The Clinical Impact of Mild Hemophilia A, Report of a Cohort Study.

Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 3210-3210 ◽  
Author(s):  
Mary-Frances Scully ◽  
David MacGregor ◽  
Meghan Walsh ◽  
Brendan Barrett ◽  
Marc Kawaja ◽  
...  
Keyword(s):  
Factor Viii ◽  
Range Of Motion ◽  
General Health ◽  
Hemophilia A ◽  
P Value ◽  
Base Line ◽  
Role Emotional ◽  
Left Ankle ◽  
Sf 36 ◽  
Significant Difference

Abstract Due to a founder affect certain regions of the Canadian province of Newfoundland and Labrador have a very high prevalence of mild Hemophilia A. To accurately plan for future health related needs of these patients, a base-line cross-sectional study of a large cohort segregating a known founder mutation (Val2016Ala) was undertaken. Characteristics of Study Participants Characteristic Affected Males Control Males P-Values (Affected vs Control) Carrier Females Control Females P-Value (Carrier vs Control) Values are expressed as number, mean*, median(interquartile range)†, or percentage Overall N 61(5–76yrs) 44(4–81yrs) 0.883 88(8–88yrs) 65(16–78yrs) 0.935 Factor VIII* 0.15(0.05) 1.29(0.37) <0.001 0.78(0.31) 1.34(0.41) <0.001 Hepatitis Bc Antibodies 15.8%(9/57) 0%(0/28) 0.012 0%(0/75) 0%(0/58) - Hepatitis C Positive 19.7%(12/61) 0%(0/33) 0.001 0%(0/88) 0%(0/65) - HIV Positive 1.6%(1/61) 0%(0/33) 0.420 0%(0/88) 0%(0/65) - Diabetes 24.4%(11/45) 6.1%(2/33) 0.031 - - - BMI†(Adults Only) 30.0(27.0–33.0) 27.0(26.0–30.0) 0.028 29.0(20–57) 28.0(19–46) .446 Statistically Significant Difference SF-36 & Colorado PE-05 Mean Scores for Affected and Unaffected Males Affected Males Affected Males Affected Males Unaffected Siblings Unaffected Siblings Unaffected Siblings t-test Difference vs References Dimension n Mean SD n Mean SD P-Value P<0.05)* Affected Males(age 23–76 years) Unaffected Siblings(age 20–81 years) SF-36 Scores General Health Scale 44 58.1 28.7 32 71.4 21.4 0.024* Role Emotional 44 89.2 19.9 33 98.0 5.5 0.007* Colorado PE-05 Musculoskeletal Scores Axial Deformity 47 0.9 1.4 33 0.2 0.8 0.008* Range of Motion 47 4.2 2.0 33 2.9 1.8 0.005* Gait 47 2.4 3.5 33 0.4 1.4 0.002* Left Ankle 47 3.3 5.0 33 1.0 2.9 0.015* Right Ankle 47 3.2 4.6 33 0.7 1.3 0.002* There was a positive association between the SF-36 General Health Scale scores and the following values of the Colorado PE-05 Scores. Gait (R -.45, p<.001), Range of Motion (R-.08, P=.508), Axial Deformity (R -.35, P<.001), Right Ankle (R -.42, p<.001), and Left Ankle (R-.49, p<.001). The association between the SF-36 Role Emotional Scales for affected males and the values of the Colorado PE-05 was also positive. Gait (R -.52, P<.001), Range of Motion (R - .30, P = .01), Axial Deformity (R -.54, P<.001), Right Ankle (R -.06, P<.001) and Left Ankle (R-.52, p<.001). No patients studied have acquired an inhibitor to Factor VIII. Obese males (BMI >30) had significantly greater impairment in their Range of Motion than non-obese males (4.59 vs (3.34), p<0.05) independent of whether or not they had hemophilia. These results are consistent with our clinical experience that mild Hemophilia A causes episodic bleeding, lack of recognition and delayed treatment leads to significant morbidity. The association with diabetes was unexpected. Future interventions will now focus on prevention and early treatment of ankle bleeds and will include strategies to reduce the risk of obesity.

Effect of Mild Hemophilia A on Quality of Life Using the SF-36.

Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 5537-5537
Author(s):  
Meghan C. Walsh ◽  
David Macgregor ◽  
Brendan Barrett ◽  
Susan Stuckless ◽  
Marc Kawaja ◽  
...  
Keyword(s):  
Quality Of Life ◽  
General Health ◽  
Hemophilia A ◽  
Bodily Pain ◽  
Adult Males ◽  
Negatively Associated ◽  
Role Emotional ◽  
Sf 36 ◽  
The Impact

Abstract Using a cross-sectional study design we determined the impact of mild hemophilia A on quality of life. Adult males (n=47) with the same mutation (Val2016ala) were compared to unaffected adult males (n=32). The Short Form-36 (SF-36) and the Health Assessment Questionnaire (HAQ) were used to measure quality of life and physical function. Measures included bleeding history, Body Mass Index (BMI), age, and viral status. Using the SF-36, mild hemophilia was found to be statistically and negatively associated with quality of life in both the general health scale (p<0.039) and role emotional (p<0.005) dimensions. Age (>40) was associated with lower quality of life in four of eight dimensions namely, physical functioning (p<0.023), bodily pain (p<0.049), general health scale (p<0.034) and role emotional (p<0.025). A positive viral status of hepatitis (HCV) was found to be negatively associated with bodily pain (p<0.044) in affected males. Finally, those who experienced bleeding episodes (>2 in previous 5 years) were also associated with lower quality of life in social functioning (p<0.02) and the health transition scale (p<0.043). Additionally, a greater than 5-point difference was found in affected males in five out of eight dimensions when compared to the Canadian male population. No significant differences were found between groups from the HAQ scores. Mild hemophilia A, age, bleeding frequency and a positive hepatitis status were found to negatively affect quality of life in our study.

Differentiation of BM-HSCs Into FVIII Producing Hepatocytes: Approach to Haemophilia Treatment

Blood ◽  
2012 ◽  
Vol 120 (21) ◽  
pp. 4388-4388
Author(s):  
Amal M. El-beshlawy ◽  
Hala Gabr ◽  
Rania Zayed ◽  
Laila Hegaz ◽  
Rania Fawzy ◽  
...  
Keyword(s):  
Factor Viii ◽  
Hemophilia A ◽  
Conflicts Of Interest ◽  
Alpha Fetoprotein ◽  
Control Group ◽  
P Value ◽  
Rt Pcr ◽  
Significant Difference ◽  
The Mean

Abstract Abstract 4388 Background: Hemophilia is caused by a single-gene defect in which a small increase in gene products could transform a severe form of hemophilia into a mild one. Hemophilia treatments are readily available in developed countries, however In Egypt, Most hemophilia patients are treated with plasma or cryoprecipitate, where the treatment is associated with a high risk of blood-borne diseases. Liver transplantation in human and canine hemophilia A results in an increase in factor VIII levels to normal. Studies showed that BMCs not only differentiated into hepatic and liver cells, but they did express the intact gene of the FVIII A3 domain. Objective: In this work we studied the ability of bone marrow derived stem cells from hemophilia patients' relatives (carrier or normal) to be differentiated into hepatocytes expressing FVIII m-RNA in vitro as a step towards transplantation in haemophilia patients. It was necessary to prove that the applied culture conditions were successful not only to obtain hepaotocyte morphology but also hepatocyte ability to produce FVIII. Methods: The study was conducted on family relatives of five hemophilia A patients attending the hematology clinic, Cairo University hospitals. From each family, one hemophilia A carrier and one healthy person were subjected to the study. Informed consent was obtained from the participants. BM-HSCs were cultured in liquid culture containing HGF for 6 days. Differentiation into hepatocytes was evaluated by alpha-fetoprotein (AFP) expression using immunohistochemistry, albumin synthesis in culture supernatant using microalbumin assay kit, factor VIII activity by one stage clotting assay and expression of FVIII mRNA by RT-PCR. Results: Cell morphology changed after 6 days culture; round or polygonal-shaped cells with moderate cytoplasm and a medium-sized nucleus were observed. Morphologic confirmation of hepatocyte differentiation was done by immunocytochemistry; human alpha fetoprotein positive cells were detected in the culture. The positive cells appeared round or pear shaped, most of them contained one nucleus. However, few cells were binucleated with brown stained cytoplasm and bluish nuclei (Figure 1 A, B). By image analysis, the mean number of alpha fetoprotein positive cells estimated in10 random high power fields was 11 ± 1.6, 11 ±1.8 cells/HPF in the carriers and controls respectively. Immunophenotyping after culture; the percentage of CD 34+ve cells for the carrier group ranged from 0.5 to 2.5 with the mean of 1.2 ± 0.8 and from 0.7 to 2.1 with the mean of 1.5 ± 0.7 for the control group. There was no statistically significant difference between the two groups (p > 0.05) and the percentage of CD 90+ve cells for the carrier group ranged from 11.1 to 14.2 with the mean of 12.7 ± 1.2 and from 12.6 to 13.8 with the mean of 13.3 ± 0.6 for the control group. There was no statistically significant difference between the two groups (p > 0.05). On comparison between immunophenotyping before and after culture in both groups, statistical analysis showed highly significant decrease in CD34 positivity (p value 0.002 and 0.001) in the carriers and controls respectively associated with highly significant increase in the percentage of CD90 positive cells (p value 0.000) in the two groups. Albumin secretion was detected in the culture supernate at the 6th day culture, the mean albumin level was 0.52 mg/L ± 0.32 and 0.6 mg/L ± 0.4 in the carriers and controls respectively. F VIII activity was estimated; with the mean of 0.14%±0.021% and 0.5%±0.4% in the carriers and controls respectively. Transcription of FVIII m-RNA was detected by qualitative RT-PCR in 2 carriers and all controls (Figure 2). Conclusion: BM derived hepatocytes showed positive AFP expression. Functional tests performed showed their ability to produce albumin and perform FVIII activity. Also FVIII mRNA expression was detected. Induction of HSCs differentiation by in vitro manipulation may become a valuable tool to provide a cell source for liver transplant procedures and treatment of haemophilia patients. Disclosures: No relevant conflicts of interest to declare.

scholarly journals Efektivitas Exercise Intradialisis Menggunakan Barbell dan Range of Motion (ROM) terhadap Adekuasi Hemodialisa pada Pasien Penyakit Ginjal Kronik

2019 ◽  
Vol 2 (2) ◽  
pp. 189-200
Author(s):  
Yestiani Norita Joni ◽  
Busjra M Nur ◽  
Fitrian Rayasari
Keyword(s):  
Chronic Kidney Disease ◽  
Standard Deviation ◽  
Range Of Motion ◽  
Intervention Group ◽  
Control Design ◽  
P Value ◽  
Significant Difference ◽  
Post Test ◽  
The Difference ◽  
Keywords Hemodialysis

The purpose of this study is to know the effectiveness of intradialysis exercise using barbells and Range of Motion (ROM) on the adequacy of hemodialysis in patients with chronic kidney disease in the hemodialysis room of RSIJ Sukapura in 2018. The design of this study uses a design with non-probability pre and post test two groups without control design . The result of the difference in the effectiveness of the adequacy values between the two intervention groups after the intervention was given was the barbell intervention obtained 1,33 with a standard deviation of 0.485, an error standard of 0.114. Whereas in the ROM intervention group 1.67 the standard deviation was 0.485, the standard error was 0.114 and the p-value was 0.047 (> 0.05). Conclusion, there was no significant difference in the value of hemodialysis adequacy between the barbellROM intervention groups after the intervention.   Keywords: Hemodialysis Adequacy, Barbell, Exercise Effectiveness, Intradialysis, Range Of Motion (ROM)

scholarly journals Lower quality of life in longstanding mild primary hyperparathyroidism

2013 ◽  
Vol 57 (2) ◽  
pp. 139-143 ◽  
Author(s):  
Andreia Veras ◽  
Juliana Maia ◽  
Patricia Mesquita ◽  
Catia Eufrazino ◽  
Francisco Bandeira
Keyword(s):  
Quality Of Life ◽  
Primary Hyperparathyroidism ◽  
Functional Capacity ◽  
General Health ◽  
Sf 36 ◽  
Significant Difference ◽  
Mild Primary Hyperparathyroidism ◽  
Recent Diagnosis ◽  
Time Since Diagnosis

OBJECTIVE: To describe the non-classical manifestations and quality of life in patients with primary hyperparathyroidism (PHPT). SUBJECTS AND METHODS: We evaluated non-classical manifestations and quality of life of 22 patients with PHPT using the SF-36 questionnaire according to the time since diagnosis. RESULTS: In the scores of quality of life, a significant difference was found comparing the groups with previous and recent diagnosis of PHPT in terms of functional capacity (39 + 22.83 vs. 76.25 + 22.37; p = 0.001), limitation of physical aspects (30 + 38.73 vs. 75 + 21.32; p = 0.006), general health (57.20 + 19.16 vs. 77.75 + 15.70; p = 0.012), and vitality (49.00 + 21.19 vs. 70.00 + 24.12; p = 0.044). CONCLUSION: We found a significant difference in quality of life in relation to the time of the diagnosis of PHPT.

scholarly journals Assessing the Performance of Extended Half-Life Coagulation Factor VIII, FC Fusion Protein by Using Chromogenic and One-Stage Assays in Saudi Hemophilia A Patients

2020 ◽  
Vol 2020 ◽  
pp. 1-7
Author(s):  
Tarek M. Owaidah ◽  
Hazzaa A. Alzahrani ◽  
Nouf S. Al-Numair ◽  
Abdulmjeed O. Alnosair ◽  
Amelita M. Aguilos ◽  
...  
Keyword(s):  
Factor Viii ◽  
Half Life ◽  
Hemophilia A ◽  
Coagulation Factor ◽  
Time Interval ◽  
One Stage ◽  
Chromogenic Assay ◽  
Significant Difference ◽  
Mean Differences ◽  
The One

Background. The one-stage assay is the most common method to measure factor VIII activity (FVIII : C) in hemophilia A patients. The chromogenic assay is another two-stage test involving purified coagulation factors followed by factor Xa-specific chromogenic substrate. Aim. This study aimed to assess the discrepancy and correlation between the chromogenic and one-stage assays in measuring FVIII : C levels in hemophilia patients receiving Extended Half-Life Elocta® as a recombinant extended half-life coagulation factor. Methods. We performed a study comparing the measurements of FVIII : C levels by the chromogenic versus the one-stage assays at different drug levels. Data of FVIII : C levels, dosage, and the time interval from administration to measurement were retrieved from the hospital records. The correlation, mean differences, and discrepancy between the two assays were calculated. The linear regression analysis was used to predict the time interval till reaching 1% FVIII : C. Results. Fourteen patients with 56 samples were included in the study. Of them, 13 patients were receiving Elocta® as a prophylactic, while one was receiving Elocta® on demand. One-third of these samples showed a discrepancy between the chromogenic and one-stage assays. The two assays were well correlated. Mean differences were significant at the individual and the time interval level. The time since the last Elocta® injection could significantly predict FVIII : C levels (β = 0.366, P<0.001). Conclusion. Our findings suggested a significant difference between both methods; the FVIII : C levels measured by the one-stage assay were less than those estimated by the chromogenic assay. However, the measurements of FVIII levels by the two assays were well correlated but discrepant in one-third of the samples. The levels of FVIII : C reach 1% after 5.4 days since the last Elocta® administration.

Thromboelastography in Children with Coagulation Factor Deficiencies.

Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 2139-2139 ◽  
Author(s):  
Meera B. Chitlur ◽  
Indira Warrier ◽  
Madhvi Rajpurkar ◽  
Wendy Hollon ◽  
Lolita Llanto ◽  
...  
Keyword(s):  
Whole Blood ◽  
Thrombin Generation ◽  
Hemophilia A ◽  
Coagulation Factor ◽  
Response To Treatment ◽  
Factor Vii ◽  
P Value ◽  
Factor V ◽  
Mean Values ◽  
Significant Difference

Abstract The thromboelastograph produces a continuous profile of the rheological changes that occur during the process of coagulation using whole blood. This information can be transformed into a dynamic velocity profile of the changes in blood elasticity occurring during clotting. We used the TEG® hemostasis analyzer in patients with hemophilia A or B with and without inhibitors and other coagulation factor deficiencies (OFD), to study the thromboelastographic profiles in these patients. Materials and Methods: 62 children (6 months-19 years old) were enrolled according to IRB regulations. 29 children had severe hemophilia A (SHA), 4 moderate hemophilia A or B (Mod.H), 2 severe factor VII deficiency, 1 combined factor V and VIII deficiency, 1 VWD (type II B), 1 severe factor V deficiency, 1 Severe PAI deficiency, 19 normal controls (NC), and 4 SHA with inhibitors (SHA+I). All patients were studied 72 hours after the last dose of factor. Citrated whole blood was activated using recombinant human tissue factor (Innovin, Dade Behring Inc®) and recalcified using 0.2M CaCl2. In patients with central lines with heparin, a heparinase cup was used. The TEG® was run for ≥ 90 min. CBC with differential was obtained on all subjects. Results: There was no significant difference in the CBC parameters among patients. Analysis of the TEG data revealed the following: Table 1 TEG Parameters (mean values) SHA (n=29) Mod.H (n=4) SHA+I (n=4) OFD (n=6) Control(n=19) MTG:Max rate of thrombin generation; TMG: Time to MTG; R: Reaction Time; K: Time to reach an amplitude of 20mm; MA: Max. Amplitude MTG(mm*100/sec) 8.7 9.6 1.3 9 17 TMG(min) 27.5 16.6 62.7 17.5 8.9 R(min) 22 14 56 15 7 K(min) 7 4 41 4 2 Max.Amplitude, MA (mm) 59 56 12 58 62 The rate of thrombin generation as visualized by plotting the 1st derivative of the TEG course, in patients with SHA without inhibitors, showed that they could be divided into 2 groups based on MTG (</>9). When analysed the 2 groups showed the following characteristics (5 representative curves from each group are shown): Figure Figure Table 2 TEG Parameters (Mean values) MTG < 9 (n=16) MTG > 9 (n=13) p value TMA: Time to MA; MTG(mm*100/sec) 5.5 12.6 <0.001 TMG (min) 33 20 0.009 R(min) 26 16 0.004 K(min) 9 3.4 0.03 MA(mm) 56.1 62.3 0.01 TMA(min) 60 38 0.006 13/29 children with SHA had target joints and 69%of patients with target joints had a MTG<9. Conclusions: SHA patients have variable bleeding tendencies as seen by the variation in MTG. A lower MTG is associated with a higher incidence of target joints. This may provide a clue as to which patients may have the greatest benefit from primary prophylaxis. Patients with OFD have a TEG® profile similar to Mod.H patients. SHA+I have poor thrombin generation as seen by a significantly longer TMG and R time (p <0.05), compared to all subjects. The TEG may provide valuable clues to the severity of bleeding tendencies in patients with factor deficiencies. In additional observations (not shown), it appears that the TEG may be used to monitor the response to treatment with factor concentrates and tailor treatment with rFVIIa.

scholarly journals The Use of Chromogenic Factor VIII Assay Changes Treatment Approach in a Portion of Mild Hemophilia A Patients with Factor VIII Assay Discrepancy

Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 906-906
Author(s):  
Muntadhar Al Moosawi ◽  
Karen L. Dallas ◽  
Steven Wong ◽  
Shannon Jackson
Keyword(s):  
Factor Viii ◽  
Gene Mutation ◽  
Roc Curve ◽  
Hemophilia A ◽  
Treatment Approach ◽  
Gene Mutations ◽  
P Value ◽  
Moderate Severity ◽  
Mutation Database ◽  
Study Population

Introduction Hemophilia A is an X-linked inherited bleeding disorder characterized by a deficiency of coagulation factor VIII (FVIII). FVIII assays used for diagnosis and classification of hemophilia are the widely used one-stage assay (OSA) and the chromogenic assay (CSA), a more costly and less commonly used test. The CSA has the advantages of improved precision and insensitivity to pre-activation effects of FVIII that can lead to erroneous results. In approximately one-third of mild hemophilia A cases, reliance on OSA can lead to overestimation of factor level in comparison to CSA, and this 'discrepancy' phenomenon is closely associated with the FVIII molecular abnormality. In mild or moderate hemophilia A, assay discrepancy can lead to misclassification or inappropriate treatment. The objectives of this retrospective study were to determine the proportion of patients with OSA/CSA discrepancies defined by OSA to CSA ratio of &gt;1.5 or &lt;0.5, to identify previously reported and new F8 gene mutations and to classify the impact of assay discrepancy on clinical treatment approach. Also, the study aimed to re-evaluate published discrepant OSA/CSA ratios to identify a ratio with the highest sensitivity to indicate that a change of treatment approach or intensity may be required. Methods We reviewed the charts of adult (&gt;18 years old) patients with mild or moderate hemophilia A followed at the Adult British Columbia Hemophilia clinic with concomitant OSA and CSA results between January 2013 and March 2019. Data collected included patient age, disease severity classification, baseline OSA FVIII:C, and F8 gene mutation. Bleeding phenotype and impact of discrepancy on treatment approach was evaluated and determined independently by two individuals and was correlated with concomitant OSA and CSA. Gene mutation data was collected to determine whether OSA/CSA discrepancy has been previously reported in the international hemophilia A mutation database (www.factorviii-db.org). Descriptive data was reported as medians and ranges. Statistical analyses using Spearman's correlation, logistic regression and receiver operating characteristics (ROC) curve were performed using IBM SPSS v. 22.0. Results 98 patients were included in the study with median age of 53 years (18-88). 75 patients were classified by OSA at baseline as mild and 23 patients as moderate severity. Median FVIII:C by OSA was 15% (6%-40%) in the mild group and 3% (1%-5%) in the moderate severity group. Median FVIII:C using CSA was 13% (2%-66%) in the mild group and 4% (1%-7%) in the moderate group. Based on ratio alone, OSA/CSA discrepancy was detected in 51 (52%) patients out of which 3 (3%) were reverse discrepancies (CSA higher than OSA). Treatment approach was changed as a result of CSA introduction in 14 (27%) patients with OSA/CSA discrepancy (14% of the entire study population). OSA had overall good correlation with CSA (r = 0.84) and as expected, there was no correlation between age and OSA to CSA ratio (r = 0.03). In the patients with OSA/CSA discrepancies, neither age nor OSA/CSA predicted a change of treatment approach (OR 1.02 for age; 95% CI 0.99-1.06; p value 0.213 and OR 1.48 for OSA/CSA; 95% CI 0.96-2.28; p value 0.08). The OSA to CSA ratio of 1.8 to 3.5 demonstrated the highest area under the ROC curve and sensitivity for identification of requirement for treatment change in patients with OSA/CSA discrepancy (AUC 0.75; sensitivity 71%, negative predictive value 75%). Six F8 gene mutations with OSA/CSA discrepancies previously not reported in the database were identified. Conclusions OSA/CSA discrepancies were detected in 52% of the patients with mild or moderate hemophilia A and resulted in change of treatment approach in 27% of discrepant patients. Use of an adjusted OSA/CSA ratio between 1.8 to 3.5 was more likely to detect cases with a requirement for change of treatment approach and use of this ratio to indicate discrepancy may be more clinically meaningful. Six gene mutations associated with OSA/CSA discrepancies previously not described in the international hemophilia A mutation database were identified in the study population, highlighting the need for genotyping discrepant cases. Disclosures No relevant conflicts of interest to declare.

scholarly journals Socio Demographic Profile and Risperidone Response in New Versus Old Schizophrenia Patients

2020 ◽  
Vol 18 (1) ◽  
pp. 82-85
Author(s):  
Mohan Belbase ◽  
Jyoti Adhikari
Keyword(s):  
Negative Symptoms ◽  
Total Duration ◽  
P Value ◽  
Base Line ◽  
Age Group ◽  
Old Patients ◽  
Syndrome Scale ◽  
Significant Difference ◽  
The Mean ◽  
Negative Syndrome

Introduction: Schizophrenia is a mental disorder characterized with disorganized thinking, perception, expression of reality with significant social and occupational dysfunction. Two groups of drugs are in recent use namely first generation (typicals) and second generation (atypical) antipsychotics. Risperidone is a broad spectrum antipsychotic and has a role as a first-line agent for first break, mild to moderately ill patients and for severely ill treatment–refractory patients. Aims: This article tries to compare the risperidone response in newly diagnosed schizophrenia patients versus old patients already on some antipsychotics other than risperidone. Methods: This is an experimental intervention study of patients attending to psychiatry OPD and indoor in Nepalgunj Medical College, Kohalpur. Total 40 patients (27 new and 13 old) were selected and sample was collected in one year from January 2018 till December 2018. Positive and negative syndrome scale questionnaire was used to record the positive and negative symptoms of schizophrenia on baseline (week 0). Patients were followed up on week 4 and week 8 and the same positive and negative syndrome scale questionnaire was applied to record the improvement. Risperidone was given in therapeutic dose (4-8mg) on the basis of symptoms and improvement. Results: The study subjects were divided into new N=27 (17 male and 10 female) and old N=13 (7 male and 6 female). Maximum number of schizophrenia cases were in age group 15-25 and 35-44 years comprising 30 % in each group. Mean total duration of illness in new group was 23.89 ± 29.51 months (median being 12.0 months) while in old group it was 123.69 ± 83.34 months (median being 96.0 months) with significant difference between two groups (p= <0.001).The mean risperidone dose in milligram on base line (week 0) was 4.15 ±  0.55 for old group while it was 4.04 ± 0.52 for new group. On week 4, the mean dose for old group was 5.08 ± 0.95 while for the new group it was 4.81 ± 1.08. On week 8, the dose for old group was 6.08 ± 1.32 while it was 5.15 ± 1.35 for new group. There was a significant difference in the drug dose on week 8 between old group and new group with p value of 0.047 (statistically significant).  Conclusion: Our study suggests that schizophrenia is found in most productive age group. Risperidone is  effective in both new and old schizophrenia patients however old patients need higher dose of risperidone than new patients.

Pharmacokinetics of Factor VIII Level in Patients Hemophilia A in Relation to Clinical and Radiological Outcomes

QJM ◽  
2021 ◽  
Vol 114 (Supplement_1) ◽  
Author(s):  
Azza Abdel gawad Tantawy ◽  
Iman Ahmed Ragab ◽  
Mohamed Gomaa Khalil
Keyword(s):  
Factor Viii ◽  
Hemophilia A ◽  
Trough Level ◽  
Peak Level ◽  
Functional Independence ◽  
Bleeding Pattern ◽  
Bleeding Events ◽  
X Ray ◽  
Significant Difference ◽  
Trough Levels

Abstract Background The benefit of pharmacokinetics (PK) -guided dosing is that both prophylactic and “on demand” dosing will be based on actual FVIII trough and peak levels instead of current FVIII estimates based on body weight and in-vivo recovery based dosing. Knowledge will increase with regards the relationship between FVIII level and bleeding in individual patients. The dose and frequency of factor VIII for patients on prophylaxis should only be reduced if clinically justified and impact should be monitored with regard to bleeding events, bleeding pattern and joint status. Objective To assess the trough and peak level of factor VIII in patients with hemophilia A on low dose prophylaxis and its impact on the clinical and radiological joint status. Patients and Methods A cross sectional study was performed in Ain-Shams University, Pediatrics Hospital, Hemophilia Clinic. It included 25 children and adolescents with hemophilia A on prophylactic factor VIII during the period from September 2018 to August 2019. Factor VIII used was recombinant 3rd generation with a dose of 45 IU/Kg/week rounded to the nearest 500IU. Trough level of factor VIII was done before prophylactic dose and peak level was done one hour postinfusion through chromogenic assay using STAGO-Immuno-Def VIII reagent. Clinical joint score and Functional Independence Score of Hemophilia (FISH) were done. Radiological joint scores were done using conventional x-ray, ultrasound and MRI. Cut-off level of trough levels was studied at 1% and 1%-5% of factor VIII. Results Patients were classified into 3 groups,4 patients (21.1%) had trough level &lt;1%, 13 patients (68.4%) had trough level between 1%-5%, 2 patients (10.5%) had trough level &gt; 5%. No significant difference between trough level of factor VIII and clinical joint scores and FISH scores of patients. Median score of the worst joint of patients with trough level &gt;5% was 5 in comparison to 8 and 7 in patients with trough level 1%-5% and &lt;1% respectively. Mean FISH of patients with trough level &gt;5% was 9.50 ± 2.12 in comparison to 12.92 ± 5.41 and 11.00 ± 3.56 for patients with troughs 1%-5% and &lt;1% that was respectively. Comparison between groups with different trough level of factor VIII according to X-ray scores of patients showed no significant difference Conclusion Low trough levels alone did not warrant intensification of the prophylaxis regimen; rather, the dose and dosing frequency should be adjusted based on individual’s bleeding pattern and many other factors as shown in our study the insignificant relation between trough levels of factor VIII and clinical and radiological outcomes.. More frequent factor VIII monitoring and incorporation into population based PK are warranted.

scholarly journals Assessment of Gingival Health Status among Patients with Fixed Orthodontic Appliance

2021 ◽  
Vol 11 (1) ◽  
pp. 38-41
Author(s):  
Shahida R. Hussein ◽  
Bayan A. Hassan
Keyword(s):  
Mean Value ◽  
P Value ◽  
Orthodontic Appliances ◽  
Base Line ◽  
Orthodontic Appliance ◽  
Gingival Health ◽  
Fixed Orthodontic Appliances ◽  
Significant Difference ◽  
The Mean ◽  
Fixed Orthodontic Appliance

Fixed orthodontic appliances corrupt plaque removal, proper oral hygiene, and gingival health so periodontal evaluation is important to be checked in every appointment. The aim of the present study was to assess gingival health among patients with a fixed orthodontic appliance. A clinical comparative study conducted on 25 patients with an aged ranged from 15 to 25 years. Clinical parameters included plaque index (PI), gingival index (GI), and bleeding on probing (BOP) were recorded at base line before starting orthodontic treatment and after 1 month of treatment. A double paired t-test was applied to the data collected for statistical analysis. The mean value of PI (1.11), GI (1.155), and BOP (0.600) scores was increased after placement of fixed orthodontic appliance, but with no statistically significant difference with base line for PI (P-value = 0.596), GI (P-value = 0.355), and BOP (P-value = 0.256), respectively. Regarding age group, mean PI (1.46), GI (1.22), and BOP (0.875) were increased with increasing age, patients who age’s ≥20 showed statistical significant difference for gingival and plaque means P-value = 0.006 and P-value = 0.03) for ≥20, respectively. The finding of this study had shown that patients with fixed orthodontic appliances had non-significant increase in the mean value of plaque, gingival, and BOP indices scores. All mean scores were increased with increasing age, but with non-significantly for BOP only.

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