Objective To investigate the diagnostic value of growth hormone(GH) stimulating test in dwarfism of children and related influencing factors .Methods Arginine and L‐dopa were applied to perform GH stimulating test in 642 children with dwarf‐ism .GH levels were detected at 30 ,60 ,90 ,120 ,150 ,180 min after stimulation .All children were divided into GH deficiency (GHD) group ,with peak value of GH less than 10 ng/mL ,and non‐GHD group ,with peak value of GH at least 10 ng/mL .Physical and la‐boratory data of the two groups were compared .Results The proportion of children with GHD and without GHD were 68 .69%and 31 .31% ,respectively ,and with statistical difference(χ2 =6 .19 ,P<0 .05) .The GH peak value ,with a delay ,mainly appeared at 30 and 150 min after stimulation ,accounting for 22 .42% and 34 .27% .Levels of age ,body height standard deviation ,body mass in‐dex ,alanine aminotransferase ,aspartate aminotransferase ,insulin growth factor‐1 and 25‐hydroxyl vitamin D were statistically dif‐ferent between the two groups(P<0 .05) .Conclusion Arginine and L‐dopa combined stimulating test could be used for the diagno‐sis of GHD in children with dwarfism .GH level might be correlated with various physical and laboratory parameters ,which should be considered for the diagnosis of GHD .

Objectives To investigate the causes of short stature in Turner syndrome (TS). Methods 86 patients were di-agnosed with TS by karyotypes from 2004 to 2013. According to the deletion types of the X chromosome short arm, growth hor-mone (GH), insulin-like growth factor-1 (IGF-1) and thyroid function, the TS patients were divided into different groups and com-parison was made among groups. Results Ht SDS in three groups with different extent of the deletion of the X chromosome short arm were (-4.39±1.08), (-3.26±1.25) and (-2.84±0.15) (P<0.05). The proportion of growth hormone deifciency (GHD) in the three groups were 62.5%, 38.9%and 0%(P<0.05). Ht SDS in groups with different degree of IGF-1 level were (-4.37±1.10), (-3.82±1.07) and (-3.25±0.91) (P<0.05). There was no signiifcant difference of Ht SDS between hypothyroidism patients with and without GHD (P>0.05). Conclusions The deletion of X chromosome short arm may cause the short stature in TS. The GH-IGF-1 axis in TS is impaired, but GHD is not related to short stature in TS.

Objective To analyze the clinical characteristic s of an infant with congenital lipoid adrenal hyperplasia (CLAH),and to sequence the acute regulatory protein (steroid acute regulatory,StAR) gene of the infant patient and her pedigree.Methods Physical examination,laboratory tests,and imaging examination of the 11-month-old patient with CLAH were collected.DNA was extracted from blood samples of the patient and her parents.The 7 exons of StAR gene were amplified by PCR and then sequenced.Results Dark skin,girl vulva,and one each 1.0 cm×1.0 cm palpable lump in inguinal area bilaterally were observed.The adrenocorticotropic hormone (ACTH) was 253 pg/ml,cortisol was 27.6 nmol/L at 8 am,17-hydroxyprogesterone was 3 nmol/L.Uhrasound showed that sign of testicular ultrasonography existed in bilateral inguinal regions.Karyotype analysis showed 46,XY.Sequencing of PCR amplified fragments showed that there were two heterozygous mutations c.229C ＞ T,p (Gln77X) and C.659A＞G,p (His220Arg) of StAR gene in this patient.By rectifying the disturbance of electrolyte,and treating with hydrocortisone and 9α fludrocortisone,etc,the patient has been stable so far.Conclusion The patient presents typical clinical manifestations.Two heterozygous mutations including c.229C ＞T,p (Gln77X) from maternal and C.659A＞G,p (His220Arg) from paternal of StAR gene were detected.Wherein the c.659A＞G,p.(His220Arg) as a novel point mutation of StAR gene,has not been reported so far.

Aim To explore the protective effects of lithium chloride ( LiCl ) on neurous injuries and phos-phorylation of tau protein at serine262 induced by okada-ic acid( OA) . Methods The neuroblastoma SK-N-SH cells were differentiated by all-trans-retinoic acid ( AT-RA) . The differentiated SK-N-SH cells were treated with OA to establish the Alzheimer′s disease cellular model. SK-N-SH cells′ viability and proliferation were measured by SRB test. Giemsa staining was used to observe cell morphology. The neurite length of SK-N-SH cells was measured by Image-Proplus software. Syn-aptophysin and phosphorylated tau protein at serine262 expression levels were tested by Western blot. Results The SK-N-SH cells which were treated with 10 μmol ·L-1 ATRA for 7 days displayed mature neuronal fea-tures. The synaptic length of SK-N-SH cells became longer. And the levels of serine262 phospho-tau was sig-nificantly elevated. 20~100 nmol·L-1 OA effectively inhibited the viability of differentiated SK-N-SH cells in a concentration-dependent manner and in a time-de-pendent manner. The OA treatment induced obvious synaptic atrophy in differentiated SK-N-SH cells. And the phosphorylation level of tau protein serine262 also greatly increased. The pretreatment with 10 mmol · L-1 LiCl significantly ameliorated the synaptic atrophy, the decrease of synaptophysin expression and the in-crease of tau phosphorylation at serine262 induced by OA in differentiated SK-N-SH cells. Conclusion LiCl could effectively inhibit OA-induced synaptic atro-phy in differentiated SK-N-SH cells, and it could also result in the increase of synaptophysin expression and the decrease of the phosphorylation of tau protein at serine262 .

OBJECTIVE:To change traditional working mode of drug storehouse by applying intelligent system,improve drug storage management,improve work efficiency and quality of drug storehouse,and to provide reference for hospital drug storehouse information management. METHODS:The changes of drug storehouse working mode and management in our hospital were compared before and after the application of intelligent drug storehouse management system. The time taken to complete individual work and the times of error occurrence 12 weeks before and after the application of the system were adopted as indexes to evaluate the effects of the system. RESULTS:Through the combination of Internet+Internet of things bar code,radio frequency identification,electronic tags,etc.,intelligent drug storehouse management system,which included temperature and humidity management,drug storehouse management,supply chain management three modules,was established in our hospital. Its application improved the drug storehouse working mode and process,strengthened the drug storehouse management in respect of staff,supplier,cold chain drugs and special drugs. Before application,average weekly time-consuming of drug shelfing,bill warehousing,outgoing and deliverying and inventory were(11.92 ± 0.701)h,(13.96 ± 0.752)h,(14.96 ± 0.542)h,(4.58 ± 0.376)h;the times of errors within 12 weeks were 10,8,9,2,respectively. After application of the system,average weekly time-consuming were(9.83±0.718)h,(11.29±0.753)h,(9.91±0.557)h,(3.00±0.316)h,respectively(vs. before application, all P<0.05). The times of errors within 12 weeks were 1,1,2,0. CONCLUSIONS:The application of intelligent drug storehouse management system optimizes workflow of drug storehouse in our hospital,strengthens the management of drug storehouse,and improves working efficiency and quality of the drug storehouse.

Objective:To investigate the diagnostic value and cutoff value of the basal luteinizing hormone (LH) level for central precocious puberty (CPP) in girls with different body mass index (BMI) values.Methods:A total of 1 087 girls aged 6 to 8 years old with breast development and visiting the Department of Endocrine Genetics and Metabolism, Fuzhou Children′s Hospital of Fujian Medical University from January 2014 to December 2018 were enrolled.The girls had the breast development of Tanner stage B2 or B3.They were divided into groups of normal weight (840 cases), overweight (133 cases) and obesity (114 cases) according to BMI percentiles corresponding to their age and sex.All individuals underwent gonadotropin-releasing hormone (GnRH) agonist test.Statistical analysis was performed using SPSS 18.0 software, and the receiver operating characteristic (ROC) curve was drawn.Results:In the normal weight group, 393 girls were positive and 447 girls were negative for GnRH test.In the overweight group, 72 girls were positive and 61 girls were negative for GnRH test.In the obesity group, 40 girls were positive and 74 girls were negative for GnRH test.The areas under the ROC curve (AUC) of the basal LH level for the diagnosis of CPP in the normal weight, overweight, and obese girls were 0.826, 0.774, and 0.801, respectively.When the Youden index was maximum, the cutoff values of the basal LH level were 0.245 IU/L, 0.225 IU/L and 0.145 IU/L, respectively; the sensitivities were 63.9%, 65.3%, and 80.0%, respectively; the specificities were 87.9%, 82.0%, and 71.6%, respectively.When the cutoff value of the basal LH level was 0.455 IU/L in the normal weight group, 0.480 IU/L in the overweight group, and 0.335 IU/L in the obesity group, the specificities of the 3 groups were all above 96.6%.Conclusions:It is reliable to use the basal LH level for CPP diagnosis for girls with different BMI values.The cutoff value of the basal LH level for CPP diagnosis in obese girls is the lowest.

Objective: To explore the association among clinical features, electromyography (EMG) and magnetic resonance neurography (MRN) in patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Methods: A cross-sectional survey was conducted to enroll consecutively typical CIDP patients in Renmin Hospital of Wuhan University from May 2017 to May 2019. The Hughes Disability Scale (HDS) was used to evaluate the illness severity of the patients. The electrodiagnostic parameters including motor conduction velocity (MCV), compound muscle action potential (CMAP), F-wave latency, sensory nerve conduction velocity (SCV) and sensory nerve action potential (SNAP) of upper and lower limbs were analyzed. The patients whose response waveform can be elicited in all nerves were defined as group A, and those without response in one or more nerves as group B. MRN quantitative technique was used to calculate the cross-sectional area of nerves roots (nr-CSA) of brachial plexus and lumbosacral plexus. The linear regression method was used to analyze the correlation among clinical features, EMG and nr-CSA. Results: A total of 32 patients with typical CIDP met the criteria, 75% (24/32) of whom were males. There were 16 patients in the mild group (group A) and 16 in the severe one (group B). The abnormal rate of F-wave latency was the highest. Cerebrospinal fluid (CSF) protein, HDS score were correlated significantly with the nr-CSA of brachial plexus and lumbosacral plexus in the two groups (group A: CSF protein and brachial plexus nr-CSA: r=0.498, P=0.004; CSF protein and lumbosacral plexus nr-CSA: r=0.479, P=0.007; HDS score and brachial plexus nr-CSA: r=0.650, P=0.000; HDS score and lumbosacral plexus nr-CSA: r=0.625, P=0.000. group B: CSF protein and brachial plexus nr-CSA: r=0.497, P=0.049; CSF protein and lumbosacral plexus nr-CSA: r=0.503, P=0.047; HDS score and brachial plexus nr-CSA: r=0.605, P=0.001; HDS score and lumbosacral plexus nr-CSA: r=0.648, P=0.000). MCV of median nerve and ulnar nerve was negatively correlated with nr-CSA of brachial plexus in the two groups (group A: MCV of median nerve and nr-CSA of brachial plexus: r=-0.309, P=0.019; MCV of ulnar nerve and nr-CSA of brachial plexus: r=-0.286, P=0.027. group B: MCV of median nerve and nr-CSA of brachial plexus: r=-0.660, P=0.000; MCV of ulnar nerve and nr-CSA of brachial plexus: r=-0.581, P=0.001). The F-wave latencies of median and ulnar nerves were positively correlated with nr-CSA of brachial plexus, and the CMAP amplitude of tibial nerve and SNAP amplitude of sural nerve were positively correlated with nr-CSA of lumbosacral plexus in group B. Conclusions Male patients with CIDP are predominant. The higher the nr-CSA in brachial plexus and lumbosacral plexus, the higher the CSF protein and disability score, and the larger the nr-CSA in brachial plexus, the slower the MCV in the median and ulnar nerve. For group B patients with more severe nerve injury, the larger nr-CSA of brachial plexus was, the longer F-wave latency of median and ulnar nerve was, and the larger nr-CSA of lumbosacral plexus was, the lower CMAP amplitude of tibial nerve and SNAP amplitude of sural nerve were. As a non-invasive test, MRN can be used to assist in the diagnosis of CIDP and to assess the severity of the disease.