Dwarfism, precocious puberty patients after treatment review notes

  Height measurement: It is necessary to measure height every month after treatment for both short and precocious patients, and since the change in height within one month is small, it is necessary to measure as accurately as possible. Accurate height measurement needs to pay attention to “four similarities”: ① the same time, because after standing or sitting up, the vertebral space of the human spine will be compressed, the height of the morning and evening will be different, the same time measurement is comparable; ② the same measuring tape; ③ the same measurer to measure the person, different people, the ruler card in the head of the tightness will be different; 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 by 2cm last month, but he hasn’t grown 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 can be averaged over several months (Note: usually do not measure height too often, in general, height and weight can be measured once a month (and it is recommended to keep good records), otherwise it is easy to cause psychological pressure to the child, too much psychological pressure is not conducive to growth). Since the drug dose is generally calculated according to body weight, if the weight change exceeds 10%, it may be necessary to adjust the drug dose (during the six-month review period, the body weight should be controlled appropriately, and the change should not exceed 10% as much as possible).  Growth hormone treatment review time and items: For those who apply growth hormone treatment, after 3 months of treatment, thyroid hormone (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 was accelerated significantly, the thyroxine requirement increased and a few people may cause a relative deficiency of thyroxine. Insufficient thyroxine requires oral levothyroxine tablets for a short period of time, otherwise the efficacy of growth hormone will be affected (there are also a few cases of slightly high thyroxine due to excessive thyroxine regulation, at this time, as long as TSH is not too low, there is also generally no need to deal with it, and continued use of growth hormone will often automatically drop to normal). In addition to thyroxine and blood glucose, bone age and insulin-like growth factor-1 (IGF-1) and insulin-like growth factor binding protein-3 (IGFBP3) should be checked when the IGF is rechecked in order to understand the situation after the medication is administered. It is also an important indicator of growth factor dose adjustment. The latest information from abroad shows that if the dose of growth hormone is adjusted according to the IGF results, the therapeutic effect is significantly better than the traditional fixed dose. Subsequent review, generally once every six months, and at 1 year of treatment, it is best to also review the liver and kidney function (can be done together with blood sugar).  Growth hormone stimulation test, as long as it is done correctly and relatively standardized, regardless of treatment or not, is in principle not necessary to be done again, except for very rare and special cases. Therefore, it is not necessary to do the growth hormone provocation test again when reviewing after treatment.  Growth hormone dose adjustment: Growth hormone generally does not need to be adjusted within a short period of time, and weight changes not exceeding 10% generally do not need to be adjusted, and even if adjusted, it is not only based on growth rate or weight. It is also necessary to consider different etiologies, the results of the growth hormone provocation test before the initial dose, the development of youth, the size of the bone age, and especially the results of the IGF-1 and IGFBP-3 review. Regular review is also necessary! Although the effect of treatment is important, the safety of medication is more important! The dose of growth hormone has a wide range. For those 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 older bones and limited treatment time, generally start with a medium or large dose of the normal range, because of the limited time and slow observation may affect the final treatment effect.  Growth hormone discontinuation index: For those who start treatment at a younger age, the treatment can be temporarily stopped for a period of time when the height reaches or slightly exceeds the height of the same age (usually takes 2~3 years or longer), and then used again when the height is significantly lower than that of the same age. For people with older 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 application may still improve 1-2mm per month at this time, but the available time is already very little, there is no substantial meaning). However, for those whose height has approached normal adult height during the period of use, discontinuation of the drug may also be considered, but a detailed assessment of whether the therapeutic goals have been achieved is needed before discontinuation.  GnRHa treatment review program and dosage adjustment: Since the dosage of GnRHa treatment in patients with precocious puberty is a difficult issue, it needs to be individualized, i.e., the dosage may be different for each person, at different times. In particular, the first six months to one year of treatment need to be reviewed from time to time. The height growth, control of sexual development, ultrasound (girls, boys only need to measure testicular size), sex hormone levels [generally sex hormone six, which need to include at least LH, FSH and E2 (female) or T (male)] should be reviewed 2 months and 6 months after treatment, and the GnRH stimulation test should be reviewed every 6 months, so that the dose can be adjusted when appropriate. Too high a dose will result in too slow growth and will not help much in improving adult height, too low a dose will make it difficult to control during puberty and will not achieve the therapeutic goal. According to the guidelines of precocious puberty, it is better to evaluate the bone age in detail, because it is difficult to identify the growth of bone age without detailed evaluation because of the short interval, which is not conducive to dose adjustment. The dose adjustment of GnRHa is mainly in the first six months, and after six months, if there are no special changes, it is usually once every six months, and the review items are the same as the review at six months.  At present, in GnRHa treatment for more than two months when the review, often used to check the injection of GnRHa about 1 hour after the method of sex hormones, so equivalent to a simple stimulation test (the first dose has been enhanced, injection of the third injection is often less than 1.5 months, generally do not check the injection after an hour value), unless the treatment is not smooth, when the review, generally rarely do the standardized GnRH stimulation test again.  GnRHa discontinuation indicators: application is generally not less than two years, in the bone age and age, and reached or close to the age of normal sexual development, the predicted height slightly higher than the target height when (generally to be 2 ~ 3cm higher) can be considered to discontinue the drug. For girls, discontinuation is generally considered when the bone age is not too old, the age is greater than 10 years old, and the height is around 150 CM. Because after stopping the drug, there is still 2~3 months of slower bone growth time, but some people may have a faster bone growth period in the following 2~4 months, and the later rapid bone growth may lower the predicted height. Alternatively, the drug may be discontinued when the bone age is greater than 13.5 years for girls and 14.5 years for boys, at which point even combined use of growth hormone may not be effective in increasing lifetime height, but growth hormone may be considered for continued use.  For pseudo or partial precocious puberty oral herbal medicine, generally is every 3 months to review ultrasound, sex hormones (generally check sex hormone six, at least need to include LH, FSH and E2), if necessary, review GnRH stimulation test. Bone age should be checked every 6 months.  Precautions after injection of recombinant human growth hormone: Since growth hormone is a hormone normally secreted by the human body every day, rhGH is exactly the same as the growth hormone secreted by the human body itself, there is no need to correlate any abnormalities that occur with rhGH during the drug administration. Growth hormone also will not promote sexual development or promote bone age growth as some people think (although growth hormone deficient people may originally have small bone age, after applying growth hormone to make it normal, there is the possibility of transition to normal bone age), otherwise it will not be used to improve lifetime height. If you are more ill, consider stopping the use for a few days as your own growth will be affected. If you have special circumstances, it is not much of a problem to stop using it for a few days. Since normal people also secrete growth hormone every day, the application of general medications or preventive vaccinations, etc., during the use of the drug is not affected.  GnRHa is an amino acid replacement of normal human gonadotropin-releasing hormone (GnRH, a 10-peptide), making it bind more strongly to the gonadotropin-releasing hormone receptor on the pituitary gland without acting as a gonadotropin-releasing hormone to inhibit pubertal growth. Overall safety is good. Some side effects on the instruction are mainly for adults, because the adult sex hormone level is high, and the sudden suppression will cause a series of side effects such as osteoporosis and other sex hormone decline, while in precocious children, the sex hormone level should be very low, and the sex hormone decline is more in line with the normal physiological process, and the corresponding side effects of sex hormone reduction will not occur in general. During the period of medication, the application of general drugs or vaccination is not affected in case of illness, and the same applies to oral Chinese medicine in case of pseudoprecocious puberty.