Validation of a new pediatric joint scoring system from the International Hemophilia Prophylaxis Study Group: Validity of the hemophilia joint health score

Repeated bleeding into the joints leads to the development of chronic and progressive joint disease in 90% of people with severe haemophilia. Owing to recurrent haemarthroses, musculoskeletal outcome remains an important hallmark of treatment efficacy in haemophilia. Physical joint assessment is commonly used to measure structural and functional joint damage. With the development and introduction of prophylaxis, the most widely used instruments for the assessment of haemophilic arthropathy were found to lack sensitivity to detect the earliest signs of joint disease. To address these inadequacies, a new international consensus tool, the Haemophilia Joint Health Score (HJHS), was developed. The HJHS appears to be a sensitive, reliable and valid tool. However, the HJHS needs further evaluation in various patient populations to assess its applicability and usefulness.

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A Pilot Study of Patients’ Views of an Oral Health Scoring System

Primary Dental Care ◽

10.1308/135576104773711246 ◽

2004 ◽

Vol os11 (2) ◽

pp. 37-39 ◽

Cited By ~ 5

Author(s):

FJ Trevor Burke ◽

Michael Busby ◽

Siobhan McHugh ◽

Anne Mullins ◽

Roger Matthews

Keyword(s):

Oral Health ◽

Pilot Study ◽

Scoring System ◽

Good Method ◽

Large Majority ◽

Study Group ◽

Health Score ◽

Dental Practitioners

An oral health scoring system (Oral Health Score: OHS) has been designed to provide a numerical measure of the overall state of a patient's oral health by means of a series of simple clinical examinations. Objective To assess, by means of a questionnaire, patients’ opinions of the value of the OHS. Methods Eight general dental practitioners who used the OHS were asked to participate in the project. The participating dentists were requested to explain the aims of the project to 50 patients. Patients who agreed to participate were asked to complete a questionnaire after a visit during which the OHS was used. These patients were provided with a reply-paid envelope for the return of the questionnaire. Results Completed, usable questionnaires were received from 315 patients. Of respondents, 97% considered that the OHS gave them a better understanding of the condition of their mouth and 98% considered that the OHS was a good method for communication between dentist and patient. Conclusion The results of the present study indicate that, within the study group, the OHS is considered by a large majority of patients to be a good method for communication of the patient's oral health between dentist and patient.

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Known-group validity of patient-reported outcome instruments and hemophilia joint health score v2.1 in US adults with hemophilia: results from the Pain, Functional Impairment, and Quality of life (P-FiQ) study

Patient Preference and Adherence ◽

10.2147/ppa.s141392 ◽

2017 ◽

Vol Volume 11 ◽

pp. 1745-1753 ◽

Cited By ~ 12

Author(s):

Tyler W Buckner ◽

Michael Wang ◽

David L Cooper ◽

Neeraj N Iyer ◽

Christine L Kempton

Keyword(s):

Quality Of Life ◽

Functional Impairment ◽

Patient Reported Outcome ◽

Health Score ◽

Group Validity ◽

Patient Reported ◽

Joint Health

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Hemophilia joint health score reliability study

Haemophilia ◽

10.1111/j.1365-2516.2006.01312.x ◽

2006 ◽

Vol 12 (5) ◽

pp. 518-525 ◽

Cited By ~ 179

Author(s):

P. HILLIARD ◽

S. FUNK ◽

N. ZOURIKIAN ◽

B.-M. BERGSTROM ◽

C. S. BRADLEY ◽

...

Keyword(s):

Reliability Study ◽

Health Score ◽

Score Reliability ◽

Joint Health

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Subchondral Bone Degradation Following Bone Marrow Stimulation -An MRI Analysis-

Foot & Ankle Orthopaedics ◽

10.1177/2473011417s000076 ◽

2017 ◽

Vol 2 (3) ◽

pp. 2473011417S0000

Author(s):

Yoshiharu Shimozono ◽

Youichi Yasui ◽

Robin Megill ◽

John Kennedy

Keyword(s):

Subchondral Bone ◽

Scoring System ◽

3 Tesla ◽

Joint Loading ◽

Thickness Change ◽

Health Score ◽

Subchondral Cyst ◽

3 Tesla Mri ◽

The Mean

Category: Ankle Introduction/Purpose: Microfracture is the most common operative procedure for the treatment of osteochondral lesions of the talus (OLT). The role of the subchondral bone (SCB)/plate (SCBP) is recognized as one of the most significant factors providing joint-loading support to the ankle. Greater attention has recently been paid to the SCBP and its effects on overlying hyaline and fibrocartilage function. However, damage to the SCB and SCBP during microfracture may irreversibly change the joint loading support of the ankle, leading to reparative cartilage degradation. The purpose of this study is to investigate the morphological change in the SCB and SCBP after microfracture for OLT by developing a novel magnetic resonance imaging (MRI) scoring system specifically for evaluating SCB and SCBP. Methods: Twenty patients who underwent microfracture for OLT and had at least a one year follow-up 3-Tesla MRI between 2008 and 2011 were selected. A SCB Health scoring system was developed to assess the condition of the SCB using 3-Tesla MRI. The SCB Health score is based on the amount of edema, subchondral cyst diameter, qualitative change in SCBP morphology and thickness change of the SCBP (Table 1). Nine of the twenty patients had two follow-up MRIs, and differences in their scores were calculated. The clinical evaluation was assessed using the Foot and Ankle Outcome Scores (FAOS) preoperatively and at final follow-up. Results: At first follow-up MRI, 65% of patients had mild or greater edema, 40% had subchondral cysts, 65% had irregularity or collapse of their SCBP and 85% had a >25% change in SCBP thickness. The overall SCB Health score of 90% of patients were abnormal. Of the nine patients who had a subsequent follow-up MRIs, the mean time between the first and second study was 22±6 months. The scores between the two time points were not statistically significant (p=0.347, p=0.559, p=0.169, p=0.347 p=0.154 for edema, subchondral cyst diameter, qualitative SCBP measurements, SCBP thickness change and overall SCB Health score, respectively). The mean FAOS score improved significantly from 55.5±16.8 preoperatively to 76.0±12.3 at final follow-up (p<0.05). Conclusion: The SCB and SCBP were not completely healed in 90% of patients at a mean 18 months follow-up following microfracture of the talus. Subsequently, morphological changes were not restored at a mean 43 months at second follow-up in a smaller subset of patients. Most previous studies have focused on the degeneration of the articular cartilage layer, and its effect on clinical outcomes. The authors of current study believe that looking at the changes in the SCB may provide additional information as to why BMS procedures fail in the long-term.

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The Clinical Features and Prognosis of Early T-Cell Precursor Subtype of Lymphoblastic Lymphoma in the Japanese Pediatric Leukemia/Lymphoma Study Group ALB-NHL03 Study

Blood ◽

10.1182/blood.v124.21.1728.1728 ◽

2014 ◽

Vol 124 (21) ◽

pp. 1728-1728

Author(s):

Reiji Fukano ◽

Shosuke Sunami ◽

Masahiro Sekimizu ◽

Tetsuya Takimoto ◽

Tetsuya Mori ◽

...

Keyword(s):

T Cell ◽

Scoring System ◽

Lymphoblastic Lymphoma ◽

Study Group ◽

Advanced Stage ◽

Sufficient Data ◽

Cell Precursor ◽

Pediatric Leukemia ◽

Significant Difference ◽

Lymphoma Study Group

Abstract Introduction: Recently, early T-cell precursor acute lymphoblastic leukemia (ETP-ALL) was identified as a subtype of T-cell ALL (T-ALL), with distinctive gene expression and cell marker profiles. Some reports revealed that ETP-ALL was associated with a high risk of remission induction failure and relapse. In precursor T-cell lymphoblastic lymphoma (T-LBL), the clinical features and prognosis of the ETP subtype are not clear yet. In this study, we analyzed the data obtained from patients of advanced stage T-LBL to clarify the prognosis of pediatric T-LBL according to the immunophenotypes, including the ETP subtype of LBL. Patients and methods: From November 2004 to October 2010, 136 children (aged 1–18 years) with newly diagnosed advanced stage LBL (stages III and IV) were eligible for the Japanese Pediatric Leukemia/Lymphoma Study Group ALB-NHL03 study. We analyzed their immunophenotyping data as well as the ETP subtype. The immunophenotype of T-LBL was classified into pro-T, pre-T, intermediate T, and mature T based on the European Group for the Immunological Characterization of Leukemias (EGIL) classification. The definition of ETP subtype LBL was based on a previous report from the Tokyo Children’s Cancer Study Group (Inukai et al, 2011) using a scoring system consisting of the following 11 markers: CD4, CD8, CD13, CD33, CD34, HLA-DR, CD2, CD3, CD4, CD10, and CD56. Both definitions were based on flow cytometric analysis. Results: In this analysis, 104 (76%) patients were diagnosed with T-LBL. Sufficient data to evaluate the EGIL classification was available for 40 out of 104 patients. The remaining patients could not be classified due to incomplete immunophenotypic data. There were 1, 9, 21, and 9 cases of Pro-T, pre-T, intermediate T, and mature T, respectively. The 3-year event-free survival (EFS) of pro-T/pre-T and intermediate T/mature T was 80.0 ± 12.7% and 76.7 ± 7.7%, respectively (P = 0.7586). For evaluating the ETP subtype of LBL, sufficient data, obtained by using the scoring system with 11 markers, was available for 40 patients. Eight (20%) and 32 (80%) patients were classified as having ETP and non-ETP subtype, respectively. Bone marrow involvement for patients with ETP and non-ETP subtype was observed in 7 (88%) and 11 (34%) cases, respectively (P = 0.014). Central nervous system involvement in patients with ETP and non-ETP subtype was observed in 2 (25%) and 0 cases, respectively (P = 0.036). Thus, stage IV classification was more frequently observed in patients with ETP subtype than in patients with non-ETP subtype (P = 0.014). The 3-year EFS of patients with ETP and non-ETP subtype were 75.0 ± 15.3% and 71.9 ± 8.0%, respectively. There was no significant difference in EFS between patients with ETP and non-ETP subtype (P = 0.8281). Conclusion: For 40 out of 104 T-LBL patients, sufficient data was available to evaluate the immunophenotype for EGIL classification and ETP subtype. There was no significant difference in EFS according to the immunophenotypic subtype of T-LBL. In contrast to T-ALL, ETP subtype in LBL was not statistically related to EFS in this analysis. Disclosures No relevant conflicts of interest to declare.

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A comparison of diaphyseal and metaphyseal techniques of ulna shortening

Journal of Hand Surgery (European Volume) ◽

10.1177/1753193412469126 ◽

2012 ◽

Vol 38 (5) ◽

pp. 542-549 ◽

Cited By ~ 6

Author(s):

G. Sennwald ◽

D. Della Santa ◽

J.-Y. Beaulieu

Keyword(s):

Retrospective Study ◽

Pain Relief ◽

Scoring System ◽

Delayed Union ◽

Study Group ◽

Self Assessment ◽

Plate Removal ◽

Non Union ◽

Ulnar Head

The aim of this retrospective study was to compare two techniques of ulna shortening for ulnocarpal abutment. The technique performed initially was diaphyseal osteotomy. Subsequently, a metaphyseal osteotomy was performed to avoid the drawbacks related to the plate. The study group consisted of 29 patients: 13 with diaphyseal and 16 with metaphyseal osteotomies. The pre-operative diagnosis was ulnocarpal abutment in all cases. The results were graded according to the scoring system of Chun and Palmer and patients’ self-assessment. Both rankings were related to indications, age, gender, occupation, and surgery. Final follow-up occurred at a median of 54 (range 15–144) months after surgery. There was no delayed union or non-union, necrosis of the ulnar head, or infection. Pain relief was the main benefit. Both techniques gave similar outcomes for pain relief, satisfaction, and objective results. The shortening was significantly greater in the metaphyseal group without any apparent consequence on function or pain. No screw removal was needed after metaphyseal osteotomy; in contrast, plate removal was required in all patients. We conclude that both techniques are valuable alternatives for treating ulnocarpal abutment.

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