, conducted to determine the firmness and pectin methyl esterase (pme) activity at 4 harvest dates (2nd, 3rd and 4th week of july, and 1st week of august). various packaging materials i.e. corrugated fiber board boxes and crates with high and low density polyethylene liners, corrugated fiber board boxe ...201425328240
cerebrospinal fluid leakage in gorham-stout disease due to dura mater involvement after progression of an osteolytic lesion in the thoracic spine.patients with gorham-stout disease (gsd), a rare disease of poorly understood etiopathophysiology, suffer from progressive osteolysis. destruction of bone matrix is caused by lymphatic vessels, which can lead to csf leakage if parts of bony structures adjacent to csf spaces are involved. so far, fewer than 200 patients have been reported in the literature; only 4 of these patients presented with csf leakage. the authors report the case of a 30-year-old man with gsd and csf leakage due to dura ma ...201425325172
complex lymphatic anomalies.complex lymphatic anomalies include several diagnoses with overlapping patterns of clinical symptoms, anatomic location, imaging features, hematologic alterations, and complications. lymphatic malformations likely arise through anomalous embryogenesis of the lymphatic system. analysis of clinical, imaging, histologic, and hematologic features is often needed to reach a diagnosis. aspiration of fluid collections can readily define fluid as chylous or not. the presence of chyle indicates dysfuncti ...201425241096
lymphatic malformations: diagnosis and management.lymphatic malformations are benign vascular lesions that arise from embryological disturbances in the development of the lymphatic system. they encompass a wide spectrum of related abnormalities, including cystic lymphatic lesions, angiokeratoma, lymphatic malformations that occur in bones (gorham-stout syndrome), lymphatic and chylous leak conditions, and lymphedema. this article will focus only on lymphatic malformation mass lesions, whereas other related disease entities will be covered elsew ...201425241095
gorham-stout's disease in the metatarsus: a case report.gorham-stout disease (gsd) is a rare disease occurring in the bone tissue and is characterized by spontaneous, progressive resorption. the etiology and treatment of the disease remains unclear. this article presents a 53-year-old male patient diagnosed with gsd in the 3rd and 4th metatarsal of his right foot.201425230274
gorham-stout disease: radiological, histological, and clinical features of 12 cases and review of literature.gorham-stout disease (gsd) is an exceedingly rare disease characterized by progressive osteolysis and angiomatosis. we investigate the features of this disease and evaluate the effects of bisphosphonates (bps) on it. the clinical, radiological, and pathological characteristics of 12 patients diagnosed with gsd were summarized. immunohistochemical staining with specific lymphatic endothelial markers (d2-40), vascular markers (cd 31, cd 34), and vascular endothelial growth factor (vegf) and vascul ...201625227772
lymphangiomatosis with dental involvement.lymphangiomatosis characterized by lymphangiomatous proliferation is a rare disease of unknown etiology, which seems to be more aggressive than gorham-stout syndrome, also known as "the vanished bone disease."201525192473
laboratory models for the investigation of lymphangiomatosis.lymphangiomatosis is an uncommon proliferative disorder of the lymphatic vasculature whose etiology remains poorly understood. the lymphangiomatosis spectrum encompasses a remarkable heterogeneity in its potential presentation, including micro- and macrocystic isolated lymphatic malformations, thoracic and intraabdominal diffuse lymphangiomatosis, and osseous and soft-tissue presentations known as gorham-stout disease. recent therapeutic advances are empirical in nature or, at best, inferential, ...201425173588
an unusual cause of recurrent chylothorax: gorham syndrome. 201425057476
redescription of platynaspisflavoguttata (gorham) (coleoptera, coccinellidae) and notes on nomenclature of platynaspiskapuri chakraborty & biswas.platynaspisflavoguttata (gorham) (coleoptera: coccinellidae) is redescribed and the male genitalia are illustrated for the first time. it is also recorded from sri lanka for the first time. platynaspisbimaculata (hoang, 1983) is a new junior synonym of platynaspisbimaculata pang & mao, 1979 (new synonym). platynaspiskapuri chakraborty & biswas, 2000, the replacement name for platynaspisbimaculata pang & mao, 1979 established by ukrainsky (2007), is also the new replacement name for platynaspisbi ...201425057247
growth medium alterations improve in vitro cold storage of pear germplasm.development of new fruit cultivars is dependent on genetic resource collections such as those at the pomological garden of the institute of horticulture and viticulture near almaty, kazakhstan. the pear germplasm collection of the pomological garden contains 615 cultivars and three species. in vitro cold storage of the collection would provide additional security to the field collection.201424997837
gorham-stout disease mimicking spondyloarthritis. 201424986965
ultrastructure of plant leaf cuticles in relation to sample preparation as observed by transmission electron microscopy.the leaf cuticular ultrastructure of some plant species has been examined by transmission electron microscopy (tem) in only few studies. attending to the different cuticle layers and inner structure, plant cuticles have been grouped into six general morphological types. with the aim of critically examining the effect of cuticle isolation and preparation for tem analysis on cuticular ultrastructure, adaxial leaf cuticles of blue-gum eucalypt, grey poplar, and european pear were assessed, followin ...201424895682
[is the wait-and-see approach in gorham syndrome a therapeutic option?]. 201424890889
nomenclatural notes on some checkered beetle (coleoptera: cleridae) types of the natural history museum, london (bmnh).lectotypes were designated (and holotypes and paralectotypes recognized) for 44 species of hydnocerinae, including the type species for isolemidia, parmius, paupris, allelidea, blaesiopthalmus and lemidia, four species of enoclerus (clerinae), and 14 species of cymatodera (tillinae). annotations include comments on additional type material, new type locality, previous (type series) locality, and questionable or mysterious types. phyllobaenus pallipes (gorham) and p. rufithorax (gorham) are synon ...201424870084
gorham-stout syndrome: a case report.gorham-stout syndrome, is an extremely rare disease of the bone, characterized for vascular and lymphatic channels proliferation in bony segments and consequent osseous resorption. there are around 200 cases reported around the world. although bisphosphonates are used for symptoms relief, there is no standardized treatment established. we present a case that was diagnosed in our centre secondary to a resistant epistaxis and a literature review of this condition.201424825048
gastrointestinal tract involvement of gorham's disease with expression of d2-40 in duodenum.we present a case of a 13-year-old boy with gorham's disease involving the thoracic and lumbar spine, femur, and gastrointestinal (gi) tract, which was complicated by recurrent chylothorax and gi bleeding. the presenting symptoms were intermittent abdominal pain, back pain, and melena. esophagogastroduodenoscopy and colonoscopy showed no abnormal lesions, but duodenal biopsy showed marked dilation of the lymphatics in the mucosa and submucosa, which revealed positive staining with a d2-40 immuno ...201424749089
rare bone diseases and their dental, oral, and craniofacial manifestations.hereditary diseases affecting the skeleton are heterogeneous in etiology and severity. though many of these conditions are individually rare, the total number of people affected is great. these disorders often include dental-oral-craniofacial (doc) manifestations, but the combination of the rarity and lack of in-depth reporting often limit our understanding and ability to diagnose and treat affected individuals. in this review, we focus on dental, oral, and craniofacial manifestations of rare bo ...201424700690
viewpoints on vessels and vanishing bones in gorham-stout disease.gorham-stout disease (gsd) is a rare disorder characterized by the proliferation of endothelial-lined vessels in bone and the progressive destruction of bone. although jackson described the first case of gsd in 1838, the clinical and histological features of gsd were not defined until gorham and stout published their report on massive osteolysis in 1955. in the years since gorham and stout's groundbreaking publication, more than 300 cases of gsd have been described in the literature. these repor ...201424583233
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