2.POEMS syndrome associated with multicentre Castleman's disease and thrombocytosis: a case report
Xu CHEN ; Mei JU ; Xiulian XU ; Juanqin GONG ; Lin LIN ; Heng GU
Chinese Journal of Dermatology 2012;45(4):259-262
A rare case of POEMS(polyneuropathy,organomegaly,endocrinopathy,M-band,skin changes) syndrome associated with multicentre Castleman's disease and thrombocytosis is reported in a 48-year-old man.Three years prior to the presentation,he developed a series of symptoms without obvious triggers,including persistent myalgia and myasthenia of extremities,abnormal skin sensation (burning),gradually increased roughness and pigmentation of skin,thickening and mild induration of lower-extremity skin giving an ichthyosiform appearance.Two years prior to the presentation,electromyography indicated peripheral neuropathy,and computed tomography(CT) revealed hepatomegaly,splenomegaly,lymphadenectasis,ascites,hydrotborax and hydropericardium.Multiple biopsies of lymph nodes indicated an obvious vascular proliferation between lymphoid follicles with the involvement of lymphoid follicles,which,together with the histochemical findings and special staining results,was suggestive of hyaline-vascular type Castleman's disease.Three months prior to the presentation,painful erythema emerged and gradually spread in both thighs,with a progressive deepening in the colour of central lesions,and flushing of periphery lesions.Two months prior to the presentation,a soybean-sized nodule arised in the erythema on the right inner thigh,which was then ulcerated with a little pus left and a shallow ulcer formed.Physical examination revealed a mild atrophy of lower-extremity muscles,with muscle strength graded as 5 for bilateral upper extremities and as 4 for bilateral lower extremities.The skin was rough,diffusely pigmented and thickened,giving an ichthyosiform appearance,which was more apparent in the extremities than in the trunk.An atropurpureus patch sized 5 cm × 2 cm was seen respectively in bilateral inner thighs; the surface of the central lesions was coarse and covered with dry crusts and scales,giving a rough paperlike appearance,and the peripheral lesions were surrounded by a faint prunosus halo,which faded on pressure.Laboratory examination found hypothyroidation,reduced insulin secretion,abnormal calcium and phosphorus metabolism,and progressive thrombocytosis.Immunofixation electrophoresis showed that the serum was weakly positive for IgG type M protein.This patient was diagnosed with POEMS syndrome associated with multicentre Castleman's disease and thrombocytosis.After treatment with methylprednisolone and thalidomide,the main symptoms of POEMS syndrome were improved,whereas thrombocytosis progressively aggravated.
3.Infantile rhabdomyofibrosarcoma.
Hong-feng TANG ; Tian-lin WANG ; Wei-zhong GU ; Long LIN ; Min-ju LI
Chinese Journal of Pathology 2005;34(9):607-608
Actins
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metabolism
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Antineoplastic Combined Chemotherapy Protocols
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therapeutic use
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Back
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Desmin
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metabolism
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Diagnosis, Differential
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Fibrosarcoma
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pathology
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Humans
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Infant
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Male
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Neoplasm Recurrence, Local
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Reoperation
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Rhabdomyosarcoma
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drug therapy
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pathology
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surgery
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Vimentin
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metabolism
4.Effects of calponin-1 gene silencing on the biological behavior of uterine smooth muscle cells.
Yong-hong GU ; Chang-ju ZHOU ; Lin-yu HU ; Qian CHEN ; Wei-she ZHANG
Journal of Southern Medical University 2010;30(6):1369-1372
OBJECTIVETo study the effects of calponin-1 expression inhibition on the proliferation , invasiveness, apoptosis and cytoskeleton of uterine smooth muscle cells, and explore the molecular mechanism of calponin-1 in the uterine smooth muscle cells for labor onset.
METHODSsiRNA-calponin-1 adenovirus plasmid was constructed and transfected into primarily cultured uterine smooth muscle cells. The proliferation, invasiveness and apoptosis of the cells were determined by MTT assay, matrigel invasion assays and flow cytometry, respectively. Rhodamine-Phalloidin was used for labeling filamentous actin (F-actin), and the morphology and the distribution of F-actin was observed under fluorescence microscopy and analyzed quantitatively.
RESULTSThe motor ability of uterine smooth muscle cells decreased significantly after transfection with siRNA-calponin-1 adenovirus plasmid (P<0.05). The transfected cells showed thinner, loosened and irregular F-actin microfibers, and the cells in the empty vector and blank control groups showed thicker and longer F-actin microfibers.
CONCLUSIONInhibition of calponin-1 expression can inhibit uterine smooth muscle cell migration and cause the morphological change and rearrangement of F-actin without affecting its proliferation and apoptosis in vitro, suggesting that the morphological change and rearrangement of F-actin of uterine smooth muscle cell may be one of the important mechanisms in the labor onset.
Apoptosis ; Calcium-Binding Proteins ; genetics ; Cell Movement ; Cell Proliferation ; Cells, Cultured ; Female ; Gene Silencing ; Humans ; Microfilament Proteins ; genetics ; Myocytes, Smooth Muscle ; cytology ; metabolism ; RNA Interference ; RNA, Small Interfering ; genetics ; Uterus ; cytology ; metabolism
5.T3/T4 thoracic sympathictomy and compensatory sweating in treatment of palmar hyperhidrosis.
Jie YANG ; Jia-Ju TAN ; Guo-Lin YE ; Wei-Quan GU ; Jun WANG ; Yan-Guo LIU
Chinese Medical Journal 2007;120(18):1574-1577
BACKGROUNDCompensatory sweating (CS) is one of the most common postoperative complications after thoracic sympathectomy, sympathicotomy or endoscopic sympathetic block (ESB) for palmar hyperhidrosis. This study was conducted to examine the relevance between CS and the sympathetic segment being transected in the surgical treatment of palmar hyperhidrosis, and thus to detect the potential mechanism of the occurrence of CS.
METHODSBetween October 2004 and June 2006, 163 patients with primary hyperhidrosis were randomly divided into two groups, T(3) sympathicotomy (78 patients) and T(4) sympathicotomy (85), who were operated upon under general anesthesia via single lumen intubation and intercostal video-mediastinoscopy (VM).
RESULTSNo morbidity or mortality occurred. Palmar hyperhidrosis was cured in all patients. Follow-up (mean (13.8 +/- 6.2) months) showed no recurrence of palmar hyperhidrosis. The difference of rates of mild CS in groups T(3) and T(4) was of no statistical significance. The rate of moderate CS was significantly lower in group T(4) than in group T(3). No severe CS occurred.
CONCLUSIONThe rates of occurrence and severity of CS are lowered with the lower sympathetic chain being transected.
Adolescent ; Adult ; Female ; Follow-Up Studies ; Humans ; Hyperhidrosis ; surgery ; Male ; Middle Aged ; Postoperative Complications ; etiology ; Prospective Studies ; Sweating ; Sympathectomy ; adverse effects ; methods ; Thoracic Surgery, Video-Assisted
6.Clinicopathological analysis of cutaneous natural killer/T cell lymphoma: 36 case report
Jiaosheng XU ; Min LI ; Xin HUANG ; Yong-hong ZHANG ; Chun-ju ZHOU ; Xue-min XUE ; Ze-jun DUAN ; Lin SUN ; Cui-ling LIU ; Cong-you GU ; Fang AN ; Zi-fen GAO
Chinese Journal of Dermatology 2011;44(9):611-614
ObjectiveTo investigate the clinicopathological features and prognosis of natural killer (NK)/T cell lymphoma and to analyze its relationship with Epstein-barr virus(EBV). MethodsTotally, 36 cases of cutaneous NK/T cell lymphoma were collected from 2000 to 2010 at the Department of Pathology, Peking University Health Science Center, and classified into primary and secondary groups according to whether there is evidence of extracutaneous involvement within 6 months after diagnosis. Clinicopathological features were analyzed and Epstein-barr virus (EBV) was detected. ResultsOf these 36 cases, 13 (36.1%) were classified as primary cutaneous NK/T cell lymphoma, 20 (55.6%) as secondary, and 3 (8.3%) remained unclassified because of the lack of clinical data. Males were more likely to develop both primary and secondary cutaneous NK/T cell lymphoma than females, but there was no striking difference in sex ratio between the patients with primary and secondary lymphoma (P > 0.05 ). Compared with the patients with primary cutaneous NK/T cell lymphoma, those with secondary cutaneous NK/T cell lymphoma showed a younger median age at onset(43.5 vs. 54 years, P < 0.05), higher prevalence of B symptoms(including fever, night sweat, body weight loss) and multiple skin lesions (P < 0.05 and 0.01, respectively). EBV was positive in 92.3% (12/13) of the primary lymphoma cases and 85%(17/20) of the secondary lymphoma cases. Moreover, the median survival was 8 months in all the cutaneous NK/T cell lymphoma cases, and was significantly shorter in secondary cases than in the primary cases(6 vs. 18 months, x2 = 6.074, P < 0.05). ConclusionsCutaneous NK/T cell lymphoma is an EBV-associated, clinica]ly aggressive disease entity. Patients with primary cutaneous NI/T cell lymphoma seem to have an older age at onset and a better prognosis as compared with those with secondary cutaneous NK/T cell lymphoma.
7.Intercostal video-mediastinoscopy: a report of 701 cases.
Jie YANG ; Jia-ju TAN ; Jun WANG ; Guo-lin YE ; Wei-quan GU ; Jun YE ; Le-wei ZHU
Chinese Journal of Surgery 2007;45(22):1524-1526
OBJECTIVETo summarize the experience of intercostal video-mediastinoscopy (VMS) in treatment for mediastinal masses, malignant pleural effusion and palmar hyperhidrosis.
METHODSThe clinical data of 701 patients received intercostal VMS from November 2001 to June 2007 were summarized retrospectively. Forty-eight patients with mediastinal masses and 46 patients with suspected malignant pleural effusion underwent intercostal VMS pleural biopsy (39 cases with talc pleurodesis) and 607 patients with palmar hyperhidrosis underwent bilateral intercostals VMS thoracic sympathectomy.
RESULTSNo mortality and morbidity were reported in this group. Definitive pathologic diagnosis had been made through VMS mediastinal masses biopsy in mediastinal masses and pleural biopsy in pleura effusion. The efficiency of talc pleurodesis was 100% for 39 cases. The symptoms of sweating of hands in 607 patients with palmar hyperhidrosis disappeared completely, all patients' hands became dry with a 1.5 degrees C to 3.0 degrees C increase of the skin temperature immediately after operation. No recurrence occurred during the follow-up.
CONCLUSIONVMS is a simple, convenient and alternative procedure for the treatment of mediastinal masses, malignant pleural effusion and palmar hyperhidrosis.
Adolescent ; Adult ; Aged ; Child ; Female ; Follow-Up Studies ; Humans ; Hyperhidrosis ; surgery ; Male ; Mediastinal Neoplasms ; diagnosis ; surgery ; Mediastinoscopy ; methods ; Middle Aged ; Pleural Effusion, Malignant ; diagnosis ; surgery ; Pleurodesis ; methods ; Retrospective Studies ; Sympathectomy ; methods ; Thoracic Surgery, Video-Assisted ; methods ; Treatment Outcome
8.Surveillance of hemagglutinin genetic variation of influenza viruses in Shanghai and Wuxi
Lu-Fang JIANG ; Li-Wen JU ; Hai-Yong GU ; Hui-Guo SHEN ; Yan-Ting LI ; Jian CHEN ; Feng-Xing YOU ; Lian-Di ZHOU ; Yu-Zun LIN ; Qing-Wu JIANG
Chinese Journal of Infectious Diseases 2001;0(06):-
Objectives To explore the type and subtype distribution of influenza viruses in influenza-like patients and the hemagglutinin(HA)genetic variation of influenza A viruses in Shang- hai and Wuxi during the influenza prevalent season from 2004 to 2006.Methods Throat swabs were collected from the influenza-like patients in the sentinel hospitals and during the outbreaks,and then inoculated into MDCK cells to isolate influenza viruses,which were subsequently identified by direct immunofluorescence(DIF)and reverse transcription-polymerase chain reaction(RT-PCR).HA seg- ments of influenza A viruses were sequenced to analyze the genetic variation of HA.Results One hundred and twenty-six strains of influenza viruses,including 53 H3N2,43 H1N1 and 30 influenza B viruses were isolated from August 2004 to September 2006,and 7 outbreaks.All these outbreaks oc- curred in February or March The pathogens were identified as H1N1 in one outbreak,H3N2 in two outbreaks,B in two outbreaks and mix infections in two outbreaks(1 H1N1 and B,1 H3N2 and B, respectively).By sequencing the HA segment,the H3 and H1 segments were all homologous to the isolates from different countries in the same period.Conclusion H3N2 and H1N1 are the major strains prevalent in Shanghai and Wuxi,which reach the peak from January to March No HA and NA recom- binant strains and new HA and NA subtypes are found in these areas.The variations of H1 and H3 are similar to those found in other countries.
9.Correlation between birth defects and dietary nutrition status in a high incidence area of China.
Bao-Yuan ZHANG ; Ting ZHANG ; Liang-Ming LIN ; Fang WANG ; Ruo-Lei XIN ; Xue GU ; Yu-Na HE ; Dong-Mei YU ; Pei-Zhen LI ; Qing-Shan ZHANG ; Jin ZHAO ; Yu-Fu QIN ; Xiu-Feng YANG ; Gong CHEN ; Ju-Fen LIU ; Xin-Ming SONG ; Xiao-Ying ZHENG
Biomedical and Environmental Sciences 2008;21(1):37-44
OBJECTIVETo investigate the association between birth defects and dietary nutrient intake in a high risk area of China.
METHODSA dietary survey was performed and serum folic acid was measured in women whose pregnancy was affected by neural tube defects (NTDs) or unaffected by any birth defects (BDs) in Zhongyang and Jiaokou Counties in Shanxi Province of China.
RESULTSThe local average consumption of foods including dark green vegetables, fruits, fat and meat, and nutrient intake (e.g., energy, protein, retinol, riboflavin, vitamin E, and selenium) were lower than the national average level. In women of childbearing age, these regions, the intake of nutrients was much lower than the recommended nutrient intake (9%-77%). The case-control dietary nutrition study of women whose pregnancy was affected by BDs (including NTDs and congenital heart defects) demonstrated that, in early pregnancy, adequate nutrition (i.e., eating meat, fresh vegetables, fruit more than once a week) was a protective factor, while eating germinated potatoes was a risk factor. The geometrical mean (p5-p95) of serum folic acid in women with NTD birth defects was 9.6 nmol/L (3.6, 23.03), which was significantly lower than that in normal women (14.03 nmol/L).
CONCLUSIONWomen of childbearing age in the two counties of Shanxi Province, China, have a marked insufficient intake of some nutrients, especially folic acid, zinc, vitamins A and B12. This nutrient deficiency may be an important risk factor for the high prevalence of birth defects in these regions. Therefore, adequate dietary nutrition in early pregnancy can prevent BDs.
Adolescent ; Adult ; Aged ; Case-Control Studies ; China ; epidemiology ; Female ; Folic Acid ; blood ; Humans ; Incidence ; Middle Aged ; Neural Tube Defects ; epidemiology ; Nutritional Status ; Surveys and Questionnaires
10.Unusual patterns of neural tube defects in a high risk region of northern China.
Gong CHEN ; Li-Jun PEI ; Jian HUANG ; Xin-Ming SONG ; Liang-Ming LIN ; Xue GU ; Jian-Xin WU ; Fang WANG ; Ji-Lei WU ; Jia-Peng CHEN ; Ju-Fen LIU ; Ruo-Lei XIN ; Ting ZHANG ; Xiao-Ying ZHENG
Biomedical and Environmental Sciences 2009;22(4):340-344
OBJECTIVETo study the prevalence of different types of neural tube defects (NTDs) in Luliang Prefecture, Shanxi province, where the prevalence of NTDs is unusually high and the correlation between NTDs prevalence and patterns.
METHODSA surveillance population-based birth defects was performed in Luliang Prefecture, Shanxi province.
RESULTSThe results of our study showed that the prevalence of NTDs was 2-fold higher in Luliang Prefecture than in other areas of Shanxi province. Unusual patterns of NTDs were found, however, multiple NTDs were relatively common in Luliang Prefecture, accounting for over 13% of all NTDs cases in China.
CONCLUSIONThe prevalence of NTDs is associated with its patterns.
Child, Preschool ; China ; epidemiology ; Humans ; Infant ; Infant, Newborn ; Neural Tube Defects ; classification ; epidemiology ; Risk Factors