[Long-term follow-up of isolated- XXXX deficiency typeIA: the clinical analysis of 2-sister cases].

@entity1 @entity407 @entity6 @entity2301 @entity28 @entity442 @entity2124 @entity71765 @entity209 @entity413 @entity214
OBJECTIVE: To elucidate the curative and adverse effect of recombinant @entity1 @entity2124 (rhGH) in 2 @entity1 with isolated- @entity2124 @entity71765 (IGHDIA), to track sexual development and pregnancy, and reassess the quality of life in the adulthood. METHOD: The authors summarized the data of 2-sister cases with IGHDIA; followed up for assessment of height, weight, blood pressure and sexual development; detected fasting blood lipids, @entity413 , insulin, @entity442 ( @entity442 ) and @entity2301 ( @entity2301 ); made an investigation of education and occupation, and so on. RESULT: After 6.2 and 7.3 years treatment with rhGH, the two sisters had considerably improved height from -7.8 @entity214 , -8.8 @entity214 to -2.6 @entity214 and -1.3 @entity214 respectively. No evident side effect was observed. They had normal sexual development and pregnancy. The levels of @entity442 and @entity2301 were still low, in the elder sister they were 46.6   g/L, 2460   g/L, and in the younger 52.4   g/L, 2430   g/L. No @entity407 , @entity6 or @entity28 occurred. CONCLUSION: Long term therapy with rhGH may improve final adult height of individuals with IGHDIA. They can have normal sexual development and pregnancy. @entity209 did not occur during the follow-up period.
