Familial glucocorticoid deficiency (FGD) known as one of primary congenital adrenal hypoplasia diseases,is a rare autosomal recessive disorder.FGD is characterised by isolated glucocorticoid deficiency,therefore the patients exhibit low serum cortisol and high plasma adrenocorticotropic hormone levels.The patients typically present with hypoglycemia,recurrent infections,hyperpigrnentation and tall stature.Much research on the pathogenic genes and molecular biology mechanisms have been performed,and presently some pathogenic genes have been discovered.In order to enhance the clinicians'understanding of familial glucocorticoid deficiency,this review focuses on the pathogenic genes,pathogenesis,diagnosis and treatments of the disease.

Objective:To analyze quantitatively individual baseline drift of immunoglobulin and complement within 24 h.Methods:Blood samples were drawn from adult volunteers on day 1 and day 2 at 7.00 am.Immunoglobulin and complement including IgG,IgA,IgM and C3,C4 were measured with automatic immunobiochemistry analyzer.The rate of changes between two times of measured value,baseline drift,could be calculated.Results:The individual baseline drifts of measured values were from 6.12% to 7.97% (mean=7.22%) in 5 immunological parameters.There were significations between two groups within 5 immunological parameters (P

Massive hemoptysis is one of the most dreaded of all respiratory emergencies and can have a variety of underlying causes. It is mostly caused by bleeding from bronchial circulation. Bronchial artery embolization is now considered to be the treatment of choice for acute massive hemoptysis. Bronchial artery embolization (BAE) is a safe and effective nonsurgical treatment for patients with massive hemoptysis. However, nonbronchial systemic arteries can be a significant source of massive hemoptysis and a cause of recurrence after successful BAE. So knowledge of the bronchial artery anatomy, together with an understanding of the pathophysiologic features of massive hemoptysis, are essential for planning and performing BAE in affected patients. In addition, interventional radiologists should be familiar with the techniques, results, efficacy, safety and possible complications of BAE and with the characteristics of the various embolic agents. Bronchial arterial catheterisation in human via a percutaneous approach has been practiced for 32 years (1973) in the world and 20 years (1986) in China, initially for direct chemotherapy treatment for bronchial malignancies and then for the embolization of patients with massive haemoptysis. A review of clinical experience to evaluate technique,embolic materials,outcome and complications of BAE is presented.

OBJECTIVE:To observe the efficacy and safety of Shuxuetong injection in the treatment of acute cerebral infarc-tion (ACI). METHODS:110 patients with ACI were randomly divided into control group and observation group. Control group was given mannitol,aspirin,atorvastatin,antidiabetic drugs and other conventional treatment;observation group was additionally given Shuxuetong injection 6 ml adding into 0.9% Sodium chloride solution 250 ml by intravenous infusion,3 times a day. The ef-ficacy in 2 groups was evaluated after 15 d,and the National Institute of Health Stroke Scale(NIHSS)score,neuron specific eno-lase(NSE),high-sensitivity C-reactive protein(hs-CRP)and tumor necrosis factor-α(TNF-α)levels before and after treatment and the incidence of adverse reactions in 2 groups were recorded. RESULTS:After treatment,NIHSS score,NSE,hs-CRP and TNF-αin 2 groups were significantly lower than before,and observation group was lower than control group,the differences were statisti-cally significant(P<0.05);total effective rate in observation group was significantly higher than control group,the difference was statistically significant(P<0.05). There was no obvious adverse reactions in 2 groups during treatment. CONCLUSIONS:Based on the conventional treatment,Shuxuetong injection has good efficacy and safety in the treatment of ACI.

OBJECTIVE:To establish a method for simultaneous determination of P-coumaric acid and ferulic acid in the couple of Sparganium stoloni and Curcuma aeruginosa,and to investigate rational proportion of the couple.METHODS:HPLC method was adopted.The determination was performed on Agilent 20RBAX XDB-C18 column with mobile phase consisted of acetonitrile-0.1％ phosphoric acid (gradient elution) at the flow rate of 1.0 mL/min.The detection wavelength was set at 266 nm,and the column temperature was 30 ℃.The sample size was 10 μL RESULTS:The linear ranges of P-coumaric acid and ferulic acid were 4.218 6-21.093 μg/mL(r=0.999 8),1.836 0-9.180 μg/mL(r=0.999 9),respectively.RSDs of precision,stability and reproducibility tests were all lower than 2.0％.The recoveries were 98.72％-100.30％ (RSD=0.18％,n=9),99.11％-100.45％ (RSD=0.46 ％,n =9).Among 4 compatibility proportions of the couple of S.stoloni and C.aeruginosa (1 ∶ 1,2 ∶ 1,1 ∶ 2,1 ∶ 0,m/m),the dis-solution rate of P-coumaric acid and ferulic acid were the highest when the ratio of S.stoloni-C.aeruginosa (m/m)was 2 ∶ 1.CON-CLUSIONS:The method is simple,accurate,stable and reproducible,and can be used for content determination of P-coumaric acid and ferulic acid in the couple of S.stoloni and C.aeruginosa.when the proportion of the couple of S.stoloni and C.aeruginosa is 2∶1 and the contents of P-coumaric and ferulic acid are the highest,the contents of main etfective components are the highest.

OBJECTIVETo investigate the significance of NADPH quinine oxidoreductase 1 (NQO1) protein overexpression on prognostic evaluation of head and neck squamous cell carcinoma (HNSCC).

METHODSNQO1 protein was detected in 162 of HNSCC, 45 cases of adjacent nontumor tissues and 26 samples of normal head and neck epithelia using EnVision immunohistochemical. Correlation between NQO1 overexpression and patients prognosis was also analyzed.

RESULTSThe positive rate and strongly positive rate of NQO1 protein were 84.0% (136/162) and 69.8% (113/162) in HNSCC, respectively, and both of which were significantly higher than either those in adjacent nontumor tissues and normal head and neck epithelia (both P < 0.01). NQO1 expression was significantly correlated with the clinical stage, pT and chemoradiotherapy of HNSCC (P < 0.01). Kaplan-Meier survival analysis showed that overall survival and disease-free survival rates were significantly higher in HNSCC patients with high level NQO1 expression than that those with low level of NQO1 expression (Log-rank = 6.625 , P = 0.010;Log-rank = 6.234 , P = 0.013). Additional analysis by Cox proportional hazard regression model showed that high level of NQO1 expression was an independent hazard predictor for overall survival of patients with HNSCC (Wald = 6.626, P = 0.008).

CONCLUSIONSNQO1 expression level is closely correlated with the progression and prognosis of patients with HNSCC. High level of NQO1 expression may be used as an important indicator for patients with poor prognostic HNSCC.

Breast ; enzymology ; Carcinoma, Squamous Cell ; enzymology ; mortality ; pathology ; Disease-Free Survival ; Female ; Head and Neck Neoplasms ; enzymology ; mortality ; pathology ; Humans ; Kaplan-Meier Estimate ; NAD(P)H Dehydrogenase (Quinone) ; metabolism ; NADH, NADPH Oxidoreductases ; metabolism ; Prognosis ; Proportional Hazards Models

During 2 years,a 6-year-old girl was hospitalized for 2 times with recurrent onset of episodes of vomiting,weakness and fever after eating dessert at the Department of Neurology & Endocrine Pediatrics,the Affiliated Hospital of Qingdao University.The arterial blood gas analysis revealed severe hypoglycemia,lacticacidemia and metabolic acidosis,the urine ketone body was positive.After intravenous infusion of glucose,bicarbonate and antibiotics,there was a dramatic clinical improvement in a short time.Physical examination showed tachypnea and mild hepatomegaly,and she had normal physical and mental development.The laboratory findings revealed transient hyperuricacidemia.Urine organic acids analysis repeatedly showed an elevation of lactic acid,ketone and glycerol.Glyceroluria was a very distinctive trait.The literatures in PubMed was searched with glyceroluria as keyword.Three related diseases were identified:FBPase deficiency,glycerol kinase (GK) deficiency and complex GK deficiency.Further reading of related literatures to understand the characteristics of diseases and laboratory tests,the clinical diagnosis of GK deficiency and complex GK deficiency was excluded.The mutation analysis of FBPase gene (FBP1) was performed by Sanger sequencing and a novel compound heterozygous mutations of c.355G ＞A and c.960delG was discovered.Full analysis of disease-related traits and targeted gene testing is one of the effective methods for accurate diagnosis and treatment of inherited metabolic disorders.

Objective:To study the effect of continuous subcutaneous insulin infusion (CSII) in the long-term treatment of type 1 diabetes mellitus (T1DM) children, to analyze the factors influencing the efficacy, and to provide theoretical guidance for the application of CSII in long-term therapy and follow-up management.Method:A total of 60 T1DM children who were under 18 years old, lived in Qingdao for a long time, had CSII for more than 6 months, and visited the Outpatient Department of Endocrinology of Qingdao Women and Children′s Hospital for a long term from January 2019 to June 2019 were followed up with questionnaires to understand their general condition and treatment-related data.Result:After the CSII therapy, the hemoglobin A1c (HbA1c) of T1DM children significantly decreased from (9.58±2.08)% to (7.12±1.11)% ( t=7.315, P<0.05), the daily insulin dosage per unit weight significantly declined from 0.91(0.86, 0.94) IU to 0.80 (0.66, 0.88) IU ( Z=-5.514, P<0.05), and the frequency of both hypoglycemia and ketoacidosis was significantly reduced.Diet control, daily exercise time, the visit frequency and the self-monitoring frequency of blood glucose affected the curative effect of CSII therapy (all P<0.05). Conclusion:CSII therapy can effectively control the blood glucose of children with T1DM in Qingdao area, significantly lower HbA1c, and reduce the insulin dosage and the incidence of hypoglycemia and ketoacidosis.Good diet control, regular exercise, regular follow-up and a high frequency of blood glucose monitoring are associated with better glycemic control.

Objective:To statistically analyze SCI paper output and research hotspots of Chinese stomatology research institutes from 2011 to 2020.Methods:The papers of Chinese stomatology research institutions in the Web of Science database were searched and analyzed by bibliometric methods in various dimensions including publication time, institutional and journal distribution, international (regional) cooperation, and research hotspots.Results:The number and quality of publications in Chinese dental research institutions have increased significantly in the past decade, and the top four institutions in terms of publication number were Sichuan University, Peking University, Shanghai Jiaotong University, and Air Force Military Medical University. The distribution of the journals to which the articles belong was relatively scattered, and the research hotspots mainly focused on squamous cell carcinoma, periodontitis, bone regeneration, and materials science.Conclusions:The output of SCI papers and the number of high-quality papers in Chinese stomatology research institutions increased year by year during 2011-2020, and the scientific research strength continued to increase.

Animals ; Bacterial Infections/metabolism* ; Glucose/metabolism* ; Homeostasis ; Insulin Secretion ; Mice ; Mice, Knockout ; Sirtuins/metabolism*