Objectives To summarize the characteristics,differential diagnosis and management of incomplete 17 alpha-hydroxylase/17,20-1yase deficiency(17 OHD)of Chinese patients.Methods Six cases of incomplete 17 OHD from Peking Union Medical College Hospital were studied retrospectively through analyzing their clinical data,and the molecular pathogenic mechanism was discussed after literature review.Results Four cases of 46,XX incomplete 17 OHD were reported.The clinical characteristics included female phenotype,various degrees of breast development and absent or sparse axillary/pubic hair, oligomenorrhea or secondary amenorrhea,recurrent luteinized ovarian cysts,hypogonadism with persistent hyperprogesteronemia or high serum 17 alpha-hydroxyprogesterone level,with or without hypokalemic hypertension.There were also 2 cases of 46,XY incomplete 17 OHD,in which ambiguous genitalia were present besides hypokalemic hypertension.Conclusions Incomplete 17 OHD is a very rare form of congenital enzymatic deficiencies of steroid synthesis,which should be included in the differential diagnosis when there are menstrual disorders,sexual infantilism,recurrent ovarian cysts or ambiguous genitalia.Under such circumstances,hyperprogesteronemia offers a valuable clue for further investigation.

Objective:To search for a method for radical resection of pancreatic and duodenal malignancy involving the mesentery root and for the long post-operation survival of patients.Methods:From Jan.2004 to Aug.2006,a total of 26(16 male and 10 female. aged 27-70)patients with pancreatic and duodenal malignancy involving the mesentery root were treated in our department.The patients included 3 with duodenal malignancy and 23 with pancreatic malignancy.Curative resection was performed by the extended pancreaticoduodenetomy(Whipple procedure)combined with mesentery root resection(MRR)for all patients.The outcomes,safety and the post-operation survival rate were analyzed retrospectively.Results:Thirteen patients were treated with Whipple procedures combined with MRR,9 were treated with partial portal vein/superior mesenteric vein(PV/SMV)and reconstruction of the vessel,and 4 patients received pre-shunt between PV and SMV with artificial vessel graft before the extended Whipple and MRR procedures.The operation time was 2.5 to 7(4.4?1.1)hour,and blood loss was 300 to 5 000(1892?1414)ml with the blood transfusion of 0 to 5 600(2 100?1 586)ml.There was no death in our group and 7(27%)had post-operation complication.The post-operation hospital stay was 10 to 30 days.The pathologic examination showed negative surgical margins for all specimens.The tumor size was 4 to 10 (6.17?2.03)cm.After a follow-up of 9 to 38 months,the pain was relieved in all patients.One of the 3 patients with duodenal adenocarcinoma had liver metastasis at 10 months after operation,and the other 2 survived 10 months and 27 months without evidence of tumor reccurence.The patient with pancreatic micro-adenocarcinoma died of local reccurence 9 months after operation.The patient with neuroendocrine carcinoma died of organ failure 24 months after operation.The patient with lymphoma have survived for 24 months after operation.The 1-year and 2-year accumulated survival rates in the 20 cases with pancreatic ductal cancer were 86.6% and 45.6%. respectively.Conclusion:The extended Whipple procedure with MRR is safe and effective.It can obtain R0 resection in patients with malignant tumors(over 5 cm in diameter)in the head,neck and uncinate process of the pancreas and duodenal.

In order to enhance the antitumor efficacy of recombinant Newcastle disease virus, rNDV-IL15 was rescued in this study. Recombinant plasmid prNDV-IL15 was constructed, and BHK21 cells were transfected with the recombinant plasmid. Finally, the recombinant Newcastle disease virus rNDV-IL15 was successfully rescued. The growth curves of these two recombinant viruses were determined. Murine melanoma B16F10 cells were infected with rNDV-IL15 at MOI of 0.1, and the expression level of IL15 in the supernatant was detected by ELISA. The antitumor efficacy of rNDV-IL15 and rNDV was compared in vitro and in vivo. Results showed that prNDV-IL15 was constructed and recombinant virus rNDV-IL15 was successfully rescued. The growth curve of rNDV-IL15 showed that the growth of rNDV-IL15 had not been changed after insertion of IL15 gene. Results showed that there was high level of IL15 expression in the supernatant of rNDV-IL5-infected B16F10 cells (1 044.3 +/- 27.7 ng x mL(-1)). rNDV-IL15 and rNDV significantly inhibited the growth of B16F10 cells in vitro in a time-dependent manner. However, there was no significant difference between them. In animal experiments, rNDV-IL15 efficiently suppressed tumor growth in vivo when compared with rNDV, and the difference was statistically significant. The results suggested that rNDV-IL15 is a more effective antitumor agent.
Animals ; Body Weight ; Cell Line, Tumor ; Cell Proliferation ; Chick Embryo ; Cytotoxicity, Immunologic ; Female ; Genetic Therapy ; Interleukin-15 ; genetics ; metabolism ; Melanoma, Experimental ; pathology ; therapy ; Mice ; Neoplasm Transplantation ; Newcastle disease virus ; genetics ; Plasmids ; Recombinant Proteins ; genetics ; metabolism ; Transfection ; Tumor Burden

To investigate the cell-killing effect and its possible mechanism of rClone30-hDR5 in combination with TRAIL on human hepatic carcinoma (HCC) cell line, first of all, recombinant plasmid pee12.4-hDR5 was introduced into HepG2 cells by liposome transfection. After five rounds of screening by flow cytometry, HepG2 cells expressing high levels of DR5 on cell surface were isolated. The cytotoxicity of TRAIL to selected cells was higher than that of TRAIL to HepG2 cells by MTT method (P < 0.01). The result suggested that the cloned hDR5 gene had biological activity. MTT assay showed that, rClone30- hDR5 in combination with TRAIL more efficiently inhibited the tumor growth of HepG2 cells compared to rClone30-hDR5 or TRAIL in vitro. The results of Annexin V-FITC/PI staining and Quantitative Real-time PCR indicated that rClone30-hDR5 in combination with TRAIL significantly increased the mRNA levels of caspase 3 and caspase 8, and induced the apoptosis of tumor cells. HepG2 cells were infected with rClone30-hDR5 or rClone30 at MOI of 1. The expression of hDR5 on tumor surface increased significantly by rClone30-hDR5 compared to that by rClone30, which contributed to the sensitivity to TRAIL. In conclusion, rClone30-hDR5 in combination with TRAIL has potential application value in cancer treatment.
Apoptosis ; Carcinoma, Hepatocellular ; pathology ; Caspase 3 ; metabolism ; Caspase 8 ; metabolism ; Drug Synergism ; Hep G2 Cells ; Humans ; Liver Neoplasms ; pathology ; Real-Time Polymerase Chain Reaction ; Receptors, TNF-Related Apoptosis-Inducing Ligand ; pharmacology ; TNF-Related Apoptosis-Inducing Ligand ; pharmacology ; Transfection

OBJECTIVETo discuss the safety line to avoid the post-hepatectomy hepatic insufficiency by combining indocyanine green test by pulse dye-densitometry (PDD-ICG) and standard remnant liver volume (SRLV).

METHODSSeventy-five hepatic cancer patients undergone hepatectomy from March 2007 to February 2008 were included. According to the liver function decompensatory grades after operation, the relationship between ICG retention rate at 15 min (ICGR15) and standard remnant liver volume by linear regression were analyzed.

RESULTSThere were 60 cases with slight hepatic insufficiency, 12 cases with moderate hepatic insufficiency, and 3 cases with severe hepatic insufficiency. There were no difference in age [(50 +/- 13) years old and (53 +/- 9) years old], prothrombin time [(13.6 +/- 1.0) s and (13.5 +/- 1.0) s], international normalized ratio (1.09 +/- 0.10 and 1.06 +/- 0.10) between slight hepatic insufficiency group and moderate and severe hepatic insufficiency group (P > 0.05). And there were difference in K value (0.20 +/- 0.04 and 0.17 +/- 0.03), ICGR15 (6 +/- 4 and 9 +/- 4), SRLV [(545 +/- 93) ml and (398 +/- 82) ml] between two groups (P < 0.05). Compared ICG test and standard remnant liver volume of the patients with moderate hepatic insufficiency after operation, the liner relationship was found (R = 0.640, P = 0.025), and the regression equation was: standard remnant liver volume (ml/m(2)) = 1594.6 x ICGR15 + 265.

CONCLUSIONSPDD-ICG test and standard remnant liver volume are effective to estimate hepatic function reserve of patient undergone hepatectomy.

Adult ; Female ; Hepatectomy ; methods ; Hepatic Insufficiency ; etiology ; Humans ; Indocyanine Green ; pharmacokinetics ; Liver ; pathology ; Liver Neoplasms ; pathology ; surgery ; Male ; Middle Aged ; Postoperative Complications ; etiology

OBJECTIVETo observe the differentiation and distribution of epidermal stem cell (ESC) after skin soft tissue expansion, and to initially probe into the growth mechanism of expanded skin tissue.

METHODSSamples of normal skin and expanded skin (mean effusion period 45 days) were harvested from head and cervical region in 15 patients who underwent II stage surgery after skin expansion. Samples were divided into scalp adjacent to the center of expander group (expanded scalp, 3 cm from the vertical axis of the expander), scalp from lateral part of the expander group (expanded scalp, 5 - 7 cm lateral to the vertical axis of the expander), cervical skin expansion group, un-expanded scalp control group, and un-expanded cervical skin control group, according to the position of skin harvested. The tissue structure of skin in each group was observed with HE staining, and the differentiation and distribution characteristics of cytokeratin 19 (CK19) positive cells were observed with immunohistochemical staining.

RESULTSCompared with those in the un-expanded control groups, uneven, relatively thickened and obviously folded epidermis with more cell layers and cells with obvious aggregation close to the basal layer were observed in the expanded groups, but those cells were not well-arranged and the transition of polarity was not obvious. The continuity of CK19 positive cells in the basal layer of skin was observed in each of the expanded group with immunohistochemical staining, and positive cells increased obviously and arranged in multilayer in certain parts of basal layer. Clustered or dispersed CK19 positive cells were also observed outside the basal layer. No above-mentioned phenomenon was observed in the un-expanded control group.

CONCLUSIONSThe proliferation and differentiation of ESC with ectopic distribution may enhance the repair process after skin soft tissue expansion.

Cell Proliferation ; Dermis ; cytology ; Epidermis ; cytology ; Humans ; Stem Cells ; cytology ; Tissue Expansion ; Wound Healing

OBJECTIVETo probe the best therapy for the patient after operation of hypertensive cerebral hemorrhage.

METHODSOne hundred cases were randomly divided into a treatment group and a control group, 50 cases in each group. The control group were treated by routine western medicine (Mannitol, Nifedipine and Caftriaxone sodium and so On), and the treatment group by the medication of the control group plus acupuncture 9 days after operation. Acupuncture was given at Neiguan (PC 6), Shuigou (GV 26), Sanyinjiso (SP 6), Jiquan (HT 1) and Weizhong (BL 40) and so on, combined with scalp acupuncture, with "restoring consciousness and inducing consciousness" needling method, for 10 days. The nervous function defect was assessed by Chinese Stroke Scale and the criteria for assessment of therapeutic effects.

RESULTSAcupuncture could improve significantly nervous function defect. The total effective rate was 86.000 in the treatment group and 14.0% in the control group with a significant difference between the two groups (P < 0.01).

CONCLUSIONThe therapeutic effect of the treatment group is better than that of the control group. Acupuncture combined with western medicine has cooperation for treatment of hypertensive intracerebral hemorrhage with a better therapeutic effect.

Acupuncture Therapy ; Adult ; Aged ; Aged, 80 and over ; Combined Modality Therapy ; Female ; Humans ; Intracranial Hemorrhage, Hypertensive ; physiopathology ; therapy ; Male ; Middle Aged ; Nervous System ; physiopathology

AIMTo study the effects of 9-cis-retinoic acid (9-cis-RA) on cell cycle and expression of cyclin D1 and cdk4 in lung cancer cells.

METHODS9-cis-RA (1 x 10(-6) mol.L-1) was used to treat lung cancer cells for 24 h; Flow cytometry (FCM) was used to detect the percent of G0/G1 phase and S phase cells of three groups including blank control, DMSO control and 9-cis-RA groups; RT-PCR was used to analyze the expression changes of cyclin D1 and cdk4 before and after treatment with 9-cis-RA in lung cancer cells.

RESULTSThe percent of G0/G1 phase cells of 9-cis-RA groups was significantly higher than that of the control groups (P < 0.01 or P < 0.05) and the percent of S phase cells of 9-cis-RA groups was lower than that of the control groups (P < 0.01 or P < 0.05); the expression of cyclin D1 of PG, SPC-A1 and L78 cells was decreased (P < 0.01) and the expression of cdk4 of PG, A549 and L78 cells was also decreased (P < 0.01) after treatment with 9-cis-RA.

CONCLUSIONMost of the proliferation and the expression of cyclin D1 and cdk4 of PG, A549, SPC-A1 and L78 were inhibited by 9-cis-RA.

Adenocarcinoma ; metabolism ; pathology ; Antineoplastic Agents ; pharmacology ; Carcinoma, Squamous Cell ; metabolism ; pathology ; Cell Division ; drug effects ; Cell Line, Tumor ; Cyclin D1 ; biosynthesis ; Cyclin-Dependent Kinase 4 ; Cyclin-Dependent Kinases ; biosynthesis ; G1 Phase ; drug effects ; Humans ; Lung Neoplasms ; metabolism ; pathology ; Proto-Oncogene Proteins ; Resting Phase, Cell Cycle ; drug effects ; S Phase ; drug effects ; Tretinoin ; pharmacology

Objective To observe the effects of cell cycle inhibitor on astrocytic proliferation and scar formation and to study neuronal apoptosis after focal cerebral ischemia in rats.Methods Ischemic model was established by photochemistry method.T_2-weighted MRI was performed on the 3rd, 7th, and 30th day after focal cerebral ischemia.The expression of glial fibrillary acidic protein(GFAP)and apoptosis was observed by immunofluorescence.The protein levels of GFAP and proliferation cell nuclear antigen (PCNA), CyelinA and CyclinB1 were measured by Western blotting from the ischemic and sham animals finished on the 3rd, 7th, and 30th day.Results A marked and significant reduction of brain infarction volume was found in Olomoucine-treated ischemic animals(2.27%?0.28% , 1.87%?0.19%, 1.08%? 0.18%)as compared with controls(5.10%?0.35%, 4.60%?0.26%, 3.96%?0.28%, P

OBJECTIVETo find a better therapy for extensive cerebral infarction.

METHODSSixty cases with extensive cerebral infarction were treated by surgical decompression. After operation, they were randomly divided into an acupuncture plus medication group and a medication group, 30 cases in each group. The patients in the medication group were treated by routine western medicine, and the patients in the acupuncture plus medication group by the routine western medicine plus acupuncture 2 days after operation with Danzhong (CV 17), Zhongwan (CV 12), Qihai (CV 6), Zusanli (ST 36), Xuehai (SP 10) and Shuigou (GV 26) selected. They were treated for 10 days. The nervous functions were assessed by Chinese Stroke Nerve-function Rating Scale and criteria for clinical therapeutic effect.

RESULTSAfter surgical decompression, the death rate was 20.0%; after operation, acupuncture could significantly improve the neurological functional deficit degree of the patient. The total effective rate of 40.0% in the acupuncture plus medication group was better than 13.3% in the medication group (P<0.05).

CONCLUSIONThe surgical decompression is able to decrease the death rate of extensive cerebral infarction; after the operation, acupuncture and western medicine have cooperative effect on extensive cerebral infarction with a good therapeutic effect.

Acupuncture Points ; Acupuncture Therapy ; Adult ; Aged ; Aged, 80 and over ; Cerebral Infarction ; drug therapy ; physiopathology ; therapy ; Female ; Humans ; Male ; Middle Aged ; Nervous System ; physiopathology