| ObjectiveTo sum up the clinical character and laboratory examination of Inherited Platelet Function Disorders (IPFD). To analyze the daily quality of life of pediatric patients with IPFD and provide references for its diagnosis and treatment.Methods1. Retrospectively study the clinical features of 64 patients diagnosed with IPFD in Children’s Hospital of Chongqing Medical University between 1996 and 2013.The questionnaires were obtained by telephone according to the daily life evaluation of children.2. SPSS 21.0 software was used for data analysis and the level of statistical significance was set at P<0.05.Results1. Of all the 64 patients, ratio of male to female was 1.21:1.2. Age of 64 patients is from 1 hour to 14 years and median age is 17m.29 cases are younger than lyear (45.3%) and 44 cases (64.1%) are younger than 3years.3. Of all the 64 patients,12 cases’father and/or mother conducted the test of platelet aggregation function, which 8 cases (66.7%) were decreased.4. The majority cases manifest spontaneous bleedings (68.8%), followed by upper respiratory infection (10.7%) and traumatic induced bleeding (9.4%).5. The majority of the bleeding organs was skin (64.1%), followed by epistaxis (54.7%), Gl bleeding (17.1%) and oral cavity bleeding (14.2%).6.21 in 27 patients had abnormal clot retraction test. But no significant difference was found between the abnormal clot retraction test rate and bleeding sites (P>0.05).7.53.1% cases were found with normal hemoglobin or mild bleeding induced anemia while 46.9% cases with moderate or severe anemia.8. There is no statistically significance between the severity of anemia with abnormality of clot retraction test rate (P>0.05).9. There is no statistically significance between the bleeding sites and platelet aggregation function (P>0.05).10. There is no significant difference of platelet aggregation function among different age groups (P>0.05).11. The children quality of daily life evaluation (Beijing children’s hospital version) was obtained from 8 cases. Five cases older than 8 years had no bleeding anymore. Two cases older than 7 years had less bleeding than before. One case was lyear old and the bleeding tendency was relieved.12. Of all the 64 patients, blood transfusion or symptomatic treatment was given and achieved bleeding preventive purpose. Two female adolescents manifesting prolonged menorrhagia obtained curative effects with oral estrogen administer.13. Age and severity of anemia are associated abnormal bleeding by univariate analysis (P<0.05). Logistic multivariate analysis indicated that anemia was an independent factor related to bleeding score (P<0.05), while gender, age, the cause of first episodic bleeding or clot retraction test had no impact on bleeding score.Conclusion1. The majority of IPFD were male and onset in infants. More than half patients had a positive family history.2. The majority cases manifest spontaneous bleedings, followed by upper respiratory infection and traumatic induced bleeding. Most bleeding organs was skin, followed by epistaxis, GI bleeding and oral cavity bleeding.3. Clot retraction test and platelet aggregation function couldn’t measure the severity of hemorrhage. The majority of bleeding induced anemia was mild.4. Anemia was an independent factor related to the bleeding score, while gender, age, the cause of first episodic bleeding and clot retraction test had no impact on it.5. Reduced bleeding tendency was found along with patient growing, but life-threaten bleeding still will be seen in IPFD. |
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