The Clinical And Quality Of Life Analysis Of137Children With Haemophilia

Objective: To explore predictive clinical manifestations and quality oflife status aiming to reduce haemophilia arthropathy and disability ofchildren with haemophilia.Methods:1. The study group was137patients with haemophilia diagnosed from2008to2014in Chongqing Children’s hospital. Retrospectively analyzethe clinical characteristics and questionnaires (FBS, CHO-KLAT2.0). Thequestionnaires were obtained in the haemophilia communication meeting.2. The statistical analysis were got by SPSS19.0software and the datawas analyzed with variance analysis, chi-square test, Kruskal-Wallis Testand t-test according to specific consideration.(The significance level wasset at P<0.05).Results:1. Out of137haemophilia children, there were114haemophilia A(HA) and23haemophilia B (HB). The factor level in81children withhemophilia were measured, including66HA and15HB.2. Out of137haemophilia children, there were1female (HA) while 136were male.114HA children’s age were from7m to275m averaged at97.70m.23HB children’s age were from18m to239m averaged at79.65m.The severity which was classified by factor level between HA and HB hadno significant difference （P>0.05）.3. The age of first episodic bleeding between HA and HB had nosignificant difference （P>0.05）, which is majored in infancy. But mildgroup is significant different with moderate and severe ones in the age offirst episodic bleeding (P<0.05）, moderate and severe group had nosignificant difference（P>0.05）.4. The majority of the reason causing first episodic bleeding in HA andHB both was traumatic bleeding, followed by spontaneous bleeding andiatrogenic bleeding. The majority of the first episodic bleeding position inHA and HB was skin, followed by deep muscle, joints and brain. Theseverity of first episodic bleeding between HA and HB had no significantdifference（P>0.05）.5. The age of the first consultation between HA and HB had nosignificant difference（P>0.05）. The first consultation age in mild HA hadsignificant difference with moderate and severe group（P<0.05），while nosignificant difference between moderate and severe ones（P>0.05）. Thefirst consultation age between mild and moderate plus severe ones in HBhad no significant difference（P>0.05）.6. The delayed consultation percentage is67.15%(92cases). 21(22.83%) cases of137patients consulted after24m of their first episodeof bleeding. The delayed consultation time in mild HA is much longerthan moderate and severe ones（P<0.05） but no difference betweenmoderate and severe group（P>0.05）. The delayed consultation timebetween mild and moderate plus severe HB had no significant difference（P>0.05）.7.32（28.07%）cases out of114HA had positive family history and9（39.13%）cases out of23HB did. The positive family history ratebetween HA and HB had no significant difference（P>0.05）.8. The age of the first arthrorrhagia between HA and HB had nosignificant differenc（eP>0.05）. The first arthrorrhagia age between HA andHB, or among the different severity groups according to factor level had nosignificant difference（P>0.05）. The majority involved first arthrorrhagiawas knee, followed by ankle, elbow and hip.9.47.27%(26/55) of the patients had developed joint deformities, themajority involved joint was kne（e23/30,88.46%）, followed by elbow（4/30,15.38%）, ankle（2/30,7.69%） and hip（1/30,3.85%）. The ages betweenjoint deformities group and normal joint group had significant difference（P<0.05）. But the disease severity between joint deformities group andnormal one had no significant difference（P>0.05）.10. The FBS score among mild, moderate and severe HA had nosignificant difference（P>0.05）. The FBS score between mild and moderate plus severe HB had no significant difference（P>0.05）. The FBS score ininfant HA group is significant different with school and preschool agegroup（P<0.05），but no significant difference between school and preschoolage group（P>0.05）. The FBS among different age groups in HB had nosignificant difference（P>0.05）. The FBS score between joint deformitiesgroup and normal joint group was significant different (P<0.05).11. The Parent-CHO-KLAT among different age groups in HA hadsignificant difference (P<0.05). In HB, the Parent-CHO-KLAT in schoolage group is significant different with infant and preschool group (P<0.05),but had no significant difference between infant and preschool group(P>0.05). Parent-CHO-KLAT among mild, moderate and severe HA had nosignificant difference (P>0.05). The Kid-CHO-KLAT among different agehaemophilia and among mild, moderate and severe group, both had nosignificant difference (P>0.05).12. None of the58haemophilia patients had prophylaxis treatment, allwere treated by on-demond treatment, only10(17.24%) cases of themwould like accept prophylaxis treatment.Conclusion：1. The majority of haemophilia were male and HA. The age of firstepisodic bleeding was toddler and the most reason of first episodic bleedingwas traumatic bleeding. The major position of first episodic bleeding wasskin and mild bleeding was the most seen. The first consultation time was usually in infancy, but delayed consultation rate was still high. The firstarthrorrhagia most happened in preschool age and the knee was majorinvolved joint followed by ankle, elbow and hip. The average age of jointdeformities was97.23m, and the joint deformities rate was increased withage.2. The family burden was affected by haemophilia but was relativelymild in infant groups. Joint deformities affected the family burden morethan normal joint. The CHO-KLAT score in our country was much lowerthan in developed country, indicating poor quality of life in our countrywhich probably was due to on-demand instead of prophylaxis treatment.