Effect Observation On Growth-Promoting And Influence On Related Indexes Of Replacement Therapy With Recombinant Human Growth Hormone In Children With Prader-Willi Syndrome In Shanxi Province

Objectives:To observe the clinical manifestations of prader-willi syndrome in Shanxi Province and explore the growth-promoting effects of recombinant human growth hormone therapy,and the effects on thyroid function,fasting plasma glucose,glycosylated hemoglobin during the treatment,so as to provide a basis for clinical treatment.Methods:Seven patients with prader-willi syndrome admitted to the out-patient department of shanxi children’s hospital from January 2017 to January 2019 were selected.Clinical data of these patients were collected,their clinical manifestations were observed and genetic testing were performed.RhGH 0.1~0.15U/(kg· d)was injected subcutaneously once a night after diagnosis,followed up for review every 3~6 months.Monitoring patients after treatment for 3 months,6 months,12 months of height,weight,growth rate,height standard deviation score,insulin-like growth factor 1 and fasting plasma glucose,glycosylated hemoglobin,thyroid stimulating hormone,three triiodothyronine and free thyroxine,collect the relevant data and statistical analysis.Results:1.After repeated measurement of variance analysis,the results showed that the overall mean values of IGF-1 were not completely equal before treatment,3,6 and 12 months after treatment,and the difference was statistically significant(F=16.54,P<0.001).Compared with before treatment,IGF-1 values were significantly increased 3 months after treatment(188.9±91.9),6 months after treatment(293.2±84.3),and 12 months after treatment(324.3±67.1)(P<0.05).The overall mean values of GV were not all equal before treatment,3,6 and 12 months after treatment,and the difference was statistically significant(F=25.2,P<0.001).Compared with before treatment,GV values were increased after 3 months of treatment(12.3±2.1)and 6 months of treatment(9.3±1.2)(P<0.05),while there were no significant increase after 12 months of treatment(7.5±0.6)(P>0.05).It cannot be considered that the overall mean values of HtSDS before treatment,3 months after treatment(-0.9±1.2),6 months(-0.9±1.2),and 12 months(-0.03±0.1)were statistically different(F=6.48,P>0.05).2.After repeated measurement of variance analysis,it cannot be considered that the overall mean values of TSH before treatment,3 months after treatment(4.5±1.0),6 months after treatment(4.6±0.7),and 12 months after treatment(5.0±0.5)were statistically different(F=0.58,P>0.05).The overall mean values of FT3 before treatment,3 months after treatment(6.2±1.3),6 months after treatment(6.6±0.7)and 12 months after treatment(6.2±0.9)were not statistically significant(F=0.54,P>0.05).The overall mean values of FT4 were not statistically significant before treatment,3 months after treatment(15.2±1.7),6 months after treatment(15.3±1.7),and 12 months after treatment(15.5±1.0)(F=0.11,P>0.05).The overall mean values of FPG before treatment,3 months after treatment(5.3±0.6),6 months after treatment(5.3±0.6),and 12 months after treatment(4.8±0.5)could not be considered statistically different(F=1.27,P>0.05).The overall mean values of HbA1 c before treatment and 3 months after treatment(5.0±0.3),6 months after treatment(5.0±0.6),and 12 months after treatment(5.1±0.6)were not statistically significant(F=0.34,P>0.05).Conclusions:1.In children with PWS,both IGF-1 and GV are increased,so rhGH can promote the growth of children with PWS.2.rhGH treatment had no significant effect on thyroid function,fasting plasma glucose and HbA1 c in children with PWS.