Review of patients with dwarfism and precocious puberty during treatment and related considerations

1. Height measurement: Whether it is dwarfism or precocious puberty, it is necessary to measure height every month after treatment. Accurate height measurement needs to pay attention to the “four same”: ① the same time, because after standing or sitting up, the intervertebral space of the human spine will be compressed, the morning and evening height will be different, the same time measurement is comparable; ② the same measuring tape; ③ the same measurer, different people, the ruler card in the head of the tightness will be different; ④ children 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 the height too often, generally once a month, otherwise it is easy to cause psychological pressure on the child, too much psychological pressure, not conducive to growth).

2, growth hormone treatment review time and items: for the application of growth hormone treatment, treatment 3 ~ 6 months after the need to review thyroxine (mainly check FT3, FT4 and TSH) and blood sugar. 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 over-regulation of thyroxine, in which case, as long as TSH is not too low, there is generally no need to deal with it, and continued use of growth hormone will often drop to normal automatically). The more the growth is significantly faster than in the past, the earlier the review is needed, preferably in 3 months. For those with precocious puberty who apply growth hormone in combination, since the growth rate is not significantly faster than in the past, a review in about 6 months can be considered. The review of IGF and insulin-like growth factor-1 (IGF-1) and insulin-like growth factor binding protein-3 (IGFBP3) is recommended for those who are initially treated in our hospital. It is also an important indicator of growth hormone dose adjustment. The latest information from abroad shows 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. After the review, generally once every six months.

3, growth hormone dose adjustment: growth hormone in a short period of time generally do not need to be adjusted, weight change of not more than 10% generally do not need to be adjusted, even if the adjustment, not only according to the growth rate or weight. Also need to consider different etiology, the results of the growth hormone stimulation test before the initial use of the drug, youth development, bone age size, especially the IGF-1 and IGFBP-3 review results. Regular review is also necessary! Although the effect of treatment is important, the safety of medication is more important! The dose of growth factor 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 the side effects are less likely to occur. For children with large bone age and limited treatment time, generally start with a medium or large normal dose, because of the limited time, slowly observed may affect the final treatment effect.

4, growth hormone discontinuation indicators: for those who start treatment at a younger age, 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), to be used again when the height is significantly lower than that of the same age. For those who are older, due to the limited treatment time, the indicator of discontinuation is to stop using the drug when the monthly growth rate is less than 4mm for three consecutive months (although the application may still improve 1 or 2mm per month at this time, but the available time is already very little and has no substantial significance). However, for those whose height has been close to the normal adult height during the use of the drug, can also consider discontinuing the drug, but before discontinuing the drug needs to be assessed in detail whether to achieve the purpose of treatment.

5, GnRHa treatment review program and dose adjustment: because the dose of GnRHa treatment in patients with precocious puberty is a difficult point, it needs to be individualized, that is, each person, the dosage may be different in different periods. In particular, the first six months to one year of treatment need to be reviewed from time to time. One month, three months (in order to reduce the patient’s pain and financial burden, it is now often simplified to review only at about 2 months) and six months after treatment, we should review height growth, sexual development control, ultrasound (girls, boys only need to measure testicular size), sex hormone levels (generally six sex hormones, including at least LH, FSH and E2), and GnRH stimulation test if necessary The dose should be adjusted at the right time. If the dose is too high, the growth will be too slow and will not help much to improve the adult height; if the dose is too low, it will be difficult to control during puberty and will not achieve the treatment purpose. 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. GnRHa dose adjustment is mainly in the first six months, after six months, if there is no special situation changes, generally is once every six months, review items with six months when the review.

6, 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 is slightly higher than the target height (generally to be 2 ~ 3cm higher) when you can consider discontinuing the drug. Because there is still 2~3 months of slower bone age growth time after discontinuation, but there is often 2~4 months of faster bone age growth period after that, the later rapid bone age growth may lower the predicted height. Or in girls bone age greater than 13.5 years, boys bone age greater than 14.5 years when the drug is discontinued, at this time, even if the combined use of growth hormone, can not play a role in improving lifelong height, but growth hormone can also be considered to continue with.

7, for pseudo or partial sexual precocious oral Chinese medicine, generally is every 3 months to review ultrasound, sex hormones (general check sex hormone six, at least need to include LH, FSH and E2), if necessary, review GnRH stimulation test. Bone age is reviewed every six months.

Since growth hormone is a hormone normally secreted by the body every day, rhGH is exactly the same as the body’s own growth hormone secretion, there is no need to correlate any abnormalities that occur with rhGH during the medication period. 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 corresponding side effects of sex hormone reduction will not occur after suppression. 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.

8. Special Note: After 2 months of GnRHa application, if you want to get guidance on medication through this website after local review of ultrasound and sex hormones, you must write down detailed medical history, bone age, predicted height and other examination results before medication, medication mode, name (it is possible for those who apply Inhibiton to only extend the injection time without reducing the dosage) and dosage, whether the first dose has been enhanced, and the height, weight and sexual development control before and after treatment. Weight change, sexual development control (such as breast and testicular size change, girl’s secretion, menstruation of those who already have menarche, etc.), etc., and post-treatment examination for comparison. Since the consultation information is not usually at hand when answering inquiries (online dose adjustment is limited to 2 months after GnRHa injection, and it is difficult to make appropriate adjustments via the Internet), we will not be able to provide you with the information you need.