Laboratory Analysis of Abnormal Closure Times with Relation to Low Ristocetin Cofactor Versus Other Causes.

Blood ◽  
2004 ◽  
Vol 104 (11) ◽  
pp. 4010-4010
Author(s):  
Emma Arthur ◽  
Andrea Cortese Hassett ◽  
Franklin A. Bontempo
Keyword(s):  
Differential Diagnosis ◽  
Clinical Utility ◽  
Congenital Defects ◽  
Reference Laboratory ◽  
Testing Methods ◽  
Normal Range ◽  
Normal Ranges ◽  
Upper Limits ◽  
History Of ◽  
Ristocetin Cofactor

Abstract Closure times (CTs) have replaced the limitations of the previous testing methods. However, the clinical utility of detecting abnormal CTs remains poorly defined, with the differential diagnosis including von Willebrand’s disease (vWD), aspirin or NSAIDs, uremia, liver disease, or congenital defects. Ristocetin cofactor (RCoF) measures the functional activity of the vW molecule and when low, most often suggests vWD. CTs are frequently done in patients suspected by history of having vWD with some laboratories preferring to perform the collagen/epinephrine (Col/Epi) CT alone, while others additionally include the collagen/ADP (Col/ADP) CT. METHODS: We analyzed 1480 consecutive CTs from a large reference laboratory done on a PFA-100 (Dade-Behring) and correlated the CTs with RCoF levels done on a BCS analyzer (Dade-Behring). Normal ranges were: Col/Epi CT 84-175 sec.; Col/ADP CT 65-117 sec.; RCoF 0.5-1.5 U/ml. Calculated means for CT of ≥ 300 sec. used a level of 300. TABLE Of the 260 studies with abnormal closure times, 131 (50%) had normal RCoF values, 47 (18%) were high and 18 (32%) were low. All studies with low RCoFs had at least one abnormal CT with 78/82 (95%) having both CTs prolonged, 2/82 (≫2%) with a long Col/EPI only, and 2/82 (≫2%) with a long Col/ADP only. Mean CTs in patients with low RCoFs and both CTs abnormal were 252 seconds for Col/EPI and 174 seconds for Col/ADP, both well above the upper limits of the normal range. Mean CTs in patients with low RCoFs and one abnormal CT were 180 seconds for Col/EPI and 123 seconds for Col/ADP, both just slightly above the upper limits of the normal range. CONCLUSIONS: These findings suggest that when RCoF is low, both Col/EPI and Col/ADP CTs are prolonged, usually markedly, and the chances that a low RCoF will be missed is low, particularly if both CTs are used. RESULTS: Total CTs analyzed: 1480 No.≥ 1 Abnormal CT 260/1480 (18%) Number (%) Both CTs Abnormal Col/Epi ONLY Abnormal Col/ADP ONLY Abnormal Total Abnormal CTs 260 (100%) 203 (78%) 42 (16%) 15 (6%) Total Low RCoFs 82 (100%) 78 (95%) 2 (≈2%) 2 (≈2%)

Follow-up of Urinary Neopterin Concentrations in two Healthy Children until Adolescence

Pteridines ◽  
2003 ◽  
Vol 14 (3) ◽  
pp. 102-107
Author(s):  
Christiana Winkler ◽  
Barbara Frick ◽  
Katharina Schroecksnadel ◽  
Barbara Wirleitner ◽  
Dietmar Fuchs
Keyword(s):  
Early Morning ◽  
Healthy Children ◽  
Normal Range ◽  
Normal Ranges ◽  
Infectious Origin ◽  
Upper Limits ◽  
Urinary Neopterin ◽  
Urine Specimens ◽  
Sensitive Marker

Abstract Neopterin is a sensitive marker of the activated cellular (= Thl-type) immune response. Neopterin concentrations in healthy children are higher compared with those of adults and are declining with increasing age. In this study, we present a follow-up of urinary neopterin to creatinine concentrations in two healthy children until adolescence. Data were available from one female (7 - 23 years) and her brother (0 - 18 years), of whom early morning urine specimens were collected on an irregular basis throughout 18 years. In total, neopterin and creatinine concentrations were measured by high pressure liquid chromatography (HPLC) in 343 urinary specimens. In both children, concentrations of urinary neopterin were highest at study entry. With increasing age neopterin concentrations decreased continuously to reach rather constant levels around the age of fifteen years. On several occasions at which individuals presented with common sickness, mostly of infectious origin, neopterin concentrations were found highly increased. In the absence of such infectious episodes, neopterin/creatinine measurements of both individuals fit well to previously established normal ranges. However this was true only for specimens collcctcd when children were older than 10 years, before that age concentrations of neopterin were frequently found to be higher than the established upper limits of the normal range.

Acquired von Willebrand’s Syndrome and Acquired Thrombopathy in a Patient with a Chronic Lymphocytic Leukemia

1975 ◽  
Author(s):  
J. L. Wautier ◽  
S. Levy-Toledano ◽  
Y. Sultan ◽  
E. Bodevin
Keyword(s):  
Platelet Aggregation ◽  
Chronic Lymphocytic Leukemia ◽  
Lymphocytic Leukemia ◽  
Normal Range ◽  
Spontaneous Bleeding ◽  
History Of ◽  
Abnormal Bleeding ◽  
Normal Hemostasis ◽  
Von Willebrand ◽  
Ristocetin Cofactor

The patient was 65 years old when she developped spontaneous bleeding. She did not have a family history of abnormal bleeding and profered clear evidence of normal hemostasis before.Bleeding time, platelet aggregation induced by collagen were abnormal. VIII related antigen, ristocetin cofactor were 10 per cent the normal range while VIII procoagulant activity was 50%.Interaction with rabbit sub endothelium was studied: contact and adhesion were 50% of the normal range and thrombi were absent.Infusion of cryoprecipitate corrected partially von Willebrand abnormalities while platelet aggregation induced by collagen remained unchanged. Velocity of platelet aggregation induced by collagen, VIII related antigen and ristocetin cofactor increased moderately after corticotherapy.The association of an acquired thrombopathy and von Willebrand’s disease in a patient with chronic lymphocytic leukemia lead one to wonder about possible relationship between these diseases in this case.

Hereditary Heparin Cofactor II Deficiency and the Risk of Development of Thrombosis

1987 ◽  
Vol 57 (02) ◽  
pp. 196-200 ◽  
Author(s):  
R M Bertina ◽  
I K van der Linden ◽  
L Engesser ◽  
H P Muller ◽  
E J P Brommer
Keyword(s):  
Liver Disease ◽  
Venous Thrombosis ◽  
Healthy Volunteers ◽  
Mean Value ◽  
Age Group ◽  
Normal Range ◽  
Heparin Cofactor Ii ◽  
History Of ◽  
Group 2 ◽  
Hereditary Nature

SummaryHeparin cofactor II (HC II) levels were measured by electroimmunoassay in healthy volunteers, and patients with liver disease, DIC, proteinuria or a history of venous thrombosis. Analysis of the data in 107 healthy volunteers revealed that plasma HC II increases with age (at least between 20 and 50 years). HC II was found to be decreased in most patients with liver disease (mean value: 43%) and only in some patients with DIC. Elevated levels were found in patients with proteinuria (mean value 145%). In 277 patients with a history of unexplained venous thrombosis three patients were identified with a HC II below the lower limit of the normal range (60%). Family studies demonstrated hereditary HC II deficiency in two cases. Among the 9 heterozygotes for HC II deficiency only one patient had a well documented history of unexplained thrombosis. Therefore the question was raised whether heterozygotes for HC II deficiency can also be found among healthy volunteers. When defining a group of individuals suspected of HC II deficiency as those who have a 90% probability that their plasma HC II is below the 95% tolerance limits of the normal distribution in the relevant age group, 2 suspected HC II deficiencies were identified among the healthy volunteers. In one case the hereditary nature of the defect could be established.It is concluded that hereditary HC II deficiency is as prevalent among healthy volunteers as in patients with thrombotic disease. Further it is unlikely that heterozygosity for HC II deficiency in itself is a risk factor for the development of venous thrombosis.

The Prevalence of Hereditary Antithrombin-III Deficiency in Patients with a History of Venous Thromboembolism

1985 ◽  
Vol 54 (04) ◽  
pp. 744-745 ◽  
Author(s):  
R Vikydal ◽  
C Korninger ◽  
P A Kyrle ◽  
H Niessner ◽  
I Pabinger ◽  
...  
Keyword(s):  
Venous Thrombosis ◽  
Antithrombin Iii ◽  
Positive Family History ◽  
Normal Range ◽  
The Family ◽  
History Of ◽  
Antithrombin Iii Deficiency ◽  
Recurrent Venous Thrombosis ◽  
Antithrombin Iii Activity ◽  
Slight Deficiency

SummaryAntithrombin-III activity was determined in 752 patients with a history of venous thrombosis and/or pulmonary embolism. 54 patients (7.18%) had an antithrombin-III activity below the normal range. Among these were 13 patients (1.73%) with proven hereditary deficiency. 14 patients were judged to have probable hereditary antithrombin-III deficiency, because they had a positive family history, but antithrombin-III deficiency could not be verified in other members of the family. In the 27 remaining patients (most of them with only slight deficiency) hereditary antithrombin-III deficiency was unlikely. The prevalence of hereditary antithrombin-III deficiency was higher in patients with recurrent venous thrombosis.

scholarly journals Giant Fibrovascular Polyp as an Unusual Cause of Globus Pharyngeus

2021 ◽  
pp. 014556132110039
Author(s):  
Jelena Sotirović ◽  
Ljubomir Pavićević ◽  
Stanko Petrović ◽  
Saša Ristić ◽  
Aleksandar Perić
Keyword(s):  
Differential Diagnosis ◽  
Asymptomatic Patient ◽  
Histopathological Examination ◽  
Complete Resection ◽  
Airway Compromise ◽  
Endoscopic Removal ◽  
Globus Sensation ◽  
Fibrovascular Polyp ◽  
History Of ◽  
Fatal Complications

Differential diagnosis of globus sensation in an otherwise asymptomatic patient should include hypopharyngeal fibrovascular polyp to avoid potentially fatal complications like airway compromise following regurgitation. We present a case of a 74-year-old man with a 13-cm long hypopharyngeal fibrovascular polyp with 9 months history of globus sensation. A narrow stalk of the giant polyp allowed endoscopic removal and complete resection with the CO2 laser. Histopathological examination was conclusive for the fibrovascular polyp.

scholarly journals The Relationship between Scapular Upward Rotation and Shoulder Internal and External Rotation Isokinetic Strength in Professional Baseball Pitchers

Healthcare ◽  
2021 ◽  
Vol 9 (6) ◽  
pp. 759
Author(s):  
Byung Gon Kim ◽  
Seung Kil Lim ◽  
Sunga Kong
Keyword(s):  
External Rotation ◽  
Professional Baseball ◽  
Isokinetic Strength ◽  
Strength Ratio ◽  
Shoulder Injury ◽  
Isokinetic Dynamometer ◽  
Normal Range ◽  
History Of ◽  
Baseball Pitchers ◽  
The Relationship

This study aims to assess the relationship between scapular upward rotation (SUR) across varying humeral-elevation angles (HEAs) and shoulder isokinetic strength and ratio in professional baseball pitchers. The subjects were professional baseball pitchers (n = 16) without a history of shoulder injury in the last six months. The subject’s SUR angles were measured with the humerus elevated at HEAs of 0° (at rest), 60°, 90°, and 120° to the scapular plane. Shoulder isokinetic strength was evaluated for shoulder internal rotation (IR) and external rotation (ER) strength (PT%BW and TW%BW), and the ER/IR strength ratios were determined at 60, 120 and 180°/s using an isokinetic dynamometer. The SUR angle at an HEA of 0° was positively correlated with IR strength at 120°/s (r = 0.535) and 180°/s (r = 0.522). The SUR angle at an HEA of 60° was negatively correlated with the ER/IR strength ratios at 60°/s (r = −0.505) and 120°/s (r = −0.500). The SUR angle at an HEA of 90° was negatively correlated with the ER/IR strength ratios at 60°/s (r = −0.574; r = −0.554) and 120°/s (r = −0.521; r = −0.589) as well as with ER strength at 180°/s (r = −0.591, r = −0.556). The SUR angle at an HEA of 120° was negatively correlated with ER strength at 60°/s (r = −0.558), 120°/s (r = −0.504; r = −0.524), and 180°/s (r = −0.543) and the ER/IR strength ratio at 60°/s (r = −0.517). In this study, we found that the ratio of isokinetic strength between ER and IR became closer to the normal range on increasing the SUR angle. In particular, an HEA of 90°, which resembles the pitching motion, showed a clear relationship between SUR, shoulder ER, and the ratio of ER/IR isokinetic strength in professional baseball pitchers.

scholarly journals Undifferentiated pleomorphic sarcoma in oropharyngeal mucosa of patients with multiple basal cell carcinomas

Rare Tumors ◽  
2021 ◽  
Vol 13 ◽  
pp. 203636132110264
Author(s):  
Andrea Dekanić ◽  
Marko Velepič ◽  
Margita Belušić Gobić ◽  
Ita Hadžisejdić ◽  
Nives Jonjić
Keyword(s):  
Differential Diagnosis ◽  
Basal Cell ◽  
Polypoid Lesion ◽  
Older Patient ◽  
Mesenchymal Tumors ◽  
Undifferentiated Pleomorphic Sarcoma ◽  
Pleomorphic Sarcoma ◽  
Basal Cell Carcinomas ◽  
History Of ◽  
Multiple Basal Cell Carcinomas

Malignant mesenchymal tumors of oropharyngeal mucosa are rare. Those with fibroblastic and histiocytic differentiation in the skin are called atypical fibroxanthoma (AFX) and in the soft tissue undifferentiated pleomorphic sarcoma (UPS). Here we present a case of an older patient with a history of multiple basal cell carcinomas and recently with a rapidly growing polypoid lesion in the mucosa of posterior oropharyngeal wall with AFX/UPS morphology. The differential diagnosis, histological pitfalls of this poorly characterized mesenchymal lesions, and the challenges associated with treatment are discussed.

scholarly journals Neonate born with ischemic limb to a COVID-19 positive mother: management and review of literature

2020 ◽  
Vol 10 (1) ◽  
Author(s):  
Shahana Perveen ◽  
Karmaine A. Millington ◽  
Suchitra Acharya ◽  
Amit Grag ◽  
Vita Boyar
Keyword(s):  
Differential Diagnosis ◽  
Compartment Syndrome ◽  
Upper Extremity ◽  
Preterm Neonate ◽  
Review Of Literature ◽  
Case Presentation ◽  
Ischemic Limb ◽  
History Of ◽  
Hand Amputation ◽  
Work Up

AbstractObjectivesTo describe challenges in diagnosis and treatment of congenital neonatal gangrene lesions associated with history of maternal coronavirus disease 2019 (COVID-19) infection.Case presentationA preterm neonate was born with upper extremity necrotic lesions and a history of active maternal COVID-19 infection. The etiology of his injury was challenging to deduce, despite extensive hypercoagulability work-up and biopsy of the lesion. Management, including partial forearm salvage and hand amputation is described.ConclusionsNeonatal gangrene has various etiologies, including compartment syndrome and intrauterine thromboembolic phenomena. Maternal COVID-19 can cause intrauterine thrombotic events and need to be considered in a differential diagnosis.

scholarly journals Genetic Background of Congenital Erythrocytosis

Genes ◽  
2021 ◽  
Vol 12 (8) ◽  
pp. 1151
Author(s):  
Mary Frances McMullin
Keyword(s):  
Receptor Gene ◽  
Cell Mass ◽  
Oxygen Affinity ◽  
Young Age ◽  
Erythropoietin Receptor ◽  
Congenital Defects ◽  
History Of ◽  
Genetic Mechanisms ◽  
High Oxygen Affinity ◽  
Hemoglobin Variants

True erythrocytosis is present when the red cell mass is greater than 125% of predicted sex and body mass, which is reflected by elevated hemoglobin and hematocrit. Erythrocytosis can be primary or secondary and congenital or acquired. Congenital defects are often found in those diagnosed at a young age and with a family history of erythrocytosis. Primary congenital defects mainly include mutations in the Erythropoietin receptor gene but SH2B3 has also been implicated. Secondary congenital erythrocytosis can arise through a variety of genetic mechanisms, including mutations in the genes in the oxygen sensing pathway, with high oxygen affinity hemoglobin variants and mutations in other genes such as BPMG, where ultimately the production of erythropoietin is increased, resulting in erythrocytosis. Recently, mutations in PIEZ01 have been associated with erythrocytosis. In many cases, a genetic variant cannot be identified, leaving a group of patients with the label idiopathic erythrocytosis who should be the subject of future investigations. The clinical course in congenital erythrocytosis is hard to evaluate as these are rare cases. However, some of these patients may well present at a young age and with sometimes catastrophic thromboembolic events. There is little evidence to guide the management of congenital erythrocytosis but the use of venesection and low dose aspirin should be considered.

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