The clinical manifestation and laboratory features of juvenile hyaline fibromatosis(JHF)are reposed in a 5-year-old girl.Clinical symptoms began at 1 year of age,which was characterized by multiple cutaneous nodules and gingival hyperplasia with normal intelligence.Histopathologic examination of a skin biopsy specimen showed that the epidermis was normal,the nodules were composed of spindle-shaped fibroblasts embedded in homogeneous,non-fibrous,eosinophilic substance,and normal tissue was disrupted.Moreover,the fibroblasts showed transparent cytoplasm arranged in an obscure bundle-like pattern.No cell atypia or necrosis was observed.PCR amplification and sequencing of the capillary morphogenesis gene-2(CMG2)revealed a homozygous splicing mutation(IVS 14+1G→T)in the patient,while both of her parents were hetemzygous for the mutation.JHF is inherited in this family in an autosomal recessive manner.

Objective To evaluate low-weight mesh for tension-free repair of inguinal hernia in the elders. Methods 120 old patients ( age at 60 -97 years) underwent tension-free repair of inguinal hernia by using low-weight mesh. The early complications, time of up and about, hospital stay, postoperative chronic pain,foreign body feelings and hernia recurrence were analyzed. Results The operations were successful in all cases and the average time of operation was (43 ± 12 ) min. 9 cases suffered from edema of the scrotum, 5 cases suffered urine retention, 4 cases suffered from incision hematoma. The average time of outof-bed was ( 17.4 ± 1.8 ) h, the average hospital days was ( 7.4 ± 1.9) d. However, there were 1.7%(2/120) patients suffering from slight chronic pain and 9. 1% (11/120) patients complaining foreign body feelings. There was no recurrence after follow-up for 6 to 48 months. Conclusions The clinical application of low-weight mesh for tension-free repair of inguinal hernia in old age is safe and effective, with an additional advantage of low occurrence of chronic pain and foreign body feelings.

Objective: To analyze the epidemiological characteristics of HIV-infected pregnant women and exposed infant in Guangdong province and identify the factors associated with infant HIV infection through mother-to-child transmission. Methods: National Information System for Prevention of mother-to-child HIV Transmission and Early Infant Diagnosis Information Management Platform were used to collect the individual information about HIV-infected pregnant women and exposed infants who were delivered in Guangdong from January 1, 2014 to December 31 in 2017. The differences in pregnant women’s demographic data, history of pregnancy and childbirth, the utilization of mother-to-child transmission prevention services and early infant diagnosis between the infected HIV exposed infants and uninfected HIV exposed infants were compared, and univariate and multivariate logistic regression analyses were conducted to identify the factors associated with mother-to-child HIV transmission. Results: Among 349 HIV infected pregnant women, the proportions of the pregnant women whose HIV infection status were confirmed before pregnancy, during pregnancy and at or after childbirth were 30.4% (106/349), 49.6% (173/349) and 20.0% (70/349) respectively. The proportions of those with sexual partners whose HIV infection status were unknown and those receiving no antiviral treatment were 39.5% (138/349) and 13.2% (46/349) respectively. Among the HIV exposed infants, the mother-to-child transmission rate was 4.2%(15/353), the HIV exposed infants had the first or second early diagnosis tests within 44 (P₂₅-P₇₅: 42-50) days and 96 (P₂₅-P₇₅: 92-106) days after birth, respectively. Univariate logistic regression analysis indicated that the risk for mother-to-child HIV transmission increased in those whose HIV infection status were confirmed at or after childbirth compared with before pregnancy (OR=5.72, 95%CI: 1.52-21.61) and in the group that antiviral treatment was given to either mothers or infants compared with the group that antiviral treatment was given to both mothers and infants (OR=33.56, 95%CI: 9.04-124.55), while there was lower mother-to-child HIV transmission risk in artificial feeding group compared with breast feeding group (OR=0.07, 95%CI: 0.01-0.76). Conclusion The risk of mother-to-child HIV transmission in Guangdong can be effectively reduced by the measures of early diagnosis, antiviral treatment and artificial feeding as well as the improvement of mother-to-child transmission prevention service.

OBJECTIVE: To clarify the correct diagnostic methods of FBPS of children, and to explore techniques and technology of surgical operation aim to complete excision of sinus tract and preservation of the recurrent laryngeal nerve through analyzing the clinical characteristics and the characteristics of open neck surgery in FBPS of children. METHOD: Clinical materials of 6 children FBPS with internal pyriform sinus were retrospectively analyzed, and literatures were studied to explore the appropriate surgical management of children FBPS; lesions of 6 patients were completely dissected by open neck surgery with the help of di-direction catheterization revealing the tract of fistula, and the openings of the fistula to pyriform apex were cauterized by monopolar coagulation at the end of the procedure; all 6 cases were with systematic endoscopic follow-up. RESULT: All cases of 6 children patients were cured by open neck surgery, no recurrence of clinical symptoms were observed during a mean follow-up of 14 months (1-3 years); 2 cases were diagnosed with an "anatomical" relapse (persistence of the sinus tract orifice without clinical symptoms) at the follow-up endoscopy(at 3 and 4 months after surgery); all 6 cases had no complication of laryngeal paralysis. CONCLUSION The children patients presenting recurrent acute suppurative thyroiditis and deep neck abscess of anterior area in the lower part of neck, especially locating left neck, should be considered as the diagnosis of FBPS. also the same as confirmed by enhanced neck CT scan and direct laryngoscopy. Patients with identification of internal pyriform sinus and Betz fold by direct laryngoscopy, and with identification of fistula which passes through the cricothyroid membrane beneath the superior laryngeal nerve can be confirmed FBPS. Completely dissection of sinus tract by open neck surgery was the effective management of children FBPS and di direction catheterization aids to reveal and identify the tract of fistula. Resection of the posterior part of the thyroid cartilage ala can be useful to aid exposure and preservation of the recurrent laryngeal nerve; but co-excision of a portion of the upper of the thyroid gland aids to the entire dissection of lesions. In addition, cauterizing the opening of the fistula to pyriform apex may be useful to obliterate the internal opening of pyriform sinus.
Adolescent ; Branchial Region ; abnormalities ; Child ; Child, Preschool ; Female ; Fistula ; diagnosis ; surgery ; Humans ; Male ; Neck ; Retrospective Studies

The unilateral biportal endoscopic technique is defined as posterior unilateral of two small incision with established percutaneous observation channel and endoscopic observation channel in monitoring field. The operation tools were placed within the channel for each operation inside and outside vertebral canal. The characteristics of dual channel intervention make this technology different from coaxial endoscopic technology. This technique has a clear vision for flexible and convenient operation with the advantages of relatively simple surgical instrument requirements. In recent years, the use of unilateral dual channel endoscopic surgery in treating spinal diseases was gradually increasing, especially in the removal of nucleus including lumbar intervertebral disc protrusion and lumbar spinal stenosis disease decompression, and in endoscopic space-occupying lesions such as vertebral fusion and spinal canal between inside and outside. It was also applied in cervical and thoracic diseases with good clinical effects. Its advantages include less surgical trauma and faster recovery process. In addition, it has a large surgical field of vision under the microscope for easy identification of structures and surgery and with relatively gentle learning curve. Thus, it is conducive to beginners' mastery. However, complications such as nerve injury and postoperative lower limb numbness are still frequently reported. Some factors have to be considered, including insufficient understanding of the new technology, the local anatomy of the dual-channel endoscope, the operation of the spinal canal under the microscope. The successful application of the technique in treating various lumbar diseases needs understanding the operation process of the technique and local anatomy under the microscope.

Objective:To develop a unilateral biportal endoscopic technique and investigate the clinical efficacy of unilateral biportal endoscopic technique in the treatment of lumbar disc herniation and lumbar spinal stenosis.Methods:Between July 2018 and June 2019, 60 patients with lumbar disc herniation or spinal stenosis were treated by unilateral biportal endoscopic technique. According to the inclusion and exclusion criteria, 51 patients were included in the study. There were 25 cases of prolapsed lumbar disc herniation, 26 cases of degenerative lumbar spinal stenosis, all of which were treated with posterior lumbar decompression by unilateral biportal endoscopic laminectomy, and contralateral stealth decompression by unilateral approach was performed on the patients with bilateral stenosis. Endoscopic interbody fusion and percutaneous pedicle screw fixation were performed in 5 patients with instability. Operation time, length of incision, hospital stay and complications were recorded. Visual analogue scale (VAS) for low back pain and leg pain, Oswestry dysfunction index (ODI) and the modified Macnab scale were used to evaluate the clinical efficacy.Results:All operations were completed successfully, and no cases were transferred to open surgery. The operative time was 70.29±19.55 min (44-151 min), while the length of incision was 1.79±0.34 cm (1.4-3.0 cm). Postoperative CT suggested complete decompression with intact contralateral structure. All patients got out of bed 1-3 d after surgery, and the postoperative hospital stay was 3.49±2.76 d (1-14 d). The postoperative follow-up time was 13.59±2.80 months (10-21 months). Forty-six patients returned to work or normal activities within 3 weeks and 5 patients with interbody fusion returned to normal activities within 4 weeks. According to the modified Macnab criteria, the final outcome was excellent in 43 cases, good in 6 cases, and fair in 2 cases. There were 3 cases of dural sac tear during operation and 2 cases of transient numbness of lower limbs after surgery and they all recovered after conservative treatment. The VAS score of low back pain of 26 patients with lumbar spinal stenosis was reduced from 6.69±1.44 before surgery to 3.27±1.43 at postoperative 1 month, 2.69±1.57 at postoperative 3 months, 2.31±1.16 at postoperative 6 months and 2.23±1.28 at the last follow-up, respectively, and the difference was statistically significant ( F=128.534, P<0.005). The VAS scoreof leg pain was reduced from 6.77±1.34 before surgery to 3.27±1.37 at postoperative 1 month, 2.88±1.48 at postoperative 3 months, 2.85±1.52 at postoperative 6 months and 2.54±1.53 at the last follow-up, and the difference was statistically significant ( F=146.951, P<0.005). The ODI score was reduced from 64.18%±8.23% before surgery to 37.53%±4.45% at postoperative 1 month, 27.51%±3.83% at postoperative 3 months, 19.91%±5.27% at postoperative 6 months and 6.84%±2.74% at the last follow-up, and the difference was statistically significant ( F=783.966, P<0.005). The VAS score of low back pain of 25 patients with lumbar disc herniation was reduced from 5.60±1.38 before surgery to 3.04±1.54 at postoperative 1 month, 2.84±1.75 at postoperative 3 months, 3.12±1.86 at postoperative 6 months and 3.44±1.69 at the last follow-up, respectively, and the difference was statistically significant ( F=22.357, P<0.005). The VAS scoreof leg pain was reduced from 5.48±1.45 before surgery to 2.88±1.64 at postoperative 1 month, 2.52±1.83 at postoperative 3 months, 2.76±1.83 at postoperative 6 months and 3.00±1.92 at the last follow-up, and the difference was statistically significant ( F=29.445, P<0.005). The ODI score was reduced from 53.59%±6.87% before surgery to 32.46%±3.78% at postoperative 1 month, 23.39%±2.78% at postoperative 3 months, 16.49%±3.49% at postoperative 6 months and 7.23%±3.15% at the last follow-up, and the difference was statistically significant ( F=790.985, P<0.005). Conclusion:Unilateral biportal endoscopic technique has the advantages of clear and wide field of vision, large operating space, relatively simple surgical instrument need and convenient and flexible operation procedure. It has excellent clinical effects in the treatment of lumbar disc herniation and lumbar spinal stenosis.

Objective To establish a model of knee joint extension contracture in New Zealand white rab-bits, and to lay the experimental foundation for further studies on the pathogenesis and treatment of joint contractures. Methods Thirty male New Zealand white rabbits with mature bones were randomly divided into 6 groups. The left knee joints of the immobilization groups ( 5 groups of 5 rats each) were fixed in extension for 1, 2, 4, 6 or 8 weeks. There was also a control group. At the end of each period the plaster was demolished and the level of transforming growth factor-β1 ( TGF-β1) in joint cavities, the degree of total contracture, myogenic contracture, arthrogenic con-tracture, and the thickness of the posterior joint capsules were measured. The significance of the differences between the immobilized groups and the control group was compared using one-way analysis. Results The level of TGF-β1 in the joint fluid differed significantly among the 6 groups. The differences in the degree of total contracture among the control group, one-week, two-week and four-week groups were also significant. The average degree of the myogenic contracture in the one-week group was significantly different from the other 5 groups' averages. The average myogenic contracture was also of significantly different between the two-week group and the control group. The degree of arthro-genic contracture was significantly different among the groups except for between the 6-week and 8-week groups. The average joint capsule thickness was significantly different among all of the groups except for between the control group and the one-week group. Conclusion This technique for modeling knee extending contracture using New Zealand white rabbits is simple and practical. It provides a better animal model for studying the mechanism of knee joint con-tracture and related treatment strategies and can be used for further exploration of the occurrence and recovery of knee contractures.

Objective To analyze the efficacy and safety of glimepiride treatment as initial monotherapy in newly diagnosed patients with type 2 diabetes mellitus (T2DM).Methods This was a subgroup analysis of the GREAT study,which investigated the efficacy and safety of glimepiride as initial monotherapy in Chinese patients with T2DM.This analysis was performed in 209 patients with disease duration less than 6 months and never received any anti-diabetic drugs.The change of HbA1C,fasting plasm glucose (FPG),2 h postprandial blood glucose (2hPPG),homeostasis model assessment for β-cell function index (HOMA-β),homeostasis model assessment for insulin-resistance index(HOMA-IR),the percentage of patients with HbA1C ＜ 7.0％ at endpoint and the incidence of hypoglycemia were evaluated after 16-weeks treatment.Results After 16-weeks glimepiride treatment,HbA1C value reduced significantly from baseline to endpoint,the reduction was statistically significant (9.21％ ± 1.65％ to 6.69％±0.83％,P＜0.001),69.7％ of the patients achieved HbA1C ＜7.0％ at study endpoint.Glimepiride-treated patients also achieved a significant improvement in FPG [from (10.15 ± 2.13) mmol/L to (7.23 ± 1.50) mmol/L,P＜0.001] and 2hPPG [from (17.21 ±4.14) mmol/L to (11.62 ± 3.34) mmol/L].HOMA-β was improved from 17.21± 15.19 [11.62 (2.90,115.8)] to 41.13 ± 44.12 [28.00 (5.1,360.00)],and HOMA-IR was reduced from 2.32± 1.90 [1.76 (0.60,12.80)] to 2.07 ± 1.74 [1.63 (0.4,12.3)].The incidence of all reported symptomatic hypoglycemia was 18.2％,and the incidence of confirmed hypoglycemia was 3.8％.Conclusion This analysis showed that glimepiride treatment as an initial mono-therapy could effectively improve blood glucose control in newly diagnosed patients with T2DM,and the treatment may improve islet β cell function,and the safety profile is reasonably good.

OBJECTIVETo investigate the relationship between aberrant methylation of Syk and Runx3 genes and recurrence and metastasis after resection of gastric cancer.

METHODSApplying methylation-specific polymerase chain reaction technique, promoter methylation of Syk and Runx3 genes in the tumor tissues and adjacent normal tissues of gastric cancer patients were detected to investigate the relationship between methylation status of the promoter region of Syk and Runx3 genes and postoperative recurrence and metastasis.

RESULTSIn the 70 cases of gastric cancer, the frequencies of promoter methylation of Syk and Runx3 genes were 45.7% (32/70) and 55.7% (39/70) in gastric cancer, and 0 (0/70) and 7.1% (5/70), respectively, in the adjacent normal tissues. The rates of promoter methylation of Syk and Runx3 genes in the gastric cancers were significantly higher than that in the adjacent normal tissues (P < 0.001 for all). The promoter methylation of Syk and Runx3 genes was significantly correlated with the degree of tumor differentiation, depth of invasion, lymph node metastasis and pathological staging (P < 0.05 for all). The frequency of postoperative recurrence and metastasis in 32 patients with Syk promoter methylation was 65.6% (21/32) and that in 38 cases with Syk promoter unmethylation was 18.4% (7/38), showing a significant difference between the two subgroups (χ(2) = 16.13, P < 0.001). The rate of postoperative recurrence and metastasis in 39 patients with Runx3 promoter methylation was 61.5% (24/39) and that in 31 patients with Runx3 promoter unmethylation was 12.9% (4/31, P < 0.001).

CONCLUSIONSThe methylation of Syk and Runx3 promoters plays an important role in postoperative recurrence and metastasis of gastric cancer. Combined detection of promoter methylation of Syk and Runx3 genes is helpful for early diagnosis and evaluation of prognosis of gastric cancer.

Adenocarcinoma ; genetics ; pathology ; surgery ; Adenocarcinoma, Mucinous ; genetics ; pathology ; surgery ; Adenocarcinoma, Papillary ; genetics ; pathology ; surgery ; Adolescent ; Adult ; Aged ; Aged, 80 and over ; Carcinoma, Signet Ring Cell ; genetics ; pathology ; surgery ; Core Binding Factor Alpha 3 Subunit ; genetics ; DNA Methylation ; Female ; Follow-Up Studies ; Gastrectomy ; Humans ; Intracellular Signaling Peptides and Proteins ; genetics ; Lymphatic Metastasis ; Male ; Middle Aged ; Neoplasm Invasiveness ; Neoplasm Recurrence, Local ; Neoplasm Staging ; Promoter Regions, Genetic ; Protein-Tyrosine Kinases ; genetics ; Stomach Neoplasms ; genetics ; pathology ; surgery ; Syk Kinase ; Young Adult