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Objective To summarize the clinical features of patients with Wilson's disease (WD). Methods A retrospective analysis was performed for the clinical data of 83 patients with WD who were admitted to The Third Hospital of Hebei Medical University from April 2013 to August 2021, including clinical manifestations, Imaging examinations, laboratory examinations, liver histopathological examinations, and ATP7B gene testing results. The patients were divided into groups based on different clinical types. A one-way analysis of variance was used for comparison between groups. Results The youngest age was 3 years for the 83 patients with WD, among whom 39 (46.99%) had an age of ≤18 years, with a mean age of 21.16±14.87 years for all 83 patients. Of all patients, 63.86% had liver-type WD, 31 patients (37.35%) had developed liver cirrhosis at the time of consultation, and 5 patients (6.2%) attended the hospital due to acute or acute-on-chronic liver failure. Of all patients, 62(74.69%) were positive for corneal K-F ring, and the positive rate of K-F ring was 66.04% in the patients with liver-type WD. Among the 83 patients, 79(95.18%) had a reduction in blood ceruloplasmin, and 73(87.95%) had an increase in 24-hour urine copper. The liver histopathological results of 25 patients showed varying degrees of inflammation, fibrosis, steatosis, and copper particle deposition in liver tissue. The ATP7B gene testing results of 25 patients showed that c.2333G > T/p.R778L of exon 8 was the most common mutation site. Conclusion Most patients with WD have the manifestation of liver diseases, and the examinations of corneal K-F ring, serum ceruloplasmin, and 24-h urine copper have their own limitations. Liver pathology and ATP7B gene testing can be performed when it is unable to make a confirmed diagnosis.
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Objective To investigate the clinical and pathological features of children with glycogen storage disease (GSD). Methods A retrospective analysis was performed for ten children with GSD who were admitted to the Third Hospital of Hebei Medical University and The Fifth Medical Center of Chinese PLA General Hospital from January 2002 to January 2022, based on medical history, liver biochemistry, and liver biopsy, and population characteristics, clinical manifestations, biochemical parameters, and liver histopathological characteristics were compared and analyzed. Results All ten children had developmental retardation and a short stature, with the manifestations of abnormal liver function, mild weakness, poor appetite, yellow urine, and yellow eyes, and four children had hepatosplenomegaly. Among the ten children, six had the clinical manifestations of hypoglycemia, and one had bilateral gastrocnemius hypertrophy and positive Gower sign. Two children had positive CMV IgG. Liver histopathological manifestations included diffuse enlargement of hepatocytes, light cytoplasm, and small nucleus in the middle like plant cells, with or without fibrous tissue proliferation. Conclusion Most patients with GSD have developmental retardation and abnormal aminotransferases, and liver pathological examination shows specific pathological features.
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Tumor necrosis factor-alpha-induced protein 8-like 2 (TIPE2) can negatively regulate innate immunity and adaptive immunity and participate in the development and progression of various liver diseases, such as hepatitis, liver cirrhosis, and hepatocellular carcinoma, by affecting a variety of signaling pathways. This article summarizes the current research on the involvement of TIPE2 in the pathogenesis of different liver diseases and points out that TIPE2 may become a new target for the treatment of liver diseases.
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Pulmonary arterial hypertension(PAH) leads to increased pulmonary artery pressure, pulmonary vasoconstriction, thickening of vascular wall, malignant proliferation of smooth muscle and endothelial cells, pulmonary artery occlusion and other pathological changes, which ultimately lead to right heart failure.With the advance of basic and clinical research of PAH, new progress has been made in the treatment of PAH in recent years, which improves the prognosis of patients.This article reviews the progress of drug therapy for PAH.
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Objective: To evaluate the clinical efficacy and side effects of icotinib combined with a subarachnoid space implantable pump for the treatment of leptomeningeal metastases (LM) from lung adenocarcinoma. Methods:Seven cases of LM from lung adeno-carcinoma with epidermal growth factor receptor mutation were included in the study from March 2011 to September 2015. With the aid of anesthetists, all patients were implanted with subarachnoid space implantable pumps to drain the cerebrospinal fluid (CSF) and then treated with icotinib (125 mg, three times a day) until disease progression or unacceptable toxicities. After 4 weeks, the efficacy and tolerability of icotinib were evaluated on the basis of symptoms, tumor markers from CSF, and brain gadolinium-enhanced magnet-ic resonance imaging scans. Results:Among the seven patients evaluated, no patient had complete response, two patients had partial response, four had stable disease, and one had progression. The patient who was progressive died at a month after therapy. The sur-vival time of all the other patients was more than 4 months. The common adverse effects of icotinib were skin rash and diarrhea, main-ly in grades 1 and 2. No infection of the local subarachnoid drainage tube was found in the abdominal wall. Conclusion:Icotinib com-bined with a subarachnoid space implantable pump for the treatment of LM from lung adenocarcinoma may be effective, and the tox-icities are tolerable. This method could also obviously alleviate the clinical symptoms and improve the quality of life of the patients, worthy of further study.
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Objective To investigate the effect of lamivudine (LAM) on serum interferon (IFN)-γ and interleukin (IL)-4 levels in patients with chronic hepatitis B(CHB). Methods Serum IFN-γ and IL-4 levels were measured with enzyme-linked immunosorbent assay (ELISA) in 66 CHB patients at baseline and 3, 6, 9 and 12 months after LAM treatment respectively. And 20 healthy volunteers served as healthy control. The comparision of pretreatment and post-treatment was done using t test and numberation data were analyzed by non parametric rank sum test. Results In HBeAg positive patients, the serum level of IFN-γ was (21.03±4.44) ng/L in complete response group, which was higher than partial response group [(13.85±3.92) ng/L] and non-response group [(10.63± 3.11) ng/L] (t=7.56,t=11.87, both P<0.01). Take that IFN-γ is 15.66 ng/L as boundry patients with high baseline IFN-γ level showed much higher complete response rate (31.0% vs 8.7%, x2 =8.391, P<0.01) and lower non-response rate (13.8% vs 52.2%,x2=4. 256, P<0.01) than those with low baseline IFN-γ levels. After LAM treatment, the IFN-γ/IL-4 ratios in complete response patients were approximate or even higher than those in healthy group, whereas the IFN-γ/ IL-4 ratios of patients with partial response and non response were lower than those in healthy group. In HBeAg negative patients, the IFN-γ/IL-4 ratios increased slowly but didn't reach the same levels of healthy group. Conclusions It is suggested that LAM treatment can increase IFN-γ release and reduce IL-4 release in CHB patients. The response type to LAM therapy is associated with the recovery of T helper cell (Th) 1/Th2 balance post-treatment and pretreatment IFN-γ levels.
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OBJECTIVE:To discuss the clinical significance of the expression of P- glycoprotein(P-gp),the multidrug resistance gene - 1(MDR - 1) expression product in the malignant lymphoma of nasalcavity and pharynx nasalis.METHODS: The expression of P - gp in the malignancy lymphoma of nasalcavity and pharynx nasalis of 37 cases was detected by S - P Immunohistochemical staining,and its correlations with patents' first visit,return visit,original sites,pathological type, immunohistochemical typing and prognosis were analyzed.RESULTS:Positive P- gp expression appeared in 87.5%(7/8) of the patients for return visit vs.34.5%(10/29) for first visit and 70.0%(7/10) with malignant lymphoma at pharynx nasalis vs.37.0%(10/27) with malignant lymphoma at nasal cavity,all showing significant differences.There were also significant differences in drug resistance between the patients with positive P- gp expression and those with negative P- gp expression (88.2%(15/17) vs.10.0%(2/20)).CONCLUSION:Positive P-gp expression serves as scientific basis for clinicians in predicating drug resistance and selecting drugs and establishing schemes.
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Objective To observe the change on living blood cells of peripheral bloodstream in patients with TIA.Methods To observe the behavior of living blood cells in the peripheral bloodstream of 18 patients with TIA using high magnification microscopy system, and compared with 20 healthy individuals.Results Erythrocyte aggregation rate, leukocyte activation rate, thrombocyte activation rate, and the extent of those changes were higher in the TIA group than in the control group. This difference was statistically significant ( P
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3626 cases of abdominal cystic mass wereclassified by authors. Results showed that ovarycystic mass was the most common among them.there were 2653 cases in all, accounting for73. 167%. In this paper 88 cases that were compar-atively rare were classified in details, according tooriginal tissue inflammatory cystic mass and reten-tive hydrops were the mose common, there were40 cases in all (45. 45%), congenital cystic masswas the second, 26 cases (29. 55%), developing lo-cation abserved was the most common in meson-tery, 32 cases (36. 36%), the second in abdominalcavity, and then, retroperitoneum, appendix, ete.
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Tumor necrosis factor-alpha-induced protein 8-like 2 (TIPE2) can negatively regulate innate immunity and adaptive immunity and participate in the development and progression of various liver diseases, such as hepatitis, liver cirrhosis, and hepatocellular carcinoma, by affecting a variety of signaling pathways. This article summarizes the current research on the involvement of TIPE2 in the pathogenesis of different liver diseases and points out that TIPE2 may become a new target for the treatment of liver diseases.