Abstract:
Background:Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening clinical syndrome. Central nervous system (CNS) involvement is a severe complication, which can lead to rapid disease development and higher morality. However, this has not been given enough attention in adult HLH. Therefore, we carried out this study to analyze the clinical features, laboratory findings, treatment outcomes, and other characteristics of adult HLH with CNS involvement. Methods:A retrospective analysis of 96 adult patients with HLH combined with CNS involvement between June 2003 and December 2016 was conducted. Clinical features, cerebrospinal fluid (CSF) features, image changes, and therapeutic outcomes were analyzed. Results:Among the 96 patients, 86 had various CNS symptoms and 33 (38.4%) had already presented symptoms before the HLH diagnosis was confirmed. A total of 59 patients received CSF examinations and showed abnormalities in 23 patients (39.0%). Seventy patients received imaging examinations and the results showed fifty patients with imaging changes (71.4%). Fifty-seven patients received multiple rounds of repeated intrathecal injection therapy and 35 patients improved (61.4%). As for the multiple analyses of effective factors on survival time, the results showed that the effects of combined Epstein-Barr virus (EBV) infection (P = 0.026, Exp(B) = 2.309, 95% confidence interval [CI] [1.108, 4.823) and intrathecal injection therapy (P = 0.013, Exp(B) = 0.422, 95% CI [0.214, 0.831]) on the survival time of the CNS-HLH patients were significant. Conclusions:Complication with EBV infection is a risk factor, and intrathecal injection is a protective factor. CNS involvement in HLH is not rare, which can result in a poor prognosis. Multiple rounds of repeated intrathecal injection therapy can improve the prognosis of CNS-HLH patients. :成人噬血细胞淋巴组织增多症伴中枢神经系统受累:单中心96例回顾性分析摘要背景:噬血细胞淋巴组织增多症(hemophagocytic lymphohistiocytosis,HLH)是一类威胁生命的临床综合征。中枢神经系统(central nervous system,CNS)受累是其一种严重的并发症,进展较快,死亡率增加,但在成人HLH中未得到广泛重视。因此,我们通过观察这类患者,来分析成人HLH伴CNS受累的临床表现、实验室检查、治疗预后等特点。 方法:回顾性分析2003年6月至2016年12月我中心96例HLH伴CNS受累成人患者的临床资料、脑脊液变化、影像学异常及治疗、转归。 结果:在收录的96名患者中,86名存在神经系统症状,其中33名(38.4%)在HLH确诊前即出现神经系统症状。59名患者接受脑脊液检查,其中23名(39.0%)存在异常。70名患者接受影像学检查,其中50名(71.4%)出现影像学改变。共57名患者接受多次鞘内注射治疗,其中35名(61.4%)患者情况改善。对于影响预后的多因素分析发现,合并EB病毒(Epstein-Barr virus,EBV)感染(P=0.026,Exp(B)=2.309,95% CI [1.108,4.823])及接受鞘内注射治疗(P=0.013, Exp(B)=0.422,95% CI [0.214,0.831])对生存时间的影响有统计学意义。 结论:对于成人HLH合并CNS受累的患者,存在EB病毒感染提示不良预后,而接受鞘内注射治疗则可改善预后。成人当中的HLH合并CNS受累并不少见,并且一旦出现CNS受累提示预后较差。多疗程的鞘内注射治疗可以有效改善这类CNS-HLH患者的预后。.
journal_name
Chin Med J (Engl)journal_title
Chinese medical journalauthors
Song Y,Pei RJ,Wang YN,Zhang J,Wang Zdoi
10.4103/0366-6999.228234subject
Has Abstractpub_date
2018-04-05 00:00:00pages
776-783issue
7eissn
0366-6999issn
2542-5641pii
ChinMedJ_2018_131_7_776_228234journal_volume
131pub_type
杂志文章abstract:OBJECTIVE:To evaluate the effect of gynostemma pentaphyllum mak (GP) on carcinomatous conversions of leukoplasia of the golden hamster cheek pouch induced by dimethylbenzanthracene (DMBA). METHODS:284 golden hamsters, ranging from 4 to 8 weeks of age, 80 to 100 grams in weight were randomly divided into three groups: ...
journal_title:Chinese medical journal
pub_type: 杂志文章
doi:
更新日期:1998-09-01 00:00:00
abstract:OBJECTIVE:To evaluate the clinical value of sequential subtraction scintigraphy (SSS) with 99mTc red blood cell (RBC) for the early detection of gastrointestinal (GI) bleeding. METHODS:Ninety patients referred with clinical evidence of GI bleeding underwent 99mTc-RBC scintigraphy with digital gamma camera after labeli...
journal_title:Chinese medical journal
pub_type: 杂志文章
doi:
更新日期:2001-01-01 00:00:00
abstract::To investigate the HBV infection and its replication in Chinese patients with chronic liver disease, polymerase chain reaction (PCR) was employed to detect hepatitis B virus DNA in sera of 410 patients with chronic liver disease and liver samples from 188 patients. The HBV DNA detectability in all serum samples was 58...
journal_title:Chinese medical journal
pub_type: 杂志文章
doi:
更新日期:1993-01-01 00:00:00
abstract:OBJECTIVE:To evaluate the effects of nitric oxide inhibitor on prostacyclin (PGI2) biosynthesis and the role of PGI2 in hyperhemodynamics of portal hypertension. METHODS:Sprague Dawley rats were divided into four groups: intrahepatic portal hypertension (IHPH) by injection of CCl4, prehepatic portal hypertension (PHPH...
journal_title:Chinese medical journal
pub_type: 杂志文章
doi:
更新日期:1999-06-01 00:00:00
abstract:BACKGROUND:Corneal dystrophy is a group of inherited blinding diseases of the cornea. This study was to identify the mutations of the keratoepithelin (KE) gene for proper diagnosis of corneal dystrophy. METHODS:Three families with corneal dystrophy were analysed. Thirteen individuals at risk for corneal dystrophy in f...
journal_title:Chinese medical journal
pub_type: 杂志文章
doi:
更新日期:2004-09-01 00:00:00
abstract:BACKGROUND:There are more than 300 genetic loci that have been found to be related to hereditary hearing impairment (HHI), including 92 causative genes for nonsyndromic hearing loss, among which 34 genes are related to autosomal dominant nonsyndromic HHI (ADNSHHI). Traditional linkage analysis and candidate gene sequen...
journal_title:Chinese medical journal
pub_type: 杂志文章
doi:10.4103/0366-6999.171440
更新日期:2015-12-20 00:00:00
abstract:BACKGROUND:The primary reasons for local recurrence and therapeutic failure in the treatment of malignant gliomas are the invasion and interactions of tumor cells with surrounding normal brain cells. However, these tumor cells are hard to be visualized directly in histopathological preparations, or in experimental glio...
journal_title:Chinese medical journal
pub_type: 杂志文章
doi:
更新日期:2012-12-01 00:00:00
abstract:BACKGROUND:Morphologic imaging after radiofrequency ablation (RFA) of liver metastases is hampered by an inflammatory response in the ablation margin, making the identification of local tumor progression (LTP) difficult. The aim of this study was to evaluate the efficacy of early (18)F-FDG PET/CT scanning to monitor th...
journal_title:Chinese medical journal
pub_type: 杂志文章
doi:
更新日期:2010-07-01 00:00:00
abstract:BACKGROUND:A simple, safe and effective therapeutic strategy for traumatic instability of the subaxial cervical spine, as well as its prognostic assessment, is still controversial. METHODS:The therapeutic options for 83 patients of traumatic instability of the subaxial cervical spine, whose average age was 35 years, w...
journal_title:Chinese medical journal
pub_type: 杂志文章
doi:
更新日期:2008-08-05 00:00:00
abstract:BACKGROUND:Excessive expression of thymic stromal lymphopoietin (TSLP) has been demonstrated in asthmatic airway epithelia and in nasal epithelia from animal models of allergic rhinitis (AR), but the evidence of expression of TSLP in nasal epithelial cells (NECs) of patients with AR is lacking. We aimed to investigate ...
journal_title:Chinese medical journal
pub_type: 杂志文章
doi:
更新日期:2009-10-05 00:00:00
abstract:BACKGROUND:The importance of heart rate as secondary prevention strategies for patients with coronary artery disease (CAD) is emphasized by multiple guidelines. However, limited information is available on the heart rate distribution and the change patterns of resting heart rate when initiating beta-blocker therapy amo...
journal_title:Chinese medical journal
pub_type: 杂志文章,多中心研究
doi:
更新日期:2013-01-01 00:00:00
abstract:OBJECTIVE:To focus on the asthmatic pathogenesis and clinical manifestations related to epithelial sodium channel (ENaC)/chlorine ion channel. DATA SOURCES:The data analyzed in this review were the English articles from 1980 to 2015 from journal databases, primarily PubMed and Google Scholar. The terms used in the lit...
journal_title:Chinese medical journal
pub_type: 杂志文章,评审
doi:10.4103/0366-6999.162494
更新日期:2015-08-20 00:00:00
abstract::Anti-HNK-1 monoclonal antibody (MAb) was reactive with non-cancerous and cancerous prostatic epithelia, as well as natural killer cells, myelinated nerves and cells from APUD systems. The expression of HNK-1 antigen on prostate cancer was investigated immunohistochemically by avidinbiotin-peroxidase complex (ABC) meth...
journal_title:Chinese medical journal
pub_type: 杂志文章
doi:
更新日期:1995-07-01 00:00:00
abstract:: ...
journal_title:Chinese medical journal
pub_type: 社论
doi:10.4103/0366-6999.247217
更新日期:2018-12-20 00:00:00
abstract:BACKGROUND:L-shaped incision of pronator quadratus (PQ) muscle along its radial and distal borders was always taken for distal radius fractures reduction and internal fixation. Repair of the PQ muscle was always recommended at the end of operation for some instructive reasons. But repair of PQ is not satisfied because ...
journal_title:Chinese medical journal
pub_type: 杂志文章
doi:
更新日期:2014-01-01 00:00:00
abstract:BACKGROUND:Volatile anesthetics (VAs) may affect varied and complex physiology processes by manipulating Ca(2+)-calmodulin (CaM). However, the detailed mechanism about the action of VAs on CaM has not been elucidated. This study was undertaken to examine the effects of VAs on the conformational change, hydrophobic site...
journal_title:Chinese medical journal
pub_type: 杂志文章
doi:
更新日期:2012-09-01 00:00:00
abstract::The results of 31 cases of sutureless esophagogastrostomy by intraluminal elastic circular ligation (IECL) with the biodegradable supporting tube were reported. The fate of the supporting tube could be tracked satisfactorily by X-ray. The tube-dislodge time was 15.03 +/- 2.23 days and unaffected by the size of support...
journal_title:Chinese medical journal
pub_type: 杂志文章
doi:
更新日期:1995-10-01 00:00:00
abstract:BACKGROUND:Activation of N-methyl-D-aspartate (NMDA) receptors and alpha-amino-3-hydroxy-5-methyl-4-isoxazole-propionic acid (AMPA) receptors play an important role in the neurons death induced by ischemia. The mitigating effect of intravenous anesthetics on ischemic neuron injury is related to their influence on NMDA ...
journal_title:Chinese medical journal
pub_type: 杂志文章
doi:
更新日期:2006-09-20 00:00:00
abstract:OBJECTIVE:To evaluate the safety and immunogenicity of lyophilized, live attenuated hepatitis A vaccine (H2 strain) in rhesus monkeys. METHODS:Nine adult rhesus monkeys were used as experimental animals. The rhesus monkeys without anti-HAV were divided randomly into the aqueous vaccination group (4 rhesus monkeys), th...
journal_title:Chinese medical journal
pub_type: 杂志文章
doi:
更新日期:2000-04-01 00:00:00
abstract:BACKGROUND:Irritable bowel syndrome (IBS) is reported associated with the alteration of gut microbial composition termed as dysbiosis. However, the pathogenic mechanism of IBS remains unclear, while the studies of Chinese individuals are scarce. This study aimed to understand the concept of dysbiosis among patients wit...
journal_title:Chinese medical journal
pub_type: 杂志文章
doi:10.1097/CM9.0000000000000192
更新日期:2019-04-20 00:00:00
abstract:BACKGROUND:Von Hippel-Lindau (VHL) disease is a hereditary tumor disorder caused by mutations or deletions of the VHL gene. Few studies have documented the clinical phenotype and genetic basis of the occurrence of VHL disease in China. This study armed to present clinical and genetic analyses of VHL within a five-gener...
journal_title:Chinese medical journal
pub_type: 杂志文章
doi:10.4103/0366-6999.147802
更新日期:2015-01-05 00:00:00
abstract:BACKGROUND:The tibial plateau is asymmetric with a larger medial plateau. We observed from clinical practice that the shape of the tibial plateau does not always present a larger medial plateau. Tibial plateau also showed other shapes. The purpose of this study was to analyze the anthropometric data of the proximal tib...
journal_title:Chinese medical journal
pub_type: 杂志文章
doi:
更新日期:2014-01-01 00:00:00
abstract:OBJECTIVE:To investigate molecular events of exocytosis in cultured human pheochromocytoma cells with stimulation. METHODS:The cultured pheochromocytoma cells prepared from human adrenal pheochromocytoma tumor were stimulated for the release of catecholamines by depolarization with the administration of 50 mmol/L KCl....
journal_title:Chinese medical journal
pub_type: 杂志文章
doi:
更新日期:1998-04-01 00:00:00
abstract:BACKGROUND:Allergic rhinitis (AR) is a Th2 dominant cytokine response. Chloride channel-3 (ClC-3) plays an important role in nasal mucosal edema and inflammatory pathologic changes in AR. Antiallergic herbal agents (AHA) are antiallergic herbal products. In the previous study, we have demonstrated that AHA clearly inhi...
journal_title:Chinese medical journal
pub_type: 杂志文章
doi:
更新日期:2010-04-20 00:00:00
abstract::Three cases with disseminated Penicilliosis marneffei (PSM) are reported. They are all natives of Guangxi Zhuang Autonomous Region. Among them, 2 died and 1 remains alive. Their clinical features were characterized by multiple organ involvement, multiple subcutaneous abscesses, inflammatory papules, nodules and pustul...
journal_title:Chinese medical journal
pub_type: 杂志文章
doi:
更新日期:1991-03-01 00:00:00
abstract:OBJECTIVE:To explore the immune promotion mechanism of retinoic acid (RA). METHODS:Reverse Transcription-Polymerase chain reaction (RT-PCR) was used to detect the gene expression of insulin like growth factor I (IGF-I) and IGF receptors (IGF-IR and IGF-IIR) in vitro, and their regulation by RA in human cord blood lymp...
journal_title:Chinese medical journal
pub_type: 杂志文章
doi:
更新日期:2001-02-01 00:00:00
abstract:BACKGROUND:Restrictive cardiomyopathy (RCM) is the least common cardiomyopathy in which the walls are rigid and the heart is restricted from stretching and filling properly. Cardiac troponin I (cTnI) mutation-caused myofibril Ca2+ hypersensitivity has been shown to be associated with impaired diastolic function. This s...
journal_title:Chinese medical journal
pub_type: 杂志文章
doi:10.4103/0366-6999.219150
更新日期:2017-12-05 00:00:00
abstract:OBJECTIVE:To determine antiganglioside antibodies (AGA) in cerebrospinal fluid (CSF) of children with neuropsychiatric lupus erythematosus (NPLE), and to investigate their association with clinical manifestations and other laboratory tests. METHODS:An enzyme linked immunoabsorbent assay was used to detect AGA in 22 CS...
journal_title:Chinese medical journal
pub_type: 杂志文章
doi:
更新日期:1997-08-01 00:00:00
abstract:BACKGROUND:As an X-linked recessive way, arginine vasopressin receptor 2 (AVPR2) gene mutation resulted in a hereditary disease - congenital nephrogenic diabetes insipidus (CNDI). We found a suspect clinical CNDI pedigree. In order to identify the genetic etiology, we performed the genetic analysis. METHODS:The clinic...
journal_title:Chinese medical journal
pub_type: 杂志文章
doi:
更新日期:2014-01-01 00:00:00
abstract:OBJECTIVE:To investigate the use of the Gibbs-Sampler method in evaluating the relationship between clinic events and health risks in a meta-analysis of multiple clinical trials. METHODS:By using a generalized linear model with random-effects, Gibbs-Sampler technique was used in a meta-analysis of multiple clinical tr...
journal_title:Chinese medical journal
pub_type: 杂志文章
doi:
更新日期:2000-06-01 00:00:00