Objective To evaluate the usefulness of Doppler micro-probe and rigid endoscope in aneurysm surgery. Methods From April 2005 to July 2006,Doppler micro-probe and rigid endoscope were applied to 96 patients with aneurysm.The number of aneurysm was 107.The diameter of the Doppler probe was 1.5 mm,with a frequency of 20 MHz.Endoscopes with 0,30 and 70 degree were used.Doppler and endoscope were employed before and/or after aneurysm clipping. Results In all the 107 aneurysms,there were 39 in which the endoscope provided information unavailable under the microscope.The clip was adjusted after endoscope and/or Doppler examination in 19 aneurysms,10 of which were due to incomplete clipping,while the other 9 compromise of small perforators. ConclusionDoppler and endoscope are useful in aneurysm surgery,and can be applied both before and/or after clipping.

To evaluate the efficacy of manipulation therapy in repairing thin-layer and thick-layer articular cartilage defects in rabbits.

Objective To assess the efficacy and safety of ketogenic diet(KD) on refractory epilepsy in children.Methods KD treatment was designed to observe the effects for 12 weeks.Totally 22 children with 16 boys and 6 girls were enrolled in the study.The epileptic syndromes included infantile spasms(13 cases),Lennox-Gastaut syndrome(4 cases),Dravet syndrome(2 cases),and the unclassified(3 cases).The KD was prepared according to the modified Johns Hopkins regimen.Urinary ketones were measured every day to ensure that ketosis state and parents′ diaries were kept to find out when it started to work and the change of seizure frequency.Effects of KD was evaluated by Engel standard.Blood chemistry was done at baseline,4 weeks and 12 weeks to analyze the effects of KD on metabolism.Side effects were monitored and treated.Results All cases completed the KD regimen for at least 2 weeks,19 cases for at least 4 weeks,and 10 cases for at least 12 weeks.Sixteen out of 22 children experienced the seizure reduction within 3 weeks(1-15 d),especially in the first week,and seizure free appeared within 5 weeks(1-32 d) in 8 cases.Overall,the diet achieved the seizure-free in 36.4%(8/22 cases) and an over 90% of seizure frequency reduction in 22.7%(5/22 cases).The efficacy of KD seemed not correlated with the sex,age,etiopathogenisis,and syndromes and so on.Blood chemistry suggested a normal range of glucose level at 4 weeks,though higher than that at the baseline.The blood triglyceride and total cholesterol level at 12 weeks increased strikingly,even beyond the normal range compared with the baseline.The side effects mainly including transient gastrointestinal symptoms and metabolic disturbances were mostly tolerable.Conclusions KD is probably a feasible therapy on refractory epilepsy in children,with quick and high efficacy and few side effects.

The main treatment strategies for chronic pancreatitis in young patients include therapeutic endoscopic retrograde cholangio-pancreatography (ERCP) intervention and surgical intervention. Therapeutic ERCP intervention is performed much more extensively for its minimally invasive nature, but a part of patients are referred to surgery at last. Historical and follow-up data of 21 young patients with chronic pancreatitis undergoing duodenum-preserving total pancreatic head resection were analyzed to evaluate the outcomes of therapeutic ERCP intervention and surgical intervention in this study. The surgical complications of repeated therapeutic ERCP intervention and surgical intervention were 38% and 19% respectively. During the first therapeutic ERCP intervention to surgical intervention, 2 patients developed diabetes, 5 patients developed steatorrhea, and 5 patients developed pancreatic type B pain. During the follow-up of surgical intervention, 1 new case of diabetes occurred, 1 case of steatorrhea recovered, and 4 cases of pancreatic type B pain were completely relieved. In a part of young patients with chronic pancreatitis, surgical intervention was more effective than therapeutic ERCP intervention on delaying the progression of the disease and relieving the symptoms.

OBJECTIVETo study the influence of Suspension Pancreatic-Duct-Jejunum End-to-Side Continuous Suture Anastomosis (SPDJCS) on the incidence of pancreatic fistula after pancreaticoduodenectomy, and to analyze its applicability, safety, and efficacies.

METHODSA prospective controlled trial was conducted with 165 cases receiving pancreaticoduodenectomy in the Department of Hepatopancreatobiliary Surgery from January 2010 to May 2012. The patients were divided into Group A (end-to-end/end-to-side invaginated anastomosis, n=52), Group B (end-to-side mucosal anastomosis, n=48), and Group C (SPDJCS, n=65). The preoperative data, intraoperative data, and operative outcomes (incidence of pancreatic fistula, operation time, intraoperative blood loss, peritoneal drainage, peritoneal hemorrhage, peritoneal abscess, delayed gastric emptying, pulmonary infection, postoperative infection, blood transfusion, and perioperative mortality) were compared among the 3 groups.

RESULTSThe total incidence of pancreatic fistula was 13.9% (23/165) in all the 165 patients. The incidence in Group A and Group B was 23.1% (12/52) and 18.8% (9/48), both higher than that in Group C [3.1% (2/65), both P<0.05]. Group C showed significantly better outcomes than group A and B in terms of the operation time (5.5±1.2 hours vs. 6.1±1.1 hours, 5.5±1.2 hours vs. 6.3±1.5 hours), volume of blood loss (412.0±205.0 mL vs. 525.0±217.0 mL, 412.0±205.0 mL vs. 514.0±217.0 mL), and postoperative drainage amount of plasma tubes (175.0±65.0 mL vs. 275.0±80.0 mL, 175.0±65.0 mL vs. 255.0±75.0 mL) (all P<0.05), while Group A and Group B displayed no difference in these aspects (P>0.05). As complications other than pancreatic fistula were concerned, the three groups were not different from each other (P>0.05).

CONCLUSIONSSPDJCS may have the effect of reducing the incidence of pancreatic fistula after pancreaticoduodenectomy. It could be safe, practical and convenient technique of anastomosis for pancreaticojejunostomy.

Adult ; Aged ; Anastomosis, Surgical ; methods ; Female ; Humans ; Jejunum ; surgery ; Male ; Middle Aged ; Pancreatic Ducts ; surgery ; Pancreaticoduodenectomy ; adverse effects ; methods ; Prospective Studies ; Suture Techniques

Obiective:To elucidate the phenotype and the genotype-phenotype correlations in Chinese patients with X-linked adrenoleukodystrophy(X-ALD).Methods:Clinical features of 40 Chinese patients with X-ALD were studied and mutation spectrums were investigated by polymerase chain reaction (PCR) and sequencing. Results:Among these patients, four were siblings from two unrelated families, the others were unrelated. There were 31 cases with childhood cerebral (CCALD), 8 cases with adolescent cerebral (ACALD) and 1 case with adrenomyeloneuropathy (AMN). Visual impairment, which presented in 12 cases (30%), was the most common initial symptom. Nine (69%) of 13 cases who had hydrocortisone and ACTH measured showed adrenal insufficiency. By follow-up date, 19 cases (47.5%) were dead. The interval from onset to death varied from 1 to 6 years and the average were 3.3 years. The mean age at death was 10.5 years. Eleven cases (27.5%) were in vegetable state. The mean interval from onset to apparently vegetable state was 2.8 years (range from 1 to 6 years). Four cases had progressive neurological disability. Four cases were lost follow-up. One case with CCALD and one case with ACALD progressed slowly. The courses of the disease of these two patients were 5 years and 15 years respectively. Thirty five mutations were identified in 40 cases. Most were located within exon 1-3 (40%, 16/40) and exon 6-8 (42%, 17/40). There is a distinct clustering of missense mutations in exon 6 (17%, 7/40). Five types of mutations were associated with CCALD, three with ACALD and a missense mutation was identified in the patients with AMN. The two patients with long disease courses had a missence mutation c.1559 T>A and a nonsense mutation c.1785 G>A respectively. The siblings with similar manifestations and onset age were observed in two families, whose mutations were c.887 A>G and c.1028 G>T. Conclusion:The phenotypes, disease severity and rate of neurodegeneration could not be predicted by the nature of mutations.

Objective To analyze the features of diagnosis, treatment and prognosis of primary renal neuroendocrine tumors, and to improve the understanding of primary renal neuroendocrine tumors. Methods From January 2008 to June 2015, 5 cases of primary renal neuroendocrine tumors were hospitalized and their data was analyzed retrospectively, with 1 male 4 females, aged 40-73 years with the middle age of 48 years.Tumors were all located in the left kidney, whose diameters arranged from 4 to 9 cm, with an average of 6.5cm.One case presented with hematuria, one case was identified because of abdominal pain, and the other three cases were identified via physical examination.Four cases underwent a renal contrast-enhanced ultrasound, which indicated a medium-hypoechoic mass in three cases and a hyperechoic mass in one case.All five cases underwent CT scan, presenting irregularly shape and density. Calcification was found in three cases on plain scanning.Significantly heterogeneous enhancement was found in three cases and moderate heterogeneous enhancement was found in one case on enhanced scanning. Results All five cases underwent operations, with two cases undergoing radical nephrectomy and three cases undergoing partial nephrectomy.Pathological examination showed three cases of tumor cells arranged in a nest slug or ribbon-like infiltrative growth, with no or rare mitosis and no necrosis, which is consistent with renal carcinoids.Three out of 4 renal hilar lymph nodes containing tumor cells were identified in one case. The tumor cells in another case exhibited a ribbon-like arrangement, with some round nuclei, no significant atypia, and rare mitosis, which is consistent with renal atypical carcinoid.Two out of two renal hilar lymph nodes containing tumor cells were identified in this case. The tumor tissue of one case showed morphologically uniform, medium-sized cells arranged in nest slug form with necrosis, a high nuclear cytoplasm ratio, an obvious allotype and frequent mitosis, which is consistent with renal small cell carcinoma.The immunohistochemistry of the five cases indicated synaptophysin( Syn) and chromogranin A ( CgA) positive in varying degree.One case of renal carcinoid relapsed approximately 78 months after partial nephrectomy, following with radical surgery.The other two cases were followed up for 8 or 27 months and no recurrence or metastasis was detected.One case of renal atypical carcinoid was followed up for 4 months after radical nephrectomy and no recurrence or metastasis was detected.One case of renal small cell carcinoma died of multiple organ failure 11 months after radical nephrectomy plus hepatic metastatic carcinoma radio-frequency ablation approximately.Conclusions Primary renal neuroendocrine tumors are rare clinically. Renal neuroendocrine tumors may be expressed as carcinoid, atypical carcinoid or small cell carcinoma, and the clinical manifestations, pathological characteristics and prognosis varied.Primary renal carcinoids may be treated by surgery with a nice prognosis.Patients with primary renal small cell carcinoma require comprehensive treatment, and their prognosis is poor.

OBJECTIVETo compare the safety and efficiency of pacing at right ventricular outflow versus right ventricular apex.

METHODPatients were divided into two groups: pacing at ventricular cardiac apex (common pacing group, n = 22) and pacing at right ventricular outflow tract (uncommon pacing group, n = 18).

RESULTSImpedance and amplitude of R-wave were similar during implantation between the two groups (all P > 0.05). The pacing threshold and electric current were significant higher in uncommon group than those in common pacing group (all P < 0.05), however, these differences disappeared at 1 month post pacemaker implantation (all P > 0.05). The mean QRS duration tended to be shorter in uncommon pacing group compared to that in common pacing group (P > 0.05). There was no pacemaker associated adverse effect in both groups.

CONCLUSIONThe safety and efficiency of pacing at right ventricular outflow was similar as those of pacing at right ventricular apex.

Adult ; Aged ; Cardiac Output ; Cardiac Pacing, Artificial ; methods ; Electrocardiography ; Female ; Follow-Up Studies ; Heart Ventricles ; physiopathology ; Humans ; Male ; Middle Aged ; Ventricular Function, Right

A(p.G888S)were detected in POLG1 gene.Sequence analysis of parental blood DNA revealed that her father carried L83P and her mother carried G888S.Conclusions The characteristics of clinical manifestation,electrophysiology,pathology and POLG1 gene mutation of the patient were highly consistent with Alpers syndrome.The prominent white matter change and increased immunological factors in CSF were first reported in Alpers syndrome.Alpers syndrome should be considered for those patients whose liver function were severely impaired after exposure to valproic acid.

OBJECTIVELysosomal storage diseases are a group of inherited disorders caused by deficiency of lysosomal enzymes or structural components. The manifestations of lysosomal storage diseases are complicated due to different enzyme deficiency. It has been reported that a range of metabolic diseases resulting in abnormal accumulation of metabolic byproducts may exhibit abnormal cytoplasmic vacuolation of lymphocytes. The aim of this study was to elicit the usefulness of vacuolated peripheral lymphocytes detection in screening and diagnosis of lysosomal storage diseases.

METHODClinical data of 42 patients who underwent microscopic and electron microscopic examination of peripheral blood specimens in our department were retrospectively evaluated between January 2008 and December 2009.

RESULTForty-two patients with the suspected lysosomal storage diseases were included, these patients presented with motor and developmental retardation and/or regression. Seizure occurred in 32 patients. Hepatosplenomegaly were found in 4 patients. Three patients presented with declined visual acuity. Atrophy and/or abnormal signals were detected on cranial CT/MRI images in 24 patients. Blood biochemical tests were normal. Serum levels of ammonia, lactic acid and pyruvate were normal. Serum amino acid profiles and urinary organic acid profiles were normal. Serum fatty acid profiles were normal. Vacuolated lymphocytes were detected on microscopic examination of blood film in 14 patients, and 8 of these patients were confirmed to have lysosomal storage disease. Curvilinear body was found on electronic microscopic examination of peripheral lymphocytes specimens in 4 patients, confirming the diagnosis of neuronal ceroid lipofuscinosis. In 3 of these 4 patients, curvilinear body were also found on electronic microscopic examination of skin and/or muscle specimens. Enzyme analysis confirmed the diagnosis of metachromatic leukodystrophy in one patient and Pompe's disease in another patient. Typical pathological changes were found on the examination of bone marrow in 2 patients with normal acid sphingomyelinase activity. So the patients were diagnosed with Niemann-Pick disease type C. The diagnosis of other 6 patients with vacuolated lymphocytes was unknown.

CONCLUSIONBecause of its usefulness and minimal invasiveness, vacuolated peripheral lymphocytes examination should be a screening test for lysosomal storage disease. As for patients with suspected neuronal ceroid lipofuscinosis, electron microscopic examination of peripheral lymphocyte specimens may provide specific clues to the final diagnosis.

Adolescent ; Child ; Child, Preschool ; Female ; Humans ; Infant ; Lymphocytes ; pathology ; Lysosomal Storage Diseases ; blood ; diagnosis ; pathology ; Male ; Microscopy, Electron ; Retrospective Studies ; Vacuoles