Volume 96  ·  Issue 47  ·  2016

In recent years, precision medicine has attracted much attention^[1]。 As a new strategy in the field of healthcare, precision medicine aims to improve personalized diagnosis, medical decision-making and clinical prognosis, that is, to provide precision medicine services such as reasonable treatment plans and drug dosages at the right time^[2,3]。 In the implementation of precision medicine, it is very important to integrate technologies such as genomics, computer biology analysis, clinical informatics, and disease-specific dynamic markers^[4,5]。 Especially for patients with cardiovascular diseases, combining biomarkers, understanding the information of epigenetics, metabolomics and clinical characteristics of patients will greatly improve the prognosis of patients^[6]。

vascular mild cognitive impairment (VaMCI) is a mild cognitive impairment syndrome caused by or related to vascular causes, which is a transitional phase between normal and dementia^[1]。 Due to our unique linguistic and cultural background and the differences in the understanding of international diagnostic criteria, the criteria, thresholds and tools related to VaMCI diagnosis are still not widely agreed^[2]。 Therefore, the Alzheimer's Disease Chinese Working Group on Guidelines (ADCWG) of the Chinese Geriatric Health Care Association published the Chinese Guidelines for the Diagnosis of Vascular Mild Cognitive Impairment (Chinese VaMCI Diagnostic Guidelines)^[1]。 Several key issues covered in the Guide are interpreted below.

To analyze the difference of dosimetric parameters between pre-plan and post-plan of ¹²⁵I radioactive seed implantation assisted by 3D printing individual non-coplanar template (3D printing template) for locally recurrent rectal cancer (LRRC).

From February 2016 to April 2016, a total of 10 patients with locally recurrent rectal cancer received ¹²⁵I seeds implantation under CT guidance assisted by 3D printing template in Department of Radiation Oncology, Peking University Third Hospital.Each patient underwent CT simulation, three-dimentional treatment planning pre-implantation, 3D printing template design, radioactive seed implantation assisted by 3D printing template and dosimetric verification post implantation. The median activity of seed was 0.63 mCi (0.58 to 0.7 mCi) (2.15- 2.59×10⁷ Bq), and the median number of seeds was 80 (19 to 192). D₉₀, D₁₀₀, V₁₀₀, V₁₅₀, CI, EI, HI, D_5cc, D_2cc of bladder and bowel of pre-plan and post-plan were calculated, respectively.Paired t test was used to evaluate the difference of dosimetric parameters between pre-plan and post-plan.

The median D₉₀ of pre-plan and post-plan were 13 761.0 and 12 798.8 cGy, respectively.The median D₁₀₀ of pre-plan and post-plan were 5 293.6 and 5 397.9 cGy, respectively.The median V₁₀₀ of pre-plan and post-plan were 90.0% and 90.0%, respectively.The median V₁₅₀ of pre-plan and post-plan were 63.8% and 62.4%, respectively.The median CI of pre-plan and post-plan were 0.73 and 0.67.The median EI of pre-plan and post-plan were 0.22 and 0.30, respectively. The median HI of pre-plan and post-plan were 0.29 and 0.31.The median bladder D_2cc of pre-plan and post-plan were 3 088.8 and 4 240.4 cGy, respectively.The median bowel D_2cc of pre-plan and post-plan were 7 051.6 and 7 903.9 cGy, respectively.

3D printing template might be helpful for locally recurrent rectal cancer patients who received ¹²⁵I radioactive seed implantation assisted by 3D printing individual template.Seed implantation might have more chances to achieve prescription dose and dose limitation of organs at risk of pre-plan, which is important for precise implantation and quality control.

To investigate the brain function changes in cirrhosis patients after transjugular intrahepatic portosystemic shunt (TIPS), resting-state functional MRI (rs-fMRI) performed and fractional amplitude of low frequency fluctuation (fALFF) was analyzed.

From January 2014 to February 2016, a total of 96 cirrhotic patients from invasive technology department and infection department in the First Affiliated Hospital of Anhui Medical University were selected , the blood ammonia data of 96 cirrhotic patients with TIPS operation in four groups were collected after 1, 3, 6 and 12 month, and all subjects performed rs-fMRI scans. The rs-fMRI data processed with DPARSF and SPM12 softwares, whole-brain fALFF values were calculated, and One-Way analysis of variance , multiple comparison analysis and correlation analysis were performed.

There were brain regions with significant function changes in four groups patients with TIPS operation after 1, 3, 6 and 12 month, including bilateral superior temporal gyrus, right middle temportal gyrus , right hippocampus, right island of inferior frontal gyrus, left fusiform gyrus, left olfactory cortex, left orbital superior frontal gyrus (all P<0.005). Multiple comparison analysis showed that compared with patients in the 1-month follow-up, patients in the 3-month follow-up showed that brain function areas increased in left olfactory cortex, left inferior temporal gyrus, left fusiform gyrus, left orbital middle frontal gyrus, left putamen, left cerebelum, and decreased in left lingual gyrus; patients in the 6-month follow-up showed that brain function areas increased in left middle temportal gyrus, right supramarginal gyrus, right temporal pole, right central operculum, and decreased in left top edge of angular gyrus, left postcentral gyrus; patients in the 12-month follow-up showed that brain function areas increased in right hippocampus, right middle cingulate gyrus, and decreased in right middle temportal gyrus.Compared with patients in the 3-month follow-up, patients in the 6-month follow-up showed that brain function areas increased in left superior temporal gyrus, left middle temporal gyrus, right temporal pole, right island of inferior frontal gyrus, and decreased in left cerebelum, left orbital inferior frontal gyrus; patients in the 12-month follow-up showed that there were no obvious increase and decrease brain function areas.Compared with patients in the 6-month follow-up, patients in the 12-month follow-up showed that there were no obvious increase brain function areas , but brain function areas decreased in bilateral middle temportal gyrus(P<0.001). Brain regions were positively related to blood ammonia in right middle cingulate gyrus, right central operculum, left parahippocampal gyrus, while as brain regions were negatively related to blood ammonia in bilateral medial prefrontal lobe, anterior cingulate and paracingulate gyrus, right top edge of angular gyrus, right middle temportal gyrus, left anterior central gyrus, left posterior central gyrus (allP<0.005).

The resting state brain function increased or decreased with course of disease in cirrhosis patients after TIPS operation. The brain activity of limbic system and sensorimotor system all had significant correlation with blood ammonia levels. The blood ammonia level and the function of relative brain regions after 6-month with TIPS operation can be gradually improved.

To identify the Androgen Receptor (AR) gene mutation of one family with complete androgen insensitivity syndrome (CAIS) and to establish the methods of prenatal genetic diagnosis for CAIS.

The AR gene exons of the family were amplified by PCR and sequenced directly. Linkage analysis was performed by using the CAG repeats in the exon1 of AR gene to assure accuracy of the prenatal diagnosis.

We found a frameshift mutation c. 2546del A (p. Asn849Ile fsX34) in the exon7 of AR gene in the proband.The mutation had not been reported before.The mother of the proband went through two times prenatal genetic diagnosis for her next pregnancies, both fetuses were male and did not get the mutation.The results of the linkage analysis were consistent with the sequencing results.

A novel AR mutations in a CAIS family have been confirmed. The method of prenatal genetic diagnosis was established, and worked effectively in the CAIS family.

To explore the clinical and scalp video electroencephalographic(EEG) characteristics of insula lobe epilepsy identified by intracranial electrode, and to provide some references for early diagnosis and clinical evaluation in patients with insula lobe epilepsy.

A total of 12 patients diagnosed with insula lobe epilepsy identified by intracranial electrode were included from Xuanwu Hospital of Capital Medical University from January 2013 to December 2015.Their clinical and EEG data were analyzed.

91.7% of patients had sensory aura before seizure, and had clear consciousness at onset initial time.Visceral motor and visceral sensation were the most common symptoms, and the epilepsy was always ended with somatic movement.There was no prominent characteristic changes in scalp EEG.

Clinical features are important for insula lobe epilepsy diagnosis.When patients present symtoms aforementioned, insula lobe epilepsy should be considered.

To compare the outcomes between anterior cervical discectomy and fusion (ACDF) and posterior laminectomy and fusion(LF) for multilevel cervical spondylotic myelopathy combined with cervical kyphosis.

From January 2010 to June 2014, 54 patients with cervical spondylotic myelopathy combined with cervical kyphosis underwent surgical treatment.Among them, 29 patients were underwent ACDF, and 25 patients were underwent LF in Department of spine surgery, Tianjin Union Medical Centre. The operation time, intraoperative blood loss, fusion segments, Japanese Orthopaedic Association (JOA)score, Neck Disability Index (NDI), Visual Analog Scale (VAS), change of cervical curvature, range of motion(ROM)and complications were recorded and compared between the two groups.

Mean operative time was (162.7±21.3)min in the anterior approach group versus (176.3±29.8)min in the posterior group(P>0.05). Mean intraoperative blood loss was (135.6±27.8)ml in the anterior approach group and (255.2±32.3)ml in the posterior approach group(P<0.05). Mean fusion levels are (4.1±0.3)in the anterior approach group and (5.3±0.5) in the posterior approach group(P<0.05). The mean preoperative JOA score were(8.3±2.7)in the anterior approach group and( 8.9±2.1) in the posterior approach group (P>0.05). Mean postoperative JOA score were(13.6±2.5) in the anterior approach group and (14.0±1.7)in the posterior approach group at final follow-up(P>0.05). Mean improvement rate was (55.7%±16.3%)in the anterior approach group and (58.3%±15.7%) in the posterior approach group (P>0.05). Mean preoperative NDI score were(33.8±11.0)in the anterior approach group and (34.4±8.7)in the posterior approach group (P>0.05). Mean postoperative NDI score were (16.9±7.5) in the anterior approach group and (15.5±8.1) in the posterior approach group at final follow-up (P>0.05). Mean VAS score were (2.9±1.5) in the anterior approach group and (2.5±1.0) in the posterior approach group before operation(P>0.05), they are improved to (1.2±1.2) and (1.2±1.3), respectively(P>0.05). Mean Cobb angle of the operative site were (-24.3±4.4)°in the anterior approach group and (-22.7±3.7)° in the posterior approach group before operation(P>0.05). At final follow-up, the Cobb angle of the operative site were (13.7±3.2)°in the anterior approach group and (6.2±4.2)° in the posterior approach group(P<0.01). Mean preoperative ROM were (29.0±6.7)°and (30.4±5.4)° in the anterior approach group and posterior approach group, respectively(P>0.05). Mean postoperative ROM were (11.7±6.5)° and (8.2±5.9)°in the anterior approach group and the posterior approach group, respectively(P<0.05). There were 16 patients with complications in the anterior approach group and 7 patients with complications in the posterior approach group(P<0.05).

For multilevel cervical spondylotic myelopathy combined with cervical kyphosis, ACDF can restore the lordosis better, fuse less levels but have more complications compared with LF. Patients treated with LF can get as good life quality as with ACDF and have less complications although fuse more levels compared with ACDF.

To evaluate the efficacy of percutaneous vertebroplasty(PVP) combined with postoperative radiotherapy and radiotherapy alone in the treatment of spinal metastatic tumors and to evaluate the prognostic factors for survival.

From December 2011 to December 2015, according to the choice of treatment, patients in group A(60 cases) were treated with PVP combined with postoperative radiotherapy and those in group B(50 cases) underwent radiotherapy alone, age, sex, primary tumor type , and other basic characteristics were analyzed in both groups in department of orthopedics and radiotherapy department, 307 Hospital of the People′s Liberation Army. The pain visual analogue scale(visual analogue scale, VAS), tumors of the spine instability score(the spinal instability neoplastic score and sins), physical status score(Karnofsky performance score and KPS) were used to evaluate pain, spinal stability improvement and physical condition. Kaplan-Meier was used to analyze the survival rates of two groups of patients and the influence of primary tumor types on the survival of patients; Cox proportional hazard model was used to calculate the correlations between survival and visceral metastases, system medical treatment, vertebral number before treatment and physical condition.

There was no significant difference in baseline data between the two groups(P>0.05). The VAS in the group A was significantly lower than the scores in the group B at 1 month, 3 months, 6 months, and 12 months after surgery. The SINS score dropped from(7.8±1.2) to(6.3±0.9)(1 month), (6.1±0.8)(3 months) in patients with PVP combined with postoperative radiotherapy(P<0.05), the SINS score of radiotherapy patients simply dropped from(7.6±0.9) to(7.4±0.7)(1 month), (7.3±0.6)(3 months), and there was no statistically significant difference(P=0.12). The survival rates of 6 months, 1 years, and 3 years were similar between two groups(P>0.05). The influence of different types of primary tumors on the survival time of the patients was statistically significant(P<0.05). Multiple analysis showed that the internal organs metastasis, systemic medical treatment, the number of vertebral bodies and the physical condition were the important prognostic factors of the survival in patients with spinal metastases.

PVP combined with postoperative radiotherapy for spinal metastases is better than radiotherapy alone in the treatment of relieving pain, maintaining the stability of vertebral body and improving the quality of life of patients. Survival prognosis was similar in two groups. The types of primary tumors, visceral metastasis, systemic medical treatment, the number of vertebral bodies and the physical condition are important prognostic factors in the survival of patients with spinal metastases.

To evaluate the medial anterior flange overhang related to the femoral prosthesis size determined by femoral sizer in total knee arthroplasty.

Sixty one cases of varus knee osteoarthritis (61 knees) were analyzed from January 2014 to January 2015, 14 males and 47 females, aged (66.8±6.8) year old. 3D models of distal femur were established by Mimics, total knee arthroplasty surgeries were simulated and the medial and lateral femoral condylar anteroposterior diameter were measured. According to the AP diameter of lateral femoral condyle (lateral condyle group) and femoral sizer (sizer group), different size of the PFC Sigma femoral prosthesis models were imported to mimics, and the coverage of medial anterior femoral flange were compared.

The AP diameter of lateral femoral condyle[(55.76±3.64) mm]was significantly less than the sizer group[(57.02±3.59) mm],P<0.05. The distal femur aspect ratio for male (1.2 %±0.1%) and female (1.2%±0.1%) has no statistically difference (P>0.05). Femoral prosthesis sizes, the uncoverage diameter of the anterior chamfer line, midpoint of the flange in the sizer group were significantly larger than the lateral condyle group (P<0.05). The femoral size, overhang ratio of anterior chamfer line and midpoint of the flange in male were significantly larger than females (P<0.05).

The AP diameter determined by femoral sizer instrument had a tendency to larger prosthesis, and the risks of medial femoral anterior flange overhang increased. Medial anterior flange overhang in male was significantly less than female, but the difference had no correlation to the distal femur asepect ratio.

To evaluate right atrial function in systemic lupus erythematosus (SLE) with normal pulmonary pressure by using two dimensional-speckle tracking imaging (2D-STI) and real-time three-dimensional echocardiography(RT-3DE).

A totoal of 40 patients with SLE and 40 control subjects were collected between December 2011 and May 2014 from Ningbo Medical Treatment Center Lihuili Hospital. Right atrial global longitudinal strain (RAGLS) and all kinds of right atrium volume index (RAVI) were detected. Relationship between tricuspid annular plane systolic excursion(TAPSE), tricuspid annulus systolic peak velocity (S′), right ventricular myocardial performance index (RVMPI), E_TV/e′_TV and the right atrium parameters were analyzed.

SLE group′s right atrium maximal volume index(RAVImax)(33.5±11.1) ml/m^2, right atrium minimal volume index (RAVImin)(13.2±4.5) ml/m^2, right atrium presystolic volume index (RAVIpre) (2.57±10.2) ml/m^2, right atrium total emptying volume index(RAVIt)(20.2±8.1)ml/m^2, right atrium active emptying volume index (RAVIa)(12.5±7.5) ml/m²) were increased .But right atrium passive emptying volume index(RAVIp)( 7.8±2.7) ml/m² and right atrial global longitudinal strain (RAGLS) (38.2%±7.7% ) were decreased (both P<0.05). RAGLS was positive correlated with S′ and TAPSE, ther value were 0.565 and 0.445 respectively (both P<0.01). E_TV/e′_TV was positive correlated with RAVIpre, RAVIa, the r value were 0.469 and 0.481 respectively (both P<0.05).

Detecting right atrial function by 2D-STI and RT-3DE could diagnose right atrial dysfunction in early stage of SLE patients with normal pulmonary pressure. The method is simple, has significant clinical value.

To investigate the relationship of single nucleotide polymorphisms of Multi-drug resistance (ABCB1/MDR1) gene with Aspirin resistance (AR) in patients with ischemic stroke.

Three hundred ischemic stroke patients from Department of Neurology, Affiliated Hospital of Qingdao University were collected from September 2014 to May 2016. According to the results of Thromboelastogram (TEG), the patients were divided into aspirin resistance (AR) group and aspirin sensitive (AS) group. The genotypes of ABCB1 gene (C1236T, G2677T/A, C3435T) were detected by polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP) and direct sequencing.

Linkage disequilibrium was found among the three loci (D′=0.8; r²=0.9). The T allele frequency of C3435T in AR group and AS group was 57.3% and 43.1% respectively, and the difference was statistically significant (χ²=9.143, P=0.002). The frequency of genotypes CT+ TT in AR group was higher than AS group, and the difference was also significant (χ²=4.369, P=0.037). But there was no difference in allele frequency and genotype distribution of C1236T and G2677T/A between the two groups.Haplotypes constituted by the three loci were analyzed, and the OR value of haplotype (C-T-T) was 1.602 (χ²=5.374, P=0.02), the OR value of haplotype (C-G-C) was 0.495 (χ²=8.775, P=0.003), the OR value of haplotype (T-G-C) was 3.010 (χ²=5.846, P=0.015), while the OR value of haplotype (T-T-T) was 3.308 (χ²=4.650, P=0.031).

The polymorphisms of C3435T in the ABCB1 gene have a relationship with AR in the patients with ischemic stroke. The haplotypes (C-T-T, T-G-C, T-T-T) are also related to the AR in the patients with ischemic stroke.

To explore the clinical characteristics of thyrotropin-secreting pituitary adenomas and the effect of the transsphenoidal approach of procedure with/without drug treatment for thyrotropin-secreting pituitary adenomas, and to investigate the correlation between the prognosis of thyrotropin-secreting pituitary adenomas and the postoperative level of thyroid stimulating hormone (TSH).

A total of 45 patients with thyrotropin-secreting pituitary adenoma who hospitalized in Peking Union Medical College Hospital from 2000 to 2015 were enrolled in the study.We collected the clinical features, hormone levels, imaging findings, treatment and follow-up data of these patients.

The average age of the patients was 40.26 years old and the average disease duration was 5.83 years.Among them, 8 cases were microadenomas and 37 cases were macroadenoma.Thirty patients were treated with somatostatin analogues before surgery.TSH level was significantly decreased after operation (P=0.012). All of them had done the pituitary surgery, including 44 transsphenoidal surgery and 1 subfrontal surgery that had recurrence one year after the transsphenoidal surgery.The TSH level of 34 cases went back to normal on the third day after surgery.The remission rates on three months and one year after surgery in patients with TSH level<0.1 mIU/L on postoperative day (POD) 3 were significantly higher than those in patients with TSH level>0.1 mIU/L on POD3.As well as the remission rates on three months and one year after surgery in patients with TSH level normal on postoperative one month were significantly higher than those in patients with TSH level abnormal on postoperative one month.

TSH level of postoperative one month could be used to predict prognosis and monitor recurrence.

To make a systematic assessment of the complications of open reduction and internal fixation versus external fixation for unstable distal radius fractures.

A computer-based online search of PubMed, ScienceDirect, EMBASE, BIOSIS, Springer and Cochrane Library were performed.The randomized and controlled trials of open reduction and internal fixation versus external fixation for unstable distal radius fractures were collected.The included trials were screened out strictly based on the criterion of inclusion and exclusion.The quality of included trials was evaluated.RevMan 5.0 was used for data analysis.

A total of 17 studies involving 1 402 patients were included.There were 687 patients with open reduction and internal fixation and 715 with external fixation.The results of Meta-analysis indicated that there were statistically significant differences with regard to the postoperatively total complications, infection, malunion, tendon rupture (I²=8%, RR=0.77(95%CI 0.65-0.91, Z=3.10, P<0.05). There were no statistically significant differences observed between two approaches with respect to nounion, re-operation, complex regional pain syndrome, carpal tunnel syndrome, neurapraxia, tendonitis, painful hardware, scar(P>0.05).

Postoperative complications are present in both open reduction and internal fixation and external fixation.Compared with external fixation, open reduction and internal fixation is lower in total complications postoperatively, infection and malunion, but external fixation has lower tendon rupture incidence.

In 1954, Whipple first classified prehepatic portal hypertension (PPH) into intrahepatic and prehepatic^[1]。 At present, surgery is the main treatment method. Large-scale clinical studies have shown that the long-term effect of various shunt surgery is stronger than that of simple devascularization surgery, and the selection of specific shunt surgery is based on the etiology and particularity of the patient's condition, which has strong flexibility. This study summarized the experience of 30 cases of prehepatic portal hypertension, hoping to provide a reference for the choice of surgical treatment for prehepatic portal hypertension.

Male, 16 years old, was seen in the Department of Neurology, Daping Hospital, Third Military Medical University in February 2014 due to episodic blurred vision for 1 week. There was a history of upper respiratory tract infection 2 weeks before the illness, accompanied by cough and expectoration. During the attack, only blurred vision appeared, which lasted for more than ten seconds and returned to normal. The condition was repeatedly unrelieved. Ocular examination: visual acuity in the naked eye was 1.0 on the right and 1.2 on the left, normal eye movement in both eyes, transparent cornea in both eyes, anterior chamber (–), and no adhesion in the iris. Visual evoked potentials suggest normal optic nerve conduction in both eyes. MRI of the head showed abnormal signal shadow in the corpus callosum (FIG. 1A，FIG. 1B)。 Electroencephalogram examination showed no abnormalities, and "demyelinating encephalopathy" was considered in the outpatient clinic. After oral treatment with idebenone, the above symptoms disappeared. It relapsed again in December 2015, and the episode process was the same as before. Physical examination: normal vital signs, clear speech, naked eye vision examination: right 1.0, left 1.2, eye movement and fundus examination of both eyes were normal, no visual field defects, and no abnormalities in other physical examinations. MRI of the head showed that the signal change in the part of the corpus callosum still existed (Figure 1C，Figure 1D), there was no abnormality in EEG, except for the high percentage of lymphocytes (49.8%) indicated by blood routine, thyroid and abdominal ultrasound, bilateral visual evoked potentials, and other biochemical indexes were not abnormal. After admission, the symptoms began to resolve after treatment with idebenone, ribavirin and vitamin B1 for 3 days. On January 25, 2016, a reexamination of the head MRI showed that the abnormal signal of the corpus callosum pressure region completely disappeared (Figure 1E，Figure 1F)。

A 55-year-old female was admitted to Beijing Chaoyang Hospital affiliated to Capital Medical University on August 5, 2015 due to "numbness and weakness of right lower limb for 5 days". Five days before admission, there was numbness in the right toe and paroxysmal pain in the back without obvious trigger, which later progressed to numbness and weakness in the whole right lower limb; Numbness and weakness of the left lower limb occurred 1 day before admission, and the enhanced MRI of the thoracic spine showed that "the abnormal signal of spinal gray matter in the thoracic 3-8 segments considered inflammatory changes" and was admitted to the neurology department. Previous Sjogren's syndrome for 2 years, taking cyclosporine 25 mg twice/d and methotrexate 2.5 mg once/week; IgA nephropathy 14 years; Immune-associated thrombocytopenic purpura for 5 years. Physical examination: clear mind, fluent speech, roughly normal cranial nerves. The muscle strength of both upper limbs was grade V, and the lower limbs were grade V-. The muscle tone was not high, the tendon reflex was symmetrical, and the finger-nose, heel-knee-tibia tests were stable and accurate. The acupuncture sensation decreased below the right chest 4. Bilateral pathological signs were negative. Soft neck, no resistance. Medical examination showed no obvious abnormalities in heart, lung and abdomen, and no enlargement of lymph nodes in the whole body.

Intracranial atherosclerosis is the leading cause of ischemic stroke in China. Thickening and strengthening of the vessel wall and the presence of unstable plaques are often overlooked by traditional imaging methods without vascular stenosis, but these features of the vessel wall are important for clarifying the mechanism of ischemic stroke. In recent years, high-resolution magnetic resonance imaging (HR MRI) has brought hope to non-invasive display of intracranial artery wall and plaque characteristics, and is considered to be the only method to study intracranial artery wall imaging in vivo.

Immunoglobulin-G4-related hypertrophic pachymeningitis (IgG4-RHP) is a manifestation of IgG4-related disease (IgG4-RD) involving the central nervous system discovered in recent years. In 2009, Chan et al. of Hong Kong Kwong Wah Hospital^[1]First reported. This disease is characterized by elevated serum IgG4 levels, headache, cranial (spinal) nerve paralysis, epileptic seizures and other symptoms, and local or diffuse dura (spinal) thickening can be seen on imaging. According to literature search, there are more than 50 cases reported abroad, but rare in China. The clinical, imaging and pathological characteristics of IgG4-rHP are reviewed systematically, aiming to improve the awareness and attention of relevant doctors and avoid misdiagnosis or misdiagnosis.

According to the different binding sites between histones and chromatin, they can be divided into core histones (H2A, H2B, H3, H4) and linked histones (H1); According to different components, it can be divided into polylysine group (H1, H2A, H2B) and polyarginine group (H3, H4); According to the different positions of histones, they can be divided into extracellular histones, cytoplasmic histones and intranuclear histones. Intranuclear histones bind and regulate DNA transcription; Cytoplasmic histones are involved in regulating immune and inflammatory responses; Extracellular histones mediate the damage of lung, liver and other tissues during inflammation, and participate in a series of physiological and pathological processes such as sepsis, thrombocytopenia and thrombin production. Current studies have shown that extracellular histones mainly come from two pathways: (1) dead parenchymal tissue cells release intranuclear histones extracellularly; (2) Activated neutrophils are actively released. During the activation of neutrophils, nuclear substances are decomposed, and the rupture of organelle membrane and nuclear membrane in cytoplasm leads to the release of extracellular DNA (cfDNA) -histone complex and granular cytoplasmic proteases such as myeloperoxidase and elastase. These substances together form network-like structures-neutrophil extracellular traps (NETs), which participate in the anti-infective response of the body after the invasion of pathogenic microorganisms, and stimulate inflammation and thrombosis through toll-like receptor (TLR) or inflammatory pathway.