Objective To explore the etiology and pathogenesis of Hirschsprung disease(HD) and the role of nitric oxide in pathophysiology of HD. Methods The whole amount preparations of dilative and transitional segments were stained by NADPH\|diaphorase histochemistry mothod,and colonic walls were digested by dispase. Then the NOS positive nervous structure were observed by light and scanning electron microscope. Results In dilative segment, under light microscope, ganglion and neuron were bigger and more darkly stained. Intraganglionic neurons distributed mostly in peripheral area of ganglion and basal part of ganglionic fiber bundles. Under scanning electron microscope, the neurons were denser, more nerve fibers from them and connected with each other in all dimensions. And more transverse connected fibers between neurons which were arranged along muscle fibers. Myenteric plexus even connected with the submucosal plexus by nerve fibers which passed through circular muscle layer. In transitional segment, under light microscope, intraganglionic neurons cytoplasm were lightly stained and also variedly. The ganglion and neurons of the segment were smaller, and the fibers from them were thinner and paler than those of the dilative segment. Under scanning electron microscope, neurons' density was lower, the fiber connection between neurons and muscle fibers/neurons was lesser. In the transitional segment, neurons and nerve fibers were distributed linearly along the longitudinal muscle fibers. Conclusion Our results indicated that an intimate relationship existed between the development of Hirschsprung disease and abnormal distribution and metabolism of NOS positive neuron in colonic wall. [

Objective To explore the clinical features,neuroimaging and histopathological findings in patients with idiopathic hypertrophic cranial pachymeningitis (IHCP) with a nodular space occupying effect.Methods Four IHCP cases with a nodular space occupying effect diagnosed in our hospital were retrospectively studied.Results All the 4 patients were men with a mean onset age of 40.25 (33 ～ 50) years old.They all had long disease duration and relapses.The common symptoms of IHCP were chronic headache,multiple cranial nerve palsies and epileptic seizures.CT and MRI of the brain revealed prominent dural partial thickening,which indicated a mass or nodular space occupying effect that mimicked intracranial tumour-like meningioma.The histopathological findings of dura in 2 cases revealed connective tissue proliferation,scattered neutrophile granulocytes and plasmacytes infiltration.Combination therapy of corticosteroid or/and immunosuppressive drugs was effective for the IHCP patients.Conclusions IHCP patients with a nodular space occupying effect usually onset with chronic headache and are often recurrent.The combination therapy of corticosteroid or/and immunosuppressive drugs is effective.The image of the brain presents prominent dural partial thickening,indicating a mass or nodular space occupying effect,which often lead to confusion with intracranial tumours or granulomatosis.

Background and purpose: Regional lymph node metastasis was significantly associated with the prognosis of patients with non-small cell lung cancer (NSCLC). This study was designed to compare paclitaxel liposome plus cisplatin (LP) with gemcitabine and cisplatin (GP) in patients with regional lymph node metastasis of advanced NSCLC as a ifrst-line treatment. Methods:A total of 55 patients were randomly assigned to receive either liposomal paclitaxel (175 mg/m2) and cisplatin (75 mg/m2) or gemcitabine (1 000 mg/m2) and cisplatin (75 mg/m2) every 3 weeks. Results:Objective response rate (ORR) of lung primary foci was 37.9%in the LP arm and 30.8%in the GP arm (P>0.05) and the disease control rate (DCR) was 91.3%and 80.8%respectively (P>0.05);ORR of regional metastasis lymph node was higher in the LP arm (44.8%vs 15.4%, P<0.05).There was no signiifcant difference in DCR (93.1%vs 73.1%, P=0.101), although slight trends favoring paclitaxel liposome were seen;There was signiifcant difference in median overall survival (17.0 vs 12.0 months, P<0.05). LP was associated with significantly less thrombocytopenia and gastrointestinal side effects (P<0.05), but no signiifcant difference was observed in hyphemia, leucopenia, hepatotoxicity, renal toxicity and allergic reactions (P>0.05). Conclusion: Liposomal paclitaxel plus cisplatin is superior to gemcitabine plus cisplatin with less toxicity and better tolerated, it deserves further research and clinic application for patients with regional lymph node metastasis of advanced NSCLC.

Objective:To explore the application value of patient data-based real-time quality control (PBRTQC) in enhancing the effectiveness of internal quality control (IQC) management.Methods:From the PBRTQC real-time quality control intelligent monitoring platform integrated with the laboratory information system (LIS), a total of 35,631 test results of red blood cell (RBC) count, white blood cell (WBC) count, and dehydroepiandrosterone sulfate (DHEA-S) were collected from patients of the Department of General Xi'an Area Medical Laboratory Center from August 1, 2023, to April 1, 2024. The platform was used in patient data distribution characteristics test, EWMA real-time quality control chart procedure establishment, performance validation, effect evaluation, best procedure selection, and real-time operation. The performance evaluation indexes of the best PBRTQC procedure establishment, the cut-off limit range, weighting coefficient, cumulative mean, standard deviation (SD), coefficient of variation ( CV) of the EWMA real-time quality control chart, and the cumulative mean, SD, and CV of its internal quality control data in the same period were counted, and at the same time compared with the quality target (1/3TEa). Coefficient of variation analyses were performed to compare the quality control status of PBRTQC and conventional internal quality control in the presence of warning or alarm prompts based on quality control process records, and alarm messages. Results:The evaluation indexes of the optimal procedures for RBC count, WBC count, and DHEA-S were the probability of error detection (Ped) between 93%-97% and greater than 90%, the false positive rate (FPR) between 0.0%-0.5%, the false negative rate (FNR) between 3.0%-7.0%, and the average number of the patient sample until error detection (ANPed) between 5-11, which is in line with the optimal quality control efficacy quality requirements for the PBRTQC procedure. The patient outcome cut-off concentrations for the optimal procedure EWMA quality control charts ranged from RBC count (3.92-5.16)×10 12/L, WBC count (4.28-7.50)×10 9/L, and DHEA-S (830-2 160) μg/L; (2 160-4 210) μg/L. The weighting coefficients were 0.05, 0.03, and 0.03, respectively. The real-world application of the EWMA real-time quality control charts showed stable and excellent analytical performance of the measurement system, such as out-of-control alarm: RBC count, 1 true alarm, Ped of 95.85%, and FPR of 0%. The cumulative CV of EWMA was less than the quality target; the cumulative CV of DHEA-S was 7.66% and 9.47%, respectively, and the cumulative CV of low level was greater than the quality target (8.33%), and the cumulative CV of high and low levels were 4.12% and 6.25%. Conclusion:The PBRTQC EWMA method can monitor the patient data - in real-time and continuous way. It can also dynamically identify and provide early indication of small changes in analytical performance during the analysis process, and can be used as a supplement to quality control products to improve the efficacy of laboratory quality management.