Congenital disorders of glycosylation(CDG) are a rapidly growing group of genetic diseases that are due to defects in the synthesis of glycans and in the attachment of glycans to other compounds. Most CDG are multisystemic diseases often involving severe psychomotor retardation. The CDG causing sialic acid deficiency of N-glycans can be diagnosed by isoelectric focusing of serum transferrin. Glycan structural analysis, yeast genetics and knockout animal models are essential tools in the elucidation of novel CDG. In this review, we focus on the current knowledge of the pathogenesis and identification of nine primary glycosylation diseases.

Objective To study the expression of distintegrin and metalloprotease (ADAM10) in serum of lung cancer patients and its clinical significance. Methods The serum protein level of ADAM10 was assayed by ELISA in 44 lung cancer patients,34 benign lung lesions and 31 healthy subjects. The serum protein level of CEA was assayed by radioimmunoassay. Results The serum concentration of ADAM10 and CEA was higher in patients with lung cancer than benign lung lesions and normal controls(P <0.01) ,theft was distinct statistical difference be-tween lung cancer and lung benign lesions,normal controls(P <0.01) ,but no statistical difference between lung be-nign lesions and normal controls(P >0.05) ; the serum concentration of ADAM10 of lung cancer with lymph node metastasis were significantly higher than those of lung cancer without lymph node metastasis, there was statistical difference (P < 0.01) ; the serum concentration of CEA of lung cancer with lymph node metastasis were slightly higher than those of lung cancer without lymph node metastasis, but there was no difference (P > 0.05) ; the serum concentration of ADAM10 and CEA in adenocarcinoma were higher than squamous cell carcinoma and small cell lung cancer(P <0.01 ,P <0.05). The single item detection of ADAM10 or CEA to the lung cancer diagnostic sensitivity is 51.7% ,56.9% respectively,the union detection reaches 84.5% . Conclusion ADAM10 is related with occur-rence and metastasis in lung cancer. The union detection of ADAM10 and CEA can raise the lung cancer diagnostic sensitivity.

AIM: To investigate the effects of uric acid sodium salt (UANa) as adjuvant on humoral and cellular immune response in BALB/c mice. METHODS: BALB/c mice were immunized with trichosanthin (TCS) as antigen together with UANa suspension as adjuvant. The antibody titers of IgG were detected by enzyme-linked immunosorbent assay. Dendritic cells (DC) were induced in vitro, the phenotypes of DC were analyzed by flow cytometry and the effect of UANa on DC maturity was evaluated. A delayed-type hypersensitivity (DTH) model was used to analyze the effect of UANa on cellular immune responses in vivo. The in vitro proliferation of lymphocytes was determined by ConA stimulation. RESULTS: Freunds adjuvant greatly enhanced the antibody response of mice to TCS, while UANa adjuvant failed to promote the antibody response but significantly reduced the antibody response as compared to TCS only. No effect of UANa on the expression of CD11c and CD83 in DC was observed by flow cytometry analysis. However, UANa significantly enhanced the expression of MHC II molecule. In the DTH model, UANa enhanced the degree of allergen-induced ear swelling and promoted the ability of lymphocyte proliferation in vitro. CONCLUSION: UANa suspension as adjuvant significantly enhances the cellular immune response but inhibits the humoral immune response to a certain degree, suggesting that UANa has potential application in the vaccine research.

This article was aimed to study the intestinal absorption about main active ingredients of pectolinarin and pectolinarigenin in Carboned Cirsium japonicum DC. The absorption rate and absorption rate constant were taken as indicators. The intestinal absorption of pectolinarin and pectolinarigenin were compared by everted rat intestinal sac method among different parts of the small intestine. The results showed that the absorption rate constant of pectoli-narin among duodenum, jejunum, ileum and colon parts were 0.505 1 ± 0.192 7, 0.936 0 ± 0.187 2, 0.732 0 ±0.133 5, 0.251 3 ± 0.027 6 (μg·h-1·cm-2). The absorption rate constant of pectolinarigenin among the duodenum, je-junum, ileum and colon were 0.059 1 ±0.008 3, 0.093 3 ±0.029 2, 0.112 3 ± 0.035 6, 0.029 4 ± 0.009 1 (μg·h-1·cm-2). It was concluded that the absorption of both ingredients increased over time. The absorption of both ingredi-ents in the jejunum and ileum was higher than other parts of the small intestine. The absorption rate of pectolinarin in the entire small intestine was much higher than the absorption rate of pectolinarigenin.

AIM:To investigate the effects of uric acid sodium salt (UANa) as adjuvant on humoral and cellular immune response in BALB/c mice. METHODS:BALB/c mice were immunized with trichosanthin (TCS) as antigen together with UANa suspension as adjuvant. The antibody titers of IgG were detected by enzyme-linked immunosorbent assay. Dendritic cells (DC) were induced in vitro,the phenotypes of DC were analyzed by flow cytometry and the effect of UANa on DC maturity was evaluated. A delayed-type hypersensitivity (DTH) model was used to analyze the effect of UANa on cellular immune responses in vivo. The in vitro proliferation of lymphocytes was determined by ConA stimulation. RESULTS:Freund's adjuvant greatly enhanced the antibody response of mice to TCS,while UANa adjuvant failed to promote the antibody response but significantly reduced the antibody response as compared to TCS only. No effect of UANa on the expression of CD11c and CD83 in DC was observed by flow cytometry analysis. However,UANa significantly enhanced the expression of MHC II molecule. In the DTH model,UANa enhanced the degree of allergen-induced ear swelling and promoted the ability of lymphocyte proliferation in vitro. CONCLUSION:UANa suspension as adjuvant significantly enhances the cellular immune response but inhibits the humoral immune response to a certain degree,suggesting that UANa has potential application in the vaccine research.

Objective To study the cause and management of difficult laparoscopic appendectomy.Method A retrospective analysis of 102 difficult laparoscopic appendectomies was performed. Results The associating factors leading to operative difficulty were as follows:adhesion of peri-appendix in 44 cases,gangrene at root of appendix in 21 cases,special location of appendix in 17 cases,abdominal adhesion in 12 cases,obesity and inflatable intestine in 8 cases. Laparoscopic appendectomy was successfully performed in 96 cases(94.1%,96/102) ,and no intestinal leakage or massive hemorrahge occurred. Conclusions Adhesion of peri-appendix is the most common cause in difficult laparoscopic appendectomy,and gangrene at root of appendix is the most difficult one. Reasonable management for appendix stump contributes to successful operation.

Objective: To introduce a new surgical technique for the correction of congenital syndactyly without skin grafts. Methods: The technique consists of a dorsal double-wing flap created from the dorsal skin of the metacarpophalangeal joint to cover the newly released web space and zigzag incisions in the fingers, thus avoiding the use of skin grafts in this area. Results: From May 2010 to October 2016, 35 web spaces in 24 patients were treated using this technique. There were no complications such as haematoma, infection or flap necrosis. The average follow-up time was 54 months (range, 6 months to 60 months). One of the 35 webs developed web creep. No patients developed flexion contractures. All webs had good appearance with 45 degrees inclination from dorsal to palmar. All fingers had no obvious scar, and the flexion and abduction function were good. Conclusions The technique is simple, rapid, safe and easily performed and does not require the use of skin grafts.

Objective To explore a simple and effective operative procedure for treatment of pa-tellar fractures.Methods The clinical data of 108 patients (including 76 males and 32 females) with patellar fractures were analyzed.The age of the patients ranged from 18 years to 82 years.There were 67 patellar fractures on the right extremities and 41 on the left.Fracture types included transfractures in 43 patients, comminuted fractures in 54, torn fractures in eight and longitudinal fractures in three.Period from injury to operation ranged from 3 hours to 10 days.During operation, the broken patella was exposed for reduction and temporary pliers fixation;then, a five-pointed star woven with two absorbable sutures was placed on the broken patella, two semi-circular sutures around the patellar edge were made with su-tures which were through five points of the five-pointed star.When two sutures were pulled and knotted,the five-pointed star was also stretched to fix the patellar fractures firmly.Models of transverse patellar fractures were made in 20 knee joints of catties, which were divided into two groups randomly.Patellar fractures in Group A were fixed with five-pointed star lattice sutures and those in Group B with AO inten-sion bands.Loading test was performed on quadriceps femoris with materials test system for measuring the width of each fractured patella after the test.Results All patients were followed up for 6-60 months (mean 20 months) , which showed that all patellar fractures were healed.According to Bostman scoring system, the efficacy was excellent in 76 patients and good in 32.The experiment showed no statistical difference in the fracture disjunction distance between two methods (P > 0.05).Conclusion For treatment of patellar fractures, five-pointed star lattice sutures have the advantages of simple operation,reliable fixation, early postoperative exercise, fast recovery, satisfactory outcome and free need of reoper-ation for removing internal fixation.

OBJECTIVE: To compare the CT scan results in patients with deviation of nasal septum and normal adults and to explore its values in evaluating the state of nasal ventilation. METHOD: Twenty-six patients with deviation of nasal septum and 15 normal people were recruited in the study. The nasal airway from anterior nostril to posterior nostril was evaluated with PhlipsTomos scan AV Expander Elspiral scanner. The indexes of nasal ventilation was measured with the image-analysis software. The results of CT scan between patients with deviation of nasal septum and normal people was compared. RESULT: (1) There was obvious difference between patients with deviation of nasal septum and normal people in the NMCSA, IMCSA, DNCAT and DICAT. There was obvious difference between patients with deviation of nasal septum with obstruction in the opposite side and normal people in the ONMCSA and ODNCAT. There was obvious difference between patients with deviation of nasal septum without obstruction in the opposite side and normal people in the ODNCAT. (2) In the patients with deviation of nasal septum, between nasal minimal cross sectional area and inferior turbinate's maximum cross sectional area, there is obvious relation, r = -0.394, P < 0.05. CONCLUSION CT is one kind of objective inspection method in evaluating the state of nasal ventilation. With the measurement of the nasal morphology and functional index, we can understand the state of nasal ventilation more comprehensively. And with this, the treatment and diagnosis of disease with nasal obstruction will be more scientific.
Adolescent ; Adult ; Case-Control Studies ; Female ; Humans ; Male ; Middle Aged ; Nasal Obstruction ; diagnostic imaging ; physiopathology ; Nasal Septum ; abnormalities ; diagnostic imaging ; Pulmonary Ventilation ; Tomography, X-Ray Computed ; Young Adult

Objective:To investigate the clinical efficacy of external fixators combined with anterolateral thigh musculocutaneous flap for treatment of Gustilo type IIIB/C open tibiofibular fractures.Methods:A retrospective case series study was conducted to analyze clinical data of 15 patients with Gustilo type IIIB/C open tibiofibular fractures admitted to Ruihua Hospital of Soochow University from March 2016 to June 2019. There were 11 males and 4 females, with the age of (48.5±12.6)years (range, 22-67 years). All patients underwent emergency debridement in stage I, the major blood vessels, nerves and tendons were inspected and repaired, and the fracture ends were fixed by external fixator. There were different degrees of wounds necrosis, infection and bone defect after operation. After debridement in stage II, the soft tissue defects with the dimension of 10.0 cm×5.0 cm to 30.0 cm×8.0 cm were repaired with anterolateral thigh musculocutaneous flaps whose areas ranged from 10.5 cm×5.5 cm to 30.5 cm×8.5 cm. All donor areas of the musculocutaneous flaps were sutured directly in stage I. The healing of the donor areas and musculocutaneous flaps were observed within 2 weeks after operation. At the last follow-up, the shape and sensory recovery of the flap, healing of fractures and related complications were observed. The lower extremity functional scale (LEFS) was used to evaluate the injured limb function.Results:All patients were followed-up for 12-32 months [(22.0±5.8)months]. All donor areas were healed by first intention, leaving only linear scars. The musculocutaneous flaps survived completely in all patients. Partial necrosis of large area of musculocutaneous flap occurred in 2 patients, and healed after debridement and skin grafting. Another patient had vascular crisis after musculocutaneous flap operation and survived after the embolized vein repaired by contralateral great saphenous. At the last follow-up, the shape of flap recovered well, and the feeling partially recovered with the two-point discrimination of 18-26 mm. All fractures healed well, and there were no serious infection-related complications such as osteomyelitis. The LEFS score was 47-69 points [(59.0±9.5)points].Conclusion:Theexternal fixator combined with anterolateral thigh musculocutaneous flaps for treatment of Gustilo type IIIB/Copen tibiofibular fractures can better restore the appearance of soft tissue defect of the lower leg, and can effectively reduce the occurrence of severe infection-related complications.