I. Clinical pathway standard inpatient procedures for dwarfism
(A) Applicable objects.
The first diagnosis of dwarfism (formerly known as dwarfism) (ICD-10UE34.3).
(B) Diagnostic basis.
According to Pediatrics (edited by Wang Weiping, Higher Education Press, 2004 edition), Guidelines for the diagnosis and treatment of children with short stature from the Endocrine Genetic Metabolism Group of the Pediatrics Branch of the Chinese Medical Association (Chinese Journal of Pediatrics, 2008, 46:428-430), Pediatric Endocinology (edited by Mark A. Sperling. Saunders Elsevier Press, 2007), Zhu Futang Practical Pediatrics (7th edition) (People’s Health Press), Pediatric Endocrinology (edited by Yan Chun and Wang Mu Ti, People’s Health Press, 2006). Yun Lu, Department of Child Health, Lianyungang Maternal and Child Health Hospital
Those whose height is below the 3rd percentile of the growth curve of normal healthy children of the same race, age and sex, or below two standard deviations (height standards refer to the standard deviation of height and weight of children aged 2-18 years in China developed by the 2005 study on the data of child physical development survey in nine provinces/municipalities).
(C) Choice of treatment plan.
According to Pediatrics (edited by Wang Weiping, Higher Education Press, 2004 edition), Guidelines for the diagnosis and treatment of children with short stature from the Endocrine Genetic Metabolism Group of the Pediatrics Branch of the Chinese Medical Association (Chinese Journal of Pediatrics, 2008, 46:428-430), Pediatric Endocinology (edited by Mark A. Sperling. Saunders Elsevier Press, 2007), etc.
1, Isolated growth hormone deficiency drug treatment: growth hormone replacement therapy.
2.Hypothyroidism: thyroxine replacement therapy.
3, congenital ovarian hypoplasia: general growth hormone replacement therapy before the age of 12 years at bone age, combined or separate estrogen and progestin therapy after the age of 12 years.
4.United pituitary hormone deficiency: corresponding lack of hormone replacement therapy.
5.Other: cause-specific and symptomatic treatment.
6.Adjunctive treatment: exercise and nutrition therapy.
(D) Standard hospitalization days ≤ 3 days.
(V) Entry pathway criteria.
1, The first diagnosis must meet the ICD-10UE34.3 disease code for dwarfism (formerly known as dwarfism).
2. There is no clear cause of dwarfism.
3.Meet the criteria for hospitalization: meet the diagnostic criteria for dwarfism and be judged by an endocrinologist or pediatric clinician to require hospitalization for examination and treatment.
4.When the patient has other disease diagnosis at the same time, such as during hospitalization without special treatment nor affect the implementation of the clinical pathway process of the first diagnosis, it can enter the pathway.
(F) Examination items during hospitalization.
1. Required examination items.
(1)Routine blood, routine urine, routine stool;
(2) Thyroid function (T3, T4, TSH, FT3, FT4), hepatitis B two to one half;
(3) Liver and kidney function, blood lipids, electrolytes, blood glucose;
(4) Bone age, pituitary MRI (reinforcement is required if tumor is suspected);
(5) Growth hormone stimulation test (including arginine stimulation test, insulin stimulation test, colistin stimulation test, levodopa, two of which must be selected, the first two must be selected).
2, according to the patient’s condition can choose the examination items.
(1) Cortisol, adrenal hormone releasing hormone, insulin-like growth factor 1 (IGF1), insulin-like growth factor binding protein 3 (IGFBP3);
(2) Bone mineral density;
(3) 25hydroxyvitamin D3;
(4) X-ray of the head, chest, spine, pelvis, and long bones of the extremities;
(5) Blood gas analysis;
(6) Sex hormones: luteinizing hormone, follicle stimulating hormone, estradiol, testosterone, prolactin, chorionic gonadotropin;
(7) Gonarelin excitation test;
(8) Chorionic gonadotropin test;
(9) Karyotype analysis;
(10) IGF-1 production test.
(vii) Treatment plan and drug selection.
1.Growth hormone treatment is given to those diagnosed with growth hormone deficiency: growth hormone powder or water, the commonly used dose in China is 0.1C0.15U/kg/d, subcutaneous injection at bedtime.
2. Symptomatic treatment drugs: choose according to the patient’s condition.
(1) Thyroid hormone replacement therapy is given to those diagnosed with hypothyroidism: Generally, eugenol is used, the dose varies according to the degree of deficiency, starting from small doses, and oral administration is required in the morning on an empty stomach, and the hormone level is reviewed and the dose is adjusted 2-4 weeks after starting the medication.
(2) Diagnosis of hypoadrenocorticism: use hydrocortisone treatment.
(3) Others: treated according to the corresponding examination results.
(H) Discharge criteria.
1.Patients complete relevant examinations as well as etiological assessment.
2.The process of growth hormone stimulation test was smooth and there were no adverse reactions.
3.No complications and/or comorbidities that require hospitalization.
(ix) Variation and cause analysis.
The examination reveals the presence of more serious medical systemic diseases such as renal insufficiency, congenital heart disease, etc. Active symptomatic management is required to improve relevant examinations, explain and inform the family of the condition, the reasons leading to prolonged hospitalization and increased hospitalization costs, etc., and withdraw from this pathway according to the corresponding pathway or guidelines for rescue treatment.