Objective To investigate the clinical,neuroimaging and myopathological features of mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes(MELAS).Methods The clinical manifestations,neuroimaging and myopathological features of 31 patients with MELAS diagnosed in our Neuromuscular Center in the recent 7 years were retrospectively analyzed.A3243G point mutations were analyzed by RFLP method in 10 patients.Results ①Clinical features:There were 18 male patients and 13 female patients.The age of onset ranged from 3 to 43 years,averaging 21.9 years.The averaged duration was 4.9 years.Thirteen patients in this group had family history of maternal inheritance pattern.The main clinical manifestations included short stature(26 patients),recurrent headache and vomiting(24 patients), muscle weakness(22 patients),epileptic seizure(21 patients),cognitive decline(19 patients),visual disturbance(17 patients),sensorineural deafness(16 patients),ataxia(6 patients),psychiatric symptom (8 patients),external ophathalmoplegia(2 patients)and diabetes mellitus(9 patients).The serum CK level was slightly elevated in 6 patients,and the fasting blood lactic acid was increased in 15 of the 18 detected patients.②Neuroimaging features:The stroke-like lesions were mostly confined to cerebral cortex, including temporal lobe(24 patients),occipital lobe(21 patients),parietal lobe(12 patients)and frontal lobe(4 patients).Three patients had deep white matter involvement.Migrating stroke-like lesions were confirmed in 4 patients by repeated cranial CT/MRI examination.In addition,cerebral atrophy(17 patients)and bilateral basilar ganglion calcification(11 patients)were found.③Myopathological features: Scattered ragged red fibers(RRF)in various number were found in all the patients by MGT staining.Other founding included strongly SDH-reactive blood vessel(27 patients),COX enzyme deficiency(19 patients), and mild to moderate lipid storage in RRF(20 patients).④MtDNA analysis showed 9 patients with A3243G point mutation in all the detected 13 patients.Conclusion The clinical and neuroimaging features may offer important clue to the diagnosis of MELAS,but a definite diagnosis of MELAS relies on the myopathology and mtDNA mutation analysis.

Objective To develop a quality of life scale for patients with congenital external and middle ear malformation, and to explore its reliability and validity. Methods The initial quality of life scale for patients with congenital external and middle ear malformation was constructed based on quality of life scales from home and abroad. A total of 140 patients with congenital external and middle ear malformation had been recruited in the study. After pretest and item sifting, the quality of life scale was constructed, and its reliability and validity were evaluated. Results Eighteen-item quality of life scale for patients with congenital external and middle ear malformation was constructed, which included three parts: physiological function, psychological status and social interaction. The retest reliability was 0. 878; split-half reliability coefficient and Cronbach's alpha coefficient were 0.927 and 0. 899, respectively. The results of factor analysis showed satisfactory construct validity. The reliability and validity of this scale was consistent with the demands of psychometrics. Conclusion Congenital external and middle ear malformation quality of life scale is believable and effective, which can be used for clinical practice.

OBJECTIVETo evaluate the feasibility and efficacy of enteral nutrition (EN) in patients underwent open-abdomen managements.

METHODSTwenty-one patients who received at least 3 days of EN after opening the peritoneal cavity between January 2003 and November 2006 were included in this study. Energy expenditure and actual caloric and protein intake were determined in some patients. The levels of serum protein and nitrogen balance before and after the EN were analyzed. Other related complications were also evaluated.

RESULTSAverage daily total caloric intake was 93% - 95% of estimated needs. The EN support was administered (8.8 +/- 5.5) d after opening the abdominal cavity and lasted for (51.5 +/- 33.6) d. Initial serum protein levels were low and below normal but increased in all of the patients after the EN. The average nitrogen balance was (-28.6 +/- 5.4) g/d. Diarrhea, gastric reflux, vomiting and abdominal distention occurred in 67%, 23%, 9.5% and 23% of the patients, respectively. All the complications were managed well.

CONCLUSIONSEN support could be effectively and safely given in patients requiring opening peritoneal cavity management.

Abdomen ; surgery ; Adult ; Enteral Nutrition ; Female ; Follow-Up Studies ; Humans ; Male ; Middle Aged ; Peritoneal Cavity ; surgery ; Postoperative Care ; methods ; Retrospective Studies ; Treatment Outcome

This paper reports the surface morphology and I-V curves of porous silicon (PS) samples and related devices. The observed fabrics on the PS surface were found to affect the electrical property of PS devices. When the devices were operated under different external bias (10 V or 3 V) for 10 min, their observed obvious differences in electrical properties may be due to the different control mechanisms in the Al/PS interface and PS matrix morphology.
Electrochemistry ; instrumentation ; methods ; Electrodes ; Electromagnetic Fields ; Gold ; chemistry ; Materials Testing ; Molecular Conformation ; Porosity ; Silicon ; chemistry ; Surface Properties

OBJECTIVETo investigate the diagnosis and treatment of patients with Crohn disease (CD) complicated with gastrointestinal fistulae.

METHODSClinical data of sixty-two cases with CD complicated with gastrointestinal fistula e from 1978 to 2004 were analyzed.

RESULTSThese were 68 external fistulae in 6 2 patients including recurrent fistulae in 6 cases, internal fistulae in 8 cases . Twenty- seven fistulae were located in the terminal ileum and 21 fistulae wer e located in ileocolic anastomosis site. The main surgery included 14 ileocecal resections with primary anastomosis and 26 resections of original ileocolic anastomosis with fistula and re-anastomosis. The incidence of recurrence was lower (15.4% ) in patients with postoperative medication including sulfasalazine and immunomodulator than that (34.8% ) in patients without postoperative immunomodulator,but the recurrence time was longer [(40+/- 17) months] in patients with postoperative medication than that [(8+/- 3)months] in the patients without postoperative specific medication.

CONCLUSIONSMost CD fistulae are external fistulae,most of the external fistulae are treated by resection of the fistula and anastomosis. Specific medication including sulfasalazine,mesalamine and immunomodulators should be used to prevent postoperative complications and CD recurrence.

Adult ; Crohn Disease ; complications ; diagnosis ; surgery ; Female ; Humans ; Intestinal Fistula ; complications ; diagnosis ; surgery ; Male ; Middle Aged

OBJECTIVETo observe the efficacy of polyglycoside of Tripterygium wilfordii(GTW) in preventing postoperative recurrence of Crohn disease(CD).

METHODSThirty-nine post-operative CD patients in whom all of the diseased gut had been removed from January 2005 to December 2006 were enrolled in a randomized, placebo-controlled trial. The patients took GTW(21 cases) or SASP(18 cases) in two weeks after operation. Crohn disease activity index(CDAI), ESR and CRP were collected at week 0, 13, 26, 52 or at the onset of symptoms. Ileocolonoscopy was performed at the end of the trial or at the onset of symptoms.

RESULTSOne patient in GTW group and 2 patients in SASP group were lost and 2 patients in GTW were excluded from the trial for non-compliance. Clinical recurrence was ascertained in one patient (5.6%) received GTW and in four (25.0%) received SASP. Four of eighteen patients in GTW(22.2%) had endoscopic recurrence compared with nine of sixteen(56.2%) in SASP. There were significant differences between the two groups(P<0.05).

CONCLUSIONTripterygium wilfordii showed good efficacy in preventing recurrence of postoperative CD which can maintain remission and prevent recurrence.

Adult ; Crohn Disease ; drug therapy ; Drugs, Chinese Herbal ; therapeutic use ; Female ; Glycosides ; therapeutic use ; Humans ; Male ; Middle Aged ; Phytotherapy ; Postoperative Period ; Secondary Prevention ; Treatment Outcome ; Tripterygium

OBJECTIVETo explore the surgical methods and the clinical results of chronic radiation enteritis.

METHODSTreatments were applied to forty-nine cases of chronic radiation enteritis complicated with intestinal obstruction, enterocutaneous fistula, intestinal stenosis, intestinal bleeding, severe proctocolitis and intestinal perforation, among whom 47 cases received an average of 2.8 +/- 2.1 operations. Twenty-six cases received resection of the injured segment with primary anastomosis, fourteen cases received intestinal resection and proximal enterostomy, among whom 6 ostomies were permanent, and another 8 cases received secondary ostomy closure. The injured intestinal segments were spared in 7 cases.

RESULTSForty-seven among 49 cases were cured (success rate, 96%) with no anastomotic leakage. Two patients died.

CONCLUSIONSSurgical complications of chronic radiation enteritis should be managed operatively. The operative method should be chosen according to the general condition of the patients and the complexity of the abdomen. Perioperative management and proper selection of intestinal segments for anastomosis are essential for the success.

Adult ; Aged ; Anastomosis, Surgical ; Chronic Disease ; Enterostomy ; Female ; Humans ; Inflammatory Bowel Diseases ; etiology ; surgery ; Male ; Middle Aged ; Radiation Injuries ; etiology ; surgery ; Radiotherapy ; adverse effects ; Retrospective Studies ; Treatment Outcome

OBJECTIVESTo evaluate the impairment of fenvalerate on reproductive and endocrine systems and clarify the mechanism of action.

METHODSDifferent doses of fenvalerate (0, 2, 4, 12, 60 mg/kg) were orally treated to the adult male SD rats for 15 days and 30 days, respectively. The levels of serum follicle stimulating hormone(FSH), luteinizing hormone (LH), testosterone(T) and testis homogenate T were determined by radioimmunoassay(RIA). Besides, the activity of testicular marked enzymes such as acid phosphatases(ACP) and gamma-glutamyl transpeptidase(gamma-GT) were examined, and sperm head counts were measured to explain the changes of daily sperm production(Spr).

RESULTSIn fifteen days, serum FSH levels markedly increased in rats exposed to fenvalerate of < or = 12 mg/kg groups(P < 0.01) and serum levels of LH increased in 12 mg/kg group(P < 0.01). In addition, T levels in testis homogenates decreased after treated with the doses of > or = 12 mg/kg groups compared with the control group(P < 0.01). In thirty days, serum contents of FSH were significantly elevated in the doses of > or = 12 mg/kg groups(P < 0.01) and homogenate levels of T were diminished in the low dose group(2.4 mg/kg) (P < 0.05). Activity of ACP increased in 12 mg/kg group after fifteen days(P < 0.05) and was restrained in the high dose group(60 mg/kg) in thirty days(P < 0.05), but the contents of gamma-GT were arrested with different doses dependently in the testis(P < 0.05). Fenvalerate caused dose-dependent reduction in sperm head counts and daily sperm production, which markedly reduced at the doses of > or = 12 mg/kg groups(P < 0.01).

CONCLUSIONSFenvalerate has obvious reproductive toxicity on male rats and can change their serum and testis homogenate levels of sex hormone or activity of testicular marked enzymes, which may be correlated with the impairment of Sertoli cell and spermatogenic epithelium.

Animals ; Endocrine System ; drug effects ; metabolism ; Follicle Stimulating Hormone ; blood ; Insecticides ; toxicity ; Luteinizing Hormone ; blood ; Male ; Nitriles ; Pyrethrins ; toxicity ; Rats ; Rats, Sprague-Dawley ; Reproduction ; drug effects ; Spermatozoa ; drug effects ; Testosterone ; blood

OBJECTIVETo summarize the principles of ear reconstruction after rupture of postauricular expanded flap.

METHODSThe rupture of the postauricular expanded flap was graded as I-IV degree. For flap rupture of I and II degree, the ear reconstruction was performed immediately with autogenous cartilage framework. For flap rupture of III degree, topical and systemic treatment should be done to relieve the redness and swelling. Then the ear reconstruction was performed. For flap rupture of IV degree, the expanded postauricular flap was smoothened. Ear reconstruction was performed 3 months later.

RESULTSThere were 67 cases with flap rupture, including 43 cases as I and II degree, 17 cases as III degree and 7 cases as IV degree. Ear reconstruction with autogenous cartilage framework was performed in 43 cases, with Medpor framework in 17 cases. Flap smoothing and secondary ear reconstruction with autogenous cartilage framework was performed in 7 cases. The results were satisfactory with complications in 4 cases (7%), which resolved after treatment. Primary healing was achieved in all the patients.

CONCLUSIONSGood result could be achieved in ear reconstruction, even after flap rupture. Proper treatment should be adopted according to the rupture degree.

Adolescent ; Adult ; Cartilage ; transplantation ; Child ; Child, Preschool ; Ear, External ; surgery ; Female ; Humans ; Male ; Reconstructive Surgical Procedures ; Ribs ; transplantation ; Surgical Flaps ; Tissue Expansion ; Young Adult