Objective To explore the clinical features,diagnosi s and prognosis of incontinentia pigmenti.Methods Analyzing and summarizing the clinical characteristic, diagnosis and prognosis of neonatal incontinentia pigmenti in 6 neonatal infants that were hospita- lized in our department during the period from January 1 998 to December 2003 were studied,and some relevant literature were reviewed. Results 1.Three of 6 infants were male which was unusual;2.Four infants had typical skin lesions at birth and 1 case at 6 days old.Four cases had typical 3 stages o f skin lesions including the erythematous and vesicular inflammatory stage,verr ucous lesions and hyperkeratosis stage,macular hyperpigmentation stage,but the re was overlap;3.Four infants were complicated by central nervous system involv ement (two cases presented mental retardation,2 infants were temporary damage). Two cases were complicated by ocular manifestations ( one case had optical nerve atrophy and blind in left eye,the other had severe bilateral retinal lesions); 4.On specific examination 5 infants were diagnosed by skin biopsy.Gene analysis was made in 1 case,but we didn′t find the mutations of NEMO. Conclusions Incontinentia pigmenti is a rare X-linked dominant multisystem disease.It may be misdiagnosed in the initial stages.Except typical clinical features,skin biops y and gene analysis are main evidence for diagnosis.Early detection and interven tion are important for prognosis. J Appl Clin Pediatr,2005,20(2):123-125

Objective To improve the recognition of intramedullary spinal abscess by a case of congenital dermal sinus with intramedullary spinal abscess and reduco the incidence of congenital dermal sinus with intramedullary spinal abscess.Methods Clinical,laboratory data and image of a confirmed case about one infant of congenital dermal sinus with multiple intramedullary spinal abscess were investigated,the related literature was reviewed.Results In this case,when the infant with congential dermal sinus had infection,he failed to gain antibiotic therapy, timely surgical treatment,his infection had diffused, and multiple intramedullary spinal abscess flared up.Conclusions Intramedullary spinal abscess is a rare disease.If treatment is delayed, the prognosis is poor and the mortality rate is high.MRI is the ideal investigation for diagnosis.Intramedullary spinal abscess can happen subsequent to congenital dermal sinus with infection, and cause neurological sequela. So an infant with congenital dermal sinus should be offered to avoid complication caused by infection.

11.0 for the pH of reaction solution,65℃～75℃for the reaction temperature,and 1:1.7 for the mass ratio of corn(dry weight)to FeCl_3?6H_2O.The corn polysaccharide-Fe(Ⅲ)complex synthesized with the optimized process was stable,with good solubility in water.The assay of Fe(Ⅲ)was 39.86%,40.20%and 40.17%,respectively for three batches of products.The RSD was

0.5 cm or beneficial anatomical vari- ations displayed on MRCP,were obviously improved and there were no significantly different among the 4 types hilar eholangiocarcinoma.Conclusion MRCP could accurately make the preoperative diagnosis and type of hilar cholangiocarcinoma; the image of second branch of bile duct and the variation of the confluence of hepatic hilar displayed on MRCP has great clinical significance for operative regimes of hilar cholangiocar- cinoma,especially for typeⅣ.It does benefit not only to improve the resection and radical rate of some hilar cholangiocarcinomas, but also to select suitable method of biliary enteric anastomosis and avoid injuring the bile duct in operation.

OBJECTIVETo compare the differences in the clinical function and lumbar and abdominal myodynamia in patiants of lumbar disc herniation treated with moxibustion at Dazhui (GV 14) and Guanyuan (CV 4) and acupuncture.

METHODSForty cases were randomized into a moxibustion group and an acupuncture group, 20 cases in each group. In the moxibustion group, the warm moxibustion was applied alternatively at Dazhui (GV 14) and Guanyuan (CV 4) once every other day, 1 h each time, once every day. In the acupuncture group, acupuncture was applied to the corresponding acupoints based on the affected lumbar vertebras, such as Jiaji (EX-B 2), Shens-hu (BL 23), Dachangshu (BL 25) and Huantiao (GB 30), etc. , once evey day 30 min each time. The treatment for 3 weeks was taken as one session in each group. Totally, one session treatment was required. Surface electromyography (SEMG) of bilateral paraspinal muscle and rectus muscle, and JOA score of low back pain were observed in the two groups.

RESULTS(1) JOA score: the score of subjective symptoms, score of activity of daily living (ADL) and total score were improved obviously as compared with those before treatment in the two groups (P<0.01, P<0.05). The results of subjective symptoms score, score of ADL and total score in the acupuncture group were superior to those in the moxibustion group after treatment (6.95+/-0.94 vs 5.50 +/-0.89,10. 90+/-1.86 vs 8.90+/- 1. 92,22.50 +/- 2.82 vs 19.35 +/- 2. 70, all P<0. 05). (2) SEMG comparison: root-mean-square value (RMS) was all reduced in SEMG of the anteflexion, rear protraction, orthostatism, bilateral bending and neck and leg rear flexion for strengthening lumbar muscle as compared with those before treatment in the two groups (P< 0.05, P<0. 01). RMS of the anteflexion and bilateral bending in the acupunture group were reduced much obviously as compared with the moxibustion group. In terms of sitting position anteflexion, rear protraction, orthostatism, bilateral bending and neck and leg rear flexion for strengthening lumbar muscle, median frequency (MF) after treatment was all improved as compared with that before treatment in the two groups (P<0. 05, P<0. 01). In terms of anteflexion, the electrode MF after treatment was improved much obviously in the acupuncture group(P<0. 05).

CONCLUSIONMoxibustion at Dazhui (GV 14) and Guanyuan (CV 4) and conventional acupuncture all improve muscle function, relieve muscle fatigue, increase the ability of anti-muscle fatigue, strengthen lumbar vertebral stability, release subjective symptoms and improve ADL. But, the effects of moxibustion are slightly lower than those of acupuncture.

Acupuncture Therapy ; Adult ; Aged ; Electromyography ; Female ; Humans ; Intervertebral Disc Displacement ; physiopathology ; therapy ; Lumbar Vertebrae ; physiopathology ; Male ; Middle Aged ; Moxibustion ; Young Adult

OBJECTIVETo evaluate the feasibility and efficacy of arterial duct stenting in neonates with pulmonary atresia and intact ventricular septum.

METHODSEleven neonatal pulmonary atresia with intact ventricular septum patients received arterial duct stenting in our hospital from December 2007 to September 2010 were involved in this study. The average age was (8.20 +/- 2.90) days (ranged from 3 to 13 days). The average weight was (3.41 +/- 0.29) kg (ranged from 3.00 to 3.88 kg). The stents were selected according to digital subtracted angiography measurements. After checking for correct position by angiography, the balloon was inflated to expand the stent to desired diameter. Oxygen saturation was monitored, echocardiography was measured and stent diameter and location were observed by chest Xray. Patients were followed up at 1, 3, 6 and 12 months post procedure.

RESULTSStents were successfully implanted in all 11 patients. The preoperative peripheral oxygen saturation was (63.27 +/- 8.47)%, while increased to (82.73 +/- 5.59)% after alprostadil application and to (86.18 +/- 3.19)% after operation (all P < 0.01). After the operation, the peripheral oxygen saturation was higher than alprostadil application (P < 0.05). The intraoperative narrowest diameter of patent ductus arteriosus was (1.69 +/- 0.37) mm, the length was (16.72 +/- 2.37) mm. The internal diameter of implant stents was 4 mm, the length was (20.18 +/- 3.40) mm. After the operation, surgical B-T shunt operation was performed in one patient due to stent shift and pulse oxygen saturation decrease. One patient died post operation with unknown reason, another patient received stent balloon dilatation due to pulse oxygen saturation decrease at 4 months after the surgery. Pulmonary atresia with intact ventricular septum surgeries were performed in 2 patients at 5 and 7 months after stent implantation.

CONCLUSIONThe neonatal pulmonary atresia with intact ventricular septum arterial stent implantation was a feasible and effective procedure and this method could be used as preferred treatment in pulmonary atresia and intact ventricular septum for neonates.

Cardiac Catheterization ; Follow-Up Studies ; Humans ; Infant, Newborn ; Male ; Pulmonary Atresia ; therapy ; Stents ; Treatment Outcome ; Ventricular Septum

Objective To explore the factors associated with the complications of cranioplasty, infection and local epidural hematoma, such as age, sex, primary disease, skull defect and concomitant hydrocephalus. Methods 211 patients after cranioplasty were reviewed. Results 8 cases (3.8%) complicated one of them, in which 6 cases with local epidural hematoma and 2 cases with infection. The infection was more likely related with the areas of skull defect (P=0.003). No factor was found related with the local epidural hematoma. Conclusion It is necessary to focus the size of the frontal sinus when repairing the the frontal bone, to avoid the screw into the frontal sinus caused infection.

Objective　In a RCT study, the safety and efficacy of sabot (a slow-release salbuteral) and volmax (controlled-release salbuterol) were compared in bronchial asthma. Methods　40 patients with moderate to severe asthma were randomly divided into two groups and treated by sabot or volmax for 2 weeks. The FEV1%, peak expiratory flow (PEF), symptom score and use of rescue ventolin were measured to evaluate the effect of treatment. Results　After treatment FEV1%, PEF and symptom score improved and the need for inhaling short-acting beta 2-agonis in both groups reduced significantly. There was no difference of these improvement between two groups. Conclusion　The safety and efficacy of sabot for treatment of asthma was similar to volmax.

BACKGROUNDThe second mitochondria-derived activator of caspases (Smac) is a novel proapoptotic gene, which plays an important role in the apoptosis-inducing effects of irradiation on tumor cells. The purpose of this study was to investigate the effects of extrinsic Smac gene transfer and its over-expression in radiotherapeutic sensitivities of cervical cancer cells.

METHODSAfter the Smac gene was transferred into the cervical cancer cell line HeLa, subcloned cells were obtained by persistent G418 selection. Cellular Smac gene expression was detected by RT-PCR and Western blot, while in vitro cell viabilities were detected by trypan blue staining assay. After treatment with X-ray irradiation, cellular radiotherapeutic sensitivities were investigated by tetrazolium bromide colorimetry. Cellular apoptosis and its rate were determined by electronic microscopy, annexin V-FITC and propidium iodide staining flow cytometry. The expression and activities of cellular caspase-3 were assayed by Western blot and colorimetry.

RESULTSSmac mRNA and protein levels in HeLa/Smac cells and the selected subclone cell line of cervical cancer were significantly higher than those of HeLa (P < 0.01). There was no significant difference in cellular viabilities between them (P > 0.05). However, after irradiation with 8 Gy X-ray, growth activities of HeLa/Smac were reduced by 22.42% (P < 0.01). When compared with those of HeLa, partial HeLa/Smac cells presented characteristic morphological changes of apoptosis under electronic microscope, with higher apoptosis rates (16.4% vs. 6.2%, P < 0.01); the caspase-3 expression levels in HeLa/Smac cells were improved significantly (P < 0.01), while its activities were increased by 3.42 times (P < 0.01).

CONCLUSIONSStable transfer of the extrinsic Smac gene and its over-expression in cervical cancer cell line could significantly enhance the expression and activities of cellular caspase-3 and ameliorate apoptosis-inducing effects of irradiation on cancer cells, which was a novel strategy to improve radiotherapeutic effects on cervical cancer.

Apoptosis ; radiation effects ; Carrier Proteins ; genetics ; physiology ; Caspase 3 ; Caspases ; metabolism ; Female ; Gene Transfer, Horizontal ; HeLa Cells ; Humans ; Intracellular Signaling Peptides and Proteins ; Mitochondrial Proteins ; genetics ; physiology ; Radiation Tolerance ; Uterine Cervical Neoplasms ; drug therapy ; enzymology ; pathology

Histone lysine methyltransferase EZH2 has been reported to be frequently overexpressed in hepatocellular carcinoma (HCC) tissues and associated with hepatocarcinogenesis. However, the exact mechanism of EZH2 up-regulation in HCC has not been determined. In this study, we used murine hepatocyte AML12 cells to investigate the role of hepatitis B virus X protein (HBx) in regulating the expression of mEZH2. Western blot analysis demonstrated that the expression level of mEZH2 protein in AML12 cells was up-regulated by HBx in a dose-dependent manner. To further investigate the mechanism of mEZH2 overexpression, the 2500 bp regulatory sequence upstream from the first exon of the mEZH2 gene was amplified from AML12 genomic DNA and constructed into a luciferase reporter plasmid. The luciferase activity of the mEZH2 promoter significantly increased in AML12 cells co-transfected with HBx plasmid, and deleting the -486/-214 promoter region decreased HBx-induced mEZH2 promoter activation by nearly 50%. The -486/-214 region was then analyzed in the TRANSFAC 6.0 database and a typical E2F1-binding site was found. Mutation of this E2F1-binding site or knockdown of E2F1 expression by RNAi led to a dramatic decrease in HBx-induced activation of the mEZH2 promoter and mEZH2 overexpression in AML12 cells. These results provide evidence that HBx up-regulates mEZH2 expression by transactivating the mEZH2 promoter through E2F1 transcription factor, thereby providing new epigenetic evidence for the carcinogenic effect of HBx.
Animals ; Binding Sites ; Cell Line ; E2F1 Transcription Factor ; genetics ; Enhancer of Zeste Homolog 2 Protein ; Hepatocytes ; cytology ; metabolism ; virology ; Histone-Lysine N-Methyltransferase ; genetics ; metabolism ; Mice ; Plasmids ; Polycomb Repressive Complex 2 ; Promoter Regions, Genetic ; genetics ; RNA, Small Interfering ; genetics ; Trans-Activators ; genetics ; metabolism ; Transfection ; Up-Regulation