Foreword
More than 70 experts from more than 50 hospitals throughout the country were organized by the Endocrine Genetic Metabolism Unit of the Chinese Medical Clinical Science Branch, the China Medical Doctors ‘ Association ‘ s Professional Committee on Adolescent Health and Medicine, the Fukuko Centre for the Medical Development of Children and the Editorial Committee of the Chinese Journal of Practical Paediatrics. The consensus of Chinese experts on the diagnosis and treatment of child-specific shorts, based on the methods and steps developed by the consensus of clinical experts, was published in recent days. The publication of this consensus has broken with the current state of the country, where there is no ISIS consensus or guidance to provide a reference for clinical precision.
ISS definition
Specially short-body (ISS) is the general description of a group of low-pregnancy diseases with high heterogeneity, with a height below the age, sex and ethnic mean height of 2 standard deviations (standard deviation, SD) or below the third percentile (P3,-1.88 SD) and a normal birth-length, weight and body mass ratio, with no evidence of whole-body, endocrine, nutrition, chromosomal abnormality or genetic mutation. ISS includes family shortness, FSS and physical growth delays (constituency of growth and development, CDGP)
ISS Diagnosis
The complexity of the causes of the child ‘ s dysentery, the identification of the causes and the mechanisms of morbidity and morbidity remain a challenge, and the recommendations of the experts in the ISS consensus can be consulted for clinical diagnosis, as shown in the table below.
The results of the expert recommendation on diagnostic criteria for special shorts
Diagnostic criteria
Outcome (%)
Not all patients need to be tested for growth hormone stimulation.
++ 70.27
Growth hormone-enabled experiment growth hormone peak tangent value 7ug/L
++ 56.76
If necessary, the chromosomal microarray will be tested for non-recognised dwarf disorders, which are directly sequenced in the occult genre.
+++89.19
Short people with no significant abnormal face or symmetry, with a recommended shortness of Ht SDS<-3.0
++ 60.00
Note: Outcome 26% ~ 50% is +; 50% ~ 75% is ++; > 75% is ++; Ht SDS is the height standard deviation; 2.5% of experts support Ht SDS<-2.5; 7.5% of experts support Ht SDS<-2.0
It is noteworthy that the new consensus states that young children with any of the following characteristics are subject to a pathological cause assessment: Ht SDS 1.0 SD/year) or growth rate of <7.0 cm/year for children aged 2 < 4 < < 5.5 cm/year, < 5.0 cm/year for children aged 4 , < 4.0 cm/year for children aged 6 to pre-adolescent year and < 6.0 cm/year for adolescent children.
Clinical assessment
Clinical assessment (systemological history and detection), auxiliary examination (conventional, endocrine hormone and image) and genetic testing assessment are equally important and should be assessed in a comprehensive manner. The specific clinical assessment process is shown in figure 1.
Auxiliary examination
Regular blood tests should be carried out for unidentifiedly short children, as shown in the table below.
Auxiliary examination to be completed prior to the diagnosis of a specific short body.
Inspection
Specific items
General laboratory examinations
Blood, urine, liver, kidney, myocardial enzyme, blood electrolyte and calcium; blood resin, blood sugar; blood and gas analysis, if necessary, etc.
Endocrine assessment
_
Basic indicators
Thyroid function; insulin sample growth factors combined with protein; adrenal cortex hormones, cortex alcohol; empty insulin; sex hormones, including yellows (age children), egg-bulb estrogen, edict, testosterone, oxytocin, etc.
Selective indicator (conditional)
Growth hormones combined with proteins, acidic unstable sub-kiss.
Motivating Test
Growth hormone stimulation test (if necessary)
Video screening
Bone age, skeletal malformation, cardiac color, ovarian B super (girls), lower ovary-diagnostic MRI (if necessary)
Genetics testing
See section 3.3 for details
Genetic testing and assessment processes
Chromosomal abnormalities, intrabone cartilage abnormalities of the osteoporosis plate, etc. all contribute to smallness. The consensus recommended the following process for the assessment of ISIS genetic tests, as shown in figure 2.
ISS treatment
hGH treatment
The experts recommended treatment standards for special shorts: the Ht SDS standard for the recommended use of hGH treatment for infants; the starting age for the use of hGH treatment for infants is 3-4 years; and the procedure for the use of hGH treatment for infants is at least 2 years or more.
For the first time, in 2021, the National Drug Control Authority approved the treatment of ISIS for human growth hormones (Ansumium). See table below.
hGH therapeutic pointer, start time, recommended dose and course
Treatment pointers
Treatment time
Dose recommendation*
Treatment
Matching ISIS diagnosis, Ht SDS<-2.0
3-4 years
Initial dose per day of 45 μg/(kg d) or 0.15 U/(kg d)
Recommended treatment for at least 2 years
* The recommended dose of hGH is higher than the dose used for GHD, based on the possible impairment of the growth hormone sensitivity of children with ISIS.
It is not recommended to reduce the hGH dose to the normal IGF-1 level at the high IGF-1 (>2SD) level, and the dose should be adjusted in conjunction with the individualized growth response indicators of the affected child. Because the IGF-1 level of ISS is usually +1 SDS or higher, in some cases IGF-1 levels above +2 SDS may be required to achieve an effective growth response.
Protogeny hormones
GnRHA and hGH are not recommended for regular joint treatment of ISS children.
Other treatments
The aromatic enzyme inhibitor (aromatase inhibitor, AI) is a combination of hGH, reorganizing insulin sample growth factors-1 (rhIGF-1) and hGH, herbal medicine, growth promotion therapy, etc., have not yet been sufficiently studied to demonstrate their effectiveness and the statement is not certified and is not currently recommended. The short-term use of testosterone has been reported to have had some effect, but for individualization options it requires careful use.
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