What are the considerations for the review of dwarfism

Regular review items and precautions for children with dwarfism after treatment: I. Height measurement It is necessary to measure the height of children with dwarfism every month after treatment, and since the change in height within one month is small, it should be measured as accurately as possible. Accurate height measurement needs to pay attention to “four similarities”: ① the same time, because the vertebral space of the spine will be compressed after standing or sitting up, the height will be different in the morning and evening, and the measurement will be comparable only at the same time; ② the same measuring tape; ③ the same measurer measures the child, but the tightness of the tape card on the head will be different for different people; ④ the child the same method of standing (requiring shoes off, heels, hips, shoulders and head all against the wall, chest up, stomach in, waist as straight as possible, eyes level, head not too tilted, otherwise the highest point of the head can not be measured). If you measure at home, you can put a piece of paper on the wall, and draw a line on the paper after each measurement. It is not necessary to measure the specific height of each measurement, but only to distinguish the difference with the previous month. When measuring at home must pay attention to the ruler and the floor level, you need to use a right angle triangle ruler, or use a hard book, one side against the wall, the other side and the floor level. Parents often respond, “My child grew 1.5-2 centimeters last month, but why did he not grow at all this month?” The main reason is that it is still related to the measurement error. After a longer treatment time, the growth rate of several months can be averaged (Note: usually do not measure height too often, generally once a month, otherwise it is easy to cause psychological pressure to the child, too much psychological pressure is not conducive to growth).

(2) Review time and items after growth hormone treatment (a) For children treated with growth hormone, after 3 months of treatment, thyroxine (mainly FT3, FT4 and TSH) and fasting blood sugar need to be reviewed. Since most of the treated patients are dwarf patients, the growth rate was too slow in the past and the thyroxine requirement was low. After the growth accelerated significantly, the thyroxine requirement increased and a few of them may cause a relative deficiency of thyroxine. Thyroxine deficiency requires oral levothyroxine tablets for a short period of time, otherwise the efficacy of growth hormone will be compromised. There are also a few people who have slightly high thyroxine due to over-regulation of thyroxine. At this time, as long as TSH is not low, there is generally no need to deal with it, and continued use of growth hormone will often automatically drop to normal.

(b) When you have a review in about six months, in addition to thyroxine and blood glucose, you should also check your bone age and insulin-like growth factor-1 (IGF-1) and insulin-like growth factor binding protein-3 (IGFBP3). The test results may vary greatly from hospital to hospital, and there are some hospitals whose reagents are not necessarily reliable. if the initial diagnosis is made in our hospital, it is recommended to continue to review the test in our hospital. the review of IGF-1 and IGFBP3 is an important indicator of both the safety of medication and the adjustment of growth factor dose. The latest foreign data show that the treatment effect is significantly better than the traditional fixed dose if the dose of growth hormone is adjusted according to the IGF result at the right time.

(C) review at 1 year of treatment, it is best to also review liver and kidney function, which can be done together with blood sugar.

Adjustment of growth hormone dose is generally not needed within a short period of time, and weight change of not more than 10% is generally not needed. Adjustment is not only based on growth rate or weight, but also needs to consider different etiologies, results of growth hormone stimulation test before initial use, pubertal development, bone age, especially IGF-1 and IGFBP -3 review results, etc. That’s why it’s so important to have regular reviews! The applicable dose of growth hormone therapy has a wide range. For people with small bone age and sufficient treatment time, we generally start with a smaller dose within the normal range, if the small dose has a better effect, the cost is relatively low and side effects are less likely to occur, if the effect is not satisfactory, then consider adjusting the dose. For children with large bone age and limited treatment time, generally start with a medium or large normal dose, because time is limited and slow observation may affect the final treatment effect.

Growth hormone discontinuation indicators For those who start treatment at a young age, they can temporarily stop for a period of time when their height reaches or slightly exceeds the height of their peers (usually requiring treatment for 2~3 years or longer), and then use it again when their height is significantly lower than that of their peers. For those who are older in bone age, due to the limited treatment time, the indicator for stopping the drug is to stop when the monthly growth rate is less than 4mm for three consecutive months, although the treatment at this time may still improve 1 to 2mm per month, but the available time is already very little, there is no substantial significance. However, for those whose height has approached normal adult height during the treatment period, discontinuation of the drug can also be considered, but a detailed assessment is needed before discontinuing the drug to see if the treatment objective can be achieved.