| ObjectiveThe aim was to retrospective analysis clinical data about childhood hemophilia including clinical classification, clinical type, the age of first onset, the age of the first bleeding,the age of diagnosis, family history,the causes of hemorrhage(spontaneous bleeding, traumatic hemorrhage or iatrogenic hemorrhage),the incidence of joint deformity, replacement therapy, the incidence of HBV,HCV,HIV and syphilis, of the children with hemophilia in the department of pediatric hematology and oncology of the Zhengzhou University First Affiliated Hospital during the last five years.To explored clinical characteristics,diagnosis and treatment in the children hemophilia at local area and provided some guidance in diagnosis and treatment for local children hemophilia in the future. MethodA total of 294 childhood patients with hemophilia were diagnosed in the First Affiliated Hospital of Zhengzhou University from January 2010 to December 2014 and were retrospectively analyzed. The age of all research object was less than or equal to 14 years old. According to the level of the activity of plasma factor VIII(FVIII: C) or factor IX(FIX: C), all research object were divided into three clinical types, including the heavy type(the activity of factor less than 1% of the normal value), intermediate type(the activity of factor between 1% and 5% of the normal value),light type(the activity of factor between 5% and 40% of the normal value).The bleeding degree of hemophilia was divided into three level. The first level was mild bleeding, including nasal bleeding, skin ecchymosis, subcutaneous hematoma and so on. The second level was moderate bleeding including the hematoma of soft tissue, oral hemorrhage, the hemorrhage of muscle, the joint bleeding and so on.The third level was severe bleeding, including the respiratory tract hemorrhage, the urinary tract bleeding, the gastrointestinal bleeding, the throat and neck hemorrhage, the intracranial hemorrhage, the retinal hemorrhage, the retroperitoneal hemorrhage, the intraperitoneal bleeding and so on.The above reference standard of the clinical classification, clinical type and the degree of bleeding/ Chinese Medical Sciences Blood Branch of Thrombosis and Hemostasis Group/ China Hemophilia Treatment Collaborative Group of Children Group/ Prevention and Treatment Group. Recommendations for diagnosis and treatment of children with hemophilia [J]. The Chinese Journal of Pediatrics, 2011, 49(3):193-195. Results1. The 294 cases of childhood hemophilia patients were male. The hemophilia A was 254 cases(86.39%). The hemophilia B was 40 cases(13.61%). The ratio between hemophilia A and hemophilia B was 6.35.2. The sereve cases was 83, the medium cases was 117 and the light cases was 94.The most of clinical type was medium.3. According to the age, the 294 cases of hemophilia children was divided into four age stages, including under the age of one years old, between one to three years old, between four to six years old, between seven to fourteen years. Every group was set for the left open, the right closed interval. The total of the age of the first hemorrhage onset less than or equal to three years old was 222 cases(75.51%). The infants and young children was the main part of the 294 cases of hemophilia children.4. The average age of first bleeding was 1.62±1.58 years old and the average age of diagnosis was 5.54±5.28 years old in 294 cases of children with hemophilia. The compare between the age of the first bleeding onset and the age of the first diagnose, the difference was statistically significant(t=-12.514?P=0.000). It indicated the presence of delayed diagnosis in hemophilia at local area.5. During all of the children with hemophilia, 57 of whom had family history(19.39%). The total of hemophilia A was 50 cases and hemophilia B was 7 cases in children with family history.6. 104 cases of children with hemophilia had spontaneous bleeding, which was the first form of the cause of the first bleeding. 166 cases had traumatic bleeding, which was the first form of the cause of the first bleeding. 24 cases had iatrogenic bleeding, which was the first form of the cause of the first bleeding(including 9 cases of intramuscular bleeding, 9 cases of vein puncture bleeding, 6 cases of preoperative examination). The ratio of severe childhood hemophilia was 44.58%, the ratio of medium childhood hemophilia was 26.50% and the ratio of mild childhood hemophilia was 38.30%. Spontaneous bleeding included 37 cases of severe hemophilia, 31 cases of medium hemophilia and 36 cases of mild hemophilia. The incidence of spontaneous bleeding between severe hemophilia and medium-light hemophilia, the difference was statistically significant(?2=4.286, P=0.038).7. 220 cases of children with hemophilia had mucocutaneous hemorrhage(74.83%), which was the first bleeding site of hemorrhage. The total of the severe hemophilia was 62 cases, the ratio of severe childhood hemophilia was 74.70% in 294 cases of hemophilia in children. The total of the medium hemophilia was 82 cases, the ratio of medium childhood hemophilia was 70.09% in 294 cases of hemophilia in children. The total of the mild hemophilia was 76 cases, the ratio of mild childhood hemophilia was 80.85% in 294 cases of hemophilia in children. The incidence of the mucocutaneous hemorrhage between severe-medium hemophilia and light hemophilia, the difference was not statistically significant(?2=2.660, P=0.103).14 cases of children with hemophilia had sereve bleeding. The total of the severe hemophilia in sereve bleeding was 7 cases. The total of the medium hemophilia in sereve bleeding was 4 cases, the total of the mild hemophilia in sereve bleeding was 3 cases. The incidence of the sereve bleeding between severe hemophilia and medium-light hemophilia, the difference was not statistically significant(?2=3.438, P=0.064)8. 95 cases(32.21%) of children with hemophilia had joint deformity. The top of joint deformity was the knee joint deformity(65 cases of childhood hemophilia, 68.42%). Ankle malformation was the second of all the children with hemophilia. Elbow deformity was the third of all the children with hemophilia. The total of the severe hemophilia was 18 cases, the ratio of severe childhood hemophilia was 21.69% in 294 cases of hemophilia in children. The total of the medium hemophilia was 52 cases, the ratio of medium childhood hemophilia was 44.44% in 294 cases of hemophilia in children. The total of the mild hemophilia was 25 cases, the ratio of mild childhood hemophilia was 26.60% in 294 cases of hemophilia in children. The incidence of the joint deformity between severe hemophilia and medium-light hemophilia, the difference was statistically significant(?2=5.971, P=0.015). The joint deformity in children with hemophilia was mainly occurred in more weight or activity of the joints, such as the knee, ankle and elbow.9. 294 cases with hemophilia in children had received replacement therapy. 177 cases(60.20%) with hemophilia in children had received the treatment of the infusion of plasma, cold precipitation and very few blood coagulation factors. 74 cases(25.17%) with hemophilia in children had received the treatment of the infusion of plasma, cold precipitation. Only 43 cases(14.63%) with hemophilia in children had received the treatment of the infusion of blood coagulation factors.10. 294 cases of children with hemophilia were performed about the detection of infectious diseases(including hepatitis B, hepatitis C, syphilis, AIDS) before replacement therapy. HCV injection was found in only one patient, without a history of HCV in the family. Conclusion1. There is a gap between the age of first onset and the age of diagnosis. It indicate that there is the phenomenon of delayed diagnosis at local area.2. The incidence of spontaneous bleeding in sereve hemophilia is higher than mild- mediun hemophilia.3. The incidence of joint deformity is high. The incidence of joint deformity in sereve hemophilia is higher than mild-mediun hemophilia.4. Replacement therapy is not standard, the dosage of coagulation factor was not enough. |
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