Score relatif aux saignements de Šrámek

Description

The Šrámek bleeding score is a short questionnaire that can either be self- or expert-administered. It is designed to assist in the clinical diagnosis of mild inherited bleeding disorders. The target population is people with mild inherited bleeding disorders.

Contact person:
FR Rosendaal: f.r.rosendaal@lumc.nl

Date of this review: August 27, 2013
Updated: April 2014

Utility

Overall utility:

• Useful in the primary care clinical setting to screen for a bleeding disorder
• May be self-administered

Limitations:

• Low discriminating power in a specialty referral setting (i.e. better for primary care)
• No validation against gold standard laboratory/clinical diagnosis
• No specific assessment of sensitivity or specificity of abnormal bleeding score
• Time to complete not known

Administration

1. Time to complete: Not identified
2. Equipment/space required: None
3. Training required:
   • None for self-administered questionnaire¹
   • For expert-administered, no training specific to the administration of the scoring tool is required^2,3
4. Cost: None
5. Scoring/scaling/interpretation of results:
   • Scale: 0–1 for each of 16 main bleeding symptoms or categories. For some categories the maximum score could be as high as 5 if sub-categories of that question were positive (e.g. severity)¹
   • Possible range of total score 0–40¹ (Podda modification range 0–42 for 12 main bleeding symptoms)²
   • Abnormal if total score ≥12 (based on the Podda-modification of the score²)

Psychometrics

Psychometric properties:

1. Construct validity:
   Convergent validity
   
   • PFA-100^® Collagen-Epinephrine cartridge closure times progressively increased in association with higher bleeding score categories (four categories in this study: 0–3, 4–7, 8–11, ≥12), however bleeding time and PFA-100^® Collagen-ADP cartridge closure times did not differ among groups.²
   • A significant correlation between PBAC score and Podda-modified bleeding score² was observed in women with and without underlying bleeding disorders (Spearman’s rho 0.44, p<0.001)³
   • Median bleeding score increased in mild to moderate forms of rare bleeding disorders and VWD (although the trend was not statistically significant). A similar non-significant trend was seen in female hemophilia carriers with factor levels ranging from normal to mild to moderate deficiencies having proportional increases in bleeding score.³
   Group differences
   • Presence of certain symptoms (e.g. bleeding post tonsillectomy and adenoidectomy) was most informative at differentiating controls from those with bleeding disorders.¹
   • Abnormal bleeding score (Podda-modified)² was observed in 49% of women with a bleeding disorder and no women without an underlying bleeding disorder.³
2. Criterion validity:
   • No studies identified
3. Reliability:
   • No studies identified
4. Responsiveness/sensitivity:
   • ROC-AUC for interview (using only presence or absence of symptoms rather than actual bleeding score) in a screening situation had high discriminating power (AUC not recorded), and in a referral clinic (hematology) setting of minimal discriminating power (AUC not recorded).¹

Languages studied: English

Groups tested:

• Healthy controls¹
• Adults with mild hemophilia A/B¹
• Adults with mild VWD¹
• Adults with platelet dysfunction¹
• Adults and children being evaluated for a possible bleeding disorder²
• Women with rare bleeding disorders, VWD, and hemophilia carriers^3,4

Age: Children and adults

Glossary of Psychometric Properties

References

1. Šrámek A et al. Usefulness of patient interview in bleeding disorders. Arch Intern Med 1995; 155: 1409-1415.
2. Podda GM et al. Usefulness of PFA-100^® testing in the diagnostic screening of patients with suspected abnormalities of hemostasis: comparison with bleeding time. J Thromb Haemost 2007; 5: 2393-2398.
3. Siboni SM et al. Gynaecological and obstetrical problems in women with different bleeding disorders.Haemophilia 2009; 15: 1291-1299.
4. Plug I et al. Bleeding in carriers of hemophilia. Blood 2006; 108: 52-56.