von Willebrand's disease characterized by increased ristocetin sensitivity and the presence of all von Willebrand factor multimers in plasma: a new subtype.

Abstract:

:The clinical and laboratory features of a patient with a recently recognized new variant of von Willebrand's disease are presented. The importance of this variant is that it is associated with a clinically significant bleeding diathesis but with a normal skin bleeding time, PTTK, factor VIIIc and platelet aggregation with 1 mg/ml ristocetin. The distinctive laboratory features are increased platelet sensitivity to low concentrations of ristocetin, and the presence of all plasma von Willebrand factor multimers, but in reduced amounts. The need for thorough investigation of patients with significant bleeding history despite apparently normal screening tests is emphasized.

journal_name

Pathology

journal_title

Pathology

authors

Wylie B,Gibson J,Uhr E,Kronenberg H

doi

10.3109/00313028809085199

subject

Has Abstract

pub_date

1988-01-01 00:00:00

pages

62-3

issue

1

eissn

0031-3025

issn

1465-3931

journal_volume

20

pub_type

杂志文章
  • Hep Par 1 in gastric and bowel carcinomas: an immunohistochemical study.

    abstract:AIMS:Hep Par 1 has been described as a specific marker of hepatocellular differentiation and its immunohistochemical use has been suggested as a helpful tool for hepatocellular carcinoma (HCC) diagnosis. Most metastatic liver tumours come from the gastrointestinal tract and usually can be distinguished from HCC only th...

    journal_title:Pathology

    pub_type: 杂志文章

    doi:10.1080/0031302021000009333

    authors: Villari D,Caruso R,Grosso M,Vitarelli E,Righi M,Barresi G

    更新日期:2002-10-01 00:00:00

  • Gamna body of the heart.

    abstract::A patient with cerebral strokes had a cardiac tumour excised. Histologically, it was an infarcted myxoma with features of a Gamna body. Findings are compared with other cardiac Gamna bodies reported in the literature. Most probably represent unusual degenerative changes in a myxoma. ...

    journal_title:Pathology

    pub_type: 杂志文章

    doi:10.3109/00313028409084740

    authors: Coard KC,Silver MD

    更新日期:1984-10-01 00:00:00

  • Development of paediatric biochemistry centile charts as a complement to laboratory reference intervals.

    abstract::Age-specific paediatric reference intervals are used in interpretation of laboratory results. However, interpretation may be problematic when a child just crosses an age bracket and the difference between the original and the subsequent age-specific reference interval is large. Moreover, details about the physiologica...

    journal_title:Pathology

    pub_type: 杂志文章

    doi:10.1097/PAT.0000000000000118

    authors: Loh TP,Antoniou G,Baghurst P,Metz MP

    更新日期:2014-06-01 00:00:00

  • Papillary cystic tumor of the pancreas coexisting with hairy cell leukemia.

    abstract::The coexistence of a pancreatic papillary cystic tumor with hairy cell leukemia is reported. To the best of our knowledge this association has never been published. A 41-year-old man diagnosed with hairy cell leukemia developed a second malignancy that corresponded to a papillary cystic pancreatic tumor. The patient u...

    journal_title:Pathology

    pub_type: 杂志文章

    doi:

    authors: Acebo E,Rodilla IG,Torío B,Hernando M,García de Polavieja M,Morales D,Seco I,Bermudez A

    更新日期:2000-08-01 00:00:00

  • Beta-carotene reduces the ductular (oval) cell reaction in the liver of Wistar rats submitted to the resistant hepatocyte model of carcinogenesis.

    abstract::The morphology of livers of Wistar rats treated with beta-carotene (BC), vitamin A (VA, retinol acetate) or corn oil (CO, controls) and submitted to the resistant hepatocyte model of carcinogenesis was studied. Preneoplastic lesions (PNL) were smaller and less numerous in the BC group. The latter group also presented ...

    journal_title:Pathology

    pub_type: 杂志文章

    doi:10.1080/00313029800169416

    authors: Dagli ML,Guerra JL,Sinhorini IL,Wu TS,Rizzi MB,Penteado MV,Moreno FS

    更新日期:1998-08-01 00:00:00

  • A combination of p40, GATA-3 and uroplakin II shows utility in the diagnosis and prognosis of muscle-invasive urothelial carcinoma.

    abstract::Recently developed antibodies against uroplakin II and ΔNp63 (p40) show utility in diagnosing primary bladder urothelial carcinoma, although application in metastatic bladder cancer and patient outcomes has been less well defined. We evaluated these antibodies by immunostain intensity and H-score, in conjunction with ...

    journal_title:Pathology

    pub_type: 杂志文章

    doi:10.1016/j.pathol.2016.05.008

    authors: Leivo MZ,Elson PJ,Tacha DE,Delahunt B,Hansel DE

    更新日期:2016-10-01 00:00:00

  • Utility of B-type natriuretic peptides and cardiac troponins for population screening regarding cardiac abnormalities.

    abstract:AIMS:The increasing importance of cardiac disease has generated an interest in improved screening strategies regarding preclinical cardiac abnormalities and employing measurement of circulating biomarkers. This review focuses on the utility of the B-type natriuretic peptides (NP) and the cardiac troponins (cTns) for th...

    journal_title:Pathology

    pub_type: 杂志文章,评审

    doi:10.1097/PAT.0b013e32834e786e

    authors: Eggers KM,Venge P

    更新日期:2012-02-01 00:00:00

  • Aeromonas septicemia: relationship of species and clinical features.

    abstract::Thirteen patients with Aeromonas species septicemia were seen at Westmead Hospital between 1983 and 1987. In 10 patients (77%) septicemia was caused by A. sobria, and in 3 (23%) by A. hydrophila, A. caviae was not isolated. Chronic underlying illness was present in 10 patients (77%), hematological malignancy being the...

    journal_title:Pathology

    pub_type: 杂志文章

    doi:10.3109/00313028909059546

    authors: Dryden M,Munro R

    更新日期:1989-04-01 00:00:00

  • Functional antithrombin-III variant (41 Pro----Leu) identified by liquid secondary ion mass spectrometry.

    abstract::A genetic variant of antithrombin with impaired heparin cofactor activity was identified in 4 members of a French family. Both the variant and normal antithrombin component were purified by affinity chromatography on heparin Sepharose. Reverse phase peptide mapping revealed a single altered peak when tryptic digests o...

    journal_title:Pathology

    pub_type: 杂志文章

    doi:10.3109/00313029109061429

    authors: Brennan SO,Borg JY,Shaw GJ,Caen J,George PM

    更新日期:1991-01-01 00:00:00

  • Large cell undifferentiated carcinoma of the urinary bladder.

    abstract::We present the clinicopathological features of eight cases of large cell undifferentiated bladder carcinoma not otherwise specified (LCUBC). LCUBC was characterised by sheets of large polygonal or round cells with moderate to abundant cytoplasm and distinct cell borders. The LCUBC component varied from 90 to 100% of t...

    journal_title:Pathology

    pub_type: 杂志文章

    doi:10.3109/00313021003767363

    authors: Lopez-Beltran A,Cheng L,Comperat E,Rouprêt M,Blanca A,Menendez CL,Montironi R

    更新日期:2010-06-01 00:00:00

  • Poems syndrome.

    abstract::A 40 year old Chinese woman presented with anasarca and later developed features of POEMS syndrome. These are peripheral neuropathy (P), organomegaly (O), endocrine dysfunction (E), monoclonal gammopathy (M), and skin changes (S) which are usually associated with plasma cell dyscrasia. In our patient, monoclonal gammo...

    journal_title:Pathology

    pub_type: 杂志文章

    doi:10.3109/00313029109060819

    authors: Leung NW,Lam CW,Pang JC

    更新日期:1991-04-01 00:00:00

  • Cancer stem cell markers in prostate cancer: an immunohistochemical study of ALDH1, SOX2 and EZH2.

    abstract::The aims of this study were to investigate the immunohistochemical expression and potential prognostic significance of putative cancer stems cell markers ALDH1, EZH2 and SOX2 in prostate cancer.A total of 142 consecutive radical prostatectomies submitted to one laboratory with a diagnosis of prostatic adenocarcinoma b...

    journal_title:Pathology

    pub_type: 杂志文章

    doi:10.1097/PAT.0000000000000325

    authors: Matsika A,Srinivasan B,Day C,Mader SA,Kiernan DM,Broomfield A,Fu J,Hooper JD,Kench JG,Samaratunga H

    更新日期:2015-12-01 00:00:00

  • Sensitivity of respiratory bacteria to lignocaine.

    abstract:AIM:Lignocaine, a topical anaesthetic agent, is generally used in variable concentrations usually between 2% and 4% on the vocal cords prior to flexible bronchoscopy and bronchoalveolar lavage (BAL) procedures. The aim of this study was to investigate whether 2% or 1% lignocaine significantly inhibits the growth of org...

    journal_title:Pathology

    pub_type: 杂志文章

    doi:10.1080/00313020500168752

    authors: Chandan SS,Faoagali J,Wainwright CE

    更新日期:2005-08-01 00:00:00

  • Inflammatory pseudotumor of epididymis and Epstein-Barr virus: a study of two cases.

    abstract::Inflammatory pseudotumor (IP) is an uncommon tumor-like lesion of unknown etiology. Two cases of IP of the epididymis were identified in our hospital file. Having read a recent report on finding Epstein-Barr virus (EBV) in some cases of IP in lymph node, spleen and liver, the two cases of epididymal IP were tested for...

    journal_title:Pathology

    pub_type: 杂志文章

    doi:10.1080/00313029700169664

    authors: Chan KW,Chan KL,Lam KY

    更新日期:1997-02-01 00:00:00

  • An immunohistochemical and molecular analysis of papillary proliferation of the endometrium.

    abstract::Papillary proliferations of the endometrium (PPEs) are uncommon lesions that are often associated with endometrial polyps. PPEs occasionally precede or co-exist with atypical endometrial hyperplasia or adenocarcinoma, but their pathogenesis and relationship to endometrial neoplasia is uncertain. In the present study 1...

    journal_title:Pathology

    pub_type: 杂志文章

    doi:10.1016/j.pathol.2017.10.013

    authors: Stewart CJR,Bigby S,Giardina T,Grieu-Iacopetta F,Amanuel B

    更新日期:2018-04-01 00:00:00

  • Clinical and histopathological predictors of progressive disease in IgA nephropathy.

    abstract::The clinical course and biopsy findings of twenty-one patients with IgA nephropathy, followed up for a mean period of 37.4 mth (range 24-54 mth) after diagnosis, were reviewed retrospectively to determine whether the clinical presentations, the laboratory findings or histopathologic changes have prognostic implication...

    journal_title:Pathology

    pub_type: 杂志文章

    doi:10.3109/00313028609090824

    authors: Ng WL,Loke SL,Yeung CK,Kwan S,Chan KW,Chan MK

    更新日期:1986-01-01 00:00:00

  • Reliability of the porphobilinogen screening assay.

    abstract::A survey conducted by the Australian Association of Clinical Biochemists Porphyrin Working Party on urinary porphobilinogen screening showed good sensitivity (75-97.5%). This is contrary to reports in the literature and to our own observations. We therefore assessed a widely used screening method (Watson-Schwartz) and...

    journal_title:Pathology

    pub_type: 杂志文章

    doi:10.3109/00313029009086660

    authors: Buttery JE,Carrera AM,Pannall PR

    更新日期:1990-10-01 00:00:00

  • Expression and prognostic significance of SIRT1 in ovarian epithelial tumours.

    abstract:AIMS:Sirtuin1 (SIRT1) is a nicotinamide adenine dinucleotide-dependent deacetylase. Recently, some studies have suggested that SIRT1 could be over-expressed in breast, prostate and colon cancers and up-regulated SIRT1 inactivates p53 by deacetylation. Therefore, we investigated the prevalence and the prognostic impact ...

    journal_title:Pathology

    pub_type: 杂志文章

    doi:10.1080/00313020902884451

    authors: Jang KY,Kim KS,Hwang SH,Kwon KS,Kim KR,Park HS,Park BH,Chung MJ,Kang MJ,Lee DG,Moon WS

    更新日期:2009-01-01 00:00:00

  • Primary cerebral neuroblastoma. Case report with light microscopy, tissue culture and electron microscopy study.

    abstract::A cerebral neuroblastoma was studied by light microscopy, tissue culture and electron microscopy. Synapse-like structures were found within tumour cells. This supports the neuroectodermal origin of this neoplasm. ...

    journal_title:Pathology

    pub_type: 杂志文章

    doi:10.3109/00313028009060082

    authors: Ojeda VJ,Jacobsen PF,Papadimitriou JM

    更新日期:1980-04-01 00:00:00

  • Cutaneous tumoural melanosis: a presentation of complete regression of cutaneous melanoma.

    abstract::Partial regression is common in cutaneous melanoma; however, complete regression manifesting as tumoural melanosis is rare, conceptually challenging and under-reported. In this study we report on clinical, histological and molecular findings in four cases of completely regressed cutaneous melanoma with nodal or brain ...

    journal_title:Pathology

    pub_type: 杂志文章,评审

    doi:10.1016/j.pathol.2019.01.008

    authors: Ching D,Amini E,Harvey NT,Wood BA,Mesbah Ardakani N

    更新日期:2019-06-01 00:00:00

  • Diagnostic performance of serum cobalamin tests: a systematic review and meta-analysis.

    abstract:AIMS:Serum cobalamin (cbl, vitamin B(12)) tests are routinely ordered for investigating conditions potentially amenable to cbl supplementation. This study aimed to systematically assess the evidence of diagnostic accuracy for serum cbl tests across patient subgroups. METHODS:Seven medical databases were searched (1990...

    journal_title:Pathology

    pub_type: 杂志文章,meta分析,评审

    doi:10.1097/PAT.0b013e3283486435

    authors: Willis CD,Elshaug AG,Milverton JL,Watt AM,Metz MP,Hiller JE,ASTUTE Health Study Group.

    更新日期:2011-08-01 00:00:00

  • Low-calcium/high-phosphorus rickets in rats. II. Osteoclast changes.

    abstract::Weanling rats were given a low Ca (0.003%)/high P (0.64%) diet with and without vitamin D for periods up to 6 weeks. An ultrastructural examination of selected areas of the proximal tibial metaphysis was carried out to study osteoclasts and their relation to calcified and uncalcified bone tissue. Osteoclasts were seen...

    journal_title:Pathology

    pub_type: 杂志文章

    doi:10.3109/00313027909059045

    authors: Feik SA,Storey E

    更新日期:1979-10-01 00:00:00

  • Insights into the molecular roles of Zika virus in human reproductive complications and congenital neuropathologies.

    abstract::The recent upsurge in the association of congenital neurological disorders and infection by the Zika virus (ZIKV) has resulted in increased research focus on the biology of this flavivirus. Studies in animal models indicate that ZIKV can breach the placental barrier and selectively infect and deplete neuroprogenitor c...

    journal_title:Pathology

    pub_type: 杂志文章,评审

    doi:10.1016/j.pathol.2017.07.008

    authors: Gharbaran R,Somenarain L

    更新日期:2017-12-01 00:00:00

  • Lipoleiomyosarcoma of the mediastinum.

    abstract::A case is described of a mediastinal soft tissue sarcoma, in a 76-year-old man, characterized microscopically by the intimate combined features of liposarcoma and leiomyosarcoma. The lipomatous component consisted of well differentiated liposarcoma with myxoid areas. The smooth muscle component was characterized by fa...

    journal_title:Pathology

    pub_type: 杂志文章

    doi:10.1080/00313029700169475

    authors: Gómez-Román JJ,Val-Bernal JF

    更新日期:1997-11-01 00:00:00

  • High Yes-associated protein 1 with concomitant negative LATS1/2 expression is associated with poor prognosis of advanced gastric cancer.

    abstract::The Hippo pathway is a tumour-suppressive pathway and its inactivation is known to be associated with progression and metastasis of various cancers. LATS1/2 (large tumour suppressor homolog 1 and 2), YAP1 (Yes-associated protein 1), and TEAD4 (TEA domain-containing sequence-specific transcription factors 4) are core c...

    journal_title:Pathology

    pub_type: 杂志文章

    doi:10.1016/j.pathol.2019.01.001

    authors: Kim E,Ahn B,Oh H,Lee YJ,Lee JH,Lee Y,Kim CH,Chae YS,Kim JY

    更新日期:2019-04-01 00:00:00

  • The value of endomyocardial biopsy in diagnosis and guiding therapy.

    abstract::Endomyocardial biopsy (EMB) is a highly-specialised procedure that is associated with some controversy as to its diagnostic role due to its inconsistency in diagnosing a wide variety of cardiac diseases. Given the advances and sophistication in echocardiography and cardiac magnetic resonance imaging (MRI), the vast ma...

    journal_title:Pathology

    pub_type: 杂志文章

    doi:10.1016/j.pathol.2017.08.004

    authors: Khan T,Selvakumar D,Trivedi S,Rao K,Harapoz M,Thiagalingam A,Denniss AR,Varikatt W

    更新日期:2017-12-01 00:00:00

  • Comparison of infliximab drug measurement across three commercially available ELISA kits.

    abstract::The monitoring of infliximab drug levels aids in the management of several autoimmune diseases, notably inflammatory bowel disease. Several commercial kits are now available and approved by the Therapeutic Goods Administration (TGA) for the measurement of infliximab levels, but there have been limited verification or ...

    journal_title:Pathology

    pub_type: 杂志文章

    doi:10.1016/j.pathol.2016.07.001

    authors: Lee MW,Connor S,Ng W,Toong CM

    更新日期:2016-10-01 00:00:00

  • Desmoplastic fibroma of bone.

    abstract::A 5-year-old Chinese boy had dull pain in his right knee for about one year. An intraosseous osteolytic lesion in the metaphysis of the right lower femur was curetted. The diagnosis of desmoplastic fibroma, possibly the first in a Hong Kong Chinese, was based on the clinical, radiological and histological findings. Ab...

    journal_title:Pathology

    pub_type: 杂志文章

    doi:10.3109/00313028709077135

    authors: Chan KW,Pun WK,Choi CH

    更新日期:1987-04-01 00:00:00

  • Effect on organism recovery rate from BacT/Alert blood cultures with reduced incubation period.

    abstract::This retrospective study evaluated 15,377 sets of BacT/Alert blood cultures to determine incubation time for blood cultures. Ninety-six per cent (1476) of total isolates signalled positive within five days and 56 isolates turned positive in five to seven days. Of the 56 organisms recovered between five and seven days,...

    journal_title:Pathology

    pub_type: 杂志文章

    doi:10.1080/00313029800169176

    authors: Mukerjee C,Heron LG,Varettas K

    更新日期:1998-05-01 00:00:00

  • Comparison of thromboplastins using the ISI and INR system.

    abstract::Twelve thromboplastins were tested against a secondary reference thromboplastin (human brain CRM BCR No. 147) or a tertiary house standard (human brain thromboplastin) with plasmas from normal healthy individuals and patients on oral anticoagulant therapy. The relationship between the prothrombin ratios of the thrombo...

    journal_title:Pathology

    pub_type: 杂志文章

    doi:10.3109/00313029009063783

    authors: Moriarty HT,Lam-Po-Tang PR,Anastas N

    更新日期:1990-04-01 00:00:00