Objective To investigate the clinical and laboratory profiles of a patient with pustular psoriasis-like skin lesion and cerebral palsy due to biotinidase deficiency. Methods A 5 year and 4 month-old boy with biotinidase deficiency was confirmed by urinary organic acid analysis with gas chromatography/mass spectrometry (GC/MS)and biotinidase activity assay of peripheral blood. His clinical features, laboratory findings, treatment and outcome were studied. Results The boy showed difficulty in taking food after birth, gradually eczema and pustules appeared at the age of 2 months, and generalized erythema and intractable pustular psoriasis-like lesion at the age of 8 months. His intellectual development was normal with retardation of locomotor system. He had muscular dystonia at the age of 6 months. Physical examination showed generalized pustular psoriasis-like lesion, generalized paralysis, hypertonic contracture of extremities, sparseness of scalp hair and severe malnutrition. Routine laboratory tests showed a mild anemia, metabolic acidosis and elevation of plasma creatine phosphokinase. Increased excretion of urinary lactate, pyruvate, 3-OH-propionate, propionylglycine, and 3-methylcrontonylglycine were observed. Biotinidase activity of his peripheral blood was below 0.1 pmol/min/3mm (normal 6.3-9.3 pmol/min/3mm). Biotin (10 mg/day) supplementation led to a dramatic recovery of the skin lesion. After the treatment of rehabilitation, his muscle power was also improved gradually. Conclusions Dermatological and neurological manifestations are the main features of biotinidase deficiency. Early diagnosis and biotin administration can greatly improve the clinical symptoms. Generalized pustular psoriasis-like lesion and cerebral palsy of this boy have improved after the supplementation of biotin, but he may be remained wheelchair-dependent because of delayed diagnosis.

Objective To evaluate the feasibility and outcomes of different surgical approaches on the basis of sentinel lymph node biopsy (SLNB) in treating early-stage vulvar cancer, and discuss the proper strategy for individualized treatment. Methods The medical charts of patients with early-stage vulvar cancer treated in Sun Yat-sen University between January 2004 and December 2013 were retrospectively collected. A total of 74 patients who received sentinel lymph node(SLN)detection in primary surgery were enrolled (average age 55). The surgical approaches contained SLNB, inguinal lymphadenectomy (IL), and extensive vulvectomy. The SLN were examed on intraoperative frozen sections. The treatment protocols, lymphatic metastasis, postoperative recovery condition, recurrence and survival data were collected and analyzed. Results At least one SLN was successfully detected in 68 (92%,68/74) patients. SLN were positive in 21 patients, of whom 12 (group A) underwent bilateral IL, and 9 (group B) received radiotherapy without performed IL. SLN were negative in 47 patients, of whom 26 (group C) underwent bilateral IL and one of them had a non-SLN metastasis, and 21 (group D) were advised to follow-up. The coincidence of pathological results between frozen and paraffin sections was 100%. The sensitivity and specificity of SLNB for diagnosis of lymph node metastasis were 95% and 100%, respectively. A total of 44 complications happened in patients underwent SLNB and IL (group A and C), including 16 poor wound healing, 14 lymphedema, 8 lymphatic fistulas, 3 phlebothrombosis and 3 infections. There were no complications happened in patients underwent SLNB alone (group B and D), among whom the operation time, bleeding amount, and hospital stay were also significantly less than those in patients underwent SLNB and IL. The median follow-up time was 41 months and the 3-year overall survival rate was 85% in the whole series. Recurrences were observed in 11 patients and 9 of them died of the tumor with the median survival time of 15 months. In patients with positive SLN (group A and B), the 3-year overall survival rate was 58% with 8 patients died of the disease, including 4 in group A and 4 in group B. In patients with negative SLN (group C and D), the 3-years overall survival rate was 97% with one patient in group D died of the tumor, and significantly higher than that of patients with positive SLN (P=0.003). The 3-year overall survival rate was significantly difference. In univariate analysis by log-rank test showed that, neither in patients with nor without SLN metastasis the prognosis differed with respect to surgical approaches (group A vs B, P=0.709;group C vs D, P=0.253). Univariate analysis by log-rank test showed that, lymph node metastasis, pathological grade, depth of invasion, and tumor location could significantly affected survival (P<0.05), whereas age, tumor diameter, and surgical approach didn′t (P>0.05). Multivariate analysis showed that lymph node metastasis (RR=21.57, 95%CI:2.68-173.10, P=0.002) and tumor location (RR=7.85, 95%CI:1.79-34.50, P=0.024) were the independent factors for overall survival. Conclusions Lymph node metastasis is an independent prognosis factor for patients with early-stage vulvar cancer. SLNB could accurately diagnose the status of lymph nodes and help to decide subsequent treatment. The omissions of IL in patients with negative SLN avoid surgical morbidity and shorten postoperative recovery period without an increased risk of recurrence.

Objective To explore the effects of general anesthesia combined thoracic paraverte-bral block on postoperative pain and fast track single-port video-assisted thoracoscopic surgery (VATS).Methods Thirty patients,including male 20 and female 10,received single-port VATS were randomly and equally divided into two groups:group C received general anesthesia only,and group T received ultrasound-guided thoracic paravertebral nerve block combined with general anesthe-sia.Both groups did not use the patient-controlled analgesia,if insufficient analgesia happened (rest-ing VAS scores>4),than used dezocine intravenously as additional analgesia (a single-dose 5-20 mg, no more than 120 mg per day).The Ramsay scores at 1,4,8,12 h after the surgery and the mechani-cal withdrawal threshold on the day before the surgery,at 4,8,12,24 h after the surgery were recor-ded.The first time of post-operation pain feedback,the consumption of dezocine in the first 24 h after surgery,the incidence rates of side effects,the first time off-bed and the hospital stays were also re-corded.Results Compared with group C,the Ramsay scores at 8,12 h postoperatively in group T significantly decreased (P <0.05),and the mechanical withdrawal threshold at 4,8 h postoperatively significantly increased (P <0.05).The first time of post-operation pain feedback in group T was sig-nificantly longer than group C (P <0.05).The consumption of dezocine in the first 24 h after surgery significantly decreased in group T (P <0.05).The first time off-bed and the hospital stays in group T were shorter than group C (P <0.05).Also,the incidence rates of nausea,vomiting in the first 24 h postoperatively were lower in group T (P < 0.05 ).Conclusion General anesthesia combined with single-injected thoracic paravertebral nerve block can effectively relieve the postoperative pain in pa-tients undergoing single-port VATS,reduce the consumption of opioids in the first 24 h postopera-tively,cutting down the occurring rates of adverse reactions,which was beneficial to early ambulate and shortened the hospital stays.

Objective To evaluate the management and survival of lymph node region recurrence of epithelial ovarian cancer (EOC),and discuss its suitable therapeutic strategy.Methods Thirty-eight patients with the recurrence of lymph node region were extracted from 1945 patients who were diagnosed EOC and treated in Sun Yat-sen University Cancer Center from January 1995 to December 2008.The clinical characteristics,therapy methods and survival of them were retrospectively analyzed.Patient age at initial diagnosis was ＞ 50 years old in 24 patients and ≤50 years old in 14 patients.There were 15 cases with stage Ⅱ and 23 cases with stage Ⅲ in terms of initial International Federation of Gynecology and Obstetrics (FIGO,1987) staging.Classified with histological grade,7 cases were in G1,14 cases were in G2,17 cases were in G3 ;according to the histological types,19 cases were with serous adenocarcinomas,and 19 cases were with non-serous adenocarcinomas (including 9 endometrioid adenocarcinoma,1 mucinous adenocarcinoma and 9 unclassified adenocarcinoma).The median follow-up time was 59 months (ranged 16 to 124 months).Results (1) Feature of recurrences:the median interval of last treatment to recurrence was 18 months (range 9 to 96 months).Most of them were absence of symptoms.The serum level of CA125 was elevated in 15 patients(39％,15/38).(2) Treatment of recurrences:of the 38 patients,19 underwent lymphadnectomy for recurrence regions and received adjuvant chemotherapy (surgery + chemotherapy group),14 received local radiotherapy and adjuvant chemotherapy (radiotherapy + chemotherapy group),5 received chemoherapy only (chemotherapy group).There were 35 cases achieved complete response (CR),including 19 patients underwent secondary debulking surgery in surgery + chemotherapy group,14 cases in radiotherapy + chemotherapy group (12 of them treated by radiotherapy,the other 2 cases reached CR after adjuvant chemotherapy) and 2 cases in chemotherapy group.While only 3 patients reached partial response in chemotherapy group.(3) Survival and second recurrences:during follow-up,14 cases died of tumor,4 cases survival with tumor while 20 cases survival without evidence of tumor.The 5-year post-recurrence survival rate of 38 cases was 66.5％,with 71.8％,68.8％ and 40.0％ in surgery +chemotherapy,radiotherapy + chemotherapy,and chemotherapy group,respectively,and there was no significant difference in survival rate between them (P ＞ 0.05).A total of 15 patients experienced second recurrences,including 7 cases with peritoneal and 8 cases with lymph node region recurrences.(4) Prognosis factors:the univariate analysis shown that survival after recurrence was significantly related to patient age,tumor-free interval and number of recurrence disease (P ＜ 0.05),while not to FIGO stage,histological type,histological grade,and lymphadnectomy during primary surgery (P ＞ 0.05).The multivariate analysis showed that patient age and tumor-free interval were independent prognostic variables for survival after recurrence (P ＜ 0.05).Conclusions The lymph node region recurrence of EOC may be have good prognosis and distinctive clinical process.Local treatment strategies including secondary surgery and radiotherapy should be considered,which may significantly improve survival in ovarian cancer patients with lymph node region recurrence.

The dosage-efficacy/toxicity relationship of the 50% alcohol extracts of Polygonum multiflorum was comparatively investigated on either normal or CCl4-induced chronic liver injury rats, by determining the general condition, serum biochemical indices and liver histopathology, coupled with the factor analysis. The dosages were 10 and 20 g raw materials per kg body weight. Compared with the normal control group, the normal high dose group showed significant increases of the serum alanine transaminase (ALT), total bilirubin (TBIL), high mobility group box 1 (HMGB-1) and interleukin-1β (IL-1β) (P < 0.05 or P < 0.01), as well the frequent incidences of inflammatory cell infiltration, hepatic sinus enlargement and fiber stripes formation in histopathological sections. Compared with the model control group, the model low dose group showed significant declines of serum ALT, aspartate transaminase (AST) and total bile acid (TBA) (P < 0.05), as well the alleviation of vacuoles of hepatocytes, but no amelioration of the inflammatory cell infiltration and fibrous tissue hyperplasia; moreover, the model high dose group showed significant degeneration declines of serum HMGB-1, tumor necrosis factor-α (TNF-α) and IL-1β (P < 0.05, P < 0.01), as well the evident alleviation of vacuoles degeneration of hepatocytes, inflammatory cells infiltration and fibrosis degree. The factor analysis showed that the low dosage treatment had almost neither injuring effect on the normal rats nor protective effect on the model rats; while the high dosage treatment showed observable injuring effect on the normal rats, expressed by the significant increases of the factor-1 (HMGB-1, TNF-α and IL-1β as the main contributors) and factor-2 (TBIL, ALT and TBA as the main contributors) relative to the normal control group. The liver protective effect of the high dosage treatment could be observed with the significant reduction of the factor-1, indicating the effective alleviation of the expression of inflammatory cytokines. In conclusion, it could illustrated the phenomenon of symptom-based prescription theory of Polygonum multiflorum on rat livers: the high dosage of the herb had either an injuring effect on normal rats, or a therapeutic effect on the rats with chronic liver injury.

The liver injury induced by Polygonum multiflorum Thunb. (PM) was investigated based on idiosyncratic hepatotoxicity model co-treated with lipopolysaccharide (LPS) at a non-hepatotoxic dose. Sprague-Dawley (SD) rats were intragastrically administered with three doses (18.9, 37.8, 75.6 g crude drug per kg body weight) of 50% alcohol extracts of PM alone or co-treated with non-toxic dose of LPS (2.8 mg·kg(-1)) via tail vein injection. The plasma alanine aminotransferase (ALT) and aspartate aminotransferase (AST) activities were assayed and the isolated livers were evaluated for histopathological changes. The dose-toxicity relationships of single treatment of PM or co-treatment of LPS were investigated comparatively to elucidate the idiosyncratic hepatotoxicity of PM. The results showed that no significant alterations of plasma ALT and AST activities were observed in the groups of solo-administration of LPS (2.8 mg·kg(-1), i.v.) or different dosage (18.9, 37.8 and 75.6 g·kg(-1), i.g.) of PM, compared to normal control group (P > 0.05); while significant elevations were observed in the co-administration groups of PM and LPS. Treatment with LPS alone caused slight infiltration of inflammatory cells in portal area but no evident hepatocytes injury. Co-treatment with LPS and PM (75.6 g·kg(-1), i.g.) caused hepatocyte focal necrosis, loss of central vein intima and a large number of inflammatory cell infiltration in portal areas. When further reduce the dosage of PM, significant increases of plasma ALT and AST activities (P < 0.05) were still observed in co-administration groups of LPS and PM (1.08 or 2.16 g·kg(-1)), but not in LPS or PM solo-administration groups. Nevertheless, the co-treatment of low dosage of PM (0.54 g·kg(-1)) with LPS did not induce any alteration of plasma ALT and AST. In conclusion, intragastric administration with 75.6 g·kg(-1) of PM did not induce liver injury in normal rats model; while the 2 folds of clinical equivalent dose of PM (1.08 g·kg(-1)) could result in liver injury in the LPS-based idiosyncratic hepatotoxicity model, which could be used to evaluate the idiosyncratic hepatotoxicity of PM.

OBJECTIVE: To investigate the role of VEGF in angiogenesis of nasopharyngeal carcinoma tissue. METHOD: Serum and tissue VEGF were detected by the quantitative enzyme-linked immunosorbent assay (ELISA) method both in 62 nasopharyngeal carcinoma without therapy and 20 nasopharyngitis. The expression of microvessel density in tissue of nasopharyngeal carcinoma and nasopharyngitis were detected by immunohistochemical method. RESULT: (1) There was no significant relationship compared VEGF and MVD in nasopharyngeal carcinoma patient, gender and ages (P>0.05), while the expression of VEGF and MVD in later stage (III+IV) were significant higher than that in earlier stage (I+I) (P<0.01), and in positive lymph node group or metastasis group were higher than in negative group (P<0.01); (2) The expression of VEGF and MVD in nasopharyngeal carcinoma serum and tissue were higher than in nasopharyngitis (P<0.05); (3) There was positive correlation between VEGF and MVD in nasopharyngeal carcinoma tissue (r=0.865, P<0.01), but negative correlation in their serum (r=0.328, P>0.05). CONCLUSION VEGF could induce angiogenesis in nasopharyngeal carcinoma tissue, and play an important role in progression in nasopharyngeal carcinoma, VEGF could be an important marker for monitoring prognosis of disease.
Adolescent ; Adult ; Aged ; Carcinoma ; Endothelium, Vascular ; metabolism ; pathology ; Female ; Humans ; Male ; Microvessels ; Middle Aged ; Nasopharyngeal Carcinoma ; Nasopharyngeal Neoplasms ; blood supply ; metabolism ; pathology ; Neoplasm Staging ; Neovascularization, Pathologic ; Prognosis ; Vascular Endothelial Growth Factor A ; metabolism ; Young Adult