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Šrámek Bleeding Score

 
Šrámek Bleeding Score

Šrámek Bleeding Score

Authors/developers
Šrámek A, Eikenboom JCJ, Briet E, Vandenbroucke JP, Rosendaal FR

Questionnaire and scoring key

N.B.: These scores are posted with the permission of the developers.

  • Description
  • Utility
  • Administration
  • Psychometrics
  • References

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

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

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 required2,3

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)

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 carriers3,4

Age: Children and adults

  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.