Volume 131 · Issue 15 · 2018

噬血细胞性淋巴组织细胞增多症（HLH）是一种严重危及生命的临床综合征，伴有过度炎症，而不是一种孤立的疾病。分为原发性HLH和继发性HLH。原发性HLH与HLH相关基因突变有关，而继发性HLH发生在严重感染、肿瘤、结缔组织疾病等后。^[1]无论原发性或继发性HLH，30-73%的患者有中枢神经系统（CNS）受累。^[2]在中国成人HLH患者中，38.4%的患者在发病时有神经系统症状，在整个病程中有神经系统症状的高达89.6%。^[3]然而，还没有关于HLH儿童中枢神经系统受累的明确报道。

Prospective real-life data on the safety and effectiveness of rituximab in Chinese patients with diffuse large B-cell lymphoma (DLBCL) or follicular lymphoma (FL) are limited. This real-world study aimed to evaluate long-term safety and effectiveness outcomes of rituximab plus chemotherapy (R-chemo) as first-line treatment in Chinese patients with DLBCL or FL. Hepatitis B virus (HBV) reactivation management was also investigated.

A prospective, multicenter, single-arm, noninterventional study of previously untreated CD20-positive DLBCL or FL patients receiving first-line R-chemo treatment at 24 centers in China was conducted between January 17, 2011 and October 31, 2016. Enrolled patients underwent safety and effectiveness assessments after the last rituximab dose and were followed up for 3 years. Effectiveness endpoints included progression-free survival (PFS) and overall survival (OS). Safety endpoints were adverse events (AEs), serious AEs, drug-related AEs, and AEs of special interest. We also reported data on the incidence of HBV reactivation.

In total, 283 previously untreated CD20-positive DLBCL and 31 FL patients from 24 centers were enrolled. Three-year PFS was 59% (95% confidence interval [CI]: 50-67%) for DLBCL patients and 46% (95% CI: 20- 69%) for FL patients. For DLBCL patients, multivariate analyses showed that PFS was not associated with international prognostic index, tumor maximum diameter, HBV infection status, or number of rituximab treatment cycles, and OS was only associated with age >60 years (P < 0.05). R-chemo was well tolerated. The incidence of HBV reactivation in hepatitis B surface antigen (HBsAg)-positive and HBsAg-negative/hepatitis B core antibody-positive patients was 13% (3/24) and 4% (3/69), respectively.

R-chemo is effective and safe in real-world clinical practice as first-line treatment for DLBCL and FL in China, and that HBV reactivation during R-chemo is manageable with preventive measures and treatment.

ClinicalTrials.gov, NCT01340443; https://clinicaltrials.gov/ct2/show/NCT01340443.

After the first examination of patients with lymphoma diagnosis, important laboratory tests such as complete blood count; albumin, kidney and liver function tests; uric acid; β2-microglobulin; C-reactive protein (CRP); erythrocyte sedimentation rate (ESR); and lactate dehydrogenase (LDH) examinations are recommended. In this study, our aim was to find the relationship between laboratory parameters and the maximum standard uptake value (SUV_max) of positron emission tomography/computed tomography (PET/CT) in patients with lymphoma at the diagnosis and after treatment.

Thirty-four lymphoma patients treated at Mustafa Kemal University Internal Medicine Clinic between 2014 and 2017 were included in this retrospective study. Results of CRP, ESR, LDH, albumin, and white blood cell (WBC) count were recorded before each PET scan test, and each parameter was analyzed for correlation with SUV_max measurements.

Spearman’s correlation test showed that the after-treatment SUV_max values were significantly correlated with the after-treatment LDH, ESR, and CRP values (for LDH, ESR, and CRP, R²: 0.453, 0.426, and 0.351; P= 0.007, 0.012, and 0.042, respectively). On the other hand, albumin and WBC count did not show a significant correlation with the after-treatment SUV_max values (all P> 0.05).

CRP, ESR, and LDH values may also be good predictors in patients for whom PET/CT imaging cannot be performed.

Although much attention has been paid to the pharmacokinetics (PKs) of different factor VIII (FVIII) concentrates in persons with hemophilia A (HA), limited information is available in young boys with severe HA. In this study, we aimed to assess the PK parameters of FVIII products in boys with severe HA in China.

A total of 36 boys (plasma-derived [pd]-FVIII, n = 15; recombinant [r] FVIII, n = 21) were enrolled between January 2015 and May 2016 in Beijing Children’s Hospital. PK characteristics of FVIII products were studied according to a reduced 4-sampling time point design (1 h, 9 h, 24 h, and 48 h postinfusion).

The mean FVIII half-life (t_1/2) was 10.99 ± 3 .45 h (range 5.52- 20.02 h), the mean in vivo recovery (IVR) was 2.01 ± 0.42 IU/dl per IU/kg (range 1.24–3.02 IU/dl per IU/kg) and mean clearance (CL) of FVIII is 4.34 ± 1.58 ml·kg⁻¹·h⁻¹ (range 2.29–7.90 ml·kg⁻¹·h⁻¹). We also analyzed the influence of several parameters that potentially modulate FVIII PK. The age was closely associated with FVIII half-life (R² = 0.32, P< 0.01). The t_1/2 of FVIII increased by 0.59 h per year. Besides age, von Willebrand factor antigen (VWF:Ag) also was associated with FVIII half-life (R² = 0.52, P< 0.01). Patients with blood Group O had a shorter FVIII half-life than patients with non-O blood group (9.40 ± 0.68 h vs. 12.3 ± 0.79 h, t = 2.70, P= 0.01). The FVIII IVR correlated with age (R² = 0.21, P< 0.01) and VWF:Ag level (R² = 0.28, P< 0.01). CL rates were faster in young patients and in those with low-VWF:Ag levels. CL rates of FVIII are higher in blood Group O versus non-blood Group O persons (5.02 ± 0.38 vs. 4.00 ± 0.32 ml·kg⁻¹·h⁻¹, t= 2.53, P= 0.02).

Chinese boys with severe HA have similar PK values to other ethnic groups and large differences in FVIII PK between individual patients. Age, blood group, and VWF:Ag levels are important determining factors for FVIII CL.

Central nervous system (CNS) involvement is found in many patients with hemophagocytic lymphohistiocytosis (HLH). In this study, we mainly analyzed neurological symptoms, imaging findings, cerebrospinal fluid (CSF), and their relationship with outcomes of HLH children.

Related data of 179 Chinese pediatric patients with HLH admitted to our center from January 2010 to December 2015 were analyzed retrospectively. Diagnosis and treatment were based on the HLH-2004 protocol. Two-tailed Chi-squared test was used to compare between different groups, and Kaplan-Meier survival curves were used to analyze the overall survival (OS) of patients with HLH.

In the present study, 21.2% (38/179) of total patients had neurological symptoms including seizure, irritability, somnolence, and unconsciousness. There were 80 (50.0%, excluding 19 patients without imaging data) patients with cranial imaging abnormalities. There were 14.7% (17/116, excluding 63 patients who did not accept lumbar puncture) of patients with abnormal CSF results. CNS involvement is defined as abnormalities in one or more of CNS symptoms, radiological findings, and CSF. Thus, 60.3% of them had CNS involvement. As for the prognosis, the median follow-up time was 3.2 years (17 lost to follow-up). The probable 3-year OS of children was higher without CNS involvement (86.0% ± 4.6%) than those with CNS involvement (68.9% ± 4.9%, hazard ratio [HR] = 2.286, P= 0.019). Among them, the probable 3-year OS of children without CNS symptoms was 76.0% ± 3.8%, higher than with CNS symptoms (59.5% ± 8.1 %, HR= 2.147, P= 0.047). The 3-year OS of children with abnormal CSF was 64.7% ± 11.6%, compared with normal CSF (85.1% ± 3.7%, HR= 0.255, P= 0.038).

HLH patients with CNS involvement might have worse outcomes compared with those without CNS involvement, and CNS symptoms and CSF changes are more important to access the prognosis than imaging abnormality.

Pulmonary Langerhans cell histiocytosis (PLCH) is an interstitial primary pulmonary disease, characterized by Langerhans cell proliferation. It is easily misdiagnosed in children. This study aimed to characterize the clinical manifestations and features of PLCH by retrospective analysis.

A retrospective analysis was performed in 117 PLCH patients out of 338 LCH patients who were admitted in our center from November 2006 to October 2013. Variables between two groups were compared by Mann-Whitney U-test and Chi-square test. Kaplan-Meier curves were constructed to compare the survival rates and Cox regression to evaluate the effect of risk factors.

The median age of PLCH group was significantly lower than that of non-PLCH group (18.63 months vs. 43.4 months, P< 0.001). All PLCH children had other organ involvement and only 11 cases (9.4%) had respiratory symptoms. The most common radiologic finding was cystic lesions (29 cases, 24.8%). Pulmonary function abnormalities were dominated by obstructive ventilatory dysfunction (63 cases, 82.9%). The 5-year overall survival (OS) of PLCH children was 93.6% ± 2.3% and the event-free survival (EFS) was 55.7% ± 5.2%. Among the 38 cases with progressed or relapsed disease, five cases (13.2%) were due to progression or recurrence of lung damage. The 5-year OS of PLCH children with "risk organ" involvement was significantly lower than those without "risk organ" involvement (86.0% ± 4.9% vs. 100%, χ²= 8.793, P= 0.003). The difference of EFS between two groups was also significant (43.7% ± 7.7% vs. 66.3% ± 6.5%, χ² = 5.399, P= 0.020). The "risk organ" involvement had a significant impact on survival (hazard ratio = 1.9, P= 0.039).

PLCH mainly occurs in young children, and only a small percentage of patients have respiratory symptoms. They generally have other organ involvement. Most of PLCH children have a good prognosis and most lung lesions could have improved or stabilized. Management of "risk organ" involvement is the key point to improving EFS.

It is known that short sleep duration adversely affects children’s behavior and physical development. This study aimed to investigate the status of sleep duration in 3–14-year-old children in Beijing and explore the related factors of sleep loss with them.

In this study, a cross-sectional study of random stratified cluster sampling was conducted on 3–14-year-old children and adolescents in Beijing. According to the proportion of children in each district and school, the final cohort included a total of 11 kindergartens, 7 primary schools, and 8 junior high schools from 7 districts of Beijing. Children of sampled classes were included, and their parents were invited to fill a series of questionnaires including the simplified Chinese version of Pediatric Sleep Questionnaire, Sleep Questionnaire Scale, and Hong Kong-Children Sleep Questionnaire about the performance of the last 6 months.

Out of the total 11,420 questionnaires, 9198 questionnaires were valid and effective with the response rate of 80.54%. The age of the investigated children was 8.8 ± 3.8 years, including 4736 males and 4462 females. The daily sleep duration of children in Beijing was 9.7 ± 0.7 h. The prevalence of sleep loss (<9 h/day) of children in Beijing was 11.8%. The daily sleep duration of children aged <6, 6 ≤ age <11, and ≥11 years was 9.7 ± 0.6 h, 9.6 ± 0.6 h, and 9.5 ± 0.8 h, respectively. The sleep duration reduced significantly in children aged ≥11 years as compared to younger children in Beijing which was mainly contributed by the variation tendency of sleep duration on weekdays. The multivariate logistic regression analysis identified factors associated with sleep loss (P < 0.05): male (odds ratio [OR] = 1.32, 95% confidence interval [CI ]: 1.15–1.51), age ≥11 years (OR = 2.37, 95% CI: 1.92- 2.93), overweight (OR = 1.34, 95% CI: 1.17–1.54), family history of snoring (OR = 1.35, 95% CI: 1.13–1.61) and activities before bedtime with watching TV (OR = 1.24, 95% CI: 1.08–1.43), sports (OR= 1.22, 95% CI: 1.01–1.48), playing cellphone (OR = 1.91, 95% CI: 1.31- 2.73) and surfing the Internet (OR = 1.27, 95% CI: 1.06–1.52) and among them age ≥11 years and playing cellphone before bedtime had greater impact on children’s short sleep duration than that of other factors.

Sleep loss was common among 3–14-year-old children in Beijing. Sleep duration decreased with age, especially among children over 11 years old. Factors associated with sleep loss covered sociodemographic characteristics, family sleep habits and routine activities before bedtime, and among those variables, age ≥11 years and playing with cellphones before bedtime had a greater impact on sleep duration, indicating that existing sleep loss in 3–14-year-old children could be, at least partly, improved by paying more attention to children aged of 11 years or entering Grade 5 and Grade 6 and to children with a family history of snoring; by reducing the use of electronic products before bedtime, especially cellphones; by managing weight and keeping fit; and by improving the bedtime routine.

The 47,XYY syndrome could result in fertility problems. However, seldom studies reported comprehensive researches on the embryonic development and pregnancy outcomes of these patients. This study aimed to evaluate the clinical outcomes of nonmosaic 47,XYY patients performed with fluorescent in situ hybridization (FISH) and preimplantation genetic diagnosis (PGD) treatment.

This was a retrospective study. Between January 2012 and May 2017, 51 infertile males with nonmosaic 47,XYY syndrome underwent FISH-PGD were included in the study. According to sex chromosomal FISH results, embryos were classified as normal signal, no nuclei fixed, no signal in fixed nuclei, suspensive signal, and abnormal signal groups, respectively. The incidence of each group, the fixation rate, and hybridization rate were calculated. Embryonic development and pregnancy outcomes were also analyzed. The measurement data were analyzed with Student’s t-test. The comparison of categorical data was analyzed with the Chi-square test and Fisher’s exact test when expected cell count was <5.

The 53 PGD cycles with 433 embryos were analyzed. The fixation rate was 89.6%, while the hybridization rate was 96.4%. There were 283 embryos with two sex chromosomal signals with clear diagnosis (65.4%). The numbers of no nuclei fixed, no signal in fixed nuclei, suspensive signal, and abnormal signal groups were 45 (10.4%), 14 (3.2%), 24 (5.5%), and 67 (15.5%), respectively. Embryos with abnormal signals were abandoned. The number of good-quality embryos was 210 (57.4%), including implanted embryos on day 4/day 5 and cryopreserved. The rates of good-quality embryos in the no nuclei fixed (22.2%), no signal in fixed nuclei (28.6%), and suspensive signal groups (33.3%) were comparable (P > 0.05), and were significantly lower than the normal signal group (66.4%, P< 0.001). The clinical pregnancy rates of fresh and frozen embryos transferred cycles were 70.6% and 85.7%, respectively.

Among embryos with a clear diagnosis of sex chromosome, about one-fifth showed abnormal signals. Embryos with two sex chromosomal signals are more likely to develop into good-quality ones. The application of the PGD by FISH may help to improve the clinical outcomes.

Cytokines play an important role in occurrence and recovery of hepatitis B virus (HBV) infection. The aim of this study was to investigate the changes of cytokines concentration and its correlation to alanine aminotransferase (ALT), HBV deoxyribonucleic acid (HBV-DNA), hepatitis B envelope antigen (HBeAg), and HBV surface antigen (HBsAg) in the development of chronic hepatitis B (CHB).

Thirteen healthy individuals (HI), 30 chronic HBV-infected patients in immune tolerant (IT) phase, and 55 CHB patients were enrolled between August 2015 and May 2017. The peripheral blood samples were collected from all individuals. The levels of interferon (IFN)-α2, interleukin (IL)-10, transforming growth factor (TGF)-β1, HBV-DNA, HBsAg, and HBeAg and liver function were measured. The quantitative determinations of cytokines levels, including IFN-α2, IL-10, and TGF-β1 were performed using Luminex multiplex technology. The correlation of cytokines to ALT, HBV-DNA, HBsAg, and HBeAg was analyzed by linear regression analysis.

IFN-α2 levels were similar between HI and IT groups (15.35 [5.70, 67.65] pg/ml vs. 15.24 [4.07, 30.73] pg/ml, Z= −0.610, P= 0.542), while it elevated significantly in CHB group (35.29 [15.94, 70.15] pg/ml vs. 15.24 [4.07, 30.73] pg/ml; Z= −2.522, P= 0.012). Compared with HI group (3.73 [2.98, 11.92] pg/ml), IL-10 concentrations in IT group (5.02 [2.98, 10.11] pg/ml), and CHB group (7.48 [3.10, 18.00] pg/ml) slightly increased (χ² = 2.015, P= 0.365), and there was no significant difference between IT and CHB group (Z= −1.419, P= 0.156). The TGF-β1 levels among HI (3.59 ± 0.20 pg/ml), IT (3.62 ± 0.55 pg/ml), and CHB groups (3.64 ± 0.30 pg/ml) were similar (χ² = 2.739, P= 0.254). In all chronic HBV-infected patients (including patients in IT and CHB groups), the elevation of IFN-α2 level was significantly associated with ALT level (β = 0.389, t = 2.423, P= 0.018), and was also negatively correlated to HBV-DNA load (β= −0.358, t= −2.308, P= 0.024), HBsAg (β= −0.359, t= −2.288, P= 0.025), and HBeAg contents (β= −0.355, t= −2.258, P= 0.027). However, when both ALT level and cytokines were included as independent variable, HBV-DNA load, HBsAg, and HBeAg contents were only correlated to ALT level (β= −0.459, t= −4.225, P= 0.000; β= −0.616, t= −6.334, P= 0.000; and β= −0.290, t= −2.433, P= 0.018; respectively).

IFN-α2 elevation was associated with ALT level in patients with chronic HBV infection. However, in CHB patients, only ALT level was correlated to HBV-DNA, HBsAg and HBeAg contents.

Currently, spectral-domain optical coherence tomography (SD-OCT) appears to be a new type of glaucoma diagnostic tool. Thus, this study aimed to evaluate the effectiveness of glaucoma diagnostic parameters from SD-OCT of patients with different severities of myopia.

This was a cross-sectional study. A total of 248 participants (248 eyes) were enrolled, including 51 cases in the early primary open-angle glaucoma group, 79 cases in the control group (0.50 D to −0.50 D, excluding −0.50 D), 47 cases in the low-myopic group (−0.50 to −3.00 D, excluding −3.00 D), 43 cases in the moderate-myopic group (−3.00 to −6.00 D, excluding −6.00 D), and 28 cases in the high-myopic group (≤−6.00 D). All participants were examined using the Humphrey visual field test and SD-OCT. The SD-OCT parameters of the retinal nerve fiber layer (RNFL) and ganglion cell complex were analyzed statistically using the receiver operating characteristic curve and area under the curve (AUC).

The AUC showed that the best parameters for the control and low-myopic groups were the inferior and inferior temporal RNFL thicknesses (AUC >0.94), respectively; for the moderate- and high-myopic groups, the best parameter was the temporal low RNFL thickness (AUC, 0.926 and 0.896, respectively). The AUC of the inferior parameters of the moderate-myopic group (0.864) was lower, ranked 15^th among all RNFL parameters. When the sensitivity was fixed at 85%, the specificity of the inferior, superior, inferior temporal, and superior temporal quadrants was higher (>80%) in the control and low-myopic groups, while they were lower (20–60%) for the moderate- and high-myopia groups. The green color based on the OCT database was also less for the high-myopic group compared with that of other groups (P < 0.05).

Glaucoma diagnostic parameters from SD-OCT were not clinically effective for the moderate- and high-myopic groups. The specificities were low. The moderate- and high-myopic groups require comprehensive analyses for the diagnoses of glaucoma. The SD-OCT database should be improved to better indicate the level of myopia based on the corresponding diopter readings.

Technical aspects of the correct placement of medial support locking screws in the locking plate for proximal humerus fractures remain incompletely understood. This study was to evaluate the clinical relationship between the number of medial support screws and the maintenance of fracture reduction after locked plating of proximal humerus fractures.

We retrospectively evaluated 181 patients who had been surgically treated for proximal humeral fractures (PHFs) with a locking plate between September 2007 and June 2013. All cases were then subdivided into one of four groups as follows: 75 patients in the medial cortical support (MCS) group, 26 patients in the medial multiscrew support (MMSS) group, 29 patients in the medial single screw support (MSSS) group, and 51 patients in the no medial support (NMS) group. Clinical and radiographic evaluations included the Constant-Murley score (CM), visual analogue scale (VAS), complications, and revision surgeries. The neck-shaft angle (NSA) was measured in a true anteroposterior radiograph immediately postoperation and at final follow-up. One-way analysis of variance or Kruskal-Wallis test was used for statistical analysis of measurement data, and Chi-square test or Fisher’s exact test was used for categorical data.

The mean postoperative NSAs were 133.46°± 6.01°, 132.39°± 7.77°, 135.17°± 10.15°, and 132.41°± 7.16° in the MCS, MMSS, MSSS, and NMS groups, respectively, and no significant differences were found (F = 1.02, P= 0.387). In the final follow-up, the NSAs were 132.79°± 6.02°, 130.19°± 9.25°, 131.28°± 12.85°, and 127.35°± 8.50° in the MCS, MMSS, MSSS, and NMS groups, respectively (F = 4.40, P= 0.008). There were marked differences in the NSA at the final follow-up between the MCS and NMS groups (P = 0.004). The median (interquartile range [IQR]) NSA losses were 0.0° (0.0–1.0)°, 1.3° (0.0–3.1)°, 1.5° (1.0–5.2)°, and 4.0°(1.2–7.1)° in the MCS, MMSS, MSSS, and NMS groups, respectively (H = 60.66, P< 0.001). There were marked differences in NSA loss between the MCS and the other three groups (MCS vs. MMSS, Z = 3.16, P= 0.002; MCS vs. MSSS, Z = 4.78, P< 0.001; and MCS vs. NMS, Z = 7.34, P< 0.001). There was also significantly less NSA loss observed in the MMSS group compared to the NMS group (Z= −3.16, P= 0.002). However, there were no significant differences between the MMSS and MSSS groups (Z= −1.65, P= 0.225) or the MSSS and NMS groups (Z= −1.21, P= 0.099). The average CM scores were 81.35 ± 9.79, 78.04 ± 8.97, 72.76 ± 10.98, and 67.33 ± 12.31 points in the MCS, MMSS, MSSS, and NMS groups, respectively (F = 18.68, P< 0.001). The rates of excellent and good CM scores were 86.67%, 80.77%, 65.52%, and 43.14% in the MCS, MMSS, MSSS, and NMS groups, respectively (χ² = 29.25, P< 0.001). The median (IQR) VAS scores were 1 (0- 2), 1 (0- 2), 2 (1–3), and 3 (1–5) points in the MCS, MMSS, MSSS, and NMS groups, respectively (H = 27.80, P< 0.001). Functional recovery was markedly better and VAS values were lower in the MCS and MMSS groups (for CM scores: MCS vs. MSSS, P< 0.001; MCS vs. NMS, P< 0.001; MMSS vs. MSSS, P= 0.031; and MMSS vs. NMS, P< 0.001 and for VAS values: MCS vs. MSSS, Z = 3.31, P= 0.001; MCS vs. NMS, Z = 4.64, P< 0.001; MMSS vs. MSSS, Z= −2.09, P= 0.037; and MMSS vs. NMS, Z= −3.16, P= 0.003).

Medial support screws might help enhance mechanical stability and maintain fracture reduction when used to treat PHFs with medial metaphyseal comminution or malreduction.

Estrogen is one of the most important reproductive steroidal hormones and plays a critical role in the maintenance of pregnancy, and its function is mediated by estrogen receptor 1(ESR1 ). The polymorphisms of ESR1 were involved in recurrent spontaneous abortion (RSA); however, the association between ESR1 polymorphisms and RSA remains controversial. The present meta-analysis was aimed to clarify the association between ESR1 PvuII (-397C/T, rs2234693) and XbaI (-351A/G, rs9340799) polymorphisms and the risk of RSA.

All the included articles were retrieved from PubMed, Embase, Cochrane Library, China National Knowledge Infrastructure, and Wanfang Med Online Database up to January 3, 2018. Data were processed in the Stata 12.0 software. The odds ratios (ORs) and 95% confidence intervals (95% CIs) were calculated using fixed-effects models (FEM)/random-effects models (REM).

Seven case- control studies with 836 cases and 1164 controls were included in the study. Generally, the ESR1 polymorphisms were not associated with RSA in any of the genetic analysis models. However, it was found that as rs9340799 polymorphism was related to increased risk of RSA in non-Asian group in the homozygous genetic model (OR = 2.40, 95% CI = 1.05–5.50, P= 0.039). Moreover, in Asian group, rs9340799 polymorphism was found to be related to decreased RSA risk in both the heterozygous model (OR = 0.53, 95% CI = 0.33- 0.85, P= 0.009) and the dominant genetic model (OR = 0.55, 95% CI = 0.30- 0.98, P= 0.042).

Generally, there was no significant association between the polymorphisms of ESR1 and the risk of RSA. However, subgroup analysis indicated that ESR1 rs9340799 polymorphism was related to increased RSA risk in the non-Asian group while associated with decreased RSA risk in Asian group.

编辑：浆细胞样树突状细胞（pDC）代表树突状细胞的一个子集。有两种不同形式的源自pDC的肿瘤；一种是母细胞浆细胞样树突状细胞瘤（BPDCN），另一种是与髓系肿瘤共存的成熟pDCs增殖。髓系肿瘤中成熟的pDC以谱系阴性的人白细胞抗原(HLA)-DR为特征⁺CD56⁻CD123⁺CD11c⁻免疫表型。在这里，我们描述了一名83岁的男性，他患上了原发性骨髓纤维化（PMF）转移的急性髓性白血病（AML），与意义不明的单克隆丙种球蛋白病（MGUS）和成熟的pDCs增殖共存；然而，骨髓（BM）的免疫表型结果显示阳性CD56表达。

致编者：Castleman病（CD）描述了一组具有异质性临床表现的淋巴增生性疾病。^[1]CD包括至少两种不同的疾病（单中心CD[UCD]和多中心CD[MCD]），具有各自的临床特征和预后：UCD通常涉及一个淋巴结区域，结果良好；MCD是一种预后相对较差的全身性疾病，涉及多个淋巴结区域，伴有全身症状（例如发热、盗汗和虚弱）和器官功能障碍。根据发病机制，MCD可根据人类疱疹病毒-8（HHV-8）感染状况进一步分为不同的亚型。在HHV-8阴性MCD（也称为特发性MCD[iMCD]）中，有一种称为TAFRO综合征的特殊亚型，其构成了一系列独特的临床表现（T：血小板减少症，A：Anasarca，F：骨髓纤维化，R：肾功能障碍和O：器官肿大）。这种综合征首先在日本报道，^[2]据报道，主要在日本人群中，偶尔在高加索人中。国内尚无此类病例报道。我们在此报告了中国首例确诊为骨髓纤维化的TAFRO综合征患者。

致编辑：过度减肥通常会导致明显的腹部轮廓畸形。腹部成形术是腹直肌壁松弛、皮肤过多和分离的最佳解决方案。^[1]反向腹部成形术是一种局部手术，在纠正上腹部皮肤松弛方面具有不可替代的作用，通常在先前的吸脂术或常规腹部成形术后进行。^[2]在皮肤明显松弛的情况下，上腹壁的残余轮廓畸形也仍然存在。

编者按：根据中国国家统计局1999年10月的数据，60岁以上的人口已达到总人口的10%，表明中国正在进入老龄化社会。截至2017年末，全国60岁以上人口24090万人，占总人口的17.3%。随着人口的迅速老龄化，老年患者胆道疾病在我国已变得多发，发病率高达8-11%。老年胆道疾病由于应激反应、防御能力、免疫力降低，表现为并存疾病增多、进展快、手术耐受性差、手术风险高、术后并发症频发、病死率高。因此，探索老年胆道疾病患者的有效治疗方法具有重要意义。根据我们治疗老年胆道疾病患者的临床经验，^[1]结合以往的研究，本报告介绍了中国老年胆道疾病患者外科治疗的现状。^[2]

致编者：经皮肾镜取石术（PCNL）是一种常用于治疗大肾结石的外科手术。术后通常伴有剧烈疼痛和不适。静脉注射阿片类药物和局部麻醉剂浸润已被常规用于控制术后疼痛。此外，已经尝试了区域阻滞，例如椎旁阻滞和肋间阻滞。然而，这些阻滞的镇痛功效尚未确定。^[1]据报道，竖脊肌平面阻滞（ESPB）可在胸腹部手术后提供有效的镇痛。^[2,3]我们描述了一例在PCNL后使用持续ESPB提供高效镇痛的患者。本研究获得我院机构审查委员会的批准。一名67岁男性（体重61 kg，身高171 cm）因右肾结石接受PCNL治疗。他已经控制了高血压和糖尿病，他的实验室检查值并不显著。PCNL在全身麻醉下以侧卧位进行。

致编者：假性醛固酮增多症II型（PHAII），又称戈登综合征，是一种罕见的常染色体疾病，由WNK1,WNK4,CUL3，或KLHL3基因。迄今为止，约有200个个人和家庭被报道患有PHAII；其中26例是由CUL3突变。^[1,2]在儿科，高钾血症并发高血压很少见，这种情况应考虑PHAII。

致编者：一名43岁男性，6年前被诊断为扩张型心肌病，最近因胸部充血恶化伴呼吸急促和腹胀而来我院就诊。患者有糖尿病病史。心电图（ECG）显示窦性心律伴心室内传导障碍，非典型左束支传导阻滞，QRS间期160 ms。超声心动图表现符合扩张型心肌病[双中心型，图1a]，其中左心房直径为44mm，左心室舒张末期直径为78mm，左心室射血分数为30%。最终诊断为纽约心脏协会III级扩张型心肌病。为了获得良好的预后、提高运动耐量和预防猝死，建议进行心脏再同步治疗（CRT）和除颤器植入。然而，在植入手术期间观察到冠状窦（CS）异常。因此，手术被迫停止，并安排了异常的心脏计算机断层血管造影（CTA）评估。

致编者：尽管对前交叉韧带（ACL）进行了大量的形态学和生物力学研究，但这种解剖结构仍在研究中；特别是，讨论了其与生物力学相关的解剖组成。目前，通常假设ACL包括两个束的结构：前内侧和后外侧。有时，甚至区分三束；然而，ACL是一个整体和连续结构的假设在文献中不断被提及。众所周知，ACL不是等长的，ACL纤维的长度随着膝关节屈伸而变化：在屈曲时，前内侧束的长度增加（收紧），而后外侧束的长度减少（松弛）。同时，在膝关节屈伸过程中，ACL的股骨部分旋转，而其胫骨部分微小移动。^[1]可以认为，嵌入ACL远端的异物迁移能力较低。

编辑：羟氯喹（HCQ）常用于系统性红斑狼疮（SLE）、类风湿性关节炎和其他自身免疫性疾病。我们报告了一例由HCQ诱导的SLE患者银屑病样红皮病。因此，风湿病学家和皮肤科医生应该意识到HCQ诱导的银屑病样病变的可能性。

在题为“蒲公英提取物对卵巢颗粒细胞增殖和激素受体表达的影响”的文章中，发表在第1694-1701页，第14期，第131卷中华医学杂志,^[1]财务披露部分不见了。应写成如下：